The doctors told us to let Simon die. Instead we tried for a baby... and ended up with twins!': The inspirational film-maker who refused to accept the 'death sentence' of Motor Neurone Disease Simon Fitzmaurice was diagnosed with Motor Neurone Disease in 2008 He eventually ended up on a ventilator and was told to 'agree' to die But instead he and his wife decided to have more children Meanwhile, Simon has been able to continue with his work making films By Jenny Friel Ruth Fitzmaurice watched as the consultant, a man they had never met before, entered the hospital room and made his way towards her husband's bed. Simon, a talented filmmaker and the father of three small boys, lay there with a tube going down his throat, pushing air into his lungs, allowing him to breathe but preventing him from being able to talk. They listened as the medic spelled out in no uncertain terms what he expected them to do. Life: Simon Fitmaurice lives at home with his wife Ruth, and breathes with the help of a ventilator device "He basically announced that this was the end of the road," explains Ruth. That was it, they had done all they could - that he had phoned Simon's own consultant in Beaumont Hospital who agreed that ventilation for Motor Neurone Disease (MND) is not advocated in Ireland. The consultant continued, telling Simon that it was now time for him to make "the hard choice" - to agree to come off the ventilator. In other words, to allow himself to die. He told them that he knew of only two cases of home ventilation in Ireland... but both patients were extremely wealthy. Ruth stood in the corner of the room, clutching on to her mother-in-law, Florence. We were in total shock. I knew he could be heading that way but I didn't think he was at that point already," she says. We just cried, holding on to each other. That Simon had even been put on mechanical ventilation when he was admitted to the Intensive Care Unit with respiratory failure, according to the Fitzmaurices, appears to have been a fluke. "We think they probably did it and then said: "Uh oh, what have we done here?"" says Ruth. And they were stuck with him in the ICU. Despite the consultant's stark and very clear recommendation, Simon refused to grant permission to take him off the machine that was keeping him alive. "Simon's family very much think for themselves, and Simon in particular is a very strong character," smiles Ruth. "He wouldn't be fazed by being told what to do by a doctor, he would question things and say: "Hang on a second." The consultant told us if he stayed on the ventilator that he wouldn't get out of the hospital. With MND [a degenerative condition that destroys the cells that control voluntary muscles and can affect speaking, walking, breathing, swallowing and general movement] it's like, "where do you think this is going? You're only going to get worse. Why would you choose to ventilate? So that's when we decided to fight. Communication: Simon can use his eyes to direct a laser device, allowing him to speech via a machine It took four long months, dozens of meetings and exhausting rounds of tense negotiations but finally a home care plan was put in place and Simon returned to his home in Greystones, Co. Wicklow in March 2011. By this stage the disease - which RTÉ broadcaster Colm Murray passed away from this summer - had left him in a wheelchair, with limited movement and requiring 24-hour nursing care. But shortly after arriving home the couple made another decision that this time even had many of those closest to them scratching their heads. Simon and Ruth, who had three young children, decided to have another baby. "Everyone thought we were a bit mad," laughs Ruth. But we felt in the face of death and with everything that had happened, well, kids are the ultimate opposite of all that, they're life-affirming. Before: Simon and Ruth on their wedding day in 2004 Their lives were certainly affirmed when 20 weeks into Ruth's pregnancy they discovered she was expecting twins. Their youngest son and daughter, Hunter and Sadie, were born in April 2012. Daily life for the Fitzmaurices is now consumed with taking care of their five children: Jack, eight, Raife, six, Arden, four, and the toddler twins. There is also Simon's condition, which has left him only able to move his eyes. A machine allows him to breathe and he is fed through a peg tube. But his mind, as always, continues to work constantly. A writer from the age of 14, he became a multi-award- winning film-maker and his short film, The Sound Of People, was shown at the prestigious Sundance Film Festival in the US just months before his diagnosis in the summer of 2008. Despite his illness, the 39-year-old has completed the script for My Name Is Emily, which he explains is a feature length movie "about a 16-year-old girl and her quest to free her father from a psychiatric institution, helped by the boy who loves her." He began writing it with his hands and finished it typing with his eyes, using a high-tech laser eye device that is connected to an on-screen keyboard. The movie has already received substantial funding from the Irish Film Board and is set to begin filming in early 2014. Simon, from his wheelchair and using the eye device to communicate with his crew, will be the director. This week he, his family and the film production team launched an online crowd funding campaign, which they hope will raise €200,000. This money