Managing Sickle Cell Disease Pain Wally R. Smith, MD Florence Neal Cooper Smith Professor of Sickle Cell Disease

The Pain Response: Psychosocial factors associated with Sickle Cell Disease and Health Care Utilization This flow chart shows a bio-psychosocial model for studying pain in SCD. The model describes the factors that affect responses to pain and determine whether patients seek treatment or stay at home. These factors include gender (men and women respond to pain differently), psychosocial factors (such as stress or depression), illness related variables (such as blood levels or pain severity), readiness to get treatment, and health status. Gender factors affect all variables, for example, men and women respond differently to psychosocial variables and to different cues to action. Psychosocial variables affect cues to action and whether a person decides to go for treatment or stay home and deal with the pain. Illness-related variables, such as severe vs. mild pain, affect readiness variables and cues-to-action. These in turn help a person determine if they should seek treatment or stay home and deal with the pain.

The Majority of SCD Patients Have Pain on Over Half Their Days

Most SCD Pain is Managed at Home

Fatigue May be Nearly as Important as Pain Common, mostly moderate in severity Correlates with hemoglobin Interferes moderately with school, work and exercise Correlates w/ pain, sleep quality, anxiety, depression, stress, QoL Ameringer S, Elswick RK, Smith W. Fatigue in Adolescents and Young Adults with Sickle Cell Disease: Biological and Behavioral Correlates and Health-Related Quality of Life. Journal of pediatric oncology nursing : official journal of the Association of Pediatric Oncology Nurses. 2014;31(1):6-17. doi:10.1177/1043454213514632.

Summary: What we Know About Pain in SCD The “What” The “Why” Pain is far more frequent than once thought, the rule rather than the exception Most pain is NOT called a “crisis” by patients, and is treated at home Fatigue rivals pain as a reported SCD symptom Sleep and quality of life suffer without pain relief The pain experience is very individual-different people respond different ways

Unanswered Questions Does chronic SCD pain only result from patients with frequent acute pain? When does it start? Is SCD chronic pain from damaged nerves or brain rewiring? Does treatment with opioids (narcotics) help or hurt?

Reasons for Opioid Underuse Examples of feelings due to opioid underuse are to deal with side effects; family gatherings; unplanned meetings; religious, spiritual and ritual activities attendance; feeling resigned to pain/ fatalism; medication ineffectiveness; concern about declining functioning; concern about running early; carelessness/forget to take medication; social stigma/concern about being labeled a complainer; fear of tolerance and concern about addiction; cost of medication/ financial difficulties; moral concerns; having conflicting feelings, attitude; feeling of being responsible to others.

Reasons for Opioid Overuse Some reasons for the overuse of opioids are to deal with severe pain intensity, anticipatory fear of adverse outcomes, avoid going to ER or admission, use of medication for other symptoms (e.g., sleep, anxiety, depression), carelessness, to avoid being labeled as a complainer, being more stable in social events, concern about not getting work done, medication ineffectiveness, and knowing what works for me.

Results: Illustrative quotes Participant #10: “Yeah, I actually, just, stopped taking it… kinda worried about my dependency”. Shows an unwillingness to take opioids, for fear of being dependent Participant #16: “You know it slows me down… or makes me drowsy, if I have, you know things to do”. Illustrates patients’ dislike of side effects of opioids, interference with planned activities Participant #20: “maybe not taking it…the kids take advantage…’cause they know it makes me drowsy, and it puts me to sleeps so they… take advantage”. Illustrates patient caught between needing to use opioids for pain control and not wanting opioids for various reasons. Hypothesis: this can lead to various levels of adherence over time