What is the Treatment for Cystic Fibrosis?

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There is no cure for CF (cystic fibrosis), so treatments focus on the symptoms of the disease. The goals of the treatments are to:

• Keep your lungs from getting infected, or control infection if it occurs
• Clear as much mucus from your lungs as possible
• Improve your breathing
• Prevent blockages in your intestines
• Make sure you are getting enough calories and nutrients

Today, there are more treatments for CF than ever before. Everyone with CF does not need the same treatments, so each person has their own treatment plan.

Treatments for Lung Problems
When mucus builds up in the lungs it blocks the airways and makes it hard to breathe. The extra mucus also makes it easy for bacteria to grow and cause a lung infection.

There are five main treatments for CF lung problems:

1. Airway Clearance Techniques
Airway clearance techniques (ACTs) loosen mucus and clear it from the lungs. ACTs are very important daily treatments. They help reduce lung infections and make it easier to breathe. There are many ACTs and most of them are easy to do. Some ACTs include coughing, deep breathing, or vibrating the chest by hand. Other ACTs involve wearing a special vibrating vest or blowing into a special device. A family member, friend, nurse, or therapist can perform airway clearance for small children. Older children and adults may not need any help.

2. Antibiotics
Most people with CF have mild, ongoing lung infections. Sometimes a lung infection can become serious. Antibiotics are medicines that fight the bacteria that cause lung infections. Bacteria can become resistant to some antibiotics. To avoid this, your doctor may sometimes change your antibiotic.

For mild infections, antibiotic pills, capsules, or liquids are taken by mouth. For serious infections, IV (put into a vein) antibiotics are given. IV antibiotics are often given if antibiotics taken by mouth do not work. Antibiotics can also be given in an aerosol mist that you inhale. One aerosol antibiotic, TOBI® (tobramycin) is used to fight the bacteria that cause most ongoing lung infections. Aerosol antibiotics are sometimes given along with antibiotics taken by mouth.

Some people need to take antibiotics every day. Others only need them from time to time. Your doctor decides what kind of antibiotic you need, and how often you need it, based on your sputum culture results. A sputum culture is a test that checks for infection-causing germs in your mucus.

3. Other Aerosol Medicines
There are two other kinds of inhaled treatments for CF besides aerosol antibiotics:

• Bronchodilators. Bronchodilators are used by a lot of people with CF. They open up the airways. When inhaled just before an ACT, a bronchodilator helps move mucus. Some bronchodilators are albuterol and terbutaline.
• Mucus-thinners. These treatments, called mucolytics, make mucus thin and easier to cough out. One medicine, Pulmozyme® (dornase alfa), is often used for those age 6 or older with mild, moderate, or severe lung disease. It helps the lungs work better.

Hypertonic saline is a different mucus thinner. It is a mist of very salty water that helps the lungs work better. It is used after a bronchodialator, to help move mucus out of the lungs.

4. Anti-inflammatory Medicines
Anti-inflammatory medicines reduce the inflammation (swelling) of the lung tissues. Reducing lung inflammation helps prevent damage to the lungs. Ibuprophen, a non-steroid anti-inflammatory (NSAID) medicine, is often used. It may also slow the progress of CF in young children with mild symptoms. Those who take ibuprophen for CF must be closely monitored by a doctor. Corticosteroids are anti-inflammatory medicines that are used to treat other diseases, but experts are not sure yet they should be used to treat CF.

5. Exercise
Most everyone with CF should exercise. Exercise forces you to breathe deeply and may even help expand your airways. A physical therapist can design an exercise program for you based on the severity of your CF, your fitness level, and the activities you enjoy. A good exercise program includes two types of activities:

• Endurance exercise such as swimming, running, cycling, or aerobics classes
• Strength-building activity such as weight training

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Treatments for Digestive Problems
In CF, mucus blocks the tube that takes enzymes from the pancreas to the small intestine. Without enzymes, food cannot be digested well. More than 9 out of 10 people with CF have trouble digesting fats and proteins. This makes it hard to gain weight and has a serious impact on health. The good news is that the digestive problems of CF can be treated.

Three main treatments are used for most people:

1. Nutrition
Good nutrition is needed for good health. If you have CF, you need more calories than other people. This is because your body has trouble getting nutrients from food, and also because it takes more energy for you to breathe. There are no restrictions on diet for adults or children with CF. The best diet for anyone with CF is a high fat, high protein, high calorie diet. Both adults and children with CF should eat high-calorie foods and high-calorie snacks such as nuts and milkshakes.

2. Pancreatic Enzymes
Most people with CF need to take pancreatic enzyme capsules whenever they eat. These enzymes replace the natural ones that are blocked by mucus in the pancreas. Enzymes must be taken with all meals and snacks. Capsules are taken at the beginning and also at the end of each meal. Babies with CF need enzymes to help them digest breast milk or formula. For babies, the capsules are opened and the enzymes are sprinkled into a soft acidic food like applesauce. Older children and adults just swallow them with liquid.

Taking enzymes helps you digest fats, proteins, and carbohydrates, which are the body's main sources of calories. People who take enzymes have an easier time gaining weight.

3. Vitamins
Vitamins A, D, E, and K are the fat-soluble vitamins. This means they dissolve in fat, not in water as the other vitamins do. Vitamins A, D, E, and K are absorbed in your small intestines along with the fat you eat. If you are like most people with CF, your body does not digest fat well and you do not get enough fat-soluble vitamins. Your doctor can prescribe water-soluble forms of these vitamins for you to take. Most people take them twice a day. Taking enzymes will help you absorb more vitamins. Those who take a lot of antibiotics may need extra vitamin K.

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References

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Cystic Fibrosis Foundation Web site. http://www.cff.org/AboutCF/Faqs/ . Accessed May 13, 2008.

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Last Modified Date: March 19, 2009 © Accordant Health Services, a CVS/Caremark company.  All rights reserved.
This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.
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