Evidence-based management of pregnant women with sickle cell disease in high-income countries.
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ABSTRACT:
SUBMITTER: Oteng-Ntim E
PROVIDER: S-EPMC9820817 | biostudies-literature | 2022 Dec
REPOSITORIES: biostudies-literature
Publications
Evidence-based management of pregnant women with sickle cell disease in high-income countries.
Oteng-Ntim Eugene E Shangaris Panicos P
Hematology. American Society of Hematology. Education Program 20221201 1
Globally, patients living with sickle cell disease are now surviving to reproductive age, with life expectancy approaching 50 years in most countries. Thus, reproductive options are now essential for patients living with the condition. However, it can be associated with maternal, delivery, and fetal complications. Outcomes may vary depending on the level of expertise and resources. In this piece we provide an optional guideline for managing sickle cell disease in pregnancy. The therapeutic optio ...[more]
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Project description:Pregnancy in women with sickle cell disease (SCD) is fraught with complications, some of which are life-threatening. Managing pregnancy in these women can be challenging, especially with poor resources, which is often the case in low-income countries. In Nigeria, for instance, up to 90% of patients pay out of pocket for medical care due to the poorly developed health insurance system, and this worsens the morbidity and mortality associated with this condition. We describe a pragmatic approach to routinely managing pregnant women with SCD in the antenatal period, showing the feasibility of effective management of these high-risk pregnancies in limited-resource settings. We also present the case of a pregnant Nigerian woman with SCD who has intrauterine growth restriction (IUGR) and acute chest syndrome (ACS), conditions that are life-threatening for the fetus and the mother, respectively, and require prompt intervention. We highlight how we successfully managed this woman in a cost-effective manner by employing relatively inexpensive tests for diagnosis and treating her effectively with oxygen, appropriate antibiotics and manual exchange blood transfusion for the ACS, and finger pulse oximeters to monitor oxygen saturation. We explore pathophysiological concepts to IUGR in women with SCD and briefly discuss the appropriate mode of delivery, including the options for pain relief in labor.
| S-EPMC9821549 | biostudies-literature
Project description:BackgroundDespite ~90% of sickle cell disease (SCD) occurring in low-and middle-income countries (LMICs), the vast majority of people are not receiving evidence-based interventions (EBIs) to reduce SCD-related adverse outcomes and mortality, and data on implementation research outcomes (IROs) and SCD is limited. This study aims to synthesize available data on EBIs for SCD and assess IROs.MethodsWe conducted a systematic review of RCTs reporting on EBIs for SCD management implemented in LMICs. We identified articles from PubMed/Medline, Global Health, PubMed Central, Embase, Web of Science medical subject heading (MeSH and Emtree) and keywords, published from inception through February 23, 2020, and conducted an updated search through December 24, 2020. We provide intervention characteristics for each study, EBI impact on SCD, and evidence of reporting on IROs.Main results29 RCTs were analyzed. EBIs identified included disease modifying agents, supportive care agents/analgesics, anti-malarials, systemic treatments, patient/ provider education, and nutritional supplements. Studies using disease modifying agents, nutritional supplements, and anti-malarials reported improvements in pain crisis, hospitalization, children's growth and reduction in severity and prevalence of malaria. Two studies reported on the sustainability of supplementary arginine, citrulline, and daily chloroquine and hydroxyurea for SCD patients. Only 13 studies (44.8%) provided descriptions that captured at least three of the eight IROs. There was limited reporting of acceptability, feasibility, fidelity, cost and sustainability.ConclusionEBIs are effective for SCD management in LMICs; however, measurement of IROs is scarce. Future research should focus on penetration of EBIs to inform evidence-based practice and sustainability in the context of LMICs.Clinical trial registrationThis review is registered in PROSPERO #CRD42020167289.
