Paula Tanabe

Management of Sickle Cell Disease: Summary of the 2014 Evidence-Based Report by Expert Panel Members

IMPORTANCE Sickle cell disease (SCD) is a life-threatening genetic disorder affecting nearly 100,000 individuals in the United States and is associated with many acute and chronic complications requiring immediate medical attention. Two disease-modifying therapies, hydroxyurea and long-term blood transfusions, are available but underused. OBJECTIVE To support and expand the number of health professionals able and willing to provide care for persons with SCD. EVIDENCE REVIEW Databases of MEDLINE (including in-process and other nonindexed citations), EMBASE, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, CINAHL, TOXLINE, and Scopus were searched using prespecified search terms and keywords to identify randomized clinical trials, nonrandomized intervention studies, and observational studies. Literature searches of English-language publications from 1980 with updates through April 1, 2014, addressed key questions developed by the expert panel members and methodologists. FINDINGS Strong recommendations for preventive services include daily oral prophylactic penicillin up to the age of 5 years, annual transcranial Doppler examinations from the ages of 2 to 16 years in those with sickle cell anemia, and long-term transfusion therapy to prevent stroke in those children with abnormal transcranial Doppler velocity (≥200 cm/s). Strong recommendations addressing acute complications include rapid initiation of opioids for treatment of severe pain associated with a vasoocclusive crisis, and use of incentive spirometry in patients hospitalized for a vasoocclusive crisis. Strong recommendations for chronic complications include use of analgesics and physical therapy for treatment of avascular necrosis, and use of angiotensin-converting enzyme inhibitor therapy for microalbuminuria in adults with SCD. Strong recommendations for children and adults with proliferative sickle cell retinopathy include referral to expert specialists for consideration of laser photocoagulation and for echocardiography to evaluate signs of pulmonary hypertension. Hydroxyurea therapy is strongly recommended for adults with 3 or more severe vasoocclusive crises during any 12-month period, with SCD pain or chronic anemia interfering with daily activities, or with severe or recurrent episodes of acute chest syndrome. A recommendation of moderate strength suggests offering treatment with hydroxyurea without regard to the presence of symptoms for infants, children, and adolescents. In persons with sickle cell anemia, preoperative transfusion therapy to increase hemoglobin levels to 10 g/dL is strongly recommended with a moderate strength recommendation to maintain sickle hemoglobin levels of less than 30% prior to the next transfusion during long-term transfusion therapy. A strong recommendation to assess iron overload is accompanied by a moderate strength recommendation to begin iron chelation therapy when indicated. CONCLUSIONS AND RELEVANCE Hydroxyurea and transfusion therapy are strongly recommended for many individuals with SCD. Many other recommendations are based on quality of evidence that is less than high due to the paucity of clinical trials regarding screening, management, and monitoring for individuals with SCD.

A prospective study of ED pain management practices and the patient's perspective

OBJECTIVE This study was conducted to describe the prevalence of pain in the emergency department and to identify factors that may contribute to its treatment. METHODS Interviews were conducted with 203 patients who entered the emergency department during the study period. Patients were interviewed regarding various aspects of their pain. Medical records were reviewed to determine what treatments were provided. RESULTS One hundred sixty of the 203 patients came to the emergency department with a chief complaint related to pain, indicating a prevalence rate of 78%. Approximately 58% of all patients received either medication or an intervention. An average of 74 minutes elapsed from the time of arrival in the emergency department to the time of treatment with pharmacologic agents. Various independent variables were examined to determine their ability to predict the treatment of pain. Chest pain was most often treated with medication, and abdominal pain was least often treated with medication. Despite high pain ratings, only 15% of the sample received an opioid. DISCUSSION This study revealed a very high prevalence of pain among patients in the emergency department and showed that, overall, pain was poorly treated. The findings suggest that chest pain is the only type of pain routinely relieved in the emergency department. An anecdotal finding was that 31 patients said they would refuse pain medications if such medications were offered. Twenty-five patients reported fear of addiction as their reason for this refusal.

Assessment of inter-observer reliability of two five-level triage and acuity scales: a randomized controlled trial

INTRODUCTION The Emergency Severity Index (ESI) is an initial measure of patient assessment in the emergency department (ED). It rates patients based on acuity and predicted resource intensity from Level 1 (most ill) to Level 5 (least resource intensive). Already implemented and evaluated in several US hospitals, ESI has yet to be evaluated in a Canadian setting or compared with the five-level Canadian Emergency Department Triage and Acuity Scale (CTAS). OBJECTIVE To compare the inter-observer reliability of 2 five-level triage and acuity scales. METHODS Ten triage nurses, who had all been trained in the use of CTAS, from 4 urban, academic Canadian EDs were randomly assigned either to training in ESI version 3 (ESI v.3) or to refresher training in CTAS. They independently assigned triage scores to 200 emergency cases, unaware of the rating by the other nurses. RESULTS Number of years of nursing practice was the only significant demographic difference found between the 2 groups (p = 0.014). A quadratically weighted kappa to measure the inter-observer reliability of the CTAS group was 0.91 (0.90, 0.99) and not significantly different from that of the ESI group 0.89 (0.88, 0.99). An inter-test generalizability (G) study performed on the variance components derived from an analysis of variance (ANOVA) revealed G(5) = 0.90 (0.82, 0.99). CONCLUSIONS After 3 hours of training, experienced triage nurses were able to perform triage assessments using ESI v.3 with the same inter-observer reliability as those with experience and refresher training in using the CTAS.

