Fentanyl Buccal Tablet: A New Breakthrough Pain Medication in Early Management of Severe Vaso-Occlusive Crisis in Sickle Cell Disease

Pain Practice ◽  
2015 ◽  
Vol 16 (6) ◽  
pp. 680-687 ◽  
Author(s):  
Lucia De Franceschi ◽  
Paolo Mura ◽  
Vittorio Schweiger ◽  
Elisa Vencato ◽  
Francesca Maria Quaglia ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Breakthrough Pain ◽  
Pain Medication ◽  
Cell Disease ◽  
Early Management ◽  
Fentanyl Buccal Tablet ◽  
Buccal Tablet

scholarly journals Clinical and Biomarker Predictors for Avascular Necrosis in Sickle Cell Disease

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3091-3091
Author(s):  
Michael Rabaza ◽  
Maria Armila Ruiz ◽  
Liana Posch ◽  
Faiz Ahmed Hussain ◽  
Franklin Njoku ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Board Of Directors ◽  
Avascular Necrosis ◽  
Sickle Cell ◽  
Research Funding ◽  
Medical Attention ◽  
Cell Disease ◽  
Advisory Committees ◽  
Early Management

Abstract Introduction Sickle cell disease (SCD) affects 1 in 365 African Americans and approximately 25 million people world-wide. A common skeletal system complication is avascular necrosis (AVN), which can cause substantial pain and a reduced quality of life. While early management of AVN is focused on increasing range of motion with physical therapy and pain relief, there are no clear predictors for who is more likely to develop AVN and earlier institution of these preventive measure could help decrease disease progression. Vascular endothelial growth factor (VEGF) is a biomarker of endothelial injury and may indicate reduced vascular supply to the femoral or humeral head. Here we describe potential risk factors and biologic pathways for AVN in SCD, as understanding these may lead to improvements in future monitoring, early detection, and early intervention practices. Methods We investigated clinical and laboratory risk factors associated with AVN in a cohort of 435 SCD patients from our center. Blood samples, clinical, and laboratory data were collected at the time of enrollment during a clinic visit. Genotyping for alpha thalassemia was performed by PCR and the serum concentration of VEGF was measured by ELISA. AVN status was confirmed by review of the medical record and available imaging. We conducted a cross-sectional analysis comparing categorical and linear variables by AVN status using the chi-square and Kruskal-Wallis test, respectively. The independent association of the clinical and laboratory variables with AVN status was determined by logistic regression analysis. The initial model included variables with a P-value < 0.1 on univariate analysis and the final model was ascertained by stepwise forward and backward selection. Median values and interquartile range (IQR) are provided. Results The median age of the cohort was 32 (IQR, 24 - 43) years, 57% (250/435) were female, and 46% (198/435) were on hydroxyurea. AVN was observed in 34% (149/435) of SCD patients. SCD patients with AVN were older, had more frequent vaso-occlusive crises requiring medical attention, and had a higher body mass index (Table I) (P ≤ 0.002). We measured VEGF in 241 of the SCD patients with serum samples available at the time of enrolment. Serum VEGF concentrations trended higher in SCD patients with versus without AVN (420 vs. 359 pg/mL, respectively; P = 0.078). In the multivariate analysis model, AVN was independently associated with increased number of vaso-occlusive crises (OR 1.1, 95% CI: 1.0 - 1.14; P = 0.02), AST concentration (natural log OR 0.5, 95% CI: 0.2 - 0.9; P = 0.03), VEGF concentration (natural log OR 1.4, 95% CI: 1.0 - 1.9; P = 0.047), and tobacco use (OR 1.9, 95% CI: 0.9 - 3.7; P = 0.078). Discussion In conclusion, we demonstrate a high prevalence of AVN in an adult cohort of SCD patients. The presence of AVN was independently associated with a greater frequency of vaso-occlusive pain episodes, which may demonstrate a shared pathophysiology between AVN and vaso-occlusion that merits further investigation. We demonstrate that serum VEGF concentrations are higher in SCD patients with AVN and may be a clinical tool to identify those at high-risk and for earlier intervention for this complication. Figure 1 Figure 1. Disclosures Gordeuk: Modus Therapeutics: Consultancy; Novartis: Research Funding; Incyte: Research Funding; Emmaus: Consultancy, Research Funding; Global Blood Therapeutics: Consultancy, Research Funding; CSL Behring: Consultancy. Saraf: Pfizer: Research Funding; Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees, Research Funding; Novartis: Membership on an entity's Board of Directors or advisory committees, Research Funding.

