Ashley was born in 1982. When the midwife put him into the cot beside Anne’s bed, she looked at him and thought: “My God, from now on this little person has to be looked after.”
He was always active. In 1998, aged 16, he played football twice a week, cycled 56 miles from London to Brighton, and ran for his athletics team. At school, computing was his best subject. He had set up his own website – quite an achievement, back then. Against a blue background, it showed a “Welcome” sign, with flashing lights, a whirling globe, and Homer Simpson bobbing up and down inside a beer glass. The centre of the page announced the time of day and greeted visitors warmly, particularly if they visited more than once: “Wow, I must be doing a darn good job!”
Over the phone from work, Anne told Philip to fetch a neighbour, Sharon. Then she called for an ambulance and hurried home. When she got there, Anne rushed into the bedroom to see her older son. He looked peaceful, as if asleep, but showed no signs of life. The paramedics confirmed that Ashley was dead. How could somebody so young die suddenly in his sleep? Ashley had always been so healthy. He’d even won awards for taking no time off school.
As it turns out, somebody young and healthy dies mysteriously every day. In fact, more often than that: eight die each week of instantaneous heart failure, classified as sudden adult death syndrome (Sads). Ciara Agnew, 14, died coming home on the school bus. Tom Baldwin, 15, died at a friend’s house. Jamie Coffey, 17, was having a kickabout after dinner when he collapsed and died. Sarah Dudhill, 19, was found dead in bed by her boyfriend. Robert Manning, 16, died playing rugby with his father and brothers. The list goes on. Each one had been regarded as healthy. So who’s to say this couldn’t happen to someone in your family?
The average age for sudden cardiac deaths is 17, but Sads does not only kill teenagers. Among the most recent – unconfirmed – cases was Dan Frank, who collapsed and died without warning in October, in the newsroom of the Catholic Herald, where he worked. He was 24. Still older victims include Tony Hughes, 35, who also died at work, and father of three Richard Abbott, 34.Deaths like these are actually so common that they’re rarely reported in the national news. So families like the Jollys usually have little idea that their private loss fits into a national pattern.
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Until relatively recently, the same could have been said for cot death, which kills barely half as many people – fewer than 250 infants each year. As awareness of cot death increased, notably thanks to the campaigning efforts of Anne Diamond, the incidence fell dramatically. If awareness of Sads reached the same levels, hundreds could be saved each year. And that might include you, or someone you love.
Ashley’s death was a mystery. The police were called at once. Nobody was allowed into the bedroom until an inspector had taken a look. Eventually, Ashley was enclosed in a body bag and taken away. The family would have to wait for a postmortem to find out how he had died.
“It sounds so dramatic when you try to explain it,” Anne says, at her home in Brentwood, Essex. “But when something like this happens, you feel like your whole life has ended. You don’t think you’re going to survive. It’s physical pain as well as emotional pain: your stomach actually hurts.” Before I came to see Anne, I expected our conversation to be extremely painful. I was ready for her to break down in tears. That’s not what happened. Considering what she’s been through, Anne’s face is remarkably unlined – a clue, perhaps, that this is a woman who keeps the more operatic emotions in check. Only after we’ve been talking for an hour or so, when she recollects that sensation of physical pain, does she blink rapidly – the first, easily missed, evidence of strong emotion.
No bereavement is easy, but the loss of a child is hardest of all. Statistics show that if you suffer the death of a child, the odds that your marriage will break up are doubled. What happened to the Jollys was arguably worse than most such bereavements, because in cases of chronic illness families can prepare for a child’s passing. Even a fatal accident can be easier, because that gives parents someone else to blame – without that, research suggests, they blame themselves.
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One of Anne’s strategies for making sense of Ashley’s death was to write poetry. “Did I dream I had a son I hugged for 16 years/ Along with the responsibility, hard work and the tears/ Did I dream I lost him one night, as he lay in bed asleep/ Did he really die so easily and leave us all to weep?” She became convinced that her son was still with her; that he sent his love by dropping white feathers. “They fell on my lap on the anniversary of Ashley’s death.”
