Management Of Sickle Cell Pain Research Articles

Evaluating the feasibility of delivering a pain management programme for adults living with sickle cell disease.

Pain is the prominent feature of sickle cell disease (SCD) and negatively affects quality of life. Delivery of pain management programmes (PMPs) has been suggested in clinical guidelines for pain management in SCD; however, further evidence of the feasibility and effectiveness of PMPs in this population is needed. This study explored the feasibility of delivering a sickle cell pain management programme (SCPMP) for adults within a haemoglobinopathies service. A single arm, repeated-measures observational design was used to determine feasibility of delivering the SCPMP at one study site. Primary feasibility outcomes were recruitment, completion of treatment and outcome measures, satisfaction, credibility and acceptability to participants. Secondary feasibility outcomes were treatment outcomes and processes, frequency of vaso-occlusive crisis (VOC) and healthcare utilisation. Four of five feasibility criteria were met. Annual recruitment of eight participants to a SCPMP was not achieved. Twenty-nine people began a SCPMP during the study period. Twenty-five (86.2%) participants attended ≥5/8 sessions and 21(84%) programme completers provided all end of programme questionnaires. Mean scores of >7 on ten-point scales were seen across satisfaction and credibility questions. At least moderate (Hedges g >0.5) effect sizes were seen in pre-post SCPMP measures of pain interference, anxiety, depression, self-efficacy, pain-related worry and acceptance. A small (Hedges g 0.4) effect size was seen in HRQoL. Following SCPMP attendance, mean frequency of self-reported VOC and hospital admissions reduced. This study suggests that, given an adequate source of referrals, a SCPMP is feasible to deliver and appears acceptable and credible to participants. Exploration of influences on recruitment, such as barriers to group interventions, would be illuminating, prior to investigating feasibility of an adequately powered randomised-controlled trial.

A Nurse-Led Education Intervention to Improve Infusion Center Management of Sickle Cell Pain

Problem Statement: Patients with Sickle Cell Disease (SCD) experience acute pain episodes for which they seek care in the Emergency Department (ED). SCD predominantly affects African Americans, and adults with SCD face significant disparities and bias when seeking care in the ED (Haywood, et al., 2013; Mills, et al. 2011). In January 2019, the UCSD Sickle Cell team established a same-day infusion center (IC) management strategy for SCD pain crises as an alternative strategy to seeking care in the ED. This program was well accepted by patients, and as the IC volume of sickle cell patient visits grew (270 visits in the two years pre-IC vs.1076 visits in two years post-IC), a need was identified to assess and improve IC staff knowledge and comfort of management of sickle cell pain. Methods: A 30-minute educational presentation (EP) was created and delivered in a hybrid (in-person/virtual) format to IC staff at three IC sites. A 9-question Google Forms survey was created with input from a validated survey tool and administered. This form comprised of 7 knowledge questions and 2 questions related to comfort levels assessing pain and administering pain interventions in persons with SCD. Responses were collected pre- and post-EP at 1-week (0-7 days). Results: Forty-three pre-surveys were recorded, and twenty-two post-surveys were returned. The overall average score of correct knowledge-based questions (7 total) increased from 2.98 to 5.18. The total responses of "Comfortable" and "Very comfortable" increased for both comfort level assessing pain in persons with SCD (40.4% pre-EP to 86.4% post-EP), and comfort level administering pain interventions in persons with SCD (47.5% pre-EP to 91% post-EP). Conclusion: Surveys confirm that knowledge deficits and comfort level gaps exist among IC staff. A nurse-led education intervention proves effective in increasing IC staff knowledge and comfort levels treating sickle cell pain in the IC setting. Implementing an education intervention to increase IC staff knowledge about SCD creates an opportunity to optimize IC staff competence and comfort in treating patients with SCD. This education is critical for compassionate care of this vulnerable population and transcending racial bias and disparities.

Role of regional anesthesia in patients with acute sickle cell pain: A scoping review.

Sickle cell disease is the most prevalent inherited blood disorder in the world, with significant morbidity and mortality. Patients often have recurrent painful vaso-occlusive episodes, and the American Society of Hematology gives a conditional recommendation for the use of regional anesthesia for acute sickle cell pain management. This scoping review summarizes the current evidence and identifies gaps for future research. Our screening process is outlined, and articles that mentioned the use of regional anesthesia for acute sickle cell crises were included. We present and interpret our results and highlight opportunities for future investigation.

Navigating Ethical Challenges for Pediatric Sickle Cell Pain Management in the Context of the Opioid Epidemic.

