The family of a Northwest Territories toddler with a rare, life-threatening condition is hoping doctors in the United States will be able to diagnose what is making him sick.
Deagan Clavette, nearly two years old, was born to a family from Hay River, N.W.T., but he has called the Pediatric Medical Unit at the Stollery Children's Hospital in Edmonton home for most of his short life.
"He's basically a bubble boy allergic to life," said his mother, Jennifer Tregidgo-Clavette.
When he was five days old, Deagan temporarily stopped breathing. At six weeks, he again went into respiratory distress and was medevaced, first to the hospital in Yellowknife then on to the Stollery Children's Hospital in Edmonton.
He returned home but at the age of seven months he went into anaphylaxis and underwent an emergency tracheostomy in order to breathe. He was medevaced again to Stollery, where he has remained for the last 15 months.
The Clavette family — Jennifer, husband Kevin and their first son Kaelin — relocated from Hay River to Edmonton to be with Deagan.
Deagan has been discharged twice since then but those visits were short, totaling less than three weeks, with Deagan landing back in the ICU each time.
A medical team has been working around the clock to keep Deagan comfortable, while trying to diagnose why he has unpredictable and potentially fatal reactions.
Tregidgo-Clavette says the reactions at times can build up gradually. "Sometimes he's red and rashy. His eyes will swell. His lip will swell."
Other times his reactions are immediate and frightening, such as losing consciousness, or going into shock.
"His airway will start to swell. He'll need a puffer and an EpiPen or eventually [he] will go into anaphylaxis."
Tregidgo-Clavette said he once had to have seven EpiPens in a 24-hour span.
"Plus [he] ended up on an epi-drip which no other child has been on in the Stollery [Children's Hospital] due to anaphylaxis, which is scary."
In many ways Deagan is a normal toddler. He likes books, walking and playing. He's curious and always exploring the world around him.
But his world is small. He needs 24-hour supervision, either from his parents or hospital staff.
Doctors have confirmed, so far, that he has 25 allergies, including carrot and sweet potato, and other substances such as latex, detergents and scents. The only food he can tolerate is a diet of amino acids fed through a feeding tube directly into his gut.
A tracheostomy remains in place in his throat to help administer medication and assist his breathing when his airway swells. He uses sign language to communicate.
Isabelle Chapados, a pediatrician and pediatric endocrinologist at the Stollery Children's Hospital, is one of Deagan's primary physicians.
"We know how to deal with allergies — it's the other type of reactions where we don't exactly know what to do for him," she said.
"It's not allergic. It's another type of reaction that we have never been able to pinpoint and that makes his life very unpredictable."
The team of doctors have been treating Deagan for a form of Mast Cell Disease. When mast cells — a type of white blood cell that helps regulate the immune system — don't function properly they can release too many chemicals causing reactions in the skin, respiratory, and neurologic systems, with or without a known trigger.
Chapados estimates MCAS affects about one in every 100,000 people and says it's "quite rare," adding that she has never seen a patient with the disease before.
However, none of the laboratory tests has confirmed a diagnosis.
The family and doctors are working to get a teleconference with the U.S. National Institute of Health which specializes in research and treatment of new and rare diseases. Up until this point the Government of the Northwest Territories has been covering his hospital stay and treatment, but the family and physicians are still figuring out who or what jurisdiction would pay if Deagan needs to go to the U.S. for a diagnosis and ongoing care.
'He needs to be out living a normal life'
Tregidgo-Clavette praises the doctors and nurses' efforts keeping him alive but wants an answer as to what is wrong with her son and how they can manage his condition as he gets older.
"Deagan will not survive without a diagnosis and without a life management plan in place," she said.
The family has been relying on a Go Fund Me campaign to help pay for their mounting bills, including renting an allergy-free home in Edmonton. Then in December, Deagan's father Kevin received a layoff notice from De Beers' Snap Lake diamond mine, when the company announced the mine's shutdown.
She said she hopes a confirmed diagnosis and treatment plan is near.
"[Deagan] cannot live in the hospital for the rest of his life," she said.
"It's not feasible. He needs to be out living a normal life with his brother playing out in the snow and the grass enjoying life like a two-year-old boy should be able to do."