Washougal girl, 5, who chose to forgo treatment dies

Most 4-year-olds cannot grasp the concept of death. Most don’t have to, but Julianna Snow was forced to consider and reckon with her own mortality at that young age.

Julianna, of Washougal, was diagnosed with Charcot-Marie-Tooth Disease, which is one of the world’s most common inherited neurological disorders, according to the NIH. It’s a progressive disease that damages the nerves affecting certain muscles. In most cases, it’s relegated to the arm and leg muscles, sometimes so mildly that doctors don’t even find it upon examination. But there are many mutations of CMT, and symptoms range in severity.

Julianna’s case was one of the most severe.

The disease affected the muscles she needed for chewing and swallowing, then those needed for breathing. Her lungs would fill with mucus and cause pneumonia. When this happened, doctors would need to perform nasotracheal suctioning, an exceedingly painful procedure in which a tube is threaded through the nose and down the throat, past the gag reflex and into the lungs to remove dangerous mucus build-up.

Julianna despised it.

Most children scream and need to be restrained during the procedure, but Julianna was eventually too weak to do anything but cry, her nurse Diana Scolaro — who often performed the procedure — told CNN. And while most children can be sedated for the process, Julianna was too weak.

She remained conscious for each one, feeling every second of pain.

After each session, Scolaro would tell Julianna, “Rest now, baby. Maybe you can make it two hours before we have to do it again.”

Scolaro told CNN, “It’s not for the faint of heart to take what she took.”

Juliana’s condition continued to worsen. She eventually required a pressurized mask to pump oxygen into her lungs, which were too weak to do it themselves. She was fed through a tube inserted into her stomach. Her tiny body had grown too frail to fight off another bout of pneumonia, or even the common cold.

In October 2014, Dr. Sarah Green sat Julianna’s parents, neurologist Dr. Michelle Moon and former Air Force pilot Steve Snow, down to have a difficult conversation. The next time Julianna got sick, she would likely die. If doctors managed to save her, her quality of life would almost certainly be poor.

It was a decision Moon and Snow could have made on their own, and they had — they were going to bring Julianna to the hospital if she got sick again.

But then they decided to ask Julianna.

• • •

As Moon explained on Anderson Cooper’s “AC360, “Julianna had to go through hundreds of rounds of nasotracheal suctioning. She knows exactly what that was. She was awake for every single one. I think she has a right. I think she has a say.”

So Moon sat her 4-year-old daughter down to have a series of the most heart-wrenching conversations a parent could imagine.

“Julianna, if you get sick again, do you want to go to the hospital again or stay home?” she asked her daughter in a conversation she blogged about on her personal blog and for The Mighty.

“Not the hospital,” the girl said.

In another conversation, Julianna asked if her mother wanted her to get treatment. Moon asked what Julianna wanted.

“I hate NT. I hate the hospital,” she said.

“Right. So if you get sick again, you want to stay home,” her mom said. “But you know that probably means you will go to heaven, right?”

“Yes.”

“And it probably means that you will go to heaven by yourself, and Mommy will join you later.”

“But I won’t be alone,” Julianna replied.

“That’s right. You will not be alone.”

“Do some people go to heaven soon?” the 4-year-old asked.

“Yes. We just don’t know when we go to heaven,” her mom said. “Sometimes babies go to heaven. Sometimes really old people go to heaven.”

“Will Alex go to heaven with me?” Julianna asked, referring to her 6-year-old brother.

“Probably not. Sometimes people go to heaven together at the same time, but most of the time, they go alone,” Moon told her. “Does that scare you?”

“No, heaven is good,” her daughter said. “But I don’t like dying.”

The two had many more conversations in this vein, Moon trying to ensure that, as much as possible, Julianna understood what dying means. Not only can most 4-year-olds not grasp the concept of death, most people can’t.

Still, a decision had to be made, and Julianna helped make one.

“I want to make it clear these are not Julianna’s decisions or choices,” Moon told People. “They are Steve’s and my decisions, but we look to Julianna to guide us.”

Her parents respected her wishes.

“She made it clear that she doesn’t want to go through the hospital again,” Michelle told CNN. “So we had to let go of that plan because it was selfish.”

• • •

On Tuesday, Julianna died at 5 years old.

“Our sweet Julianna went to heaven today,” Moon wrote on the blog dedicated to her daughter. “I am stunned and heartbroken, but also thankful. I feel like the luckiest mom in the world, for God somehow entrusted me with this glorious child, and we got almost six years together.”

Since Moon first posted on The Mighty, a blog about people with disabilities, and began her own blog, Julianna’s story has received national attention. CNN wrote a two-part series on the family, which was also featured on “AC360.” People magazine profiled the family. Even the Korean Broadcasting System traveled to Washington to film Julianna for a Christmas Day documentary.

As her story grew, so too did backlash in response to their decision.

“Your daughter sounds super smart but, please, don’t let her be anything like the decision-maker on this. I’m so grateful my parents heard my articulate little 4 year-old thoughts, factored them in, and didn’t confuse my strong verbal skills with an older emotional age,” wrote one commenter on The Mighty.

“The fact that she used leading questions does not sit well with me. As an attorney, I cannot even use those types of questions when cross examining. So, to present the questions in such a format induces the child in subtle prompts to answer in a particular way . . . furthermore, a four year old lacks the full capacity to decide their own morality.[sic]” wrote another.

“I am so sorry for the lack of support you and your husband get from people who do not know you or young Julianna,” one wrote in response.

Even bioethicists weighed in. New York University bioethics professor Arthur Caplan told CNN, “This doesn’t sit well with me. It makes me nervous. I think a 4-year-old might be capable of deciding what music to hear or what picture book they might want to read. But I think there’s zero chance a 4-year-old can understand the concept of death. That kind of thinking doesn’t really develop until around age 9 or 10.”

Others disagree.

“To say her experience is irrelevant doesn’t make any sense,” Dr. Chris Feudtner, Director of the Department of Medical Ethics at The Children’s Hospital of Philadelphia said. “She knows more than anyone what it’s like to be not a theoretical girl with a progressive neuromuscular disorder, but to be Julianna.”

Karla Langlois, a hospice nurse who worked with Julianna, agreed.

“I think she’s very capable of having input into the end of her life,” Langlois told People. “I don’t know that it’s appropriate for every child, but in this scenario it’s very appropriate.”

Moon wrote a second blog post explaining her decision: I realize that the idea of not doing “everything” to prolong the life of a young child is perhaps unacceptable to some. But when your child’s illness is terminal, when the disease takes more and more and the treatments become riskier and offer less return, there are choices. The choices seem impossible, and they’re painful. We believe that sometimes it’s an act of love not to do “everything” to extend life and focus instead on giving your child the most beautiful life possible for as long as you’re allowed.