Sickle Cell Disease

Sickle cell disease (SCD) is an inherited blood disorder. SCD causes changes in a protein called hemoglobin in the body’s red blood cells. Healthy red blood cells are round and flexible and can travel easily through blood vessels. In SCD, changes in the hemoglobin protein cause the red blood cells to become hard, sticky, and shaped like the letter “C.” These misshaped cells can stick to the inside of blood vessels, causing blockages and slowing blood flow to organs. People with SCD can have painful episodes called vaso-occlusive crises (VOC) caused by these blockages.   

  • SCD is a genetic disorder that runs in families. It is inherited when a child receives two sickle cell genes, one from each biological parent. These genes affect how hemoglobin works. There are different types of SCD, depending on the inherited genes.

    Approximately 100,000 people in the United States have SCD. SCD is more common among people whose ancestors came from sub-Saharan Africa, Spanish-speaking regions in the Western Hemisphere (South America, the Caribbean, and Central America), Saudi Arabia, India, and Mediterranean countries (Turkey, Greece, Italy).

    In the United States, one in 365 newborns born to people identifying as African American or Black are diagnosed with SCD. All newborns are screened for SCD.

  • All people seeking pre-pregnancy or prenatal care should be offered a complete blood cell count (CBC). Some physicians may also recommend hemoglobin electrophoresis, a test that can look for abnormal types of hemoglobin in the blood. If you have sickle cell trait (you have one sickle cell gene) or SCD (you have two sickle cell genes), your partner should also be screened.

    Your baby’s chances of getting SCD depend on the specific genes that you and your biological partner have. Your healthcare professional or a genetic counselor can help explain the different genetic screening and testing options.

  • SCD can range from mild to severe, and it can cause varying complications, such as chronic pain, painful crises, anemia, and infections. These complications can develop or worsen during pregnancy. Pregnancy complications like preeclampsia, placental abruption, stillbirth, and preterm birth also happen more frequently in people with SCD. Pregnant people with SCD also have a higher risk of fetal growth restriction or having a baby with a lower-than-average weight at birth. Fatigue, depression, and anxiety can worsen during pregnancy or the postpartum period.

    The risk and severity of complications depend on your current health and medical history. For people with pre-existing heart and lung problems, pregnancy may not be recommended. However, many people with SCD have successful pregnancies.

  • Ideally, a maternal-fetal medicine subspecialist and a hematologist experienced in SCD should be involved in managing your pregnancy. A genetic counselor, pain management specialist, behavioral health provider, and social worker may also be helpful.

    Experts recommend the following pregnancy care plan for people with SCD:

    • Schedule a pre-pregnancy visit with your healthcare team. This is an opportunity for you and your healthcare team to evaluate how pregnancy may affect your future health and your baby’s health. During this visit, your doctors will assess your general health, review your medications, and provide genetic counseling. Some medications may not be recommended during pregnancy and may need to be adjusted.

    • Get vaccinated with all vaccinations recommended for your age group before pregnancy. You should also get the 4 vaccinations recommended during pregnancy: COVID-19, flu, Tdap, and respiratory syncytial virus (RSV). Also, make sure that your pneumococcal and meningococcal vaccines are up to date.

    • Follow your doctors’ schedule for prenatal care visits. Regular prenatal care throughout pregnancy is important to stay as healthy as possible. You may also need additional ultrasounds to check fetal growth and monitor your fetus’s health during pregnancy.

  • If you don’t have any complications, delivery is usually recommended at 39 weeks of pregnancy. If problems arise, it may be recommended that you deliver your baby earlier than your due date. Vaginal delivery is recommended unless other complications happen that make a cesarean delivery the safer option.

Quick Facts

  • SCD is an inherited blood disorder. 

  • Genetic testing can help identify if your baby is at risk of inheriting SCD. 

  • SCD may increase the risk of anemia, infections, and painful crises during pregnancy. It may also increase the risk of preeclampsia, preterm birth, and fetal growth restriction (FGR).   

  • Pregnancy with SCD should be planned and managed with a hematologist and MFM specialist. 

Glossary


Anemia: A condition where the body has a lower-than-normal number of red blood cells.  

Fetal Growth Restriction: A condition in which the fetus measures much smaller than expected for the gestational age.  

Fetus: During pregnancy, the stage of development from nine weeks to birth. 

Gene: The basic unit of inheritance passed from parents to offspring. Genes contain the instructions for a person’s physical and biological traits.  

Genetic counselor: A healthcare professional who specializes in helping individuals and families understand how genetic conditions might affect them. 

Hematologist: A doctor specifically trained in the treatment of anemia and other blood disorders.  

Hemoglobin: A protein inside red blood cells that carries oxygen from the lungs to other parts of the body. It also picks up carbon dioxide (a waste product from your cells) and returns it to your lungs so you can breathe out carbon dioxide. 

Hemoglobin electrophoresis: A test that measures the different types of hemoglobin in the blood. It is used to screen for sickle cell disease and sickle cell trait.   

Maternal-fetal medicine subspecialist: An obstetrician with specialized training in prenatal care for women with high-risk pregnancies.  

Placental abruption: A serious complication of pregnancy in which the placenta separates too early from the uterus. It usually occurs in the third trimester or during childbirth. Symptoms include pain in the abdomen and vaginal bleeding.    

Preeclampsia: A disorder that can occur during pregnancy in which the blood pressure gets too high. It can damage many organs in the body, including the kidneys, brain, and liver. 

Red blood cells: The cells that carry oxygen in the body. 

Sickle cell disease: A genetic disorder that causes red blood cells to be misshaped. Individuals with this disease have two sickle cell genes, one inherited from each biological parent.  

Sickle cell trait: A genetic condition caused by inheriting one sickle cell gene from one biological parent. Individuals with sickle cell trait can pass the gene to their offspring. 

Ultrasound: Use of soundwaves to create images of internal organs or the fetus during pregnancy. 

Vaso-occlusive crisis (VOC): A painful episode that occurs when a blood vessel becomes blocked, leading to severe pain.  

 

Last Updated: April 2024