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Information ⬇

Author: Jo Howard

Consider painful sickle cell crisis if there is acute pain in the spine, abdomen, chest or joints in a patient of Afro-Caribbean, Arabic or Indian origin. Management is summarized in Figure 104.1.

Priorities ⬆ ⬇

  1. Make the diagnosis
    • Most patients with sickle cell disease who are having an acute painful crisis will recognize their pain as typical in character and location. If the patient says the pain is unlike their usual sickle pain, consider alternative diagnoses. See the relevant chapters for other causes of acute pain in the chest (Chapter 7), abdomen (Chapter 21), joints (Chapter 28), spine (Chapter 29) and limbs (Chapter 30).
    • If the patient does not have a prior diagnosis of sickle cell disease, confirm the diagnosis with a full blood count, blood film and high-pressure liquid chromatography (HPLC) or haemoglobin electrophoresis. Patients with HbSS will usually have Hb 60–90g/L, but in other genotypes it may be 90–130g/L. The blood film will show sickle cells, target cells and irregularly contracted or boat cells. Sickle solubility test is positive in sickle trait as well as in sickle cell disease, so if negative will exclude the diagnosis, but if positive does not confirm it.
  2. Relieve pain
    • Establish the patient's previous requirement for analgesia and how much has been taken in the past 24h. Most patients will know their usual analgesic regimen or have an individual pain protocol.
    • Offer pain relief within 30 min of arrival at hospital (as per NICE guidance). Monitor the level of pain with an age-appropriate pain assessment tool.
    • For severe pain or for moderate pain which has not responded to initial analgesia, offer a bolus of strong opioid, for example morphine 5–10 mg SC, IV or PO; oxycodone 2.5–5 mg can be used if morphine-intolerant, with monitoring of oxygen saturation and sedation score.
    • For moderate pain where the patient has not yet received any analgesia offer a weak opioid (e.g. codeine).
    • All patients should be offered regular paracetamol, NSAID and weak opioid unless contraindicated.
    • Do not use pethidine for treatment of acute painful sickle cell crisis because of the risk of fits.
  3. Look for precipitating factors and treat these
    • Focus the history and examination on detecting evidence of infection, as this is the most common precipitating factor. Patients with sickle cell disease are effectively splenectomized and thus at particular risk of infection with encapsulated bacteria: pneumococcus, meningococcus and H. influenzae type B.
    • Low-grade fever may occur without infection (reflecting tissue necrosis).
    • Take appropriate microbiological samples (blood, urine and sputum samples and viral swabs), other blood tests and arrange a chest X-ray (Table 104.1).
    • Antibiotic therapy should be started after cultures have been taken. Use a broad-spectrum penicillin and macrolide if there are chest symptoms; antibiotic choice should depend on local microbiology advice.
    • Other precipitating factors include stress, cold weather and dehydration.
  4. Refer to a haematologist for urgent advice if the patient has:
    • Chest, abdominal or neurological symptoms (Table 104.2)
    • Priapism
    • Pain which is atypical or does not respond to usual analgesia

Further Management ⬆ ⬇

Outline

Titration of Analgesia!!navigator!!

  • Assess adequacy of pain relief using a pain assessment tool every 30 minutes until pain is relieved and at least four-hourly thereafter. If the patient has ongoing severe pain, offer repeated bolus doses of analgesic.
  • Consider Patient Controlled Analgesia (PCA) if repeated bolus doses of analgesic are needed.
  • Consider a long-acting opioid (e.g. morphine sulphate modified release).
  • Monitor the patient for adverse effects of opioid with repeat observations (including sedation score) hourly for first six hours and at least four-hourly thereafter.
  • Refer for haematology review.

Supportive Care!!navigator!!

  • Monitor the patient for other sickle complications, including acute chest syndrome, throughout hospital admission.
  • Give an oral laxative, antiemetic and antipruritic while receiving opioid analgesia.
  • Consider venous thromboembolism risk and prescribe appropriate prophylaxis.
  • Fluids can be taken orally in most patients. Aim for an intake of 3L/day (in adults). IV fluids should be given if the patient has clinical signs of dehydration, vomiting, diarrhoea, abdominal pain or is unable to take adequate oral fluids.

Blood Transfusion!!navigator!!

  • Blood transfusion is not usually indicated for treatment of a simple painful crisis.
  • A decreased haemoglobin (below the patient's baseline) may be caused by increased haemolysis, splenic sequestration (raised reticulocyte count) or aplastic crisis (parvovirus infection, decreased reticulocyte count).
  • Do not transfuse without discussion with a haematologist.
  • If blood is required please let the transfusion laboratory know that this is for a sickle cell patient and ensure that the blood is matched for full Rh and Kell type.

Reducing Analgesia!!navigator!!

  • Reduce doses of analgesia once pain is under control.
  • Introduce oral analgesics as soon as possible, at least 24h before discharge.

Further Reading ⬆

Howard J, Hart N, Roberts-Harewood M, et al. on behalf of the BCSH Committee (2015) Guideline on the management of acute chest syndrome in sickle cell disease. British Journal of Haematology , 169, 492–505. http://onlinelibrary.wiley.com/doi/10.1111/bjh.13348/epdf.

National Institute for Care and Health Excellence (2012) Sickle cell disease: managing acute painful episodes in hospital. Clinical guideline (CG143). https://www.nice.org.uk/guidance/cg143.