MY HEALTH EXPERIENCE:Motor neurone disease is a killer. But so is life, says SIMON FITZMAURICE

YOU’RE IN A room in Dublin. A man walks in whom you’ve never met. He starts to talk to you. He asks you if you have any children. You say you do. He tells you not to go home tonight. That when you leave the building you must turn right instead of left, head north instead of south. That you must keep going in an unknown direction, knowing only that you will never see anyone from your life again. The journey he is asking you to take is death.

I come from a different country to you. A different place. There are only a few of us there. My norm is very different to yours. You find it difficult to understand me. And looking at me, you must think me quite strange. I have motor neurone disease (MND).

I am in a bed in the Beacon Hospital. I came in with pneumonia and three days of no sleep and continuous coughing left me exhausted and unable to breathe. Moved into intensive care, I went into respiratory failure, collapsed unconscious and was put on a ventilator.

I now have a tube up my nose and a tube down my throat. One for feeding, one for breathing. Both of which prevent me from speaking. Motor neurone disease prevents me from moving my arms and legs. I communicate with my family through text messages on my phone.

A man has just walked in the door. I have never met him before and he starts to speak to me. He says his name is John Magner, consultant anaesthetist for the ICU. He tells me he has just got off the phone from Prof Orla Hardiman in Beaumont Hospital, after I requested that he ring them to ensure that I was getting the best care for MND in the Beacon.

He tells me that Prof Hardiman has said that they do not advocate ventilation (see panel) in this country for MND patients. That it is time for me to make the hard choice. He tells me that there have only been two cases of invasive home ventilation, but in both cases the people were extremely wealthy.

My mother and my wife start crying in the corner of the room. I look at him but I cannot reply. He looks at me. “This is it now for you. It is time for you to make the hard choice, Simon.” My mother and my wife are now holding each other, sobbing.

While he is looking at me, my life force, my soul, the part of me that feels like every part, is unequivocal. I want to live. It infuses my whole body to such an extent that I feel no fear in the face of this man. We find out two days later that the home ventilator is covered by the HSE, while the home care package needed to run it can be funded between my family and the HSE.

A day later, myself and my father are watching a movie on my laptop. Every movie watched after talking to that man feels like a vindication to me. Every moment lived is a moment lived. We are watching the movie and another man walks in the door whom I have never met. He introduces himself as Ronan Walsh, neurologist. He begins to ask me about the history of my motor neurone disease, despite the fact that I am unable to speak due to the tubes.

My father attempts to fill him in (medical details would not be his strong point). The man quickly gets down to the point: “Why would you want to ventilate? You have motor neurone disease and you are only going to get worse. At the moment you have use of your hands, but the paralysis will grow, will get worse. Why would you want to ventilate?”

For these people the questions, “Why would you want to ventilate?” “Why would you want to live, having motor neurone disease, not being able to move your arms and your legs?”, are rhetorical. But the irony is that they are asking the right questions.

Why would you want to ventilate, why would you want to live? I have many reasons, if they were prepared to listen. But that is not why they are there. I believe they are there because they have made a decision about my standard of living. I think that to them, it is inconceivable that I would want to live. But not for me. For me, it’s not about how long you live, but about how you live.

They ask me why I want to live and the answer is the same as given by “mostly-dead” Westley in The Princess Bride, when replying to the question posed by Miracle Max, “What’s so important? What you got here that’s worth living for?” “Truue loove” is his response. That’s how I feel. Love for my wife. Love for my children. My friends, my family. Love for life in general. My love is undimmed, unbowed, unbroken. I want to live. Is that wrong? What gives a life meaning? What constitutes a meaningful life? What gives one life more value than another? Surely only the individual can hope to grasp the meaning of his or her life. If not asked if they want to live, it negates that meaning.

You have motor neurone disease, why would you want to live? Motor neurone disease is a killer. But so is life. Everybody dies. But just because you die, just because you will die at some point in the future, does that mean you should kill yourself now? For me, they were asking me to take my own life. Or to endorse euthanasia. I refused.

For days they stood around, scratching their heads and wondering what to do with me. In Ireland, MND patients are not routinely ventilated. Instead, patients are sedated, helped, counselled, eased into death. They are not given a choice. Not like in other countries, including the US. Not here.

I have been in this hospital now for four months and I am going home on a home ventilator. I am one of the first people with MND to ever go home ventilated in Ireland with the help of the HSE. The HSE seems to get nothing but bad press, but they have been nothing but exemplary in their support of my move home. The nurses in the Beacon have been inspirational, guiding me through the terror of respiratory failure and the panic of recovery. I have been educated here in the vocation of caring. And the Irish Motor Neurone Disease Association (IMNDA) has been unendingly supportive, helping us with equipment, aids and in every way possible.

For the consultants, whom I have no doubt believed that they were doing the right thing, believed that they were delivering the hard truth, I have nothing against them. But I do wish they would open their eyes. There is no hard truth, only truth on a given day by a given person.

