scholarly journals Implementation of an Intravenous Lidocaine Guideline for Children and Adolescents with Sickle Cell Vaso-Occlusive Pain

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 2962-2962
Author(s):  
Ugochukwu Agbakwuru ◽  
Jacob D AuBuchon ◽  
Bobi Toebe ◽  
Anne LaBarge ◽  
Jorge Di Paola ◽  
...  
Keyword(s):  
Pain Management ◽  
Side Effects ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Research Funding ◽  
Management Service ◽  
Intravenous Lidocaine ◽  
Cell Disease ◽  
Care Guideline ◽  
Patient Reported

Abstract Introduction Sickle cell disease (SCD) affects approximately 100,000 Americans.[1] Vaso-occlusive episodes (VOEs) are the leading cause of hospitalization in SCD; in 2016, out of 134,000 SCD hospitalizations, 81% included VOE. [2] Opioids are the mainstay of VOE treatment, but side effects include sedation, hyperalgesia, and dependency risk. Lidocaine, which inhibits voltage-gated sodium channels in peripheral sensory nerves, is given intravenously (IV) for postoperative pain management and holds promise for VOE treatment.[3] At St. Louis Children's Hospital (SLCH), children admitted with VOEs receive an opioid by continuous infusion plus patient-controlled analgesia (PCA) and non-opioid adjuvants. In 2019, the Sickle Cell Disease Program and Pain Management Service jointly developed a guideline for VOE management that includes: 1. Standardized dosing of IV opioids and IV or oral NSAIDs 2. Pain management service consultation for all children age ≥10 y admitted with VOE 3. Initiation of IV lidocaine infusion (1-1.5 mg/kg/h x 48 h) within 24 h of admission for severe pain 4. Addition of non opioid adjuvants, such as muscle relaxants, topical agents, and agents for neuropathic pain 5. IV lidocaine is prescribed by the Pain Management Service. Patients are monitored for lidocaine side effects including tinnitus, perioral tingling, vital sign changes, and seizures. A 24 h serum lidocaine level is drawn to ensure levels are not supra-therapeutic. We aimed to evaluate the implementation of this care guideline, focused on IV lidocaine as a safe, tolerable and effective adjunct to opioids for VOE treatment in children. Methods This retrospective cohort study reviewed records of children with SCD age ≥10 years admitted for VOE at SLCH during 2018-2020. Data collected included patient demographics, impact on pain, lidocaine levels, and reported side effects. This study was approved by the institutional review board. Results We identified 61 patients (31 males), median age 15.7 y (range 9-21), with 174 IV lidocaine administrations during 164 hospitalizations. Hemoglobin (Hb) SS comprised 60.7% of the cohort; 32.8% had Hb SC disease, 4.9% had Hb Sβ0 thalassemia and 1.6% had Hb Sβ+ thalassemia. IV lidocaine was started within 24 h for 78.7% (129/164) of included admissions. The mean blood lidocaine level was 2.14 mcg/ml (SD 1.23). Of the 164 admissions, 9 individuals had lidocaine levels above the limit of 4.5 mcg/ml, none had symptoms of toxicity. Few side effects were noted: lip paresthesia in 1 child (lidocaine stopped, but received in future admissions); nausea in 1 child (declined lidocaine in future admissions); pain increase in 1 teen (lidocaine was stopped). One child stopped lidocaine infusions prematurely during 2 admissions due to refusal of phlebotomy for the lidocaine level. Overall, 59 children perceived benefit of IV lidocaine and chose to receive it again during later admissions. During 10 prolonged hospitalizations, a second 48-hour lidocaine infusion was given due to patient reported benefit. We identified falsely elevated lidocaine levels when subcutaneous lidocaine was used before phlebotomy, but no patients with high lidocaine levels experienced toxicity. We altered our response to supratherapeutic levels by pausing the lidocaine infusion, redrawing lab at a peripheral site without subcutaneous lidocaine contamination, and resuming lidocaine infusion if level was normal. Every repeated lidocaine level was within normal limits. Discussion In our cohort, IV lidocaine was safe, tolerable, and improved pain control. The care guideline was initiated within 24 hours for 78.7% of admissions. Mild side effects occurred in only 3 patients, highlighting safety. Future considerations include a prospective study focused on length of stay, patient-reported outcomes, opioid exposure, and factors influencing the care guideline's utilization. References: 1. American Society of Hematology. Sickle Cell Disease. www.hematology.org/Patients/Anemia/Sickle-Cell.aspx 2.Fingar K, Owens P, et al (2019). Characteristics of Inpatient Hospital Stays Involving Sickle Cell Disease, 2000-2016. Agency for Healthcare Research and Quality. https://hcup-us.ahrq.gov/reports/statbriefs/sb251-Sickle-Cell-Disease-Stays-2016.jsp 3.Dunn L, Durieux ME, (2017). Perioperative use of intravenous lidocaine. Anesthesiology, 126(4), 729-737. https://doi.org/10.1097/aln.0000000000001527 Disclosures Di Paola: CSL Behring: Consultancy, Honoraria. Hulbert: Pfizer: Current Employment, Current holder of individual stocks in a privately-held company; bluebird bio: Consultancy; Global Blood Therapeutics: Research Funding; Forma Therapeutics: Consultancy, Research Funding.

