Friday, August 16, 2019

Resident Journal Review: Update on Attitudes Towards Patients with Sickle Cell Disease and Effects on the Provision of High-Quality Care in the Emergency Department

Authors: Hannah Goldberg, MD; Sharleen Yuan, MD PhD; Samantha Yarmis, MD
Editors: Kami M. Hu, MD FAAEM and Kelly Maurelus, MD FAAEM
Originally published: Common Sense January/February 2019

Do emergency physicians have biases towards patients with sickle cell
disease and do biases affect the delivery of appropriate care?

Many patients with sickle cell disease (SCD) have disease that is well managed in the outpatient setting. However, among patients with severe symptoms of SCD, there is a high recidivism rate in the emergency department (ED).[1] Care of patients with sickle cell disease with vasoocclusive crises (VOC) can often elicit frustration on the part of both the patient and the emergency physician due to many factors. These patients tend to have pain that is difficult to assess, as well as a high opiate tolerance requiring large doses to control pain. Additionally, physicians can have negative feelings about patients with sickle cell disease, with hesitancy regarding redosing of parental opioids due to concerns about opiate addiction and drug-seeking behavior.[2] These exist despite evidence that patients with sickle cell generally present with less outward distress or vital sign abnormality despite sincere pain and that lab-work does not correlate to presence of VOC or severity of associated discomfort.[3] We attempt to discern how pervasive these negative biases may be and whether or not they affect patient care.

Glassberg J, Tanabe P, Chow A et al. Emergency provider analgesic practices and attitudes toward patients with sickle cell disease. Ann Emerg Med. 2013;62(4):293-302.
The American Pain Society and National Heart, Lung, and Blood Institute (NHLBI) have issued national guidelines making recommendations for emergency department management of acute pain crises.[4] There is, however, a perception among patients and specialists that emergency physicians often deviate from these guidelines while treating patients with SCD in the emergency department. It had been unclear whether this nonadherence was due to emergency providers being unaware of the
existence of these guidelines, or whether providers were aware but their compliance was hindered by conscious or subconscious negative feelings towards patients with SCD.

In this 2013 cross-sectional convenience sample survey study, Glassberg, et al., surveyed 795 emergency medicine providers and examined the association of negative attitudes toward patients with sickle cell disease and adherence to the NHLBI guidelines. Surveys were made available
to attendees of the 2011 American College of Emergency Physicians (ACEP) Scientific Assembly in San Francisco, California. A 33-question survey assessed physicians’ demographics, provider practice patterns, and provider attitudes towards patients with SCD. Specifically, practice patterns assessed included pharmacologic approach including choice of agent, route, dose, and frequency of selection. Of the 795 surveyed, 671 answered completely and were included in analysis. In analysis of provider attitudes, six survey items were grouped together to form a “negative attitudes scale” (mean score 39.5±21.9; potential range 0-100). Higher scores indicated more negative feelings about sickle cell disease patients. Four items grouped together to form a “positive attitudes scale” (mean score 37.1±23.1; potential range 0-100), where higher scores indicated more positive views about patients with SCD. Five items grouped together to form a “red-flag behavior scale” (mean score 58.7±22.4; potential range 0-100), where higher scores indicated a greater belief that certain sickle cell disease patient behaviors indicate that the patient is inappropriately drug-seeking.

Glassberg, et al., found that negative views of patients with SCD were associated with lower guideline adherence. Of survey responders, pediatric providers had more positive attitudes, and physicians working in environments that see a higher frequency of patients with SCD had worse attitudes. Providers who identified themselves as black had more positive attitudes and lower scores on the red-flag behavior scale. Interestingly, when controlled for outside factors, working at institutions that had comprehensive sickle cell disease clinics did not significantly influence provider attitudes towards patients with SCD.

The most common medications prescribed to treat pain were morphine (95% of physicians) and hydromorphone (91% of physicians). Eighty-five percent of those surveyed indicated they were comfortable reassessing and redosing opioids if the patient’s analgesia was inadequate. Pediatric emergency providers were six times more likely than adult providers to use patient-controlled analgesic devices for pain management. Highvolume ED providers were less likely to reassess and redose pain medication after 30 minutes.

