‘Our children were going to die and there was nothing we could do about it’

The last Sunday in November last year was the day that Santa Claus was to turn on the Christmas lights in the town of Roscommon. For the Naughton family, it was a tradition they never missed.

It was an event the three Naughton boys, Archie (then 10) and his brothers, twins George and Isaac (six), were all excited about, asking constantly when would it be time to go.

Half an hour before parents Paula and Padraic were due to put the boys in the car and leave the house, Archie found a leaflet on their kitchen table. Within minutes, he was hysterical.

[ Parents of boy with genetic muscle disease announce death of ‘adored’ son ]

What Archie found by accident was a fundraising leaflet. It had a photograph on it of him, and his twin brothers. What he read was: “Duchenne Muscular Dystrophy is a fatal and catastrophic disease. Currently no treatment or no cure.

"Archie, George and Isaac Naughton are three brothers diagnosed with this genetic disease. Children affected lose ability to walk between the ages of eight and 12 and die from respiratory and/or cardiac failure. Average life expectancy is mid-to-late 20s."

“Archie came to me with the leaflet in his hand and he was hysterical,” Paula recalls. “All he wanted was to be with his father. He didn’t want me. The other two boys were shouting that they wanted to go and see Santa. They had no idea what was going on.”

Grotesque challenge

It was yet another grotesque parenting challenge in Paula and Padraic’s lives that had to be faced.

“Padraic and I decided that I would take the twins to see Santa and he would stay with Archie. So I went with the twins to see the lights switched on. You’re standing in the middle of a community, full of happy, healthy children, and your son is at home breaking his heart, because he’s found out he is going to die prematurely.”

We get so much support from this community but sometimes I feel like we're a family of freaks."

Paula is sitting in the livingroom of the family home in Roscommon, trying to compose herself, as she tells this story. It’s half-term and the three boys are in the kitchen, playing games together.

When I arrived in the house first, I sat with Paula for some time in the big kitchen-conservatory, drinking tea and chatting, while the boys played around us. Archie speaks with a distinct midlands accent, like his father, and his brothers have more of Paula’s English accent.

When I look at the three boys first, nothing unusual registers – only that identical twins George and Isaac (known by his pet name, Teddy), are astonishingly gorgeous, with their shaggy brown hair and strikingly lovely faces. Their older brother, Archie, has a face full of character. After a while, I begin to notice other things about the boys.

I notice how Archie, after playing his allotted half-hour of X-Box, gets up from the sofa. Most 11-year-old boys would spring up in a burst of energy. Archie has to slowly push himself up from the sofa, using his hands, and he wobbles when on his feet.

There are ham sandwiches and hot chocolate on offer, and Archie walks with a rolling gait across the kitchen like a ballet dancer, balancing on the balls of his feet, rather than his heels. The muscles in his calves and hips are already so damaged that the carriage of his entire body has been pushed upwards.

Telling symptom

At one point, Teddy pulls up his trouser-leg to scratch an itch, and I see how this six-year-old has the muscular-looking calves of an adult jockey; a telling symptom of the disease.

George runs to fetch a toy he wants us to play with, and it's an odd, waddling run of poor motor skills; more like the way a toddler just learning to walk moves, not a six-year-old boy.

Paula and Padraic, who is from Westmeath, met in London in 1994, when they were both training as nurses. They became a couple soon after, and married in 2003. Two years later, they came to Ireland, to help with the care of Padraic’s parents.

At that point, Paula was pregnant with Archie. They had not intended staying long in Ireland, but they decided to stay, to offer more long-term support to Padraic’s family. They bought their Roscommon house in 2006.

When Archie was still a toddler, he contracted meningitis, and was brought to Portiuncula Hospital in Ballinasloe. They were warned he might not survive. Paula describes those days of waiting for Archie to emerge from unconsciousness as akin to “standing on the top of a volcano, trying to stop your child from falling in”.

Archie did wake up, and in the joy and relief at his recovery, his parents were not unduly troubled by the fact their son was now limping, and weak on his left side.

They put it down to side-effects of meningitis, and Archie did a course of exercises. But he continued to drag his leg when going upstairs. Meanwhile, his twin brothers were born in 2010.

