By Alison Harper
In the Stevens’ household, in a fridge in the kitchen, is a stack of small boxes.
It does not look much, but not only is it worth thousands of pounds, it is quite literally a lifesaver.
It contains Elaprase, an enzyme replacement drug (ERT), which has given Oliver and Samuel Stevens a chance to be typical boys.
Oliver, nine, Samuel, seven, have Hunter Syndrome, also known as MPS (mucopolysaccharide disease), a genetic illness passed via the mother.
It is a degenerative illness which causes tissue to thicken, leading to heart problems, organ damage and stunted growth.
The boys were not expected to reach adulthood.
It is four years since I first met Claire and Bob Stevens at their home in Badshot Lea, Surrey.
"Before the treatment started there was a fair degree of certainty that we would outlive our children – now there is uncertainly"
Then they could only hope that a ‘wonder’ drug would pass tests and be made available.
When it did, there was a long wait for a UK licence, but finally 18 months ago, the boys had their first dose.
Claire was understandably over the moon.
“For five years we waited for them to get it,” she said.
“It is now been a year and a half since they have been having the infusions, it has been absolutely fantastic.”
The boys used to have to go to London’s Great Ormond Street Hospital for the weekly four-hour long infusions.
But now Claire has learnt how to medicate the boys at home – and that has made a massive difference.
“Oliver hasn’t had a hospital stay, apart from operations, since he has been on the treatment and Samuel hasn’t either,” she said.
So now every week, Claire brings out two blue trays and a mixture of medical paraphernalia, and lays it out on the dining room table.
She begins the ritual of sterilising the kit before attaching it to a portacath which takes the medicine via the heart.
To be able to physically help with the infusions has helped her deal with the boys’ illness.
“Hunter’s is carried by the mother and passed on to the children so for me to be able to give the boys something to make the them better – it doesn’t get any better than that,” she said.
The whole process takes five hours.
The medicine is kept in a small bag carried over the boys’ shoulder which looks like it could hold an MP3 player, and they carry on, watching TV and playing while they receive it.
“We can’t run or ride bikes,” says Oliver, but his brother is more upbeat: “We eat pizza!” he adds, “it is better than going to the doctors because there you have to wait and my mum now does it and it doesn’t take long.”
The ERT helps by softening the tissue, which means the visible features of Hunter’s – the heavy eyebrows and forehead – are lessened. The medication also softens the texture of the boys’ hair, and skin.
Internally, damage to the organs should not get worse – but it is not reversed.
"ERT in some cases has saved lives, and without question in others it is preventing physical degeneration"
Neither does ERT help the skeletal structure, so Oliver and Samuel will continue to find their joints stiffening.
There are approximately 90 people in the UK with Hunter Syndrome and because of the nature of the illness most of them are children.
Christine Lavery, chief executive of the MPS Society, has known Oliver and Samuel since they were first diagnosed.
“They were quite poorly children before they had ERT,” she said.
There are about 50 people with Hunter’s who are suitable to be treated with Elaprase and Christine says in many cases the results have been obvious.
“ERT in some cases has saved lives, and without question in others it is preventing physical degeneration,” she said.
“Some children with more progressive problems have become fitter, more well in themselves and they have regained some skills.”
It is that time of year where everyone reflects on the past 12 months.
Claire and Bob can see their children making progress and Bob’s guardedly optimistic about the future.
“Before the treatment started there was a fair degree of certainty that we would outlive our children which is something that is pretty awful to contemplate but now there is uncertainty.”
There is NHS funding for Elaprase until 2012, Claire and Bob are desperately hoping that after then, the government will continue to support them
This article is from the BBC News website. © British Broadcasting Corporation