Sickle cell disease is an inherited red blood cell disorder caused by defective hemoglobin residing within it. Red blood cells transport oxygen from your lungs to the rest of your body. Hemoglobin is the actual protein that carries the oxygen.
Usually red blood cells are round, soft and pliable and can therefore easily pass though your blood vessels even if they are narrow. If the hemoglobin protein in the red blood cells is defective, such as in sickle cell disease, the blood cells become smaller and much stiffer, preventing them from squeezing through narrow blood vessels. The blood cells will get stuck and start clumping together which will eventually completely block off the blood flow in the vessel. Your body’s tissue near that area will then die due to lack of oxygen. This is a very painful process and is called a sickle crisis.
The gene defect that causes this abnormal hemoglobin is present mostly in people of African descent. About 1 in 10 African-Americans have the gene. It is also present, but less commonly so, in other ethnic groups such as the Latino-American population where about 1 in 100 have the gene.
You can either have one or two of this sickle cell gene. If you only have one gene, only half of your hemoglobin will be abnormal and you have condition called sickle cell trait which is a much milder form of the disease. Sickle cell trait will not affect your pregnancy much except for an increased risk for bladder infections.
If you have two sickle cell genes, however, all of your hemoglobin will be abnormal and you will have the full-fledged sickle cell disease where pregnancy complications are common and you should be followed by a health care provider who is experienced dealing with this problem.
During pregnancy your body needs more oxygen due to the increased metabolism of pregnancy and the demands of your growing baby. The frequent blockage of blood vessels from the malformed and stiff blood cells seen in this disease will result in both you and your placenta receiving less oxygen. The consequences of this are numerous. There will be an increased risk for bladder, kidney and lung infections, severe anemia, preeclampsia, miscarriage, growth restriction, early labor and stillbirth.
If you carry the sickle cell gene, it is important to determine if your baby has inherited it and if so, and to what degree. You need to test the father of the baby early in the pregnancy to help determine how your baby is affected. If both of you have the single gene also known as the trait form, there is a 1 in 4 chance of your baby ending up with the 2 gene full fledged disease, 1 in 2 chance of your baby having the one gene milder trait form, and 1 in 4 chance of not having any sickle cell genes at all.
If you baby has inherited two of the sickle genes, it won’t be noticed initially. Your baby will be protected while unborn and during the first few months of it life from the presence of fetal hemoglobin, a hemoglobin made by all babies’ blood cells in the beginning of their lives. The fetal hemoglobin will act similar to normal hemoglobin and mask the soon-to-come abnormal sickle hemoglobin. It is only when the baby reaches about 3 months of age that the fetal hemoglobin will be replaced by your baby’s innate hemoglobin, and symptoms may then appear if your baby has inherited the 2 gene disease.
If you have the full fledged 2 gene sickle cell disease while pregnant, it is important that you stay well hydrated and that you treat any infection promptly.
You will often be recommended to take high dose of folic acid to help your red blood cell production. You will also be watched closely for bladder and other infections (the key to not having bladder infections is staying hydrated). Your baby’s well-being will be monitored closely with frequent ultrasounds and receive antenatal surveillance such as non-stress tests. Due to the increased risk for preeclampsia, some providers will also start you on low dose aspirin to take daily by mouth.
Blood transfusions may finally be given to you during the pregnancy to help mix your blood with red blood cells that have normal hemoglobin in them in order to prevent problems associated with this disease.
For the delivery, vaginal delivery is usually recommended. Frequently, extra oxygen and Intravenous fluids will be given to you during labor to keep you continuously hydrated and decreasing the cells from sickling.