Increased Blood Pressure in the Emergency Department : Pain, Anxiety, or Undiagnosed Hypertension?

STUDY OBJECTIVE We determine the proportion of patients with increased emergency department (ED) blood pressure and no history of hypertension who have persistently increased blood pressure at home, describe characteristics associated with sustained blood pressure increase, and examine the relationship between pain and anxiety and the change in blood pressure after ED discharge. METHODS This was a prospective cohort study. Patients with no history of hypertension and 2 blood pressure measurements of at least 140/90 mm Hg who were treated in an urban ED were enrolled, provided with home blood pressure monitors, and asked to take their blood pressure twice a day for 1 week. Outcome measures were increased mean home blood pressure (140/90 mm Hg or greater), and correlations between ED anxiety (Spielberger State Anxiety Scale) or pain (10-point scale) and the change in blood pressure after discharge. Potential relevant predictors were recorded and a multivariate model was constructed to assess the relationship between these predictors and increased home blood pressure. RESULTS 189 patients were enrolled and 156 returned the monitors and completed the protocol. Increased mean home blood pressure was present in 79 of 156 (51%) patients and was associated with older age and being black. Of patients with ED blood pressures meeting criteria for stage I hypertension, 6% had home blood pressures meeting stage II hypertension, 36% stage I, and 52% prehypertension, and 6% had normal blood pressure For patients with ED blood pressures meeting stage II criteria, the corresponding percentages were 28%, 31%, 33%, and 8%, respectively. The difference between home and ED systolic blood pressures was not associated with anxiety (r=-.03; P=.69) and showed a slight association with pain in the opposite direction from what was expected (r=.18; P=.03). CONCLUSION Patients without diagnosed hypertension and increased ED blood pressures often have persistently increased home blood pressures, which does not appear to be related to pain or anxiety in the ED.

The influence of triage systems and triage scores on timeliness of ED analgesic administration

OBJECTIVES The aim of the study was to examine the association between triage scoring systems and triage priority scores on time to initial emergency department (ED) analgesic administration. METHODS An observational, multicenter, prospective, cohort study was conducted at 20 US and Canadian EDs. Centers from the United States used the Emergency Severity Index triage system or 1 of 3 unvalidated triage systems. Canadian centers used the Canadian Triage and Acuity Scale. Patients aged 8 years or older who presented to the ED with a chief complaint of moderate to severe pain (>3 on a 10-point numerical rating scale) and who were ultimately discharged home were eligible for study enrollment. Triage score, triage system, pain rating on arrival, and time of initial analgesic administration were recorded. RESULTS Among 842 enrolled subjects, 506 (60%) received an analgesic while in the ED. Lower-acuity patients consistently waited longer for analgesics. On multivariate modeling, presenting pain intensity, total time spent in the ED, white ethnicity, and triage system were associated with time to initial analgesic administration. Emergency departments using the Canadian Triage and Acuity Scale triage system exhibited the lowest rates of analgesic use and displayed longer median times to initial analgesic administration. CONCLUSIONS Although there were some differences between triage systems, all sites and systems demonstrated unacceptably long times to analgesic provision. Many patients with moderate to severe pain received no analgesic during their ED stay. Future studies should examine whether ED overcrowding impacts timeliness of analgesic administration and identify specific strategies to improve pain management practices in this challenging environment.

The Impact of Race and Disease on Sickle Cell Patient Wait Times in the Emergency Department

STUDY OBJECTIVE To determine whether patients with sickle cell disease (SCD) experience longer wait times to see a physician after arrival to an emergency department (ED) compared to patients with long bone fracture and patients presenting with all other possible conditions (General Patient Sample), and to attempt to disentangle the effects of race and disease status on any observed differences. METHODS A cross-sectional, comparative analysis of year 2003 through 2008 data from the National Hospital Ambulatory Medical Care Survey, a nationally representative sample of nonfederal emergency department visits in the United States. Our primary outcome was wait time (in minutes) to see a physician after arrival to an ED. A generalized linear model was used to examine ratios of wait times comparing SCD visits to the two comparison groups. RESULTS SCD patients experienced wait times 25% longer than the General Patient Sample, though this difference was explained by the African-American race of the SCD patients. SCD patients waited 50% longer than did patients with long bone fracture even after accounting for race and assigned triage priority. CONCLUSIONS Patients with SCD presenting to an ED for care experience longer wait times than other groups, even after accounting for assigned triage level. The African-American race of the SCD patients, and their status as having SCD itself, both appear to contribute to longer wait times for these patients. These data confirm patient anecdotal reports and are in need of intervention.

