PORTLAND – Five-year-old Julianna Snow has never been healthy enough to attend Sunday school at the City Bible Church in Portland, Oregon, where her family belongs, so most of what she knows about heaven, she knows from her parents.
They tell her that heaven is where she’ll be able to run and play and eat, none of which she can do now. Heaven is where she’ll meet her great-grandmother, who shared Julianna’s love of shiny, sparkly, mismatched clothes.
God will be in heaven, too, they tell her, and he will love her even more than they do.
But Michelle Moon and Steve Snow explain that they won’t be in heaven when Julianna arrives there, and neither will her big brother, Alex. She’ll go to heaven before them because she has a severe case of an incurable neurodegenerative illness called Charcot-Marie-Tooth disease.
Her coughing and breathing muscles are so weak that the next time she catches even a cold, the infection could settle in her lungs, where it could cause a deadly pneumonia. Her doctors believe that if they can save her under those circumstances — and that’s a big if — she will likely end up sedated on a respirator with very little quality of life.
There’s no debate about the medical facts of Julianna’s condition. But there is debate about something her parents have chosen to do: They asked their daughter, at the age of 4, if she would want to go to the hospital the next time she becomes dangerously ill, or would she want to stay home, where she would die.
Julianna has said she doesn’t want to go to the hospital. She wants to go to “heaven.”
Before having these discussions with her daughter, Michelle looked online for guidance about end-of-life talks with a 4-year-old. Finding nearly nothing, she started a blog of her own in hopes of helping other families in the same situation. Later, she contributed to The Mighty, a website where people write about grappling with disabilities and devastating diseases.
Michelle: Julianna, if you get sick again, do you want to go to the hospital again or stay home?
Julianna: Not the hospital
Michelle: Even if that means that you will go to heaven if you stay home?
Julianna: Yes… I hate NT (naso-tracheal suction, where a tube was placed down her nose into her lungs without sedation). I hate the hospital.
Michelle: Right. So if you get sick again, you want to stay home. But you know that probably means you will go to heaven, right?
Michelle: And it probably means that you will go to heaven by yourself, and Mommy will join you later.
Julianna: But I won’t be alone.
Michelle: That’s right. You will not be alone.
Julianna: Do some people go to heaven soon?
Michelle: Yes. We just don’t know when we go to heaven. Sometimes babies go to heaven. Sometimes really old people go to heaven.
Julianna: Will Alex (her 6-year-old brother) go to heaven with me?
Michelle: Probably not. Sometimes people go to heaven together at the same time, but most of the time, they go alone. Does that scare you?
Julianna: No, heaven is good. But I don’t like dying.
Michelle: I know. That’s the hard part. We don’t have to be afraid of dying because we believe we go to heaven. But it’s sad because I will miss you so much.
Julianna: Don’t worry, I won’t be alone.
Michelle: I know. I love you.
Michelle: Yes, I love you madly. I’m so lucky.
Julianna: And I’m so lucky.
Julianna: Because you love me madly.
Before these conversations, Michelle says she and Steve had planned to take Julianna to the hospital if she were to get another infection. But after hearing Julianna’s wishes, they changed their minds.
“She made it clear that she doesn’t want to go through the hospital again,” Michelle wrote CNN in an email. “So we had to let go of that plan because it was selfish.”
While most readers left supportive comments on her blog post, some thought Michelle and Steve had made the wrong choice.
“Unbelievable that any parent would think a 4-year-old is able to understand or make a decision on life,” commented one mother of a child with a chronic illness. “Clearly that mother asks her leading questions. This article sickens me.”
The next week Michelle posted another blog post, further explaining their decision.
“She’s scared of dying, but has, to me, demonstrated adequate knowledge of what death is. (J: ‘When you die, you don’t do anything. You don’t think.’),” Michelle wrote. “She hasn’t changed her mind about going back to the hospital, and she knows that this means she’ll go to heaven by herself. If she gets sick, we’ll ask her again, and we’ll honor her wishes.”
