expert roundtables

Sickle Cell Disease: Managing Pain Without Encouraging Opioid Dependence

by Stella T. Chou, MD; Michael Rutledge DeBaun, MD, MPH; and Julie Kanter, MD

Overview

Patients with sickle cell disease (SCD) experience numerous pain episodes throughout the course of their disease. Educating patients and their families about pain, embracing a comprehensive approach to pain management that incorporates nonpharmacologic treatments, and reducing the risks associated with opioid analgesics are central to the successful long-term care of individuals with SCD.

Q:

How do you approach pain management in patients with SCD, and what are your thoughts on the role of opioid analgesics?

Stella T. Chou, MD

Associate Professor of Pediatrics
Chief, Division of Transfusion Medicine
Children’s Hospital of Philadelphia
Perelman School of Medicine at the University of Pennsylvania
Philadelphia, PA

“For those patients who do require extended opioid therapy, weekly outpatient visits can help them develop much closer relationships with their physicians.”

Stella T. Chou, MD

When treating patients with SCD, specifically as it relates to the issue of pain management, one of the most important things that we can do is to spend adequate time with the patient and their family to truly understand what is causing that pain. We know that SCD impacts the whole family. From the parents’ perspective, trying to navigate the pain management of your own child is going to be difficult. No parent wants to see their child in pain or have an adverse outcome from something that was intended to treat that pain.

We do rely on parents to help us with the pain assessment, especially for the younger children. For instance, parents can offer insights on how the pain is manifesting this time, as opposed to past experiences, and they provide insight into what else may be happening in the child’s life. We can ask them whether they believe that any recent stressor or other health issue may be contributing, which can help tease out whether this is typical childhood pain or, in fact, an acute vaso-occlusive pain event.

For those patients who do require extended opioid therapy, weekly outpatient visits can help them develop much closer relationships with their physicians. More frequent visits with the other members of their SCD health care team, including psychologists and social workers involved in their pain management, can also greatly improve the care of patients. Obviously, these types of interventions require time; in fact, it typically requires up to a year to be successful in weaning chronic opioid dependence. While this can be a major time commitment, it can prove very effective.

Michael Rutledge DeBaun, MD, MPH

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

“A comprehensive approach to pain management empowers clinicians and individuals with SCD to seek individualized, evidence-based solutions to acute and chronic pain beyond simply adding more pain medication.”

Michael Rutledge DeBaun, MD, MPH

When we educate families about the management of acute vaso-occlusive pain, our first objective is to identify individualized nonpharmacologic strategies. The families must understand that the approach to pain management does not always include taking a nonsteroidal anti-inflammatory drug or an opioid analgesic. Thomas D. Coates, MD, and colleagues conducted novel research demonstrating that the onset of stress in individuals with SCD decreases microvascular blood flow and increases the likelihood of red blood cell entrapment and progression to vaso-occlusive pain events.  

Clinicians can support individuals with SCD and their families in learning to recognize pain triggers and that pain management does not always involve an opioid. While opioids have a place in acute pain management during a vaso-occlusive pain episode, unfortunately, some clinicians have mistakenly assumed that having a diagnosis of SCD is equated with the need for lifelong daily pain medication.

Part of the onus for improving pain management in SCD is on us, as clinicians, to spend the time educating individuals with SCD and their families about nonmedicinal strategies to manage pain and to help the patient distinguish SCD-related pain from the other types of pain that occur in the general population (eg, lower back pain and overuse pain of selected joints with sports injuries).  

Ultimately, to optimally manage acute and chronic pain in children and adults with SCD, health care providers must engage in a lifelong learning process about the pharmacologic and the nonpharmacologic approaches to pain management. Cognitive behavioral therapy is emerging as a promising strategy for patients with SCD. Further, both clinicians and patients should acknowledge the implications that depression and anxiety are associated with an increased incidence of acute and chronic pain in the general population and in patients with SCD. Such a comprehensive approach to pain management empowers clinicians and individuals with SCD to seek individualized, evidence-based solutions to acute and chronic pain beyond simply adding more pain medication.

Julie Kanter, MD

Associate Professor, Division of Hematology and Oncology
Director, Adult Sickle Cell Program
Codirector, Lifespan Comprehensive Sickle Cell Center
University of Alabama at Birmingham
Birmingham, AL

“As we move forward, knowing that our patients are hopefully going to survive for many years to come, well into adulthood, I think that it is important to take a slight step back to see the full picture. We should continue to be very aggressive in managing acute SCD pain crises, but perhaps we should be less aggressive in moderating normal childhood pain.”

Julie Kanter, MD

I agree with Dr Chou and Dr DeBaun on the importance of a comprehensive approach, and I would add that there are many reasons to be avoidant of opioid analgesics in SCD, with the risk of opioid dependence being just one of these reasons.

Part of the problem is how we (as medical providers) have approached SCD in the past and what we need to change as more individuals are growing into adulthood. Specifically, a major component of our counseling and discussion with patients and families has been related to pain recognition and management. We have spent time developing pain plans with escalating medication regimens and have suggested that patients need to seek acute care if their pain does not relent. Now, this may even be specific to the United States in some respects in that we did not spend enough time in the past teaching patients how to cope with pain. For example, a patient with SCD was recently hospitalized and receiving intravenous hydromorphone for several days for what was later determined to be acute tendonitis. Tendonitis is, of course, something that is very common and can be painful, but, certainly, if you or I sought medical attention for tendonitis, intravenous hydromorphone would not be the expected result. Thus, we need to make sure that we are tailoring the use of opioids for severe SCD-related vaso-occlusive crisis and not using them in places where other modalities may be both more effective and better in the long run for the patients.

As we move forward, knowing that our patients are hopefully going to survive for many years to come, well into adulthood, I think that it is important to take a slight step back to see the full picture. We should continue to be very aggressive in managing acute SCD pain crises, but perhaps we should be less aggressive in moderating normal childhood pain.

References

Chalacheva P, Ji Y, Rosen CL, DeBaun MR, Khoo MCK, Coates TD. Nocturnal peripheral vasoconstriction predicts the frequency of severe acute pain episodes in children with sickle cell disease. Am J Hematol. 2021;96(1):60-68. doi:10.1002/ajh.26014

Khaleel M, Puliyel M, Shah P, et al. Individuals with sickle cell disease have a significantly greater vasoconstriction response to thermal pain than controls and have significant vasoconstriction in response to anticipation of pain. Am J Hematol. 2017;92(11):1137-1145. doi:10.1002/ajh.24858

Osunkwo I, O’Connor HF, Saah E. Optimizing the management of chronic pain in sickle cell disease. Hematology Am Soc Hematol Educ Program. 2020;2020(1):562-569. doi:10.1182/hematology.2020000143

Osunkwo I, Veeramreddy P, Arnall J, et al. Use of buprenorphine/naloxone in ameliorating acute care utilization and chronic opioid use in adults with sickle cell disease. Blood. 2019;134(suppl 1):790. doi:10.1182/blood-2019-126589

Shah P, Khaleel M, Thuptimdang W, et al. Mental stress causes vasoconstriction in subjects with sickle cell disease and in normal controls. Haematologica. 2020;105(1):83-90. doi:10.3324/haematol.2018.211391

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