Anne and Omar Shamiyeh first learned something was wrong with one of their twins during an ultrasound, when Anne was 18 weeks pregnant.
"The technician was, like, 'Well, there's no visualization of his stomach,' " says Anne. "And I was like, 'How does our baby have no stomach?' "
It turned out that the baby's esophagus was not connected to his stomach. He also had a heart defect. At the very least, he was likely to face surgeries and a long stay in intensive care. He might have lifelong disabilities.
This was only the start of an eight-month ordeal for the Shamiyeh family.
Decisions about how much care to offer very sick family members are always challenging. But they can be particularly wrenching for parents like the Shamiyehs, who face harrowing choices during what is supposed to be a wonderful time — the start of a life.
As doctors and families consider how far to push medical care, a chasm can open between the parents' hopes and what providers consider realistic.
For the Shamiyehs, the first major decision was whether to "selectively reduce," the clinical term for aborting one fetus in a multiple pregnancy. "Omar and I were very uncomfortable with that," Anne says. "We really wanted to see what he was going to be like, and what life had to offer."
That decision meant the twins, a boy and a girl, would likely be born prematurely. As it turned out, they were delivered by cesarean section at 30 weeks — about two months early — at UCSF Benioff Children's Hospital, in San Francisco.
The boy was named Kai, the girl Malia. Each weighed about 3 pounds. They were rushed immediately to the neonatal intensive care unit. That night Kai had his first surgery.
Malia went home after about five weeks. But Kai was far from ready. He was on a ventilator, had to be fed through a tube directly into his stomach and was still struggling to survive. Eventually, he was diagnosed with CHARGE syndrome — a rare genetic condition that can result in severe cognitive and physical disabilities.
About the time Malia went home, the doctors and nurses sat down with the Shamiyehs to discuss Kai's treatment. They needed to know whether the family wanted a tracheostomy — surgeons would insert a breathing tube directly into Kai's neck to ease passage of air into his lungs.
"It seemed awful," Anne recalls. "We were both really unhappy with that, but we understood it wasn't a choice. It was something we had to do."
But Dr. Liz Rogers, a UCSF neonatologist who cared for Kai, saw it as a significant decision.
"To be very honest," Rogers says, "for many, many of our families, the point of decision around a tracheostomy is a major, major time when families say, 'This has gone on for too long, and it's not what I want for him.' "
Anne had real hope for Kai's future, despite the pessimism of some doctors.
"I kept thinking, maybe that doctor's view of quality of life is different from mine. And maybe, for me, loving my child and having him feel love is enough," Anne says. "And it's OK if he can't talk. Maybe he'll wear a diaper until he's 5, and maybe he'll be in a wheelchair, but that's OK. Because he'll be alive, and he'll be my child."
Studies suggest that health care providers do tend to have a different view of quality of life than parents do. In Kai's case, many of his day-to-day caregivers — the nurses — felt Kai was suffering unnecessarily.
Deidre Miller, a registered nurse in the NICU, says she was one of just a handful of nurses willing to be part of his primary care team. It was clear to all of them, she says, that Kai wasn't going to make it. Miller says she felt comfortable caring for Kai, but faced pressure from other nurses.
"A lot of people thought, 'OK, well, let's just offer the Shamiyehs the opportunity to withdraw care today.' And, as a primary nurse, you knew that the Shamiyehs were never going to agree to that, and you knew that he had joy in his life," she recalls. "But you go into the break room, and everybody wants to talk about it, and everyone wants you to be the person to tell the Shamiyehs."
There's often a lag between when health care providers and parents sense a child isn't going to make it. In one study, for instance, oncologists realized that their young patients would not recover months before the parents recognized it.
"As easy as it is to say we knew Kai was going to die, and we knew he was going to have a difficult life — gosh, what if we had been wrong?" Miller says.
From Anne's and Omar's perspective, Kai had many happy moments. They visited every day, always with Malia in tow. He smiled, cooed and connected with them. But he wasn't getting better.
In May 2013, five months into Kai's stay in the NICU, the Shamiyehs and their doctors sat down to talk about whether they wanted to go forward with the heart surgery that had been on the calendar since his birth. It would have to be done if Kai was ever to leave the hospital.
The surgery wouldn't help, doctors explained, and Kai might die during the procedure. This time, Anne and Omar decided not to go forward.
"So that was the day we found out we wouldn't ever be bringing Kai home," Anne says.
Two weeks later, Kai developed an infection the doctors couldn't treat. On June 5, 2013, he died in his mother's arms.
There were real costs to Kai's long stay in the NICU. Based on billing statements, the Shamiyehs calculate that the charges for Kai's care added up to more than $11 million, though their insurer very likely negotiated a lower rate.
There were also consequences for Kai's twin sister, Malia, whose parents were mostly focused on her brother during her first six months of life. Born two months prematurely herself, she had physical and speech delays; although at age 3, she's already caught up.
Looking back, Omar says he wonders if they went too far. "It's really hard to think — for five months he was going through all this pain and all this stress," Omar says. "You wonder if you made the right decision in keeping it going, you know?"
But Anne, who is now studying to become a nurse in the NICU, says she does not regret giving their son the best possible chance at life.
She's at peace with both their decisions — to try to save Kai, and to let him go.