Ver el código de «Vaso-occlusive pain crisis»

==Background==
*Common complication of [[sickle cell anemia]], constituting 79-91% of ED visits<ref name="Lovett">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 name="uptodate">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-children-and-adults</ref>

===Precipitating Factors===
*Stress
*Cold weather
*[[Dehydration]]
*[[Hypoxia]]
*[[Infection]]
*[[Acidosis]]
*[[Alcohol intoxication]]
*[[Pregnancy]]
*Exertional stress

==Clinical Features<ref name="Lovett" /> <ref name="uptodate" />==
*Pain
**Upper and lower [[back pain|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 DDX}}

==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. <ref name="uptodate" />
**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 <ref name="uptodate" />
**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. <ref name="uptodate" />
**Non-opioid analgesia
***[[NSAIDs]] (e.g. [[ketorolac]] 15mg q6h)
***[[Acetaminophen]]
***IV/IM [[haloperidol]] or [[droperidol]] 5-10mg<ref name="Lyness">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 name="Lyness" />
**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]]===
*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 name="uptodate" />

===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
*[[leukocytosis|WBC >30K]]
*[[thrombocytopenia|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==
*[[Sickle cell crisis]]

==External Links==

==References==
<references/>

[[Category:Heme/Onc]]