A woman married to a sufferer of the fatal ‘elephant man’ condition says the disease doesn’t scare her.
Angie Richards, 34, first met Brian, 33, whose bones and organs won’t stop growing, on an online dating website seven years ago.
He has Proteus syndrome, an ultra-rare condition made famous by Joseph Merrick who inspired the film ‘The Elephant Man’.
But she was never put off by his deformities – despite knowing he was only expected to live until he was 18.
Angie Richards, 34, first met Brian, 33, whose bones and organs won’t stop growing, on an online dating website seven years ago
Ms Richards, from Dover in Delaware, US, said: ‘From his dating profile picture, I could tell he had something wrong but didn’t know what it was, nor did I care; disease and handicap, doesn’t scare me.
‘Having a condition was not enough for me to think of Brian as a “no”.
‘I grew up in an environment where you don’t care about what a person looks like, you care about who they are and how they treat others.
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‘Brian is very caring, loving man, early on in our relationship he drove three hours for my daughter’s birthday and even made her a woodwork penguin toy – which is her favourite animal.’
The food inspector added: ‘He can’t change his condition and didn’t choose it to happen, it was just luck of the draw.
‘People asking questions never bothers me, but I have to bite my tongue when they point or whisper.
Mr Richards has Proteus syndrome, an ultra-rare condition made famous by Joseph Merrick who inspired the film ‘The Elephant Man’ (pictured after surgery to stop his seizures)
‘As he grows older, things continue to change and he is gradually getting worse. Despite all of this is, he always has a smile on his face and is always optimistic, he never complains.’
With only 120 documented cases, Mr Richards’ condition led to cruel bullying and all prospective dates were scared off.
Before meeting his wife online in 2010, he doubted he would ever find love.
He added: ‘When people meet me they normally like me for who I am, but don’t like how I look.
‘But with Angie, she cares about the beauty inside not what’s on the outside.
‘Before her I didn’t have a lot of emotions. I had to shut down to protect myself but now I’m learning to be able to talk about problems and tell her when I’m in pain.’
Since getting married, he has become a stay-at-home father to Mrs Richards’ nine-year-old daughter Samantha, helping her with homework and doing the school runs.
But in their current home, he struggles to fit down the corridors with his crutches.
Mrs Richards was never put off by his deformities – despite knowing he was only expected to live until he was 18 (pictured: his feet are one of his worst affected areas)
Now they are approaching their fourth wedding anniversary, but they are aware he could die at any time from the condition
Ms Richards said: ‘From his dating profile picture, I could tell he had something wrong but didn’t know what it was, nor did I care; disease and handicap, doesn’t scare me.’
The family are now hoping to move away to a specially adapted home to suit his disabilities.
Mr Richards was diagnosed with Proteus syndrome when he was three – the chances of being born with the condition are less than one in a million.
His condition was amplified during puberty when his bones grew at an increased rate and required many surgeries.
WHAT IS PROTEUS SYNDROME?
Proteus syndrome, thought to affect only a few hundred people worldwide, results in bone overgrowth.
The word ‘Proteus’ comes from the name of the ancient Greek god of change.
Overgrowth becomes apparent between the ages of 6 and 18 months and gets more severe with age.
In people with Proteus syndrome, the pattern of overgrowth varies greatly but can affect almost any part of the body.
This overgrowth is usually asymmetrical, so corresponding body parts are not affected in the same way.
Some people with Proteus syndrome have neurological abnormalities, including intellectual disability, seizures, and vision loss.
Proteus syndrome results from a mutation in the AKT1 gene.
This genetic change is not inherited from a parent; it arises randomly in one cell during the early stages of development before birth.
The AKT1 gene helps regulate cell growth and division and cell death.
A mutation in this gene disrupts a cell’s ability to regulate its own growth, allowing it to grow and divide abnormally.
More men are affected than women, but it is not known why.
Source: National Institutes of Health
Surgeons tried to destroy the growth plate in his knee because it was growing so large, but it didn’t work.
It regrew and another kneecap formed above it, meaning he now has two kneecaps in one knee, making it difficult for him to bend the joint.
He has undergone 41 surgeries to help his quality of life and stop his bones from growing, including toe amputations, bone removal from his knee and most recently he’s had a lung removed. While he also underwent surgery to reduce his seizures – a common symptom of the condition.
At school, he was bullied and called ‘ugly’ and ‘Frankenstein’s monster’ which led to depression.
However, when he was 18-years-old, his mother encouraged him to embrace who he was.
Mr Richards said: ‘I broke down in the doctor’s office, crying over a massive surgery scar on my knee that looked like a gash from a gang fight.
‘My mum told me, “you’re just going to have to accept this is what happens” and from there I stopped caring about what other people thought.
‘There’s nothing I can do to get rid of it, so I either had to understand what it is and how it works or have a horrible, miserable life fearing what was going to happen.’
He is unable to work, due to his feet swelling to more than double in size and having a second knee-cap grow in the one leg that means he struggles to walk.
Alongside other internal issues, including heart problems, an enlarged spleen and one of his lungs that was removed two years ago.
He continued: ‘My feet are hard and have bumps were the toes used to be, I’ve been unable to wear shoes and the only way I can walk is by treading on the sides of my feet.
‘Then my second kneecap also stops me from being able to bend, we’re hoping that surgery will help to relieve it and the get the tendon over my kneecap.
‘The older I’m getting the more issues I’m having, we don’t understand what’s causing a lot of them but I try to stay positive.’
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He has undergone 41 surgeries to help his quality of life and stop his bones from growing, including toe amputations and bone removal from his knee
A medical scan from 2005 shows how his skull has been mishaped by the condition proteus – aka elephant man condition