scholarly journals Clinical Guideline Highlights for the Hospitalist: Management of Acute and Chronic Pain in Sickle Cell Disease

2021 ◽  
Author(s):  
Charles D Pham ◽  
Duong T Hua
Keyword(s):  
Chronic Pain ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pediatric Patients ◽  
Clinical Guideline ◽  
Target Population ◽  
Cell Disease ◽  
Release Date ◽  
History Of ◽  
American Society

GUIDELINE TITLE: American Society of Hematology 2020 Guidelines for Sickle Cell Disease: Management of Acute and Chronic Pain RELEASE DATE: June 19, 2020 PRIOR VERSION: Not applicable DEVELOPER: American Society of Hematology Guideline Panel on Sickle Cell Disease-Related Pain FUNDING SOURCE: American Society of Hematology TARGET POPULATION: Adult and pediatric patients with a history of sickle cell disease with acute and chronic pain.

scholarly journals American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain

2020 ◽  
Vol 4 (12) ◽  
pp. 2656-2701 ◽  
Author(s):  
Amanda M. Brandow ◽  
C. Patrick Carroll ◽  
Susan Creary ◽  
Ronisha Edwards-Elliott ◽  
Jeffrey Glassberg ◽  
...  
Keyword(s):  
Chronic Pain ◽  
Pain Management ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Practice Research ◽  
Management Approach ◽  
Evidence Based ◽  
Cell Disease ◽  
Targeted Interventions ◽  
American Society

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.

NT-Pro Brain Natriuretic Peptide Levels and the Risk of Stroke and Death in the Cooperative Study of Sickle Cell Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1541-1541 ◽  
Author(s):  
Roberto F Machado ◽  
Mariana Hildescheim ◽  
Laurel Mendelsohn ◽  
Gregory J Kato ◽  
Mark T Gladwin
Keyword(s):  
High Risk ◽  
Sickle Cell Disease ◽  
Incidence Rate ◽  
Sickle Cell ◽  
Pediatric Patients ◽  
Cooperative Study ◽  
Cell Disease ◽  
Risk Of Death ◽  
History Of

Abstract Abstract 1541 Poster Board I-564 Background Elevations in NT-proBNP levels are associated with hemolysis-associated pulmonary hypertension and mortality in adults with sickle cell disease. The association of this vasculopathy with the risk of stroke has not been explored. The Cooperative Study of Sickle Cell Disease (CSSCD) is the largest cohort of adult and pediatric patients with sickle cell disease with the longest median follow-up. Using stored plasma samples from patients enrolled in the CSSCD, we tested the hypothesis that adult patients with high levels of NT-proBNP would be at a high risk of death and stroke and that pediatric patients with a high BNP might be at a high risk of stroke. Methods A threshold NT-pro-BNP value previously identified to predict mortality in adults with sickle cell disease was used to determine the association between the risk of stroke and mortality in a cohort of 758 participants (pediatric cohort, n=428 and adult cohort, n=330) in the CSSCD. Results The prevalence of an abnormal NT-proBNP level ≥ 160 pg/ml was 27.6 % in patients in the adult CSSCD cohort. The prevalence of a NT-proBNP level ≥ 160 pg/ml was 27.4 % in the pediatric cohort, which is at least in part explained by known higher normal NT-proBNP levels in children, especially during the first year of life. The incidence of the development of a high NT-proBNP level in subjects with a normal baseline level was 6 % over a mean follow-up time of 5.9 years (incidence rate per 100-person years of 1.03) in the pediatric cohort and 16.5 % over a mean follow-up time of 1.9 years in the adult cohort (incidence rate per 100-person years of 8.59). In subjects with normal baseline levels, multivariate logistic regression analysis of clinical and laboratory factors associated with the development of a high NT-proBNP level included increasing age (OR 3.43, 95% CI 1.6-7.2, P = 0.001), low hemoglobin (OR 0.47, 95% CI 0.3-0.7, P < 0.001), high white blood cell count (OR 1.69, 95% CI 1.05-2.7, P = 0.03), high creatinine (OR 2.22, 95% CI 1.1-4.3, P = 0.02), blood urea nitrogen (OR 1.64, 95% CI 1.2-2.3, P = 0.004), alanine aminotransferase (OR 1.26, 95% CI 1.01-1.6, P = 0.04) and uric acid levels (OR 2.32, 95% CI 1.4-3.8, P = 0.001), and history of leg ulcers (OR 7.46, 95% CI 2.0-28.5, P = 0.003). Elevated NT pro-BNP levels were associated with indices of hemolytic anemia (hemoglobin: OR 0.33, 95% CI 0.2-0.4, P < 0.001) and a history of stroke (OR 1.86, 95% CI 0.9-3.7, P = 0.02). An NT-pro-BNP level ≥160 pg/ml was a predictor of mortality (RR 6.24, 95% CI 2.9-13.3, P < 0.001) and stroke (RR 3.84, 95 % CI 1.0-14.3, P = 0.05) in this cohort. Conclusions A high NT-proBNP level is a major risk factor for death in patients with sickle cell disease. These findings provide further support for a mechanistic link between hemolytic anemia and the development of cardiovascular complications in this patient population. Finally, we have identified a novel widely available biomarker of the risk of death and stroke in sickle cell disease. Disclosures No relevant conflicts of interest to declare.