scholarly journals Prevalence of High BMI Status in Adults with Sickle Cell Disease

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 2039-2039
Author(s):  
Stephanie O Ibemere ◽  
Laura Niederer ◽  
Charity I Oyedeji ◽  
Ebony Burns ◽  
John Myers ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Medical Records ◽  
Research Funding ◽  
Normal Weight ◽  
Cell Disease ◽  
Epidemiological Analysis ◽  
Patient Reported ◽  
Pain Frequency ◽  
High Bmi

Abstract Introduction Emerging literature suggests body mass index (BMI) may be increasing in individuals with sickle cell disease (SCD), a condition historically associated with underweight status. However, the current prevalence of overweight and obese adults with SCD remains unclear in a country where high BMI is prevalent in the general population. We present an epidemiological analysis of the prevalence of overweight and obese individuals in a large representative sample of adult SCD patients, identifying associations between BMI, sociodemographic and clinical characteristics. Methods Using the Sickle Cell Disease Consortium (SCDIC) registry, we compiled a detailed clinical and demographical from a non-random sample of adults, aged 20-45 years. The SCDIC collects data from eight academic centers providing comprehensive care throughout the U.S. Adult participants were excluded from the analysis if they were under 20 years of age, pregnant at the time of enrollment, or were without medical records or patient enrollment surveys. Sociodemographic information and patient-reported outcomes, including pain frequency and severity, SCD complications, and hydroxyurea use, were collected at the time of enrollment. Non-SCD medical conditions and anthropometric measurements were abstracted from medical records. We stratified BMI, measured in kilograms per meters squared (kg/m2) following CDC criteria: underweight (<18.5 kg/m2), normal weight (18.5-24.9 kg/m2), overweight (25.0-29.9 kg/m2), and obese (>30 kg/m2). An epidemiological analysis was performed of BMI in those with SCD. Bivariate analyses were conducted using Chi-square test, t-test, and ANOVA; non-parametric tests were used when appropriate. Data were analyzed using SAS 9.4 (SAS Institute; Cary, NC). Results In total, 1,664 adults met the inclusion criteria for this cross-sectional analysis of SCDIC registry data. The median BMI for the entire sample was 23.9 kg/m² (IQR: 21.1-28). The majority of participants were African American (99.1%), female (56.6%), and had an HbSS genotype (69.6%). The prevalence of an obese BMI (17.5%) was greater than underweight BMI (6.4%) among the entire cohort (Table 1). When compared to under/normal weight participants, those who were overweight/obese were older (median age 31.2 versus 