Photo by Robert Hood / Fred Hutch News Service
If you drive by the Adams’ house — tucked at the end of a cul-de-sac in Redmond, Washington — there’s a decent chance you’ll spot Andrew Adams sitting on his front porch, quietly snuggling a chicken.
Andrew, who is 23, does many things quietly — he’s autistic and has little use for small talk. He also does many things with a chicken by his side. When Andrew is home, if he’s not sitting on the porch, he’s often playing video games in his room with his favorite bird — Daisy Duke — on his lap.
Daisy Duke is a stately, rotund Buff Orpington chicken with feathers the color of light caramel. She and her three fellow coop-mates act more like housecats than hens.
“They’ve been handled since they were chicks,” said Kristin Adams, Andrew’s mother.
Andrew calls them his “chickadees” and sometimes shares more with the birds than with his human family, Kristin said. In turn, the hens talk to him in a series of soft chirps and burbles. He’s been a chicken owner since the age of 8, when he instantly bonded with a little brown Araucana chick at a feed store that he named Frightful, after a hawk in a favorite book.
A few months after Frightful came into their lives, Kristin overheard her son telling his beloved pet that he thought his body was trying to kill him. Since birth, Andrew had experienced bouts of severe abdominal pain, high fevers and painful ulcers in his mouth that made eating impossible
It would be months more before the family got anywhere close to understanding what was causing the symptoms. And it would be many more years before he finally received an experimental stem cell transplant at age 18 in a last-ditch effort to stop the ravages his immune cells were inflicting on his body.
A team of specialists from Seattle Children’s Hospital, Seattle Cancer Care Alliance and Fred Hutchinson Cancer Research Center got Andrew to the point he is today, on the other side of a transplant that’s allowed him to turn a huge corner from his life-threatening and extremely rare genetic disorder known as Trisomy 8 Mosaicism.
But Kristin credits someone else for helping pull her son through the ordeal: Frightful. The beloved chicken died three years ago, not long after Andrew was finally released from the hospital to live at home again, after recovering from his transplant.
When Kristin prods her son to talk about Frightful now, he simply says: “She was quite the chicken.”
Andrew also referred to Frightful as his “secret-keeper,” Kristin said. Recently, she leant more words to that succinct phrase about Andrew and his secret-keeper. In a memoir published in April titled “The Chicken Who Saved Us: The Remarkable Story of Andrew and Frightful,” she describes the bond between son and fowl — and Andrew’s journey from a life full of pain to a rare diagnosis that raised more questions than it answered and, finally, to a transplant.
One of 53
Very early on in Andrew’s life, his parents began to worry that something was not quite right. They were in and out of the pediatrician’s office with alarming regularity with their feverish, inconsolable baby, but the doctors would always say Andrew just had the standard cold, flu or other virus that was going around.
By the time Andrew was a toddler, those explanations weren’t cutting it any more.
“I had that mommy gut feeling that this isn’t just a cold, this isn’t just a flu,” Kristin said.
Photo courtesy of Kristin Adams
It would be several more years before that gut feeling led to a diagnosis that explained Andrew’s symptoms. By third grade, Andrew’s cyclical illnesses had become routine for the family — and they were getting worse, often resulting in hospitalization.
They came on about once a month, starting with a sudden, high fever, Kristin said. She’d drop him off at school seemingly fine, then get a call a few hours later that he was in the nurse’s office with a temperature of 105 F. Then, he’d develop blisters or ulcers around his mouth. Then searing pain in his stomach. Each episode would last several days, maybe up to a week.
Complicating matters even more was Andrew’s inability to communicate the nuances of his symptoms or his feelings. His parents received the neurologist’s letter with Andrew’s diagnosis of autism when he was 3 years old — the same day he said his first words.
Even without a full description of his symptoms, Andrew’s pain and the accompanying fevers were clear to his parents. The family and an ever-changing team of doctors muddled through, trying treatment after treatment with no explanation for the illnesses and no way to prevent them.
Finally, at age 9, he received a diagnosis thanks to an immunologist at Seattle Children’s Hospital, Dr. Troy Torgerson, and a team of human genome researchers at the National Institutes of Health in Bethesda, Maryland. The family learned Andrew had three copies of one of his chromosomes in some of his cells but not others. Known as a mosaic trisomy, Andrew’s case was especially rare because of the particular chromosome affected in his body, chromosome 8.
Most trisomies — where cells carry three copies of a given chromosome instead of two — are fatal, causing miscarriages very early in pregnancy. (Trisomy 21, which causes Down syndrome, is a notable exception.) And even mosaic cases like Andrew’s, where some of a baby’s cells have the normal two copies while some carry three versions of chromosome 8, often lead to miscarriage.
The diagnosis, while definitive, didn’t answer many questions for the family. Trisomy 8 mosaicism is incredibly rare and its symptoms are variable. At the time Andrew was diagnosed, there were only 52 other people reported with the disorder in the world. And as far as anyone knew, none of the others had anything resembling Andrew’s symptoms.
