Sickle cell disease (SCD) is a major public health burden worldwide with increasing morbidity and mortality. We studied the risk factors associated with mortality in SCD patients, between the years 2006 and 2020 at three hospitals in Oman. We analyzed clinical manifestations, haematological, biochemical, and radiological parameters, use of antibiotics, and blood and exchange transfusions. Our cohort included 123 patients (82 males, 41 females), with a median age of 27 (IQ 21–35 years). SCD related complications included acute chest syndrome (ACS) in 52.8%, splenic sequestration in 21.1%, right upper quadrant syndrome in 19.5%, more than > 6 VOC /year in 17.9%, and stroke in 13.8%. At the terminal event, patients had cough, reduced O2 saturation, crepitation and fever in 24.4%, 49.6%, 53.6% and 68.3% respectively. Abnormal chest x-ray and chest CT scan were seen in 57.7%, and 76.4% respectively. Laboratory parameters showed a significant drop in hemoglobin (Hb) and platelet counts from baseline, with a significant rise in WBC, LDH and CRP from baseline (p < 0.05, Wilcoxon Signed Ranks test).. All patients received antibiotics, whereas, 95.9% & 93.5% received simple blood transfusions, and exchange transfusions respectively, and 66.6% required non-invasive ventilation. Among the causes of death, ACS is seen in 32 (26%), sepsis in 49 (40%), and miscellaneous in 42 (34%). Sudden death was seen in 32 (26%) of patients. Male gender, with low HbF, rapid drop in Hb and platelet, and increased in WBC, LDH, ferritin, and CRP, correlated significantly with mortality in this cohort.

Introduction: Sickle cell disease (SCD) is a major public health problem in Oman with high morbidity and mortality. The ability to identify the risk factors that are associated with mortality among SCD patients would permit accurate prognostication and provide an opportunity to use effective prophylactic management. Objective: The study aimed at identifying the risk factors associated with mortality in SCD patients by studying the clinical course of 86 patients who died at two tertiary care hospitals in Oman. Methods: We analyzed the electronic records of 86 SCD patients who died between 2006 to 2016. These data included cardiac parameters, SCD manifestations like frequency of painful crisis, acute chest syndrome, splenic and hepatic sequestration, dactylitis, and stroke. Furthermore, we also analyzed the haematological, biochemical and radiological parameters as well as use of antibiotics and exchange blood transfusion. Data were transcribed to Excel and later analyzed by IBM SPSS Ver23. Results±13.7. Previous complications prior to death included acute chest syndrome in 66.3% cases, followed by stroke in 20.9%, hepatic sequestration in 6.9%, splenic sequestration in 3.5% and dactylitis in 2.3% cases. At the terminal event, patients presented with fever, cough and crepitation in 47%, 29% and 36% respectively, while abnormal pulse, blood pressure, respiratory rate and O2 saturation were seen in 55%, 64%, 46% and 55% respectively. Further abnormal chest x-ray, CT scan and abdominal ultrasound were seen in 57.5%, 88.5% and 50% respectively. Amongst the haematology parameters, there was a significant drop in the mean hemoglobin and platelet counts from baseline, whereas there was a significant rise in the WBC counts (p<0.05, student's t test). The biochemical parameters showed a significant rise in the LDH and CRP levels (p<0.05). All patients received antibiotics, 88% received blood transfusions, 35.7% received exchange transfusions, whereas 87.5% required NIV/ventilation. Finally, all patients presented with multi-organ terminal events that varied in the severity, but sepsis showed the commonest cause of death (54.3%). Conclusion: Multi-organ failure was mostly associated with the terminal events relating to mortality. Although sepsis played a major role as a cause of death in association with multiorgan failure, low hemoglobin, and low platelet counts with increased WBC counts along with elevated LDH and CRP played a significant role in the terminal event in this cohort of SCD patients. Disclosures No relevant conflicts of interest to declare.