Blogging About Cystic Fibrosis (CF)

Exercise and Cystic Fibrosis

2008-09-28T14:06:00.000-07:00

The Benefits of Exercising when You Have Cystic Fibrosis
The risk of dehydration in patients with cystic fibrosis is a legitimate concern, however, it is not reason enough to exclude people with cystic fibrosis from participating in as many normal activities as possible.
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CF Goes Back to School

2008-08-25T11:12:00.000-07:00

Sharpened pencils, new backpacks and notebooks; ‘Tis the season for heading back to school. Sending a child off to school for the first time can be an emotionally charged event. The typical anxiety level may be ratcheted up a notch for parents of children with cystic fibrosis. This does not need to be the case. With a little bit of help and a few simple tips, you can send your child to school confident that his or her CF needs will be met.

In August, 2007, we surveyed our members in a CF Parents Survey. Throughout this article you’ll see quotes by members of CysticFibrosis.com regarding their expectations, excitement, and apprehensions about sending their children off to school.

Larasmommy: "Mostly I’m worried about the germs and how sick she gets when she goes there."

Bbedrock: "Starting a school year is always exciting. My son loves to experience and learn new things and I hope that continues as he starts a new school year. The biggest fear is that he will contract something at school; he's entering third grade; it's never happened, but the fear is still there."

First Timers

Parents of younger children who are starting school for the first time or who are transferring to a different school, often worry about how much information to give their child’s teacher. The Cystic Fibrosis Research Inc. (CFRI) has an excellent publication, "CF in the Classroom". This booklet outlines the basics of CF and offers guidelines for ensuring you make the most of your child’s school experience. In order to make sure the teacher understands the material you are presenting, be sure to give it to them in person. Encourage your child’s teacher to ask questions about CF and provide an opportunity to discuss it further. The CFRI has done a wonderful job of gathering the most relevant information for parents and teachers, and combined them in this informational booklet. If your child’s school has a nurse or counselor on staff, make the information available to them as well.

TonyaH: "I always meet with his teacher before the start of school to discuss the disease, supply literature, go over his meds, etc. I also make a little trip to his PE teacher to ask him/her to be aware."

Whomever you disclose your child's health information to, remind them that because this information is health-related, it is considered private. It is inappropriate for them to share or disclose the details of your child’s condition with others, including the rest of the class. As your child progresses in school, he or she may want to let others know about cystic fibrosis. Determining how and when to do that is a matter of personal preference.

Simple Answers

The day will come when a student with CF will have to explain why he or she takes those pills at lunch time, why he or she is so skinny, or why that pesky cough never goes away.

David: "I have always told him to be honest and tell them he has cystic fibrosis and that they can’t catch it."

Depending on their age and relationship to the student, some people will ask nosey questions, and others will be genuinely interested in understanding. Many people have found the direct, non-specific or detailed approach to be the best.

Michelle43: "He made this up himself, I have CF it makes me cough and is like asthma, I have to take pills and do treatments, but I'm very lucky and healthy because my mommy keeps good care of me and I've only been to the hospital once; most CF kids go more often."

Below are some of the experiences that parents of CFers and their students have offered to explain CF to teachers and classmates.

Bbedrock: "My son's first grade year wasn't too good. The teacher admitted she felt sorry for him - no reason to; he appears and acts healthier than non-CF kids. The day before second grade started we met with everyone who was going to have anything to do with him - from the lunch aid to the phys ed teacher to the principal and went through everything CF related and the repercussions to him, emphasizing our desire to have him treated like everyone."

Consmom: "[We stay involved by] keeping in contact with the teacher, nurse and catering manager. We have an education assembly in September so he gets to show off his vest, etc. and we can explain CF to everyone."

Michelle43: "I send a "CF in the Classroom" booklet to all of his new teachers. I highlight all of the things that have to do with my son and staple a picture of Collin and phone number list where to reach me or Collin’s dr. and an invite to feel free to ask me anything and to call if he's not acting well."

Preventing Infection

Avoiding every germ is an impossible task. Getting sick is inevitable. Prevention is key, and seeking treatment at the first signs of infection is a must. Some of the basic ways to prevent illness include:

Vaccinations
Flu (yearly)
Pneumonia (every 5 years)
Hand washing

One of the most important things a child with CF can do to stay healthy is to minimize exposure to germs and harmful bacteria. Frequent hand hygiene, which includes washing one’s hands with soap and water, and/or using alcohol-based hand gel, is best. A good rule of thumb is if there is visible dirt on a child’s hands or the child has just used the restroom, he or she should use soap and water. After sneezing and coughing, an alcohol based gel or antibacterial wipe is sufficient.

TheDickens: "Make sure your child keeps his/her hands clean and try to keep a safe distance from children with colds and other illnesses."

Making the Grade: Keeping Up Despite Absence

Frequent absences from school are sometimes part of life with CF and can make both students and parents anxious. It is important for students to keep up with their peers in terms of coursework to the greatest extent possible. This is where an Individualized Education Plan (IEP) and the Americans with Disabilities Education Act (IDEA) serve a purpose. Another law that protects students with health impairments is Section 504 of the Rehabilitation Act of 1973, which offers modifications for students who need it.

Jane: "My kids both have a 504 plan to help with accommodations. I have found most teachers and school staff to be very helpful. I teach in the same school as my children, so I am on top of things."

It is best to have an IEP or 504 plan in place before the student becomes sick. This way, no additional time is lost trying to establish whether the student needs special accommodations.

David: "[My advice to parents is to] set up a 504 plan so that he can use the restroom when needed as well as make sure he is allowed to carry some kind of bottle with him for drinks."

Sample modifications for elementary/secondary education might include:

Student carries and takes own enzymes
Student may sit at a desk away from classmates who have colds
Snacks are permitted during class time
Child does not need to ask permission to go to the bathroom or to get a drink
Second set of textbooks for home

Section 504 also applies to colleges, graduate schools, and any other institutes of higher education that receive federal funds. Under Section 504, institutes of higher learning cannot refuse to accept students based on their disabilities. The school must provide accommodations if the student is accepted.

Depending on the school’s schedule and an individual’s stage of disease, an IEP may be required. Additionally, it ensures that the school is compliant with the IDEA, and will make reasonable accommodations for students in need. This is especially valuable for college students. Note-taking services or extended deadlines for coursework might be required in the event that the student experiences an exacerbation and cannot attend class.

A Balancing Act

Keeping up with health care in addition to a school schedule, extra curricular activities, and homework can be tiresome. It’s important that students learn effective time management skills and self discipline in order to balance the demands put upon their time and energy. Here are some strategies employed by members of CysticFibrosis.com:

JORDYSMOM: "The week before school starts, earlier bedtimes start. To ensure that things are going smoothly at school, I just communicate with him daily about how he feels, how his day went etc."

Kelli Myers: "As a former teacher, I think it is important for the parent(s) to meet with the teacher, explain the disease, and leave a typed list of instructions/helpful hints. Also, let the teacher and school nurse know of any med changes, illnesses, etc. and specify if the matter is a private matter (not to be shared with classmates, other parents, etc.)."

Michelle43: "Good communication is very important. The nurse checks Collin's temp at lunch for me if I think he may be coming down with something or I let the teacher know that we're increasing his treatments and the albuterol may make him fidget and lose concentration in class. I also mention to the gym teacher that it's so hot out that Collin has needed extra salt tabs at home; I usually see the gym teacher daily. I had one teacher’s aid who was sort of snotty and when she saw a list of Collin's meds by accident her whole attitude changed and she started asking questions, and she is much more understanding towards my son now. Sometimes education is all that's needed."

Conclusion

Starting school or going back to school can be an exciting while somewhat nerve-wracking experience. When parents, students, and educators work together, students with CF will benefit fully.

With the newest advances in medications and treatments for cystic fibrosis, more and more patients are able to not only graduate from high school, but go on to institutes of higher learning. The future for students with CF is growing brighter every day.

What Lowers Blood Sugar?

2008-08-24T15:27:00.000-07:00

When the body metabolizes (i.e., breaks down) molecules of carbohydrates, it converts it into blood sugar. Blood sugar or glucose is the fuel the body needs to carry out its day to day functions. Compared to fats and proteins, carbohydrates are easier to break down.

Glucose is used as rapidly as it is produced. It enables the brain, nervous system, muscles and other organs to function. The body can also store the energy it takes from metabolizing carbohydrates. When it does so, a compound called glycogen is made. Glycogen gets stores in the liver and muscles.

Diabetes is a condition in which the body cannot adequately convert blood sugar into energy. When this happens the blood sugar, called glucose, builds up in the blood stream and does not reach the muscle tissue as it should. This is how diabetes begins to impair major organ functions.

Read the rest of the article here: What Lowers Blood Sugar?

Naturally High Fiber Foods

2008-08-07T15:33:00.000-07:00

Nutritionists recommend consuming 20-30 grams per day of dietary fiber. Time to get out the Metamucil? Think you need to add a fiber pill supplement to your daily meal?Not necessarily. Eating enough fiber can happen simply by selecting the right foods as part of the diet.

Dietary fiber is fiber that comes from plant sources. It is usually referred to as roughage and is useful to prevent constipation, relieve diverticulosis, reduce the risk of colon cancer and helps lower blood cholesterol levels. It can also be used as an effective way to help control weight.

Read the rest here:

Natural High Fiber Foods

Myth #5: People with CF are Thin and Sickly-Looking

2008-08-06T09:56:00.001-07:00

Fact: People with CF look just like anyone else.

Cystic fibrosis patients who have pancreatic insufficiency have trouble gaining weight even with ravenous appetites, but with the help of pancreatic enzyme supplements, they can and do achieve a healthy, normal weight for their age and sex. CF patients who are pancreatic sufficient may actually be slightly overweight since their bodies work normally to absorb calories and nutrients from what they eat.

It’s impossible to tell if someone has cystic fibrosis just by looking at them. Everyone with CF has a different set of complications relating to their disease. Some have more lung problems, while others have more digestive issues. Some are barrel-chested because of the extent of lung disease. Some look just like their non-CF siblings or relatives.

The only way to truly know if a person has CF is if they tell you. Never assume.

Myth #4: People with CF should not drink milk because it makes too much mucus

2008-08-05T15:28:00.000-07:00

Fact: While it may feel like it, milk does not increase mucus production.

A 2005 review of studies concluded that there was no link between milk consumption and mucus production.

In one study, participants infected with the common cold virus reported symptoms of increased in mucus production after drinking milk, but when their mucus production was actually measured there was no statistical difference.

People with CF need to be able to perform airway clearance in order to get as much mucus as possible out of their airways. There are many devices available to assist with this such as vibrating vests, percussors, and even a technique called “clapping” which is performed by another individual.

Additionally, there are medications that have been developed to help thin the mucus and make it easier to cough out. These include hypertonic saline, DNase (Pulmozyme) and guafeneisin, which is an expectorant.

Milk, particularly whole milk, is actually a good choice of beverage for people with CF. It contains plenty of calories, fat, protein and vitamin D to help keep the body healthy. Milkshakes made with ice cream and whole milk are one way for people who are pancreatic insufficient to put on some much needed weight.

Source: http://www.abc.net.au/health/talkinghealth/factbuster/stories/2007/11/28/2102703.htm

Myth #3: Men with CF are Sterile

2008-08-04T22:13:00.000-07:00

Fact: Men with CF are not sterile. 2% of men with CF can father children naturally without the assistance of an IVF (in vitro fertilization) clinic. In the remaining 98% of men with CF, congenital bilateral absence of the vas deferens (CBAVD) is a relatively frequent cause of male infertility.

It is important to note that men with CBAVD are not, as was previously believed, sterile. They produce sperm except the sperm have nowhere to go because the pathway to release them (i.e., the vas deferens) is absent. These sperm ducts are not blocked with mucus; they are completely absent.

CBAVD may not necessarily be a sign of CF, but rather, is a co-occurring disease in patients with more mild mutations of the CF gene. Some of the less common mutations of the CF gene can also result in CBAVD.

Men with CF who have CBAVD can still father children. Family planning options that use their sperm rely on assisted reproductive technology (ART).

Fertility clinics specializing in a process called MESA (micro-surgical epididymal sperm aspiration) can assist a couple who wish to have children naturally. The MESA process is performed in conjunction with two other procedures in which the sperm are injected directly into an egg. The first is called ICSI (intracytoplasmic sperm injection). Once fertilization has taken place, the embryo is then inserted into the womb.

For more information about family planning when cystic fibrosis is a concern, please visit the following site:

FAQs About CF and Family Planning

Myth #2: Only Children are Diagnosed with CF

2008-08-04T14:05:00.000-07:00

Fact: Although it’s true that most people who have cystic fibrosis are diagnosed before age 2, it’s possible to be diagnosed later in life.

Symptoms that lead to eventual diagnosis in adults may include:

Very salty-tasting skin
Persistent coughing, at times with phlegm
Frequent lung infections, like pneumonia or bronchitis
Wheezing or shortness of breath
Small, fleshy growths in the nose called nasal polyps
History of bronchiectasis

With only 112 Cystic Fibrosis Care centers in operation, the number of doctors who are familiar with cystic fibrosis are few and far between. Even pulmonologists (heart-lung specialists) may not believe that a person with CF could survive into adulthood. Ignorance on the part of medical professionals is what makes the path to adult diagnosis so frustrating and discouraging.

Attempting to diagnose cystic fibrosis based on symptoms alone is unreasonable, as every case of cystic fibrosis is different. Even sweat tests which measure the concentration of salt lost when a suspected CF patient perspires are inconclusive and may give false negatives/positives.

Scientists have identified more than 1,500 mutations of the CF gene. Only a full genetic panel, like that offered by Ambry genetics can confirm or deny whether a person has cystic fibrosis. The Ambry panel tests all the known mutations not just the 24 most common ones.

One study has shown that individuals who are diagnosed later in life (i.e., past the age of 40) are less likely to have the deltaF508 mutation. These patients are also less prone to Pseudomonas Aeruginosa infections. The same study showed that late diagnosis was more common for females than males. Another commonality among adults with late diagnosis (LD) cystic fibrosis was the prevalence of pancreatic sufficiency.

Ethnicity and Common Mutations

2008-08-03T21:14:00.000-07:00

Statisticians and geneticists at institutes around the globe have been able to determine that certain CFTR mutations are more commonly associated with specific races and ethnicities. They have identified 24 mutations, all of which have become part of carrier screening as recommended by the American College of Obstetrics and Gynecologists (ACOG.) Studies have gone so far as to associate the mutations not only with ethnicities, but by geographical region as well.

Below is a list of the 24 common mutations for which carrier testing, prenatal and newborn screening is conducted. These are listed numerically and alphabetically, and not frequency of occurrence.

Not every ethnicity has the same mutations. In North America for example, researchers have identified only twenty-one occurrences of 711+1G- >T in a sample population of greater than 5,000 individual chromosomes. This means that the particular mutation is rather rare for Americans in the United States and Canada.

Interestingly, the DeltaF508 mutation is the most common mutation worldwide. This is why a majority of cystic fibrosis patients have 2 copies of the delta F508 gene—one from each carrier parent.

For more information and a complete list of the statistics of ethnicity/region and CF mutations, visit http://www.genet.sickkids.on.ca/cftr/resource/rptTable1Full.html

Myth #1: Only Caucasians have the cystic fibrosis gene.

2008-08-03T20:33:00.000-07:00

Fact: Although Caucasians and people of European descent are the ones most likely to have the CF gene, people of any ethnicity can have it. The statistics below show CF mutations* broken down by race and ethnicity.

Caucasian

Odds that both parents are carriers: 1 in 625

Chance of having a child with CF: 1 in 2500

Mutation detection by screening: 90%

Hispanic American

Odds that both parents are carriers: 1 in 2,100

Chance of having a child with CF: 1 in 8,500

Mutation detection by screening: 57%

African American

Odds that both parents are carriers: 1 in 4,200

Chance of having a child with CF: 1 in 17,000

Mutation detection by screening: 69%

Asian American

Odds that both parents are carriers: 1 in 8,100

Chance of having a child with CF: 1 in 32,400

Mutation detection by screening: unknown

* the most commonly known 25 mutations

These statistics and probabilities will be very different for mixed race couples. This data is based on Genetic Medicine 4(5):379-91. 2002.

Topic of the Month: CF Myths

2008-08-03T20:16:00.000-07:00

This month I will be focusing on dispelling some of the most common myths surrounding cystic fibrosis. As always, I welcome your comments and questions on the subject.

Coping With Home Infusion Therapy

2008-05-23T23:49:00.000-07:00

Home IV therapy for some is much more comfortable than continuing treatment as a hospital inpatient. However, it's not without its own set of frustrations and difficulties. Many cystic fibrosis patients are well-seasoned professionals when it comes to managing the schedule of home IV therapy; for the newly diagnosed or parents who are learning the home IV routine for the first time, it can all be a bit overwhelming. This article is written with the first-timers in mind in hopes that it will allay your fears and give you the confidence you need to cope with this new aspect of managing this aspect of life with cystic fibrosis.

Click here to read the full article which includes information about:

Top Ten Reasons to Support the Cystic Fibrosis Foundation

2008-05-20T13:30:00.001-07:00

1. The Foundation is efficient. The Foundation is one of the most efficient organizations of its kind with nearly 90 cents of every dollar raised available for investment in vital CF research, care and education programs.

2. The Foundation is a leader. Virtually every approved CF drug available today was made possible because of Foundation support. Since the 1980s, the Foundation has played an integral role in the development of Pulmozyme(r), TOBI (r) azithromycin and hypertonic saline for use as CF treatments. To date they have committed nearly $300 million to CF drug discovery and development.

3. The Foundation is innovative. The Foundation invests more money in drug discovery and development than any other disease foundation in the country. Forbes, Business Week, USA Today and Newsweek have recognized the Foundation's innovative approach to research, and its venture philanthropy business model is the subject of two Harvard Business School studies.

4. The Foundation is accountable. In 2008 the Foundation achieved the coveted four-star rating for sound fiscal management from Charity Navigator, the largest independent charity evaluator in the United States. The Foundation is also an accredited charity of the Better Business Bureau's Wise Giving Alliance, meeting all of its Standards for Charitable Accountability.

5. The Foundation is adding tomorrows every day. When the Foundation was established, children born with CF were not expected to live long enough to attend elementary school. Thanks to research and care supported by the Foundation, the median predicted age is now 37 years; more than double what it was 25 years ago.

6. The Foundation is making history. For the first time in the history of the disease, there are therapies in the Foundation's drug development pipeline that addresses the basic defect of CF (a faulty protein.) If successful, these therapies could add decades of life for people with the disease. Early results in the clinical trial for VX-770, an oral drug aimed at addressing the basic defect in CF, show that the compound significantly improves several key trial indicators of the disease--the most promising results the Foundation has seen from a Phase 2 trial.

7. The Foundation is making a difference. Today, more than 44 percent of all people with CF are age 18 or older, thanks to wise investments made by the Foundation in research and comprehensive care. To ensure excellent care, the Foundation has implemented a quality improvement program at its nationwide network of 113 CF care centers. In addition, the Foundation has launches the Program for Adult Care Excellence (PACE) a $5-million initiative to enhance care for adults with CF.

8. The Foundation is taking action. The efforts of the Foundation and its volunteers provide those with CF a voice in Congress. The Congressional CF Caucus has more than 125 members, making it one of the largest caucuses in Congress.

9. The Foundation is forward-thinking. To supplement contributions raised by its dynamic agenda of GREAT STRIDES and special fund-raising events, the Foundation's Milestones to a Cure Campaign was launched to raise $175 million by 2010. To date, Milestones has raised $126 million to support critical therapeutics development.

10. The Foundation is far-reaching. The Foundation has 80 chapters and branch offices throughout the country and supports and accredits a nationwide network of care centers. The CF Services Pharmacy, the Foundation's wholly-owned subsidiary, makes therapies available to nearly 8,000 CF patients nationwide and advocates on behalf of patients working with insurance plans to cover prescription medication.

[the above is reprinted with permission from the Cystic Fibrosis Foundation]

Restrictions on Activities for Kids with CF?

2008-05-19T23:09:00.000-07:00

Children with CF can and should do as much physical activity as their peers do. Regular exercise, especially anything that gets the heart rate elevated and forces a child to breathe more forcefully is a good way to improve lung health. Regular vigorous exercise is beneficial for the patient with CF, and should be encouraged. There are few, if any,activities that the individual with CF cannot participate in, however on occasion contact sports are discouraged due to the positioning of implantable venous access devices.

Another benefit of any organized group activity or sport is that it teaches your child discipline and the importance of being part of a team. As your child gets older, discipline will be key in keeping them compliant with their medications for CF. Also, by learning to be a team player, they will gain an appreciation for how important it is to be an active participant in their health care at the CF Team Clinic.

For a sample list of activities for people with CF and the pros and cons of them, please read the following article:

No Limits on Activities for Children With Cystic Fibrosis

Mixed Messages: When the Sweat Test Is Negative but the Symptoms Look Like CF

2008-05-15T17:50:00.000-07:00

The sweat test used to be the gold standard for determining whether a person has cystic fibrosis. With the advent of more definitive diagnostic procedures such as pre-implantation, prenatal and newborn screening for cystic fibrosis, getting a correct diagnosis is far easier than it used to be.

But what happens when you or your child still exhibits symptoms that are common to cystic fibrosis? How do you determine whether to keep pushing for more genetic testing to confirm CF, or whether it’s time for you and your doctor to start looking at other possibilities?

