Most children with cystic fibrosis are fairly healthy until they reach 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 37 years, a dramatic increase over the last three decades.

Death is usually caused by lung 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)

Call your health care provider if an infant or child has symptoms of cystic fibrosis.

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

Call your health care provider if you or your child experiences:

• Fever, increased coughing, changes in sputum or blood in sputum, loss of appetite, or other signs of pneumonia
• Increased weight loss
• More frequent bowel movements or stools that are foul-smelling or have more mucus
• Swollen belly or increased bloating

Images

Clubbing

Clubbed fingers

Postural drainage

Cystic fibrosis

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Review Date: 05/01/2011
Reviewed By: David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc., and Denis Hadjiliadis, MD, Assistant Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, University of Pennsylvania, Philadelphia, PA.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)