When Julianna Snow was nine months old, her parents, Michelle and Steve, noticed that she could not sit up steadily, something most babies can do around six months. At her first birthday, when children typically start to walk, Julianna could not even pull herself up to a standing position.
Juliana’s pediatrician said she was just a “late walker” and would catch up soon. He affirmed that five percent of children don’t walk until they are eighteen months old. Michelle wanted to believe him, but her mother’s intuition and her training as a neurologist told her there was something more worrisome. After lengthy research, she discovered that Julianna likely had spinal muscular atrophy, a hereditary disease where neurons in the brain and spinal cord are progressively destroyed.
Steve’s odd-looking feet, with high arches, turned out to be the culprit. Two neurologists confirmed that Steve had a mild case of Charcot-Marie-Tooth (CMT) disease which manifested as a severe case in his daughter. In 2012, just after Julianna turned two, Michelle and Steve brought her to the University of Iowa to be diagnosed by Dr. Michael Shy, the world’s leading expert on CMT. He confirmed the diagnosis explaining that the myelin sheath covering Julianna’s nerves had never formed correctly and her nerves were progressively degenerating.
Michelle describes a “slow-motion horror story” from this time on. CMT was attacking not only Julianna’s nerves but her ability to breathe. She needed more than just a tube of oxygen in her nose. She needed a pressurized mask pumping air into her. By the time she was four, she was hospitalized three times at Doernbecher Children’s Hospital in
Portland, Oregon, where Steve and Michelle moved to be close to his parents.
Julianna was fitted for a back brace to fix the scoliosis that developed as a result of her weak muscles, and for ankle and feet braces. She underwent physical and occupational therapy. But she got worse. She could not even hold a small toy without help and her chewing and swallowing muscles were so weak, she was fed through a tube in her stomach. Repeatedly, mucus was settling in her lungs and causing pneumonia.
Several times daily in the hospital she underwent nasotracheal suctioning when 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 this procedure. Stronger children usually scream and have to be restrained when undergoing this procedure. Julianna was so weak all she could do was cry.
It was at this crucial point that Steve and Michelle asked her if she wanted more treatments or die at home. Julianna did not want to go back to the hospital again. She wanted to go to heaven and said, “God will take care of me.” Steve and Michelle decided to honor her wishes.
While there is no debate about the medical facts of Julianna’s condition, there is concern about what her parents have chosen to do: they asked their four year old daughter to make a decision between treatment or dying.
If Julianna were an adult, there would be no debate about her decision to forego painful and burdensome treatment that only prolongs her dying. If she chose further treatment, she would likely be sedated, unable to think and talk, and die in a short time.
Bioethicist Art Caplan believes that Steve and Michelle made a wrong decision. He believes “there’s zero chance a 4-year-old can understand the concept of death. 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.”
Dr. Chris Feudtner, a pediatrician and ethicist at Children’s Hospital in Philadelphia, disagrees. She believes that to conclude that Julianna’s “experience is irrelevant doesn’t make any sense. She knows more than anyone what it’s like to be not a theoretical girl with a progressive neuromuscular disorder, but to be Julianna. Her choice for heaven is reasonable and her parents are right to listen to her. This little girl has chosen how she wants to live.” Julianna’s pulmonologist agrees.
I believe that the fact that the parents are agreeing to what Julianna wants is beside the point as this is in fact the equivalent of them making the decision, although in an unusual manner given that she is so young. As far as the law goes, the question is whether this decision is clearly contrary to Julianna’s best interests, and is therefore medical neglect. I would conclude that it is not as further treatment seems unduly burdensome with little likelihood that Julianna will be significantly benefited.