Here are the most common diseases that present with symptoms very similar to cystic fibrosis:

Shwachman-Diamond Syndrome (www.shwachman-diamond.org)

Diagnosed by: blood test or genetic test

failure to thrive
feeding problems
recurrent infections
slow growth
Diarrhea with fatty, oily loose stools
Blood clotting problems (similar to vitamin K deficiency in cystic fibrosis)
Enlarged liver
Diabetes mellitus
Abnormal pulmonary function test

Celiac Disease (www.celiac.org)

Diagnosed by: 1) celiac blood panel 2) duodenal biopsy and biopsy of multiple locations in the small intestine 3) observed response to gluten free diet

Recurring bloating, gas or abdominal pain
Chronic diarrhea or constipation or both
Unexplained weight loss
Pale, foul-smelling stool
Anemia
Vitamin K deficiency
Fatigue
Failure to Thrive (infants)
Male infertility

Primary Ciliary Diskinesia

Diagnosed by: biopsy of lung or sinus tissue

Chronic sinusitis
Bronchiectasis
Susceptibility to pneumonia and lung infections
Male infertility

Alpha-1 Antitrypsin Deficiency

Diagnosed by: simple blood test

Impaired liver function
Recurrent lung infections
Airway inflammation
Hemoptysis
Swollen abdomen due to enlarged liver
Wheezing and shortness of breath
Chronic cough
Bronchiectasis

Corticosteroids (Prednisone)

2008-05-14T17:03:00.000-07:00

Corticosteroids are often prescribed to cystic fibrosis patients as a way to decrease inflammation in the body. When given in an initially large (sometimes called a “burst”) then tapering dose, corticosteroids such as prednisone have been shown to be effective in dealing with the short-term inflammation problems caused by asthma and allergies. Corticosteroids, specifically forms of fluticosone, are also commonly used as a treatment for allergic bronchopulmonary aspergilliosis (ABPA.)

Cystic fibrosis patients who culture P. Aeruginosa infection have demonstrated an increase in their forced vital capacity (FVC, i. e., the total amount of air they’re able to breathe in at once) measure on part of their pulmonary function tests.

Long term use of corticosteroids must be monitored carefully. When the body is relying on corticosteroids for a long period of time, it ceases production of its own steroids. In some cases, cystic fibrosis patients may become fully dependent on a course of corticosteroids just so their bodies can function properly in response to natural biological stressors such as illness or injury.

The most documented complication of long term or high dose treatment using corticosteroids is their ability to induce diabetes mellitus or cystic fibrosis related diabetes. Prolonged use of prednisone can weaken the skeletal system and can cause growth problems, osteoporosis and adrenal suppression. These side-effects are even more devastating in younger patients with cystic fibrosis. Side effects of prednisone include insomnia, elevated mood, and in some cases, even mania. Using prednisone for long periods can cause side effects such as puffy face (called ‘moon face’) weight gain, osteoporosis, glaucoma and type II diabetes mellitus. Stopping the medication without tapering down the dose may cause some patients to suffer from depression.

Inhaled steroids such as Flovent, Azmacort, or Pulmicort have been effective in their long term use for controlling the inflammatory effects of asthma. These inhalers do not provide immediate relief of symptoms like a bronchodilator would, but when used as part of a regular treatment regimen they can help keep wheezing and chest tightness under control. Because the benefits of oral corticosteroids are apparent, current studies have turned to the efficacy of inhaled corticosteroids on lung function in individuals with CF. A pilot study examined inhaled corticosteroids over a 30-day period and concluded that patients treated with inhaled steroids had better pulmonary function. Inhaled delivery of corticosteroids has far fewer adverse effects than other forms of the medication.

For more information:

http://www.cysticfibrosismedicine.com/htmldocs/CFText/steroids.htm

http://www.buzzle.com/articles/prednisone-side-effects.html

What is BiPAP?

2008-05-13T22:23:00.000-07:00

BiPAP stands for Bi-level Positive Airway Pressure. It is a breathing apparatus that helps people get more air into their lungs. The airways are kept open using a BiPAP machine. BiPAP machines are adjusted to dual settings so that the pressure of the air coming in on inspiration can be different from the pressure of the air being exhaled. This is beneficial because it allows the patient to get more air in and out of the lungs without having to exert a lot of muscle energy.

A person’s forced vital capacity (FVC) as determined by a Pulmonary Function Test is largely what determines if a BiPAP machine is necessary. BiPAP is often used for people who have experienced pneumothorax (or collapsed lung) and as a result have loss of ability of the air sacs at the outermost portions of the lungs.

BiPAPs are usually used while a person is sleeping. It is used for conditions other than just cystic fibrosis. Sleep apnea sufferers for example, are often fitted with a BiPAP machine and face mask. Unlike supplemental oxygen, BiPAP masks are worn so that they completely enclose the mouth and nose. Most manufacturers of BiPAP face masks have different models available and all are designed to be as comfortable as possible.

Failure to Thrive (FTT)

2008-05-12T00:00:00.000-07:00

The first few years in a child's life are critical period during which the most dramatic changes in growth and mental development take place. When a baby or toddler doesn't gain weight and grow at the predicted rate, a doctor may say that the child is failing to thrive. In many cases, failure to thrive and its accompanying symptoms can be the key to effectively diagnosing cystic fibrosis as early as possible.

Failure to Thrive (FTT) is detected when a child falls below the 80th percentile for height and weight at a given age. Failure to thrive is a somewhat ambiguous term, is not a formal diagnosis. However, it serves as a starting point for exploring the underlying cause of the condition.

Symptoms or behaviors that may indicate failure to thrive include:

-Frequent diarrhea or vomiting

-Irritability and colic

-Unfocused eyes, inability to concentrate

-Developmental benchmarks such as sitting up, talking, etc. are not reached when expected

-Refusing food

-Repeated infections

In many cases, failure to thrive is connected to a baby's inability to eat or utilize calories and nutrients properly. The causes can be either physical, as in the case of a child with a cleft palate who cannot suckle for breast or bottle feeding. Failure to thrive may also be the result of a chronic condition like cystic fibrosis, in which the pancreas cannot secrete the enzymes required to help the body absorb food. Food allergies, celiac disease, and inability to tolerate milk protein are just a few of the underlying causes of failure to thrive in infants.

A child who is failing to thrive may also be the product of a poor socio-economic environment. Living in poverty without access to health care, or an inability to buy the appropriate foods for an infant, can cause failure to thrive. Sadly, failure to thrive is sometimes caused by child abuse.

Doctors rely on growth charts to determine whether an infant is growing normally. Specifically, a baby's birth weight should double by the age of 4 months, and should grow almost 10 inches in the first year. Although birth weight may drop initially, when an infant does not recover that lost weight in a suitable amount of time (2 weeks), the doctor will look into the reasons as to why. Even babies who are born prematurely are expected to progress according to an adjusted growth chart.

Correctly diagnosing the cause of FTT as early as possible is essential to prescribing the most appropriate and effective treatment plan.

Controlling Asthma

2008-05-11T23:56:00.000-07:00

Effectively controlling asthma requires a great deal of self-awareness and discipline. It involves being diligent in controlling one's personal environment to the greatest extent practicable. For example, dust and dust mites are a major source of problems for asthmatics. Eliminating dust in the home, particularly in the areas in which the most time is spent, can greatly decrease the number of asthma attacks.

Here are a few suggestions for limiting asthma triggers in the home:

-vacuum carpets at least once per week

-dust and sweep at least once per week

-enclose pillow cases in an allergen reducing cover

-purchase a portable HEPA air filtration unit for the home

-eliminate clutter in the form of knick-knacks

-use your stove's fume hood when cooking (this reduces the amount of Nitrogen in the air-a common asthma trigger)

-limit the use of air freshening aerosols or candles

Asthma is a chronic respiratory condition, characterized by severe narrowing of the airways upon exposure to air-borne pollutants, or allergens. Anything that sets as asthma attack in motion is called a "trigger."

Read the rest of the article here.

Anti-Anxiety Medications

2008-05-11T23:48:00.000-07:00

Coping with cystic fibrosis is a life-long challenge. The needs and demands of such an all-encompassing condition can cause a person to develop anxiety and depression. Many patients with cystic fibrosis are prescribed anti-depressant medication to help them keep from feeling overwhelmed by their disease. Constant fear and worry about becoming sicker or being exposed to a difficult-to-treat infection can also lead to problems with anxiety.

When panic and anxiety attacks interfere with your ability to function in your day-to-day routine, especially to the extent that it interferes with a cystic fibrosis patient's ability to remain compliant with their treatment program, it may be time to look into medical treatment. Determining which anti-anxiety medication is best for your particular symptoms should begin with a visit to a mental health professional to obtain a proper mental health evaluation, and definitive diagnosis.

Panic disorder is just one of five types of anxiety disorders, not including specific phobias. Evidence from a study published in 2005 suggests that more than 6 million people suffer from panic disorder.

The symptoms of panic disorder can be prevented with a variety of medications, some of which are effective all by themselves and others which provide relief when combined with another psychotropic medication.

Benzodiazepines ("benzos") such as Xanax and Klonopin are the heavily favored for treating panic disorder. Their main benefit is that they can produce the desired relief sooner than some other medications. Almost half of the time, low doses of Xanax or Klonopin are effective in blocking a panic attack. All benzos do carry a certain degree of dependency risk. One of the most positive aspects of using Xanax, is that it is fast acting, and can be taken at the first sign of a panic attack, and will reduce the level of anxiety associated with it in under ten minutes.

Klonopin, however, works best when taken regularly as a maintenance medication. People who don't want to take medication daily, or are forgetful about taking it would probably prefer a different type of anxiety medication. One benefit with Klonopin, is that when it's a regular part of your regimen, situations that would normally produce anxiety and panic don't seem to be as stressful. Compared to Xanax, a person taking Klonopin feels more "normal" even when taking it, and not "dulled" by the medication.

Antidepressants, although they take longer to produce the overall desired effect in the brain chemistry (weeks instead of days) have a much higher rate of success when taken regularly for the treatment of panic disorder. Examples of antidepressants include Anafranil, Tofranil, Elavil, and Adapin.

Selective Serotonin Re-uptake Inhibitors (SSRIs) are perhaps the most commonly recognized class of medications for the treatment of panic disorder. Zoloft, Paxil and Prozac, are names many people have heard of or seen on television or in magazines. Because the have fewer side effects than other medications, many people prefer them. In terms of effectiveness, they are just as beneficial as antidepressants. Deciding which medication is the right one should be based on the severity of your symptoms, the frequency of the attacks, and your willingness to be patient while your body adjusts to the medication.

Medication for anxiety attacks is not the appropriate solution for everyone, but for some people it can make a world of difference. Many find that a combination of cognitive behavioral therapy (CBT) and medication can greatly improve their anxiety symptoms.

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Choosing the proper anti-anxiety medication

Treatment of Mold Allergies

2008-05-11T23:45:00.000-07:00

Molds and fungi are very resilient and can linger even in the most inhospitable environments. Spring to late fall is generally considered "allergy season" since this is when pollen becomes airborne. Mold spores are much smaller than pollen, however, and pervasive in all seasons Whether they are found indoors or outdoors, mold typically grows in moist areas and releases its spores to the air at any time. These spores are tiny enough to enter the smallest airways of the respiratory tract, where they wreak havoc with a person's immuno-response system. Itchy, watery eyes, continuous sneezing, wheeze and breathlessness, all are symptoms of a mold allergy.

Treatment options include:

1. Over the Counter Medications
2. Bronchodilators
3. Corticosteroids
4. Immuno-therapy (allergy shots)

. Anti-fungals

Determining the appropriate treatment for mold allergy begins by obtaining a full diagnosis from a doctor, dermatologist or allergist.

Read the rest of the article here.

What is Stenotrophomonas maltophilia?

2008-05-11T22:35:00.000-07:00

Stenotrophomonas maltophilia or (sten malt) as it is often abbreviated, is a strain of gram negative bacteria. Similar to B. cepacia in its ability to resist treatment with antibiotics, it is often found living in aquatic environments. A study in 2002 found that unlike B. Cepacia, Stenotrophomonas maltophilia doe not reduce survival for people with cystic fibrosis. Cystic fibrosis patients can become infected with Stenotrophomonas maltophilia when they come in contact with contaminated, moist environments such as:

1. Sinus flushes
2. Mouthwash
3. Nebulizers used by other CF patients
4. Other equipment used by CF patients
5. Respiratory secretions
6. Urine or catheters
7. Open sores

Healthy people are not susceptible to stenotrophomonas maltophilia. As is the case in any situation involving the hospitalization of a patient with CF, the necessary contact precautions should be used to prevent the spread of Stenotrophomonas maltophilia. Strict adherence to proper cross-infection protocol has been very effective in reducing the risk of transferring a stenotrophomonas infection between patients in a hospital setting.

Fortunately, sten. malt. is far less likely to cause infection than PA. "Several studies in the US and elsewhere have been performed to find out the effect of Steno colonization on lung function and morbidity in CF patients, and none of them found that Steno actually causes a significant effect, unlike B. cepacia, for example. It just seems to like to sit in the cf lung." (M B Avison, PhD. University of Bristol, UK. www.bris.ac.uk/bcare/BGG)

It has a very limited ability to cause infection in humans and is only potentially lethal when it infects the bloodstream causing bacteremia. Overall, infections of sten. malt. are quite rare. They can be treated using a synergistic approach of intravenous medications that are targeted to gram-negative bacteria. This may involve treatment with colymycin and tobramycin used together.

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Supporting documentation

Synergistic Activities of Macrolide Antibiotics against Pseudomonas aeruginosa, Burkholderia cepacia, Stenotrophomonas maltophilia, and Alcaligenes xylosoxidans Isolated from Patients with Cystic Fibrosis

Prevalence and antimicrobial susceptibility of microorganisms isolated from sputa of patients with cystic fibrosis.

Factors Affecting the Incidence of Stenotrophomonas maltophilia Isolation in Cystic Fibrosis^*

Transitioning to Adult Care

2008-05-09T22:31:00.000-07:00

Cystic fibrosis has long been considered a disease that affects mainly children. Many CF patients are seen by a pediatrician from infancy well into their early twenties. Thanks to earlier diagnosis and more effective therapies that can be done early in a child’s life, these children are well on their way to a healthy adulthood.

But what about those years in between? How can parents prepare their children for the transition to adult care? When should the child begin taking responsibility for their own medical needs? What are the things that children need to learn before they’re ready for the transition?

Cystic fibrosis patients and their physicians, especially the pediatricians and the rest of the CF Team, often form strong bonds. Together the patients, their parents and the doctors have learned how to deal specifically with the individual. This tremendously supportive network takes years to build, and the thought of walking away from it all to begin with a new doctor and an unknown CF team can be frightening. The fear of the unknown and the worry about having to start all over with someone new can fill a CF patient with anxiety.

Patients who have a great deal of self-confidence and a solid understanding of their particular needs as a CF patient handle the transition much better than those who have never involved themselves in their own health care. Preparing to transition to adult care is just that—a transition. As the parents gradually take a lesser (but no less supportive) role in their child’s health care, the patient gradually learns to take control. Ultimately this promotes self-esteem, a sense of empowerment, and even better health for those who are compliant with doctor’s orders.

Studies have proven that people with chronic diseases who frequently set challenging but reasonable goals for themselves are better able to cope with the world around them. They are able to manage their disease with an optimistic yet realistic attitude. This contributes to a greater overall quality of life.

The files below are something of a game plan. The goal: being well prepared to leave the pediatrician as a confident, competent adult who knows how to cope with CF on its many levels and to have the best quality of life possible. Please note that these files are for disabilities in general and aren't specifically targeted to cystic fibrosis.

Health Care Transition Workbook (English, age 12-14)
Health Care Transition Workbook (Spanish, age 12-14)

Health Care Transition Workbook (English, age 15-17)
Health Care Transition Workbook (Spanish, age 15-17)

Health Care Transition Workbook (English, age 18+)
Health Care Transition Workbook (Spanish, age 18+)

Career Spotlight: Architectural Designer

2008-05-09T21:41:00.001-07:00

Just because a person has cystic fibrosis doesn’t mean they can’t plan for and enjoy an interesting, fulfilling and challenging career. People with CF have virtually endless options available to them when it comes to the future. Improved therapies, better health education and a “can do” attitude are just a few of the ingredients that go into making life’s goals a reality.

Meet Amy—a 27 year old adult with cystic fibrosis. She is just one of many adults with who has answered the call to the work force. A full time architectural designer, she enjoys the challenge of using her computer skills to create architectural drawing to be included with construction permits. “I love my job,” she says. Rendering, which is creating color drawing by hand “is my absolute favorite thing.”

Like other adults who have to find a way to combine their creative passions and pursuits with the ever-present need to maintain good health. Amy’s work ethic and how she manages her workload stems largely from the skills she has utilized for managing life with cystic fibrosis. “With CF you must be very diligent and pay attention to the details” she explains. She recognizes that this is important not only for keeping on top of her health, but “with my job as well.”

Initially Amy was concerned about how to inform her employer about her disability. To her relief, she found her employer to be very accommodating to her needs as a CF patient. Her 8:30 to 5:30 schedule isn’t very physically demanding, but the projects that land on her desk often have sensitive deadlines that must be met. Nonetheless, “I’ve not had any problems with taking time off,” she explains. “We can come and go” for necessary doctor appointments. Her employer only requests that she adjust her schedule to make up any time missed for such things.

Some cystic fibrosis patients worry about their job security, especially if they must be hospitalized for a lengthy period. Time spent away from the office while on sick leave or disability cause a lot of worry and anxiety. Fortunately for Amy, her employer and her coworkers were supportive when she hit a rough patch with her health during the winter.

“I became sick and needed to go inpatient. I told my employer then [that I have cystic fibrosis]. They were very supportive and when I went back in less than 2 months later there were no issues [about job security].”

What does the future hold for Amy? She admits that her health isn’t as strong as she’d like it to be. When asked about how hard she plans to work in the future, she candidly shares “I am considering going part time this year.” She is hopeful that her health will improve once she takes a lighter schedule at work, and that she can work awhile longer before needing to leave work permanently.

Amy sets a positive example for younger patients who may be struggling with their disease. She keeps from focusing too much on herself by doing things to reach out to other members of the community who have chronic conditions. For example, she recently donated her beautiful, long hair to “Locks of Love” a charity that uses donated hair to make lovely wigs for cancer patients who have lot their hair from chemotherapy.

Her advice for others who may be interested in working in the field of architectural design? "Take some drafting classes and design classes at a local community college." She also recommends looking through design books, and encourages future architectural designers to "be prepared to work very hard in school."

Hill-Burton Helps CF Families Needing Reduced Cost or No-Cost Medical Care

2008-05-09T18:49:00.000-07:00

Living with cystic fibrosis takes its toll on a person's stamina, mental well-being, and almost always the family's finances. By applying through the United States' Department of Health and Human Services, families who are struggling to handle the heavy financial burden of cystic fibrosis can qualify for low-cost or no cost medical services as provided by certain hospitals that are contracted with Hill-Burton.

Although the application has very specific parameters, it's at least worth looking into. Extensive medical services to treat cystic fibrosis are hard to find, the list below may help you identify a Hill-Burton obligated facility in your area. You may apply for coverage before, after or during the time you are receiving health care.

Services that are applicable for cystic fibrosis patients and their families include newborn screening tests, and other lab work. Some facilities can provide home health care, such as home infusion therapy. Eligibility depends primarily on household income.

Unfortunately, there are no Hill-Burton obligated facilities in the following states:

Indiana
Nebraska
Nevada
Rhode Island
Utah
Wyoming

Steps to Apply for Hill-Burton Free or reduced-cost Care

Find the Hill-Burton obligated facility nearest you from the list of Hill-Burton obligated facilities.
Go to the facility's admissions or business office and ask for a copy of the Hill-Burton Individual Notice. The Individual Notice will tell you what income level makes you eligible for free or reduced-cost care, what services might be covered, and exactly where in the facility to apply.
Go to the office listed in the Individual Notice and say you want to apply for Hill-Burton free or reduced-cost care. You may need to fill out a form.
Gather any other required documents (such as a pay stub to prove income eligibility) and take or send them to the obligated facility.
If you are asked to apply for Medicaid, Medicare, or some other financial assistance program, you must do so.
When you return the completed application, ask for a Determination of Eligibility. Check the Individual Notice to see how much time the facility has before it must tell you whether or not you will receive free or reduced-cost care.

For more information, please check out the Hill-Burton fact sheet and FAQ

What is Aztreonam?

2008-05-09T18:29:00.000-07:00

Aztreonam is an antibiotic developed by Gilead that is currently in Stage 3 clinical trials. Stage 3 is the last step before the product becomes available to patients. Aztreonam is a beta-lactam, which means it is in the same family of antibiotics as meropenem.

This medication represents a very exciting new treatment against P. Aeruginosa infection. Study data in the United States has demonstrated the drug to be 88% effective against P. Aeruginosa (PA.) This is true not only in preventing PA infection in young patients, but in treating older patients who have been colonized with PA. Aztreonam is also showing a certain amount of effectiveness against certain strains of B. Cepacia.

Studies also show that Aztreonam is similar in effectiveness to ceftazadime for the treatment of pulmonary exacerbations. The drug will be available as an inhaled form to be used in the eFlow.

For more information about Aztreonam and press releases from Gilead Pharma, please see the following links:

Gilead Announces Detailed Results of Phase III Study of Aztreonam Lysine for Inhalation in Patients with Cystic Fibrosis

Gilead Submits New Drug Application to Use Aztreonam Lysine for Inhalation for CF

Other Press Releases

How We Breathe: Understanding Oxygenation of the Blood

2008-05-09T17:48:00.000-07:00

One of the main functions of the lungs is to oxygenate (bring oxygen into) a person's blood. When a person takes a breath of air, either through the mouth or the nose, it travels down the trachea (windpipe) where it then flows to tubes called bronchioles. At the end of the bronchioles are little sacs. These sacs are called alveoli and are the part of the lungs where oxygenation takes place.

Alveoli have very thin walls, but they have amazingly large surface area. This allows the oxygen in the alveoli to diffuse across the surface as much and as quickly as possible. Oxygen diffuses into the blood, and carbon dioxide is removed from the blood. This process is called gas exchange. The red blood cells, specifically hemoglobin, now full of life-giving oxygen, go out from the lungs via the pulmonary vein, and dispersed throughout the rest of the body.

In patients with cystic fibrosis, the thick, sticky mucus that blocks the airways, bronchioles and alveoli, greatly impedes the body's ability to perform the function of gas exchange. As a result, the blood stream retains too much carbon dioxide. As the carbon dioxide builds up in the bloodstream, a person may begin to experience grogginess, confusion or dizziness. Carbon dioxide in the blood stream also has the potential to damage muscle tissue, including the heart, when it builds up to the point of forming carbonic acid.

