Peripheral muscle function and body composition in people with cystic
fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: a cross-sectional,
observational, single-centre study
Abstract
Background: People with cystic fibrosis (pwCF) often have
multifactorial peripheral muscle abnormalities attributed to, for
example, malnutrition, steroid use, altered redox balance and,
potentially, CF-specific intrinsic alterations. Malnutrition in CF now
includes an increasing prevalence of overweight and obesity,
particularly in those treated with CF transmembrane conductance
regulator (CFTR) modulators. We aimed to characterise peripheral muscle
function and body composition of pwCF on
Elexacaftor/Tezacaftor/Ivacaftor (ETI) CFTR modulator treatment,
compared to healthy controls. Methods: Fifteen pwCF on ETI, and
15 healthy age- and sex-matched controls (CON), underwent whole-body
dual-energy X-ray absorptiometry scans, and a comprehensive evaluation
of peripheral muscle function. These tests included quadriceps maximal
isometric force measurement, an intermittent isometric quadriceps
fatiguing protocol, handgrip strength dynamometry, squat jump height
assessment, and a 1-minute sit-to-stand test. Results: No
differences in quadriceps maximal isometric force (CON: 181.60 ± 92.90
Nm vs. CF: 146.15 ± 52.48 Nm, P = 0.21, d = 0.47),
handgrip strength (CON: 34 ± 15 kg vs. CF: 31 ± 11 kg, P = 0.62,
d = 0.18), peripheral muscle endurance, fatigue, or power were
observed between the groups ( P>0.05). Moreover, no
differences in whole-body, trunk or limb lean mass, fat-free mass, fat
mass, or whole-body bone mineral density were evident (
P>0.05). Conclusion: Comparable peripheral
muscle mass and function may be achievable in pwCF on ETI, especially
those with good lung function. Research is needed to confirm these
improvements in pwCF who have more severe lung disease, are less
physically active, and have less optimal nutrition and exercise support.