29.3 years; p<0.0001) and had a higher prevalence of hypertension (45.1% versus 28.3%; p<0.0001). Most participants in the overweight/obese BMI categories had genotype HbSS (59.0%), however, genotype HbSS accounted for 77% of the under/normal weight category. Most participants with an overweight/obese BMI also had some college or vocational training (39.9%) and had Medicare, Medicaid, or military insurance (71.9%). Median BMI did not differ on reported use or non-use of hydroxyurea (23.9 [IQR: 21.1-27.6] vs 23.9 [IQR: 21.0-28.4] p=0.1), mean number of patient-reported SCD complications (2.6 versus 2.6; p=0.6), nor mean pain frequency (49.6 versus 50.6; p=0.06). However, overweight/obese participants reported significantly higher mean pain intensity than their under/normal weight counterparts (51.8 versus 50.8; p=0.03). Conclusion To date, this is the largest analysis of adult BMI among individuals with SCD in the U.S. Among the eight SCDIC sites spanning from the Northeast to Southwest U.S., the prevalence of underweight BMI was less than that of overweight or obese BMI status, challenging previous understandings of weight status and further aligning with the growing rates of overweight and obesity in the general U.S. population. Significant associations between high BMI and hypertension, age, and pain intensity highlight an opportunity for further research to understand the impact of increasing BMI on SCD outcomes and non-SCD comorbidities. Figure 1 Figure 1. Disclosures Ibemere: bluebird bio Insights Council: Consultancy, Honoraria; Ugali Youth: Consultancy, Membership on an entity's Board of Directors or advisory committees. King: Health Resources and Services Administration: Research Funding; National Cancer Institute: Research Funding; Global Blood Therapeutics: Research Funding; National Heart, Lung, and Blood Institute: Research Funding. Hankins: Global Blood Therapeutics: Consultancy; UpToDate: Consultancy; Bluebird Bio: Consultancy; Vindico Medical Education: Consultancy. Tanabe: CSL Behring: Consultancy. Shah: Emmaus: Consultancy; GLG: Consultancy; Alexion: Speakers Bureau; Guidepoint Global: Consultancy; Bluebird Bio: Consultancy; CSL Behring: Consultancy; Novartis: Research Funding, Speakers Bureau; GBT: Consultancy, Research Funding, Speakers Bureau.

scholarly journals Implementation of a Standard Order Set at a Sickle Cell Acute Care Observation Unit

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3396-3396
Author(s):  
Renee Cheng ◽  
Rishi Patel ◽  
Sandra Kang ◽  
Frances Tian ◽  
Xu Zhang ◽  
...  
Keyword(s):  
Pain Management ◽  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Acute Care ◽  
Sickle Cell ◽  
Research Funding ◽  
Mixed Effects ◽  
Data Sets ◽  
Cell Disease ◽  
Data Set