Non-Cardiopulmonary Factors Affecting the Six-Minute Walk Distance in Patients with Sickle Cell Disease: Results From the Walk-PHaSST Study

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 1074-1074 ◽  
Author(s):  
Ruchika Goel ◽  
Kathryn L. Hassell ◽  
Roberto F Machado ◽  
Robyn J. Barst ◽  
Nancy Yovetich ◽  
...  
Keyword(s):  
Chronic Pain ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
P Value ◽  
Cell Disease ◽  
Walk Distance ◽  
Factors Affecting ◽  
History Of ◽  
Minute Walk Distance ◽  
Minute Walk

Abstract Abstract 1074 Non-Cardiopulmonary Factors Affecting the Six-Minute Walk Distance in Patients with Sickle Cell Disease: Results from the Walk-PHaSST Study. INTRODUCTION: The six-minute walk (6MW) test is frequently used to assess exercise capacity. Patients with sickle cell disease (SCD) can have decreased 6MW distance (6MWD) compared to controls. The 6MWD in conjunction with the TR-jet velocity (TRV) and NT-proBNP have recently been proposed to have a greater predictive value for screening SCD patients suspected of having pulmonary hypertension (PH) than TRV alone. (Parent et al, NEJM, 365; 1, 2011 365 (1):44–53). The American Thoracic Society guidelines recommend caution in controlling for sources of variability in the 6MWD (Am J Respir Crit Care Med 166. 111–117, 2002). Age and height are known confounders of the 6MWD. However, non-cardiopulmonary factors including skeletal-mechanics and pain may also impact the 6MWD. AIM: This study explores whether non-cardiopulmonary factors affect the 6MWD in SCD patients. METHODS: We analyzed data from subjects screened for the walk-PHaSST trial. Walk-PHaSST was a multi-center, placebo-controlled, double-blind, 16-week trial evaluating the safety and efficacy of oral sildenafil for the treatment of Doppler-defined PH (TRV '2.7m/s) in subjects with SCD aged >12 years. The primary endpoint in the trial was change in 6MWD. During screening, subjects were evaluated by self-reported medical history, physical examination, blood sampling, echocardiography and 6MWD. Univariate and multivariable linear regression was performed. A two sided p value <0.05 was considered significant. RESULTS: Of the 673 subjects screened, 671 had a 6MW test. The median (inter-quartile range) of 6MWD was 438m (503 – 381m = 122 m). On univariate analysis, there was no statistically significant effect of the SCD genotype on the 6MWD (p=0.26). Further, when combining the severe genotypes (HbSS and HbSβ0 thalassemia) vs the less severe genotypes, there was no significant difference in the 6MWD (p=0.22). By multivariable linear regression (Table 1), after adjusting for age, gender and TRV, the presence of the following (self-reported by subjects) were independently associated with an estimated decrease in the 6MWD: a) chronic pain (n = 260/671, 38.9%) by 24.3m (95% CI: 9.1–39.4m, p-value <0.01), b) history of avascular necrosis (AVN) of the hip (N =127/671, 18.9%) by 27.8m (95% CI: 9.2–46.4m, p-value <0.01), and c) osteopenia (N = 46/671,6.9% ) by 31.2m (95% CI: 2.7.-59.6m, p-value <0.05) There were no significant two-way interactions between chronic pain, AVN of the hip and/or osteopenia. History of leg ulcers, osteomyelitis and rheumatoid arthritis were not significant predictors of the 6MWD. DISCUSSION: In this multi-center study of patients with SCD, history of self-reported: 1) chronic pain 2) AVN of the hip and 3) osteopenia were independently associated with decreased functional capacity (as assessed by the 6MW test), after adjusting for age, gender, and TRV. While the 6MWD in patients with SCD is significantly related to cardiac function (i.e., PH and LV diastolic dysfunction), the potential effects of pain, osteonecrosis and osteopenia on 6MWD suggest that if using the 6MW test as the primary endpoint in future trials, one should consider documentation of the presence of these factors and use a stratified randomization based on a composite of these non-cardiopulmonary variables. Disclosures: Hassell: NIH:. Gladwin:Patents filed related to treating hemolysis.: Patents & Royalties.

