PreviousAubrey was planning for a stem cell transplant to cure her aplastic anemia, then everything fell apart
One phrase has come to define this past year of Aubrey Rivera’s life: “We have a small window of opportunity.” Every time Aubrey and her mom heard it, Aubrey was facing incredible odds to stay alive. Every time, she pulled through, thanks to her strength, her family’s hope, and her care team.
Aubrey is an old soul, as her mom, Margarita, puts it. Her music tastes range from ’80s and ’90s rap to Michael Jackson and Fleetwood Mac. She also loves running, sports, and cooking. Her doctors describe her as extremely smart, a curious kid who is always listening and asks great questions.
Last August, Aubrey had just celebrated her 12th birthday when she started experiencing shortness of breath, her heart raced, she felt fatigued, and she started developing bruises. After a visit to urgent care, she ended up in the ER of a hospital close to home. After many tests, she was diagnosed with severe aplastic anemia.
Aplastic anemia is a rare blood disorder whereby the bone marrow stops making enough new blood cells, including white blood cells, red blood cells, and platelets. The missing white blood cells make people with aplastic anemia more susceptible to infections. The lack of red blood cells causes fatigue and shortness of breath, as oxygen doesn’t move as easily around the body. Not having enough platelets can cause bruising and bleeding.
Stem cell transplantation, also called bone marrow transplantation, is a curative option for aplastic anemia, and it was presented to Aubrey and her family early on.
“At that time, I didn’t really know what it meant,” Aubrey said.
The doctors gave Aubrey and her family a choice of different hospitals to perform the stem cell transplant. The family picked Lucile Packard Children’s Hospital Stanford because they had family nearby.
They were introduced to Jessie Alexander, MD, MSCR, a pediatric stem cell transplant specialist at Stanford Medicine Children’s Health. Aubrey’s sisters were tested to determine if they were good matches to donate stem cells to her.
While neither of Aubrey’s sisters was a perfect match, Dr. Alexander explained that stem cell transplantation was still possible.
“A newer method is a haploidentical, or half matched, transplant, which opens up your options a lot,” she said. “Biological parents are expected to be at least half matched, and each sibling has a one-in-two chance of being at least half matched. Using a special method of administering some of the chemotherapy after the stem cell infusion, cells from a haploidentical donor can be accepted and functional. This method has shown great promise in patients with severe aplastic anemia.”
Aubrey received blood and platelet transfusions to temporarily restore her missing blood cells until stem cell transplant. Because of her low levels of white blood cells, she started getting sick more frequently and more severely. She had an E. coli infection and started developing a cough that escalated into severe pneumonia. She was admitted to the intensive care unit at a hospital close to home.
“Aubrey was such a trouper—she was talking, jumping up onto the bed, listening to what was going on,” said Margarita. “She’s always wanted to know what was going on.”
Aubrey’s doctors wanted to put in a chest tube to help her breathe. Margarita stepped out of her room during the procedure.
“Within minutes, everything just went sideways,” she said. “I saw Aubrey struggling for air and the doctors saying they needed to intubate her. That whole night, the doctors kept telling me the situation was hour by hour.”
That was when Margarita heard the “small window of opportunity” phrase for the first time during Aubrey’s illness.
“By the next day, her doctors said they had stabilized her a little, and they thought they had a chance to get her to Stanford, so I said please do,” she said.
At Stanford Children’s, Dr. Alexander was working to make space for Aubrey in the Pediatric Intensive Care Unit (PICU) and assemble a team of experts to care for her.
“I spent that weekend on the phone with the other hospital, trying to talk through what was going on,” Dr. Alexander said. “We thought we were going to lose her, and were just trying to figure out how we could get her to us. Thinking of a patient like this for a stem cell transplant sounds crazy, totally preposterous. But if we didn’t take her to definitive therapy, she would have died. The thing that really sets our hospital apart is the significant multidisciplinary collaboration that needed to happen in a precisely orchestrated way and the multiple individuals who were willing to think outside the box and place patient opportunity and life ahead of what seemed most logical or most realistic.”
Aubrey arrived at Stanford via helicopter on Nov. 8.
She was on a ventilator and was experiencing multiple organ dysfunction. Her stomach was hugely distended, she was very swollen, and she had gained almost 40 pounds.
“Every day, the providers just really put their heads together, and they kept trying and trying,” Margarita said. “They told me to call family to say their goodbyes because they didn’t know if she would make it.”
The doctors knew there was a fungal infection in Aubrey’s blood, and every time they tested her blood, she had no detectable levels of neutrophils, one type of white blood cells that help fight fungal infections.
