Pregnancy in sickle cell disease

Powars, Darleen R.; Sandhu, Meenu; Niland-Weiss, Joyce; Johnson, Cory; Bruce, Samuel L.; Manning, Phil R.

doi:10.1016/0020-7292(87)90269-4

Cited by 29 publications

(5 citation statements)

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“…Such figures are difficult to interpret because the improving overall survival may have allowed women with intrinsically more severe disease to become pregnant. It is hardly surprising that maternal and fetal outcomes are influenced by socio‐economic conditions, 16,29,30 but the mortality rate of 7% in the present study is difficult to interpret against this background as although the per capita income of Jamaica is relatively low, the country has excellent public health and preventive medicine standards.…”

Section: Discussionmentioning

confidence: 63%

“…Reported maternal mortality rates in SS disease have varied widely but are generally higher in low‐ and middle‐income countries, rates of 4.3–12.9% being reported from Nigeria, 17,25–27 and 11.4% in Tanzania, 28 the latter attributing 82% of maternal deaths to infection. Maternal deaths in the USA fell from 4.1 to 1.7% at the same institution before and after 1972 29 and two meta‐analyses found that rates fell with better socio‐economic status 15,16 . This mortality may be reduced by better or more comprehensive antenatal care in some settings, 4,27,30 although a program addressing maternal mortality by the Jamaican Ministry of Health doubled the number of antenatal clinic visits in SS mothers but did not affect mortality, 31 and three of the five deaths in the current study occurred despite close monitoring by obstetricians experienced in sickle cell disease.…”

Section: Discussionmentioning

confidence: 97%

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Pregnancy outcome in homozygous sickle cell disease: observations from the Jamaican Birth Cohort

Lewis

Thame

Howitt

et al. 2021

BJOG

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Objective To document pregnancy outcome in homozygous sickle cell (SS) disease and in age‐matched controls with a normal haemoglobin genotype followed from birth for up to 45 years. Methods A total of 100 000 consecutive non‐operative deliveries screened for sickle cell disease at the main Government maternity hospital in Kingston, Jamaica between 1973 and 1981 detected 311 (149 female) babies with SS disease who were matched by age and gender with 250 (129 female) controls with an AA haemoglobin phenotype. These individuals have been followed from birth with prospective assessment of menarche and detailed documentation of all pregnancies. Results There were 177 pregnancies in 71 SS patients and 226 pregnancies in 74 AA controls. Mothers with SS disease had more spontaneous abortions (adjusted relative risk [aRR] 3.2, 95% CI 1.6–6.1), fewer live births (aRR 0.7, 95% CI 0.6–0.9) and their offspring were more likely to have a gestational age <37 weeks (aRR 2.1, 95% CI 1.1–3.7) and low birthweight <2.5 kg (aRR 3.0, 95% CI 1.6–5.3). They were more prone to acute chest syndrome (aRR 13.7, 95% CI 4.1–45.5), urinary tract infection (aRR 12.8, 95% CI 1.3–125.9), pre‐eclampsia/eclampsia (aRR 3.1, 95% CI 1.1–8.8), retained placenta (aRR 10.1, 95% CI 1.1–90.3), sepsis (Fisher's Exact test 0.04) and pregnancy‐related deaths (Fisher's Exact test 0.02). Four of five deaths were attributable to acute chest syndrome. There was no genotypic difference in pregnancy‐induced hypertension or postpartum haemorrhage. Conclusion Pregnancy in SS disease carries risks for both mother and child. The variable characteristics of pregnancy‐related deaths complicate their prevention. Tweetable abstract Pregnancy in SS disease compared with controls showed increased abortions and stillbirths, fewer live births and maternal deaths in 7% patients.

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Section: Discussionmentioning

confidence: 63%

Section: Discussionmentioning

confidence: 97%

Pregnancy outcome in homozygous sickle cell disease: observations from the Jamaican Birth Cohort

Lewis

Thame

Howitt

et al. 2021

BJOG

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“…Pregnancy is associated with an increased incidence of acute painful crises, [52][53][54][55][56] in both HbSS and to a lesser extent HbSC disease. 7 In a prospective UK study, acute pain was the most common complication, affecting 57% of pregnant women with SCD.…”