Carbonic acid is the same thing that gives a soda its "fizz." Have you ever accidentally burped soda through your nose? Remember how much it burned and irritated your throat and nasal passages? That's because the carbonic acid was damaging the sensitive tissue. The same thing happens in the body when gas exchange cannot proceed normally.

Read the rest of the article here...

Nasogastric (NG) Tube Feeding

2008-05-09T17:43:00.001-07:00

Cystic fibrosis patients are encouraged to follow a diet that is high in calories. Doctors recommend that the calories come from high energy foods such as animal proteins and fats. Patients who are pancreatic insufficient have a difficult time gaining and maintaining a healthy weight. As a result, these patients are malnourished. Research has demonstrated that up to 30% of young adult patients with cystic fibrosis (CF) are malnourished.

Failure to maintain a healthy body mass index (BMI) can lead to lower pulmonary function, since the body is not absorbing the nutrients needed to build up strong respiratory muscles. This is just one of the reasons why weight loss is often a precursor or indicator of a respiratory infection. When patients are severely malnourished they may become candidates for what is called "nutritional rehabilitation." This means that the patient is given additional calories and energy via tube feeding.

When Is It Used?

The most popular way of tube feeding is called nasogastric (NG) feeding. Nasogastric feeding involved inserting a thick, flexible tube through the nose, back down the esophagus and into the stomach. From there liquids can either be added to or removed from the stomach. There are different scenarios in which NG feeding is used. These may include instances when a patient is experiencing a bowel blockage and must not eat solid food by mouth; post-operational use to prevent the acidic contents of the stomach from being aspirated (inhaled.) Perhaps the most familiar use of NG tubes is when a person has to have their "stomach pumped" in order to remove poison, or an overdose of oral medication.

NG-tubes are intended for infrequent, short-term use. For general purposes such as post-operative emptying of the stomach, they are only in place for two or three days. However, in the case of cystic fibrosis patients who require tube-feeding, this may be longer. Some cystic fibrosis patients have a new NG-tube inserted on a nightly basis, so they can receive nutrition while they sleep.

Read the rest of the article here...

CF Statistics that Aren't Frightening

2008-05-08T19:15:00.000-07:00

Cystic fibrosis patients are surviving well into adulthood. This change has been rapid and dramatic. Sadly, there's still far too much information out there that can be overwhelming and downright discouraging.

Here is just a smattering of paraphrased information from medical professionals about their optimism (guarded or not) for the future and quality of life for cystic fibrosis patients. Where applicable, I've backed up their quotes with meaningful statistics.

...with only conventional treatments, survival rates for people with CF have improved dramatically...

[Patients continue to] have 100% lung function even 10 years after Pseudomonas colonization

Roughly 85% of the CF patients born in the ‘80’s survive today, and for the CF child born in the ‘90’s, there is no real decrease in life expectancy attributable to CF.

Dr. Birgitta Strandvik, CF Center Director,
University Hospital,Göteborg, Sweden

In an average year, between 120-150 patients with cystic fibrosis are given a double-lung transplant.

Thanks to new rules regarding who gets priority on the transplant list, people who are wait-listed for new lungs receive them in under 2 years.

"lung transplantation can improve survival and quality of life for many cystic fibrosis patients."

Jonathan Orens, MD, Johns Hopkins School of Medicine

Pre-natal and Newborn screening for cystic fibrosis allows the child faster access to life-improving therapies and medications.

Thanks to newborn screening for CF, the average age at diagnosis is now 2 years old.

Clinical trials are progressing at a fast pace for therapies and medications to treat the symptoms of CF.

Genetic testing for the most common types of CF mutations is now affordable. A full panel of genetic screening is also available, which makes the future bright for treatments that focus on specific genotypes (i.e., mutations ) of CF.

What is a Pulmonary Exacerbation?

2008-05-08T17:55:00.001-07:00

Pulmonary exacerbation [pull-moh-NARE-ee eks-a-sur-BAY-shun] is defined simply as a temporary worsening of the lung function due to an infection or inflammation. Although no formal definition exists, an exacerbation is generally characterized by the following symptoms:

1. Shortness of breath
2. Fatigue
3. Increased cough
4. More productive cough
5. Drop in FEV1 or other markers of the pulmonary function tests

There is really no way to categorize what constitutes an exacerbation for everyone with CF. Generally, if something is happening that feels out of the ordinary with regard to breathlessness or lung health, then it might be an exacerbation. In adults with cystic fibrosis, P. Aeruginosa is most often the cause of a pulmonary exacerbation, and is diagnosed with a sputum culture.

Exacerbations or "Flare Ups" associated with lung disease in cystic fibrosis are the body's way of asking for help. A doctor may diagnose an exacerbation by listening to the lungs for any unusual crackles or wheezes, and looking at comparative pulmonary function tests.

Some people with cystic fibrosis experience exacerbations at rather predictable times in the year. For some, seasonal allergies can bring about a flare. For others, it's humidity. For still others, an exacerbation may be caused by an upper respiratory infection or pneumonia.

Treating an Exacerbation

Cystic fibrosis specialists usually like to treat an exacerbation initially with a short course of prednisone. CF patients who have an asthma in addition to CF most often benefit from these corticosteroids. Bronchodilators are also useful to open up tightened airways.

In moderate exacerbations where the FEV1 drops more than a few mL from the normal baseline PFTs, oral antibiotics or inhaled antibiotics such as TOBI, Tazicef or Colistin might be prescribed. The more serious exacerbations that occur with fungal infections or bacterial infections may require a "tune-up" in the hospital where the patient can benefit not only from medication, but additional chest physiotherapy, a monitored diet and, when possible, rest.

Positive Information

It's important to remember that an exacerbation is not necessarily the precursor to declining health. People who experience predictable flare-ups at certain times of the year benefit from doing preventive tune-ups to help them enjoy longer periods of good health with relatively few symptoms. Also, just because a cystic fibrosis patient experiences an exacerbation, it doesn't mean that they have been colonized or infected with a new type of bacteria. It could simply be that an existing bug is growing too fast for the body to fight without antibiotic intervention.

An Eye to the Future

Cystic fibrosis researchers are still studying the link between exacerbations, their severity, and antibiotic therapy. Clinical trials for certain types of inhaled antibiotics are ongoing. The hope is that preventing exacerbations rather than just treating them will greatly improve the quality of life and mean survival age of people with CF.

Wonderful Progress from the Cystic Fibrosis Foundation

2008-05-02T05:32:00.001-07:00

The Cystic Fibrosis Foundation just published the following press release:

We have some exciting news. We are pleased to tell you that, over the past seven days, Congress and the President took action on four separate bills that champion the rights and health of people with cystic fibrosis.

The Cystic Fibrosis Foundation played a hand in each of the following developments:

The Senate officially recognized May 2008 as National Cystic Fibrosis Awareness Month.

Congress passed the Genetic Information Nondiscrimination Act (GINA) today. This law makes it illegal for employers and insurance companies to discriminate based on genetic information.

President Bush signed into law the Newborn Screening Saves Lives Act, which provides resources and funding for new and existing newborn screening programs through 2012.

The House of Representatives reauthorized the Small Business Innovation Research (SBIR) program. This program awards grants to small biotechnology companies that conduct important CF research.

Each of these initiatives has tremendous potential to help people with CF. These bills help increase awareness of cystic fibrosis, focus research funding on CF, and establish critical legal protections to help people with the disease live longer, healthier lives. Click here to learn more.

These successes would not be possible without you. Thank you for your diligence and dedication.

Understanding a Fecal Fat Study

2008-04-10T17:53:00.000-07:00

Background on Malabsorption and Pancreatic Insufficiency

Cystic fibrosis is a genetic disease in which a faulty protein in a person's genes causes their body to produce abnormally thick, sticky mucus For some people with CF, this mucus causes mainly respiratory complications such as chronic bronchitis, sinusitis or recurring pneumonia. However, CF is not just a respiratory disease. The thick mucus is also present in the person's digestive system, where it prevents the pancreas from secreting the enzymes needed to absorb nutrients and energy from food. When a person experiences malabsorption due to the lack of naturally produces pancreatic enzymes, that person is said to be pancreatic insufficient.

One of the ways to determine whether a person with cystic fibrosis will need supplemental pancreatic enzymes is to do what's called a "fecal fat study." Additionally, a fecal fat study can help the doctor or dietician determine which type of pancreatic enzyme supplements to prescribe, and how many pills are to be taken with food.

Fecal Fat

Not all fats are bad. In fact, the body relies on certain types of fat to store energy, and help absorb vitamins A, D, E and K. A person with normal pancreatic and liver function readily absorbs dietary fat from digested food as it passes into the small intestine. The small intestine is the last stop before foods become fecal matter. The technical term for fatty stool is "steatorrhea."

When the body is unable to absorb fat from food, the fat gets passed through the body and ends up in a person's stool or bowel movement (BM.) Too much fat in the stool can cause BMs to float, have a bad odor, or be loose and oily. People who are pancreatic insufficient or who have pancreatitis may experience a great deal of pain, bloating and gas when they eat foods that are high in fats.

The Fecal Fat Study

A fecal fat study begins by the patient maintaining a diet high in fat (at least 100 grams per day) for three days. Then, the patient is to collect his or her bowel movements for three days (72 hours.) A laboratory technician or your family doctor will provide the container you will use to collect the sample. When three days of collection are complete, the sample is brought to the lab where it will be dried, and initially weighed. The sample will then be subject to a process by which the fats are separated from the rest of the fecal material. The fat is then weighed and compared to the total weight of the initial sample. This is used to calculate a percentage or amount of fat per day that was excreted in the stool. In healthy individuals, fat is expected to make up about 5% of the total fecal material. In patients with CF, this percentage is much higher. These patients are said to be pancreatic insufficient.

The Results

People who are pancreatic insufficient as determined by a fecal fat study require supplemental digestive enzymes in order to maintain healthy nutritional status. Fats and proteins are essential to proper growth and development. In patients with cystic fibrosis, the pancreas is blocked and cannot secrete amylase, lipase, trypsin and chymotrypsin, which are necessary to absorb not only fats, but proteins as well.Supplemental pancreatic enzymes are formulated in such a way that they contain three specific types of digestive enzymes that help the body absorb fats and proteins. The manufacturers of enzyme supplements combine the three types of enzymes in different ratios. Each supplement is formulated this way.

Nasal Irrigation

2008-04-01T12:31:00.000-07:00

Cystic fibrosis patients are prone to nasal congestion, which can inflame the upper respiratory tract and lead to sinus infections. One of the ways to help keep the sticky mucus from staying trapped in the sinuses is to gently flush them with a saline solution. Such rinses are effective in reducing the inflammation and clearing the symptoms of allergies or rhinitis.

Commercially available sinus rinses and saline sprays are available in different concentrations of salinity. Some kits come with the salt water already mixed, while others require you to add a salt packet to warm water that you put into specifically designed container such as a squeeze bottle.

To make your own saline rinse, you can follow this easy recipe:

Isotonic saline

½ teaspoon of sea salt or kosher salt

1 cup of warm water (or room temperature will do)

½ teaspoon of baking soda

Combine all ingredients in a cup or bowl. Using a bulb syringe, draw the salt water into the bulb.

Proper administration of a saline flush is necessary in order to reap its benefits. A thorough flushing can be a messy process, so it’s best to lean over the bathroom sink or shower when rinsing with it. Start by tilting your head down toward the bowl of the sink. Leave your mouth open. With the filled bulb syringe (or squirt bottle that came in the kit) squeeze firmly into one nostril. The water should flow out the other nostril. If your sinuses are quite congested, some water may flow down the back of your throat and out your mouth. This is normal. Continue rinsing with the other nostril.

Follow up the procedure by gargling with water. This will keep the salt from irritating your throat and causing a cough reflex.

A more aggressive way to flush congested sinuses is to run the saline mixture through a Water-Pik. Don’t use the attachment as is; cut it back about an inch so that the pressure of the water coming out is reduced. This method is quite effective for dislodging stubborn mucus that lingers following a sinus infection. Water-Pik now makes a nasal flush attachment tip that makes flushing your sinuses even easier.

Hypertonic saline is useful in stimulating the linings of the nasal passages to produce some moisture of their own. This helps give the mucus a more slippery surface to slide across, making it easier to clear out any congestion. Hypertonic saline is a saltier concoction and therefore may sting sensitive sinuses. It’s best to slowly increase the salinity (saltiness) of the rinse solution by adding gradually more salt.

CF Patient Preferred Sinus Rinse Products

Breathe Ease
Ocean
Oasis
Salt-Air

How is an Antibiotic Desensitization Done?

2008-04-01T12:27:00.000-07:00

When a sputum sample is taken, the laboratory technicians will let the sample grow in a number of different types of environments to see what grows from the sample. Once bacteria is cultured, it is treated in the lab with different types of antibiotics in order to determine whether that particular bug is sensitive or resistant to specific types of antibiotics. If a culture shows sensitivity to a drug, that is the drug prescribed. But what happens when a person has an allergy to drug that the doctor wants to use to treat the infection? Suppose a culture is sensitive to a broad-spectrum antibiotic such as Cipro or Septra, but the patient experiences pulmonary edema or a severe allergic reaction called anaphylaxis when exposed to the drug?

When there are few or no alternative medications with which to treat the infection, a doctor may recommend a procedure called antibiotic desensitization. This process takes place under extremely careful supervision in the intensive care unit (ICU) of the hospital. A patient is started on a very small dose of the antibiotic and watched carefully for any signs of allergic response. When the patient is observed to tolerate that dose, the pharmacist will prescribe a slightly higher concentration of the drug. Little by little, stronger concentrations of are delivered to the body until the full recommended treatment dose is achieved.

At this point, if the patient has exhibited no severe allergic reactions to the antibiotic, he or she is said to be successfully desensitized. From that point forward, that antibiotics should always be used first to treat infection so that the patient does not redevelop sensitivity to it.

Antibiotic desensitization in adults with cystic fibrosis

Successful Meropenem Desensitization in a Patient with Cystic Fibrosis

Desensitization Useful for Allergy to Life-Saving Drug

Antibiotic allergy in cystic fibrosis

Understanding Cystic Fibrosis Associated Arthritis

2008-04-01T12:20:00.000-07:00

As cystic fibrosis patients are surviving longer into their adult years and “middle age,” some may find themselves experiencing episodes of arthritis. Although cystic fibrosis associated arthritis has been observed to affect only 5-10% of the cystic fibrosis population, there’s no denying it happens.

Aches and pains of the joints and particularly large joints such as knees and ankles can cause extreme pain. During an episode, these sites may feel warm to the touch as well as appear swollen and red. Flu-like symptoms such as fatigue and fever may also be present. The interesting thing about cystic fibrosis related arthritis is that flare-ups are rather intermittent; patients typically experience no other symptoms in between episodes.

Defining CF Associated Arthritis

Cystic fibrosis associated arthritis (CFAA)is described as having episodes that last less than a week, involve the large joints (ankle, knee, wrist, shoulder) and responds to treatment with anti-inflammatory medication.

Non-arthritic Joint Pain

Since CF patients are often malnourished and have osteopenia or osteoporosis, aches and pains may not necessarily be from arthritis, but rather from a condition called hypertrophic pulmonary osteopathy (HPOA.) HPOA is not specifically tied to CF, but is observed in other lung diseases. HPOA is believed to be the culprit behind finger clubbing.

Diagnosis

Diagnosis cystic fibrosis associated arthritis can only be diagnosed once other likely possibilities for joint pain have been ruled out. A doctor will ask questions about when the symptoms appeared, whether the pain was the same on both sides of the body, and whether a rash or fever was present. The doctor also needs to know approximately how long the episodes of pain last. It is important for the patient to distinguish whether the pain is sharp or dull, throbbing or steady.

Treatment

As with most inflammatory conditions, non-steroidal anti-inflammatory (NSAID) medications are the first course of action. Ibuprofen in prescription strength doses (i.e., 600mg or greater) is usually enough to gain control over an episode cystic fibrosis associated arthritis. There is a small percentage of people with CF-associated arthritis that are resistant to NSAID treatment, but do respond to corticosteroids such as prednisone. An anti-inflammatory diet may also be helpful to lessen the severity of an episode and prevent further ones.

Understanding Vitamins

2008-03-31T16:44:00.000-07:00

Cystic fibrosis patients who are pancreatic insufficient and have to rely on enzyme supplements in order to have adequate nutrition, tend to have low vitamin levels. Vitamins A, B, D, E and K are the ones of primary concern. Routine blood work can determine how much of a particular vitamin is being absorbed. In cases where a person is vitamin deficient, vitamin supplements are recommended. Products like ADEKS, AquADEKs and SourceCF ABDEKS are three of the most commonly recognized vitamin supplements for patients with cystic fibrosis.

In order to understand the importance of each vitamin, we must look at how they function in the body. Vitamins are divided into two main categories: fat-soluble and water-soluble. Cystic fibrosis patients, due to their low body fat, have trouble absorbing fat-soluble vitamins.

Fat Soluble

A: divided into retinols and beta carotenes, vitamin A is needed for growth and cell development. It helps the body maintain healthy skin, hair and nails, not to mention gums, bones and teeth. Some studies have even linked it to being a likely way to prevent lung cancer.

When the body doesn’t get enough vitamin A, nightblindness is the most noticeable symptom. In children, insufficient levels of vitamin A can cause stunted growth or small stature. Cystic fibrosis patients without enough vitamin A are more prone to infection of any type. Dry skin is also a symptom of lack of vitamin A in the body.

D: also called calciterol, vitamin D is a precursor to calcium absorption. It helps the body maintain bone density. This is one vitamin that the body is able to generate on its own. Exposure to direct sunlight in reasonable amounts is what triggers the body to produce this vitamin.

Food sources of Vitamin D include milk, butter, egg yolks, and fatty fish. These are excellent food choices for people with cystic fibrosis since they provide high calories, good and some protein. The body doesn’t need much vitamin D—only 5 micrograms.

Vitamin D deficiency commonly presents itself as diarrhea, loss of appetite and headaches. Determining whether these symptoms are caused by lack of vitamin D or are signs of some other complication of cystic fibrosis can be difficult, especially in children.

E: tocopherol is the technical term for vitamin E. An important antioxidant, this vitamin helps the body maintain muscle structure and red blood cells. Healthy red blood cells are important for people with cystic fibrosis, since adequate oxygen saturation is needed to maintain good health.

There are no physical symptoms that are associated with low levels of vitamin E, but inadequate concentrations of it in can interfere with a woman’s ability to carry a pregnancy to term. Food sources of vitamin E are those high in “good cholesterol,” for example, eggs, vegetable oils, margarine and mayonnaise and nuts. Dark green leafy vegetables, particularly spinach and asparagus are high in vitamin E.

K: Without vitamin K, the body cannot clot blood properly. In people with cystic fibrosis, deficiency in vitamin K can lead to challenging bouts of hemoptysis (coughing up blood) even to the point of requiring an embolization to stop the bleeding. Excess bleeding, especially for women, can also result in iron deficiency. This impairs the body’s ability to heal from infection. People who bruise easily may be deficient in vitamin K.

One amusing way to tell if a person is deficient in vitamin K is to take a piece of real gold (14k or better) and press it to the person’s cheek and “draw” a line with it. If the person’s cheek develops a dark gray line where the gold was, the person is vitamin deficient.

Water-Soluble Vitamins

Biotin: This vitamin sets the pace of a person’s metabolism and is a source of energy for the body. Egg yolks, soybeans, cereal and yeast products (excluding beer) are foods that contain biotin. Lack of biotin can lead to depression, dry, scaly skin and hair loss. Although uncommon in people with cystic fibrosis, high blood cholesterol is a sign of biotin deficiency.

Folate: Also known as folic acid and folacin, this vitamin is needed to make DNA, RNA and the ever-essential red blood cells. It also helps the body synthesize amino acids. Without it, weight loss, anemia and digestive issues can occur. Women who are pregnant and have insufficient folic acid levels are at a greater risk for having a baby with birth defects. Folate also helps liver function—which is useful for cystic fibrosis patients with liver damage.

Sources of folate in food include: liver, avocados, raw vegetables, and crunchy vegetables like broccoli and celery.

Niacin: B3 is the form of niacin needed to metabolize energy. It’s important as it promotes normal growth of the body, especially early in life. Sources of niacin include: lean meat, chicken and turkey, seafood, eggs, legumes and fortified cereal.

Lack of niacin can lead to diarrhea and particularly watery (as opposed to fatty) stool. It can also cause mouth sores.

B: along with niacin (B₃) B₅, B₂, B₁ and B₆are required for energy. In patients with cystic fibrosis, a lot of energy is used in fighting lung infections, carrying out airway clearance, and remaining as active as possible. One particularly useful role of vitamin B1, also called thiamine, is that it promotes normal digestion, and appetite. The other B vitamins are essential in helping the body convert carbohydrates (sugars and starches) into usable energy. They also contribute to healthy growth and synthesis of oxygen-carrying red blood cells.

Insufficient levels of vitamin B have been linked to depression and mood swings. Along with depression is a lack of appetite and weight loss. In people with cystic fibrosis it is important to properly assess whether any mood changes are the result of vitamin insufficiency, or if there is something else going on. Weakness and anemia are other symptoms of low vitamin B.

Patients with cystic fibrosis related diabetes should also be sure they are getting adequate amounts of vitamin B₅ (pantothenic acid) in the diet since one symptom of deficiency is low blood sugar. This, combined with a decreased ability to fight infection can cause instable blood sugar levels.

Studies have shown that the leading cause of vitamin B₁₂ deficiency is caused by an inability to properly absorb the vitamin in the intestinal tract. This is largely the case in patients with cystic fibrosis. The thick, sticky mucus in the digestive tract is what keeps the small intestine from absorbing B vitamins.

C: ascorbic acid, found mostly in citrus fruits, melons and other brightly colored fruits and vegetables, was once considered a panacea for the common cold. Though its cure-all reputation has been debunked, there’s no denying the benefits of vitamin C. The body uses this vitamin to strengthen the walls of blood vessels and prevent atherosclerosis or hardening of the veins—good news for patients who need PICC lines or midlines.

Signs of vitamin C deficiency include: loose teeth, bleeding gums, poor or decreased appetite and the inability to heal from a simple infection. Since vitamin C increases iron absorption, extremely low levels of vitamin C can lead to internal bleeding or massive hemoptysis.

