What happens when you run the risk of passing on your heart condition to your child?

Mention of Fabrice Muamba's name makes the hairs stand up on the back of Noirín Nolan's neck. Ten years before his very public and distressing cardiac arrest during an FA Cup match in March 2012, from which he recovered, she was equally lucky to survive a similar near-death experience.

Noirín's last memory of October 7th, 2002, was putting a towel out on the washing line at her home in Limerick and walking with friends towards Pembroke College, where she was sitting a beauty therapy exam. Aged 18 at the time, she had felt unwell for about a week and was taking a prescribed, over-the-counter medicine for gastric upset.

Fellow students have since filled her in on what happened. She had just completed the exam, when she collapsed.

Fortunately, two of her classmates had trained in cardiopulmonary resuscitation (CPR) and they immediately started to work on her – one of them doing chest compressions and breaths while the other counted aloud. When the paramedics arrived, they “shocked” her and got her heart going again.

"If it wasn't for the early intervention of the CPR, it would be a very different story because they kept the oxygen supply going to my brain," she says. "I was out for 19 and a half minutes."

Chances of survival
Every minute without CPR reduces your chances of survival by 10 per cent, explains the medical director of the Irish Heart Foundation (IHF), Dr Angie Brown. Part of the IHF's current "Happy Hearts" appeal (see panel) is to raise funds for its CPR programme, which trains 65,000 people a year in this life-saving skill.

After her cardiac arrest, Noirín spent 10 days in intensive care at Mid-West Regional Hospital in Limerick before being transferred to Cork South Infirmary to have an implantable cardioverter-defibrillator (ICD) fitted. This acts as both pacemaker and defibrillator – it regulates her heart's rhythm if it goes too fast or too slow and kick-starts it if it stops.

She had an inkling at the time, she says, that she had the inherited condition Long QT Syndrome (LQTS) – a disturbance of the heart’s electrical system, which causes problems with its rhythm. “But it wasn’t confirmed until seven years later. It’s in the family and very, very dominant. They had no idea.”

Suddenly all the stories she had from her childhood of fainting – such as after a spelling test or at the altar while serving Mass – were explained for her. While palpitations, black-outs and cardiac arrest are symptoms of LQTS, other affected people may have no symptoms.

An estimated 2,000 people in Ireland have LQTS, based on projections from statistics in other countries, explains Brown. "There are others probably carrying the gene, who may or may not show it. You can have Long QT and have a normal ECG [electrocardiogram]."

There are also cases where LQTS is not inherited but caused by taking medication for some other condition – for example, it may occur with drugs used in treatment of low potassium, she says.

"If you are diagnosed, we search for the gene in you and if we get it, that's good because we check the rest of the family," says Brown. "Sometimes, if we don't find it, it may be because it's something we haven't discovered yet." About 14 different genes associated with the condition have been identified so far.

Cardiac screening
Noirín's immediate family – her three siblings and parents – all went for cardiac screening at Tallaght hospital in Dublin and were then referred for genetic testing.

“In one way we haven’t looked back since,” she says. “It has been brilliant because it is better the devil you know . . .”

Noirín was aware soon after her cardiac arrest that if LQTS was confirmed and if she were to become pregnant, there would be a 50 per cent chance of the baby inheriting the condition. So she was adamant for a long time that “I am not passing on this gene, I am not having any children.”

But shortly before her marriage to Darren Nolan in May 2011, she started to change her mind. “I saw my brother and sisters having their kids and the difference they make.”

She had been going out with Darren before her cardiac arrest, so he had been through it all with her and had told her that he was fine with not having children, if she didn’t want to.

Just over six months after their wedding Noirín was pregnant and classified as “high risk” due to her condition. She had decided to go off her heart medication before that as it is not 100 per cent effective for her type of LQTS and, with an ICD fitted, it was a case of “belt and braces”, as one doctor put it.

“I don’t see the point in pumping something unnecessarily into your body,” she comments. The important thing is to avoid stress and not to take any of the long list of drugs that pose varying degrees of risk to people with LQTS – such as the one she was prescribed the week before her cardiac arrest, as she found out afterwards.

Although the Nolans live in Borrisokane, Co Tipperary, their baby's delivery was planned for Cork University Maternity Hospital, two and a half hours' drive away, as her cardiologist is based there. "If anything went wrong, he wanted to be present."

There were concerns about the possible effect of pregnancy on Noirín’s heart rate but all was fine. However, she had to take it easier than most pregnant women, as tiredness has been a feature of her life since her arrest.

The couple was spared the possibility of a mad dash to Cork when it was decided to induce Noirín a day before her due date because of a leakage of amniotic fluid. She was determined to keep calm to try to prevent the risk of the ICD going off unnecessarily, which has happened.

Device confusion
On those occasions – once in a car crash and another when dancing too close to speakers – the device confused a surge in adrenaline with her heart rate going up too high.

“It scared the living daylights out of me,” says Noirín who had been warned it would be “like a kick of the horse in the middle of my stomach”.

In fact, “it felt like a phone vibrating in my chest – very, very strongly”. If you don’t lie down after the first shock, it goes again, getting stronger every time.

She certainly didn’t want to bring that on in labour “although, to be honest, it would have been the least of my problems”, she says with a laugh. But she was calm, Darren was “brilliant” and their baby girl, Éabha, arrived safely on September 19th, 2012.

Six weeks later they went to Our Lady’s Children’s Hospital Crumlin in Dublin to have a blood sample taken from Éabha for genetic testing. She was six months old by the time the result came back.

"It makes it that bit quicker since they found the gene in me and they knew what they were looking for," says Noirín. "But when you're waiting for something, weeks feel like months."

Inherited condition
They had hoped their daughter would not have inherited the condition but the test came back positive. "It was absolutely gutting but you pick yourself up and get on with it."

Éabha is on medication three times a day and will probably need an ICD fitted at the age of four or five.

“We have to try to keep her away from competitive sporting activities but it all depends on her personality,” says Noirín. “At the moment we are giving her the medication but we are going to raise her as a normal child.”

Having once been dead set against having children, Noirín says she is “so delighted now when I see Éabha that I actually did”.

Although LQTS may impinge on their daughter’s lifestyle, “it is not the end of the world. It isn’t the hardest thing to live with.”

Knowledge and treatment of the condition are improving all the time. And life for Noirín is much easier now she has a monitor at home, which she puts over her ICD for a reading that is relayed down the phone line to the cardiology department in Cork.

“I can lie on my bed at home and send this down – it’s brilliant.” It means she has to go to Cork for a check-up only once a year, as long as nothing untoward shows up in the read-outs.

“We’re looking forward to a very full and happy life with Éabha,” Noirín adds. “I am much happier that we know that she has this and we can work around it.”

swayman@irishtimes.com