Background: The studies in the Middle East Arabs have found that there are specific risk factors making Sickle cell disease (SCD) more prevalent in that part of the world, which has the fast growing in the population and the increase in consanguinity marriage. Despite of that, there are no studies conducted in Makkah Al-Mukarramah or even in all Saudi Arabia (KSA) regions that, focusing on the complications of SCD and its related risk factors.
Objectives: To determine the pattern of admission and estimate the prevalence of different complications of SCD among children in Makkah Al-Mukaramah, KSA, 2017.
Patients and methods: A retrospective, analytic, noninterventional cohort study was carried out in Makkah Al- Mukaramah, KSA over a 6 months period (from first of June 2017 to end of December 2017). The study reviewed the medical files of all sickler patients who have been admitted to the pediatric departments of the involved hospitals in the given time. Records of patients having combined thalassemia disease and/or Glucose 6 Phosphate Dehydrogenase Deficiency (G6PD) were excluded from the study. The required data were collected by using hard copies of a well-designed, semi-structured, valid and reliable checklist.
Results: The study included 145 medical files of pediatric patients with SCD. Their age ranged between one day to 14 years with a Mean ± SD of 7 ± 3.6 years. Males represent 54.5% of them. Almost two-thirds of patients (67.6%) were Saudis. All cases live outside Makkah (n=5) were diagnosed as Sickle cell anemia with Vaso-occlusive crisis (VOC) whereas 47.1% of those live in Makkah were diagnosed by VOC at admission. The most frequent complication was VOC (55.9%), followed by infection (9%). Overall, 41.4% of SCA pediatric patients were admitted once whereas 25.5% were admitted more than 5 times. Acute splenic sequestration crisis was reported among 3.1% of Saudi patients compared to 12.8% of non-Saudi patients. This difference was statistically significant. Other complications were not significantly related to patients` nationality.
Conclusion: The most frequent complication of SCD was VOC. Primary prevention of the acute complications of SCD should base on health care provider with expertise in SCD, preferably hematologist. In addition to prevention of complications through the use of penicillin prophylaxis started in the newborn period, appropriate immunizations, and blood transfusions for those at risk for stroke.
Mohammad Alkot, Walaa A Almaghrabi, Noura Al-Najdi, Mohammad Al-Otaibi, Mokhtar Shatla and Hossam Abdelbaki