Germelle Scott was just 10 weeks old when he was admitted to Children’s Wisconsin for the first time. But it would not be his last hospital admission. In fact, for the first three years of Germelle’s life, he spent more days in the hospital than at home.
“He’s what you call a frequent admitter,” said Trina, Germelle’s mom. “He would have a crisis and go to the hospital for four or five days, then come home for two or three days and then get sick again.”
Germelle was born on August 9, 2015. One month later, he was diagnosed with sickle cell disease.
Normal blood cells are smooth and round, allowing them to freely pass through veins and arteries without issue. But for those with sickle cell disease, the blood cells are misshapen, like crescents. This causes them to get stuck together and clog up the flow of blood. When this happens, it can lead to intense pain, anemia (too few red blood cells), fatigue as well as infection and stroke.
Since he inherited an affected gene from both parents, Germelle had what is known as hemoglobin SS sickle cell anemia. Making up about 65 percent of all cases, it’s the most common type of sickle cell disease. But Germelle had an especially serious case.
“Germelle had very severe sickle cell disease,” said Larisa Broglie, MD, a Pediatric Blood and Marrow Transplant Specialist at the MACC Fund Center for Cancer and Blood Disorders at Children’s Wisconsin. “He had an extraordinary number of hospitalizations for pain, and it was also causing damage to organs, including his liver. It was certainly impairing his ability to participate in school and live a normal life.”
In addition to the weekly hospital admissions for his sickle cell episodes, Germelle suffered a stroke and had his gallbladder removed when he was 2 years old, and then he had another stroke when he was 3. It was after that second stroke that Trina and Germelle’s care team began to discuss a bone marrow transplant.
Finding a Match
Bone marrow is the soft substance inside of bones that produces blood cells — red blood cells that carry oxygen throughout the body and white blood cells that fight off disease and infection.
The first step in a bone marrow transplant is finding a matching donor.
“We want the immune systems to be as close a match as possible,” said Dr. Broglie. “A closer match allows those cells to get into the body and become familiar with this new location and this new person a little easier. It’s a higher chance of success.”
Siblings are usually the best bet — with a 25 percent chance of being a full match and a 50 percent chance of being a half match. But neither of Germelle’s two brothers were a full match.
Since a child gets half of their bone marrow from each parent, both Trina and Germelle’s father were a half match. As recently as 10 years ago, a half match wasn’t considered close enough to move forward with a bone marrow transplant.
“Over the past decade or so, there's been a big shift in bone marrow transplants and we've gotten very good at using half matched donors and parents,” said Dr. Broglie, who is also an associate professor of pediatric blood and marrow transplantation at the Medical College of Wisconsin, the academic partner of Children’s Wisconsin. “We can now safely offer transplants to families with a parent as a donor.”
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In fact, Children’s Wisconsin is part of a HaploIdentical Sickle Cell consortium that has been researching the use of half-match parent bone marrow donors to treat sickle cell disease in children.
“20 years ago, we had a lot of problems with infections and graft-versus-host disease,” said Julie-An Talano, MD, Medical Director of Blood and Marrow Transplant at Children's Wisconsin and primary investigator of the HaploIdentical Sickle Cell consortium. “We have learned a lot over the years about how to safely do transplants for patients with a half match donor.”
How a bone marrow transplant typically works is doctors take bone marrow or peripheral stem cells from a donor and give them to a patient, after the patient has received chemotherapy to remove their own blood forming cells. This process, however, places the patient at high risk for infection.
Through this clinical trial, the doctors add some of the donor T-cells back to reduce those risks. But it’s a highly delicate balance because too many T-cells can cause the donor’s immune system to attack the patient’s cells, a condition known as graft-versus-host disease. With that right balance, the half-match transplant can be successful.
