Abstract
OBJECTIVE
Vaso-occlusive crisis (VOC) is the most common problem reported by patients with sickle cell disease (SCD). The objective of this study was to evaluate the impact of individualized pain plans in pediatric patients with SCD admitted for VOC.
METHODS
This was a pre- and post-study of patients with SCD admitted to Riley Hospital for Children for VOC from July 1, 2019, through July 1, 2020. The primary outcome was length of inpatient stay for VOC. Secondary outcomes included final pain score, days on scheduled opioids, days on breakthrough opioids, and average morphine milligram equivalents (MME) used per day.
RESULTS
Nine patients were included. The mean age was 16 years (range, 10–20 years). Key clinical findings were decreases in median [IQR] for final pain scores (7 [4.5–9] vs 6 [2.5–8], p = 0.396) and number of days of breakthrough opioid use (5 [3–8] vs 4 [2.5–5.5], p = 0.233). Following implementation of an individualized pain plan, there was an increase in median average MME per day (65.94 [53.1–97.7] vs 82.85 [41–114.3], p = 0.844). Median length of stay and days on scheduled opioids remained the same.
CONCLUSIONS
This study demonstrated that use of individualized pain plans in a small population of patients with SCD might result in decreased pain scores and decreased days on breakthrough opioids.
Keywords: analgesics, opioid; anemia, sickle cell; child; hospitalization; length of stay; MME; pain management
Introduction
Sickle cell disease (SCD) is an inherited autosomal recessive disorder that results in sickle-shaped red blood cells due to a mutation in the beta globin gene.1 Sickled cells obstruct circulation, prevent tissue oxygenation, and contribute to severe complications including acute painful vaso-occlusive crises (VOCs).1
Accounting for approximately 230,000 annual admissions, VOC is the most common cause for emergency department visits and hospitalizations in children with SCD.2 In a publication examining school absenteeism in adolescents with SCD, study participants missed an average of 12% of the school year with their absences directly correlating to pain crises.3 Similarly, Barakat et al4 identified a significant association between pain frequency and lower quality of life. VOC not only affects patients with SCD, but also places a large financial burden on the health care system.2 Because of these negative outcomes, it is crucial to establish effective and timely pain management for the treatment of VOC in the pediatric population.
In recent years, the restriction of opioid use due to the national pandemic of opioid abuse has facilitated increased provider apprehension in prescribing opioids and negatively impacted adequate pain control in patients with SCD.5,6 Conventional weight-based dosing of opioids may not provide sufficient analgesia for pediatric patients with SCD presenting with VOC. Larger or more frequent dosing of opioids may be needed to provide adequate analgesia, which some physicians may be uncomfortable initiating.7 Current research suggests better management of SCD relies on optimal management of VOC rather than the restriction of opioid use.8 To establish satisfactory opioid analgesia, current data and guidelines promote the use of individualized pain plans for treatment optimization and prescriber reassurance.7 The objective of this study was to evaluate clinical outcomes in children with SCD admitted for a VOC with use of a patient-specific pain plan.
Materials and Methods
This pre- and post-study was conducted at Riley Hospital for Children at Indiana University Health. Patients with SCD with an individualized pain plan and hospitalized for VOC during the period of July 1, 2019, to July 1, 2020, at Riley Hospital for Children were included in this study. Patients were excluded if they developed acute chest syndrome or complications unrelated to SCD, if they were transferred from an outside hospital, if their pain plan included use of a patient-controlled analgesia pump (owing to limitations with integration into the electronic medical record), or if their individualized pain plan was not followed at any point during their inpatient stay.
Individualized pain plans were created by using data from the patient's most recent VOC admissions. Specifically, medications that successfully provided pain relief by patient and provider report were recommended for immediate initiation during subsequent VOC admissions. Medication regimens for each patient were assessed for safety and appropriate dosing. The finalized pain plan included a complete drug regimen for VOC pain management with details regarding dose, route, frequency, and dosing adjustment criteria. After a thorough review, pain plans were uploaded into the electronic medical record for reference during subsequent VOC admissions.
Before pain plan implementation, the multidisciplinary pediatric hematology team was educated on the new guidelines for individualized VOC pain management. Education included information about study goals, where to access pain plans, when to use pain plans, and how pain plans were created.
Data collection was completed for VOC admissions before and after the implementation of patient-specific pain plans. Data collection included patient demographics, length of stay, days on scheduled therapy while inpatient, days on breakthrough therapy while inpatient, average morphine milligram equivalents (MME) per day, and final pain score. Per institutional standard, pain was rated numerically on a scale of 1 to 10. It was also noted if patients were using hydroxyurea therapy at home. Data from a patient's most recent admission prior to pain plan creation were compared directly with data from a VOC admission, using a pain plan from July 1, 2019, to July 1, 2020. If a patient had multiple VOC admissions during the study period, data were averaged for comparison to the most recent admission not using a pain plan. All data were evaluated by using Wilcoxon statistical analysis. Statistical calculations were conducted by using Minitab Statistical Software, version 21.1.0.
