scholarly journals Optimizing the care model for an uncomplicated acute pain episode in sickle cell disease

Hematology ◽  
2017 ◽  
Vol 2017 (1) ◽  
pp. 525-533 ◽  
Author(s):  
Paul Telfer ◽  
Banu Kaya

Abstract The pathophysiology, clinical presentation, and natural history of acute pain in sickle cell disease are unique and require a disease-centered approach that also applies general principles of acute and chronic pain management. The majority of acute pain episodes are managed at home without the need to access health care. The long-term consequences of poorly treated acute pain include chronic pain, adverse effects of chronic opioid usage, psychological maladjustment, poor quality of life, and excessive health care utilization. There is no standard protocol for management of an acute pain crisis in either the hospital or the community. The assumptions that severe acute pain must be managed in the hospital with parenteral opioids and that strong opioids are needed for home management of pain need to be questioned. Pain management in the emergency department often does not meet acceptable standards, while chronic use of strong opioids is likely to result in opioid-induced hyperalgesia, exacerbation of chronic pain symptoms, and opioid dependency. We suggest that an integrated approach is needed to control the underlying condition, modify psychological responses, optimize social support, and ensure that health care services provide safe, effective, and prompt treatment of acute pain and appropriate management of chronic pain. This integrated approach should begin at an early age and continue through the adolescent, transition, and adult phases of the care model.

2020 ◽  
Vol 4 (12) ◽  
pp. 2656-2701 ◽  
Author(s):  
Amanda M. Brandow ◽  
C. Patrick Carroll ◽  
Susan Creary ◽  
Ronisha Edwards-Elliott ◽  
Jeffrey Glassberg ◽  
...  

Background: The management of acute and chronic pain for individuals living with sickle cell disease (SCD) is a clinical challenge. This reflects the paucity of clinical SCD pain research and limited understanding of the complex biological differences between acute and chronic pain. These issues collectively create barriers to effective, targeted interventions. Optimal pain management requires interdisciplinary care. Objective: These evidence-based guidelines developed by the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care professionals in pain management decisions for children and adults with SCD. Methods: ASH formed a multidisciplinary panel, including 2 patient representatives, that was thoroughly vetted to minimize bias from conflicts of interest. The Mayo Evidence-Based Practice Research Program supported the guideline development process, including updating or performing systematic reviews. Clinical questions and outcomes were prioritized according to importance for clinicians and patients. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations, which were subject to public comment. Results: The panel reached consensus on 18 recommendations specific to acute and chronic pain. The recommendations reflect a broad pain management approach, encompassing pharmacological and nonpharmacological interventions and analgesic delivery. Conclusions: Because of low-certainty evidence and closely balanced benefits and harms, most recommendations are conditional. Patient preferences should drive clinical decisions. Policymaking, including that by payers, will require substantial debate and input from stakeholders. Randomized controlled trials and comparative-effectiveness studies are needed for chronic opioid therapy, nonopioid therapies, and nonpharmacological interventions.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3743-3743 ◽  
Author(s):  
Samir K. Ballas ◽  
Carlton Dampier

The transition of medical care of patients with sickle cell disease (SCD) from pediatric to adult providers represents a milestone in their lives. Major concerns among adolescents and young adults about transition include taking responsibility for self, making own decisions, cost of medical care, fear of suboptimal pain management, and reluctance to leave known providers. In this study we present our experience in the process of transition to adult care and its outcome over the last ten years. Adolescents and young adults were given information about the nature of medical care provided by adult internists and hematologists. The sickle cell programs available in the city were described. Moreover, site visits to the hospitals where adult care was to be provided were arranged. During these visits, adolescents and young adults had the chance to meet the hematologist and other potential providers and ask questions, visit the emergency room, the clinic, and the sickle day unit if applicable. Patients were empowered to choose the program to which they wished to be transitioned. During the last 10 years, 90 adolescents and young adults (See Table) with SCD (Sickle Cell Anemia [SS], Hemoglobin SC Disease, and Sickle Thalassemia [ST]) were transitioned to the adult sickle cell program of Thomas Jefferson University. Age of transition varied between 18 and 25 years. Eighteen patients (20%) died. Age at death was 24.9 ± 2.95 years and the male/female ratio was 10:8. Complications of sickle cell disease after transition included leg ulcers, stroke, avascular necrosis, anxiety, depression, and priapism. Nineteen patients (10 males, 9 females) were employed. Twenty-nine (32%) patients developed chronic pain syndrome and its sequelae. Many patients failed to achieve their childhood goals. The data show that a significant number of patients die within 10 years after transition. The quality of life of survivors is suboptimal and drifts into issues of chronic pain management in the adult environment. Identifying these issues may provide predictors that identify children at risk to have undesirable outcomes after transition. Aggressive management and refining the process of transition should improve the outcome after transition. Distribution of the Transitioned Patients SS SC ST Total Male 31 8 4 43 Female 34 8 5 47 Total 65 16 9 90


Hematology ◽  
2017 ◽  
Vol 2017 (1) ◽  
pp. 406-411 ◽  
Author(s):  
Joshua J. Field

