Rare disorder is linked to the mystery death of little Nicole
Tuesday, 29 January 2008
A rare disorder which affects less than 1% of the population may have caused the baffling death of an eight-year-old Ulster girl.
Little Nicole Black from Portadown died two days after she had hurt herself
while playing in the garden of a friend's house on June 12, 2006.
After her death, Nicole's heart was donated to save the life of
four-year-old Londonderry boy Paul Donnelly, who had been suffering from
dilated cardiomyopathy - an incurable enlargement of the heart.
Initially, it was thought that some form of trauma might have led to
Nicole's death.
Those who tended to the distressed youngster
immediately afterwards noticed that she had a small cut at her left eye
which became swollen.
Nicole, from Corbrackey Road, complained of
feeling sick and was taken to Craigavon Area Hospital but was later
transferred to the Royal Hospital for Sick Children in Belfast, where she
died two days later.
Nicole's cause of death was initially given
as cerebral oedema, or swelling of the brain, due to trauma.
At an
inquest yesterday in Craigavon Courthouse, none of the witnesses before
Coroner John Leckey, was able to explain how exactly Nicole had hurt
herself. The inquest heard that when asked about what she may have done at
the time, Nicole had repeatedly said, "I don't want to talk about it"
.
The inquest heard an extract from a report by Mr Peter Richards, a
consultant neurosurgeon from the John Radcliffe Hospital in Oxford which
highlighted the rare veins disorder Arteriovenous malformation (AVM) .
Mr Leckey said that although Mr Richards was unable to identify with certainty
the underlying reason for the cerebral oedema, he accepted "on the
balance of probabilities" Mr Richards' opinion that "a low-level
fall as is speculated happened in Nicole's case would not be expected to
cause her death from massive intra-cerebral haemorrhage".
Mr Leckey went on to quote from Mr Richards' report that: "If she had
suffered a severe primary head injury from an event ... I would not have
expected her to have been talking and walking immediately following the
injury. I therefore consider it likely that some other event has led to her
haemorrhage and I would speculate that a small arteriovenous malformation
has ruptured coincidentally and in the process destroyed itself, such that
no trace of it is identifiable on pathological examination."
Mr Leckey said that he was not sufficiently confident that trauma was the
underlying reason for the oedema, and ruled that the cause of death had been
from cerebral oedema, with a secondary cause of death of subdural and
sub-arachnoid haemorrhage.
Mr Leckey also paid tribute to Nicole's
family for allowing her organs to be donated. Four people, including young
Paul, were among those who were helped following her death. Such was the
gratitude of Paul's mother, Bella, that she named her daughter Nicole in
tribute when she was born in September, just a few months later.
Speaking after the inquest, Nicole's mother, Barbara, told of her relief at
the findings.
"I definitely came out of (the inquest) feeling
better. We went in wondering if we were going to get any answers at all, and
we did," she said.
AVM is described as "a tangle of
abnormal blood vessels", which is not inherited or infectious. It can
occur anywhere in the body, with brain AVMs considered rare and affecting
less than 1% of the population.
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