I NTRODUCTION: Acute painful vaso-occlusive crises (VOC) in sickle cell disease (SCD) are the leading cause of emergency department (ED) encounters and frequent hospital admissions. For patients presenting with an uncomplicated VOC, acute care observation units (ACOU) have previously been shown to reduce admission rates and length of stay. We wished to evaluate if implementing a standardized acute care order set (ACOS) at the University of Illinois Hospital Sickle Cell ACOU would decrease the time to first dose of analgesic medication, inpatient hospital stays, and subsequent admissions to the ACOU. METHODS: The ACOS includes standard orders for laboratory tests to monitor severity of sickle cell hemolysis, intravenous fluids, and analgesic medications including opioids. We conducted a retrospective analysis to evaluate if the ACOS enhanced workflow and improved the timeliness of treatment in patients experiencing a VOC. The ACOS was created in April 2017 and we compiled data from the three months before (January-March 2017) and after (May-July 2017) ACOS creation. We collected data on the time it took to administer the first opioid dose, admission rate, length of stay, number of acute care visits, ED visits, and inpatient hospitalizations in a three-month span, and demographics including variations in age, gender, and sickle cell disease genotype. Patient data was collected from a pharmacy-generated list of patients who received narcotics in the ACOU during the aforementioned time period. We analyzed the effect of the ACOS on the aforementioned variables. A mixed effects linear model was used to compare time to first dose of opioids and length of stay between data sets. A mixed effects logistic model was used for binary outcomes. Covariates of age (years), gender, and severity of sickle cell hemoglobin genotype (severe: HbSS, HbS beta0 thalassemia; mild: HbSC, HbS beta+ thalassemia) were included in the models. Statistical analyses were carried out in R version 3.4.3. DISCUSSION: The pre-ACOS data set contains 291 patient encounters for 76 patients with a median age of 37 years (interquartile range [IQR], 30-47 years), 66% female, and 71% with severe genotypes. The post-ACOS data set contained 289 patient encounters for 80 patients with a median age of 32 years (IQR, 27-45 years), 80% female, and 73% with severe genotypes. Implementation of an ACOS was associated with decreased time to pain management by 3.7 minutes (p=0.077) in patients presenting with uncomplicated VOC and with fewer repeat visits to the ACOU in the studied 3-month period [OR 0.35 (95% CI 0.13-1.00), p=0.049]. The median number of opiate doses received by patients in both data sets was 3. Using 3 as a cutoff, the implementation of the ACOS was also associated with more patients receiving >3 doses of opiates [OR=1.84 (95% CI 1.05-3.19), p = 0.033]. We demonstrate that in SCD patients experiencing VOC, a standardized ACOS was associated with a trend to reducing time to receiving pain management, with increased total opioid doses during the ACOU admission (suggesting better pain control), and subsequently with a statistically significant reduction in the number of repeat ACOU visits in the studied 3-month period. We have shown that a standardized ACOS that streamlines workflow in an ACOU may play an important role in delivering timely and quality care to patients with SCD. Disclosures Gordeuk: Pfizer: Research Funding; Novartis: Consultancy, Honoraria, Research Funding; Global Blood Therapeutics: Consultancy, Honoraria, Research Funding; Modus Therapeutics: Consultancy, Honoraria; Ironwood: Research Funding; CSL Behring: Consultancy, Honoraria, Research Funding; Imara: Research Funding; Inctye: Research Funding; Inctye: Research Funding; Pfizer: Research Funding; Emmaus: Consultancy, Honoraria; CSL Behring: Consultancy, Honoraria, Research Funding; Ironwood: Research Funding; Novartis: Consultancy, Honoraria, Research Funding; Imara: Research Funding; Modus Therapeutics: Consultancy, Honoraria.

Effects Of GMI 1070, a Pan-Selectin Inhibitor, On Pain Intensity and Opioid Utilization In Sickle Cell Disease

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 775-775 ◽  
Author(s):  
Laura M. De Castro ◽  
Ted Wun ◽  
Sophie Lanzkron ◽  
Wally R. Smith ◽  
Kathryn L. Hassell ◽  
...  
Keyword(s):  
Placebo Group ◽  
Pain Management ◽  
Sickle Cell Disease ◽  
Pain Intensity ◽  
Sickle Cell ◽  
Research Funding ◽  
Study Drug ◽  
Cell Disease ◽  
Opioid Use ◽  
Vas Scores

