Summary Guidelines include recommendations intended to optimize patient care; used appropriately, they make healthcare consistent and efficient. In most lower‐middle income countries (LMICs), there is a paucity of well‐designed guidelines; as a result, healthcare workers depend on guidelines developed in Higher Income Countries (HICs). However, local guidelines are more likely to be implemented because they are applicable to the specific environment; and consider factors such as availability of resources, specialized skills and local culture. If guidelines developed in HICs are to be implemented in LMICs, developers need to incorporate local experts in their development. Involvement of local stakeholders may improve the rates of implementation by identifying and removing barriers to implementation in LMICs. Another option is to encourage local experts to adapt them for use in LMICs; these guidelines may recommend strategies different from those used in HICs, but will be aimed at achieving the best practicable standard of care. Infrastructural deficits in LMICs could be improved by learning from and building on the successful response to the human immunodeficiency virus/acquired immunodeficiency syndrome pandemic through interactions between HICs and LMICs. Similarly, collaborations between postgraduate medical colleges in both HICs and LMICs may help specialist doctors training in LMICs develop skills required for guideline development and implementation.

In Africa, sickle cell disease (SCD) is a major public health problem with over 200,000 babies born per year. In Ghana, approximately 15,000 (2%) of Ghanaian newborns are diagnosed with SCD annually. A retrospective review of medical records of all SCD patients aged 13 years and above, who presented to the sickle cell clinic at Ghana Institute of Clinical Genetics (GICG), Korle-Bu, from 1st January 2013 to 31st December 2014, was carried out, using a data abstraction instrument to document their phenotypes, demographics, attendance/clinic visits, pattern of attendance, and common complications seen. During the period under review 5,451 patients were seen at the GICG, with 20,788 clinic visits. The phenotypes were HbSS (55.7%) and HbSC (39.6%) with other sickle cell phenotypes (4.7%). Out of the 20,788 clinic visits, outpatient visits were 15,802 (76%), and urgent care visits were 4,986 (24%), out of which 128 (2.6%) patients were admitted to the Teaching Hospital for further management of their acute complications. There were 904 patient referrals (out of 5,451 patients) for specialist care; the 3 specialties that had the most referrals were Obstetrics and Gynaecology (168 patients), Orthopaedics (150 patients), and Ophthalmology (143 patients). In 2014, complications seen at KBTH included 53 patients with avascular necrosis (AVN) and 61 patients with chronic leg ulcers. Our centre has a large number of patients living with sickle cell disease. From our experience, early recognition and referral of sickle cell related complications can reduce morbidity and mortality associated with this disease. A multidisciplinary approach to care of SCD patients is therefore important.

Sickle cell disease (SCD) is associated with adverse pregnancy outcome. In women with SCD living in low-resource settings, pregnancy is associated with significantly increased maternal and perinatal mortality rates. We tested the hypothesis that implementing a multidisciplinary obstetric and hematology care team in a low-resource setting would significantly reduce maternal and perinatal mortality rates. We conducted a before-and-after study, at the Korle-Bu Teaching Hospital in Accra, Ghana, to evaluate the effect of a multidisciplinary obstetric-hematology care team for women with SCD in a combined SCD-Obstetric Clinic. The pre-intervention period was assessed through a retrospective chart review to identify every death and the post-intervention period was assessed prospectively. Interventions consisted of joint obstetrician and hematologist outpatient and acute inpatient reviews, close maternal and fetal surveillance, and simple protocols for management of acute chest syndrome and acute pain episodes. Primary outcomes included maternal and perinatal mortality rates before and after the study period. A total of 158 and 90 pregnant women with SCD were evaluated in the pre-and post-intervention periods, respectively. The maternal mortality rate decreased from 10 791 per 100 000 live births at pre-intervention to 1176 per 100 000 at postintervention, representing a risk reduction of 89.1% (P 5 0.007). Perinatal mortality decreased from 60.8 per 1000 total births at pre-intervention to 23.0 per 1000 at post-intervention, representing a risk reduction of 62.2% (P 5 0.20). A multidisciplinary obstetric and hematology team approach can dramatically reduce maternal and perinatal mortality in a low-resource setting.

