CFTR modulators drive shift to tailored child nutrition plans
Study findings based on surveys completed by parents/caregivers of CF children
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CFTR modulators for cystic fibrosis (CF) can change a child’s weight and nutrition needs, so each child should receive an individualized nutrition plan based on how they are growing, what they prefer to eat, and how they are responding to treatment, a survey study found.
The study, “Parents’/Caregivers’ perspectives on weight management in children with cystic fibrosis receiving modulator therapy: A cross-sectional survey,” was published in Nutrition in Clinical Practice by researchers in the U.S. who interviewed 49 parents and caregivers of children with CF.
Appropriate weight gain seen in most children in study
CF causes thick, sticky mucus to build up in the lungs and other organs in the body, including those of the digestive tract. CFTR modulators are medications that increase the functionality of the CFTR protein, whose deficiency or defects causes CF. These treatments are known to ease lung and digestive symptoms, and they may also help children with CF gain weight more easily.
Because of these changes, the usual approach to nutrition may need to change. This study looked at how parents and caregivers viewed their child’s weight and nutrition after starting treatment with a CFTR modulator. The researchers used a survey to ask about diet, communication with the care team, and concerns about eating behaviors.
The parents and caregivers who answered the survey represented a total of 60 children with CF. The children had a mean age of 7.9 years, and they visited the clinic two to 10 times each year. Most children (88.3%) were on pancreatic enzyme replacement treatment, which helps the body digest food when the pancreas does not release enough digestive enzymes due to sticky mucus.
All 60 children were receiving treatment with a CFTR modulator, most commonly Trikafta (elexacaftor/tezacaftor/ivacaftor). Of the 46 children on a CFTR modulator for more than a year, about two-thirds (65.2%) were reported to have gained an appropriate amount of weight. However, nine (19.5%) gained too little, four (8.6%) did not gain weight, two (4.3%) gained too much, and one (2.2%) lost weight.
We no longer have to monitor everything she eats. She no longer drinks weight gainer shakes and is at a healthy weight.
Most parents and caregivers agreed with the doctor’s classification of their child’s weight (86%), as well as about the child’s overall weight trajectory (75%). A weight trajectory refers to how a child’s weight changes over time rather than looking at one single measurement. While parents generally agreed with doctors, some differences in how weight was viewed were reported.
The way families managed nutrition also changed for some children after starting treatment. Of the 46 children on a CFTR modulator for more than a year, about half (52%) had some type of change to their diet. Some families reduced high-calorie foods, calorie-dense supplements, or supplemental feeding because their child’s weight had improved.
Nearly half (46%) of those children had followed a high-fat diet during the previous six months. These findings are important because nutrition care has traditionally focused on helping children with CF gain enough weight. With CFTR modulators, some children may no longer need the same high-calorie nutrition plan.
“We no longer have to monitor everything she eats. She no longer drinks weight gainer shakes and is at a healthy weight,” one participant said about their child.
At the same time, about one-third (35%) of children on a CFTR modulator for more than a year were reported by their parents to have less-than-ideal weight. This supports the need to tailor nutrition advice to each child rather than using the same strategy for everyone, the scientists suggested.
Some families ‘struggled with the topic of weight’
However, some parents and caregivers reported behaviors that could be considered disordered eating in about one-third (35%) of the children. These included skipping or changing how they used pancreatic enzymes, limiting the types of foods they ate, exercising excessively, focusing heavily on body image or weight, skipping meals, having strict food rituals, frequently weighing themselves, and binge eating.
Talking about weight was generally acceptable to families, although some “struggled with the topic of weight,” the researchers wrote. Most parents and caregivers (91%) discussed their child’s weight with the clinic at every visit.
“It’s probably the biggest challenge we have faced in CF care, and I definitely have some trauma from it,” one participant said.
Parents and caregivers generally agreed with doctors about their child’s weight and were willing to discuss nutrition, but concerns about weight and eating behaviors should be considered carefully. Some children may need fewer extra calories or supplements after starting treatment, while others may still need help gaining weight, the researchers noted.
Study limitations noted by the authors included the relatively small group of participants and the fact that 80% of children with CF were from Western U.S. states.
“Therefore, the results may not represent global perspectives of parents/caregivers of [children with CF] because of the variability in practices in CF centers and cultural views of weight.”
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