Early recognition of cystic fibrosis and a comprehensive, multidisciplinary treatment program can lengthen survival time and improve quality of life. Specialty clinics for cystic fibrosis can be found in many communities and may be helpful.

Medications include antibiotics for respiratory infections and pancreatic enzymes to replace those which are missing. Vitamin supplements are sometimes prescribed. Inhaled bronchodilators are used to relieve chronic obstruction of the airways.

A recent study showed 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. More research is being done.

Other treatments include postural drainage and chest percussion, and other breathing treatments.

Lung transplant may be considered in some cases.

New treatments include replacement of the DNAse enzyme. This is available as a medication called dornase (Pulmozyme). Genetic research is ongoing in hopes of correcting the disease by artificially inserting a "normal" gene into the person. This treatment is called gene therapy, and an intranasal form of gene therapy is currently undergoing clinical trials. Research on possible methods which could be used to correct the disorder before birth seems promising.

About 40% of children with cystic fibrosis live beyond age 18. The average life span for those who live to adulthood is 30-33.

Death is usually caused by pulmonary (lung) complications.

• Pneumonia, recurrent
• Pneumothorax
• Hemoptysis (coughing up blood) needs to be linked
• Chronic respiratory failure
• Cor pulmonale
• Liver disease
• Diabetes mellitus
• 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.