Physical Impairment and Function in Children and Adolescents With Sickle Cell Disease: A Systematic Review

Published:September 27, 2021DOI:



      To examine physical impairments and physical function in children and adolescents with sickle cell disease (SCD).

      Data Sources

      PubMed, Embase (, 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.


      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.


      List of abbreviations:

      BOT (Bruininks-Oseretsky Test of Motor Proficiency), CHQ (Child Health Questionnaire), FDI (Functional Disability Inventory), IPAQ (International Physical Activity Questionnaire), NHANES (National Health and Nutrition Examination Survey), PROMIS (Patient-Reported Outcome Measurement Information System), SCD (sickle cell disease)
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