It was Archie’s teacher who eventually told his parents that it was noticeable in class that their son couldn’t sit up properly, or get up properly. “We were told that, developmentally, he wasn’t the same as the other children.”

They still thought this was a residual problem from his meningitis. Meanwhile, they had noticed that Teddy, the younger twin by 90 minutes, was having trouble sitting up.

Referrals

In the autumn of 2012, both Archie and Teddy saw various pediatricians, receiving a number of referrals. As the referrals continued, and Paula and Padraic started to pick up on the ensuing silence after tests and the body language from the consultants, they began to increasingly worry.

On November 6th 2012, they took Archie and Teddy to a pediatrician in Portiuncula. They also brought George with them. The twins were two, and Archie was seven.

I got this sudden feeling that there was something wrong

They went into the consulting room, and he examined Archie and Teddy. “At the end of it, he said to us, I’m not 100 per cent sure, but I think there is something wrong with their muscles. I need to do a blood test.”

All eyes in the room travelled to George, obliviously playing, the only child not there for tests. “I said to him, the twins are identical, and so we’ll have to test George too. Then I had to get out of that room. I thought I was going to suffocate,” Paula says.

In the intervening two days, before returning for the blood tests, Paula googled phrases such as, “large calves in children” and “inability to walk upstairs properly”. What she found seemed to her like a checklist of the symptoms both Archie and Teddy had displayed. They were symptoms of a condition called Duchenne Muscular Dystrophy, a genetic disease that filled her with so much horror she could not sleep.

On the Thursday, all three boys had blood tests. “We were told we’d be rung in the morning about when to come in for the results.”

That night in the kitchen, when the boys were in bed, Paula told Padraic that she thought their three children had an incurable, fatal muscle-wasting condition. “He leaned back against the sink and said, ‘You’re wrong, you’re really, really wrong.’”

By this point in our conversation, Padraic has come home from work. The two take turns in relating the events of a week they will never forget.

‘Part denial’

“I had not looked up anything on the internet,” he says. “It was part denial, but they do always say, don’t consult the internet for medical matters. For me that night in the kitchen, I was hopeful that the boys would be okay and if they were not, that there would be a treatment for it, so we could fix it. But Paula already had a hunch about what they had.”

At 8.50am the next day, the doctor rang. He told her that the results were back, and that he would see them personally at 2pm that day. Padraic came home from work early, and they drove to Portiuncula with the twins.

At 2pm that November day in 2012, Paula and Padraic were told that all three of their boys had Duchenne Muscular Dystrophy.

“I didn’t really know what it meant,” Padraic says, “but I knew it was something awful, and the condition Paula had told me the previous night that she thought they had. I couldn’t catch my breath. I was in a state of shock. Paula was crying.”

"When he told us what they had, I knew that our children were going to die and there was nothing I or anyone else could do about it,"

Paula says. “Nothing can prepare you for hearing news like that, especially coming from a doctor you trust. Padraic was ashen. I kind of had this out-of-body experience then, where I am looking down on them all in the room from above, as if it wasn’t really happening. I asked the doctor about miracles and he said, you had to believe in hope.”

At this point in the conversation, Paula has to take a break, because she’s crying, and apologising for crying. Then Teddy opens the livingroom door, wondering how much longer his parents are going to be.

“When can you come play with us?” he asks. “Just another little while, Teddy,” Paula replies, her face suddenly transformed into a smiling mask until the door closes again, and the sound of small footsteps can be heard retreating. None of us says anything for a while.

“We walked to the car in complete silence,” Padraic says.

I said, I think we should keep on driving and drive to Galway Bay with the boys and then just keep driving

In their state of utter shock, as Padraic drove them away from the hospital, they found themselves formulating a plan, whereby they would all die together.

Paula says, and Padraic confirms this. “I don’t think that was an irrational thing to say at the time, and anyone who blames us for having those thoughts doesn’t know what it was like to have been given that news.”

Shock and grief

They drove. Then Paula looked back, into the back seat, and realised that in their daze of shock and grief, they had forgotten Archie was not with them; he was still at school. “We only had two boys with us.”

“We couldn’t have left Archie behind with nobody,” Padraic says. “We said to each other, things happen for a reason; Archie is not in the car and we will get through this.”