The aim was to describe the impact of the opioid epidemic on pain management practices in pediatric sickle cell disease (SCD) and propose a conceptual framework for navigating ethical decision-making in pediatric sickle cell pain management. A review of the literature on ethical challenges in the management of sickle cell pain was conducted and considered in the context of the opioid epidemic and psychosocial factors affecting youth with SCD. The Integrated Ethical Framework for Pain Management (IEFPM) was applied to pediatric sickle cell pain management using a clinical case example. Implicit bias, health-related stigma, and potential neurocognitive impairment all present unique challenges in ethical decision-making for youth with SCD. National guidelines for prescribing opioid medication may complicate providers' clinical decision-making and affect their sickle cell pain management practices. The IEFPM was found to be applicable to ethical decision-making for pediatric sickle cell pain and captures both patient-related and provider-related aspects of clinical pain management. The opioid epidemic has exacerbated existing ethical challenges for pain management among youth with SCD. The IEFPM provides a conceptual model that can be integrated into health care settings to facilitate ethical decision-making and promote greater health equity in the clinical management of pediatric sickle cell pain.

Compliance with Expert Recommendations of Sickle Cell Pain Management—a Single Institution Review

Introduction:Sickle cell disease in a lifelong disorder associates with the occurrence of recurrent painful vasoocclusive crisis that often result in frequent emergency room trips and inpatient hospital stays. There are several goals in the management of sickle cell disease including early resolution of painful crisis and reduction of end stage events such as pulmonary hypertension and stroke. Unfortunately, the painful crisis leads to frequent ER visits and sometimes results in lengthy hospital stays. In 2014, The US Department of Health and Human Services came out with an expert report panel dictating recommendations on care for patient with sickle cell disease recommending pain medicine in the emergency room within one hour of triage and use of personal controlled analgesia (PCA) pumps. We opted to look at patient records within Orlando Health, a center with a large sickle cell patient population, to determine if we are following these recommendations.Methods: We reviewed emergency room and inpatient records of 158 patients. We documented if pain medications were started within two hours of arrival to the emergency room (we allowed additional time to account for registration and triage) and we reviewed inpatient records for PCA use. We also looked for trends in PCA use in terms of complexity of hospital stays and length of stay.Results:For sickle cell patients coming into the emergency room, 107/158 (68%) were given pain medication within 2 hours of registration.Of patients admitted to the hospital, only 10/158 (6%) were given a PCA pump. The patients given a PCA pump had an average length of stay of 16 days compared to the general population of 6.1 days. PCA use increased during lengthy hospitalizations rather than being started immediately.The patients who were given a PCA pump were assigned an ER acuity score of 3 compared to the general population of 2.1.Discussion:In caring for patients with sickle cell disease, we often aim to provide quick and effective management of pain crisis. In terms of emergency room management, triage to pain medication time is ideally within one hour. By looking at an extended window to account for registration time, we were able to provide pain medication on time 68% of the time. Per pharmacy staff, the pain medications are kept in the emergency room pharmacy system and should be immediately available upon request. Emergency room physicians identify overcrowding of the ER and the prospect of patient addiction as potential barriers to more immediate access. This thought process can lead to unnecessary suffering from patients.Inpatient PCA use was very infrequently started. Currently the hospitalist service is starting most of the inpatient pain medication. When asked about PCA use, the hospitalist physicians mostly stated that their experience hasn't shown a trend toward shorter length of stay. Additionally, patients frequently state that they prefer push medication to PCA use. Data does exist that the opposite is true, PCA use is associated with shorter length of stay. Education on this data and encouragement of PCA use with patients should be encouraged per expert recommendations. It does appear that PCA use is reserved for patients who are deemed “sicker” or have longer length of stay.We believe these trends are reflective of other institutions and broad education on best management of sickle cell pain crisis may be required. DisclosuresLandau:celgene: Speakers Bureau; sanofi: Speakers Bureau; alexion: Speakers Bureau; novartis: Speakers Bureau; BMS: Speakers Bureau.

Acupuncture for Sickle Cell Pain Management in Pediatrics: A Promising Non-Opioid Therapy Emerges

Acupuncture for Sickle Cell Pain Management in Pediatrics: A Promising Non-Opioid Therapy Emerges

Acupuncture for Sickle Cell Pain Management in Pediatrics: A Promising Non-Opioid Therapy Emerges

Acupuncture for Sickle Cell Pain Management in Pediatrics: A Promising Non-Opioid Therapy Emerges

Acupuncture for Sickle Cell Pain Management in Pediatrics: A Promising Non-Opioid Therapy Emerges

Purpose In response to our nation’s epidemic drug overdose deaths, the Joint Commission has mandated that non-pharmacologic therapies, for example acupuncture, be offered to patients with pain. Acupuncture has been used for thousands of years in China for pain, and has gained global utilization. Acupuncture is recognized by the National Institute of Health as safe and effective for pain. It is widely administered in the Veterans Administration for pain and has positively contributed towards the decrease in opioid dosing. …

Emergency Department Provider Survey Regarding Acute Sickle Cell Pain Management.

