ISSN: 2578-501X
Authors: Leal CVF, dos Santos Barros G, Visconti ACC, Novak AM, de Souza CM, Caparrotti G, Varga TG, Yamasita GAM, Archangelo RV and Cancado RD*
Sickle cell disease (SCD) is a severe inherited and multisystem blood disorder characterized by hemolytic anemia, vasoocclusive crises (VOCs), progressive multiorgan damage, and increased mortality. SCD is the most common inherited disorder worldwide, with well over 300,000 children born with SCD yearly, 75% of whom are born in Africa. Based on the Brazilian neonatal screening program, the annual average of new cases of children diagnosed with SCD was 1,087, with an incidence of 3.78 per 10,000 live births and an estimated number of 60,000 to 100,000 individuals living with SCD in Brazil. Our objectives were to describe the sociodemographic and genotype characteristics and clinical complications of patients with SCD and compare the laboratory profile and the impact of healthcare resource utilization between patients with and without hydroxyurea during five years of observation. Objectives: To describe the sociodemographic and clinical complications, compare the laboratory profile and the impact of healthcare resources utilization between patients with and without hydroxyurea of SCD patients during five years of observation. Method: This was a retrospective cross-sectional descriptive study conducted at one Brazilian reference center on SCD - Santa Casa de Sao Paulo, in Sao Paulo (Southeast region of Brazil) during a 5-year observation period (2016-2020). Results: Among a total of 100 eligible adult patients, the main sociodemographic and genotypic characteristics of patients with SCD were: median age of 31 years, 84% of patients between 18 and 45 years of age, only one patient over 60 years of age; the majority were female (59%), Hb SS genotype (91%), black or mixed race, with complete secondary education and low family income; 100% of patients used the SUS insurance plan, although 29% also had private health insurance. VOC, infection, stroke and priapism, and cholecystectomy and avascular osteonecrosis were the most frequently observed acute and chronic complications, respectively. Most patients used hydroxyurea (71%), 22% were on a chronic transfusion regimen, and 17% used an oral chelator. Patients using HU had a statistically significant improvement in laboratory values (Hb, Fetal Hb, reticulocytes, DHL, bilirubin, leukocytes, and platelets) compared to patients without HU. Despite the improvement in laboratory parameters, patients using HU did not have significantly lower rates of medical care in the emergency room and hospitalization compared to patients without HU.
Keywords: Sickle Cell Disease; Acute And Chronic Complications; Hydroxyurea; Public Healthcare Resources
