Heaven over hospital: 5-year-old changes the conversation about death

This is the third part of the series “Heaven over hospital.” Read the second part here.

When Stephanie Hunter had to withdraw life support from her mother, her friends and family understood. They expressed their condolences, said she’d made the right decision, and some even shared stories about the tortured choices they had made for their own dying parents.

But four years later, when she and her husband had to make a similar decision for their terminally ill baby, they didn’t feel like anyone understood. A few family members thought they were giving up all hope for their child.

That’s why Stephanie and her husband, Justin, have remained quiet about the manner in which their son died three years ago. “They made us feel so ashamed,” she said.

But last week that ended. Stephanie said a CNN story about a family’s decision for their terminally ill 5-year-old was an important first step in changing the way people think about end-of-life decisions for terminally ill children. Now, she says, the shame is starting to disappear, the taboo starting to fade.

“I am so thankful for your (family’s) unbelievable bravery and strength in telling and standing by the decision your family has made,” she wrote in response to a blog written by the mother of the 5-year-old girl, Julianna Snow.

‘We felt so alone’

“We (chose) to forego further medical intervention as a means to end our child’s pain and unimaginable suffering,” she added on the Facebook page for a local television state that carried CNN’s story.

When Wyatt Hunter was a week old, blood clots burst in his brain. Doctors told the Hunters that he would be in severe pain the rest of his life – pain that not even around-the-clock morphine would erase. The doctors said he would be in a completely vegetative state and would have no quality of life. There was no chance he would ever be able to eat, see, hear, walk or talk. The only thing he would be able to do is breathe.

The Hunters didn’t want their son to live that way, so they made a decision no parent should ever have to make: They decided to take him off all forms of life support, including a ventilator, nutrition, and life-prolonging medications.

A few relatives didn’t agree with the Hunters’ decision to end life support and judged them for it.

Wyatt died comfortably two weeks later. His doctors supported the couple’s decision.

“We knew it was the right decision. Our doctors told us it was the right decision. But we felt so alone,” Stephanie remembers.

The circumstances of Julianna’s illness are very different, but her parents also felt alone. When organizations such as the National Institutes of Health talk about end-of-life decisions, it’s about adults. While there are a few notable exceptions, such as the Courageous Parents Network and the Conversation Project, not many places talk about end-of-life care for dying children.

Julianna has an incurable neuromuscular disorder, and the next time she gets a cold, her body will be too weak to fight it off. The hospital likely won’t be able to save her – but even if it could, she would likely face a terrible quality of life.

Her parents, Steve Snow and Michelle Moon, asked her if she wanted to go to the hospital the next time she got a cold. She chose heaven, and they say they’ll abide by her wishes to die at home.

Following the story’s publication last week, most people have been supportive of the Snow family, but there have been critics.

“The parent’s should go to prison for death row (for) letting their child kill herself,” wrote one Facebook user.

Stephanie Hunter, who’s been silent all these years, spoke up online to defend the Snow family against these critics.

“How can those who judge be rso hurtful with their words when they admittedly have never walked in the shoes of families like you, like me,” she wrote on Michelle Moon’s blog.

The executive director of the Courageous Parents Network, Blyth Lord, who lost her own daughter in 2001, said she wishes people were less judgmental of the Snow family and others who find themselves having to make horrific decisions no parent should ever have to make.

“It is clear how beautifully they parent and love their daughter amidst the most heartbreaking of stories,” Lord wrote in an email to CNN. “It is not for anyone else to AGREE or DISAGREE with their choice, but only to say that we know they are being the best parents that Julianna could have.”

A search for a mystery gene

Much of the negative response to Julianna’s story has come from people living with neuromuscular diseases. Some of them started a Facebook group called “Dear Julianna” where they tell Julianna that if she keeps fighting, she can live a fruitful life just as they do.

“There’s a ton of us out there who have experienced what she’s going through. We’re all confident with support and medical technology she can get through this,” said Emily Wolinsky, one of the page’s founders, who has muscular dystrophy.

But Dr. Michael Shy, director of the division of neuromuscular diseases at the University of Iowa Carver College of Medicine, said no one has experienced exactly what Julianna is going through. Wolinsky and many of the others don’t even have the same disease Julianna has, he said.

And even if the little girl did have the same disease as the “Dear Julianna” writers, he said, it would be impossible to compare their lives, since there are so many variations even within one disease. For example, there are 83 different genes known to cause the illness Julianna has, called Charcot-Marie-Tooth disease, and each gene causes a different version of the disease.

Doctors don’t even know what bad gene Julianna has. They’ve tried for years to figure it out, but to no avail.

After the CNN story, the Charcot-Marie-Tooth Association donated $6,000 to use new technology to do whole-genome sequencing on Julianna and her parents. Dr. Stephan Zuchner, a professor of genetics and neurology at the University of Miami Miller School of Medicine, said there’s a 30% to 50% chance they’ll be able to find the culprit gene in Julianna’s case. He said they should know in a few weeks.

But even if they are successful, Julianna’s parents know it’s unlikely it will help their daughter. Pinpointing the gene is just the first step. Several CMT genes were found in the early 1990s and there is still no treatment available.

Even with no cure in sight, Julianna is living life to the fullest, dressing up as a princess, singing songs from “Frozen” and enjoying a stream of visitors.

This week, Moon wrote about how she asked her husband six months ago if he thought it was a good idea to go public with their story. He said yes.

“Julianna is a bright light,” Snow told his wife. “Maybe she has something to teach the world.”

This is the third part of the series,”Heaven over hospital.” Read the second part here.