Other Effects of Cystic Fibrosis

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By ACCORDANT HEALTH SERVICES

Many people know that CF causes problems with the lungs and the digestive system. But CF can also affect some other parts of the body too.

Muscles
CF can make the leg and arm muscles weak. It can also sometimes cause the diaphragm muscle to be weak. The diaphragm is a thin, parachute-shaped muscle that separates the heart and lungs from the other organs. It is the most important muscle that you use when you breathe. It moves down when we breathe in, and up when we breathe out. Problems with the diaphragm can worsen breathing. CF affects the arm and leg muscles more often than it affects the diaphragm.

Muscle weakness is common among those with CF. It can be caused by many things. Some of the possible causes are:

• Poor nutrition
• Not using the muscles very much
• Weight loss
• Not enough calories from proteins
• Inflammation
• Corticosteroid medicines (the amount of weakness depends on the dose)

Good nutrition and regular exercise can help keep muscles working well.

Bladder
People with CF cough a lot – an average of 643 times a day! Coughing so much puts ongoing stress on the bladder muscles. This can cause a loss of control over urine flow when coughing, sneezing, laughing, or lifting something. It happens when the pelvic floor muscles are weak. The pelvic floor muscles are like a sling that runs from your pubic bone to your tail bone. Urine leakage occurs because the pressure inside the bladder is greater than the pressure from these muscles that control the release of urine.

Loss of control over urine flow affects both men and women, but it is more common in women. About 65 out of 100 women with CF have this problem. Women with poor lung function are most likely to have it.

If you have urine leakage tell your doctor. There are good ways to solve this problem. You can learn how to strengthen your pelvic floor muscles with exercises. The exercises are done by pretending to stop the flow of urine without using any muscles in your abdomen or legs. The pelvic floor muscles are tensed for about 10 seconds, then relaxed. If the exercises do not work for you, there are other treatment options too.

Kidney Stones
About 6 out of 100 people (6%) with CF get kidney stones. Of these people, about 40% get kidney stones more than once. People who do not make much urine are more likely to get kidney stones. Those who often have diarrhea may be more likely to get calcium-based stones. Calcium-based stones are the type that occur most often in those with CF.

Kidney stones cause pain, fever, nausea, and vomiting. If kidney stones are not treated, infection can occur. The main treatments are drinking a lot of water and taking pain relievers. Most kidney stones pass out of the body within in 48 hours. If they do not pass out, other treatments can be done.

Upper Airway
The upper airway is made up of the nose, the sinuses, and the throat. The main job of the upper airway is to filter and warm the air you breathe before it goes to the lungs.

CF can cause two problems in the upper respiratory tract:

• Sinusitis
  

  Sinuses are air spaces in the bones of your head. There are four pairs of sinuses. They are lined with mucus membranes. Sinusitis is swelling of the mucus membranes in the sinuses. In CF this inflammation can be caused by thick mucus that blocks the sinuses. The blockage may be hard to clear up, and this can lead to a sinus infection.

  Adults with chronic (long-lasting) sinusitis may have bacteria in their sinuses that is like the bacteria often found in the lungs. Symptoms of a sinus infection can include a low fever, nasal drainage, tiredness, and nasal polyps. A sinus infection is treated with medicines such as antibiotics, antihistamines, and decongestants. Sometimes the sinuses are drained with surgery. It is very important to treat chronic sinusitis before lung transplant surgery. If surgery is done before sinusitis is treated, the new lungs may get infected.

• Nasal Polyps
  

  Nasal polyps are small growths of flesh on the lining of the inside of the nose. Nasal polyps are less common than sinusitis. They are often found in children with CF. Polyps do not usually cause problems, but sometimes they can make it harder to breathe. If they block breathing, polyps can be surgically removed or treated with medicine. The cause of nasal polyps is not known.

References

Johns Hopkins Cystic Fibrosis Center Web site. http://hopkinscf.org/main/whatiscf/treat_muscle.html. Accessed June 3, 1008.

A.D.A.M. Medical Encyclopedia [Internet]. Atlanta, GA: A.D.A.M., Inc.; ©2005. Diaphragm; [updated 2007 July 26; cited 2008 July 10]; [1p.]. Available from: http://www.nlm.nih.gov/medlineplus/ency/imagepages/19072.htm.

Med Terms Web site. http://www.medterms.com/script/main/art.asp?articlekey=2983. Accessed July 10, 2008.

Barry SC, Gallagher CG. Corticosteroids and skeletal muscle function in cystic fibrosis. J Appl Physiol. 2003:95(4):1379-84.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/main/whatiscf/effects_bladder.html. Accessed June 3, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://hopkinscf.org/main/whatiscf/effects_muscleb.html. Accessed June 3, 2008.

Med Terms Web site. http://www.medterms.com/script/main/art.asp?articlekey=6650. Accessed July 11, 2008.

Johns Hopkins Cystic Fibrosis Center Web site. http://www.hopkinscf.org/main/whatiscf/effects_sinus.html. Accessed June 3, 2008.

Cunningham JC, Taussig LM, An Introduction to Cystic Fibrosis for Patients and Families. Fifth edition. Cystic Fibrosis Foundation; 2003.

Med Terms Web site. http://www.medterms.com/script/main/art.asp?articlekey=22702. Accessed July 10, 2008.

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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