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Physical Impairment and Function in Children and Adolescents With Sickle Cell Disease: A Systematic Review
Victoria Marchese1, Kelly Rock1, Andria Harpold1
1Department of Physical Therapy and Rehabilitation Science, University of Maryland School of Medicine, Baltimore, MD.
Insights
Children and adolescents with sickle cell disease (SCD) experience significant physical impairments and functional limitations. These deficits impact muscle, bone, and overall physical performance, highlighting a critical need for targeted interventions.
Area of Science:
- Pediatric Hematology
- Rehabilitation Medicine
- Clinical Research
Background:
- Sickle cell disease (SCD) is a genetic blood disorder affecting red blood cells.
- Children and adolescents with SCD often face multifaceted health challenges beyond hematological aspects.
- Understanding physical impairments is crucial for comprehensive care and improving quality of life.
Purpose of the Study:
- To systematically review and synthesize evidence on physical impairments and physical function in pediatric SCD.
- To identify the range and severity of physical deficits in this population.
- To inform clinical practice and future research directions.
Main Methods:
- A comprehensive systematic literature search was conducted across multiple databases (PubMed, Embase, CINAHL, Cochrane, Dissertations & Theses).
- Inclusion criteria focused on SCD, physical impairments/function, and pediatric populations (1990-2020).
- Rigorous screening and data extraction processes were employed by independent reviewers.
Main Results:
- The review identified significant physical impairments and functional limitations in children and adolescents with SCD compared to controls.
- Deficits were observed across various domains including muscle/bone composition (19%-58%), muscle strength, cardiopulmonary function, and motor performance.
- Limitations also extended to physical activity levels and self-reported physical function in quality of life measures.
Conclusions:
- Children and adolescents with SCD exhibit notable physical impairments and functional limitations.
- There is a need for collaborative development of standardized methods to define and measure physical function in pediatric SCD.
- Further research is warranted to understand the underlying factors contributing to these physical deficits.
Objective:
To examine physical impairments and physical function in children and adolescents with sickle cell disease (SCD).
Data Sources:
PubMed, Embase (embase.com), Cumulative Index to Nursing and Allied Health (EBSCO), Cochrane Central Register of Controlled Trials (Wiley), and Dissertations and Theses (ProQuest) were searched from January 1, 1990, to September 25, 2020. References retrieved were required to include a term for SCD and a term for physical impairments or physical function. Results were limited to articles with children and adolescents and in the English language.
Study Selection:
A total of 3054 nonduplicate articles were independently screened by 2 reviewers, resulting in 240 articles for full-text review. The full-text review, performed by 2 independent reviewers, resulted in 67 articles.
Data Extraction:
Data were extracted from each full text to a custom Excel document by a single reviewer and were verified by a secondary reviewer.
Data Synthesis:
The studies identified in this systematic review offer evidence that children and adolescents with SCD demonstrate physical impairments and physical function limitations compared with control participants as noted by varying percentages in deficits up to 19%-58% in muscle and bone composition and/or symptoms, muscle strength, cardiopulmonary function, motor performance, physical activity, and physical function domains of quality of life questionnaires.
Conclusions:
Children and adolescents with SCD present with physical impairments and physical function limitations. Scientists and clinicians should consider developing collaborative standards to define and objectively measure physical impairment and function in this population to comprehensively examine the underlying factors that contribute to physical impairments and function.
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