Organ damage mitigation with the Baskent Sickle Cell Medical Care Development Program (BASCARE)

Area of Science:

• Hematology
• Public Health
• Medical Informatics

Background:

• Sickle cell disease (SCD) is prevalent in the Eastern Mediterranean region.
• Early childhood interventions can postpone morbidity and mortality in SCD patients.
• The Baskent Sickle Cell Medical Care Development Program (BASCARE) has 25 years of regional experience.

Purpose of the Study:

• To evaluate the organ damage-reducing effects of the BASCARE program.
• To assess the impact of BASCARE on patient care and outcomes in SCD.
• To analyze the efficacy of specific program components.

Main Methods:

• Development of an electronic health recording system (PRANA) and electronic vaccination system.
• Implementation of low citrate infusion in automatic erythrocyte exchange (ARCE) for pregnant women.
• Utilized leukocyte-filtered, irradiated blood, MRI (T2) for hemosiderosis, and allogeneic stem cell transplantation protocols.

Main Results:

• Vaccination rates increased significantly (influenza: 21.5% to 50.8%; pneumococcal: 21.5% to 49.2%).
• Effective ARCE maintained in 352 subjects; maternal/fetal mortality prevented in 35 pregnancies.
• Chelating therapy reduced from 6.7% to 5%; 13 adult stem cell transplant patients achieved cure without major complications.

Conclusions:

• The BASCARE program significantly improved SCD patient care and prolonged lifespan (42 ± 13 years vs 29 ± 7 years).
• Individualized, holistic programs like BASCARE are recommended for SCD centers.
• The program demonstrated effective management of SCD complications and improved patient outcomes.