OAKLAND — For an 11-year-old, Gabriel Walker has lofty career goals. He wants to be either a mechanical engineer or a cardiothoracic surgeon who operates on the heart, lungs and other chest organs.
“I’m good,” Gabriel said when asked how he feels. That isn’t always the case, however. Sometimes the calm, quiet scholar — who is among thousands of children in California with sickle cell disease — feels tired or in pain, or is dehydrated, and he knows those symptoms can quickly escalate into a life-threatening situation.
Still, he tries to live an active life — he just received his white belt in karate. His favorite subject in school is math, “but I really like my science teacher,” he adds.
“Our day-to-day is the same as everybody else,” said his father, Casey Walker, a nuclear engineer. “The biggest problems come with pain crisis in sickle cell or if his hemoglobin drops or if he gets really tired. We have to look for things like that — there are certain triggers we all have to be aware of at home and at school.”
Every two months, Gabriel is driven by his parents from the family’s Vallejo home to UCSF Benioff Children’s Hospital Oakland, which has the largest and most comprehensive sickle cell program west of the Mississippi River.
The Oakland hospital’s Comprehensive Sickle Cell Center offers inpatient and outpatient services and a 20-bed infusion center hospital, where patients come in for transfusions, infusions and other treatments for the day. It treats 250 children and 250 adults a year with sickle cell disease and provides screening and treatment for stroke risk, hypertension and pulmonary and neurovascular disease.
UCSF Benioff Children’s Hospital Oakland is one of the few medical centers that receives National Heart, Lung and Blood Institute (NHLBI) grants specifically to treat adults with sickle cell disease. Last year it was awarded one such grant for $4.1 million, according to hospital spokeswoman Melinda Krigel.
The hospital has been a center for research in sickle cell disease for 35 years, and has led national and international multi-center trials in sickle cell disease research, including transfusion therapy, acute chest syndrome and bone marrow transplantation, Krigel said, adding that it’s one of the few centers currently conducting trials in cellular therapies for sickle cell patients.
In 2016, a team of Bay Area scientists — researchers from UCSF Benioff Children’s Hospital Oakland, UC Berkeley and the University of Utah — used a new gene-editing technique to fix the mutation that causes sickle cell disease, considered a breakthrough for a possible cure for the deadly immune system disease. The study was published in the Oct. 12, 2016, issue of Science Translational Medicine.
“This is an important advance because for the first time we show a level of correction in stem cells that should be sufficient for a clinical benefit in persons with sickle cell anemia,” said Dr. Mark Walters, a pediatric hematologist and oncologist and director of UCSF Benioff’s blood and marrow transplantation program.
Sickle cell is an inherited blood disorder that causes anemia. It’s a condition where there aren’t enough healthy red blood cells to carry oxygen throughout the body. Normally, red blood cells are round and flexible and move through a person’s blood vessels easily. In people who have sickle cell disease, the red blood cells are instead shaped like bananas or half-moons. The irregular shape of sickle cells cause them to become stuck in blood vessels, blocking the flow of blood and oxygen delivery.
“You can drink water, you can get good rest, you can provide nutrition, and despite doing everything you can to prevent complications, this is a disease where things can still happen along the way,” said Gabriel’s physician, Dr. Anne Marsh, director of the Pediatric Sickle Cell Clinic at UCSF Benioff Children’s Hospital Oakland.
“Gabriel is an incredible young man,” Marsh continued. “”He’s bright, he’s articulate, he’s talented, he’s resilient, and he’s surrounded by a family that has been incredibly loving and supportive.”
Gabriel was born at John Muir Medical Center in Walnut Creek, where he was diagnosed with sickle cell. The Walkers were told by John Muir officials that UCSF Benioff Children’s Hospital Oakland would provide the best care for their son.
Casey Walker said he and his wife Andrea, a computer programmer in curriculum design, both have the sickle cell trait, as do Gabriel’s two older sisters.
“So, when we found out, we weren’t surprised,” Walker said. “We weren’t devastated or anything.”
The Walkers made the first appointment for Gabriel when he was only 2 months old. At 6 months, he developed his first crisis — high fever, lots of coughing and wheezing. He was diagnosed with Acute Chest Syndrome (ACS), which is common among sickle cell patients. For the next six months, Casey Walker said his son returned to the Oakland hospital at least once a month because of it, sometimes staying four to five days in the hospital.
After his first birthday, Gabriel was injected with hydroxyurea, the first successful medicine in 100 years to treat sickle cell disease.
“He’s been a poster child for hydroxyurea since he’s been on it,” Walker said. “His fetal hemoglobin got higher, and he hasn’t had more than four or five hospital stays since that first year.”
However, Marsh said, “Gabriel has had a rough year (in 2018). He’s had a lot in terms of complications.”
On New Year’s Eve, on a trip to Universal Studios in Orlando, Florida, Gabriel said he suddenly couldn’t breathe while flying and took inhaler medication, but had to stop because his heart was beating too fast.
“We contacted Children’s and they told us to take a blood test — and told us where to go to take a blood test,” Walker said. “They’re still directing his care. Mind you, this is from Oakland — and we’re in Orlando, Florida.”
Gabriel’s hemoglobin was low and he was admitted into the Arnold Palmer Hospital for Children. He received a transfusion that night and was released the next morning — and got to see The Wizarding World of Harry Potter.
In addition, Gabriel had his spleen removed in April after it had enlarged. The condition lowers the amount of oxygen-carrying red blood cells in the body. In addition, as a result of the operation, he developed pneumonia-like symptoms of Acute Chest Syndrome.
“I think his family has done a particularly good job at being diligent along the way and working together with us as a team — this really does take a team approach,” Marsh said. “To provide comprehensive care for a child, the communication we’ve had with (Gabriel’s parents) Casey and Andrea around Gabriel’s health has been really outstanding.”
At Wednesday’s checkup, Gabriel was quick to tell Marsh what refill he needed and noted some pain in his arm — which Marsh wanted to monitor in the next week.
“You can see he has lofty aspirations,” Marsh said. “We want to help do everything we can to allow him to continue down that road of success. I want to meet him someday when he’s operating on people’s chests.”
FYI
For more information about UCSF Benioff Children’s Hospital Oakland’s Comprehensive Sickle Cell Program: https://www.childrenshospitaloakland.org/main/departments-services/comprehensive-center-for-sickle-cell-disease-41.aspx
For tips parents of children with sickle cell: https://www.childrenshospitaloakland.org/main/tips-for-parents-of-kids-with-sickle-cell-disease.aspx
