I was diagnosed with Raynaud’s 13 years ago, aged 20. There had been a couple of defining moments when I realised my circulation wasn't quite right. I'd been working for a retail company and had the tedious task of working in a cold warehouse, pre Christmas, for sale preparations, marking down the stock. Everyone else seemed to manage with the temperature, except me. My hands were blue, and so numb I couldn't hold my pen. Another time I was on holiday in California - it was 80 odd degrees in the sun but a slight breeze or the air conditioning would send my fingers and toes freezing cold and blue.
Referral
Three years later I was lucky enough to have a GP who had the insight to refer me to a rheumatologist at Addenbrookes in Cambridge, just in case there was something more to my Raynaud's. After blood tests and a capillaroscopy, I was diagnosed with scleroderma. Of course my parents and I had done our internet research and had scared ourselves something silly with what we found, so when the consultant confirmed it, we were devastated. How do you process the fact that you have a chronic debilitating condition that could affect you for the rest of your life, when you're 23 years old and have all to live for? I had to tell my boyfriend, my friends, my colleagues. I wanted them to be aware of what could happen to me but I wanted to protect them too. I didn't want them to be as terrified as I was.
So first I had the standard tests; a heart echo, lung function and chest x-rays. The results of these were all ok, and as far as I was concerned, I really only had the Raynaud's to deal with, so I carried on with life, bought a flat, got a new job, all with numerous fears nagging deep in the back of my mind - what would become of me in the future? How would the disease progress? How will it affect my everyday life?
I was told that every case was different but there were a few paths that the disease generally followed. I was pointed in the direction of the Raynaud's & Scleroderma Association and was told I could meet others in the same boat, but I didn't want to then. I didn't want to see or hear what the condition could potentially do to me - that would make it all too real and scary.
Changes were becoming apparent
Two years later, aged 25, changes in me must have become apparent. The company I worked for were keen to have Health & Safety and the ergonomics team look after me. My fingers began to change, stiffen - and odd things began to happen, silly injuries and other tell tale signs that only made sense years later. Not willing to face any of it, I decided 'the disease wouldn't beat me’ and I went off travelling the world with 2 of my best friends! It was only years after my return that my parents, specialists and friends said they knew it wasn't the best idea I'd had but they knew they wouldn't be able to stop me from going.
We managed northern Thailand, Bangkok, Ko Samui, Vietnam and Singapore. It was a fantastic experience, despite my struggles but after 9 weeks I was forced home. I had no energy, my joints and bones ached. I was tired all the time, not hungry, finding it hard to breathe but blaming the humidity. I wasn't sleeping and I was losing weight by the day. I couldn't bend down to put my shoes and socks on, I struggled to lift my arms to brush my hair and I could no longer lift my rucksack. But I didn't want to go home, it felt like giving in to the disease, failing, my fears were becoming reality. When I got home I was a mess; I only had the energy and mobility to sit on the sofa all day - my joints and muscles hurt, I couldn't get comfortable just sitting or sleeping, I was weak and down to around 7 stone in weight. I could hardly walk or get dressed. After a lot of persuading I gave in and went back to my consultant at Addenbrookes, where I was promptly admitted to hospital. I had tests galore; bloods taken
virtually every day, tests for tropical diseases, bone marrow biopsy, muscle biopsy, liver biopsy, CT scans, a barium meal, endoscopy, to name but a few. No stone was left unturned. Sticky blood was discovered, I was also diagnosed with Lupus, myositis and pernicious anaemia, finally concluding: mixed connective tissue disease. I was in hospital for six weeks.
Medication
I was given intravenous steroids over 3 days to kick start me back into action and a simple walk down the ward corridor to the bathroom became my greatest achievement in months. I started hydrotherapy to help build my muscles and gain strength. I went home with a mountain of medications, a cocktail of steroids, immuno-suppressants, blood thinning pills, pills to help my digestion and supplement drinks. I was issued with a disabled badge and ferried around in a wheelchair. I was back living with my parents, so they could help feed me up, look after me and generally do all they could for me. I began physio for my hands and occupational therapy to provide me with aids to help with everyday life. I was taught how to try and balance my life-style but my recovery didn't happen over night - it was a long slow process and a lot to deal with. My life was never going to be how it once was.