For the most part, the family was left on the same path that it had been on before the diagnosis. For the next several years, Andrew was treated with an escalating series of immune-suppressing drugs to try to quell the crests of fevers and pain. They bounced around from specialist to specialist, department to department in the hospital.
Ultimately, things got worse.
“We hit critical mass when he was a sophomore in high school,” Kristin said. “[The spells] basically overlapped to where it was every single day; it never resolved itself.”
Andrew was sick so often that he had to drop out of school — it was supposed to be temporary but his hiatus ended up lasting several years. At 5 feet 10 inches tall, he weighed only 100 pounds. Eating was excruciating — he threw up all the time and the feeding tube he’d had placed could only do so much. During one particularly painful spell Kristin describes in her memoir, in which Andrew was vomiting blood and hadn’t been able to eat in weeks, she carried her teenage son into the emergency room in her arms. He didn’t leave the hospital again for nearly eight months.
Andrew missed Frightful terribly during that time, and even when he was home he often couldn’t be in contact with the bird because his immune system was so weak. His younger sister, Hannah, who would go on to be his bone marrow donor, came up with the idea of fixing an iPad to the chicken coop so the boy and chicken could Facetime together.
It wasn’t the same, though.
During his long hospital stay, the family reconnected with Torgerson, the immunologist who’d originally helped diagnose Andrew years earlier. He went to work on their behalf and, through colleagues, found a young man in Japan with trisomy 8 mosaicism who seemed to share Andrew’s painful symptoms. That man’s doctor had identified certain immune molecules — cytokines — that had gone haywire in his body. Torgerson tested Andrew’s bone marrow — the birthplace of all his body’s immune cells — and found that it was chock full of cytokines and cells carrying three copies of chromosome 8. Somehow, the genetic abnormality had taken over his marrow entirely. And it was causing the immune cells to viciously attack the cells they perceived as foreign, which amounted to much of Andrew’s body.
In essence, Andrew was right when he’d told Frightful eight years earlier that his body was killing itself.
This was the turning point, Kristin said. The immunologist believed that Andrew needed a reboot in the form of a bone marrow transplant. Kristin and her husband, Jon Adams, agreed.
One among many
With that decision, Andrew and his family entered an even smaller world, a world in which he was the only person on the planet — as far as his family knows — with trisomy 8 mosaicism to receive a bone marrow transplant for the condition.
Because of his young age and the rarity of his disorder, the family connected with a doctor who specializes in rare diseases — Fred Hutch and Seattle Children’s transplantation researcher and physician Dr. Lauri Burroughs. A pediatric oncologist by training, Burroughs now specializes in “non-cancer” cases — children with non-malignant blood disorders who can be treated or cured by a transplant.
Fred Hutch file
Although most of her patients’ conditions aren’t quite as unusual as Andrew’s, many of them do have very rare diseases, Burroughs said. Taken together, these patients are a force to be reckoned with. Children with non-malignant blood disorders make up roughly a third of the pediatric patients undergoing transplants in the U.S. Like Andrew, many of Burroughs’ patients have genetic conditions that alter how their immune systems function. And, like Andrew, many are incredibly sick going into transplantation, which can be a problem since the high doses of chemotherapy (with or without radiation) typically used to “condition” patients for a transplant can be very toxic.
Burroughs’ own research has focused on developing gentler but still-effective methods of pre-transplant conditioning for this unique group of patients. And gentle treatment was exactly what Andrew needed, Burroughs said.
“We were worried about giving him a more aggressive transplant-conditioning regimen because we weren’t sure he would survive it,” she said.
Instead, the team used a version of transplantation originally developed at the Hutch by transplantation researcher Dr. Rainer Storb, known as a “mini-transplant.”
At the time of Andrew’s treatment, mini-transplants were used fairly routinely in adults too sick or weak for a traditional transplant, typically the elderly. But their use in children was still experimental — her team and other researchers had published some data, Burroughs said, but not much. Andrew’s doctors ended up designing a new transplant protocol just for him. His sister Hannah, age 15, was his bone marrow donor.
When asked what he remembered about that time in the hospital, Andrew replied quickly: “Pure pain.” He also remembered playing a special Hunger Games board game prototype someone had given him, in fact, he had a lot more to say about the Hunger Games than about his symptoms.
“Do you want to tell Rachel about when you made red velvet cake in the hospital?” Kristen prompted during the interview. Andrew has had a long interest in baking and cooking and is now working as a part-time food prep chef on the Microsoft campus.
“I don’t remember that well,” Andrew said.
“Do you remember some of it?”
“I remember putting a whole gallon of food coloring in it.”
