There is currently no cure for cystic fibrosis. Treatment for cystic fibrosis is focused on easing symptoms, slowing the progression of the disease, and preventing and treating complications.

Types of Treatments

Treatments for cystic fibrosis include:

• Airway clearance
• Medications
• Surgery
• Long-term pulmonary rehabilitation
• Other therapies

Airway clearance – Patients with cystic fibrosis are taught airway clearance techniques that are used several times a day.

Airway clearance techniques used for cystic fibrosis include:

• Chest physical therapy
• Cupping with hands on the front and back of chest
• Breathing and coughing techniques
• The use of machines, such as a vibrating vest, that pulse air into the lungs

Medications – Medications used to treat cystic fibrosis may include:

• Antibiotics
• Anti-inflammatory drugs
• Mucus-thinning drugs
• Bronchodilators (to keep airways open)
• Oral pancreatic enzymes (to improve nutrient absorption)
• Stool softeners
• Acid-reduction medications

Antibiotics – Inhaled antibiotics, such as tobramycin and aztreonam, may be used to treat lung infections in patients with cystic fibrosis.

Anti-inflammatory drugs, such as ibuprofen, are used to lessen swelling associated with cystic fibrosis

Mucus-thinning medication – The drug, dornase alfa, and hypertonic saline, may be used to thin the thick mucus associated with cystic fibrosis.

Bronchodilators – Inhaled bronchodilators, such as albuterol, are used to open the airways in patients with cystic fibrosis.

Oral pancreatic enzymes – Oral pancreatic enzymes, such as lipase, protease, and amylase, are taken before meals by patients with cystic fibrosis to help break down food.

Stool softeners – Stool softeners, such as polyethylene glycol, are used to prevent intestinal obstructions in patients with cystic fibrosis.

Gene-targeted therapy – Patients with cystic fibrosis who have certain gene mutations may be given gene-target therapy to improve lung function, weight, and to reduce the amount of sodium in sweat.

Gene-targeted therapy for the treatment of cystic fibrosis may include:

• Elexacaftor
• Ivacaftor
• Tezacaftor
• Combination of tezacaftor and invacaftor
• Combination of lumacaftor and ivacaftor

Acid-reducing drugs – Acid-reducing drugs for the treatment of cystic fibrosis may include the proton pump inhibitors:

• Ecomeprazole
• Lansoprazole
• Omeprazole

Surgery – Some patients with cystic fibrosis may need surgery to remove nasal polyps, to treat chronic sinusitis, or to repair an intestinal obstruction.

In some selected patients with severe lung damage due to cystic fibrosis, lung transplantation may be an option.

For patients with cystic-fibrosis liver disease, such as scarring of the liver, also called cirrhosis, liver transplantation may be an option. Liver transplantation can be combined with lung or pancreas transplantation.

Other therapies – Additional therapies for cystic fibrosis may include:

• Oxygen therapy to prevent pulmonary hypertension, which is high blood pressure in the main lung artery
• Noninvasive ventilation, also called CPAP, which uses a machine and mask to assist breathing while sleeping
• Placement of a feeding tube to counteract or prevent malnutrition
• Vitamin therapy with vitamins A, D, E, and K
• Diet therapy

Long-term pulmonary rehabilitation – Pulmonary rehabilitation for cystic fibrosis includes:

• Exercise
• Nutritional counseling
• Supportive counseling
• Cystic fibrosis education

New, Experimental Treatments

The existing antifungal drug, amphotericin, has recently been shown to help improve lung function in patients with cystic fibrosis. Amphotericin can also help to fight off the chronic bacterial infections that are a hallmark of the disease.

Long-Term Follow Up

Long-term follow-up is necessary for patients with cystic fibrosis to prevent and treat infections and complications and monitor treatment effectiveness.

Sources

This content was written by the MediFind Medical Team. Last updated: 6/9/2022