Sickle Cell Disease in Pregnancy

Trait and disease are not the same conversation

RCOG's patient information is clear on this and it is the first thing to establish. If you have two sickle genes, one from each parent, you have sickle cell disease. If you have one, you are a carrier, and that is sickle cell trait.

On trait, RCOG says directly that carriers "do not usually experience symptoms", and that pregnancy is not affected, but that you can pass the condition on if your partner is also a carrier or has the disease. That is the whole of it for trait. Everything else on this page is about the disease.

For scale, RCOG states that between 12,000 and 15,000 people in the UK have sickle cell disease and over 300 babies are born with the condition here each year. The NHS Sickle Cell and Thalassaemia Screening Programme offers screening in pregnancy and newborn screening in the first week of life.

Before pregnancy: the medicine that has to stop

RCOG advises that if you are taking hydroxycarbamide, also called hydroxyurea, you should stop it and continue using contraception for 3 months before you become pregnant, and that if you become pregnant while taking it, it should be stopped as soon as you know. RCOG adds a reassurance worth repeating: "stopping it should not affect your health".

The annual review RCOG describes should already include an echocardiogram, blood pressure, urine and blood tests, and retinal screening. If you have not had those in the last year, they are done in early pregnancy. Vaccinations against hepatitis B, influenza and pneumococcus should be up to date, and RCOG notes these are safe in pregnancy.

Because sickle cell disease increases infection risk, daily preventive antibiotics are usual, and higher-strength folic acid is prescribed. Both are named here without doses; they are prescribed by your haematology team.

What the pregnancy schedule looks like

RCOG sets out a specific appointment pattern that is far more intensive than routine care: you should be seen at the antenatal clinic "at least every 4 weeks until your 24th week, and then every 1-2 weeks until you have had your baby". At each visit blood pressure and urine are checked, and beyond the routine scans you should have extra scans to check that your baby is growing normally.

Your team should include an obstetrician, a midwife and a haematologist with expertise in sickle cell disease. The British Society for Haematology guideline on the management of sickle cell disease in pregnancy is the current UK professional guidance behind that care, and it covers preconception screening and antenatal, intrapartum and postnatal management. It replaced the older RCOG Green-top Guideline on this topic, which has been archived.

The risks, stated plainly

RCOG's summary is that most women with sickle cell disease "will have a straightforward pregnancy and not have serious problems", while listing what can go wrong. Painful crises can be more common in pregnancy, and cold, dehydration and overexertion can bring them on. Sudden anaemia and lung problems can occur. Venous thrombosis risk, already raised by pregnancy, is raised further by sickle cell disease. Pre-eclampsia is more likely later in pregnancy. And because the condition affects placental function, babies are more likely to be small, so growth is watched by scan.

RCOG also says you are more likely to go into labour early, and that if you do not, induction before your due date is likely to be advised to reduce risks, with a higher chance of caesarean birth. Note the wording: more likely, not certain.

If you have a crisis

RCOG's instruction is to contact your maternity unit as soon as possible so you can be seen urgently. The treatment it describes is strong pain relief, oxygen, fluids through a drip if you are dehydrated, checks for other causes, antibiotics if indicated, and heparin injections to reduce clot risk, with close monitoring often in a high-dependency area and checks on your baby's wellbeing.

The practical preparation is to have the contact number for your maternity unit saved before you need it, and to know that morning sickness matters more for you than for most people because dehydration is a crisis trigger. Persistent vomiting is a reason to be seen, not to wait it out.

Labour, birth and pain relief

RCOG advises giving birth in a hospital able to manage sickle cell complications, being kept warm to reduce the risk of a crisis in labour, fluids to prevent dehydration, and compatible blood being available in case transfusion is needed, with close monitoring of your baby's heartbeat. Vaginal birth should be possible if there are no complications.

On pain relief, RCOG says you should see an anaesthetist before labour and that all the usual methods should be suitable except pethidine, "as it could cause complications". That single exception is worth having written in your notes.

NICE guideline NG121, which covers intrapartum care for women with existing medical conditions, sets the general expectation that this plan is agreed in advance by a multidisciplinary team rather than assembled during labour.

After birth, and your baby

RCOG advises keeping warm and well hydrated, extra oxygen if needed, early mobilisation, compression stockings and daily heparin injections for at least a week, continuing for 6 weeks where other risk factors such as caesarean birth apply. Breastfeeding is recommended and supported.

Your baby will be offered newborn screening for sickle cell disease as part of the heel prick blood spot test at around day 5. NICE CKS on sickle cell disease and the NHS pages describe the condition and its lifelong management, which is the context your baby's result sits in if it is positive.