Living in blisters and pain

Assya is almost 15 years old and has junctional epidermolysis bullosa, an autosomal recessively inherited condition. A defect caused by gene mutations within the components of the skin allows tissue to separate and blisters to form whenever there is friction or trauma to an area. In many instances, the blistering is spontaneous.

Each day begins painstakingly unwrapping the bandages that have been protectively enveloping Assyas blisters for the past day. Stuck to the weeping skin beneath, the last layer is stripped off while she grits her teeth. As I try to imagine a well adhered plaster being ripped from a partial thickness burn, Im surprised she makes no sound or mention of pain. Its difficult to imagine what the experience of pain might be for someone who has been experiencing it almost continually since birth, and even Assya finds it hard to find a description that fits:

“I met a man once, who wanted me to try laughing gas for pain relief. He kept questioning me on whether I felt like I had been burnt everywhere, but I just burst out laughing in annoyance. I dont even know what it feels like to be burnt. It hurts, but then it has done since birth so I cant tell you how bad it actually is.”

A simple task like getting into the bath is torturous. A dose of morphine is normally needed before each limb is slowly immersed into the water. It stings like hell. This simple activity of daily living for most people undoubtedly requires much determination and mental power on her part.

Wrapped in soft cotton sheets, Assya must endure the last part of her daily ordeal: bursting each blister, releasing the pus-like watery liquid, before they enlarge and spread. It is this self inflicted painful measure (part of management in the long run), that makes you squeamish even to think about it. I watch, initially horrified, while mother and daughter burst one after another, releasing the swollen bubbles with needles (which I am only used to seeing on the ends of syringes), methodically puncturing her entire body. Later, I cringe to hear her first squeal as her mum attempts to pop one under the small toe.

I watch them in admiration but also with disbelief. Assya willingly tells me more: her first memory is that of her mother and nurses holding her down in an attempt to burst her blisters while she screams and cries in pain, a pain that she describes as being worse than a dog bite. Her father left them because he couldnt bear to live with her. She attends a special school and is working towards GCSEs in eight subjects.

Assya

“I only go to special school because of how I look,” she tells me in a matter of fact way. “It is not because my fingers are a bit twisted or that I am not so agile. I have visited a mainstream school and seen how cruel the children can be; there is a lot of bullying. I have decided to do a course in business studies and hope to start my own business one day.” I wonder what keeps a person like her going without becoming depressed?

The worst experiences are usually social occasions. “A 15 year old cousin saw me at a wedding and she just burst out crying. Her mum came up to my mum and told us to leave and go home as we were upsetting everyone.”

Assya is the only affected member of the family to have lived past a few days. “When my mothers nephew died I was a bit older, and, although at first I was happy that there was someone else like me, when he died I was really angry out of guilt because I felt that it was my fault. Times like meeting John Major and his wife to receive a bravery award have been wonderful experiences. They make me want to live for as long as the Queen Mother did, but then at other times when I cannot cope with the pain I wish I was dead.

“I suppose when you think of skin diseases, eczema, and psoriasis might come to mind. Epidermolysis bullosa is a rare condition but I think any health professional should realise the impact such a disease can have on other people around the patient, more than the patient themselves. I might feel someone is staring at me, but I have realised how shocked they probably are when they first see me. They probably think I am contagious. I have learnt to be thick skinned, at school, on the street, and even in hospital where taking a few steps outside the ward draws so much attention.

Although skin diseases are generally itchy or sore looking, epidermolysis bullosa restricts life in the same ways as any other disability, as you cannot take part in any rough activity, like sports, for fear of more blisters. People can work together to help patients feel at ease with their condition so that they can live a normal life, rather than feel depressed and lonely. I find it so helpful to have seen and talked to others with epidermolysis bullosa. I have always been made to feel important both in hospital and school. I just hope my condition allows people to see how abnormal skin can be the most awful handicap in the world. It is the first thing a person sees. Unlike a problem inside you, no one can hide from their skin.”