Abstract

Objective: To study the management and the prognosis of sickle cell disease in its homozygous (SS) and composite (SC) forms, this is particularly morbid when it is associated with pregnancy, in the quest to propose a protocol for its management.
Patients and methods: This were a retrospective, descriptive and analytical study carried out at the Pikine National Hospital Center, in suburb of Dakar, over a period of four successive years, from April 1, 2009 to July 31, 2019. Were included all pregnant women with major sickle cell syndrome who gave birth in hospital. Data entry and analysis were done by the Statistical Package of Social Science (SPSS) and Excel software.
Results: We identified twenty-four (24) pregnant women with major sickle cell disease, representing an overall prevalence of 0.1%. The average age was 27.2years. The SS phenotype was the most found with Twenty-two cases (91.6%). Their average pregnancy rate was 1.5 pregnancies. The majority (53.8%) were diagnosed with sickle cell disease during the current pregnancy, in the event of a vaso-occlusive crisis or during a systematic pregnancy check-up. The average hemoglobin level was 7.7g/dl with extremes of 10.1g/dl and 2.3g/dl. Urogenital infections were present in 50% of cases. One case of gonalgia was noted in a patient with an SS phenotype. The average gestational age at delivery was around 31.7 weeks with extremes of 26 to 39 weeks. Thirteen (13) pregnancies were complicated by intrauterine growth restriction (IUGR) in SS patients. The stillbirth rate was 125‰. We noted 69.2% of live births, 23.1% of intra uterine fetal death and 7.7% of abortions. Almost half of the patients (13 cases) had received a postpartum blood transfusion.
Conclusion: The results of our study perfectly show the seriousness of this pathology and the importance of rigorous hematological management. This is a pregnancy at high risk of maternal-fetal complications requiring appropriate obstetric follow-up, which must be based on several axes: pregnancy planning, multidisciplinary follow-up, screening for signs of severity and supervised management of acute accidents.

Keywords

sickle cell disease, type SS and SC, pregnancy, complications, multidisciplinary, pregnancy planning, Urogenital infections, gestational age, gonalgia, intrauterine growth, retrospective, vaso-occlusive