| S-EPMC7888630 | biostudies-literature
Project description:Pregnancy in Sickle Cell Disease (SCD) women is associated to increased risk of clinical and obstetrical complications. Placentas from SCD pregnancies can present increased abnormal findings, which may lead to placental insufficiency, favoring adverse perinatal outcome. These placental abnormalities are well known and reported, however little is known about the molecular mechanisms, such as epigenetics. Thus, our aim was to evaluate the DNA methylation profile in placentas from women with SCD (HbSS and HbSC genotypes), compared to uncomplicated controls (HbAA). We included in this study 11 pregnant women with HbSS, 11 with HbSC and 21 with HbAA genotypes. Illumina Methylation EPIC BeadChip was used to assess the whole placental DNA methylation. Pyrosequencing was used for array data validation and qRT-PCR was applied for gene expression analysis. Our results showed high frequency of hypermethylated CpGs sites in HbSS and HbSC groups with 73.5% and 76.2% respectively, when compared with the control group. Differentially methylated regions (DMRs) also showed an increased hypermethylation status for the HbSS (89%) and HbSC (86%) groups, when compared with the control group methylation data. DMRs were selected for methylation validation (4 DMRs-HbSS and 3 DMRs the HbSC groups) and after analyses three were validated in the HbSS group, and none in the HbSC group. The gene expression analysis showed differential expression for the PTGFR (-2.97-fold) and GPR56 (3.0-fold) genes in the HbSS group, and for the SPOCK1 (-2.40-fold) and ADCY4 (1.80-fold) genes in the HbSC group. Taken together, these data strongly suggest that SCD (HbSS and HbSC genotypes) can alter placental DNA methylation and lead to gene expression changes. These changes possibly contribute to abnormal placental development and could impact in the clinical course, especially for the fetus, possibly leading to increased risk of abortion, fetal growth restriction (FGR), stillbirth, small for gestational age newborns and prematurity.
2022-09-03 | GSE212603 | GEO
Project description:BackgroundSARS-CoV-2 has infected a large number of pregnant women.ObjectiveTo compare clinical, perinatal outcomes of women with COVID-19 from high-income countries (HICs) and low- to middle-income countries (LMICs).Search strategyOnline databases were searched.Selection criteriaOriginal studies on pregnant women with COVID-19 were included.Data collection and analysisInformation on clinical presentation, co-morbidities, pregnancy outcomes, neonatal outcomes, and SARS-CoV-2 infection in neonates was extracted.Main resultsThe pooled estimate of SARS-CoV-2 positive neonates is 3.7%. Symptomatic presentations are less common in LMICs compared to HICs (odds ratio [OR] 0.38). Diabetes (OR 0.5), hypertension (OR 0.5), and asthma (OR 0.14) are commonly reported from HICs; hypothyroidism (OR 2.2), anemia (OR 3.2), and co-infections (OR 6.0) are commonly reported in LMICs. The overall risk of adverse pregnancy outcomes is higher in LMICs compared to HICs (OR 2.4). Abortion (OR 6.2), stillbirths (OR 2.0), and maternal death (OR 7.8) are more common in LMICs. Preterm births and premature rupture of membranes are comparable in both groups. Neonatal deaths (OR 3.7), pneumonia (OR 7.5), and neonatal SARS-CoV-2 infection (OR 1.8) are commonly reported in LMICs.ConclusionsIn LMICs, pregnant women and neonates are more vulnerable to adverse outcomes due to COVID-19. PROSPERO registration no: CRD42020198743.
| S-EPMC7612435 | biostudies-literature
Project description:BackgroundIn low- and middle-income countries (LMICs) and territories, maternal infections impose a non-negligible disease burden. We aimed to analyse the secular trends, age distribution, and associated factors of maternal sepsis and other maternal infections (MSMI) across 131 LMICs from 1990 to 2019.MethodsWe collected yearly data of incidences, deaths, and disability adjusted life years (DALYs) on MSMI in 131 LMICs from 1990 to 2019 from the Global Burden of Disease 2019 (GBD 2019). The sociodemographic index (SDI) and universal health coverage effective coverage index (UHCI) were also acquired. Relative percent change and estimated annual percentage change (EAPC) were used to assess the secular trends. Correlation analyses were also employed to explore the associations between the burden of MSMI with SDI and UHCI.ResultsBetween 1990 and 2019, the age-standardised incident rates (ASIRs), age-standardised maternal mortality ratios (ASMMRs) and age-standardised DALYs rates of low-income countries (LICs) were much higher than that of lower-middle-income countries (LMCs) and upper-middle income countries (UMCs), although they all continued to decline. At least six of 131 LMICs had ASMMR greater than 70.00 per 100 000 live births in 2019. The incidences of MSMI increased first till 20-24 years and then decreased with age both in 1990 and 2019, while the ASMMRs were higher in the youngest and the oldest age group. With the growth of SDI and UHCI in 2109, the decreasing trend of ASIR, ASMMR, and age-standardised DALYs rates slowed down.ConclusionsAlthough the progress has been made in reducing the burden of MSMI in 131 LMICs, the disease burden in LICs far exceeded that of LMCs and UMCs. Socio-economic status and universal health coverage were both associated with the MSMI burden, and further research is needed to explore the underlying factors contributing to these disparities and to identify effective strategies for reducing the burden of MSMI.