“This has proven to be a safe way to do transplants, particularly with African Americans who have sickle cell disease,” said Dr. Talano, who is also a professor of hematology and oncology at the Medical College of Wisconsin. “When a sibling isn’t a match, we search the national marrow donor registry. Unfortunately, there are not a lot of minorities in the donor registries, so it is difficult to find a complete unrelated donor match. Being able to successfully use a related half-match donor makes transplant much more accessible.”
Children’s Wisconsin and the Medical College of Wisconsin are one of nine centers in the United States participating in this trial — and the only one in the Midwest. To date over 40 patients have been treated on this protocol with sickle cell disease.
It was because of this work that Dr. Broglie and the rest of Germelle’s care team felt comfortable and confident moving forward with a bone marrow transplant using Trina as his donor.
For a mother, giving life to a child is one of the most precious and rewarding things imaginable. But doing it for a second time is something else altogether.
“I don’t really know how to explain it,” said Trina. “I was able to give him a second chance at life — a kid life, a healthy life.”
Counting Down to Day Zero
Once Trina was identified as the donor, the process was long and deliberate. There were frequent doctor’s appointments to gather information, perform tests and go over the process in depth — all of its potential risks and benefits.
“A bone marrow transplant is a difficult process,” said Dr. Broglie. “There are very prolonged hospitalizations and serious risks. We meet with families multiple times because it’s important for parents to hear everything so they can make an informed decision.”
“It's a hard process to go through,” said Dr. Talano. “But it really can change the course of a patient’s life.”
Dr. Broglie first met with Germelle and his family in 2019. They met again in 2020 and a third time in August 2021. In December 2021, Trina made the ultimate decision to move forward with a bone marrow transplant. From there, the process moved quickly.
The next month, January 2022, Germelle began preparation. He started taking daily medication to suppress his immune system.
On March 1, 2022, Germelle was admitted to Children’s Wisconsin and underwent a round of chemotherapy. This was done for two reasons. First, his current bone marrow needed to be eliminated to make room for the donation. Second, his immune system needed to be fully wiped out so that it wouldn’t attack the donor marrow once implanted.
At this same time, Trina was receiving four shots a day for two weeks to help boost her bone marrow production.
Finally, transplant day arrived — March 18, 2022. Doctors call it “day zero.”
The procedure was simple enough. Doctors hooked Germelle up to an IV and over an hour or so, the new bone marrow flowed into his blood stream. In general, it takes about two to three weeks for those new cells to fully implant and start producing new blood cells.
Finally, on May 18, 2022, after 61 days in the hospital, Germelle was ready to go home.
“It was hard, a lot of balancing, a lot of back and forth,” said Trina. “But the MACC Fund Center team were so great and gave me so much support. They became family.”
A Second Chance at Life
Once home, it didn’t take long for Germelle to adapt to his new life. He was reborn. For the first time in his life, he could be a kid. Before, he couldn’t spend a lot of time outside — the summer heat would cause him to spike a fever or the chill of winter would trigger a crisis.
“Within the first month of being home, he was showing signs of being totally healthy and normal,” said Trina. “He was able to do things he could never do. Simple things like playing with the hose, he was so amazed by.”
For his 7th birthday, Trina threw him a pool party. It was the first time Germelle had ever swam in a pool.
“He was so excited,” said Trina. “I couldn’t keep him out of the water.”
Today, two years since his transplant, Germelle is doing great. He’s off all his medications and he hasn’t been admitted to the hospital since. While Dr. Broglie is hesitant to say “cured,” Germelle no longer has symptoms of sickle cell disease.
Looking back at everything they’ve been through as a family, Trina can’t help but be grateful. Grateful for all the support she received from her care team, grateful that her son is healthy and has a second chance at life.
For Germelle, even though he didn’t fully understand it at the time, he still remembers.
“Now that he's 9, he understands,” said Trina. “Out of the blue he’ll say, ‘I remember when my back used to hurt’ or ‘I remember when I used to spend so much time in the hospital.’ He's just really glad it's behind him because he's just a kid now.”