Sickle cell disease (SCD) can lead to potentially devastating complications that occur secondary to vaso-occlusion. Current national clinical guidelines are largely based on expert opinion, resulting in significant variation of management. Provider awareness regarding emergency department (ED) management of vaso-occlusive crises (VOC) remains unknown. A 23-question assessment of VOC management was administered to all eligible ED providers at Riley Hospital for Children between September and November 2018. Univariate analyses were performed to evaluate responses between groups. Of 52 respondents comprised of ED staff attendings (27%), resident trainees (58%), and ED nurses (15%), the majority were not aware of SCD management guidelines being available. Approximately 54% of providers endorsed a high comfort level in managing VOC, with staff and nurses more likely to report this than trainees (P=0.02). Less than 10% of all providers knew the recommended timeframe from triage to initial medication administration. Prolonged time between pain assessments was reported by 25% of providers with a high comfort level in managing VOC, which was similar to providers with a lower comfort level (13%, P=0.217). Only one fourth of all respondents appropriately did not use vital signs as an indication of a patient's pain level, and >10% reported not utilizing patient-reported pain scores. This was not significantly different between provider comfort levels (P=0.285 and 0.412, relatively). Our results suggest education regarding recommended practices was inadequate regardless of reported provider comfort. Further provider education and/or standardized ED VOC management guidelines may serve as areas for improvement in SCD care.

Acupuncture for pediatric sickle cell pain management: A promising non-opioid therapy

ObjectiveTo describe the use of acupuncture for pain management in children with sickle cell disease. DesignA retrospective chart review of a single-institution experience on the use of acupuncture in pediatric patients with sickle cell disease was evaluated between 2012–2019. Demographic characteristics, presenting pain location, pain scores pre- and post-acupuncture, and adverse events were collected. SettingColumbia University Medical Center, NewYork-Presbyterian Morgan Stanley Children’s Hospital Pediatric Hematology outpatient and inpatient units. InterventionsAcupuncture was performed by six licensed acupuncturists. Point prescriptions were based on pain location, philosophies of Traditional Chinese Medicine and Japanese Style of Kiiko Matsumoto acupuncture. Main outcome measuresPain reduction as measured by two Verbal Pain Scales. ResultsNinety acupuncture treatments were administered to twenty-four patients with sickle cell disease: median age 17.5 years, 62 % female, 37.5 % African American, 50 % Hispanic. The mean treatment duration was 18.5 ± 4.8 min. Fifty-five treatments had documented pre/post-acupuncture pain scores. Pain reduction was achieved in 65.5 % of these treatments. A 0–10 pain scale used in 13 treatments reported a mean pre-acupuncture score of 7.31 ± 1.75, post-acupuncture score of 6.08 ± 1.85, and a mean pain score change of 1.23 ± 1.09 (p = 0.11); A 0–4 pain scale used in 42 treatments reported a mean pre-acupuncture pain score of 3.31 ± 0.72, post-acupuncture score of 2.33 ± 0.98, and a mean pain score change of 0.98 ± 0.99 (p < 0.0001). No adverse events were noted. ConclusionAcupuncture therapy decreased pain for our patients with sickle cell disease, providing a safe non-opioid therapeutic option.

Management of Sickle Cell Disease Pain among Adolescent and Pediatric Patients

Management of sickle cell pain in adolescent and pediatric patients is inadequate, and the employment of proper management guidelines and practices are highly variable among different regions and populations. APPT, the multidimensional adolescent pediatric pain tool, promotes optimal pain management and introduces best practical guidelines for pain management. The goal of this study is to assess pain and pain management among young patients diagnosed with sickle cell disease (SCD) by introducing the APPT as a tool for pain management, and analyze factors contributing to pain management. Information relevant to demographic data, SCD characteristics, APPT assessment, and satisfaction of patients regarding pain management were collected using a structured questionnaire. Results showed that SCD is highly associated with gender (p = 0.022), consanguinity (p = 0.012), and number of surgeries (p = 0.013). Most patients (58.9%) indicated the involvement of more than six body areas affected during pain crisis. Severe pain was described by more than half the patients (55.6%), while moderate pain was reported by 31.1%. Most patients described their pain by sensory, affective, and temporal words. The number of painful areas, pain intensity, and use of descriptive pain words was correlated and interpreted by age, BMI, school absence, and number of surgeries. Results of this study could provide guidance to healthcare providers to improve current practices for SCD pain management in order to improve health outcomes and patients’ satisfaction.