| S-EPMC11377970 | biostudies-literature
Project description:Hydroxyurea is widely used in high-income countries for the management of sickle cell disease (SCD) in children. In Kenyan clinical guidelines, hydroxyurea is only recommended for adults with SCD. Yet many deaths from SCD occur in early childhood, deaths that might be prevented by an effective, disease modifying intervention. The aim of this review was to summarise the available evidence on the efficacy, effectiveness and safety of hydroxyurea in the management of SCD in children below 5 years of age to support guideline development in Kenya. We undertook a systematic review and used the Grading of Recommendations Assessment, Development and Evaluation system to appraise the quality of identified evidence. Overall, available evidence from 1 systematic review (n=26 studies), 2 randomised controlled trials (n=354 children), 14 observational studies and 2 National Institute of Health reports suggest that hydroxyurea may be associated with improved fetal haemoglobin levels, reduced rates of hospitalisation, reduced episodes of acute chest syndrome and decreased frequency of pain events in children with SCD. However, it is associated with adverse events (eg, neutropenia) when high to maximum tolerated doses are used. Evidence is lacking on whether hydroxyurea improves survival if given to young children. Majority of the included studies were of low quality and mainly from high-income countries. Overall, available limited evidence suggests that hydroxyurea may improve morbidity and haematological outcomes in SCD in children aged below 5 years and appears safe in settings able to provide consistent haematological monitoring.
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Project description:Vaccination during pregnancy is a safe and effective intervention to protect women from potentially severe consequences of influenza and reduce risk of influenza and pertussis in their infants. However, coverage remains variable. In this mini-review we update findings from a 2015 systematic review to describe results from recent studies in high income countries on the uptake of influenza and pertussis vaccination in pregnancy, reasons for vaccine hesitancy and barriers to increasing uptake, from maternal and healthcare provider (HCP) perspectives. Studies reported highly variable uptake (from 0% to 78%). A main facilitator for uptake among pregnant women was receiving a recommendation from their HCP. However, studies showed that HCP awareness of guidelines did not consistently translate into them recommending vaccines to pregnant women. Safety concerns are a well-established barrier to uptake/coverage of maternal immunization; 7%-52% of unvaccinated women gave safety concerns as a reason but these were also present in vaccinated women. Knowledge/awareness gaps among pregnant women and lack of confidence among HCPs to discuss vaccination were both important barriers. Several studies indicated that midwives were more likely to express safety concerns than other HCPs, and less likely to recommend vaccination to pregnant women. Women who perceived the risk of infection to be low were less likely to accept vaccination in several studies, along with women with prior vaccine refusal. Findings highlight the importance of further research to explore context-specific barriers to vaccination in pregnancy, which may include lack of vaccine confidence among pregnant woman and HCPs, and policy and structural factors.
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Project description:BackgroundMany high-income countries (HICs) have now vaccinated a substantial proportion of their population against COVID-19. Many low-income countries (LICs) may need to wait until at least 2022 before even the most vulnerable 20% of their populations are vaccinated. Beyond ethical considerations, some redistribution of doses would reduce the risk of the emergence and spread of new variants and benefit the economy, both globally and in donor countries. However, the willingness of HIC governments to donate vaccine doses is likely to depend on public support. While previous work has indicated strong average levels of public support in HIC for donation, little is known about how broad-based this support is.ObjectiveTo investigate the extent to which support for donation holds across both pre-specified and exploratory subgroups.MethodsFrom 24 November-28 December 2020 we conducted an online survey of 8209 members of the general public in seven HIC (Australia, Canada, France, Italy, Spain, UK and USA). We conducted tests of proportions and used Bayesian ordinal logistic regression models to assess the extent of support for donation across population subgroups.ResultsWe found broad-based support for donations in terms of age, gender, socio-economic status and political ideology. We found no strong evidence that support for donations was higher among those with greater income or a university education. Support for donation among those on the political right and centre was lower than on the left, but 51% (95% confidence interval 48-53%) of respondents who identified with the right supported some level of donation. Those in the more altruistic half of the sample (as captured by willingness to donate money to a good cause) were more likely to support donation than those who were not, but around half of the less altruistic group supported some level of donation.ConclusionThere is broad-based support for policymakers in HICs to donate some of their countries' COVID-19 vaccine doses for distribution to LICs.
