LISTENER SEPTEMBER 7 2019by Ruth Nichol
HEALTH
ThisLife
they’d never been diagnosed in the
first place.”
Haemophilia, which affects about
one in 5000 males, is caused by low
levels of one of two blood-clotting
factors – either factor VIII or factor IX.
More than half of those affected have
severe haemophilia, which means
their factor levels are less than 1%.
People with haemophilia don’t bleed
faster than other people, but they do
bleed for longer – with potentially
fatal consequences. Haemophilia
can’t be cured, but even severe hae-
mophilia can be treated with regular
intravenous injections of the missing
clotting factor.
Haemophilia is known as asex-linked disorder because the genes
that govern factors VIII and IX are
situated on the female (X) chromo-
some. A boy whose mother carries
the gene has a 50% chance of having
haemophilia. Her daughters have a
50% chance of carrying the gene,
which may in turn affect their sons.
However, in about 30% of cases
there is no family history of the
disorder. Instead, it develops from
a spontaneous genetic mutation.
It’s now known that some women
carriers also have lower-than-normal
levels of blood-clotting factors. This
can cause problems such as heavy
periods or excessive bleeding during
pregnancy and labour.Living
carefully
ever after
Men with haemophilia are
living much longer than
they used to, but that brings
new challenges.
NUTRITION • FOOD • WINE • SPORT
Deon YorkG
ET
TY
IM
AG
ES
I
f Deon York, who has severe haemophilia,
had been born several decades earlier, he
probably wouldn’t have survived to his
late thirties – let alone be working full-time
and travelling regularly overseas to do
volunteer work for the World Federation of
Hemophilia.
In 1960, life expectancy for a person with severe
haemophilia – a genetic bleeding disorder that
affects mostly males – was about 20 years. They
died from either external or internal bleeding.
And as York knows from his work overseas, that
is still the case in developing countries where hae-
mophilia often goes undiagnosed and untreated.
When he went to Laos last year, he met the coun-
try’s entire identified patient group- just 17 of them in a population
of almost seven million people.
That compares with a New
Zealand patient group of about
430.
“They were mostly kids,” he
says. “Everyone else had moved
away or, very likely, died – or