Controlling Asthma

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."

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How We Breathe: Understanding Oxygenation of the Blood

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.

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What is a Pulmonary Exacerbation?

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.

Nasal Irrigation

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

Understanding B. Cepacia

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

Different Types of Coughs

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)

Recognizing the Dangers of MRSA and CAMRSA

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.

Therapies that Use Positive Expiratory Pressure (PEP)

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

What is Bronchiectasis?

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.

What You Need to Know About Pseudomonas Aeruginosa (PA)

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

Bronchodilators and Nebulized Medications

"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.

When Your Spouse Doesn't Believe You're Ill

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.

1. 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.
2. 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.
3. 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.
4. 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.
5. 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.
6. 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

Types of Airway Clearance Devices

Cystic fibrosis patients have abnormally thick, stick mucus that is difficult to remove from their airways. In order to prevent infection a combination of medications and airway clearance techniques are used. In the past, the methods of airway clearance we cumbersome, time consuming, and, at times, downright uncomfortable for both patient and care giver. One such example is the "clapping" technique. Sometimes referred to as "beatments" or "thumps" by CF sufferers, the process involved having a caretaker cup his or her hands and physically beat the patient's back in a rhythmic manner. By clapping each section of the lung's lobes, the mucus was dislodged to a point that it could be coughed up and spit out.

Over the last decade many developments have been made in the world of airway clearance devices that make expelling the mucus much easier. Although some patients do still prefer the clapping methods to which they have grown accustomed, newcomers to the disease have many options for airway clearance.

Intrapulmonary Percussive Ventilator (IPV)

This device, available in some hospitals and often called the "Percussion Air" stimulates the airways by directing several bursts of air into the lungs at a predetermined rate. A pneumatic flow interuptor sends a pulsing flow of air at a rate of 100-300 blasts or "cycles" per minute. The device is attached to a special mouthpiece and nebulizer cup so that the patient can benefit from medication simultaneously with the percussion treatment. Due to the high frequencies at which the air pulses, some patients have difficultly tolerating such a method. However, great benefits exist for those who have good posture and have been properly instructed in the technique of using and IPV.

The Flutter(r) Mucous Clearance Device

The Flutter is a small, handheld device that creates positive expiratory pressure (PEP) in order to stimulate the lungs to vibrate or "flutter" enough to dislodge the sticky mucous. The device itself consists of a weighted metal ball resting in a cone that sits atop a diaphragm through which air can flow. When the patient exhales, the cone and ball move around creating the "fluttering" motion that results in PEP. This causes pressure in the airways, keeping them open longer than would happen just through simple tidal breathing. The cap on the end of the device can be twisted to different settings to make the pressure lesser or greater depending on the patient's ability.The range of benefit of the Flutter depends largely on the patient's ability to exhale against the device. Longer, sustained breaths and the use of a technique called "huff coughing" are the best ways to benefit from using the Flutter.

Acapella (tm)

Similar to the Flutter, the Acapella employs the use of positive expiratory pressure. This device is considerably larger than the Flutter, but has a greater range of resistance than can be "dialed in" on one end. One advantage of the Acapella is that the t-piece of a nebulizer can be attached to it, allowing the patient to inhale medication while also engaging in airway clearance. The only drawback is that many home nebulizer-compressor systems do not deliver a high flow rate through the nebulizer cup. This results in wasted medication and decreased benefit for the user. In the hospital setting, however, the air flow can be adjusted to a rate that is high enough to push the medication through the device and reach the mouthpiece, ultimately benefiting the patient. Most often, the Acapella is used independently of nebulizer treatment.

Vests

The most favored and beneficial airway clearance device used by CF patients is the Vest. Different models are available from different companies, and determining which one to use is left to personal preference. The vest works by using a method called high frequency chest compression. The Vest is inflatable, and is connected to a compressor by two air hoses. Once switched on, the vest fills with air, and the air begins to oscillate, thereby loosening the mucus. The frequency (measures in Hertz) and Pressure (psi) can be adjusted according to the needs of the individual. Some patients wear their Vests while exercising, or while inhaling aerosolized medication. The technique of "huff coughing" is also applied while a patient is undergoing a vest treatment. Huff coughing is a type of deep exhalation that enables the airways to stay open longer. It's similar to how a person would breathe if they wanted to see their breath in the cold air, or breathe fog onto a window. When breathing in this way during a vest treatment, it's easier for the patient to cough up the mucous that is being dislodged.

