The increasing prevalence of childhood sickle-cell disease in Ireland.
BACKGROUND
Ireland has been relatively free of sickle-cell disease (SCD) and a care policy for the disease has not been established.


OBJECTIVE
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.


CONCLUSIONS
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.
National Children's Hospital, Tallaght, Ireland.
C
C
McMahon
C O
CO
Callaghan
D
D
O'Brien
O P
OP
Smith
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006450:Hemoglobin SC Disease / Q000175:diagnosis / Q000453:epidemiology*; D006451:Hemoglobin, Sickle; D006801:Humans; D007223:Infant; D007494:Ireland / Q000453:epidemiology; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies
D006451:Hemoglobin, Sickle