Evaluation of Intravenous Diphenhydramine Use in Patients with Sickle Cell Vaso-Occlusive Crisis

2020 ◽  
pp. 001857872095417
Author(s):  
Katherine Rector ◽  
Shelby Merchant ◽  
Rachel Crawford ◽  
Justin R. Arnall ◽  
James Symanowski ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Sickle Cell ◽  
Cell Disease ◽  
Median Length ◽  
Significant Difference ◽  
Oral Group ◽  
Icd 10 ◽  
Secondary Endpoints ◽  
Group 2

Purpose: To compare the incidence of oversedation between oral and parenteral diphenhydramine therapy for treatment of opioid-induced pruritus in patients with sickle cell disease vaso-occlusive crisis (SCD VOC). Methods: This retrospective, single-center, cohort study included patients greater than or equal to 18 years old with sickle cell disease admitted for vaso-occlusive crisis who received either intravenous or oral diphenhydramine for opioid-induced pruritus. Patients were identified through ICD-9 and ICD-10 codes from June 1, 2016 through July 1, 2017. Rates of oversedation were compared between the 2 formulations. Secondary endpoints included length of stay, amount and duration of diphenhydramine, rate of acute chest and indication for IV therapy. Results: Fifty unique patients were included in the analysis representing 121 admissions. Seven patients received both formulations on separate admissions and were included in both groups. Twenty-nine percent of patients in the IV diphenhydramine group experienced oversedation (12/42) versus 13% in the oral diphenhydramine group (2/15, P = .312). The average number of admissions was significantly higher in the IV versus oral group (2.45 vs 1.20; P = .005) with average and median length of stay also significantly higher in the IV versus oral group (30.57, 16.0 vs 10.67, 10.0; P = .003). Conclusion: While there was no statistically significant difference in the rates of oversedation with use of IV versus oral diphenhydramine formulations, patients with SCD VOC who received IV diphenhydramine had more frequent admissions and a longer length of stay. Clinicians may consider oral diphenhydramine preferentially in appropriate patients over IV administration.

Vasoocclusive Painful Episodes and the Risk of Acute Chest Syndrome in Children with Sickle Cell Disease: Does the Selection of Parenteral Opioid Matter?

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 442-442
Author(s):  
Ram V. Kalpatthi ◽  
Matt Hall ◽  
Jignesh Dalal ◽  
Gerald M Woods
Keyword(s):  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Respiratory Depression ◽  
Sickle Cell ◽  
Patient Population ◽  
Lower Risk ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Median Length ◽  
Hospitalization Costs

Abstract Background: Vaso-occlusive painful episodes (VOC) is the most common cause of hospitalization in patients with sickle cell disease (SCD) that often requires opioid analgesics and intravenous fluids. However, due to respiratory depression, the use of opioids during these painful episodes has been associated with the risk of development of acute chest syndrome (ACS), the major cause of early mortality in these patients. Nalbuphine is a unique opioid analgesic that effectively treats pain but lacks significant respiratory depression. Previous small scale studies (Pediatr Blood Cancer 2005 and J Pediatr Hematol Oncol 2011) suggested a lower risk of ACS in patients receiving Nalbuphine during VOC when compared with morphine. We sought to compare outcomes and the risk of ACS in pediatric sickle cell patients hospitalized for VOC in relationship to different opioid treatments. Methods: We used the Pediatric Health Information System (PHIS), an administrative database of children's hospitals in the US. Patients ≤ 21 years of age with SCD from 43 hospitals from 2005-2013 were included in the study. SCD patients who had a primary diagnosis VOC (ICD-9 codes 282.62, 282.64, 282.42 and 282.69) were included. VOC hospitalizations were divided into four different narcotic analgesic groups. In bivariate analyses, we compared patient demographics, treatment details, risk of ACS, length of stay, hospital costs, and readmissions across groups using chi-square tests or Kruskal-Wallis tests as appropriate. Multivariable models accounted for hospital clustering with generalized estimating equations for binary outcomes and generalized linear mixed effects models with random hospital intercepts and an exponential distribution (due to skewness of the data) for continuous outcomes. A p-value <.05 was considered statistically significant. Results: From 2005 to 2013, a total of 11260 unique pediatric SCD patients were identified. These patients had 42688 VOC hospitalizations, and received a single parenteral narcotic analgesic during each hospitalization (Morphine 82.2%, Hydromorphone 13.3%, Fentanyl 2.4% and Nalbuphine 2.1% of VOC hospitalizations). Table 1 describes the outcomes of VOC hospitalizations for all four narcotic groups. In unadjusted analysis, patients who received Nalbuphine only had significantly lower risk of ACS diagnosis (5.6%), ICU admissions (0.9%), shorter median length of stay (2 days) and lower median hospitalization costs ($4345) [Table 1]. Patients who received Morphine had less readmission within 3 and 7 days of discharge after VOC hospitalizations (Table 1). Multivariate analysis after adjusting for age, gender, hospital, season, race, payer source, and complex chronic conditions confirmed that patients who received Nalbuphine had significantly lower risk of ACS diagnosis, ICU admissions, shorter median length of stay, and lower median hospitalization costs compared to patients who received Morphine, Hydromorphone or Fentanyl (Table 2). Conclusions: In our largest pediatric in-patient sickle cell cohort, Nalbuphine was associated with significantly lower risk of development of ACS when compared with other opioids. In addition, patients received Nalbuphine had significantly lower ICU admissions, shorter hospital stay and lower hospitalization costs suggesting better pain control. However, our study shows that Nalbuphine in this patient population is rarely utilized. Prospective studies are needed to confirm this association and to elucidate the mechanisms that underlie these beneficial effects of Nalbuphine in this patient population. Figure 1 Figure 1. Figure 2 Figure 2. Disclosures No relevant conflicts of interest to declare.