will help pay for, among other things, specialised equipment and a longer shoot, which will allow Simon to work shorter days. It is an extraordinary challenge that they have set themselves. But one, Ruth says, that is helping them keep their spirits up through some extraordinarily difficult times. She also believes Simon's writing talent has only got better since the onset of MND: he has recently finished the first draft of a book. "It has been quite startling to watch," she says. Everything he says now is pretty much profound. It's all condensed into this amazing creativity. At their comfortable home in Wicklow, the love between Ruth and Simon is clear to see. She turns to him regularly throughout the interview, checking facts and dates, waiting patiently for him to answer via his laser eye device. The words Simon types in are spoken out loud through the computer's voice, which is male with an American accent. It took some getting used to, but the family has learned to accept it and are even quite fond of it now. Family: This photograph shows Simon and Ruth with their eldest two childrenm Raife and Jack Dozens of family photos line the walls of the hallway and kitchen. Many are recent, showing the twins as small babies in the arms of various family members. Others are older, from before Simon was diagnosed. They show an extremely handsome, dark-haired man with bright blue eyes and a broad smile. One wedding photo in the hallway is particularly lovely. "Ah yes," grins Ruth. We call that one the money shot. The couple first met when in college. "But Simon always had a girlfriend," says Ruth. Then a few years later I was at a bus stop outside work. He walked by with his parents and stopped to say hello. I gave him my phone number but he didn't ring me. "She was out of my league," interrupts Simon. "I bumped into him again on the way to work, we began emailing and that was that," says Ruth, 38, who was working for Today FM at the time. The couple were married in 2004 and shortly afterwards moved to a cottage in the north-east of the country. "We lived in a cottage in the middle of nowhere," says Ruth. We ditched in the day jobs, me to do my writing, Simon to do his films, to have children and a big garden. It was all going great - and then Simon got MND. "I ruined it," says Simon. "It wasn't your fault," smiles Ruth gently. You didn't ask to get MND. Ruth was pregnant with their third son when Simon travelled to the Sundance Festival over Christmas 2007 to show his acclaimed short film. While there, he noticed he had a pain in his foot. "There was nothing before that," says Ruth. If anything, he was the kind of guy, when everyone else was struck down with a cold or something, he would be fine, full of energy. He lived life to the max: that was Simon, always. His career was just starting out, the festival was a real wow moment, it was really going somewhere and he won these awards, then it all happened at once, it was crazy. "When he came home with his sore foot I had the typical wifey reaction; "Oh you're being a hypochondriac. It's probably the clutch in your car, it's quite stiff, or the fancy trainers that you're wearing that aren't very practical. I didn't think much of it; we were very busy at the time, we had two little boys and another on the way. My dad is a GP and he sent him to a neurologist when it got a little worse, when he was limping with it. Earlier days: Simon, right, films in the Himalayas In June 2008, the test results all finally came back. "I was around four months pregnant," says Ruth. My dad drove us up that day, he was possibly waiting for bad news. I remember I was in the waiting room, Simon had gone into the doctor on his own. My dad had come in with mud all over his shoes and I was giggling at him dropping clumps all over the carpet. "I was kind of in my own world, then Simon came out and he said: "You better come in here." We went in and the doctor told us he had MND. Like anyone else, I'd heard of Stephen Hawking (the theoretical physicist was diagnosed with MND when he was 21, given two years to live but is now 71) but I didn't really know anything more than that about it. "My neurologist said I had three to four years to live," explains Simon. That was five years ago. The prognosis does not include being on a ventilator. Most people with MND die with respiratory failure. A ventilator enables me to breathe, because my muscles lack the strength to do so. Over the next two years, Simon lost the use of his legs and arms. In the meantime, the family moved to Greystones, where Simon grew up and where his family still lives. "The progression either starts in your limbs or your voice and your lungs," explains Ruth. Simon's was limb onset, his legs and arms went first. Then once your diaphragm goes you can't breathe any more. That's usually the end of the road for most people because in Ireland they don't advocate ventilation. In 2010 Simon developed pneumonia. Exhausted and unable to breathe, his family brought him to their closest hospital, where he went into respiratory failure. For some reason it was decided to intubate him and put him on a ventilator. The consultant, however, later advised him to come off the machine, which came as a huge shock. "We thought we were really far from respiratory failure because Simon was still talking," says Ruth. In my head I thought the progression would be that he would lose his voice first and then his breathing would go. Simon's will to live was, and is still, great. He refused to follow the consultant's advice. "In fairness to the hospital, once they realised we weren't going to turn the ventilator off, they began to help us put together a home plan with the HSE," says Ruth. "The HSE have been absolutely great," says Simon. "Yes, once they committed to the package they have been great," says Ruth. We have a nurse 24 hours, one during the day and a night nurse who sits in the study. We have a night monitor hooked up so they're listening in all night as we sleep, which is obviously a strange way for a couple to live but you do adapt. We have a carer for about nine hours a day because it takes two people to get him up and all of that. 