Tuesday, October 27, 2015

Heaven or the hospital

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.”
Credit: CNN
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?
Julianna: (nods)
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.
Julianna: Madly.
Michelle: Yes, I love you madly. I’m so lucky.
Julianna: And I’m so lucky.
Michelle: Why?
Julianna: Because you love me madly.



  1. Julianna Snow is dying of an incurable disease. She's stable at the moment, but any germ that comes her way, even just the common cold virus, could kill her. She's told her parents that the next time this happens, she wants to die at home instead of going to the hospital for treatment.

    If Julianna were an adult, there would be no debate about her case: She would get to decide when to say "enough" to medical care and be allowed to die.

    But Julianna is 5 years old. Should her parents have let her know how grave her situation is? Should they have asked her about her end-of-life wishes? And now that those wishes are known, should her parents heed them?

    When she was 9 months old, Julianna's parents noticed she couldn't sit up steadily, something most babies can do around 6 months. At her first birthday, when children typically start to walk, Julianna couldn't even pull herself up into a standing position...

    With Julianna's symptoms -- her developmental delays and her somewhat floppy arms and legs -- this is what kept popping up: spinal muscular atrophy, a hereditary disease where neurons in the brain and spinal cord are progressively destroyed. Many children with the disease don't live to see their second birthday.

    To get spinal muscular atrophy, a child must inherit a defective gene from both parents. Michelle had herself tested and didn't have the gene. She was relieved, but stumped. By now Julianna was 1½ and couldn't walk without the help of a walker, and then only for short distances. Her doctors were mystified, too.

    Then Michelle remembered her husband's funny-looking feet...

    In spring 2004, after flying fighter jets over Baghdad and Kuwait, Air Force Capt. Steve Snow was assigned to Osan Air Base in South Korea. On his first day, not knowing a soul, he headed to the officers' club in search of company.

    That's when she walked in: Capt. Michelle Moon, the flight surgeon for his squadron...

    The two fell in love and were married in 2006. Two years later, their son Alex was born, and Julianna came along two years after that on August 25, 2010.

    Over the years, Michelle had never given her husband's feet much thought, except to occasionally notice that they were sort of odd looking, with high arches.

    But as Michelle searched for the cause of their daughter's slow development, Steve's funny-looking feet became the key to solving their daughter's mystery

    By some awful quirk of genetics, Steve's mild case of CMT had manifested as a severe case in his daughter...

    In fall 2012, just after Julianna turned 2, Steve and Michelle brought her to the University of Iowa to see Dr. Michael Shy, one of the world's leading experts on CMT. He explained that the myelin sheath covering her nerves had never formed correctly, and so underneath, her nerves were degenerating, as a wire frays if the insulation around it breaks down.

    The severity of CMT depends on the specific genetic mutation that has caused it. Shy tested Julianna and couldn't find anything amiss, so he sent her DNA to a specialized genetics lab at the University of Miami for a wider search. They couldn't find anything definitive, either.

    This didn't mean Julianna didn't have CMT; scientists just haven't discovered her particular variation. Without knowing what genetic mutation was causing her disease, it was hard to give Michelle and Steve a prognosis.

    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. (continued)

  2. (continued) 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." ...

    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...

    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.

    She (Dr. Sarah Green) 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…(continued)

  3. (continued) 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…

    "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.
    Now Michelle and Steve had to decide: Would they abide by her wishes?


  4. 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."


  5. 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."


  6. 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 used to turn circles in her wheelchair. Now her hands are too weak to work the controls.

    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.


  7. "She made it clear that she doesn't want to go through the hospital again," Michelle Moon, Julianna's mother, wrote in an email to CNN. "So we had to let go of that plan because it was selfish."

    The medical community and the public are still debating on the ethical implications of this course of action. While some gave their support to the Snows, others criticized them for thinking that a child is able to understand enough to make life-or-death decisions.

    From a psychiatrist's stand point, however, the argument that young children don't have a clear understanding of death still stands. Children six years old and below tend to think that death is temporary and reversible.

    At this stage, children adopt a "magical thinking" view of death and can be reversed through the use of magic words, thoughts or actions, or that death is merely a punishment for bad behavior that can be lifted. They may also have difficulty understanding the concept of heaven or hell.

    Knowing this, it is unusual for doctors to ask children to make such crucial life and death decisions, especially when it comes to their own.