The Effect Of Microalbuminuria On Duration Of Hospitalization In Adult Sickle Cell Disease Patients With Pain Crisis

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4694-4694
Author(s):  
Mohammed Shaik ◽  
Borys Hrinczenko
Keyword(s):  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Categorical Variables ◽  
Cell Disease ◽  
Exact Test ◽  
Blood Disorder ◽  
Significant Difference ◽  
Pain Crisis

Introduction Sickle cell disease (SCD) is a genetic blood disorder of hemoglobin resulting in severe morbidity and early mortality. The prevalence of microalbuminuria and/or proteinuria in children with SCD varies from 18 to 28% and increases with age. However, the exact prevalence in adults in not known. In the general population, the presence of albuminuria has been shown to be associated with all cause mortality. The urine microalbumin to creatinine ratio (MA) is considered to be an early sign of impending sickle cell nephropathy. We sought to investigate the association of MA with various SCD genotypes (HbSS, HbSC, HbS/β-thalassemia) in our clinic and also the length of stay (LOS) in hospitalized SCD patients with acute pain crisis. Methods Twenty-eight consecutive SCD patients diagnosed in our clinic by hemoglobin electrophoresis were included in our study. The patients (pts) age, gender, hemoglobin electrophoresis, and baseline MA were obtained. Based on their MA level they were divided into two groups, abnormal MA (MA ≥ 30 mg/g creatinine) or normal MA (< 30 mg/g creatinine). Eleven of these 28 patients were eventually hospitalized for a sickle cell related pain crisis. Their MA level was obtained within 24-hours of hospital admission and their hospitalization length of stay (LOS) was also recorded. We analyzed the association between the two groups of patients, those with normal or abnormal MA, with the different genetic variants of SCD in both our clinic and hospitalized pts. Furthermore, for hospitalized pts we also assessed an association of MA with their mean LOS. The Chi-square test/Fisher’s exact test was used for categorical variables and the T-test/Mann-Whitney test was used for numerical variables. Results All twenty-eight patients were African American without significant renal impairment, with 11, 10, and 7 pts with SS, SC and S β-thalassemia (Sβ), respectively. Fifteen of these pts had abnormal MA, 12 pts were female. The median age was 35.5 yrs (range, 19 - 59), median LOS was 3.5 days (range, 2-8). The SS pts had higher abnormal MA levels followed by SC and then Sβ (p=0.03) (Table 1). There was no significant difference in gender between the two groups (p=0.2). SCD pts admitted to the hospital for pain crisis with an abnormal MA within 24-hours of admission had a significantly higher mean LOS when compared to pts with normal MA (p=0.0089) (Table 1). Conclusion Microalbuminuria is more prevalent in the severe genotypes of SCD disease such as SS and SC vs. Sβ pts. Thereby, MA might be a useful biomarker of generalized SCD vasculopathy, in addition to a known marker of progressive nephropathy. Furthermore, MA has a significant impact on length of hospitalization. An abnormal MA obtained within the first 24-hrs of hospitalization of a SCD pt in pain crisis was predictive of prolonged hospital duration. Early and more aggressive supportive care symptom management for those patients might be a reasonable option but requires more study. Further large prospective clinical trials are needed to validate our findings. Disclosures: No relevant conflicts of interest to declare.

