Ken and Gina’s story: A sister’s gift for her brother’s future

Miranda was with her oldest child, 18-year-old Gina, in one part of the hospital. Her husband, Oscar, was with their youngest, 7-year-old Ken, in another part of the hospital.

“It was one of the most challenging days because we had two children in the hospital, both going through very delicate procedures," Oscar says. “But it was comforting to know that Gina was there, not because she was sick, but because she wanted to help her little brother.”

Gina was having a procedure to extract her bone marrow – which could cure her little brother's sickle cell disease. Oscar breathed a sigh of relief when the doctors came into Ken’s room with the donated bone marrow and said Gina was doing well.

Then he joked: “Now let's give the bone marrow to Ken and see if he starts acting like Gina.”

A surprising diagnosis

Oscar and Miranda were shocked to learn from Ken's newborn screening that he had sickle cell disease. Normally, red blood cells are round, soft, and flow easily through the body. With sickle cell disease, they become sticky and curved like a crescent moon, so they don't move as easily. This can cause severe pain, organ damage, stroke and other problems.

Sickle cell disease is passed down from parents to children. Many people carry one copy of the gene without knowing it, since it usually doesn't cause symptoms. But if both parents carry one copy, each child has a 1 in 4 chance of being born with the disease.

“We have three other kids and none of them have sickle cell, so we didn’t even realize it was a possibility,” Miranda says.

When Ken was 2, they started coming to Children's Health℠ for his sickle cell care.

“We picked Children’s Health because it was the closest hospital after we moved to Texas,” Miranda says. “And that’s a decision we’ll never regret.”

A cure for sickle cell

The care team at The Pediatric Sickle Cell Disease Program at Children’s Health discussed options for treatment, including a potential cure with a stem cell transplant.

Victor Aquino, M.D., Pediatric Hematologist/Oncologist at Children’s Health and Professor of Pediatrics at UT Southwestern, explained that a stem cell transplant can replace the bone marrow that makes sickle cells with someone else’s bone marrow that makes regular red blood cells.

“With sickle cell, we almost always use sibling donors and 25% of siblings are a match,” Dr. Aquino says. “This gives the best chance for long-term quality of life and a much lower chance of a complication called graft-versus-host disease.”

Ken’s three siblings were tested and Gina was a match.

His parents considered all the steps of the transplant process: Gina would need surgery to have her bone marrow extracted and would likely have some pain after the procedure. Ken would need chemotherapy to eliminate his own bone marrow, which comes with many side effects. Then he’d be in the hospital for several weeks after and with a high risk of infection.

“At the time he wasn’t having too many complications, so we decided to wait to do the transplant,” Miranda says.

An ICU stay

A few years later, Ken started experiencing more pain crises, which happen when sickle-shaped cells build up and block the flow in blood vessels.

Miranda remembers bringing him to the emergency room because his care team said to go there right away if he ever had a fever higher than 101.4˚F – an early warning sign of serious complications.

“We were driving to the ER and he was being playful, we were having this great conversation,” Miranda says. “I thought they were going to tell me I was crazy for bringing him in.”

But Ken’s oxygen was so low that he needed to be admitted to the hospital. Then he was transferred to the intensive care unit (ICU) and diagnosed with splenic sequestration, an emergency where sickle cells get trapped inside the spleen.

Gina remembers her brother’s pain crises and seeing him so sick in the hospital.

“At that point, I was like OK, this is really serious,” she says.

Re-considering a transplant

Ken’s experience in the ICU led his family to re-visit the idea of a transplant. Gina started researching sickle cell disease and was fascinated by the science behind transplants. But she was nervous about going under anesthesia and the pain donors described after the procedure.

She said yes to being his donor. About two weeks before the transplant, Ken began chemotherapy treatments to make space for Gina’s bone marrow.

Gina woke up at 5 a.m. on July 26, the day of the procedure – and it was over before she knew it. That same day, Ken received her bone marrow.

Gina had some pain in the hip where they extracted the bone marrow, but it faded after a few days.

Then, it was time to wait.

Waiting

Over the next few weeks, the care team monitored Ken closely, to make sure Gina’s bone marrow was starting to grow. He stayed in his hospital room to avoid infections, since he was vulnerable to them in the weeks that followed.

“Those weeks were long and a bit boring,” Miranda says. “It was like you had planted a seed and now you are waiting for it to grow. And seeds do not grow in one day.”

His parents spent all the time they could with him. Ken occupied himself playing Roblox while Miranda wrote songs in her notebook. His siblings came to see him and play games and visits from physical therapy, occupational therapy and child life helped pass the time.

“You could really tell that his care team are all experts at working with children because Ken was so comfortable with them, they built a great rapport," Oscar says.

They were relieved when testing revealed that only 11% of Ken's blood cells were sickle cells, compared to 86% pre-transplant.

“Anything below 50 percent can really benefit their health,” Dr. Aquino says.

After five weeks in the hospital, Ken finally went home.

A regular life

Ken needed to take it easy for a while but was grateful to be home with “his sisters, his couch, his mom’s spaghetti and his Legos.”

His family hopes that the transplant will mean fewer trips to the hospital and Ken being able to do all the things he wants to do – like playing basketball and keeping up with the other kids at school.

“We often tell families that our goal with a transplant is one year of hospital and recovery, for 90 good years of life,” Dr. Aquino says.

Gina is headed to college to study medical laboratory science, a field that sparked her interest, in part, from learning about sickle cell disease.

“Ken and I are really close and look the most alike. Plus, my other sisters are more scared of needles, so it worked out that I was the match,” Gina says. “I love that I could be that person for him.”

Explore treatment options for sickle cell disease

For some children with sickle cell disease, treatments such as stem cell transplant and gene therapy may offer a cure. Children’s Health specialists work closely with families to determine which treatment options may be appropriate based on a child’s health, disease severity and other factors. Learn more about our Sickle Cell Disease Program and curative options for the disease.