Imagine having sharp, tingling pain running throughout the tiny blood vessels in your bones. What if you couldn’t stop it? What if prescriptions, such as opioids, following rounds of hospital visits, numbed these sensations but never alleviated this discomfort? Shirlanna Station, a self-described sickle cell warrior, no longer experiences this pain, an acute, or sudden, event called vaso-occlusive crisis, which previously sent her to the emergency room and made her bones feel heavy. “Right now, I feel amazing,” Station said about her life after receiving a blood and bone marrow transplant from her younger sister, which provided her body with healthy red blood cells. “I am no longer fatigued like I used to be. I am trying to get used to the new energy I have. With the absence of that pain and with the absence of opioids there is a weight lifted from my shoulders. There is no more stop-and-go. It’s just going.”
Shirlanna’s story: On Oct. 11, 2019, Shirlanna received a stem cell transplant from her sister as part of a trial led by Courtney Fitzhugh, M.D., a sickle cell disease and bone marrow transplant researcher with the National Heart, Lung, and Blood Institute. As a participant in another sickle cell disease study at the NIH, Shirlanna learned about the stem cell transplant trial and enrolled. Fitzhugh is studying how to transplant adults with stem cells from half-matched relatives, which could be a child or parent. In Shirlanna’s case, her younger sister qualified as a half-match and provided the stem cells for the transplant. Shirlanna went through extensive preparations so she could receive her sister’s stem cells, which act as the seeds of the bone marrow. Because her sister’s cell didn’t have the abnormal protein responsible for sickle cell disease, which can cause anemia, fatigue, bouts of pain, and serious organ damage, a transplant provided the possibility of a cure.
To help prevent an immune response that rejects the transplanted blood cells, Fitzhugh provides adults in the trial with immune-suppressing medication. Shirlanna also received chemotherapy and radiation as part of the protocol. She stayed in the hospital for 100 days to recover. She’ll stay in the study indefinitely as the clinical research team continues to monitor her progress.
Shirlanna did experience a surprise side effect from the blood and bone marrow transplant. Her skin, which she says is now glowing, started shedding as a reaction to a new medication. Laughing about the surprise sunburn and flaking, she mentions this was the “old shedding away for the new to flourish.”
For Shirlanna, these temporary side effects and future risks are “well worth” the benefits of the trial. Previously, her daily pain was a level six, on a scale from one to 10. When she was a child, her pain level was four, although it was more sporadic. Now, it’s zero. “I’m exercising more and doing things I haven’t been able to do my whole life,” Shirlanna says. “I have no more pain. It feels truly amazing.”
By maintaining a healthy lifestyle and taking care of herself, Shirlanna envisions she’ll have a healthy future. She’s completing a degree in cybersecurity. She’s also mentoring another NIH trial participant awaiting a similar stem cell transplant.
“Aha” moment: “Oh wow, there is something more to life than what I had been experiencing,” Shirlanna recalls thinking after learning about the blood and bone marrow trial. If the new type of stem cell treatment worked, she realized she could “live and live how I wanted to live.”
Invisible badge: Previously, when Shirlanna went dancing, on bike rides, or for a jog or walk, she felt pulsing pain throughout her arms and legs. Dehydration, humidity, colder weather, sugar, and alcohol, such as having a social drink with friends, triggered these episodes. As a social butterfly, Shirlanna wanted to go dancing with friends when she was younger, but the pain of living with sickle cell disease made social activities, as well as attending school and work, challenging. However, these experiences taught Shirlanna how to push through pain. “This is what a lot of warriors do,” Shirlanna explains. Warriors wear “a badge that can’t be seen.”
Advice: Shirlanna mentions that anyone with sickle cell disease or waiting for a bone marrow transplant should know that “there is light at the end of the tunnel.” Her advice is to “keep going and be that sickle cell warrior that we all are.”
Navigating a diagnosis: “Pursue your purpose,” Shirlanna said about navigating a sickle cell disease diagnosis. “Those simple words help you reflect on yourself and what you want out of life. Sometimes that’s all we have to think about to get through things.”
Magic wand: If Shirlanna had one wish for sickle cell disease it would be to “cure all of the children” who inherited this blood disorder. She would also host educational classes about sickle cell disease, particularly in Africa. This would be a way to give back to the underprivileged children and adults there, almost all of whom experience pain without the resources of those living with sickle cell disease in the U.S.
Advocacy: As a sickle cell disease advocate, Shirlanna shares her experiences of navigating emergency room visits, waiting for bone marrow transplant availability, participating in sickle cell disease research, and about the impact sickle cell disease funding has had for patients, like herself, with physicians, researchers, and the public.