    "You wouldn't normally ask a child to make crucial decisions because they lack the ability to understand completely the circumstances or the complexity of the situation," said Professor Paul Komesaroff, director of the Centre for Ethics in Medicine and Society at Monash University.

    However, Komesaroff added that he can understand why her parents asked Julianna and believes that it is not a case of the parents wanting to be relieved of the responsibility of caring for a terminally ill child.

    "I see it as a genuine act of concern and love, in which they've introduced the aspect of respect for the little child herself," Komesaroff explained.

    Bridget Haire of the University of NSW said that the decision to continue Julianna's treatment is one that lies with all the members of her family but respects that Julianna should have a say in the matter as well.

    "A person, however young, is going to have some idea of what is or isn't acceptable to them," Haire said, adding that Julianna's decision to stay at home over undergoing further treatment is a decision that shows emphasis on the importance of quality over prolonging life.


  8. Snow family blog: http://juliannayuri.com/

    J: I will love you forever.

    M: I will love you forever.

    J: I will always be your baby.

    M: And I will always be your mom.

    J: Not when we die…

    M: Yes, even when we die.

    J: Mom, will you miss me when I die?

    M: Oh, Julianna. I nod.

    J: I don’t want you to be sad. I want to give you flowers so that you remember me.

    M: Julianna – it could be a long time – you’ve done so well. Do you worry about dying?

    J: Nods. Sometimes. I’m used to things here. I’m not used to dying.

    M: Of course not. No one is. We only die once. Sweetie, I don’t want you to worry about it.

  9. Julianna’s family is painfully aware of what they have been through and what potentially could lie ahead. Her mother, Michelle Moon, is a neurologist. As CNN reports, the child’s hospital visits have been progressively more brutal. “Over and over, she told her parents how much she hated the hospital, especially ‘NT,’ or naso-tracheal suctioning…. 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.”...

    It’s hard to imagine anything more gut-wrenching than having to prepare for the loss of a child, and having to prepare that child for her own death. Death is not something we like to face head on in our culture, even as adults. Last year, when Brittany Maynard shared her choice to end her life with dignity before brain cancer could do its worst, she was met with both admiration and condemnation. The Vatican’s Monsignor Ignacio Carrasco de Paula called her act “an error” and said, “Suicide is not a good thing, it is a bad thing because it is saying no to life.”

    Julianna and her family have no plans for giving death an assist, but they are making a conscious decision to not prolong life, and with a medical industrial complex that has the means to extend a heartbeat and a breath well beyond its comprehension of whether that heartbeat and that breath should be let go, the right to refuse every means necessary is hard for some to grasp. Harder still for the critics of Julianna’s family are the questions of whether a child her age is capable of deciding on her own course of end of life care, particularly when her choices are so clearly informed by such a decisive and certain religious point of view. “This little girl doesn’t even understand what she is agreeing to, this is borderline assisted murder,” wrote one appalled reader on USA Today. Another reader, responding to the family’s original post, said, “That mother asks her leading questions. This article sickens me.”

    My own children are being raised in a Christian household, but one which gives everybody room for asking questions and expressing doubt. I tell my kids that we don’t know where their grandfather or my friend Debbie are now that they’re gone, and that anyone who tells them they do know is not telling the truth. That’s the wager of faith we make here. Others, in their grief, find talk of heaven and the afterlife only insults their own beliefs. But I also get that a young child who has had to face much too much ugly, painful truth already deserves the comfort of belief more than any outside stranger’s skepticism. And when a family doesn’t have the luxury of assuming a long life for one of its members, when it has had so much taken from it and so much yet to lose, who’s to deprive them the hope then of something else, something better, on the other side of it?


  10. Children younger than 6 years old often do not understand death. They tend to think it is somehow temporary and reversible, like sleep. They may engage in magical thinking, like that they will see everyone they love in heaven.

    Palliative care has developed to offer comfort to patients -- whether young or old -- whose diseases we cannot alleviate or cure. And while we might not be able to save them, we can seek to improve their quality of life, relieve their pain, suffering and distress, support them and their families, and help them cope as best as possible.

    Yet many patients and their families -- no matter what their age -- are unsure whether to accept palliative care. They think it may be best to pursue aggressive interventions that may have limited, if any chance of success -- and that may at times cause side effects more painful than those of the illness itself...

    This is the dilemma that Michelle Moon and Steven Snow have confronted since their daughter, Julianna, was diagnosed with Charcot-Marie-Tooth disease. Julianna is 5 years old, and now, she has told her parents, she would prefer to go to heaven than go back to the hospital.

