21 February 2008
No breakfast and gallons of water later I was sent for an abdominal ultrasound scan and some liver tests. Following the test I understood from the radiologist that there did not appear to be anything abnormal, at least not with the liver.
Between the early-morning tests and the evening consultation with the neurologist I went out and had a cracking lunch of lobster and champagne, at Sheekey’s, one of my favourite restaurants, with my dear old friend Geoffrey Hanscomb. That put me in the right frame of mind to hear the worst, whilst still hoping for the best.
That evening, ‘my lovely’ (Alice, my wife) met me at the Café Rouge for a little more champagne and a light snack before our meeting with the neurologist for the moment of truth. I had previously told him that whatever he had to say, provided I had more than a month to live I intended to go to Australia whatever. I was, of course, putting on a great show of bravado whilst at the same time feeling very apprehensive.
In the event, the Dr. confirmed, what I had feared all along, that all the indications were that I was suffering from some form of Motor Neurone disease (MND) or something allied to it.
This is what I had suspected, having looked up the symptoms on the Internet. I read that MND refers to a group of diseases which affect the motor neurones which are the cells that control voluntary muscle activity such as speaking, walking, breathing and swallowing. The prognosis, in any event, was not good. There are broadly four various types of MND.
Most cases of MND progress quite quickly with noticeable decline over the course of months. Symptoms may present in one region and they will typically spread. MND, it said, is typically fatal within 2-5 years, with around 50% of patients dying within 14 months of diagnosis. One in five patients may survive for five years and one in 10 for 10 years. Â Mortality normally results when control of the diaphragm is impaired and the ability to breath is lost.
The worst of the four forms of MND being ASL – that is when MND is the presence of both upper and lower motor neurones.
The other three are primary natural sclerosis (PLS) ; progressive muscular atrophy (PMA) and bulbar -pseudobulbar palsy – spastic and progressive bulbar palsy -spastic and flaccid, appear to be less serious inasmuch as one might live a little longer. No treatment is currently effective for any of these conditions. Although the use of a drug (Riluzule) , in the case of ALS, can extend the lifespan of a patient by 2-3 months. None of which is, what you might call, terribly encouraging.
Nor was the consultation with the neurologist very encouraging. Although he was unable to definitively confirm the precise form of MND from which I was suffering he was encouraged by the fact that it did not, so far in any rate, seem to be affecting my throat and he therefore suspected that I was suffering from one of the lesser forms. In other words, I wasn’t going to die tomorrow and it may well be years rather than months. At this point he was awaiting the outcome of some of the blood tests which apparently take three weeks for culture the to grow, but he did say that he would be surprised if any of them showed anything significantly different from his initial diagnosis. He did however, want me to see yet another neurologist, one of world leading authorities on MND, Professor Nigel Leigh at Kings College Hospital, for a second opinion.
Funnily enough having received the neurologist’s diagnosis I felt more optimistic, at least I had some idea what was wrong with me and possibly what is going to happen to me but, at this stage, I don’t know when. In the meantime I have been put onto Riluzole for 56 days to see what effect, if any, they have on my liver function.