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Additional Resources:

American Thoracic Society List of Airway Clearance Devices (includes Insurance and Cost Information for Acquiring Them)

How To Huff Cough Effectively

Respirtech inCourage vest

The Flutter Valve (From Axcan Pharma)

Hill-Rom Vest

Acapella PEP Therapy

Percussionaire Company

Anti-inflammatory Agent: Corticosteroids

Public domain image courtesy of National Library of Medicine (NLM)

Prednisone is perhaps the most recognizable and most prescribed anti-inflammatory agent for cystic fibrosis patients. This corticosteroid has been proven effective in suppressing inflammation of the lungs. Studies have demonstrated that doses of 60mg are useful in lowering the IgE levels of patients who suffer from allergic bronchopulmonary aspergilliosis (ABPA.)

Despite its success in treating inflammation, many patients who have undergone corticosteroid therapy report unfavorable side effects. The most challenging side effect is the onset of steroid-induced diabetes. Blood glucose levels must be monitored frequently when a patient is being treated with prednisone. Rapid weight gain due to fluid buildup is also common. This can cause something called “moon face” which is when most of the weight makes the patient’s face appear quite puffy and round. Frequent usage of prednisone has been linked to osteoporosis, which is already a co-occurring condition in patients with CF.

A study conducted in 1999 suggested that ongoing treatment with prednisone may have adverse effects on a patient’s growth pattern. This was observed most in male patients who had not yet gone through puberty.

The benefits of corticosteroids are best observed when they are used to treat and control the effects of asthma. For example, oral soluble prednisolone (Prednisone), inhaled budesonide (Pulmicort Turbuhaler) or fluticasone proprionate (Flonase intranasal spray) are helpful particularly in young patients who are wheezy and have associated asthma.

In general, corticosteroids such as prednisone are prescribed when other attempts to reduce inflammation of the airways have failed.

Overview of Inflammation Treatment

The airways of patients with cystic fibrosis (CF) are in constant danger of becoming inflamed. Airborne pathogens such as allergens and pollutants are the leading cause of inflammation. Viral or bacterial infections can also cause the airways to tighten, making breathing more difficult, and reduce a CF patient’s ability to perform mucus clearance maneuvers. It is believed that the lungs of cystic fibrosis patients are prone to inflammation as a result of the malfunction of the CFTR protein.

Repeated infection, especially as a result of mucoid Pseudomonas aeruginosa (PA) can result in lasting damage to lung tissue. PA has also been observed to cause a hyperimmune inflammatory response. In other words, the body reacts to PA even more strongly than it does to other bacterial infections.

Lung inflammation is present even in patients who are not exhibiting signs of active infection. Again, this is due to the body’s inability to utilize the chloride ion transport across cells. Studies conducted on newborns with cystic fibrosis have hypothesized that at birth the lungs are still normal. However, with each subsequent infection, damage to the airways becomes cumulative as the airways continue to narrow. In order to avoid the lasting effects of inflammation, it is important for cystic fibrosis patients to work with their physicians to determine an appropriate preventive maintenance routine.

One of the prevailing treatments to control inflammation includes the use of high dose ibuprofen. Typically this is effective for younger patients who have not yet experienced many pulmonary exacerbations. Other therapies include:

1. Corticosteroids[1]
2. Macrolide antibiotics[2]
3. NSAIDs (non-steroidal anti-inflammatory drugs)[3]
4. Defensins and cathelicidins[4]
5. Leukotriene receptor antagonists[5]
6. Alpha-1-antitrypsin and secretory leukoprotease inhibitor (SLPI)[6]

For more information, talk to your CF specialist or pulmonologist about whether preventive anti-inflammatory agents is appropriate for you or your child.

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[1] Medications (eg, prednisone) that are related to cortisone, a naturally occurring hormone. Corticosteroids lessen inflammation, swelling, and pain. In some cases, corticosteroids are injected directly into a joint; however, they can have serious side effects - such as damage to bones and cartilage - especially when used over long periods of time.

www.ehealthmd.com/library/osteoarthritis/OSA_glossary.html

[2] The macrolides are a group of drugs (typically antibiotics) whose activity stems from the presence of a macrolide ring, a large lactone ring to which one or more deoxy sugars, usually cladinose and desosamine, are attached. The lactone ring can be either 14, 15 or 16-membered. Macrolides belong to the polyketide class of natural products.
en.wikipedia.org/wiki/Macrolides

[3] Non-steroidal anti-inflammatory drugs, usually abbreviated to NSAIDs, are drugs with analgesic, antipyretic and anti-inflammatory effects - they reduce pain, fever and inflammation. The term "non-steroidal" is used to distinguish these drugs from steroids, which (amongst a broad range of other effects) have a similar eicosanoid depressing anti-inflammatory action. NSAIDs are sometimes also referred to as non-steroidal anti-inflammatory agents/analgesics (NSAIAs). ...
en.wikipedia.org/wiki/NSAID

