scholarly journals Opioid Dependency Significantly Increases Complications and Mortality in Sickle Cell Disease

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4701-4701 ◽  
Author(s):  
Stuthi Pavani Perimbeti ◽  
Kevin Ye Hou ◽  
Sabarina Ramanathan ◽  
Adonas Woodard ◽  
Daniel Kyung ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Hospital Mortality ◽  
Sickle Cell ◽  
Opioid Dependence ◽  
Conflicts Of Interest ◽  
Primary Diagnosis ◽  
Cell Disease ◽  
Opioid Use ◽  
Increased Risk ◽  
Complications And Mortality

Abstract Introduction: According to the National Center for Health Statistics, there was a twenty-one percent increase in deaths from drug overdose in the USA, with opioids contributing to about two-thirds of these deaths. Despite nationwide efforts to reduce opioid use, narcotic pain medications are still the most frequently used method of pain control in patients with sickle cell disease (SCD). We sought to examine the burden and complications of opioid dependence in patients with SCD. Methods: The National Inpatient Sample (NIS) for the years 1999 to 2014 was queried to yield adult admissions with a primary diagnosis of sickle cell disease (ICD-9 codes: 2826, 28260, 28261, 28262, 28263, 28264, 28268, 28269) and the admissions were stratified based on the presence of opioid dependence (ICD-9 codes: 30400-30403, 30470-30473, 30550-30553). Univariate and bivariate analyses were performed using the Chi square test. Cox proportional hazard regression was used to control for multiple confounders in calculating the hazard ratios of occurrence of complications and mortality. Results A total of 216,438 (Weighted N=1,066,536) admissions were identified between 1999 and 2014, out of which 1.6% (N=3603) had opioid dependence. The median age of patients with opioid dependence was 26 years, compared to 31 years in patients without opioid dependence. Average cost and length of hospitalization for patients with and without opioid dependence was $29,883 & $20,638 and 6.4 days & 5.1 days, respectively. The rates of various complications and Hazard Ratio (HR) of event occurrence among patients with and without opioid dependence are depicted in Table 1. After adjusting for demographics, hospital characteristics (region, bed size, location), and baseline comorbidities, SCD patients with opioid dependence had a 50% increased risk of in-hospital mortality (H.R 1.5, 95% C.I. 1.2-1.6, p<0.001) compared to those without. Conclusions: There is a correlation between opioid dependence and SCD complications among patients hospitalized with a diagnosis of SCD. Perhaps, patients predisposed to vaso-occlusive events require more frequent use of narcotics and become opioid dependent. Alternatively, the dependence on opioids may increase the incidence of complications from sickle cell disease. Regardless, patients with opioid dependence have significantly higher in-hospital mortality. The main limitation of this study is the use of ICD codes for identifying opioid dependence. In our opinion, the abnormally low incidence of opioid dependence in our data is a poor representation of the actual population. Opioid abuse is a serious concern that has heavy financial, social and public health implications on the welfare of the community, particularly those with SCD. This study highlights the need for more effective disease modifying agents for the treatment of this chronic and debilitating disorder. Disclosures No relevant conflicts of interest to declare.

Polymorphisms in TNFα Are Associated with Cerebrovascular Events in Sickle Cell Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1540-1540 ◽  
Author(s):  
Latorya A Barber ◽  
Allison E Ashley-Koch ◽  
Melanie E. Garrett ◽  
Karen L Soldano ◽  
Marilyn J. Telen
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Umbilical Vein ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Data Set ◽  
Clinical Complications ◽  
Cerebrovascular Events ◽  
Increased Risk

