Life-changing transplant gives sickle cell patient a second chance

October 3, 2018 | by Abe Rosenberg

Cierra, Sickle Cell Patient | City of Hope Sickle cell patient Cierra Danielle Jackson
Cierra Danielle Jackson recalls the pain.
“I just remember crying, and my mom holding me,” she said.
She was 6 years old.
Mom remembers an even earlier incident.
“When she was 2,” recalled Cheryl Jackson, a medical secretary, “she ran a fever with a lot of swelling. I knew what it was.”
Looking at Jackson now, you'd never suspect anything was ever wrong.

'Always in the Hospital'

Jackson, an up-and-coming actress and former beauty queen was born with sickle cell disease, a genetic disorder that afflicts some 100,000 Americans, 80 percent of whom are African American.
People with sickle cell experience chronic, severe pain because their defective, sickle-shaped red blood cells don't flow naturally. Instead they clump, clogging arteries and depriving vital organs of the oxygen they need. Eventually the organs fail.
“It's a devastating disease that means a horrible quality of life and a very short life expectancy,” said City of Hope pediatric hematologist and the Barron Hilton Chair in Pediatrics Joseph Rosenthal, M.D.  Very few sickle cell patients live past 30, and much of their time is spent coping with one pain emergency after another.
“By the time I reached ninth grade,” recalled Jackson, “I was always in the hospital.”
Along with the physical pain came emotional distress.
“Sickle cell can give you jaundice,” she said. “Kids in school called me the 'green-eyed gremlin.' Total strangers would come up to me and ask, 'Are you OK? Are you contagious? Do you have liver problems?' There were constant reminders, and they were annoying. Also, I was always sad, because I knew sickle cell patients don't live long.”
Sad, and heartbroken too, because normal, everyday ways of having fun could be dangerous.
“Mom took me to a pool but wouldn't let me swim, because the cold water could trigger an attack,” she recalled.
“But one time I went to this party and got on a jet ski. I knew I shouldn't, but I went anyway. Suddenly, I'm out in the middle of the ocean, in excruciating pain. I managed to get back somehow, but I was in the hospital for a week.”

Chance for a Cure

Many sickle cell patients manage their condition with blood transfusions, along with a variety of medications, often powerful opioid painkillers or the chemotherapy drug hydroxyurea (a newer drug, L-Glutamine, was approved by the Food and Drug Administration in 2017).
But only a bone marrow transplant can “cure” sickle cell disease, and so, after enduring the pain through high school and into her college years at UCLA, that's what ultimately brought Jackson to City of Hope.
Though less well-known than its world-famous cancer and diabetes efforts, City of Hope's sickle cell program is a leader in one critical area.
“We've done 27 transplants (for sickle cell patients), the largest number in the West,” said Rosenthal, pointing out that the focus of City of Hope's program is different. “We partner with the larger sickle cell community that's treating the symptoms. We're striving to find a cure.”
City of Hope is the natural place to develop that cure, because so much of the needed experience and infrastructure already reside here. Over the past 40 years, City of Hope physicians have performed more than 14,000 stem cell transplants to treat leukemia, lymphoma, myeloma and other blood cancers.
“We can apply the same transplant technology to our sickle cell patients,” said Rosenthal.

Finding a Match

But transplants can only be successful if the right donor is found.
Ideally, both donor and patient should carry the same 12 blood proteins. This “full match” minimizes the possibility of graft-versus-host disease, when the donated cells attack the patient's system. Relatives, especially siblings, are the likeliest source of such a match. In fact, when a child receives bone marrow from a brother or sister, the cure rate can reach 95 percent.
Transplants are less successful in adults, and the odds of finding a fully matched donor outside the family are not good in the African-American community.
That's why so much recent research has focused on so-called “half matches,” where perhaps only six proteins line up. A decade ago it was found that half-matches can work if strong chemotherapy was administered both before and after the transplant. The chemo killed the remaining sickle cells without harming the transplanted stem cells, which took root and produced healthy bone marrow.
But Rosenthal is poised to take the half-match process even further, thanks to a new $5.74 million grant from the California Institute for Regenerative Medicine. His new approach uses lower doses of chemo, but prior to the transplant, the donor's marrow is depleted of CD4+ immune cells, reducing the chance that the donated cells will attack their host. A phase 1 clinical trial is about to begin.
Further down the road is the possibility of using gene editing to repair defective blood cells, then reinfusing them into the patient, eliminating the need for a donor altogether.
“My dream,” said Rosenthal, “is to leverage City of Hope's strength in cancer and gene therapy to come up with a sickle cell treatment that can be delivered early and prevent the disease.”
For Jackson, the dream was simple:
“I wanted to be rid of this,” she said. “And I always knew that one day I'd be away from it.”
It would take longer than she expected.

A Year of Discovery

Jackson met Rosenthal in 2012. Together they decided to wait until Jackson finished college, so she could then devote the several months required for the long process of preparation, transplant and recovery.
“As soon as I graduated (in 2014), City of Hope called,” she remembers. A date was set.
But a bizarre snafu stopped everything.
During a preliminary blood transfusion, word came down that Jackson's insurance would not cover the “experimental” transplant. The procedure was abruptly halted. Jackson, who'd left her job and moved out of her apartment, was dumbfounded. And unemployed. And homeless.
“I really struggled,” she says of the months that followed. “I prayed a lot.”
Her deep faith grew during this time which she now calls her “year of discovery,” and she's convinced that her temporary setback served a greater purpose.
Cierra, Sickle Cell Patient | City of Hope Cierra Jackson with her doctor, Joseph Rosenthal, M.D.
Rosenthal meantime, hadn't given up. He promised Jackson, “We're going to make this right.”
“He's a wonderful doctor,” said Jackson. “He really fought for me.”
A year later, City of Hope called again. They would cover the transplant through a clinical trial.

The Rocky Road to Wellness

This time everything fell into place. A donor from the U.K. was a 99.9 percent match, and on May 12, 2015, the transplant was performed.
Jackson describes her slow recovery as “a rocky road, definitely not a walk in the park.” Three years later, though she's considered “cured,” there is some disappointment because she continues to experience pain in her limbs, the result of avascular necrosis, a lingering legacy of her sickle cell disease and the drugs used to treat it.
“Unfortunately we can't reverse it,” says Rosenthal.
Still, she's adjusting to her new reality, especially the happy realization that she can now look forward to adulthood.
“I'm tearing down the old mental strongholds, like my fear of not living very long. I'd gotten so used to being sick. It's different now. I'm a new Cierra!”
A new Cierra with new plans. She's pushing forward with her acting career. She's writing a book about her journey and also creating a nonprofit to support and counsel others with sickle cell disease. It's all part of what she came to understand as she faced her worst moments, finding “beauty in the pain” and calling it “the leverage God gives you to help someone.” It's more than a spiritual bromide, she insists. Jackson's been told that Rosenthal's team learned many lessons from her transplant, which will help future patients.
To those patients Jackson sends a message, and, from deep within, a personal promise:
“There's hope, and you're not alone,” she said. “I know what you're going through and I'll be your friend, even if you're a million miles away.
“Keep going. Don't stop!”
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