Munson Health
 
Cystic Fibrosis

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by Carson-DeWitt R

(CF)

 

Symptoms

The abnormally thick mucus of CF blocks certain organs. This causes many of the symptoms of CF.
Symptoms in infants may include:
  • Difficulty passing the first stool
  • Intestinal obstruction, sometimes requiring surgery
  • Salty sweat
Mucus that causes blockages in the lungs may lead to:
  • Coughing and wheezing
  • Shortness of breath
  • Difficulty with exercise
  • Abnormally shaped clubbed finger tips
  • Malformed chest
Mucus can also block the pancreas. This can block enzymes used to help you digest food. This can lead to:
Other symptoms may include:
  • Mildly decreased fertility in females
  • Prolapsed rectum
  • Prevention of sperm production in males
  • Nasal polyps
  • Chronic nasal congestion from chronic sinus infections
  • Jaundice or other symptoms of liver disease
  • Excessive thirst or urination that may indicate diabetes mellitus type 2
  • Stomach pain or swelling from intestinal blockage
Overall, girls are affected more severely than boys.
 

Treatment

There is no cure for CF. Treatment is aimed at:
  • Improving the amount of nutrition your body receives
  • Preventing and treating lung and sinus infections
  • Keeping the airways and lungs as clear as possible
Treatment for CF includes:

Nutritional Support

Better nutrition will help improve overall health. It will also improve growth and development in children. Children who have returned to normal weight within two years of the diagnosis have fewer coughing episodes and better lung function. Some nutritional steps that may help include:
  • A 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 and salt replacement, especially in hot weather or during illnesses

Managing Lung Infections

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:
  • Vaccination
  • Antibiotics (usually inhaled)

Other Treatment

Surgery may be required to treat blockages in the intestine. Lung and liver transplants may also be considered.
Support is important for those with CF and their families. Ask you doctor about local support groups or counseling options.
If you or your child is diagnosed with CF, follow your doctor's instructions . Keep all follow-up appointments as advised by your doctor.
If you or your child is diagnosed with CF, follow your doctor's instructions . Keep all follow-up appointments as advised by your doctor.
 

RESOURCES

American Lung Association
http://www.lungusa.org

Cystic Fibrosis Foundation
http://www.cff.org

 

CANADIAN RESOURCES

About Kids Health
http://www.aboutkidshealth.ca

SickKids
http://www.sickkids.ca

 

References


About cystic fibrosis: what you need to know. Cystic Fibrosis Foundation website. Available at: http://www.cff.org/AboutCF. Accessed May 21, 2013.


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 May 21, 2013.


Cystic Fibrosis. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated May 2, 2013. Accessed May 21, 2013.


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.page. Accessed May 21, 2013.


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;123:714-722.


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 Jul 25;7.

 

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