27320461

PMC4918094

Introduction

Advances in primary prophylaxis have resulted in improved outcomes for patients with sickle cell anemia (SCA; i.e., hemoglobin SS- and Sβ°-thalassemia). Standard prophylactic measures include a first pneumococcal polysaccharide vaccine (PPV) and transcranial Doppler ultrasound (TCD) at age 2 years. Though efficacious, evidence suggests that delivery of these interventions is suboptimal. This study reports adherence to these measures and examines concordance across different data sources, using Registry and Surveillance for Hemoglobinopathies project data.

Methods

Retrospective database and SCA center chart review identified children with SCA aged 24–36 months between January 1, 2004, and December 31, 2008. PPV and TCD administration were determined through Medicaid and Children's Health Insurance Program administrative claims data, medical record review, and Georgia Registry of Immunization Transaction and Services. Analysis was conducted in 2015.

Results

A total of 125 children met inclusion criteria. Forty-five (36.0%) children had documentation of both interventions whereas 19 (15.2%) had no documentation of either intervention. Sixty-one (48.8%) children obtained only one intervention. Of these, more were likely to have had PPV than TCD (77.0% vs 23.0%, respectively, p<0.001). Agreement between claims data and medical record review was moderate for PPV (κ=0.55) and substantial for TCD (κ=0.74).

Conclusions

No single, reliable data source for tracking standard of care for children with SCA statewide was found. Based on study data, prophylaxis measures were not universally implemented during the surveillance period. Further research is needed to adequately track changes over time, determine risk groups, and develop methods of evaluating important metrics.

CDC Public Access

U50 DD000582/DD/NCBDD CDC HHS/United States
U50 DD001010/DD/NCBDD CDC HHS/United States