Early fortified breastfeeding may aid growth in infants with cystic fibrosis
Researchers say preventive strategy can minimize risk of poor development
Most infants with cystic fibrosis (CF) must be fed fortified milk, that is, breast milk, formula, or a combination of both, within the first six months of life to achieve normal growth by age 3, a study suggests.
This lets babies receive all the benefits from breast milk, plus additional nutrients that will contribute to their normal growth. Children breastfed until 6 months of age, either fortified or not, had less severe lung disease than those who started fortified formula early in life.
“For these infants, the proactive/preventive strategy of fortified breast milk feedings starting soon after CF diagnosis … can minimize the risk of prolonged postnatal growth faltering, accelerate the potential of attaining catch-up growth, and decrease the likelihood of experiencing more severe early-onset lung disease,” the researchers wrote in the study, “Breastfeeding, growth, and lung disease in the first 3 years of life in children with cystic fibrosis,” which was published in the Journal of Cystic Fibrosis.
CF is caused by mutations in the CFTR gene and leads to no or faulty production of the CFTR protein that normally regulates the flow of chloride through the cell membrane. This results in thick, sticky mucus accumulating in several organs, particularly the lungs and digestive system.
The disease features a range of symptoms, including respiratory problems like shortness of breath and frequent lung infections, and digestive issues that may include food malabsorption and poor growth. Optimizing nutrition is critical to ensure normal growth and improve lung function in children with CF.
The 2009 CF Foundation infant care guidelines recommend breast milk as the initial feeding for infants, but scientific evidence is limited, and the guidelines don’t specify if breastfeeding should be supplemented with formula.
Affect of breastfeeding on growth in CF
Here, researchers analyzed data on breastfeeding and its impact on CF children’s growth and lung function using a comprehensive CF early onset lung disease (CFELD) scoring system. The score is defined by systematically recording CF-related respiratory symptoms, Pseudomonas aeruginosa infections, pulmonary exacerbations, and hospitalizations, and varies from asymptomatic to severe. The study enrolled 172 CF children from six centers in the U.S. who were born between 2012 and 2017, and diagnosed with CF through newborn screening.
Most infants had pancreatic insufficiency (86%), a common complication of CF wherein the enzymes that help break down food don’t reach the digestive tract due to mucus accumulation.
At birth, 72% of the children were breastfed. By 3 months, only 20% continued with unfortified breastmilk. At 6 months, 64% of the children were receiving fortified feedings, with a mean caloric density of 25.1 kcal/oz., to help them grow.
Fortified breastmilk and fortified formula were equally effective at improving growth, with weight-for-age z-score catching up with normal values at 12 months, and length/height-for-age z-score by age 3. Z-scores reflect the difference between a child’s weight or height and reference values in healthy children, adjusted for age and sex.
“The most important finding … is that infants in all three fortified groups experienced marked growth improvements from early infancy to 3 years of age,” the researchers wrote.
Lung disease and breastfeeding
As for early-onset lung disease up to age 3, children who were fed with unfortified breast milk in the first six months saw fewer P. aeruginosa infections and pulmonary exacerbations than those who started formula within the first three months of life or later.
Up to 3 years of age, they were more likely to have milder lung disease than those who started fortified feeding at 2 months (30% vs. 76% in moderate/severe categories).
Within the unfortified group, there were no significant differences regarding P. aeruginosa infections and pulmonary exacerbations between children fed predominantly with breast milk, with formula, or a combination. However, the CFELD score was milder among those only predominantly breastfed.
As for the fortified group, results showed no differences comparing rates of lung infections and exacerbations. Fewer infants fed with fortified breast milk had severe or moderate CFELD than those fed formula or combination (30% vs. 53% vs. 62%), however.
Other factors may influence growth and pulmonary outcomes within the first three years of life with CF. Helpful factors included pancreatic sufficiency, higher growth scores at birth, treatment with Kalydeco (ivacaftor), parents with some college education, no exposure to cigarette smoke, and shorter daycare attendance.
“This timely information underscores the implications and challenges associated with predicting the early outcomes of infant nutrition in CF and provided the evidence base necessary for updating the clinical practice guidelines on nutritional management,” wrote the researchers, who said one limitation of the study was that Trikafta wasn’t indicated for this age group when it was conducted, which means its potential effects on the links between breastfeeding, growth, and pulmonary outcomes couldn’t be explored.
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