Abstract Abstract 1540 Poster Board I-563 Tumor necrosis factor alpha (TNFα) is a pro-inflammatory cytokine that stimulates phagocytosis, neutrophil recruitment, and expression of adhesion molecule VCAM-1. Plasma levels of TNFα have been found to be increased in sickle cell disease (SCD), and in vitro studies show that TNFα causes increased adherence of sickle red blood cells to human umbilical vein endothelial cells. A polymorphism in the promoter region of the TNFα gene has previously been associated with stroke in children with SCD (Hoppe et al., 2007). The current study was designed to identify associations of additional TNFα single nucleotide polymorphisms (SNPs) with SCD clinical complications. We analyzed five SNPs in the TNFα gene in 509 DNA samples of SCD patients from Duke University, University of North Carolina at Chapel Hill, and Emory University. In our data set, cerebrovascular events (CVEs), including overt stroke, seizures, and transient ischemic attacks, occurred in 133 out of 509 SCD patients (26.1%). SNP genotyping was performed using Taqman genotyping assays from Applied Biosystems. Due to low minor allele frequencies (<0.05) for all the SNPs examined, genetic associations with SCD clinical complications were examined by using allele tests. After controlling for age, gender, and use of hydroxyurea, two of the five TNFα SNPs, rs2228088 and rs3093665, were significantly associated with CVEs (p=0.013 and 0.029, respectively). The odds that SCD patients with a G allele at rs2228088 suffered from CVEs were 0.485 times that for patients with the T allele, suggesting that the G allele had a protective effect. The odds of having the A allele at rs3093665 and suffering from CVEs was also reduced, at 0.45 compared to the C allele. Neither SNP was found to be in linkage disequilibrium (LD) with any of the other SNPs analyzed (r2≤0.002). There was also strong association of SNP rs2228088 with acute chest syndrome (ACS; p=0.003), occurring in 382 out of 509 SCD patients (75%). However, in this analysis, the G allele was associated with increased risk for ACS (OR=2.313). In addition to the association with CVEs, the SNP rs3093665 was also significantly associated with priapism (p=0.03), reported by 86 of 223 male SCD patients (38.6%). In this analysis, the A allele was protective, as had been observed for CVE (OR=0.188). Additionally, we found no difference in steady state plasma TNFα levels between genotypes for the two SNPs. The functional significance of these SNPs is presently unknown. SNP rs2228088 is a synonymous SNP located in the coding region, and rs3093665 is located in the 3' untranslated region of the TNFα gene. While the G to T change at SNP rs2228088 does not translate to a change in amino acid sequence, the A to C change at SNP rs3093665 may affect mRNA stability due to its location. It is also possible that one or both of these SNPs is in LD with another functionally relevant SNP. Our findings thus support previous data implicating TNFα polymorphisms in risk for central nervous system events. Interestingly, ACS has been previously associated with seizures, stroke and altered mental status in adults and children with SCD (Vinchinsky et al., 2000) and with silent cerebral infarcts and reversible posterior leukoencephalopathy syndrome in children with SCD (Henderson et al., 2003). However, in our dataset, ACS and the occurrence of CVEs were not associated (p=0.24). Further studies are required to elucidate these and other factors that potentially correlate with SCD clinical complications. Disclosures No relevant conflicts of interest to declare.

Alloantibody Formation In Sickle Cell Disease

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2395-2395 ◽  
Author(s):  
Joep Sins ◽  
Saurabh Zalpuri ◽  
Marjon Cnossen ◽  
Anita W. Rijneveld ◽  
Jean-Louis Kerkhoffs ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
General Population ◽  
Sickle Cell ◽  
Cumulative Incidence ◽  
Cox Regression ◽  
Conflicts Of Interest ◽  
Cumulative Number ◽  
Cell Disease ◽  
Cox Regression Analysis ◽  
Increased Risk

