I’m the real life Tin Man – it feels like I’m wrapped in barbed wire & I have to perform a Mermaid routine just to move
FOR Nicola Whitehill, life was once filled with nights out, sun-kissed holidays with friends and ambitions to make a name for herself in the legal profession.
But her hopes were turned on their head after a devastating diagnosis at the age of 24.
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Told she was living with systemic sclerosis scleroderma, doctors gave her a bleak prognosis - she could have as little as 15 months to live.
But now, at 51, Nicola has beaten the odds and has lived with the rare autoimmune disease for more than half her life.
Although she says its catastrophic impact means she “exists rather than lives”.
“Before my diagnosis, I’d never even heard of scleroderma,” says Nicola, from Southport, Merseyside. “It all happened so quickly.
“I hadn’t been feeling too well for maybe a week or two, and was generally run-down.
“But I thought it was because I’d been burning the candle at both ends.
“I was working and studying at the same time, I was out a lot and I’d just been on a girls’ holiday to Malia, and on the plane home, all my joints were completely swollen.”
After a trip to the GP, where blood tests for arthritis came back negative, she was referred for further investigations.
“I knew something was wrong,” says Nicola. “I normally had so much energy, and suddenly I was exhausted.
“These tests confirmed I had scleroderma, and I was also diagnosed with Raynaud’s, a condition that makes the blood vessels, mainly in your fingers and toes, narrow.
“When I realised how serious scleroderma was, particularly diffuse systemic sclerosis, the type of the condition I had, I was stunned.”
Scleroderma is a rare, chronic disease of the immune system, blood vessels and connective tissue.
It affects an estimated 19,000 people in the UK, and is four times more common in women than men.
As an autoimmune condition, the immune system becomes overactive and attacks healthy tissue within the body.
My mouth and face have totally changed. I now have a dot-to-dot puzzle on my face with a cat's arse mouth
Nicola Whitehill
For most people, scleroderma causes problems with the skin, including itching and tightness that makes it harder to move the joints, as well as calcinosis, hard lumps under the skin.
People with the condition can also develop digital ulcers, painful sores on their fingers and toes, which must be treated swiftly to prevent infection.
With diffuse systemic sclerosis, the type of scleroderma that Nicola has, internal organs like the heart, oesophagus, kidneys, lungs and digestive system, can be affected too.
The impact of Nicola’s condition has been life-changing.
“It feels like my body has been wrapped in barbed wire then dipped in concrete,” she says.
“My muscles and joints are just so stiff and painful.”
While she qualified as a barrister in 2004, more than six years after her diagnosis, the severity of her symptoms and the side effects of continuous chemotherapy used to suppress her condition soon forced her to step back from the career she loved, aged just 31.
Now, Nicola says she’s all but housebound, only able to go outdoors with the help of a mobility scooter.
And her once-vibrant social life now revolves around medical appointments.
“Every day starts the same way,” she says. “I spend hours on what I call my ‘Mermaid routine’.
“That’s at least three hours where I bathe in liquid paraffin and warm water to get my circulation and joints moving and covering my body in emollient.
“Even that is exhausting, and there are days where it’s all I’m able to do.
“It’s a whole rigmarole, but without it I can barely function.
“For years I’ve said I’m like the real-life Tin Man, the character from the Wizard of Oz.
“It’s like trying to start an old car as it takes me so long to get moving.”
It’s been soul-destroying. That’s the thing with this illness, it feels almost as if it’s out to make you look ugly
Nicola Whitehill
Among the agonising symptoms Nicola endures are painful ulcers on her hands and feet.
She can often have as many as eight fingers bandaged at one time, with gangrene - where a loss of blood supply causes body tissue to die - recurring on one finger.
“Gangrene is very painful and takes so long to heal,” she says.
“If it doesn’t get better, you’re looking at amputation. I’ve been lucky enough to avoid that so far, but it’s still a struggle.
“My hands are in constant pain, not only with the ulcers and intermittent gangrene, but they’re curled and shrunken which means even really simple things like holding a knife and fork or brushing my hair are almost impossible, and really time-consuming.”
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One issue that has come as a huge blow for Nicola in recent years is the loss of most of her top teeth, something that’s not just impacted her ability to speak and eat, but her confidence.
“They started going in 2015 as my gums began to shrink,” she says.
