patient care perspectives

Pain Management and Opioid Analgesics for Sickle Cell Disease

by Michael Rutledge DeBaun, MD, MPH

Overview

The assessment of pain in a patient with sickle cell disease (SCD) should elicit whether the pain is acute, chronic, and/or related to the SCD. Appropriate use of opioid analgesics in SCD-related pain episodes relies on recognition of the multidimensional aspects of pain, pharmacologic principles, and trust between patients and health care providers.

Expert Commentary

Michael Rutledge DeBaun, MD, MPH

Professor of Pediatrics and Medicine
Vice Chair for Clinical and Translational Research
J.C. Peterson Chair in Pediatrics
Director, Vanderbilt-Meharry Center for Excellence in Sickle Cell Disease
Vanderbilt University Medical Center
Nashville, TN

"No single objective tool can measure pain satisfactorily. Trust between the affected individual in pain and the health care provider is essential, and pain assessment tools must reflect the multidimensional aspects of pain."

Michael Rutledge DeBaun, MD, MPH

Patients with SCD can experience both acute pain episodes caused by vaso-occlusive events and debilitating chronic pain syndromes, and we need to take the time to have a conversation with our patients to uncover the degree and frequency of their pain. I ask my patients to describe their pain. Is it a stabbing pain? Is it a sharp pain? Is it a chronic pain? If I touch your back, does it hurt? Is it a pain that is associated with bad body posture and weak core muscles? These are some of the questions I ask. If we do not ask these types of questions, we cannot accurately assess the patient’s pain and we will not be able to provide appropriate pain relief. Further, no single objective tool can measure pain satisfactorily. Trust between the affected individual in pain and the health care provider is essential, and pain assessment tools must reflect the multidimensional aspects of pain (ie, pain intensity is important, but so are other aspects of pain, such as chronicity and functional impact). 

Misinformation about the appropriate use of opioid analgesics in SCD-related pain episodes is a formidable challenge. Unfortunately, there is a strong bias among many health care providers that patients with SCD have opioid abuse syndrome. Many clinicians are wary of even the appropriate use of opioid analgesics (eg, continuous infusion of pain medication at night, during an inpatient crisis), and this may be due, in part, to backlash from the opioid epidemic.

Once hospitalized, there are 3 broad approaches to pain management in children and adults with SCD. The first is to maintain a continuous infusion of pain medication and titrate the dose to the point where the patient is comfortable after several boluses. The goal is to have the continuous infusion be within the therapeutic window. If the dose is too high, the patient will be sedated. If the dose is too low, the patient will remain in pain. Even when the patient is in the ideal therapeutic window, breakthrough acute pain and brief acute exacerbations of the pain may occur, and patient-controlled analgesia (PCA) can be used to provide immediate pain relief. We use such a strategy with PCA where the majority of the opioid dose is given by a continuous intravenous route and the demand dosing is provided every 20 minutes at a dose equivalent to one-sixth of the hourly infusion dose. The demand dose is used for breakthrough pain and is not intended to substitute for a continuous infusion. This strategy rarely results in sedation, and the number of times the patient tries to activate the PCA in 1 hour, when observed over a 2- to 4-hour window, also provides a sense of the pain relief (ie, a patient who hits the PCA 10 times in a 3-hour period can often safely go up in their hourly infusion and their PCA dose). The patient can then be observed closely at the new upwardly adjusted hourly infusion for side effects, with reevaluation of the increase in the use of PCA per hour. The increase in the new dose is not likely to cause respiratory depression because the continuous infusion dose of the opioid is what the patient was given in the prior hours.

Another approach that relies primarily on the PCA pump as the main source of achieving the therapeutic window has not been our strategy, owing to a number of limitations with that method. The third approach is generally not accepted anymore (ie, as-needed pain medication written for oral, intravenous, or both) and may result in an antagonistic relationship between the patient and the nurses when as-needed requests are perceived as too frequent or inappropriate.

References

Ataga KI, Kutlar A, Kanter J, et al. Crizanlizumab for the prevention of pain crises in sickle cell disease. N Engl J Med. 2017;376(5):429-439.

Balsamo L, Shabanova V, Carbonella J, et al. Improving care for sickle cell pain crisis using a multidisciplinary approach. Pediatrics. 2019;143(5). pii: e20182218.

Brandow AM, DeBaun MR. Key components of pain management for children and adults with sickle cell disease. Hematol Oncol Clin North Am. 2018;32(3):535-550.

Dampier C, Palermo TM, Darbari DS, Hassell K, Smith W, Zempsky W. AAPT diagnostic criteria for chronic sickle cell disease pain. J Pain. 2017;18(5):490-498.

Murad MH, Liem RI, Lang ES, et al. 2019 sickle cell disease guidelines by the American Society of Hematology: methodology, challenges, and innovations. Blood Adv. 2019;3(23):3945-3950.

Niihara Y, Miller ST, Kanter J, et al; Investigators of the Phase 3 Trial of I-Glutamine in Sickle Cell Disease. A phase 3 trial of l-glutamine in sickle cell disease. N Engl J Med. 2018;379(3):226-235.

Ruta NS, Ballas SK. The opioid drug epidemic and sickle cell disease: guilt by association. Pain Med. 2016;17(10):1793-1798.

Uwaezuoke SN, Ayuk AC, Ndu I, Eneh CI, Mbanefo NR, Ezenwosu OU. Vaso-occlusive crisis in sickle cell disease: current paradigm on pain management. J Pain Res. 2018;11:3141-3150.

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