Sickle cell disease in pregnancy: care and risks

Sickle cell disease is an inherited condition of the red blood cells. The NHS explains that the cells can become misshapen, block blood vessels and trigger pain crises, which can be set off by dehydration, sudden temperature changes and stress. Pregnancy places extra demands on the blood and circulation, so these problems can become more frequent.

The RCOG lists more painful crises, sudden worsening anaemia and lung problems, pre-eclampsia, blood clots, early labour and higher caesarean rates for the mother. For the baby, the concerns are poor growth because of reduced placental function, and premature birth.

The RCOG advises telling your sickle cell team about plans for pregnancy, having a yearly review that includes echocardiography, blood pressure, urine and blood tests and retinal screening, and finding out your partner's status. Tommy's explains that when both parents are carriers, each pregnancy has a one in four chance of a child with the disease and a one in four chance of a child with neither the condition nor the trait.

Where a partner is a carrier, specialist genetic counselling is offered, including the option of diagnostic testing in pregnancy. Tommy's notes chorionic villus sampling and amniocentesis carry a risk of miscarriage of about 1 in 200.

• Stop hydroxycarbamide (hydroxyurea) three months before conception, with your haematologist's guidance.

• Start 5mg folic acid daily before pregnancy.

• Update vaccinations, including hepatitis B, flu and pneumococcal vaccines, as the RCOG recommends.

• Ask for a check of organ function if one is due.

In addition to 5mg folic acid, the RCOG recommends continuing daily penicillin to prevent infection and starting low-dose aspirin 75mg from early pregnancy. For pain, paracetamol and codeine are acceptable, while ibuprofen-type medicines are avoided before 12 weeks and after 28 weeks.

The NHS vitamins page confirms the higher folic acid dose for women with sickle cell conditions. Never alter doses without checking with the specialist team.

Care is shared between a haematologist and an obstetric team, usually in a hospital. The RCOG describes appointments every four weeks up to 24 weeks and every one to two weeks after that until delivery, with blood pressure and urine tested each time and extra ultrasound to follow growth.

Screening has an important role. The NHS says antenatal sickle cell screening is arranged through a midwife referral before 10 weeks, while newborn babies have a heel-prick test. If a baby is found to carry the trait or have the disease, follow-up is arranged.

A painful crisis in pregnancy is an emergency. The RCOG describes treatment with strong painkillers, oxygen, intravenous fluids if dehydrated, antibiotics and heparin injections to reduce clot risk, in a monitored setting with checks on the baby's wellbeing. Do not wait at home: go to the maternity unit or A&E and mention your sickle cell diagnosis, and call 999 for chest pain, difficulty breathing, weakness on one side, or severe pain not relieved by your usual plan.

Birth is recommended in a hospital able to manage sickle cell complications. The RCOG advises keeping the mother warm to reduce the chance of a crisis, using fluids and oxygen as needed, having compatible blood available, and continuous fetal monitoring. A vaginal birth is acceptable if there are no complications, and most standard pain-relief options can be used except pethidine.

After the baby is born, warmth, fluids, early mobilisation and heparin injections with compression stockings reduce clot risk. The RCOG says injections are given for at least a week, and six weeks if there are additional risk factors. Breastfeeding is supported, and progesterone-only options, implants and the Mirena coil are suggested contraceptive choices.