Opioid Use in Adults With Sickle Cell Disease Hospitalized During Vaso-Occlusive Crisis: A Systematic Review

BACKGROUND Sickle cell disease (SCD) is a genetic disorder of red blood cells that results in acute and chronic health problems, including painful syndromes. Opioid analgesia is the mainstay of moderate to severe pain management in SCD, although adjunctive psychosocial approaches such as cognitive behavioral therapy (CBT) are increasingly incorporated. CBT has been used in populations of various ages to address a wide range of issues, such as mood disorders and chronic pain. It is unclear if effective CBT reduces the use of opioids to manage pain in pediatric SCD. OBJECTIVE The aim of this study is to evaluate the association between CBT and decreased opioid use in children with SCD. METHODS In this systematic review protocol, we describe our approach to applying predetermined eligibility criteria to searches of PubMed (including Medline), Embase, Cochrane, Web of Science, and PsycINFO databases, as well as Google Scholar and grey literature. In particular, we will use keywords to search for English-language studies of individuals with SCD aged 21 years old and younger published before November 2018. Keywords will allow us to assess for the primary outcome—total use of opioid medications—and the secondary outcomes—pain intensity and emotional functioning—during pain management using a combined opioid and CBT approach, opioids alone, or CBT alone. The review team will use standardized abstraction forms to review articles at the title, abstract, and full-text levels. Finally, reviewers will assess the risk for bias, quality of evidence, and adequacy of data for quantitative versus qualitative synthesis. If meta-analysis is deemed inappropriate, a narrative review will be conducted. RESULTS We will report a summary of findings across studies that meet eligibility criteria to compare the extent to which adjunctive CBT is associated with decreased opioid use among children with SCD. CONCLUSIONS This systematic review will present the current state of the evidence on CBT and opioid use in pediatric SCD, which may inform clinical practice and health policy to support optimized pain management. INTERNATIONAL REGISTERED REPOR PRR1-10.2196/13211

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The factors influencing utilization of hospital services by adult sickle cell disease patients: a systematic review protocol

The JBI Database of Systematic Reviews and Implementation Reports ◽

10.11124/01938924-201513090-00004 ◽

2015 ◽

Vol 13 (9) ◽

pp. 18-29 ◽

Cited By ~ 2

Author(s):

Irina Benenson ◽

Yuri T. Jadotte ◽

Mercedes Echevarria

Keyword(s):

Systematic Review ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Hospital Services ◽

Systematic Review Protocol ◽

Cell Disease ◽

Factors Influencing ◽

Review Protocol

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Re: Manuscript titled, “Liver transplantation for sickle cell disease: a systematic review”

HPB ◽

10.1016/j.hpb.2021.01.015 ◽

2021 ◽

Author(s):

Juliet Emamaullee ◽

Linda Sher ◽

Kambiz Etesami ◽

Jeff Kahn ◽

Jim Kim ◽

...

Keyword(s):

Systematic Review ◽

Liver Transplantation ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Cell Disease

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The effect of iron chelation therapy on overall survival in sickle cell disease and β-thalassemia: A systematic review

American Journal of Hematology ◽

10.1002/ajh.25103 ◽

2018 ◽

Vol 93 (7) ◽

pp. 943-952 ◽

Cited By ~ 11

Author(s):

Samir K. Ballas ◽

Amer M. Zeidan ◽

Vu H. Duong ◽

Michelle DeVeaux ◽

Matthew M. Heeney

Keyword(s):

Systematic Review ◽

Overall Survival ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Chelation Therapy ◽

Iron Chelation ◽

Iron Chelation Therapy ◽

Cell Disease

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Efficacy of Allogeneic Hematopoietic Cell Transplantation (HCT) in Sickle Cell Disease: Results of a Systematic Review/Meta-Analysis

Blood ◽

10.1182/blood-2019-124955 ◽

2019 ◽

Vol 134 (Supplement_1) ◽

pp. 2106-2106

Author(s):

Madiha Iqbal ◽

Tea Reljic ◽

Ernesto Ayala ◽

Hemant S. Murthy ◽

Ambuj Kumar ◽

...

Keyword(s):

