Rachel Willn would settle into bed next to her husband and know it could be her last night alive.  It sounds extraordinary but if any sudden noise – a scream or even an alarm clock – had woken her during the night, her heart could have stopped suddenly.  ‘We had to take the phone out of our bedroom,’ says Rachel, who’s married to Nigel, 32, and has two children, Jack, now six, and Adam, two.  ‘It was too much of a risk – someone could have called while I was asleep.’  Rachel, 29, who’s from Nuneaton, has Long QT syndrome. 

It’s an inherited electrical abnormality in the heart, causing an erratic heart rhythm.  The biggest danger is a sudden noise while the sufferer is asleep – this is the time when the heart rate is slowest and can be shocked the most.  There are dangers, too, with exercise when there’s most strain on the heart.  Rachel’s older sister, Lisa Browne, had the same condition, although she didn’t know it.  She died when she was just 27.

The two sisters were very close – both trained as nurses and they married within 10 weeks of each other in 1995.

The year before, Lisa had started to feel tired and faint all the time.  Her GP thought she was depressed and prescribed antidepressants.  ‘She knew it wasn’t depression,’ insists her  mum, Doreen Harley, 54.  ‘She was lethargic, not unhappy.’  On January 10 1998, Lisa’s husband panicked when he couldn’t rouse her from sleep and she was rushed to hospital by ambulance.  By the time Doreen and her husband Terry, 58, got there, it was too late.  ‘Nurses took us into the relative’ room and told us they’d tried to resuscitate Lisa but she’d died,’ says Doreen, from North Wales.  ‘I insisted on seeing Lisa in A&E, where I clung to her body, sobbing.  Losing a child is the most painful event a parent can ever experience.  Rachel was hysterical when we phoned to tell her the news.’

At the time, Lisa’s death was a mystery.  Doctors thought she could have had a deep vein thrombosis (DVT) or a brain haemorrhage, but a post mortem proved inconclusive.  ‘It was a black period.’ Says Doreen.  ‘The inquest that August was particularly hard – the cause of death was ‘unascertainable.’  But the pathologist hinted that her sudden death may have been due to an electrical abnormality in her heart, which can’t be detected after death as the heart isn’t beating.  He suggested that other members of the family should be screened.’

Doreen got information from CRY (Cardiac Risk in the Young), a national support group for families of sudden death victims, and was put in touch with the medical team at St George’s Hospital in London.  It specialises in screening families where a sudden death has occurred.  ‘I had to find an answer to Lisa’s death,’ says Doreen.  ‘I also desperately wanted to prevent another premature death in my family.’  Doreen and Terry were tested in December 1999.  Rachel was heavily pregnant at the time, so she missed out.  The results showed that Doreen was clear but that Terry had Long QT syndrome.  As he was older, the dangers from a sudden noise were less likely to affect him – he’d already survived his most active time of life.  It was probable that Lisa had inherited the condition from him – and others in the family could have Long QT, too.  The circumstances surrounding Lisa’s death now seemed clearer.  On that fateful morning, her alarm clock had gone off as usual at 6 am.  It killed her either because her heart was beating in a particularly erratic pattern at the time, or she was in a much deeper sleep than usual.  ‘I was worried about Terry,’ says Doreen.  ‘But my main concern was for Rachel and her family – she was younger and more active, so therefore at greater risk.’

I couldn’t let my boys lose their mum - so I was screened

For 2 years, Doreen begged Rachel to be screened.  ‘It became my obsession,’ she admits.  ‘I couldn’t bear if it Rachel died too.  Also, there was a good chance her sons could have it.’  But Rachel wouldn’t be pushed.  ‘I was busy being a mum,’ she says.  ‘I wanted minimum upheaval in our lives.  It was only when mum sent me a newspaper article about a family with the same condition, who had lost 16 members over three generations, that it struck home how at risk I was.  I couldn’t let my boys lose their mum.’  Rachel was screened last March, and it showed she had Long QT syndrome.  Jack did, too, but the condition doesn’t become actively dangerous until adolescence, when the heart’s fully developed.  Adam was screened recently and the family are still awaiting the results.  ‘I was in pieces,’ says Rachel.  ‘Deep down I knew though, I’d felt breathless recently, and had some blackouts, all signs of the condition.  I needed to know for sure, for myself and also for the boys.  When he’s older, jack will have it drummed into him about avoiding sudden noises and excessive exercise.’

As Rachel’s in the age group most at risk, she’s had to take as many steps as she can to avoid sudden noises in her sleep – no phone in the bedroom, and no alarm, either.  ‘I think I’d had some near-misses already.  I’d woken with the alarm a couple of mornings over the previous few years and blacked out.  My head was spinning when I came around.’  Rachel was offered a life-saving operation.  She was fitted with an electrical device which contained a pacemaker regulating her heart beat, plus a defibrillator to kick-start her heart if it suddenly stopped.  The operation involved a three-day stay in hospital, and she took 10 weeks to recover. 

Last August, it saved her life.  When Adam suddenly woke screaming in the bedroom next door to Rachel’s it woke her up and stopped her heart.  Mercifully, the defibrillator kicked in and got her heart going again.  Doreen’s relieved that everyone’s been tested.  ‘We’ve all suffered terribly.  Lisa’s death was dreadful – but if it hadn’t happened we’d never have known about the dangers to the rest of my family.’

Doreen’s now become a trainee counsellor for CRY and is campaigning for a national screening programme.  ‘Since Lisa’s death, my relationships with Rachel and Terry have suffered.  We’ve all had to come to terms with some terrible times.  Thankfully, it’s gradually getting better.  And at least we know what caused Lisa’s death.’ 

What Causes This?

Long QT syndrome is one of 10 of the most common causes of sudden young cardiac deaths.  It’s caused by a faulty gene which can be passed through generations.  Blackouts are the most common problem, but it can cause sudden death during sleep or exercise.  Research is ongoing but the greatest danger comes in youth (during or after adolescence) when you’re at your most active and after your heart has fully developed.  Risks are lessened with age because, as you’ve already survived through your more active years, there’s less likelihood of sudden death occurring when you have a less active lifestyle.  Unless you’re screened, the problem will go undetected.  It’s thought that four to eight ‘apparently fit and healthy’ young people die each week from an undetected heart condition.