Treatment

Although there is still no cure for cystic fibrosis, there are effective methods of treatment. Today, more and more youngsters are reaching adulthood with a minimal amount of lung damage. The discovery of the CF gene holds the promise of better methods of treatment in the future. Knowledge of the CF gene is allowing investigators, for the very first time, to trace the links between the cause of the disease and all its signs and symptoms.

PULMONARY

The primary focus of CF treatment is on pulmonary problems since most CF illnesses and deaths are due to lung infection and dysfunction. The goals of pulmonary therapy are to loosen and thin out the thick mucus that clogs the airways, and to maintain good lung function. Several methods are used.

To prevent or combat lung infections, affected individuals may take antibiotics frequently. Special aerosol solutions may be used to thin and loosen the mucus in the lungs as well.

"Postural drainage" is used to help maintain clear airways. This is a form of physical therapy in which the child is placed in special positions, depending on the region of the chest to be drained, and "clapped" over the lobes of the lungs. Often, a great deal of mucus may be cleared in this way. Parents are instructed on how to perform postural drainage and "clapping"- hitting with a cupped hand- so that they can carry out the procedure at home.

Recent studies have demonstrated that controlled breathing against a positive end-expirator pressure (PEP) mask is also a very effective mucus clearance technique. The "PEP mask" gives independence to patients who are old enough to perform the techniques reliably.

In very advanced cases, the lungs may become so badly scarred from constant infections that the only solution is a lung transplant.

GASTROINTESTINAL

The gastrointestinal problems associated with cystic fibrosis occur in approximately 85% of patients, and result in disturbed digestion and malabsorption of fats, proteins, and carbohydrates. Before treatment, a person with CF may consume twice the quantity of food that a healthy person would eat. Persons with CF usually require special pancreatic enzyme supplements to aid digestion, a special diet with increased calories and protein, and vitamin supplements including vitamin E, and sometimes vitamin K.

Pancreatic enzymes, which are taken orally, correct most of the deficits caused by the disease. They help the body absorb nutrients from food, and reduce both the number and bulk of stools, and the amount of flatulence, abdominal pain, and distension.

In addition, persons with CF need to add salt to their diet to replace the excessive amounts they lose in their sweat.