About one-third of cystic fibrosis patients face problems obtaining life-saving treatment, research suggests.
Cystic fibrosis clogs the lungs with thick, sticky mucus
A study of 2,300 patients by the Cystic Fibrosis Trust uncovered wide variations in specialist treatment and prescribing practice across the UK.
It also said all 38 of the UK's specialist CF centres were under-funded, with many short-staffed.
Health officials said concerted efforts were under way to tackle CF through research, screening and better care.
More than 7,500 people - about half of whom are children - have CF, the most common genetically inherited life-threatening disease in the UK.
The average life-expectancy for patients is 31 years, but many die in their late teens or early 20s. The length and quality of life are dependent on a high level of medical care.
The research found:
- None of the 38 specialist CF centres receives sufficient funds to provide recommended levels of care for patients
- Only nine are able to employ even a half of the staff they need - with one centre saying they have funding to employ only 8% of the required staff
- More than 30% of the 2,300 patients who replied to the survey reported difficulties in obtaining vital drugs and therapies
- For one drug of proven efficacy, the proportion of prescribing ranged from 65% in one area to 23% in another.
More than 7,500 people in the UK have CF
About half are children
CF is the most common, life-threatening inherited disease in the UK
The disease attacks a number of organs in the body
It targets the lungs and pancreas in particular
CF clogs them with thick, sticky mucus, making it difficult to breathe and digest food
One person in 25 is a carrier of the CF gene - more than 2.3 million people in the UK
CF is managed through a constant daily regimen of medication and physiotherapy
Rosie Barnes, chief executive of the CF Trust, said: "The research clearly demonstrates what patients and medical staff have been telling us for some time: that the welcome increase in NHS funding over recent years is simply not getting to the front line of CF care.
"Without extensive and ongoing treatment, most babies born with CF would die before their first birthday.
"Yet this study shows that many children and young adults do not get the treatment they should."
Ms Barnes said she was particularly shocked at the fact that levels of care varied so widely depending on where a patient lived.
She said there had been modest improvements in the standards of care since the trust launched a concerted campaign in 1997.
However, she said that, overall, there was still a shortfall of over 50% in funding for CF care by the NHS.
The CF Trust estimates that providing adequate care for all patients would cost £308m a year.
Health Minister Stephen Ladyman said a concerted effort to tackle CF was already taking place through research, screening and better treatment and care.
"A package of research was announced last year in the Genetics White Paper, including £2.5m over five years to support gene therapy research for CF and up to £4m to provide gene therapy vector production for the NHS and other public sector researchers.
"A national roll-out of neonatal cystic fibrosis screening is currently under way."
Mr Ladyman said the aim was to bring existing provision up to the highest standard.
"We expect to see full roll-out of screening within three years, but we can't do everything overnight.
"The NSF for long-term conditions, which will be published next year, will initially focus on neurological conditions but will extend to other long term conditions including CF.
"Everyone with a long-term condition will have a personalised care plan and be supported by community matrons in every PCT."