Difference between revisions of "Vaso-occlusive pain crisis"

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==Background==
 
==Background==
 +
*Common complication of [[sickle cell anemia]], constituting 79-91% of ED visits<ref>Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47</ref>
 +
*Sickled red blood cells obstruct vasculature, causing ischemic injury and pain
 +
 
===Precipitating Factors===
 
===Precipitating Factors===
 
*Stress
 
*Stress
 
*Cold weather
 
*Cold weather
*Dehydration
+
*[[Dehydration]]
*Hypoxia
+
*[[Hypoxia]]
*Infection
+
*[[Infection]]
*Acidosis
+
*[[Acidosis]]
*Alcohol intoxication
+
*[[Alcohol intoxication]]
*Pregnancy
+
*[[Pregnancy]]
 
*Exertional stress
 
*Exertional stress
  
 
==Clinical Features<ref>Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47</ref>==
 
==Clinical Features<ref>Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47</ref>==
 
*Pain
 
*Pain
**Most common manifestation of SCA (79-91% of ED visits)
+
**Lower [[back pain|back]], long bones most commonly affected
**Lower back, long bones most commonly affected
 
  
 
==Differential Diagnosis==
 
==Differential Diagnosis==
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*CBC
 
*CBC
 
**Because anemia can precipitate a crisis, must check for acute hemoglobin drop
 
**Because anemia can precipitate a crisis, must check for acute hemoglobin drop
*Chemistry / LFT / lipase
 
**If abdominal pain, may be cholecystitis, mesenteric ischemia, or perforation
 
 
*Retic count (if aplastic crisis considered - rare in adults)
 
*Retic count (if aplastic crisis considered - rare in adults)
 
**Should be >0.5%
 
**Should be >0.5%
*T&S/T&C
+
*Consider:
*Urine pregnancy
+
**Chemistry / [[LFTs]] / lipase
*[[CXR]]
+
***If abdominal pain, may be cholecystitis, mesenteric ischemia, or perforation
**If cough, shortness of breath, or febrile
+
**T&S/T&C
 +
**Urine pregnancy
 +
**[[CXR]]
 +
***If cough, shortness of breath, or febrile
  
 
==Management==
 
==Management==
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***[[IVF]] bolus may lead to atelectasis, pulmonary edema, and acute chest syndrome. Hyperchloremic metabolic acidosis due to normal saline may further precipitate sickling. <ref> Glassberg, J., Evidence-based management of sickle cell disease in the emergency department.Emerg Med Pract, 2011. 13(8): p. 1-20</ref> <ref> Carden, M.A., et al., Variations in pediatric emergency medicine physician practices for intravenous fluid management in children with sickle cell disease and vaso-occlusive pain: A single institution experience.Pediatr Blood Cancer, 2018. 65(1) </ref> <ref> Carden, M.A., et al., Normal Saline Bolus Use in Pediatric Emergency Departments is Associated with Worse Pain Control in Children with Sickle Cell Anemia and Vaso-occlusive Pain.Am J Hematol, 2019. </ref>
 
***[[IVF]] bolus may lead to atelectasis, pulmonary edema, and acute chest syndrome. Hyperchloremic metabolic acidosis due to normal saline may further precipitate sickling. <ref> Glassberg, J., Evidence-based management of sickle cell disease in the emergency department.Emerg Med Pract, 2011. 13(8): p. 1-20</ref> <ref> Carden, M.A., et al., Variations in pediatric emergency medicine physician practices for intravenous fluid management in children with sickle cell disease and vaso-occlusive pain: A single institution experience.Pediatr Blood Cancer, 2018. 65(1) </ref> <ref> Carden, M.A., et al., Normal Saline Bolus Use in Pediatric Emergency Departments is Associated with Worse Pain Control in Children with Sickle Cell Anemia and Vaso-occlusive Pain.Am J Hematol, 2019. </ref>
 
***There are no randomized controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid.<ref>Okomo U, Meremikwu MM. Fluid replacement therapy for acute episodes of pain in people with sickle cell disease. Cochrane Database of Systematic Reviews 2015, Issue 3. Art. No.: CD005406. DOI: 10.1002/14651858.CD005406.pub4</ref>
 
***There are no randomized controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid.<ref>Okomo U, Meremikwu MM. Fluid replacement therapy for acute episodes of pain in people with sickle cell disease. Cochrane Database of Systematic Reviews 2015, Issue 3. Art. No.: CD005406. DOI: 10.1002/14651858.CD005406.pub4</ref>
*O2 if [[hypoxic]]; otherwise may inhibit erythropoiesis
+
*O2 if [[hypoxia]]; otherwise may inhibit erythropoiesis
  
===Anemia===
+
===[[Anemia]]===
 
*Transfusion [[PRBCs]] 10 mL/kg over 2hr period
 
*Transfusion [[PRBCs]] 10 mL/kg over 2hr period
 
*Indications:
 
*Indications:
 