Abstract Background Transfusion of red blood cells (RBC) is a common intervention to treat and prevent complications in sickle cell disease (SCD). However, frequent transfusions may lead to erythrocyte alloimmunization, thereby complicating donor matching procedures and posing patients at risk for hemolytic transfusion reactions. Little information is available about the risk of alloimmunization of sickle cell patients living in European countries. In the Netherlands extensive matching procedures to prevent alloimmunization were introduced a decade ago, but the effect on alloimmunization has not been evaluated yet. Aims The primary aim of this study is to evaluate the cumulative incidence of first alloantibody formation in a Dutch cohort of transfused SCD patients, and to compare this with a general Dutch RBC-transfused population. In addition, the effect of extended RBC matching protocols on the incidence of alloantibody formation in SCD and potential clinical determinants of alloimmunization will be assessed. Methods We conducted a retrospective cohort study and collected data on SCD patients (genotypes HbSS, HbSC, HbSβ0 and HbSβ+ thalassemia), diagnosed in three Dutch Sickle Cell Treatment Centers that received non-extended matched (ABO, RhD) RBC transfusions between 1984-2004 and extended matched (at least ABO, Rhesus phenotype, Kell) RBC transfusions between 2004-2011. In addition, we compared this population with a general population of 3 042 patients that received non-extended matched (ABO, RhD) RBC transfusions between 2005-2009 in the Leiden University Medical Center (Zalpuri et al. 2012). Cohorts were not matched for ethnicity. Alloimmunization risk was calculated as Kaplan-Meier incidence with cumulative number of transfusions as time variable. The association with the clinical determinants gender, SCD-phenotype and ethnicity was analyzed with Cox-regression analysis. Results A total of 291 SCD patients received 7 957 RBC units. Alloantibody formation occurred in 52 (17.9%) patients. The cumulative incidence of alloimmunization was 9% after 5 RBC units, 15% after 10, 24% after 20 and 34% after 40 RBC units. Multivariate analysis, correcting for the cumulative number of transfusions, demonstrated a significantly increased risk of alloantibody formation in our SCD cohort when compared to a general population of transfused patients (HR 7.5 (95% CI: 5.06-11.14), where the cumulative incidence of alloimmunization was 1.1% after 5, 2.4% after 10, 3.4% after 20 and 6.5% after 40 RBC units. No association could be demonstrated between alloantibody formation and clinical determinants such as gender, SCD-phenotype or ethnicity. However, a significant reduction in alloimmunization was observed in SCD patients that received their first transfusion from the year 2004 onwards, after preventive matching for Rhesus phenotype and Kell was introduced for SCD patients (HR 0.48 (95% CI: 0.24-0.97)). Conclusion The overall rate of first RBC alloantibody formation in our cohort was 17.9% and the risk of alloimmunization increased substantially with an increasing number of RBC transfusions. A unique comparison with a general cohort of Dutch transfused patients demonstrates a significantly higher risk of alloantibody formation in SCD, acknowledging earlier findings. This may partially be explained by differences in RBC antigens between patients of African descent and the predominantly Caucasian donors. Besides the number of RBC units, no other clinical risk factors for allo-immunization in SCD could be identified. The effectiveness of extended RBC matching protocols in the prevention of alloimmunization for chronically transfused patients in the participating centers was confirmed. Disclosures: No relevant conflicts of interest to declare.

The Cerebral Vasculopathy In Malian Sickle Cell Anemia Children: Lesson From Routine Transcranial Doppler Screening

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4687-4687
Author(s):  
Dapa Aly Diallo ◽  
Aldiouma Guindo ◽  
Alain Dorie ◽  
Boubacari Ali Touré ◽  
Baba Fané ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Conflicts Of Interest ◽  
Fetal Hemoglobin ◽  
Preventive Treatment ◽  
Cell Disease ◽  
Control Center ◽  
Increased Risk ◽  
Patients At Risk

