Sickle Cell Health Awareness, Perspectives, and Experiences (SHAPE) survey: Perspectives of adolescent and adult patients, caregivers, and healthcare professionals on the burden of sickle cell disease.

OBJECTIVES: Sickle cell disease (SCD) is an inherited disorder that causes lifelong complications, substantially impacting the physical and emotional well-being of patients and their caregivers. Studies investigating the effects of SCD on quality of life (QOL) are often limited to individual countries, lack SCD-specific QOL questionnaires, and exclude the caregiver experience. The SHAPE survey aimed to broaden the understanding of the global burden of SCD on patients and their caregivers and to capture the viewpoint of healthcare providers (HCPs). METHODS: A total of 919 patients, 207 caregivers, and 219 HCPs from 10, 9, and 8 countries, respectively, answered a series of closed-ended questions about their experiences with SCD. RESULTS: The symptoms most frequently reported by patients were fatigue/tiredness (84%) and pain/vaso-occlusive crises (71%). Patients' fatigue/tiredness had one of the greatest impacts on both patients' and caregivers' QOL. On average, patients and caregivers reported missing 7.5 days and 5.0 days per month, respectively, of school or work. HCPs reported a need for effective tools to treat fatigue/tiredness and a desire for more support to educate patients on long-term SCD-related health risks. CONCLUSIONS: The multifaceted challenges identified using the SHAPE survey highlight the global need to improve both patient and caregiver QOL.

Sickle cell disease and the need for blood: Barriers to donation for African, Caribbean, and Black young adults in Canada.

BACKGROUND: Many blood operators around the world face the challenge of increasing the number of donors of African ancestry to meet the transfusion needs of people living with sickle cell disease. This article reports results of the barriers to blood donation for young adults (aged 19-35) in Canada who identify as African, Caribbean, or Black. STUDY DESIGN AND METHODS: A community-based qualitative study was conducted by researchers from community organizations, blood operator, and universities. In-depth focus groups and interviews (n = 23) were conducted from Dec 2021 to Apr 2022 and thematic analysis was completed. RESULTS: Applying a socio-ecological model, multiple levels of interacting barriers to blood donation were identified. These included macro-level barriers (e.g., systemic racism, mistrust of the healthcare system, sociocultural beliefs and views about blood and sickle cell disease), mezzo-level barriers (e.g., deferral criteria, minimum hemoglobin levels, donor questionnaire, access, parental concerns), and microlevel barriers (e.g., limited knowledge of blood needs for people with sickle cell disease, lacking information about blood donation process, fear of needles, personal health concerns). DISCUSSION: This study is the first to focus on barriers to donation for African, Caribbean, and Black young adults across Canada. Parental concerns, informed by parents' experiences of inequitable healthcare and mistrust, emerged as a novel finding in our study population. Results suggest that higher order (macro-level) barriers influence and may reinforce lower order (mezzo- and microlevel) barriers. As such, interventions aimed at addressing barriers to donation should keep in view all levels with particular attention paid to higher order barriers.

Patient and Caregiver Perspectives on Care-Seeking During a Vaso-Occlusive Crisis in Sickle Cell Disease: Results from Qualitative Interviews in Canada.

Purpose: There is little research on care-seeking preferences during active pain crises for sickle cell disease (SCD) patients and their caregivers. The objective of this study was to identify relevant, patient or caregiver narratives of the pain crisis experience, to understand the factors that contribute to care-seeking during a pain crisis, and to identify preferences when making care-seeking decisions during a pain crisis. Patients and Methods: Qualitative semi-structured interviews were conducted with Canadian residents with a self-reported SCD diagnosis, who were either ≥18 years of age or an adolescent between the ages of 12-18. Interviews were hosted virtually, audio-recorded, and transcribed verbatim. Results: A total of 23 individuals participated (74% female; 26% male), including six adolescents with parent dyads and 11 adults. Almost all (N = 21, 91.3%) participants were Black/African American. Participants mentioned many factors that influenced care-seeking decisions, mainly the symptom and pain experience; institutional factors (waiting time, the presence of and adherence to treatment guidelines, and the empathy or racial bias felt from medical staff); and subject-level factors (age and a flexibility in daily responsibilities). Conclusion: This study identified important institutional and subject-level considerations involved in care-seeking decisions. Most importantly, this study highlights the racial stigma faced by many patients when care-seeking in the ER and the lack of care protocol implemented, which hinders care-seeking in a dedicated medical facility. From the patient perspective, these are clear gaps to fill to encourage patients to seek and receive the care they deserve.