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What treatments or therapies are available for Cystic Fibrosis?

Cystic Fibrosis is an inherited disease which involves a defective gene that causes the body to produce abnormally thick, sticky mucus. This mucus builds up in the lungs and digestive tract which can result in severe lung damage, nutritional deficiencies, and early death. There is no current cure for cystic fibrosis.

The main goals for treatment are preventing infection, improvement of airflow by reducing the amount and thickness of lung secretions, and nutrition maintenance. There are several treatments for cystic fibrosis depending on the needs of the individual patient. These include:
• Antibiotics – Drugs that fight the germs called bacteria that cause lung infections in people with cystic fibrosis. Example: TOBI® (tobramycin for inhalation).
• Mucus-thinners – Drugs that make the mucus thinner so it is easier to cough out. Example: Dornase alfa (Pulmozyme).
• Inhaled bronchodilators – Drugs that help open airways for easier breathing. Example: Proventil, Ventolin.
• Oral enzymes and vitamins – Thick mucus can block the digestive tract and pancreas. It can stop digestive enzymes from breaking down food which can lead to malnutrition. People usually need to supplement their diet with oral pancreatic enzymes and vitamins A, D, E, and K (antioxidants).
• Pain relievers – Studies have shown that ibuprofen (Motrin, Advil) may slow lung damage in children with cystic fibrosis by reducing inflammation (swelling) in the lungs.
• Bronchial airway drainage and chest percussion – These procedures help people with cystic fibrosis physically remove the thick mucus from their lungs.
• Lung transplantation – For people with severe breathing difficulties, life-threatening lung complications, or increased resistance to antibiotics, transplantation of both lungs may be an option. This is not a cure since the defective genes exist in cells throughout the entire body, not just the lungs. The benefits must outweigh the risks since there can be complications with any organ transplant such as rejection or infection.
• Gene therapy – Currently there are no good gene therapies for cystic fibrosis because researchers have been unable to find an effective way to deliver healthy genes into the body. Clinical trials are under way to determine the safest, most effective way to deliver these genes in hopes of successfully treating the actual cause of cystic fibrosis in the future.

Sources:
Diseases and Conditions. “Cystic Fibrosis”. www.mayoclinic.com. Mayo Clinic Health. Last accessed 12/2006.
Medline Plus Encyclopedia. Medline, 2006.About Cystic Fibrosis. www.cff.org. Cystic Fibrosis Foundation. Last accessed 12/2006.

This answer prepared 12/12/2006.

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