Table of Contents Information on Cystic Fibrosis? How Common Is Cystic Fibrosis? Cystic Fibrosis Symptoms Suspecting a Child May Have Cystic Fibrosis Cystic Fibrosis Symptoms & Diagnosis What Makes Cystic Fibrosis a Genetic Disease? Cystic Fibrosis Treatment How the Gene Mutation Causes Cystic Fibrosis Gene Therapy as a Cystic Fibrosis Treatment Detecting Cystic Fibrosis in an Unborn Baby Can Cystic Fibrosis Be Prevented? How Patients and Their Families can Cope Keeping on Top of Your Conditio

Cystic Fibrosis Treatment

At present, neither gene therapy nor any other kind of treatment exists for the basic causes of cystic fibrosis, although several drug-based approaches are being investigated. In the meantime, the best that doctors can do is to ease the symptoms of cystic fibrosis or slow the progress of the disease so the patient's quality of life is improved. This is achieved by antibiotic therapy combined with treatments to clear the thick mucus from the lungs. The therapy is tailored to the needs of each patient. For patients whose disease is very advanced, lung transplantation may be an option.

CF was once always fatal in childhood. Better treatment methods developed over the past 20 years have increased the average lifespan of CF patients to nearly 30 years. These treatment approaches are detailed more fully below:

• Management of lung problems

  A major focus of cystic fibrosis treatment is the obstructed breathing that causes frequent lung infections. Physical therapy, exercise, and medications are used to reduce the mucus blockage of the lung's airways.

  Chest therapy consists of bronchial, or postural, drainage, which is done by placing the patient in a position that allows drainage of the mucus from the lungs. At the same time, the chest or back is clapped (percussed) and vibrated to dislodge the mucus and help it move out of the airways. This process is repeated over different parts of the chest and back to loosen the mucus in different areas of each lung. This procedure has to be done for children by family members but older patients can learn to do it by themselves. Mechanical aids that help chest physical therapy are available commercially. Exercise also helps to loosen the mucus, stimulate coughing to clear the mucus, and improve the patient's overall physical condition.

  Medications used to help breathing are often aerosolized (misted) and can be inhaled. These medicines include bronchodilators (which widen the breathing tubes), mucolytics (which thin the mucus), and decongestants (which reduce swelling of the membranes of the breathing tubes). A recent advance, approved by the Food and Drug Administration, is an inhaled aerosolized enzyme that thins the mucus by digesting the cellular material trapped in it. Antibiotics to fight lung infections also are used and may be taken orally or in aerosol form, or by injection into a vein.

• Management of digestive problems

  The digestive problems in CF are less serious and more easily managed than those in the lungs. A well-balanced, high-caloric diet, low in fat and high in protein, and pancreatic enzymes (which help digestion) are often prescribed. Supplements of vitamins A, D, E, and K are given to ensure good nutrition. Enemas and mucolytic agents are used to treat intestinal obstructions.