Unfortunately, as I was beginning to get back on my feet, my boyfriend of 8 years, broke up with me. I was devastated. I blamed myself, I blamed my health, I blamed him but do you know what, it could well be the best decision he ever made. It made me more determined than ever, a kind of 'I'll show you' attitude appeared. I wanted my life to be how it was before the diseases. I wanted to prove that I could still go out, have fun, have a drink, go dancing and I did, but at a price.
Stress and exhaustion
My confidence was at an all time low. I used to easily wear myself out trying to 'prove' myself; ending up tired and achy, and ultimately pushing myself to the limits. Stress and exhaustion are not positive factors for an already struggling circulation. Ulcers and sores on my fingers became more common, and so did the hospital stays for intravenous iloprost infusions and as some of you may know, iloprost isn't a quick fix either - ulcers and sores can still take months to heal even after this treatment.
I was in hospital at least 3 or 4 times for iloprost that year but in 2005 it was a little less and I managed to start commuting, part-time, back into London for my job. But there was still the ongoing bore of ulcers, sores, infections, endless antibiotics, lancing of nails, bandaging, not being able to use my hands much, and having to go back to my parents for support.
In 2006 age 29, I picked up a muscular-skeletal virus. It hurt to breathe, sleep, sit still or just about do anything for a good 3 weeks! I couldn't sneeze, yawn, or have a good ole' hearty laugh without sharp shooting pains in my chest. Later in the year, with little improvement, there was concern it could be the beginning of pulmonary hypertension, so I was admitted to Papworth Hospital in Cambridge for more tests and iloprost. Thankfully it was just pneumonia and slight fibrosis of the lungs. But since then it's been my chest that is my tell-tale sign for over doing it. When I get tired and run down, my breathing becomes shallow and my chest gets tight and uncomfortable.
It was around this time that the RSA had a regional meeting at a local hospital in Essex and I was introduced to the Broomfield Scleroderma Clinic. I could finally have my iloprost infusions close to home - making the hospital stays so much more bearable because it was easier for people to visit.
In 2008 I had a particularly nasty ulcer on the forefinger of my left hand. I'd already had 2 doses of iloprost that year but it hadn't seemed to help. It got worse, and went black and the pain was horrific, to the point where it was affecting my sleep and concentration. I was admitted to hospital again. After several more weeks of excruciating pain and the blackness increasing, I was told there was nothing more the local hospital could do. I was at an all time low again, not being able to fend for myself, spaced out on pain killers, fearing that I may lose half my finger, whether it be from amputation or just rotting away. The worries mounted up; would it happen to other fingers too at a later stage? What was to become of my hands?
Feeling brighter
I was sent to the Royal Free to see Prof Denton and his team, where I was taken in for 11 days and a new plan of action was formed; more iloprost, more antibiotics, a stint on statins and a stint on Viagra - which has of course, caused untold amounts of laughs. I was also referred to have an operation called a digital sympathectomy on my hand to take away the tiny nerves endings at the base of my finger, so the blood vessels would remain open. Finally I started to feel brighter, things were being done again, there was a little bit of hope, so I grabbed it!
A while after the operation and another dose of iloprost, the blackness scabbed and began to come away. Now I would get the answers to my fears. What would be left under the scab? Would it be hollow? Would I lose my nail forever? How grim would it look? Three months after the operation the scab came away and I was pleasantly surprised with what was left. It's not unattractive, just a little shorter, bent and got an odd looking nail growing on it.
What now?
This was the hard part but it's been a bit like therapy for me - reminding myself that these conditions affect me every day and are never far from the forefront of my mind. It doesn't consume me because I don't let it - I find something else to do, busy myself with friends and family, find myself a project to work on but there's no getting away from the fact that with my now wonky misshapen stiff fingers, most simple things have become a bit of a chore.
