SCD: SICKLE CELL DISEASE IN PREGNANCY -BRITISH SOCIETY OF HEMATOLOGY RECOMMENDATIONS

SCD: SICKLE CELL DISEASE IN PREGNANCY -BRITISH SOCIETY OF HEMATOLOGY RECOMMENDATIONS (updating previous RCOG GTG)

SCD Complications 

Hemolytic Anaemia 

Vaso occlusion 

Acute painful crisis

Acute chest syndrome 

Pulmonary hypertension 

Stroke

Renal dysfunction 

Retinal disease

Leg ulcer

Cholelithiasis 

A vascular necrosis Hip complications in pregnancy 

HbSS HbSC- increased risks

Preconception care

Genetic screening 

Annual review from child

Partner testing

High risk couples discuss reproductive options

No intervention, Prenatal diagnosis, PGD

Preconception review of complications 

Prior to pregnancy:

Oxygen saturation 

BP, creat, PCR.RFT

anti hypertensive >130/80

Doppler ECHo(PH,Diastolic dysfunction, early cardiac death) Screening for PH: TRV increase 

Preconception clinics specialist 

Preconception medications review 

Folic acid 5mg NTD prevention

VIT D supplements 

Hyposplenic-Infection (Penicillin prophylaxis young child)

Pregnancy to continue (Pneumonia) -Daily antibiotics prophylaxis 

Vaccination up to date - Pnemococcal Influenza vaccine if not in 5y

ARBs to be stopped ACEi conversion 

Hydroxycarbamide to be discontinued 

Iron chelators stopped when attempting to conceive 

Antenatal hemoglobinopathy screening 

Partner screening must

If partner carrier - counselling regarding affected fetus risk

Enable prenatal diagnosis 

If affected fetus options of MTP

Recommendations

As part of the annual review, all women with SCD should be encouraged to engage with partner testing prior to embarking on pregnancy.

High-risk couples should be counselled prior to pregnancy about their reproductive options: non-intervention, prenatal diagnosis or pre-implantation genetic diagnosis.

Recommendations

Pregnancy and conception should be discussed with women of child-bearing age with SCD as part of their annual review.

Women should be reviewed by a specialist prior to conception to ensure optimisation of health and screening for disease complications.

Preconception clinics should be available and accessible.

Recommendations

Folic acid (5 mg daily) should be given from before conception and throughout pregnancy.

Women should be given vitamin D as per national recommendations for all pregnant women.

Daily antibiotic prophylaxis is recommended.

Vaccinations should be kept updated as per national recommendations for SCD and should include flu vaccination.

When attempting to conceive ARBs should be stopped and there should be a plan for stopping/converting ACEi.

Hydroxycarbamide should be discontinued when attempting to conceive unless the woman is considered to be at high risk of serious complications relating to SCD and blood transfusion is not feasible.

Iron chelators should be stopped when attempting to conceive.

Recommendations

If the woman has not been seen preconceptually, she should be offered partner testing.

If the partner is a carrier, counselling regarding the potential for an affected fetus should take place in the first trimester to enable prenatal diagnosis and if an affected fetus is identified the option of termination should be offered.

Recommendations

A full assessment, as for preconception care, should be repeated as early as possible in the antenatal period, including review of vaccinations and medications, organ damage and red cell alloantibodies.

Antenatal care should be provided by a multidisciplinary team including an obstetrician and midwife with experience of high-risk antenatal care and a haematologist with links to a Specialised Haemoglobinopathy Team.

Regular antenatal appointments for women with SCD should provide routine antenatal care as well as care specifically for women with SCD.

Women with persistent vomiting should be advised to seek medical advice early.

Women should be reminded to take daily folic acid (5 mg) and prophylactic antibiotics (if not contraindicated) and avoid drugs that are unsafe in pregnancy.

Iron supplementation should be given if there is laboratory evidence of iron deficiency.

Women with SCD should be considered for low-dose aspirin 75–150 mg once daily from 12 weeks of gestation in an effort to reduce the risk of developing preeclampsia and should be monitored for blood pressure rises and proteinuria.

Aspirin prescription should be reviewed at 36 weeks of gestation to consider stopping prior to delivery.

Recommendations

Women should be offered serial fetal biometry scans (growth scans) every four weeks from 24 weeks of gestation.

Recommendations

If transfusion is needed, pregnant women with SCD should be given ABO-compatible, extended Rh- and Kell-matched, CMV-negative units. If there are clinically significant red cell antibodies (current or historical) then the red cells selected should be negative for the corresponding antigens.