As the Naughtons explain it, Duchenne Muscular Dystrophy manifests itself gradually from the feet up, as children lose control of their muscles. (The disease is almost exclusively found in boys.) A child will lose their ability to walk between the ages of eight and 12.

Archie already uses a wheelchair for journeys of any distance outside the house, and has had it for two years. In time, all muscle functions cease, and the affected person will need help with everything, including eating, and breathing.

“The ultimate muscle is the heart, and that, in the end, is what almost everyone dies of, heart failure,” Paula says.

For a long time, the Naughtons did not tell anyone the news, beyond their immediate family and staff at Archie’s school, whom they praise for their “phenomenal” support. They also express gratitude for the kindness shown to Archie by his young classmates, who carry his bag for him and generally look out for him.

“Our relationship has become much stronger, because we have to be strong; the boys need us,” Padraic says. “We are on the same mission; to do the best for our boys. We have to be together for the boys, and the boys will need a Mummy and Daddy in their lives, if and when they start to deteriorate.”

Urgency

“Duchenne’s never leaves my head or my heart,” Paula says. “Even on really nice occasions, like weddings or parties, if there are other children there, I will compare our boys to them. I accept that now. I used to think it would dissipate. But as Archie gets worse, the urgency to find a solution for him has only increased, and I can never stop thinking about it.”

The Naughtons tried to keep their lives normal, and private, for as long as possible, but their commitment to try and find treatment or a cure for the boys eventually drove them to go public, as the need to fundraise for the boys’ future also became more obvious.

A trust, Join Our Boys, has been established for Archie, George and Isaac. There are three trustees, and nobody is paid. It has two aims: to fundraise to build the €1.5 million bungalow to facilitate future needs for the boys, who will all eventually be in motorised wheelchairs and need three sets of feeding tubes, hoists and oxygen.

The house will be owned by the trust, to whom it will eventually revert. The architect drawings have been done for free. A site has been bought, but building cannot commence until the full amount is raised, or the necessary materials are donated, to offset costs.

The other aim of the trust is to raise awareness of Duchenne, and to work in collaboration with registered organisations to find a treatment for the disease.

To date, some 250 fundraising events have been held across the country to benefit the family. A sum in excess of €850,000 has been raised, with €189,000 of that donated to research organisations.

Many of the fundraisers have been held locally. “Knowing that the community in Roscommon is supporting us is like having the wind to our backs. We feel nurtured by them,” Padraic says.

It was one of these publicity fundraising leaflets that Archie found on the kitchen table before Christmas. Prior to that, he had unquestioningly gone along with wearing the orange Join Our Boys T-shirts made to publicise the trust, and which he was often photographed in, along with his twin brothers.

He had become accustomed to fundraising events happening, many of which he associated with meeting new people, fun activities and being made a fuss of. He knew his “muscles didn’t work properly, and that we were looking for a cure,” as Paula puts it.

Inconsolable

But until that Sunday last November, Archie didn’t really understand what Join Our Boys was fundamentally about. When Paula returned to the house with the twins, after the visit to Santa and the ceremony for switching on the Christmas lights, 10-year-old Archie remained inconsolable.

“He cried and cried,” Paula says. “When we came home, he kept saying: ‘I’m frightened. I’m frightened. Am I going to die? I didn’t know I was going to die so young.’”

It is an unthinkable, cruel and tortuous situation for any parent of a young child to find themselves in. But in this horrendous scenario, as in all the others they had faced to date, the Naughtons had no choice except to manage the situation as best they could, and to try and deal with Archie’s questions.

“We tried to deflect it, and say that any of us could die at any moment,” Paula says. “We said, as things are at the moment, we don’t have medicine for your muscles, but Mummy and Daddy are doing their best to help find it. He asked us, ‘Will they find the medicine?’and we said, we hope so.”

Since Christmas, Padraic feels that Archie has sometimes been getting scared at night-time. “He knows fear now. He’ll say things like, ‘I can’t run as fast as the other guys anymore, and I can’t kick the ball as high as the others.’ He’s more emotional with me, and needs a cuddle in the night.”

“Knowing our boys have Duchenne’s is not a nightmare, because we can never wake up from it, whereas eventually, you do actually wake up from a nightmare,” Paula says.

[ gofundme ]