Cystic fibrosis services have received extra funding but they still fall well below international best practice. Fiona Tyrrell reports
The news earlier this month that €4.78 million has been earmarked for extra staff resources for cystic fibrosis services, has been welcomed by patients, medical professionals and support groups.
However, with staffing levels for cystic fibrosis services more than 400 per cent below minimum accepted standards and lack of segregation and isolation facilities putting patients' lives at risk on a daily basis, much more is needed, according to Cystic Fibrosis Association of Ireland (CFAI).
Ireland has the highest incidence of the disease in the world with more than 1,100 cystic fibrosis sufferers and one in every 19 people in Ireland are cystic fibrosis carriers.
Cystic fibrosis causes a build-up of mucus and leaves patients exposed to frequent infections. This leads to the deterioration of the lungs and in some cases a lung transplant is vital for survival.
As there is no cure for cystic fibrosis, patients rely heavily on the health system to manage their often severely debilitating symptoms.
Twenty years ago there were very few people with cystic fibrosis, now the medical community worldwide is seeing an aging population. In Ireland there are many people aged 30 and 40 living with cystic fibrosis and a small number aged 50 and 60. No two cases of cystic fibrosis are identical.
The death rate among people with cystic fibrosis is intrinsically linked to the severity of the genetic phenotype of the disease but it is also closely correlates to the number of chronic lung infections that a patient would receive during their life time, according to Godfrey Fletcher chief executive of the CFAI.
A serious cause for concern is the fact that many cystic fibrosis patients are being treated in units with few if any isolation facilities, putting them at risk of picking up infections from other patients.
International requirements stateclearly that people with cystic fibrosis have unique infection control issues and should be looked after in single en suite rooms, says Dr Charles Gallagher, consultant in respiratory medicine, St Vincent's Hospital and chairman of the medical and scientific council of the CFAI.
The reality in Ireland is that an acutely sick cystic fibrosis patient going into hospital today will have an "extremely small chance" of getting an en suite single room, he says.
Services and facilities for cystic fibrosis suffers in Ireland remain well below acceptable international standards, he says.
In February 2005 a report compiled by UK consultant Dr Ronnie Pollock concluded that the majority of Irish adults with cystic fibrosis are being treated in facilities that are "seriously inadequate".
The situation at the National Adult Referral Centre St Vincent's Hospital was described as bleak and the lack of isolation facilities there was deemed dangerous.
The creation of a specific cystic fibrosis treatment centre at St Vincent's is part of the current Health Service Executive (HSE) hospital redevelopment programme, however as yet no confirmed funding or start date has been fixed for this work. A spokeswoman for the Eastern Region Health Authority said a dedicated unit forms part of the ongoing development at St Vincent's and is still at the planning stage.
This year, the Minister for Health Mary Harney allocated €4.78 million in ongoing funding in the 2006 Budget estimates to increase specialist staffing needs across the State.
This is a step in the right direction, but is only one-third of what is needed, according to Dr Gallagher.
Cystic fibrosis services are provided in 13 locations around the State. At present, there are 3.5 consultants treating more than 1,100 patients, when there should be 29. A further two are due to be appointed in a month.
There are only 40 professional cystic fibrosis staff, nurses, dieticians, social workers etc, in the State when there should be 175, adds Dr Gallagher.
At the National Adult Referral Centre there is just one consultant to treat 60 per cent of the adult cystic fibrosis population in the State.
In many situations patients are admitted to hospital for treatment via A&E because there are no beds available on the wards. Waiting sometimes for 24 hours to be admitted, they are exposed to serious risk of infection.
Once patients make it to the wards serious overcrowding means that cystic fibrosis sufferers are forced to share wards and bathrooms with other respiratory disease patients and in some cases adults are treated in paediatric wards. All ideal scenarios for cross-infection.
An investment of between €30-50 million would guarantee proper facilities, single rooms with en suites, in all 13 cystic fibrosis centres as well as a 30-bed unit and centre in St Vincent's, Fletcher says.
A HSE working group established to review the delivery of services for cystic fibrosis patients in Ireland will publish a report shortly.
It will make specific recommendations regarding a range of service improvements required for persons with cystic fibrosis, in particular the need to increase the level of clinical, nursing and allied health professional staffing in units, according to a spokesperson for the Department of Health.
• Cystic Fibrosis Awareness Week runs until Saturday. For further information contact the Cystic Fibrosis Association of Ireland on 01-496 2433. Donations to the association can now be made via your mobile phone. Just text CF to 57800 and a donation of €5 will automatically be given to the association.
Cystic fibrosis explained
• A genetically inherited disease, cystic fibrosis primarily affects the lungs and digestive system, but can also damage other organs including the pancreas, liver and reproductive system.
• In the lungs it causes a build-up of mucus, which leads to a cycle of lung infections and inflammation. The build-up of mucus in the pancreas can also make it difficult to digest and absorb food.
• It is a recessive gene. Parents who are carriers of the cystic fibrosis gene have a one in four chance of having a child with full-blown cystic fibrosis.
• Treatment includes consumption of pancreatic enzymes with food, daily chest physiotherapy, nutritional supplements, tube feeding, antibiotic treatment and organ transplant.
• The severity of the illness can vary. Generally it gets progressively worse with age and becomes increasingly more difficult to manage.
• Respiratory infections are the main clinical problems and leading cause of death. The average age of death is around 34.
• Research suggests that carriers of cystic fibrosis with no symptoms have better survival rates against other gastric diseases. The scientific community believe it is possible that the extremely high incidence rate in Ireland dates back to famine times. Then gastric illnesses were rife due to malnutrition and carriers of the cystic fibrosis gene survived better because of this increased immunity to disease.