Sickle Cell: Management of Acute Pain Crisis Clinical Pathway

Pathway Background and Objectives

Sickle Cell Disease (SCD) is the most common genetic disease in the United States. It is caused by a mutation in the hemoglobin beta chain in which glutamic acid is substituted with valine. The Centers for Disease Control and Prevention (2019) estimates that over 100,000 Americans are affected by SCD. The disease can affect multiple organ systems and decrease life expectancy. There are no national guidelines for acute pain management in patients with SCD. Likewise, there was no standardized approach to emergency department and inpatient management of acute pain for patients with SCD at Connecticut Children’s. This inconsistency in care can lead to high readmission rates, prolonged intravenous opioid management, opioid crisis, and opioid induced hypersensitivity. There is currently a national goal to reduce the use of long-acting opioids for patients with non-cancer pain to mitigate the increased side effects and risks associated with opioid use, and also because of its decreased efficacy in treating this type of pain. The specific objectives of this pathway are to:

Standardize sickle cell acute pain treatment
Decrease LOS and readmission rates
Decrease the duration patients receive intravenous opioids
Improve timely consultation of the Pain Team (if needed)
Improve timely administration of multi-modal treatments
Encourage early mobilization

Algorithm Educational Module

Quality Metrics

Percentage of eligible patients who utilize the pathway order set
Average time from ED arrival to first opioid administered (oral or IV) in ED (minutes)
Percentage of ED patients who are admitted
Average time on IV opioids after arrival to medical-surgical floor (hours)
Average time from arrival to ED to PCA initiation (minutes)
Average time from arrival to medical-surgical floor to PCA initiation (minutes)
Percentage of admitted patients with pain team service consult ≤ 24 hours from admission
Percentage of admitted patients who have orders for any of the following adjuvant therapies: PT, Psychology, Integrative Medicine, or Massage Therapy
ALOS (days, IP/OBS) and ALOS (minutes, ED)
Readmissions within 7 days
Readmissions within 30 days

Key References

Balsamo L, Shabanova V, Carbonella J, Szondy MV, Kalbfeld K, Thomas DA, Santucci K, Grossman M, Pashankar F. Improving Care for Sickle Cell Pain Crisis Using a Multidisciplinary Approach. Pediatrics. 2019 May;143(5):e20182218. doi: 10.1542/peds.2018-2218.
Brandow AM, Nimmer M, Simmons T, et al. Impact of emergency department care on outcomes of acute pain events in children with sickle cell disease. Am l Hematol. 2016 Dec;91(12):1175-1180.
Brandow AM, Zappia KJ, Stucky CL. Sickle cell disease: a natural model of acute and chronic pain. Pain. 2017 Apr;158 Suppl 1(Suppl 1):S79-S84.
Dampier C, Palermo TM, Darbari DS, Hassell K, Smith W, Zempsky W. AAPT Diagnostic Criteria for Chronic Sickle Cell Disease Pain.J Pain. 2017 May;18(5):490-498.
Field, JJ, Ballas SK, Campbell CM, Crosby, LE, Dampier C, Darbari DS, McClish DK, Smith W, Zempsky WT. AAAPT Diagnostic Criteria for Acute Sickle Cell Disease Pain. J Pain. 2019 Jul;20(7):746-759.
Sheehy KA, Lippold C, Rice AL, Nobrega R, Finkel JC, Quezado ZM. Subanesthetic ketamine for pain management in hospitalized children, adolescents, and young adults: a single-center cohort study. J Pain Res. 2017 Apr 5;10:787-795.
Zempsky WT, Loiselle KA, Corsi JM, Hagstrom, JN. Use of Low-dose Ketamine Infusion for Pediatric Patients With Sickle Cell Disease-related Pain. Clin J Pain. 2010 Feb;26(2):163-7.

Disclaimer

The clinical pathways in the above links have been developed specifically for use at Connecticut Children’s and are made available publicly for informational and/or educational purposes only. The clinical pathways are not intended to be, nor are they, a substitute for individualized professional medical judgment, advice, diagnosis, or treatment. Although Connecticut Children’s makes all efforts to ensure the accuracy of the posted content, Connecticut Children’s makes no warranty of any kind as to the accuracy or completeness of the information or its fitness for use at any particular facility or in any individual case.