A comparison of analgesic management for emergency department patients with sickle cell disease and renal colic.

ObjectivesTo determine whether there is a difference in time to initial analgesic for patients with acute pain from sickle cell disease (SCD) versus renal colic (RC) and to identify factors contributing to variance in time to analgesic. MethodsA retrospective cohort study of the adult emergency department (ED) patients with acute pain from SCD and RC in an urban ED (final ED discharge ICD-9 diagnosis codes were included). A structured medical record review abstracted the demographics, arrival shift, triage level, initial pain score, triage time, and time of initial analgesic dose. Data were compared with Kaplan-Meier plots of time to initial analgesic for both RC and SCD with the log-rank test to test for differences by disease category. A multivariable Cox regression model estimated differences in time to initial analgesic by disease category while controlling for other possible confounders. ResultsMedian time to initial analgesic was 80 minutes for patients with SCD (interquartile range, 48 to 145) versus 50 minutes for patients with RC (interquartile range: 30 to 96). Patients with SCD reported a higher pain score on arrival when compared with RC patients and were more frequently assigned a higher triage priority level (P=0.05). Covariates that contributed the most delays to the model were afternoon arrival [hazard ratio (HR): 0.35, P<0.01], low acuity triage level (HR: 0.42, P<0.01), SCD diagnosis (HR: 0.61, P<0.01), and inability to obtain intravenous access (HR: 0.71, P=0.01). DiscussionED patients with SCD experienced longer delays in the administration of the initial analgesic compared with RC patients, despite higher arrival pain scores and triage acuity levels.

Emergency Provider Analgesic Practices and Attitudes Toward Patients With Sickle Cell Disease

STUDY OBJECTIVE We determine whether emergency provider attitudes and demographics are associated with adherence to national guidelines for the management of acute sickle cell disease pain. METHODS We conducted a cross-sectional survey of emergency providers at the 2011 annual American College of Emergency Physicians Scientific Assembly, using a validated instrument to assess provider attitudes and self-reported analgesic practices toward patients with sickle cell disease. Multivariable, relative risk regressions were used to identify factors associated with adherence to guidelines. RESULTS There were 722 eligible participants, with a 93% complete response rate. Most providers self-reported adherence to the cornerstones of sickle cell disease pain management, including parenteral opioids (90%) and redosing opioids within 30 minutes if analgesia is inadequate (85%). Self-reported adherence was lower for other recommendations, including use of patient-controlled analgesia, acetaminophen, non-steroidal anti-inflammatory drugs and hypotonic fluids for euvolemic patients. Emergency providers in the highest quartile of negative attitudes were 20% less likely to redose opioids within 30 minutes for inadequate analgesia (risk ratio 0.8; 95% confidence interval [CI] 0.7 to 0.9). High-volume providers (those who treat more than 1 sickle cell disease patient per week), were less likely to redose opioids within 30 minutes for inadequate analgesia (risk ratio 0.9; 95% CI 0.8 to 0.9). Pediatric providers were 6.6 times more likely to use patient-controlled analgesia for analgesia (95% CI 2.6 to 16.6). CONCLUSION The majority of emergency providers report that they adhere to national guidelines about use of opioids for sickle cell disease-related acute pain episodes. Other recommendations have less penetration. Negative attitudes toward individuals with sickle cell disease are associated with lower adherence to guidelines.

Adult emergency department patients with sickle cell pain crisis: results from a quality improvement learning collaborative model to improve analgesic management.

