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Published on: March 14, 2017
Improved survival of children and adolescents with sickle cell disease
Charles T Quinn1, Zora R Rogers, Timothy L McCavit
1Division of Hematology-Oncology, Department of Pediatrics, The University of Texas Southwestern Medical Center, Dallas, TX, USA. charlestquinn@gmail.com
Insights
Most children with sickle cell disease (SCD) now survive to adulthood. However, young adults face high mortality risks after transitioning to adult medical care, highlighting a critical care gap.
Area of Science:
- Hematology
- Pediatric Medicine
- Public Health
Background:
- Childhood survival for sickle cell disease (SCD) has improved.
- Limited data exists on survival and mortality patterns in older children and adolescents with SCD.
Purpose of the Study:
- To estimate 18-year survival for newborns with SCD.
- To document changes in SCD mortality causes and ages over time.
- To explore the association between improved medical care quality and survival.
Main Methods:
- Analysis of the Dallas Newborn Cohort (DNC) data.
- Inclusion of 940 subjects with 8857 patient-years of follow-up.
- Examination of mortality incidence, causes, ages, and quality of care over time.
Main Results:
- Contemporary 18-year survival is high: 93.9% for sickle cell anemia and 98.4% for milder SCD forms.
- Mortality patterns have shifted; sepsis is no longer the leading cause of death.
- Recent deaths occurred in patients ≥18 years, primarily post-transition to adult care.
- Improved quality of care in DNC, with more timely visits and interventions for young children.
Conclusions:
- Most children with SCD now survive childhood.
- Young adults transitioning to adult care are at high risk of early death.
- Addressing the transition to adult care is crucial for improving long-term outcomes in SCD.
Abstract:
The survival of young children with sickle cell disease (SCD) has improved, but less is known about older children and adolescents. We studied the Dallas Newborn Cohort (DNC) to estimate contemporary 18-year survival for newborns with SCD and document changes in the causes and ages of death over time. We also explored whether improvements in the quality of medical care were temporally associated with survival. The DNC now includes 940 subjects with 8857 patient-years of follow-up. Most children with sickle cell anemia (93.9%) and nearly all children with milder forms of SCD (98.4%) now live to become adults. The incidence of death and the pattern of mortality changed over the duration of the cohort. Sepsis is no longer the leading cause of death. All the recent deaths in the cohort occurred in patients 18 years or older, most shortly after the transition to adult care. Quality of care in the DNC has improved over time, with significantly more timely initial visits and preventive interventions for young children. In summary, most children with SCD now survive the childhood years, but young adults who transition to adult medical care are at high risk for early death.
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