'I've no idea how much it costs. The HSE provide some of it, we provide some and our health insurance provides some. It's a huge operation to keep him going. Ruth is also keen to point out that ventilation is not for every MND patient. ""It's a very personal choice," she says. Simon chose the ventilation; not everyone would. For some people, respiratory failure is the end point of what they want or put up with. Simon will probably never reach that point, he just loves being alive and wants to be with us, and that's his choice. Coming home from the hospital with all the equipment, Ruth admits, was a nerve-wracking prospect. "We were scared; it's like bringing the hospital home with you," she says. But when you saw him in the ICU, with no window, it was breaking him. Over the next year, they got used to the new regime of having 24-hour nursing care in their home and the birth of the twins, who were conceived naturally. "Yes," laughs Ruth. As Simon's dad has said, Simon's equipment still works! "The kids keep us in the moment all the time when this is going on around us," she adds. It gives us a focus and a motivation. Simon interrupts at this point, saying simply in his American accented computer voice: "Best decision ever." "For both our families," agrees Ruth. When you're in the hospital everyone goes into this mode of death approaching, everyone is carrying it. Suddenly these babies came along, this life-affirming thing happened. Everyone was bowled over by it and it became this beacon of hope. So while there have been some huge positives, what have been the hardest things about Simon's condition that Ruth has experienced? "Losing his voice, although we've got used to the American accent now," she says. And the food, I found that very hard. Simon has a peg tube now and he doesn't eat. We were big foodies, a lot of our relationship was based around dinner and going out to eat together. But the family has adapted to their new way of life. "MND is a part of me now," says Simon. It impacts every part of my life and the lives of my family. You live with MND, you don't fight it. You create a new normality and you build a life. The biggest challenge over the last five years is that MND constantly changes the condition of your life. You are building a normality on shifting sands. So adapting becomes part of your existence. In the first years, it is mourning each loss - not adapting. But we have got better at living this life. Allowing room to enjoy, to be happy. His relationship with each of his children has also had to change as he grew weaker. Some, like the eldest Jack, remember how he was before, while the younger ones have never known him to be any different. "Simon is really good at adapting," says Ruth. As each new little person comes along, it gets harder to connect with them. With the others he had more facial movement, he could interact with them. But he still found a way. Sadie will sit on his lap and he puts on this lovely guitar music and puts up photos on his computer screen. She sits there quite happily and will chat to his face and give him a kiss. He finds a way in, he never gives up on that. As for any present prognosis, Ruth says they have learned to do without one. "We don't look too far into the future," she explains. Of course, sometimes you can't help it, but in general I find it pointless. We live very much in the now. "I've just had so many times when the doctors have said: "This is the end of the road," and it hasn't been. There have been so many times that you begin to not believe in statistics. This is what it is, just let's go with it. "Everyone dies," adds Simon. Stephen Hawking and my friend Tim (who also has MND) are 70. "Simon is at what you would term end stage now," says Ruth. The only thing left that really move are his eyes - and whether or not his eyes will be affected is debatable. And there's always a danger of chest infection with the ventilator. There's been a few bad chest infections and we thought he wasn't going to make it through, but he has. The day after we meet, at the launch of his funding campaign Simon tells again how, for as long as he can, he will cling on to the life he has been given. "I want to live," he says. Ruth and my five children are my life. I worry all the time about the effects of my MND on them... I don't know the extent of the cost, I simply know it's worth it. I'm in love with this life and it's worth every hardship to me. Contributions to the campaign raising funds to help Simon direct his film can be made at indiegogo.com/projects/my-name-is-emily. For further information about Simon and to view his previous work, visit mynameisemilythefilm.com