Pets and Asthma

2008-03-30T21:11:00.000-07:00

Asthmatics are often discouraged from keeping certain types of pets. Determining whether the pet is a problem for the asthmatic involves knowing specifically which allergens trigger an asthma attack. Asthma attacks are most often brought on by tiny airborne contaminants or allergens, such as pet dander. Some people with asthma have no trouble at all with the animal's fur or dander, however their symptoms may flare up due to what the pet brings into the home.

Pets that have the opportunity to be outdoors inadvertently track in environmental allergens. Small amounts of dust, pollen or spores may cling to the animal's coat. When the pet grooms or is stroked by its owner, these allergens have the potential to bring about bronchospasms.

Indoor pets or low dander animals are recommended for asthmatics. Although no animal can be truly hypo-allergenic, hairless or short-haired breeds of cats and dogs are generally preferred because they do not shed as much. Additionally, certain types of birds, such as the cockatoo, produce a fine powder on their bodies which can aggravate a person's symptoms of asthma or allergies.

An individual's quality of life and the enhancement of joy as a pet owner may take precedence over certain manageable health risks.

Understanding B. Cepacia

2008-03-28T20:01:00.000-07:00

People with cystic fibrosis are prone to a variety of lung infections. Bacteria can easily take hold in the thick, sticky mucus of the airways and begin to grow. Different types of bacteria may only cause occasional flare up for some people with CF, while others may develop chronic infection. In the clinical setting such as hospitalization or office visits, strict anti-infection measures must be implemented in order to prevent the spread of resistant forms of bacteria. The most devastating type of resistant bacteria is a species identified as Burkholderia cepacia (B. cepacia.) This persistent microorganism can occasionally be spread from person to person or object to person. This is of great concern because of the limited number of antibiotics available to fight this bacteria.

History and Epidemics of B. Cepacia

Burkholderia (previously known as Pseudomonas) cepacia, a nutritionally versatile, gram-negative organism, was first described in 1949 by Walter Burkholder of Cornell University, as the phytopathogen responsible for a bacterial rot of onions. Ironically, B. cepacia is now being considered by agricultural microbiologists as an agent to promote crop growth.

The threat of B. cepacia infection led to severe control measures. The popular summer camps like "Camp Pillfeather" were closed once doctors discovered how easily cepacia could spread from person to person. Even transplant centers ceased to accept B. cepacia–infected CF patients as transplant candidates. At first doctors assumed that all species (aka genomovars) of cepacia were equally dangerous, having high morbidity and mortality rates.

In reality transmissibility varies markedly from strain to strain, and that most strains don't cause epidemics, but seem to be relegated to individual cases. Many people with cepacia aren't even sure how they first contracted it. One bit of positive information is that lack of transmission of some strains has also been observed between siblings with CF.

Numerous CF-associated B. cepacia epidemics have now been described, and the epidemic strains have been characterized. One particular highly transmissible strain, epidemically spread within and between CF centers on both sides of the Atlantic, carries the cblA gene. This cblA+ strain has spread across Canada and has now been isolated in 50% of CF centers in the United Kingdom. Another strain of B. cepacia has spread among CF centers in four regions of France.

However, it has become clear that transmissibility varies markedly from strain to strain, and that most strains are not involved in epidemics but appear to be independently acquired and show no evidence of transmission. For example, in 8 years no cases of transmission were detected at the University of North Carolina CF center, despite clinical and social contact between patients and the absence of stringent infection control measures. Independent acquisition of B. cepacia with no evidence of transmission between CF patients was also reported from Denmark.

Shedding Light on B. Cepacia

In the CF community, this exceptionally resistent bacteria is a bone of contention for patients and healthcare professionals alike. Strict adherence to cross-infection avoidance protocols must be a priority. The gravity of the potential probelms for CF patients cannot be denied. However, many patients and their caregivers seem to have almost an irrational fear of being exposed to cepacia.

Because of the different genomovars (i.e. species) of cepacia, it is impossible to predict how an individual's lung function and overall health will be affected once cepacia has been cultured. For some people, decline is rapid and devastating. Others (like myself) may experience some degree of loss of lung function and become more susceptible to other infections.

Most of the anxiety about cepacia stems from the basic reality that cepacia is highly transmissible from patient to patient(1). However, not every species has the same transmissiblity characteristics.

The CFF patient registry tracks approximately 23,000 patients with cystic fibrosis who receive care at CF Centers throughout the country. Overall, only 3-5% of CF patients culture cepacia in any form. This translates to anywhere from 690 to 1150 patients who culture cepacia. In the United States there are 115 care centers. Just from rouhg estimation, this could mean that only 10 patients at each care center have cepacia. Keep in mind that not all of them are present each time the clinic holds appointments, and not all of them continually culture cepacia.

I am not intending to downplay the seriousness of cepacia, nor am I expressing the idea that precautions should be ignored. There are epidemic strains of cepacia. According to an academic paper published in November, 2003 on the study of cepacia in the UK (2)

"Minimising the risk of transmission of this organism from one individual to another requires a multifactorial approach. The polymerase chain reaction [PCR] has allowed for earlier detection of this organism in the clinical progression of the infection, which may allow for earlier and more aggressive intervention with appropriate antibiotics. In addition, such molecular diagnostic approaches may help by giving direction to segregation policies, thus minimising transmission in both the in-patient and the out-patient setting. Most importantly perhaps is vigilance on the part of the CF patient to adhere to infection control practices. Although tedious and socially difficult to maintain properly, these practices do work. " (Emphasis mine)

The more common pathogens of concern in CF clinics are those such as Pseudomonas aerginosa, and various staph infections. I am confident in my doctors' ability to execute proper infection control measures that protect me and others with CF. It's the rest of the world I worry about--I find myself far more likely to catch a nasty bug from the world beyond my health care center's walls. Public places like movie theatres, swimming pools, shopping malls, etc. pose a greater risk to my overall health.

Some Tips on "Germ Etiquette"

The best way to prevent infection is good hand washing with soap and water.
Whenever you interact with someone at a doctor's office or hospital, it's best to wash your hands before and after contact. CF doctors are usually very good about washing their hands. As a CF patient it is your right to insist that your nurses wash their hands as well. Not all medical professionals or caregivers understand just how critical this is for people with CF. Hand washing should be done before and after contact with others, or contact with objects or surfaces that have become contaminated with respiratory secretions. Not only do patients have to wash their hands, but nurses, doctors, therapists and aides do as well.

Use common sense and practice good hygiene when in the clinic or hospital.
Cough into your shoulder or the crook of your elbow. Some CF clinics ask that people who have a cough should wear a mask. Patients with cepacia should also be courteous to wear gloves and maintain a minimal distance of 3 feet away from other patients. CF Clinics recommend that patients with cepacia have either the first or last appointment of the clinic so that they are not posing an exposure risk. Another way to protect yourself from any lingering bacteria on surfaces is to avoid using your hands to touch things. Shut the water faucet off with your elbow. Push the elevator button with your knuckle instead of your fingertip.

For more information be sure to visit the Cystic Fibrosis Foundation's B. Cepacia information page.

Sources and Related Articles:

-
(1) source: Prevention of Cross Infection in cystic fibrosis. Claus Moser, MD, PhD & Neils Hoiby, MD
(2) source: Burkholderia cepacia and cystic fibrosis. John E. Moor & J. Stuart Elborn. November, 2003. Northern Ireland Public Health Library.

ABC News: Living Dangerously With Cystic Fibrosis

B. Cepacia and Cystic Fibrosis

Agricultural Use of B. Cepacia: A Threat to Human Health?

Infection of B. Cepacia in Cystic Fibrosis Patients

Nutrition, Exercise and Well Being

2008-03-25T03:40:00.000-07:00

Lungs aren’t the only organs affected by the thick, sticky mucus that cystic fibrosis produces. The pancreas, an exocrine gland that normally secretes digestive enzymes necessary to absorb nutrients from food, can be coated with mucus as well. In fact, nearly 85% of CF patients demonstrate pancreatic insufficiency before age 10. This can lead to issues of malabsorption and malnutrition due to the body’s inability to break down fats, carbohydrates and protein before they pass into the intestinal system.

Nutrition and Calories

In order to remedy this problem, many CF patients are prescribed digestive enzyme supplements to help the body better absorb the vitamins and minerals that are present in the diet. However, digestive enzymes are only part of the solution. A person with CF needs to incorporate more calories into their diet than other healthy people the same age. Important ways that you can help your child with CF grow healthy and strong include providing good overall nutrition along with extra fat and calories as well as prescribed enzymes.

Table 1. Caloric Intake Ranges Based on Age Group and Activity

It is important for everyone with CF to combine good nutrition with appropriate exercise. One of the challenges that await school-aged children upon returning to the classroom is that eating habits may need to change. For example, a child who has been able to “graze” frequently during the day may have trouble adjusting to the pre-determined eating schedule set by the school or classroom.

Another problem that may indirectly affect CF students is the obesity epidemic. Many schools are adopting low-calorie, low-fat meal programs in an attempt to address the alarming rise in the number of overweight and obese children. If your child is participating in a school lunch program, be sure that the meal offers the appropriate amount of calories for your child’s age and activity level.

On school days, offer your child or young adult a large, balanced breakfast. Meals that include complex carbohydrates (e.g. whole grain toast) take longer for the body to break down and provide energy for a longer period of time. You the school nurse, who in turn may speak with your child’s teacher or the school administration, to permit additional snack breaks as necessary.

Correlating Nutrition and Lung Health

The need for adequate nutrition cannot be stressed enough. The Cystic Fibrosis Foundation (CFF) has identified a correlation between nutritional health and lung health. Studies have shown that patients with better Body Mass Index (BMI) values for their age, height, and weight, typically perform better on pulmonary function tests. Many CF physicians stress the importance of patients reaching healthy weight and BMI goals before age 6 in order to reduce the likelihood of hospitalizations due to pulmonary exacerbations.

Certain sports related activities, such as running and jumping, generate vibrations in the chest cavity which may stimulate coughing. Coughing should never be suppressed, because it is how the body rids itself of infection-prone mucus in the lungs. Getting the body moving acts as a natural form of chest physiotherapy.

Exercise is also an effective way to prevent infection and inflammation of the lungs. Scientific studies have demonstrated that physical exercise is just as important as chest physiotherapy. Any sort of activity that forces the respiration rate to increase will naturally strengthen the muscles used to cough. Strong back, chest, and abdominal muscles that are well toned from exercise can make coughing easier and less exhausting. Aerobic (oxygen using) and cardio-pulmonary exercise is particularly important for people with CF. As the heart rate increases, the rate of blood flow increases, thereby delivering vital oxygen throughout the body.

Although it may be tempting for parents of students with CF to want their child to opt-out of physical education class, this is not a good idea. The school nurse should speak with coaches and PE teachers about the importance of exercise and its benefit to the student with CF. Students with CF should be encouraged to participate as much as they are able in sports or other fitness activities. If outdoor activity aggravates allergy symptoms, look for other ways to encourage rigorous cardio-pulmonary activity.

Community classes in martial arts, dance, or gymnastics may be an alternative. Another option is to have your student enroll in the school’s band or orchestra. Wind instruments such as the clarinet, trombone, trumpet or French horn are excellent for improving and maintaining lung function. This can also open the doors to scholarship opportunities when your student is ready to apply for college.

The combination of nutrition and exercise can lead to lasting benefits for all ages of those with cystic fibrosis. Many CF Team Clinics have nutritionists and/or dietitians on staff to help determine the best way to incorporate adequate nutrition and exercise into the busy lives of CF patients and their care givers.

How To Be a Good Patient

2008-03-21T11:31:00.001-07:00

Doctors, nurses and respiratory therapists agree that when it comes to being a good patient, individuals who have cystic fibrosis tend to lead the pack. Although they develop some of their “patient personality” largely out of necessity, there’s no question about their ability to face the challenge of CF with every ounce of dedication and diligence they have to give.

Good patients typically embody the following qualities:

Actively involved
Self-awareness
Inquisitiveness
Willingness to learn
Compliant

-Active Involvement-

Being actively involved in your own health care is just one of many ways to take back some control in what is largely uncontrollable. You may not be able to avoid catching a bacteria or virus from the world at large, but you can be proactive in remedying it. A patient who is actively involved in their care understands the importance of making appointments in advance even when they don’t feel particularly sick. They know that managing CF requires ongoing monitoring of their status, particularly when it comes to charting the results of pulmonary function tests (PFTs) and sputum cultures. An actively involved patient understands the value of “an ounce of prevention.”

Good patients who are actively involved in what their doctors are doing, often bring along a supportive friend or family member to their clinic appointments. A second set of ears to hear what the doctors have to say is a good way to make sure you don’t miss anything important or new. Active involvement may also involve bringing a notebook to each appointment to record the doctor’s instructions and recommendations. Making lists of questions for the doctor ahead of time, as well as a list of any medications that need refills are things that good patients do to stay on top of things.

-Self-Awareness-

Similar to being actively involved in your health care program, you must be self-aware. By learning what constitutes your particular “base line” or “status quo” you will be more adept at recognizing when things are starting to decline. For example, if your cough is usually dry and tight but starts to become wetter and more productive, you get on the phone to schedule an appointment at the CF Clinic.

Another aspect of self-awareness is that you have a healthy attitude about your limitations because of CF. You are able to live life to the fullest, but do so without exhausting yourself or putting yourself in harm’s way. A good patient who is self-aware knows how to set appropriate boundaries to keep friends and even family from pressuring you into giving too much of yourself.

Patients who are self-aware are honest with their doctors about the non-physical aspects of life with CF. They understand that the emotional burden that comes with having a chronic condition like CF is sometimes too much for one person to bear. A good patient asks for help when they feel overwhelmed.

-Inquisitiveness-

Good patients ask questions and expect answers. They ask medical professionals about any new treatments or medications they’ve heard about or read about online. An inquisitive nature not only helps a CF patient understand his or her own body, but enables them to gain clearer understanding of what the doctors are doing and why.

An inquisitive patient does not assume that s/he has all the answers. Patients who are inquisitive don’t assume that everything they read on the Internet about CF is true or applicable to their individual situations. They bring their questions to the professionals and ask for clarification. They respect the doctor’s expertise and training, and ask questions about things they don’t understand. A good patient is not afraid to ask a doctor to explain things more than once. After all, cystic fibrosis has so many facets, it’s unreasonable to expect to learn it all in a short clinic appointment.

-Willingness to Learn-

When a doctor recommends a new course of treatment or a new therapy, a good patient is willing to learn to adapt. Instead of refusing to try something unfamiliar, they give it a fair chance before making a final decision. For example, if your doctor recommends that it’s time to get a port-a-cath instead of a PICC line, a good patient will be willing to learn why this is so. Similarly, if a patient is diagnosed with cystic fibrosis related diabetes (CFRD), the willingness to learn how to manage the new condition should override any desire to complain or become depressed at the news.

Although there is certainly a time and a place for complaining and “venting” about the struggle of life with CF, a patient with a healthy outlook and willingness to learn will be able to successfully balance it all with a bit of help. Patients who are willing to learn are not afraid to step outside their comfort zones and learn how to let other people help them with things.

-Compliant-

Rarely are cystic fibrosis patients 100% compliant with all their treatments and medications, though there are some who set the bar quite high! Compliant patients stand a much greater chance of finding a sense of peace and acceptance of their disease. Patients who are compliant can rest assured to know that they are doing everything within their power to make their lives as full and beautiful as possible, even with CF.

Doctors appreciate their patients who are compliant, because it makes their jobs a bit easier. For example, if a patient is compliant to take their enzymes as directed suddenly begins to have a change in their bowel movements, the doctor will know that either the diet has changed or the enzymes are no longer effective at that particular strength. Less time is wasted in trying to find an appropriate solution.

Compliant patients keep their appointments. They understand the need for preventive care in treating cystic fibrosis, and they take every opportunity to monitor their health.

Managing life with cystic fibrosis can be tough, but it doesn’t need to be overwhelming and depressing. If you work on being a good patient, chances are you’ll cope with the disease much better.

Understanding Meconium Ileus

2008-03-21T11:24:00.000-07:00

Meconium ileus is one of the prevalent first signs of cystic fibrosis in infants. 1 in 5 people with cystic fibrosis have undergone treatment for meconium ileus. Meconium refers to a baby’s first bowel movement. Often it is greenish in color, and has built up in the infant’s bowels prior to birth. Normally this material is expelled early on. In babies with CF however, the thick, sticky mucus present in the intestines prevents the thick, tar-like meconium from moving. It remains in the bowel, causing an obstruction and a bloated abdomen. In many cases surgery is required to remove the blockage.

The blockage, which accumulates at the lower end of the small intestine (i.e., the ileum) must be removed to prevent damage to the bowel. Treatment for meconium ileus depends on the severity of the blockage. Early detection using an x-ray or CT scan helps determine whether treatment will require surgery or simply a strong course of mucolytics.

-What is an Ileostomy?-

Treating meconium ileus with surgery requires that the blocked portion of the intestine be removed. In order for them to heal properly from the procedure, an ileostomy must be done. An ileostomy is the technical term for the surgery in which an opening in the abdomen, called a “stoma” is created. The ileus, which is the end portion of the small intestine, is brought through the stoma. A small bag is placed at the site and taped to the skin. Fecal material is excreted into the bag instead of at the anus.

Once the intestine heals and blockage is no longer a risk, another surgery called an “ostomy reversal” is done to put everything back together inside the body. The first bowel movements upon reversal are quite frequent. It’s normal to have several per day, possibly in excess of 10.

-Life After Ostomy-

CF patients who have undergone an ileostomy and reversal must consider carefully the foods they eat. Failure to absorb nutrients and vitamins from food is a common problem for all CF patients who are pancreatic insufficient. Those who have had bowel surgeries should take extra care to make sure they eat enough calories per day to off-set the nutritional loss that occurs as the result of frequent stools.

Maintaining adequate hydration is also key, since people who have had intestinal blockages in the past are most likely to experience them again.

Different Types of Coughs

2008-03-19T17:42:00.000-07:00

Whether you've got one yourself or are sharing an elevator with someone who has one, a cough is an unwelcome disturbance. But did you know that not all coughs are alike, and not all people who cough are contagious? Let's consider the different types of coughs, ways to remedy them, and how to identify whether a simple cough may be something more sinister.

Whooping Cough

Common mostly in children and toddlers, whooping cough is a highly contagious condition caused by a bacterial infection. It is characterized by a long coughing fit, during which the sufferer cannot draw in an adequate breath. The coughing may be so hard that it causes the person to shake or spasm. In some instances the blood vessels of the eyes may break due to the intense effort of the cough, or the coughing spasm turns into dry heaving or vomiting.

Chronic Cough

People with certain health conditions or diseases such as cystic fibrosis, chronic obstructive pulmonary disease (COPD), emphysema, bronchiectasis, or bronchitis tend to cough involuntarily. This is the body's response to inflamed or otherwise blocked airways, and its attempt to exchange oxygen. A chronic cough may sound dry or raspy as in the case of COPD, or can be wet-sounding and fully of phlegm as sometimes happens for people with cystic fibrosis.

Asthma and Allergies

Asthmatics tend to have a dry, non-productive cough accompanied by a tight wheeze or crackling sound. Asthmatics and allergy suffers alike experience coughing spells when their airways become constricted in response to a trigger such as airborne pollutants, pollen, animal dander, cigarette smoke or products containing fragrance. This is most often remedied with a short acting bronchodilator in the form of an inhaler, or can be treated with longer acting corticosteroids.

Remedies

Determining the appropriate cough remedy depends on the underlying reason for the cough. Cough associated with the common cold is usually caused by post-nasal drip. Throat lozenges and sprays containing menthol are effective in soothing the throat. Over-the-counter (OTC) medications like pseudoephedrine (Sudafed) help dry up the mucus in the sinuses and eliminate post-nasal drip.

Homeopathic remedies such as taking a hot shower with a lot of steam can help thin and loosen mucus in the chest. Inhaling a vapor mist from a humidifier also works well for some people, especially those suffering from a common cold. Sipping hot tea in flavors like vanilla, peppermint, or chamomile is a relaxing and calming way to sooth your throat when you have a cough.

Hydrocodone cough syrup is a strong cough suppressant. Not only does it calm your body's urge to cough, it can relieve the pain of an aching ribcage or strained abdominal muscles that occur with hard coughing. Hydrocodone cough suppressant requires a prescription from a doctor, and should be taken exactly as prescribed. Because it depresses your body’s respiration rate, patients with cystic fibrosis should use products containing hydrocodone with caution.

Other OTC cough syrups or even pills include an expectorant. Expectorants are useful to thin mucus secretions, making them easier to cough out. This is important especially in cases where the cough is a symptom of a respiratory infection. An expectorant combined with a mucolytic is a good option for people with cystic fibrosis not only because it helps break up the mucus, but it makes coughing it out that much easier. Look for products that contain the ingredient guaifenesin. Like hyptertonic saline, it will draw water into the lung tissue, making thick mucus less likely to “stick around” so to speak.

When to Seek Medical Attention

Not all coughs require medical attention. However, there are some serious conditions in which a health care professional should be contacted. These include:

-an atypical cough that lasts longer than 10 days

-a previously diagnosed cough changes in nature

-secretions have changed in color and appear more yellow or green

-blood is present in secretions or only blood is being coughed up (hemoptysis)

-cough is followed by a severe sharp pain on one side of the chest (could be a collapsed lung, i.e., pneumothorax)

Spotlight On: ShopforCF.com

2008-02-21T21:50:00.000-08:00

There's nothing that inspires me more to be active and compassionate in the CF community than to see others with CF doing great things. I'd like to tell you about a website that is doing just that. ShopforCF.com is just one recent example of one person's commitment and drive to do something positive for the CF community. Ronnie, a cystic fibrosis patient in Arizona, started the site as a way to raise money for cystic fibrosis research.

Here's what Ronnie has to say about the site's goal and purpose:

The sole purpose of this website is to raise funds for Cystic Fibrosis research, so people that live with the disease might have a chance to have a fuller, longer life. The way this happens is through money donated to the Cystic Fibrosis Foundation who in turn supplies pharmaceutical companies with the necessary grants and funds to continue vital research. The ultimate goal of course being, to find a cure for this disease that affects roughly 35,000 patients.

