Overweight and cystic fibrosis: An unexpected challenge

Abstract

Achieving a healthy weight balance has been a central focus of care for people who have cystic fibrosis (CF). Over the years, the emphasis has primarily been on promoting weight gain to optimize pulmonary outcomes. With continued improvements in CF care, including highly effective CF modulators available for many people, the CF community is now experiencing a new challenge: addressing the concern that some people are gaining weight excessively. While at this time, we do not know to what extent overweight and obesity will affect health outcomes for people with CF, it is likely that excessive weight gain may have negative health impacts similar to those seen in the general population. In this paper, we review the history of nutritional guidelines for people with CF, as well as more recent trends toward overweight and obesity for some. A multidisciplinary approach is needed to collaboratively start the oftentimes difficult conversation regarding excessive weight gain, and to identify resources to help people achieve and maintain a healthy weight through diet, exercise, and behavioral modification.

Elexacaftor/Tezacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for the F508del Mutation and Advanced Lung Disease: A 48-Week Observational Study

“…A similar increase in BMI after using CFTR modulators in severely affected pwCF was described in patients with the F/MF genotype [22] and in patients with mutations determining CFTR residual function [23]. Although over the last decades an emphasis has been on promoting BMI gain to optimise pulmonary outcomes, the risk of overweightness/obesity in an adult population should be monitored in the long-term [24][25][26].…”

Effects of Exercise on Nutritional Status in People with Cystic Fibrosis: A Systematic Review

“…We were surprised at the lack of high-quality RCTs that included nutritional status as an outcome of exercise interventions. Future research should not only include longer-term outcomes of exercise training on body composition in patients with CF, but should include adults as well as differentially look at the impact of exercise on patients that are underweight, normal weight and overweight, and should include alternatives to BMI and body mass in assessing nutritional status (e.g., dual-energy X-ray absorptiometry, skinfold thickness, bioelectrical impedance, and peripheral quantitative computed tomography) [ 12 , 33 ].…”

“…Most people think of exercise as a way to maintain or lose weight; thus, some people with CF that are underweight or at their goal weight may be reluctant to begin an exercise program. On the contrary, with the advent of highly effective modulator therapies, some patients are now concerned about gaining too much weight [ 12 , 13 ].…”

“…This question has clinical relevance due to the morbidity and mortality implications of malnutrition in this patient population and the perceived risk of weight loss in a population that has historically been underweight. In this new era of highly effective modulator therapies, there is now the potential risk for both normal weight obesity (increased fat mass with an otherwise normal body mass) as well as outright overweight and obese, especially as more people begin these drugs at a younger age and are on them for longer periods of time [ 12 , 13 ].…”

Eating Disorders and Disordered Eating Behaviors in Cystic Fibrosis: A Neglected Issue

“…Attaining an adequate body weight is important for achieving improved respiratory health and low infection risk [ 1 ]. However, struggles with weight status are common in CF, with patients of younger ages often failing to maintain and gain body weight [ 4 ] and older individuals facing overweight and obesity more frequently [ 5 , 6 ].…”