But her immediate response was to puzzle over his death. Ashley wore a brace for his teeth. Anne wondered if something had come loose and he’d choked on it. She asked herself what he’d eaten that last day. A lot of bacon. Could that have done it? When something doesn’t make sense, it’s difficult to be logical.
Eventually, the official verdict came in: Ashley had suffered a fatal asthma attack. But something didn’t seem right. Ashley had never had asthma. And his peaceful appearance in death gave no sign of disturbance. Anne – a light sleeper – would surely have heard him have an asthma attack. So would Philip. It didn’t make sense.
She asked to see a copy of the postmortem. “With someone you have loved and nurtured for 16 years, you don’t just say, ‘Oh well, it’s just one of those things.’ Not knowing what happened became all-consuming, overwhelming.”
Her surviving son, Philip, became severely introverted. He lived in his room, and Anne practically had to post his dinner under the door. He went through an angry phase, getting in fights at school. John, her husband, struggled to cope with what had happened. “He didn’t want to speak about it. I could see it in his face, and he wouldn’t answer when I brought the subject up.” So she locked herself away in Ashley’s computer room. She started writing to the teaching hospitals in London, asking about asthma and respiratory problems. Eventually, she got a letter from Guy’s Hospital saying the problem was more likely to have been Ashley’s heart. So Anne had her son’s heart sent to a specialist. But they found nothing wrong. It was perfectly normal.
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At the top of the human heart sits a group of blood vessels shaped like a crown, or corona. The coronary arteries supply blood to the heart muscle. Most heart-related death involves some kind of malfunction here. Typically, the coronary arteries become furred with cholesterol, severely restricting blood flow. Not all such “attacks” are fatal, but when they are, the cause of death is easy to identify at postmortem.
That kind of heart problem, relatively common in older people, is rarely seen in young adults. Here, a likelier cause of death is “arrhythmia”, or disturbed heartbeat. This can arise because the heart muscle is exceptionally enlarged, or due to weaknesses in the heart’s right ventricle. In both cases, again, the cause of death can usually be found at postmortem.
An entirely different set of potentially fatal problems, only recently identified, stems from the heart’s electrical circuitry. The muscle of the heart comprises millions of cells. Each cell has pores allowing the passage of chemicals. These provide an electrical signal that stimulates the heart to beat and thus pump blood. A disordered electrical signal can show up only while the person is alive; in death, the heart appears normal.
Altogether, one in every 20 cases of sudden cardiac death, or about 500 every year in the UK, shows no cause. This “invisible” killer can strike suddenly. Symptoms include breathlessness and fainting, but in a third of cases the first symptom is sudden death. This can be provoked by stress and exercise, the deep relaxation of sleep, or shock. Even the sudden noise of a doorbell has been known to trigger a fatal arrhythmia.
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Up to 10 years ago, if a young person died suddenly and nothing showed up at postmortem, it was put down as a “natural” event, and families were told not to worry. Even specialists had no idea what was going on, says Dr Sian Hughes, a pathologist specialising in heart problems at University College London. In her windowless office she pulls a massive volume from her shelves: Muir’s Textbook of Pathology. It’s the 12th edition, published in 1985. “Let’s have a look,” she says, turning to the section on hearts. “There’s nothing. These problems were not even known about then.”
Sads is still not fully understood. Sudden arrhythmic death syndrome (as doctors more correctly term it) is usually inherited. The whole family may have the condition. If individual members aren’t checked, a second – or third, or fourth – shocking bereavement may occur. In many families, that has happened.
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Susie Murray lost two close relatives before doctors realised the whole family was at risk. The first time I telephoned her, she couldn’t speak to me. “I’m sorry,” she said, “I’m expecting a call. My son had three peculiar episodes over the weekend. We’re trying to get hold of a consultant.” I called back later, but nobody was at home. They’d rushed Alex, 11, to hospital. He was okay, but it’s hard to imagine a better insight into the life of families with Sads – constantly on the alert, waiting for someone else to die.
