A Lifetime Coping with EPP
Situated in my home town of Hythe, one of the original Cinque Ports, and only 22 miles from the French ports of Calais and Boulogne, I’m glancing at the morning sunshine creeping through my partially closed curtains.
A warm 19degrees and I’m reflecting on how my life has been affected by E.P.P. over the last 59 years.
Managing the effects of E.P.P. are very simple, I have become single minded not to allow others to decide my level of light intolerance , I now say sorry "I’m unable to accompany you today. " with some persuasion friends generally understand.
To the uninitiated Erythropoietic Protoporphyria is a very rare skin condition, ALA and PBG (aminolevulinic acid and porphobiinogen) the raw materials for making haem go through an 8 stage process in which individual enzymes get put together to form HAEM, unfortunatley the ferrochelatase enzyme which gets added to the process is underactive, Leaving EPP patients with light sensitivity to longer ultraviolet wavelengths in UVA and visible light.(quote taken from an introduction to the group of Porphyrias) published by the British Porphyria Association.
There are three skin porphyrias PCT/CEP/EPP. King George 111 1760-1820 was said to suffer from a rare form of Porphyria. Which type has not been established but according to some reports he suffered from bouts of "madness" probably caused by porphyria.
In April, with the fragrance of cut grass gently wafting in the air, I become vigilant by reducing my exposure to the direct sunlight, although my tolerance marginally increases over the course of summer , my skin will tan, not a lovely bronze colour, but as the skin weathers my actual time spent in the sun increases. The seasonal changes cause problems whilst a degree of acclimatization takes place, leaving winter and into spring my skin is very susceptible to burn with little exposure to the warming sun.
By the time autumn leaves are falling and the October nights are drawing in my skin begins to recover from the degradation caused by the bright rays that summertime brings, it can be a lovely time of the year especially if we get an "Indian summer" here in the UK.
My skin begins to recover quite quickly losing the thick leathery appearance which builds up through the summer; I help the process by using moisturizing creams and Petroleum Jelly.
The Early Years
Mum had no idea of my future problems to come and like other mothers would walk me to the park on nice summer afternoons, but alas I would get upset and cry when left in the sun for long periods. My mother had no knowledge of EPP in 1952, it was not until 1972 twenty years later that I was properly diagnosed at St John Hospital London, after a very difficult period of uncertainty throughout my early life.
For many years my parents, especially my mother spent a lot of time visiting the doctors surgery attempting to identify my illness, our GP had little knowledge of my condition, prescribing yellow and blue tablets, to this day I don’t know what they were, my memory tells me they had no beneficial effect. My parents became desperate to identify various substances which would improve my ailment, we tried Yeast tablets and odd creams but nothing seemed to work.
There appeared to be little that could be done to help me apart from staying out of the sun, I therefore remember watching a lot of sport on TV whilst my family would go to the beach and swim.
As a Young Boy
As an infant boy growing up, and having a brother and sister all born within 4 years of each other, I was unaware of the risks of being in the sun, quite naturally I would want to play outdoors, wherever it be in the streets, or the park, and to explore the nearby firing ranges which the army used for target practice.
During the summer, my teachers at school would find it difficult to understand why I could not take part in any outside activities, although eventually with some compromise on both sides I was allowed to take part wearing long sleeve shirts and trousers on school sports days. I remember feeling a bit out of place not being able to conform, but when I competed and won some races all concern for my appearance didn’t seem to matter
any more, I always had a competitive nature which remains to this day.
Many hours were spent wondering near the seashore, picking up live rounds of ammunition left by soldiers on the firing ranges, we would take them home and put each one into a vice, to extract the cordite, this brittle substance when light made quite a nice explosion of fire, unlike today with the introduction of
Health and Safety rules there were no fences to keep us out. The real danger for me out on the firing ranges was the "direct sunlight" often I would become stranded under the heat of the midday sun, without shade to protect me from the fierce rays of sunlight, with the skin tolerance levels reached, I became very pink and swollen, so getting home quickly became very important, the damage had been done by the time I arrived home, I would be in a poor state of high anxiousness, and near to collapse.
We learnt early on that using cold water was a good method of easing the pain and reducing the swelling. I would lay on my bed with the curtains drawn covered in wet towels to my arms, legs, and face, these would have to be constantly refreshed to keep me cool and eventually after very little sleep the pain would pass by the next morning.
