Joanne Has Rare and Life-limiting Condition: Vascular Ehlers-Danlos
Joanne gets alot of support from her friends and family while struggling with Vascular Ehlers-Danlos.
The 39-year-old occupational therapist had just put children Pete, then two, Alice, three and Will, seven, to bed and was sitting with husband Danny when she began to feel unwell.
“We were just sitting on the sofa talking when I suddenly had chest pains,” she recalls.
“They got so bad Danny called an ambulance.”
Joanne was taken from her home in Llantwit Major to hospital, where she was shocked to be told she’d had a heart attack and needed open-heart surgery.
Doctors were mystified to find a tear in her heart – what later turned out to be a tell-tale sign of the rare and life-limiting condition Vascular Ehlers-Danlos, which usually leaves sufferers with a life expectancy of just 41.
“I was kept in hospital and a few days later I had open-heart surgery and a heart by-pass at the age of 39, just out of the blue,” Joanne says, still shocked by the speed of events.
Discharged from the University Hospital of Wales in Cardiff less than a week later, she was sent home reeling.
“It had all happened so fast. I was just terrified,” she said.
Friends devised a rota to sit with Joanne, gradually giving her the confidence to be alone.
As she slowly got better she was referred to a genetic counsellor, but had to wait 18 months for a final diagnosis as the condition is so rare, affecting around one in 250,000 according to some research.
Joanne then found out the full horror of what she had – the inherited syndrome usually kills people by the age of 41 and there is no treatment or cure.
Despite her fear the young mother knew she must carry on as normally as possible for her three children.
“It was very scary in the first months. The diagnosis was devastating, worse than the original heart attack because by that time I felt better,” she says.
“No-one can tell me what the prognosis is because no-one really knows.”
Determined to create happy memories for their children, Joanne and Danny, a weapons technician at RAF St Athan, decided to take them out of school for a once-in-a-lifetime six-month trip across Europe.
If Joanne’s life was going to be shorter than she’d hoped, she wanted it to be good.
Doctors told her she mustn’t play squash, jump into very cold water or do any activity that exerted her heart too much – but that still left plenty to do and she wanted to swim in warm seas.
In 2006, three years after her heart attack, Joanne, Danny and their children set off on a six-month tour of Europe to a rousing farewell from friends.
“We wanted to build family memories and we did,” says Joanne.
Driving in a people carrier they visited France, Italy, Greece, Austria, Switzerland and their favourite place, Corfu.
Some of Joanne’s most treasured memories are the funniest, like the time a Portaloo in Paris opened while she was using it and the time her and Danny were caught on CCTV in Corfu taking a late-night skinny dip.
The family bumped into singer Aled Jones in EuroDisney, negotiated riots in Athens and stayed in a house which flooded in Italy.
“We were directed by our satnav ‘Kylie’ who constantly got us lost,” laughs Joanne.
“In Florence we drove into the city centre and came out right by the Duomo. Leaving we got lost so we followed a bus and ended up in the bus terminal.
“Corfu was our favourite place. It was so beautiful and the sea was lovely.
“We stayed in houses, static caravans, villas and a traditional stone cottage in Puglia which flooded. There was no water or electricity.”
Taking the children to see sites like the Eiffel Tower, the Sistine Chapel, the Acropolis and the Coliseum, Joanne wanted to soak up as much of life as possible.
Will, now 18 and a pupil at Llantwit Major Comprehensive, Alice, now 14 and Pete, 13, who go to Cowbridge Comprehensive, had a wonderful time and it was such an educational trip it didn’t affect their schooling, recalls their mum.
Seven years on from the trip and 10 since her heart attack, Joanne is still alive, against the odds, and the family still talk about the trip.
“I don’t know what the prognosis is. We take every day as it is. We don’t plan too far ahead,” she explains..
“I never take anything for granted. My condition is a shadow that always hovers over me. It covers everything you do, even holidays.
“This summer we went to Croatia, Danny wouldn’t go to any islands, we had to be on the mainland, near a hospital.”
He was remembering a holiday to Spain a few years back when Joanne had to be flown home ill to hospital.
But making the most out of what might be a shortened life is vital, and staying home isn’t an option for the family.
“Having this condition changes the way you approach things,” she said.
“You don’t get het up about minor things and celebrate things while you can. I don’t put things off. I make sure we have holidays and spend time with the children.”
Joanne returned to work as soon as she could after having her heart attack, crediting being an occupational therapist as being part of her identity.
“I work part time and I’m self-employed so I work shorter hours but it’s important for me to work. It’s part of who I am,” she said.
When she reached her 50th birthday earlier this year friends organised a special party at St Donats Arts Centre as Joanne wanted to ‘shout my age from the rooftops’ rather than hide it.
Her joy at her unexpectedly good health took a blow when she lost her brother Mark to the same condition last year at the age of 56.
“He reached 54 with no symptoms and then two-and-a-half years ago he became ill,” she said.
“He had scans and surgery then last October he had a massive rupture to his aorta.”
His loss made Joanne even more determined to make the most of what time she has left. Determined to keep happy memories alive for her family, she’s written a book about their six-month trip which is published this week.
See You In September follows the highs and lows of travelling with three small children as well as how Joanne has come to deal with the condition she is determined won’t stop her enjoying life.”
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