Vaso-occlusive pain crisis: Difference between revisions

(/* Clinical FeaturesLovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47 and DeBaun, M. Acute vaso-occlusive pain management in sickle cell disease. UpToDate. 2022 Mar. Accessed via: https://w...)
 
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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
*Provider misconceptions can interfere with adequate pain assessment and can lead to insufficient treatment. Avoid use of the terms "sickler" or "frequent flier." Early, adequate, and aggressive pain treatment is essential. <ref>DeBaun, M. Acute vaso-occlusive pain management in sickle cell disease. UpToDate. 2022 Mar. Accessed via: https://www.uptodate.com/contents/acute-vaso-occlusive-pain-management-in-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=14519&source=see_link#H317569</ref>
===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> <ref>DeBaun, M. Acute vaso-occlusive pain management in sickle cell disease. UpToDate. 2022 Mar. Accessed via: https://www.uptodate.com/contents/acute-vaso-occlusive-pain-management-in-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=14519&source=see_link#H317569</ref>==
*Pain
*Pain
**Most common manifestation of SCA (79-91% of ED visits)
**Upper and lower [[back pain|back]], long bones most commonly affected
**Lower back, long bones most commonly affected
**Can also include abdomen, chest, arms
**Because anemia can precipitate a crisis, must check for acute hemoglobin drop
**Often multiple sites are involved
**Intensity can be debilitating and pain can last from hours to days with some individuals never being completely without pain


==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)
**Pain assessment comes from the patient or patient family. No lab or clinical findings determine, confirm, or change if a patient has pain. Absence of hemolysis or baseline hemoglobin level should not be used to withhold or decrease pain medication dosing. <ref>DeBaun, M. Acute vaso-occlusive pain management in sickle cell disease. UpToDate. 2022 Mar. Accessed via: https://www.uptodate.com/contents/acute-vaso-occlusive-pain-management-in-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=14519&source=see_link#H317569</ref>
***Avoid IM route (use SQ if necessary)
**Ask the patient what medications have worked for them in the past
***Avoid meperidine
**Pain medication should be dosed within 30 minutes, IV route preferred with SC or IN routes for those with access issues <ref>DeBaun, M. Acute vaso-occlusive pain management in sickle cell disease. UpToDate. 2022 Mar. Accessed via: https://www.uptodate.com/contents/acute-vaso-occlusive-pain-management-in-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=14519&source=see_link#H317569</ref>
***Use PCA pump if available
**IV [[opioids]] ([[morphine]] or [[hydromorphone]])
***Redose in 30min if inadequate
***Redose in 30min if inadequate
***Normally admit if needs more than three doses
***Can also use IN fentanyl particularly in pediatric patients. Restrict to 2 doses in the ED. See below. <ref>DeBaun, M. Acute vaso-occlusive pain management in sickle cell disease. UpToDate. 2022 Mar. Accessed via: https://www.uptodate.com/contents/acute-vaso-occlusive-pain-management-in-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=14519&source=see_link#H317569</ref>
**Use of concurrent acetaminophen encouraged, unless contraindicated
**Non-opioid analgesia
***[[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
**Transfusion is not a treatment for vast-occlusive pain without anemia <ref>DeBaun, M. Acute vaso-occlusive pain management in sickle cell disease. UpToDate. 2022 Mar. Accessed via: https://www.uptodate.com/contents/acute-vaso-occlusive-pain-management-in-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=14519&source=see_link#H317569</ref>
 
===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==

Latest revision as of 15:37, 20 April 2022

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
  • Provider misconceptions can interfere with adequate pain assessment and can lead to insufficient treatment. Avoid use of the terms "sickler" or "frequent flier." Early, adequate, and aggressive pain treatment is essential. [2]

Precipitating Factors

Clinical Features[3] [4]

  • Pain
    • Upper and lower back, long bones most commonly affected
    • Can also include abdomen, chest, arms
    • Often multiple sites are involved
    • Intensity can be debilitating and pain can last from hours to days with some individuals never being completely without pain

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
    • Pain assessment comes from the patient or patient family. No lab or clinical findings determine, confirm, or change if a patient has pain. Absence of hemolysis or baseline hemoglobin level should not be used to withhold or decrease pain medication dosing. [5]
    • Ask the patient what medications have worked for them in the past
    • Pain medication should be dosed within 30 minutes, IV route preferred with SC or IN routes for those with access issues [6]
    • IV opioids (morphine or hydromorphone)
      • Redose in 30min if inadequate
      • Can also use IN fentanyl particularly in pediatric patients. Restrict to 2 doses in the ED. See below. [7]
    • Non-opioid analgesia
    • Nonpharmacologic measures (e.g. heating packs, distraction)
  • 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. [10] [11] [12]
      • There are no randomized controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid.[13]
  • 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. DeBaun, M. Acute vaso-occlusive pain management in sickle cell disease. UpToDate. 2022 Mar. Accessed via: https://www.uptodate.com/contents/acute-vaso-occlusive-pain-management-in-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=14519&source=see_link#H317569
  3. Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47
  4. DeBaun, M. Acute vaso-occlusive pain management in sickle cell disease. UpToDate. 2022 Mar. Accessed via: https://www.uptodate.com/contents/acute-vaso-occlusive-pain-management-in-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=14519&source=see_link#H317569
  5. DeBaun, M. Acute vaso-occlusive pain management in sickle cell disease. UpToDate. 2022 Mar. Accessed via: https://www.uptodate.com/contents/acute-vaso-occlusive-pain-management-in-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=14519&source=see_link#H317569
  6. DeBaun, M. Acute vaso-occlusive pain management in sickle cell disease. UpToDate. 2022 Mar. Accessed via: https://www.uptodate.com/contents/acute-vaso-occlusive-pain-management-in-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=14519&source=see_link#H317569
  7. DeBaun, M. Acute vaso-occlusive pain management in sickle cell disease. UpToDate. 2022 Mar. Accessed via: https://www.uptodate.com/contents/acute-vaso-occlusive-pain-management-in-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=14519&source=see_link#H317569
  8. *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
  9. *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
  10. Glassberg, J., Evidence-based management of sickle cell disease in the emergency department.Emerg Med Pract, 2011. 13(8): p. 1-20
  11. 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)
  12. 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.
  13. 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
  14. DeBaun, M. Acute vaso-occlusive pain management in sickle cell disease. UpToDate. 2022 Mar. Accessed via: https://www.uptodate.com/contents/acute-vaso-occlusive-pain-management-in-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=14519&source=see_link#H317569