In the United States, there are roughly 100,000 sickle cell warriors. When disaggregated by race, 1 in 365 African Americans are sickle cell warriors. 1 in 16,300 Latinx are sickle cell warriors. 1 in 13 African Americans are trait carriers. (Disabilities, 2020). Know Your Status! This phrase is synonymous with Get Yourself Test Month (GYT). Very rarely do we do we think to use this statement to convey the importance of knowing your sickle cell status. September is Sickle Cell Awareness Month. Unless you know you know sickle cell intimately as a warrior living with the hemoglobin disease and/or are a caretaker of someone that is; it is not a condition that is top of mind. Much of the widespread global understanding of sickle cell consists of frequent hospital visits and short life expectancy.
Awareness and education is key. What is sickle cell screening? What are they truly checking for? Sickle cell screening involves a blood draw to identify abnormal types of hemoglobin. Red blood cells contain hemoglobin. It carries oxygen throughout the body. (Disabilities, 2020). For 40+ years, this screening is done as part of newborn screening for babies. Typically, within 24-48 hours after birth. Hematologists want to determine if indication of disease (abnormal hemoglobin S) or if you are a trait carrier. If you are not a trait carrier but have abnormalities; the next step is to determine what type of sickle cell do you have. An important point of clarification is that the newborn screening indicates the likelihood of a child having the trait or disease. Confirmation of disease occurs within the first 1-3 years of life. While the newborn screen has helped with reduce morbidity and mortality of people living with SCD, there are there remains inequities in accessing quality racially concordant care.
Sickle cell disease is most commonly diagnosed in those of African Descent, Hispanics of Caribbean descent, and in some cases the trait can be found in those who identify as Middle Eastern, Indian, Native American, and of Mediterranean ancestry. The image below provides a visual of the probability that a child will either be a trait carrier, have the disease, and/ or have normal hemoglobin.
Sickle Cell Disease Foundation explains:
“How Sickle Cell Trait is Inherited?
- If both parents have SCT, there is a 50% (or 1 in 2) chance that any child of theirs also will have SCT, if the child inherits the sickle cell gene from one of the parents. Such children will not have symptoms of SCD, but they can pass SCT on to their children.
- If both parents have SCT, there is a 25% (or 1 in 4) chance that any child of theirs will have SCD. There is the same 25% (or 1 in 4) chance that the child will not have SCD or SCT.
- If one parent has SCT, there is a 50% (or 1 in 2) chance that any child of this parent will have SCT and an equal 50% chance that the child will not have SCT. “ (Foundation, n.d.)
“Hb S can form crystals that change the shape of the RBC from a round disc to a characteristic sickle shape. This altered shape limits the RBC’s ability to flow smoothly throughout the blood vessels in the body, limits the hemoglobin’s ability to transport oxygen to tissues, and decreases RBC lifespan from 120 days to about 10-20 days. A person with sickle cell disease (homozygous for Hb S) can become severely anemic because the body cannot produce RBCs as fast as they are destroyed. The affected person can suffer painful episodes and a variety of complications when sickled cells become lodged in and obstruct small blood vessels (vaso-occlusion).” (Online, n.d.)
Many adults do not know their sickle cell status. In addition, there are many who’ve been misdiagnosed as well. In a prospective cohort study conducted by Southern Community between 2002-2009, 86,000 adults residing in 12 southeastern states between the ages of 40-70 participated. The study cross-walked the self-reported sickle cell statuses with actual confirmed laboratory results. 40% self-reported having the sickle cell disease, while 60 % reported they did not. Their findings reported that of those who self-reported having sickle cell disease, only 6% had confirmed lab results, 63% as confirmed trait carriers, 6% having hemoglobin sc, and 35% with neither the trait nor disease. (Bean C, 2014). In 1985, I was told I was a trait carrier. 26 years later, I learned through my prenatal care genetic screening that I have sickle cell sc. Since then, I’ve met many sickle cell sc warriors with a similar experience. Prior to my confirmed diagnosis, I was aware of the probability that at least one of my future children will have the sickle cell disease. To have a more informed understanding, my husband made sure he was screened prior to us having children to confirm that he was in fact a trait carrier. Adults can request to be screened for SCD through quick blood draw in regular metabolic labs from a vein in the arm. For partners interested in having children and want to assess their risks, meeting with a genetic counselor is an additional option.
As sickle cell research continues to improve, dedicated funding is much needed. Especially for research regarding the sickle cell population, 60 and older. All individuals living with any of the six types of sickle cell disease are warriors. Their caretakers are their advocacy champions. SCD does not define the warrior! Continue to spread love, education, awareness, and support. Join your local SCD walks and donate to https://www.sicklecelldisease.org/.