Celebrity Status

To catch you all up I’d just been diagnosed with Leber Hereditary Optic Neuropathy (LHON) mutation 11778. For about eight weeks I was on permanent rotation in and out of military hospitals and the Eye and Ear. Apart from the fact I’d now got blurry vision and chronic fatigue, I really had no idea what it all meant. Apart from getting an introduction to the Irish Health Service and the Army Medical Corps, I still as of yet, had not been given as much as a paracetamol. But to make me feel better doctors kept telling me ‘you have a very rare condition’. Yeahie the best news I’ve ever been give. Not sure if, ‘hi I’ve a very rare condition,’ would work as a chat up line.

On my last visit to the Eye and Ear I’d been told I’d be completely blind within the next month. So now what? The prospect of not being able to see a thing within the next month wasn’t exactly all that appealing. Reporting back to Commandant Collins, in the Medical Aid Post in Baldonnel, I informed him of the prognosis of being totally blind within a month. ‘It was ok,’ I explained, ‘we could get an appointment with a neurologist in six weeks’. As you can imagine neither Commandant Collins or Sister Bomfield knew what to say. If they were finding it hard to process all the information, you can only imagine how numb my brain was.

At the same time my father was doing work in a Highfield Hospital in Santry, Dublin. There he spoke to his friend Dr. Dennis and explained the situation. Within minutes Dr. Denis was on the phone to his neurologist friend and neurologist colleague Dr. Staunton. The next day we met the consultant in his private rooms in Beaumont Hospital in Dublin.  I do recall him asking every question you could think of; diet, exercise, what treatment the Eye and Ear had given me, family history, when did my sight start going? The latter was an interesting one. When I really thought about it there had been little signs (my right eye to be exact) going back around four months. Things like trying to focus reading a book. My left eye had obviously compensated for the right eye. As he explained he had no cure but something had to be done to try and stop the deterioration. The next morning, I was in Beaumont Hospital. Staunton had taken me in as his private patient, publicly. When we arrived to check in this did not go down well with the lady in admissions, who wanted us to go on the public waiting list. Dr. Staunton intervened, as time was against us.

Well I went from not been given a paracetamol, to being drip fed a cocktail of god knows what drugs. The consultant explained he was pumping me full of steroids and other medication I can’t remember the names of, in an attempt to stop the deterioration. Every test that the Eye and Ear had done, I had to go through again. The eye tests are a pain. You have to stick you head in a head clamp. Dye is put in your eyes. Then you’re asked not to blink as a doctor looks in your eye with a magnifying device with a light that literally ‘blinds’ you. Another MRI, and more blood tests like they are going out of fashion. I could’ve made a fortune if I was selling it.

Having a ‘rare condition’ had its perks. I was like a celebrity. I had a room to myself with an ensuite and a TV – for all the good it did. (The TV was no more than a 12” screen and mounted on a stand in the top corner of the room. To me it was no more than a radio.) I wasn’t allowed walk anywhere. A porter wheeled me around in a wheelchair with me giving the royal wave to passers-by. The medical profession I found were very serious indeed and didn’t’ seem to appreciate a patient taking the piss.

Celebrity status afforded me every day a rotation of neurologists coming to look in my eyes to see what LHON 11778 looked like. When you think about, they got to see what LHON looks like, I still have no idea.

One trip took me to a meeting room full of white jacketed specialists and student doctors. The contrast to a military hospital was amusing. I was in a sea of white rather than a sea of green. I was 18 and naturally I was having fun getting sympathy of lady student doctors and nurses. After a while though, the novelty of signing autographs wore off.  ‘Can I look in your eyes,’ from pretty student doctors just doesn’t have the same appeal after day seven. There’s only so much poking an eyeball can take.

Thinking of the day I had the lumbar puncture (spinal tap) done still makes me shiver. For those who have not had the pleasure it is a procedure in which fluid is taken from the spine in the lower back through a hollow needle, usually done for diagnostic purposes.

It all happened in seconds, but it felt like hours. First the anaesthetic. Then the hollow needle pierces the skin in the lower back. The feel of cold metal etching the bone of the spine was like someone scrapping their fingers on a chalkboard. The doctor then punches through with a load crack. ‘Good god the pain’. Then came the relentless migraine. It lasted for around ten days. Every second, every minute, every hour, constant pounding resulted in days of no sleep. Believe me when I tell you, I broke. There were tears and lots of them. This, along with the cocktail of steroids and god knows what else, had left me extremely weak. I do recall a kind nurse on night shift keeping me company on those sleepless nights.

My mother remembers me saying to her that I shouldn’t be in hospital, as there were really sick people in the ward and I wasn’t sick. There really was. A few patients had brain injuries, Motor Neurone Disease, chronic pains; a whole list of conditions I’d never heard of before. You got to know them all. ‘What test you have done today?’ Was usually how every conversation started.

The common room at the time was also a smoking room. Now I’m not sure what it’s like now, but then in the hospital you could only open a window by a crack. They had stoppers on them to prevent patients jumping out. So the common room was literally a smoking room. You could smell it down the ward. As you got closer you were met by seeping smoke coming through the cracks of the door. Once stepping inside, visibility was almost zero. An older man, who was almost immobile and permanent fixture in the room, had one cigarette in his mouth and another cigarette in the ash tray. The idea was so he could just lean over and with his old cigarette, light the other one. It went on like this all day.

Everyday friends and family came in to see me. I demanded food and lots of it. Whatever effect the steroids were having on my eyes I couldn’t tell, but I wanted to eat everything around me. For some reason oranges were king. The guys from my class in the Air Corps were given passes to come in every day. It was not long before the locks on the windows were off and the devise requiring 50p for an hours TV was dismantled. The matron was not impressed. ‘Nothing to see here,’ she was told.

So after nearly two weeks I was to be discharged. The consultant had done all he could do. Whatever cocktail he had given me seemed to have stabilised the deterioration. And it has remained so ever since. I went into the hospital though lean and fit. I left looking like the Michelin Man, zero energy, and covered all over from the top of my head to my little toes in acne. It was gross. So much for celebrity status.

One thing I did learn from this experience was if I was on my own and had to rely on the public system I’d be completely blind now. So the lesson is if you are going to get sick make sure you have the best family and friends or else have one hell of an insurance policy.