Madam, - Many congratulations to Orla Tinsley, Jennifer Tormey, Denise Saul and other families for highlighting the major deficiencies that exist in the adult hospitals they attend for the management of cystic fibrosis (CF), and for their call for urgent action to rectify this intolerable situation.
As the clinician in the multi-disciplinary CF team at Our Lady's Children's Hospital, Crumlin, I am writing to draw attention to the equally important need to improve and enhance medical services and facilities for CF children and adolescents throughout the country, which I hope is not being overshadowed by the crisis in adult CF services.
The treatment of CF in adulthood should not be divorced from its treatment in childhood or adolescence but must be viewed as a continuum with smooth transition from paediatric to adult care. Early and intensive management of CF in children will result in healthier adults with better quality of life and less need for in-hospital care.
It is hoped that, in the future, novel forms of treatment (including gene therapy) will permit more specific and targeted interventions in CF and eventually a definitive cure for the condition. However, these therapies are most likely to be effective in CF individuals with mild lung disease.
This underlines the need for comprehensive treatment of CF with currently available therapies starting at the time of diagnosis and continuing into adulthood.
Of the 1,100 people with CF in this country, approximately 650 (60 per cent) are younger than 18 and are cared for in specialist paediatric units. These young people and their parents require advice and holistic care from multi-disciplinary teams with appropriate skills in catering for the unique needs of babies, children and particularly teenagers. In the past three years, the HSE has made significant investments in these teams, leading to major improvements in the level of care available for CF families.
However, as in adult hospitals, many of our paediatric CF units (including the clinic at this hospital) do not have ring-fenced, dedicated in-patient wards with single en-suite rooms to prevent cross-infection. These need to be established urgently in tandem with the development of new facilities for CF adults.
There has been some mention of the better outlook for CF in Northern Ireland. Genetic differences and the fact that CF care is centralised to well-resourced units in Belfast might partly explain this. The other major difference is that since 1983 all new-born babies in Northern Ireland have been screened at birth for CF via a simple blood test. This allows the diagnosis to be established a few weeks after birth and appropriate treatment commenced.
This approach has been shown to significantly improve the outcome for CF children, particularly in terms of growth, nutrition and long-term development. In the absence of a new-born screening programme, delays in the diagnosis of CF are inevitable. The average age of CF diagnosis in this country is two years and there is a gender gap in that girls are diagnosed later than boys.
It is therefore important to introduce a new-born screening programme for CF without delay. The requirements for implementing such a programme were submitted to the Department of Health and Children in 2003 and a response is awaited. - Yours, etc,
GERARD J CANNY MD,
Cystic Fibrosis Unit,
Our Lady's
Children's Hospital,
Crumlin,
Dublin 12.