Cystic fibrosis (CF) is an inherited disease. It causes a defect in certain cells of the lung and digestive systems. The defect makes the cells produce a thick, sticky mucus. This mucus can cause:
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CF is a serious life-long condition, but the severity of the illness can vary greatly. The average life expectancy for someone with CF is about 35 years. Although, some with mild forms of CF can live to age 60 or beyond.
CF is a genetic disorder. A child with CF inherits defective genes from each parent. Parents who have the gene, but do not have CF, 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 in infants may include:
Mucus that causes blockages in the lungs may lead to:
Mucus can also block the pancreas. This can block enzymes used to help you digest food. This can lead to:
Other symptoms may include:
Overall, girls are affected more severely than boys.
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.
CF is often diagnosed by symptoms, family history of CF, or a positive screening test in newborns. The diagnosis may be confirmed with genetic testing. Other lab tests that may be used to confirm CF include:
Your doctor may need to check your lungs. This may be done to look for symptoms or to determine treatment. Tests may include:
Tests may also be needed to check the pancreas. These tests may be done to assess symptoms or determine treatment.
There is no cure for CF. Treatment is aimed at:
Treatment for CF includes:
Better nutrition will help improve overall health. It will also improve growth and development in children. Children who have returned to normal weight within 2 years of the diagnosis have fewer coughing episodes and better lung function. Some nutritional steps that may help include:
Thick mucus in the airways increases the risk of respiratory infections. The infection can also be more severe because of the mucus. Treatment of a current infection often requires antibiotics. Prevention of new infections may be done with:
Medications help keep the airways clear. Most will be delivered through an inhaler or nebulizer. Medications may include:
Other steps that may help clear mucus from the lungs include:
Oxygen therapy may be required as the disease progresses. Ventilation may also be needed. Talk to your doctor about whether these are options for you.
If you have the defective genes, there is no way to prevent CF.
Adults can be tested to see if they carry the genes before having children. Prenatal testing can determine if a baby will have CF.
About Kids Health—The Hospital for Sick Children
The Lung Association
About cystic fibrosis: what you need to know. Cystic Fibrosis Foundation website. Available at: http://www.cff.org/AboutCF. Accessed February 12, 2016.
Cystic fibrosis. American Academy of Pediatrics Healthy Children website. Available at: https://www.healthychildren.org/English/health-issues/conditions/chronic/Pages/Cystic-Fibrosis.aspx. Updated November 21, 2015. Accessed February 12, 2016.
Cystic fibrosis (CF). EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated January 26, 2016. Accessed February 12, 2016.
Genetic testing. American Medical Association website. Available at: http://www.ama-assn.org/ama/pub/physician-resources/medical-science/genetics-molecular-medicine/related-policy-topics/genetic-testing.page. Accessed February 12, 2016.
4/16/2009 DynaMed's Systematic Literature Surveillance http://www.ebscohost.com/dynamed: 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(2):714-722.
10/1/2013 DynaMed's Systematic Literature Surveillance http://www.ebscohost.com/dynamed: Elphick HE, Mallory G. Oxygen therapy for cystic fibrosis. Cochrane Database Syst Rev. 2013;7:CD003884.
Last reviewed February 2016 by Rimas Lukas, 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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