Integrating palliative care and emergency medicine for optimal management of sickle cell pain in the wake of the United States opioid epidemic.

Having lost a sibling to sickle cell disease (SCD) related complications, and having heard multiple accounts from a friend with sickle cell about how much she suffers, and how her pain is poorly managed, there is certainly a need to share insights on this topic.

Pre-hospital management of pain associated with an acute sickle cell crisis

Sickle Cell Disease and its acute manifestation as a sickle cell crisis has a severely deleterious effect on predicted mortality and quality of life of its sufferers. Serious sequelae can occur, resulting in severe neurological and cardiovascular impairment. In the majority of cases these patients will present to pre-hospital clinicians with acute intractable pain unmanaged by the patient's own analgesia. Unfortunately, some evidence reports that pain is commonly under-treated with assumptions made about drug-seeking behaviour. The evidence presented in this paper will indicate that Entonox should no longer be routinely used for the management of sickle cell pain. Instead, emphasis should turn to the use of potent intravenous opiates (where intravenous access can be reliably and aseptically gained). The use of diamorphine and ketamine for extreme pain should be investigated by pre-hospital services, as should the use of non-steroidal anti-inflammatory drugs.

Healthcare injustice in patients with sickle cell disease

Purpose – The purpose of this paper is to detail experiences that sickle cell disease (SCD) patients associate with healthcare justice and injustice in pain control. Design/methodology/approach – A content analysis study of open-ended comments written by 31 participants who completed a 20-item healthcare injustice questionnaire-revised twice: once in reference to experiences with doctors and once in reference to experiences with nurses. Findings – Participants’ mean age was 33±10 years; most were African-Americans and women. Themes showed: the four domains of healthcare justice were represented in patients’ comments; examples of justice and injustice were provided; specific incidents and interactions with healthcare providers were memorable to patients; and setting was a factor important to healthcare experiences because expectations about services vary by setting. Research limitations/implications – Patients were self-selected. Future work will include qualitative interviews and focus groups to uncover more details about how patients experience healthcare injustice. Practical implications – Additional training is needed for SCD providers and about proper management of sickle cell pain; educational modules are also needed that address areas of healthcare injustice by patients. Originality/value – The authors are the first to report how patients define healthcare justice and injustice. Specific details about memorable SCD patient-provider interactions and pain control are described.

The Opioid Drug Epidemic and Sickle Cell Disease: Guilt by Association.

In 2008, for the first time in Centers for Disease Control and Prevention (CDC) history, death due to motor vehicle accident was no longer the leading cause of injury mortality non-medical death [1]. Drug overdose was the number one cause of non-medical death in the United States [1]. Since 2008, the number of deaths due to drug overdose has been increasing steadily [2–9]. The total number of deaths due to drug overdose was 36,450 in 2008 [10]. In 2013, the total amount of deaths due to drug overdose was 43,982 [10]. Given the alarming nature of these statistics, patients who requested an opioid prescription became targets of suspicion and possible accusation of being drug addicts and/or drug seekers and possibly potential victims of overdose [8,9,11–15]. The advent of precision medicine [16], however, seems to justify such behavior. Since the pharmacodynamics and pharmacokinetics of an opioid vary among patients considerably depending on their individual specific genetic and epigenetic factors, it is conceivable that a certain opioid or a certain dose of an opioid do not apply to all patients all the time. Patients with sickle cell disease (SCD) whose pain was treated with opioids chronically learned which opioid and which dose of an opioid is best to relieve or minimize their sickle pain. Nevertheless, management of sickle cell pain with opioids must follow two tiers as is the case in the management of other types of pain with opioids. The first tier is to optimize analgesia by prescribing adequate dose to minimize pain severity, and the second tier is to minimize risk by frequent monitoring and assessment of opioid related adverse effects and outcomes related to substance use disorders [17]. Although …

Acupuncture for Sickle Cell Pain Management in a Pediatric Emergency Department, Hematology Clinic, and Inpatient Unit

Abstract Background: Acupuncture has been shown to mitigate certain painful conditions in children. However, utilization of acupuncture for addressing acute and chronic pain in children with sickle cell disease has not been well-studied. The aim of this article is to report the use of acupuncture to treat acute and chronic sickle cell pain in one patient in a pediatric emergency department (PED), hematology clinic, and inpatient unit of an inner-city children's hospital. Case: A 16-year-old Hispanic male with sickle cell disease was treated with acupuncture for 3 years. A retrospective chart review was performed to collect details on the acupuncture treatments, their effects on this patient's pain and anxiety, feedback from the patient and his family, and frequency of PED visits and admissions for pain. Results: Fifty-eight acupuncture treatments were administered for this patient's sickle cell pain during the period that was reviewed. His pain was reduced by an average of 2.3 points and 2.2 points for in...

