PETER DERBYSHIRE: He can breathe on his own during the day, but not when he goes to sleep.
KIM DERBYSHIRE: Doctors believed he wouldn’t live for longer than six weeks.
KIM DERBYSHIRE: It was like, ‘Oh right, okay. Game on!’
PETER DERBYSHIRE: You alright?
LIAM DERBYSHIRE: Yeah.
COMM: 17-year-old Liam Derbyshire suffers
from an extremely rare condition that affects less than 1,500 people worldwide.
KIM DERBYSHIRE: Liam has a condition called
Congenital Central Hypoventilation Syndrome or Ondine’s Curse. Ondine was a fairy and
he was cursed that if he ever fell asleep, he would die, because he wouldn’t breathe.
So we rely on a machine to make him breathe. And without that machine, he wouldn’t be alive.
COMM: Congenital Central Hypoventilation Syndrome
or CCHS affects Liam’s central and autonomic nervous system, which controls functions such
as blood pressure, heart rate and breathing, and can increase the risk of organ damage,
especially to the brain.
PETER DERBYSHIRE: If you ask me if I had a
good night’s sleep, the answer is no. Not since the day he was born, to be quite frank.
KIM DERBYSHIRE: You don’t know from one
day to the next, one minute to the next minute, whether he is going to have a seizure, an
episode, he is going to get tiered, he can collapse on you at any time. I always remember
one senior nurse saying to me many times, ‘It will get easier as he gets older.’
That is the biggest load of rubbish going because it does not get easier. If anything,
it gets harder. You go from having what you think is a normal child to someone who actually
gets rarer by the day, because he is surviving so long as he is.
COMM: Every night, Liam relies on a ventilator
to keep him alive when he sleeps. A carer is also on hand to watch over him, in case
anything goes wrong.
PETER DERBYSHIRE: Basically, Liam’s sleeping
arrangements are the electric bed, which has three positions in order to give him some
quality of sleep. There is a plate underneath the mattress, that will sense after 12 bursts
of fitting, the idea’s that this ventilator supplies positive pressure, so his lungs are
forced to exhale. Ever anybody’s sort of had the night dream where you falling off
a cliff, that’s your body telling you your phrenic nerve is alarming and it makes you
take a deep breath. So, equated to that, that’s his condition, he has that all the time.
PETER DERBYSHIRE: It’s also his room, so
we’ve got his PlayStation, his set TV, then obviously Liam’s big forte is LEGO, which
is all around the room.
COMM: It’s currently not known how Liam’s
condition will affect his learning abilities long term.
PETER DERBYSHIRE: His mental state is unknown,
to be perfectly frank, we don’t know which is the spectrum, so where it starts and where
it finishes.
KIM DERBYSHIRE: He can build these incredible
LEGO models and there is thousands and thousands of parts, and yet he can put that all together.
PETER DERBYSHIRE: He will check the LEGO website
to see if there is any new one’s out and you know full well what’s coming on either
his birthday or Christmas list.
INTERVIEWER: Which is your favourite one, Liam?
KIM DERBYSHIRE: Liam is 17, nearly 18, so
it’s a big birthday this year. He is an annoying, grumpy teenager. So he is no different
to any other teenager. But he does have a sense of humour. He is very caring. He has
got a really soft side. He has one older brother, three half sisters. His two older sisters,
they have both got children so he has got eight nieces and nephews.
RHYS GIBSON: Me and Liam, we do a lot of things
together. We play with the trains, build LEGO.
RHYS GIBSON: He loves trains. You ask him
anything about trains, he’ll tell you, yeah.
RHYS GIBSON: Like, some people don’t understand
what he says, but I do. You just got to take a bit more time and proper listen to what
he says to understand and fully be aware of what he is saying.
PETER DERBYSHIRE: Life expectancy wise for
Liam, nobody knows for sure. But... KIM DERBYSHIRE: You cannot regret or have
any remorse about the life he has had. Because he wouldn’t have had it. If we would have
listened to some of the doctors, by that, he shouldn’t even be alive now. We never
really thought we would get this far.
PETER DERBYSHIRE: Liam’s living proof that
there’s always hope. A future for Liam is I would say it’s not going to be totally
independent, but with us two, it’s going to be as independent as we could possibly
make it.
KIM DERBYSHIRE: It’s 18 years since we started this journey, you see.
PETER DERBYSHIRE: Yeah, I don’t regret any day of it.