Our whole family was living with a timebomb

When Benita Davies 16 year old son died, she had no idea that the rest of her family was on borrowed time, too, due to a genetic condition.

When the police arrived at the hospital where I worked as a nurse, I knew something was wrong.  But nothing prepared me for the shocking news that my 16-year-old son Paul had died suddenly.

As they sat me down and started to explain what had happened, I listened in complete disbelief.  Paul had been riding his bike to meet a friend when he collapsed.  Passers-by tried to revive him, but it was too late.  My son was already dead.

“But healthy teenagers don’t just die,” I cried.

A post -mortem revealed that Paul suffered from a heart condition known as Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC).

Hardly anyone’s heard of it, but it’s believed to be the second most common cause of unexpected death in young people.  The condition involves the heart muscle being slowly replaced by fibrous tissue and fat, which ultimately makes the heart turn almost rigid and stop beating.  We were told that Paul could have died at any time.  His heart was a time-bomb waiting to go off.

A few months later his sister Nicola 23,went on holiday to Magaluf.  While she was there she passed out after going swimming,and spent a few days in intensive care.  She told hospital staff that her brother had died recently and they did a series of tests that revealed she, too, had suspected ARVC.

I was devastated by the news , I couldn’t bear the thought of losing another child.  When Nicola got home, I rushed her to the doctor for tests as I wanted the condition confirmed.  She had an electrocardiogram (ECG) and an X-ray, but nothing showed up.

“She’ll be fine” the consultants insisted.  But instinct told me otherwise and I wasn’t prepared to wait for the worst to happen.

So, I turned to a charity, CRY (Cardiac Risk in the Young). They warned me that ARVC is often a genetic condition with females as carriers and my entire family should be tested.

I insisted the doctors reassess my daughter, and eventually she was admitted to St George’s Hospital in London for further tests.

They revealed she had an advanced form of ARVC, and like Paul, was in danger of dying.  She had an operation to fit a defibrillator to kick-start her heart if it did suddenly stop working.

I was so relieved, but more traumatic news was to come.  I was tested and the condition was found in me.  Doctors immediately put me on a course of medication and scheduled further tests every six months- and my whole family decided to get tested straightaway.

My eldest daughter Lyndsey, 26, was shown to be a carrier, although she is not showing any signs of developing the condition yet.  It was also revealed my younger sister, Claire, 47, had an advanced form of the condition.  As a teenager she used to faint regularly, but teachers at school used to blame her blackouts on standing too long in queues or assemblies. Little did we know she risked dying suddenly at any time.

Most deaths due to ARVC occur between the ages of 12 and 25.  It appears that fit and healthy teenagers are most at risk. It is uncommon, but not unheard of, for older people to develop symptoms.  Now Claire also takes regular medication.  Her children Shelley, 19 and Stacey, 16, are also tested every six months for the development of ARVC.

My other sisters Gillian, 42, and Vivien,41, also have five teenage children between them.  Gillian’s son John is now 15, and because he is almost the same age as Paul was, when he died, he may be at increased risk and needs testing more than the others.  Her other children are also suspected of having the condition.

ARVC is difficult to diagnose and was only discovered in the Seventies.  Most victims don’t have any symptoms at all.  The only usual symptoms are fainting fits and rapid heart beats.  But if you have it, there is always a potential risk of sudden death.

I traced my family tree for a heart specialist to examine and I was shocked by the results of my research. I knew my granny had had her first heart attack aged, 38, but I was staggered to discover that nine of my great-aunt’s sons had died suddenly, in their 30s or 40s from heart problems. I had even suffered from an attack of angina when I was 38.

It was only after Paul’s death that family members started to talk about how many people had suffered from heart conditions, and then I realised there was a massive family link that couldn’t be ignored.  I am concerned for my nieces and nephews.  I just couldn’t face seeing my sisters go through what we did when we lost Paul so young.

Thankfully the rigorous testing we are constantly undergoing as a family should save us from losing anyone else.  But the risk is always there.

Nicola’s life was undoubtedly saved by Paul’s death, but if we had known about his condition sooner then Paul would also be alive today.  He would have needed a full heart transplant, but he never had the chance.

Find out more about ARVC