After the swelling had subsided I was left with thousands of pin head sized purple spots showing just below the surface of my skin, it would be highly sensitive for two to three days afterwards.
As a consequence virtually every part of my exposed skin has broken open, its only now after a life time of experiences that I fully understand my own restrictions.
My infant and junior years were very painful trying to understand my illness, explaining to my friends and adults that I could not go outdoors and play in the sunshine. Becoming a victim of my own condition was something I wanted to avoid at all cost, because staying indoors is not always the solution, the condition does vary from day to day. Allowing more time outdoors when conditions dictate.
Family holidays as such did not happen, our standard of living was very working class, and father was very strict and would not let us talk at the dinner
table, and if we left anything on our plate, we were told to think of all those starving people in Africa, certainly if we did not finish our main meal we would not get a pudding, food rationing had just ended after the war.
Discipline then was very tough, at junior school we would be given a number of punishments, the slipper a soft shoe would be given in front of the class to the backs of legs, for serious offences we were sent to stand outside the Headmasters office, the cane was the ultimate corporal device at his disposal, but I avoided the cane many times because of my skin condition. I would add my secondary school teachers were not quite so sympathetic. At home whilst being threatened with the belt a good old fashion clip round the ear or worse a good hiding were used to enforce the ethic of the day that children should be seen but not heard. Many times we would be sent to our rooms for being naughty.
My teenage years brought additional pressures, trying to take part in sporting activities such as Cricket and Athletics. Joining the army cadets was another painful experience, when trying to complete my Duke of Edingborough awards scheme, I was brought home after only one day when I became quite ill due to the sunlight, I was mistaken, I thought it would be safe under the protection of a tent, how wrong I was.
On leaving school I entered into a 5 year Plumbing apprenticeship, between April and October I would suffer considerably and consequently required time off work ill, this was very difficult to explain, because at the time, I had not received my full diagnosis, my employer and colleges could not understand why I was having time off for getting sun burnt.
By then I became aware of how important my academic studies were, and began planning a future in plumbing and heating design. With the help of my employer I managed to get a place on a specialist Plumbing design course, and four years later with the necessary qualifications I left Vauxhall College in London to pursue a career in a design office, operating in an urban landscape seeking the shade where I could.
I married in 1972 and we had two children, Joanna 1975, Andrew 1977 both fortunately do not suffer from EPP.
Up until 1975 I had no contact with anybody else suffering with a
similar sun problem, then out of the blue the Daily Mirror featured an article on Thursday 31
July 1975, about Sara Hulbert aged seven years, a little girl entitled "WHY SARA CAN’T PLAY IN THE SUN" I could not believe it at last somebody else who appeared to have my condition, my reaction was to make immediate contact, I wrote a letter to her parents and received a detailed letter back explaining how they as parents had been coping with their daughters care, her medication of Beta Carotene tablets, was the same as my own prescription. Sometime later I stopped taking these because I became aware of the side effects and the huge number of tablets that I was required to take.
1975 the year my daughter Joanna was born, was recorded as one of the hottest summers on record, EPP patients had a very difficult time that year.
Another article appeared in 1980 entitled "A GIRL HIDES FROM THE SUN published by Alex Linsay a reporter for the Sunday Express wrote about a complaint called Hydroa Vacciniforme (Hutchinson Summer Eruption, Hydrao Aestivalis) not to be confused with EPP although the symptoms are quite similar.
I was now beginning to search for other EPP patients that I could communicate with, I felt alone and needed the support of fellow sufferers who could really understand my true difficulties.
I became resolved to the idea that little could be done to improve my condition, so at this point I lost heart with the medical practitioners and basically stopped turning up month after month at the clinic, I decided to just get on with my life the best way I knew how. There was no internet super highway then enabling us to talk with one another. I was on my own not knowing. It’s a vastly different story today, with us wallowing in excruciating self pity, giving every bit of detail imaginable.
Weather patterns here in the UK are very mixed and varied between Autumn and Winter, with the sun strength lost, skin irritation is at a minimum, Spring through Summer are periods of great uncertainty, when walking outside I instinctively seek the shade like a magnet attracts particles of metal, although when I find myself inadvertently in the sunshine, I like the warmth it brings, a pleasant feeling, making life seem healthier. But the cold winds from the east are a major irritator and will start off a reaction, especially during April and May.