    We physicians try to respect patients' wishes, while doing good and avoiding harm to patients. Here, the best we can do may be to provide comfort, because Julianna's doctors apparently say she cannot be cured. Countless patients greatly appreciate such comfort, and it gives them quality time and dignity at the end of their lives...

    In the meantime, Julianna Snow's family is not alone in having to face a potentially life or death choice. And while their daughter may not fully understand the options, the parents seem determined that their daughter will enjoy the time she has left. That is a courageous decision that may inspire many others as well.


  11. Five-year-old Julianna Snow is at the center of that debate right now. She was born with Charcot-Marie-Tooth disease, a neurodegenerative disorder that gets more and more aggressive until it destroys. Julianna can’t eat. She can’t walk. She can’t move. She wears a mask to breathe and stays connected to machines. There is no cure.

    This isn’t one of those situations where if she fights hard enough or sacrifices enough she can get healthy. In those cases, you push her through the rain so she can stand in the sun again.

    For Julianna, a cold could kill her. She is going to die sooner rather than later; the question is how? The next time she gets an infection, will her family rush her to the hospital, where a surgery would likely allow her to live a bit longer in sedation? Julianna has taken a stand: She wants to stay at home no matter what, alert enough to talk and to love, surrounded by family as she dies.

    When you’ve lived that kind of life, the whole of it in death’s shadow, you are not an everyday 5-year-old. I think you know enough about yourself to have a say in your suffering

    Read more here: http://www.kansascity.com/living/liv-columns-blogs/jenee-osterheldt/article41943531.html#storylink=cpy

  12. 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.


  13. 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, that support disappeared. No one understood. Family and friends said the Hunters were killing their child.

    That’s why Stephanie and her husband, Justin, have remained quiet about the death since it occurred 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.

    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. He would never be able to do anything more than breathe on his own. He wouldn’t be able to blink his eyes, let alone walk or talk.

    The next time Julianna Snow, 5, catches even a cold, the infection will likely leave her sedated on a respirator with very little quality of life. She has a severe case of an incurable neurodegenerative illness called Charcot-Marie-Tooth disease. After debating the issue, her parents decided to honor Julianna’s wish not to return to the hospital but die at home instead.

    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 withhold nutrition from Wyatt.
    His doctors supported that decision, but some close friends and relatives told them they were starving their baby. Stephanie’s grandmother threatened to come to the hospital and feed Wyatt when the parents were out of his room.

    Wyatt died two weeks later.

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


  14. The best experts in the U.S., and indeed the whole world, cannot save Julianna’s life. Thus far, she has been sustained through painful therapies of mechanically sucking mucus out of her lungs by passing a tube down her nose and throat. The doctors’ verdict: buy a little more time by continuing the painful therapy each time she is brought to the hospital or let her die quietly at home. Her chances of survival with the continuation of the therapy in the hospital are very slim and she may not survive a fresh attack. Whichever way, Julianna seems certain to die. The choice of whether to bring her again to hospital or leave her at home to die was left to the parents.

    Next, the question had to be put to Julianna herself then aged four. Her mother asked her what she would prefer if she were to get very sick again: go back to hospital for more treatments or die at home. She chose to rather die at home and go to heaven. Now five, Julianna is more determined. After a series of clarifications on the implications of her choice of heaven over hospital by her mother, Julianna said she perfectly understood that further treatments could extend her life and give her more time with her family. She also understood that her parents would not be coming to heaven with her right away and that she would be going all by herself. Her valedictory: “Don’t worry. God will take care of me. He’s in my heart.”

    Rather than wallow in self-pity, Steve and Michelle have been excellent parents and coaches to Julianna. At age four, they, like experienced counselors, had lovingly shared the consequences of choosing heaven over hospital without compelling her to decide otherwise. The decision was left to her and not manipulated to assuage the family’s sense of loss in event of her expected death. Her elder brother has also been prepared for the eventuality that his only sister may soon be gone for good.

    They are even better prepared for Julianna’s eventual exit now that she is five and still resolved to go to her maker rather than suffer further pain. They have not inundated God with the questions of why all this is happening to Julianna and the family. Or why God would not heal her. Even in death as is most likely and perhaps imminent in Julianna’s case, God is already glorified in her faith and confidence in Him and choice of heaven over temporary earthly reprieve.


  15. Weeks after their story became a topic of discussion around the globe, Michelle Moon and her husband Steve Snow took time to thank people who helped promote awareness for their daughter’s neuromuscular disease.

