Sickle Cell Crisis

Painful Crisis

Severe pain is the most common reason that patients with sickle cell, will attend the ED. The pain can be agonising (and often underestimated by us), we need to act fast to help ease the symptoms

  • Paracetamol & NSAID’s: We should offer these to all patients (if not contraindicated) presenting in painful crisis, as they are good adjuncts to opiate analgesia.
  • Opiates: This is often the mainstay of initial treatment, and depending on the patient and their current symptoms, you may consider; intranasal diamorphine, Oramorph, or IV morphine. (its worth noting that sickle patients may have a tolerance to opiates)
  • Pain Scoring: Patients may need significant amounts of analgesia and its important to record frequent pain scores to help guide treatment.
  • Precipitants: It is important to look for and treat the cause of the crisis
    • Dehydration: both a precipitant and consequence of a crisis, IV access should be established early and rehydrated carefully.
    • Hypoxia: This is not common in painful limb crisis but target SaO2  ≥95%
    • Infection: this is a common cause, as sickle patients are immunocompromised and can have functional asplenia/hyposplenia (despite the size of their spleen). Temp >38°C you should cover with IV Co-Amoxiclav (Call Microbiology if Penicillin allergy)
  • Tests:
    • ALL: FBC, G&S, U&E, LFT, CRP
    • Chest Signs/SaO≥90% on air: CXR, ABG/CBG
    • Fever: Blood Culture
    • Jaundice: Amylase
  • Admission: The majority of patients presenting with painful crisis will need admission, involve the teams early
    • Adults: Haematology (with general medical support)
    • Kids: Paediatrics

Acute Chest Syndrome

Reduced lung volume and basal opacity Radiopaedia [Click Pic 2 Link]
Signs:

  • Fever: Often >38.5°C
  • Tachypnoea
  • Increased Work of Breathing
  • Added sounds: Wheeze/Creps/Bronchial
  • Hypoxia: Predictor of severity
  • CXR infuriates: The clinical picture often precedes the X-Ray findings.

Tests

  • ABG/CBG
  • Venous bloods: FBC, G&S, U&E, LFT, CRP
  • Cultures: Blood, Throat
  • CXR

Management:

  • Early Specialty involvement (Haem/Paeds, +/- ICU)
  • Oxygen (+/- respiratory support): Aim SaO2  ≥95% (do ABG OR CBG)
  • IV Fluid: Careful monitoring as overload could worsen picture.
  • IV Antibiotics: Discuss with microbiologist

 

Abdominal Crisis

Signs:

  • Insidious onset: which can make recognition difficult
  • Abdominal Pain: Generalized tenderness , often with no grading or rigidity
  • Anorexia
  • Distention
  • Constipation
  • Diminished bowel sounds
  • Girdle/Mesenteric Syndrom:
    • Vomiting and Ileus
    • Silent Abdomen
    • Distended small bowel loops on X-Ray

Tests

  • Venous bloods: FBC, G&S, U&E, Amylase, LFT, CRP
  • Cultures: Blood
  • AXR OR US: as indicated
  • CXR & ABG/CBG: if SaO2  ≥90% on air

Management:

  • Early Specialty involvement (Haem/Paeds)
  • Oxygen: Aim SaO2  ≥95%
  • IV Fluid: Careful monitoring.
  • IV Antibiotics: Discuss with microbiologist
  • Measure Abdominal Girth: @umbilicus

 

Aplastic Crisis

Sudden drop in the patients Haemoglobin, is due to Parvovirus B19, there is often a viral prodrome and occasionally classic ‘slapped Cheek syndrome’. Urgent Transfusion MAY be required if Hb <60g/l, – CHECK patient’s baseline Hb and discuss with haematologist for transfusion decision.

  • Venous bloods: FBC + reticulocyte
    , G&S, U&E, LFT, CRP, LDH

 

Splenic/Hepatic Sequestration

This can be insidious or acute, and may be recurrent, however, it can lead to rapid deterioration of the patient, and death. (Splenic sequestration  is more common in children <3yrs)

Signs:

  • Abdominal Pain: Upper abdomen
  • Distention: with hepato/splenomegaly
  • Fever: Sepsis is common
  • Shock

Tests

  • Venous bloods: FBC, G&S, U&E, Amylase, LFT, CRP
  • Cultures: Blood
  • CXR & ABG/CBG: if SaO2  ≥90% on air

Management:

  • Early Specialty involvement: (Haem/Paeds) Transfusions may be needed
  • IV Fluid: Careful monitoring.
  • IV Antibiotics: Discuss with microbiologist
  • Analgesia
  • Oxygen: Aim SaO2  ≥95%

 

Priapism

this is a sustains and painful erection, that if left untreated can lead to long term sequelae, through, ischaemic penile injury and fibrosis. Unfortunately this will effect 9/10 males by the age of 20yrs old, and is a UROLOGICAL EMERGENCY.

  • Fulminant/Acute
    • Pain: Severe
    • Duration: >4hr
    • Penis: Fully erect
    • Urological Emergency
  • Stuttering
    • Pain: Variable
    • Duration: 30min-3hr
    • Penis: May not be fully erect
    • High Risk of Fulminant

Management:

  • Early Management:
    • Urination: this may require a catheter
    • Warm bath
    • Fluids
    • Analgesia
  • Early Treatment fails:
    • Urology referral: as needs assessment for aspiration/injection
    • Haematology/Paediatric referral : As exchange transfusions and medical management may also be required
    • Etilefrine OR Pseudoephidrine: may be useful but should be directed by specialist and pharmacy.

 

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