| S-EPMC8627792 | biostudies-literature
Project description:Organ transplantation has become an effective means to extend lives; however, a major obstacle is the lack of availability of cadaveric organs. India has one of the lowest cadaver organ donation rates in the world. If India could increase the donor rate, the demand for many organs could be met. Evidence from high-income countries suggests that an organ donor registry can be a valuable tool for increasing donor rates. The purpose of this study is to determine whether the implementation of an organ donor registry is a feasible and appropriate policy option to enhance cadaver organ donation rates in a lower-income country.This qualitative policy analysis employs semi-structured interviews with physicians, transplant coordinators, and representatives of organ donation advocacy groups in Mumbai. Interviews were designed to better understand current organ donation procedures and explore key informants' perceptions about Indian government health priorities and the likelihood of an organ donor registry in Mumbai. The 3-i framework (ideas, interests, and institutions) is used to examine how government decisions surrounding organ donation policies are shaped.Findings indicate that organ donation in India is a complex issue due to low public awareness, misperceptions of religious doctrines, the need for family consent, and a nation-wide focus on disease control. Key informants cite social, political, and infrastructural barriers to the implementation of an organ donor registry, including widely held myths about organ donation, competing health priorities, and limited hospital infrastructure.At present, both the central government and Maharashtra state government struggle to balance international pressures to improve overall population health with the desire to also enhance individual health. Implementing an organ donor registry in Mumbai is not a feasible or appropriate policy option in India's current political and social environment, as the barriers, identified through the 3-i framework lens, may be too difficult to overcome. Despite the evidence supporting the use of donor registries as a means to enhance organ donation rates, it is clear that context is critical and that it is not always practical to apply evidence-based policy solutions from high-income countries to lower-income settings.
| S-EPMC4712496 | biostudies-other
Project description:ObjectiveTo explore digital health interventions that have been used to support pregnant women at high risk for pre-eclampsia/eclampsia (HRPE/E) in low-income and middle-income countries (LMICs).DesignScoping review.Data sourceEMBASE, MEDLINE, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews and CINAHL were searched between 1 January 2000 and 20 October 2020.Eligibility criteriaThe review included original research studies that were published in English, involved pregnant women at HRPE/E and implemented digital health interventions for PE/E in LMICs.Data extraction and synthesisTwo reviewers independently completed the data extraction for each of the 19 final articles. An inductive approach was used to thematically organise and summarise the results from the included articles.ResultsA total of 19 publications describing 7 unique studies and 9 different digital health interventions were included. Most studies were conducted in South Asia and sub-Saharan Africa (n=16). Of nine unique digital health interventions, two served the purpose of predicting risk for adverse maternal health outcomes while seven focused on monitoring high-risk pregnant women for PE/E. Both of these purposes used mobile phone applications as interface to facilitate data collection, decision making, and communication between health workers and pregnant women. The review identified key functions of interventions including data collection, prediction of adverse maternal outcomes, integrated diagnostic and clinical decision support, and personal health tracking. The review reported three major outcomes: maternal health outcomes including maternal and neonatal morbidity and mortality (n=4); usability and acceptability including ease-of-use, and perceived usefulness, (n=5); and intervention feasibility and fidelity including accuracy of device, and intervention implementation (n=7).ConclusionAlthough the current evidence base shows some potential for the use of digital health interventions for PE/E, more prospective experimental and longitudinal studies are needed prior to recommending the use of digital health interventions for PE/E.
| S-EPMC8830260 | biostudies-literature