What're the complications of cystic fibrosis?
Most people with cystic fibrosis also have digestive problems. Mucus can block the ducts of the pancreas, preventing enzymes produced by that organ from reaching the intestines to help digest food. Problems with digestion can lead to diarrhea, malnutrition, and weight loss. Insulin-dependent diabetes develops in 10% of adult patients, and multilobular biliary cirrhosis with varices and portal hypertension develops in 4 to 5% of adolescents and adults. Chronic and/or recurrent
abdominal pain may be related to intussusception, peptic ulcer disease, periappendiceal abscess, pancreatitis, gastroesophageal reflux, esophagitis, gallbladder disease, or episodes of partial intestinal obstruction secondary to abnormally viscid fecal contents. Some babies with cystic fibrosis have meconium ileus, a blockage of the intestine that occurs shortly after birth.
People with cystic fibrosis have an increased risk of lung infections. Many of them will have repeated bouts of pneumonia. These usually lead to worsening lung disease. This, in turn, can result in respiratory failure and arrest. Cystic fibrosis patients often cannot interact with each other socially due to worries of cross-infection of Pseudomonas, MRSA, Burkholderia cepacia, and other bacteria. These infections thrive in the thick mucus of cystic fibrosis patients' lungs and cause complications and possibly death. Therefore cystic fibrosis patients who do not have a certain bacteria type cannot meet with those who do. Because of this risk cystic fibrosis patients must remain in isolation during hospital stays, and special precautions must be taken. This risk previously caused many cystic fibrosis clinics to recommend that cystic fibrosis patients live in isolation and never meet. However recently these views have been changed because of the possible psychological problems this may cause; instead cystic fibrosis patients are encouraged to exercise caution, avoid direct physical contact, and possibly wear surgical masks.
In addition to pulmonary infections, most persons with cystic fibrosis also have problems with digestion, particularly the digestion of fats. This leads to malabsorption and difficulty gaining and maintaining weight, which in turn affects overall health. This is due to the abnormally sticky mucus that blocks the release of digestive enzymes from the pancreas. Pancreatic insufficiency is treated with supplemental enzymes. Usually water-miscible forms of the fat-soluble vitamins A, D, E, and K are required as the decreased fat absorption can lead to deficiencies of these vitamins.
Excessive sweating in hot weather or with fever may lead to episodes of hypotonic dehydration and circulatory failure. In arid climates, infants may present with chronic metabolic alkalosis. Salt crystal formation and a salty taste on the skin are highly suggestive of cystic fibrosis. Men and women with cystic fibrosis are often unable to have children because of abnormalities in their reproductive systems. Cystic fibrosis also causes a defect in the vas deferens causing sterility in approximately 98% of males with the disease.
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.