AN ULVERSTON dad diagnosed with a rare and incurable condition that leaves him at risk of internal decapitation has said a 'big thank you' after receiving a life changing donation.

Phil Clark along with sons Owain, 13, and Steffan, five, and daughter Rhiannon, 10, all have Elhers Danlos Syndrome – a genetic disorder that weakens the collagen in the body.

The condition is thought to affect just one in every 5,000 people.

The 39-year-old's 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.

So with the help of his wife Sallyann, the former Ulverston Victoria High School pupil, has been fundraising £55,000 to pay for a life changing operation in Spain.

After hearing of the family's plight the Lake District Farmers Armstrong Family Charitable Fund has donated £1,000 to help them pay for the operation set for March 15.

The money was raised at the charitable fund's annual celebrity chef events, hosted and sponsored by Clarence House Country Hotel and Restaurant, Dalton.

Phil, of from Birkrigg Common, said: "The support we have received has just been so humbling.

"People who don't even know me have lent their support and donated.

"I just want to say a massive thank you, it means so much."

The family hope the operation, which 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, will give him a better quality of life.

Phil, who continually wears a neck brace, said he was looking forward to the operation, which has a three to four week minimum recovery time, so he could regain a sense of 'normality'.

The dad-of-three, who is still technically employed by Amazon as a team leader, but hasn’t been able to work for the last three years, said: "I am hoping the operation will give me back a sense of normality.

"I was a really active person before I started suffering from EDS.

"I want to get back to exploring the world with my children.

"I also hope that by sharing my story we raise awareness of this condition.

"Not enough is known about it."

Sallyann added: "We hope to turn this situation into something positive for the children.

"It is something we are facing together and we are managing.

"We want them to know they can do anything and support is out there."

The family, who had being living in South Wales returned to Cumbria after struggling to access NHS support.

Sallyann said: "More needs to be understood about this condition.

"We found the consultant is Spain by doing our own research.

"The GP and doctors here have been supportive but they haven't got any other cases like Phil's to refer to.

"They have just told us to go to Spain as that is what Phil needs."

The children, who are under the care of Great Ormond Street Hospital, are at earlier stages of the condition.

The whole family will be flying out to Spain next week to support Phil. To date the fund has reached £39,125.

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

Visit www.nwemail.co.uk/ldf to apply for The Mail and LDF’s monthly £1,000 grant giveaway.