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In Africa, the maternal mortality rate in sickle cell disease (SCD) is~10%. Our team previously demonstrated an 89% decrease in mortality rate in a before-and-after feasibility study among women with SCD living in low-resource setting in Ghana. In the same cohort including additional participants with and without SCD, we used a prospective cohort design to test the hypothesis that implementing a multidisciplinary care team for pregnant women with SCD in low-resource setting will result in similar maternal and perinatal mortality rates compared to women without SCD. We prospectively enrolled pregnant women with and without SCD or trait and followed them up for 6-week postpartum. We tested the newborns of mothers with SCD for SCD. We recruited age and parity matched pregnant women without SCD or trait as the comparison group. Maternal and perinatal mortality rates were the primary outcomes.A total of 149 pregnant women with SCD (HbSS, 54; HbSC, 95) and 117 pregnant women without SCD or trait were included in the analysis. Post-intervention, maternal mortality rates were 1.3% and 0.9% in women with and without SCD, respectively (P = 1.00); the perinatal mortality rates were 7.4% and 3.4% for women with and without SCD, respectively (P = 0.164). Among the mothers with SCD,~15% of newborns had SCD. Multidisciplinary care of pregnant women with SCD may reduce maternal and perinatal mortality rates to similar levels in pregnant women without SCD in low-resource settings. Newborns of mothers with SCD have a high rate of SCD.

Pregnancy is a life-threatening occurrence in women with sickle cell disease (SCD), with increased odds of maternal and perinatal mortality compared to pregnant women without SCD. 1 During pregnancy, women with SCD can also experience SCD related maternal morbidities, including acute vaso-occlusive pain episode, acute chest syndrome (ACS), and venous thromboembolism with expected increased incidence rates when compared to not being pregnant. 2 We previously demonstrated in a case series that 87% of all maternal deaths were due to ACS, with almost 80% of episodes preceded by an acute pain event. 3 In another prospective study, we demonstrated increased incidence rates of acute pain and ACS in pregnant women with SCD 4 compared to historical non-pregnant women with SCD. 5 As a planned follow-up to our prospective cohort study, we extended the outcome in our pre-existing cohort of pregnant women with SCD 4 to determine the impact of pregnancy on acute pain events pre-and postpartum. We tested the hypothesis that pregnant women with SCD have higher incidence rates of acute pain requiring hospitalization and ACS during pregnancy compared to one-year postpartum.

Sickle cell disease (SCD) is most prevalent in sub-Saharan Africa with 15,000 newborns per year in Ghana. 1,2 Pregnant women with SCD have increased risks of both SCD-specific and pregnancy-related complications, compared to pregnant women without SCD. 3 In a recent meta-analysis, our team reported that SCD in pregnancy increases the risk of maternal death by over 20-fold compared to pregnant women without SCD, in lowand middle-income countries. 4 In sub-Saharan Africa, maternal death rate of SCD is 7%-12%, 3,5,6 without clearly identifiable etiologies.Following our before and after study that demonstrated a 89% relative risk reduction in maternal death in women with SCD with our combined SCD-obstetric team care model, 7 we undertook a medical records and autopsy review of all SCD maternal deaths over a sevenyear period from 2010 to 2016, at the Korle-Bu Teaching hospital (KBTH), in Accra, Ghana. Ethical approval was obtained from the institutional review boards of the College of Health Sciences, University of Ghana and Vanderbilt University Medical Centre. At the KBTH, about 11 000 deliveries occurred annually including 200 women with SCD, and about two-thirds are HbSC. of all maternal deaths in women with SCD at KBTH. All deaths that occurred prior to arrival at the hospital, and those without documented evidence of hemoglobin phenotype, were excluded. Our research team, including obstetricians, hematologists, a pediatrician, anesthesiologists and nurses, adjudicated all cases of SCD maternal deaths to reach a consensus on the cause of death. Pathologists performed autopsies. In this series, we defined maternal death, direct and indirect obstetric cause of death according to WHO ICD-10 classification (WHO 2012). 8 The maternal mortality records, patients' admission and discharge files, and death certificates of all SCD-related maternal deaths within the study period were retrieved and reviewed by the team. Data were summarized as simple descriptive statistics. The Mann-Whitney U-test and Fisher exact test were used to evaluate differences between pregnant women with HbSS versus HbSC. A P-value of < .05 was considered statistically significant. SPSS version 24 (IBM Corp, USA) was used for analysis.HbSS was attributable to stroke. Two-thirds (6/9) of venous thromboembolism occurred in women with HbSC disease. There was no statistically significant difference in VTE as cause of death between HbSC and HbSS (P 5 .69).In our case series of maternal death among pregnant women with SCD at a single centre in Accra, Ghana, the main causes of death were related to SCD complications. Most maternal deaths were preceded by acute pain episodes; the commonest cause of death was ACS (86%).The number of deaths from venous thromboembolism was similar in 0 | V C 2018 Wiley Periodicals, Inc.wileyonlinelibrary.com/journal/ajh Am J Hematol. ;1-3.[8] Organization WH. The WHO application of ICD-10 to deaths during pregnancy, childbirth and puerperium: ICD MM; 2012. SUPPORTING INFORMATIONAdditional Supporting Information may be found on...

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