Who to refer
Patients with known sickling disorders or thalassaemia who have moved to the area (e.g. university students) should be referred to the specialist haemoglobinopathy clinic at UHBW. This clinic provides multidisciplinary care for patients with: sickle cell disease (HbSS, HbSC, HbSβ thalassaemia and other compound heterozygotes), β thalassaemia major, β thalassaemia intermedia and HbH disease (α thalassaemia).
Unaffected carriers of significant haemoglobinopathies:
Carrier status may be suspected because of family history, ethnicity, or FBC abnormalities. In general, these patients do not need to be referred to haematology. Haemoglobinopathy screening is routinely undertaken for pregnant women and for neonates.
It may be important to diagnose asymptomatic carriers because:
- Partner testing with prenatal and antenatal genetic counselling may be indicated. Discuss with Sickle Cell and Thalassaemia co-ordinator.
- Results may explain FBC abnormalities, avoiding unnecessary further investigations and ineffective or harmful treatment
- Rarely, in very extreme circumstances carriers may become symptomatic (in sickle cell trait).
Please see the Sickle Cell & Thalassaemia Clinical Nurse Specialist - UHBW page of Remedy
See BSH guidelines:
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