Cystic fibrosis

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Clubbed fingers

Clubbing

Cystic fibrosis

Postural drainage

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.  

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 shown that 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.

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

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.

• Pneumonia, recurrent
• Pneumothorax
• Coughing up blood
• Chronic respiratory failure
• Cor pulmonale
• Liver disease
• Diabetes
• Osteoporosis and arthritis

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.

Review Date: 06/08/2005
Reviewed By: Thomas A. Owens, MD, Departments of Internal Medicine and Pediatrics, Duke University Medical Center. Review provided by VeriMed Healthcare Network.