While dementia is normally associated with old age, 5-year-old Harley Bond was unfortunately diagnosed with Sanfilippo syndrome when he was 3, a rare type of childhood dementia.
When Harley stopped saying ‘mommy’ and ‘daddy’ because he didn’t recognize them, parents Wayne Bond, 47, and Emma Siddal, 38, from Sheffield, were left heartbroken.
Watch to find out more of this boy’s story below.
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Video credit: Rumble
But a clinical trial at Great Ormond Street Hospital has given them hope as Harley’s speech and ability slowly improved and even started to recognize them again. In the ongoing trial, a chip was implanted into Harley’s head.
The chip releases minuscule quantities of the enzyme that he lacks. While Harley still has bad days, at least his condition doesn’t seem to be worsening.
Emma said: “It was heart-breaking in the months he couldn’t recognize us, he struggled to make eye contact with us, and didn’t call us mom and dad.
“The trial is doing well, and it’s really giving us a little bit of hope for the future, getting on it was a potluck and we’re happy he is responding well.
“We realize, however, that it won’t cure him, but it might give us a few extra precious years with our brave son.”
While Harley was developing normally in his first few months, a spate of infections and colds, as well as his speech going backward when he was three, started to worry his parents. In 2016, he was diagnosed with Sanfilippo Syndrome type B.
After the devastating diagnosis, Harley started to get worse. He would sometimes stop breathing for a short while at night and he couldn’t discern what was safe to eat. They had to constantly watch over him as he was just as likely to eat a rock as he would baby food.
In 2017, Harley’s parents enrolled him in an experimental clinical trial after hearing about the possibilities it offered.
Wayne said: “He was born completely fine and developing well, but then everything changed.
“He was really boisterous with other kids and his nose was always runny, he kept getting infections and started to lose his speech.
“When he went to the park or was with other kids, we knew there was something up because he just didn’t act like everyone else, he was really boisterous.
“He couldn’t remember simple things like knowing how to use the toilet or what was food and what was not.
“The hospital spoke to us about the option and let us know about this new trial, but they told us he would need to do a year of studying to assess his memory.
“Luckily his memory was good enough to be able to get onto the trial, he could remember enough of what he was told to show there was hope he could be saved.
“It was going well but then he had a bad patch for a few months, he didn’t know who we were and couldn’t even call us mommy or daddy, the whole thing is soul-destroying because we’re looking at our little boy and he’s dying.
“We try to do everything we can to stay positive and being mentally positive is the hardest part, kids with this syndrome don’t make it to adulthood so we know we just have to enjoy the time we have with him.
“Knowing that our son has dementia was hard to even comprehend, but that’s why it’s so important that everyone starts to know that it is possible and that it isn’t just older people this disease can affect.
“With the clinical trial he is on, he has good days and bad days but naturally this condition is supposed to just get worse and worse, which isn’t the case with Harley, sometimes he’s completely in control of himself but other days he can’t move.”
Several US pharmaceutical companies are running the trial. In essence, it’s an enzyme replacement therapy and the implant bypasses the blood-brain barrier by directly supplying the missing enzyme to the brain.
Allievex is one of the firms running the trial.
Thomas Mathers, Allievex CEO, explained: “In general Sanfilippo is a disorder, and in these children, they have a genetic mutation which stops them from being able to produce a certain enzyme that they need otherwise their neurons will be killed off.
“We have a device that is implanted into the child’s head which releases a synthetic version of the missing enzyme directly into the brain that washes the sugar build-up which causes the neurons to be killed.
“Worldwide, we have 22 children who have used or are using this trial.”
Despite the challenges, Harley still manages to be a kid.
Wayne said: “When you watch him go through all of this, he seems invincible – he rarely complains about any of it.
“We have good and bad days, but we’re cherishing the time we have with him and hopefully the treatment will work, and we’ll have him for many more years to come.
“Sometimes he forgets who we are and it’s awful, but we always remain strong and it’s made our family a unit of strength because we have been through it all and remain resilient together.
“Before the trial started, Harley was just getting worse but since he has been on it, he has remembered words he could no longer say, he recognizes us as mum and dad and the condition seems to of stabilized.
Emma said: “We are so proud of everything our little boy is achieving, we thought he would just get worse but he seems to be improving very slightly in some cases.”
Wayne added: “He’s such a strong lad, and he gives us glimpses of his personality when he can, which we love.”
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