Cerebral vasculopathy is one of the major complications of Sickle Cell Disease (SCD). 11% of the homozygous SCD patients experience stroke by age of 20 years. The use of Transcranial Doppler (TCD) allows identification of patients at risk for stroke and leads to implementation of a preventive treatment that contributes to limit the burden of SCD particularly during childhood. Using TCD screening, we evaluated the prevalence and incidence of cerebral vasculopathy in Malian SCD children. During the years 2008 to 2013, 572 children, 249 girls and 323 boys, age range (2-17yrs) were routinely screened by TCD at our Sickle Cell Disease Research and Control Center of Bamako, Mali. The overall prevalence of cerebral vasculpathy defined by conditional (1.5-1.79 cm/sec) and abnormal TCD (≥ 1.80 cm/sec) in this population is 17.1%. The highest proportion (92.9%) was observed in homozygous SCD patients while the percentage of affected patients was much lower in S/β0thalassemia (4.1%) and in SC (3.1%) patients. No cases were observed in S/β+thalassemia patients. SCD children <9 years old were more susceptible to cerebral vasculopathy complications than those above this age threshold (P<0.001). Low hemoglobin levels and low fetal hemoglobin were associated with an increased risk of cerebral vasculopathy. During the study, 4 of 444 children with normal TCD converted to conditional TCD, while 5 of 68 children with conditional TCD converted to abnormal TCD. This conversion from conditional to abnormal TCD was associated with a mean decrease in Hb value of 0.37g/dL (P=0.002). In conclusion this study shows high prevalence and incidence of cerebral vasculopathy in Malian SCD children. While chronic transfusion programs significantly reduces the risk of stroke in SCD patients with abnormal TCD, at present there are no well articulated strategies to prevent conversion from conditional to abnormal TCD. A more comprehensive approach would hopefully reduce the morbidity and mortality due to cerebral vasculopathy in SCD affected children. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Sickle Cell Disease and Venous Thromboembolism: Hospital Mortality, Length of Stay and Cost

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2378-2378
Author(s):  
Michael Rainone ◽  
Stuthi Pavani Perimbeti ◽  
Rishi Shrivastav ◽  
Jeffrey A. Glassberg ◽  
Lawrence Cytryn
Keyword(s):  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Sickle Cell ◽  
Treatment Guidelines ◽  
Conflicts Of Interest ◽  
Primary Diagnosis ◽  
Cell Disease ◽  
Chi Square ◽  
Blood Institute

Abstract Introduction: It is estimated that there are 100,000 Americans living with Sickle Cell Disease (SCD). Patients with SCD experience a number of complications that frequently require hospitalization. SCD is a prothrombotic state that is commonly complicated by venous thromboembolism (VTE) and recurrent VTE. The National Heart, Lung and Blood Institute do not include VTE as one of the complications of SCD in their latest guidelines, and the topics of prophylaxis and treatment of VTE in SCD are not discussed. There are no guidelines specifically designed for the prophylaxis or treatment of VTE in the SCD population, and traditionally management guidelines for VTE in the general population are followed. Recent information on national prevalence, mortality, length of stay, and cost for hospitalized patients with with SCD complicated by VTE is limited. Methods: We used data from the Healthcare Cost and Utilization Project's National Inpatient Sample (NIS) from 1999-2014 to examine these variables. The data on SCD from 1999-2014 was analyzed using ICD-9-CM codes for SCD (ICD-9-CM: 282.41, 282.42, 282.6, 282.60, 282.62, 282.63, 282.64, 282.68, 282.69) in the primary diagnosis field, and VTE (ICD-9-CM: 453.40, 453.41, 453.42, 453.82, 453.83, 415.11, 415.19) in the secondary diagnosis field which includes codes for venous thrombosis and pulmonary embolism. Univariate and bivariate statistical analysis was performed using the chi-square test. Multivariate analysis was performed using cox proportional hazard regression. The alpha was set at 0.05. Results: Over a 15 year period, from 1999-2014, a total of 217,791 (weighted N = 1,073,215) admissions with SCD were identified. A total of 7,898 admissions were associated with VTE. Mean age at admission of those with VTE was 27.42 (+/- 0.05) years and those without VTE was 34.00 (+/- 0.51) years. In patients with SCD and VTE, the average inpatient mortality was 3.08% (p < 0.0001) versus mortality of 0.27% in patients that did not have VTE. The hazard ratio for mortality was 4.18 (CI: 2.95-5.93) (p < 0.0001). Length of stay in the SCD with VTE group was 10.45 days (+/- 0.43) versus 5.09 days (+/- 0.02) (p < 0.0001) in SCD without VTE. Overall hospital cost was higher in those with VTE at $60,055 (+/- $1,940) versus $28,729 (+/- 232.97) (p < 0.0001) in those without VTE. Conclusions: Patients with SCD and VTE experience significant morbidity, mortality, prolonged hospitalization and increased cost associated with this complication of the disease as was observed in this study. Furthermore, patients who experience VTE are significantly younger than those who do not, with mean age of 27 versus 34. After controlling for multiple confounders like age, race, sex, income, comorbidities, the presence of VTE is associated with a significantly higher risk of mortality in SCD. Currently, there are no prophylaxis or treatment guidelines designed specifically for patients with SCD and VTE. We recommend the use of antithrombotic prophylaxis or therapy in patients with SCD be evaluated in prospective studies. Disclosures No relevant conflicts of interest to declare.

