Sickle Cell Disease-Related Worry and Associations with Healthcare Utilization in Youth with Sickle Cell Disease
Authors: Pizzo Alex , Kang Guolian , Okhomina Victoria , Porter Jerlym , Hodges Jason , Rai Parul , Takemoto Clifford M. , Hankins Jane S , Alberts Nicole M. 
Published: December 2024

Background and Aims: Disease-specific worries and anxiety (e.g., concerns of medical or treatment complications and disease progression) are common among youth with a chronic illness (e.g., cancer, rheumatoid arthritis). Moreover, they are associated with a host of negative outcomes, including pain, psychological distress, and increased healthcare utilization. Despite the impact of disease-specific worry among youth with chronic illness and the significant lifelong burden of sickle cell disease (SCD), little is known about SCD-related worry among youth with SCD. The current study aimed to characterize SCD-related worry across two developmental stages: school-aged children (8-12 years) and adolescents (13-19 years), and to identify risk factors (i.e., demographic, treatment-related, diagnostic, psychosocial factors) associated with elevated SCD-related worry. Associations between SCD-related worry and healthcare utilization were examined as a secondary aim.
Methods: A sample of youth (N = 538, mean age = 14.0 years, standard deviation [SD] = 3.0 years, range = 11-19 years, 49.6% female, 60.4% SS/Sβ0, 27.5% SC, 8.9% Sβ+) living with SCD enrolled in the Sickle Cell Research and Intervention Program (SCCRIP) cohort (Hankins et al. Pediatric Blood & Cancer 2018; PMID 29797644) completed study procedures. Within this sample, patients completed 9 items rated on a 5-point Likert scale (0 = never, 1 = almost never, 2 = sometimes, 3 = often, 4 = almost always) from the Pediatric Quality of Life Inventory Sickle Cell Disease Module (PedsQL-SCD) that assessed SCD-related worries (e.g., concerns of pain, acute medical complications). Patients also completed PedsQL-SCD items that assessed low mood (1-item), pain (Pain and Hurt subscale), and pain interference (Pain Impact subscale). All items were completed via self-report. Response scales of items were converted to a 100-point scale (0 = 0, 1 = 25, 2 = 50, 3 = 75, 4 = 100). Demographics, treatment-related factors, SCD-genotype, physical comorbidities, and acute healthcare utilization were obtained from the SCCRIP database. To examine SCD-related worry, we calculated the average response across all SCD-related worry items. We also calculated the percentage of school-age and adolescent patients scoring ≥75 on individual SCD-related worry items. Regression models adjusted for age and sex were calculated to examine the effect of demographic, disease, disease modifying therapy, low mood, pain, and healthcare utilization on SCD-related worry.
Results: The mean average response for SCD-related worry items from the overall sample was 24.2 (SD = 20.4) indicating overall low levels of worry. The mean average response on SCD-related worry items was comparable for school-age children (mean = 22.9, SD = 19.9) and adolescents (mean = 25.9, SD = 20.9). Regarding specific elevated worries, the most common worry amongst the overall sample was “I worry others will not know what to do if I have pain,” with 14% of youth reporting this worry occurred often or almost always (i.e., score ≥75 on the item) in the past month. In addition, a greater number of adolescents than school-age children reported often or almost always on this item (17.5% vs. 11.2%, p = 0.049). In contrast, a higher percentage of school-age children compared to adolescents reported often or almost always on the item “I worry I might have a stroke” (9.3% vs. 4.4% p = 0.04). Among all youth, increased SCD-related worry was associated with a greater number of individuals living in the household (beta-coefficient [standard error]; 1.4[0.6], p = 0.02) as well as low mood (0.3[0.03], p &lt; 0.01), pain intensity (0.5[0.03], p &lt; 0.01), pain interference (0.6[0.04], p &lt; 0.01), and increased number of pain-related hospital admissions in the past year (2.8[1.1], p = 0.01). Male sex (-3.0[1.8], p = 0.09) and a history of stroke (5.8[3.5], p = 0.1) were both marginally associated with SCD-related worry.
Conclusions: Little research has examined SCD-related worry among youth with SCD. Demographic factors, history of stroke, low mood, pain, and healthcare utilization were associated with elevated SCD-related worry. Similar to other studies examining disease-specific worries among youth with a chronic illness, robust associations emerged between SCD-related worry and low mood, pain, and pain-related disability. These findings are notable and more in-depth examination of SCD-related worry is warranted.