Treating and Managing Cystic Fibrosis

How Cystic Fibrosis Is Treated

There have been many advances in CF treatment. Patients can take medications to help thin and clear the thick mucus from the airways, enzymes to help absorb fat and nutrients, and antibiotics to treat infections. There are also new treatments that target fixing the CFTR protein.

Airway Clearance Therapy

People with CF need to perform “airway clearance therapy” (ACT). This can be done using manual chest physical therapy or a device worn over the chest that helps clear airway secretions by shaking the mucus in the airways, enabling you to cough it up. Another portable mucus clearing device involves breathing through a mask or mouthpiece. This device forces you to breathe out with more pressure, dislodging mucus in trapped in the airways.

Mucus Thinning Medication

A nebulizer or inhaler is often used before performing ACT. Commonly used medications are albuterol that relaxes the airway and helps clear and thin mucus so you can cough it up more easily.

Enzymes and Nutrients

Pancreatic enzyme replacement therapies help the body absorb food and necessary nutrients. Enzymes have to be given before every meal or snack. People with CF also have to take certain vitamins that are absorbed with fat. Additional salt needs to be provided in formula or food.

Antibiotics and Anti-inflammatories

Antibiotics are frequently needed to treat bacteria that grow in the mucus. These can be given in one of three ways:

  • Orally or by mouth – this is the easiest and cheapest route.
  • By inhalation – this is more expensive but very effective.
  • Intravenously (IV) – this is usually reserved for those who are sicker.

Anti-inflammatory medications have also been found to be helpful in CF. Two medications are currently in use, azithromycin (an antibiotic that’s used as an anti-inflammatory agent in CF), and ibuprofen.

Managing Cystic Fibrosis

Regular check-ups with your doctor are needed as patients with CF will require constant monitoring and health management to control symptoms and prevent complications.

Your doctor may request that you receive some other tests depending on your condition. Common tests include blood tests, bone mineral density tests, glucose monitoring, pancreas functionality test, and respiratory cultures. Ultrasounds, chest CTs or X-rays, colonoscopy and a lung biopsy may be needed in some situations.

In addition to doctor visits, lifestyle changes can help manage symptoms. Practicing good hygiene and receiving all recommended vaccines can prevent patients with CF from getting an infection which in turn could lead to more severe complications. To maintain a healthy weight many patients with CF follow healthy, high-calorie, high-sodium diets. Physical activity is also encouraged to help improve and maintain lung function.

Most importantly all patients with CF need to continue treatments, including medicines, supplements, and daily airway clearance techniques as directed by their physicians. Your physician should also be alerted immediately to any complications so that they can provide supplemental medication to help the immune system fight off infection as quickly as possible.

For More Information:

  • Local CF chapters of the CF Foundation are available to help support parents of newly diagnosed CF in children, as well as in older individuals with CF.
  • The CF Patient Assistance Foundation helps people with CF afford medication and the devices needed to stay healthy. It also assists individuals in applying for supplemental social security and social security disability benefits.
  • The CF legal hotline that provides information for individuals with CF about their rights.

Finding Support

The Lung Association recommends patients and caregivers join our Living with Lung Disease Support Community to connect with others facing this disease. Join a Better Breathers Club to learn better ways to cope with a CF diagnosis. You can also call the Lung Association's Lung Helpline at 1-800-LUNGUSA to talk to a trained respiratory professional who can help answer your questions and connect you with support.

Reviewed and approved by the American Lung Association Scientific and Medical Editorial Review Panel.

Page last updated: March 12, 2020

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