When babies with congenital heart defects grow up to live normal lives
One million children a year are born with CHD – 800,000 of those have no access to care
Lucy Allen (16), who has hypoplastic left heart syndrome, addressing the Assembly’s All-Party Group on Congenital Heart Disease hosted by the Children’s Heartbeat Trust at Stormont last November, following the launch of the consultation process on the future of paediatric cardiac services in Northern Ireland. Photograph: Press Eye
Patricia McCann and her son Cormac McCafferty who spent the first three months of his life in hospital, where he had ‘back-to-back’ surgery to widen his aortic arch and repair a hole in his heart. Photograph: Joe O’Shaughnessy
When Patricia McCann first heard that her newborn son had four problems with his heart and needed surgery right away, she had no idea what the future held.
“It is kind of shocking and you don’t know what’s happening,” she says.
She and her husband, Maurice McCafferty, who live in Galway, spent the first three months of baby Cormac’s life at Our Lady’s Hospital for Sick Children in Crumlin, Dublin, where he had “back-to-back” operations to, first, widen his aortic arch, and then to repair a hole in his heart.
Further surgery was needed when he was two and a half, and again at age 14, but now Cormac (18) leads a remarkably normal life.
He is in his first year at NUI Galway studying engineering and, although told to give up competitive sport after his last operation, he has since taken up running, swims and cycles regularly and has completed a couple of triathlons for fun.
“Nothing too competitive, to stay fit more than anything,” he says. He knows his own fitness levels and limits. “I wouldn’t be doing really intense racing; the stuff I’d be running would never put a real strain on the heart.”
While his mother says she “nearly had a heart attack” at the sight of him swimming in a lake in May at the Lough Cutra triathlon in Co Galway, Cormac feels that his congenital heart defect (CHD) does not compromise his life, apart from having to remember to take daily medication, and that he is no different from his peers.
Thanks to advances in medical expertise and technology, an estimated 95 per cent of the one in every 100 children born with heart defects will now live into adulthood – at least in countries such as Ireland.
As European Heart for Children pointed out in an awareness-raising campaign a few years ago, one million children a year worldwide are born with CHD and while 200,000 have access to proper care, 800,000 per year have no hope.
When the Adult Congenital Heart Service started in Dublin’s Mater hospital just more than 10 years ago it was receiving 220 new referrals a year and now it gets more than twice that number, says clinical nurse specialist Rhona Savage.
Some 90 per cent of those are children “transitioning” from Crumlin’s paediatric service after the age of 16.
The transfer to adult services is often more difficult for the parents than the patients, says Savage who sees every patient on every visit.
The first time she makes sure they understand their condition, their medication, what surgery they’ve had and which symptoms they should be concerned about and report in between visits to the clinic.
She spends a lot of time on lifestyle advice with these young adults, covering exercise, diet, alcohol, recreational drugs, smoking and contraception.
If they want to get a tattoo, they need to be on antibiotics to prevent infection and, for similar reasons, they have a CHD card to show dentists when getting dental treatment.
There are 36 different sorts of CHD so recommended exercise, in particular, is tailored to each individual, says Savage. However, advice many young men find most challenging is being warned not to lift more than 30kg in the gym and not to take supplements such as creatine.
Resort to scare tactics
“The young guys laugh you out the door,” says Savage, who, if she hears on subsequent visits that they’re still lifting 100kg, will sometimes resort to scare tactics. But if they are feeling physically very well, they’re less inclined to heed the advice.
However, they begin to engage when there’s “an event”, she remarks. And if they’re feeling absolutely wretched with palpitations and other heart failure symptoms the morning after the night before, the recommendation of no more than two alcoholic drinks and no shots is suddenly likely to make more sense.
Savage and her nursing colleague are at the end of a phone for any query. For instance, she says: “Maybe they have been invited to go paintballing with their mates and wonder if that is acceptable.”
This generation is treading new paths when it comes to lifestyle and long-term services. And next month, Heart Children Ireland is launching a support group in Dublin for young adults aged 18-plus with CHD.
“There is a need to look at what services there are for young adults and what we can do to empower our young adults,” says the chief executive of Heart Children Ireland, Margaret Rogers.
At a preliminary meeting of young adults with CHD, the main issues raised, she says, were ongoing healthcare, peer support and the determination “to thrive, not just survive”. Although Cormac won’t be able to make next month’s gathering, he thinks it is a good idea and intends to be in touch by email.
“It is good to talk to people who have been through something similar,” he acknowledges. In his own social circles, he doesn’t mind people knowing about his condition “but I just don’t like talking about it”.
Cormac has few worries about his future health. “I have been told I will have to have another surgery but don’t even know when – 20 or 25 years from the last one. I don’t even think about that really. It will be when I am in my 40s hopefully.”
His mother is delighted that he is so blasé but, she points out, he, of course, doesn’t remember his early days at Crumlin.
“That’s why I have a different perspective, I am still traumatised,” says Patricia with a laugh. “It’s kind of Russian roulette; you come out the other end and you think ‘I survived that and life is back to normal’.”
Theirs is the sort of story she would have loved to have read when Cormac was first diagnosed and they were in the “see-saw situation”.
“You can actually live a normal life afterwards,” she wants parents of newly diagnosed babies to know. “And you meet wonderful people along the way. I wouldn’t change anything – although Cormac probably would.”
Give up competitive sport
For Ryan Malone of Belfast, it was a much bigger blow being told that he had to give up competitive sport. He was a promising squash player and was on the verge of being sent for trials for the all-Ireland under-14 squad.
