Sickle Cell Disease Survivor
- Born with sickle cell disease
- Experienced severe pneumonia at age seven
- Received bone marrow transplant at age eight in 2005 from marrow donation from older sister
- Cured of sickle cell disease ever since
As a young girl, Tola Oriade often felt hemmed off from a normal life. “I was kind of weak, and couldn’t do a lot of things that my friends were doing—like going to the beach, playing soccer, going for a swim,” she says. Diagnosed with sickle cell disease (SCD) at birth, Tola couldn’t risk getting too hot or too cold, getting injured—or getting sick. Activities that were innocuous for most kids might trigger a life-threatening complication.
What began as a grave event for Tola at the age of seven—a serious case of pneumonia—ultimately led to a transformative cure. One year after her pneumonia, on May 13, 2005, Tola had a bone marrow transplant at Seattle Children’s. For people like Tola who live with sickle cell disease, a successful transplant is a complete cure. After eight years of living with SCD, she says, “I felt like a normal person.”
Two dangerous pain episodes
SCD gets its name from the sickle-like shape of the red blood cells of people who have inherited this blood disorder. About 100,000 people in the U.S. have SCD. In the recent past, those who contended with it faced an average life expectancy of less than 50 years. Today, life expectancies are rising, as efforts in training parents of children with SCD are helping them avoid the most common triggers of life-threatening complications.
Tola’s parents, Bosede and Caleb, had done everything right during Tola’s early years. Bosede had been a stay-at-home mom to her three daughters (Tola is the middle child, between Tayo and Abi), and was dedicated to safeguarding Tola’s health. The family had moved to Mountlake Terrace, Washington when Tola was five, and she was seen regularly at the Comprehensive Sickle Cell Clinic at Odessa Brown Children's Clinic in Seattle.
Until that bout of pneumonia, Bosede recalls, “We thought Tola had a very mild case of SCD, because she was very healthy. Most patients usually have pain crises. She never got sick until she was seven; but once it started it was very bad.”
Also called a pain episode or sickle episode, this occurs when red blood cells stick to other blood cells and the blood vessel wall, forming a “sludge” that blocks blood flow. It can cause excruciating pain, organ damage, and other serious complications, and may occur anywhere in the body. In Tola’s case, the sickle cells clumped in the small vessels of the lungs, and made it difficult for her to breathe.
“At first we thought the pneumonia was an aberration. But a year after that, Tola had a stroke,” says Bosede. Another stroke could prove fatal, and at the very least, Tola faced several years—or a lifetime—of repeated blood transfusions.
A perfect marrow match
One of Tola’s doctors, SCCA pediatric oncology/hematology specialist Michael A. Bender, MD, PhD, suggested the family explore having Tola undergo a transplant to cure her disease. Transplants themselves pose serious risks, and must be weighed in the context of the quality of life, as well as the life span of a person with this disease. “The decision to have a transplant is often more difficult for families with sickle cell than for other diseases,” says Dr. Bender. “For many diseases, a transplant provides the only high chance of long-term survival, and thus it is often a ‘life-or-death’ decision."
In contrast, he says, “people with sickle cell often live into their 40s and 50s, although life can be filled with severe medical complications and excruciating pain. After transplant, a patient’s quality of life dramatically improves—they stop having sickle cell pain, no new complications occur, and in fact, some resolve.”
A transplant for sickle cell improves the life of someone destined to have increasing pain and medical complications rather than preventing an imminent death, as is the case with transplant for many cancers. “Many families would rather continue with standard care for sickle cell than go through the difficult and risky transplant process,” he says.
Whether or not a transplant is advisable, Dr. Bender points out, depends on both the severity of the disease and the availability of a donor. A sibling transplant, for example, is the safest and most effective option. For the Oriade family, Caleb says, “Our minds were made up. If it was possible, we were going to go for it.” There’s a one-in-four chance that a sibling will be a perfect donor match; having two siblings upped Tola’s odds to 50 percent.
Luckily, this perfect match was found in Tola’s older sister Abi, who was 10 at the time. “I was happy to save my little sister’s life—I felt like a hero,” Abi recalls. She describes the process as “very painful. I had to be in a wheelchair for a week, and couldn’t do any sports for a month. But my sister’s worth it.”
Not a walk in the park
“A bone marrow transplant is not a walk in the park," Caleb says. "It was a long and difficult process, with many complications.”
To begin with, Tola had to undergo a course of chemotherapy to kill off all of her bone marrow and weaken her immune system to prevent her body’s rejection of Abi’s donated marrow. “I lost all my hair. And my skin turned like... a dark chocolate purple,” Tola says. “They put me in isolation—my immune system was wiped out so I could get sick very easily.”
Following the transplant, Tola was hospitalized for about a month before returning home. One complication she developed was “gut” graft-versus-host disease (GVHD). GVHD occurs when the transplanted cells recognize the recipient’s (Tola’s) tissues as foreign and attack them. With GI or gut GVHD, the cells that line the gut are damaged, and are less able to absorb nutrients. In Tola’s case, she threw up every time she ate. “They put Tola on immunosuppressants to fight the GVHD longer than we would have wanted,” says Caleb, “but she got rid of it, and it never came back.”
Tola also worked with an endocrinologist for a time, as the immunosuppressants temporarily slowed her development. “I missed half of third grade,” Tola says. “It took a while for me to go back to school.” Tola was home-schooled by her school district for the fourth grade, and she returned to her elementary school for fifth grade.
Life after transplant
For Tola and her family, the transplant was worth the risks and temporary hardships. After about a year-long recovery process, all the activities that were closed to her in the past, like soccer and swimming, became possible. “It’s funny, I’m not interested in them anymore,” laughs Tola. “I have new hobbies.”
Currently living with her family in Virginia, she says her two favorite pursuits are drawing and dancing—especially hip-hop and jazz dances. Tola dreams of someday working as a graphic designer or a cartoon animator. “I feel blessed,” she says.