**[[Aplastic crisis]]
 
**[[Aplastic crisis]]
**Sequestration crisis
+
**[[splenic sequestration|Sequestration crisis]]
 
**hemoglobin <6 with inappropriately low retic count
 
**hemoglobin <6 with inappropriately low retic count
 +
 +
===Pediatric Pain Crisis===
 +
''Ideally should have institutional protocols to initiate pain management at triage''
 +
 +
*Consider early intranasal [[fentanyl]] even prior to establishing IV
 +
**Dosage 2 mcg/kg intraNASAL
 +
*IV pain medication options
 +
**Morphine 0.1 mg/kg
 +
**Hydromorphone 0.02 mg/kg
 +
**Toradol 0.5 mg/kg
 +
*PO options
 +
**Oxycodone 0.1 mg/kg
 +
**Morphine 0.5 mg/kg
 +
**Hydromorphone 0.05 mg/kg
 +
**Ibuprofen 10 mg/kg
 +
 +
 +
''Repeat pain medications q20 minutes at 50% of initial loading dose until adequate pain control achieved''
  
 
==Disposition==
 
==Disposition==
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*[[Sepsis]], [[osteomyelitis]], or other serious infection is suspected
 
*[[Sepsis]], [[osteomyelitis]], or other serious infection is suspected
 
*[[Priapism]], [[aplastic crisis]], [[hypoxia]], splenic sequestration
 
*[[Priapism]], [[aplastic crisis]], [[hypoxia]], splenic sequestration
*WBC >30K
+
*[[leukocytosis|WBC >30K]]
*Platlet <100K
+
*[[thrombocytopenia|Platelet <100K]]
 
*Pain is not under control after 3 rounds of analgesics in ED
 
*Pain is not under control after 3 rounds of analgesics in ED
 
*<1yr old
 
*<1yr old

Latest revision as of 01:24, 27 November 2019

Background

  • Common complication of sickle cell anemia, constituting 79-91% of ED visits[1]
  • Sickled red blood cells obstruct vasculature, causing ischemic injury and pain

Precipitating Factors

Clinical Features[2]

  • Pain
    • Lower back, long bones most commonly affected

Differential Diagnosis

Sickle cell crisis

Evaluation

  • CBC
    • Because anemia can precipitate a crisis, must check for acute hemoglobin drop
  • Retic count (if aplastic crisis considered - rare in adults)
    • Should be >0.5%
  • Consider:
    • Chemistry / LFTs / lipase
      • If abdominal pain, may be cholecystitis, mesenteric ischemia, or perforation
    • T&S/T&C
    • Urine pregnancy
    • CXR
      • If cough, shortness of breath, or febrile

Management

  • Analgesia
  • Hydration
    • Controversial
      • Dehydration promotes sickling
      • IVF bolus may lead to atelectasis, pulmonary edema, and acute chest syndrome. Hyperchloremic metabolic acidosis due to normal saline may further precipitate sickling. [5] [6] [7]
      • There are no randomized controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid.[8]
  • O2 if hypoxia; otherwise may inhibit erythropoiesis

Anemia

Pediatric Pain Crisis

Ideally should have institutional protocols to initiate pain management at triage

  • Consider early intranasal fentanyl even prior to establishing IV
    • Dosage 2 mcg/kg intraNASAL
  • IV pain medication options
    • Morphine 0.1 mg/kg
    • Hydromorphone 0.02 mg/kg
    • Toradol 0.5 mg/kg
  • PO options
    • Oxycodone 0.1 mg/kg
    • Morphine 0.5 mg/kg
    • Hydromorphone 0.05 mg/kg
    • Ibuprofen 10 mg/kg


Repeat pain medications q20 minutes at 50% of initial loading dose until adequate pain control achieved

Disposition

Consider admission

Consider discharge

  • Pain is under control and patient can take oral fluids and medications
  • Ensure appropriate oral analgesics are available
  • Provide home care instructions
  • Ensure resource for follow-up

See Also

External Links

References

  1. Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47
  2. Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47
  3. *Lyness, D., & Motov, S. (2016, July 7). CERTA Opioid Alternatives and Analgesics. Retrieved July 25, 2016, from http://www.propofology.com/infographs/certa-opioid-alternatives-as-analgesics
  4. *Lyness, D., & Motov, S. (2016, July 7). CERTA Opioid Alternatives and Analgesics. Retrieved July 25, 2016, from http://www.propofology.com/infographs/certa-opioid-alternatives-as-analgesics
  5. Glassberg, J., Evidence-based management of sickle cell disease in the emergency department.Emerg Med Pract, 2011. 13(8): p. 1-20
  6. Carden, M.A., et al., Variations in pediatric emergency medicine physician practices for intravenous fluid management in children with sickle cell disease and vaso-occlusive pain: A single institution experience.Pediatr Blood Cancer, 2018. 65(1)
  7. Carden, M.A., et al., Normal Saline Bolus Use in Pediatric Emergency Departments is Associated with Worse Pain Control in Children with Sickle Cell Anemia and Vaso-occlusive Pain.Am J Hematol, 2019.
  8. Okomo U, Meremikwu MM. Fluid replacement therapy for acute episodes of pain in people with sickle cell disease. Cochrane Database of Systematic Reviews 2015, Issue 3. Art. No.: CD005406. DOI: 10.1002/14651858.CD005406.pub4