Thanks to everyone who tweeted for our case!
The learning points are summarised below.
The case was based around a nulliparous 27 yr old women with sickle cell disease. She wished to seek prior to conception. Ideally pregnancy’s should be planned to allow discussion regarding sickle cell affects on pregnancy, how pregnancy affects sickle cell disease and to create a management plan to improve outcomes for mother and baby.
Planning also allows the opportunity to screen the partner to allow counselling regarding risks to the foetus of inheriting a haemoglobinopthy.
Prior to pregnancy an assessment of chronic disease complications should be undertaken. This would include:
- echo – screening for pulmonary hypertension
- BP and urinalysis – to identify women with hypertension/proteinuria
- Renal and liver function
- Retinal screening – for signs of proliferative retinopathy
- screening for iron overload. if evidence of iron overload aggressive iron chelation should be undertaken pre-conception
- screening for red cell antibodies – as this may increase the risk of haemolytic disease of the newborn.
Management of a patient during pregnancy with sickle cell disease:
- pencillin prophylaxis
- up to date vaccinations
- folic acid 5mg daily
- STOP hydroxycarbamide 3 months before conception
- ACE-I and angiotensin receptor blockers should be stopped.
- Iron replacement if evidence of deficiency
- low dose aspirin 75mg should be considered from 12 weeks to reduce the risk of pre-eclampsia
- MDT led care
- avoidance of precipating factors of sickle cell crises – extreme temps, dehydration and overexertion. Dehydration can be caused by vomiting during pregnancy – and exerly medical review should be sought by the patient
- during hospital admissions patients should recieve LMWH
USS during pregnancy
- viability scan at 7-9 weeks
- routine first trimester scan
- anomaly scan at 20 weeks
- then every 4 weeks from 24 weeks gestation
Routine prophylactic transfusion is not recommended during pregnancy. Although early studies have recommended transfusion as there was a decrease in maternal morbidty and perinatal mortality, transfusion itself have associated risks including alloimunisation, delayed transfusion reactions, transmission of infection and iron overload.
Top-up transfusion is indicated for acute anaemia, and a level under 6g/dl or a fall of >2g/dl is used as a guide.
Acute exchange transfusion is required if the patient presents with acute chest syndrome or stroke during pregnancy, followed by a transfusion regime.
Blood should be matched for an extended phenotype includign rhesus typing (C,D.E) as well as KELL typing. At delivery – blood should be crossmatched if there are atypical antibodies.
Blood transfusion should be CMV negative during pregnancy.
Labour and delivery
Patients should be offered elective birth by c-section or induction of labour
LMWH should be administered for 7 days following normal vaginal delivery or 6 weeks following c-section
HbSC VS HbSS
HbSC has previously been considered to have a more benign phenotype in pregnancy. However recent studies have confirmed high rates of maternal and fetal morbidity and mortality in both HbSS and HbSC. Both HbSS and HbSC have higher rates of delivery before 34 weeks when compared to national data.
Management of sickle cell disease in pregnancy Green-top guideline no. 61. Royal College of obstetricians and gynaecologists,
Pregnancy outcome in patients with sickle cell disease in the UK–a national cohort study comparing sickle cell anaemia (HbSS) with HbSC disease.