Sickle Cell

Question 1

Inheritance pattern?

Answer 1

Autosomal recessive

Question 2

What occurs in sickle cell disease?

Answer 2

Sicling of red cells in low oxygen conditions causing vast-occlusion in small vessels and cells prone to increased haemolytic breakdown

Question 3

What are risks of sickle cell?

Answer 3

Painful crises
Perinatal mortality
Premature labour
Fetal grwoth restriction

Increased spontaneous miscarriage
Maternal infection
Thromboembolic events
Pre-eclampsia
Antepartum haemorrhage

Question 4

What should you advise pre-conception?

Answer 4

Factors affecting sickling crises: cold, hypoxia, dehydration - hence N/V of pregnancy, over exertion, stress

Pregnancy worsens anaemia so increases risk of crises and acute chest syndrome - chest pain, cough, tachypnoea and new infiltrates on CXR (Treat as for pneumonia + blood transfusion)

Address change of fetus being affected

Daily penicillin or erythromycin 
Update vaccines: Hepatitis B, single does HiB
Meningiococcla C
5-yearly pneumococcal
Annual H1N! with seasonal influena

Stop ACE, ARB, hydroxycarbamide > 3 months preconceptually

5mg folic acid

Question 5

What investigation preconception?

Answer 5

Pregnancy increases risks of incfection
Screen for red cell antibodies (if present - increased risk of haemolytic disease of newborn)
Assess partner’s blood to check if carrier
Assess current disease with echocardiography if not done in last year to exclude pulmonary hypertension
BP
Urinalysis
U&E
LFT
Retinal screening
Iron studies

Question 6

What antenatal care in sickle cell disease?

Answer 6

Aspirin from 12 weeks to reduce risk of pre-eclampsia
TEDs in pregnancy
Heparin thromboprophylaxis if hospitalised
Check BP at all visits and MSU monthly

Viability scan at 7-9 weeks
Dating scan at 11-14 weeks
Anomaly scan at 20 weeks
Growth scan 4 weekly from 24 weeks

Supplement iron if proven deficiency

Question 7

What should you do in a crises?

Answer 7

Admit if fever, severe or atypical pain, chest pain or breathless

Use morphoin/diamorphine if pain needs IV opiates
Give nasal O2 if SaO2<95%
Adequate fluid intake unless pre-eclampsia then specialised.

Question 8

Describe [prstpartum care

Answer 8

38-40 week delivery
Keep warm and hydrated
Continuous monitoring of fetus and maternal saO2
7days of heparin thromboprohpylaxis post delivery, 6 week if Caesarean section