She continued: “Very clearly, my 4-year-old daughter was telling me that getting more time at home with her family was not worth the pain of going to the hospital again. I made sure she understood that going to heaven meant dying and leaving this Earth. And I told her that it also meant leaving her family for a while, but we would join her later. Did she still want to skip the hospital and go to heaven? She did.”
Should a child decide her own end-of-life care?
For adults, end-of-life decision-making is relatively straightforward: Basically, we get to decide how much medical treatment we want and don’t want.
But how much say should a child get? And at what age?
Without realizing it, Michelle and Steve had stepped into a heated debate.
Bioethicist Art Caplan has read Michelle’s blogs, and he thinks she’s made the wrong decision.
“This doesn’t sit well with me. It makes me nervous,” he says. “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.”
He says Julianna’s parents shouldn’t put any stock in what she has to say about end-of-life decisions. Maybe she chose heaven over the hospital because she feels how much her parents hate to see her suffer; young children often pick up cues from their parents and want to please them, he says.
Caplan, before he started the bioethics program at New York University a few years ago, worked at the University of Pennsylvania and consulted on end-of-life cases at Children’s Hospital of Philadelphia with Dr. Chris Feudtner, a pediatrician and ethicist there. Caplan respects him a great deal.
Feudtner, it turns out, disagrees with Caplan about Julianna.
“To say her experience is irrelevant doesn’t make any sense,” he says. “She knows more than anyone what it’s like to be not a theoretical girl with a progressive neuromuscular disorder, but to be Julianna.”
At his hospital, he has asked dying children her age what they want to do, and in the appropriate circumstances, he has taken it into consideration.
For example, he doesn’t take their opinion into account when it’s a black and white decision — children with treatable leukemia must get chemotherapy, for example, no matter how hard they protest. But when the choice is gray, when there’s more than one reasonable option, as there is with Julianna, he has put stock in their wishes.
As for a 4-year-old not having the mental capacity to think through death, he’s found that even adults often don’t think through such issues as carefully as one might like.
“My 86-year-old father died in April, and I’m not sure he truly got it,” he says. “He was bed-bound from cancer, and he said, ‘If this is the best I get, get me a Smith & Wesson.’ Did he mean that? I don’t know.”
Feudtner, the chairman of the American Academy of Pediatrics section on hospice and palliative medicine, says as best he can tell from Michelle’s blogs, Julianna’s choice for heaven over the hospital is reasonable and her parents are right to listen to her.
“Palliative care isn’t about giving up. It’s about choosing how you want to live before you die,” he says. “This little girl has chosen how she wants to live.”
Julianna is no ordinary 5 year old
Art Caplan’s words sting Michelle when she hears them. How could he deign to understand her child? But then a few days later, she wrote to CNN to say that she understands how someone could have a difficult time understanding what she and Steve have decided.
If you haven’t met Julianna, she says, it’s hard to explain how even at such a young age, she understands the choice that’s in front of her, how she’s the one who experiences the suffering and should get a say in what will happen to her.
The doctors and nurses who know Julianna best agree.
Dr. Danny Hsia, her pulmonologist, observes that Julianna is wiser than most 5-year-olds. “In that case, it makes a lot of sense to listen to her. I have the utmost faith in her mother and father. They’re phenomenal parents and have her best wishes at heart,” he says.
He believes that when Julianna gets another infection, the hospital will likely not be able to save her. “For her, there is no light at the end of the tunnel,” he says, his voice trailing off. “She doesn’t have a long time to live.”
Diana Scolaro, the nurse who took care of Julianna through three stays in the intensive care unit, also supports Steve and Michelle’s decision to listen to their daughter. “You have to know what it’s like to hold down a child and hear them scream so you can stick a tube down their nose. It’s one thing to do that when you know you’ll have a success at the end, but for Julianna, there is no success,” she says. “We pulled her from death’s door so many times last year, but she’s sicker now than she was then, and I don’t think we could pull her through another big crisis.”
Diana cries as she tries to express her wishes for Julianna. “I want her living and dying in her princess room, at home, surrounded by her family, not in the cold technology of a hospital,” she says. “There is no cure for her. Every day is a blessing. Every day is a gift.”
