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Cystic Fibrosis

Authors:
  • Cynthia M. Johnson, MA
Publication Type:

Condition

Cystic Fibrosis

(CF)

Definition

Cystic fibrosis (CF) is an inherited disease. It causes the body to make thick and sticky mucus. This can cause problems with breathing and digestion.

CF can range from mild to life-threatening.

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 children, adolescents, and people who are White. Other things that raise the risk are:

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

Symptoms

Some people have few or no symptoms. Others can have severe, life-threatening 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 for CF. It can be managed by:

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

Options are:

Diet Changes

Diet changes are needed 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 help treat and prevent them. Yearly flu shots should also be given.

Lung and Airway Support

Medicines help keep the airways clear. Options 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, a special type of salt water
  • Chest percussion— 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. This is used when other methods do not help. Surgery may also be done to treat blockages in the intestine.

Prevention

There are no current guidelines to prevent CF.

RESOURCES:

CANADIAN RESOURCES:

CANADIAN RESOURCES:

References

  • Cystic fibrosis (CF). EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/cystic-fibrosis-cf
  • Cystic fibrosis. Healthy Children—American Academy of Pediatrics website. Available at: https://www.healthychildren.org/English/health-issues/conditions/chronic/Pages/Cystic-Fibrosis.aspx.
  • Cystic fibrosis. Johns Hopkins Medicine website. Available at: https://www.hopkinsmedicine.org/health/conditions-and-diseases/cystic-fibrosis.
  • Farrell PM, White TB, et al. Diagnosis of cystic fibrosis: consensus guidelines from the Cystic Fibrosis Foundation. J Pediatr. 2017;181S:S4-S15.e1
  • Intro to cystic fibrosis. Cystic Fibrosis Foundation website. Available at: https://www.cff.org/intro-cf#overview-of-cf.

Contributors

  • Daniel A. Ostrovsky, MD
Last Updated:
2022-09-01

(C) Copyright 2022 EBSCO Information Services

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.