Sickle cell disease made the news earlier this year when rapper Prodigy died from complications related to SCD this past June at the age of 42. It’s actually very common among African-Americans with 1 in 13 being carriers for the disease and 1 in 365 having sickle cell disease. There are other ethnic groups that are frequently affected as well. In some parts of Central India and eastern Saudi Arabia, as much as 40% of the population carries the sickle cell trait or is affected by sickle cell disease. People with Mediterranean and Central American heritage have higher rates as well. (People with Mediterranean heritage often have a variety of SCD known as thalassemia.) Because of the high number of people with Sub-Saharan African ancestry in the Caribbean and parts of South America, sickle cell trait is more common there as well.
For a long time, sickle cell disease was treated as a fatal children’s disease because children who were born with it frequently died very young. But in the last 3o years, advances in medications and treatment have led to longer lifespans for people with sickle cell disease- resulting in more women of childbearing age with the disease. While there are more specialists going into pediatric care for SCD, there are fewer care providers who are confident or knowledgeable about treating adults.
This gap between pediatric care and adult care is more of a problem as we have more children living to adulthood with birth defects and genetic conditions. I feel a similar frustration with CJ’s care. I feel like specialists are often thinking mostly of the “here and now” instead of the impact on his life as he grows to adulthood. To me, it kind of feels like that part in Dark Knight where the Joker says, “I’m like a dog chasing a car. I wouldn’t know what to do with one if I caught it.” As we have more children with disabling and genetic conditions living longer, our healthcare professionals are going to have to raise the bar beyond “Your child is still alive”. (But I digress…)
Sickle cell disease testing has only only been widely implemented since 1986 and universal testing only started in the 2000’s. The American Society of Hematology estimates that as many as 30% of infants with SCD may be lost to follow-up. So the current situation is that many women of childbearing age who are at risk may not know that they have SCD or that they are carriers for the disease. We also have a growing population of immigrants from areas where SCT is common who may not have had genetic testing available to them and may not know their sickle cell status.
If you want the whole ten page paper I wrote on this in all it’s APA cited glory with recommendations for public health strategies, you can find it here. (A little light reading before bedtime anyone?)
How SCD causes pregnancy complications
Most of us have red blood cells that are round shaped, but people with sickle cell disease have red blood cells that are shaped like a crescent or sickle. This causes the red blood cells to become stiff and sticky and clump together. When the blood cells clump together, it cuts off oxygen to the body’s tissues and organs causing pain and organ damage. Sickle cell trait (SCT) can cause all kinds of problems, but it also seems to give carriers a level of protection against malaria that other people don’t have. Scientists think it may have evolved as a way to protect people living in malaria prone areas from the disease.
Sickle cell disease impacts many of the body’s systems, it can cause many different types of complications for a pregnant woman and her baby:
- Infections- UTI’s, but also kidney and lung infections
Heart enlargement and heart failure from anemia
- Severe anemia for baby
- Intrauterine growth restriction (IUGR)
Low birth weight (less than 5.5 pounds).
Stillbirth and newborn death
One little known fact is that even women who are carriers of sickle cell trait are at risk for pregnancy complications that can result pre-term birth. Women who are carriers of the sickle cell trait are more likely to have urinary tract infections during pregnancy and a condition called pyelonephritis during pregnancy. (Pyelonephritis is a serious and potentially life-threatening condition where the kidneys develop inflammation from a bacterial infection.) With the increased risk of kidney/urinary tract infections comes an increased risk of preterm birth and even death for the mother and baby. This could be a strong contributing factor to the distressingly high rates of maternal and infant morbidity and mortality among African-American women.
What Can We Do?
If you read the above link to the CDC’s short article on the high rates of preterm birth for African-American women, you can see that they’re not talking about SCD and SCT as a possible contributing factor. The March of Dimes mentions “clotting abnormalities” as one of many possible contributing factors. Awareness about how much this could be impacting African-American women seems to be pretty low in the public health and medical fields. The relationship between pregnancy and sickle cell disease and sickle cell trait is still relatively new. But with an increasingly diverse population in the United States which includes immigrants from India, the Caribbean and the Middle East, SCD/SCT related pregnancy complications could become an even bigger problem if it’s not addressed.
There are a few things that moms who are at risk for SCD/SCT can do:
- Get tested for your sickle cell status. The blood test can be performed on adults and will tell you whether you are a carrier, have SCD or are unaffected. If other women in your family have had serious infections during pregnancy or given birth prematurely it might be an indicator that SCD/SCT runs in your family. The CDC has a short pamphlet on SCD and pregnancy that you can find here.
- Know the signs and symptoms of preeclampsia and preterm birth. Having SCD is a HUGE risk factor for preeclampsia and other hypertensive disorders of pregnancy. If you are experiencing any of the following symptoms during pregnancy and have SCD, DO NOT IGNORE IT. With symptoms like changes in vision, you should go to the emergency room immediately. Changes in vision can be a sign that the preeclampsia has progressed to a life threatening stage. I recommend reading the updated guidelines for diagnosing preeclampsia. ACOG revised their guidelines because they found that even women who weren’t displaying “classic” signs of preeclampsia like spilling protein in urine or blood pressure greater than 160/110 were still developing organ damage from hypertensive disorders of pregnancy.
- Keep a record of your blood pressure. High blood pressure in pregnancy can be a sign of preeclampsia. But the catch is that blood pressure readings can vary from person to person, so even if you have “low” blood pressure, you could still have preeclampsia. Keep a record of your blood pressure; maybe even request a copy from your doctor at each visit. With a record of your blood pressure readings, you can help the doctors and nurses at the ER better understand your condition if you ever need to go there.
- Get help! People with SCD have to strike a fine line on exercise and activity. If they do too much, it can bring on a sickle cell crisis (clotting problems and pain), but doing too little could increase problems with congestive heart failure and lung problems. Getting too cold, hot or over tired can trigger a sickle cell crisis, so get whatever help you can with housework, cooking and caring for older children both before and after the baby comes.
- Stay healthy. Eat plenty of fruits, vegetables, protein and whole grains. Drink water and cut back on soft drinks, fast food, sugar and processed food. Wash your hands to prevent viral and bacterial infections and help everyone else in the household to do the same. For exercise, listen to your body and stay as active as you can without getting too overtired.
Communities should get involved too:
- Hold awareness events- Encourage women to get screened for SCD/SCT, even they are not pregnant yet.
- Help out- In some European countries, all mothers are entitled to in-home visits from a nurse to help the mother and family adjust to the baby. They typically refer mothers to resources that might help them, watch for signs of postpartum depression or postpartum complications, and even just listen to the mother talk about her situation. Unfortunately, we don’t have that kind of option in the US, but we can provide support to expectant mothers- with and without SCD/SCT- through community groups, churches, family and friends. Visitors who help the mother by caring for older children, preparing meals or doing housework can actually be very welcome if their focus is on helping instead of just “seeing the baby”. (Got dietary restrictions? Check out this post on how neighbors, friends and family can help out with meals.)