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sickle cell vaso-occlusive crisis

Etiology: - sickle cell disease Pathology: 1) precipitation of hemoglobin SS under low oxygen tension resulting in loss of erythrocyte structure & deformability 2) microvascular occlusion & infarction 3) overactivity of SLC12A4 may contribute to erythrocyte dehydration Clinical manifestations: - vaso-occlusive episodes or "painful crises" a) may be very painful & debilitating for the patient b) there are no objective findings to confirm the presence of pain Management: 1) analgesia for painful crises: a) non-steroidal anti-inflammatory agents (NSAIDs) b) acetaminophen c) parenteral opioids for severe pain [2] - morphine, oxycodone agents of choice - therapeutic management of the pain is a frequent source of conflict between the physician & patient or patient's family d) agents used in combination with analgesics - antihistamines: diphenhydramine, hydroxyzine - tricyclic antidepressants - phenothiazines for nausea (ondansetron might be an option for nausea) e) ref [1] states scheduled morphine is treatment of choice citing ref [3] 2) general management principles include: a) hydration with 3-4 L of oral fluid/day b) intravenous fluids for dehydration or acute illness c) avoid iatrogenic fluid overload in patients with renal, cardiac or hepatic insufficiency d) infusion centers with better outcomes than emergency departments for managing uncomplicated crises [5] e) incentive spirometry to avoid acute chest syndrome 3) nitric oxide of no benefit [3]

Related

sickle cell (hemoglobin SS) disease

General

complication sign/symptom

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 17, 19. American College of Physicians, Philadelphia 2015, 2022
  2. Green D Managing Sickle Cell Disease: An Update NEJM Journal Watch. Sept 16, 2014 Massachusetts Medical Society (subscription needed) http://www.jwatch.org - Yawn BP et al. Management of sickle cell disease: Summary of the 2014 evidence-based report by expert panel members. JAMA 2014 Sep 10; 312:1033 PMID: 2520308
  3. Gladwin MT et al Nitric Oxide for Inhalation in the Acute Treatment of Sickle Cell Pain Crisis JAMA. 2011;305(9):893-902 PMID: 21364138 http://jama.ama-assn.org/content/305/9/893.full
  4. Yale SH, Nagib N, Guthrie T. Approach to the vaso-occlusive crisis in adults with sickle cell disease. Am Fam Physician. 2000 Mar 1;61(5):1349-56, 1363-4. PMID: 10735342
  5. Lanzkron S, Little J, Wang H et al. Treatment of acute pain in adults with sickle cell disease in an infusion center versus the emergency department: A multicenter prospective cohort study. Ann Intern Med 2021 Jul 6; [e-pub]. PMID: 34224261 - Kanter J. One small step for sickle cell disease: Many more to go. Ann Intern Med 2021 Jul 6; [e-pub] PMID: 34224263