Vaso-occlusive pain crisis: Difference between revisions
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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
**Because anemia can precipitate a crisis, must check for acute hemoglobin drop


==Differential Diagnosis==
==Differential Diagnosis==
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==Evaluation==
==Evaluation==
*CBC
*CBC
**Assess for significant anemia
**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%
*[[CXR]]
*Consider:
**If cough, shortness of breath, or febrile
**Chemistry / [[LFTs]] / lipase
*O2
***If abdominal pain, may be cholecystitis, mesenteric ischemia, or perforation
**If hypoxic; otherwise may inhibit erythopoesis
**T&S/T&C
*[[ECG]]
**Urine pregnancy
*T&S/T&C
**[[CXR]]
*blood culture2
***If cough, shortness of breath, or febrile
*VBG
*[[Urinalysis]]
*Urine pregnancy
*Head CT/MRI
**If symptoms of stroke


==Management==
==Management==
*Analgesia
*[[Analgesia]]
**IV opiods prefered (Morphine or hydormorphone)
**IV [[opioids]] ([[morphine]] or [[hydromorphone]])
***Avoid IM route (use SQ if necessary)
***Avoid meperidine
***Use PCA pump if available
***Redose in 30min if inadequate
***Redose in 30min if inadequate
***Normally admit if needs more than three doses
**Non-opioid analgesia
**Use of concurrent acetaminophen encouraged, unless contraindicated
***[[NSAIDs]] (e.g. [[ketorolac]] 15mg q6h)
***[[Acetaminophen]]
***IV/IM [[haloperidol]] or [[droperidol]] 5-10mg<ref>*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</ref>
***[[Ketamine]] (IN 1mg/kg 1ml per nostril or IV 0.3mg/kg)<ref>*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</ref>
**Nonpharmacologic measures (e.g. heating packs, distraction)
*Hydration
*Hydration
**Initial bolus should be 1/2NS with goal of increasing MCV of sickled cells<ref>Guy, R. Treatment of Sickle Cell Crisis with Hypotonic Saline Clinical Research 1971; 19: 420</ref><ref>Pathogenesis and Treatment of Sickle Cell Disease H. Franklin Bunn, M.D. N Engl J Med 1997; 337:762-769</ref>
**Controversial
**1.5 times maintenance with D5 1/2NS
***Dehydration promotes sickling
**Normal Saline boluses for hypovolemic shock
***[[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>
*O2 is not useful in the nonhypoxic patient
***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 [[hypoxia]]; otherwise may inhibit erythropoiesis
 
===[[Anemia]]===
*Transfusion [[PRBCs]] 10 mL/kg over 2hr period
*Indications:
**[[Aplastic crisis]]
**[[splenic sequestration|Sequestration crisis]]
**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
 


===Anemia===
''Repeat pain medications q20 minutes at 50% of initial loading dose until adequate pain control achieved''
*Transfusion
**Indications:
***Aplastic crisis
***Sequestration crisis
***hemoglobin <6 with inappropriately low retic count
***hemoglobin <10 with acute crisis
**Transfuse 10 mL/kg over 2hr period


==Disposition==
==Disposition==
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*[[Acute chest syndrome]] is suspected
*[[Acute chest syndrome]] is suspected
*[[Sepsis]], [[osteomyelitis]], or other serious infection is suspected
*[[Sepsis]], [[osteomyelitis]], or other serious infection is suspected
*[[Priapism]], [[aplastic crisis]], [[hypoxia]]
*[[Priapism]], [[aplastic crisis]], [[hypoxia]], splenic sequestration
*WBC >30K
*[[leukocytosis|WBC >30K]]
*Platlet <100K
*[[thrombocytopenia|Platelet <100K]]
*Pain is not under control after 2-3 rounds of analgesics in ED
*Pain is not under control after 3 rounds of analgesics in ED
*<1yr old
*<1yr old


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==See Also==
==See Also==
*[[Sickle cell crisis]]


==External Links==
==External Links==

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
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