The ‘slow-motion horror story’
By longstanding tradition, when an Air Force pilot takes his final flight, his colleagues douse him with champagne in celebration as he emerges from the cockpit. Steve had decided to leave the Air Force and his “fini flight,” as it’s called, was set for Davis-Monthan Air Force Base in Tucson, Arizona, where the Snow family had recently moved from Texas. It was January 30, 2013, about three months after their visit in Iowa with Shy.
Steve didn’t want to stop flying, but being an Air Force pilot means working long hours and transferring frequently from base to base. Steve’s family needed him and it would be much better if they could live in one place, near extended family who could help. Julianna was getting sicker and sicker and required more attention. Alex, a 4-year-old bundle of energy, needed attention, too.
Later, Michelle would write in a blog that Steve “sacrificed a career he loved to become Julianna’s main caregiver. …(He) is simply the most decent human being I know. … He is my rock star.”
As the last bottle of champagne rained down, Steve became sad, thinking about how he would never again fly the A-10 jet, his favorite aircraft.
He looked out into the crowd for a supportive smile from Michelle, but he couldn’t find her.
He did see Alex and his babysitter, who explained to Steve that Julianna was in the hospital. Steve ran inside to change out of his wet uniform and drove straight there.
Inside, Julianna was having trouble breathing. What had started as a cold had turned into something more sinister. The doctors assured Steve and Michelle that after a round of antibiotics and some supplemental oxygen through a tube in her nose, she should be back home soon.
But that’s not the way it turned out. In Michelle’s words, a “slow-motion horror story” unfolded as Julianna spent 11 days in the hospital struggling to breathe, most of it in the intensive care unit. She needed more than just a tube of oxygen in her nose; she needed a pressurized mask pumping air into her.
Up until that point, the Charcot-Marie-Tooth disease had weakened only Julianna’s arms and legs. Now it was attacking the nerves that controlled her breathing muscles.
The Snow family will always remember Steve’s fini flight as the beginning of a new and horrible chapter in their lives.
The worst was yet to come.
The start of tough choices
Dr. Sarah Green was nervous to meet with Steve and Michelle. Just 33 years old and only four years out of her training, she was relatively new to the delicate task of talking to parents of dying children. For difficult discussions like this one, she and her colleagues usually worked in pairs, but on that October day in 2014, there had been a scheduling problem and she was on her own.
Julianna was now 4 and in her third stay in 10 months at Doernbecher Children’s Hospital in Portland, Oregon, where Steve and Michelle had moved to be close to Steve’s parents.
Up until this point, the family had been action-oriented, getting Julianna fitted for a back brace to fix the scoliosis that had developed as a result of her weak muscles, arranging for braces on her ankles and feet in hopes that she might walk one day, making appointments for physical and occupational therapy.
But none of this was helping her get better. In fact, she was getting worse. At 2, she could sit up unsupported and walk with a walker, but now, she couldn’t do either. She once had nearly full use of her arms, but now couldn’t even hold a small toy without help. At one point she ate food, but now her chewing and swallowing muscles were so weak, she was fed through a tube in her stomach.
Most significantly, it was getting harder and harder for her to breathe and cough. Repeatedly, mucus was settling in her lungs and causing pneumonia.
The doctors had been able to pull Julianna out of it each time, but by definition, because CMT is a progressive disease, her breathing muscles would only get weaker and weaker. If she got another infection, her doctors weren’t sure they’d be able to help.
Green proceeded with the conversation slowly and carefully. She asked Steve and Michelle: If Julianna were to get another infection, would they want to bring her back to the hospital? There was a reasonable chance Julianna would die there after being subjected to painful procedures. Even if she lived, it would likely be for a short time, and she’d likely be sedated, unable to think and talk as she could now.
The other option would be for her to stay home and forgo treatment, in which case Julianna would most certainly die. But she would be dying at home and without painful medical interventions.
There was no right or wrong answer, Green told them. The choice was up to them.
Enduring difficult treatment
For several months after Julianna was discharged from the hospital, her dolls kept needing to be admitted. Julianna assured them there would be no shots or IVs there, just Band-Aids and new toys.
