Transition Readiness Assessment of Adolescents with Sickle Cell Disease and Their Caretakers at the Sickle Cell Clinic in Kumasi, Ghana

Background: Sickle Cell Disease (SCD) is associated with high child mortality in sub-Saharan Africa (SSA). Improved medical services within an established SCD program in Kumasi, Ghana have focused on enhanced newborn screening and comprehensive pediatric SCD care (Ohene-Frempong et al., 2008). These efforts have improved SCD survival and increased the need for adolescents to transition to adult care. We sought to understand patient and caretaker perspectives on transition to adult care for adolescents with SCD visiting the Komfo Anokye Teaching Hospital Sickle Cell Clinic (KATH SCC) in Kumasi, Ghana, where the transition occurs by age 15 years.

Methods: Structured interviews were conducted with adolescents aged 12-15 years and adults who accompanied them to KATH SCC. Interviews queried 4 areas that are relevant for assessing transition readiness in the U.S.: SCD medical knowledge, symptom self-management, psychosocial impact, transition preparation. Interview questions were adapted from two surveys that assessed transition readiness of adolescents with SCD in the U.S. (Telfair et al., 2004; Treadwell et al., 2016) and modified to fit the Ghanaian context. Interviews were conducted in English or Twi (the prevalent local language), per participant preference. Responses were transcribed during the interview, and later translated into English, as needed, by one interviewer (MKS) for descriptive statistics and qualitative analysis.

Results: In total, 46 children (mean age 13 years) and 46 accompanying adults were consented (or assented) and interviewed. Most participants selected Twi for interviews. Adults were either parents (70%) or close relatives, with mean age 39.8 years (range 18-70). SCD type was determined by clinic records: 59% of children interviewed had HbSS, 35% HbSC, and 6% had no SCD type recorded. Most children and their caretakers had some knowledge about SCD and management: 61% of children knew that SCD is a blood disorder, while 74% knew that SCD is an inherited disorder. Majority of children knew what to do to ease pain in crisis (61%), where to go for care (85%), what care is helpful (72%), and could engage with the hospital team independently (87%). Gaps in knowledge were evident for up to one third of children for each question. Their responses were corroborated by at least 46% of adults. Significantly fewer children (39%) knew their SCD type compared to the adults (63%) (p=0.02). Children who reported knowing how to handle a pain crisis used a combination of analgesics, folic acid, penicillin, and a local ointment, but did not understand that folic acid and penicillin are maintenance medications that do not relieve painful episodes. Children appeared more concerned than adults about their current health and social stigmatization than about future health-related challenges. Seventy-six percent of children worried about having SCD, 28% felt like burdens to family, 37% told their friends about having SCD, but 96% had plans for their future. Adults had different perspectives of disease burden, where only 48% and 6% believed their child worried and felt like a burden, respectively. Few children (35%) and adults (37%), were aware of the requirement to switch to adult care by age 15, but most (72% or 74%) were willing to transition when asked to do so.

Conclusions: To the best of our knowledge, this is the first study of perspectives about care transition for adolescents with SCD and their caregivers in SSA. Our clinic-based assessment at a single site suggests that the youth and their adult caregivers are informed about SCD but lack information relevant to adequate medical care, are concerned about peer stigmatization and were unaware of the need to transition. Challenges to successful transition in the country may include the relatively young age of transition and gaps in knowledge needed for successful self-management. Future efforts to improve transition to adult SCD care may need to include education of adolescents and families about the disease, self-management, and transition and address sense of self-burden to families and stigmatization among peers. Generalizability of our findings and practical methods to address needs for SCD transition within Africa remain to be tested.

Acknowledgments: Support to MKS by David E. Rogers Student Fellowship Award from New York Academy of Medicine and IFAP Global Program of Columbia University. We thank KATH SCC staff for facilitating this research.