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The increasing prevalence of childhood sickle-cell disease in Ireland
C McMahon1, C O Callaghan, D O'Brien
1National Children's Hospital, Tallaght, Ireland.
Insights
Ireland
Area of Science:
- Paediatric Haematology
- Genetic Blood Disorders
Background:
- Sickle-cell disease (SCD) is rare in Ireland, with no established care policy.
- Increasing refugee numbers necessitate a review of SCD prevalence and care needs.
Purpose of the Study:
- Determine childhood SCD prevalence in Ireland.
- Predict requirements for a comprehensive SCD care and assessment program.
Main Methods:
- Retrospective analysis of children with sickle-cell crisis (Jan 1999-Apr 2001).
- Assessment of haemoglobinopathy phenotype nature and severity.
Main Results:
- 92 patients with haemoglobinopathy registered; 23 with SCD.
- Majority of patients from Nigeria, Angola, and Congo.
- 32 sickle-cell crises observed, primarily haemolytic or splenic sequestration.
Conclusions:
- Rising SCD cases due to increased refugee populations require a comprehensive care approach.
- Care strategies should mirror those for paediatric haemophilia for optimal outcomes.
Background:
Ireland has been relatively free of sickle-cell disease (SCD) and a care policy for the disease has not been established.
Aim:
To determine the prevalence of childhood SCD in Ireland and to predict requirements for a comprehensive care and assessment programme.
Methods:
We retrospectively analysed the data of children with SCD presenting with sickle-cell crisis to one institution from January 1999 to April 2001. We also determined the nature and severity of the presenting haemoglobinopathy phenotype.
Results:
Ninety-two patients with haemoglobinopathy have been registered with the Paediatric Haematology Service. The majority are from Nigeria with a smaller number from Angola and the Congo. Sixty have sickle-cell trait, 23 SCD, four haemoglobin SC disease and two haemoglobin E (HbE). There have been 32 sickle-cell crises. The majority were haemolytic or splenic sequestration events with a smaller number of aplastic and vaso-occlusive events and one osteomyelitis.
Conclusion:
The increasing number of children presenting with SCD as a result of the increasing refugee numbers requires a comprehensive care approach similar to that required for paediatric haemophilia to ensure optimum care.
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