Susie’s sister Dawn, 33, was the first to die. She got up early one morning in the autumn of 1999 and collapsed. The family was told she’d died as a result of Sads and was advised to have tests. But the results of those tests were misinterpreted. As a consequence, wrongly believing that all was well, Susie also lost her daughter, Nadine.
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Two years after Dawn died, Nadine, 21, was at home in bed. She was on the phone to her boyfriend, Matt, who was waiting to board a flight. “He heard a muffled noise,” Susie recalls, “then Nadine sighed. Matt actually heard her die, but he didn’t realise that. He thought she’d been cut off.” When Nadine failed to appear at college, friends alerted the family. Susie’s husband, Alan, went round to find Nadine dead in bed, the phone at her side.
Susie and her son are now being treated, but living with the condition remains terrifying. “You have a constant, gnawing fear that it’s going to happen again. Imagine what it’s like for Alex. He has a deep fear that he will die in the same way.” He also has to deal with other people. His school insisted he mustn’t take part in sports. “He’s not allowed on the school bus either,” Susie says. “They said, ‘It wouldn’t be nice for the other children if something happened to Alex.’”
At the Heart Hospital in central London, the clinic devoted to cardiac problems in the young boasts sophisticated technology. But the clinic’s work begins with less high-tech family trees.
Constructing a pedigree for each family, explains Eileen Firman, can indicate a pattern of death at a young age. She shows me a sample on an A4 sheet. The chart provides insight into family tragedy. Individuals who have died have a line through them; the age and supposed cause of death is written underneath. It’s impossible to miss the pattern of untimely mortality. The patient’s aunt died at 39, her grandmother at 33. Two cousins died as infants, victims of cot death; two others lost babies the same way. (It remains unclear how closely cot death is linked to Sads.) Patients are often surprised to see the patterns. They thought their parents or grandparents died of unrelated causes, such as epilepsy, pneumonia or asthma. Others may have been identified as drowning or dying in car accidents – while actually suffering an arrhythmia in the pool or behind the wheel. Once the pedigree has been completed, families come to the clinic for tests. About half the individuals are found to have a problem. “It’s extremely difficult when one sibling has the condition and the other doesn’t,” says Firman. “There can be guilty feelings in the person that doesn’t, and often everybody devotes all their attention to the child that has it.”
Treating Sads is not complex. Prescribing beta-blockers can often do the job. Or patients can be fitted with an implantable cardioverter defibrillator (ICD). In the Heart Hospital’s cafe, Firman takes an ICD out of a plastic bag and hands it to me. It’s covered in a dull metal, shaped like a symbolic heart, and weighs about the same as my mobile phone. This model costs more than £15,000. The ICD detects abnormal heart rates and, after allowing a short period for problems to rectify themselves, sends an electric charge into the ventricle. It’s not pleasant – patients say it feels like being kicked in the chest – but it keeps them alive. Firman knows of one woman who tends to get a shock every day.
“She passes out first. I was in a car with her when suddenly her head dropped onto my shoulder. Then I felt this huge jolt go through her. She was out for about 20 seconds altogether.”
One young patient who has cheated death thanks to the ICD is James Maskell, who experienced chest pains and dizziness during exertion. After extensive testing, doctors found that his heart was stopping for several seconds during the early hours of the morning. He might have been found dead one day, but he was lucky enough to be checked and now wears a device.
When the government issued its first National Service Framework (NSF) six years ago, describing the rights of patients, it declined to acknowledge Sads; “only” eight young people die each week, compared with nearly 2,000 people killed by coronary heart disease in the same period. But Professor William McKenna, who set up the clinic at the Heart Hospital, argues that Sads deaths are preventable, and their impact is disproportionately severe. “It’s not like losing your grandfather at 92. Families fall apart.”
The latest NSF, issued last year, acknowledges this. A chapter has been added to cover diseases of the heart’s electrical system, indicating that patients should be sent to centres of excellence. Meanwhile, the Royal College of Pathologists is putting out advice to its members, many of whom remain unaware of Sads.
