Introduction
Sickle cell disease (SCD) is a genetic disorder that leads to vaso-occlusive phenomena and hemolysis and a myriad of other major complications that could be life-threatening. It is one of the commonest inherited diseases globally and is inherited as an autosomal recessive disease caused from the substitution of valine for glutamic acid at the sixth amino acid of the beta-globin chain. 1 This amino acid substitution leads to the production of a hemoglobin that is poorly soluble when deoxygenated. The clinical features such as vaso-occlusive phenomena, results from the polymerization of deoxygenated hemoglobin S. In pregnancy, SCD is associated with significant maternal morbidity and mortality. The recognized complications include maternal mortality, preeclampsia, eclampsia, venous thromboembolism, cesarean delivery, intrauterine fetal death and fetal growth restriction.2
The prevalence of SCD varies between countries. For example, data from the United States showed that the overall prevalence is roughly about 4.83 per 10,000 deliveries.3 Among those women with SCD, 28.5% of them develop a crisis at the time of delivery. The maternal mortality rate was reported to be 1.6 per 1000 deliveries in women with SCD, compared to 0.1 per 1000 without SCD.3
Information about the prevalence in Saudi Arabia is probably underestimated and varies between the different provinces, with the highest prevalence being in the Eastern province, followed by the Southwest province.4
SCD in pregnancy tends to cause higher episodes of painful crises and a higher frequency of blood transfusion2. Although complications of SCD are more commonly associated with genotype HbSS, other genotypes such as: HbSB and HbSC, are considered part of SCD and should receive the same level of care as those with HbSS. The development of a multidisciplinary care approach and comprehensive sickle cell centers seem to be associated with a decrease in the incidence of perinatal complications.5,6
To date, in Saudi Arabia, there is no National Clinical Practice Guidelines to provide guidance for management of SCD in pregnant women.
Clinical Practice Guidelines (CPGs) were defined, by the Health and Medicine Division (HMD) of the American National Academies, formerly the Institute of Medicine (IOM), as ‘statements that include recommendations intended to optimize patient care that are informed by a systematic review of evidence and an assessment of the benefits and harms of alternative care options.7
The Second edition of the Appraisal of Guidelines for Research and Evaluation Instrument (AGREE II) is the gold standard for quality assessment or critical appraisal of CPGs. It was first published in its original form in 2003 and lastly updated in 2017 by the AGREE enterprise. AGREE II is a validated quantitative tool that has been cited in well over 1013 articles and endorsed by several healthcare organizations.8,9
AGREE II identifies constituents that must be addressed by CPGs to improve their quality and henceforth ensure their expected trustworthiness and positive impact on healthcare outcomes.9
We decided as a university referral teaching hospital to take the initiative and conduct a systematic review of published evidence-based CPGs and critically appraising eligible CPGs using the AGREE II instrument in preparation for adapting a CPG for management of pregnant women with SCD as part of our CPG adaptation program that follows a formal methodology for adaptation of CPGs, the ‘King Saud University Modified ADAPTE ’ method, where details of which were reported in a previously published article.10