Information on the management of sickle cell and thalassaemia for primary care

Sickle cell disease (SCD) and thalassaemia are prevalent inherited blood disorders in the UK, with an estimated 15,000 individuals affected by SCD and approximately 2,300 by thalassaemia.

These conditions predominantly impact individuals of African, Mediterranean, Asian, or Middle Eastern descent. Primary care practitioners play a crucial role in the early identification, management, and ongoing support of patients with these haemoglobinopathies.

St George’s is the specialist haemoglobinopathy team for patients with sickle cell and thalassaemia in the SW London area and parts of Surrey, Sussex and Hampshire.

We support the local haemoglobinopathy teams at Epsom and St Helier’s Hospital, Frimley Park Hospital, Royal Surrey County Hospital, East Surrey Hospital, Ashford and St Peters Hospital and Kingston Hospital.

Patients living in our region will be seen at St George’s at least once a year for their annual review and will have access to our specialist nursing and psychology services. We provide transfusion therapy at St George’s and support local hospitals with other treatments such as hydroxycarbamide, iron chelation and consideration of new therapies.

All patients known to SGUH will have a universal care plan.

St George’s contact details

Sickle Clinical Nurse Specialist (CNS):

Tel: 07825 978812, Email: haemoglobinopathy.nurses@stgeorges.nhs.uk

Haematology secretaries:

Tel: 020 8725 0885, Email: haematology.secretaries@stgeorges.nhs.uk 

Sickle emergencies

Patients with sickle cell disease may often need admission to hospital

Please do contact the team for advice above but in the case of an emergency please do send to the emergency department.

Key considerations in patients with sickle cell disease and thalassaemia

• Acute painful crises – may not be manageable with oral analgesia at home and admission required
• Acute chest syndrome – sickling in lung leading to hypoxia and needs urgent treatment and consideration of transfusion
• Acute anaemia (especially in context of some infections such as parvovirus causing aplasia)
• Overwhelming sepsis – patients with sickle cell and those who have had splenectomy are immunocompromised and may need more aggressive treatment
• Stroke – patients with sickle cell disease are at increased risk of stroke from childhood. Any neurological concern in a patient with sickle cell disease should be treated as an emergency
• Priapism – sickle cell disease is a cause of priapism and patients should be advised to attend the emergency department if the condition lasts for more than 2 hours or is unmanageable at home

Key Information for Primary Care

Vaccinations

Adults with SCD who have not received primary vaccination as part of the national schedule in the UK should be offered:

• One dose of Hib/Men C and one dose of pneumococcal polysaccharide vaccine (PPV23); followed by,
• One dose of MenACWY conjugate vaccine one month later
• Two primary doses of MenB vaccine one month apart [this can be at the same visits as the other vaccinations above]
• A single 0.5 ml dose of pneumococcal conjugate vaccine (PCV13) which should be given at least six months after PPV Adults with SCD should also be offered:
• Pneumococcal polysaccharide vaccination (PPV23) at five yearly intervals ·

Annual influenza vaccination

• Hepatitis B vaccine if they have not previously received it and are non-immune

Please check the green book for up to date advice for example Covid-19 boosters

Patients who have had a splenectomy

• Vaccinations as per the green book

Regular medications

All patients with sickle cell disease should be offered penicillin V 250mg bd prophylaxis unless contraindication or concern (please discuss with sickle cell team)

All patients with sickle cell should be on regular folic acid 5mg daily

Please consider regular analgesia on prescription if needed

Travel vaccinations and antibiotics

Patients should be encouraged to seek travel advice and to accept all the offered immunisations relevant to the area to which they are travelling; this includes live vaccines such as yellow fever.

There is a common misconception among patients – and some healthcare professionals – that people with sickle cell disease do not get malaria infections; this is incorrect, and malaria infection can be very serious and fatal. Patients with SCD should receive malaria prophylaxis when travelling to malarial areas, in line with general guidance for the area of travel.

All chemoprophylactic agents are acceptable in patients with SCD although it should be noted that there is an increase of glucose-6-phosphate-dehydrogenase (G6PD) deficiency in this patient group and in these patients certain agents should be avoided.

Blood pressure monitoring

Patients with SCD generally have a lower mean blood pressure (BP) than age and sex matched · controls

Hypertension in SCD should be monitored and treated in primary care.

Patients with hypertension and ACR <3.5mg/mmol should be treated with a BP target of 140/90mmHg. Patients with hypertension and ACR >3.5mg/mmol should be treated with a BP target of 130/80mmHg

Vitamin D and bone health

Vitamin D levels should be measured at least annually in all patients with SCD. ·

Patients who have vitamin D insufficiency or deficiency should be started on appropriate vitamin D replacement.

If there is any evidence of pathological fracture, bone mineral densitometry (BMD) assessment should be carried out.