Sunday 25 September 2016

Watch: This video could save an Irish boy's life

Having a child with a potentially fatal condition would push most people to despair. But Alan and Michelle Finglas tell about their ambitious campaign to save their son Dylan's life, and the lives of others

Joy Orpen

Published 18/05/2015 | 02:30

Michelle and Alan Finglas with their son Dylan. Photo: Gerry Mooney.
Michelle and Alan Finglas with their son Dylan. Photo: Gerry Mooney.

Dylan Finglas is an incredibly cute two-year-old.

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He's one of those immensely happy children who never stops smiling, and who loves to interact with everyone and everything around him.

But the unbearable truth about this funky little guy is that he is the only known person in Ireland living with a devastating genetic condition. This disorder is caused by an enzyme deficiency, which in turn affects the central nervous system. If its progress is not halted, it will result in Dylan's organs shutting down, ending in his tragic, early death.

His softly spoken, pretty mum, Michelle, says there was no indication that anything was wrong for most of the pregnancy. However, when she went to the hospital for a routine appointment two weeks before her due date, she insisted on a scan, as she had some serious concerns. "When they did the scan, they discovered there was very little [amniotic] fluid around the baby, so they admitted me straight away," she volunteers. "All I had with me was my handbag."

Baby Dylan Finglas was safely delivered that night by Caesarean section. Alan, his proud dad, was there to welcome him into the world. "I felt on top of the world," says Alan. "I just remember thinking I wanted to be a really, really good dad." Dylan turned out to be a model child. "He slept well, took his bottle happily and there were no real problems," Michelle recalls.

Alan and Michelle, who are in their 30s, live in a spacious home in Tyrrelstown, north Dublin. Back then, they felt their life was complete in every way. Following a year's maternity leave, Michelle returned to her job at Bank of Ireland, leaving Dylan in the care of his doting grandmother, while Alan continued working as a carpenter in the film industry.

As the months jogged on, Alan and Michelle became concerned that Dylan was slower than other children of the same age in his development and in achieving milestones. "We kept thinking, 'he'll get there in the end'," says Michelle.

But when he was still not walking unaided at 18 months, a public-health nurse arranged for him to have physiotherapy. When he was 22 months old, a doctor then recommended that Dylan be referred to consultant paediatrician Professor Alf Nicholson at Temple Street Children's University Hospital for an assessment.

"He did blood and urine tests," says Alan, "to rule out a number of conditions, including some that were rare. The bloods all came back fine, but one of the urine tests was slightly out of range, so he suggested doing another test to eliminate that. It was sent to a specialist laboratory in Manchester."

It took almost two months to get the results back, and following one further test, which only took a week, Prof Nicholson asked to see Alan and Michelle. "We weren't too worried," Alan recalls. "Dylan was well in every way. So we really weren't expecting anything too serious." However, what they learned in Prof Nicholson's office that day, was very, very serious indeed.

 

"He told us Dylan had Multiple Sulfatase Deficiency (MSD)," Alan says. "We didn't have a clue what that meant. He said it could have an impact on Dylan's life expectancy, and that was absolutely devastating. We were trembling and could hardly breathe."

Alan says MSD is caused by a recessive gene defect, and that both he and Michelle are carriers of that gene. "Dylan is the only known person in Ireland with this condition, and the youngest known person in the world to have MSD at present."

Michelle and Alan Finglas with their son Dylan. Photo: Gerry Mooney.

Currently, Dylan is under the care of metabolic consultant Professor Gregory Pastores and consultant metabolic paediatrician Dr Ellen Crushell at Temple Street; consultant paediatrician Dr Jane Leonard oversees his care at the Central Remedial Clinic, which he attends once a week.

Prof Pastores says: "MSD is a very complicated medical condition, which is caused by a defect in the SUMF1 gene. This gene is responsible for promoting the activity of several enzymes that would normally work to cleanse the body of various potentially toxic metabolites.

"Deficiency of SUMF1 leads to a loss of enzyme activity and the build-up of toxic material in different organs of the body, but mainly the brain. If left untreated, this leads to brain degeneration, manifested initially by developmental delay, but which unfortunately is associated with regression [that is, the loss of milestones], loss of interest in surrounding stimuli and ultimately unresponsiveness. Affected children rarely see their 10th birthday."

However, Alan, who now devotes his life entirely to Dylan, has discovered through exhaustive research that scientists in Italy have already identified a cure for MSD. "They managed, using enzyme replacement therapy (ERT), to reverse the effects of MSD in mice," says Alan.

He believes the next step is to fund research to develop treatment for humans with MSD using ERT. And he firmly believes this is totally achievable.

So much so, that Alan has founded the MSD Action Foundation and savingdylan.com. An accountant, a business manager and a scientist, all of whom are volunteers, sit on the board of the Foundation. Their sole aim is to fund research leading to a cure for MSD. Alan believes it will cost about €2m to fund the initial research.

"That would get it to the grant stage," he says. "Once positive data is produced, further funding is likely from the EU and the USA." In order to kick-start the process, Alan, with the help of Mark Quinn, MD of High Wire Film & Television Production Facilities, has produced a heart-melting short clip on YouTube - it's also on savingdylan.com - called This Video Will Save a Little Boy's Life.

They need this video to go viral so that there will be a global response. Alan believes that successful MSD treatment could benefit other conditions, such as Alzheimer's and Parkinson's. In the meantime, he and Michelle continue to battle for a "chance of life" for their son. And perhaps these lyrics, penned by another Dylan, sum up what they feel for their darling boy: "I love you more than ever, more than time and more than love/ I love you more than money and more than the stars above."

 

Finally, the last word goes to Alan: "Please watch and share the video."

For further information or to donate, see savingdylan.com

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