There are plenty of gadgets available to help with buttons, undoing jars, chopping and grating food, getting plugs out of sockets but there are many other simple things that are taken for granted - trying to use the latest touch screen technology, trying to grip coins and push them into the car park machines, trying to flush the loo using those push button flushes - the circular ones you have to push inwards or downwards - who invented them? I struggle filling the car with petrol, because I can't squeeze the pump and as for putting the butterfly backs on my earrings, that can take minutes rather than seconds, providing I can hang on to them at all. Trying to get a cash card out of the hole in the wall can also be tricky, with or without gloves on. As for reaching into my bag and trying to avoid knocking my overly sensitive fingers with anything hard or sharp - that's impossible! I could go on forever with examples. We learn to adjust, though it takes time and can be frustrating but we do it because we have to. I always find it amazing that I can still type reasonably fast.
Every one of my fingers has had an ulcer at some point but where those fingers can't take the touch of a key for weeks on end, another one seems to take its place.
Asking for help
I feel like I'm slow at everything now. Getting dressed, going to the loo, eating my dinner. I have got a lot better at asking for help; asking strangers to open a bottle of drink for me and even to the point where I've asked a taxi driver to undo the back of my dress when dropping me home after a wedding - I'd have had to sleep in it otherwise! My friends will automatically take my bags from me, and are ready to do up the fly and button of my jeans when necessary. But they still call me a nightmare because I am a little too independent for my own good at times - they would much rather help than see me struggle but I like to struggle and then ask if I have to.
So what have I learned?
Don't hide away from these conditions, talk to people, be honest and tell them how you feel. I always thought that by expressing the way I felt was moaning but you know what, we have a right to say how it is. Explaining what the diseases do and how they make you feel will help people to understand.
They may never fully comprehend what I go through as I open the fridge door or prepare to step inside a supermarket. Or how frustrating it can be just trying to put on a pair of tights but unless we actually explain when we're asked how we are, or what's happening, how will they ever know? I bet we've all used the standard “I'm fine” line, which I'd say is perfectly OK to say to strangers but not to friends and family. Sometimes it helps to be honest, to explain if you're not fine and tell them how you feel.
Admitting you need help in any way or seeking out an 'outsider' to talk to, like a counsellor or psychotherapist, is not a sign of weakness, it's a sign of strength and courage. If talking doesn't completely help and you need anti-depressants to see you out of a bad patch, then there's no shame in that either. In my experience, seeing my psychotherapist is the best thing I've done and I've tried all sorts of things in the past - crystal healing, yoga, acupuncture, flower power, reflexology, etc. but there's nothing like telling someone your fears to make you realise that you are human and some of your thoughts are only natural.
I recognise now that everyone's problems are their own and it doesn't matter whether you think yours are big or small compared to anyone else's, they're yours and you are entitled to feel exactly how you feel about them.
For a long time I thought Raynaud’s and scleroderma defined me, but it doesn't. There's so much more to me than these conditions. OK I don't ski anymore, not because of the cold, there's solutions for that, but for fear of falling and further damaging my hands. I don't go on bike rides as I don't wish to risk death by not being able to squeeze the brakes. I don't play tennis/badminton anymore without the risk of thwarting my opponent with the racket when it slides from my dodgy grasp. But I can drive, I go to work - I'm lucky that I can work from home when I need to. I've found I'm quite good at baking cakes.
It's not easy and can take forever but where there's a will there's a way. I can still go out and party with my friends, I still go on holidays, live my life to the fullest I can and of course there's always something around the corner to test me but I'll deal with that when it comes to it.
Relationships
As for relationships, old friends and family are my comfort zone. They know my history; I don't need to explain myself to them. I sometimes find it hard meeting new people, trusting them. It's my confidence but I'm getting it back slowly.
But someone new? A new man, even... how will they understand all of this? why would anyone else want my condition to become part of their life? how would they cope with the fact that I often have indigestion at night and sleep on a hundred pillows? what will they think of my awkward caresses. Without talking things through with people, these issues can fester and get so big in your own mind. Negativity breeds more negativity so try seeking out the positives and if you find the courage to share your thoughts with someone, it becomes real and they'll help you to deal with it. Just hearing “why wouldn't anyone want to love you?” can sometimes be just enough. I've never really dwelled on the 'why me?’ - it's just the way it is. I like to think that I was chosen to test my resilience, courage and strength.
Nicki Hunt, Essex