[4] Similar to antimicrobial peptides. These peptides are excellent candidates for development as novel therapeutic agents and complements to conventional antibiotic therapy because in contrast to conventional antibiotics they do not appear to induce antibiotic resistance while they generally have a broad range of activity, are bacteriocidal as opposed to bacteriostatic and require a short contact time to induce killing. A number of naturally occurring peptides and their derivatives have been developed as novel anti-infective therapies for conditions as diverse as oral mucositis, lung infections associated with cystic fibrosis (CF) and topical skin infections. Source: wikipedia

[5] Leukotriene receptor antagonists, such as montelukast sodium (e.g. Singulair tablets) or zafirlukast (e.g. Accolate tablets), treat asthma via a totally different pathway to other available medicines. They work by blocking substances in your lungs called leukotrienes, which cause narrowing and swelling of the airways. Blocking leukotrienes can improve asthma symptoms, including night-time symptoms, and can help prevent asthma attacks. http://www.mydr.com.au/default.asp?article=2700

[6] Acid-stable proteinase inhibitor (elastase, cathespin) against proteinases liberated from liberated from cervical mucus disintegrating leukocytes; from sputum in bronchial infections. source: http://cancerweb.ncl.ac.uk/cgi-bin/omd?antileukoprotease

What is Mycobaterium Avium Complex (MAC)?

Mycobacterium is a type particularly strong organism that has been known to cause tuberculosis leprosy and Mycobacterium avium complex (MAC). There are two main classifications of Mycobaterium: fast-growing and slow-growing. Mycobaterium are primarily found in water and food. Even water that has been disinfected with chlorine can still harbor a member of the Mycobacterium family. There are strains of non-tuberculosis mycobacterium (NTM) that are generally referred to as “TB cousins.”

How Prevalent is It?

People with cystic fibrosis are more susceptible to infection than their healthy peers. According to a study published in 2002 in the American Journal of Respiratory and Critical Care, Mycobacterium Avium Complex (MAC) was cultured in over 70% of the test subjects.

Causes and Symptoms

Avoiding contact with some source of MAC is virtually impossible. Animal, water, air, food, soil, tobacco products are all suspected vectors for the mycobacterium avium complex. Unlike B. Cepapcia complex, Staph Aureus, Psuedomanas Aeruginosa, or other infectious conditions common in the cystic fibrosis community, MAC is not spread through person-to-person contact. This The most common TB Cousin, MAC, presents itself like many other illnesses. Without a the conclusive results from a sputum sample, MAC may be misdiagnosed as just another pulmonary exacerbation, or the flu. Common symptoms of infection include:

• Fever

• Chills

• Swollen glands

• Nigh sweats

• Fatigue

How it is Diagnosed

MAC is diagnosed by drawing blood to determine the function of the liver. This is because the condition causes the number of liver enzymes to be elevated. A sputum culture is also necessary for detecting the presence of MAC.

Treatment

Treating MAC is difficult, especially becaue it typically requires dual antibiotic therapy. Medical professionals agree that Azithromycin should be one of the drugs used. However, this can be challenging, especially for CF patients who are already taking a prophylactic dose of azithromycin. Unfortunately, MAC cannot be eradicated by the usual 10 day course of antibiotics. Treatment for the condition is ongoing and life-long.

MAC fact sheet

Multicenter Prevalence Study in CF

Patient Registry Report (Part 4)

Preventing Exacerbations and Prolonging Lung Health

Preventive maintenance is an important part of living well with cystic fibrosis. Over the last two decades, research has opened the doors to many new medications and therapies for the treatment of CF. As a result, the number of adults with severe lung disease is decreases. In fact, the number of patients with severe lung disease in 2005 is only half of what it was in 1985. Adherence to a preventive treatment regime enables more patients to reach adulthood with only moderate lung disease.

Frequently prescribed medications that are used to prevent exacerbations include:

-TOBI
-Pulmozyme
-Zithromax
-Ibuprofen

Each of these has been proven effective in promoting lung health and reducing the risk of potentially damaging infections.

TOBI is a formulation of the antibiotic, tobramycin. Nebulizing this medication instead of receiving it as an infusion helps direct more of the drug directly to the site where the infection is occurring. Patients who culture P. aeruginosa (PA) and have moderate to severe lung damage are those who are likely to benefit from TOBI inhalation solution. Nearly 58% of patients included in the 2005 CF Patient Registry are using TOBI.

