Cystic Fibrosis: Treatment

How is Cystic Fibrosis Treated?

Cystic fibrosis is treated with a personalized treatment plan that includes symptom management techniques, a fitness plan, nutritional therapy and medications. Following your treatment plan will help you to keep your disease under control.

CF Treatment Plan

Treatment plans are personalized to your specific health needs, age, lifestyle, goals and medical test results. Your CF care team will work with you to create a treatment plan that meets your needs and goals. Treatment plans can include:

• Regular visits to CF care center

• Airway clearance techniques

• Medications

• Avoiding infection

• Treating infections

• Lung transplant

• Nutrition plans

• Fitness plans

Visiting Your Cystic Fibrosis Center

The visit to your CF center is a time for your care team to check on your health status, help you troubleshoot problems and adjust your treatment plan if necessary.

Since cystic fibrosis is a complex disease, it requires a team approach, with you or your child at the center. Your care team includes a group of CF health professionals who partner with you to provide specialized, comprehensive CF care.

Airway Clearance Techniques

Airway clearance measures are treatments designed to clear trapped mucus from the airways.

Oscillating positive expiratory pressure devices (OPEPD): These include devices such as the Aerobika^®, Acapella^® or the Flutter Valve^® that help clear mucus from your lungs. These are small devices you inhale and/or exhale into.

High-frequency chest wall oscillation vests: These include The Vest®, SmartVest®, AffloVest^®, InCourage® Vest and Monarch® Vest.  Inflatable vests that you put on. The vest shakes your chest to help dislodge the mucus from the airway walls. Sometimes the Aerobika® or Acapella® is used after the inflatable vest. Once the mucus is dislodged, the device can help clear the mucus.

Postural drainage and clapping use gravity to promote drainage of mucus from the lungs.

Each technique can be prescribed by your health care provider. Correct technique using these devices is very important. Make sure a health care provider, often a respiratory therapist, experienced in the use of the device, shows you how to use it. It is also important to have your technique checked periodically to make sure you continue to use it correctly to obtain the most benefit.

Medications for Cystic Fibrosis

A variety of inhaled medications may be used along with the airway clearance techniques. The CF Team will review the medications recommended for you.

• Inhaled TOBI® or Tobramycin Using AeroEclipse® XL BAN (Breath Actuated Nebulizer)

• Using A Pari LC® Plus Nebulizer With Gentamicin

• Using A Pari LC® Plus Nebulizer With Meropenem

• Using A Pari LC® Plus Nebulizer With Colistimethate

• Using A Pari LC® Plus Nebulizer With Ceftazidime

• Inhaled Amikacin Using AeroEclipse® XL BAN (Breath Actuated Nebulizer)

Although there is not yet a cure for cystic fibrosis, there have been many treatment advances that have improved digestion, lung function and longevity, while enhancing mucus clearing and restoring CFTR function.

See the Cystic Fibrosis Foundation website for up-to-date information about approved CF medication (Opens in a new window) (Opens in a new window) (Opens in a new window) and those under development.

Avoiding infections with CF

There are a number of measures that can help avoid infections.

• Vaccines are recommended. The influenza or flu vaccine is recommended yearly, often in the fall. There are two pneumonia vaccines; both are recommended. These vaccines help prevent severe infection from a bacterium that is a common cause of pneumonia.

• Good hand washing with soap and water is also very important to help prevent the spread of germs and infections. Alcohol-based gels are also effective. Use a quarter-sized dollop in your palm, and rub your hands together until they are dry.

• Avoid touching your eyes, nose and mouth. Germs are often spread this way.

• Avoid contact with others when they are sick.

• Remember the six-foot rule. All people with CF should stay six feet away from each other in all settings, unless living in the same household. The thick mucus seen with CF leads to a person being more at risk of infection. This will help decrease the risk of transmitting germs seen with CF from person to person.

Treatment of Infections

Antibiotics are used to treat bacteria and other infectious organisms causing infection in the lungs in order to improve respiratory symptoms and prevent further damage to the airways. Antibiotics may be taken as a routine chronic regimen; these can be oral or inhaled for specific bacteria in the lungs. Occasionally the bacterial burden in the lungs becomes overgrown, and a course of oral or IV antibiotics is needed. The typical amount of time for a course of antibiotics for an acute infection is two to three weeks.

Lung Transplantation: Is It Right for You?

Cystic fibrosis may progress despite therapy. If this happens, discuss with your health care provider if lung transplantation may be an option for you.
 
Lung transplant surgery replaces one or two diseased lungs with healthy lungs from a non-living donor. With improved surgical techniques and post-transplant care, this may offer you improved quality of life and longer survival. Cystic fibrosis doesn’t recur in transplanted lungs.

Lung transplantation is performed only at specialty medical centers. Your health care team may determine that lung transplant is the best option for you and that you are healthy enough for surgery. After an extensive evaluation, appropriate candidates are placed on a waiting list. A position on the waiting list is determined by disease severity. Wait times vary from transplant center to transplant center.

After a lung transplant, people need to take anti-rejection medicines for the rest of their lives to help prevent the body from rejecting the new lungs. These medicines can cause complications, including infection. Having emotional support is an important part of recovering and staying healthy after lung transplant surgery.

Learn more about a lung transplant.

Nutrition Plan

People with CF often need to eat a high-calorie, high-fat diet. Medicines may be recommended to help with digestion. Vitamin and mineral supplements may also be recommended. Each person’s nutritional needs are different, so your care team will help create a nutritional plan just for you.

Fitness Plan

Staying physically active helps the lungs function better, reduces pulmonary exacerbations, slows the rate of lung function decline and helps you maintain a normal life. Make physical activity an important part of your lifestyle, like sleeping and eating. Choose activities that you can do consistently despite the weather, such as walking, running and cycling. Keep hydrated while you are exercising to maintain your salt level.