Arky is one of only two kids in New Zealand with congenital athymia, which means he’s unable to develop an immune system. This ultra-rare disorder came along shortly after his primary diagnosis of CHARGE Syndrome – so, to help him keep beating the odds, Satan’s Little Helper provided a few tools to keep him charging ahead!
Lots of parents think their kids are one of a kind, but three-year-old Arky really almost, is. He is one of only two children in New Zealand to have. But Arky’s health is complicated by CHARGE syndrome – an anagram for a cluster of conditions that include coloboma of the eye, heart defects, atresia of the choanae, restriction of growth and development, and ear abnormalities and deafness.
Beating the odds
“Arky has abnormally small ears and his nose wasn’t fully formed when he was born. He has under-developed extremities, deafness, and visual impairment; and he’s non-verbal and needs a full-time tracheostomy to help him breathe. He’s also fed through a tube,” explains his mum, Erin.
“Doctors told me he was unlikely to leave the hospital alive. The odds were stacked against him!”
Arky was also born with congenital athymia, which means he was missing a thymus gland. Responsible for the production and maturation of immune cells, the thymus gland is needed to develop an immune system. The average lifespan of a child born with congenital athymia is around two years old.
To beat the odds, Arky flew to the UK for a life-saving transplant.
Taking a child with no immune system on a commercial flight was scary enough, but to make matters worse, Erin had to leave husband Nathan and daughter Ruby behind during the initial stages of the Covid-19 pandemic.
Alone in a pandemic
“His operation was at the Great Ormond Street Children’s Hospital in London, which is one of two centres in the world that treats athymia. We had to stay there for four months under strict Covid restrictions.”
Armed with a new thymus, and a tracheostomy in the mix of it all, Arky and Erin made it back to NZ and out of managed isolation in time for Christmas 2020!
“He now has an 80-90% fully functioning immune system, although we still have to navigate his CHARGE syndrome.”
Because of the time spent overcoming his thymus problem, Erin couldn’t focus on his cognitive development, which has been held back by hearing and vision loss and sensory issues caused by CHARGE.
A bit of catching up to do
“We avoid public spaces and haven’t done a lot beyond our home due to his lack of an immune system, he has delayed development emotionally and physically as a result of prolonged hospitalisation. Over the next few years, we’ll need to do lots of character building, personality development and physical stimulation,” she adds.
However, there have been some positive milestones in Arky’s development!
“A year ago, he couldn’t crawl. Now he pulls himself around on furniture or waddles on his knees! He can also count to 10 and knows his ABCs.”
To help continue that momentum, Satan’s Little Helper sent him some sensory and cause-and-effect toys.
“The ocean wave projector is amazing,” says Erin. “It helps to calm and relax him and it’s a good source of sensory stimulation and de-sensitisation at the same time.”
“The wooden rolling rainbow tracks is a marble game that he loves so much. It’s helped his hand-eye coordination and core strength, because it encourages him to go to his knees and reach up to play with the tracks. It also helps his communication! I’m shocked to see how one simple toy can have so many benefits.”
Working on his core
Arky’s lung development remain a concern and he’s currently ventilated over night to support his sleep.
“I know we can cut that down, but the only way to get him off the ventilator is if his core and lungs are strong enough. My goal is to wean him off the ventilator completely, hopefully in the next three years, and I’d love to decannulate his tracheostomy further down the line!”
With toys like the rolling rainbow tracks, Arky has another tool to navigate CHARGE syndrome and continue his (almost) one-of-a-kind story!