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Paediatric Sickle Cell & Thalassaemia

Checked: 07-08-2024 by Vicky Ryan Next Review: 06-08-2026

Nurse support

UHBW provides a dedicated haemoglobinopathy service based at Bristol Royal Hospital for Children to offer care and support to patients with sickle cell and thalassemia and their parents/carers.

Refer by contacting:

Paediatric Benign Haematology CNS Team on 0117 342 8721 or mobile 07920545620 / 07747004996 (Monday to Friday, 9am to 5pm)

For non-urgent advice you can also email: paedbenignhaemcns@uhbw.nhs.uk

Haemoglobinopathy Guidelines

Affected patients:

Patients with known Sickling disorders or thalassaemia who have moved to the area should be referred to a specialist haemoglobinopathy clinic. This clinic provides comprehensive multidisciplinary care for patients with:

Sickle cell disease (HbSS, HbSC, HbS/Beta thalassaemia and other compound heterozygotes)
Beta thalassaemia major
Βeta thalassaemia intermedia
HbH disease (most commonly 3 gene deletion α thalassaemia)

The clinic also sees children with other rare inherited anaemias:

Diamond Blackfan anaemia
Congenital Sideroblastic anaemia
Congenital Dyserythropoetic anaemia
Red cell membrane (eg Hereditary Spherocytosis)
Enzyme disorders (eg Pyruvate Kinase deficiency)

Please refer via eRS.

Alternatively, you can request non-urgent Haemoglobinopathy advice via email or above telephone number.

Urgent advice can also be obtained from the CNS team on the number above or from the benign haematology SpR on 07880163927.

Unaffected carriers of haemoglobinopathies:

Carrier status may be suspected because of family history, ethnicity, or FBC abnormalities. National screening is routinely done for neonates and pregnant women.

Unaffected carriers do not usually need to be seen in the paediatric haemoglobinopathy clinic.

Primary Care information on Sickle Cell Disease

Please see the attached primary care information on sickle cell.

This includes information on:

risk of infection
managing pain
complication
sexual health and contraception

Primary Care information on Thalassaemia & RIA

Please see the attached Primary care information on Thalassaemia and RIA.

This includes information on:

regular transfusions
iron overload
complications
sexual health and contraception

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