Cystic Fibrosis

Medically reviewed by Drugs.com. Last updated on Apr 6, 2025.

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Cystic fibrosis (CF) is a lifelong condition that affects your lungs, digestive system, and other organs. Your mucus, tears, sweat, and saliva become so thick and sticky that they clog your lungs and digestive system. CF usually causes problems with breathing and with breaking down and absorbing food.

WHILE YOU ARE HERE:

Informed consent

is a legal document that explains the tests, treatments, or procedures that you may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done. Make sure all your questions are answered.

Nutrition:

You may need to eat foods that have extra calories, fat, vitamins, or calcium. You may also need vitamin supplements if the level in your blood is too low.

Oxygen:

You may need extra oxygen if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils. Ask your provider before you take off the mask or oxygen tubing.

Drugs used to treat this and similar conditions

Medicines:

• Antibiotics help fight or prevent an infection caused by bacteria.
• Steroids help decrease inflammation in your lungs and help you breathe more easily.
• Mucus-thinning medicine is breathed in to help thin lung mucus so you can cough it up more easily.
• Bronchodilators help open the air passages in your lungs, and help you breathe more easily.
• Pancreatic enzymes help your digestive system break down food and absorb nutrients.
• Cystic fibrosis transmembrane regulators (CFTR) may help improve your symptoms if your CF is caused by a certain protein mutation. Your healthcare provider or CF specialist can give you more information about CFTRs.

Tests:

• Blood tests may be used to find signs of infection and to check kidney function.
• Pulmonary function tests will show how much oxygen your body is getting. You breathe into a mouthpiece connected to a machine. The machine measures how much air you breathe in and out over a certain amount of time.
• A sputum sample is tested to find common sources of infection that can happen along with CF. Mucus from your lungs is collected in a cup when you cough. You may need to give 3 samples of your sputum, usually first thing in the morning.
• A fecal fat test is used to check the amount of fat in your bowel movements. Your body may not be able to absorb fat from the foods you eat. You will be told how to get a sample of each bowel movement you have over a 72 hour period.
• An oral glucose tolerance test may be given each year to check for diabetes.
• CT scan pictures of your sinuses may show growths, swelling, and mucus buildup. You may be given contrast liquid to help problems show up better in the pictures. Tell the provider if you have ever had an allergic reaction to contrast liquid.
• Genetic counseling can help you learn why you have CF. If you plan to have a baby, genetic counseling may show if your child is at risk for CF.

Treatment:

• Airway clearance techniques are exercises your provider teaches you to help remove mucus so you can breathe more easily. These exercises may be used along with machines or devices to help decrease your symptoms and risk for infection.
• Surgery may be needed if you have severe damage to organs, such as your liver or lungs. Ask your provider for more information about surgery.
• A ventilator is a machine that gives you oxygen and breathes for you when you cannot breathe well on your own. An endotracheal (ET) tube is put into your mouth or nose and attached to the ventilator. You may need a trach if an ET tube cannot be placed. A trach is a tube put through an incision and into your windpipe.

Treatment options

The following list of medications are related to or used in the treatment of this condition.

• Pulmozyme
• Creon
• Kalydeco
• Trikafta
• Cayston

View more treatment options

RISKS:

Your symptoms may not go away, or they may come back after treatment. Your lungs, liver, or pancreas may be damaged. You may not get enough nutrients, and you may have severe weakness. You may be at increased risk for broken bones. You can develop pancreatitis or diabetes. You may be at higher risk for lung infections. Your liver can develop cirrhosis (scarring) and not work normally. You may have severe breathing or bleeding problems, or a collapsed lung. These problems can be life-threatening.

CARE AGREEMENT:

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your healthcare providers to decide what care you want to receive. You always have the right to refuse treatment.

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The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

Further information

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