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What're the symptoms of cystic fibrosis?

The symptoms of cystic fibrosis usually develop during early childhood. The severity of symptoms varies from person to person. Almost always, a productive cough is present, and very often patients appear barrel-chested. Frequent hospitalizations are a result of recurring respiratory, gastrointestinal, and nutritional problems.

Some people have severe symptoms involving various parts of the body; others have much milder disease involving only one area of the body. Both lungs and pancreas produce abnormally viscous mucus. The mucus in the lungs can become a growth medium for bacteria, resulting in chronic respiratory infections and eventual permanent damage to the lung tissue. As lung function deteriorates, cystic fibrosis patients develop pulmonary hypertension and eventually cor pulmonale. Death usually occurs from severe infection or heart failure. These thick secretions also obstruct the pancreas, preventing digestive enzymes from reaching the intestines to help break down and absorb food.

Cystic fibrosis is characterized by the production of very thick and sticky mucus. The ducts leading from the pancreas become obstructed, preventing normal digestion of food. This leads to poor absorption of the nutrients in the food, and ultimately to malnutrition. About 90 percent of cystic fibrosis cases involve the lungs. Lung infections are common among children with cystic fibrosis and may lead to extreme weakness. Severe cases of cystic fibrosis ultimately may lead to heart disease. About half of people with cystic fibrosis have repeated chest infections and pneumonia. If they are not treated early and properly, these are very difficult to treat. Symptoms include persistent coughing, excess production of sputum (saliva and mucus), wheezing, and shortness of breath with ordinary activities. If people with cystic fibrosis do not have proper treatment, they will continue to have oily bowel movements, abdominal pain, and problems putting on weight. Constipation is also a frequent symptom. Occasionally the gut becomes completely blocked, resulting in extreme stomach pain.

More information on cystic fibrosis

What is cystic fibrosis? - Cystic fibrosis is a hereditary disease of the exocrine glands. Cystic fibrosis affects the pancreas and the glands that secrete sweat and mucus.
What causes cystic fibrosis? - Cystic fibrosis is caused by a defect in the person's genes. The defective gene that is responsible for causing cystic fibrosis is on chromosome 7.
What're the complications of cystic fibrosis? - Most people with cystic fibrosis also have digestive problems. People with cystic fibrosis have an increased risk of lung infections.
What're the symptoms of cystic fibrosis? - The symptoms of cystic fibrosis usually develop during early childhood. Cystic fibrosis is characterized by the production of very thick and sticky mucus.
How is cystic fibrosis diagnosed? - Family history, persistent respiratory disease, or clinical evidence of pancreatic insufficiency may suggest the diagnosis of cystic fibrosis.
What's the treatment for cystic fibrosis? - Treatment for cystic fibrosis consists of the intake of digestion enzymes, nutritional supplements, percussion and postural drainage of the lungs, improved antibiotics.
What cystic fibrosis medications are available? - Cystic fibrosis medications include H2 receptor antagonists, oral corticosteroids, ibuprofen, erythromycin, amoxicillin-clavulanate, etc.
What cystic fibrosis diet is suggested? - People with cystic fibrosis usually require high-calorie diets and vitamin supplements. Most people with cystic fibrosis need to take pancreatic enzymes.
What's the gene therapy for cystic fibrosis? - Gene therapy for cystic fibrosis typically aims to supplement a defective mutant allele with a functional one.
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