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