Cystic fibrosis

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Clubbing

Cystic fibrosis

Treatment:

An early diagnosis of CF and a comprehensive treatment plan can improve both survival and quality of life. Specialty clinics for cystic fibrosis may be helpful and can be found in many communities.

text continues below

Treatment includes:

Antibiotics for respiratory infections.
Pancreatic enzymes to replace those that are missing.
Vitamin supplements, especially vitamins A, D, E, and K.
Inhaled bronchodilators, such as albuterol (Proventil, Ventolin), help open the airways.
DNAse enzyme replacement therapy.The medication dornase (Pulmozyme) contains an enzyme that thins the mucus and makes it easier to cough up.
Pain relievers. Research has shownthat the pain reliever ibuprofen may slow lung deterioration in some children with cystic fibrosis. The results were most dramatic in children ages 5 to 13.
Postural drainage and chest percussion.
Lung transplant may be considered in some cases.

Support Groups:

For additional information and resources, see cystic fibrosis support group.

Expectations (prognosis):

Disease registries now show that 40% of patients with cystic fibrosis are over age 18.

Today, the average life span for those who live to adulthood is approximately 35 years, a dramatic increase over the last three decades.

Death is usually caused by lung complications.

Complications:
The most common complications are chronic respiratory infections.

Calling your health care provider:

Call your health care provider if symptoms develop that suggest an infant or child may have cystic fibrosis.

Call your health care provider if a person with cystic fibrosis develops new symptoms, particularly severe breathing difficulty or coughing up blood.

References:

US Food and Drug Administration. FDA Approves First DNA-based Test to Detect Cystic Fibrosis. Rockville, MD: National Press Office; May 16, 2005. Press Release P05-23.

Cystic Fibrosis Foundation. What is CF? Available at http://www.cff.org/about_cf/what_is_cf/. Accessed May 16, 2005.