The earlier a diagnosis is made the better so that early treatment can slow the progression of lung damage caused by infection. Prenatal tests are available to determine if a baby will be born with cystic fibrosis. In newborns, blood tests indicating high levels of digestive enzymes suggest cystic fibrosis, but a certain
diagnosis requires a sweat test to determine the amount of salt in the sweat. Sweat tests provide a valid diagnosis in babies over 24 hours old, and this test is also used to confirm diagnosis in older children and adults.
Cystic fibrosis remains incurable; existing treatments aim to relieve discomfort and delay the devastating and inevitable effects of the disease. Meconium ileus, the intestinal obstruction occurring in newborns, may require surgery. Patients with pancreatic blockage must take pancreatic enzymes with meals. Even with such enzymes, people with cystic fibrosis must consume adequate amounts of protein, vitamins, and higher-than-normal amounts of fat to ensure growth.
Those with respiratory infections are treated with antibiotics, often in aerosol form. When inhaled, these medicated vapors fight infection and relieve constriction of the airways. Using a procedure called chest physical therapy or postural drainage, caregivers of people with cystic fibrosis repeatedly and vigorously pound on the patient's back and chest to dislodge mucus obstructing the airways. Increasingly, cystic fibrosis patients with severe, irreparable lung damage turn to lung transplantation surgery (see Medical Transplantation). Although complications with transplantation surgery may pose problems for some patients, lung or combination heart and lung transplants provide nearly 80 percent of cystic fibrosis patients with severe lung damage an entirely new lease on life.
Although no cure has yet been found, cystic fibrosis presents one of the most promising areas of research in modern medicine. Scientists are investigating the use of gene therapy to...