Along with medications, they also attacked the infection with granulocytes, a type of white blood cell that includes neutrophils and other infection-fighting cells.
“This is the point where you do a hail Mary,” said Anju Goyal, MD, a pediatric hematologist who cared for Aubrey. “She had no other option or she would die.”
Over six weeks, Aubrey was given 23 infusions of granulocytes. She slowly improved and was eventually extubated and weaned off blood pressure support.
Once again, there was another small window of opportunity. Aubrey’s family gathered with many members of her care team, including Dr. Alexander, Dr. Goyal, and representatives from the PICU, infectious diseases, and palliative care teams, as well as nursing staff.
“There must have been 20–25 people crammed in that room discussing what we should do next,” said Dr. Goyal. “Her family said, ‘She’s a fighter and we want to give her a chance.’ We were going to try to honor the family’s wishes.”
During the discussion, Dr. Alexander told Margarita that she estimated a 5% to 10% chance that Aubrey would successfully make it through the transplant and be cured of her aplastic anemia.
“I told mom, there’s actually a really decent chance that she will die during the conditioning, the preparation for the transplant,” Dr. Alexander said. “But if we don’t do the transplant, she will certainly die. She has no neutrophils, so unless she gets donor cells, she’s not going to get better. However, without taking a leap, there is a significant risk she’s going to get even worse, and then she’s really not going to be a candidate for a stem cell transplant.”
For Aubrey’s family, there was never a doubt that they would take any opportunity to get Aubrey well.
“When we transferred Aubrey to Stanford, the last thing on my mind was Aubrey ever being able to get a stem cell transplant because she was so sick,” said Margarita. “So when I heard Dr. Alexander say those famous words, that we had a small window of opportunity, I knew, even though they gave me the worst-case scenarios, in my gut I knew Aubrey was stronger than all that.”
Aubrey’s sister, Alyssa, agreed to donate stem cells to Aubrey.
“Aubrey’s sister said, ‘We have to try,’” said Margarita. “I know in my heart, we’re doing it. Aubrey’s doctors are giving me this opportunity—that’s hope.”
Aubrey started the conditioning regimen for her stem cell transplant, which used chemotherapy and radiation to suppress Aubrey’s immune system and remove any remaining cells in her bone marrow to make room for her sister’s cells. Somehow, Aubrey pushed through it.
“Every day, she would get chemo, get a fever, and curl up in a little ball,” said Margarita. “She’d get some Tylenol and then an hour or two later, there she was on her iPad. I thought, ‘Look at you, girl, you’re flying right through this.’ She was amazing through each day of conditioning, blowing the minds of her providers.”
Then, on Dec. 23, Aubrey received her stem cell transplant, surrounded by family.
“It was a wonderful day—that was my Christmas miracle,” Margarita said. “Everyone was on call ready for the worst-case scenario, but it didn’t happen; Aubrey tolerated it.”
On Christmas Day, Aubrey also received a stuffed St. Bernard named Patchy that she takes everywhere, from CT scans to biopsies.
“Everyone on the care team knows who Patchy is,” she said.
Two weeks after the stem cell infusion, the donor cells engrafted, or settled into Aubrey’s bone marrow, and started circulating new cells. She now has the same number of blood cells as someone without aplastic anemia, meaning she will have more energy and fewer infections.
Aubrey slowly got stronger and started doing physical therapy to be able to sit up, then stand, then walk. Her next goal: get back to running, which she loves.
Even the fact that Aubrey is walking surprises some of her providers who saw her at her sickest.
“It’s surreal—I saw her and her mom walking in the lobby the other day, and it’s just extraordinary. I wasn’t sure she was ever going to leave that PICU bed,” said Dr. Goyal. “I’m so thankful that she is alive; truthfully, she feels very much like a miracle to me.”
Aubrey said she doesn’t feel back to normal yet, but she’s using her considerable strength to get there. She’s excited to return home in a few weeks, see her friends and (non-stuffed) dog, and start seventh grade in the fall.
She hopes that her story can help inspire other kids who are going through impossibly difficult times.
“I hope other kids know you’re not alone; no matter what comes at you, ready or not, you’ll always have some type of strength, whether you see it or not,” she said. “Don’t be scared.”
Margarita offered the same message of hope for parents and families.
“Don’t give up hope,” she said. “Kids are so strong—never give up on them. They’re resilient, no matter what; they’re super-strong,” she said. “Thank you to all of the doctors and nurses who cared for Aubrey—they had hope also. Nobody ever gave up on her: They tried and tried and tried, and she pulled through.”
Learn more about stem cell transplantation at Stanford Children’s >
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