The Hematologist

March-April 2018, Volume 15, Issue 2

What We Know and Do Not Know About Chronic Pain in Adults With Sickle Cell Disease

Michael DeBaun, MD, MPH Professor of Pediatrics and Medicine, Director of the Vanderbilt-Meharry Center for Excellence in SCD
Vanderbilt University Medical Center, Nashville, TN

Published on: February 02, 2018

Field J. Five lessons learned about long-term pain management in adults with sickle cell disease. Hematology. 2017;2017:406-411.

The current opioid epidemic presents unique challenges for adults with sickle cell disease (SCD) and the providers who care for them. Given the public discourse about opioid misuse, health care providers for children and adults with SCD must be especially cognizant of their prescribing practices and emerging state-based opioid regulations that may impact management of acute and chronic pain in children and adults with SCD.

For decades, a disproportionate number of physicians and nurses have harbored negative attitudes about pain management in adults with SCD. In one study, 53 percent of emergency department physicians and 23 percent of hematologists surveyed thought that more than 20 percent of adults with SCD have opioid abuse syndrome.1 Similarly, 63 percent of nurses surveyed believed opioid abuse syndrome was common in the adult SCD population, and 30 percent were reluctant to give the recommended dose of opioids upon evaluation.2 The consequences of this systematic bias is undertreatment of pain for many adults with SCD.

Fortunately, health care provider attitudes about pain management are not immutable. A Johns Hopkins eight-minute video serves as an educational resource for health care providers focused on general challenges of pain management and the patient’s perspective of acute pain management in patients with SCD.3 Viewing this short video has improved health care provider attitudes about treating pain in adults with SCD using standard care guidelines.4

Dr. Joshua J. Field highlighted the five lessons for management of pain in adults with SCD. These lessons are: 1) Focus on adults with high rates of resource use for pain; 2) treat underlying causes of pain, including SCD, aggressively; 3) apply principles of chronic pain management to a clinic model; 4) understand that higher doses and larger quantities of oral opioids often do not help; and 5) realize that parenteral opioids are not the optimal treatment of chronic pain. These lessons highlight the challenges of managing pain in adults with SCD and provide clear strategies for assessment of common problems.

Missing from the lessons learned is the significant overlap between chronic pain and depression and the role of the primary care provider. In a recent review, individuals with SCD and depression had almost a three times greater relative risk of being high health care utilizers and 50 percent greater rates of hospitalization (2.9 vs. 1.8 hospitalizations per year) when compared with individuals without depression.5 The optimal management of depression in adults with SCD with chronic pain is not known. However, the presence of depression, a common and potentially modifiable risk factor in adults with SCD, warrants further evaluation. If adults with SCD do not have primary care providers, then the adult hematologist or health care provider is compelled to ensure that the shared strategies for management of chronic pain in the general population are included in the treatment of adults with SCD.

As Dr. Field points out in his article, there is a paucity of evidence in management of acute and chronic pain, and even less evidence as to the modifiable risk factors driving chronic pain and optimal strategies for its management. Key to Dr. Field’s approach is a trusting relationship between the patient and the provider, coupled with a stepwise approach for increased intensity in medical management of chronic pain. Specifically, if the patient averages at least one episode of pain per month and is on a maximum-tolerated dose of hydroxyurea therapy, then regular blood transfusion therapy is offered to decrease the maximum hemoglobin S concentration to less than 50 percent. If this approach does not abate the frequency of admissions for acute pain and the hemoglobin S concentration is kept to less than 50 percent, then Dr. Field recommends erythrocytapheresis with a goal of keeping the maximum hemoglobin S concentration significantly lower than 50 percent.

Is there a randomized controlled trial to support Dr. Field’s practical strategy of managing recurrent severe acute pain episodes in adults with SCD? No. However, all interventions do not require randomized controlled trials when the evidence is overwhelmingly positive and the benefits clearly outweigh the risks. Even after hematopoietic stem cell transplantation, individuals cured of SCD can have residual chronic pain syndrome for a prolonged duration after complete engraftment of donor cells. Thus, regular blood transfusions are not expected to be the definitive cure for chronic pain, but in a subset of patients, they decrease the frequency and intensity of pain episodes. Clearly more investigation is required to develop a rational approach to prevent and treat chronic pain.

What are hematologists and other health care providers to do when the most common problem affecting 50 percent of adults with SCD, recurrent severe pain, is not part of the private or public funding agencies’ research agendas? We use the strategy that Dr. Field has described, which is less than optimal, but pragmatic, compassionate, and carefully crafted to address the pressing needs of our patients and their families.


  1. Shapiro BS, Benjamin LJ, Payne R, et al. Sickle cell-related pain: perceptions of medical practitioners. J Pain Symptom Manage. 1997;14:168-174.
  2. Pack-Mabien A, Labbe E, Herbert D, et al. Nurses' attitudes and practices in sickle cell pain management. Appl Nurs Res. 2001;14:187-192.
  3. CRISIS: experiences of people with sickle cell disease seeking health care for pain. Last accessed January 25, 2018.
  4. Haywood Jr. C, 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;26:518-523.
  5. Jonassaint CR, Jones VL, Leong S, et al. A systematic review of the association between depression and health care utilization in children and adults with sickle cell disease. Br J Haematol. 2016;174:136-147.

Conflict of Interests

Dr. DeBaun indicated no relevant conflicts of interest. back to top