Sunday, September 28, 2008
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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Monday, August 25, 2008
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."
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.
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."
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:
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."
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.
Sunday, August 24, 2008
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?
Thursday, August 7, 2008
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.
Natural High Fiber Foods
Wednesday, August 6, 2008
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.
Tuesday, August 5, 2008
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.
Monday, August 4, 2008
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.
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
For more information about family planning when cystic fibrosis is a concern, please visit the following site:
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.
Sunday, August 3, 2008
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.
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
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.
Odds that both parents are carriers: 1 in 625
Chance of having a child with CF: 1 in 2500
Mutation detection by screening: 90%
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%
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%
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.