Matty Jones, 15, from Chester, suffers from a rare condition known as Dense Deposit unwellness (DDD,) that is slowly destroying his kidneys.
The unwellness, that affects simply 2 in each million individuals, means that giant proteins roll up excessive quantities within the organs, inflicting the filters to become blocked and die.
The only drug that his family claim will stop his inevitable deterioration is presently not on the market - as NHS European nation say there's no proof it helps DDD patients.
He applied to possess the drug funded by his native hospital, however his oldsters, Nick and Tibeto-Burman language, were left sorrowful once the appliance was rejected.
The drug is prescribed in European country and alternative European countries and insurance firms procure it within the United States.
Now, the family ar writing to health secretary Jeremy Hunt to boost awareness to Matty's case within the hope the NHS can fund the drug before it's too late.
But NHS England same there's no proof the drug would profit Matty or anyone else together with his condition and it'd offer 'false hope' to DDD patients.Mr Jones, 53, said: 'There is just about no probability that he can ad lib endure this and he'll then get on a permanent cycle of qualitative analysis, transplant and temporary remission.
'It's not possible to place variety thereon however his expectancy in these circumstances - while not the drug - are going to be considerably affected.
'He's comparatively lucky at the instant, along with his main symptoms being managed through diet and drugs - however will not|this would possibly not|this may not} continually be the case and it positively won't cure it.
'Already he does not have enough energy and stamina to participate properly in sport and do the items a 15-year-old boy ought to be doing - although he usually tries.
'He's additionally been severely restricted in what he will eat.'
The family don't grasp once Matty's kidneys can fail - however once it happens he can undergo chemical analysis and eventually would like a transplant.
However, even a transplant is unlikely to save lots of him.
Even other than the apparent risks of operation and organ rejection - the DDD is probably going to come and destroy his new kidneys too.
The unwellness is caused by a defective a part of the system, inflicting the formation of additional proteins.
The drug Eculizumab blocks the assembly of a macromolecule referred to as C5b9 - that is accumulating in his kidneys.
The patent for the drug expires in 2020 thus albeit it's expensive currently, it's seemingly to be plenty cheaper in 5 more or less years.
However, for Matty, that's too late, as his kidneys can virtually actually have failing by then, his oldsters claim.
They have already virtually lost their son double to the unwellness.
Mrs Jones, 45, said: 'Matty shrunk respiratory disorder on a college trip to Oesterreich at Easter and that we were moments removed from losing him as he was transferred between 3 hospitals over one night.
'Then once he had simply recovered from that we have a tendency to were rush into Alder Hey Hospital with terribly high metal levels that may cause a coronary failure.
'We completed that the NHS were simply not moving quickly enough and Matty was too fragile to attend.
'We pushed to induce the freelance Funding Request submitted for Matty - that in itself took months - and it absolutely was heart-breaking once it absolutely was rejected with none right of attractiveness.
We knew then we have a tendency to had to require things into our own hands.'
Matty's family, that conjointly includes his 17-year-old sister Sophie, area unit currently effort for the treatment to be approved before it's too late for him.
Mr Jones continued: 'We started the petition on Sat evening and that we have already got quite one,100 signatures.
'We think that's fantastic and square measure terribly grateful to everyone WHO has supported America.
'We've barefacedly mammary gland deep into our contact list to arouse support and it's been heart-warming to receive messages of support from individuals we have not seen in a few years.'
NHS European nation aforementioned NICE administered a review last year, observing all the revealed analysis on whether or not Eculizumab may well be accustomed treat DDD.
It review failed to realize proof to recommend that the drug would be a good treatment, associate degree NHS European nation advocator aforementioned.
He added: 'These square measure implausibly troublesome things, and that we have each sympathy with all patients with rare diseases that no reliable treatment exists.
'Decisions on extremely pricy treatments for rare diseases square measure taken on the recommendation of clinical specialists WHO measure the most effective on the market proof.
'For the indications evaluated to this point sadly there's no reliable proof that eculizumab could be a safe or effective treatment for patients with this condition.'