AbstractChronic pain affects one-half of adults with sickle cell disease (SCD). Despite the prevalence of chronic pain, few studies have been performed to determine the best practices for this patient population. Although the pathophysiology of chronic pain in SCD may be different from other chronic pain syndromes, many of the guidelines outlined in the pain literature and elsewhere are applicable; some were consensus-adopted in the 2014 National Heart, Lung, and Blood Institute SCD Guidelines. Recommended practices, such as controlled substance agreements and monitoring of urine, may seem unnecessary or counterproductive to hematologists. After all, SCD is a severe pain disorder with a clear indication for opioids, and mistrust is already a major issue. The problem, however, is not with a particular disease but with the medicines, leading many US states to pass broad legislation in attempts to curb opioid misuse. These regulations and other key tenets of chronic pain management are not meant to deprive adults with SCD of appropriate therapies, and their implementation into hematology clinics should not affect patient-provider relationships. They simply encourage prudent prescribing practices and discourage misuse, and should be seen as an opportunity to more effectively manage our patient’s pain in the safest manner possible. In line with guideline recommendations as well as newer legislation, we present five lessons learned. These lessons form the basis for our model to manage chronic pain in adults with SCD.


2016 ◽  
Vol 13 (4) ◽  
pp. 409-416 ◽  
Author(s):  
Kerri A Nottage ◽  
Jane S Hankins ◽  
Lane G Faughnan ◽  
Dustin M James ◽  
Julie Richardson ◽  
...  

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4700-4700
Author(s):  
Brian Pennarola ◽  
Patrick Demartino ◽  
Dale W. Steele ◽  
Susan J. Duffy

Abstract Introduction: Qualitative research identifies delayed analgesia and under-dosing as common reasons for patient/caregiver dissatisfaction with the treatment of acute vaso-occlusive pain in sickle cell disease (SCD). Experts have identified quality measures for emergency department (ED) management of acute pain in SCD including early administration of IV analgesics (NHLBI 2014). Adolescent and young adult (AYA) patients are especially at risk for dissatisfaction, and qualitative studies identify the transition from pediatric (PED) to adult emergency department (AED) care as the most difficult site of transition. Information from empirical studies of SCD pain management in PEDs and AEDs is needed to facilitate overall improvements in care and facilitate transitions of care for AYA patients. The goal of this study was to examine differences in management of SCD pain between a PED and an AED. The primary outcome was time from triage to first opioid. Methods: We retrospectively reviewed patients with SCD and acute pain, age 3 - 27 years, seeking care from June 2015 to December 2016 in the AED and/or PED within our academic, tertiary care institution. Four visits per individual per ED were abstracted. Visits were excluded if no opioid was administered, for confounding sources of pain (e.g. post-operative), transfer from another ED or critical illness. Important encounter characteristics and outcomes were summarized by mean with standard deviation (SD) or median with interquartile range (IQR) for skewed data. We compared the difference between EDs for the primary outcome using a Cox proportional hazards model with a patient-level random effect. Results: Our initial electronic health record query yielded 353 visits by 66 patients to the PED and AED. Two patients accounted for 46% of visits (77 and 84 visits). After excluding visits by patients transferred from an outside facility (n=1), with confounding illness (n=6) or no opioid administered (n=3), we extracted data on up to 4 visits per ED per patient and analyzed 127 visits by 55 patients. Demographics, initial pain score, treatments and treatment timeline are summarized by ED (Table 1). The Kaplan-Meier plot (Figure 1) shows the proportion of patients receiving the first intravenous opioid dose, by ED, as a function of time from triage. At any given time, the probability of receiving the first opioid dose in the PED was approximately 3 times greater than in the AED (hazard ratio of 2.95 (95% CI 1.93, 4.50), p < 0.001). Patients in the AED were more likely to receive hydromorphone than morphine and adjunctive NSAIDs were rarely given. More than an hour elapsed between the 1st and 2nd opioid doses in both EDs. Intravenous hydration with normal saline boluses was common in both EDs. An individualized prescribing and monitoring protocol, written by the patient's SCD provider (pain plan) was rarely available to ED providers. Conclusions: Optimal management of acute pain in patients with SCD is difficult, a challenge exacerbated by practice and cultural differences in pediatric versus adult settings. Considering the primary outcome, the PED administered IV analgesia more quickly than the AED, although neither site provided treatment consistently within 30 minutes from triage as per the NHLBI guidelines. Much of the delay in time to first opioid in the AED (Figure 1) is explained by longer rooming times. Our data are limited in that we cannot differentiate whether the delay is due to longer overall wait times or lower prioritization of patients. Nonetheless, this difference remains a known driver of patient dissatisfaction. Our results highlight multiple opportunities in both EDs to improve care including earlier administration and individualized dosing of opioids, reducing the time interval between subsequent doses, routine administration of NSAIDs, avoidance of fluid boluses in euvolemic patients and development of patient specific pain plans. Emergency providers could benefit from education and localized practice guidelines with written protocols and electronic alerts, targeted at quality improvement. The AED could also potentially benefit from focused effort to reduce time from triage to room. Subspecialty providers could assist the ED by routinely providing pain plans; this could help achieve more uniform care across individual encounters in both EDs, particularly in the few patients accounting for a large proportion of all encounters. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4849-4849
Author(s):  
Samuel Wilson ◽  
Frances Wright ◽  
Marcus A. Carden