Abstract Background Management of acute, painful vaso-occlusive crisis (VOC), the hallmark of sickle cell disease (SCD), continues to be limited to symptomatic relief with oral and/or intravenous (IV) opioids. The efficacy of GMI 1070, a novel pan-selectin inhibitor, was recently evaluated in SCD patients hospitalized for VOC. We report in-depth analysis of pain intensity variation, duration and extent of pain management and other pain-related responses in this study. Methods A multi-center, randomized, double-blind, placebo-controlled Phase 2 trial of multiple IV doses of GMI 1070 in subjects (12-60 yrs) hospitalized for VOC was conducted. A loading dose to achieve steady state drug level was followed by q12 hours (h) maintenance doses. Study drug dose was doubled per protocol after interim PK analysis. Pain intensity was assessed by a 10 cm Visual Analog Scale (VAS); sustained reduction of 1.5 cm or higher and transition to oral analgesics were included in the composite primary endpoint “time to resolution of VOC,” as were readiness for discharge and time to discharge. Opioid utilization was recorded, converted to Morphine Equivalent Units (MEU) based on standard guidelines, and normalized by body weight. Patient controlled analgesia (PCA), oral analgesic and other therapies were not mandated and reflected institutional and individual practices. Comparisons were made between pooled GMI 1070 (low and high dose) and placebo. Analysis of covariance (ANCOVA), adjusting for sex and age, was used to determine differences between groups. Kaplan-Meier (KM) analysis (log rank test) was performed to compare time to event between treatment groups. Results Mean (SD; range) VAS at presentation to ED for all subjects was 8.6 (1.6; 5-10) and decreased before study drug start (baseline) to 6.7 (2.3; 0.8-10), reflecting initial pain management. Mean VAS scores at presentation and at baseline were not different between GMI 1070 and placebo groups. However, VAS changes from baseline by elapsed hours since drug start and probability of sustained 1.5 cm reduction in VAS over time showed that patients receiving GMI 1070 had earlier changes than those in the placebo group, starting at 48 h, (p=0.43, Fig 1a). Similarly, pain intensity reduction was greater by ∼1.5 cm and more rapid within the first 100 h of study treatment for those on GMI 1070 (p=0.58, Fig 1b). In subjects still hospitalized 48 h after study drug initiation, the GMI 1070 group with concomitant hydroxyurea (HU) had the lowest VAS scores. Mean (SD; range) VAS at discharge was 4.03 (2.97; 0-10) in the placebo group and 3.02 (2.75; 0-9.8) in the GMI 1070 group. ANCOVA analysis showed that time to resolution of VOC and time to discharge were shorter in the GMI 1070 group compared to placebo, independent of HU use. Not only was mean [SE] time to transition from parenteral to oral pain medications reduced in the GMI 1070 group vs placebo (108.6 [20.8] vs 155.6 [23.8] h; p=0.14), but active drug exposure was associated with a significant reduction in initial (24 h) and cumulative (during hospitalization) opioid use (oral, IV or both routes) as compared to placebo (Table). Opioid use decreased compared to baseline in the GMI 1070 group within 4 h, while the placebo group did not go below baseline rate until 96h. Duration of IV or oral non-steroidal anti-inflammatory drug (NSAID) use was also lower in the GMI 1070 group vs placebo. Conclusion GMI 1070 therapy was associated with a significant reduction in IV opioid requirement and overall pain medication utilization in subjects hospitalized for SCD VOC. There was also an early decrease in pain intensity as measured by VAS, although this did not reach statistical significance. These effects were independent of HU use and suggest that GMI 1070 has a rapid onset of action and should be investigated for initial and early treatment of VOC in SCD, with the goal of reducing pain intensity and duration, as well as need for opioid therapy. Disclosures: De Castro: Novella Clinical: Consultancy; GlycoMimetics, Inc.: Research Funding. Wun:Emmaus, Inc.: Clinical Adjudication Committee Other; Pfizer, Inc.: Consultancy; GlycoMimetics: Research Funding. Lanzkron:GlycoMimetics, Inc.: Research Funding. Smith:GlycoMimetics, Inc.: Research Funding. Hassell:Glycomimetics, Inc: Research Funding. Kutlar:GlycoMimetics, Inc: Research Funding. Smith-Whitley:GlycoMimetics, Inc: Research Funding. Rhee:GlycoMimetics, Inc.: Research Funding; Rho, Inc.: Employment. Telen:GlycoMimetics, Inc.: Research Funding; Dilaforette, NA: Research Funding; Pfizer, Inc.: Consultancy. Thackray:GlycoMimetics, Inc.: Employment, Equity Ownership.

Microsurgery in the Sickle Cell Trait Population: Is It Actually Safe?

