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Sudden noises danger for sisters Sudden noises danger for sisters
(40 minutes later)
When Chloe and Evie Church wake for school, they cannot use an alarm clock like other children.When Chloe and Evie Church wake for school, they cannot use an alarm clock like other children.
The sisters, aged 11 and nine, have a syndrome that means a loud noise or a sudden shock could be dangerous or even fatal.The sisters, aged 11 and nine, have a syndrome that means a loud noise or a sudden shock could be dangerous or even fatal.
The girls from Swansea both have Long QT syndrome, a condition which affects some 1 in 7,000 people.The girls from Swansea both have Long QT syndrome, a condition which affects some 1 in 7,000 people.
The syndrome, which can be passed on through families, causes abnormalities in the heart's electrical system.The syndrome, which can be passed on through families, causes abnormalities in the heart's electrical system.
They know what they can do and what they can't do Samantha Church, mother of Chloe and EvieThey know what they can do and what they can't do Samantha Church, mother of Chloe and Evie
"We try not to shout, the girls aren't allowed to have alarm clocks in their bedrooms so we've got to go quite quietly into their rooms in the morning, and wake them gently," said their mother, Samantha."We try not to shout, the girls aren't allowed to have alarm clocks in their bedrooms so we've got to go quite quietly into their rooms in the morning, and wake them gently," said their mother, Samantha.
When the school is planning a fire drill, they have to tell Chloe and her younger sister so they can expect to hear the alarm ringing.When the school is planning a fire drill, they have to tell Chloe and her younger sister so they can expect to hear the alarm ringing.
They also have to be supervised by someone who can perform cardiopulmonary resuscitation (CPR) and who is able to take a pulse.They also have to be supervised by someone who can perform cardiopulmonary resuscitation (CPR) and who is able to take a pulse.
But although the family have had to adapt to the girls' condition, they can still enjoy a relatively normal life.But although the family have had to adapt to the girls' condition, they can still enjoy a relatively normal life.
Last week they were able to go to a Guy Fawkes night party safely because they knew to expect sudden, loud noises from the fireworks. LONG QT SYNDROME Causes the heart's electrical system longer to recover after heartbeatsSymptons include fainting during exercise or when scaredAround 1 in 7000 people are effectedDiagnosed easily by examining a electrocardiogram Last week they were able to go to a Guy Fawkes night party safely because they knew to expect sudden, loud noises from the fireworks. LONG QT SYNDROME Causes the heart's electrical system to take longer to recover after heartbeatsSymptons include fainting during exercise or when scaredAround 1 in 7000 people are affectedDiagnosed easily by examining an electrocardiogram
A sudden noise, or too much exercise is enough to cause a rush or adrenaline or a sharp rise in blood pressure.A sudden noise, or too much exercise is enough to cause a rush or adrenaline or a sharp rise in blood pressure.
This could be fatal, and so the girls will have to take beta-blockers for the rest of their lives.This could be fatal, and so the girls will have to take beta-blockers for the rest of their lives.
The girls were diagnosed two months ago with the condition, which also affected their grandmother.The girls were diagnosed two months ago with the condition, which also affected their grandmother.
Symptoms normally first appear in childhood, but can occur in babies and during middle age.Symptoms normally first appear in childhood, but can occur in babies and during middle age.
Beta-blockers control the condition in about 90% of cases, but some people may need a pacemaker or defibrillator.Beta-blockers control the condition in about 90% of cases, but some people may need a pacemaker or defibrillator.
"The girls were diagnosed in September and we've adjusted our life to them, slowing down a bit - they know what they can do and what they can't do," said their mother."The girls were diagnosed in September and we've adjusted our life to them, slowing down a bit - they know what they can do and what they can't do," said their mother.