Cystic fibrosis natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Natural History

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Complications

The most common complication is chronic respiratory infection.

• Bowel problems, such as gallstones, intestinal obstruction, and rectal prolapse
• Coughing up blood
• Chronic respiratory failure
• Diabetes
• Infertility
• Liver disease or liver failure, pancreatitis, biliary cirrhosis
• Malnutrition
• Nasal polyps and sinusitis
• Osteoporosis and arthritis
• Pneumonia, recurrent
• Pneumothorax
• Right-sided heart failure (cor pulmonale)

Prognosis

Most children with cystic fibrosis are fairly healthy until they reach adolescence or adulthood. They are able to participate in most activities and should be able to attend school. Many young adults with cystic fibrosis finish college or find employment.

Lung disease eventually worsens to the point where the person is disabled. Today, the average life span for people with CF who live to adulthood is approximately 35 years, a dramatic increase over the last three decades.

Death is usually caused by lung complications.