Predictors of In-Hospital Mortality and Charges in Sickle Cell Disease: Results from the California Discharge Databases 1998–2005.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 432-432 ◽  
Author(s):  
John J. Strouse ◽  
Carlton Haywood ◽  
Sophie Lanzkron
Keyword(s):  
Logistic Regression ◽  
Sickle Cell Disease ◽  
Hospital Mortality ◽  
Sickle Cell ◽  
Odds Ratio ◽  
Private Insurance ◽  
Charlson Index ◽  
Cell Disease ◽  
Increased Risk ◽  
Age Of Death

Abstract Most studies of survival in sickle cell disease (SCD) include only patients followed at referral centers. We used the public set of the California Patient Discharge Databases (1998–2005) to compare in-hospital mortality and charges by patient characteristics. We included discharges with any diagnostic code for SCD (282.41–2, 282.60–69). We used the Charlson index (adapted for administrative data) to adjust for comorbid conditions. Hospital charges were adjusted to 1998 levels. Statistical methods included ANOVA, Wilcoxon rank-sum, and multivariate linear and logistic regression. We identified 57,887 admissions for SCD and 376 in-hospital deaths (0.65%) from 1998–2005. The mean age of death was 40 years (95% CI 38–42) for sickle cell anemia (HbSS) and 48.3 years (95% CI 44.1–52.5, p<.0005) for all other SCD. Median length of stay was 4 days (IQR 2–7 days) and charges were $10,027 (IQR 5547–18,302) for HbSS and 4 days (IQR 2–6 days) and $8045 (IQR 4375–16,253) for all other SCD (p<.0001). Women with SCD were older at the time of death (43.6 years) than men (41.4 years, p=.29) and age of death increased from 40.6 years in 1998–2000 to 44.2 in 2004–2005 (p=.17) but these differences were not significant. Mortality was increased with a Charlson Index of 1 or 2, older age, private insurance, a diagnosis of HbSS, and transfusion during the admission (Table 1). Women had a lower odds of death than men (OR 0.7, p<.05). Average annual charges for hospitalization in patients with SCD, adjusted to 1998 values, were $117,000,000 and decreased $1,960,000/year (−4.1%, p<.0001) for children and increased $1,150,000/year (1.4% p<0.05) for adults. Adjusted charges for all children were stable and increased 1.5% per year for adults. Charges per admission were $3167 higher for adults than children, $5608 higher per comorbid diagnosis, $6506 higher in transfused patients, and $3595 higher with a diagnosis of crisis (p<.0001 for all). Compared to MediCal, charges were $1186 higher for Medicare (p<.05), $2651 lower for other government insurance (p<.0001), and $3521 lower for self-pay (p<.05). Government (77%) and private insurance (21%) paid for most admissions. We identified a greatly increased risk of in-hospital morality with comorbid diagnoses and older age and moderately increased risk with private insurance, HbSS genotype, and transfusion during the admission. The increased risk with private insurance was surprising, as higher socioeconomic status is often associated with better health outcomes. This may reflect more stringent requirements for admission or financial barriers to outpatient and preventive services for SCD. Total charges decreased dramatically in children, possibly reflecting increased use of hydroxyurea for frequent pain and chronic transfusions for primary stroke prevention. Table 1: Odds Ratio for In-Hospital Death Based on Multivariate Logistic Regression Variable Odds Ratio (95% CI) P-Value NS indicates not significant Charlson Index=1 3.3 (2.4–4.6) <.0001 Charlson Index=2 12.9 (9.3–18) <.0001 Age (per year) 1.04 (1.03–1.05) <.0001 Private Insurance vs. MediCal 1.6 (1.2–2.2) <.0005 Medicare vs. MediCal 0.9 (0.7–1.3) NS Other SCD vs. HbSS 0.7 (0.5–0.9) <.0005 Transfusion 1.6 (1.2–2.0) <.0001

