Nothing Normal About It: Sickle Cell Community’s Fight for Understanding, Compassion, and Quality Care
by Rejane Frederick
“There is nothing more disheartening than to go through life and no one talks or even acknowledges that you are different.” “I want them to know that we are NOT normal; there’s nothing normal about my life.”– asserts Judy Gray Johnson, a Sickle Cell survivor and author of “Resilience: A Personal Story of Coping with the Ravages of Sickle Cell Disease…Against All Odds.” And what difficult odds still face Ms. Johnson and the many other Sickle Cell survivors despite significant gains made over the past 41 years –odds that compelled her and a large group of advocates to descend upon Capitol Hill for the annual Sickle Cell Advocacy Day.
Sickle cell disease (SCD) is an inherited blood disorder that results from a genetic mutation, causing normal donut-shaped red blood cells to lose oxygen and warp into a crescent “C” shape, making it difficult for the cells to pass through the tiny blood vessels located throughout the body. As a result, the cells get stuck and build up in the passageways, producing acute pain, anemia, increasing the risk of infection, stroke and severe organ damage. While there are several forms of the sickle cell disease, one of the most common in the U.S. is Sickle Cell Anemia.
Usually associated with people of African descent, in reality SCD is present all over the world, in Asia, Central America, the Middle East, South America, the Caribbean, and throughout the Mediterranean. In the U.S., over 70,000 people have SCD, approximately 3 million carry the gene, and around 1,000 babies are born with the disease–that’s roughly 1 out of every 500 African-American births and 1 out of every 900 Hispanic American births.
One inherited copy of the gene from each parent causes the disease; thus couples should get screened for the trait, prior to pregnancy, to avoid passing both SCD genes down to their children.
Despite advancements in early detection and treatment that have substantially extended the lives of both children and adults, researchers have yet to find a cure. Thankfully, today, sickle cell no longer carries the grim life sentence of uncontrollable pain (called “pain crises”) and early death it once did; survivors are leading longer and relatively healthier, happier lives.
One of the oldest living persons with sickle cell in the U.S., Richard Mitchell, recently made news when he turned 70 years old –28 years longer than the average 42 year life expectancy for men with SCD (48 year life expectancy for women). Mr. Mitchell credits his long life to eating right, exercising, and effectively managing both dehydration and especially stress.
Unfortunately, for the average SCD survivor, stress is a constant companion as they face daily physical, psychological, and social obstacles in their never-ending quest for a healthy, pain-free, stable life. According to a 2013 study in the Journal of the American Society of Hematology, sickle cell gravely impacts survivor’s ability to secure and keep gainful employment due, in large part, to the sudden, unpredictable nature of pain crises that can keep them away from the workplace for days if not weeks on end.
Adults living with SCD face some of the highest rates of unemployment (40-60%) with men being nearly three times as likely to be unemployed. SCD survivors face significant stigma in the workforce as a result of their disease, primarily stemming from a lack of awareness and understanding of sickle cell and its numerous complications.
Sadly, the stigma faced by SCD survivors within the healthcare community is equally high: “I want them to know how we fare in the health community: we are treated like mud.” – states Ms. Johnson. At the Congressional Caucus on Sickle Cell Disease legislative briefing, Dr. Julie Kanter, M.D., Director of Sickle Cell Disease Research at the Medical University of South Carolina, outlined numerous barriers faced by the adult sickle cell community. SCD survivors, particularly adults, face inadequate insurance coverage and access to both informed specialists and comprehensive quality care, often resulting in their high usage of emergency room services. SCD adults are also often under-treated for their pain and misperceived by healthcare providers as engaged in drug seeking/addictive behavior despite evidence to the contrary. In fact, studies have frequently found that healthcare providers perceived a much higher prevalence of addiction than actually existed.
This revelation points to a disturbingly widespread and well-documented racial empathy gap within the U.S. in which patients of color are habitually perceived by their healthcare providers as feeling less pain than their white counterparts and, as such, receive inadequate pain management.
The lack of awareness and understanding around sickle cell disease is a matter of life and death. Recently, in Washington, DC, 9-year-old survivor Blake Ford made news for being hospitalized with a 105 fever after he was repeatedly denied leave from his classroom because his teacher thought he was feigning illness to get out of class work. Hours later, Blake was finally dismissed only to be placed on a bus home instead of an ambulance. This occurred all in spite of his school administrators and teachers having participated in a meeting about Blake’s SCD condition after he suffered a heart attack the previous summer. Being unaware and ill-informed is simply not an option.
“Don’t Let Sickle Cell Put Limitations on Your Life!” 11-year-old National Child Ambassador Tiahna Hughes counseled Sickle Cell Advocacy Day participants. So confident and full of energy, no one could suspect that she had been hospitalized for five days just the previous week. Sickle cell survivors are far from normal; they and their family are warriors constantly staring down odds and triumphing over feats few ever have to encounter. The sickle cell community is the embodiment of courage, resilience, hope, and determination in one, all at once; to echo Judy Johnson’s sentiments, there is nothing normal about that.
Catching Up With Tiahna Hughes
By: Ernest Dickens IV, Communications Intern – Spring 2014
In a recent interview with the Sickle Cell Disease Association of America (SCDAA), National Child Ambassador, Tiahna Hughes, shared with us how life has been for her since becoming our youth representative in 2013. Tiahna has been very excited and driven to increase awareness of Sickle Cell Disease (SCD). She believes increasing awareness is important because if her friends and family know about SCD and are not fully aware; how is SCD perceived by everyone else?
To Tiahna, SCD is not a hindrance, it is a lifestyle. “Having SCD has been a positive impact on my life. It has given me something to tell others about and at the same time it has allowed me to stand out. I love being different,” said child ambassador Hughes.
While living a Sickle Strong lifestyle, Tiahna is very active in and out of the classroom. When she is not in school or participating in SCD advocacy and awareness events, Tiahna is active on several sports teams and hosts two YouTube Channels. Tiahna plays softball and basketball, but enjoys bowling the most. “I have been bowling since I could remember, and I love it because bowling is different. There is just something about formulating a strategy [with throwing the bowling ball] to get through what is in your way [bowling pins],” said child ambassador Hughes.
Hosting her own YubeTube Channels has allowed Tiahna to explore her creative side. From sharing her love of The Simpsons, to Q&A sessions and talking about SCD, Tiahna has embraced growing up as part of the digital generation. Her infectious energy and personality has led to a second YouTube Channel; a collaborative effort between her and Arlo, another child of the digital generation who lives in New Zealand. At a young age, Tiahna has been able to take her voice beyond borders, sharing her message that she is “proud even though I have Sickle Cell, Sickle Cell doesn’t have me!”
We have to “Be Strong About SCD,” let’s be the bowling ball and SCD the pins. Each pin left standing represents someone who is unaware of SCD; it’s about time we get a strike.
#MakeSureYouShareSickleCellWithOthers – Tiahna Hughes