Predictors of Adverse Outcome in Sickle Cell Disease Patients from Oman

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.