“It’s been soul-destroying. That’s the thing with this illness, it feels almost as if it’s out to make you look ugly.
“My mouth and face have totally changed. I now have a dot-to-dot puzzle on my face with a cat's arse mouth.
“It’s a horrible thing to deal with and I had to wait quite some time before I could get a plate fitted.
“It was a massive knock to my confidence, and now I wear it most of the time because it helps support the shape of my face and the structure of my jaw.”
Scleroderma is the name for a range of conditions that affect your immune system and can cause hard, thickened areas of skin.
Sometimes, they can also cause problems with muscles, bones, internal organs and blood vessels.
The two main types of scleroderma are:
- Localised scleroderma (morphoea) – this mostly affects the skin but some types can also affect tissues under the skin, such as muscle and bone
- Systemic sclerosis – this may affect blood circulation and internal organs as well as the skin
Some types may eventually improve on their own, while others can lead to severe and life-threatening problems.
Although there's no cure for scleroderma, symptoms can usually be managed by a range of different treatments.
Everyone’s experience of scleroderma is different. It depends on what type you have, how severe it is, and what parts of your body are affected.
For most people, scleroderma causes skin changes. These symptoms can include:
- Patches of thick, hard skin that may become discoloured
- Itching
- Tight skin that makes it harder to move your joints
- Hard lumps under your skin
- Tiny blood vessels (spider veins) appearing just beneath your skin, called telangiectasia
- Painful sores on your fingers and toes, called digital ulcers
Normally, the body's immune system fights off any germs that infect the body.
It responds like this to anything in the body it doesn't recognise, and settles down when the infection has been cleared.
It's thought scleroderma occurs because part of the immune system has become overactive and out of control.
This leads to cells in the connective tissue producing too much collagen, causing scarring and thickening of the tissue.
It's not clear why this happens. Certain genes are thought to be involved, and having a close family member with the condition may increase your risk.
But that’s not all. “Because my skin is so sensitive I can’t wear the things I used to love like jeans, nice tops and even nice shoes,” says Nicola.
“I’m constantly in slippers, thick socks or Uggs because my feet are always cold due to the Raynaud’s.
“I’ve also lost the padding on the soles of my feet and have calcinosis in my toes.”
As well as symptoms which are outwardly visible, Nicola suffers muscular and skeletal pain, which means she’s lucky if she gets a single block of four hours’ sleep a night and is always fatigued.
“The disease hits you internally too,” she says.
“It’s affected my gastrointestinal system which means I have terrible acid reflux.
“I’ve got to be so careful with my diet, and almost have to force myself to eat because if I can’t do that, the next step will involve being fitted with a gastric peg, something I really don’t want to do.
“My experience shows just how true the expression ‘health is wealth’ really is.”
However, despite the devastating impact of her condition – one which has left her unable to have children – Nicola does her best to look on the bright side, and push for a brighter future for other people with rare diseases.
She campaigns for much-needed medical research into rare illnesses alongside organisations including Genetic Alliance UK, which advocates on behalf of the 3.5million people living with rare diseases in the UK.
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“In many ways I’ve been lucky,” says Nicola, who raises awareness about her condition on social media with the hashtag #SclerodermaFreeWorld.
“I’m still here after all these years, and not everyone makes it this far.
“I know of people with my condition who’ve had limbs amputated, who carry oxygen tanks because of the effect it’s had on their lungs or have passed away as a result.
“I do all I can to keep myself well, even if that means it sometimes feels like I exist rather than really live.
“I’ve made great friends through my advocacy work, because while we may be small in numbers in terms of each individual illness, rare diseases affect millions of people in the UK.
“We face huge challenges because not enough is known about what we’re going through – or how to make things better.
“After years as a bit of a social butterfly, medical appointments are now about as exciting as it gets, and I have to set aside lots of time on either side of those to make sure I’m fit to go to them and can recover properly afterwards.
“Other than that, I keep myself busy with my two rescue dogs, Ted and Poppy, who I try my very best to take out every day.
“I head out on my mobility scooter, with the two of them running alongside me down at the seafront, and it’s the best part of my day.
“There’s nothing better than feeling the wind in my hair down by the coast and chatting to people when I’m out and about.
“It makes me remember that life’s going on out there in the world – and that I’m still part of it.”
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