Cystic fibrosis Health Article

Signs and tests

A blood test is available to help detect CF. The test looks for variations in a gene known to cause the disease. Other tests use to diagnose CF include:

• Immunoreactive trypsinogen (IRT) test is a standard newborn screening test for CF. A high level of IRT suggests possible CF and requires further testing.
• Sweat chloride test is the standard diagnostic test for CF. A high salt level in the patient's sweat is a sign of the disease.

Other tests that identify problems that can be related to cystic fibrosis include:

• Chest x-ray or CT scan
• Fecal fat test
• Lung function tests
• Measurement of pancreatic function
• Trypsin and chymotrypsin in stool
• Secretin stimulation test
• Upper GI and small bowel series

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

The most common complications are chronic respiratory infections.

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