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Sickle Cell & Thalassaemia

Checked: 04-06-2024 by Vicky Ryan Next Review: 03-06-2026

Nurse support

UHBW provide a dedicated nurse based at Bristol Haematology and Oncology Centre to offer care and support to patients with sickle cell and thalassemia and their carers.

Refer by contacting:

Nicole Paterson on 0117 34 22774 or mobile 0782 594 8038 (Monday to Friday, 9am to 5pm)

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

Haemoglobinopathy Guidelines

Affected patients:

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

Sickle cell disease (HbSS, HbSC, HbSΒthalassaemia and other compound heterozygotes)
Β thalassaemia major
Β thalassaemia intermedia
HbH disease (α thalassaemia)
Diamond Blackfan anaemia. 

Please refer via eRS.

Alternatively you can request Haemoglobinopathy advice & guidance via eRS:

Haemoglobinopathy Advice - BHOC - UHBW - RA7

Priority - Routine
Specialty - Haematology
Clinic - Haemoglobinopathies & Red Cell Disorders

Urgent advice can also be obtained from the nurse help line above or haematology SpR on call via switchboard on 0117 923 0000.

Unaffected carriers of haemoglobinopathies:

Carrier status may be suspected because of family history, ethnicity, or FBC abnormalities which are not acquired. In general, these patients do not need to be referred to haematology. National screening is routinely done for neonates and pregnant women.

It may be important to diagnose asymptomatic carriers because:

Partner testing with prenatal and antenatal genetic counselling may be indicated. This can be arranged through Nicole Paterson (sickle and thalassaemia nurse specialist) 0117 3422774. Partner testing is warranted for asymptomatic carriers of HbS, C, D, E, O, βo, β+, αo. This is because HbS can combine with HbS, HbC, βo, β+, (More rarely with: HbDPunjab, HbO-Arab) to result in sickle cell anaemia, αo-thalassaemia can combine with αo (More rarely can combine with nondeletional a+ (e.g. aCSa/) to result in HbBarts Hydrops and Beta thalassaemia major or intermedia can result from various combinations of βo, β+, E and more rarely βo and Hb lepore thalassaemia (fusion of & β genes).
Results may explain FBC abnormalities, avoiding unnecessary further investigations and ineffective or harmful treatment.

Primary Care information

Please see the attached Primary Care information on Sickle Cell Disease.

This includes information on:

risk of infection
managing pain
complication
sexual health and contraception

Please see the attached Primary Care information on Thalassaemia and rare inherited anaemia.

This includes information on:

health issues associated with thalassaemia
treatments
sexual health and contraception

Efforts are made to ensure the accuracy and agreement of these guidelines, including any content uploaded, referred to or linked to from the system. However, BNSSG ICB cannot guarantee this. This guidance does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer, in accordance with the mental capacity act, and informed by the summary of product characteristics of any drugs they are considering. Practitioners are required to perform their duties in accordance with the law and their regulators and nothing in this guidance should be interpreted in a way that would be inconsistent with compliance with those duties.

Information provided through Remedy is continually updated so please be aware any printed copies may quickly become out of date.