What's the Treatment for Cystic Fibrosis?

There’s no cure for cystic fibrosis (CF). But many treatments can reduce your symptoms and improve your quality of life. Here’s an overview of the most common.

Medication

Your doctor will likely prescribe several different medications to treat your CF. These might include:

Antibiotics: These are the main medicines used to prevent and treat lung infections. Often, you’ll take these in pill form. They’re also available in inhaled form. But if you have a severe infection, you may have to get them through an IV in the hospital.

Anti-inflammatory drugs: Repeat infections can leave your airways swollen and make it harder for you to breathe. Anti-inflammatory drugs can help. One example is Tezacaftor/ivacaftor (Symdeko). This medication is available as a tablet that can help air move easier through your lungs, allowing you to blow more air out of your lungs. This medicine is available to anyone 12 years and older.

Bronchodilators : These medications work to open your airways. They’re often given in an inhaler or nebulizer. This turns medicine into a mist you breathe in through your nose. Your doctor may want you to take this medicine before chest physical therapy or to help other medications work better.

Mucus thinners: This type of medicine loosens thick, sticky mucus and helps improve the way your lungs work.

Digestive Treatments

Many people with CF have trouble digesting food and getting the nutrition they need. A dietitian can help you plan a healthy diet that’s high in the calories, fat, and protein. Here are some other things that can help:

Digestive enzymes: Taking these throughout the day will help your body better absorb nutrients from meals.

Vitamins: Your doctor may advise you to take vitamins -- especially A, D, E, and K. People with CF have a hard time absorbing enough of them from food.

Laxatives: If constipation is a problem, your doctor can prescribe a gentle laxative or stool softener that’s safe to take for a long period of time.

Chest Physical Therapy (CPT)

Airway clearance techniques (ACTs) can help you breathe better. They may also help reduce the number of lung infections you get. For instance, clapping or pounding on your chest and back helps loosen mucus so you can cough more of it out.

You can do different types of ACTs at home with the help of a family member or friend. Or, you might prefer to use a medical device. You could use an electric chest clapper or a mask that pulls mucus away from your airways with vibrations. Special therapy vests use high-frequency airwaves to loosen mucus.

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Exercise

When you work out, you breathe faster and harder, which helps you cough up more mucus. Exercise also improves your mood and protects other parts of your body, like your bones and heart.

If you work out on a regular basis, you may not need as much CPT. Check with your doctor first about what types of activities are safe for you.

Gene Therapy

Cystic fibrosis is caused by a defect on a gene known as CFTR. New medicines called “CFTR modulators” can fix this gene so it functions like it should.

This treatment isn’t for everyone. Many different gene mutations (changes) cause CF. These medications can only fix a few specific mutations. If your CF is caused by another defect, these medications won’t help.

Clinical Trials

Scientists are always researching new treatments -- be it a drug or medical device -- to make sure they work as well as expected. These clinical trials rely on volunteers.

If you choose to sign up for one, you may find a new treatment that helps your CF. There’s also a chance that the treatment you try won’t help. Still, many people who take part in a clinical trial like knowing that they’re helping in CF research.

Surgery

Sometimes CF causes problems that can only be fixed by surgery or another type of medical procedure. These might include:

Sinus surgery: Many people with CF have inflamed or infected sinuses. Your doctor may need to remove nasal polyps (growths inside your nasal passages). She can also do a procedure called an “endoscopy and lavage” that suctions mucus from your airways. This will make it easier for you to breathe.

Feeding tube: If you’re malnourished, you may need a feeding tube. This allows you to get extra nutrition while you sleep. Your doctor might thread it through your nose or place it into your stomach.

Bowel surgery: Some people with CF have very thick, sticky poop. This can lead to a blockage or cause the bowel to fold in on itself. In both cases, surgery may be necessary.

Lung transplant: If you have severe lung problems and medicine isn’t helping, you may need to consider a lung transplant. This surgery has risks, but it can provide you with a new set of lungs that hasn’t been harmed by CF.

WebMD Medical Reference Reviewed by Dan Brennan, MD on August 17, 2018

Sources

SOURCES:

Mayo Clinic: “Cystic Fibrosis -- Treatment,” “Cystic Fibrosis -- Symptoms and Causes.”

Cystic Fibrosis Foundation: “Life with CF: Treatments and Therapies,” “Nebulizer Care at Home,” “CFTR Modulator Therapies,” “Chest Physical Therapy,” “Why Participate in a Clinical Trial?” “Weighing the Benefits and Risks,” “Fitness.”

National Heart, Lung, and Blood Institute: “How is Cystic Fibrosis Treated?” “What Are Clinical Trials?”

Johns Hopkins Cystic Fibrosis Center: “Treatments: Pancreas/Gastrointestinal Tract.”

Cold Spring Harbor Perspectives in Medicine: “The Cystic Fibrosis Intestine.”

NHS: “Treating Cystic Fibrosis.”

University of Michigan Medicine: “Bronchodilators for Cystic Fibrosis.”

Cystic Fibrosis Center at Stanford: “Sinusitis.”

FDA. Drug Trials Snapshots: SYMDEKO.
 

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