Pain Interference and Fatigue in Limb-Girdle Muscular Dystrophy R9

Anna M. Reelfs; Carrie M. Stephan; Shelley R.H. Mockler; Katie M. Laubscher; M. Bridget Zimmerman; Katherine D. Mathews

doi:10.1016/j.nmd.2023.05.005

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Pain Interference and Fatigue in Limb-Girdle Muscular Dystrophy R9

Journal article

Open access

Peer reviewed

Pain Interference and Fatigue in Limb-Girdle Muscular Dystrophy R9

Anna M. Reelfs, Carrie M. Stephan, Shelley R.H. Mockler, Katie M. Laubscher, M. Bridget Zimmerman and Katherine D. Mathews

Neuromuscular disorders : NMD, Vol.33(6), pp.523-530

05/2023

DOI: 10.1016/j.nmd.2023.05.005

PMCID: PMC10527028

PMID: 37247532

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https://pmc.ncbi.nlm.nih.gov/articles/PMC10527028/pdf/nihms-1906392.pdfView

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Abstract

•Individuals with LGMDR9 experience pain interference levels similar to the general population.•Pain interference and fatigue levels often fluctuate in individuals with LGMDR9.•Pain interference and fatigue levels increase with age in individuals with LGMDR9.•Fatigue is elevated in adults with LGMDR9 and correlates with declining motor function.•Fatigue may be a useful outcome measure for future studies of LGMDR9. Pain is prevalent in individuals with limb-girdle muscular dystrophy (LGMD) R9, but impact on daily living and correlation with fatigue remain unknown. Patient-Reported Outcomes Measurement Information System (PROMIS) pain interference and fatigue short forms were completed annually by 23 children and 54 adults with biallelic fukutin-related protein (FKRP) variants for up to six years. Concurrent motor and pulmonary function were evaluated. Pain interference T-scores were near the normal mean of 50 by linear mixed model analysis (48.5 in children, 51.6 in adults). 58% of participants experienced pain interference levels greater than the general population on at least one assessment. Fatigue T-scores were elevated in adults but not children (49.0 in children, 55.1 in adults), and 75% had at least one elevated fatigue score. Of participants with at least two visits, serial scores were not consistent across visits, without a clear pattern. Pain interference and fatigue were positively correlated (r=0.55). Both increased with older age (r=0.21 and 0.41 respectively). Neither differed by sex or ambulation status. Motor (r=-0.32) and pulmonary (r=-0.25) function correlated with fatigue in adults, not children. Results suggest that pain in those with LGMDR9 is variable and episodic, limiting impact on daily life, while fatigue increases over time.

10-meter walk/run

6-minute walk test

FKRP

motor function

PROMIS short form

pulmonary function

Details

Title: Subtitle: Pain Interference and Fatigue in Limb-Girdle Muscular Dystrophy R9
Creators: Anna M. Reelfs - University of Iowa Carver College of Medicine, 375 Newton Rd, Iowa City, IA 52242, USA
Carrie M. Stephan - University of Iowa
Shelley R.H. Mockler - Center for Disabilities and Development, University of Iowa Stead Family Children's Hospital, 100 Hawkins Drive, Iowa City, IA 52242, USA
Katie M. Laubscher - University of Iowa
M. Bridget Zimmerman - Department of Biostatistics, College of Public Health, University of Iowa, 145 N. Riverside Drive, Iowa City, IA 52242, USA
Katherine D. Mathews - Department of Pediatrics, University of Iowa Carver College of Medicine, 200 Hawkins Drive Iowa City, IA 52242, USA
Resource Type: Journal article
Publication Details: Neuromuscular disorders : NMD, Vol.33(6), pp.523-530
DOI: 10.1016/j.nmd.2023.05.005
PMID: 37247532
PMCID: PMC10527028
NLM abbreviation: Neuromuscul Disord
ISSN: 0960-8966
eISSN: 1873-2364
Publisher: Elsevier B.V
Language: English
Date published: 05/2023
Academic Unit: Neurology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Biostatistics; Physical Therapy and Rehabilitation Science; Neurology (Pediatrics)
Record Identifier: 9984419355302771

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