Results
Nine patients were included in the study from July 1, 2019, through July 1, 2020. The study had representation of various genotypes with HbSS (66.6%), HbSC (22.3%), and HbSB thalassemia (11.1%). Most were male (66.6%), the mean age was 16.1 years (10–20 years), and most were receiving hydroxyurea therapy (66.7%). Three patients had more than 1 VOC admission during the study period (33.3%).
Both the length of stay (5 [4–11.5] days vs 5 [4–8.5] days, p = 0.396) and the number of days on scheduled opioids 5 [3–11] days vs 5 [4–7] days, p = 0.32) remained the same after implementation of individualized pain plans (Table). Final pain score was lower after pain plan implementation (7 [4.5–9] vs 6 [2.5–8]), but was not statistically significant (p = 0.396). Likewise, the number of days on breakthrough opioids was lower (5 [3–8] vs 4 [2.5–5.5]); however, the finding was not statistically significant (p = 0.233). Average MME needed per day also increased (65.94 [53.1–97.7] vs 82.85 [41–114.3]), but this outcome was not statistically significant (p = 0.844).
Table.
Outcomes for Pre and Post Plan expressed as Median (IQR)
Outcome | Pre-Plan | Post-Plan | p value |
---|---|---|---|
Length of stay, days | 5 (4–11.5) | 5 (4–8.5) | 0.396 |
Scheduled opioids, days | 5 (3–11) | 5 (4–7) | 0.32 |
Breakthrough opioids, days | 5 (3–8) | 4 (2.5–5.5) | 0.233 |
MME per day, mg | 65.94 (53.1–97.7) | 82.85 (41–114.3) | 0.844 |
Final pain score (1–10) | 7 (4.5–9) | 6 (2.5–8) | 0.396 |
MME, morphine milligram equivalents
Discussion
The implementation of individualized pain plans in the treatment of patients with SCD with VOC may lead to optimization of pain management, prevention of delayed or inadequate analgesia, decreased length of time on breakthrough opioids, and alleviation of provider hesitancy to initiate seemingly aggressive opioid therapy. Strategies are needed to improve assessment and treatment of pain associated with VOC. The use of individualized pain plans strives toward the goal of increasing familiarity of the clinician with the patient.7,9 According to the National Heart, Lung, and Blood Institute SCD guidelines, it is recommended that an individualized prescribing and monitoring protocol written by an SCD provider be used with patients with VOC to promote effective, timely, and safe analgesic management and resolution of the VOC.7
Although the results of this study on the use of individualized pain pains were not statistically significant in this small cohort, clinical significance was seen with improved pain management for patients with SCD who experienced VOC. Decreased interquartile ranges in length of stay, days on scheduled therapy, days on breakthrough therapy, and final pain score were all observed. These findings are similar to both pediatric and adult studies that used patient-specific pain plans and found shorter length of stay and better pain control.2,10–12 While the focus of this study was not health care–associated cost savings, we would expect this, as well, in patients who had fewer days of hospitalization.
Patients enduring pain related to SCD receive care based on a complex sociocultural system built on beliefs and attitudes of the patient, the patient's family, the health care system, and the health care team.13 Multiple health care professionals are typically involved in the care of chronic illnesses such as SCD, introducing potential shortcomings in effective communication, coordination, and continuity of care.13 A survey assessing pediatric resident perceptions toward opioid use in SCD pain management reported that increased patient and provider barriers led to distrust and ultimately to undertreatment of pain and inadequate care.14
The increase in average MME needed per day suggests better optimization of pain management as a result of individualized pain plan use. Larger doses more appropriate for the patient's weight and opioid use history were used upfront to allow for better and quicker pain relief. With the proper education and creation of individualized pain plans, providers unfamiliar with managing VOC in SCD can gain confidence with the dosing and rapid initiation of opioids in patients who present with severe pain along with diminishing conscious or unconscious biases, restoring the relationships between providers and patients.
Some limitations to this study include a single center study design and a short data collection time of 1 year. Patients on patient-controlled analgesia pumps were not included in the results because medication administration within the institution for patient-controlled analgesia was manually entered rather than system reported. While these patients did follow individualized pain plans, it is difficult to determine overall improvement with pain score, MME requirements, dose titrations, and breakthrough need. This study also used a numeric pain scale. While this is the standard at many institutions when documenting in the electronic medical record, many patients with SCD live in chronic pain and may have a hard time differentiating between their chronic and acute pain or may find it difficult to relate pain to a numeric value. The goal of pain management is to get to a level of tolerability and being able to manage the pain as an outpatient. This numeric value has large interpatient variability.
Additionally, provider education was deemed a significant challenge for successful pain plan implementation owing to many rotational prescribers such as medical students, residents, and fellows practicing on the hematology team for a short period.