Background: Sickle cell disease (SCD) is the most common inherited blood disorder in the United States (US), affecting approximately 100,000 individuals in the country who are primarily of African descent. One of the most prevalent complications of SCD is pain as a result of episodic vaso-occlusive crises. Over time, many individuals with SCD develop chronic pain and opioid dependence for pain management. L-glutamine (EndariTM) was approved by the US Food and Drug Administration in 2017 for patients 5 years-old and older to reduce complications from SCD after reviewing a phase-III placebo-controlled trial. In this study, L-glutamine led to a reduction in median number of pain crises and increased time to first pain crisis when compared to placebo (Niihara et al, NEJM, 2018). However, the impact of L-glutamine on opioid use over time remains unknown. In this study, we evaluated the effect on opioid use in individuals who were started on L-glutamine for worsening SCD related pain. Methods: After institutional review board approval, we retrospectively reviewed the electronic medical record (EMR) of individuals with SCD followed at the University of North Carolina Pediatric and Adult Sickle Cell clinics prescribed L-glutamine in 2018-2019 for worsening acute and chronic SCD-related pain. The North Carolina state controlled substance reporting system, an online clinical tool which collects information on dispensed controlled substance prescriptions to patients that is freely available to prescribers, was also reviewed for filled opioid prescriptions (and milligram morphine equivalents - MME) for each patient. Data, including health care utilization (e.g. hospitalizations and emergence room (ER) visits) and hemoglobin levels for each patient were also evaluated in the EMR for the four months preceding and the four months after L-glutamine was started to determine if changes were sustained. Results: We identified four female patients (ages ranging from 9 to 24 years-old) with SS genotype and chronic pain with acute exacerbations who had significant opioid prescription reduction after starting L-glutamine. Three individuals were taking the maximum tolerated dosing of hydroxyurea and experiencing escalating pain crises prior to initiation of L-glutamine. One patient was intolerant of hydroxyurea and was on a chronic transfusion program for chronic pain management when she was started on L-glutamine for worsening chronic pain. All patients, or caregivers, reported a reduction in acute on chronic pain after initiating L-glutamine. Each patient had a reduction in 4-month total opioid prescription use (in MME) after starting L-glutamine, ranging from a 21% reduction to 100% reduction (Figure 1). Heath care utilization significantly decreased in 1 patient after starting L-glutamine, with 3 ER visits and 2 hospitalizations in the pre-treatment period and no ER visits or hospitalizations in the post-treatment period. There was no difference in the average hemoglobin levels pre-and-post L-glutamine initiation among the patients (9.8g/dL vs. 9.7g/dL). Discussion: L-glutamine appears to have some benefit in reducing pain and opioid use, as well as healthcare utilization, in a subset of patients with SCD and chronic pain. Although we evaluated a small number of patients, all individuals (or caregivers) reported decreased pain very soon after starting L-glutamine. One patient stopped opioid use altogether in the time period evaluated. Future studies should investigate if effectiveness of L-glutamine may be based on unique red cell metabolic profiles, SCD genotype, or timing of drug initiation in these and similar patients. Future investigations will also determine long-term tolerability of L-glutamine and if the reduction in opioid use is sustained for longer periods among these patients and other responders. Disclosures Carden: GBT: Honoraria; NIH: Research Funding.


2009 ◽  
pp. 550-563
Author(s):  
Jaya L. Varadarajan ◽  
Steven J. Weisman ◽  
Henry McQuay

2020 ◽  
Vol 4 (Supplement_1) ◽  
pp. 785-785
Author(s):  
Keesha Roach ◽  
Brenda Dyal ◽  
Srikar Chamala ◽  
Yingwei Yao ◽  
Roger Fillingim ◽  
...  

Abstract Purpose: Emotional stress is a known pain trigger in patients with sickle cell disease (SCD). The Arginine vasopressin receptor 1A gene (AVPR1A), SNP rs10877969, is associated with acute pain and stress-related pain. Our study investigated the association between AVPR1A genotype with stress and age in adults with SCD pain. Methods: 169 participants with SCD and chronic pain (100% African descent; mean age 36.4 ± 11.6 years [range =18-74 years]) completed the Perceived Stress Questionnaire. The SNP was evaluated as the imputed score was R2&gt;0.8. ANOVA compared stress by genotype and age. Findings: Mean stress scores were significantly lower (p&lt;0.05) for the older adults (0.35 ± 0.18) than the younger adults (0.41 ± 0.17). Mean stress scores were not significantly different by genotype for younger or older adults. Discussion: The rs10877969 genotype frequency was not different by age. In contrast to prior research, there was no association between genotype and stress.


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