scholarly journals Average Chronic Pain Medication Needed in Adult Patient with Sickle Cell Disease and Its Relation to Vitamin D Level

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4857-4857
Author(s):  
Samip Master ◽  
Shajadi Patan ◽  
Shashank Cingam ◽  
Runhua Shi ◽  
Richard Preston Mansour
Keyword(s):  
Vitamin D ◽  
Chronic Pain ◽  
Standard Deviation ◽  
Sickle Cell Disease ◽  
Adult Patient ◽  
Retrospective Analysis ◽  
Sickle Cell ◽  
Pain Medication ◽  
Cell Disease ◽  
Vitamin D Levels

Abstract Introduction: The chronic pain in sickle cell disease (SCD) arises from chronic bone damage as a consequence of bone marrow infraction during vaso-occulsive events. There are certain barriers to adequate pain management in adult patients with SCD, namely, limited knowledge among the clinicians, inadequate assessment, concerns about addiction, and biases against opioid use. We did retrospective analysis to investigate the average pain medication needed by adult patient with SCD. We also did analysis to see if there was a relation between plasma vitamin D level and amount of pain medication needed. Methods: We take care of approximately 300 active adult SCD at Hematology clinic at our institute. We did a retrospective analysis of 458 adult patients with SCD seen at our clinic between 2001 and 2016. We collected data on type of SCD, plasma 25 -hydroxyvitamin d level and amount of opioid pain medication in mg. To get uniform units of opiates, we converted all the different opiates into morphine. Results: The average morphine dose in a 24 hours period needed to manage chronic pain in an adult patient with SS type was 84 mg with standard deviation of 72, for SC type was 60 mg with standard deviation of 72 and for sickle beta thal type was 72 mg with standard deviation of 71. There were 4 patients with SS with hereditary persistence of hemoglobin F and average opiate dose in them was 84 mg. We obtained vitamin d level on 223 patients and out of them, 47 had vitamin d level of <4.2 ng/ml (lowest level reportable by our lab). We also found negative correlation between amount of pain medication and vitamin D level. The spearman correlation coefficient was -0.2 and p value was <0.01. Conclusion: Because we were unable to find any previous reports of the correlation of vitamin D levels and opiate use in an adult population with SCD, we believe that this is the first study to report this correlation. It also provides a rough estimate regarding average amount of opiates that an adult patient with SCD needs. This correlation between Vitamin D levels and opiate requirement supports our current practice of screening all patients for Vitamin D deficiency using 25hydroxy vitamin D levels and treating all patients who are found to be deficient. We do not know if this Vitamin D replacement will reduce pain, bone health or the amount of opiate medication needed in the future. Disclosures No relevant conflicts of interest to declare.

scholarly journals Optimism Predicting Daily Pain Medication Use in Adolescents with Sickle Cell Disease

2007 ◽  
Vol 33 (3) ◽  
pp. 302-309 ◽  
Author(s):  
Laura Pence ◽  
Cecelia R. Valrie ◽  
Karen M. Gil ◽  
Rupa Redding-Lallinger ◽  
Charles Daeschner
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Medication Use ◽  
Pain Medication ◽  
Cell Disease ◽  
Daily Pain

scholarly journals Sickle cell disease: a comprehensive program of care from birth

Hematology ◽  
2019 ◽  
Vol 2019 (1) ◽  
pp. 490-495
Author(s):  
Mariane de Montalembert ◽  
Léon Tshilolo ◽  
Slimane Allali
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Care Professionals ◽  
Improve Patient Care ◽  
The United States ◽  
Cell Disease ◽  
Pneumococcal Infections ◽  
African Countries ◽  
Early Management ◽  
High Resource