The cost of life-saving research is high, and so are the stakes. The Cystic Fibrosis Foundation is the leading and nearly sole provider of the funds needed for CF research. Sources estimate that just 5 minutes of research costs $1,200. That's a mighty steep bill when you consider the length of time it takes to develop a new product and make it available to cystic fibrosis patients ShopforCF.com is helping put a few more pennies in that piggy bank with each transaction initiated through its site.

100 percent, as in ALL of the proceeds generated by ShopforCF.com are donated directly to the Cystic Fibrosis Foundation. There's zero effort on your part. All you have to do is shop online as you normally would availing yourself of the links on the ShopforCF.com site. I know it's tax season and the economy isn't booming, but please consider spending your money on ShopforCF.com before you use another online retailer. You'll feel good about your purchase, knowing that you have helped improved the quality of life for me, Ronnie, and the thousands of CF patients worldwide.

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Understanding Zinc

2008-02-18T11:11:00.000-08:00

Zinc is one of the essential nutrients that the body needs in order to function properly. Found mostly in red meats and other high protein foods, zinc is required in small amounts for the body to not only carry out vital chemical reactions, but also to promote a healthy immune system.

-Zinc in the Diet-

As a dietary supplement, the benefits of zinc are very closely tied to the body's requirement and use of vitamin A. For example, both zinc and vitamin A are useful to promote good eyesight, particularly night vision. Also, zinc and vitamin A are required by red blood cells in order for the body to heal itself from wounds or infections. Scientists and medical professionals estimate that there are no fewer than 3,000 proteins in the body that rely on zinc to carry out their normal functions. Zinc works in the body by activating a type of protein called enzymes. When the human body has adequate amounts of zinc, certain types of the body's cells will secrete a small amount of a zinc ion that works as a signal to stimulate a specific biological process.

Oysters are considered one of the best sources of zinc. However, for people with allergies to shellfish this is impractical. Other foods that promote good zinc absorption include:

Lean beef
Fortified breakfast cereal
Chicken (dark meat)
Yogurt
Nuts and legumes

-Zinc Deficiency-

If the body is deficient in zinc any of the following symptoms may occur:

Diarrhea
Hair loss
Diminished eyesight
Poor memory
Organ malfunction (particularly the liver)
Poor appetite
Bleeding disorders
Congenital defects (caused by pregnant women who are zinc deficient)

-Who Needs Zinc?-

Preventing zinc deficiency is easy done by ensuring that the diet is rich in foods that are known to have adequate levels of zinc. Other risk factors for developing zinc deficiency include alcoholism (which impairs the liver) and conditions like Chron's disease, Cystic Fibrosis or Colitis which may lead to frequent bouts of diarrhea. The recommended daily intake of zinc for adults is actually quite small. Males and females respectively only need 11mg and 8mg per day.

-Zinc and Colds-

There is some anecdotal evidence suggesting that zinc can help the body fend off the common cold. However, since colds are caused by a virus (specifically a rhinovirus), the best course of treatment for a cold is to try to alleviate the symptoms and just wait for it to run its course. Products such as Cold-Eeze lozenges, may help some people, particularly those who are harder hit by the cold due to being immunocompromised and already zinc deficient; however, such products cannot truly be relied upon as a means to speed up the healing process from a cold.

Understanding Vitamin A

2008-02-17T12:22:00.001-08:00

Everyone knows that vitamins are important for good health. Of the 13 essential vitamins, the human body can only manufacture a small portion of these. The rest must come from the diet or, in some cases, supplementation. But what exactly are the benefits of these vitamins and how much of it do we need to be healthy? In particular, what are the benefits of Vitamin A, and what are its sources?

As an antioxidant, vitamin A prevents cell damage caused by free radials and may be helpful in preventing some types of cancer. It is believed that vitamin A may help protect against lung cancer. Vitamin A has also played a part in helping heal and prevent acne. The potent acne medication, Accutane (isotretinoin), is a derivative of vitamin A.

Good sources of the retinol form of vitamin A in the diet include the following:

Cheeses
Egg yolks
Fortified whole milk
Liver
Salmon

Retinol plays many important roles in the body. It prevents night blindness and is essential for good eye health. Without retinol, cell division and cell growth cannot proceed normally. Bones and teeth need the proper amounts of vitamin A in order to develop properly. Other parts of the body, such as the tissue that lines intestines and airways also require retinol.

Vitamin A is a fat soluble vitamin that exists in multiple forms. Retinol, retinoic acid, and retinyl esters are stand-alone forms. This means that they are readily available in foods in their current form, and do not need to be combined with other minerals in order to be effective. The two types of vitamin a are retinols and beta carotene. Most of the time Vitamin A is referred to interchangeably with either of these terms. Vitamin A in the diet comes from two main sources: animal products and plants. Retinols are obtained from animal products, whereas the form beta carotene comes from plants such as yellow and orange vegetables.

Beta carotene is abundant in the following plant sources:

Broccoli
Carrots
Cantaloupe
Pumpkins
Spinach
Yellow squash
Yams

Healthy adults only need 800-1000 micrograms of vitamin A on a daily basis. Too much vitamin A can cause headaches and vision problems, joint aches, loss of appetite, and skin problems. Women who are pregnant should not take vitamin A supplements, since excessive amounts of retinol have been linked to birth defects.

Recognizing the Dangers of MRSA and CAMRSA

2008-02-17T12:11:00.000-08:00

Methicillin Resistant Staphylococcus Aureus ("MRSA") is a type of bacteria that occurs naturally in approximately 1 in 4 healthy individuals. In the general population, Staph. Aureus is harmless. It stays mostly in a person's nasal passages or on their skin. Occasionally, the bacteria becomes exceptionally resistant to the antibiotics that would normally work well to stem the tide of infection. When this happens, MRSA becomes increasingly resistant to a particular family of antibiotics called beta-lactams. When resistance occurs, commonly prescribed medications such as Meropenem, which would normally wipe out the infection with ease, are rendered ineffective against MRSA.

MRSA is spread when someone with an active Staph. Aureus infection comes into direct contact with someone who is immuno-supressed. Because of the body's inability to ward off infection, Staph is able to take hold and begin to multiply rapidly. Hospitals are often to blame when a patient contracts MRSA. In the cystic fibrosis community, the MRSA may be acquired through indirect contact with CF patients who are colonized with MRSA; for example their bedding, curtains, doctors' coats, nurses scrubs, etc. Community Acquired-MRSA ("CA-MRSA") infections can cause the same type of infections as other strains of Staph. Studies conducted in Minnesota have found that CA-MRSA is more likely to cause skin and soft tissue infections and that healthcare-associated MRSA is more likely to be found in sputum or urine.

The good news about MRSA is that patients who are colonized do not exhibit deterioration in lung function as a result. Patients who are MRSA-positive will more often than not have MRSA in their sputum, even when they're not experiencing an exacerbation due to infection. Additionally, CA-MRSA bacteria are usually susceptible to more types of antibiotics than are healthcare-associated strains of MRSA.

Recently, a newer, stronger, more resistant strain of Staph. Aureus has been identified by the Centers for Disease Control. CA-MRSA is a huge public health threat.

How Worried Should I Be?

Some people can culture CA-MRSA without ever presenting an infection. This is because staph occurs naturally in the body. The CDC reports that only 1% of the population is infected with CA-MRSA. According to the 2005 Cystic Fibrosis Patient Registry Report, the overall percentage of patients with MRSA is 17%. CF patients between the ages of 11 and 17 had the highest number of occurrences of infection. A likely explanation is that people in that particular demographic are not as fastidious about personal hygiene.

Taking reasonable precautions to prevent infection is always in the best interest of a person with cystic fibrosis. The easiest ways to protect yourself from CA-MRSA are as follows:

1. Keep your hands clean by washing thoroughly with soap and water or using an alcohol-based hand sanitizer.
2. Keep cuts and scrapes clean and covered with a bandage until healed.
3. Avoid contact with other people's wounds or bandages.
4. Avoid sharing personal items such as towels or razors.

Adhering to a strict cross-infection control plan when in the presence of other cystic fibrosis patients is also encouraged. When sitting in the waiting room for a clinic appointment, keep a minimum distance of 3 feet between yourself and others. Always cover your mouth when coughing, or wear gloves and a mask.

The effects of CA-MRSA can be devastating within a short period of time. It has the potential to destroy healthy tissue very rapidly. Cystic fibrosis patients especially should be careful to steer clear of people who are coughing or sneezing repeatedly. It's important not to ignore symptoms that may indicate infection.

Symptoms of CA-MRSA

Some people can be colonized with Staph. Aureus yet never suffer from an infection. In many cases, exposure to MRSA will not result in symptoms of disease for long periods of time. Unlike MRSA, people infected with CA-MRSA will exhibit symptoms almost immediately or within a period of a few days.

Symptoms of MRSA include:

* Abscesses at the site of broken skin
* Pustules or other pockets of pus that resemble boils or pimples
* Redness, pain and swelling at the site of presumed infection

In severe cases, CA-MRSA can manifest as serious infections such as:

* Pneumonia
* difficulty breathing
* fever or chills

It's always best to contact your physician if you or your child exhibits a rapid onset of the above mentioned symptoms.

CA-MRSA at School

Younger children should be instructed of the importance of hand-washing. Teach them to wash with warm water and soap. Singing the alphabet song (30-45 seconds) while washing is a good way for them to remember how long they should rinse. Be sure to tell your child with CF to keep his or her hands to herself on the school bus. Cuts, scrapes, and wounds should be covered with a bandage, and not picked at.

Gym class is where CA-MRSA is most likely to raise its ugly head. Locker rooms and showers are warm, moist environments where bacteria like to thrive. College students who live in crowded dorms should also be careful to limit their exposure to areas where staph may like to grow.

In the world not-restricted by the walls of a classroom, there are other ways to lower your risk of contracting CA-MRSA. Use your knuckles instead of your fingertip when pressing elevator or ATM buttons. Keep a small bottle of hand sanitizer or disinfectant wipes in your purse, briefcase or glove compartment, and use whenever necessary.

How is it Treated?

Most skin infections resolve without treatment. Other infections, however, require incision and drainage or antibiotic treatment to cure the infection. Skin infections that are left untreated can develop into more serious life-threatening infections such as infections of the bone or blood. More serious types of Staph. Aureus infections (such as pneumonia or bloodstream infections) typically require hospitalization and treatment with intravenous antibiotics. Although CA-MRSA and MRSA are difficult to eradicate once a person has become infected, a combination of drugs has demonstrated effectiveness. Vancomycin, or a similar medication can be prescribed. There is a possibility for longer lasting or more severe infections with CA-MRSA if the initial antibiotic prescribed is not capable of killing the bacteria. There is some evidence that preventive therapy with flucloxacillin is useful. Additionally Chloramphenicol and clindamycin tend to have activity against CA-MRSA.

The Choice for Children

2008-01-26T13:10:00.000-08:00

For decades women with cystic fibrosis were told not only that they shouldn’t expect to have children, but that they possibly couldn’t even withstand a full term pregnancy. Much has changed in recent years, particularly in regard to the mean survival age of patients with CF being nearly 37 years. Even so, the choice for children is a matter that weighs heavily on the hearts and minds of couples with cystic fibrosis.

Some women with CF feel very strongly about bearing biological children. This decision is just one of a myriad of difficult ones that adults with CF face. Discussing these things with the CF specialists, not to mention a potential spouse, should take place frequently and candidly. Matters of family planning and contraception are best not left to chance. Depending on a couple’s moral or religious convictions, discussions with their clergy or other leader in their religious community may help the decision process.

The experiences and situations of families in which women with CF are becoming mothers are every bit as diverse as the families themselves.

Couples who decide not to have biological children or even to adopt, face just as many emotional challenges as their parenting peers. Coming to terms with the limitations of a couple’s physical, mental, and emotional resources takes a great deal of introspection and self-awareness. One of the most important things to keep in mind is that there is such a thing as a two person family.

Men with cystic fibrosis are up against a different challenge when it comes to fatherhood. Many cannot father a child without medical intervention, and may need to consider different alternatives with their wives.

People with cystic fibrosis carry a great deal of weight upon their shoulders when they consider the biological ramifications of bearing children. There are those who believe it is simply unethical to produce children who will have a high likelihood of testing positive for cystic fibrosis, or will undoubtedly be carriers of the deadly gene. Others believe that by curtailing reproduction, God is affronted.

No matter which decision a couple with cystic fibrosis makes, friends, family, and even the CF team, should respond with grace and respect. Living well with cystic fibrosis requires positive input and support from many sources, all of which promotes better quality of life.

Therapies that Use Positive Expiratory Pressure (PEP)

2008-01-25T17:09:00.000-08:00

Cystic fibrosis patients have difficulty clearing the mucus from their lungs not only because the mucus itself is so sticky, but because the airways are inflamed or otherwise tightened. Inflammation may be from bronchiectasis, bacterial infection, allergies or pneumonia. Clearing the airways is much better accomplished when the bronchi are less inflamed and constricted.

Therapies involving Positive Expiratory Pressure (PEP) are designed to keep the airways opened when the patient breathes out. There are a few different PEP devices, each having their own limitations and benefits. The way that PEP therapy works is that it keeps the lungs inflated with air. Basic science tells us that two things cannot occupy the same space. In people with cystic fibrosis, the mucus occupies precious space in the airways, preventing them from taking a full, deep breath.

PEP devices are either stand-alone, or can be used with a nebulizer. All PEP devices work by having a one way valve that creates resistance when the patient breathes out against it. With PEP therapy, the pressure difference that occurs when a patient breathes out allows the lungs to fill with additional air, which in turn will push the mucus out as the lungs try to return to an equilibrant volume.

In simpler terms, PEP therapy functions very similarly to trying to get mustard out of a squeeze bottle. If you were to just squeeze the bottle without shaking it, you would only get a puff of air, and that little bit of watery mustard (which I call a “musquirt”.) When you shake the bottle, you move the amount of air in the bottle to the bottom, which will force the mustard out when you squeeze.

PEP therapy is very effective when combined with postural drainage. However, the postural drainage positions, especially the ones in which a patient must lie face down, may make breathing against a PEP device too difficult. Some PEP therapies combine vibratory pressure, which helps loosen the mucus and dislodge it from the linings of the airways. The Flutter ®, Acapella ® and the Quake ® use a combination of vibrations and PEP to promote airway clearance.

Below are some pictures of different types of PEP therapy:

1) PEP mask

2) Acapella

3) Flutter

4) Quake

5) PEP attachment with nebulizer

Postural Drainage and Chest Physiotherapy

2008-01-24T20:09:00.000-08:00

From mist tents to vibrating air-filled vests, the world of airway clearance therapy for cystic fibrosis patients has undergone many changes over the last few decades. Their underlying goal is the same: stimulate the lungs to move as much sticky mucus out of the airways as possible. Patients with cystic fibrosis who are old enough to remember the “olden days” of chest physiotherapy (CPT) are excited about the latest advancements, but wish they had come so much sooner. Some respiratory therapists and CF patients themselves, still insist on utilizing the more archaic therapies, mostly because of familiarity and routine. Newer medical technology is of course, expensive, and the high cost of obtaining certain airway clearance devices poses a distinct barrier to patients in need. This next few articles outline the various types of mainstream CPT methods over the years.

Postural Drainage and CPT

Also called bronchial drainage, this refers to the positions that use of gravity to aid in the mucus clearance. In order to be effective, the head must be lower than the chest. This allows the mucus to flow toward the trachea, where it can be more easily coughed out. Different postures are useful for targeting different regions in the lungs. These 6 positions are to be used in a systematic manner, working from the upper to the lower lobes. There are a total of 9 regions that can be drained in this manner. Achieving the correct positions can be done using a tilt table, a positional hospital bed, or pillows.

Postural drainage is most effective when combined with chest percussions and vibrations. This is called CPT. It has also been called “clapping” or “thumping.” In years past, the caregiver administering CPT did so by cupping his or her hand, and beating on patient’s back in each of the regions of the lungs. Its effectiveness was almost solely determined by technique. Improper cupping of the hand produced only a slapping sensation, painful to both patient and caregiver. Also, as fatigue set in, the steady, rhythmic pounding would slow down and lose intensity.

To ensure a more uniform cupping, a small rubber device was developed that could be easily gripped by the caregiver. The device looked like the paddles used for air hockey, but had a hollowed out bottom which functioned in the same manner as cupping the hands.

The “clapping method” of CPT, is no longer in widespread use for adults having CF. Certain aspects of postural drainage, however, are still useful for infants. Instead of clapping, however, the caregiver instead taps with two fingers on the different lung regions, while holding the child in the different postural drainage positions.

In place of clapping, which as mentioned before was sometimes fatiguing, a motorized massage/percussion tool is used. Respiratory therapists in hospitals use a professional model, such as the GK3 Postural Drainage and Percussion Massagers (see image below).

The product, which looks quite similar to a car buffer, is available for home use, but costs anywhere from $1,300 to $1700.

Understanding Bowel Movements

2008-01-17T18:58:00.000-08:00

Much to the chagrin and embarrassment of CF patients everywhere, bowel movements are an important part of our lives. In many ways our bowel movements serve as a benchmark that lets us know how things are going, so to speak, with our digestive systems. Cystic fibrosis patients with pancreatic insufficiency should be especially aware of all things related to their digestive process, and be able to identify what’s normal for them and what isn’t.

When the pancreas is too blocked by mucus, it can’t deliver the enzymes necessary to break down the foods we eat, particularly proteins and fats. As a result, people with CF may have more frequent bowel movements because they are not absorbing the protein and fat that they are eating. As if that weren’t embarrassing enough, CF patients are also prone to flatulence (gas). Their gas and BMs can be particularly foul-smelling as the result of proteins and fats not being absorbed by the body.

So how can a person with CF determine what’s a normal BM and what isn’t? Consider these three factors:

1) Color

2) Condition

3) Quantity

COLORS

Brown is generally the “preferred” color of healthy stool, though there may be different variations on that theme.

The liver excretes bile salts into the stool, giving it a normal brown color. Obstruction to the flow of bile out of the liver (you may see the word "cholestasis"), or liver infections like viral hepatitis, may produce clay-colored stools.

Black, tarry stools with a foul odor can be the result of eating certain foods, taking iron supplements, or possibly from internal bleeding. Foods that are dark blue or black in color may also cause black stools. Examples of foods and minerals causing dark stools include: black licorice, blueberries, iron supplements, lead, Pepto Bismol (contains bismuth.)

Green, leafy vegetables contain chlorophyll which could be coloring the stool green. Another possibility is that the stool has passed too quickly through the large intestine and has not yet had all of the bile absorbed. Sometimes newborns have green colored stools, and this is perfectly normal.

Patients with cystic fibrosis often have gall bladder issues, which can cause the stools to appear orange. This is because bile, which normally helps process fats in foods may not be excreted adequately. Stools are usually orange if they contain a lot of oil and fat that the bile salts have not had enough time to break down. A lack of bile (because of bile blockage such as gall stones) can also cause stools to appear orange. Orange, greasy stools are particularly foul-smelling, and may be difficult to control.

CONDITION

Healthy stools should be uniform in shape and consistency. They should sink and not float. Floating stool is evidence that you are not absorbing enough fat from your food.

Loose, watery stools may indicate a stomach virus, especially if they are concurrent with vomiting. In young children especially, it’s important not to let diarrhea lead to dehydration. Dehydration as a result of lost water through the stools may also contribute to intestinal blockage. If the small intestine, which usually adds water to digested food, is unable to do so, the fecalization process cannot take place and the stool can back up into the upper gastrointestinal system.

Stools that are difficult and painful to pass are often the result of not enough fiber in the diet. Patients with cystic fibrosis have to strike a delicate balance between having just enough BMs to prevent bowel obstruction, but not so many that they lose water and vital nutrients.

QUANTITY

Cystic fibrosis patients are encouraged to eat several small meals throughout the day instead of 3 large ones, as a way to avoid painful bloating and stomach pains. This means that for some people, going to the bathroom 6 times a day is just part of life. Some patients with cystic fibrosis are very regular in their bathroom habits, and can accurately predict which foods will go through more quickly than others. Usually it’s the foods that are high in fat content that cause a person to use toilet shortly after eating.

One of the earliest signs of cystic fibrosis is a condition called meconium ileus. Approximately 20% of cystic fibrosis will be diagnosed at birth because of mecomium ileus. Meconium is the material present in the intestines of a newborn. In meconium ileus, intestinal obstruction results from the impaction of thick, tenacious meconium in the small bowel.

What is Bronchiectasis?

2008-01-16T18:24:00.000-08:00

Cystic fibrosis is a chronic condition in which a mutation of the cystic fibrosis trans-membrane regulator (CFTR) protein causes the body to produce abnormally thick, sticky mucus. When this mucus becomes lodged in the respiratory passages, infection is likely to result. Blockage and subsequent infection can cause the airways to become weak and stretched out, scarred, and otherwise deformed. This condition is called bronchiectasis.

Nearly 50% of all reported instances of bronchiectasis are associated with the inflammation and infections associated with cystic fibrosis. Sometimes, the diagnosis of bronchiectasis is made prior to a formal diagnosis of cystic fibrosis. Bronchiectasis can also develop in patients who have allergic bronchopulmonary aspergilliosis (ABPA).

The severity of bronchiectasis is related to which of three types of the condition are present.

1) Fusiform (Cylindrical)

2) Varicose

3) (Saccular) Cystic

Cylindrical forms are often caused by acute bronchitis or other lung infection. In this form, the respiratory passages are only slightly widened, similar to how a garden hose would expand when filled with water. This type of bronchiectasis can be reversed, as there are no collapsed sections of the bronchial wall. Varicose bronchiectasis, is a more serious form, characterized by extended and collapsed portions of the bronchial wall. The most serious and irreversible form is cystic bronchiectasis, in which the bronchi are greatly ballooned.

Bronchiectasis is usually identified during a routine chest x-ray as part of a cystic fibrosis patient’s annual lab tests. Further imaging such as a CT scan as well as pulmonary function tests and a sputum cultures will help the doctor determine the underlying cause, extent and severity of lung damage.

