AUDIE CORNISH, HOST:
Decisions about end-of-life care are always difficult. They can be particularly wrenching for the parents of very sick infants. A chasm can open between parents' hopes and what doctors see as realistic. Jenny Gold has this story of one family's struggle to decide what's right for their newborn.
JENNY GOLD, BYLINE: Anne and Omar Shamiyeh are a truly San Francisco couple. They're hip, easy-going and diehard Giants fans. He works for an airline and she's a teacher. They already had a 2-year-old daughter when Anne got pregnant in 2012.
A. SHAMIYEH: I'd called Omar at work and (laughter) said, are you sitting down? And he's like, oh, I need to call you back, I can't breathe.
GOLD: They were thrilled. It was twins. But at the 18-week ultrasound, they learned something was wrong with one of the twins.
A. SHAMIYEH: The technician was like, well, there's no visualization of his stomach. And so I'm like, how does our baby have no stomach?
GOLD: It turned out his esophagus was not connected to his stomach. He also had a heart defect. It meant he faced a long stay in the neonatal intensive care unit, or NICU, and possibly long-term disabilities.
A. SHAMIYEH: Their next offering was to selectively reduce.
GOLD: That's the clinical term for ending one twin's life and allowing the other to live.
A. SHAMIYEH: And Omar and I were very uncomfortable with that. So we really wanted to see what he was going to be like and what life had to offer.
GOLD: That decision meant both babies would likely be born premature. At 30 weeks, about two months early, Anne Shamiyeh gave birth to twins Kai and Malia through a C-section at the UCSF Benioff Children's Hospital. Each weighed about three pounds. They were rushed immediately to the NICU.
A. SHAMIYEH: He had his first surgery that night.
GOLD: The girl, Malia, went home after about five weeks. But Kai had a long road ahead. Eventually, he was diagnosed with CHARGE syndrome, a rare genetic condition with severe cognitive and physical disabilities. Here's UCSF neonatologist Liz Rogers, who took care of Kai.
LIZ ROGERS: I think we all hoped he would survive. Unfortunately, the hardest part of my job is having to explain to a family that a bad prognosis is certainly within the realm of possibility.
GOLD: After about five weeks, the doctors and nurses sat down with the Shamiyehs to discuss a major step - whether to do a tracheostomy in which in which surgeons would insert a breathing tube directly into Kai's neck. Here's Anne Shamiyeh.
A. SHAMIYEH: It seemed awful. We were both really unhappy with that, but we understood it wasn't a choice - it was something we had to do.
GOLD: But Dr. Roger saw it as a big decision.
ROGERS: To be very honest, for many, many of our families the point of decision around a tracheostomy is a major, major time when families say, this has going on for too long and it's not what I want for him.
GOLD: But the parents had real hope for his future, even if it wasn't what they'd first imagined, even though some of the doctors had a much more pessimistic view.
A. SHAMIYEH: And I kept thinking, well, maybe that doctor's view of a quality of life is different than mine and maybe - you know, for me, loving my child and having him feel love is enough. And that's OK, and it's OK if he can't talk. Maybe he's going to wear a diaper until he's 5, and maybe he's going to be in a wheelchair. But that's OK with me because he'll be alive and he'll be my child.
GOLD: Studies suggest that providers do tend to have a different view of quality-of-life than parents. In Kai's case this caused much consternation among his day-to-day caregivers, the nurses. Deidre Miller says it was clear to all of them Kai wasn't going to make it. She felt comfortable caring for him, but she faced pressure from other nurses who felt he was suffering unnecessarily.
DEIDRE MILLER: A lot of people thought, well, let's just offer the Shamiyehs the opportunity to withdraw care today. And, as a primary nurse, you knew that the Shamiyehs weren't ever going to agree to that and you knew that he had joy in his life. But you go into the break room and everybody wants to talk about it, and everybody wants you to be the person to tell the Shamiyehs.
GOLD: Researchers have found there can be a lag of months when health care providers and parents sense a child isn't going to make it.
MILLER: As easy as it is to say we knew Kai was going to die and that we knew he was going to have a difficult life, gosh, what if we had been wrong?
GOLD: The Shamiyehs say Kai lit up when they walked in the room. They visited every day. He smiled, cooed and connected with them. He especially loved the music from the mobile on the end of his bed. Anne still keeps it in a closet downstairs.
A. SHAMIYEH: Island and nature were his two favorite ones.
GOLD: But he wasn't getting better. In May of 2013, five months into Kai's stay in the NICU, the Shamiyehs and their doctors sat down to talk about whether or not to do the heart surgery he needed. The doctors said it wouldn't help and it might kill him.
A. SHAMIYEH: So that was the day that we found out that we wouldn't ever be bringing Kai home.
GOLD: They decided not to go forward. They didn't know how long they'd have so they made a list of all the things they wanted to do with him.
A. SHAMIYEH: So the first thing we did was give him a bath because he had never been immersed in water, and he totally loved that.
GOLD: The next thing was to take Kai outside. He'd never breathed fresh air or felt the sun on his skin. So one very hot day in San Francisco, the entire Shamiyeh family brought Kai out to the hospital courtyard. Anne and Omar Shamiyeh say it was an enormous undertaking including a portable ventilator and a support staff of six.
A. SHAMIYEH: We were laughing, Zara was running around, Malia was crying. Kai was, like, squirming 'cause he was too hot. It was the most normal day that our family had felt together.
O. SHAMIYEH: That was huge. I always wanted him to feel like a little boy, you know?
GOLD: Two weeks later, Kai developed an infection that they couldn't treat. He passed away in his mother's arms. Looking back, Omar Shamiyeh says he wonders if they went too far.
O. SHAMIYEH: It's really hard to think for five months he was going through all this pain and all this stress. You wonder if you made the right decision in keeping it going, you know?
GOLD: There were other costs to Kai's six-month stay in the NICU. The Shamiyehs calculate that the hospital bills added up to $11 million, though their private insurer probably negotiated a lower rate. There were also consequences for Kai's twin sister, Malia. Her parents' attention in her first six months was really focused on her brother. She had speech and physical delays. But at 3, she's already caught up. Still, Anne says Kai's life was a blessing.
A. SHAMIYEH: This is life-changing for us, you know? Everything about it - the way that we parent out kids, the way that we live our lives - it forever changed us for the better.
GOLD: Anne Shamiyeh is now studying to become a nurse in the NICU herself. In the end, she's glad they gave their son the best possible chance at life. She's at peace with their decisions - to try to save him and to let him go. For NPR News, I'm Jenny Gold.
CORNISH: Jenny Gold is a reporter with our partner, Kaiser Health News. She reported this story as a California health journalism fellow at USC Annenberg.
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