By Maureen Gilmer, IU Health senior journalist, firstname.lastname@example.org
For years, Tremesha and Terrence Preddie grew accustomed to seeing their son live just half a life.
He couldn’t run very fast or very far, couldn’t play sports. He couldn’t even jump around in the sprinkler in the backyard.
That’s all changed now. After a stem cell transplant one year ago, Elliot Preddie has been declared free of sickle cell disease, the deadly illness that stole his strength, his endurance and too often his laughter.
Sickle cell disease is an inherited blood disorder that causes red blood cells to become hard and sticky. When they clog up blood vessels, the blood cannot bring oxygen to the body’s tissue. This often results in serious infections, chronic pain and organ damage. It strikes African-Americans the hardest, along with Hispanic-Americans.
Elliot’s disease was so severe he was receiving monthly blood transfusions. A scar in his skull reveals where he had surgery for a condition called Moyamoya, a rare cerebrovascular disorder caused by blocked arteries at the base of the brain. That put Elliot at high risk for stroke. It was, his mom said, “a ticking time bomb.”
Last year, Elliot underwent a stem cell transplant under the care of Dr. Jodi Skiles at Riley Hospital for Children at IU Health. His donor was an adult cousin. As is the case with cancer patients, the best donor is often a matched sibling, but Elliot’s younger brother, Carter, also has the disease. New research at Riley and IU Health University Hospital has shown the benefits of using half-match donors, which is what Elliot’s family did.
It marked the beginning of his new life.
“We were released from the hospital after 37 days,” Tremesha said. “You have to wait for the immune system to reboot before they can actually test it. The first test showed the bone marrow when it grew back was 100% donor marrow. None of his sickle cell bone marrow survived, which is what we want.”
That was the first indication that Elliot was cured.
“It was a huge sigh of relief,” his mom said. “We shed some tears for sure.”
Follow-up tests and Elliot’s continued recovery are proof that the seventh-grader has turned the corner on the disease.
While Elliot, 12, still carries sickle cell trait (his donor also carries the trait), he has 75% to 80% normal hemoglobin, Tremesha said.
“When we got those results, I kind of lost it. It was finally real and confirmed that everything he had gone through and my husband and I had gone through on his behalf was worth it.”
Dr. Seethal Jacob, director of Riley’s Sickle Cell Program, is thrilled for Elliot and his family, knowing the stress they had been under since Elliot’s condition began to worsen.
“When we initially made the recommendation regarding transplant, it was because we knew that without it he would need chronic blood transfusions for the rest of his life,” Dr. Jacob said. “We became concerned when he started developing antibodies to the blood products he was receiving.”
That made it harder to find blood that was a good match, which in turn made it harder to prevent bad outcomes including stroke, she said.
“Knowing Elliot and his family, we thought transplant was a good option for him.”
Not that it was without its own difficulties and complications, Dr. Jacob added.
“It still requires a lot of intense therapy, isolation, close monitoring and more medications. It’s a stressful time for the patient and the family as well. Their whole lives are remodeled for a significant amount of time.”
But it was all worth it, Tremesha says. She knew for sure this summer when she saw her son dancing through the sprinkler on the lawn.
“He couldn’t do that before because the cold water would cause a pain crisis. And now he goes outside and plays in the sprinkler for hours. The first time he did it, I started crying. He has energy and stamina that he never had.”
Now, she said, she and her husband don’t have to worry about their son getting blood transfusions anymore. They don’t worry that he will suffer a stroke. They have let go of their biggest fear – that they might outlive their child.
“Life expectancy for someone today with sickle cell disease is mid-40s, so when Dr. Skiles told me all of that is gone and he can live a long and healthy life, I just broke down because you don’t want to have to think about burying your own child,” Tremesha said. “But that was a very real possibility for us.”
Advocating for her own children is one thing. But the mother of two also speaks out on behalf of other kids and adults who suffer from sickle cell disease and sickle cell trait. She and her husband both carry sickle cell trait, which means they carry the gene for the disease, but it generally doesn’t manifest itself in serious health crises.
There are exceptions, however, she said, so she implores the healthcare community to listen closely to patients.
“I never shy away from talking about it,” she said. “I always try to challenge doctors and what they think they know and what they’ve been taught from Western medicine textbooks.”
“As a caregiver, as a parent, I always tell other parents, don’t believe everything you’ve heard or read. You have to challenge doctors and educate yourself.”
That kind of perseverance has made a real difference for Elliot and his story, Dr. Jacob said.
“His mom wanted better for Elliot. She wanted more for his life, and that led her to look at other options and to be a great advocate,” the physician said. “I encourage others not to settle, but to ask questions and to advocate for better funding and better access to care.”
Riley’s Pediatric Sickle Cell Program, the largest in the state, benefits from comprehensive and multidisciplinary care, Dr. Jacob added. Doctors at Riley often diagnose sickle cell disease through a newborn screening test, which is performed on every child born in Indiana.
“Patients have access to other subspecialties and experts like Dr. Skiles and our transplant team. They have access to various treatment options and a care model focused on what is best for each patient and family,” she said.
“That’s what makes our program at Riley so special. Our focus is not just on the medical care but on the person as an individual and the family as a whole.”
For more information about Riley’s sickle cell education program, email email@example.com.
Photos submitted and by Mike Dickbernd, IU Health visual journalist, firstname.lastname@example.org