2020 ◽  
pp. 1-2
Author(s):  
Michael Alperovich ◽  
Eric Park ◽  
Michael Alperovich ◽  
Omar Allam ◽  
Paul Abraham
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Free Flap ◽  
Near Infrared ◽  
Sickle Cell Trait ◽  
Cell Disease ◽  
Flap Transfer ◽  
Free Flap Transfer ◽  
Patient Reported ◽  
Deep Inferior Epigastric Perforator

Although sickle cell disease has long been viewed as a contraindication to free flap transfer, little data exist evaluating complications of microsurgical procedures in the sickle cell trait patient. Reported is the case of a 55-year-old woman with sickle cell trait who underwent a deep inferior epigastric perforator (DIEP) microvascular free flap following mastectomy. The flap developed signs of venous congestion on postoperative day two but was found to have patent arterial and venous anastomoses upon exploration in the operating room. On near-infrared indocyanine green angiography, poor vascular flow was noted despite patent anastomoses and strong cutaneous arterial Doppler signals. Intrinsic microvascular compromise or sickling remains a risk in the sickle cell trait population as it does for the sickle cell disease population. Just like in sickle cell disease patients, special care should be taken to optimize anticoagulation and minimize ischemia-induced sickling for patients with sickle cell trait undergoing microsurgery.

Parent Perspectives on Pain Management, Coping, and Family Functioning in Pediatric Sickle Cell Disease

2007 ◽  
Vol 46 (4) ◽  
pp. 311-319 ◽  
Author(s):  
Monica J. Mitchell ◽  
Kathleen Lemanek ◽  
Tonya M. Palermo ◽  
Lori E. Crosby ◽  
Alisha Nichols ◽  
...  
Keyword(s):  
Pain Management ◽  
Sickle Cell Disease ◽  
Family Functioning ◽  
Sickle Cell ◽  
Parent Perspectives ◽  
Cell Disease ◽  
Pediatric Sickle Cell Disease

scholarly journals Impact of voxelotor (GBT440) on unconjugated bilirubin and jaundice in sickle cell disease

2018 ◽  
Vol 10 (2) ◽  
Author(s):  
Paul Telfer ◽  
Irene Agodoa ◽  
Kathleen M. Fox ◽  
Laurie Burke ◽  
Timothy Mant ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Oxygen Affinity ◽  
Well Being ◽  
Unconjugated Bilirubin ◽  
Cell Disease ◽  
Case Patient ◽  
Disease Manifestation ◽  
Patient Reported ◽  
Hemoglobin Oxygen Affinity

For many patients with sickle cell disease (SCD), jaundice is a significant clinical disease manifestation that impacts on patient well-being. We report a case of a patient with SCD and chronic jaundice treated with voxelotor (GBT440), a novel small molecule hemoglobin oxygen affinity modulator and potential disease-modifying therapy for SCD. The case patient is a 27- year-old Black male with a long history of SCD with clinical jaundice and scleral icterus. After starting voxelotor, the patient reported that his jaundice cleared within one week, and that he felt much better with more energy, and was relieved after his eyes cleared. Voxelotor reduced bilirubin and unconjugated bilirubin (by up to 76%), and hemoglobin improved from 9.9 g/dL at baseline to 11.1 g/dL at 90 days. Jaundice impacts many adults with SCD, significantly impacting self-image. Voxelotor treatment reduced bilirubin levels and improved jaundice, resulting in an improved sense of well-being in our case patient.

scholarly journals Ticagrelor does not impact patient‐reported pain in young adults with sickle cell disease: a multicentre, randomised phase II b study

2018 ◽  
Vol 184 (2) ◽  
pp. 269-278 ◽  
Author(s):  
Julie Kanter ◽  
Miguel R. Abboud ◽  
Banu Kaya ◽  
Videlis Nduba ◽  
Carl Amilon ◽  
...  
Keyword(s):  
Young Adults ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Phase Ii ◽  
Cell Disease ◽  
Patient Reported
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