Cystic Fibrosis

Cystic Fibrosis

(CF)

Definition

Cystic fibrosis (CF) is an inherited disease that affects the lung, airways, and digestive systems. It causes the body to make thick and sticky mucus. This can cause problems with breathing and digestion.

Cystic Fibrosis

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Causes

A child with CF has faulty genes from each parent. Parents who have the gene, but do not have CF, are called carriers.

Risk Factors

This problem is more common in people with:

  • Parents who carry the CF gene
  • Brothers or sisters with CF
  • A mother with CF—most men with it cannot father children

Symptoms

Common problems are:

  • Salty sweat
  • A lasting coughing
  • Coughing up thick or bloody mucus
  • Wheezing or problems breathing
  • Weight loss
  • Poor growth (in children)
  • Being less active, such as missing school or work

Diagnosis

This problem is diagnosed based on symptoms, family history, or a positive screening test in newborns. It can be confirmed with genetic testing. Other lab tests that may be done are:

  • Sweat chloride testing
  • Transepithelial nasal potential difference measurement

Treatment

There is no cure. It can be managed by:

  • Making dietary changes
  • Managing lung and sinus infections
  • Keeping the airways and lungs clear

Choices are:

Dietary Changes

Dietary changes will need to be made to improve nutrition. This can be done with:

  • A high-calorie diet planned by a dietitian
  • Supplements, such as fat-soluble vitamins and pancreatic enzyme tablets
  • Adding salt to the diet during hot weather or illnesses

Managing Infections

Thick mucus raises the risk of severe infections. Antibiotics will be used to help treat and prevent them. Yearly flu shots should also be given.

Lung and Airway Support

Medicines help keep the airways clear. Choices are:

  • Bronchodilators to relax muscles and open the airway
  • Mucolytic agents to reduce mucus and help it move out of the lungs
  • Steroid inhalers to ease swelling and irritation (only when needed)

Other things that may help clear the airways include:

  • Inhaling hypertonic saline, which is a special type of salt water
  • Chest percussion is rhythmic clapping over the chest

People with severe breathing problems may need oxygen therapy or ventilation.

Other Treatment

Some people with severe symptoms may need lung transplant surgery when other methods do not help. Surgery may be also be done to treat blockages in the intestine.

Prevention

There are no current guidelines to prevent CF.

RESOURCES:

American Lung Association
http://www.lung.org
Cystic Fibrosis Foundation
http://www.cff.org

CANADIAN RESOURCES:

About Kids Health—The Hospital for Sick Children
http://www.aboutkidshealth.ca
The Lung Association
http://www.lung.ca

References:

About cystic fibrosis: what you need to know. Cystic Fibrosis Foundation website. Available at:
...(Click grey area to select URL)
Accessed May 1, 2020.
Cystic fibrosis. Healthy Children—American Academy of Pediatrics website. Available at: https://www.healthychildren.org/English/health-issues/conditions/chronic/Pages/Cystic-Fibrosis.aspx. Updated November 21, 2015. Accessed May 1, 2020.
Cystic fibrosis (CF). EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/cystic-fibrosis-cf . Updated April 13, 2020. Accessed May 1, 2020.
Elborn JS. Cystic fibrosis. Lancet. 2016 Nov 19;388(10059):2519-2531.
Last reviewed February 2020 by EBSCO Medical Review Board Daniel A. Ostrovsky, MD
Last Updated: 05/01/2020

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