Bronchodilators such as albuterol, are prescribed to help open the airways. Fluticason and ipatropium are useful in controlling inflammation. Percussive respiratory therapy and postural drainage designed to dislodge the mucus is also used. Patients with cystic fibrosis will often use a combination of respiratory therapy including nebulized antibiotics to help control infection, bronchodilators, mucolytics and a vibrating vest such as the InCourage or ThAIRapy vest. Each of these therapies is important not only to control infection, but to slow the rate of declining lung function. Bronchiectasis is less likely to develop if infections are treated early.

In 2005 the FDA approved an inhalation powder called Bronchitol. Phase II clinical studies demonstrated adequate safety and effectiveness in patients at risk for developing bronchiectasis. The medication works by stimulating mucus hydration, enabling the usually thick, sticky mucus to be cleared more easily. Nebulized hypertonic saline produces a similar effect.

Tune-Ups Explained

2008-01-15T12:55:00.000-08:00

Preventive care is a cornerstone of effective treatment for cystic fibrosis. A “tune-up” is a term used in the cystic fibrosis community to refer to a preventive course of intravenous antibiotics and respiratory therapy. Depending on the doctor’s preference and the specific case of the individual with CF, a “tune up” can last from 2 to 4 weeks. Usually a tune up is started in the hospital, where the CF specialist and team can keep a close eye on things with regard to dosages of antibiotics, and any changes in pulmonary function.

Some CF specialists and CF centers maintain a practice of preventive “tune ups” every six months.

The practice of regularly scheduling a tune-up can be especially beneficial for young adults with cystic fibrosis who may not always be the most compliant with their medications. By giving them a boost of antibiotics and bronchotherapy before they exhaust themselves and deplete their health reserves, they will be in a better state health-wise to continue as many normal activities as possible with their peers.

Similarly, many college students with CF have found that by scheduling a tune-up during their semester breaks, they are able to regain some of the ground they lost due to stress during the school term. Checking yourself into the hospital for a tune-up while classes are not in session is a great way to keep from missing important class notes and lectures.

The most difficult part of a preventive tune-up is that the patient may not feel as sick and worn out as during other times requiring IV therapy. This makes the resting and recuperating part of a tune-up a challenge, because the temptation is there to remain active and continue in all normal activities. The powerful combination of antibiotics may actually make a person feel worse for a time, but a rebound is almost always sure to follow. Even though a person may feel “fine” during a tune-up, it’s important that they rest, stay well hydrated, and use the time to replenish the body.

The hope and goal is to prevent repeated hospitalizations due to pulmonary exacerbations, pneumonia, or other worsening of symptoms.

Surviving the Rollercoaster of Teen Rebellion in CF

2008-01-15T12:47:00.001-08:00

Teenagers and young adults rebel. It’s what they do. Pushing the limits and testing boundaries is part of the process of developing self-identity. Watching your “baby” seemingly disregard a more than a decade’s worth of instruction and wisdom is, no doubt, a frustrating and frightening time for parents. That feeling is compounded ten-fold when your teen has cystic fibrosis, and the rebellion manifests itself as non-compliance or outright refusal to participate in the daily rigors of caring for his or her health.

Some adult cystic fibrosis patients confess that they rebelled in the following ways during their adolescence:

- “forgetting” to take enzymes with meals

- Hiding multivitamins and other medications in the houseplants

- Throwing away their sack lunches at school because they were embarrassed to have to eat so much

-turning on the nebulizer/compressor so it would make noise and fool parents into thinking a treatment was being done

-lying to the CF doctor about taking medications

-engaged in reckless behavior because they believed they wouldn’t live to be an adult

Here are some suggestions and considerations for parents whose teens are in the throes of out-and-out rebellion against cystic fibrosis:

Remember that rebellion is normal. Even if only for a moment or two, be glad that this phase of your son or daughter’s life is normal and typical teen behavior.

Rebellion can serve as a path to greater self-awareness for your teen. Allow him or her to make mistakes and suffer the natural consequences. As s/he pushes against the limitations of cystic fibrosis, s/he will learn what happens when the body is deprived of what it needs to function well with CF. This in turn, will help your teenager to be better prepared to identify what is “normal” and healthy, and what is not. Think of it as a training exercise for the day when your teen will be off on his own and will have to make his own decisions about when to call the doctor.

Do keep a watchful eye on your young adult, but resist the urge to argue over compliance issues. Develop a discipline system that works best for your family, and implement it as you see fit. For younger teens, you may want to use a reward system. Teach them that failure to do the required things such as medications and treatments, means a loss of privileges of the more amusing and enjoyable aspects of life, such as having friends over, going out to a movie, etc.

Rarely does this rebellion become life-threatening. If your teen is in relatively stable condition with respect to lung health, a short season of rebellion is not likely to trigger an acute or dramatic downfall. Although it’s inevitable to remain completely unscathed when refusing to take medications or properly care for one’s body, many cystic fibrosis patients, now in their adult years, are none the worse for wear despite their turbulent teenage years.

Don’t argue with your teen in an attempt to get him or her to understand that failure to comply with treatments now could mean he or she will die younger. Although you as a parent can see the potential long-term benefits of compliance, that line of thinking simply doesn’t penetrate the thick skulls of young adults. Whether you consider your teen to be very mature for his or her age, or very smart about CF doesn’t matter. They will be in a state of somewhat ignorant bliss of the complications of CF until they are adults and have to manage every facet of their health regime themselves.

Make the most of opportunities to talk to your son or daughter about the frustrations of cystic fibrosis, but don’t dwell on every negative aspect of it. Remind him or her that it’s okay to need help with the emotional side of things, and ask if he or she would be interested in talking to a professional or non-family member (such as a clergy member) about what is going on. If he or she is not interested, don’t force the issue.

If your teen’s rebellion takes on a truly unhealthy form--such as experimentation with illegal recreational drugs and alcohol abuse—or begins to change into depression or self-mutilation, take action. Coping with cystic fibrosis is difficult enough without these added challenges.

Teens with cystic fibrosis will rebel just as their peers will. How long this turbulent phase and wild ride will last depends on the individual. The role of parents during this time is to help steer their adolescents safely through this period with as little heartache as possible. Parents, you are not alone in this frustrating battle, and neither are your kids.

What You Need to Know About Pseudomonas Aeruginosa (PA)

2008-01-14T00:44:00.000-08:00

Pseudomonas Aeruginosa, commonly referred to in the cystic fibrosis community as simply "PA," is one of the most prevalent and difficult pathogens to eradicate. As an opportunistic pathogen, this bacteria takes hold mostly in people who are in weak health, or are already fighting another type of infection. In people who have normal immune systems and are otherwise healthy, P. aeruginosa will not affect them. P. aeruginosa is often referred to as a "water bug" because of it's ability to survive and grow in moist environments. Whirlpools, jacuzzis, and public swimming pools are likely places for P. aeruginosa. to grow.

P. aeruginosa infections can occur anywhere in the body. Cystic fibrosis patients are particularly susceptible to acquiring P. aeruginosa infection in the lungs. Patients who are undergoing intravenous antibiotic therapy, are on a respirator, or have chronic bouts of pneumonia or bronchitis are at greater risk of contracting PA. Patients with cystic fibrosis with pseudomonas lung infections experience coughing, decreased appetite, weight loss, tiredness, wheezing, rapid breathing, fever, blue-tinted skin, and abdominal enlargement.

P. aeruginosa can produce a number of toxic proteins which not only cause extensive tissue damage, but also interfere with the human immune system's defense mechanisms. Part of what makes PA so strong is that it has very little requirements in order to live. It readily adheres itself to the body's cells. This increases the risk of the infection reaching multiple systems within the body as the infected cells are transported. Additionally, P. aeruginosa doesn't need much food to survive. Even in a body that is already severely depleted and immunocompromised, PA can live. It should also be noted that P. aeruginosa is a common problem in hospitals. Because of its ability to survive, proper contact precautions and cross-infection protocol should always be used by patients and caregivers.

Perhaps the biggest challenge in treating PA is that it is resistant to many antibiotics. Medications that doctors often use as their first defense in treating lung infections in CF, such as ciprofloxacin, have little or no effect on PA. There are two forms, mucoid and non-mucoid P. aeruginosa. In the mucoid form, the bacteria has an even stronger outer-coating, making it difficult for antibiotics to penetrate the cell to kill the infection. Furthermore, the mucoid form is able to survive and reproduce more readily than the non-mucoid form. The mucoid form is what leads to more chronic respiratory infections in cystic fibrosis,

Treating an infection may involve using inhalation therapy, oral antibiotics, intravenous antibiotics, or some combination thereof. Most often doctors will treat a PA infection by prescribing "cocktail" or combination of intravenous antibiotics in the hopes that the benefits of each antibiotic will combine to produce what's called a synergistic effect. Medications utilized for this purpose include ceftazidime (Ceftaz, Fortaz, Tazicef), ciprofloxacin (Cipro), imipenem (Primaxin), tobramycin (Nebcin), ticarcillin-clavulanate (Timentin), or piperacillin-tazobactam (Zosyn). A full course of antibiotics usually lasts from 4 to 6 weeks. Studies have demonstrated that a combination of respiratory therapy (such as inhaled Tobramycin or Ceftazadime) combined with intravenous therapy, works best to deliver the medication to where infected mucus is produced, and where it later resides in the airways.

Many cystic fibrosis patients who have not been infected by P. aeruginosa, are prescribed prophylactic (preventive) antibiotic therapy. Azithromycin for example, is one such medication that has been researched in accordance with the Cystic Fibrosis Foundation, and has demonstrated positive results in improving the lung function of patients infected by P. aeruginosa. Further studies are being done to determine how well Azithromycin will reduce the instances of infection caused by P. aeruginosa. Currently there are studies being carried out to develop a vaccine for PA.

Supporting links:

Cystic Fibrosis Foundation: Azithromycin

Inhibition of PA using Azithromycin and other macrolide Antibiotics

Todar's Online Textbook of Bacteriology, "Pseudomonas" (2004)

Heatlh A to Z: Pseudomonas Infections

Eradication of Pseudomonas Aeruginosa in Cystic Fibrosis

Microbe Magazine "PA Resists Antibiotics"

Acquisition of PA in Children with CF

Blood Work: Tobramycin Peak and Trough

2008-01-11T16:26:00.001-08:00

A tobramycin trough, is a blood test that involves drawing blood during the half hour before which the next dose is to be administered. If your results show that the concentration of the drug is <2>2 µg/mL, indicate toxicity. The prescribing doctor and pharmacy are alerted, and your next dose is either delayed, or the concentration of your doses are reformulated.

A tobramycin peak, is another round of bloodwork drawn between 60 and 90 minutes after you have infused the medication. If the concentration is 4-10 µg/mL, nothing needs to be changed. If it's >12 µg/mL, again, the doctor will have you wait with your next dose, or will have the pharmacy reformulate the concentration.

Another thing the doctor might do if your kidneys are in peril, is to switch you to a low protein diet. Proteins are particularly tough for the kidneys to process, so removing or limiting it in the diet gives the kidneys a chance to rest a bit, and possibly regain function. Regaining function is entirely within the realm of possibility, especially if the doctor and patient are diligent to keep an eye on the symptoms of possible renal failure at its earliest signs. Drink plenty of water (more than you think you need) when on antibiotics. If you notice that your urine is staring to darken or smell like your medication, (this happens frequently with ceftazadime, which smells like cat urine) let your doctor know. Also be sure to let your doctor know if, during any time during your course of antibiotic treatment, you experience pain in the middle to lower portion of your back. A dull ache in that area may be indicative of kidney dysfunction.

For people who have exhibited kidney problems in response to tobra before, and you happen to be in the hospital, doctors will most likely start you on a very low dose, and have lots of labs drawn. Your dosage concentration will be ramped up with each new administration of the med until they find a safe and tolerable range for you. In my case, Tobra is one of the few meds I can tolerate, so my doc knows exactly which dose to start me at each time.

In addition to monitoring peak and trough concentrations, many CF centers are now implementing hearing screening exams. The ototoxicity (ear damaging) effect of tobra has been well established; the same was true of gentamycin, which is no longer in wide usage for that very reason.

CF Fundraising Event in Orlando, FL

2008-01-07T21:35:00.000-08:00

What:

Beginning Tuesday, January 15th, The Glenlivet City Links - an urban-scaled, nine-hole indoor golf course fashioned in the likeness of traditional Scottish greens - will open in the heart of Orlando's shopping district at Pointe Orlando.

When:

11am -2:30pm, 1/15/08 through 1/25/08

Cost:

Free (donations for the Cystic Fibrosis Foundation will be accepted)

Gall Bladder Issues in Cystic Fibrosis

2008-01-07T10:50:00.000-08:00

The gall bladder is a small, pear-shaped organ that connects to the liver by a series of ducts. As part of the digestive system, the gallbladder’s role is to store bile (a bitter liquid produced by the liver) that will be used to digest food, particularly fat, on its way to the small intestine.

Nearly every cystic fibrosis (CF) patient has or will develop gallbladder problems at some point during his or her lifetime. In people with CF, the gallbladder is abnormally small and fails to function properly due to blockage by thick bile. In many cases, the gallbladder becomes atrophied because of non-use. Gall stones may form when the gall bladder fails to empty properly for long periods of time. In cases where the gall bladder becomes blocked because of gall stones, the risk of pancreatitis (swollen pancreas) increases.

Symptoms that may indicate gallbladder irregularities include:

-abdominal pain in the pit of the stomach

- pain and discomfort when eating

-difficulty taking deep breaths

-vomiting

-pale stools

-dark urine

-abnormal or unusual bloating with indigestion

In order to determine if the gall bladder is functioning properly, or if gall stones are creating a problem, your doctor may order imaging tests, such as an ultrasound, abdominal CT scan, or x-rays. One of the most effective ways to see how well the gall bladder is working is by performing a CT scan with contrast. In this type of scan, a special type of dye is injected into the patient’s arm. The CT scanner will then take a series of images as the dye moves from the liver to the bile duct, gall bladder, and ultimately, small intestine.

Occasionally the gall bladder will need to be surgically removed. This is often done via laparoscopy, a procedure in which only a few small incisions are made in the abdomen. This type of surgery is relatively simple, typically performed on and outpatient basis. Removal of the gall bladder will not impair digestion. The only difference is that your body will no longer be able to store bile between meals. Once the gall bladder is removed, bile will move directly from the liver to the bile duct and into the small intestine.

According to Dr. Brian K. Weinstein, "Up to 27% of patients with CF have gallstones, but only about 4% are symptomatic. Biliary colic is the pain associated with stones as they pass. This pain usually passes, but can recur. When the gallbladder is swollen, with pain, this can be cholecystitis, an inflammation and infection of the gallbladder. This can initially be treated with antibiotics, but usually is an indication for surgery."

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Supporting links

Gall Bladder Disease in Cystic Fibrosis

The Gall Bladder and Biliary Tract in Cystic Fibrosis

Bile Acid Secretion in CF: Evidence for a Defect Unrelated to Fat Malabsorption

Bronchodilators and Nebulized Medications

2007-12-16T11:33:00.000-08:00

"Recurrent wheeze and breathlessness are common in people with cystic fibrosis, and bronchodilators are commonly prescribed," according to lead author Clare Halfhide, M.D., of the Royal Liverpool Children's Hospital in England. "Despite their wide-scale and often long-term use, there is limited objective evidence about their efficacy in cystic fibrosis."

Source: Center for Advancement in Health. Article "Little Evidence Behind Bronchodilator Therapy For Cystic Fibrosis"

The findings of that study were published in October, 2005. It is the most current information I can find on the topic. Dozens of health-related news media ran the article, so I'm confident that it's not just a hack study published by some entity with a hidden agenda.

I used to do CPT without a bronchodilator unless my asthma was acting up or I'm fighting infection. Now that I have a ThAIRapy Vest from Respirtech, I always take a puff of my Xopenex HFA inhaler before starting my airway clearance regimen. Xopenex is very similar to albuterol. It is a bronchodilator. Many patients find that Xopenex does not make them as jittery or anxious as albuterol does.

I also use bronchodilators both as a "rescue med" and as part of my preventive maintenance even if I don't do them at the same time as CPT. Bronchodilators are most effective when they are done first in the lineup of "usuals." Below is the proper order of med-neb treatments, as reported by another CF patient's clinic's recommendation:

1. Bronchodilator (albuterol or xopenex)

2. Mucolytics (pulmozyme THEN hypertonic saline)

3. Inhaled antibiotic (TOBI or colistin)

4. Long acting bronchodilator (serevent or foradil)

5. Inhaled steroid (flovent, advair, or pulmicort)

Here's why the order of operations is so important when managing CF.

The bronchodilator helps open up the airways. The more open the airways, the better the chances of inhaling the other medications deeply enough into the lungs where they can be most effective. Mucolytics are "mucus cutting" medications. They help break up the secretions so that they can be coughed up more easily. In CF patients, the mucus and the lungs are typically dry, which is why hypertonic saline is so useful. Hypertonic saline works by adding moisture to the lining of the lungs, resulting in a slippery surface conducive coughing out sputum. Following sputum clearance of the airways, the lungs are further able to take in the nebulized mist of the antibiotics. At this point there is a greater likelihood of the antibiotics taking hold in both small and large airways. The treatment concludes with inhaled steroids which help keep the airways open for an extended period of time (i.e. 8-12 hours when it is time for another treatment.)

The length of time to complete all these steps varies somewhat depending on the type of nebulizer/compressor system being used. Before I had a PARI eFlow to nebulize my tobramycin antibiotic, the process took approximately an hour. Also, when I was not feeling well, or was more congested or taking TOBI, it used to take me as long as 1-1/2 hours to complete the process. Now that I have an eFlow, the process is dramatically faster and takes 45 minutes or less.

Although the article I cited at the beginning of this post says that bronchodilators are shown to be ineffective treatment, bronchodilators used in tandem with the other treatments CF patients need are effective indeed. My opinion is that they are an important piece of the overall treatment. While they may be ineffective on their own, they should not be discounted altogether.

Adoption and Cystic Fibrosis

2007-12-14T17:04:00.001-08:00

Today's Q&A is courtesy of Laura Christianson, author of The Adoption Decision and The Adoption Network.

Q. If I have a chronic medical condition, will it exclude me from being eligible to adopt a child?

A. Not necessarily. While adoption professionals do evaluate the health of the prospective adoptive parent(s), their main concern is whether a person's health issues will interfere with or prevent that person from being an effective parent.

If you have a chronic medical challenge and want to adopt, it's important to share openly and honestly with your adoption social worker, and explain the ways in which you are being proactive about managing your health issues. Adoption social workers aren't looking for excuses to turn prospective parents down; they are looking ways to say "yes."

I know several adoptive parents who have chronic medical conditions—they often adopt a child who has the same condition. Adoption social workers are delighted when this happens, because children with medical issues are considered "hard-to-place." When a parent adopts a child who has the same medical challenge, the child often receives the best treatment possible because the parent knows exactly what the child needs.

Laura is a freelance journalist specializing in adoption-related issues. She is the author of The Adoption Decision: 15 Things You Want to Know Before Adopting and The Adoption Network: Your Guide to Starting a Support System.

Her Exploring Adoption blog received Forbes magazine's 'Best of the Web' rating. Laura has written numerous general-interest features and essays for national and regional publications. She speaks regularly at writers' conferences and other events.

Newborn Screening for Cystic Fibrosis Offers Hope

2007-11-20T22:44:00.000-08:00

Imagine how difficult it would be as a parent, to take your sick child from doctor to doctor, and none knew what was wrong. The doctors seem to think your baby is merely battling a stubborn cold, or has food allergies of some sort. You know that can’t be the correct diagnosis, so you exhaust yourself searching websites that describe your little one’s symptoms, but never can quite put your finger on anything that explains it. Then you find it.

Cystic fibrosis.

Cystic fibrosis is the most common genetic disease affecting the Caucasian population. It is caused by a mutation in a particular protein in a person's DNA. This mutation causes a malfunction in the sodium and chloride transport channels. As a result, large amounts of salt are secreted in the sweat of a person with cystic fibrosis. Additionally, the body produces abnormally thick, sticky mucus which creates problems for nearly every major body function, particularly the respiratory and digestive systems.

People with cystic fibrosis are prone to frequent bouts of pneumonia and other lung infections. This is because bacteria become easily trapped in the sticky mucus that blocks the airways. Airway clearance devices and preventive maintenance medications are typically used to avoid infection. Such methods include inhaled antibiotics, bronchodilators, aerosolized medications, and chest physiotherapy.

Digestive problems also arise due to the thick mucus in the intestines and around the pancreas. Pancreatic insufficiency results when the pancreas cannot secrete the necessary enzymes to break down food. Enzymatic supplements are taken orally to aid in digestion. However, even with the supplements, people with cystic fibrosis are typically malnourished because the mucus lining the intestines prevents major vitamins like A, D, E and K from being properly absorbed into the body.

Treatment of cystic fibrosis
Over the last two decades there have been incredible developments in the treatment and understanding of cystic fibrosis. Ongoing research and the development of new medications are enabling some cystic fibrosis patients to survive into adulthood-something that was virtually unheard of in the past. Unfortunately, a cure remains to be found. The Cystic Fibrosis Foundation reports that the mean life expectancy is a mere 36 years of age.

---------

You’re stunned as you read about it. It sounds so scary, but all the symptoms add up and are exactly what your child has been experiencing over the last year or more. Loose, greasy, smelly, bowel movements; persistent cough and recurring lung infections; digestive problems; failure to gain weight; those funny shaped fingers. The list goes on and you can hardly process it. You are frustrated that the doctors never even thought of testing your child for this monster disease, and you wonder how much damage may have already been done to your little one’s body. You are angry, confused and sad all at once and your only thought is to get your child tested for cystic fibrosis as soon as possible.

Now imagine the empowerment of knowing as soon as your baby was born, about the unique health care requirements he or she would need. You and the doctors could work together to devise a treatment plan that would ensure the best possible health and quality of life for your son or daughter. Instead of fear of the unknown, you are given hope for the future.

Mandatory newborn screening for cystic fibrosis is not yet widespread. As of June, 2007, only 38 (see list, below) states in the U.S., including Washington D.C., had mandatory screening programs in place. Although more are planned, they cannot be implemented soon enough.

