Women with Sickle Cell Disease

Sickle cell disease (SCD) is a disorder due to inheritance of an abnormal hemoglobin gene. This gene is not on the sex chromosome. This means there is no sex-related difference in the rate of SCD.

However, SCD can cause unique problems in women. Along with the major complications of SCD, women may also have delayed puberty and more pain crises during their period. SCD can also cause problems getting pregnant, pain during sex, and complications during pregnancy.

How does sickle cell disease affect puberty?

Girls who have sickle cell anemia (HbSS) may get their period about 2 years later than girls who do not have SCD. Milder types of SCD, such as HbSC, may only cause a 6-month delay.1

Also, girls with HbSS or HbS beta zero thalassemia usually weigh less and are less sexually mature than girls with HbSC or HbS beta plus thalassemia.1

Are periods affected?

Many women with SCD experience more pain crises just before and during their period. The frequency of pain crises is also higher during reproductive age. This means that hormone changes may play a role in pain crises.1

Heavy menstrual bleeding may be overlooked in women with SCD. This can lead to iron deficiency anemia. Doctors may prescribe birth control or hormone injections to reduce heavy bleeding or pain during menstruation. However, some birth control pills increase the risk of stroke for women with SCD.1,2

How does SCD affect sexual health?

Women with SCD are more likely to experience pain during sex (dyspareunia). Pain during sex is more common for women who have a history of chronic pain or who experience more frequent pain crises. It may be caused by pain drugs that affect hormone levels. Pain during sex can affect women’s:3

  • Body image
  • Personal relationships
  • Physical exercise
  • Sexual activity

Women with SCD can use normal birth control methods. Many doctors do not recommend combination hormonal birth control because it increases the risk of blood clots and stroke. Some women with SCD also report that pain crises happen more often with combination hormonal birth control. Birth control that uses only one type of hormone, like progesterone, may lower risk and control pain.2

How is pregnancy affected by SCD?

Complications from SCD may make it more difficult to get pregnant. Some common treatments for SCD and pain medicines may also affect fertility. This includes hydroxyurea, blood transfusions, opioids, and NSAIDs.4

If you have SCD and want to have a child, talk to your doctor about how to prepare. If you or the baby’s father are unsure about your type of SCD, it is good to get tested. This can help assess the baby’s risk of inheriting each type of SCD. For example:5

  • If you and the father both have SCD, the baby will also have it
  • If you have SCD and the father has sickle cell trait, there is a 50 percent chance the baby will have SCD
  • If both parents have sickle cell trait, there is a 25 percent chance the baby will have SCD

Your doctor may try to increase your care before pregnancy to reduce complications during the pregnancy. They may advise you to:5

  • Stop taking hydroxyurea months before trying to get pregnant
  • Stop taking some other medications
  • Get vaccinations and vaccine boosters
  • Get screened for pulmonary hypertension, liver and kidney function, or iron overload

While you are pregnant, you may see different doctors. This may include a hematologist, who specializes in blood diseases. You may need to see a specialist trained in managing high-risk pregnancies. Your doctor may also refer you to other specialists or social workers.5

You may need to visit your doctor and change your treatment. Doctors may also monitor you frequently for anemia and other factors that trigger pain crises. During pregnancy, avoid:

  • Cold temperatures
  • Dehydration
  • Rigorous physical activity
  • Stress

This is because pregnancy can increase the risk of SCD complications, including:2,5

Women with SCD in the United States do not have higher risk for pregnancy-related death, but they do have a higher risk of problems during pregnancy. These include:6

  • Babies who are smaller than expected
  • Birth defects in the baby
  • Cesarean section (C-section) delivery
  • Going into labor before the expected due date
  • High blood pressure (preeclampsia)
  • Infection
  • Pregnancy loss

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Written by: Matthew Zajac | Last reviewed: January 2021