FORT MCMURRAY ( 660 NEWS ) — Reign Johnston was born with a concave chest and a small curvature of his spine. After months of doctor’s visits his parents, Alex and Ryan, requested that he see a new pediatrician. After being referred to River Valley Pediatrics in Edmonton, they were soon sent to Edmonton’s Stollery Children’s Hospital. His mother, Alex, says that her son was breathing three times as fast as he should have been, and had failure to thrive. He was too weak to swallow and because of this could no longer breastfeed.
“During that visit at the Stollery he got an NG tube,” Alex says “which is a skinny tube that goes in his nose and down to his stomach. I had to feed him with a syringe through the tube every two hours. It was constant.”
Doctors ran tests and soon diagnosed Reign with Spinal Muscular Atrophy (SMA) Type One. SMA is a disease that causes rapid muscle deterioration, and can cause a child to struggle with things like walking, breathing, eating, and sitting.
“His lungs were collapsing, he had two PICC lines removed. During his second PICC line he had a seizure reaction to anesthetic, so now he can’t get anaesthetic at all.” Alex says, “During his Spinraza, all kids are sedated. But he can’t be sedated, he has to do it awake. It’s really a lot for him.”
AUDIO: 660’S Devon Banfield speaks with Alex Johnston, the mother of Reign – a two-year-old boy from Edmonton with SMA Type One
Spinraza is a treatment for children with SMA. Once a month Reign receives a lumbar puncture, where doctors remove five milliliters of spinal fluid and replace it with the drug Spinraza. After his diagnosis Reign spent a few weeks at the Stollery before being discharged, only to return months later.
“One day we took him to a restaurant to meet my grandparents. There he stopped breathing, he had cardiac arrest,” Alex says, adding “He had to be resuscitated by ambulance and firefighters. He was taken back to the Stollery, and we had to stay there for seven months.”
Reign spent four months in the pediatric intensive care unit, and another three months receiving general care. His mother says that Reign has to be constantly supervised to make sure he continues to breath, and has nurses that care for him while his family sleeps at night.
The family says they were denied access to Canada’s “Zolgensma Lottery”, also called the Managed Access Program and the Special Access Program because Reign relies on a BiPap machine, which helps him to breath, for over 16 hours a day. When Alberta announced case by case funding for Zolgensma in February, Alex says they thought this was their chance.
“We felt like it was for Reign,” she says “But a month after our application, we still have no word back.
Johnston says that she’s done everything she can think of, including writing to her son’s doctors and the office of Alberta Minister of Health Tyler Shandro. The family says this new development is frustrating.
“Now Reign is two years old and his symptoms this whole time have only been progressing. We’ve been fighting for two years now, and we just don’t understand why Reign hasn’t got it yet.” Alex says, adding that so many doors have been closed in their faces.
If you want to help Reign’s family, you can donate on GoFundMe.
My McMurray reached out to the Government of Alberta for a statement
Karin Campbell, a spokesperson for the Government of Alberta Health, said “While we can’t speak about specific children and their families, we recognize that any family who has submitted an application would be eagerly awaiting a decision” adding that “In Alberta children with SMA will be considered for funding on a case-by-case basis. Medical experts reviewing these applications give careful consideration to each and every situation before a decision is reached. Their evaluation includes assessing the latest scientific evidence, current recommendations for the treatment’s use and data that shows whether a patient will benefit from this particular treatment. These specialist physicians recognize the seriousness of each child’s circumstances and each application is given the time and attention needed to make this important decision.”