Dr. Eibhlin Conneally:

There is significant variability in terms of sickle cell disease. Sickle cell disease does not affect some people's day-to-day life that much, but others suffer huge disability and incapacity as a result. This makes matters difficult in the context of who should have a bone marrow transplant. Obviously, we would not do a transplant for someone who has a relatively mild phenotype, whereas someone with a severe phenotype would need it.

In regard to life expectancy, the median life expectancy in the United States is only in the mid-40s, but the black community is over-represented in sickle cell community in the US; it is probably more socially disadvantaged. In Jamaica, where the care is probably better, the life expectancy is better than in the US, but it is still nowhere near normal life expectancy. We do not propose every hospital in the country should have a sickle cell disease treatment unit. Currently, sickle cell care is provided through the network of haematologists, but there is not a haematologist in every hospital. For example, if someone attends Portlaoise hospital, that person will be referred to Tullamore hospital where there is a haematologist. What we suggest should happen is that there would be an adult centre and that all those with sickle cell disease who would be initially in the paediatric centre could then transition to the adult centre. If they were well, we could see them on an annual or biennial basis, but if they were ill, we could see them more frequently. The primary care and day care could then be provided in their local hospital through the local haematologists. This is how we would see care being provided. This same mechanism is in existence for haemophiliacs. If we kept electronic patient records, the local hospital would know exactly what to do for the specific patient and could contact the national centre, paediatric or adult.

Currently, there is no commitment to an adult service, but there is awareness of the need for such a service. St. James's Hospital has the biggest haematology department in the country and is the national adult bone marrow transplant centre. Given the paediatric hospital will move to the St. James's site, it is the obvious choice for a service for those with sickle cell disease. St. James's Hospital also provides the regional apheresis service, where cells are withdrawn and replaced with normal red cells. Therefore, for many reasons St. James's Hospital is the rational choice for the service. There is support for this at CEO level, but the creation of a service is dependent on resources.

A question was asked about bone marrow transplantation. The committee will be pleased to hear we provide the unrelated bone marrow transplantation for the North of Ireland in Dublin. Therefore, the resource is coming the other way. This is for leukaemia or lymphoma transplantation, as transplantation has not been a realistic option for adult sickle cell disease patients.