VIDEO: Tony and Mary Heffernan on loss: 'They were immense children... They are still our children, just in a different way'

Tony and Mary Heffernan have watched both their children die of a very rare and incurable disease. Barry Egan spent a day with the People of the Year at their home to hear their extraordinary, heartbreaking story.

Barry Egan

There is a strange silence around The Slieve Mish Mountains this morning - and in a particular house in Keel, county Kerry, that silence, that stillness, is felt most of all.

The last time I was in this house in January there were dinosaurs talking animatedly on the television and stories of the Gruffalo and the owl being told on the couch. In the middle of it all was a very ill five-year-old, blind and partially deaf, being fed by his devoted mother and father. The beautiful young child kicked his legs and waved his arms about. It was a very busy home.

Today, that is all changed.

The five-year-old little boy is dead. He is buried in the graveyard near the back of the house with his little sister, who died here at home on the Dingle Peninsula at 1am on January 18, 2011. Only five years, seven months and 14 days old, Saoirse died of an extremely rare, and incurable, neuro-degenerative condition called Batten's disease too. On May 4 at dawn this year Saoirse's brother Liam, only five himself, succumbed finally to the same disease. They both died in the arms of their loving parents, Tony and Mary Heffernan.

"It is very hard to pick up a coffin," Tony told me 11 months ago. "It is harder to put it down. In the next few months we are to pick up Liam's coffin and put it down ... for the second time." "It is unimaginable," said Mary, "in a few more months he will dead."

"In a few more months, there is going to be nothing. It's not something I like to think about too much. You can't help it though," Tony said. "We have to concentrate on now, to keep as positive as we can." Mary added that she banished sadness from the house. No crying in front of Liam. "Obviously we cry a lot," she said. Her kids' lives have been sad enough, she continued, but Mary didn't want Liam to go to heaven seeing his mummy and daddy sad.

Tony and Mary Heffernan sitting on their son Liam's bed where he passed away in 2014 from Battens Disease. His sister Saoirse died in the room next door from the same disease in 2011.

Liam and Saoirse and Mary and Tony

Today in Kerry, the house is eerily quiet, and empty but for Tony and Mary. Saoirse and Liam's bedrooms are kept as they were when the two children were alive. You can see their toys on the bed, the pictures of Mickey Mouse on the wall, their shoes by the bed. In Liam's bedroom, there is a framed Munster rugby jersey signed by Mick Galwey. It is just after 11am, with the sun streaming in through the windows, Mary and Tony are sitting on their son's bed remembering Liam's last night."He was unconscious, obviously," says Mary. "The two of us just lay on the bed. He was here between us and we just talked to him and we took pictures. We told him some stories. It was really quite calm, quite peaceful."

"I suppose in the few days leading up to that we would have had a lot of family around," says Tony. "Everyone who has been supportive of us got their time to say goodbye really. It was a very emotional time. When we brought him home in the BUMBLEance [the ambulance that Tony and Mary's charity, The Saoirse Foundation: Bee Is For Battens, provides for sick kids] we were told 24 to 74 hours max."

"But Liam had other plans," says Mary. "He stayed with us five days before he passed away."

I ask them what were the last words they said to Liam. "That he was going up to Saoirse," says Mary. "Give Saoirse a big hug from us," says Tony.

It is heartbreaking to walk around the house and see so much evidence of the two children who are no longer here. Downstairs, on the kitchen wall, next to pictures of Liam and Saoirse (on the beach at Inch, playing with their dogs, bouncing on the trampoline, with horse-riding helmets on, etc) is a framed quote. It reads: 'A mother holds her children's hands for a while but she holds their hearts forever.' Over the hall door is another framed quote: 'When the future seems overwhelming, remember it comes one moment at a time.'

Asked how they are coping, Mary and Tony both answer with a pained look. "To be honest, there's good and bad days. There's been a lot of heavy days recently, hasn't there?" Tony says to his wife (they were married in Kenmare on May 4, 2001.) "It's been quite tough of late," he continues, "coming up to Christmas. Christmas was a huge thing in this house. We're going to try and make it a good Christmas, to the best of our ability, this year."

"We'll have our little Santas making a little bit of noise, and Christmas tunes on the stereo, because the house is very quiet otherwise. If you say nothing here for a second, if I stop talking, the house is so silent that it is frightening. We are just trying to get through it," says Tony.