Renal Disease in Sickle Cell: Clinically Varied and Associated with Increased Mortality

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 90-90
Author(s):  
Sabarish Ayyappan ◽  
Paul Drawz ◽  
Mehdi Nouraie ◽  
Mariana E Hildesheim ◽  
Yingze Zhang ◽  
...  
Keyword(s):  
Renal Function ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Multivariate Analyses ◽  
Conflicts Of Interest ◽  
Laboratory Data ◽  
Red Cell ◽  
Cell Disease ◽  
Cell Destruction ◽  
Increased Risk

Abstract Abstract 90 The Walk-Phasst Study of sickle cell disease (SCD) affords a unique opportunity to examine renal function in a large number of adults with SCD. Extensive clinical and laboratory data were obtained on 463 adults with HbSS and 127 adults with HbSC. Where possible, correlates for estimated glomerular filtration rate (eGFR, calculated by the 4-value MDRD equation) and albuminuria/proteinuria were also evaluated in historical data, from SS adults in the CSCCD cohort and the Multicenter Study of Hydroxyurea (MSH) in sickle cell disease. Adjusted decline in eGFR was more rapid in SS compared with SC, at −2.6 and −0.92 ml/min/1.73 m2/year, respectively (p<0.001). In SS, similar declines in cross-sectional eGFR with age were seen in adults in the CSCCD (n=705) and MSH (n=298) cohorts, at −2.9 and −1.9 ml/min/year (BSA corrected), respectively. Multivariate analysis of the SS cohort in of Walk-Phasst revealed that depressed eGFR values were associated with an elevated estimated pulmonary arterial systolic pressure (as reflected in a higher tricuspid regurgitant jet velocity, measured by echocardiogram, p=0.007) and with evidence for inflammation (an elevated white blood cell count, p=0.018). In SC adults, in contrast, lower eGFRs were primarily associated with a diminished erythroid reserve (depressed absolute reticulocyte counts, P=0.005). Albuminuria (≥30 mg albumin/gm creatinine) was detectable in 66 and 41 % of adults with HbSS and HbSC in Walk-Phasst (185/287 evaluable SS and 25/61 evaluable SC, p=0.001). In Walk-Phasst, albuminuria in SS was associated with evidence of increased red cell destruction (lower total hemoglobin (P=0.07), higher LDH (P<0.001), and higher reticulocyte count (P=0.017)). In SC, albuminuria was associated with a higher LDH (P=0.01). 159/657 (24%) of adult SS subjects in the CSCCD cohort had trace to 4+ proteinuria, using a method (urine dipstick analysis) that is less sensitive and less quantitative than the albumin-to-creatinine ratio used in Walk-Phasst. Multivariate analyses again suggested a strong association between a depressed Hgb and proteinuria (P<0.001) in CSCCD, and LDH was also associated with proteinuria, in univariate analyses (P<0.001). In Walk-Phasst the absence of albuminuria in all subjects with SCD was associated with lesser mortality in multivariate analyses (Hazard ratio 0.62 (0.4–0.9, P=0.02). No similar association was seen between eGFR and mortality. Subjects with SS in Walk-Phasst had depressed serum bicarbonate levels, of 23.8±3.4 compared with 24.7±3.2mEq/dL in SC (p<0.005) and 24.8±3.3 mEq/dL in the non-CKD general population (Shah et al, 2009). In multivariate analyses, acidemia was associated with both increased destruction and decreased production of red cells, e.g. higher EPO (P=0.003) and total bilirubin levels (P<0.001), but lower reticulocyte counts (P=0.06). Impaired physiologic functioning in acidemic subjects with HbSS, manifest as a depressed 6MWD (P<0.001) and a lower eGFR (P<0.001), was seen. No effect of bicarbonate level on mortality in SCD patients was evident in Walk-Phasst. In conclusion, renal function is perturbed in sickle cell syndromes in several ways, including more rapid decrement in eGFR, highly prevalent albuminuria, and, in SS, marked abnormalities in acid-base balance. Albuminuria is associated with anemia in two large cohorts of sickle cell disease patients and, in Walk-Phasst, with evidence for enhanced red cell destruction. Importantly, albuminuria correlated with an increased risk for mortality in sickle cell disease. (We acknowledge the many contributions made by the Walk-PHASST Investigators: Badesch DB, Barst RJ, Castro OL, Girgis RE, Gibbs JS, Goldsmith JC, Hannoush H, Hassell KL, Kato GJ, Krishnamurti L, Lanzkron S, Machado RF, Morris CR, Novelli EM, Rosenzweig EB, Sachdev V, Schraufnagel DE, Taylor JG 6th.) Disclosures: No relevant conflicts of interest to declare.