Management of severe chronic pain with methadone in pediatric patients with sickle cell disease

2018 ◽  
Vol 65 (8) ◽  
pp. e27084 ◽  
Author(s):  
Zachary LeBlanc ◽  
Chris Vance ◽  
Jason Payne ◽  
Jie Zhang ◽  
Lee Hilliard ◽  
...  
Keyword(s):  
Chronic Pain ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pediatric Patients ◽  
Cell Disease ◽  
Severe Chronic Pain

scholarly journals Investigation of Chest X-Ray Use in the Emergency Department in Pediatric Patients with Sickle Cell Disease Presenting with Fever Compared to Age Matched Controls

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 26-26
Author(s):  
Urania Dagalakis ◽  
Henna Butt ◽  
Natalie Davis ◽  
Regina A. Macatangay
Keyword(s):  
Emergency Department ◽  
Chest Pain ◽  
Sickle Cell Disease ◽  
Radiation Exposure ◽  
Sickle Cell ◽  
Pediatric Patients ◽  
Asthma Diagnosis ◽  
Inclusion Criteria ◽  
Cell Disease ◽  
History Of

Background In sickle cell disease (SCD), acute chest syndrome (ACS) is associated with prolonged hospitalization, increased risk of respiratory failure, future lung disease and 25% mortality in hospitalized patients(Bakshi, & Krishnamurti, 2017; Vinchinsky et al. 1997). Pediatric patients with SCD frequently present to the Pediatric Emergency Department (PED) with complaints of fever, chest pain, and cough, all of which may or may not be related to ACS. It is challenging for PED providers to determine which patients are at highest risk of ACS, so chest X-Rays (CXR) are frequently ordered which increases radiation exposure and healthcare costs. The objective of this study was to identify incidence of CXR performance, as well as ACS diagnosis, in SCD patients presenting to our PED with or without fever. Our goal was to identify significant clinical predictors of ACS in this population in order to implement a diagnostic algorithm for PED providers. Methods This was an IRB-approved retrospective medical record review of subjects diagnosed with SCD with inclusion criteria: ages 2-12 years, who presented to the University of Maryland PED between 2016-2018. We performed bivariate analyses comparing these variables between subjects who were febrile vs. afebrile on presentation to the PED, as well as those who were ultimately diagnosed with ACS compared to those who were not. Analysis of categorical variables was performed using Chi-square or Fischer exact test as appropriate. We performed a multivariable logistic regression model to identify significant predictors of ACS diagnosis. Analyses performed using SAS 9.4. Results We identified 424 SCD subjects who presented to our PED meeting inclusion criteria, with 25% (n=108) presenting with fever. Of these, 69% received a CXR on presentation vs. 42% of afebrile subjects (p=&lt;0.0001). In our febrile group 21% (n=23) patients had more than 2 febrile episodes and 100% received CXRs. There were no significant differences between the febrile and afebrile subjects when it came to sex, asthma diagnosis/comorbidity, hydroxyurea use, folic acid supplementation, or pneumococcal prophylaxis. Overall, 10% of patients presenting to the PED were diagnosed with ACS (n=42), made up of 13% of those presenting with fever vs. 9% of those presenting without fever. Those subjects ultimately diagnosed with ACS were significantly more likely to present with chest pain (p=0.003), tachypnea (p=0.001), and hypoxia (p&lt;0.0001), and were more likely to have a past history of asthma (p=0.0085). Sickle cell variant, home medications, and history of splenectomy were not significantly associated with ACS diagnosis. Upon multivariable modeling, when adjusting for fever and pre-existing asthma diagnosis, the only significant predictors of ACS diagnosis were chest pain and hypoxia. Patients without chest pain had an odds ratio (OR) =0.3 of ACS diagnosis [95% Confidence Interval, CI 0.14-0.67], indicating they had 70% lower odds of ACS compared to patients with chest paint. Patients without hypoxia had OR=0.12 of ACS compared to those with hypoxia [CI 0.06-0.25], indicating an 88% reduced odds of ACS diagnosis. Conversely, those with chest pain had 3.3x the odds of ACS diagnosis [CI 1.5-7.4] and those with hypoxia had 8.4x the odds of ACS diagnosis [CI 4-17.9] compared to those without these symptoms. Conclusion In ACS, current guidelines recommend that patients presenting with fever, hypoxia, tachypnea, tachycardia and abnormal respiratory exam findings should be treated empirically as well as receive a CXR. However radiological signs can be delayed compared to physical signs so a normal CXR does not preclude the diagnosis of ACS if there is clinical suspicion(Howard et al. 2015). Our data demonstrate that clinical findings such as chest pain, tachypnea and hypoxia were most likely to correlate to a diagnosis of ACS. While 69% of our febrile patients received a CXR in the PED, only 13% were ultimately diagnosed with ACS, indicating that more CXRs and radiation exposure occurred in the febrile population than may have been necessary. When adjusting for fever and asthma, the most notable predictors of ACS were hypoxia and chest pain. When present, these findings are significant predictors of ACS; when absent, subjects had significantly decreased odds of ACS. Incorporating the presence or absence of chest pain and hypoxia may help focus the use of CXR on the appropriate patient population. Disclosures No relevant conflicts of interest to declare.

