Muscle Properties, Gross Motor Performance, and Quality of Life in Children With Sickle Cell Disease

Kelly Rock1, Simon Ho, Vicki L Gray

  • 1Departments of Physical Therapy and Rehabilitation Science (Drs Rock, Ho, Gray, Addison, and Marchese) and Pediatrics (Dr York), University of Maryland School of Medicine, Baltimore, Maryland; Department of Hematology/Oncology, University of Maryland Medical Center, Baltimore, Maryland (Dr York and Mss Keegan Wells and DeLuca).

Insights

Children with sickle cell disease (SCD) show weaker muscles and poorer motor skills. These factors are linked to reduced quality of life (QoL), highlighting the need for tailored physical therapy.

Area of Science:

  • Pediatric Health
  • Hematology
  • Rehabilitation Medicine

Background:

  • Sickle cell disease (SCD) is a genetic blood disorder affecting red blood cells.
  • Children with SCD often experience chronic pain and complications impacting physical function.
  • Limited research exists on the interplay between muscle properties, motor performance, and QoL in this population.

Purpose of the Study:

  • To investigate muscle characteristics, gross motor function, and quality of life (QoL) in children with SCD.
  • To compare these outcomes between children with and without SCD.
  • To examine the relationships between muscle properties, motor performance, and QoL.

Main Methods:

  • Cross-sectional study involving 24 children with SCD.
  • Muscle properties assessed via dynamometry (strength), ultrasonography (thickness), and electromyography (EMG) for neuromuscular activation (rate of activation [RoA]).
  • Gross motor performance and QoL evaluated using standardized tests and questionnaires.

Main Results:

  • Children with SCD exhibited significantly lower knee extension strength and VL EMG RoA compared to controls.
  • Impaired gross motor performance and QoL were observed in children with SCD.
  • Significant relationships were found among muscle properties, gross motor performance, and QoL.

Conclusions:

  • Children with SCD experience deficits in muscle strength, neuromuscular activation, motor skills, and QoL.
  • These findings underscore the importance of assessing muscle function and QoL in managing SCD.
  • Individualized physical therapy plans are recommended to address these multifaceted challenges.
Abstract