Improving Emergency Providers’ Attitudes Toward Sickle Cell Patients in Pain

BackgroundProvider biases and negative attitudes are recognized barriers to optimal pain management in sickle cell disease, particularly in the emergency department (ED). MeasuresThis prospective cohort measures preintervention and postintervention providers' attitudes toward patients with sickle pain crises using a validated survey instrument. InterventionED providers viewed an eight-minute online video that illustrated challenges in sickle cell pain management, perspectives of patients and providers, as well as misconceptions and stereotypes of which to be wary. OutcomesNinety-six ED providers were enrolled. Negative attitude scoring decreased, with a mean difference −11.5 from baseline, and positive attitudes improved, with a mean difference +10. Endorsement of red-flag behaviors similarly decreased (mean difference −12.8). Results were statistically significant and sustained on repeat testing three months after intervention. Conclusions/Lessons LearnedBrief video-based educational interventions can improve emergency providers' attitudes toward patients with sickle pain crises, potentially curtailing pain crises early, improving health outcomes and patient satisfaction scores.

Pathophysiology and principles of management of the many faces of the acute vaso-occlusive crisis in patients with sickle cell disease.

Effective management of sickle cell pain entails a thorough understanding of its pathophysiology and the pharmacogenomics of the opioids used to manage it. In recent years, there has been significant progress along these two lines. At the pathophysiologic level, there is evidence that the severity and frequency of painful stimuli modulate their transmission at the level of the dorsal horn of the spinal cord. This modulation is achieved via two channels: the α-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) and NMDA receptors. Initially, the AMPA channel controls the transmission of stimuli of mild-moderate severity. Once the AMPA channel reaches its limit of membrane depolarization, the NMDA channel is activated and facilitates the transmission of painful stimuli in a progressive fashion leading to central sensitization and glial activation. At the level of pharmacogenomics, the metabolism of each opioid is patient-specific. Glucuronidation is unique for the metabolism of morphine, hydromorphone, and oxymorphone. The metabolism of all other opioids requires specific Cytochrome P450 (CYP) isoenzymes. The activity of each isoenzyme and the activity of the metabolites of each opioid vary among patients depending on their genetic makeup and coexistent environmental factors such as the use of other medications that may enhance or inhibit the CYP isoenzyme activity.

(254) A fast-track emergency department acute sickle cell pain management algorithm results in fewer hospital admissions, decreased length of stay and increased hospital revenue

(254) A fast-track emergency department acute sickle cell pain management algorithm results in fewer hospital admissions, decreased length of stay and increased hospital revenue

Timely Analgesia, Reduced Hospitalization Rate, and Improved Economic Efficiency Following Implementation of a New Emergency Department Sickle Cell Pain Management Algorithm

Context: Adults with sickle cell disease (SCD) presenting to emergency departments (EDs) with severe pain often experience treatment delays and long, costly hospitalizations. Objectives: A fast-track ED SCD pain management algorithm was developed and then evaluated for its clinical and economic impact. Methods: We conducted a retrospective chart review to compare outcomes of ED visits for SCD-related pain two years before and after algorithm implementation. Patient demographics, ED utilization, and treatment outcome measures including time from registration to first opioid treatment, ED length of stay, hospitalization rate, hospitalization length of stay, and both hospital and ED revenue production were compared. Results: There was a total of 699 consecutive ED visits for SCD-related pain (131 pre- and 568 postalgorithm). Median time to first opioid dose decreased from 53 to 32 minutes (p <0.001). Disposition was determined more efficiently (210 vs. 168 minutes, p <0.001) leading to reduction in ED length of stay (345 vs. 271 minutes, p<0.001). Although ED utilization increased, this was due to a few high utilizers whose patterns of use skewed the mean. Hospitalization rate per ED visit significantly decreased (54% vs. 38%, p=0.001). Average length of stay per hospitalization decreased from 12 to 8 days (p<0.03) which was economically beneficial to the hospital in that revenue production per inpatient day increased by 28%. Conclusions: Our findings suggest algorithmic ED SCD pain management significantly improves quality and economic outcomes.