scholarly journals To Give or Not to Give: RhD Immunoglobulin for an RHD*39 Pregnant Woman with Sickle Cell Disease

2021 ◽  
pp. 1-5
Author(s):  
Justin E. Juskewitch ◽  
Craig D. Tauscher ◽  
Sheila K. Moldenhauer ◽  
Jennifer E. Schieber ◽  
Eapen K. Jacob ◽  
...  
Keyword(s):  
Pregnant Woman ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pregnancy Termination ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Childbearing Age ◽  
Procedural Complications ◽  
History Of ◽  
Chronic Hemolysis

Introduction: Patients with sickle cell disease (SCD) have repeated episodes of red blood cell (RBC) sickling and microvascular occlusion that manifest as pain crises, acute chest syndrome, and chronic hemolysis. These clinical sequelae usually increase during pregnancy. Given the racial distribution of SCD, patients with SCD are also more likely to have rarer RBC antigen genotypes than RBC donor populations. We present the management and clinical outcome of a 21-year-old pregnant woman with SCD and an RHD*39 (RhD[S103P], G-negative) variant. Case Presentation: Ms. S is B positive with a reported history of anti-D, anti-C, and anti-E alloantibodies (anti-G testing unknown). Genetic testing revealed both an RHD*39 and homozygous partial RHCE*ceVS.02 genotype. Absorption/elution testing confirmed the presence of anti-G, anti-C, and anti-E alloantibodies but could not definitively determine the presence/absence of an anti-D alloantibody. Ms. S desired to undergo elective pregnancy termination and the need for postprocedural RhD immunoglobulin (RhIG) was posed. Given that only the G antigen site is changed in an RHD*39 genotype and the potential risk of RhIG triggering a hyperhemolytic episode in an SCD patient, RhIG was not administered. There were no procedural complications. Follow-up testing at 10 weeks showed no increase in RBC alloantibody strength. Discussion/Conclusion: Ms. S represents a rare RHD*39 and partial RHCE*ceVS.02 genotype which did not further alloimmunize in the absence of RhIG administration. Her case also highlights the importance of routine anti-G alloantibody testing in women of childbearing age with apparent anti-D and anti-C alloantibodies.

scholarly journals Concurrent Bilateral Central Retinal Artery Occlusion Secondary to Sickle Cell Crisis

2021 ◽  
Vol 9 ◽  
pp. 232470962110283
Author(s):  
Gowri Renganathan ◽  
Piruthiviraj Natarajan ◽  
Lela Ruck ◽  
Roberto Prieto ◽  
Bharat Ved Prakash ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Vision Loss ◽  
Central Retinal Artery Occlusion ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Cell Disease ◽  
Sickle Cell Crisis ◽  
History Of ◽  
Retinal Artery

Vascular occlusive crisis with a concurrent vision loss on both eyes is one of the most devastating disability for sickle cell disease patients. Reportedly occlusive crisis in the eyes is usually temporary whereas if not appropriately managed can result in permanent vision loss. A carefully managed sickle cell crisis could prevent multiple disabilities including blindness and stroke. We report a case of a 24-year-old female with a history of sickle cell disease who had acute bilateral vision loss during a sickle crisis and recovered significantly with a timely emergent erythrocytapheresis.

scholarly journals History of Pain is Associated with Hospitalization and Severe Course of COVID-19 in Children with Sickle Cell Disease

2021 ◽  
Vol 22 (5) ◽  
pp. 607-608
Author(s):  
Lana Mucalo ◽  
Amanda Brandow ◽  
Mahua Dasgupta ◽  
Sadie Mason ◽  
Pippa Simpson ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
History Of ◽  
History Of Pain
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