Abstract As more children are appropriately being diagnosed, the burden of sickle cell disease is increasing greatly in Africa and in high-resource countries such as the United States and Europe. Early management is mandatory, but newborn screening is not implemented everywhere. Point-of-care testing devices are increasingly being used in low-resource countries, showing good sensitivity and specificity. Because the diagnosis is often traumatic for the families, the announcement should be made by an experienced person. The development of care networks is urgently required to facilitate daily life by defining the respective functions of nearby and highly specialized health care professionals, who should work in close collaboration. Comprehensive programs targeting the prevention of pneumococcal infections, malaria in infested zones, and stroke may substantially improve patient care. Hydroxyurea is increasingly being used, but whether it should be systematically prescribed in all children is debated, and its access is still limited in many African countries. Yearly checkups should be organized early in life in order to screen and then treat any organ impairment. Enhancing parents’ and patients’ knowledge and skills is mandatory.

scholarly journals Parents’ pain medication underdosing is associated with more emergency department visits in sickle cell disease

2017 ◽  
Vol 65 (4) ◽  
pp. e26906 ◽  
Author(s):  
Andrea K. Morrison ◽  
Matthew P. Myrvik ◽  
David C. Brousseau ◽  
Amy L. Drendel ◽  
J. Paul Scott ◽  
...  
Keyword(s):  
Emergency Department ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pain Medication ◽  
Emergency Department Visits ◽  
Cell Disease

scholarly journals Medical Marijuana Certification for Patients with Sickle Cell Disease: A Survey Study of Patient's Use and Preferences

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1094-1094
Author(s):  
Susanna A Curtis ◽  
Jonathan Spodick ◽  
Dana Lew ◽  
John D. Roberts
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Medical Marijuana ◽  
Marijuana Use ◽  
Pain Medication ◽  
Survey Study ◽  
Cell Disease ◽  
Opioid Use ◽  
Application Process

Abstract Background: In June 2016 the Yale Sickle Cell Disease (SCD) Clinic began to certify adult patients for medical marijuana (MM). Illicit marijuana (IM) use is common in patients with SCD and most who use it feel it treats their pain. While there is limited data on the efficacy of marijuana for SCD-related symptoms, our team opted to certify patients due to speculation that it would be safer based on improved regulation and reduced legal risk of MM compared to IM. We designed a survey to assess why and how participants used IM and MM and how they felt the two substances compared with respect to SCD-related symptom management. We hypothesized that most participants who were certified for MM had been previous IM users who felt that marijuana had a positive effect on pain. Methods: All patients were educated on safety risks of MM before certification. After certification patients were required to submit regulatory documents the state of Connecticut and pay a $100 annual fee prior to obtaining MM from dispensaries. Patients who had been certified for MM at our clinic were contacted by phone or at regularly scheduled clinic visits. Survey questions were read to participants and responses were recorded. Questions regarding reasons for inability to access marijuana, reasons for marijuana use and method of marijuana use were open-ended and all answers were recorded and then categorized. For questions regarding the comparisons of IM to MM subjects were asked to answer either agree, disagree, or don't know. Rates of opioid use, reasons, frequency, and methods of marijuana use before and after obtaining MM were compared using Fisher's exact test. Results: Subject Demographics: Our clinic serves approximately 150 patients, 50 of whom have been certified for MM. 27 subjects were offered the survey and 24 chose to participate. Of those who participated 12 (50%) have been able to obtain MM from a dispensary. Those who obtained MM were 29.7 ± 7.8 (mean ± SD) years old and 42% female. Those who did not obtain MM were 37.8 ± 15.3 years old and 50% female. When asked why they were unable to obtain MM 67% noted the cost of certification and 33% noted challenges with the application process. Prior to certification 79% used IM. Of those who had not accessed MM after certification 64% reported using IM, and of those who had accessed MM 44% reported also using IM. Reasons for, frequency, and methods of marijuana use: The reasons subjects reported using IM were similar to the reasons they reported requesting MM certification (Table 1). Those who obtained MM used at the same rates as subjects had previously used IM (Table 1). Though subjects who obtained MM continued to have high rates of leaf/bud/flower use, they were more likely to also utilize ingestible products such as oil and edibles (Table 1). Changes in marijuana and opiate use: After obtaining MM 58% felt they had less pain and 53% reported they used less pain medication. However, there was no change in rates of reported opioid use after obtaining MM (Table 1). Comparisons of IM and MM: When asked questions comparing MM to IM 92% of participants felt that MM was safer, and 92% noted it was less likely to cause legal toxicity. 46% of patients felt MM was stronger than IM and 31% did not. 62% of subjects felt MM controlled their symptoms better than IM. However, 39% of subjects felt that MM was less convenient than IM (31% felt it was not and 31% did not know) and 62% of subjects felt that it was more expensive. Conclusions: To our knowledge this is the first report of medical marijuana certification in a SCD clinic. One third of our patients requested MM certification, the majority of whom were already utilizing IM. They used MM for similar reasons and at similar rates as they had previously used IM. Once MM was obtained patients were more likely to use oral marijuana such as edibles and oil. Oral marijuana may be preferable to inhaled due to decreased risk of lung toxicity. Patients also reported that MM was better for their pain, allowed them to reduce pain medication use, and was safer than IM. Cost of MM was both a barrier to obtaining access and to purchasing IM. In conclusion, certifying patients for MM appears preferable to patients and may be associated with an improved safety profile. Table Table. Disclosures No relevant conflicts of interest to declare.

