Cystic fibrosis (CF) is an inherited disease. The defect occurs in epithelial (or lining) cells. These cells normally create a mucus. The mucus is a vital tool for many organs. CF causes the cells to produce a very thick and rubbery mucus. This most commonly causes:
CF is an autosomal recessive disorder. This means the child inherits one defective gene from each parent. Usually the parents do not have the disease but carry the gene. These parents are called carriers.
Factors that increase your chance of CF include:
The abnormally thick mucus of CF blocks certain organs. This causes many of the symptoms of CF.
Symptoms include:
CF is a serious life-long condition. It significantly reduces longevity. It also results in illness and poor health for most. Girls tend to be affected more severely than boys. The severity of illness can vary greatly. Some persons with mild forms of CF can live to age 60 or beyond.
The doctor will ask about symptoms and medical history. A physical exam will be done. CF is suspected in a child with classic symptoms, especially if a sibling has CF.
Tests may include:
There is no cure for CF. Treatment is aimed at:
Treatment for CF includes:
Researchers are exploring gene therapy. This may slow the progression of CF, or even cure it.
If you or your child is diagnosed with CF, follow your doctor's instructions .
RESOURCES:
American Academy of Pediatrics
http://www.aap.org/
American Lung Association
http://www.lungusa.org/
Cystic Fibrosis Foundation
http://www.cff.org/
References:
American Academy of Pediatrics website. Available at: http://www.aap.org .
American Medical Association website. Available at: http://www.ama-assn.org/ .
Conn HF, Rakel. Conn’s Current Therapy. 54th ed. Philadelphia, PA: WB Saunders Company; 2002: 720-721.
Cystic Fibrosis Foundation website. Available at: http://www.cff.org .
Kleigman RM, Jensen HB, Behrman RE, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, PA: Saunders Elsevier; 2007.
*¹4/16/2009 DynaMed's Systematic Literature Surveillance http://www.ebscohost.com/dynamed/what.php : Lai HJ, Shoff SM, Farrell PM; Wisconsin Cystic Fibrosis Neonatal Screening Group. Recovery of birth weight z score within 2 years of diagnosis is positively associated with pulmonary status at 6 years of age in children with cystic fibrosis. Pediatrics. 2009;123:714-722.
Last reviewed January 2009 by Kari Kassir, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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