Sickle Cell Disease in Newborns in Germany: Analysis of the AOK Health Insurance Data

Abstract

Sickle cell disease (SCD, ICD-10:D57) is a hemoglobinopathy of global public health relevance. As an autosomal recessive genetic disorder, it affects 70-150 newborns in Germany per year (1-2 per 10,000). Early detection during the asymptomatic first 3 months of life would meet national treatment recommendations and prevent disease sequelae. Objectives are 1) to measure the prevalence of SCD in newborns in the AOK's claims data, 2) to determine the age at first diagnosis D57, and 3) to count typical hospitalizations. Inpatient and outpatient data on 204 children born in 2009 and 2010, insured with the AOK for at least one day in their year of birth and coded as D57 during 6 calendar years were analyzed. The number of newborns in the AOK in 2009 and 2010 (denominator) is known. Valid SCD cases (numerator) fulfilled the requirement for D57 (except D57.3) to be coded at least 2 times in different quarters of years. The prevalence proportion (PP) was calculated with a bootstrapped confidence interval (CI). The first mention of D57 per case was dated in quarters of years (Q). The Standardized Incidence Ratio (SIR) was calculated to compare hospitalizations from the month 13 to month 61 with the age- and sex-matched population (1-4-year olds). The calculations were performed with SPSS 22 and Excel. The validation resulted in 78 SCD cases. The prevalence was 1.96 (95% CI 1.53-2.41) per 10,000 newborns. Regions of higher prevalence were Berlin, Bremen and Hamburg (summarized) and North Rhine-Westphalia. In only 15.4% of the cases, D57 was mentioned early (in Q1-2). The median date was in Q7. There were 266 hospitalizations in relation to 78 children, with an SIR=6.8. The rate of hospitalizations was thus 6.8 times higher than in the population. Feared outcomes such as pneumonia, septicemia and splenic disorders were among the hospital discharge diagnoses. SCD in Germany is evident in claims data of Germany's largest statutory health insurance. At present, the opportunity for early detection is being widely missed. The time to diagnosis could be shortened by the inclusion of SCD in the universal newborn screening. Until this policy is implemented, parents with a relevant family background and health professionals should pay more attention to SCD.