Conclusion
This study demonstrated that use of individualized pain plans in a small population of patients with SCD might result in decreased pain scores and decreased days on breakthrough opioids. Further prospective studies are needed to fully explore additional outcomes associated with the implementation of individualized pain plans in pediatric patients with SCD, including health care cost, patient satisfaction, quality of life, comfort of providers, and time to first opioid.
Acknowledgments
Results were presented as a student poster at the American Society of Health-System Pharmacists' Virtual Midyear Clinical Meeting on December 8, 2020.
ABBREVIATIONS
- MME
morphine milligram equivalents
- SCD
sickle cell disease
- VOC
vaso-occlusive crisis
Footnotes
Disclosures. All authors declare no conflicts or financial interest in any product or service mentioned in the manuscript, including equipment, grants, medication, employment, gifts, and honoraria. The authors had full access to all the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.
Ethical Approval and Informed Consent. The authors assert that all procedures contributing to this work comply with the ethical standards of the relevant national guidelines and have been approved by the appropriate IRB.
References
- 1.Centers for Disease Control and Prevention What is sickle cell disease? Accessed January 19, 2021. https://www.cdc.gov/ncbddd/sicklecell/facts.html.
- 2.Balsamo L, Shabanova V, Carbonella J et al. Improving care for sickle cell pain crisis using a multidisciplinary approach. Pediatrics . 2019;143(5):e20182218. doi: 10.1542/peds.2018-2218. [DOI] [PubMed] [Google Scholar]
- 3.Shwartz LA, Radcliffe J, Barakat LP. Associates of school absenteeism in adolescents with sickle cell disease. Pediatr Blood Cancer . 2009;52(1):92–96. doi: 10.1002/pbc.21819. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 4.Barakat LP, Patterson CA, Daniel LC, Dampier C. Quality of life among adolescents with sickle cell disease: mediation of pain by internalizing symptoms and parenting stress. Health Qual Life Outcomes . 2008;6:60. doi: 10.1186/1477-7525-6-60. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 5.National Institutes of Health Opioid crisis adds to pain of sickle cell patients. Accessed March 23, 2021. https://www.nhlbi.nih.gov/news/2017/opioid-crisis-adds-pain-sickle-cell-patients.
- 6.Waldrop RD, Mandry C. Health professional perceptions of opioid dependence among patients with pain. Am J Emerg Med . 1995;13(5):529–531. doi: 10.1016/0735-6757(95)90163-9. [DOI] [PubMed] [Google Scholar]
- 7.National Heart, Lung, and Blood Institute Bethesda, MD: National Institutes of Health; 2014. Evidence-based management of sickle cell disease: expert panel report, 2014. Accessed May 16, 2021. http://www.nhlbi.nih.gov/health-pro/guidelines/sickle-cell-disease-guidelines. [Google Scholar]
- 8.Kang HA, Barner JC, Richards KM et al. Association between vaso-occlusive crises and opioid prescriptions among patients with sickle cell disease: a retrospective claims-based study. J Health Econ Outcomes Res . 2020;7(1):94–101. doi: 10.36469/jheor.2020.13348. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 9.Haywood C, Jr, Lanzkron S, Hughes MT et al. A video-intervention to improve clinician attitudes toward patients with sickle cell disease: the results of a randomized experiment. J Gen Intern Med . 2011;26(5):518–523. doi: 10.1007/s11606-010-1605-5. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 10.Krishnamurti L, Smith-Packard B, Gupta A et al. Impact of individualized pain plan on the emergency management of children with sickle cell disease. Pediatr Blood Cancer . 2014;61(10):1747–1753. doi: 10.1002/pbc.25024. [DOI] [PubMed] [Google Scholar]
- 11.Schefft MR, Swaffar C, Newlin J et al. A novel approach to reducing admissions for children with sickle cell disease in pain crisis through individualization and standardization in the emergency department. Pediatr Blood Cancer . 2018;65(10):e27274. doi: 10.1002/pbc.27274. [DOI] [PubMed] [Google Scholar]
- 12.Della-Moretta S, Delatore L, Purcell M et al. The effect of use of individualized pain plans in sickle cell patients presenting to the emergency department. Ann Emerg Med . 2020;76(3S):S21–S27. doi: 10.1016/j.annemergmed.2020.08.008. [DOI] [PubMed] [Google Scholar]
- 13.Shapiro BS, Benjamin LJ, Payne R, Heidrich G. Sickle cell-related pain: perceptions of medical practitioners. J Pain Symptom Manage . 1997;14(3):168–174. doi: 10.1016/S0885-3924(97)00019-5. [DOI] [PubMed] [Google Scholar]
- 14.Fearon A, Marsh A, Kim J, Treadwell M. Pediatric residents' perceived barriers to opioid use in sickle cell disease pain management. Pediatr Blood Cancer . 2019;66(2):e27535. doi: 10.1002/pbc.27535. [DOI] [PMC free article] [PubMed] [Google Scholar]