MANAGEMENT OF SICKLE CELL CRISIS

 BASILDON AND THURROCK HOSPITAL GUIDELINES (Revised Feb 2001)

The following guidelines apply to adult patients with sickle cell disease who present in painful crisis.  The main objectives are the early institution of adequate analgesia, the correction of dehydration if present and treatment of any underlying illness (eg infection).

1.  A rapid assessment of identity of the patient and severity of the pain should be made.  (Many patients will carry a haemoglobinopathy identification card which will assist in this respect).

2. Intramuscular (IM) dose of morphine (or diamorphine) 5-15mg should be given immediately (assuming no obvious contraindications).  This should be repeated at 60-120 minute intervals according to the response.  (NB.  maximum dose schedule of morphine should not exceed 20mg/hr).  Certain patients will have haemoglobinopathy cards with guidelines as to recommended loading and maintenance opiate schedules based on previous admissions.

3. If the patient is able to hydrate orally and does not specifically require intravenous drug therapy, then the siting of an intravenous drip is unnecessary and may be counter-productive: the prolonged application of a tourniquet may induce sickling in the affected limb and drip sites may become a source of infection.  Alternative methods of rehydration including sub-cutaneous fluids and nasogastric fluids should be considered.

4. After the patient’s pain has been brought under control, a concise and relevant history and examination should be undertaken with particular relevance to the identification of underlying precipitating factors such as chest infection, urinary tract infection, dehydration, over exertion, exposure to cold etc.

5. Blood should be sent for urgent FBC, reticulocyte count, U&E and group and save in all cases.  Possible causes for an unexpectedly low Hb include bleeding, sequestration crisis, haemolytic crisis and aplastic crisis (ie Parvovirus infection associated with a low reticulocyte count).

6. Blood cultures should be taken if the patient is pyrexial.

7.  A midstream urine sample (and sputum if available) should be sent to Microbiology.

8.  There is no need to administer inhaled oxygen if there is no respiratory compromise and the arterial oxygen saturation is normal.

9.  A chest X-ray is necessary only where there are definite respiratory symptoms or signs.  If there are bilateral chest signs or bilateral infiltrate on chest X-ray, or if the oxygen saturation <90% (or Pa0₂ <11) despite 40% inhaled O₂, then the probable diagnosis is ‘Sickle Cell Syndrome’ and an emergency red cell exchange is indicated.  (cf Exchange Red Cell Transfusion Policy).

10. Other indications for emergency red cell exchange/transfusion include: Sickle cerebrovascular crisis; liver/spleen sequestration syndrome; priapism; emergency operation.

We must stress that the above guidelines are no replacement for the clinical judgement of the casualty or the admitting officer.  If particular problems arise, which are outside of the experience of the admitting team, then the haematologist on-call should be contacted by phone for advice. 

Consultants:            Dr Paul Cervi and Dr Eric Watts