Sickle cell disease (SCD) is a group of inherited red blood cell disorders. Normal healthy red blood cells are round, but in SCD, the red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle”.
The following are the most common types of SCD:
SS sickle cell disease is usually the most severe form of the disease. People who have this form of SCD inherit two sickle cell genes (“S”), one from each parent.
This is usually a milder form of SCD. People who have this form of SCD inherit a sickle cell gene (“S”) from one parent and from the other parent a gene for an abnormal hemoglobin called “C”.
HbS Beta Thalassemia
People who have this form of SCD inherit one sickle cell gene (“S”) from one parent and one gene for beta thalassemia, another type of anemia, from the other parent. There are two types of beta thalassemia: “0” and “+”. Those with HbS beta 0-thalassemia usually have a severe form of SCD. People with HbS beta +-thalassemia tend to have a milder form of SCD.
The Centers for Disease Control and Prevention (CDC) says that women with sickle cell disease are more likely to have problems during pregnancy; these problems can affect both the pregnant woman and her unborn baby.
The CDC estimates there are 80,000 to 100,000 Americans with sickle cell disease (SCD), which is a group of inherited conditions that affect red blood cells. In SCD, the normally round red blood cells resemble the C-shaped farm tool, the sickle; these red blood cells also become hard and sticky. Their shape and stickiness cause affected red blood cells to become stuck when traveling through small blood vessels and block blood flow. This causes intensely uncomfortable “pain episodes,” especially in the hands and feet.
Sickle cell disease can cause a variety of complications to both the mother and the baby during pregnancy and delivery, according to Brigham and Women’s Hospital. The mother may suffer complications including preeclampsia, eclampsia, rupture of the membrane and premature labor. About 6 percent of pregnancies end in miscarriage and another one percent in stillbirths. Many babies are small for gestational age (SGA) and are delivered early.
Additionally, sickle cells do a poor job of carrying oxygen and tend to die off quickly, leaving a shortage of the oxygen-rich blood cells that the mother and unborn baby can rely on. Doctors refer to this common complication as sickle cell anemia. Lack of oxygen causes the expectant mother to experience tiredness, irritability, dizziness or lightheadedness, and difficulty breathing. She may have a pale skin color or show signs of jaundice, which is yellowing of her skin or eyes. Pregnant women with severe sickle cell anemia often require blood transfusions, especially those experiencing infection or enlargement of the spleen. All of these complications increase the risk of problems during pregnancy.
Advancements in sickle cell treatment are helping more men and women reach childbearing age; many scientists are now turning their attention to helping these individuals have healthy pregnancies and positive birth outcomes. Many studies suggest sickle cell disease can cause infertility. One study conducted in Paris looked at 108 sperm samples collected from 44 men with sickle cell disease and found at least one abnormal sperm parameter in 91 percent of the samples.
Sickle cell anemia can become more severe during pregnancy and pain episodes may occur more often. A pregnant woman with this blood condition is at higher risk for preterm labor and having a low birth weight baby.
Proper prenatal care and careful monitoring can improve the likelihood of a healthy pregnancy. Men and women with sickle cell disease should discuss their options with their family physician or reproduction specialist.
- "Sickle Cell Awareness Month." Centers for Disease Control and Prevention. Office of the Associate Director for Communication, Division of News and Electronic Media. 2 Sept 2011. Web. 9 Sept 2013.
- "Sickle Cell Disease and Pregnancy." Centers for Disease Control and Prevention. 16 Sept 2011. Web. 9 Sept 2013.
- Berthaut I, Guignedoux G, Kirsch-Noir F, de Larouziere V, Ravel C, Bachir D, Galactéros F, Ancel PY, Kunstmann JM, Levy L, Jouannet P, Girot R, Mandelbaum J. Influence of sickle cell disease and treatment with hydroxyurea on sperm parameters and fertility of human males. Haematologica. 2008 Jul;93(7):988-93. doi: 10.3324/haematol.11515. Epub 2008 May 27