Sickle-cell Disease in Pregnancy

Sickle-cell disease (SCD) is caused by a group of hemoglobin disorders (single gene recessive) which predispose to ‘sickling’ of red cells in low oxygen conditions causing vaso-occlusion in small vessels, and cells prone to increased haemolytic breakdown.
Sickle cell disease is most prevalent in those of African descent but it is also prevalent in the Caribbean, Middle East, Mediterranean, parts of India, South and Central America.

Disease complications include 

• painful crises, 
• stroke,
• pulmonary hypertension,
• renal dysfunction, 
• leg ulcers, 
• retinal disease, 
• avascular necrosis (eg of hip).

Pregnancy complications include

• maternal painful crises,
• prematurity 
• fetal growth restriction. 
• increased chances of maternal infection, 
• thromboembolic events and 
• pre-eclampsia.

Preconception Care 

• Women with SCD should be under annual clinic review to monitor disease. Arrange hematology specialist for a preconception review. 
• Advice should cover factors affecting sickling crises (cold, hypoxia, dehydration-hence nausea and vomiting of pregnancy, over-exertion, stress). 
• Pregnancy worsens anemia, so there is increased risk of crises and acute chest syndrome (ACS)-chest pain, cough, tachypnoea and new infiltrates on Chest X Ray. It should be treat as for pneumonia along with blood transfusion.
• Screen for red cell antibodies (if present there is increased risk of haemolytic disease of newborn). Pregnancy increases the risks of infection (especially UTI). 
• Address the increased  risk of fetal growth restriction (hence the need for induction, or c-section). 
• Address chance of fetus being affected (partner’s blood to check carrier/haemoglobinopathy status: genetic counselling if needed). 
• Assess current disease: echocardiography if not done in last year to exclude pulmonary hypertension ; BP & urinalysis, U&E, LFT; retinal screening (proliferative retinopathy common); screen for iron overload if multiply transfused (if significantly overloaded, preconception chelation therapy is advised). 
• SCD is a hyposplenic state; advise daily penicillin or erythromycin and update vaccines: hepatitis B, single dose haemophilus influenza B & meningococcal C, 5-yearly pneumococcal, and annual H1N1 with seasonal influenza.
• Stop ACE inhibitors & hydroxycarbamide ≥3 months), preconceptually. 
• Give 5 mg folic acid daily preconceptually .

Antenatal Care 

• Ensure preconception measures addressed. 
• Manage by specialist multidisciplinary team. 
• If fetus has haemoglobinopathy risk, offer prenatal testing by 8–10 weeks. 
• From 12 weeks give 75 mg aspirin daily to reduce risk of developing preeclampsia.
• Suggest graduated compression stockings in pregnancy. 
• If hospitalized, give heparin thromboprophylaxis. 
• Check BP at all antenatal visits.
• Offer viability scan at 7–9 weeks, dating scan at 11–14, anomaly scan at 20, and growth scan 4-weekly from 24 weeks. 
• Supplement iron is given only if there is a proven deficiency. 
• Blood transfusion is not routinely required.

In Case of Sickle cell Crises: Crises affect 27–50%. 

• Admit if fever, severe or atypical pain, chest pain, or breathless. 
• If severe pain and there is needs of IV opiates use morphine/diamorphine (not pethidine, it risks fits);
• Give nasal O2 if oxygen sats <95% , and
• Adequate fluid intake 60mL/kg/h PO/IV . 
• Exchange transfusion is needed for acute chest syndrome or if stroke.
• If there is associated pre eclapmpsia it should be treated accordingly under specialist care in ICU. 

Intrapartum Care 

• Aim for delivery at 38 weeks at hospitals able to manage SCD and high-risk pregnancy. 
• Keep warm and hydrated in labour/post-partum.
• Monitor fetus, and maternal O2 sats. 
• Give 7 days heparin thromboprophylaxis post vaginal delivery, 6 weeks if caesarean.
• Progestogenic contraception is that of choice.