    Julianna Snow is “a really happy girl” who often acts like a typical girl. “I would say 99% of the time she’s just a 5-year-old. She likes to play,” Moon told KOIN 6 News.

    But the Vancouver family made headlines around the world after their story was told in a series of reports on CNN titled, “Heaven over hospital.”...

    Julianna’s CMT began displaying itself through delayed motor skills, Moon said. It began to affect her ability to breathe and swallow and she now needs support to breathe.

    “Last year was really, really difficult. She spent a lot of time in the ICU with respiratory issues. For her, a trivial runny nose turns into something much worse,” she said.

    Michelle Moon blogs about the experiences with Julianna. CNN saw a post where Julianna’s parents talked with her about what she wanted if she got sick again — to go to the hospital or stay at home.

    “I felt I wanted to share and ultimately CNN picked it up,” she told KOIN 6 News...

    A donation jar inside Natural Body Works states Julianna is an inspiration and living example of joy in the face of a debilitating disease. The money raised during November will be donated to the CMTA.

    Other businesses participating include Niche wine bar and art gallery and Cake Happy in Camas.

    “It’s truly about the quality of life, and I think if you look at this year compared to what it was like before when we weren’t focused on that, it’s night and day,” Moon said.


  16. A recent CNN story about a gravely ill girl’s choice to die at home rather than in a hospital had such an emotional impact on two Conejo Valley residents that they decided to take action.

    Moon was genuinely moved by the offer. “We were touched when he got the book to us, and it’s gotten only better from there,” Moon said in a note to CV Happening. “I’ve never tried to raise money for anything before, and it’s been more difficult than I thought…so every little bit counts!” The money will go to CMTA, the national organization that raises awareness and funds for CMT research/treatment, according to Moon.

    Children’s book author Will Thach was stirred by the story of Julianna Snow, a 5-year-old girl from Seattle suffering from a severe hereditary neuromuscular disease called Charcot-Marie-Tooth (CMT). There is no cure, and even the slightest cold could prove to be fatal.

    “I watched the story on TV with a few of my friends and knew I had to do something to help,” said Thach, a Malibou Lake resident who wrote The Girl Who Saved Christmas. “My friends told me to send her my book. And I said, ‘of course, she should have this for Christmas.”

    Shortly thereafter, he received a note from her mother, Michelle Moon, with information about how to help. “I told her I’d like to donate $1 from the sale of each book,” he said.

    Moon was genuinely moved by the offer. “We were touched when he got the book to us, and it’s gotten only better from there,” Moon said in a note to CV Happening. “I’ve never tried to raise money for anything before, and it’s been more difficult than I thought…so every little bit counts!” The money will go to CMTA, the national organization that raises awareness and funds for CMT research/treatment, according to Moon.

    Angelo Jayamantri, owner of SweetXO in Agoura Hills, said he, too, was affected by Julianna’s story and wanted to help. “I contacted Will to see what we could do,” he said. The two teamed up by scheduling a book signing event on December 23 from 4-7 p.m. at SweetXO. Jaymantri will donate 15 percent of the sales to Julianna’s charity. In addition to meeting Thach, Wednesday’s event will feature children’s activities and free hot chocolate.

    Moon said that Julianna loves the book so much that she has memorized some passages. “It’s a beautiful book – beautifully put together and written,” she wrote.

    - See more at: http://conejovalley.happeningmag.com/author-business-owner-team-up-to-aid-family-of-dying-girl/#sthash.7D5VTFXM.dpuf

  17. When Lisa Koziell-Betz read about the extraordinary conversations about death Michelle Moon had with her terminally ill 4-year old daughter, Julianna Snow, last year, she just had to reach out.

    "I have never done that before, but I wanted them to know I have been where they are at now," Lisa, 50, tells PEOPLE. "My heart went out to Michelle and to the family and to Julianna."

    Lisa's 7-year-old daughter, Morgan, died of stage 4 neuroblastoma in January 2007...

    The family made headlines across the world last October after CNN.com posted a two-part story about them. They have been criticized for allowing Julianna to have input into her care, saying a child that age does not understand what death is.

    "That's what got me mad," Lisa says. "They do know – or some know. Morgan knew what death was. And it sounds like Julianna knows.

    "I sent her an email that said, 'Bless you. Hang in there. Do what feels right,' " says Lisa, a recreation programmer who lives in Peterborough, New Hampshire.