Systematic Review ◽

Sickle Cell Disease ◽

Clinical Outcomes ◽

Sickle Cell ◽

Pediatric Patients ◽

Medical Literature ◽

Meta Analysis ◽

Adult Patients ◽

Cell Disease ◽

Allogeneic Hct

Background: Sickle cell disease (SCD) is an inherited hemoglobinopathy which affects over 300,000 children born each year worldwide. In spite of improvement in supportive care in recent years, there is still a lack of effective treatment options. SCD leads to debilitating and cyclic episodes of erythrocyte sickling with progressive organ injury, contributing to lifetime morbidity and shortened life expectancy. Allogeneic HCT (allo-HCT) is a potentially curative therapy for SCD because engraftment is associated with resolution of the clinical phenotype of the disease and abrogation of its complications. Medical literature on allo-HCT for SCD is largely limited to children. Recent studies have evaluated the efficacy of allo-HCT in the adult population. Here, we conduct a systematic review/meta-analysis to assess the totality of evidence pertaining to the efficacy (or lack thereof) of allo-HCT in children and adults. Materials and methods: We performed a comprehensive search of the medical literature using PubMed/Medline, EMBASE and Cochrane library on July 3rd, 2019. We extracted data on clinical outcomes related to benefits (overall [OS] and disease free/event free survival [EFS/DFS]) and harms (non-relapse mortality [NRM] and graft failure [GF]), independently by two authors. Our search strategy identified 1001 references but only 30 studies (n= 1995 patients) were included in this systematic review/meta-analysis. We also performed a sub analysis on clinical outcomes for studies that included only pediatric patients (defined as <18 years) and those in patients ≥18 years of age. Results: Median age for patients enrolled in all the studies was at 10 years. Recurrent veno-occlusive crises represented the most common indication for allo-HCT followed by acute chest syndrome and stroke; nevertheless, most patients had more than one indication. Matched related donors (MRD) were the most common donor source (93%). Bone marrow was the most common source of hematopoietic stem cells (77%). Majority of patients underwent conditioning with myeloablative regimens (77%). Pooled OS rates (n=29 studies, 1681 patients) after allogeneic HCT was 95% (95%CI=93-96%) with low heterogeneity (I2=6.4%) among included studies (Figure 1). Pooled EFS/DFS rates (n=29 studies, 1894 patients) post-allografting was 90% (95%CI=87-93%) with moderate heterogeneity (I2=54%). Pooled NRM rates from 30 studies (1995 patients) was 4% (95%CI=2-6%) with low heterogeneity (I2=29.4%). Pooled GF rates from 28 studies (1851 patients) was 4% (95%CI=2-6%) with moderate heterogeneity (I2=55%). A subset analysis specifically for pediatric patients (n= 11 studies, 1009 patients, median age at 9.7 years) showed a pooled OS rate of 96% (95%CI=94-97%) with low heterogeneity (I2=0%); and for adult patients (n=3 studies, 51 patients, median age at 33.4 years) the pooled OS was 94% (95%CI=80-100%) with moderate heterogeneity (I2=52%). Pooled EFS/DFS for pediatric patients (n= 11 studies, 1009 patients) was at 89 %( 95%CI=84-93%) with moderate heterogeneity (I2=55.1%); and for adult patients (n=2 studies, 30 patients) was at 95% (95%CI=83-100%) with high heterogeneity (I2=96.5%). Pooled NRM from 10 studies with pediatric patients (281 patients) was at 6 % (95%CI=3-10%) with low heterogeneity (I2=0%); and from 3 studies with adult patients (51 patients) was at 1% (95%CI=0-7%) with low heterogeneity (I2=15.1%). Pooled GF from 10 studies with pediatric patients (281 patients) was at 3 % (95%CI=1-7%) with moderate heterogeneity (I2=40%); and from 2 studies with adult patients (30 patients) was at 5% (95%CI=0-17%) with high heterogeneity (I2=95.4%). Conclusions: The results of our systematic review/meta-analysis show excellent OS, EFS/DFS in children and adults undergoing allo-HCT with pooled OS rates exceeding 90%. The main limitation to offering an allo-HCT in SCD remains the availability of a suitable donor as 85% of patients meeting criteria do not have a MRD. We anticipate that with emergence of haploidentical transplantation the number of allo-HCT will increase in the future. GF remains a significant concern in this population and future studies should focus on novel immune suppression strategies to help reduce GF. Disclosures Kharfan-Dabaja: Pharmacyclics: Consultancy; Daiichi Sankyo: Consultancy.

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Hospitalization Events among Children and Adolescents with Sickle Cell Disease in Basra, Iraq

Anemia ◽

10.1155/2015/195469 ◽

2015 ◽

Vol 2015 ◽

pp. 1-8 ◽

Cited By ~ 4

Author(s):

Zeina A. Salman ◽

Meaad K. Hassan

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Readmission Rate ◽

Length Of Hospital Stay ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Opioid Use ◽

Asthma Symptoms ◽

The Mean ◽

High Readmission Rate

Objectives. Despite improvements in the management of sickle cell disease (SCD), many patients still experience disease-related complications requiring hospitalizations. The objectives of this study were to identify causes of hospitalization among these patients and factors associated with the length of hospital stay (LOS) and readmission.Methods. Data from 160 patients (<14 years old) with SCD who were admitted to the Basra Maternity and Children’s Hospital from the first of January 2012 through July 2012 were analyzed.Results. The main causes of hospitalization were acute painful crises (73.84%), infections (9.28%), acute chest syndrome (8.02%), and acute splenic sequestration crisis (6.32%). The mean LOS was4.34±2.85days. The LOS for patients on hydroxyurea (3.41±2.64days) was shorter than that for patients who were not (4.59±2.86days),P<0.05. The readmission rate (23.1%) was significantly higher among patients with frequent hospitalizations in the previous year (OR 9.352, 95% CI 2.011–43.49), asthma symptoms (OR 4.225, 95% CI 1.125–15.862), and opioid use (OR 6.588, 95% CI 1.104–30.336). Patients on hydroxyurea were less likely to be readmitted (OR 0.082, 95% CI 0.10–0.663).Conclusions. There is a relatively high readmission rate among patients with SCD in Basra. The use of hydroxyurea significantly decreases the LOS and readmission rate.

