Journal Club

Sickle Cell Disease: Perceptions, Behaviors, and Management

Washington University Emergency Medicine Journal Club- September 2024

Hello all,

In honor of National Sickle Cell Awareness Month, the September Journal Club will look at a handful of articles addressing perpections, behaviors, and management related to sickle cell disease in the Emergency Department. A huge thanks to the DEI Committee, and in particular Drs. Jenna Thomas and Ernesto Romo for leading the charge in selecting this topic, choosing the articles, and leading our discussion.

Take special note:
The PGY-1 article will be appraised using the Cohort form…

The PGY-2 article will be appraised using the Therapy form…

The PGY-3 article will be appraised using the Cross-Sectional form…

The PGY-4 article will be appraised using the Qualitative Study form.


Vignette

You’re working a shift at one of your community emergency medicine sites when
you encounter Ms. Z, a 28-year-old woman with a history of sickle cell anemia who
presents with severe, generalized body pain. She reports that the pain began the
previous evening and has progressively worsened, describing it as a “deep,
throbbing pain” in her arms, legs, and back, typical of her vaso-occlusive crises. She
has had multiple admissions for similar episodes in the past and is currently taking
hydroxyurea and scheduled opioids at home. She rates her pain at 10/10 and
appears visibly distressed.


On initial evaluation, her vital signs are stable, but she is in obvious discomfort. She
explains that she typically requires higher doses of opioids due to her chronic pain
management and expresses frustration over being labeled as “drug-seeking” in the
past. A review of her medical records shows a history of frequent emergency visits,
with varying regimens of opioid use.


While you attempt to manage her pain, you note challenges in balancing adequate
pain control with concerns about opioid overuse. You initiate IV fluids, administer a
dose of IV opioids, and consider adjunctive therapies. Despite these efforts, Ms. Z
remains in significant pain and requests additional medication, stating that she is
often undertreated in the ED. You consult with the hematologist, who recommends
continuing opioids and reassessing frequently.


As you care for Ms. Z, you reflect on the complexities of pain management in sickle
cell patients. You wonder about the latest evidence on effective pain protocols, the
role of opioid-sparing strategies, and the psychosocial aspects of managing chronic
pain in this population. You head to the literature to explore these questions…


Article 1: Puri Singh A, Haywood C Jr, Beach MC, et al. Improving Emergency
Providers’ Attitudes Toward Sickle Cell Patients in Pain. J Pain Symptom Manage.
2016 Mar;51(3):628-32.e3. [Answer Key].
Article 2: Della-Moretta S, Delatore L, Purcell M, Huang Y, Heinlein M, Adkins E,
Desai P. The Effect of Use of Individualized Pain Plans in Sickle Cell Patients
Presenting to the Emergency Department. Ann Emerg Med. 2020 Sep;76(3S):S21-
S27. [Answer Key].
Article 3: Abdallah K, Buscetta A, Cooper K, et al. Emergency Department Utilization
for Patients Living With Sickle Cell Disease: Psychosocial Predictors of Health Care
Behaviors. Ann Emerg Med. 2020 Sep;76(3S):S56-S63. [Answer Key].
Article 4: Young AJ, Richardson F, Fitzgerald D, Heavrin BS, Tweddell B, Gettings L,
Cooper A, Grant CC. Let Their Voices Be Seen. Ann Emerg Med. 2020 Sep;76(3S):S73-
S77. [Answer Key].


Bottom Line

Sickle cell disease (SCD) is a chronic, debilitating illness that affects approximately 100,000 people in the United States, the vast majority of whom are African American. Patients with SCD frequently present to the emergency department for vasoocclusive pain crises, which are often under-treated (Decosterd 2007), likely in part due to negative perceptions of these patients on the part of emergency medicine providers (Shapiro 1997). It is not uncommon for patients to delay seeking care in the emergency department for such crises. Researchers at the NIH Clinical Center in Bethesda, MD and the Montefiore Medical Center in Bronx, NY, sought to identify and quantify the factors leading to such delay, and found that higher experience of stigma associated with SCD was strongly associated with delaying care (OR 1.09, 95% CI 1.03 to 1.16), as were more frequent pain episodes and more frequent ED visits in the preceding year.

In an attempt to improve healthcare providers perceptions of patients with SCD, researchers at a large urban inner city ED studied the effects of an eight-minute video featuring SCD patients and ED providers in which misinformation, stereotypes, and biases often held by ED providers toward the SCP population were examined and reviewed along with actual experiences and characteristics of SCD patients. A group of 96 healthcare providers—57% of whom were nurses, 10% attending physicians, and 33% residents and midlevel providers—were asked to complete the General Perceptions about Sickle Cell Patients Scale, a survey assessing negative attitudes, positive attitudes, and red-flag behaviors related too SCD, before, immediately after, and three months after viewing the video. They found that  negative attitudes, positive attitudes, and red flag behaviors all improved immediately after viewing the video. These improvements persisted, though were quite attenuated, three months later.

Current guidelines from the NHLBI recommend that upon presentation to the emergency department for acute, vasoocclusive pain crises, analgesia therapy be initiated within 30 minutes of triage or within 60 minutes of registration. Multiple studies have demonstrated that this measure is frequently not met in both adults (Singh 2023) and pediatric patients (Brousseau 2020). Researchers at the Ohio State University Wexner Medical Center in Columbus, Ohio sought to implement individualized pain plans for patients with SCD—including opioid, dose, and administration frequency—accessible in the medical record. Education was provided on the need to check for, and how to access, the plan. Workflow allowed for a first dose of pain medication to be administered in the triage area and frequent feedback was provided to healthcare providers regarding opioid performance. The primary goal—a decrease in the time to first opioid administration—was achieved following implementation of these individualized pain plans. Average and median time to opioid usage were 173.5 minutes and 132 minutes, respectively, before the protocol implementation, and decreased significantly to 86.3 minutes and 60 minutes after the protocol was initiated. A 22% reduction in ED length of stay was also observed.

Given the chronic pain associated with SCD, a known decrease in life expectancy, and the stigma often heaped upon theses patients, it is no wonder that many such patients feel misunderstood and mistreated by the medical community. To better understand the perceptions of patients living with SCD, researchers in Memphis, TN enlisted the aid of 8 adults with SCD in using photovoice—a worldwide project that seeks to “combine ethical photography and community engagement”—to elucidate themes related to communication with clinicians, living with pain, ED experiences, and support systems. Participants were provided an instructive handbook on taking photographs and a journal to record their thoughts. They were tasked with taking photographs over a 4-week period, after which they came together for a critical dialogue, moderated by a member of the team. Each participant discussed his or her favorite photograph and introduced it to the group by answering these questions:

  1. What were your thoughts when you took this photograph?
  2. What does it represent to you?
  3. What do you hope someone else will see in it?

Over the course of the 2.5-hour discussion, the moderator and a note taker kept track of themes that emerged from the discussion. The following six themes were agreed upon by all participants:

  1. Unpredictability of SCD
  2. Fickleness of time
  3. Coping with pain
  4. Proximity to death
  5.  Avoidance of the ED
  6. A need for improved communication.

By raising awareness of these themes, which are likely experience by many patients with SCD, the authors hoped to improve communication, trust, and shared decision-making between ED providers and SCD patients cared for in our environment.