It was “not only devastating but aggravating as well”, he says. His first reaction, typical of his competitive nature, was “who is going to take my place?”
Now aged 15, Ryan was born with transposition of vessels in his heart and needed major surgery when one week old.
“They didn’t think he was going to live,” says his mother, Caroline. However, he defied the odds and, for much of his childhood, felt no different from his peers.
Ryan remembers being told he could do anything anybody else could do – except play rugby. That wasn’t a problem, as he didn’t really like rugby. “It was more football I was into.”
But about three and a half years ago, he started to tire very easily, had no energy and fainted a few times. He was diagnosed with sub-aortic stenosis – narrowing of the left ventricle – and needed major surgery again.
“It was out of the blue,” says Ryan, who has struggled with his exclusion from sport and a sense of isolation from friendships being built up on the playing fields. He can’t do PE, only a bit of walking and swimming.
“I took up music – guitar. It’s good. It brought me round a bit. While they were out playing on the pitches, I would play guitar.” He meets people through school and Scouts, but wishes he still had sporting outlets too.
“I think it is good to meet friends from all over the place. I have my own crowd in school but they head out at the weekends. They say you don’t need to be invited but I am one of those people who doesn’t like to just turn up.”
His closest friends “are really protective”, he says. “In a way over-protective, but that’s not a bad thing.” But Ryan doesn’t like having to explain himself or standing out as different.
“I would keep my private life and heart condition quiet but the whole year at school know.”
He doesn’t believe having to cope with CHD has made him more mature than his peers but he thinks he is perhaps more appreciative of life, noticing that other people complain a lot more.
Does he worry about getting sicker? “Not really,” he replies. “I just take one day at a time.”
The Adult CHD and Friends group will meet on February 11th at 7pm in the Carmichael Centre, Dublin 7. For more information see heartchildren.ie or tel 01-8740990.
‘After surgery, I am going to be really sore and tired and will just want to get home’ It’s only a matter of time before Rhianna McAteer (15) needs another operation to repair or replace a valve in her heart. But while her last surgery was in Belfast in 2013, the Co Antrim teenager knows next time it is likely to be in Dublin.
Open-heart surgery has been stopped at the Royal Belfast Hospital for Sick Children and, within 18 months, all youngsters from Northern Ireland requiring operations will go to Our Lady’s Hospital for Sick Children in Crumlin.
“I am not really a fan of that,” says Rhianna, who was born with an atrioventricular septal defect that was not picked up until she was six weeks old. “It’s a really long journey down and, after surgery, I am going to be really sore and tired and will just want to get home but I am going to have to spend two or three hours in the car.”
Her mother, Andrea, says the younger of her two daughters could need surgery within weeks or, if they’re lucky, not for another 10 years. Of the prospect of travelling to Dublin, it is not so much the distance but the practicalities that worry her.
“I would go to hell and back with a can of petrol to fix my child,” she says. “But it is the logistics of everything. You are staying in a hotel and there is the financial burden of keeping two homes.”
After a review concluded that heart surgery at the Belfast hospital was no longer sustainable, Birmingham was first suggested as an alternative before the proposals for an all-island service at Crumlin were agreed by Minister for Health Leo Varadkar and his Northern counterpart, Jim Wells, last October.
Rhianna was one of several teenagers who earlier this month attended a consultation at Stormont on the future of the North’s paediatric cardiac service, in conjunction with Children’s Heartbeat Trust. The charity is fighting to ensure that there will still be a high-quality support service in the city for children with congenital heart defects (CHD).
“In the life of a child or teenager the surgical event is obviously critical – but it is one event every few years,” says the chief executive of Children’s Heartbeat Trust, Sarah Quinlan.
Meanwhile, it is very important to be able to access the rest of their care, medication and support locally.
The transition from children’s to adult services is also an issue, as it is in the Republic. Quinlan would like to see transition clinics established, where a teenager meets his or her future adult service cardiologist in the company of the paediatric cardiologist. She thinks such a handover would be helpful both for the young person and parents.
Looking at Rhianna, who is 5ft 8in tall, you’d never think there is anything wrong with her, says her mother.
“The ironic thing is she loves sport and she can’t do it,” says Andrea. “Rhianna would be active one day and, if she is too active, she is lying down the next day and can’t lift her head. She gets frustrated.”
The teenager agrees: “I get annoyed when I can’t do PE at school. I am usually sitting out on the bench while all my friends are away doing all that fun stuff.” She can do some, for about 15 minutes before having to take a break, and she prefers to do that rather than miss the class altogether.
After dropping horse riding, she took up archery last summer and attends her local Causeway Archers club in Ballymoney every Tuesday.
“I am enjoying that. I can compete because it doesn’t take too much out of me – I still get tired but you can take breaks whenever you want.”
Is she good at it? “I’m all right at it,” she says. “I’m going to stick at it.”
Rhianna doesn’t like talking about her condition to people, unless it is relevant to a situation.
But she’s really enjoying a teenage support group that the Children’s Heartbeat Trust started last August with an overnight weekend event. There were specialist nurses onsite to alleviate parental concern, as some of the participants had never stayed away from home before.
The group has continued since with monthly outings but the charity is not bringing teenagers together to talk about their heart conditions, Quinlan stresses, but to socialise with other young people who understand their issues.
“I’m really enjoying it because nobody pushes you and they don’t slag you. We just hang out,” says Rhianna, who doesn’t think her parents are over-protective.
“They trust me enough and are not saying I can’t do certain things,” she adds. “They want me to know myself better, so I can tell them my limits.”
For more information, see childrensheartbeattrust.org