Walter Clemont Noel migrated to Chicago from the island of Grenada for dentistry school when in 1910, he began to experience an onset of unusual symptoms of anemia and pain. Experienced doctors not interested in his case, passed the information over to novice medical residents. It was the evaluation of the novice resident which found an abnormal shape of Noel’s blood cells. He described it as a sickle shaped phenomenon. Ultimately, Noel died nearly seven years later from unknown complications.
It wasn’t until the late 1940s, a better understanding of the Sickle Cell Disease (SCD) emerged. For over 100 years, there has been little discovery into the disease that primarily plagues the African Descent population. To fully understand sickle cell disease, one must understand a basic overview of how it affects the human body. In the US, there are approximately 100,000 people diagnosed with SCD, the #1 genetic disease in the US. SCD occurs when a sickle cell trait is passed from both parents causing a condition with several cascades of complication due to the blood disorder. Additionally, 1 out of 365 African Americans and 1 out of 16, 300 Hispanic Americans are affected with SCD (“Sickle Cell Disease,” 2016) . The hallmark sign of SCD is pain all over the body; however, there are several misconceptions about what happens in the body.
A person affected with SCD receives the trait from each parent creating an active SCD. These traits can present as 1 of 6 types: HbSS, HbSC, HbS beta thalassemia, and rare forms HbSD, HbSE, or HbSO. The abnormal traits cause the blood to form into an abnormal sickled shape which compromises the natural blood flow. Symptoms of SCD normally present around five months of age with subtle signs, such as hand and/or feet swelling. Today, SCD screening is mandatory in all 50 states upon birth. In early childhood, symptoms can be described as aches, pains, or recurrent bacterial infections. Over time, these complications can worsen into what is called a pain crisis, which is caused by the abnormal shape of the red blood cells clogging the natural flow of the blood. This can last from several hours to several days. The damage of the cells can cause chest pain, pneumonia, dehydration, and even a stroke. Although each patient is affected differently, some triggers can include dehydration, stress, and temperature changes. The life expectancy of a SCD patient is between 40-60 years, with each crisis putting the individual at risk for increased death.
We will take an intimate journey into the lives of those most affected; Sickle Cell patients and the Physician who cares for them. I had the pleasure of interviewing Tampa Hematologist and Medical Oncologist, Dr. Jawan Ayer- Cole, and her patients to get a better understanding of SCD and what the Tampa community can do to increase awareness. Dr. Ayer- Cole is one of the few physicians that is specialized in SCD care since 2004. She became intrigued with SCD while training at Grady Memorial and Emory University hospitals in Atlanta, Georgia. To date, she has treated over 100 SCD patients
In their shoes: “You’re not going to make it until an adult.”
Mrs. Tyson is a 70 year old African American female living with SCD for over 60 years. She was around eight years old when she became sick and was examined by her grandmother’s employer, a white doctor. It was then that she was diagnosed with SCD. Her earliest remembrance of her condition was a lot aching pains. The doctor told her grandmother, “There was not too much to do; she may live to be 30.” She remembers growing up sheltered, as her grandmother wanted to protect her. “It was taboo in those days; no one ever talked about it,” said Mrs. Tyson. Nowadays, she complains of being tired. She is limited in her treatment options due to having a kidney transplant over 27 years ago as a result of complications from her SCD. She doesn’t go into a pain crisis as often, but she suffers from ulcers, frequent blood transfusions, and weakness. When asking her how she controls her symptoms she replies, “Prayers, prayers, prayers!”
Renee, a 37-year-old Caucasian male born in Puerto Rico, was 30 years old when he was diagnosed with SCD. He describes frequent trips to the hospital since birth and a misdiagnosis of polio and growing pains. He recalls about six years of no pains in his teenage yea rs. As an adult, he remembers missing days at work due to his crisis. He admits to chasing his lifelong pain with drugs and alcohol. It wasn’t until he met Dr. Ayer-Cole that did he gained a full understanding of his SCD and ways to control it. Renee shares his daily experience of the misconceptions of SCD. He explains the disbelief of others when he discloses his SCD.
When asked about preventing SCD in 2017, Dr. Ayer-Cole stated, “1 in 10 African American have sickle cell trait, unless you’re limiting who you date up front, it is extremely difficult to prevent sickle cell disease by genetic screening.” Among her primary care goals, her office is designed to help keep her patients out of the hospital. “It’s underestimated in the healthcare system as to how sick they are. It’s a huge life limited disease.” Although, there is no cure, a stem cell transplant can improve life expectancy.
So how does one create more awareness? Dr. Ayer- Cole shares, “It’s really hard to get people to care about what they don’t have and internalize it. There is such a gap between the average white doctor and the average black patient. Educating the community is important but how do I get to the people who will make a difference?” According to Dr. Ayer- Cole, “They [lower social economic patients] don’t have enough economic power to drive waves in research.”
“With pain comes tolerance, but with suffering comes strength”- Alexis, 23 yr. old African American female living with SCD.
To learn more about Sickle Cell Disease, also known as Sickle Cell Anemia, please visit the National Heart, Lung and Blood Institute’s website at https://www.nhlbi.nih.gov/health/health-topics/topics/sca