Both developments owe a lot to charities such as Cardiac Risk in the Young (Cry), run by Alison Cox, and Sads UK, which Anne Jolly established after losing Ashley. Today, Sads UK has about 300 members. One is Penny Hurrell, whose daughter Louise died suddenly aged 12. She lost consciousness on the sofa one evening, after a short episode of palpitations, and never came back. For the first few weeks afterwards, Penny wanted to put up Louise’s picture all over the high street, perhaps even a statue. “It was like I wanted to introduce her to everybody. The feelings of pain and loss are indescribable. And the guilt can never be appeased. I can’t believe I was not given the knowledge that would have enabled me to protect her.” Eventually, a friend gave Penny one of Anne’s leaflets. “And that opened my eyes. It’s tempting to think this has never happened to anyone else. When you realise that it has, you don’t feel so lonely.”
Between them, the charities have put on conferences (at one event, when Anne stepped up to the podium to speak, she found a white feather waiting for her). They’ve recruited celebrity supporters, and set up parliamentary pressure groups. They’ve raised money to buy heart monitors, and written to 44,000 GPs explaining Sads, so that in the statistically unlikely event that doctors come across a case, they refer the entire family for checks.
(Firman, at the Heart Hospital, confirms that the vast majority of families come to her through the charities, not GPs.) Anne would like to see the introduction of a national monitoring scheme, with children attending GP surgeries when they turn 11 and recording any history of fainting or palpitations and details of family members who may have died prematurely.
The results would help to identify the children most at risk, who could then be checked more thoroughly. She’s also encouraging schools to take advantage of the British Heart Foundation’s Heartstart UK programme, in which both children and teachers are taught resuscitation techniques.
Her husband, who used to hate the way Anne dwelt on Ashley’s death, now says he is glad she did, because he didn’t believe Ashley had died of asthma either. Today, John Jolly is no less committed to the campaign than his wife. Their son Philip, now 22, has largely regained control over his life; he recently got engaged.
Because she lives locally, Penny Hurrell has been able to get closely involved with Anne’s work. She is often at the house. When I visit, Penny makes the tea and hands out the biscuits. The two women are close friends. They feel they knew each other’s children, though they were never able to meet them.
In one of Anne’s poems, she writes about moving on: “I didn’t have a dream, Ash, for you were real enough/ I have to live my life now, but I shall find this tough/ I know what you would say, if you could speak to me/ Get on with your life, Mum, for what will be will be.’”
Has she got on with her life? In many ways, she has. She hasn’t hoarded Ashley’s possessions; the few things she’s kept, such as his West Ham football kit and his computer, are packed away. The closest to a shrine is his old website, which continues cheerfully greeting visitors who happen to come across it.
The real memorial is the ongoing campaign to raise awareness of Sads. These women are strongly motivated to prevent other families from going through what they’ve endured. It doesn’t detract from that to point out that the work is not exclusively altruistic. It answers an intensely personal need, as Penny concedes: “By doing what we are doing, I believe we’re attempting to prolong our children’s lives.”
Anne, recalling that moment when her newborn son was placed beside her, agrees. “It’s my way of looking after Ashley,” she says. “But not in a sense I would ever have imagined.”
SPORTING TRAGEDIES
Even the fittest young people can die suddenly — and unnecessarily — as a result of inherited heart conditions. Daniel Yorath, the son of the former Leeds United and Wales player Terry Yorath, died aged 15 while playing football with his father. Ian Bell, 16, died shortly after signing for Hartlepool. John Marshall, also 16 and a junior international footballer of the year, died the day after signing for Everton. Jason Erics, a Tottenham Hotspur youth player, died at 17. David Longhurst, 25, died playing for York City. And the Manchester City player Marc-Vivien Foe, 24, died during an international match. Cyclists, swimmers, tennis players and cricketers have also succumbed. “Nearly every case I read about,” says Terry Yorath, “someone is quoted as saying, ‘He was a fit young lad.’ If someone is a fit young man, they don’t think it can happen to them.” The Premier League now requires clubs to test young recruits, but screening for the general population is not economically viable. The best hope is to raise awareness of Sads, and to train fitness instructors and PE teachers in resuscitation techniques.
For further information, visit www.sadsuk.org