    "It is a hard journey, but not to honor a child in this type of situation because they are a kid, is wrong," she adds. "Some kids have a deep knowing – it cannot be discounted."...

    Lisa understands on another level as well. She made a decision for her child that some found controversial. When her daughter's cancer came back after a lengthy remission in the spring of 2006 – with 0 percent of a cure – she opted to forego any kind of treatment for the summer and just let her be a kid.

    "The doctors wanted us to get her into a treatment protocol," she says. "I said, 'Can we give her the summer?' And they said, 'Sure.' "

    She, too, criticized for her decision.

    "Some people believe that we should have treated in the hopes of a cure, at any expense," she says, "but I am proud that we chose to honor Morgan for who she was – an individual – rather than her becoming a statistic….all in hopes of 'being the one" who [beat] all the odds.

    "This, in my book would have been wrong and would have been incredibly selfish on our part," she says.

    Morgan had "an awesome summer," she shares. "She swam for the swim team. She actually got some ribbons. She was tan. She was running around. She did the monkey bars, which was something she hadn't done for years. In fact, the previous year she'd broken her arm on the monkey bars."

    Seeing her daughter so carefree was amazing, she says.

    "You look at a kid who knows she's terminal and she's out there having a ball, enjoying life for what it is," she says. "When she's in treatment, she can't have close contact with people; she's got to take a nap because of chemo, but here she was living in the moment instead of going by expectations and clocks and everything else."

    By October, though, Lisa opted for chemo and radiation –"for comfort not treatment" – to help her with the pain she was experiencing from the cancer spreading to other parts of her body.

    Morgan died at home in January 2007.


  18. Though much attention has been focused on the extraordinary conversations about death Michelle Moon had with her terminally ill 5-year-old daughter Julianna Snow last year, each day it takes an equally extraordinary amount of efforts just to keep her alive.

    Julianna, who doctors say has a rare, aggressive form of Charcot-Marie Tooth Disease (CMT) is hooked up to a breathing machine called BiPAP 24 hours a days via a mask on her nose and mouth.

    Her disease has slowly taken away her mobility, leaving her only able to move her shoulders a little.

    Michelle, 43, a neurologist, rises at 5:30 am each day to start her daughter's feeding tubes while she's still sleeping.

    After Julianna wakes up between 6:30 and 7 a.m., she and her husband, Steve Snow, 38, help Julianna do range of motion exercises, after which they begin a series of respiratory treatments to help her breathe, which takes about an hour.

    She also gets suctionioning throughout to remove excess mucus.

    Michelle, who works four days a week but takes no vacation so she can get full time pay, leaves for work around 8 a.m. – and then Steve, a U.S. Air Force veteran, takes over.

    After her morning treatments, Steve helps Julianna brush her teeth, get her dressed and take her meds: an anti-nausea drug, an antacid and a steroid inhaler. Steve also has to regularly flush out her feeding tubes.

    "These are her 'chores' as we call them," she says then adds, laughing: "Actually getting dressed is a chore to her – anything that isn't playing is a chore."

    During the week Julianna goes to kindergarten via Facetime at 9:15 a.m. for 30 minutes.

    "Her teacher is really good and interactive," says Michelle.

    Her nurse is there from 10 a.m. to 6 p.m. each day during the week to help with Julianna – which includes playing with her. (A different nurse paints her toe nails and fingernails).


  19. Julianna has significant scoliosis and gets tired from sitting upright for too long. So every two hours (at least) they give her what they call a body break where she lays down flat on her bed for 30 minutes.

    "She doesn't like that either," says Michelle," because she can't play."

    From 2 to 4 p.m. used to be her nap time but is now more of an extended body break.

    "She doesn't fall asleep but we have her lay down so she can watch a movie," says Michelle. "The TV's positioned so she can watch it from a prone position."

    Julianna picks the movies, of course.

    "It's usually Mary Poppins or one of her cartoon movies," she says.

    Around 4 p.m. they repeat the same respiratory treatments from the morning.

    Afterward, it's bath time then Julianna is wheeled out to the family's main living area (her bedroom is on the first floor) in the special cart her grandfather made to transport her and her equipment. Michelle gets home from work between 5:30 and 6 pm, so they all can enjoy some quiet family time together.

    Julianna goes to bed around 8:30 p.m. when she gets one more med.

    Steve or Michelle used to stay with her until she fell asleep around 10 p.m. – which is also when Michelle and Julianna had so many of their conversations about death. But after Julianna caught a small cold and laryngitis in December, they started taking turns sleeping in the room with her, Michelle says.

