Planning a pregnancy with sickle cell disease: what to do before, during and after

Our March 30 Cells meeting, “Clinical Conversations: OB-GYN,” inspired this guide. It covers pregnancy with sickle cell disease from planning to the weeks after birth, plus what to know if you carry the trait.

Why pregnancy with sickle cell is considered high risk

Canadian guidance lists more frequent pain crises, infections, lung complications and blood clots during pregnancy with sickle cell, and a higher risk of pre-eclampsia, a serious blood pressure condition. Babies are more likely to grow slowly or be born early. None of this rules pregnancy out, but it does mean planning with the right team from the start.

Build your team before you try

Ask your hematologist for a pre-pregnancy visit. Canadian guidance recommends care from a high-risk obstetrics team, often called maternal-fetal medicine, working with hematology, anesthesia and newborn specialists, and a birth in a centre equipped for close monitoring. Aim to conceive when your sickle cell is relatively stable, and use the months before to:

• Review every medicine you take, including pain medicines
• Bring your vaccines up to date, including your yearly flu shot
• Ask how much folic acid you need; it is often more than a prenatal vitamin contains
• Complete baseline tests, such as blood work, an eye exam and a heart ultrasound

Talk about hydroxyurea and other medicines first

Hydroxyurea is not recommended during pregnancy or breastfeeding. Canadian guidance advises stopping it before conception, and research on the safest timing continues. Plan the change with your hematologist instead of stopping on your own, and ask what will protect your health in the meantime, which for some people means regular transfusions.

Review other medicines too. Iron chelation is usually paused, and anti-inflammatories such as ibuprofen are avoided at certain stages. If you need opioid pain medicine, your team can plan for it, including checking on your baby after birth. Men living with sickle cell who take hydroxyurea should also talk to their hematologist before trying to conceive.

Know your partner’s status

Your partner’s genes shape your child’s chances. If you live with sickle cell disease and your partner carries sickle cell trait or another hemoglobin trait, each pregnancy has a 1 in 2 chance of a child with sickle cell disease. If your partner carries no trait, your child will carry a trait but not the disease.

Ask for a full hemoglobin test for your partner, such as a hemoglobin electrophoresis, ideally before pregnancy; Canadian guidance warns that quick solubility tests can miss traits. Get screened explains how to ask. If your partner carries a trait, ask for genetic counselling to understand your options, which may include testing during pregnancy or IVF with embryo testing.

What extra monitoring can look like

Expect more appointments than usual, and a birth plan focused on keeping you warm, hydrated and comfortable, with good pain relief. Monitoring varies, but often includes:

• Regular blood counts to track anemia
• Urine tests, since urinary infections may trigger a crisis
• Blood pressure checks for signs of pre-eclampsia
• Ultrasounds to follow your baby’s growth
• Transfusions in some situations, decided case by case

Warning signs: get care right away

Some symptoms cannot wait. Call your care team or go to the nearest emergency department if you have:

• A fever
• Chest pain, a new cough or trouble breathing
• Pain that is worse than usual or not easing
• A severe headache, vision changes or swelling in your face and hands
• Pain and swelling in one leg
• Vaginal bleeding, leaking fluid or contractions before 37 weeks
• Your baby moving less than usual

After your baby arrives

Blood clot risk stays higher after birth, so ask whether you need preventive treatment. Before you go home, ask when to restart your usual medicines, which are safe while breastfeeding, and how your baby will be tested for sickle cell. Talk about contraception and spacing future pregnancies, too.

New parenthood is a lot to carry alongside pain and broken sleep. Cells connects you with warriors and caregivers who understand, and registered participants in Canada who attend at least one session a quarter can access free therapy.

If you have sickle cell trait

Sickle cell trait is not sickle cell disease, and carriers usually have no symptoms. Tell your prenatal team you carry it, and test your partner: if you both carry the trait, each pregnancy has a 1 in 4 chance of a child with sickle cell disease. Genetic counselling can explain your options.

Questions people ask

This article is general information, not medical advice. Talk to your own hematology team about your care. In an emergency, call 911.