Section: Management Of Acute Pain Episodes During Pregnancymentioning

confidence: 99%

Management of sickle cell disease in pregnancy. A British Society for Haematology Guideline

et al. 2021

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This guideline was compiled according to the British Society of Haematology (BSH) process at https://b-s-h.org.uk/guide lines/proposing-and-writing-a-new-bsh-guideline/. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) nomenclature was used to evaluate levels of evidence and to assess the strength of recommendations. The GRADE criteria can be found at http://www.grade workinggroup.org Literature review detailsThis BSH guideline was developed and updated from a previous Royal College of Obstetricians and Gynaecologists (RCOG) Green-top guideline 1 in accordance with the standard method of producing BSH guidelines. Medline, Embase, the Cochrane Database of Systematic Reviews, the Cochrane Control Register of Controlled Trials (CONTROL), the Database of Abstracts of Reviews and Effects (DARE), the ACP Journal Club and the Ovid database were searched for relevant randomised controlled trials, systematic reviews and meta-analyses between 2000 and August 2018. In all, 218 papers were identified. Search terms included: 'sickle cell', 'hydroxycarbamide', 'antenatal', 'pregnancy', 'intrapartum', 'penicillin prophylaxis', 'ACE inhibitor', 'transfusion', 'ultrasound', 'Doppler', 'echocardiogram', 'anti-coagulation', 'prophylaxis', 'sickle cell and risk factors', 'preconceptual' and 'sickle cell crisis' and included all relevant Medical Subject Headings (MeSH) terms and subheadings. The search was limited to humans and the English language. The National Library for Health and the National Guidelines Clearing House were also searched for relevant guidelines. Review of the manuscriptReview of the manuscript was performed by the BSH Guidelines Committee, General Haematology Task Force, the BSH Guidelines Committee and the members of the sounding board of BSH. It was also placed on the members' section of the BSH website for comment. It has also been reviewed by the Royal College of Obstetricians and Gynaecologists, Sickle Cell Society and BSH Obstetric Haematology Special Interest Group; these organisations do not necessarily approve or endorse the contents.

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“…1 The risk varies in different geographical areas. Very high maternal mortality rates of 11.5% have been reported from West Africa and from black American groups.…”

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confidence: 99%

Pregnancy in Sickle Cell Disease in the Al Hassa Region of Saudi Arabia

2000

Ann Saudi Med

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Pregnancy in sickle cell disease (SCD) patients is associated with increased risk to both mother and fetus. 1 The risk varies in different geographical areas. Very high maternal mortality rates of 11.5% have been reported from West Africa and from black American groups.2 These high mortality rates have led to suggestions by clinicians that pregnancy in SCD patients is contraindicated and have advised therapeutic abortion and sterilization. [3][4][5] In the Eastern Province of Saudi Arabia, relatively mild manifestations of SCD have been noted, as compared to the SCD on the African continent. 6 The present study was undertaken to assess the outcome of pregnancy among SCD patients in this region. Subjects and MethodsDuring the year 1997-1998, the medical records of patients admitted at the Obstetric and Gynecology Department of King Fahad Hospital, Hofuf, were retrospectively reviewed. The records of 61 SCD patients were analyzed. The criteria for SCD were based on absence of HbA 1 , HbS of more than 40%, HbA 2 of less than 3.5% and presence of HbF. These were compared with 53 sickle cell trait (SCT) patients. The criteria for SCT were based on presence of high HbA 1 , HbA 2 of less than 3.5% and HbS of less than 40%. Red cell indices in both groups showed normocytic normochromic anemia. Association of β-thalassaemia with SCD and SCT was excluded on the basis of red cell indices and HbA 2 concentration of less than 3.5%. Glucose-6-phosphate dehydrogenase (G6PD) estimation was not carried out in most of the patients, hence the association of G6PD with SCD and SCT could not be correlated. Both SCD and SCT groups were cross-matched with 84 randomly selected control patients admitted during the same period to compare the morbidity in these groups. Age, gravidity, parity, birth weight of fetus, mode of delivery, sickle cell crisis, blood transfusion and sickle cell hemoglobin percentage were recorded. Statistical analysis was done by using an Epi-Info software. Hemoglobin electrophoresis was. done on cellulose acetate in alkaline buffer, using Helena's Electrophoresis System and staining by Ponceus stain. Scanning with Clinician 2 (Helena Laboratories Densitometer) was used for quantitative evaluation of different hemoglobin bands. Hemoglobin electrophoresis which was carried out without history of blood transfusion during the previous four months was taken into consideration. ResultsIn the SCD group, the mean age was 26.3 years, mean gravidity was 4.0, and the mean parity was 3.7. The average birth weight of the fetus was 2856 g. In this group, 12 babies (19.6%) had low birth weight (less than 2500 g), 12 patients (19.6%) had fetal distress, and 31 patients (50.8%) had sickle cell crisis. There were 48 episodes of crisis (average 1.5 episodes per patient) during the pregnancies of these patients. The crises were precipitated by urinary tract infection (24 cases), respiratory tract infection (14), sore throat (6) and septicemia (4). E. coli, Kleβsiella spp. and group B Streptococcus were the most common bacteria respon...

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Pregnancy in sickle cell disease

Cited by 29 publications

References 0 publications

Pregnancy outcome in homozygous sickle cell disease: observations from the Jamaican Birth Cohort

Pregnancy outcome in homozygous sickle cell disease: observations from the Jamaican Birth Cohort

Management of sickle cell disease in pregnancy. A British Society for Haematology Guideline

Pregnancy in Sickle Cell Disease in the Al Hassa Region of Saudi Arabia

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