HNY Policy and Pathway Repository - North Yorkshire

The haematology department at South Tees NHS Foundation Trust provides specialist care for all people with clinically significant haemoglobinopathies; this includes all patients with sickle cell disease (HbSS, HbSC, etc) or thalassaemia major (B thalassaemia, HbH disease or transfusion dependent thalassaemia). As this is a low prevalence area, the majority of care relating to the haemoglobin disorder is provided within secondary care. This guideline aims to cover information for general practitioners in helping to care for this group of people.

None provided

Patients who are carriers of a haemoglobinopathy e.g. sickle cell carrier, carrier of B thalassaemia trait do not require secondary care follow up. At diagnosis these patients will be sent an information leaflet via the requesting clinician which explains the condition. If further advice is required please use Advice and Guidance available through the Choose & Book system. Newborn screening results will be communicated by the newborn screening coordinator.

Prevention of Infection

All patients with sickle cell disease are considered to be hyposplenic. Some patients with thalassaemia major will have had a splenectomy. Guidelines for management of hyposplenism should be followed for these patients.

Guidance on antibiotic prophylaxis and vaccinations can be found in the DOH Green Book.

1. Prophylactic penicillin

• All patients should receive prophylactic penicillin or equivalent. This will be initiated in the paediatric clinic at the initial consultation, but should be continued on a regular prescription in primary care.
  
  Penicillin dosage

  Age	Dose of penicillin
  3 months 1 year	62.5mg twice daily
  1 year 5 years	125mg twice daily
  5 years - adult	250mg twice daily

• If patients do not wish to take regular penicillin they should have an emergency supply of antibiotics at home to take in the event of infection (e.g. amoxicillin 250mg tds).
• In the event of penicillin allergy, prophylactic erythromycin may be prescribed, although is not as effective and patients over 16 years may wish to consider using emergency antibiotics instead.

2. Vaccinations

• All patients should be encouraged to receive vaccinations according to the standard UK childhood immunisation schedule, including meningitis vaccines. People with haemoglobin disorders should receive the following additional vaccinations:
• Pneumovax PCV23 at age 2 years. This is repeated 3 years after the initial vaccination (aged 5 years) and five yearly thereafter.
• Hepatitis B
• Annual influenza vaccine

3. Travelling

• Patients receiving hydroxycarbamide should not receive live vaccines eg. yellow fever.
• Patients are at increased risk of malaria infection and it is important that they take appropriate anti-malarials when travelling to an 'at risk' area.

Pain Management

People with sickle cell disease may experience frequent episodes of severe pain relating to sickling crises. We encourage patients to manage their crises at home, where possible. Patients are advised to drink 1.5 2 x daily maintenance fluids (for an adult this will be 3-4 litres) and to take analgesia using an analgesic ladder according to the severity of the pain. Patients will usually take paracetamol and ibruprofen initially, but require access to opiate analgesia at home for more severe pain this will usually be Oramorph or equivalent. Pethidine should be avoided. If a patient presents with severe pain they should receive analgesia according to their emergency health care plan (of which they should have a copy).

Hydroxycarbamide

Hydroxycarbamide has been shown to reduce mortality and morbidity is both adults and children with sickle cell disease by reducing the frequency of chest and painful crises. It is currently offered to patients with recurrent painful crises, chest crises, raised transcranial Doppler velocities and more recently to children. Hydroxycarbamide works by increasing the percentage of haemoglobin F and changes in nitric oxide metabolism which reduce haemolysis and increase the baseline haemoglobin level.

In South Tees NHS Hospitals hydroxycarbamide is prescribed and monitored by the haematology team. General practitioners will not be expected to be involved in the prescribing of hydroxycarbamide, but should be aware of the potential side effects and actions to take in the event that a patient presents to primary care.

Potential presentations and clinical actions required:

Pregnancy or breast feeding	Stop drug
Family planning	Men and women should use safe contraceptive methods whilst taking hydroxycarbamide and for 3 months after stopping. Patients who want to father a child or get pregnant should stop hydroxycarbamide for at least 3 months prior to conception
Blistering rash	Stop drug and contact haematology team
Alopecia	Usually mild, stop only on patient request
Hyperpigmentation of nails	Usually mild, stop only on patient request
Fever and hepatitis	Stop drug and contact haematology team
Sore throat or mouth ulceration	Stop drug and contact haematology team
Cytopenias (Hb <45g/L, neutrophils <1.0, platelets<80)	Stop drug and contact haematology team

 

Iron Chelation

People with haemoglobin disorders often require blood transfusions and can become iron overloaded as a result of this. Iron overload is monitored in secondary care using ferritin, transferrin saturation and T2* MRI of the liver and heart. Those with evidence of iron overload will be prescribed iron chelation. In this region all iron chelation is prescribed and monitored in secondary care. Commonly used medications are:

• Deferasirox (Exjade FCT, oral preparation)
• Desferrioxamine subcutaneous or intravenous infusion

Side effects which may present to primary care:

Fever, abdominal pain and diarrhoea	Consider Yersinia infection. Stop iron chelation and prescribe ciprofloxacin 500mg bd
Skin reactions	Topical corticosteroid may be helpful in reducing local erythema
Tinnitus or worsening hearing loss	Withhold chelation and discuss with haemoglobinopathy team
Visual disturbance (night blindness or central scotoma)	Withhold chelation and discuss with haemoglobinopathy team
Drug interactions	Avoid phenothiazines due to increased risk of neurological toxicity
Abnormal blood tests: raised transaminases or cytopenias	Withhold chelation and discuss with haemoglobinopathy team

None provided

Haematology Handbook - South Tees Hospitals NHS Foundation Trust
 

Clinical Team

• Dr Dianne Plews (lead clinician, consultant haematologist)
• Dr Jonathan Lane (paediatric consultant)
• Sr Lindy Defoe (lead nurse / newborn screening)

Open Access & Clinical Advice

All patients with a clinically significant haemoglobinopathy have 24 hour open access to hospital services in the event that they are unwell.

Adults - Haematology Day Unit, JCUH. 01642 282763 (9am 5pm, Monday Friday) Ward 33, JCUH. 01642 835996 (out of hours)

Children - CYPED, JCUH. 01642 854896 (24 hours)

If a patient presents to primary care acutely unwell please contact the open access numbers. Patients should not be advised to attend A&E unless they have been involved in acute trauma or have central chest pain which seems cardiac in origin.

Urgent clinical advice for both adult and paediatric patients can be obtained from the haematology registrar on-call (via hospital switchboard 01642 850850). For non urgent queries please use the Advice and Guidance choose service or telephone Dr Plews' secretary if the patient is known to the service (01642 854381).

• South Tees Hospitals