VOC Frequency Negatively Associated With HRQOL in Children, Caregivers Living With SCD

The frequency of vaso-occlusive crises (VOCs) in children with sickle cell disease (SCD) has a negative effect on both the child and their caregiver, primarily in regard to health-related quality of life (HRQOL), according to a study published in Patient Related Outcome Measures.

SCD causes the deformity of red blood cells in patients with the disease, leading to hemolysis, anemia, and vaso-occlusion. HRQOL is heavily impaired in children with SCD due to the many symptoms and complications of the disease, not limited to VOCs. This study aimed to quantify VOC burden in children with SCD aged 12 years and younger, assess the association between the frequency of VOCs and HRQOL in children, and also to describe the burden of SCD in both children and their caregivers.

Clumps of sickle cells block the blood vessel | Image credit: Dr_Microbe - stock.adobe.com

A survey was conducted in the United States from February to April of 2021 using a cross-sectional design. Caregivers of children with SCD aged from 2 months to 11 years completed the survey, and their data were used for this study. Caregivers were required to be aged 18 years and older, living in the United States, and the primary caregiver of a child with SCD; caregivers of children who had sickle cell trait and those who did not know their child’s sickle cell type were not included. All caregivers reported on a single child and only 1 caregiver per household was permitted.

The researchers used the Infant-Toddler Quality of Life-Short Form 47 (ITQoL-SF47) to measure HRQOL in children aged 2 months to 4 years. This survey included 9 scales to judge the well-being of the child and 3 scales for the caregiver, with a scale range of 0-100 where 100 indicated the best HRQOL. Children aged 5 to 11 years used the Child Health Questionnaire-Parent Form 50 (CHQ-PF50) to assess HRQOL, with 11 scales for children and 4 for caregivers. Caregivers provided the quantity of VOCs that were experienced.

There were 167 caregivers included in this study who had a mean age of 34 years and the majority of whom were female (77.84%) and Black (92.81%). The children included had a mean age of 5.4 years, 49% were female patients, and 94.61% were Black. Children experienced 5.11 VOCs within the year on average, with 71.26% experiencing at least 3.

Children with SCD who were aged 2 months to 4 years had lower scores in all domains of HRQOL compared with the controls, with the average score of general health perceptions for children with SCD being 45 points lower compared with the average child in the United States (33.33 vs 78.36). Children aged 5 to 11 years also experienced lower scores in all domains but behavior and the mean score for general health perceptions was 36 points lower in the children with SCD compared with controls (36.82 vs 73.19). Pain interference (55.40 vs 50.00) and sleep disturbances (57.13 vs 50.00) was higher in children with SCD.

Children aged 2 months to 4 years who had 3 or more VOCs in the previous year had worse HRQOL compared with children who had 0 to 2, with overall health (14.87 vs 55.75) and bodily pain/discomfort (47.80 vs 80.30) having at least 30 point differences in the scale. This result held true in children aged 5 to 11 years, with global health (21.74 vs 56.42), physical functioning (56.04 vs 87.40), role/social limitations (51.21 vs 89.01), and bodily pain (49.80 vs 88.79) having the highest difference between children who had more frequent VOCs compared with those with less frequent VOCs.

Caregivers of children aged 2 months to 4 years with SCD reported lower scores on the HRQOL compared with the controls, with emotion domain (53.78 vs 89.97) and parent impact-time domain (40.92 vs 92.59) having significantly different scores. The same results were found in caregivers of children aged 5 to 11 years in regards to the emotion domain (54.35 vs 81.42) and the parent impact-time domain (63.77 vs 88.67) being lower in parents with children who had SCD compared with controls.

There were some limitations to this study. No causal or longitudinal relationships can be inferred due to the observational and cross-sectional nature of the study. Variables that are uncontrolled or not able to be measured could have contributed to the relationships reported. The observed associations could also change should those variables be included. Hybrid or distance learning could have reduced the amount of absences in school. HRQOL could be difficult to evaluate in an infant through the report of the caregiver.

The authors concluded that future research into the burden on children with SCD and their caregivers should “focus on examining how treatment, and possibly non-pharmacologic interventions, may reduce this burden, focusing on the child and on the entire family unit.”

Reference

Campbell A, Rizio AA, McCausland KL, et al. The burden of sickle cell disease on children and their caregivers: caregiver reports of children’s health-related quality of life and school experiences, caregiver burden, and their association with frequency of vaso-occlusive crises. Patient Relat Outcome Meas. 2023;14:369-381. doi:10.2147/PROM.S419607