Health Care Transitions in Sickle Cell Disease

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Over the past three decades, advances in the care for children with sickle cell disease (SCD) have increased their life expectancy and necessitated their transition to adult health care. However, there is a limited understating of health care transition in SCD and the impact it has on outcomes beyond perspective on and satisfaction with the process from the perspective of the adolescent and young adult (AYA) and their families. The purpose of this dissertation was to provide a better understanding of health care transitions in AYAs with SCD. This purpose was achieved through an examination of the state of the science on health care transition in AYAs with SCD, the challenges of shifting self-management from the parent to the adolescents with SCD prior to transition to adult care, and the patterns of health care utilization during transition and the factors associated with these patterns.

Findings from our integrative review indicated that few studies examined health care transition in AYAs with SCD. Most of the research on health care transition in AYAs with SCD focused on individual, family support, and the health care domain, yet results are inconclusive. For example, there is evidence that parents were heavily involved in the AYA’s health care. Although this involvement was viewed as supportive by the AYA as well health care providers, it’s not clear how parental involvement and the parent-AYA relationship can be utilized to facilitate health care transition for the AYAs with SCD. In addition, very few studies examined health care transition outcomes or examined how AYAs utilize the health care system to meet their health care needs during transition.

To further understand the parent-AYA relationship in managing SCD, we examined the shifting of management responsibility from the parent to the adolescent with SCD. Our findings indicated that shifting management responsibility is a critical and challenging process. The challenges that adolescents and parents face are adaptive-type challenges, rather than technical-type challenges. While technical-type challenges are easily fixed with technical work, more complex adaptive work is needed to address adaptive-type challenges, making the shifting of management responsibility more complex.

To understand health care transition in AYAs with SCD, we conducted a longitudinal examination of health care utilization for AYAs with SCD during transition to adult care and examined the factors associated with the different trajectory groups. Most AYAs in our sample had low clinic, hospital, and emergency department (ED) utilization trajectories. Few AYAs had high utilization in the clinic, hospital, and/ or ED. However, this group usually accounts for the highest costs of care. The sample mean emergency reliance scores were also below the cutoff point of 0.33 for high ED reliance. However, we did find evidence of increasing reliance on the ED compared to ambulatory care with increasing age indicating that a small group of AYAs with SCD might be at increased risk for high reliance on the ED. Individual, contextual, and transfer related factors were associated with the different health care utilization trajectory groups for AYAs with SCD. These factors varied between the different health care services indicating the complexity of health care utilization in AYAs with SCD and the factors influencing them. Receiving hydroxyurea was the only common predictor between higher clinic and higher hospital utilization trajectory groups, while distance to the sickle cell center was the only common predictor between higher clinic and higher ED utilization trajectory groups. Depression, chronic pain, long-acting narcotics, and chronic transfusion at age 19 predicted higher hospital and higher ED trajectory utilization groups.

A major finding in this study was the association of several mental health conditions, including depression, with health care utilization group membership. This finding underscores the importance of routine screening and adequate management of mental health care conditions in AYAs with SCD, especially during transfer to adult care.

One goal of this dissertation was to describe successful transition. The majority of our sample has transferred to adult care. The mean age at transfer was 19 years. We also examined the continuity of care after transfer and the majority of participants who transferred to adult care had at least one additional encounter in the adult sickle cell clinic, and around 65% had more than ten encounters. Only a few (5.83%) had no adult encounters after transfer. As a result, we considered the vast majority of participants who had at least one encounter in the adult sickle cell clinic to have successfully transferred and integrated into adult care.






Kayle, Mariam (2017). Health Care Transitions in Sickle Cell Disease. Dissertation, Duke University. Retrieved from


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