- Blockages in the lungs and airways
- Problems digesting and absorbing nutrients
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- Parents who are known carriers of the CF gene
- Siblings with CF
- Parents with CF (mostly mother since men with CF are often sterile)
- Difficulty passing the first stool (meconium)
- Meconium ileus (intestinal obstruction), sometimes requires surgery
- Salty sweat
- Coughing and wheezing
- Shortness of breath
- Difficulty with exercise
- Repeated pneumonia
- Severe, chronic sinusitis
- Abnormally shaped clubbed finger tips
- Barrel chest
- Nasal polyps
- Trouble gaining weight
- Poor growth
- Failure to thrive
- Bulky, bad-smelling, floating stools, due to poor digestion of fats
- Insulin-dependent diabetes
- Liver cirrhosis
- Salty sweat
- Mildly decreased fertility in females
- Prolapsed rectum
- Prevention of sperm production in males
- Sweat chloride testing (still the standard for making a diagnosis of CF)
- Transepithelial difference measurement
- Improving the amount of nutrition your body receives
- Preventing and treating lung infections
- Keeping the airways and lungs as clear as possible
- High-calorie diet planned by a registered dietitian
- Nutritional supplements, including fat-soluble vitamins
- Pancreatic enzyme tablets with meals to improve digestion and absorption of nutrients
- Drinking lots of fluids, especially in hot weather or when ill
Managing Lung Infections
- Inhaled and/or systemic antibiotics
- Antibiotics to treat recurrent lung infections
Lung and Airway Support
- Bronchodilators—to relax muscles and open the airway
- Steroid inhalers—to decrease swelling and irritation
- Mucolytic agents—to reduce mucus and help it move out of the lung
- Hypertonic saline is a special type of salt water. A nebulizer machine creates a mist of this saline, which is inhaled by the user. The mist may help thin out the mucus in the lungs.
- Chest percussion and postural drainage or high-frequency chest wall oscillation are rhythmic clapping over the chest. They may help clear mucus from airways.
American Lung Association http://www.lungusa.org/
Cystic Fibrosis Foundation http://www.cff.org/
About Kids Health http://www.aboutkidshealth.ca/
Sick Kids http://www.sickkids.ca/
About cystic fibrosis: what you need to know. Cystic Fibrosis Foundation website. Available at: http://www.cff.org/AboutCF/ . Accessed December 11, 2012.
Cystic fibrosis. American Academy of Pediatrics Healthy Children website. Available at: http://www.healthychildren.org/English/health-issues/conditions/chronic/Pages/Cystic-Fibrosis.aspx . Updated January 9, 2012. Accessed December 11, 2012.
Cystic Fibrosis. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed . Updated November 7, 2012. Accessed December 11, 2012.
Cystic fibrosis 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/cystic-fibrosis-testing.shtml . Accessed December 11, 2012.
Conn HF, Rakel RE. Conn’s Current Therapy. 54th ed. Philadelphia, PA: WB Saunders Company; 2002: 720-721.
Federico, MJ, Kerby, GS, Deterding, RR, et al. Respiratory Tract & Mediastinum. In Hay W.W., et al, Eds. Current Diagnosis & Treatment: Pediatrics . 20th ed. New York, NY: McGraw-Hill Companies, Inc; 2011.
Kleigman RM, Jensen HB, Behrman RE, Stanton BF. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, PA: Saunders Elsevier; 2007.
4/16/2009 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.
- Reviewer: Michael Woods, MD
- Review Date: 11/2012 -
- Update Date: 11/26/2012 -