Cystic fibrosis guidelines for preschool children

REVIEWED BY THOMAS LAHIRI, MD.

Tracking early disease in young children with cystic fibrosis (CF) is critical to ensure these children achieve optimal nutritional status needed to maintain lung health. Clinical care guidelines on monitoring cystic fibrosis in infants aged up to 2 years and for children aged 6 years and older have been available for years,^1-3 and have been synthesized for pediatricians in “Cystic fibrosis: An essential update,” published in October 2014 in Contemporary Pediatrics.⁴

Since this article, a new guideline has been published that addresses what has been a gap in care: monitoring cystic fibrosis in children aged between 2 and 5 years. Published in April 2016 in the journal Pediatrics, “Clinical practice guidelines from the Cystic Fibrosis Foundation for preschoolers with cystic fibrosis” highlights the key issues for pediatricians and other healthcare providers in caring for children with cystic fibrosis during this critical stage of development.⁵

“Intervention at this age can have significant consequences on long-term morbidity and mortality,” says the lead author of the guidelines, Thomas Lahiri, MD, Pediatric Pulmonology, University of Vermont Children’s Hospital, and Department of Pediatrics, University of Vermont College of Medicine, Burlington. “These guidelines will hopefully provide a framework for practitioners to institute best practices for prevention, monitoring, and treatment of pulmonary and gastrointestinal disease in CF.”

Related: An essential update for cystic for fibrosis

Saying that pediatricians are an important ally to the multidisciplinary CF care team, Lahiri emphasizes that pediatricians play a vital role in helping to ensure the child receives early treatment of symptoms and is monitored for growth, as well as to reinforce the use of chronic therapies. A fundamental role for the general pediatrician, he says, is to ensure children with CF receive appropriate well-child care that includes adhering to the full immunization schedules.

This article provides a brief overview of the 2016 guidelines, highlighting key issues underscored by Lahiri regarding monitoring preschool-aged children with CF. Pediatricians are encouraged to review the full list of recommendations in the guidelines, which can be accessed at bit.ly/cf-2016-guidelines.

Monitoring and nutritional care

Of paramount importance in monitoring and caring for children with cystic fibrosis aged between 2 and 5 years is the need for nutrition support, says Lahiri. “Achieving normal growth during this important time of development will have positive impacts on pulmonary and overall outcomes,” he says, adding that pancreatic insufficiency can also develop during this period and, if left untreated, can have dramatic negative effects on growth.

Table 1 lists general issues for routine monitoring and nutritional care in children aged 2 to 5 years.⁵

In addition, Lahiri emphasizes the need to identify and address common behavioral problems in preschoolers that may adversely affect eating. Table 2 lists these general issues to consider.⁵

Pulmonary screening

Because lung disease in children with CF can silently progress with no obvious clinical signs or symptoms, Lahiri points out the critical need for pulmonary screening. Table 3 lists the consensus recommendations on types and frequency of screening methods.

Pulmonary therapies

The guidelines also provide guidance on a number of effective therapies that may benefit preschool-aged children with CF in whom abnormalities have been detected. Saying that a normal “cold” may evolve into a significant pulmonary infection in children with CF, Lahiri emphasizes the need to identify symptoms early and use increased airway clearance and antibiotics to prevent more serious exacerbations. Table 4 lists the types of therapies recommended.

Summary

The 2016 guidelines provide pediatricians and other healthcare providers with the needed guidance on monitoring and caring for preschool-aged children with CF. Highlighted in the guidelines is the paramount need for nutritional support and monitoring to ensure the developmental and growth needs of these children are met, which is critical for pulmonary function. Also critical is the need for pulmonary screening to identify complications early and treat as necessary. Primary care pediatricians play an important role in the overall multidisciplinary approach to caring for these children.

REFERENCES

1. Cystic Fibrosis Foundation; Borowitz D, Robinson KA, Rosenfeld M, et al. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155(6 suppl):S73-S93.

2. Mogayzel PJ Jr, Naureckas ET, Robinson, KA, et al; Pulmonary Clinical Practice Guidelines Committee. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013;187(7):680-689.

3. Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H; Clinical Practice Guidelines on Growth and Nutrition Subcommittee; Ad Hoc Working Group. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008;108(5):832-839.

4. Cystic fibrosis: An essential update. Contemp Pediatr. 2014;31(10:21-24. Available at: http://contemporarypediatrics.modernmedicine.com/contemporary-pediatrics/news/cystic-fibrosis-essential-update. Accessed February 23, 2017.

5. Lahiri T, Hempstead SE, Brady C, et al. Clinical practice guidelines from the Cystic Fibrosis Foundation for preschoolers with cystic fibrosis. Pediatrics. 2016;137(4):e20151784. Available at: http://pediatrics.aappublications.org/content/pediatrics/early/2016/03/22/peds.2015-1784.full.pdf. Accessed February 23, 2017.

Ms Nierengarten, a medical writer in Minneapolis, Minnesota, has over 25 years of medical writing experience, authoring articles for a number of online and print publications, including various Lancet supplements, and Medscape. She has nothing to disclose in regard to affiliations with or financial interests in any organizations that may have an interest in any part of this article.