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.

Treatment for lung problems includes:

• Antibiotics to prevent and treat lung and sinus infections
• Inhaled medicines to help open the airways
• DNAse enzyme replacement therapy to thin mucus and make it easier to cough up
• Lung transplant, in some cases

Treatment for intestinal and nutritional problems may include:

• A special diet to help with nutrition (see: Cystic fibrosis nutrional considerations)
• Pancreatic enzymes to replace those that are missing
• Vitamin supplements, especially vitamins A, D, E, and K

Support Groups:

For additional information and resources, see: Cystic fibrosis support group

Expectations (prognosis):

Today, the average life span for persons 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.

Complications:

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

Calling your health care provider:

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, particularly severe breathing difficulty or coughing up blood.