Driving the car has always been difficult on hot sunny days, side windows can be screened, but there is no protection through the front windscreen, I therefore adopted a system of rotating my hands off the steering wheel and driving with the aid of both knees. Please do not try my method it’s extremely dangerous.
I don’t think I miss out on to much as a result of EPP, there are some activities I’m not able to take part in , spending long hours sunbathing in Antigua is an example, but with skin cancers on the increase, perhaps it’s not a good idea anyway. But there are many things which are achievable.
I have heard it said that there is a book in all of us, a hidden talent perhaps we did not know existed, well in 1985 aged 33 I walked into a bookshop in the ancient city of Rochester, where once Charles Dickens lived. Why I don’t know, but I decided I wanted to learn how to paint in watercolour, I have been interested in the arts ever since going to see major artists work all over the world. My own art whilst never been exhibited hangs privately in my house in Hythe only accessibly to family and friends. For me its pictures, but the same principle holds good.
Recent reports of the Scenesse clinical trials has reawakened my interest again to the possibility of a cure for EPP, but the process of reengagement with the
Health system can be almost as painful as having the condition itself. So how does one cope with the personal discomfort, which has plagued me for more than half a century, does spending time in cues travelling hours on trains attending appointments, sitting in medical waiting rooms, having regular blood tests effectively being treated like some pin cushion inspire me, no, not at all. But here in the UK we respond well to emergency situations that require an operation, but our illness has a slow burn type flow to it and hours upon hours can be lost attending appointments that produce little beneficial outcome. I’m grateful for the sacrifices made by those on the trial for the benefit of us all.
I understand at this stage the trial results have not been published and it’s not yet possible to say whether the drug was effective or not. We all hope for a positive outcome and look forward to the day when the drug becomes licensed for general use here in the UK.
My best plans to avoid the sun can still fail, an example of this happened very recently when I visited the new Turner contemporary Gallery in
Margate, on arrival we cued up, the cue was moving nicely in the shade, then we turned the corner into the direct sun shine which was no problem, but unfortunately the security guard stopped the cue just as we were about to enter, I was left waiting in the sun, I became very agitated, I approached
the guard and asked to be let inside, to his credit he agreed and let me pass, but in a short time my skin became quite sore.
The physical damage to skin can be healed by time and carefully applying creams. But emotionally, I get very anxious, grumpy and short tempered, this part of the illness has never really been addressed properly, its a side effect of my condition ignored by all including myself, we can only bury it in the back of our minds, and try and carry on with our lives.
I avoid group activities, such as open air concerts, but will try and attend these events during the early evening when the sun sets.
As a young teenager, waking up after a minor operation to remove a large toe nail, the chap in the bed next to me leant over and said "are you all right" I was being sick the after effects of the anesthetic, I replied "my foot aches" are you ok "not bad" was his reply, he then revealed his whole leg amputation,
Considering the catastrophic loss of a major limp he was remarkably cheerful. You have these moments in life, this was just one of those occasions, when it’s time to shut up and grin and bear it, and just get on with life the best way you know how. But consider for example Hay Fever, with its side effects of a constant running nose, squinting eyes and puffy faces, equally Psoriasis, Dermatitis and Eczema patients have a very poor outlook.
So I conclude I’m quite fortunate really. I’m really lucky, having forged a life that remains happy and contented.
Adversary can sometimes be your friend, by not giving in to your weakness’s you can find that core of inner strength, and above all remain positive, life owes nobody a living, treat each new day as though it be your last, every day is so precious try and enjoy them all. For what it’s worth, that’s my philosophy.
Notes and acknowledgements
My reasons for undertaking this article are to give thanks especially to mum for always being there when I needed her, showing me genuine support and
sympathy throughout my infant years, at a time of real hardship, before the coming of Mobile Phones, the Internet, Twitter, and Facebook, for sure my early years were very dark times in comparison to our modern world. We had very little hope of any medical understanding of my condition.
Medical sciences today have made huge advances leading the field in electronic gadgetry to the benefit of many specialist areas of medicin; these new technologies are quite outstanding.
To make my story accessible to my family, friends and future generations, who after reading my story will have a greater depth of knowledge, into the rather unusually weird problems I have to cope with during the summer time.
Dr Kurt Ayerst Consultant Dermatologist for helping me to understand the important work of the patient trials here in the UK. I now feel encouraged to go forward with more hope. We all look forward to the day when this new drug from Scenesse will be fully approved and therefore licensed for use here in the UK
15th may 2011