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

Abstract

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.

Perinatal outcomes in women with sickle cell disease: a matched cohort study from London, UK

“…The GSST is a large tertiary referral hospital in London, UK, serving a diverse deprived inner‐city population. The hospital has been running a specialist sickle‐obstetric service since 2005, and the service leads have been closely involved in the development of the UK guidelines on SCD in pregnancy 6,7 . Over the study period, care for pregnant women with SCD at this centre was closely aligned to the 2011 UK RCOG guideline 6 .…”

“…The hospital has been running a specialist sickle-obstetric service since 2005, and the service leads have been closely involved in the development of the UK guidelines on SCD in pregnancy. 6,7 Over the study period, care for pregnant women with SCD at this centre was closely aligned to the 2011 UK RCOG guideline. 6 Briefly, standard management includes daily aspirin (75-150 mg) from pregnancy confirmation until 36 weeks of pregnancy, and prophylactic anticoagulation as per the RCOG guideline.…”

The management of haemoglobinopathies in pregnancy and childbirth

“…Guidelines should be in place to aid management of painful crisis with most care resembling that outside pregnancy, although nonsteroidal anti-inflammatory drugs should be used with caution before 12 weeks and avoided after 31 weeks of gestation. 41 Table 4 summarises other potential acute presentations in women with SCD.…”

“…41 Women with SCD should be advised to give birth in hospitals that are able to manage both the complications of SCD and high-risk pregnancies. 41 The mode of delivery should be guided by obstetric indications and that, without contraindications, women should be encouraged to aim for a normal vaginal delivery. 24,41 For SCD patients requiring caesarean section, routine transfusion is not recommended, but should be considered if haemoglobin levels are <70 g/L or if the haemoglobin falls more than 20 g/L below baseline.…”

“…41 The mode of delivery should be guided by obstetric indications and that, without contraindications, women should be encouraged to aim for a normal vaginal delivery. 24,41 For SCD patients requiring caesarean section, routine transfusion is not recommended, but should be considered if haemoglobin levels are <70 g/L or if the haemoglobin falls more than 20 g/L below baseline. 24 During the intrapartum period, women with SCD are at increased risk of painful crisis or ACS, especially if labour becomes prolonged (more than 12 hours).…”