On Feb. 19, Tanjila Bolden was at home getting ready for bed when she began to feel something unusual in her chest.
“It wasn't painful, but it was weird, like fluttery on my left side of my chest, it was going down my left arm into my elbow … and up into my jaw,” she said. “I had never felt it before.”
Bolden was nervous, so she called a few family members but to no avail. Finally, she got in touch with one of her girlfriends from her weekday morning prayer circle and asked her to drive her to the hospital because she knew something was wrong.
The 45-year-old African American mother of one had a mild heart attack. She is all too familiar with excruciating pain, because she has been living with sickle cell anemia — a form of sickle cell disease – since she was 18 months old, and strangely for her, the heart attack was painless.
“For me living with sickle cell disease, I assumed that a heart attack would be worse than a sickle cell crisis, and it was not,” Bolden said.
Her inherited blood disorder contributed to her recent heart attack. Bolden spent many days and nights in the hospital with complications from the disease. Over the years, she has incurred an overwhelming number of sickle cell crises that brought about blood transfusions, left hip replacement surgery, avascular necrosis, gallbladder removal, a partial hysterectomy and acute chest syndrome.
There are more than 100,000 Americans living with a variation of sickle cell disease, which disproportionately affects Black Americans. According to the Centers for Disease Control and Prevention, one out of every 365 African Americans will be born with sickle cell disease and one out of every 13 Black babies will be born with sickle cell trait. Many people with the disease live with unimaginable pain.
To help ease the agony for thousands of people with the disease — including the nearly 2,000 St. Louisans living with the disease — the Food and Drug Administration approved two new cell-based gene therapy treatments in December. The Biden administration believes Casgevy and Lyfgenia are breakthrough advancements for curing the rare disease and can save millions of lives. However, Black people in the St. Louis area are hesitant to try the gene-editing therapies and curious about the accessibility of the treatments and their costs.
“I would never want to put something in my body or do something to my body that would cause me to be worse than I was before, because then I will regret it,” Bolden said.
Bolden has been taking hydroxyurea since 2008. The chemotherapy drug used to increase the fetal hemoglobin makes her crises infrequent. She fears that if she switches from her daily sickle cell medication routine and decides to get the treatment, then her body might reject it and her disorder becomes worse. She said she is open to learning more about the gene-editing treatments.
Sickle cell disease is a disorder that causes the red blood cells to turn hard and sticky and take the shape of a sickle. People with the disease can have a crisis at any moment if their blood vessels become blocked by the sickled cells. Healthy red blood cells are circular and can move through the small blood vessels without igniting pain throughout the body.
Bolden said when she has crises they feel as if someone is hitting her entire body with an ax and stabbing it with sharp darts at the same time. Other people describe the pain as shattered glass piercing the body for an unknown time frame.
Rosemary Britts was ecstatic when she heard the news of the potential cures for sickle cell disease.
“This has been a long time coming in getting something. They have used bone marrow transplants for people, which was a little difficult because you have to find a match or a donor,” said Britts, executive director of Sickle Cell Association–St. Louis. “So, with this gene therapy, you will be using the individual's own cells.”
Lyfgenia, which is produced by Bluebird Bio, uses a viral vector that genetically modifies the blood stem cells. Casgevy, which is made by Vertex Pharmaceuticals and CRISPR Therapeutics, is a one-time intravenous infusion that edits particular genes to reproduce fetal hemoglobin. Both therapies can treat people age 12 and older.
Britts said people living with the disease and caretakers of those with the disease call her daily asking a myriad of questions about the new treatments. They want to know about accessibility, side effects and costs.
“There are about 60% of the individuals that live with sickle cell disease on Medicaid and you have others that have private insurance, and are their insurance companies going to pay for it?” Britts said. “It's not just those with Medicaid … I'm looking at it in terms of costs beyond just getting the treatment.”
The Biden administration is trying to improve access to the new therapies. Britts hopes anyone who wants the treatment will receive the results they expect and live free from pain.
“Individuals with sickle cell are just like anyone else, in terms of wanting to be an integral part of society and to just live their lives,” said Britts.
That is all Tameeka Watson wants for her 17-year-old son who has been living with sickle cell disease since birth. Malachi had his first crisis as a child. In the earlier years, Watson took her son to the hospital every couple of months for pain management due to his crises.
“He’s had over a hundred hospitalizations,” Watson said.
Watson dreams of the days when her son can play sports, hang out with friends and go away for college without her worrying about him. She has lost many jobs and friends, and her mental and physical health has decreased, because she is constantly thinking about her son’s well-being.
“It's just so much weight on just one person,” Watson said. “I got to work two jobs because I got to pay bills here.”
Watson thought her prayers had been answered when she found out about the sickle cell treatments.
“I was like, ‘Yes, we are getting it,’’’ she said.
But her son had other plans. He decided he did not want either treatment because he said they are too new and the side effects scare him. He does not want to take the risk of developing cancer.
Two of 32 patients in a trial of the gene therapies, Lyfgenia, developed blood cancer, though the cause is uncertain. So instead, Malachi plans to have a bone marrow transplant in July. Watson is terrified with the decision because of its many risks, but she said she will support her son through it.
Although Watson’s son is against getting a gene therapy treatment, she hopes it will bring peace to people living with sickle cell disease and to the families who take care of them.
“He is ready to have a family. He wants children. He wants to just work,” Watson said. “He's just ready to be cured and go on about his business.”
