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Cystic fibrosis (CF)
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Cystic fibrosis (CF)

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Cystic fibrosis (CF) - a life-threatening disorder that causes severe lung damage and nutritional deficiencies - used to be a genetic mystery, and most people with the disease didn't live beyond their teens. But researchers have made progress in unraveling the genetic basis of CF, which has led to earlier detection. In addition, improved and more consistent treatments now allow people with CF to live into their 30s and 40s and even beyond, and to have fuller, more comfortable lives.


Many treatments exist for the symptoms and complications of CF. The main goal is to prevent infections, reduce the amount and thickness of secretions in the lungs, improve airflow, and maintain adequate calories and nutrition.

To accomplish these objectives, treatments for CF may include:

  • Antibiotics. Newer antibiotics may more effectively fight the bacteria that cause lung infections in people with CF. Among these are aerosolized antibiotics that send medication directly into airways. One of the major drawbacks of long-term use of antibiotics is the development of bacteria that are resistant to drug therapy.

  • Mucus-thinning drugs. When your white blood cells attack bacteria in your airways, DNA in the cells is released, making the mucus in your airways even thicker. The aerosolized drug dornase alfa (Pulmozyme) is an enzyme that fragments DNA, making mucus thinner and easier to cough up. Side effects of Pulmozyme may include airway irritation and sore throat. 

  • Bronchodilators. Use of medications such as albuterol (Proventil, Ventolin) may help keep open the bronchial tubes by clearing thick secretions.

  • Bronchial airway drainage. People with CF need a way to physically remove thick mucus from their lungs. This is often done by manually clapping on the front and back of the chest — a procedure that's best performed with the person's head over the edge of the bed so that gravity helps clear the secretions.

In some cases an electric chest clapper, known as a mechanical percussor, is used. An inflatable vest that vibrates at high frequency can also help people with CF cough up secretions. Both adults and children with CF need to have bronchial airway drainage at least twice a day for 20 to 30 minutes. Older children and adults can learn to do this themselves, especially if they use mechanical aids such as vests and percussors. Young children need the aid of parents, grandparents or older siblings.

  • Oral enzymes and better nutrition. CF can cause you to become malnourished because the pancreatic enzymes needed for digestion don't reach your small intestine, preventing food from being absorbed. As a result, you may need many more calories than you otherwise would. Supplemental high-calorie nutrition, special vitamins and enteric-coated oral pancreatic enzymes can help you maintain or even gain weight.

  • Lung transplantation. Your doctor may suggest lung transplantation if you have severe breathing problems, life-threatening pulmonary complications or increasing resistance to antibiotics used to treat lung infections. Whether you're a good candidate for the procedure depends on a number of factors, including your overall health, certain lifestyle factors and the availability of donor organs. Because both lungs are affected by CF, both need to replaced. If your chest isn't large enough to hold two adult donor lungs, your surgeon is likely to use two lower lobes contributed by two living donors. However it's performed, lung transplantation is a major operation and may lead to serious complications, especially post-surgical infections. The five-year survival rate has increased in the last 10 years, however.

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