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1065 Sickle Cell Disease-Related Worry and Associations with Healthcare Utilization in Youth with Sickle Cell Disease

Program: Oral and Poster Abstracts
Type: Oral
Session: 904. Outcomes Research: Hemoglobinopathies: Non-Malignant Conditions: Transforming Care: Insights into Healthcare Utilization, Outcome Measurement, and Treatment Impact in Sickle Cell Disease
Hematology Disease Topics & Pathways:
Research, Sickle Cell Disease, Health outcomes research, Clinical Research, Hemoglobinopathies, Pediatric, Diseases, Patient-reported outcomes, Study Population, Human
Monday, December 9, 2024: 5:00 PM

Alex Pizzo, MSc1*, Guolian Kang, PhD2, Victoria Okhomina, MSc2*, Jerlym Porter, PhD, MPH3, Jason Hodges, PhD4, Parul Rai, MD, MBBS4, Clifford M. Takemoto, MD5, Jane S Hankins, MD, MS6 and Nicole M. Alberts, PhD7*

1Department of Psychology, Concordia University, Montreal, QC, Canada
2Department of Biostatistics, St. Jude Children's Research Hospital, Memphis, TN
3Department of Psychology and Biobehavioral Sciences, St. Jude Children's Research Hospital, Memphis, TN
4Department of Hematology, St. Jude Children's Research Hospital, Memphis, TN
5St. Jude Children's Research Hospital, Memphis, TN
6Department of Global Pediatric Medicine, St. Jude Children's Research Hospital, Memphis, TN
7Concordia University, Montreal, QC, Canada

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 < 0.01), pain intensity (0.5[0.03], p < 0.01), pain interference (0.6[0.04], p < 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.

Disclosures: Rai: Global Blood Therapeutics: Consultancy. Takemoto: Novo Nordisk: Research Funding; Pfizer: Research Funding; Novartis: Other: DSMB; Merck: Consultancy, Honoraria.

*signifies non-member of ASH