'Magical' drive-by for boy, 4, with rare disorder

Louie was just four months old when he was diagnosed with XIAP deficiency

The mother of a four-year-old boy with a rare genetic disorder has described her shock at the community response to an event designed to make him feel "special".

Motorbikes, fire engines, police cars and tractors are among the vehicles set to take part in a drive past for Louie, from Bridgwater, later.

He is just one of 100 people diagnosed with XIAP deficiency, which can cause a number of complications including an increased risk of developing infections.

His mum Shannon, 29, has organised the event ahead of her family having to isolate in preparation for Louie's second bone marrow transplant this autumn.

"Never in a million years did I think it would go this crazy, but it just goes to show [there are] strangers all pulling together to make little boys' day," she said.

Mum Shannon and dad Louie are hoping the event will make Louie and his brothers feel 'special' for the day

XIAP deficiency largely affects males due to the genetic mutation affecting the X chromosome and Louie was just four months old when he began to display symptoms of the condition, including a rash.

It was initially suspected that he had cancer after he was blue-lighted to Bristol Children's Hospital from Musgrove Park in Taunton, Somerset.

But after extensive investigations, specialists diagnosed him with the rare genetic condition and he was transferred to the care of Great Ormond Street Hospital in London.

It was there that he underwent his first bone marrow transplant in August 2023 which, Shannon said, "didn't go very well" and led to Louie developing a brain infection.

"He lost all mobility, all speech," she said. "He had to relearn to walk, talk - everything again. He's still delayed globally and he struggles with speech now."

Louie faces another bone marrow transplant in October - this time with an increased risk of a similar infection despite being closely monitored.

Louie spent months in hospital after his first transplant

"We've got to keep Louis [isolated] throughout September [...] because obviously they don't want him to go in with any type of infection on board," Shannon said.

With this in mind, around three weeks ago she said she had the "wild idea" of setting up a "ride-in" event for Louie and his two older brothers Oscar, 12, and Jenson, eight, whose childhoods have been heavily impacted by what Louie has been through.

"I wanted to do something special for all three boys because they've all had a tough ride the past few years and they all know what's to come in October," Shannon said.

"With Louis obviously being immune compromised we can't really do much as a family," she added. "He's not allowed to go to busy [places] like theme parks, soft plays, literally anything.

"He's mainly been around like family kids and family so just for the two older boys [...] just to have that special day and just to feel a bit normal maybe, to have a bit of a childhood and just included - I think it will just be magical for them all."

Shannon said she hoped her older sons Oscar (right) and Jenson (second right) would enjoy the event too

Vehicles of all sizes and shapes, from classic cars to diggers, have been invited to take part in the event, which will set off from Morganians Rugby Club, along the Kingsdown Estate and finishing at Stogursey Village Hall.

With hundreds of people sharing her Facebook post and offers of attendance from the fire service and police, Shannon said the response "means everything".

"I'm definitely going to be overwhelmed with emotions and the kindness of people," she added.