Prevalence and Predictors of Chronic Organ Damage in Adults with Sickle Cell Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4624-4624
Author(s):  
Syed N Haider ◽  
Jun Tang ◽  
Raymond U. Osarogiagbon
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Organ Damage ◽  
Early Mortality ◽  
Categorical Variables ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Early Management ◽  
End Organ Damage ◽  
Objective Evidence

Abstract Abstract 4624 Introduction Sickle cell disease (SCD) affects about 80,000 individuals in the US and about 1 of every 400 African Americans. Although SCD is characterized by acute painful crisis events, pain rates are a weak indicator of mortality risk. The main determinants of early mortality in adults are painless events such as sickle cell nephropathy (SCN), stroke due to cerebrovascular disease (CVD) and pulmonary hypertension (PHT). Sickle cell retinopathy (SCR) is a common cause of morbidity. There is a powerful correlation between end organ damage and early mortality. The prevalence of preclinical target organ damage has not been fully elucidated. We examined the prevalence of the full spectrum of end organ damage in a cohort at entry to our adult SCD program. We also contrasted the clinical and laboratory characteristics of patients with and without organ dysfunction. Patients and Methods Retrospective review of data on 118 adults (mean age 26.48±11.85 years) gathered upon entry into our program between February 2005 and October 2008. Historical presence of episodes of acute chest syndrome, pneumonia, stroke, avascular necrosis, osteomyelitis, leg ulcers, priapism, and cholecystectomy was quantitated. All patients underwent a standardized battery of tests to evaluate hematological and biochemical parameters at entry. Objective evidence of end organ damage was defined as follows: PHT, tricuspid regurgitant jet velocity (TRJV) ≥2.5 on echocardiography; SCN, glomerular filtration rate (GFR) < 90ml/min. and/or 24-hour protein>300mg and/or urine protein/creatinine ratio>0.3, all measured or calculated from 24 hour urine sample; CVD, evidence of previous ischemic and/or hemorrhagic infarct and/or aneurysmal dilatation on brain MRI or MR angiogram; SCR, background to proliferative retinopathy on fluorescent retinal angiography. Characteristics of patients were evaluated using t-test for continuous and the chi-square test for the categorical variables. Results There were 58 (49%) males and 60 (51%) females (p-value 0.85). Hemoglobin genotype: 74(63%) had SS/Sβ0, 27(23%) had SC, 11(9%) had Sβ+ while 5(4%) had S/hereditary persistence of fetal hemoglobin (S/HPFH).Of the patients who were tested for individual end organ damage 26% (24/91) had PHT, 52% (48/92) had nephropathy, 40% (31/78) had CVD while 38% (26/68) had retinopathy. The relevant statistically significant correlative variables for each type of end organ damage are shown in the table: Conclusions End organ damage was highly prevalent in this cohort of adults with SCD. Objective clinical and laboratory factors may predict the risk for end organ damage. This information can be used to develop an objective scoring system for disease severity based on early organ damage which may enable identification of patients at highest risk for severe morbidity and early mortality who might be candidates for more intensive evaluation and early management with disease-modifying interventions, beyond treatment of acute painful events. Disclosures: No relevant conflicts of interest to declare.

Hemolysis in sickle cell disease

1974 ◽  
Vol 133 (4) ◽  
pp. 624-631 ◽  
Author(s):  
T. A. Bensinger
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease
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