Her son also did a lot of drawing and storytelling through his illnesses, Kristin said. He and his caretakers would make up stories based on Frightful, his favorite video game characters and other superheroes. He’d draw fantastical stories, but sometimes the chicken superhero character would sport both a cape and a Hickman line (a type of semi-permanent catheter used to administer chemo). Kristin thought these stories helped Andrew cope with his difficult recovery.
After they’d decided on the transplant, the doctors quickly tested Hannah and found she was a perfect match. But then things seemed to grind to a halt as the clinical teams tried to figure out Andrew’s transplant protocol and debated whether he was well enough to go through with the procedure, Burroughs said.
Part of this was due to the lack of data on pediatric mini-transplants. But part was because Andrew had been so sick for so long, and his case was so different. No one was sure how his body would withstand the treatments once the transplant got underway — or even if it would work.
“We didn’t know if a transplant would actually cure his disease,” Burroughs said. “Yet we knew that because of his disease and how sick he was, he had a high chance of not tolerating the transplant. That made us concerned. We thought it would work; we hoped it would work; but there was no guarantee.”
All that uncertainty boiled down to a lot of waiting time for the family.
“That was probably the most difficult time for my husband and I, because we had committed to OK, we’re willing to accept what the consequences are, one way or the other, for transplant,” Kristin said. “Watching him being in a place where he was suffering was really, really hard.”
They’d already been watching that suffering for too many years, she said. She wasn’t surprised that Andrew didn’t remember many details about those months.
“He was really sick. He was pretty much on a morphine drip at that time. There were times that he wasn’t very conscious,” she said. But for her and for Jon, once they’d committed to a transplant, the choice was clear. Even though they knew there was a risk that the conditioning for the transplant could be toxic or even deadly to Andrew, they couldn’t watch him in pain any more without doing something.
“Having watched our son for 10 really critical years of some very, very intense pain, the option of transplant and even just the process of it seemed less painful than the other option,” she said.
Photo by Robert Hood / Fred Hutch News Service
‘I can see him taking a deep breath’
Once the transplant process got underway, they noticed a change in Andrew, Kristin said. The conditioning regimen, gentle as it was, reigned in some of Andrew’s haywire immune cells, giving him a small amount of relief from his pain. It was like “putting a fire extinguisher on,” Kristin said.
“We could kind of see him come out of a morphine fog,” she said. “It was miniscule, it wasn’t like he was better, but we could see that maybe something was going to shift.”
Andrew still required some chemotherapy and radiation, which wreaked their own havoc on his weak body. Transplant day was especially grueling, Kristin wrote in her book — Andrew had to undergo two rounds of radiation at the same time that Hannah had her marrow harvested. In the evening, he received his sister’s cells.
The transplant itself was just one day in a very long illness, and when Kristin tells the family’s story, she doesn’t dwell for long on that day itself. They had to wait a few weeks to see whether the transplant would work. But it did — Hannah’s cells engrafted and Andrew’s symptoms began to slowly subside.
It hasn’t been a straight path, Kristin said. “It was up and down for a long time,” she said. “It was a very slow process. When he went into it he was so, so ill. We had a long ways to crawl up and out of it.”
Andrew had some bouts of graft-vs.-host disease, in which donor cells attack the transplant recipient’s healthy cells. A year ago, four years after his transplant, he was discharged from the frequent post-transplant monitoring.
It wouldn’t be accurate to say the family is finally getting back to normal, because Andrew’s "normal" before was riddled with bouts of tremendous pain. It also wouldn’t be accurate to say he’s now 100 percent healthy. But things are getting better.
Andrew still lives at home, but he finished high school and he has a job that he loves. He’s more independent. Kristin, who left her job as an interior designer when Andrew’s illness was at its peak, finally had the time and breathing space to take writing classes and finish her book.
She gestured proudly at a single box on her kitchen counter, full but not overflowing with Andrew’s medication bottles. “This is our arsenal,” she said. “It used to be three boxes deep.” Andrew’s now able to be responsible for the pills on his own.
“For the last year, it’s been really, really great,” Kristin said. “I can just kind of see him taking a deep breath.”
Kristin rooted around in a kitchen drawer for her son’s drawings featuring the chicken superheroes that he made in the hospital, but she couldn’t find them. She prompted Andrew to talk about his drawing.
He likes to draw, he said, but he doesn’t do it much anymore.
Did he remember where he put his drawings from the hospital?
“I don’t have any of them. I didn’t want them anymore, so I got rid of them,” he said. “I don’t need them anymore. I really didn’t.”
Rachel Tompa, a staff writer at Fred Hutchinson Cancer Research Center, joined Fred Hutch in 2009 as an editor working with infectious disease researchers and has since written about topics ranging from nanotechnology to global health. She has a Ph.D. in molecular biology from the University of California, San Francisco and a certificate in science writing from the University of California, Santa Cruz. Reach her at firstname.lastname@example.org or follow her on Twitter @Rachel_Tompa.
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