States with Mandatory Cystic Fibrosis screening for Newborns

Alabama*
Alaska
Arizona*
California
Colorado
Connecticut
Delaware
District of Columbia
Florida
Georgia
Hawai'i
Illinois*
Iowa
Kentucky
Maryland
Massachusetts
Michigan*
Minnesota
Mississippi
Missouri*
Montana
Nebraska
New Hampshire
New Jersey
New Mexico
New York
North Dakota
Ohio
Oklahoma
Oregon
Pennsylvania
Rhode Island
South Carolina
South Dakota
Virginia
Washington
Wisconsin
Wyoming

* these states have not yet implemented their mandatory screening program

According to the Cystic Fibrosis Foundation, the nation’s largest provider of funding for CF research, “Early diagnosis allows for immediate intervention with specialized therapies” which “have been shown to result in improved height, weight and cognitive function, and also may help maintain respiratory function. The greatest long-term benefit of newborn screening and early intervention is an increased life expectancy fewer hospitalizations over the course of a CF patient’s lifetime.”

California is the most recent state to require mandatory screening in newborns. It is the hope of many that the remaining states will soon be on board with this potentially life-enhancing measure. The following states have yet to mandate a newborn screening process for CF:

Arkansas
Colorado
Idaho
Indiana
Kansas
Louisiana
Maine
Nevada
North Carolina
Tennessee
Texas
Utah
Vermont
West Virginia

If your state is one of those listed above, consider taking action. Let your legislators know that it's important to implement newborn screening for CF. Here's how you can make that happen.

1. Contact the state health department to find out if CF is being considered for newborn screening.

2. Call or write to your governor. Visit the Web site of National Governor's Association www.nga.org to learn how to contact your governor.

3. Call or write the state legislators. www.ncsl.org is the website listing all the contact information for your state.

Partnership between Patient and Physician

2007-11-16T18:07:00.001-08:00

Back when I was in college, I was still attending a pediatric CF clinic. My doctors were wonderful and knowledgeable--about pediatric things. Basically whenever I came in for an appointment it was because I needed something. I would request to start IVs on such-and-such a date because it worked well with my course schedule. When claritin didn't seem to be helping me take care of my allergy symptoms, I told them I wanted to be switched to Zyrtec.

Primarily they may have seemed like mere suppliers, but there was much more to it than that. I saw them not only as suppliers, because together we had developed a real synergy. They knew I was serious about my health and that I was good at reading my own body. They gave me the freedom to be an independent CF adult, and reminded me that they were always there for me when I needed them. My stage of CF at that time was quite routine, which is also why I saw them as suppliers. There were really no surprises with my health.

It came to a point however, where I had truly outgrown the pediatric clinic. They sat me down and said "we'll be happy to keep seeing you, but we think you'd benefit from the adult clinic." They left the decision up to me, again reinforcing the importance of being my own advocate when it came to my health.

Now that I'm at an adult clinic, I see things as a partnership. I tell my doctor what's going on, and he tells me how to manage it. He always lays out a few options for me, and together as a team, which includes my wonderfully supportive husband, we decide what is the best course of action for my health and quality of life. There have been new challenges over the last couple years. Things that were unfamiliar to me, which is why I needed to rely heavily on my doctor's expertise.

Even with all his knowledge of CF, he's still learning the specifics about ME. That's where our partnership comes in again. It's a give and take. He needs me to communicate with him effectively about what's working or not working for me; I need him to take my feedback into thoughtful consideration and present the best plan to meet my needs. When that happens, I benefit tremendously.

I like being actively involved in my own health care. I like knowing how and why things work--that's just my nature. On the other hand, I like the freedom of not having to be the one with all the answers. Because I have a doctor I trust, I don't have to waste precious energy second-guessing his opinions or treatment plans.

Blood Work: Iron

2007-11-11T06:07:00.000-08:00

Blood Work: Complete Blood Count

2007-11-11T05:26:00.001-08:00

Click on the images below to see a larger, printable version of the table, which is in two pieces.

Blood Work: Electrolytes

2007-11-10T21:14:00.000-08:00

Electrolyte (acid-base) balance is measured as part of a comprehensive metabolic panel (CMP). Click on the image below for a larger version that you can print and use as a reference tool.

Liver Function Tests

2007-11-10T18:17:00.000-08:00

Comprehensive Metabolic Panel

2007-11-10T18:10:00.000-08:00

Click on the images above for a full screen version of the table. You can print these and keep them as handy reference tools.

Sources of Financial Assistance

2007-09-17T12:40:00.000-07:00

America's Challenge Foundation

To assure and provide financial support to children and their parents that do not have the financial resources to properly maintain adequate care that is required to; pay doctor, hospital, travel, lodging, and pharmaceutical expenses to improve the quality of life for those with CF disease. While other Cystic Fibrosis Foundations are focusing on funds for research and the prevention of Cystic Fibrosis, America's Challenge is assisting the patient and the parent's in their day to day existence.

The Genentech Endowment

Genentech is the maker of Pulmozyme. They have 3 methods of assistance. Visit this link to learn more. There are options for the insured as well as the uninsured.

Digestive Care, Inc.

This is the maker of Pancreacarb. They offer some assistance with paying for the all-important enzyme supplements.

The Reaching Out Foundation

Emergency Assistance

The Foundation provides emergency funds to families who must live away from home to be near their loved one during their hospitalization for CF related illnesses. These funds pay for such items as hotel, transportation, food and other quality of life concerns during times of crisis.

Insurance Assistance

The Foundation provides funds to families who would otherwise lose health insurance due to employment changes or other events. The fund helps qualified families with medical co-pay payments, COBRA payments or other insurance premiums.

Nutritional Assistance

The Foundation provides funds to pay for prescribed nutritional supplements for Cystic Fibrosis patients. The ability to gain and maintain weight is a challenge to CF patients. Many must purchase special nutritional supplements and enzymes to achieve this goal and these supplements are often not covered by insurance.

When Your Spouse Doesn't Believe You're Ill

2007-09-14T16:27:00.000-07:00

When your spouse doesn't believe you are ill

The doctor couldn't find anything wrong with her, despite her complaints about pain and extreme fatigue. He left the room and her husband came back a few minutes later.

"Hi, Honey," she replied to her spouse, as she carefully reached out for his hand. But he didn't reach out to take her outstretched hand; he just let it sit there empty on the scratchy hospital blanket. He looked at her with tired eyes, full of frustration.

"The doctor said that there is nothing wrong," he said. "I think maybe you've just been making all of this up the last year."

She was heartbroken and pulled her hand back. He was her lifeline, the one thing that was getting her through all of this. He was the only person who she trusted would always believe her, understand and offer love and sympathy. And now he thought she'd been deceptive, lying about her pain

Unfortunately, this scenario is a true story... and all too familiar for many couples. Illness can have a devastating effect on relationships, especially when it is invisible, difficult to diagnose, and hard to even understand. So what's the answer if your spouse doubts that you have an illness? While there are no easy answers or tips that will guarantee results, here are a few ideas to make the journey easier.

Pray. Rather than praying as a last resort, start praying first. Ask God to give your spouse wisdom and discernment, especially regarding your illness. Pray that God will send people into your spouse's life that can be a godly influence, such as other spouses of people with illness. Pray for a diagnosis so that you can have the benefit of the medical world on your side.
Allow your spouse some time to see that the illness is real. Don't expect him/her to be convinced by what you say. Let him/her see how you are over a period of time.
Surround yourself with supportive people who do understand that you have an illness and it's implications. Join a support group or a women's bible study. Ask for prayer for your marriage. Let others encourage you and stand by you when you need the emotional support. See a pastor if you need to talk to someone about your marriage and the effect his or her doubts are having on the relationship.
Refrain from trying to do more than you should physically. Every time you do too much you are sending signals that you are able to do more, even though only you will know how much you will pay for it later in pain.
Do the research on your illness or symptoms and continue to fight for a diagnosis. If you already know what your illness is, get as much information on it as possible and keep well-informed.
Invite your spouse to accompany you to a support group meeting, a workshop or conference on your illness. Explain to him that you just want to find out more information about this illness, and that he might find it interesting.

~ Reprinted from ...And He Will Give You Rest
Volume II, Issue 9. © *Now called HopeKeepers

Social Security Administration's Defintion of Disabled

2007-09-13T16:23:00.000-07:00

How does Social Security define

By Scott Davis, Esq.

Have you ever wondered what the “definition” of disability is? I know you have…we all believe we know “who” is disabled and “who” is not. Heck, you can tell just by looking at a person! Doctors are usually certain they know whether their patients meet the “definition” of disabled.

The obvious question becomes…whose definition of disability are we talking about? If you think everyone, including the Social Security Administration (SSA) are on the same page…think again! This article is my humble attempt to educate everyone, including physicians, regarding SSA’s definition of disability. Please understand it is difficult to capture the entire definition in a short article; however, it is possible to set forth a general framework.

First, allow me to debunk several longstanding myths about what is means to be disabled.

Myth #1 You have to be Totally Disabled to be eligible for Disability Benefits

Not true. I am not sure what “totally” disabled means; I do know it is a term of art our society uses, but not one SSA uses.

I suspect that if you put 100 doctors in a room and asked for a definition of “totally disabled,” you would get many different answers. My guess is that it means one is unable to function at all in any capacity.

In general, to be eligible for disability benefits, SSA does require that you not be working in any capacity. However, this does not mean you have to be to physically and/or psychological unable to function in any capacity.

Myth #2 You have to be Permanently Disabled to be eligible for Disability Benefits

Not true. Again, I am not sure what “permanently” disabled means; it is a term of art our society uses, but not one SSA uses. If a doctor supports a patient’s claim for disability, they will frequently state the patient is “permanently” disabled. The statement may be true in many cases, but my experience is that most people who apply for disability benefits with SSA are not “permanently” disabled. Even if they were, it is usually too early to tell if a 45 year old will return to work before the age of 65.

Thankfully, SSA does not require that you be permanently disabled. In fact, the issue of whether a disability is or can be permanent is never an issue at SSA.

What is SSA’s Definition of Disability?

Simply put, SSA and federal law defines disability as follows, “The inability to engage in any substantial gainful activity by reason of any medically determinable physical or mental impairment which can be expected to result in death or which has lasted or can be expected to last for a continuous period of not less than 12 months.” (citing 42 U.S.C. §§ 423(d)(1)(A) and 1382c(a)(3)(A).

First, please note that SSA’s policy is that disability can be due to any medical or psychological diagnosis (disorder). Second, contrary to popular myth, SSA does not require that you be “totally” disabled. Third, with regard to duration, the impairment does not have to be permanent, it only has to prevent you from working for a minimum of 12 months.

Scott E. Davis is a social security and long-term disability insurance attorney in Phoenix, Arizona. Mr. Davis represents clients throughout the United States. Although Mr. Davis has experience representing clients with a broad spectrum of physical and/or psychological disorders, the majority of his disability practice is devoted to representing individuals with chronic pain and chronic fatigue disorders. In almost every case, a fee is charged only if his client obtains benefits. Mr. Davis invites your questions and inquiries regarding representation via his web site.

50 Ways to Encourage a Chronically Ill Friend

2007-09-13T00:33:00.000-07:00

By Lisa Copen

"A good friend is a connection to life - a tie to the past, a road to the future, the key to sanity in a totally insane world." ~Lois Wyse

Ask, "What events in your life are changing and how are you coping with the changes?"
Understand that she lives in a constant state of making decisions for which there is no guarantee that she is making the right choice.
Put meals in disposable containers and attach a note saying "This doesn't need to be returned."
Add stickers to envelopes for a cheerful touch.
Arrange for your friend's kids to have a night with your children.
Don't make a person into a project.
Ask, "Would you be willing to talk to a friend of mine who has recently been diagnosed with a chronic illness and offer her some encouragement?" It makes one feel good to know that her experience can offer someone else hope and that God still has a purpose for her life.
Wash his car and put a little note inside for him to find later.
Remember important anniversaries, both the good and the bad. No one else will.
Ask, "Do you want company the day that you wait for the test results? I could come over for a couple of hours."
"No matter how little you have, you can always give some of it away." ~Catherine Marshall

Just listen . . . until it hurts to not say anything. And then listen some more.
Ask her, "How do you feel God is working through-or despite-this illness in your life? I'm interested."
Ask, "What do you wish people understood about your illness?"
Don't make her feel guilty about things that she cannot do.
Treat her to a gift of movie rentals via postal mail through a service ($7-15 a month).
Ask, "Would you be comfortable with having your name on a prayer list, so that others can pray for you?" Don't assume.
Instead of saying, "I will pray for you," say, "I'd like to pray for you right now, if that's okay."
Mop the floors.
Ask if she would be interested in writing something for the church newsletter, maybe even about the subject of living with chronic illness.
Buy a brightly colored umbrella as a gift.
Ask, "Do you have an errand I can run for you before coming over?"
Ask her to do spontaneous things, like go to a concert in the park, or just for a picnic. She may be more likely to participate since she knows if it's a good day or a bad day.
Don't say, "So, why aren't you healed yet?" or "I wonder what God is trying to teach you that you just aren't learning!"
For a unique gift, provide brightly colored paper plates, napkins, and utensils in a gift bag with a note that says "For when you don't feel like doing dishes."
Get her a pretty box to keep all of her notes of encouragement. Remind her to get it out and read things when she is feeling down.
Be her advocate. If you are at an event and walking/seating is an issue because of her disability, ask her if she'd like you to take care of it. If she says you can, be firm but not rude. Don't embarrass her by making accusations of discrimination or by making a scene.
Ask, "Would you be interested in a prayer partner from our church?"
Purchase matching coffee mugs for you and your friend, and then commit to pray for one another each morning while using them.
Say, "While you're in the hospital I'd be happy to take care of your pet."
Don't tell her about your brother's niece's cousin's best friend who tried a cure for the same illness and. . . (you know the rest).
Find out which charity is most important to her and then give a donation in her honor.
Ask, "What are your top three indulgences?" and then spoil her soon.
Hold the door open for her. They are heavy!
Don't tease her and call her "hop along" or "slowpoke." Comments you mean in fun can cut to the quick and destroy her spirit. Proverbs 18:14 says, "A man's spirit sustains him in sickness, but a crushed spirit who can bear?"
Say, "I know you must need someone to just vent to occasionally. I may not fully understand how you feel, but I'm here to listen anytime."
Ask your church youth group to come over and clean up the yard during seasonal changes.
Don't ask her, "How are you able to make it financially?" If she wants to share a burden she will.
Ask, "What would you advise me to look for in a new doctor?"
If your friend has a disabled parking placard and you are driving, allow her to tell you where she wants to park. If she's feeling particularly good that day, she may not want to park in the "blue space." Don't be disappointed that you'll have to walk farther.
Don't gossip about others. She'll wonder what you say about her. "Do not let any unwholesome talk come out of your mouths, but only what is helpful for building others up according to their needs, that it may benefit those who listen" (Ephesians 4:29).
Proverbs 25:11 says,
"A word aptly spoken is like apples of gold in settings of silver." Be kind, gentle, and respectful.
Accept that her chronic illness may not go away. If she's accepting it, don't tell her the illness is winning and she's giving in to it.
Don't say, "Let me know if there is anything I can do." People rarely feel comfortable saying, "Yes, my laundry." Instead pick something you are willing to do and then ask her permission. Try the coupon in back!
Ask her to share her testimony at an event.
Buy a magazine subscription for her on her favorite topic.
Plant a rosebush to view from a window.
Understand that you don't need to know all of the details about the illness in order to be helpful.

He'll share with you what he's comfortable with you knowing.
Don't ask, "Why can't the doctors help you?" or insinuate that it must be in her head. There are millions of people who are in pain with illnesses that do not have cures.
Avoid having gifts be "pity gifts." Just say, "I saw these flowers and their cheerfulness reminded me of you."
Send tapes of church services your friend misses to her with a copy of the bulletin and a note.
If she doesn't have a cordless phone, get her one. Phone headsets are also nice.

Reinvent Your Dreams

2007-09-12T16:14:00.000-07:00

By Joanna Wasmuth

Read an article about Joanna that just appeared in the San Diego Union Tribune August 2006.

Often when we are sidelined with pain and illness, we stop dreaming - about who we want to become, what we want to do, things we want to experience, how we want to impact the world. This often leads to a profound sense of loss, fear, frustration, and sometimes depression. We can become trapped and immobilized by not only our physical pain, but our emotional distress as well.

But your pain doesn't have to stop you from living a fulfilling, exciting life! Your journey with pain is a unique opportunity to slow down, take a look at your life up to this point, and reinvent it. It all starts with reinventing your dreams! Dreams are the fuel that moves you from frustration to fulfillment and satisfaction! I invite you to start reinventing today!

Exercise

Make a list of dreams and goals you've had in life.
Choose two or three that leap out at you. As you do this, you might hear yourself saying things like, 'Yeah right. Not anymore! I hurt too much to do that." If that's true, I think you'll get tremendous value from this exercise.
Really think about those dreams. What is it that drew you to them? What's the essence? Is it something that's going to increase your pain? For how long? Will the excitement be worth an increased pain level for a few days? Is there a way you could accomplish the dream without increasing your pain? Example I used to love hiking, until my health problems turned any attempt at a hike into a painful nightmare. So I reinvented that dream - maintaining the essence, but adjusting the method. Now I enjoy the beauty of nature in a way that is conducive to my current health by going for short walks on flat paths in the woods, or sitting on the beach, or riding my bike leisurely through the park.
Brainstorm creative ways that you could reinvent the dream so that you experience what you really want in a way that supports your overall health and wellness. Ask for input from friends if you get stuck. Example If you love to snowboard, but are not up to riding, maybe you could go watch a snowboard competition; take a gondola ride to the top of a local ski resort just to be in the midst of the energy (make sure you can also ride back down!); ride for an hour on the bunny hills; look into snowboard adaptations that would enable you to sit while riding; try inner tubing; and much more!
Choose one item from your brainstorm list that you will look into in the next 24 hours. Be in motion towards achieving your dreams!
You can live a dream come true life. Even if your life is marked by pain!

Joanna Wasmuth, founder of Harmony Coaching Group, is a Certified Dream Coach®, speaker and author. She is also the creator of the LifeThr!ve Personal Coaching Series for People with Chronic Pain. Joanna coaches business professionals who want to fast track achieving their dreams in life and work. Her specialty is empowering professionals with chronic pain from injury or illness to make a comeback in life by reinventing and living their dreams. To contact Joanna, visit www.harmonycoaching.com or email thrive@harmonycoaching.com.

Who Is Allowed To Park In those Disabled Parking Spaces, Anyway?

2007-09-11T16:30:00.000-07:00

Who Is Allowed To Park In those Disabled Parking Spaces, Anyway?

Written by Sherri L. Connell, BA
Copyright © 1997, Revised 2002
The Invisible Disabilities Advocate

Hey! They Don't LOOK Disabled!

Have you ever seen someone get out of a car parked in a space reserved for the disabled, who did not LOOK disabled? Did it make you very uncomfortable or even upset? Did you let them know of your disapproval by giving them a dirty look or yelling something at them?

Well, you are not alone. Many people are very disturbed by the sight of a seemingly mobile person stealing the space of someone who is truly in need of it. After all, we want to protect the rights of people for whom these spaces are reserved!

However, in wanting to help those who deserve these parking spaces, we actually may be hurting someone who does have a legal right and a legitimate need to park there. How can this be true, you ask? Isn’t it obvious who is and who is not disabled? The answer is... NO.

The qualifications for the general accessible parking spaces include those using chairs, walkers, crutches and canes, as well as some whose impairments are not always so obvious to the onlooker. I refer to these impairments which cause disabilities that are not so apparent from the outside, as "invisible disabilities."

There are thousands of people who are forced to contend with serious illnesses, injuries and circumstances, which have left them with mountains to climb every time they take a step. Most people do not realize that a person can have hindrances that come from the inside and may not even be visible on the outside. Their restrictions may not be conspicuous at a glance, but their pain, limitations and inability to function normally is all very debilitating in reality.

What may seem easy to you, may seem like a 14,000 foot hurdle to them. Being able to park close to the entrance of a building when they need to, allows them to run an errand they otherwise would not have been able to conquer. Many even collapse in stores, become very dizzy and weak or even black-out..

Here are just a few invisible reasons a person may be able to park in the accessible spaces:

Arthritis, Back Injury, Brain Injury, Chronic Fatigue Syndrome, Cystic Fibrosis, Diabetes, Heart Condition, Lupus, Lyme Disease, Muscular Disorders, Multiple Sclerosis, Neurological Disorders, Osteoporosis, Organ Transplant, Oxygen Impairment, Parkinson’s Disease, Difficult Pregnancy, Prosthetic, Seizure Disorder, Spinal Disorders, Surgery and several others.

Wow! So, They Really Need It?

For many, the shortened distance from the parking lot allows them to: walk into a building to use an electric cart or wheelchair; avoid dangerous exposure to heat, cold and exhaust fumes; use their energy for shopping; get back to their car when they have used up all of their energy inside; or simply to remember where they parked.

As you can imagine, it is very uncomfortable when people stare, because they think you do not look like you need to park in a reserved parking space. As a result, many people with these circumstances are left feeling afraid to use the very spaces that were intended to help them, even when they need it!

Honorably, most with invisible disabilities genuinely want to leave these spaces open for others if possible. Most will: not park in a space that is intended for wheelchairs and scooters; try to park somewhere else if there are not several spaces left for those who may need one; just have someone drop them at the door; or not park in an accessible space at all on a "better" or "good" day.

What we usually do not realize, is that most people with illnesses and injuries would jump at the chance to trade their plates and placards in for the ability to walk from the farthest parking space! To those who are healthy and able to walk, they see these spaces as a bonus or luxury! But, for those who are sick or in pain, it is just a reminder of what they have lost. After all, these spaces do not make life easy, they make it possible.

So, How Do You Know If They Should Be Parking There?

First of all, no person has the right to park in the access isles, which are placed next to both accessible and van accessible spaces. These are the striped areas next to the parking places and are designed to help those maneuver themselves and their assistive devices out of the car door. When someone fills up these isles, a person could get blocked in or out of their vehicle.