Another preventive antibiotic used to fight P. aeruginosa is Zithromax. In the mainstream medical environment, this drug is prescribed as a powerful 3 day course of oral antibiotics. Patients with CF are prescribed this antibiotic on an ongoing basis.

Pulmozyme is a mucolytic (i.e. mucus cutting) medication. Although some patients have difficulty tolerating it, approximately 70% of patients with moderate to severe lung disease use it.

Ibuprofen is an over-the-counter medication called NSAID (non-steroidal anti-inflammatory drug.) CF researchers have studied the benefits of prescribing high doses of ibuprofen for pediatric patients aged 6-12 with CF. The goal is to prevent infection that often follows lung inflammation. A mere 5% of patients use ibuprofen as part of their treatment.

The goal of these therapies is to reduce the number of exacerbations patients experience each year. Each exacerbation has the potential to cause irreversible lung damage. The longer a patient can go without an infection, the better the chances of reaching adulthood with only mild to moderate disease severity. As treatment options and preventive care progresses, more and more patients are able to stay infection free. In 2005 nearly 65% of CF patients reported not succumbing to a lung infection. This is a growing trend, and one that the CFF hopes will continue.

The following is the list of recommendations by the CF Foundation for avoiding infection:

1) Getting a flu shot every fall for you/your child and everyone living in the house;
2) Regular exercise to strengthen your muscles;
3) Doing airway clearance to keep the lungs as clear as possible of mucus;
4) Taking the medicines as prescribed by your CF care center;
5) Telling your CF care center early when you or your child do not feel well; and
6) Avoiding germs by using good hand hygiene and properly cleaning and disinfecting equipment



Related Links:

Use of TOBI (Tobramycin) inhalation solution
TOBI Reduces Occurrence of Hospitalizations for CF Patients
Azithromycin (Zithromax) in CF Patients
Ibuprofen instead of Corticosteroids for CF
Effectiveness of Pulmozyme in Infants Being Studied
The Importance of Being Compliant (blog entry)

Understanding and Preventing Mold Growth

Indoor air quality is a topic of great importance to everyone, but particularly to persons with cystic fibrosis.

Understanding Mold Reproduction and Growth

In order to reproduce, mold generates what are called "spores." Spores are small enough to drift through the air in a home with ease. Mold thrives when it has a chance to settle in a damp, dark area. Some types of mold are particularly fond of warm environments. Mold is exceptionally resilient and is opportunistic, meaning it will lie in wait for just the right conditions to reproduce, even if that means waiting for a long time.

The warm, moist environments like the human body provide an ideal setting for mold spores to reproduce and colonize. Effective way to control mold is to control moisture.

At some point, the colony generates additional spores, which may become airborne. Some are small enough to be respired into the human body where they irritate the small airways, thus triggering allergic reactions in some individuals.

Controlling the Mold

Despite the fact that there is no way to remove all mold and mold spores from the home, there is good news. Certain things can be done to effectively control mold and avoid serious health effects such as allergic reactions. Furthermore, dealing with potential moisture issues is beneficial to the soundness of your home.

I make a point to combat mildew and mold in my home to the greatest extent practicable. We have laminate floors in the bedroom and our entire downstairs, since those are the areas where we spend the most time. By having less carpet, there is less likelihood that spores being tracked in from outside will lie unnoticed in the carpet threads.

The bathroom can be a breeding ground for mold and mildew since there is a lot of moisture there. When showering, it is a good practice to run the exhaust fan. Once a week I use Tilex in our sinks and in the shower to kill off anything that may be growing in there.

In the kitchen we make good use of our refrigerator's built-in humidity control function. This keeps mold from taking hold of the soft cheeses and other foods that may be potential breeding grounds.

People with allergies and CF should look into HEPA (High Efficiency Particulate Air) filter systems. When an air filter unit is categorized as a true HEPA, its ability to remove certain contaminants from the air is 99.97% effective at removing particles that are .3 microns and larger in diameter. A micron is one millionth of a meter; a grain of salt is approximately 60 microns in size. Mold spores can range from 1-60 microns in diameter. The smaller the diameter, the easier it is for the pathogen to get into the body's small airways and cause irritation.

Conclusion

Mold spores can lie dormant for long periods of time and can reproduce again when just the right conditions are in place. Although mold cannot be completely avoided, and eradicating the offending spores if difficult but not impossible. With minimal effort and effective control measures, a home's overall indoor air quality can be a more healthful environment.

This article has been adapted from the article "Limiting Exposure to Mold in the Home" by Lauren G. Beyenhof. Please read the full article on Associated Content.