Changing Trends in Sickle Cell Disease-Related Mortality in the United States over Four Decades

Introduction: Sickle cell disease (SCD) causes significant morbidity and early mortality. The Cooperative Study of Sickle Cell Disease estimated median life expectancy for individuals with SCD at 42 years for males and 48 years for females in the early 1990s. However, this calculation was made prior to now standards-of-care in SCD, including routine transcranial doppler screening, pneumococcal prophylaxis, and the introduction of the primary disease-modifying therapy hydroxyurea and the widespread use of chronic blood transfusion. Over the last thirty years, with expanded access to disease modifying therapies, increased evidence exists for the preservation of organ function and less frequent acute vaso-occlusive pain. It remains unclear whether the introduction of these therapies has affected overall SCD-related mortality or the specific causes of death of those with SCD in the United States, particularly in the last decade when the use of hydroxyurea has expanded most significantly.