    "It makes us feel better knowing we're even closer," she says.

    They have no extra help on the weekends, Michelle says, but on Tuesday mornings she has occupational and physical therapy; on Wedneday mornings a hospice nurse comes in and on Thursdays, a grief counselor comes. She also has a tutor that comes in three times a week in the afternoon.

    The family also takes an extraordinary amount of care to ensure Julianna doesn't get sick. Alex takes a shower when he gets home from school each day while Michelle does the same when she gets home from work.

    "It's really, really scary when she does," says Michelle. "That's why we go to such lengths. It's also really hard when Alex gets sick."

    When that happens – which it hasn't lately – "one person will be devoted to Alex and one to Julianna and we don't cross," she says.

    Through it all, Julianna somehow remains cheerful, though she does ask her mom to hold her hand at times.

    "She's very happy and resilient," says Michelle. "The only time she's down is when she's in the hospital."


  20. Julianna Snow, the 5-year-old girl who sparked an international conversation about wrenchingly difficult decisions for terminally ill children, died Tuesday.

    "Our sweet Julianna went to heaven today," her mother, Dr. Michelle Moon, wrote on a blog devoted 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."...

    Julianna was in hospice for the last 18 months of her life.
    During that time, she loved to wear princess dresses, make up elaborate stories and games, do arts and crafts projects with hospice volunteers, and have her toenails painted.

    In a text to CNN, Moon said the end was very quick.

    "She got sick very suddenly and it was back to the same battle of helping her breathe," she wrote. "This time, she didn't bounce back -- she got worse and worse and worse and was gone in just over 24 [hours].

    "We had the full support of hospice and we had the things we needed to keep her comfortable. She died at home, in her princess room and in my arms. I don't know that she would have wanted it any other way."...

    In her blog, Moon wrote that she wants the world to remember Julianna as the "bright light" who "loved love and 'everyone except the bad guys.' "

    "Please do not forget her," Moon wrote. "She lived, she was real, and she mattered."

    "Her mind was always going. She took us to a beautiful place without limits," she added. "She encouraged us to be our most colorful and fabulous self."

    In the end, Julianna got her wish to die without medical interventions.

    "She fought hard to be here, harder than I've seen anyone fight, with a body that was too frail for this world. She was so brave -- and I hated that she had to be so brave," Moon wrote.

    "Today she is free. Our sweet Julianna is finally free."


  21. On Tuesday, Moon wrote, “Our sweet Julianna is finally free. Please do not forget her. She lived, she was real, and she mattered.”

    From Moon’s blog post:

    Dear friends,

    Our sweet Julianna went to heaven today. 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. I wanted more time, of course, and that’s where the sadness comes in. But she is free now.

    I will have more to say later. For now, this is what is in my heart.

    Today, I just want the world to know that there was a girl named Julianna.

    She was a bright light. An old and delightful soul.

    She loved love, and “everyone except for bad guys.”

    She was an unabashed princess and she elevated everyone around her. We were all kings, queens, princes and princesses by association.

    She urged us to play, to really focus on just playing. She encouraged us to be our most colorful and fabulous self. (One of her last words to me: “What’s that?” to my gray sweater. . . .)

    Her mind was “always going.” It took us to a beautiful world without limits.

    Her words were startling. Sometimes I thought that people wouldn’t believe the conversations I recorded. How could a five year old know those things? But if you spent any time with her, you knew.

    She fought hard to be here, harder than I’ve seen anyone fight, with a body that was too frail for this world. She was so brave - and I hated that she had to be so brave. This last fight was not to be won by her body. It was tired, and it needed to rest. And when it did, she was comfortable.

    Today, she is free. Our sweet Julianna is finally free.

    Please do not forget her. She lived, she was real, and she mattered.

    I cannot believe that she’s gone. Already, I worry that some of her has faded, and I need to remember all of her. The way her warm little hands felt, the hugs she would give by asking you to drape her arm around your neck. The kisses she blew. They never ran out.

    Please remember our precious girl: she was Julianna.

    Read more here: http://www.sacbee.com/news/nation-world/national/article83901777.html#storylink=cpy

  22. 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, Wash., was diagnosed with Charcot-Marie-Tooth Disease, which is one of the world’s most common inherited neurological disorders, according to the National Institutes of Health. 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 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 in order 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.”

    Read more here: http://www.sacbee.com/news/nation-world/national/article83901777.html#storylink=cpy