A teenager battling an extremely rare condition risks his life every time he goes to sleep.
17-year-old Liam Derbyshire suffers from Congenital Central Hypoventilation Syndrome, sometimes known as Ondine’s Curse, which causes his lungs to stop functioning as soon as he nods off.
The syndrome, which affects less than 1500 people worldwide, means that Liam has to be hooked up to a ventilator every night and have a carer on hand in case anything goes wrong.
When Liam was born, doctors told his parents, Peter and Kim, that he would only survive for 6 weeks.
But thanks to their dedication and around-the-clock care for their beloved son, Liam has defied all odds as he approaches his 18th birthday.
Mum Kim explains, ‘we rely on a machine to make him breathe and without that, he wouldn’t be alive. If he doesn’t breathe, he dies – that’s it.’
‘You don’t know from one day to the next, from one minute to the next minute whether he’s going to have a seizure, an episode, whether he’s going to get tired. He can collapse on you at any time.’
‘His condition is extremely rare and if we would have listened to some doctors he shouldn’t be alive right now.’
‘He had the intent for life and so that became our job to make sure he was offered that.’
Understandably, given his condition, Liam has a strict bedtime routine in order to keep him as safe as possible.
Liam’s dad Peter is in charge of hooking him up to the ventilator each night and checking that he is comfortable before the carer comes to watch him whilst he sleeps.
‘If you ask me if I had a good night sleep the answer is no, not since the day he was born to be quite frank’, Peter admits.’
‘You are constantly on the alert and you worry that perhaps it’s the last time you are saying good night.’
‘Liam’s sleeping arrangements are the electric bed which has three positions – it can be up or down and the head and feet can be lifted independently in order to give him some quality sleep.’
‘On the left hand side we have all of his ventilator equipment. The idea is that a ventilator supplies positive pressure so that Liam is actually forced to exhale CO2, which normally you or I would [naturally].’
‘There is also a plate under the mattress that will sense any sort of fitting and then the only way of making sure that he is still breathing is via that type of mask.’
Despite his syndrome and the developmental issues associated with it, Liam is thriving in mainstream school and spends plenty of time socialising with his large family.
Although further research is currently being carried out in order to find new treatments for Congenital Central Hypoventilation Syndrome, no one is quite sure what age Liam could live to. But despite the uncertainty surrounding their son’s future, Peter and Kim are determined to help him live the most independent life possible.
Peter says, ‘Life expectancy wise for Liam, nobody knows for sure.’
‘We have gone from nothing towards 18. The future for Liam I would say is, 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.’
‘Liam is living proof that there’s always hope.’
Watch the video above to find out more about Congenital Central Hypoventilation Syndrome and how Liam copes with his condition.