Delays in Cystic Fibrosis Care Tied to Persistent Impairment in Growth

Aug 11, 2023

THURSDAY, Aug. 10, 2023 (HealthDay News) — Delays in infant care for cystic fibrosis (CF) are associated with differences in growth parameters that persist through childhood, according to a study published online July 3 in The Journal of Pediatrics.

Stacey L. Martiniano, M.D., from the University of Colorado Anschutz Medical Center in Aurora, and colleagues examined whether age at initial CF care influences outcomes using data from participants in the U.S. CF Foundation patient registry, born between 2010 and 2018. Infants were classified according to age at first event (AFE); those in the youngest and oldest AFE cohorts (less than 14 days; median AFE, 10 days [early cohort; 551 infants] and 33 days or later; median AFE, 47 days [late cohort; 551 infants]) were matched by sex, race, ethnicity, and CF transmembrane conductance regulator (CFTR) variant class. The AFE proxy was age at sweat test, CF Care Center encounter, or hospitalization, with more than one event possible on the same day.

The researchers found that hospitalizations were rare but were more common in the early cohort. Hospitalizations in the late cohort were most often due to pulmonary exacerbations (59.1 versus 4.8 percent). Hospitalizations as the first CF event tended to be longer for the late cohort (12 versus six days). Infants in the late cohort were more likely to have only one CFTR variant detected on the two most common commercial variant panels. There was no difference between the cohorts in the median birth weight z-score or median birth length z-score. However, median weight-for-age z-score was significantly lower for the late versus the early cohort by time of first encounter and remained impaired through 1 year. Through 1, 3, and 5 years, the late cohort remained at a significantly reduced height. A reduced rate of breastfeeding was seen in the late versus early cohort.

“Public health, primary care, and cystic fibrosis center professionals must come together to improve timeliness and efficiency so that all babies with a positive newborns screening tests are seen promptly,” senior author Susanna McColley, M.D., of the Ann & Robert H. Lurie Children’s Hospital of Chicago, said in a statement.

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