Once in a Lifetime

Ka’apeha was born in a Honolulu, Hawaii, in October 1997. His parents, Averi and Ross, gave him a name like an epic poem—Ka’apeha Aaron Keli’ika’apunimoku Kanae Kaleimamahu—without knowing that his earliest years would hold tales of struggle and survival that would be epic indeed.
 
At just 6 weeks of age, Ka’apeha was diagnosed by his doctors in Hawaii with biliary atresia, a condition in which the ducts that carry bile from the liver to the intestine are closed or not present. Three weeks later, during surgery to correct the problem, his doctors discovered that his bile ducts were present, but were flowing in the wrong direction. As a result, Ka’apeha’s liver was in bad shape. He would need a liver transplant, but first needed to grow and get stronger in preparation.
 
Over the next few months, nourished by special formula, Ka’apeha grew slowly but his health gradually worsened. In June 1998, his doctors referred him to Lucile Packard Children’s Hospital.
 
Ka’apeha was admitted to the pediatric intensive care unit (PICU) at Packard Children’s, where he received several blood transfusions. Then, at just 9 months old, his liver failed. There was no time left to grow—little Ka’apeha needed a transplant immediately.
 
Ross was an eligible organ donor for his son, but even a small portion of his liver was too big for Ka’apeha. Instead, Dr. Samuel So and his team transplanted a smaller donor liver and supported their tiny, fragile patient as he struggled to survive.
 
Ross and Averi were relieved to have their son out of surgery, but the battle had really just begun. Almost immediately, Ka’apeha’s body began to reject his new liver. “Dr. So was cautious and careful,” recalls Averi. “I felt like he cared about us personally. He had a lot of concern and took the time to really listen to us. He took care of Ka’apeha as if he were his own child.”
 
Dr. So was aware that in some rare cases, a very sick liver is worse for a patient than having no liver at all. He explained that one of his patients had survived without a liver for 66 hours—the longest known survival of its type. Such unconventional care involved support from a continuous dialysis machine (a tool for filtering and cleaning the blood outside the body) until a new liver could be obtained for transplant.
 
Although this new method of “liver assistance,” known as CVVH (continuous venovenous hemofiltration), had been used in only a handful of cases to sustain patients with no liver, Dr. So knew that it was Ka’apeha’s best chance. The care team and the family discussed the options and the risks, and decided together to proceed.
 
Following surgery to remove his liver, Ka’apeha was placed on CVVH and listed as “status 1A,” the highest medical urgency, on the national organ transplant waiting list. As the hours passed, careful management was necessary at all times. At the same time, Dr. So phoned colleagues around the country trying to secure a donor liver for his patient.
 
The family prayed as Ka’apeha’s dad Ross played guitar at his son’s bedside in the PICU. The soft music helped keep Ka’apeha’s low blood pressure up. But as the hours turned into days—with no donor liver available—Ka’apeha’s kidneys, too, began to fail. Dr. So reluctantly told the family to prepare for the possibility that they would have to say goodbye to their son. “We didn’t want him to suffer anymore,” said Averi, “so we came to peace with that even though it was the most difficult decision we ever had to make.”
 
“We sang and prayed around his bed all night,” said Averi. “Then, the next morning there was a lot of activity.” The nurse told them Ka’apeha had urinated. Somehow, his kidneys were working again.
 
The CVVH machine, blood pressure medicines and mechanical ventilation were helping Ka’apeha sustain through the effects of his failed liver. As he improved, a donor liver match became available. With the liver en route to Packard Children’s, Ka’apeha’s care team prepared him for another surgery.
 
“I was always worrying,” recalls Ross. “Was this situation going to pull him back? We were on our toes all the time.” But this time, the liver transplant was a success. With constant attention and expert management by his team of caregivers, Ka’apeha had lived for more than 80 hours, longer than any child at the time had ever survived without a liver. “Our faith was really tested,” said Averi, “and really strengthened.”

The Kaleimamahu Family

Nine years later, Ka’apeha is a happy 10 year-old. Innovations in anti-rejection therapy developed at Packard Children’s have helped reduce his ongoing medication to a once-a-day pill, while also ensuring his good health.
 
Like their son, Averi and Ross were irreversibly changed by the experience. “I was inspired by the care that everyone gave to our son at Packard. They gave so much to us; I wanted to give back, and to give that same support to other families with sick children.” Averi switched her studies from elementary education to nursing. With Ross’s strong support, she passed her boards in August 2004. The following February, the Kaleimamahu family moved to the Bay Area, where Averi started work as a nurse at Packard Children’s Hospital.
 
Doctors regularly refer patients like Ka’apeha to Packard Children’s for care they wouldn’t be able to receive at their local hospital, and for the outstanding results they might not be able to attain anywhere else. Regular outreach programs, innovative treatment and family-centered care helped ensure that Ka’apeha’s epic experience had a very happy conclusion.