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Cystic Fibrosis (CF) is a genetic disease in which mucus builds up in the body, causing blockage in some organs (especially the lungs and pancreas).  CF is very dangerous, and there are varying degrees of severity of this disease that result from the type of mutated gene that a patient has.  Mucus blocking the lungs causes breathing difficulty, inflammation, swelling, and increased infection (mucus causes microbes or germs to get stuck in the airways where they build up more quickly than the body can destroy them).  When mucus blocks the pancreas and digestive tract, the enzymes needed for the body to break down food are prevented from getting where they are needed.  The result is that the body cannot break down food like it is supposed to, and the patient does not get the nutrients he/she needs from food.  This affects the patients’ health and growth.  CF occurs when a person gets a mutated or defective CF gene from both parents (if they only inherit a defective gene from one parent, they do not have CF but are “carriers” who can pass on the disease to their children).  A child whose parents are both carriers has 1 out of 4 chance of inheriting both copies and having CF, a 1 out of 2 chance of inheriting one defective copy and being a carrier of the CF gene, and 1 out of 4 chance of having two normal copies of the gene.  There are around 30,000 people (children and adults) in the US with CF, and there are 10 million more who are carriers of the defective CF gene.  Caucasians are more likely to be affected by CF, but all races can be affected.

Though symptoms of CF are individual to each patient, there are common symptoms that frequently occur in CF patients in general.  Also, the CF patient may have more or less severe symptoms depending on the specific type of mutation in their CF gene.  Some common CF symptoms are:

  • Persistent cough, sometimes producing phlegm (thick mucus)
  • Shortness of breath and/or wheezing
  • Frequent occurrence of lung infections (such as pneumonia)
  • Salty taste on the skin
  • Failure to grown or gain weight normally, even with normal eating patterns
  • Abnormal stools (constipation or difficulty with bowel movements or frequent bowel movements that are greasy and bulky)

Treatments of CF

There is no cure for CF, but there are many drug therapies that, along with proper nutrition, allow CF patients to live long healthier lives.  There are special care centers that work with CF patients to provide the best care possible for each individual with CF.  Some common medications used in treatment of CF are:

  • Mucus-thinners (or mucolytic agents): enzymes that help break down components of mucus, thinning the mucus so the body can clear it from the patient’s lungs (examples: inhaled saline and Pulmozyme (dornase alfa)
  • Pancreatic enzymes: medications taken to supplement lack of normal enzymes
  • Bronchodilators: these drugs open up the airways by relaxing the smooth muscles in the chest and lung area.  Care must be used in deciding to give bronchodilators to CF patients that are children because sometimes children have the opposite expected reaction to the medications (their airways close up). (examples: albuterol, Proventil, Ventolin)
  • Vitamins: used to supplement what the patient needs but does not absorb from food
  • Antibiotics: used to treat the frequent infections suffered by CF patients.  CF patients often clear antibiotics more quickly than average and are often frequent antibiotic users, and therefore they are more likely to be colonized (when bacteria live in a person’s body at low levels, putting the patient at higher risk of overgrowth of these bacteria leading to infection with resistant bacteria when the average person.  As a result, CF patients often require treatment with varied types of antibiotics including those with unusual routes of administration in order for the infection to be cleared. (example: Tobi)
  • Cystic Fibrosis Transmembrane Conductance Regulator: Improves the regulation of salt and water absorption and secretion in various tissues in the lungs and GI tract.  (Kalydeco- ivacaftor)

For more information, please visit:

The Cystic Fibrosis Foundation

References:

“Cystic Fibrosis, “ Medscape Reference: Cystic Fibrosis

“About Cystic Fibrosis: Frequently Asked Questions,” Cystic Fibrosis Foundation

“Cystic Fibrosis Treatment and Management,” Medscape Reference: Cystic Fibrosis

Services provided by Avita Pharmacy are not intended to replace the services of a physician. This information is provided for informational purposes only and is not a substitute for professional medical advice. You should consult a physician in all matters relating to your health, and particularly in respect to any symptoms that may require diagnosis or medical attention.