‘I’ve never known pain like it’

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Over the past two decades Paul Wilkinson has had new hips, ankles, knees, elbows and a shoulder.

In fact, the 68-year-old has been given so many joint replacements he likes to joke that he’s the ‘Bionic Man.’

Paul Wilkinson and his wife Susan with grandchildren Ellie Jenkins (3), Ewan Jenkins (1) and daughter Natasha Jenkins. Picture: Malcolm Wells (14582-4492)

Paul Wilkinson and his wife Susan with grandchildren Ellie Jenkins (3), Ewan Jenkins (1) and daughter Natasha Jenkins. Picture: Malcolm Wells (14582-4492)

Ever one to be upbeat about his circumstances, Paul nevertheless suffers serious pain and problems with rare medical condition alkaptonuria (AKU).

And recently the Cowplain grandfather was given an excruciating and terrifying reminder of the serious nature of AKU.

After dislocating his replacement hip a few weeks ago, Paul was in so much agony that paramedics couldn’t administer strong enough painkillers to move him.

A nearby air ambulance was called in because the doctor on board had the authority to give stronger medication.

Typically, Paul feels embarrassed about it all now – and extremely grateful to the health professionals involved.

But even he can’t play down the extent of the pain.

‘I was screaming, I’ve never known pain like it,’ says Paul.

‘I’m having a job to get my head round that now, it’s really not something I want to go through again.

‘At the time, I just wanted the pain to end and, to be honest, I wasn’t bothered how they did it. I’m embarrassed to say that now but that’s how I felt.’

This is coming from a man who says he has developed a high pain threshold.

After all, Paul has coped with arthritis symptoms (caused by AKU) for more than 20 years and recovered from multiple operations.

Paul was diagnosed with AKU when he was 16.

The genetic condition is extremely rare, only affecting one person in every 250,000 to one million.

People with AKU have trouble breaking down protein and this leads to a build-up of an acid which eats away at cartilage in the joints and other parts of the body.

Patients suffer severe arthritis of the joints, heart valve damage, fractured bones, hearing problems and kidney damage.

The acid causes joints to turn black – and another characteristic of the condition is black urine.

‘It’s sometimes called black urine disease,’ says Paul. ‘It doesn’t cause any problems but it does stain. My mum always knew who it was who hadn’t flushed the toilet.’

Paul will be seeing a consultant about his hip problem and wants to thank everyone who helped him.

And he wants to raise awareness for the condition and the AKU Society, which supports patients and their families.

The retired baker has been taking a drug yet to be licensed (see panel) and says the results are encouraging.

‘It blocks the enzyme that produces this acid,’ he says. ‘I’m not sure how much it’s going to do for me at 68, but it could help prevent young people from ending up in my position.’

Paul’s problems with his joints began in his mid 40s. For various reasons, including his health, he had to give up work as a baker and his mobility problems became increasingly worse.

He has had replacement joints in both ankles, both knees, both hips, both elbows and one shoulder, as well as a foot fusion (when the joints are fused together).

He says having operations doesn’t get any easier but believes people develop higher pain thresholds with ongoing conditions.

He walks, but has a mobility scooter and has spent time in a wheelchair.

Enjoying time with two of his grandchildren, who are visiting from Frome, the dad-of-two and grandad-of-three says his problems are nothing compared with other people’s, including members of his own family.

These two youngsters – Ellie, three, and one-year-old Ewan – are little miracles as Paul’s daughter Natasha was diagnosed with Hodgkin lymphoma twice. Not only is she lucky to be alive but thought she would never have children.

On top of that, Paul’s wife Susan has had breast cancer.

They have all battled through and appreciate every day they have.

‘People must think we’re a family of hypochondriacs but as you can see, we’re really not,’ says Paul smiling.

But he becomes more reflective when he admits. ‘Natasha was so ill. There were times when I wondered how I was going to take this family through this.’

AKU

A national centre of expertise, providing a full assessment for AKU patients, was opened at the Royal Liverpool University Hospital in 2012.

Those with the condition have regular health checks and many are receiving a new treatment called nitisinone.

This prevents the deterioration of joints in patients and is undergoing a European trial.

Currently it is being issued off licence, but it is hoped a licence will be secured in the next few years.

The AKU Society unites people affected by the condition, their families and doctors.

The charity supports patients and their carers, provides information and advice, raises awareness, supports research into the causes, effects and treatments and brings together all the resources needed to find a cure.

For information and to support the charity, visit akusociety.org or call (01223) 322897.