Joseph Saliba enjoys activities that most eight year olds typically like, such as being tickled and riding his bike.
However, unlike most children, Joseph has a rare genetic metabolic condition called Sanfilippo Syndrome, which is commonly referred to as “Childhood Alzheimer’s.”
“[Children with] Sanfilippo Syndrome, their brain starts to go, like dementia,” said Francine Saliba, Joseph’s mother. “Say he’s walking and talking one minute, and then like with Alzheimer’s, they start to lose their memory, their bodily function, speaking, everything. Until they die pretty much.”
People with this disease lack or have a malfunctioning enzyme that the body needs to break down molecules in our cells.
“These are long chains of sugars or carbohydrates in our cells and they help build bones, cartilage, tendons, skin, and connective tissue,” explained Kim Angel, executive director of Canadian MPS Society, an advocacy group for people with this condition.
“It primarily affects the central nervous system at first, so the brain and the spinal cord.”
The life expectancy for people with Sanfilippo Syndrome is between five to 15 years old.
“When I found out that the average age of these kids, I just felt like I was having a heart attack. That was it, I’m losing everything, all of my dreams. All I ever wanted was to have a family,” said Francine.
“I don’t get to see my son grow up, I don’t get to see my son graduate, I don’t get to see him get married, or anything like that.”
Francine said when Joseph was born he was very advanced for his age. He said his first word at three and a half months and started walking at nine months old.
However, at age one, Joseph started getting chronic ear infections.
“We notice he started declining in his verbal skills and every day learning skills,” Francine said.
Francine said for two years, she took him to several doctors to figure out what was wrong with him. Finally, after going to a geneticist at McMaster, he was diagnosed with Sanfilippo at age four.
“The main signs for these kids is a protruding belly, a hernia. And then, also, very strong facial features, so he has a predominant nose, predominant lips,” Francine said.
Approximately one in 70, 000 people are diagnosed with this disease. There is no cure.
Currently, there is a trial underway for gene therapy, that could help prolong life.
“The gene therapy would basically allow the body to start producing the enzymes on its own, and thus halting the progression of the disease,” said Angel.
However, the trial is only available for children under two years old.
“What happens is around three years old, the developmental portions start rapidly declining. So the gene therapy cannot bring that developmental portion back up. It cannot rebuild the neurocognition or the damage that’s been already done by this disease,” Angel said. “If it can be administered before the age of two years old, the damage isn’t that bad.”
By the time Joseph was diagnosed with the condition, he was too old to start gene therapy.
Francine now wants to bring awareness to Sanfilippo, “that way it’s not too late for other parents.”
Her hope for her son is to have a long life and said she is grateful for every day she gets to spend with him.
“That’s all I can do, is just give him the best of what I can, every day. He’s my little ray of sunshine.”