Registry-based study of gastrostomy tube placement and nutritional recovery in children with cystic fibrosis.

BACKGROUND

Although highly effective modulator therapy may reduce future gastrostomy tube (g-tube) placements, a better understanding of the benefits of g-tube placement is needed.

OBJECTIVES

To describe the characteristics of children with cystic fibrosis (CF) who underwent g-tube placement and determine if earlier placement is associated with improved clinical outcomes.

DESIGN

Retrospective cohort analysis of children with CF born 2003-2019 and followed in the US CF Foundation Patient Registry.

RESULTS

There were 2337 children with CF (22.9% of the study cohort) who underwent g-tube placement. Compared with children with CF who did not receive a g-tube, a higher proportion of g-tube recipients were diagnosed via meconium ileus or failure to thrive, born at <37 weeks' gestation or had only class I-III CF transmembrane conductance regulator mutations. G-tube recipients at age 0-1 years who were malnourished had improved body mass index (BMI) z-scores post-placement, though moderately to severely malnourished recipients did not achieve BMI z-scores >0. In contrast, only g-tube recipients at age ≥2 years who were severely malnourished achieved BMI z-scores >0. For g-tube recipients at age 0-1 years, per cent predicted of forced expiratory volume in 1 s (ppFEV1) at age 6-7 years was inversely correlated with malnutrition severity. In contrast, g-tube recipients at age ≥2 years had similar median ppFEV1 at age 6-7 years regardless of malnutrition severity, except those with severe malnutrition had slightly lower median ppFEV1.

CONCLUSIONS

Timing of g-tube placement in children with CF, both in relation to age and severity of malnutrition, was associated with varying degrees of improvements in BMI z-scores and ppFEV1.