OBJECTIVES The aims of this study were to 1) estimate differences in pain management process and patient-reported outcomes, pre- and postimplementation of analgesic protocols for adults with sickle cell disease (SCD), and 2) examine the effects of site and visit frequency on changes in pain scores and time to analgesic. METHODS A multicenter, prospective, longitudinal study enrolled patients from three academic medical centers between October 2007 and September 2009. All ED patients 18 years or older with a chief complaint of a sickle cell pain episode were enrolled. Sites formed a SCD quality improvement (QI) team and implemented standard nurse-initiated emergency department (ED) analgesic protocols; outcomes were compared between study periods defined as pre- and postimplementation of protocols. Medical record review was conducted to measure time to administration of initial analgesic, opioids used, route of opioid administration, the change in pain scores from arrival to discharge (negative numbers reflect a decrease in pain scores), and the number of ED visits per individual patient during the study period at each site. On day 7 after the ED visit, a follow-up phone interview was conducted. Patients were queried about their ED pain management using a scale from 1 to 10 (1 = outstanding, 10 = worst). Descriptive statistics are used to report the results. Ordinary least-squares regression models were constructed to measure the effect of time period, site, and number of visits per patient on change in pain score. RESULTS During the study period, 342 unique patients (57% female, mean ± SD age = 32 ± 11 years) were enrolled and had a total of 2,934 visits. There was no difference in time to administration of the initial analgesic between study periods. Overall, there was a significant decrease in pain scores from arrival to discharge between the pre- and postintervention study periods: the average difference in arrival to discharge pain scores (cm) was greater during the postimplementation period than during the preintervention period (-4.1 vs. -3.6, t = 2.6, p < 0.01). Site 1 had significant improvement between study periods (mean difference = -0.87, t = 2.63, p < 0.01; F = 14.3, p < 0.01). Patients with few ED visits (one to six annual visits, mean difference = -1.55, t = 2.1, p = 0.04) and those with frequent ED visits (7 to 19 annual visits, mean difference = -1.65, t = 3.52, p < 0.01) had a significant decrease in pain scores compared to patients with very frequent ED visits (>19 visits). There was an overall decrease in the use of morphine sulfate (MS) and increase in the use of hydromorphone (χ(2) = 105.67, p < 0.001) between study periods and a significant increase in the use of oral (PO) and subcutaneous (SC) routes, with a corresponding decrease in the intravenous (IV) route (χ(2) = 13.67, p < 0.001). There were no statistically significant differences in patient-reported satisfaction with the attempt to manage pain in the ED between study periods (p = 0.54). CONCLUSIONS While the use of a learning collaborative and implementation of nurse-initiated analgesic protocols was not associated with improvement in time to administration of the initial analgesic, improvements in the decrease in the arrival to discharge pain score and increased use of hydromorphone and the SC route were noted in adults with SCD in the ED.

Adult Emergency Department Patients with Sickle Cell Pain Crisis: A Learning Collaborative Model to Improve Analgesic Management

OBJECTIVES The objectives were to report the baseline (prior to quality improvement interventions) patient and visit characteristics and analgesic management practices for each site participating in an emergency department (ED) sickle cell learning collaborative. METHODS A prospective, multisite longitudinal cohort study in the context of a learning-collaborative model was performed in three midwestern EDs. Each site formed a multidisciplinary team charged with improving analgesic management for patients with sickle cell disease (SCD). Each team developed a nurse-initiated analgesic protocol for SCD patients (implemented after a baseline data collection period of 3.5 months at one site and 10 months at the other two sites). All sites prospectively enrolled adults with an acute pain crisis and SCD. All medical records for patients meeting study criteria were reviewed. Demographic, health services, and analgesic management data were abstracted, including ED visit frequency data, ED disposition, arrival and discharge pain score, and name and route of initial analgesic administered. Ten interviews per quarter per site were conducted with patients within 14 days of their ED discharge, and subjects were queried about the highest level of pain acceptable at discharge. The primary outcome variable was the time to initial analgesic administration. Variable data were described as means and standard deviations (SDs) or medians and interquartile ranges (IQR) for nonnormal data. RESULTS A total of 155 patients met study criteria (median age = 32 years, IQR = 24-40 years) with a total of 701 ED visits. Eighty-six interviews were conducted. Most patients (71.6%) had between one and three visits to the ED during the study period. However, after removing Site 3 from the analysis because of the short data enrollment period (3.5 months), which influenced the mean number of visits for the entire cohort, 52% of patients had between one and three ED visits over 10 months, 21% had four to nine visits, and 27% had between 10 and 67 visits. Fifty-nine percent of patients were discharged home. The median time to initial analgesic for the cohort was 74 minutes (IQR = 48-135 minutes). Differences between choice of analgesic agent and route selected were evident between sites. For the cohort, 680 initial analgesic doses were given (morphine sulfate, 42%; hydromorphone, 46%; meperidine, 4%; morphine sulfate and ibuprofen or ketorolac, 7%) using the following routes: oral (2%), intravenous (67%), subcutaneous (3%), and intramuscular (28%). Patients reported a significantly lower targeted discharge pain score (mean +/- SD = 4.19 +/- 1.18) compared to the actual documented discharge pain score within 45 minutes of discharge (mean +/- SD = 5.77 +/- 2.45; mean difference = 1.58, 95% confidence interval = .723 to 2.44, n = 43). CONCLUSIONS While half of the patients had one to three ED visits during the study period, many patients had more frequent visits. Delays to receiving an initial analgesic were common, and post-ED interviews reveal that sickle cell pain patients are discharged from the ED with higher pain scores than what they perceive as desirable.