Sickle Cell Crisis

OVERVIEW

Sickle cell disease is caused by HbS haemoglobinopathy which produces rigid, distorted and dysfunctional erythrocytes called sickle cells

CAUSE

Types of sickle cell disease

  • sickle cell anemia (usually homozygous SS genotype)
  • sickle beta thalassemia
  • sickle HbC disease

PRECIPITANTS

Commonly:

  • infection
  • dehydration
  • hypoxia
  • drugs (e.g. sedatives, local anaesthetics)

Patients with sickle cell disease are at risk of infection due to underlying immunosuppression

  • functional aspleina typically develops in childhood
  • prophylactic treatment with hydroxyurea can cause neutropenia and cardiomyopathy

PRESENTATIONS

Types of sickle cell crisis presentations:

  • fever
    — consider veno-occlusive disease, acute chest syndrome, osteomyelitis, local or systemic infection
  • vaso-occlusive crisis
    — assume this is the cause of any painful presentations
  • acute chest syndrome
    — life-threatening lung infarction
    — assume if hypoxia + chest pain
  • acute splenic sequestration
    — typically occurs in infants
    — associated with increased spleen size
    — fall in Hb by >20 g/L, thrombocytopenia but normal or increased reticulocytes
  • aplastic crisis
    — fall in Hb as well as reticulocytes <1%
    — triggered by parvovirus infection
  • stroke
  • priapism
    — stuttering (2-4h duration and may be recurrent)
    — severe (>4 hours and may lead to permanent impotence)

ASSESSMENT

Always consider the presence of all types of sickle cell crisis, regardless of the dominant presentation

  • symptoms and signs of local and systemic infection
  • respiratory signs and symptoms
  • increasing spleen size
  • shock and evidence of organ failure
  • baseline and current Hb
  • CXR if fever, chest pain or hypoxia
  • CT Head if stroke suspected
  • consider FBC, reticulocytes, bilirubin, haemolytic screen and cross-match
  • blood film: sickle cells and evidence of haemolysis (e.g. target cells, schistocytes)

MANAGEMENT

Resuscitation

  • correct hypoxia
  • ensure adequate hydration
  • transfuse of symptomatic anemia targeting Hb >50 (avoid over-transfusion e.g. Hb100, due to risks of hyperviscosity or rebound if splenic sequestration)

Specific treatment

  • fever
    — empiric flucloxacillin and gentamicin
  • vaso-occlusive crisis
    — aggressive pain management e.g. paracetamol, +/- NSAIDs, opiates
    — rehydrate with oral or IV fluids according to severity of pain
    — chronic pain may be an issue
  • acute chest syndrome
    — O2 and aggressive pain management (prevent hypoventilation)
    — consider empiric antibiotics in case of pneumonia
    — consider plasmapheresis
  • acute splenic sequestration
    — oxygen, hydration and RBC transfusion (avoid over-transfusion due to rebound when sequestration resolves)
  • aplastic crisis
    — oxygen, hydration and RBC transfusion
  • stroke
    — avoid thrombolytics; avoid hyperviscosity
  • priapism
    — oxygen, rehydration, analgesia
    — may need penile aspiration and washout (consult urology)
    — may need exchange transfusion (consult hematology)

Supportive care and monitoring

Consult hematology

  • most presentations require admission
  • acute chest syndrome, severe anaemia, fluid management issues, severe sepsis usually need HDU/ ICU admission

References and Links

  • Ballas SK. Current issues in sickle cell pain and its management. Hematology Am Soc Hematol Educ Program. 2007:97-105. PMID: 18024616.
  • Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. Radiographics. 2001  Jul-Aug;21(4):971-94. PMID: 11452073.
  • Meremikwu MM, Okomo U. Sickle cell disease. Clin Evid (Online). 2011 Feb 14;2011. PMC3217656.
  • Vijay V, Cavenagh JD, Yate P. The anaesthetist’s role in acute sickle cell crisis. Br J Anaesth. 1998 Jun;80(6):820-8. PMID: 9771314.

CCC 700 6

Critical Care

Compendium

Chris is an Intensivist and ECMO specialist at the Alfred ICU in Melbourne. He is also the Innovation Lead for the Australian Centre for Health Innovation at Alfred Health and Clinical Adjunct Associate Professor at Monash University. He is a co-founder of the Australia and New Zealand Clinician Educator Network (ANZCEN) and is the Lead for the ANZCEN Clinician Educator Incubator programme. He is on the Board of Directors for the Intensive Care Foundation and is a First Part Examiner for the College of Intensive Care Medicine. He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives.

After finishing his medical degree at the University of Auckland, he continued post-graduate training in New Zealand as well as Australia’s Northern Territory, Perth and Melbourne. He has completed fellowship training in both intensive care medicine and emergency medicine, as well as post-graduate training in biochemistry, clinical toxicology, clinical epidemiology, and health professional education.

He is actively involved in in using translational simulation to improve patient care and the design of processes and systems at Alfred Health. He coordinates the Alfred ICU’s education and simulation programmes and runs the unit’s education website, INTENSIVE.  He created the ‘Critically Ill Airway’ course and teaches on numerous courses around the world. He is one of the founders of the FOAM movement (Free Open-Access Medical education) and is co-creator of litfl.com, the RAGE podcast, the Resuscitology course, and the SMACC conference.

His one great achievement is being the father of two amazing children.

On Twitter, he is @precordialthump.

| INTENSIVE | RAGE | Resuscitology | SMACC

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