Results of the survey administered in this study indicate that emergency department treatment (and particularly administration of opioid pain medication) of SCD patients with VOC is significantly affected by provider attitudes towards this patient population. Of the eight recommendations made by NHLBI, those less accepted by emergency physicians included use of subcutaneous over intramuscular opioids, hypotonic fluid administration, frequent acetaminophen use, and sparing use of non-steroidal anti-inflammatory drugs. Of particular importance in our patient population, the study found that providers who frequently treat patients with SCD were more likely to have negative attitudes and were also less likely to adhere to the main recommendation from the NHLBI: to reassess pain every 30 minutes and redose opioid medications as needed.

Limitations in this study are mostly related to sample population. As this study was given at a large academic assembly, a disproportionate number of those surveyed (67.9%) were affiliated with academic medical centers and were more likely to be aware of national guidelines than emergency providers in the community. Additionally, a frequent limitation to surveys is a bias towards selecting the “correct” response when presented with a number of options where one option is obviously accepted as the correct action, even if this is not representative of the provider’s actual practice. A major criticism of ED providers is non-compliance in reassessing and redosing pain medication every thirty minutes. It is important to consider potential confounding variables; emergency physicians may be tending to multiple or critically-ill patients and simply do not have the time available to reassess stable patients every thirty minutes. Given this limitation, it is more difficult to arrive at the conclusion that ED physicians are unwilling to reassess SCD patients frequently as recommended by the guidelines.

Overall, this study suggests that emergency physicians are well-trained inselecting the appropriate pain medication but are not generally in compliance with the NHLBI guidelines, and often do not reassess pain or readminister pain medication frequently enough in patients with SCD. Data from this study further suggests that adequate pain management is likely hindered by physician negative attitudes towards patients with SCD and that physicians may benefit from self-awareness of their own potential biases, education regarding the presentation of pain in patients with SCD, and greater empathy in the treatment of their patients.

Glassberg J, Tanabe P, Richardson L, & DeBaun M. Among emergency physicians, use of the term “sickler” is associated with negative attitudes toward people with sickle cell disease. Am J Hematol. 2013;88(6):532-533. 
The use of labels to describe patients with chronic diseases is not uncommon, but in SCD the label of “sickler” correlates with negative attitudes in emergency medicine. Another survey conducted by Glassberg and colleagues at the 2011 ACEP annual meeting hypothesized that physicians utilizing the “sickler” nomenclature both entertained negative attitudes and were less likely to follow practice guidelines/protocols regarding SCD management.

A total of 655 emergency physicians from 49 states (2% of the 32,000 ACEP members) were surveyed. Individual physicians were approached at ACEP 2011 to provide information regarding demographics, provider practice patterns, and provider attitudes. The question “How often do you refer to a patient with SCD as a ‘sickler’?” was the primary predictor variable using the 4-point Likert scale. Survey data indicated that the term “sickler” was commonly used, with 8.7% of responders answering “always,” 43.3% “frequently,” 34.7% “rarely,” and 13.1% “never”. Using the “rarely” reference group as the control group, negative attitudes were 17.1 points higher on the negative attitudes scale in the “always” reference group and 7.8 points lower on the negative attitudes scale in the “never” group. While there was a significant difference of attitudes regarding SCD and the “sickler” terminology, there was no significant correlation to lower adherence to existing NHLBI guidelines. There was, however, a difference in the redosing of opioids within 30 minutes: over 70% of physicians that use the term “sickler” would redose, but those who used the term “frequently”
were 12% less likely to do so.

There are limitations of this survey, including a small percentage (2%) of ACEP members, who may not be representative of general practice. A majority of respondents were also from academic emergency departments. Additionally, self-reporting may not accurately describe adherence to guidelines or practice.

