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Alexander Marmureanu

Alexander Marmureanu

Cystic Fibrosis

Cystic fibrosis (CF) is a severe, genetically-determined disease involving the lungs, the gastrointestinal tract, and other organs. With cystic fibrosis, the body produces abnormally thick, sticky mucus (sputum). This mucus clogs the lungs, causing lifethreatening lung infections, and obstructs the pancreas, impairing the ability of the intestines to break down food.

Cystic fibrosis occurs in about 1 in 2,000 live births among white children and at a far lower rate in black and Asian children. Boys and girls get cystic fibrosis equally. At present, only 50% of those diagnosed with CF as infants or young children live beyond their late twenties. However, that percentage is rising as an increasing number of patients are only mildly affected and survive into adult life.

Diagnosis

Children with cystic fibrosis typically experience repeated pulmonary infections. Since cystic fibrosis affects the glands that secrete sweat, they may also suffer from salt depletion while sweating during hot weather.

Fever, cough, difficulty in breathing, fast respiration, flaring of the nostrils, poor appetite, and reduced activity are typical features of acute cystic fibrosis. Cystic fibrosis is diagnosed with a sweat test, which measures the amount of salt in perspiration. If that test is positive, it should be confirmed with a second test. Since a child's siblings run an increased risk of also having cystic fibrosis, they should be tested as well.

Treatment

Cystic fibrosis is often treated at home with antibiotics, as well as through special exercises for draining sputum—including physical therapy for the chest, physical exercise, and aerosols. Diet therapy, which emphasizes the replacement of deficient digestive enzymes, is also critically important.

Some of the tenacity of the mucus and secretions results from increased amounts of DNA. An inhaled medication, Pulmozyme® contains an enzyme that breaks down the DNA, thereby liquefying the secretions and making them easier to clear.

Surgery is sometimes performed to correct physical complications. For advanced cases of CF, lung transplantation may be required.