Joseph Dendekker – Saliba was a God given child since birth. Joseph had a rare and terminal metabolic disorder likened to childhood Alzheimer’s disease.
The condition is marked by severe symptoms like progressive dementia, mental regression, developmental delays, declining motor skills, aggressive behaviour, hyperactivity, some deafness and vision loss, and the inability to sleep for more than a few hours at a time. By the time Joseph was 14 months old some other symptoms started to appear that led Francine questioning. It all started with chronic ear infections leading to 3 separate operations for ear tubes.
Chronic ear infections are one of the first symptoms that show up, and a lot of doctors end up wrongly diagnosing the cause, instead pointing to more common issues, like allergies.
Then other symptoms started to show up: a constant runny nose; he had a protruding belly and an umbilical hernia. By four-years-old, he began developing predominant facial features. After chasing a diagnosis for two years, they finally saw a geneticist at McMaster, who knew it was Sanfilippo right away.
They took bloodwork and urine samples for genetic testing from Joseph and parents, which confirmed the diagnosis: that it was one of seven rare mucopolysaccharide (MPS) disorders.
Children with Sanfilippo Syndrome, or MPS IIIA (rarest form), are missing an essential enzyme that breaks down a complex body sugar called heparan sulfate, which is a glycosaminoglycan, or GAG for short . Because it can’t be broken down, the GAGs will keep getting bigger and bigger; it will slowly build up in the bones, the brain and other organs, stopping normal development and causing brain damage. Resulting in a slow death.
While each kind of MPS is clinically different, those afflicted with it will typically start out with normal development followed by a decline of physical and mental function.
Because it attacks the brain the same way dementia does, they typically forget the first words they learned, losing their language skills, followed by a loss of motor function. By age 10, they usually lose the ability to walk. Eventually, their liver, their heart, their intestine, everything starts to enlarge, to the point where they have to be in a wheelchair or in a vegetative state, or have breathing tubes and a feeding tube. Over the years Joseph progressed in a very different way due to the alternative holistic health practices Francine introduced.
Joseph was a vibrant little boy who loved to play and enjoyed music just as much as his mother Francine does. His smile would always light up a room and the laughter was unforgettable. Although Joseph had this rare condition, Francine never gave up. Joseph had his own language that his mother knew and understood which allowed her to listen to her intuition in hospital situations until the day Joseph decided to leave with peace.
Joseph has touched so many hearts with his infectious smile, his beautiful eyes and his silly personality. We hope to share Josephs story in such a large way to show that other families suffering with this condition aren’t alone and this journey is anything but easy.
Thank you for taking the time to read about our story and to support us along on this rare journey. This is now just the beginning of our new journey of sharing the story of Joseph.