The risks and benefits of prophylactic transfusion during pregnancy should be discussed with the patients as part of the haematology/obstetric consultation.

 Prophylactic transfusion is not routinely recommended for sickle pregnancy, but should be considered for women with: 

a. Previous or current medical, obstetric or fetal problems, related to SCD. 

b. Women previously on hydroxycarbamide due to severe disease.

c. Multiple pregnancy.

Women receiving long-term transfusions for stroke prevention or for the amelioration of severe sickle complications should continue with regular transfusions throughout pregnancy.

Transfusion may be required in women with worsening anaemia.

Transfusion should be considered for those with acute SCD complications (e.g., acute chest syndrome, stroke)

Recommendations

Pregnant women with SCD should have an agreed pain management plan for the treatment of acute painful crisis.

If pregnant women are admitted with acute pain crises they should be looked after by the MDT.

The NICE guidelines58 on the management of acute painful episodes should be followed.

NSAIDs should be used with caution in the first trimester and avoided after 31 weeks of gestation.

Fluid and oxygen balance should be monitored regularly in women admitted with sickle pain crisis.

Women with SCD should be prescribed prophylactic low-molecular-weight heparin during any antenatal hospital admission.

Recommendations 

Patients with SCD can present with acute chest syndrome (ACS) or it may develop after the onset of severe pain. Therefore vigilance should be maintained throughout hospital admission.

All hospitals should have a treatment pathway for ACS which should include a referral pathway to the high dependency unit or intensive care unit.

Antibiotics, with cover for atypical organisms, should be used even if blood cultures and sputum cultures are negative.

Simple (‘top-up’) transfusion should be considered early in the hypoxic patient but exchange transfusion is necessary if there are severe clinical features or evidence of progression despite initial simple transfusion.

Recommendations

Acute stroke should be considered in women presenting with acute neurological impairment and requires urgent consideration of exchange transfusion.

Acute erythrovirus infection should be considered in women presenting with acute anaemia.

Recommendations: (adapted from RCOG Guideline 37a60)

All women with SCD should have risk assessments performed in early pregnancy, if admitted to hospital, in the intrapartum and early postpartum period.

Women with SCD should be considered for prophylactic low-weight heparin (LMWH) from 28 weeks of pregnancy until six weeks postpartum and if women have additional risk factors, prophylaxis should start from the beginning of pregnancy.

Women admitted to hospital with a vaso-occlusive crisis or for other reasons should be offered LMWH throughout their admission unless there are contraindications.

Recommendation

Pregnant women with SCD who have a normally growing baby should be delivered between 38 and 40 weeks of gestation.

Recommendations

Women with SCD can be offered vaginal delivery and vaginal birth after previous caesarean (VBAC) if there are no other contraindications.

In women who have hip replacements (because of avascular necrosis) suitable positions for delivery should be discussed prior to delivery.

Recommendations

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.

The relevant MDT (senior midwife in charge, senior obstetrician, anaesthetist and haematologist) should be informed as soon as labour is confirmed.

Blood should be cross-matched for delivery if there are atypical antibodies present (since this may delay the availability of blood).

Women should be kept warm and given adequate fluid during labour, using a fluid balance chart to avoid fluid overload.

Continuous intrapartum electronic fetal heart rate monitoring is recommended owing to the increased risk of fetal distress, which may necessitate operative delivery.

Women with SCD should be offered anaesthetic assessment in the third trimester of pregnancy.

Opiates may be used for analgesia, except for pethidine.

Regional analgesia is recommended for caesarean section.

Recommendations

Women should be advised that they have an increased risk of pain episodes in the postnatal period and precipitants should be avoided.

Women should receive thromboprophylaxis with LMWH for six weeks after delivery.

Recommendations

Contraceptive advice should be given and conveyed to the woman’s primary care team.

The choice of contraception should be individualised but methods that eliminate user failure, such as LNG IUS and intramuscular DMPA are preferred .

There is some evidence for reduction in sickle pain associated with progesterone-only preparations.

CHCs are an option for women with SCD but cardio

vascular risk factors should be minimised to mitigate potential risk.

Reference:

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

Eugene Oteng-Ntim,1,2  Sue Pavord,3  Richard Howard,4 Susan Robinson,1 Laura Oakley,5,6 Lucy Mackillop,3 Shivan Pancham,7 Jo Howard8,9  on behalf of the British Society for Haematology Guidelines Committee

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