Singh A, Haywood Jr C, Beach MC, et al. Improving emergency providers’ attitudes toward sickle cell patients in pain. J Pain Symptom Manag. 2016;51(3):628-632.
The surveys above indicate that some emergency medicine providers have negative attitudes regarding patients with SCD. Previous research has shown that attitudes improved in internists and nurses regarding SCD patients after viewing a short video about interventions.[5] This study focuses on emergency physicians and their attitudes after viewing a video about SCD and commonly used interventions.

The study used a single group pretest/multiple posttest design, surveying a total of 96 participants including attendings, residents, nurse practitioners (NPs), physician assistants (PAs), and nurses in a large, urban, academic emergency department. The primary outcome was an assessment of the attitudes of providers using the Sickle Cell Patients Scale (which examines positive and negative attitudes, red flag behaviors). Participants completed a baseline survey on SCD attitudes prior to watching an 8-minute video featuring SCD patients and ED providers discussing challenges, misinformation, stereotypes, biases, and perspectives of sickle cell disease. A posttest survey was given at one week and then again at three months after the video viewing.

The authors discovered that after watching the video, there was a statistically significant decrease in negative attitude scores (-11.5 difference, 95% CI -14.3 to -8.7) and in red flag behavior scores (-12.8 difference, 95% CI -16.3 to -9.3), with a significant increase in positive attitude scores (+10 difference, 95% CI 6.6 to 13.45). The scores at three months displayed a small amount of attenuation from the initial intervention effect (4.5 difference, 95% CI -7.9 to -1.0).

Limitations of this study include the urban environment, which may limit generalizability. There is also potential selection bias in that academic providers knowledge and behaviors might be different from the general healthcare provider population, and surveys are always subject to possible
social desirability bias – bias introduced when respondents answer questions in a manner that would be most accepted to others though they may not be consistent with their actual views or practices. Additionally, the control group design was within groups as a before-and-after study, as opposed to between groups.

Overall, this study demonstrated that brief video-based interventions can improve providers’ attitudes regarding patients with SCD in the emergency department. Perception and attitude adjustments can change barrier to management and treatment, which could improve patients’ quality of care and experience.

Binding A, Ward R, Phua C, et al. An innovative short-stay health care model for treatment of uncomplicated vaso-occlusive crisis in adult sickle cell disease patients in Canada to reduce emergency department utilization. CJEM 2017:1-8.
Approximately 90% of acute health care visits for patients with SCD occur for vaso-occlusive crisis (VOC). Management of VOC involves early analgesia, preferably within 30 minutes, but this goal is frequently not met in the ED. Short-stay units have been developed in several countries specifically to treat VOC outside of the ED, with a goal of treating symptoms more promptly, reducing costs, and decreasing hospital admission rates. This study was the first evaluation of a short-stay unit in Canada and its effect on patient satisfaction and clinical outcomes.

The study included patients aged 18 or older with a known diagnosis of SCD who were followed at University Health Network in Toronto. The study was designed as a historical case-controlled study, with a comparison of patients during the existence of the short-stay unit (October 2014 – July 2016) to those at a prior time period (August 2009 – September 2012). Patients who were followed in the SCD clinic could call a clinic nurse who directed them either to the short-stay unit or to the ED.
Patients were excluded if they had symptoms concerning for a “complicated VOC,” defined as pain with neurologic symptoms, chest pain, severe abdominal pain, shortness of breath, priapism, or fever >38.3°C. Historical controls were excluded if they received a transfusion in the ED or did not have a diagnosis of SCD based on clinic notes. The short-stay unit was open 24 hours per day Monday to Friday, and consisted of an internist, nurses, and a pharmacist. Patients received a protocolized treatment with supplemental oxygen, intravenous (IV) fluids, and opioids. Patients were transferred to the ED if they developed signs or symptoms of complications, if their IV pain control requirement exceeded 72 hours, or if they continued to require care after the unit closed for the weekend. The primary outcome was time from patient arrival to receiving first dose of opioid pain medication. Secondary outcomes included the total opioid dose per hour, pain scores on arrival and at certain intervals, unit length of stay, and disposition.

