A life beyond sickle cell disease, thanks to a stem cell donor

Ufuoma Muwhen has always known that she had sickle cell disease. No one in the 23-year-old’s immediate family has the disease, but her maternal aunt in Nigeria died from sickle cell at a young age many years ago. And both of her parents and her sisters are carriers of the sickle cell trait. People with sickle cell trait usually do not have any of the symptoms of sickle cell disease, but they have inherited the gene and can pass it on to their children.

“My earliest memory is that at a young age, I always loved the swimming and waterparks,” recalls Ufuoma. “I remember that I had to stop swimming lessons early because I would get sick after almost every lesson.”  

What was happening to Ufuoma in those instances was a sickle cell crisis.

“I would go swimming and come out of the water and eventually, I would start feeling some joint pain, boney pain in my knee, my elbow, my upper chest area, and lower back,” she recalls. “It was a dull warm pain that aches all around. It would start slowly and get more intense. “

Ufuoma’s treatment regime, including for severe pain, evolved over time.

“At a young age I would start with Tylenol or Advil but if it got worse, we would go to oral codeine and topical analgesics like Voltarin or Rub A-535,” she shares. “As I grew older, the codeine was no longer as effective, and they switched me to oral morphine, so I always had it on hand. If this didn’t handle the pain, or if it was getting worse, my mom allowed me to make the decision about whether to go to the hospital or not.”

Treatment in hospital would include morphine infusion, saline, and oxygen. At least once a year, Ufuoma would receive a blood transfusion to replenish her hemoglobin level.

She was typically admitted to hospital for treatment three to four times a year and experienced an equal number of sickle cell crises that she was able to manage at home. In her teens, she was treated with hydroxyurea, a drug commonly used in cancer treatment to slow the growth of cancer cells, and which is often effective in preventing the formation of sickle-shaped red blood cells.

Although this treatment appeared to be working, Ufuoma’s condition was still deteriorating.

Stem cell transplant as an option for sickle cell disease

When Ufuoma was 17 years old and planning to go to university, her doctors presented her with another option: a stem cell transplant.

“My doctors told me that even though I was getting sick less often, my crises were becoming more intense and causing more damage,” she says. “They wanted me to understand that if I wanted to live a full life, a stem cell transplant was the best option.”

Stem cells, specifically blood stem cells, are immature cells that can develop into any cell present in the bloodstream. They can be used to treat more than 80 blood diseases and disorders. However, for stem cells to be transplanted successfully, donor and recipient must be a close genetic match.

Ufuoma was hesitant at first, but in consultation with her family, she agreed to learn more about this option and its potential to give her a brand-new life. The first step was screening her immediate family to see if there was a match.

“I have two younger siblings, a brother and a sister,” she said. “My sister Nyerhovwo, who was eleven years old at the time, was a 100 per cent match!”

Despite her young age, Nyerhovwo had seen her sister sick and in pain her whole life and said, “I’m going to do this because it will make you better,” Ufuoma recalls.

“I still remember the day in November 2015,” says Ufuoma. “I had gotten into university and was so happy, and the next day the transplant option was on the table. It was scary as they outlined the possible side effects. Then my family and I went to Calgary to meet the team and walk through the process, and it made me feel better and I agreed.”

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Opportunity to dream for one young woman with sickle cell disease

 In September 2016, thanks to her sister, Ufuoma had a stem cell transplant. Today, she is thriving.

Ufuoma (right) and her now 17-year-old sister Nyerhovwo rode camels in Abu Dhabi during a trip in April 2018. The trip was a “wish” granted to Ufuoma by Make-A-Wish Canada.

“I remember the first time I went for a walk post-transplant. I was able to take a deep breath for the first time in my life! I felt so healthy,” Ufuoma says.” The life I live now is unimaginable. I can go to the gym every day and the only pain I feel now is muscle soreness from working out. I can swim and I can do anything!!”

She is grateful that she was able to find a match and would like to raise awareness about the registry to help more people find matches to cure their illnesses.  A diverse pool of prospective stem cell donors in Canada and around the world can also support the clinical trials required to advance stem cell transplant science.

In 2018, Ufuoma officially launched a non-profit called Not Just You, a sickle cell support organization devoted to creating a support system for individuals and families affected by sickle cell disease.

“Our mission is to shed celebratory light on one’s sickle cell journey and provide a sense of hope for these individuals.” she said. “We wanted to be a hub that people could lean on, where parents could share their experiences and learn about different treatment regimens, remedies or options like stem cell transplants.

“Unfortunately sickle cell disease has such a stigma, people don’t even want to talk about it. For that reason, I wanted to do something ― to provide people with a place where they won’t be shamed and will be embraced for what their situation is.” 

Canada’s national stem cell registry should be as diverse as our country. A patient's best chance of finding a matching stem cell donor is among those who share their ethnic background.

We encourage members of diverse communities to support people in Canada with sickle cell disease by donating blood and joining Canadian Blood Services Stem Cell Registry. Blood donors and stem cell registrants can also show their support for these patients by joining the Sickle Cell Disease Association of Canada’s Partners for Life team.