It is people who are hard or soft. And for every moment of hardness, there was an equal moment of kindness from a nurse or a different doctor, and I had many. A moment of kindness to a panicked, terrified patient, to the most vulnerable of people, allows that part of me that feels like every part, to take a breath.

A consultant anaesthetist, Silviu Gligor, who I had gotten to know over the long days here, came into me yesterday. He wanted to say goodbye as I am going home in two days’ time after being almost four months in the hospital. He stood there, wrestling with his emotions, clearly wanting to say something of meaning to me, not just platitudes or farewells. The silence hung about him as he tried to work out what he wanted to say. It was an emotional moment. When he finally did speak, this is what he said: “Go home and teach your children many things.”

I do not speak for all people with motor neurone disease. I only speak for myself. Perhaps others would question whether or not to ventilate. But I believe in being given the choice, not encouraged to follow the status quo.

Change is possible. John Magner, the same consultant who told me I would have to switch off the ventilator, came into me after four months and told me I had come a long way and that he had learned a lot.

I am not a tragedy. I neither want nor need pity. I am full of hope. The word hope and MND do not go together in this country. Hope is not about looking for a cure to a disease. Hope is a way of living. We often think we are entitled to a long and fruitful Coca-Cola life. But life is a privilege, not a right. I feel privileged to be alive. That’s hope.

It’s not important that you know everything about where I come from. About who I am. It’s not important you know everything about motor neurone disease, about the specifics of the disease, about what it’s like to have it. It’s only important that you remember that behind every disease is a person. Remember that and you have everything you need to travel through my country.

MOTOR NEURONE DISEASE: INVASIVE VS NON-INVASIVE VENTILATION

Ventilation can be used to assist the breathing of those with motor neurone disease.

Fidelma Rutledge, a nurse specialist with the Irish Motor Neurone Disease Association, says the association “doesn’t really advocate ventilation by tracheostomy,” also known as invasive ventilation.

If it was a treatment that could cure the illness and people could live a relatively normal life, the association would do absolutely everything in our power to support people, but we are very much into quality of life.”

The association instead advocates non-invasive ventilators, comprising a portable machine and facemask. “It’s not a life-saving mechanism, it’s symptom management. It makes the person comfortable at home . . . but because the condition continues to deteriorate, generally someone would get a chest infection at some stage and that is generally the cause of death,” says Rutledge.

Of invasive ventilation, whereby a tube is inserted in the neck and breathing is controlled mechanically, she says, “generally the person is in hospital because you are looking at 24-hour nursing care which the country is not in a position to offer”.

Rutledge, who has worked with 400 people with the disease, says the option for those invasively ventilated to move home “generally wouldn’t be presented in hospital because the HSE isn’t in a position to fund it”. She says the association knows of just two people with invasive ventilation who are at home.

Prof Orla Hardiman, a consultant neurologist at Beaumont Hospital, says the issues are discussed very openly with all patients. “Once they become aware of the issues of mechanical ventilation, people don’t really want it,” she says.

“It raises important ethical issues later on such as: how do you communicate that you want to come off the ventilator if you can’t communicate any more?”

Prof Hardiman says non-invasive ventilation, available to all MND sufferers in Ireland, can be set up at home and integrated with palliative care services. As its efficacy to support breathing diminishes, “we escalate palliative care involvement and maintain the patient so that they are comfortable and, where we can, control any distress they might have”.

“Advance care direction means discussing all the options with patients and being really, really honest about the implications of ventilation, which in Ireland unfortunately are that if you get [invasive] ventilation, the chances of getting home are vanishingly small,” says Prof Hardiman.

“The HSE doesn’t have the funding to put in the care package that would be required to have someone fully ventilated at home: we just don’t have those resources.”

– JOANNE HUNT

Simon Fitzmaurice

Simon Fitzmaurice is an award-winning writer and film director. His films have screened at film festivals all over the world and won prizes at home and abroad, including Best Short Film at The Cork Film Festival and the Belfast Film Festival (twice), the Grand Jury Prize at the Opalcine Film Festival, Paris, the Jimmy Stewart Memorial Award at the Heartland Film Festival and Jury Award at the Palm Springs International Film Festival. His latest short film, The Sound of People, was selected to screen at the prestigious Sundance Film Festival. Simon holds honours master’s degrees in both Anglo-Irish literature and drama, and film theory and production. His short fiction has been shortlisted for the Hennessy Literary Award and his poetry has appeared in the quarterly publication West 47. Simon is back at home now, talking, eating, drinking, and using his hands to independently drive his powerchair and to write and work again. He is currently working with the Irish Film Board on his first feature length film. He lives in Greystones with his wife Ruth, their three sons, Jack (5), Raife (3) and Arden (2) and their Basset Hound, Pappy.

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