Sickle cell disease and pregnancy Flashcards
What is sickle cell disease?
Single gene autosomal recessive disorder which affects haemoglobin structure (N.B. thalassaemia affects haemoglobin synthesis)
What end organ damage can sickle cell disease cause?
Brain - stroke
Eyes - proliferative retinopathy
Lungs - pulmonary hypertension
Liver - failure
Renal - failure
Spleen - hyposplenism (all HbSS treated as hyposplenic)
If iron overload (less common in HbSS than beta thal major - then can also cause heart failure)
What maternal risks are associated with sickle cell disease and pregnancy?
Increased frequency of painful crises and acute chest crisis
Increased risk of UTI, hypertension, pre-eclampsia
Increased risk of preterm labour, emergency c-section
What fetal risks are associated with sickle cell disease and pregnancy?
Fetal growth restriction
Fetal and neonatal haemolysis - if autoantibodies
Inheritance of sickle cell disease
What assessments should be included in pre-pregnancy counselling?
Eyes - assessment of proliferative retinopathy
Lungs - assessment by echocardiography for pulmonary hypertension
Liver and renal function tests
Blood pressure
If suspicion of iron overload - MRI T2* cardiac and liver as per beta thalassaemia
Genetic screening in partner
Which medications should a patient with sickle cell disease be taking in pregnancy?
Hyposplenism - penicillin prophylaxis (or erythromycin if pen allergic)
PET prevention - aspirin from 12/40
folic acid deficiency (haemolysis) - folic acid 5mg
VTE prophylaxis - SCD is a mild risk factor
Stop - hydroxycarbamide, stop Ace inhibitors
How should a painful crisis be managed?
Rule out other causes FBC, reticulocyte count, CRP, U&E Hydration Analgesia - WHO step ladder (no pethidine) LMWH if admitted
How should an acute chest crisis be managed?
Chest xray, FBC, reticulocyte count, CRP, U&E +/- ABG
Oxygen, antibiotics
If Hb <65 - transfuse
If hypoxic and Hb higher - consider exchange transfusion
What are the indications for transfusion in sickle cell disease and pregnancy?
Top up for acute anaemia
Exchange transfusion for stoke and acute chest crisis
If on a regular transfusion plan pre pregnancy e.g. to prevent stroke - this should be continued
If iron deficient oral iron replacement can be considered
When should women with sickle cell disease be advised to deliver?
38-40 weeks
Which pain relief can not be given to patients with sickle cell disease?
pethidine (increases the seizure threshold)
When can NSAIDs be given for analgesia?
Postnatally and 12-28 weeks
How should pregnant women with sickle cell disease be managed in the postpartum?
Continue to avoid precipitants of crises (cold, stress, exertion, hypoxia)
LMWH
Encourage breastfeeding
Contraception - progesterone first line but cocp not contraindicated
Baby - genetic testing