It was the wishful thinking of a 4-year-old, and even she knew it wasn’t real. Over and over, she told her parents how much she hated the hospital, especially “NT,” or naso-tracheal suctioning.
Several times a day in the hospital, a nurse would put a tube down her nose and throat, forcing it past her gag reflex and into her lungs to suck the mucus out of the tiny pockets in her airways. It was too dangerous to sedate her for the procedure, so Julianna had to feel everything.
The task of doing NT suction often fell to nurse Diana Scolaro, who took care of Julianna during all three of her intensive care unit stays at Doernbecher in 2014. Stronger children usually scream and have to be restrained when someone tries to put the tube down their nose. But Julianna was so weak all she could do was cry.
When she was done, Scolaro would say to Julianna, “Rest now, baby. Maybe you can make it two hours before we have to do it again.”
But she seldom lasted that long. “It’s not for the faint of heart to take what she took,” Scolaro says.
When Julianna told her parents how much she hated NT suctioning, her mother tried to make her understand why they’d done it. “I told her that even though it was really hard in the hospital, it let her get better and come back home to us, so wasn’t it worth it?” Michelle remembers.
Julianna would never answer. That’s when Michelle decided to have a conversation about heaven.
Michelle asked Julianna, then 4 years old, if she were to get very sick again, did she want to go back for more treatments, or did she want to die at home?
Julianna’s answer was loud and clear. She chose heaven over the hospital.
Planning for heaven, enjoying life
For now, Julianna spends nearly all her time in that princess room. In her closet hang dresses for pretty much every Disney princess there is, and each morning she picks out which one she wants to be. Some days she’s Rapunzel, other days she’s Snow White or Belle or Cinderella or Sleeping Beauty.
She’s happy, watching videos and chatting nonstop with a steady stream of family, friends and nurses. She makes up elaborate fantasies, that her bed is a magic carpet, that she and her grandmother go on a shoe shopping trip together; Julianna buys one high-heeled red pair and one “low-heeled” blue pair. Then, they go to a restaurant and she orders macaroni and cheese.
Julianna displays no bitterness that she can’t actually eat real food or go on a shopping trip. Instead, Julianna smiles as she watches her brother race up and down the hallway, showing no envy. Her father wheels her into the dining room to eat with the family, and takes a piece of cheese off the pizza and rubs it on Julianna’s lips so she can have a taste, and then does the same with a piece of pineapple topping. She never once says how nice it would be if she could eat the pizza like everyone else at the table.
Her parents watch as her disease gets worse in just a matter of months. Last year, on the Fourth of July, Julianna impressed the neighborhood kids by turning circles in the driveway in her wheelchair, working the hand controls by herself. About six months later, her hands were too weak to work the controls at all. In those same six months, she went from needing the breathing mask only while sleeping to needing it nearly all the time.
Michelle and Steve try to prepare themselves and Alex for Julianna’s death. Counseling for all of them has helped. Alex is just 6 and doesn’t say much, but then he’ll surprise his parents by blurting out something insightful.
Once, he asked Michelle if her heart would stop beating when Julianna dies because she will be so sad.
Some days Michelle feels like it will. Other times she doesn’t focus on Julianna’s death, and just feels grateful to be with her. In a post this summer on The Mighty, she described her daughter’s mind and spirit as “bright, sharp and lovely.”
Julianna is planning for her future in heaven, and for her parents’ eventual arrival there.
“Do you want me to stand in front of the house, and in front of all the people so you can see me first?” she asks.
“Yes,” her mother answers.
“And will you run to me?” she asks.
“Yes, I will run to you,” Michelle answers. “And I think you’ll run to me, too.”
“I’ll run fast,” Julianna responds, shaking her head back and forth to show how fast she will run.
It has been a year since Julianna last had an infection, and these months have been “a dream,” Michelle says. But she knows it won’t last forever. Any little cold could ruin the lovely dream. Her medical training and her faith tell her that she and Steve and Julianna will know when the end is near. And they will make a decision about what to do. Together.