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

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.

Macronutrient intake in preschoolers with cystic fibrosis and the relationship between macronutrients and growth

“…Children meeting the optimal nutritional threshold (≥50 th BMI percentile) were consuming an average of 94 kcal/kg per day, within the ≥90–110 kcal/kg per day recommendation to sustain growth [6]. (Table 1).…”

“…The results of this study combined with the Clinical Practice Guidelines [6] indicate that the macronutrients contributing to energy intake may be important in preschool children with CF, particularly in relationship to growth. With few trials conducted in preschool-aged children with CF these findings offer important information for future research and clinical practice, yet need to be interpreted within the context of the limitations.…”

“…Studies investigating macronutrient intake other than fat in children with CF are limited. Until the recent Clinical Practice Guidelines from the CF Foundation for preschoolers were published [6], dietary goals for protein intake received little attention particularly for preschool-aged children. The Clinical Practice Guidelines are the first ever published specifically for preschool-aged children with CF, and there is a specific recommendation for protein, that 2–3 year-olds consume at least 13 grams of protein per day and 4–5 year olds consume at least 19 grams of protein per day [1, 6] which are based on the recommendation for the general population.…”

Clinical and microbiological impact of inhaled tobramycin treatment on cystic fibrosis patients with Pseudomonas aeruginosa

“…This is to be expected, since alternate-monthly inhaled tobramycin is the recommended treatment for CF patients with PA infections [9], particularly mucoid PA infections. If a patient never had a PA infection, there would be less of a need for them to be on tobramycin.…”

Guía de Práctica Clínica para el manejo nutricional de personas con Fibrosis Quística (GPC-FQ)

“…Se deberá monitorizar el estado nutricional cada vez que se acuda a la consulta, realizando como mínimo una valoración nutricional completa al año 9,23,25,26 . Es esencial, al diagnóstico, evaluar la ingesta dietética y su grado de malabsorción.…”