OBJECTIVES: Descriptive analysis of patients suffering from Sickle-Cell Disease (SCD) with CNS (Central Nervous System) crises, and the correlation of different variables as demographics, major complications, hematologic and genetic factors on the degree of CNS crisis, for predictor generation and better future management.

METHODS: A retrospective patients’ chart review study was built with total of 146 SCD patients with CNS crisis inside the Hematology Department in Prince Mohamed Bin Nasser Hospital in Jazan (PMBNH) Saudi Arabia from Jan.1^st to Dec. 31^st, 2021. Analysis was done using SPSS for descriptive analysis and Jaccards for regression and multiple regression analysis.

RESULTS: Patients with SCD and CNS crisis were 72(49.3%) male and 74(50.7%) female with age range from 14 to 47 years and mean of 24.4. Most of them were single (76.7%) and non-employed (80.8%). Hemoglobin electrophoresis showed (73.3%) of patients to be homozygous SCA (HbSS), while (26.7%) were SC/β-thalassaemia (HbS/ßo and HbS/ß+). The frequency of CNS crisis was once in (89.0%), twice in (8.2%), and three times in (2.7%). The mean age at onset of first CNS crisis was 15.1 years. About (52.1%) developed the first CNS crisis before 10 years of age. The complications associated SCD were vaso-occlusive crisis (100%), avascular necrosis (16.4%), acute chest syndrome (7.5%), pulmonary hypertension and intrahepatic cholestasis (2.7% for each). The apparent CNS sequelae were found among (36.3%) of patients, included motor disability (60.4%), intellectual disability (32.1%), epilepsy (17.0%) and dysphasia (11.3%). About 70(47.9%) were successfully referred to higher centers for stem cell transplantation (SCT) and the case fatality rate within the study was 2.74%.

CONCLUSIONS: CNS crises are common morbidity among SCD patients, the optimized management of cases need close monitoring of CNS crises and follow-up for improved patients’ outcome and avoiding further complications.