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GUIDELINES FOR THE MANAGEMENT OF

SICKLE CELL CRISES

These guidelines are produced by The BRITISH ASSOCIATION FOR ACCIDENT & EMERGENCY MEDICINE (BAEM) and are reproduced with their full permission.

You can visit the BAEM website by clicking HERE

GUIDELINES FOR THE MANAGEMENT OF SICKLE CELL CRISES

REMEMBER AT ALL TIMES

The patient or relative generally knows if he/she has sickle cell disease and is usually knowledgeable about the disease. The pain of sickle cell crisis is genuine, can be excruciating, and can be greater than that of childbirth. Sickle cell disease patients often have a higher pain threshold than others.

DISEASE DEFINITION:

Those at risk include patients with an ethnic background from Africa, West Indies, India, the Mediterranean, or the Middle East. Sickle cell crisis may present with a variety of clinical syndromes including severe pain in limbs, chest, abdomen, and/or systemic conditions. The pain is often severe but limb, hip or back pains may be vague in the early stages of an attack. The pain is caused by blockage of small vessels by sickled red blood cells. The immediate result of sickling is a reduction in oxygen delivery to tissues. The local fall in oxygen saturation may be precipitated by a number of factors, such as minor infections.

MANAGEMENT AIMS:

1. Parenteral analgesia within 15 minutes

2. I.V. fluids within 30 minutes

3. Admit to ward within two hours

4. Warmth. Reasurrance

5. Identify the cause of pain

6. Obtain expert haematological advice early and/or contact the patient's base hospital if any of the following is present:

a. shock

b. pregnancy > 12 weeks

c. dyspnoea

d. silent abdomen

e. organ involvement

f. vomiting/dehydration > 10%

g. abdominal pain/distention

h. you are inexperienced in the management of this condition.

TREATMENT OF THE PAINFUL CRISIS:

TRIAGE NURSE

Place on a trolley

Check oxygen saturation

Give oxygen

Wrap in warm blankets if patient feels cold

Triage to resuscitation room if:

· Sa02 < 85%

· Neurological deficit

· Hgb < 5

· Shock

· Organ involvement

· Priapism

Obtain an interpreter if needed

Ask for urgent medical assistance

Check Sickle Cell Disease Register if available

Contact patient's base hospital for information

A&E DOCTOR

Give high flow oxygen.

Ensure adequate ventilation.

Obtain I.V. access.

Take bloods for F.B.C., reticulocytes, group/save, urea/electrolytes, viral titres, blood cultures.

Commence I.V. infusion. (1 litre 0.9% NaCl over 3 hours in an adult)

Do not x-ray sites of pain.

Give amoxycillin 500 mgs and penicillin G 1200 mgs I.V In case of allergy give chlarithyromycin/erythromycin and seek advice.

Give I.V. morphine except in case of allergy/pregnancy/liver disease. (seeking advice regarding liver disease)

Body Weight Dose of Morphine:

30-50 kg 5mg

>50 kg 10mg

If unable to obtain I.V. access, give morphine 0.1 mgs. kg IM/SC Repeat after one hour if required. If morphine contra-indicated, seek advice urgently.

DO NOT USE PETHIDINE ROUTINELY.

SECONDARY SURVEY:

There are certain recognised syndromes and complications which may occur during sickle cell crises.

CHEST SYNDROMES

Suspect from chest pain, tachypnoea chest wall tenderness and Sa02 < 95%. Often misdiagnosed as clinical signs and chest x-ray changes may present late. Look for signs of coincidental ilieus. Monitor arterial gases: If pA02 < 9 kp contact anaesthetist, consider CPAP. If pA02 < 8 kp ask haematologist about possible exchange transfusion.

NEUROLOGICAL

Strokes, fits and T.I.A.'s can present with no associated pain. Discuss these with a haematologist.

INFECTIONS

Prone to pneumococcus and parvovirus B.19 especially if hyposplenic. Pyrexia may be only sign. Do blood cultures, sputum cultures, throat swab, M.S.U./urinalysis. Discuss with Consultant Microbiologist and Haematologist.

ABDOMINAL SYNDROME

Usually pain with no peritoneal signs and normal bowel sounds. Sometimes like surgical abdomen. Do abdominal x-rays and serum amylase. Keep nil by mouth and monitor carefully. If the patient is on desferrioxamine and develops diarrhoea, stop the drug. Check yersinia titre, and treat if positive.

APLASTIC CRISIS

Characterised by: High output CCF Decreased haematocrit and reticulocytes Associated with parvovirus B.19 infection. Siblings may be source of parvovirus Senior Haematology opinion essential.

SEQUESTRATION

Presents with severe anaemia Age < 5 years generally splenomegally. Age > 5 years generally hepatomegally. May need urgent transfusion. Seek immediate advice from a Consultant Haematologist.

MANAGEMENT OF CHILDREN:

Seek expert assistance early. I.V. access may be difficult; start oral rehydration immediately. Give paracetamol early orally/rectally. Morphine sulphate SC as follows: Up to 1 month - 0.150 mg/kg; 1 to 12 months - 0.200 mg/kg; 1 - 12 years - (0.1 - 0.2 mgs/kg). Guide to weight in kg for children aged less than 10 years = (age + 4 ) x 2. Provide good emotional support for the child and parents. Some children may appear angry and rebellious but try and imagine what they have been through.

PITFALLS:

· Do not under-estimate the requirement for pain relief.

· Avoid damage to veins if I.V. access is very difficult and take patient's advice on the best vein.

· If I.V. access impossible, try N.G. or rectal fluids.

DEFINITIVE CARE:

Admit to medical/paediatric ward for ongoing treatment and further haematological opinion.

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These guidelines are produced by The BRITISH ASSOCIATION FOR ACCIDENT & EMERGENCY MEDICINE (BAEM) and are reproduced with their full permission.

You can visit the BAEM website by clicking HERE