Some patients with sickle cell disease (SCD) are at increased risk of “life-changing complications” due to lack of access to chronic blood transfusion programmes at St James’s Hospital in Dublin.
St James’s provides the only specialist service in Ireland for adults with SCD, a life-threatening inherited blood disorder. However, it has insufficient ambulatory care and laboratory capacity to provide transfusion programmes to all patients requiring them, the Medical Independent (MI) understands.
SCD, the commonest haemoglobinopathy, is most prevalent in people of African or Caribbean background. Chronic transfusion is an essential treatment for many patients.
The St James’s service highlighted “urgent” requirements, including sufficient day-ward capacity to transfuse patients, in business cases submitted to the HSE in 2021 and 2022, according to documents obtained under Freedom of Information law.
Consultant Haematologist at St James’s, Dr Emma Tuohy, told MI there is an urgent requirement for an ambulatory care unit and additional medical scientists for the transfusion laboratory to facilitate access to transfusion programmes.
Currently, 12 of her patients are on a waiting list to commence transfusion programmes (one patient with thalassaemia; 11 with SCD).
“There are patients who could develop life-changing complications if I cannot transfuse them,” said Dr Tuohy, who leads the haemoglobinopathy/red cell disorders service at St James’s.
There are just two treatment bays designated to the haemoglobinopathy service, which has approximately 280 patients (255 have SCD). Sixty-four of the patients are receiving chronic transfusion programmes (56 have SCD, the remainder have thalassaemia/rare red cell disorders).
In addition, around 90 young adults aged 18 and over (including around 40 on transfusion programmes) are attending Children’s Health Ireland (CHI) at Crumlin, which has about 380 patients with haemoglobinopathies. The high-risk transition process is managed carefully between the two services. Patients are scheduled to move to the adult service at 18/19 years of age/ after the first year of college. It is understood a large proportion of the over-18s are awaiting immediate transfer to St James’s, but it does not have the requisite capacity.
CHI Crumlin and St James’s are the only national comprehensive services with expertise in management of haemoglobinopathies. They are EU-recognised expert centres for haemoglobin/red cell disorders as part of the Eurobloodnet European Reference Network in Rare Haematological Diseases. However, they are not designated as national referral centres with associated resourcing and a national model of care.
The clinical leads of both services, Dr Tuohy and Prof Corrina McMahon at CHI Crumlin, believe a national clinical programme for haemoglobinopathies is urgently required to provide a national framework for care and raise awareness in the health system.
See news feature Advocating for an Irish model of care in sickle cell disease – Medical Independent
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