scholarly journals The Effect of Health Care Disparities on Complications and Mortality in Sickle Cell Disease

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5886-5886
Author(s):  
Stuthi Pavani Perimbeti ◽  
Kevin Ye Hou ◽  
Sabarina Ramanathan ◽  
Adonas Woodard ◽  
Daniel Kyung ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Healthcare Cost ◽  
Sickle Cell ◽  
Significant Role ◽  
Conflicts Of Interest ◽  
Acute Chest Syndrome ◽  
Insurance Status ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Increased Risk

Abstract Introduction: Sickle cell disease (SCD) is a chronic and debilitating disorder that affects approximately 100,000 Americans and results in the development of significant complications, leading to high numbers of hospitalizations, healthcare cost and mortality. Despite the advent of newer therapies, the overall rate of complications has continued to rise. We aimed to study the prevalence of complications in SCD as well as its relation to differing insurance status. Methods: Patients with SCD were identified using ICD-9 codes 2826, 28260, 28261, 28262, 28263, 28264, 28268 and 28269 from the Healthcare Cost and Utilization Project's Nationwide Inpatient Sample from 1999 to 2014. Admission with acute chest syndrome, acute myocardial infarction (AMI), avascular necrosis of the hip (AVN), end stage renal disease (ESRD), pneumococcal infections, splenic sequestration and stroke. Univariate and bivariate analyses were performed using the Chi square test. Cox proportional hazard regression was used to control for multiple confounders in calculating the hazard ratios of an event occurrence and mortality. Results: A total of 216,438 (Weighted=1,066,536) observations were identified from the years 1999 to 2014. The median age for male patients was 25 years and that for females was 27. Observing the trends from 1999 to 2014, the prevalence of acute chest syndrome increased from 1.22% to 8.82% (p=0.002), splenic sequestration from to 0.08 % to 1% (p=0.01) and AVN from 1 % to 8.8% (p=0.001). The prevalence of stroke and ESRD were unchanged over the interval studied. After controlling for confounding factors such as race, age, sex, income, comorbidities and insurance status, the hazard ratio of mortality for various complications is significantly elevated. Also, after controlling for multiple confounders, the patient's insurance status plays a significant role in the risk of developing a complication and subsequent mortality (Table 1). Discussion: The data indicates that the rate of complications from SCD have risen since 1999. With newer therapies and better understanding, the life expectancy of SCD patients has risen over time, nearly doubling from 1951 to 2018. The increased frequency of complications may be attributed to better survivorship and a rising number of older SCDs patients. However, our data also suggests that insurance status plays a significant role in the complication rate of SCD. The uninsured and patients with Medicaid have significantly increased risk of developing disease complications and resultant mortality. This could be the result of reduced access to care and health disparities due to race, socioeconomic status and insurance status. Disclosures No relevant conflicts of interest to declare.

