The history of a five-year-old girl, Julianna Snow, with Spinal Muscular Atrophy (SMA), who decided to die progressively because of her incurable disease instead of living with medical treatment, has been described by Elizabeth Cohen, CNN Senior Medical Correspondent in a 2-part piece, titled “Heaven over hospital: Dying girl, age 5, makes a choice.”
The parents, Michelle Moon and Steve Snow, declared that they decided not to intervene in their daughter’s decision. Julianna’s SMA is a terminal disease that affects the part of the nervous system that controls voluntary muscle activity.
SMA is the most common genetic cause of infant death. This aggressive disease is caused by a genetic defect in the SMN1 gene, which encodes SMN, a protein widely expressed in all eukaryotic cells.
The degenerative disease manifests in various degrees of severity, which have in common progressive muscle wasting and mobility impairment. First it attacks proximal muscles and lung muscles.
Evolution of the disease
When Julianna had 9 months, their parents started to notice that she was having problems with sitting straight. When she was getting older, her condition got worse, and she reached a point where she didn’t even pull herself into a standing position. Michelle Moon was a neurologist and she really got worried about her daughter’s condition, but she explained that, at the first moment, she really tried to remain calm.
Mrs. Moon looked for answers when she realized that her husband had a problem with his tooth muscles, and she decided to evaluate him. She attached electrodes to his arms and legs to determine if the problem of her daughter was genetic.
After doing the whole process, she diagnosed Steve with Charcot-Marie-Tooth Disease, or CMT, a group of inherited disorders that affect the peripheral nerves, which are the nerves outside the brain and spinal cord. There are more than 70 kinds of CMT, each kind is caused by a different kind of mutation, and more cases are being discovered every year.
His CMT genetic problem had manifested as a severe case in his daughter. When Julianna turned two, Michelle and Steve decided to look for some experts’ opinions and sent her DNA to a specialized genetics lab at the University of Miami. For their despair, the severity of CMT depends on the specific genetic mutation that has caused it and they couldn’t find anything definitive.
The interview – ‘The hard part’
The emotive interview made by the CCN medical correspondent, Cohen, allowed Michelle and Steve to realize how hard and difficult was the whole treatment process for the girl and that ‘she was ready to go with god’. The girl never had the healthy conditions needed to attend Sunday school at the City Bible Church in Portland, Oregon, where her family belongs.
When Julianna’s mother asked her if she wanted to come back to the hospital she said “Not the hospital.” Instead, she preferred heaven before doing more therapy or treatment without hope. She let her mother know that she knew how they will miss her, and she also said that god would take care of her because ‘he was on her heart.’
“She made it clear that she doesn’t want to go through the hospital again […] So we had to let go of that plan because it was selfish.” Michelle wrote CNN in an email.
The girl who endured for years a difficult treatment feared death, but what she feared most was coming back to the hospital, she made clear that she was more than ready to go. For her parents, this might be the hardest decision of their life, but they were happy because they let her daughter decided her destiny.
“Unbelievable that any parent would think a 4-year-old is able to understand or make a decision on life.” said Michelle and Steve after hearing Julianna’s wishes.