Bristol pub fundraiser to help cousins' condition
Tracy's son Alex, 14, and two of Sarah's children, Ashley, five, and Kai, eight, suffer from the extremely rare – and often deadly – syndrome.
So, they are absolutely delighted friends and relatives are gearing up to raise thousands of pounds for the charity at the White Horse pub in Hambrook on Sunday.
When Alex was three years old doctors discovered he had dilated cardiomyopathy – a serious condition that meant his heart was enlarged and not working properly.
He was put on the waiting list for a heart transplant and, luckily, within two months a donor was found. In July 1998, Alex's life was saved by a transplant at Great Ormond Street hospital.
But tests revealed he did not just have a heart defect. He, and his two cousins, had Barth syndrome, a hereditary disease with many serious symptoms.
Tracy, 37, of Barrs Court, Longwell Green, said: "We take every day, every week and every month as a bonus because we do not know what might be around the corner. Alex has weak muscles and he has his limitations, but 11 years down the line, he is doing very well."
Tracy, who also has a 13-year-old son called Nick, added: "There are only about 130 people in the world who have been diagnosed with Barth syndrome but doctors believe it is under-diagnosed in most cases."
Sarah, of Tenniscourt Road, Kingswood, sensed all was not right with Ashley when he was not feeding, nor putting on weight as a baby. Specialists Bristol Children's Hospital discovered he was hypoglycaemic – another symptom of Barth syndrome, which only affects males. And the family was shocked to discover Kai's regular illnesses were down to a depleted immune system, also caused by the disease.
Even now, Ashley is fed through a tube in his belly button and has to be force-fed water to keep him hydrated.
"It was such a big shock," said Sarah. "We had just found out our son had a chronic illness and we were told about the symptoms Kai was showing. It was pure panic."
On Sunday afternoon, there will be a charity auction, hog roast and raffle at the pub on Bristol Road.
Family friend Dave Baber organised the event with the help of White Horse landlord Les Brooker and Ryan Edwards, the landlord of the Crown Inn, Hambrook. And they have secured some lucrative lots for people to bid for, with Bristol City legend Russell Osman auctioneer for the day.
They include a Manchester United shirt signed by the squad, signed Bristol City, Bristol Rovers and Bristol Rugby shirts, a Steven Gerrard signed montage and a pair of Harlequins rugby player David Strettle's boots.
There are also two golf days and a gold chain worth £500 donated by jeweller Bob Payne and a day's water-skiing for six donated by Dave.
Dave, 46, who runs Cherry Lodge pig farm in Iron Acton with his wife Lisa, said: "We're just trying to raise as much as we can. It's a terrible condition affecting boys that not many people know about."
Sarah, whose other children Corey, 11, and Josie, two, do not have the syndrome, said: "All I can say about Dave Baber is that he is an absolute hero, he's been absolutely fantastic. He's got no other reason to do this other than hearing about what has happened with the boys."
Starting at 4pm, tickets for the event are £2 each on the door and all proceeds will be going to the Barth Syndrome Trust.
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