Thanks to Cystic Fibrosis Foundation-supported research, people with cystic fibrosis (CF) have more therapy options than ever before. There are drugs to help clear the thick CF mucus from airways and reduce inflammation, aerosolized antibiotics and, most recently, a breakthrough therapy that addresses the underlying cause of CF. The Foundation is dedicated to investing in promising research that will lead to more new treatments and a cure for cystic fibrosis.
Kalydeco™ is a pill for people ages 6 and older who have the following CF mutations: G551D, G178R, S549N, S549R, G551S, G1244E, S1251N, S1255P and G1349D.
In people with these mutations, Kalydeco helps the defective CFTR protein work at the surface of the cell. This allows salt and fluid to move into the airways, which helps thin the thick, sticky mucus caused by CF that builds up in the lungs so it is easier to cough out.
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Clearing the Airways
People with cystic fibrosis do airway clearance techniques (ACT) to loosen and get rid of the mucus from the lungs. Clearing mucus helps to reduce the severity of lung infections and improve lung function.
Some airway clearance techniques require help from family members, friends or therapists. Adults with cystic fibrosis can do many airway clearance techniques themselves.
One technique is called “postural drainage and percussion.” People with cystic fibrosis sit, stand or lie in a position that will help free up mucus as their chest and back are pounded or clapped. Sometimes a patient will use a mechanical “vest,” or blow into a device that shakes the mucus loose, to help clear their airways.
Inhaled drugs are commonly used in cystic fibrosis care because they reach the airways quickly and easily. Inhaled treatments can be given by aerosol — a mist made from liquid medicines. The medicines go into a cup (nebulizer) that is attached to a small air compressor. The compressor blows air through the cup and makes a mist. People with cystic fibrosis breathe the mist in through a mouthpiece or mask for several minutes. Some medicines can also be given as metered dose inhalers (MDI), which deliver one dose of medicine at a time.
There are several kinds of inhaled medications used to treat CF symptoms:
Mucolytics like Pulmozyme® to thin mucus so people can cough it out easier.
Antibiotics to treat infections. Inhaled TOBI®
(tobramycin solution for inhalation) is a widely used antibiotic treatment. TOBI can be effective against the most common source of chronic lung infections, a bacterium called Pseudomonas aeruginosa
to draw more water into the airways and make it easier to cough out the mucus.
Antibiotics are used to fight infection-causing bacteria. Infections are common in the lungs of people with cystic fibrosis, so antibiotics are an important part of regular care.
The antibiotic drug, the dosage, and the length of time to take the drug, all vary from person to person. The infection-causing bacteria can become resistant to some drugs.
Antibiotics come in three different forms:
- Oral antibiotics – liquids, tablets or capsules that must be swallowed.
- Intravenous (IV) antibiotics – liquid medicine that goes directly into the blood through an IV catheter. An IV may require a hospital stay, but can also be done at home.
- Inhaled antibiotics – an aerosol or mist that can reach the airways directly.
Research supported by the Cystic Fibrosis Foundation has led to other respiratory treatments that are now considered “standard therapies” for many people battling cystic fibrosis.
For example, ibuprofen, which is an anti-inflammatory, was found by CF Foundation-supported researchers to benefit people with cystic fibrosis. It can slow the rate at which lung function usually declines for some people with CF. However, patients need to be closely monitored by their CF physician if taking this drug for CF.
Azithromycin, a common antibiotic, was shown to have special benefits for some people with cystic fibrosis. To study this possibility, the Foundation conducted a Phase 3 trial and found that, in some cases, the drug helped to preserve and improve lung function. The drug also reduced the number of hospital stays for many people who participated in the clinical trials.
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Implanted devices that allow repeated and long-term access to the bloodstream for frequent or regular administration of drugs as an alternative to shots are called ports and PICCs.
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Nutrition & Eating Right
Nutrition needs change with age — especially for people with cystic fibrosis. Dietitians at cystic fibrosis care centers offer specially tailored dietary programs for each stage of life.
Children and teens with cystic fibrosis need extra calories to grow and develop. Everyone with CF, no matter their age, need good nutrition to stay strong against lung infections and other challenges. Occasionally, patients need to take nutrition through a tube ("tube-feeding") to provide extra calories that help the body grow and stay strong.
Body Mass Index, or “BMI” is a calculation used to assess how healthy an individual’s weight is relative to their height. Click here for more information and to find out your BMI.
Almost half of all people with cystic fibrosis in the United States today are adults. They are facing diseases most often associated with the middle-aged or elderly. These diseases include CF-related diabetes and brittle bones (osteoporosis or osteopenia). By working closely with CF care centers, good nutrition can help to prevent or lessen the impact of these and other health problems.
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Over the years, some research have indicated that people can gain health benefits by increasing their intake of antioxidants, either by eating more fruit and vegetables or by taking supplements.
Healthy, non-cystic fibrosis lungs appear to have more natural antioxidants than cystic fibrosis lungs. The lack of natural antioxidants is considered by researchers to be a possible factor in the inflammation and infection cycle in CF.
The Cystic Fibrosis Foundation supports laboratory research and clinical trials to evaluate the possible benefits and safety issues involved in taking drugs that are the building blocks of antioxidants, like glutathione.
All Foundation-supported studies are peer-reviewed and closely monitored. People with cystic fibrosis should discuss with their CF care center the potential benefits and risks of any alternative therapy, diet supplements or extra vitamins not prescribed by your doctor (e.g. nutriceuticals).
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