Five-year-old Julianna Snow is suffering from an incurable neurodegenerative disease that robbed her from the perks of being a child, urging doctors to predict that if she contracts pathogens as simple as the common cold virus, it could kill her. Being in and out of the hospital whenever she gets sick, she loathes the idea of going back there and her parents allowed her to choose "heaven or hospital."
Charcot-Marie-Tooth (CMT) disease, also known as hereditary motor and sensory neuropathy (HMSN), is a hereditary condition characterized by damage on peripheral nerves affecting around one in 2,500 people in the United States. Peripheral nerves are found outside the brain and the spinal cord, which relays motor and sensory information.
When these are damaged, the patient will suffer from muscle weakness in the foot, lower legs, arms, fingers, wrists and even the tongue. In later stages of the disease, it could weaken the muscles in the lungs, making it difficult for the patient to breathe.
The disease's onset is expected to start during adolescence or adulthood. However, in Julianna's case, her parents noticed her condition when she was about nine months old as she was unable to sit while other babies can sit upright starting at six months.
Michelle Moon, the little girl's mother, made a blog post in May when she shared her conversation with Julianna about going back to the hospital. She recalled that her daughter wanted to stay home even if she gets sick.
"So if you get sick again, you want to stay home? But you know that probably means you will go to heaven, right?" Moon told her daughter. Julianna knows that if she dies, her mother and father won't be able to go with her. "But I won't be alone," the intelligent girl answered.
The family has been attending the City Bible Church in Portland, Oregon where Julianna learned a lot about God and heaven. However, the family's decision earned mixed reactions from many people.
"This doesn't sit well with me. It makes me nervous," Bioethicist Art Caplan said upon reading Moon's blog. He thinks she made the wrong decision when letting her daughter decide about death at just age 4.
"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 nine or 10," he added.
Moon is a neurologist and she unfolded the mystery of her daughter's delayed developmental milestones. Worried when her daughter could not walk by age 1, she conducted her research.
She read something about CMT disease and when she came across its symptoms, it was just like Julianna's. She immediately got herself tested for the defective gene but it was negative. At an instant, she remembered her husband's weird-looking feet and when he got tested after his deployment as an Air Force in Korea, it turned out he had a mild case of the hereditary disease.
Apparently, his mild case turned out to be a severe one for their daughter. The onset was early and the progression of her muscle weakness was fast that she suffered from respiratory difficulties starting when she was 3 years old.
She spent days to months in the hospital's intensive care unit being connected to tubes of all sorts. The traumatized child hated the hospital and the procedures being done to her.
"When we started hospice in November 2014, she could come off BiPAP for about two hours at a time. She is now completely BiPAP dependent. She has also lost almost all use of her arms. It's painful to witness the regression of your child, but now my focus is different," Michelle said in a second blog post in June.
The couple were decided that if their daughter would get sick, they would definitely bring her back to the hospital. However, when they heard Julianna's wish, they decided to honor it.
"We agreed that we would continue to talk to her about this. If she changed her mind, so be it. Our plans became plans 'for now'," she wrote.
"We've been given a second chance to enjoy our Julianna. I'm still scared of what is to come and can't really imagine life without my baby girl. I know that this won't last forever, but I'm grateful for the time we have now," she added.
