A leading respiratory expert has called for an overhaul of the way patients suffering from a debilitating genetic lung disease are treated across Europe.
Dr Jane Lucas, a respiratory consultant at Southampton General Hospital, said that while changes to care for patients with primary ciliary dyskinesia (PCD) in England would ensure excellence in treatment, the situation across Europe was “fragmented”.
The condition, which affects around 3,000 people in the UK, causes defects in cells which line and clear the airways, ears and throat and can lead to frequent infection and permanent damage without early diagnosis, regular lung clearance and antibiotics.
She spoke out following the creation of four PCD centres in England, based in Southampton, Leicester, Leeds and London, to ensure all patients were assessed regularly and treated by dedicated specialist teams for the first time.
“We have reached a major milestone in this country through the development of the four centre network,” said Dr Lucas, who is also a reader in respiratory and allergy paediatrics at the University of Southampton.
“This is a landmark moment for patients and families affected by PCD and an indication of how determined we are to offer the best possible services.”
Previously, patients across England received varied levels of clinical care after diagnosis with patients cared for at 32 different hospitals and, although expertise existed in some areas, patients in other areas received either inappropriate or no respiratory care.
People with PCD usually have symptoms of a wet cough and blocked nose from birth and ongoing problems with chest, sinus and ear infections throughout childhood and adolescence. Without appropriate treatment this progresses to irreversible lung damage and hearing impairment.
Fiona Copeland, chair of the PCD Family Support Group, said: “We welcome the new national PCD management service which will ensure that our PCD children receive appropriate treatment throughout childhood regardless of their postcode.”
Following the creation of a PCD service in England, Dr Lucas and a team of 13 co-authors studied the structure of centres in 26 European countries and found only three – Hungary, Cyprus and Denmark – had a centralised system of care for patients.
The study, published in The European Respiratory Journal, found 36% of centres used variations of the saccharine test for diagnosing the disease, which is known to be unreliable, whereas nasal nitric oxide, the recommended screening test, was only available in 46%.
To confirm the condition, two of the key techniques, electron microscopy and ciliary function tests, were used by only 77% and 57% respectively, with 16% of centres unable to provide either.
As treatment, international guidelines suggest airway clearance therapy, which was not used in 22% of centres, should be standard, along with formal exercise, which was only practised by 28%.
Dr Lucas, based at the NIHR Southampton Respiratory Biomedical Research Unit, added: “PCD care in Europe is fragmented, with splits among a large number of centres, and this affects the quality of diagnostics and care and can prove the difference between preserving lung function and not.
“The development of larger centres, as we have established in England, along with these findings suggest there is no excuse for governments not to provide the basis for specialist care to ensure all patients, regardless of where they are in Europe, have access to the highest standards of care.”
Dr Lucas and her colleagues have successfully used this evidence to obtain European Union funding (€3m) to undertake further research and implement changes to ensure better provision of services for patients with PCD across Europe.
Posted on Wednesday 1 May 2013