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  The Silent Killer October 7, 2001 Reporter :Paul Ransley Producer : Nick Farrow  Three years ago, Paul Ransley put together a story on a little known disease called Long QT. The syndrome has a great deal of relevance here in Australia. It's a devastating disease for those whose lives it touches, and is little known to those who have not come into contact with it; but who may well be at risk.For those who suffer from it, Long QT Syndrome is a time bomb in their chest. It is increasingly common, but most doctors seem unaware of its existence, and it often strikes without warning, killing young people in the full bloom of life.  The syndrome gets its name from the distinctive "signature" or shape of the wave — an unusually long interval between the "Q" and "T" wave points — shown on an electrocardiogram or heart monitor. This shows how long it takes for the heart to return to "normal" after contracting and pumping blood through the body.The hearts of people who have Long QT syndrome work perfectly normally most or nearly all of the time. However, there is a greater risk than normal that the heart will spontaneously get out of rhythm. In some people this leads to fainting spells. In a few people with long QT syndrome, if the rhythm does not recover spontaneously (as it usually does), this can lead to sudden death, according to the National Heart Foundation in NSW. Physical exercise, emotional shock or surprise — even an alarm clock going off — may trigger a fast, irregular and lethal heartbeat. When this happens, no blood is pumped out from the heart and the brain is deprived of blood, causing sudden loss of consciousness and death. Often the only warning signs are fainting or unexplained collapses — symptoms easily misdiagnosed, especially by doctors who may not even be ready to consider it. Common misdiagnosis of LQTS includes low blood sugar levels, a virus, febrile convulsions, epilepsy and hypotension (low blood pressure). Some doctors, who recognise that the condition is so subtle that physicians often misdiagnose it, say LQTS may account for a third of all unexplained deaths in children and young people.  A constant hindrance to doctors in the quest to diagnose patients with Long QT is the unstable nature of the syndrome. The usual way to identify the presence of Long QT is with an ECG. However, people with LQTS don't necessarily have a prolonged QT interval all the time; so when they have an ECG, the QT interval may actually be normal. As a consequence, doctors recommend that in any family where repeated episodes of fainting or a history of sudden death exist, an investigation of the cause(s), including Long QT syndrome, should be undertaken.As Paul Ransley found, experts are unsure exactly how many people die from Long QT in Australia every year. Estimates range from 20 to 230. In America, the figure is up to 4000 deaths a year. Research done in the past three years has revealed about one in 5000 suffer from Long QT. But there are a number of people with milder forms, and they may be as common as one in a thousand. Long QT is usually hereditary, but there is now increasing concern about how Long QT develops in healthy people exposed to certain drugs and chemicals. Long QT is easily treatable, usually with medication called beta-blockers. In patients who do not respond to medication, the surgical cutting of certain nerves in the neck — called left cardiac sympathetic denervation — or the insertion of a pacemaker or automatic defibrillator, can be used in treating the syndrome.  In Sunday's original Long QT story (June 14, 1998) we followed a family touched by Long QT. One Melbourne couple, David and Yvonne Jones, lived for years not knowing why their apparently healthy teenage daughter Chelsea died while competing in an equestrian event. Only when their younger daughter Felicity began fainting soon afterwards — and after enduring unsympathetic treatment from doctors — did an ambulance heart monitor reveal she had Long QT Syndrome, the likely cause of Chelsea's death. Felicity is now fitted with a defibrillator: an implanted device that will restart her heart if it ever stops.Since we last visited Felicity, her defibrillator has saved her life numerous times. One such time was after consuming two energy drinks containing caffeine and a herbal extract called guarana. Dr Brian Bailey told Sunday: "Guarana supposedly is similar to caffeine. But I don't think there's been a great deal of medical research about it. So, like many herbal products, it may have actions over and beyond what we believe it has, or it may be present in the drinks or chocolate bars, or whatever, in high quantities which are potentially dangerous." So why are stimulants, such as caffeine and guarana, so dangerous to Long QT sufferers? Felicity's cardiologist, Dr Jeffrey Alison, explains: "Long QT is a disorder where events are triggered by excess stimulation of whatever form. Therefore, in someone who is yet to be diagnosed, these stimulants may unmask their problem and cause their first event. And there's a significant number of people with Long QT Syndrome whose first event is a lethal event." Felicity now knows to avoid high-energy guarana and caffeine drinks, but cardiologists are concerned about their increasing popularity, especially for those who are undiagnosed. Paul Ransley, in our 1998 report, found another Melbourne family, the Foleys, whose children were collapsing without warning to a less than sympathetic reception from some doctors. Thanks to the persistence of their mother Julie, the children were eventually given ECG tests. The results vindicated her concerns. Three of the five children have had pacemakers fitted. Four now take beta-blocker drugs to regulate their heartbeats, which they will need to take for the rest of their lives. Julie and Lloyd Foley have had difficulties trying to educate Australians about the serious nature of Long QT. In an effort to spread the word, they run their own support group and website for other Long QT families.  Awareness is clearly the key to saving future lives, as Paul Simpson discovered. Paul's wife, Leeanne, was a 31-year-old mother of three when she apparently drowned. Leeanne had always been fit and healthy. Long QT wasn't considered as a cause for her death. The connection between her death and Long QT would not be made until our story in 1998, as Paul explains: "My sister saw the Sunday program featuring Long QT Syndrome and she sort of put two and two together and she researched it further and rang me, and I took the kids up to the local doctor who did an ECG on all of them and sent them away to the cardiologist, who then did a stress test where my daughter was diagnosed." Paul believes learning how his wife died may have saved the life of his 10-year-old daughter, Nicole.For further information on Long QT Syndrome, here is a summary of some of the best websites on the Internet: http://www.sads.org.au The Australian site for the Sudden Arrhythmia Death Syndrome (SADS) Foundation. Established by the Foley family in 1996, when they had become frustrated with the lack of LQTS help in Australia. It contains current LQTS research as well as contact details for those seeking help. There are links for international SADS groups. http://www.heartkids.org.au A support group for children suffering from heart conditions, run by the Royal Children's Hospital in Melbourne. It contains easy to understand facts and figures about heart defects, as well as stories written by children who are sufferers. http://www.heartdisease.8m.com St Vincent's Hospital's cardiology site. It includes support groups, contact numbers and general cardiology information. http://www.mydoctor.com.au General Australian medical website that explains LQTS as well as other medical disorders. http://www.qtsyndrome.ch LQTS sufferer Jon Biele started the Internet project, The European LQTS Information Centre, to collect and provide actual information about the condition. Its symptoms still lead to misunderstandings, which can be fatal. http://www.gwi.net/seahorsepress Kara Mia is the story of Kara Anglim, a 14-year-old girl who suffers a cardiac arrest at running practice. She was later diagnosed as being a LQTS sufferer. This is the story of coping with the condition, written by her mother Maryann Anglim, RN, and her father Walter Allan, a child neurologist. |
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