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Published on: May 2, 2021
Functional outcomes of children with sickle-cell disease affected by stroke
L M Hariman1, E R Griffith, A L Hurtig
1Department of PM&R and Pediatrics, University of Illinois College of Medicine, Chicago 60612.
Insights
Children with sickle-cell disease (SCD) who experience strokes often recover physically but face significant cognitive and psychosocial challenges. These functional deficits, including intellectual and language impairments, outweigh physical independence following stroke events.
Area of Science:
- Pediatric Neurology
- Hematology
- Neuroscience
Background:
- Sickle-cell disease (SCD) is a genetic blood disorder associated with an increased risk of stroke in children.
- Functional recovery and long-term outcomes after stroke in pediatric SCD patients remain incompletely understood.
- Previous research has not extensively investigated the nature and degree of functional recovery post-stroke in this population.
Purpose of the Study:
- To retrospectively evaluate the functional status of children with SCD who have experienced strokes.
- To compare the functional outcomes of SCD children with stroke history against age- and gender-matched SCD children without stroke history.
- To quantify physical and cognitive functional outcomes and assess the impact of stroke on pediatric SCD patients.
Main Methods:
- Retrospective analysis of 14 children (5 boys, 9 girls) with SCD and stroke history (1-3 strokes).
- Participants' mean age was 11.6 ± 4.3 years; mean age at first stroke was 6.1 ± 5.2 years.
- Physical and psychosocial functions were assessed using standardized tests at least one year after the last stroke.
Main Results:
- All SCD-stroke children achieved physical independence, with few experiencing hand function impairments or mild self-care difficulties.
- The majority exhibited intellectual deficits (borderline to moderate mental retardation), reduced language function, and adjustment problems.
- Children with SCD and stroke showed significantly lower intelligence quotients compared to non-stroke SCD controls, indicating adverse cognitive effects.
Conclusions:
- In children with SCD, stroke primarily results in significant psychosocial and cognitive deficits rather than severe physical disability.
- Cognitive functioning, including intellectual and language abilities, is adversely affected by stroke in pediatric SCD patients.
- Psychosocial deficits appear to be more prominent than physical disabilities in the long-term outcomes of stroke survivors with SCD.
Abstract:
The nature and degree of functional recovery after stroke in children with sickle-cell disease (SCD) has not been extensively investigated. The purpose of this study was to evaluate retrospectively the functional status of 14 SCD children who had had strokes and to compare them with age-matched and gender-matched SCD children who had not had strokes. By doing so, we would be able to quantify the eventual physical and cognitive functional outcomes of survivors of stroke secondary to SCD and assess the impact of stroke on these patients. These children (five boys and nine girls) with SCD and stroke(s) were 11.6 +/- 4.3 years of age (range five to 18 years). They experienced one to three strokes at a mean age of 6.1 +/- 5.2 years (range one to 17 years). A series of tests were administered to these subjects to evaluate physical and psychosocial functions. These tests were performed at least one year after the latest stroke. This study showed that all of the SCD-stroke children were physically independent. Only a few had impairments of hand functions and mild difficulties in self-care activities. However, most of these children demonstrated intellectual deficits ranging from borderline to moderate mental retardation, reduced language functions ranging from low normal to retarded range, and problems in adjustment. Intelligence quotient of the children with SCD-stroke(s) was significantly lower than those of age-matched and gender-matched nonstroke SCD children, suggesting that stroke caused an adverse effect on the cognitive functioning of these children. The results indicate that in the SCD-stroke children psychosocial deficits outweighed physical disabilities.(ABSTRACT TRUNCATED AT 250 WORDS)
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