Cystic fibrosis

Treatment

Is there treatment for cystic fibrosis?

Although there is no cure for cystic fibrosis (CF), there are FDA approved treatments for different health problems seen in cystic fibrosis. Some treatments attempt to loosen up the mucus from the lungs and thus decrease the frequency of lung infections. Treatment is broken up into medications, respiratory therapies, nutritional support, and exercise. Medications for cystic fibrosis include constant antibiotics, which can prevent and reduce the frequency of lung infections. Airway clearance or respiratory therapies are aimed at loosening up the thick mucus from the lungs. Typically, high frequency chest wall oscillations are provided through a vest. The oscillations shake up and loosen the mucus. Manual coughing techniques can help loosen up the mucus from the lungs also. Additionally, exercise is beneficial in clearing out mucus. Surgeries for sinus complications may be necessary. As the disease becomes worse, lung transplant may be necessary for some individuals with CF. Newer treatments are targeted for individuals with certain gene changes (mutations) and help correct the underlying defect in the CFTR gene.

Pancreatic enzymes before meals can help digestion. These are dosed by a dietician, and taken at specific times before each meal to help digestion. Diet supplements may also be necessary to help people with CF get proper nutrition. A feeding gastric tube (G-tube) may help some people with this. Liver complications may need treatment with medication also. Speak with your care team to find out what specialists you may need to see.

If someone with CF develops cystic fibrosis related diabetes mellitus, treatment would depend upon the severity of the diabetes. An endocrinologist can help manage diabetes mellitus in an individual with CF.

Males who want to have children may need to consult with an infertility specialist to be able to father a child.

Treatment guidelines have been developed and are available on the Cystic Fibrosis Foundation website.

Moskowitz S.M. et al. "CFTR - related disorders." Gene Reviews. 19 February 2008. Web GeneReviews

CFF - About CF

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