"What else can you do really?" says Mary.

"We'll have a little bit of Christmas cheer in the house," says Tony, "but it's not going to be easy."

"It's never going to be easy," says Mary.

"Last Christmas, we were only told in November that he had six months to live, but he was quite well at Christmas. He was interacting with us - you know, laughing, giving a kiss." "He had a good Christmas, " says Tony.

Mary: "We had a beautiful last Christmas with Liam. Whereas Saoirse's last Christmas was horrendous - because she was really near death, and in a lot of pain. Whereas Liam was clapping his hands, kicking his legs, to the DVD we had on for him. It was lovely. After Liam's death, we were so busy. We just threw ourselves into the charity. Then four weeks after he died we got The Pride Of Ireland award. We decided we'd go to everything.

"It was the polar opposite when Saoirse died," says Mary, "we locked ourselves away; in a misery kind of way. This time, we said if we're invited we'd go to things, the best we can. But there is only so much rope in that, really."

I say that regardless of whether they lock themselves away or go out, Tony and Mary always return to the same reality. "For me, personally," says Mary, "I paint on the smile and put up the wall. If anyone asks me how I am, I say, 'Ah, sure, grand', and maybe change the subject. But it is when I am here on my own, or it is just myself and Tony, that you have your breakdowns.

"We're not robots," she says. "I cry every day. Ten times a day probably, in private. That's not to say if you got caught out somewhere or you saw something, you wouldn't get a bit emotional." Mary explains that three weeks ago she and Tony went for a walk to Muckross Park in Killarney when they came by accident almost to a part of the park that meant something to them. There's a gap in the trees that overlooks Lough Leane. There's also a bench that Tony and Mary used to sit on, with Liam in his buggy. It was at this exact spot that they used to play-act with Liam that this is where the Gruffalo - a character from Julia Donaldson's children's book The Gruffalo - lived in the cave while the owl was in the tree. "We were just walking along then..." says Mary. "Things just catch you unawares. All of a sudden you look up and it all comes back to you."

What came back to Mary and Tony was how Liam loved coming here and spending time with his favourite characters the Gruffalo and the owl. "He would get so excited," says Mary. "This was in the start of November last year, when we got the diagnosis that he had six months to live. We said we would bring him into the park while he can still interact with us.

"At that stage his speech was just kind of sounds, but he turned around when we said we must leave now and say goodbye to the Gruffalo, he said, as clear as day - 'Bye owl.'

"That's the last thing he ever said," recalls Mary with what can only be described as heartbreak etched deep into her face.

Tony: "He never spoke again."

After all Tony and Mary have been through - the unimaginable agony of losing two of their children, one after another from the same rare disease - would they try for another child?

"I'd love to but the answer is no," says Mary initially. "It isn't on my radar at the moment, obviously, because Liam will be gone seven months in a few days. Obviously, it is immensely difficult because you have children with a view to that you'll rear them, they'll grow up and you'll have grandchildren. All that is taken away from you. At the same time, they were immense children. I do feel an immense sense of being blessed having had them.

"Of course, I'm angry and all those other emotions. But I wouldn't change it in the sense that my children were Saoirse and Liam - and they are Saoirse and Liam. They are still my children, just in a different way. I suppose that's how I cope. I know they are gone. I don't live in a world where I think they are still here. But I do talk to them every day. I communicate with them every day.

"It is something we have spoken about at length to be honest," Mary says returning to the subject of whether they would consider having another child. "Tomorrow morning, I would love it. But it's the fear. I'm so afraid. Because I've had two. We haven't had miscarriages. We've had two children. They've both had diseases. They've both died. So the odds are stacked against us . . ."

"It is not a lottery either," explains Tony, "when you look at the odds. Genetics don't lie. When you put myself and Mary together, the odds are 3-1 that a child would have Batten's disease. We are only one of seven couples in the world who have two kids with Batten's disease. It's ultra rare. That's just the luck of the draw, really," says Tony, again, with what can only be described as heartbreak etched deeply into his face.

