Sickle cell disease

Question 1

Pathophysiology of sickle cell disease

Answer 1

polymerisation of abnormal sickle haemoglobin in low oxygen conditions leading to the formation of rigid and fragile sickle shaped red cells.
These are prone to increased breakdown which causes haemolytic anaemia and vaso-occlusion of small vessels

Question 2

Consequences of sickle cell disease

Answer 2

acute painful crisis, acute chest syndrome, pulmonary hypertension, stroke, renal dysfunction, retinal disease, leg ulcers

Question 3

Fetal consequences of sickle cell disease?

Answer 3

increased chance of pre-term birth, low birth weight

Question 4

What medications should be commenced in women with sickle cell disease who are trying to conceive?

Answer 4

Folic acid 5mg
Vitamin D
Daily antibiotic prophylaxis
Vaccinations
Aspirin from 12-36 weeks

Question 5

Which medications should be stopped in women with sickle cell disease who are trying to conceive?

Answer 5

ARBs, ACEi
Hydroxycarbamide
Iron chelators

Question 6

Fetal monitoring in sickle cell disease

Answer 6

Fetal biometry scans from 24 weeks, 4 weekly

Question 7

Delivery plan for women with sickle cell disease

Answer 7

between 38 and 40 weeks

Question 8

Considerations for women with sickle cell disease in labour?

Answer 8

Keep warm, adequate fluids and fluid balance charting
Continuous fetal monitoring
Opiates for analgesia, but not pethidine