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Cystic fibrosis

What is it?

  • Cystic fibrosis (CF) is a long-term disease that causes the exocrine (EKS-o-krin) glands to stop working normally. The exocrine glands make thin secretions, such as sweat, mucus, tears, and saliva (spit). Exocrine glands also help make juices in the digestive system. This system includes the esophagus (e-SOF-uh-gus), stomach, and intestines (bowels). With CF, the digestive and respiratory (RES-per-uh-tor-e) systems are most affected. The respiratory system is the breathing system, which includes the lungs.
  • With CF, the exocrine glands make mucus and secretions that are too thick and sticky. These secretions may plug up ducts or other passages in your body. A duct is a narrow tube through which secretions flow. Mucus plugs may plug up ducts in the lungs, pancreas, and intestines. These plugs can cause breathing and food digestion problems. CF also affects sweat glands by causing high amounts of salt in your sweat.
  • There are about 40,000 people in the United States with CF. This disease used to be known as a children's disease, but adults may also have it. Scientists have found new treatments that may help you live a longer life with CF.

Causes:

CF is a genetic disease. One in 29 Americans is a carrier of CF, which means they have the CF gene. Genes are pieces of code that are responsible for telling cells how to grow. For example, your genes "told" your body to grow your color hair, and your eye color. A person must get a CF gene from both parents to develop the disease. You cannot "catch" CF from someone with CF.

Signs and Symptoms:

CF affects each person differently. It may be found in babies because of digestive problems, obstruction (blockage) of the intestine, or breathing problems. CF may not be found in some people until later in life, if they have a milder case of the disease. You may have salty-tasting skin, a long-term cough, pneumonia (new-MOAN-yuh), or wheezing. Wheezing is the loud noise heard when you breathe in or out. You may have problems with nutrition and gaining weight, even though you eat a lot. You may get many lung infections. Some other signs of CF that may develop over time are:

  • Clubbing of the fingers and toes. This is when the ends of your fingers and toes get round and large.
  • Thick mucus in your intestines may cause abdominal (belly) pain, gassiness, or blocked intestines. You may have BMs (stools) that are large, greasy, and bad smelling. Because of these problems, you are at higher risk for problems with your rectum (where you have BM's).
  • Redness, swelling, and pain in the nose passages. This is called sinusitis. Nasal polyps (PAW-lips), which are fleshy growths inside your nose, may also occur.

Medical Care:

There is no cure for CF. Treatment for CF depends upon what stage the disease is in and what organs are affected. Antibiotics are used to treat lung infections. Digestive enzymes are used to improve digestion. Medicine that thins the thickened airway secretions may be needed. You may need to go into the hospital for more care. You may have any of the following tests or treatments.

  • Blood tests.
  • Bowel movement (BM) tests.
  • Breathing exercises.
  • Chest x-ray.
  • Genetic counseling.
  • Intravenous (IV) fluids.
  • Medicine breathing treatments.
  • Medicines and vitamins.
  • Postural drainage.
  • Pulmonary (lung) function tests.
  • Sputum (spit) test.
  • Sweat test.

Dietary Measures:

  • Eat a high fat (100 grams fat a day), high calorie diet. This will keep you stronger and may also help prevent lung infections.
  • You may want to eat many small meals each day rather than 3 large meals. Eat healthy foods from all of the food groups, which includes fruits, vegetables, breads, dairy products, meat and fish. Eating healthy foods may help you feel better and have more energy.
  • Foods like fruit, bran, and prune juice can help you have a BM. Drinking water can also help you pass BMs easier. Caregivers may give you fiber medicine or stool softening medicines to make your BMs softer and regular.
  • If you do not have to limit your liquids, drink 6 to 8 cups of water each day. This helps thin the sputum (spit) so it can be coughed up more easily.
  • Food allergies may be more common in CF patients. Keep a food diary to learn what food may trigger a symptom.

Herbs and Supplements:

Before taking any herbs or supplements, ask your caregiver if it is OK. Talk to your caregiver about how much you should take. If you are using this medicine without instructions from your caregiver, follow the directions on the label. Do not take more medicine or take it more often than the directions tell you to. The herbs and supplements listed may or may not help treat your condition.

Supplements:

    Complementary Therapies:

    • Biofeedback and relaxation techniques may help CF patients.
    • Hypnosis, like biofeedback, may be helpful for CF patients.
    • Exercise can help improve lung function in CF.

    Other ways of treating your symptoms : Other ways to treat your symptoms are available to you.

    Talk to your caregiver if:

    • You would like medicine to treat CF.
    • Your symptoms have not gone away or improved by these self-help measures.
    • You are urinating less, have a dry mouth and cracked lips, or are dizzy. These are signs of dehydration (d-hi-DRA-shun).
    • You have chills or feel weak or achy. These are signs that you may have an infection.
    • You have questions about what you have read in this document.

    SEEK CARE IMMEDIATELY IF:

    • You have trouble breathing.
    • The skin between your ribs is being sucked-in with each breath.
    • Your lips or fingernails are turning blue or white.

    Care Agreement:

    You have the right to help plan your care. To help with this plan, you must learn about your health condition and how it may be treated. You can then discuss treatment options with your caregivers. Work with them to decide what care may be used to treat you. You always have the right to refuse treatment.

    References:

    1. Belsky J & Khanna P: The effects of self-hypnosis for children with cystic fibrosis: a pilot study. Am J Clin Hypn 1994; 36(4):282-292.

    2. Darling PB, Lepage G, Leroy C et al: Effect of taurine supplements on fat absorption in cystic fibrosis. Pediatr Res 1985; 19(6):578-582.

    3. Delk KK, Gevirtz R, Hicks DA et al: The effects of biofeedback assisted breathing retraining on lung functions in patients with cystic fibrosis. Chest 1994; 105(1):23-28.

    4. Duijvestijn YC & Brand PL: Systematic review of n-acetylcysteine in cystic fibrosis. Acta Paediatr 1999; 88(1):38-41.

    5. Gavin J, Ellis J, Dewar AL et al: Dietary fibre and the occurrence of gut symptoms in cystic fibrosis. Arch Dis Child 1997; 76(1):35-37.

    6. Leser C, Rothe TB & Karrer WL: Rehabilitation of cystic fibrosis in adulthood. Schweiz Rundsch Med Prax 1997; 86(50):1984-1990.

    7. Lucarelli S, Quattrucci S, Zingoni AM et al: Food allergy in cystic fibrosis. Minerva Pediatr 1994; 46(12):543-548.

    8. Roum JH, Borok Z & McElvaney NG: Glutathione aerosol suppresses lung epithelial surface inflammatory cell-derived oxidants in cystic fibrosis. J Appl Physiol 1999; 87(1):438-443.

    9. Shepherd RW, Holt TL, Thomas BJ et al: Nutritional rehabilitation in cystic fibrosis: controlled studies of effects on nutritional growth retardation, body protein turnover, and course of pulmonary disease. J Pediatr 1986; 9(5):788-794.


    Last Updated: 4/4/2014

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