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 | ||
**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 | ||
*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== | ||
*[[Analgesia]] | |||
**IV [[opioids]] ([[morphine]] or [[hydromorphone]]) | |||
***Redose in 30min if inadequate | |||
**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 | |||
**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. <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> | |||
*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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*[[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]] | ||
* | *[[thrombocytopenia|Platelet <100K]] | ||
*Pain is not under control after | *Pain is not under control after 3 rounds of analgesics in ED | ||
*<1yr old | *<1yr old | ||
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
- Stress
- Cold weather
- Dehydration
- Hypoxia
- Infection
- Acidosis
- Alcohol intoxication
- Pregnancy
- Exertional stress
Clinical Features[2]
- Pain
- Lower back, long bones most commonly affected
Differential Diagnosis
Sickle cell crisis
- Vaso-occlusive pain crisis
- Bony infarction
- Dactylitis
- Avascular necrosis of femoral head
- Acute chest syndrome
- Asthma
- Pulmonary hypertension
- Gallbladder disease
- Acute hepatic sequestration
- Infection
- Parvovirus B19
- Splenic sequestration
- CVA
- Cerebral aneurysm and ICH
- Priapism
- Papillary necrosis
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:
Management
- Analgesia
- IV opioids (morphine or hydromorphone)
- Redose in 30min if inadequate
- Non-opioid analgesia
- NSAIDs (e.g. ketorolac 15mg q6h)
- Acetaminophen
- IV/IM haloperidol or droperidol 5-10mg[3]
- Ketamine (IN 1mg/kg 1ml per nostril or IV 0.3mg/kg)[4]
- Nonpharmacologic measures (e.g. heating packs, distraction)
- IV opioids (morphine or hydromorphone)
- 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]
- Controversial
- O2 if hypoxia; otherwise may inhibit erythropoiesis
Anemia
- Transfusion PRBCs 10 mL/kg over 2hr period
- Indications:
- Aplastic crisis
- 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
Repeat pain medications q20 minutes at 50% of initial loading dose until adequate pain control achieved
Disposition
Consider admission
- Acute chest syndrome is suspected
- Sepsis, osteomyelitis, or other serious infection is suspected
- Priapism, aplastic crisis, hypoxia, splenic sequestration
- WBC >30K
- Platelet <100K
- Pain is not under control after 3 rounds of analgesics in ED
- <1yr old
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
- ↑ Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47
- ↑ Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47
- ↑ *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
- ↑ *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
- ↑ Glassberg, J., Evidence-based management of sickle cell disease in the emergency department.Emerg Med Pract, 2011. 13(8): p. 1-20
- ↑ 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)
- ↑ 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.
- ↑ 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