Second, there are spots strictly set aside for those using wheelchairs or motor scooters. Not every parking lot has them, but for those that do, they are clearly marked, "Van Accessible." These spaces are 96" wide, with a stripped 96" space to the side, allowing the person to maneuver their chair or scooter out with a lift or ramp. It is not illegal for someone without a chair to park in a van accessible space, but it should be left open for those with the specific need.

On the other hand, the rest of the reserved spaces are properly referred to as "accessible parking spaces." They are marked with a sign that often has a logo of a wheelchair. However, this does not mean that it is only for those using chairs. This logo is the universal and international symbol for "disability" or "disabled."

The purpose of the accessible spaces is to assist those with all types of disabilities and disabling conditions. For those with limitations, the spots help to make it possible for the visitor to shop and run errands.

How do you know who can park in an accessible space and who cannot? Look for a temporary or permanent placard in the front window or a disabled license plate. These items are received through an application form in which a patient’s doctor must fill out for them, through the Department of Motor Vehicles.

The DMV has specific guidelines and requirements the person must meet in order to receive a placard or license plate. They take into consideration the impairments due to the illness or injury, as well as the implications and aggravations of symptoms and limitations. Therefore, if a person is issued a license and is displaying it, then they have the LEGAL, MEDICAL RIGHT TO PARK THERE.

But, What If They

DON'T Have A License To Park There?

Anyone who parks in a space reserved for those with accessibility needs, must display their placard or license or they can be fined. You can call the sheriff’s department of that county if it is not on private property; however, the vehicle must be illegally parked when the officer arrives (do not call 911!). Or, you can notify a security guard to ticket them or the store manager to page the owner by license plate number and vehicle description.

But, what about those people who use their relative’s placard, when their relative is not even with them? Well, this is definitely immoral, selfish and disrespectful as well as being illegal; there is NO excuse of this dishonest behavior. However, unless you know them and know without a doubt is not their placard, it is in the best interest of those of us who suffer from invisible disabilities to just smile and assume they have a right to be there.

These reserved spaces are designed to help those in need of them for a number of reasons. Without these spaces, seemingly simple tasks in life would be excruciatingly painful, overwhelming, impossible or even life threatening for thousands of people, whether the disability is visible or invisible.

Finally, it is honorable for you to care if these spots are being abused by those who do not need them. Just remember, as shown in this article, you cannot be the judge of who deserves to park in the accessible spaces and who does not, just by looking at them.

Therefore, if a person is displaying a license to park in an accessible parking space, try offering a hand, instead of a visual judgment; after all..."the people you are graciously intending to defend, may be standing right in front of you!"

###

This article contains excerpts from the booklet “But You LOOK Good: A Guide to Understanding and Encouraging People with Chronic, Debilitating Illness and Pain!”

This booklet explains how a person can be damaged by an illness and/or injury on the inside, but still “look fine” to others on the outside. It gives detailed suggestions of “what not to say” and explanations as to why. In addition, the booklet provides many examples of “what to say” to be an encouragement and offers practical “ways to help.”

For ordering information and to read more articles written by Sherri, visit The Invisible Disabilities Advocate at: www.InvisibleDisabilities.com Please contact IDA for permission to distribute or publish this article.

Winning Your Disability Case in Three Words

2007-09-11T00:43:00.000-07:00

By Scott Davis, Esq.

In a disability case, almost any symptom or limitation can be disabling; but to determine whether they preclude work, the relevant questions are how frequent, how severe, and how long do they last?

A critical point I make to people who contact me everyday is that their disability case is won or lost based on symptoms/limitations and not on their diagnosis! Clearly, under federal law, a disability claimant has to have a legitimately diagnosed physical and/or psychological disorder to even allege disability, but this is only the beginning of the analysis.

Disability cases are almost always won or lost based on the quality (documentation) of your medical records and the subsequent opinions rendered by your treating physicians regarding your ability to sustain full-time employment. The documentation of symptoms and limitations in your medical records is critical as it provides tremendous credibility to and an understanding as to why your doctors’ have concluded you are unable to work.

Once a diagnosis is established, the disability inquiry immediately shifts to why you are unable to work due to the symptoms and limitations that result from the diagnosis. I tell my clients that of the total time spent in a disability hearing before a federal judge, 5% is spent on the diagnosis and 95% is spent determining the frequency, severity and duration of symptoms and limitations, and whether they prevent all work. Ignoring this fact places the success of your claim in great peril…don’t ever forget this!

A common problem disability claimants frequently make is having “tunnel vision” and focusing solely on their diagnosis, as if the fact they have been diagnosed with a disorder automatically confirms they are disabled and entitled to benefits. This is especially true of people suffering from chronic pain and fatigue disorders such as fibromyalgia and chronic fatigue syndrome. I believe this is true because these folks have almost universally been sent on an “odyssey” by the medical community, simply to obtain a diagnosis.

Never forget that obtaining a physical and/or psychological diagnosis is the very beginning, and not the end of your disability case.

Thus, the question becomes, “How do I document the frequency, severity and duration of my symptoms and limitations?”

Tip #1: What should I be Documenting? Simply put, whatever it is that prevents you from working. For example, let’s use chronic pain and fatigue. It is critical you distinguish why the pain and fatigue is different from what an average person may experience. If I say “I am in pain and fatigued,” that does not tell you much. Why? From time to time we all experience some degree of pain and/or fatigue. But if you tell your doctor “I am unable to function as I experience severe daily pain and exhausting fatigue lasting most of the day without relief,” or “I am unable to function 2 days per week due to migraine headaches that last all day even with medication,” now you’ve given the doctor and a judge an idea of why your symptoms are so debilitating.

Tip #2: Obtain a Copy of your Medical Records from your Treating Physicians

After you have followed Tip #1, the next question becomes, “Did the doctor write down what I just told him/her?”

I am often surprised at how many people applying for disability benefits have never seen their medical records. Obtaining a copy of your current treating physician’s records is important because it will give you an idea of whether your symptoms and limitations are being recorded. You may be surprised to find that your complaints do not appear in the records or if they do, the doctor’s notes are totally illegible! Illegible handwriting is a real problem, because judges who decide your claim are just like you and I, they don’t (and generally won’t) try too hard to decipher what the notes say.

If you are not satisfied with the documentation, address the issue tactfully with your doctor and explain the importance of documentation to your disability case. If they are receptive, I suggest you give them a copy of this article for reference.

Tip #3: Keep A Short Diary of Your Symptoms and Limitations before your next Visit to the Doctor

Whether you know it or not, your daily lives tell a compelling story about your inability to work. But how do you remember the frequency, severity and duration of your symptoms especially if you can’t spell your name at times!

I advise clients to keep a short and simple diary one week before their next visit with their doctor. For simplicity sake, the entries should short and not detailed (otherwise you will not do it). On a day when you were unable to get out of bed due to pain or fatigue, document it. Or document when you slept for only three hours the night before and then took a couple of naps the next day. Or document the migraine headache that lasted for two days in spite of medication.

Then, on your next doctor visit, when he/she asks “How are you doing?” you will have a laundry list of symptoms and limitations rather than giving them a blank stare!

Of course, winning your disability case is more complicated than this article has the time for; however, following these tips will significantly increase your odds of winning.

Best of luck to you and remember to keep fighting for the benefits you deserve!

Making Desireable Changes

2007-09-10T16:32:00.000-07:00

By David Spero, RN
Adapted from the book, The Art of Getting Well: Five Steps to Maximizing Health When You Have a Chronic Illness.

Health problems and symptoms, with the possible exception of pure genetic disorders, carry a signal to change. Even the flu tells us to rest and take Vitamin C. The message can be as obvious as, "get better shoes," if our feet hurt, or it may be hard to decipher. It can relate to an internal issue, like a headache that means, "Stop trying to be the perfect mother, already!" Or it could be telling us about an external change, like asthma that means, "Get the mold out of this apartment or move away!" Changing such harmful situations makes it possible to achieve higher states of wellness.

We usually think of health changes in terms of diet, exercise, rest, and a few other areas, but a whole range of life factors come into play. These modifications can be huge decisions, like leaving a miserable relationship, or simple choices, like a better mattress to sleep on, or cutting down on coffee. Even small changes can have large payoffs. By giving us a sense of control, they set the stage for further growth. It doesn't always matter much what change we decide to make. Just doing something, anything, for ourselves, for our bodies, makes a huge difference.

How change works
To help us survive when life was much more dangerous than now, our brains learned to like things they've gotten used to, even if they're awful. So it's normal to fear change to some degree, but the actual process of change is pretty straightforward. Here are some guidelines:

1. The best changes are things you want to do, not things someone else tells you to do. In Stanford University's Arthritis Self-Management Program, each participant has to make a weekly action plan. One woman, Martha, planned three weeks in a row to do more walking, and never did it. Finally, the truth came out. She said, "I don't really like to walk; I just thought I should." Substituting another form of exercise got her going.

2. You need to believe that the change you plan will actually help. If you need convincing, talking with others who share your issues, reading books and articles, or listening to your doctor can provide evidence of effectiveness.

3. Changes should be realistically attainable. People tend to want things to go too far, too fast. They turn self-care into a form of self-abuse. "I will run on the treadmill an hour a day." "I want to lose 100 pounds in three months, like that person on the TV ad." Good luck! We have much better chances if we make changes that feel good as we go along, and set realistic timetables.

4. Start small, breaking large goals into achievable chunks. It is far better to start with a less ambitious goal and achieve it, then to shoot for some gigantic transformation and fall short. The first pattern leaves you feeling good about yourself and ready for more; the second makes you want to forget the whole thing.

5. There will be ups and downs. The great leap to fitness, the steadily improving ability to speak up for ourselves, or the sudden, permanent adoption of a healthy, natural diet; these things do not happen often. In real life, there are good and bad days, good and bad weeks, even months. Coming back from the bad patches is part of the process.

6. For change to be worth the trouble, our lives have to be worth living. If we don't have a reason to get out of bed in the morning, it really won't matter how healthy we are, will it? So look for ways to get more pleasure, more purpose, and more fun into your life. Some people say that being healthy means giving up everything you like. They have it completely backward. Give up the things you don't like, and appreciate the heck out of the things you do. One good place to start is by rewarding yourself when you do something positive for your health. For example, after you exercise, you might want a long, hot bath, just to enjoy.

6. Change happens when we're not looking. We work and work toward getting in shape, say, or being more disciplined about finishing what we start. Nothing seems to happen for the longest time. Then one day, we notice that we are not getting tired nearly as fast. We're breathing easier, feeling better. When did it happen? Probably, it happened when we stopped watching. When we give up the need for miracles is when miracles happen. And in the field of self-motivated behavior change, miracles happen every day. If you keep these guidelines in mind, they can happen for you, too.

David Spero, RN is a 51-year-old nurse, journalist and health educator living in San Francisco. In 1989, as a father of two young children, he was diagnosed with multiple sclerosis. He struggled for years before beginning to absorb the lessons of his illness. Continuing to work part-time and raise a family, he utilized such practices as yoga, swimming, meditation, psychotherapy, and guided imagery, and made several other life changes to optimize his physical, mental and spiritual condition.

Seeing is Not Always Believing when it Comes to Chronic Illness

2007-09-10T16:08:00.000-07:00

By Carol Sveilich, MA

Has someone ever said to you, "You look great!" while inside you felt fatigued or were in profound pain? People who live with ongoing pain and chronic health challenges face such dilemmas every day. Since many ongoing symptoms often do not manifest outwardly, people sometimes have a difficult time believing that a person with a healthy appearance and lively demeanor can be experiencing so many profound symptoms and limitations.

Well-meaning acquaintances and family members who say, "but, you look fine," can often unleash a sense of anger or compound the feelings of isolation in the person who lives with physical challenges that are hidden from view. It would be nicer to hear, "I cannot imagine the difficulties you are experiencing. You are incredible!" or "I didn't know you were dealing with such challenges. Tell me more about it. How can I help?" or "You certainly make it look easy, but it must not be easy at all." Acknowledging the condition, rather than belittling or dismissing it, is always welcomed.

Unfortunately, no instruction book exists to guide people living with such ongoing challenges. People with chronic symptoms must sometimes learn to adapt to new routines or limitations. There was a time in their lives when they would promptly recover a bout of feeling ill. But now they find themselves riding a wave of symptoms that wax and wane and never quite go away. This is a tricky ride to maneuver, especially for someone who is accustomed to experiencing decent health and a prompt recovery.

For some, the most challenging aspect of living with ongoing pain is not having control over whether or not they will have a nice day. It is sometimes impossible to make plans, commit to vacations, or even take a trip to see a movie. This lack of control over symptoms can leave a person feeling as though they live within the body of an unpredictable stranger. Not surprisingly, Nazi concentration camp survivor and psychiatrist, Viktor E. Frankl, compared his loss of control in prison to that of a person imprisoned in a body with chronic illness. His inspiring attempt to control attitude, if not circumstances, is one that can easily apply to the growing population who live with chronic pain.

Many individuals do not give in to their pain or illness. When faced with strenuous situations, they tend to push themselves beyond their comfort level. As a result, they pay a high price for overexertion, which often aggravates the condition or illness. Symptoms may worsen for days or even weeks. Sometimes having a good day is simply having a day that is realistically paced. Honoring limitations is one of the most difficult challenges for those whose lives are invaded by pain. They tend to move beyond their comfort levels into a more psychologically comfortable space so that they appear normal and perfectly capable. Over time, they must learn the fine art of managing their condition and learning to say no to many of the activities and chores most people take for granted.

Some people who live with chronic conditions and the associated limitations grow tired of being tired. They sometimes decide to challenge their limitations or else let limitations be overridden by the sheer force of their willpower. Some people have the mental determination but lack the corresponding physical stamina. It's as if the mind and physical body vibrate at different frequencies and race or rest at entirely different levels. In your mind, you might have all of these ideas or projects that you'd like to accomplish, articles you'd like to write, jobs you'd still like to hold. You are still an energetic person even though your body may not be energetic. It's difficult to merge the desire to do, with a body that is unable to accomplish what the mind wants to direct.

The person must always remember to get past the guilt that is sometimes associated with taking frequent rest periods during the day. The person who lives with chronic symptoms must constantly play the trade-off game. What can I cut today? What has to be done, and what can be shelved for awhile? How can I save my energy? How can I reshuffle the responsibilities before me so that I can maintain my stamina? If they don't alter their lives in significant ways by juggling, pacing, and simplifying activities, symptoms can become even more difficult to manage and adjust to.

Let's face it. Human nature, for the most part, is visually oriented. We believe what we see and often make character judgments based solely on visual perceptions. Society is simply not attuned to the needs of these people with easily concealed disorders such as chronic pain. While many feel compelled to help someone with an obvious physical challenge, they may respond negatively when asked to help or provide special accommodations to someone who appears healthy and looks just fine.

But, what happens when the person who appears healthy, energetic, and just fine to family, friends, and coworkers, is quietly suffering with chronic pain. Often, they simply learn to play the part of being pain-free. "Sometimes I think I should just go into acting!" Shawna laughs. She lives with the painful symptoms of endometriosis and already feels like a seasoned actress. "I should win an Oscar for some of the roles I've had to play in trying to hide my pain and symptoms from others."

Marilyn was brought up to do everything for herself. Independent by nature, she certainly does not like to admit that she needs help. "I'm sure I give the impression that I don't have a problem with pain, and that confuses people. On the one hand, I'm saying 'I hurt and am tired,' but if they look at me, they don't see the pain and fatigue."

"It's discouraging and demoralizing to be in pain every single day," says Peggy, who lives with the chronic symptoms of fibromyalgia, a condition characterized by widespread musculoskeletal pain. It is one of many heterogeneous illnesses; meaning that each person may experience the same disorder in a variety of ways. "Sometimes I become overwhelmed with the pain. It wears me down and takes a lot of explaining because most people cannot relate to being in pain all of the time."

Chronic pain is real. Yet it is sometimes difficult to talk to friends and family members about it. Not only do people want to be free of chronic pain, they do not want to feel like a burden. "It's a large part of who I am. I just don't want to be pitied by my friends or be known as 'the suffering one' to those I love or the people I work with," says Donna. "Everyone becomes tired of hearing about how much I hurt, including myself! Some people think I am making it up or exaggerating my symptoms. But chronic pain is my reality. Even the medical community doesn't always take my pain seriously."

Pain management seems to be at the vortex of incompatible agendas among government watchdogs, insurance companies, doctors, and patients. Fear of addiction is the key issue. Concerned that the patient will become addicted, doctors are sometimes hesitant to prescribe painkillers. However, individuals with chronic pain, or those who require significant pain management, rely on analgesics just as a diabetic depends on insulin. Erica, a mother of three, counts herself fortunate. "I now have a good doctor who does not shy away from the issue of pain management. He prescribes the appropriate medication when needed and necessary. I go about my life, but when the disease acts up, I'm not afraid to medicate and then move on."

Some people who live with painful conditions feel the need to be stoical about their plight. They see their pain as a sign of weakness, or feel their character is being disparaged because of their suffering, and thus refuse to manage their symptoms with effective treatments. They deprive themselves of the relief that may allow them to regain a better quality of life.

Jill is just beginning to learn the intricate rules of the pacing-game. It is terribly tempting for her to try to play catch-up when she happens to have a good hour or day. "Sometimes I start to feel better, get excited, and feel that I should take advantage of feeling better. So I begin to get things done and end up doing too much. I've only just learned that instead of trying to get a lot done while feeling well, I need to temper that phase so that I don't 'crash.' This is so obvious and yet so hard to do, because it is exciting to feel good."

The struggle of pain and exhaustion is a daily concern. Fatigue is a part of many chronic conditions, and making a place for this unrelenting weariness becomes a way of life. After awhile, it becomes impossible to remember what it is like not to feel exhausted. It is important to recognize that you may be able to perform some of your previous activities, but less frequently or for a shorter duration of time. People with chronic disorders can learn to work with their bodies and to recognize their limitations, but it often takes a bit of time and some trial and error.
People who live with concealed disorders talk in terms of good days and bad days. Performing one major task per day helps many people to manage their world, even if only in small bites. Deciding what is a priority and what can wait becomes a new and crucial skill. Pacing and juggling tasks and pleasurable activities become skills that optimize chances for a manageable life.

Families may have to find a new way of playing and having fun together when one member of the family becomes chronically ill. Old hobbies and recreational trips may make way for new and creative replacements. Playing board games rather than camping, for instance, can keep families connected and involved in each others' lives.

Delegating is a crucial survival skill to develop. This is often difficult especially if you link self-worth to tasks completed. For instance, if you live with chronic back pain or fibromyalgia, you may have difficulty asking the grocery store-bagger to assist them to the car. Accustomed to being self-sufficient, many with pain may equate help with weakness and the loss of dignity. They desperately want to remain self-sufficient and resist surrendering to fatigue and pain. Others, however, may regard such a request as a way to reserve and restore some energy and preclude the most persistent pain. It's important to keep at the forefront of your mind that learning to accept help can actually be a favor to others. Accepting help from friends and family may be easier if you look at it in this way: You are helping them to cope.

But what is chronic pain really like to live with? If you have not experienced it, it is difficult to describe such a steadfast companion. Albert Schweitzer once said, "Pain is a more terrible lord of mankind than even death itself." Many who have lived with chronic pain would echo those sentiments. However, is crucial to remember that, in the end, nothing can compromise your spirit. You are not your pain. You are not your illness. Look at your physical state of health as the movement of the ocean. There will be mighty waves at times, but in other hours or future days, the tide will also recede, and there will be calm periods where you will find relief. The good news is that we have the capacity to make some issues foreground and some issues background in our lives. This is a choice that we can make all day long throughout our lives. What part of your illness can you put in the background, if only for a short while? Have a visit with someone you love. For that moment, you can welcome your joy to the foreground, even if only slightly. Keep inviting those inspiring, meaningful moments into your life. Most of the time, life is all about small and precious moments.

The goal is not necessarily to become well, but to learn to accept what life has handed us. It may not always be a welcome gift-this one of chronic symptoms and unpredictable days-but rather an opportunity to learn more about our inner strength and the importance of the people in our lives. It is also a chance to use the gifts within that would have otherwise sat dormant. Sometimes, learning to be compassionate with ourselves is a far more difficult assignment than caring for others. Fortuitously, chronic physical pain or symptoms often force us to care for and about ourselves in new and profound ways.

Learning to feel hopeful again is a vital stage towards acceptance of our condition and making peace with the pain. Today, there are more beneficial treatments and resources for chronic pain than ever before. Support groups for specific conditions meet regularly, both in person and online. Camaraderie and information are only a mouse click or telephone call away. Numerous organizations for particular illnesses and conditions, such as the National Pain Foundation provide educational information to those with health challenges and to their family members. There is also a vast amount of research underway. Medical scientists and alternative healthcare professionals are learning to recognize, control, and in some cases prevent, a host of chronic disorders. There are many reasons to feel hopeful if you shift your focus and maintain awareness that the future holds possibilities that cannot even be imagined today.

No one chooses to have a chronic illness or experience unyielding pain. When struck with a disability, people usually feel compelled to return to their former selves and rejoin previous routines. Almost primordial in nature, we intuitively ache for a bygone era when life was predictable and comfortable. To accept a new and limited way of functioning is not very appealing. Why should a person want to live happily in a body that is out of order? There are many who fight an illness or condition that seems resistant to interventions. Some become gifted at taking flight. They ignore and run from a chaotic set of symptoms that has shattered their habitual schedule. Despite the frustrations and various stages of grief, most people eventually move on and coexist in relative peace with their disorder. They learn to dance with the pain using new steps and discover what they can now do rather than what they used to do. Having lost the ability to kick up their heels, they learn to tap their toes to a new tune, perhaps a bit closer to the ground.

Carol Sveilich, M.A., is a group facilitator and counselor in San Diego, California. Her background includes conducting support groups for those with health challenges, serving as an academic counselor, and developing newsletters, columns and articles to assist and educate others with chronic health disorders. Called by many a "support group in a book" and endorsed by medical and mental health professionals, as well as by actor, ED ASNER, former Miss America, MARY ANN MOBLEY, and San Diego Chargers, ROLF BENIRSCHKE, Sveilich's new book Just Fine: Unmasking Concealed Chronic Illness and Pain is now available directly from the author or may be ordered through bookstores everywhere. FREE COPING TIPS may also be requested from the author via email: writefaceforward@yahoo.com