Cystic Fibrosis: Treatments

How is cystic fibrosis treated?

There is no cure for cystic fibrosis, but early diagnosis and consistent compliance with a treatment program can help to reduce symptoms and complications and extend life. Treatments for cystic fibrosis include a multifaceted approach that is tailored to your specific symptoms, the presence of coexisting diseases and complications, your age and medical history, and other factors.

The goals of treatment are to keep the airways in the lungs open, minimize symptoms, such as shortness of breath and frequent cough, and reduce the development of complications, such as frequent lung infections.

Drug treatment of cystic fibrosis

Cystic fibrosis is treated with a variety of medications, which vary depending on the specific symptoms and body systems that are affected. Medications can include:

• Antibiotics to treat bacterial pneumonia and other bacterial lung infections

• Bronchodilators to help relax and open up the lower airways in the lungs

• Digestive enzymes to help with digestion

• DNase enzyme treatment to thin mucus and make it easier to cough up and keep the airways clear

• Oxygen therapy to treat moderate to severe cystic fibrosis, which results in low levels of oxygen in your blood

• Steroid inhalers to reduce airway inflammation

• Vitamins and other nutritional supplements to minimize vitamin deficiencies and malnutrition

Other treatments of cystic fibrosis

Cystic fibrosis is also treated with a variety of therapies, lifestyle and dietary interventions, and possibly surgery. Other treatments of cystic fibrosis include:

• Chest physical therapy, such as chest percussion and postural drainage, which are techniques performed daily to several times a day to physically loosen and remove mucus in the lungs

• High-protein, high-calorie diet

• Lung transplant

• Participation in a clinical trial that is testing promising new treatments

• Prevention measures to avoid diseases that can seriously complicate cystic fibrosis and become life threatening, such as preventing pneumonia and the flu with vaccination

• Pulmonary rehabilitation, which is a medically supervised exercise program

• Regular but moderate exercise program

• Staying hydrated by drinking lots of fluids to keep mucus as loose as possible

What are the possible complications of cystic fibrosis?

Complications of cystic fibrosis are serious, progressive, and eventually become life threatening and fatal. Respiratory failure is the most common cause of death in people with cystic fibrosis. You can reduce the risk of serious complications or delay their development by following the treatment plan you and your health care provider design specifically for you. Complications of cystic fibrosis can include:

• Diabetes

• Disability

• Gastrointestinal conditions, such as pancreatitis, gallstones, and intestinal obstruction

• Growth deficiencies in children and infants

• Heart failure

• Infertility

• Lung disease, such as asthma, permanent lung damage, and respiratory failure

• Recurrent and chronic sinus infections and lung infections, such as bronchitis and pneumonia

• Serious liver problems, such as cirrhosis, portal hypertension, and liver failure

• Sinus infections

• Skeletal diseases, such as arthritis and osteoporosis

• Vitamin and mineral deficiencies and malnutrition