AN Ulverston dad and his three children have been diagnosed with an incurable condition that leaves them at risk of internal decapitation.

Phil Clark along with sons Owain, 13, and Steffan, five, as well as daughter Rhiannon, 10, all have Ehlers-Danlos syndrome – an incurable genetic disorder that weakens the collagen in your body.

It is thought to affect one in every 5,000 people.

Phil, a former Ulverston Victoria High School pupil, condition is now so advanced that without life-saving complex surgery, that isn’t available on the NHS, he is at risk of being internally decapitated as the progressively weakened ligaments in his neck could simply snap.

Phil's wife Sallyann said she treasures every moment knowing at any time that simplest of pleasures could cruelly be taken away.

The 38-year-old said: “We have been dealt the cruellest of blows.

"Knowing Phil and our precious children are battling against such a terrible fate is just horrendous.”

Sallyann, a former primary school teacher, from Birkrigg Common, said: “This is our greatest fear. My husband could suddenly die and won’t be here to watch his children grow up and help them cope with the same dreadful condition.”

The family have been left with no choice but to fundraise for the expensive operation in Spain that will cost a minimum of £55,000 and is scheduled for next month.

Up to now they have raised £36,000.

Phil, 39, who is still technically employed by Amazon as a team leader, but hasn’t been able to work for the last three years, said: “It is so hard for us to appeal for help.

"I don’t like asking for money, but it’s our final chance for me to be here for my children, who are going to need so much support as they get older.”

The surgery will fuse Phil’s upper vertebrae together to stabilise and strengthen his neck, as well as remove part of his skull to make way for his brain that is seeping into his neck.

Phil, who is now virtually dependant on a mobility scooter and continually wears a neck brace, added: “I have gone from running up mountains and windsurfing to being left with the most horrendous muscle pain, severe fatigue and fighting for my life.”

Phil’s condition was only diagnosed after their daughter, Rhiannon, began suffering with mobility problems.

Sallyann said: “Although she was a healthy happy toddler, by the time she reached six, Rhiannon, could only walk for a few minutes at a time, suffered from severe joint pain and had no bladder control.”

After several tests, she was diagnosed with the incurable genetic disorder Ehlers-Danlos hypermobility, at Great Ormond Street.

Sallyann said: “We were absolutely devastated. The fact there is no cure and the future looks so daunting for our daughter was just unbearable.” The couple were warned that their daughter’s condition would get more severe with age, especially as she hit puberty due to the change in her hormones which naturally relax ligaments.

Already Rhiannon, who is now home-schooled with her brothers, is constantly in pain and some days is too weak to get out of bed.

Sallyann said: “The positive news was we had some answers as to what was happening to Phil, who by then was also suffering from severe leg pain which he had originally put down to all the running and mountain climbing he did.

“At the same time Owain had started complaining of agonising pain in his knees and severe headaches and later on Steffan began waking in the night screaming in agony.”

The couple were initially warned by specialists that normally 50 per cent of children in a family would inherit the condition.

“We have just been incredibly unlucky that all three of our kids have it,” said Sallyann.

“But we are helping them as much as we can. Research shows the stronger the muscles are, the more support the ligaments have - reducing the symptoms.

“So we encourage them all to use their bikes and chase around the garden – obviously this is more difficult for Rhiannon, as she tires very easily, but she tries her very best."

Sadly for Phil though, his condition is now so severe he has been diagnosed with the added complication of suffering from cranial and cervical instability, as well as Chiari malformation – known side effects of Ehlers-Danlos syndrome.

Consequently the tissue in his neck is unable to support the weight of his head and the ligaments in the base of his skull are too soft to hold his brain in place resulting in a small section falling into his neck.

Without the surgery, his symptoms will become so severe it will lead to paralysis, organ failure and then internal decapitation.

At the moment there are no doctors in the UK performing the surgery on EDS patients, so the couple are fundraising to secure the money to pay for Phil’s operation abroad.

Phil said: “More than anything I want to be here to see my children grow up. The thought of not being around is unbearable.

"They need me and on top of everything else they are facing, I don’t want them to have to lose a parent – no child should ever endure that."

To donate go to https://www.justgiving.com/crowdfunding/phil-clark.

PANEL

- Ehlers-Danlos syndromes (EDS) are a group of rare inherited conditions that affect connective tissue such as tendons, ligaments, blood vessels, internal organs and bones.

- There are 13 types of EDS and most are very rare. It is thought to affect 1 / 5000 people but our belief is this is an under estimation as often so many sufferers are misdiagnosed.

- If a parent has EDS there is a 50/50% chance of any child inheriting the condition