"The reality is," adds Mary, "we've had two children, they've both had a terminal disease. It's a case of chances really. Would you take the chance? Liam is just gone. Who knows in the future. . ." Batten's disease, explains Tony, is an inherited disorder of the nervous system that usually manifests itself in childhood. In some cases the early signs are subtle, he adds, taking the form of personality and behaviour changes, delayed speech, slow learning, clumsiness or stumbling.

Over time, Tony continues of the disease that took, one by one, his two kids, affected children suffer mental impairment, worsening seizures, "and progressive loss of sight and motor skills. Children become totally disabled and eventually die," says Tony who works in the global LNG (liquid natural gas) shipping industry based out of Oslo, Norway. "I took a year out in 2014 to spend more time with Liam. At the moment I have been putting my energy into helping others via the charity."

In the hallway, hang, side by side in frames, the little Kerry football jerseys of Liam and Saoirse. We go into the kitchen and sit around the table. Mary has a story. On a rare respite from their troubles, they were at the All-Ireland Final at Croke Park on September 21, and, says Mary with an equally rare smile, Kerry won. She just broke down crying. She didn't know why and she felt very awkward about it, as public weeping is not what she does. Then she remembered in the moment of crying the reason. . . "The day before Liam died," she recalls, "we were dressing him up. I put on his Kerry jersey and I said to him: 'Look, buddy, when you go to heaven, you might have a little word with Jesus - we could do with the Sam Maguire down here in Kerry.

"He passed away the next day, and it completely went out of my head. Through any other Kerry match, it never came into my head. Obviously, the moment Kerry won the All-Ireland it did come into my head. Gosh, it just broke me for a few minutes. I really got a sense that Liam was a part of it that day with Kerry winning the All-Ireland."

"There you go - Liam has brought home Sam for us," Tony says meaning the Sam Maguire cup.

"I want Liam to help me win Euro-Millions, so we can build Liam's Lodge and buy five more BUMBLEances, and a helicopter.

"The last five years have been beyond an emotional roller-coaster," says Tony. "We have had extreme lows, which not many people can understand - the loss of all your children opens a wound that can never be forgotten," he adds with a look in his eyes that is hard to put into words. Tony says that he has no choice but to have an outlook which "demands me to focus on the good and not let the negatives feature. I also have had extreme highs. I am still married happily to my best friend, without whom I know I would not survive to be the person I am today," he says, looking at Mary.

"We have received awards this year," he says referring to The Pride Of Ireland Award earlier in the summer, and, last night on RTE, The People Of The Year Award ('For their selfless dedication and commitment to helping the families of children, especially those with rare and complex conditions.') "Do all of these matter?" Tony asks rhetorically as it starts to get dark in the afternoon in Kerry.

"Personally no, not really. They mark something from the past. I personally believe it's not what you have done in life that's the most important, it's what you are going to do. I want to continue to become a better person, I want to fill the lives of the sickest children in Ireland with smiles, and smiles change everything.

"I personally don't smile much, but when I see the BUMBLEance on the road, when I see the development of Liam's Lodge ... " Tony says in reference to the charity he and Mary set up to help kids, and parents, in similar situations as their own in March, 2012, just after Liam was diagnosed with Battens disease ... "and when I see my wife, I smile, knowing that my family are making a difference in this world. My kids, by name alone, are making Ireland a better place," Tony says, meaning The Saoirse Foundation.

I ask what next. "I personally want to spend more time with my wife and helping other sick kids and their families," Tony says. "Our kids have taught me one thing: it's not quantity of life that makes you fulfilled as human, it's quality. They only lived to age five."

Tony is 43 while Mary is 39. "I hope during the remainder of my life, " he says, "I can smile and feel as happy as Saoirse and Liam did during their short lives. You would have had to have met Saoirse and Liam, to know how life should be experienced, now it's time to give that same experience to other sick children in Ireland."

The last thing I see before I leave Tony and Mary in Keel is one of Liam's toys on the couch in the living room. It's his little Gruffalo.Perhaps he left it behind to watch over his mother and father.

To support the BUMBLEance go to www.BUMBLEance.com This Christmas, if you want to help the Heffernans - why not get involved in the #SantaSelfie? Take a photo and post it on social media: text STAR to 50300 to donate €4 and then nominate three friends.

You can watch Barry Egan's interview, produced by Kryan O'Brien, with Tony and Mary Heffernan on independent.ie