Preschoolers With Cystic Fibrosis

Publication Date: June 1, 2020
Last Updated: March 3, 2023

Objective

Objective

This patient summary means to discuss key recommendations from the Cystic Fibrosis Foundation for preschoolers (age 2–5 years) with cystic fibrosis.

Overview

Overview

  • Cystic fibrosis is a progressive, inherited disease of the lungs, digestive tract, and other organs.
    • It is caused by a defective gene.
  • We will use the abbreviation CF throughout this summary to refer to cystic fibrosis.
  • Symptoms include problems breathing, coughing, frequent lung and sinus infections, foul stools, poor growth, pain, and fatigue.
  • This document includes recommendations in the following areas: routine surveillance for pulmonary disease, therapeutics, and nutritional and gastrointestinal care.

Diagnosis and Monitoring

Diagnosis and Monitoring

  • In the US, newborns are routinely screened for cystic fibrosis by blood tests.
    • At 2 weeks of age, a skin test for the salt content of sweat can be done.
    • Genetic testing is also available.
  • Given that CF often progresses in a clinically silent manner, objective and sensitive tools that detect and track early disease are important in this age group.
  • Monitoring
    • General health and growth
    • Oxygen levels
    • Spirometry (measuring effective breathing)
    • Airway surveying
      • Imaging every 2-3 years with x-ray or CT
      • Cultures for potential infections every 3 months
        • Antibiotics for infections

Treatment

Treatment

  • Treatment addresses several areas:
    • Caregiver engagement
      • Quarterly assessment by doctors and parents
    • General Health
      • Routine well-child care
      • Immunizations
      • Smoke-free environment
    • Airway maintenance
      • Clearing secretions
      • Inhaled hypertonic saline to loosen secretions
    • Medications
      • Dornase alfa to loosen secretions
      • Routine asthma inhalers are not favored.
      • Chronic use of cortisone is not favored.
    • Nutrition
      • Increased intake of energy, vitamins, and quality nutrition to maintain growth
        • Including tube feeding, if needed
      • Attention to and possible treatment of eating behavior disorders
      • Monitoring for gastrointestinal conditions more likely in CF patients
      • Added table salt, especially during hot weather
      • Pancreatic enzyme replacement if needed
  • Optimum care requires continuous attention to these multiple areas of concern by parents, caregivers, and health professionals from multiple disciplines.

Abbreviations

  • CT: Computed Tomography
  • US: United States

Source Citation

Thomas Lahiri, Sarah E. Hempstead, Cynthia Brady, Carolyn L. Cannon, Kelli Clark, Michelle E. Condren, Margaret F. Guill, R. Paul Guillerman, Christina G. Leone, Karen Maguiness, Lisa Monchil, Scott W. Powers, Margaret Rosenfeld, Sarah Jane Schwarzenberg, Connie L. Tompkins, Edith T. Zemanick, Stephanie D. Davis. Pediatrics Mar 2016, peds.2015-1784; DOI: 10.1542/peds.2015-1784
 

Disclaimer

The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.