Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With Cystic Fibrosis

Author:

Lahiri Thomas1,Hempstead Sarah E.23,Brady Cynthia4,Cannon Carolyn L.5,Clark Kelli6,Condren Michelle E.7,Guill Margaret F.38,Guillerman R. Paul9,Leone Christina G.10,Maguiness Karen11,Monchil Lisa12,Powers Scott W.13,Rosenfeld Margaret14,Schwarzenberg Sarah Jane15,Tompkins Connie L.16,Zemanick Edith T.17,Davis Stephanie D.11

Affiliation:

1. Pediatric Pulmonology, University of Vermont Children’s Hospital and Department of Pediatrics, University of Vermont College of Medicine, Burlington, Vermont;

2. The Dartmouth Institute for Health Policy and Clinical Practice, Lebanon, New Hampshire;

3. Geisel School of Medicine at Dartmouth, Lebanon, New Hampshire;

4. Children’s Respiratory and Critical Care Specialists and Children’s Hospitals and Clinics of Minnesota, Minneapolis, Minnesota;

5. Texas A&M Health Science Center, College Station, Texas;

6. Department of Pediatrics, University of North Carolina, Charlotte, North Carolina;

7. University of Oklahoma College of Pharmacy and School of Community Medicine, Tulsa, Oklahoma;

8. Allergy and Pediatric Pulmonology, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire;

9. Department of Radiology, Baylor College of Medicine and Department of Pediatric Radiology, Texas Children’s Hospital, Houston, Texas;

10. Cystic Fibrosis Center, Children’s Hospital Colorado, Aurora, Colorado;

11. Section of Pediatric Pulmonology, Allergy and Sleep Medicine, Department of Pediatrics, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, Indiana ;

12. Armond V. Mascia, MD Cystic Fibrosis Center, Maria Fareri Children’s Hospital at Westchester Medical Center, Valhalla, New York;

13. Department of Pediatrics and Cincinnati Children’s Research Foundation, University of Cincinnati College of Medicine and Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children’s Hospital, Cincinnati, Ohio;

14. Division of Pulmonary Medicine, Seattle Children’s Hospital and Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington;

15. Pediatric Gastroenterology, Hepatology and Nutrition, University of Minnesota Masonic Children’s Hospital, Minneapolis, Minnesota;

16. Department of Rehabilitation and Movement Sciences, University of Vermont College of Nursing and Health Sciences, Burlington, Vermont; and

17. Department of Pediatrics, University of Colorado School of Medicine, Aurora, Colorado

Abstract

Cystic fibrosis (CF) clinical care guidelines exist for the care of infants up to age 2 years and for individuals ≥6 years of age. An important gap exists for preschool children between the ages of 2 and 5 years. This period marks a time of growth and development that is critical to achieve optimal nutritional status and maintain lung health. Given that disease often progresses in a clinically silent manner, objective and sensitive tools that detect and track early disease are important in this age group. Several challenges exist that may impede the delivery of care for these children, including adherence to therapies. A multidisciplinary committee was convened by the CF Foundation to develop comprehensive evidence-based and consensus recommendations for the care of preschool children, ages 2 to 5 years, with CF. This document includes recommendations in the following areas: routine surveillance for pulmonary disease, therapeutics, and nutritional and gastrointestinal care.

Publisher

American Academy of Pediatrics (AAP)

Subject

Pediatrics, Perinatology and Child Health

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