During the study period, there were 21 visits to the short-stay unit by 12 patients. The mean age was 28.2 years old. The mean time from arrival to first opioid dose was 23.5 minutes and the average length of stay was 28.7 hours. Three patients (16%) were admitted, of which two had persistent pain and one had signs of acute chest syndrome. Overall patient satisfaction scores based on surveys administered prior to discharge were high (>4/5 on a Likert scale), with the exception of satisfaction with unit location. In comparison to 80 historical cases, the mean time to first opioid dose was significantly lower (23.5 vs 100.3 min, p<0.001) and mean total opioid dose per hour was higher (46.7 vs. 11.9 morphine equivalent mg/hr, p<0.001). Discharge rate was also higher from the short stay unit (84.2% vs. 69.7%, no p value provided).

This study demonstrated that a short-stay unit is a feasible model to decrease time to analgesia and decrease admission rates for patients with sickle cell disease presenting with uncomplicated VOC. Patient surveys revealed that major barriers to short-stay unit utilization were lack of information regarding its availability and travel distance to the unit. A major limitation of the study was low recruitment, with only 12 unique patients presenting to the unit during the nearly two-year study period. Patients were not sent directly from ED triage, but rather had to call a nurse coordinator from the clinic, which likely resulted in low utilization. In addition, the historical case-control design may contribute to confounding. The authors conclude that a short-stay unit with a protocolized approach is a feasible model for management of uncomplicated VOC. Other medical centers have also displayed promising outcomes utilizing the short stay model for care of SCD patients. Amdemariam, et al., found that after enacting a similar short stay algorithmic model in the ED, there was a significant
decrease in admission rate with shorter hospital length of stays.[6]

There remains a negative attitude towards patients with sickle cell disease amongst some emergency physicians. The recognition of these attitudes is important because studies suggest that physicians with more negative feelings towards patients with SCD provide less adequate care of patients, with the resultant effect of souring physician-patient relationships and further perpetuating negative attitudes. It is important to reassess and redose analgesic medication for VOC every 30 minutes to ensure adequate pain management. Many emergency physicians may not be aware of the NHLBI guidelines for treating patients with SCD. Both physicians and patients would benefit from utilization of these guidelines to help standardize care of patients with SCD and remove the factor of physician attitude and thus the influence that negative attitudes have on patient care. Additionally, at institutions with a high SCD population, it is worth further investigating the implementation of a short-stay model which could standardize care of SCD patients with VOC, thereby decreasing ED utilization and hopefully removing potential contributors to negative attitudes resulting in sub-standard care.

Negative biases regarding patients with sickle cell disease persist amongst the emergency physician population, with evidence that providers with negative attitudes regarding this population provide suboptimal care, which can result in incomplete pain management, poor physicianpatient relationships, prolonged emergency department length of stays, and increased hospital admission rates.1,6 It is important that we seek to assess our personal attitudes, improve them through education, and adhere to existing guidelines for care.


1. Haywood C Jr, Lanzkron S, Ratanawongsa N, et al. Hospital self-discharge among adults with sickle-cell disease (SCD): associations with trust and interpersonal experiences with care. J Hosp Med. 2010;5:289-294.

2. Brown SE, Weisberg DF, Balf-Soran G, Sledge WH. Sickle cell disease patients with and without extremely high hospital use: pain, opioids, and coping. J Pain Symptom Manage. 2015;49(3):539-47.

3. Lovett PB, Sule HP, Lopez BL. Sickle cell disease in the emergency department. Hematol Oncol Clin North Am. 2017;31(6):1061-79.

4. Gibbons GH, Shurin SB. Evidence-Based Management of Sickle Cell Disease. Expert Panel Report, 2014. Last accessed October 29th, 2018.

5. Haywood C Jr, 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;26:518-23.

6. Andemariam B, Odesina V, Owarish-Gross J, et al. A fast-track emergency department acute sickle cell pain management algorithm results in fewer hospital admissions, decreased length of stay, and increased hospital revenue. J Pain. 2014;15(4): S39.

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