CF - Cystic Fibrosis

What are the treatments for the disease?

Treatment for cystic fibrosis focuses on treating symptoms and deficiencies. Pancreatic enzymes and diet changes may lead to better digestion.

Lung and airway infections can be treated with potent antibiotics. Inhaled bronchodilator medications used in asthma, such as albuterol, have improved breathing in some people with cystic fibrosis.

Chest physiotherapy and postural drainage techniques are used to help clear the excess mucus out of the lungs. Drugs that help break up the mucus, such as dornase alfa, may help clear the lungs. Anti-inflammatory medication like ibuprofen may reduce the inflammation in the lungs. Inhaled antibiotics are used to prevent lung infections that may lead to hospitalization.

Now that the gene for cystic fibrosis is known, the possibility of gene therapy looks hopeful. Already scientists have transferred normal genes from chromosome 7 into laboratory animals and seen promising results.

What are the side effects of the treatments?

Side effects depend on which medications are used. Bronchodilators can cause a rapid heart rate and trembling of the hands. Antibiotics can cause rashes and other allergic reactions.

What happens after treatment for the disease?

Rehabilitation therapy helps most people to use their energy more efficiently, in a way that requires less oxygen. Many people also use oxygen therapy. This improves shortness of breath and overall survival, especially in those with advanced disease.

How is the disease monitored?

Regular lung function testing may be done to check on how well the lungs and airways are working. Sputum may be collected and sent to the lab to test for infection. Cystic fibrosis is a life-long disease. It helps to pay ongoing attention to physical conditioning, nutrition, and good mental health. Monitoring growth is an important consideration for assessing adequate nutrition.