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What cystic fibrosis medications are available?

H2 blockers, also known as H2 receptor antagonists, are a class of drugs that help reduce stomach acid. They are prescribed for cystic fibrosis patients in an effort to create a more alkaline environment so the pancreatic enzymes can do their job. They can help break up clumps of undigested food that lead to distal intestinal obstruction. Oral corticosteroids are indicated in infants with prolonged bronchiolitis and in those patients with refractory bronchospasm, allergic

bronchopulmonary aspergillosis, and inflammatory complications (eg, arthritis and vasculitis). Long-term use of alternate-day corticosteroid therapy can slow the decline in pulmonary function, but because of steroid-related complications it is not recommended for routine use. Patients receiving corticosteroids must be closely monitored for evidence of carbohydrate abnormalities and linear growth retardation.

Ibuprofen, the anti-inflammatory drug ibuprofen, an ingredient in many over-the-counter painkillers, can preserve lung function in cystic fibrosis. When given at a dose sufficient to achieve a peak plasma concentration between 50 and 100 µg/mL over several years, has been shown to slow the rate of decline in pulmonary function. The treatment was most effective in younger patients under 13 years of age. Researchers warn that ibuprofen treatment should be performed only under medical supervision because the high drug doses required must be determined individually for each patient.

The choice of antibiotic depends on the particular organism or organisms found. Some antibiotics are given as aerosols directly into the lungs. Antibiotic treatment may be prolonged and aggressive. A penicillinase-resistant penicillin (eg, cloxacillin or dicloxacillin) or a cephalosporin (eg, cephalexin) is the drug of choice for staphylococci. Erythromycin, amoxicillin-clavulanate, ampicillin, tetracycline, trimethoprim-sulfamethoxazole, or occasionally chloramphenicol may be used individually or in combination for protracted ambulatory therapy of pulmonary infection due to a variety of organisms. For some patients, ciprofloxancin effectively substitutes for a course of intravenous antibiotic, if the patient's germs are sensitive and the illness is mild.

An aerosol form of the antibiotic tobramycin significantly reduced Pseudomonas infections in cystic fibrosis patients. The inhaled drug directly reaches the infected lung tissue, reducing the dose required and the potential for side effects. Tobramycin by aerosol form is easier and less expensive to administer than by intravenous injection.

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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