Transition to adult care in sickle cell disease: A longitudinal study of clinical characteristics and disease severity

Abstract

Background. Sickle cell disease (SCD) is a chronic blood disorder in which mortality has increased for adolescents and young adults (AYA). Procedure. A longitudinal analysis of medical records was conducted to describe the clinical course among AYAs (ages 12–27 years) during transition to adult care. Measures included sociodemographic, complications, SCD severity (modified pediatric SCD severity index), comorbidities, and transfer. Group-based trajectory modeling (GBTM) to identify subgroups with distinct severity trajectories and chi-square and unpaired student t-test to explore subgroup differences were used. Results. Overall, 339 AYAs (97% black, 56% male, 69% hemoglobin SS) had 10848 clinic, 3840 hospital, and 3152 emergency department visits. Complications included vaso-occlusive crises (80%) and acute chest syndrome (41%). Comorbidities included depression (19%) and anxiety (14%). Most AYAs transferred to adult care (n=220) at 19 years. Fourteen AYAs died, 10 within seven years from transfer. GBTM identified both stable and increasing severity trajectory groups: stable-low (n=31, 23%), stable-medium (n= 61, 46%), stable-high (n=6, 5%), low-increasing (n=13,10%), and medium-increasing (n= 22,17%). AYAs with increasing severity (25%) were older, lived closer to the clinic, and had higher risk for SCD complications and comorbidities. They had less pediatric clinic visits, however, they were more likely to transfer and remain longer in adult SCD care. Conclusions: Whereas most AYAs had stable severity, nearly a quarter had increasing severity, over time. AYAs with increasing severity had more complications, were more likely to transfer to adult care, and demonstrated higher and longer adult SCD care utilization compared to AYAs with stable severity.

Correlates of successful transition in young adults with sickle cell disease

“…This suggests that, in our cohort, disease severity leading to hospitalization may negatively influence transition outcomes. In contrast, other studies in SCD and other chronic diseases found milder disease and decreased health care utilization correlated with increased care gaps . Thus, while co‐morbidities and disease severity are related to adverse health outcomes during transition, disease‐related characteristics and use of health care services are variably linked to transition outcomes.…”

“…4 Most children with SCD live to adulthood, increasing the number of patients transitioning medical care from pediatric to adult hematology providers. 5,6 Transition is associated with high rates of morbidity and mortality in patients with SCD. 7 Medical complications due to acute chest syndrome, pain, and renal disease contribute to this risk.…”

“…7 Medical complications due to acute chest syndrome, pain, and renal disease contribute to this risk. 3,5,6 In addition, rates of depression and anxiety are increased in young adults with SCD and are associated with increased disease severity. 5,8,9 Systemwide challenges to accessing care include limited availability of adult comprehensive sickle cell programs.…”

“…3,5,6 In addition, rates of depression and anxiety are increased in young adults with SCD and are associated with increased disease severity. 5,8,9 Systemwide challenges to accessing care include limited availability of adult comprehensive sickle cell programs. 4 Furthermore, adolescents make Abbreviations: ED, Emergency Department; IQR, interquartile range; JHH, Johns Hopkins Hospital; PCP, Primary Care Provider; SCD, sickle cell disease.…”

Sickle-Cell Disease Co-Management, Health Care Utilization, and Hydroxyurea Use

“…19,20 Predictor Measures Age-related differences in SCD health service utilization have been found in several studies. 16,[21][22][23][24] Young adults that are transition aged (16 to 25 years) have particularly high rates of acute (emergency and inpatient) care utilization. 23 However, 18-to-30-year-olds with SCD have the highest reported rates of acute care encounters per patient per year, before decreasing throughout middle and older ages.…”