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Updated: Mar 31, 2026
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Health-related Quality of Life in Infants With Sickle Cell Disease
Lauren M Beverung1, Christina Bemrich-Stolz, Sylvia Torres
1*Pediatrics †Divisions of Hematology/Oncology/Bone Marrow Transplant §Community Pediatrics, Medical College of Wisconsin/Children's Hospital of Wisconsin Milwaukee, WI ‡Division of Hematology/Oncology, University of Alabama at Birmingham/Children's Hospital of Alabama, Birmingham, AL.
Insights
Infants with sickle cell disease (SCD) experience impaired health-related quality of life (HRQL). This decline is evident by 13-24 months, impacting physical and psychosocial well-being compared to healthy peers.
Area of Science:
- Pediatrics
- Hematology
- Quality of Life Research
Background:
- Sickle cell disease (SCD) is a genetic blood disorder affecting red blood cells.
- Health-related quality of life (HRQL) is a crucial measure of well-being in pediatric populations.
- Understanding HRQL in infants with SCD is vital for comprehensive care.
Purpose of the Study:
- To assess and compare the health-related quality of life (HRQL) of infants with sickle cell disease (SCD) against historical cohorts.
- To identify age-specific differences in HRQL for infants with SCD.
Main Methods:
- A cross-sectional study involving 90 infants with SCD across two major medical centers.
- Parents completed the PedsQL Infant Module for infants aged 1-24 months.
- Comparison with historical cohorts of healthy, acutely ill, and chronically ill infants.
Main Results:
- Infants with SCD showed lower Physical HRQL than healthy infants at 1-12 months.
- By 13-24 months, infants with SCD had worse HRQL across all domains compared to healthy infants.
- A significant increase in impaired Physical and Total HRQL was observed in infants with SCD from 1-12 months to 13-24 months.
Conclusions:
- Infants with sickle cell disease (SCD) exhibit impaired health-related quality of life (HRQL) that worsens with age.
- HRQL deficits in infants with SCD are detectable and significant, necessitating targeted interventions.
- Findings highlight the importance of monitoring and supporting HRQL in early childhood for infants with SCD.
Abstract:
Using historical cohorts of healthy, acutely ill, and chronically ill infants for comparison, we sought to determine whether infants with sickle cell disease (SCD) have impaired health-related quality of life (HRQL). We conducted a cross-sectional study at 2 sites: the Medical College of Wisconsin/Children's of Wisconsin and the University of Alabama School of Medicine/Children's of Alabama. Parents of 90 infants with SCD completed the PedsQL Infant Module corresponding to their infant's age (1 to 12 mo or 13 to 24 mo) during a regular clinic visit. At 1 to 12 months, infants with SCD displayed lower Physical HRQL than healthy infants, but better HRQL than chronically ill infants. By 13 to 24 months, infants with SCD had worse HRQL in all areas than healthy infants and worse Physical and Total HRQL than acutely ill infants. Compared with chronically ill infants in this age group, infants with SCD had similar Physical HRQL and better Psychosocial and Total HRQL. By 13 to 24 months, a greater proportion of infants with SCD had impaired Physical and Total HRQL compared with infants aged 1 to 12 months. All differences were significant at the (P<0.05) level. Impaired HRQL can be detected in infants with SCD.
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