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Age-related prescription medication utilization for the management of sickle cell disease among Texas Medicaid patients

Journal of Opioid Management ◽

10.5055/jom.2021.0662 ◽

2021 ◽

Vol 17 (4) ◽

pp. 301-310

Author(s):

Nidhi Shukla, MS, MBA ◽

Jamie C. Barner, PhD, FAACP, FAPhA ◽

Kenneth A. Lawson, PhD, FAPhA ◽

Karen L. Rascati, PhD

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Age Groups ◽

Dual Therapy ◽

Cell Disease ◽

Opioid Use ◽

Medication Utilization ◽

Age Related ◽

Nonopioid Analgesics ◽

Chi Square Analysis

Introduction: Sickle cell disease (SCD) is associated with recurrent complications and healthcare burden. Although SCD management guidelines differ based on age groups, little is known regarding actual utilization of preventative (hydroxyurea) and palliative therapies (opioid and nonopioid analgesics) to manage complications. This study assessed whether there were age-related differences in SCD index therapy type and SCD-related medication utilization.Design and patients: Texas Medicaid prescription claims from September 1, 2011 to August 31, 2016 were retrospectively analyzed for SCD patients aged 2-63 years who received one or more SCD-related medications (hydroxyurea, opioid, or nonopioid analgesics).Outcome measures: The primary outcomes were SCD index drug type and medication utilization: hydroxyurea adherence, and days’ supply of opioid, and nonopioid analgesics. Chi-square, analysis of variance, and Kruskal–Wallis tests were used.Results: Index therapy percentages for included patients (N = 2,339) were the following: opioids (45.7 percent), nonopioids (36.6 percent), dual therapy-opioids and nonopioids (11.2 percent), and hydroxyurea (6.5 percent), and they differed by age-groups (χ2 = 243.0, p 0.0001). Hydroxyurea as index therapy was higher among children (2-12:9.1 percent) compared to adults (26-40:3.7 percent; 41-63:2.9 percent). Opioids as index therapy were higher among adults (18-25:48.0 percent; 26-40:54.9 percent; 41-63:65.2 percent) compared to children (2-12:36.6 percent). Mean hydroxyurea adherence was higher (p 0.0001) for younger ages, and opioid days’ supply was higher for older ages.Conclusions: Texas Medicaid SCD patients had low hydroxyurea utilization and adherence across all age groups. Interventions to increase the use of hydroxyurea and newer preventative therapies could result in better management of SCDrelated complications and reduce the frequency of pain crises, which may reduce the need for opioid use.

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Medical marijuana certification for patients with sickle cell disease: a report of a single center experience

Blood Advances ◽

10.1182/bloodadvances.2020002325 ◽

2020 ◽

Vol 4 (16) ◽

pp. 3814-3821 ◽

Cited By ~ 1

Author(s):

Susanna A. Curtis ◽

Dana Lew ◽

Jonathan Spodick ◽

Jeanne E. Hendrickson ◽

Caterina P. Minniti ◽

...

Keyword(s):

Health Care ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Medical Marijuana ◽

Care Utilization ◽

Cell Disease ◽

Opioid Use ◽

Admission Rates ◽

Single Center Experience ◽

Few Data

Abstract More than one-third of adults with sickle cell disease (SCD) report using cannabis-based products. Many states list SCD or pain as qualifying conditions for medical marijuana, but there are few data to guide practitioners whether or whom should be certified. We postulated that certifying SCD patients may lead to a reduction in opioid use and/or health care utilization. Furthermore, we sought to identify clinical characteristics of patients who would request this intervention. Retrospective data obtained over the study period included rates of health care and opioid utilization for 6 months before certification and after certification. Patients who were certified but failed to obtain medical marijuana were compared with those who obtained it. Patients who were certified were invited to participate in a survey regarding their reasons for and thoughts on certification. Patients who were certified for medical marijuana were compared with 25 random patients who did not request certification. Fifty adults with SCD were certified for medical marijuana and 29 obtained it. Patients who obtained medical marijuana experienced a decrease in admission rates compared with those who did not and increased use of edible cannabis products. Neither group had changes in opioid use. Patients who were certified for medical marijuana had higher rates of baseline opioid use and illicit cannabis use compared with those who did not request certification. Most patients with SCD who requested medical marijuana were already using cannabis illicitly. Obtaining medical marijuana decreased inpatient hospitalizations.

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