Aaden Adams remembers waking up in his room in the Cardiac Intensive Care Unit (CICU) at Seattle Children’s Hospital and asking for red Popsicles.
“Everybody wanted me to talk and I wanted a red Popsicle,” said the precocious 6-year-old with a face full of freckles. “So I asked for Popsicles. Do you remember that, Mom?”
“Yes, we remember that well,” answered Aaden’s mom, Cheree Adams. “That was a good day. That was the day we knew that you were on the right path. We knew that you were coming back to us.”
Just two weeks prior, Aaden, who was born with a congenital heart defect, was so near death that his parents were preparing themselves for the reality that he might not make it out of the hospital. It was a situation they had not even considered.
“He came here for a pretty minor procedure,” said Andrew Adams, Aaden’s father. “He was supposed to be in and out of the operating room, but then his body just shut down. His heart wouldn’t restart.”
Dr. Erin Albers, Aaden’s attending cardiologist, said the complication was so unusual that no one on the care team had seen it before.
“When Aaden came off of bypass his left ventricle wasn’t working,” she said. “We don’t know why it happened, but after 24 hours when his situation didn’t change, we assumed he would not recover and began looking to a heart transplant.”
But Aaden’s road to transplant was not easy. In fact, it seemed his body was fighting his care team every step of the way.
“We were in uncharted territory”
While on Extracorporeal Membrane Oxygenation (ECMO), a machine that functions for the heart and lungs when the organs fail or need to rest, Aaden developed a resistance to a common anticoagulation drug called Heparin. The drug helps prevent blood clots, which can be deadly, especially for patients who are on ECMO.
“I got a call from the evening shift pharmacist that Aaden needed to go off of Heparin,” said Christa Kirk, the Seattle Children’s CICU pharmacist who had previously been working with Aaden and asked to be paged if anything came up. “There is another drug, Argatroban, that also works as an anticoagulant but there is very little literature on how to manage patients on ECMO with that medication, much less a pediatric patient. We were in uncharted territory.”
Kirk’s task was daunting. She needed to determine exactly how much Argatroban to give Aaden not only while he was waiting on ECMO, but also when it came time for his heart transplant surgery – too little and his blood would likely clot; too much and he could bleed to death. She read as much literature as she could find and consulted with colleagues across the United States. About six hours after the initial call, she had a recommendation. Aaden’s entire care team then gathered to discuss his situation and decide how to proceed.
“That’s one of the things I have always loved about working (at Seattle Children’s),” Kirk said. “We truly work as a team where experts from a variety of fields come together and are invested in deciding what’s best for the patient.”
A donor heart comes, but more hurdles emerge
The Argatroban worked and Aaden was surviving on ECMO, waiting for his new heart. Two weeks later, the team received news that a donor heart was available. It seemed everything was coming together, that was, until Aaden’s body decided to throw another wrench in the plan. He developed antibodies against several tissue types, which would make it very difficult for his body to accept a donor heart, Albers said.
“There was a high risk of Aaden’s body attacking the donor heart,” Albers said. “We were faced with the decision to leave him on the high-risk ECMO or take the donor heart and figure out a protocol that would prevent his body from rejecting the heart.”
Albers added, “We weigh risks and benefits every day. In Aaden’s case, we put our heads together and made a team decision that – given our experience and success in treating antibody mediated rejection – the risks of accepting this heart for him were far less than allowing him to wait longer on ECMO with all of the ongoing anticoagulation concerns in addition to the cumulative risks of ECMO itself.”
While the care team is frequently faced with making difficult decisions, Aaden’s case was especially tough for them because everything had transpired so quickly, Albers said.
“Most of the time, when we do a transplant, the patients have been here for a long time and we have time to process the various options,” she said. “This was so different. This was an asymptomatic child who came in for an elective procedure, and suddenly he needed a heart transplant. I can’t imagine how difficult this situation was for his parents.”
Andrew and Cheree Adams said they “took it one day at a time.”
“Honestly, I don’t even remember how they figured it all out,” said Andrew Adams. “They talked to us constantly and we trusted them completely knowing they were doing everything they could to save him, but we were preparing. We didn’t know which way things were going to go.”
Dr. Jonathan Chen, chief of pediatric cardiovascular surgery and co-director of Seattle Children’s Heart Center, said that patients like Aaden usually don’t get a donor heart because it’s so difficult to find a heart that’s a perfect match to their body’s specific needs. But the Seattle Children’s team was not ready to give up, and worked to develop a unique approach to helping Aaden’s body accept the heart that was necessary to save his life.
“Aaden is a fighter,” Chen said. “He was not going to make it easy for us, but we felt good about the plan. We felt confident that it would work.”
The plan takes hold in transplant
“It was April 11,” Andrew Adams said. “We were sleeping and got the call from the nurse at 2 a.m. who told us they had a heart for us. It was so bittersweet because I knew that someone else’s child had to die for my son to live, but we were so grateful.”
Chen and Dr. Michael McMullen did the transplant. The first step was to completely drain Aaden’s blood and replace it with new blood to help prevent his antibodies from attacking the new heart. The next step was to determine how much of the Argatroban would be needed during the surgery, where excessive bleeding was even more of a risk and the drug’s interactions with anesthesia and other medications were unknown.
Anesthesiologist Greg Latham remembers “feeling nervous.”
“I was completely unfamiliar with this medication,” he said. “We just didn’t know how it was going to work. There just isn’t enough experience out there with Argatroban in pediatrics, but we were determined to figure it out to the best of our ability for Aaden.”
Success and a fond farewell
To everyone’s relief, the surgery went well, and Dr. Chen talked to Andrew and Cheree Adams immediately afterward.
“He said everything was looking good,” Andrew Adams said. “It was finally one good step forward. Now we just needed Aaden to wake up.”
Aaden said he remembers his doctors and nurses as well as his parents talking to him. They wanted him to talk back. He was afraid his voice would sound funny because he hadn’t talked in a long time, so he stayed quiet. Then he decided he wanted Popsicles.
Aaden’s recovery from that point on “was simply remarkable,” said Albers. “He’s an amazing little boy. Quite inspiring.”
Kirk remembers the day Aaden rode a tricycle through the CICU, just before he was discharged.
“I couldn’t stop the tears,” she said. “We all cared about this little boy so much. To see him so, so sick and then to see him going home…it was the best outcome we could have ever hoped for. I will never forget him.”