As a journalist I am forever being hounded by people who think they have a story to tell. The 21st century obsession with the quick path to fame without achievement is bordering on the ridiculous. Spurred on by a glut of TV shows, from Big Brother through to Pop Idol, for many people reality and fantasy shelter under the same umbrella these days and it has left me increasingly sceptical over whether there really are any credible tales left worth telling.So, when a relative alerted me to a friend of hers she thought I should contact, I wasn’t bowelled over with enthusiasm by her declaration that the woman in question was a “modern day vampire.” Two days later, as I hurried to meet her, visions of bats and crucifixes spinning in my head, I consulted my notes a third time, still not entirely confident I was making the right decision.Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, potentially life-threatening disease of the blood characterised by hemolytic anemia, thrombosis and red urine(link is external) due to breakdown of red blood cells. PNH is the only hemolytic anemia caused by an acquired intrinsic defect in the cell membrane. I entered the coffee bar, looking for the pasty-faced “Goth” I felt sure I would be meeting. To my surprise, the 32 year old Melanie Williamson who greeted me was completely different to all my preconceived expectations. “So you’re a modern day vampire then!” She laughed a little awkwardly, clearly uncomfortable with my brash assessment.“Well – without trivialising the illness – what I’m suffering from does bear similarities to this fictional image but I don’t want to sensationalise a very serious condition. I do have to take blood every two-three weeks and my body is rejuvenated by the transfusion but that’s pretty much where the comparisons end. I can go out in the sun, I’m not afraid of crosses and I love garlic in my food!” “I’m sorry. Perhaps you could educate me with the real story behind PNH,” I said, feeling slightly silly.“Paroxysmal nocturnal hemoglobinuria – to give it its full title – is a misunderstood illness. I didn’t even know I had it for about a year and when it was eventually diagnosed I discovered that there are only two experts on the disease in the UK: one based in London and the other up in Leeds ”. “I first remember feeling something was wrong around the tail end of 2005. I’ve always been a healthy, active person. I’ve never smoked or drank excessively and I’ve been a vegetarian since I was a child. Added to this, I studied diet and nutrition at university so I have a good understanding of the body.
“It was whilst I was on a skiing trip in France that I noticed I was feeling a little more tired than normal. I vividly recall not even being able to walk up the hill and had a terrible shortness of breath. When I returned home I went to see my doctor and initially he could not find anything wrong with me. “It was only after going back to the GP several times and pressing the matter that I was eventually put in for a series of blood tests and the true nature of my illness became apparent. Soon after that I was referred to a doctor at St George’s in London.”
Certainly my research into the condition did not make for comfortable reading. In real terms, sufferers of PNH find that the red blood cells are weak and therefore destroyed more rapidly than normal. The acute condition which Melanie suffers extends from aplastic anaemia (bone marrow failure) which means she also has a low white cell count, leaving her open to infection. The low platelet count means she is prone to excessive bruising and bleeding and is forced to endure regular blood transfusions to keep her system going.“I have a transfusion every 3 weeks. The process takes between 6 and 9 hours and certainly after the transfusion is complete, I feel rejuvenated and much more buoyant. I am physically lifted. I guess that’s where your “vampire” analogy really comes into play because after the process is complete I am close to my normal, healthy self. “However, as the days pass I do notice the physical decline again and within a week the disease starts to take its toll on my stamina again.”
In the UK , PNH affects about 1 in every 2 million individuals and presently there are 25 people who are registered with the sickness. “Imagine if I had those odds in the lottery! I’d be a millionaire a hundred times over!”
When discussing her illness with the few experts that are familiar with PNH, she found – perhaps predictably – that a definitive answer to some of her concerns was not easily come by.“Doctors are really reluctant to answer specific questions on the illness. A lot of research tends to suggest that in about 80% of cases, the disease is terminal within 10 years. Of the remaining 20%, half actually do cure themselves naturally whilst the other 10% can be completely healed via a bone marrow transplant. That’s what I am hoping for. I think I have found a perfect donor match with a relative and we are just in the process of preliminary investigations.”
For Melanie, PNH has brought about a huge number of challenges on a daily basis and I was impressed by her maturity in dealing with many of these.“Life for me has been all about accepting enforced changes. I didn’t have a choice in any of this and sometimes when I have a quiet moment I still can’t believe what has happened. My illness has robbed me of a lot of the wonderful things I used to enjoy. My active existence is now a thing of the past. Skiing, running and the gym have been replaced by regular walking and a brief jog now and again on the treadmill. “Another sacrifice for the moment is children. Pregnancy and PNH don’t sit well together and I’ve been told the strain of carrying a baby would be too much for me. That doesn’t mean I wouldn’t like to have them one day, mind you!”
For Melanie, the future is a different place to the one many of us look forward to. “I don’t dream about winning the lottery or having fantastic holidays away in exotic places. My hopes are simple ones. “I take each day as it comes and I have learnt to enjoy my time. I don’t live for tomorrow because tomorrow is a luxury I can’t afford at the moment. Right now, the immediacy of life is my driving force. Realising you suddenly have no idea of when it might all be taken away from you changes your perspective some what. “I keep myself busy and I have thrown myself into a variety of projects. I am in the process of completing a book about my experiences, which I hope to have published by the start of next year.” When I came away from the interview with Melanie I did so with an odd mix of celebration and guilt.
Here was a woman in the prime of her life who was facing a significant challenge with a maturity and courage that was truly inspiring. The hurdles she has to regularly leap dwarf all the mundane problems so many of us gripe about by the water cooler or bitch over during a coffee morning with our friends.
Most of us are fortunate in that we can change our outlook because we are essentially in control of our lives. For Melanie Williamson there is only the reality of her situation and she tackles it without complaint. I was impressed by her optimistic spirit and passionate nature and perversely, she made me realise just how lucky the majority of us really are in this world.
So, next time you want to moan about the state of the English weather remember this parting comment that Melanie made to me as we headed out into the drizzle of a late February afternoon.“It’s raining again! I used to hate the rain. But now even the most mundane of events is an endorsement of life. For me, beyond money, love and career, that sense of life is the greatest feeling of all.”
And in that moment I understood exactly what she meant.