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.

Lead Neuropathy and Sickle Cell Disease

PEDIATRICS ◽  
1974 ◽  
Vol 54 (4) ◽  
pp. 438-441
Author(s):  
Gerald Erenberg ◽  
Steven S. Rinsler ◽  
Bernard G. Fish
Keyword(s):  
Case Report ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Lead Poisoning ◽  
Cell Disease ◽  
Hemoglobin S ◽  
Increased Risk ◽  
Rare Manifestation ◽  
Poisoning In Children

Four cases of lead neuropathy in children with hemoglobin S-S or S-C disease are reported. Neuropathy is a rare manifestation of lead poisoning in children, and only ten other cases have been well documented in the pediatric literature. The last previous case report of lead neuropathy was also in a child with hemoglobin S-S disease. The neuropathy seen in the children with sickle cell disease was clinically similar to that seen in the previously reported cases in nonsicklers, but differed in both groups from that usually seen in adult cases. It is, therefore, postulated that children with sickle cell disease have an increased risk of developing neuropathy with exposure to lead. The exact mechanism for this association remains unknown, but in children with sickle cell disease presenting with symptoms or signs of peripheral weakness, the possibility of lead poisoning must be considered.

scholarly journals A case of severe multi-system disease in a sickle cell SC patient caused by parvovirus B19 infection

2021 ◽  
Vol 2 (5) ◽  
Author(s):  
Mohamed Almuqamam ◽  
◽  
Swetha Madhavarapu ◽  
Nataly Apollonsky ◽  
◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Parvovirus B19 ◽  
Acute Infection ◽  
Organ Injury ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Increased Risk ◽  
Parvovirus B19 Infection

Sickle Cell Disease (SCD) is an inherited hemoglobinopathy, which results in production of abnormal hemoglobin S. HbSC disease is a variant of SCD, which shares a similar clinical complication profile to HbSS disease, but often thought to be a milder condition. In patients with SCD, Hb S in deoxygenated state undergoes polymerization, leading to hemolysis, vaso-occlusive events, and eventually end-organ damage. Among other complications in patients with SCD is increased risk of complications caused by parvovirus B19. We present a case of a 14-year-old female with HbSC disease who presented to the emergency room with complaint of abdominal pain and found to have splenic sequestration. Splenic sequestration progressed rapidly, Hemoglobin (hb) dropped to 4.6 g/dl and acute chest syndrome (ACS) developed. She was treated following the ACS protocol, received 4 units of Packed Red Blood Cells (PRBC) and subsequently underwent a single volume PRBC exchange transfusion. Considering her unusual presentation, with severe ARDS from alveolar hemorrhage requiring mechanical ventilation and multi-organ injury, several autoimmune and infectious conditions with a cytokine storm component including COVID-19 disease, were considered. Results of viral testing revealed parvovirus B19 IgM antibodies signifying an acute infection. She fully recovered with supportive care and was discharged home. Multisystem involvement simulating connective tissue disorders or malignancies with acute parvovirus B19 infection has been reported and is considered extremely rare. To our knowledge, there were no reports of pediatric patients with SC disease presenting with splenic sequestration and ACS in the setting of parvovirus B19 multisystem disease. Keywords: sickle cell disease; acute respiratory distress syndrome; acute chest syndrome; parvovirus B19.

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