Saturday 29 April 2017

Dad and daughter diagnosed with the same life-threatening heart condition within months of each other

Recently Paul Dawe discovered that he and two of his children suffer from a genetic, life-threatening heart condition

Paul Dawe with his daugther, Sarah. Photo: Tom Conachy
Paul Dawe with his daugther, Sarah. Photo: Tom Conachy

Joy Orpen

Paul Dawe (47) met his wife Rosemary when he was just 11 years old. One of the first things he told her was that he was already in secondary school. This, it later emerged, was a fib. But, nonetheless, the relationship did flourish. Now the Dawes, who live happily in Dundalk, have three children, including 14-year-old identical-twin boys.

However, their lives took an unexpected trajectory in recent years, following the discovery that some members of the family carry a specific gene which causes an enlargement of the heart muscle.

Paul says the warning signs appeared a long time ago when his brother John was diagnosed with a cardiac problem when he was just seven. Tragically, he died just 14 years later. As a result, Paul and his two sisters were examined at the Mater Miseracordiae University Hospital, Dublin. "Back then, testing was pretty rudimentary," says Paul. "So we got the all-clear."

In the ensuing years, Paul worked hard to take care of his children. He did contract work for the ESB; he drove lorries, and is currently employed as a caretaker in a secondary school in Dundalk. "I was sent for a medical when I joined the school," Paul explains. "Their doctor checked my heart, and he picked up a slight murmur. When I told him about my family history, he advised me to see my GP, who referred me to Drogheda [Our Lady of Lourdes's Hospital]."

A cardiologist then recommended that Paul have an echocardiogram, also known simply as an echo, which uses sound waves to image the inside of the heart, and to check muscle function. He had an electrocardiogram (ECG) which examines the electrical activity of the heart. And he was given a stress test, which involved having his heart function monitored, while walking on a treadmill, the incline of which was gradually increased. Finally, he went for an MRI scan, which can produce detailed images of various parts of the body.

Then Paul went back to the cardiologist in Drogheda. "I do remember that visit vividly. While I was waiting, I looked out the window. It was a nice summer's day, but nonetheless, I was thinking about my late brother John. By then, I knew for certain something was on the cards and I realised that my life was about to change forever."

Which it did; and quite dramatically.

The consultant explained to Paul that one side of his heart muscle was enlarged. He was given a diagnosis of hypertrophic cardiomyopathy (HCM).

According to consultant cardiologist Dr Catherine McGorrian, at the Mater Hospital, cardiomyopathy can affect the size and shape of the heart and the thickness of the muscle wall. This makes it more difficult for the heart to pump blood around the body. This rang true for Paul. "I'd noticed symptoms for years," he says. "But I always blamed my breathlessness on being unfit and not getting enough exercise. Now I knew the real cause."

Since cardiomyopathy is one of the causes of premature sudden cardiac death, and sudden arrhythmic death syndrome (SADS), early diagnosis and interventions are crucial. It can affect people of all ages. In many cases, it is an inherited condition caused by a genetic abnormality, so several family members can be implicated. Paul was prescribed beta blockers, which help improve the function of the heart, and he was advised to avoid any form of strenuous physical activity.

Following the diagnosis, Paul phoned his wife. "It was so scary. When I told Rose, she was absolutely shocked," he recalls. He was then referred to the Mater's Family Heart Screening Clinic at Heart House. This is a charity-funded public screening centre for people at risk of having a genetically inherited heart disease. "These people may have a family history of sudden cardiac death or SADS, or may have a living family member with an inherited cardiac disease," says, Dr McGorrian, speaking for the organisation.

Soon after, Paul and Rose's daughter, Sarah, had an MRI scan, which revealed that she was suffering from the same condition as her dad. "I was absolutely shocked," says Sarah, a gentle, charming 25-year-old accounts assistant. "I had never felt that I had anything at all wrong with me." Like her father, she used to get tired when she exercised, and, just like him, she ascribed it to lack of fitness.

Initially, Sarah was not given beta blockers, but she was monitored on a regular basis. As part of that process, she had another MRI scan last year, which revealed that the enlargement of her heart had increased. She is now waiting for a defibrillator to be implanted. "They had told me I might need one in the future; but I thought they meant when I was in my 40s," Sarah says. "So I was stunned when they told me recently I'd need one soon. They used to put them in the chest, but now they insert them more to the side, where they are less invasive."

The defibrillator will ensure Sarah's heart beats at a regular rhythm. "I've already met the doctor who will do the procedure," says Sarah, "and I have been told that this will not prevent me from having a family in the future."

In 2015, Heart House referred Paul and Rose's other children, Brandon and Jonathan, the twins, to Our Lady's Children's Hospital, Crumlin, where they initially tested negative for the heart condition. However, a further test last December revealed that Brandon had early signs of cardiomyopathy. "Rose burst into tears," Paul remembers. "And, because they are identical twins, it is likely Jonathan will also develop the condition."

This will not be the first time these lively boys face medical challenges. They were born at 28 weeks, each weighing about the same as a 2lb bag of sugar. "If they'd been born 30 or 40 years earlier, they wouldn't have survived," says Paul. Luck seems to be Brandon and Jonathan's middle name. Now they are benefiting from the good work being done by the Mater's Family Heart Screening Clinic.

"The service is just brilliant," says Paul. "They make sure you have access to all the treatment you need, even if they have to fight for it.

"Thanks to them, some of my family members are going to be spared what happened to my brother. If you feel breathless, and there was anyone in your family with heart problems, then go for testing," he urges passionately.

The Mater Foundation supports the Family Heart Screening Clinic by purchasing vital screening equipment and investing in research.

To donate, tel: (01) 830-3482, or see materfoundation.ie

Volunteers are invited to sell heart badges to help purchase a new echo machine (costing €95,000) which is crucial for early diagnosis

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