Emergency Department Physicians Management Versus Hospitalists Service Management Admittance Rates for Sickle Cell Disease Patients from an Emergency Department Observation Unit

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5568-5568
Author(s):  
Taylor Mueller ◽  
LaShon Sturgis ◽  
Patrick Loeffler ◽  
Ann-Marie Kuckinski ◽  
Abdullah Kutlar ◽  
...  
Keyword(s):  
Emergency Department ◽  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Sickle Cell ◽  
Service Management ◽  
Return Rate ◽  
Cell Disease ◽  
Return Rates ◽  
Patient Disposition ◽  
Significant Difference

Abstract Background: Emergency Department Observation Units (EDOUs) and Sickle Cell Pathways (SCPs) allow for protocol based rapid initiation of analgesic treatment for sickle cell disease (SCD) patients during a vaso-occlusive crisis (VOC). Initially, Emergency Department (ED) physicians managed patients on the SCP in the EDOU. After an administration change in September 2013, the Hospitalists service provided protocol based care in the EDOU for the SCP after the initial ED assessment. The Hospitalists service management model incorporates mid-level providers (Nurse Practitioners and Physicians Assistants) into the management of patients on the SCP. The SCP utilizes an individual dosage database using patient-controlled analgesia delivered narcotics. No other changes were made to the pathway during the study period other than the type of physician management. Objective: To compare admission rates between ED physicians and Hospitalists service management of patients on a SCP. Our secondary objective was to ascertain if there was a difference in the three and 30-day return rates of SCD patients managed by ED physicians as compared to hospitalists. Methods: This study was a retrospective database review using a database created from visits of patients with VOC to the EDOU. Data were analyzed for a 21-month period (December, 2011 - August, 2013) in which patient care was managed by ED physicians and the 21-month period (September, 2013 -May, 2015) in which it was managed by the Hospitalists service. 773 patient encounters were included from the ED physicians management period and 727 from the Hospitalists management period. The database was reviewed for information on length of stay (LOS), disposition, three and 30-day return, and readmission rates. Only uncomplicated pain crisis visits due to SCD presenting to the ED were included in this study. Results: The average EDOU length of stay (LOS) for a SCD patient during the ED management period was 17 hours and 54 minutes; during the Hospitalists management period, the average LOS was 18 hours and 23 minutes. The data were analyzed by patient disposition, three and 30-day return rate as shown in Figure 1 and Figure 2 below. Figures 1 and 2 show that the admittance rate for SCP patients during the ED management period was 16% while during the Hospitalist management period the admission rate was 24.8%. Statistical analysis shows that this difference is significant (p<0.0001). The 30-day return rates for patients who did not return at three days were not significantly different. Of the patients initially admitted to the hospital, the 30-day readmission rate following EDOU return under the ED management period (not included in the figure) was 8.9% while it was 15.6% for the Hospitalist management period (p<0.001). Discussion: Analysis of the data shows that the EDOU LOS was not statistically different between groups (p=.1853), suggesting that the disposition decision time and treatment time was similar for both types of physician management. Patients were admitted at a statistically significant higher rate under Hospitalist management than ED management. However, there is no statistically significant difference in the three day return rates between the two groups, indicating that this increased admittance did not lead to a decrease in return rates. The three-day return rate is a marker of the efficacy and appropriate disposition from the pathway. Similarly the 30-day return rates of the two groups were nearly identical (41.1% versus 39.8%). However, while the patients returned to the EDOU at the same rate, they were statistically more likely to be readmitted upon their return by hospitalists as compared to ED physicians (p<0.001). This study demonstrates Hospitalists service management, staffed with midlevel providers, produces different outcomes than ED management. Although the study does not explain this difference it raises important questions for further study regarding adherence to treatment protocol, confidence in disposition decisions, and inter-professional communications. Despite the differences in the outcomes between the two management groups, the outcomes of the Hospitalists group are better than national averages. Thus, this is a viable model for the pathway management of patients with SC VOC. Figure 1. ED physician management of the EDOU (December, 2011 - August, 2013) Figure 1. ED physician management of the EDOU (December, 2011 - August, 2013) Figure 2. Hospitalist management of the EDOU (September, 2013 - May, 2015) Figure 2. Hospitalist management of the EDOU (September, 2013 - May, 2015) Disclosures No relevant conflicts of interest to declare.

scholarly journals Pregnancies and Neonatal Outcomes in Patients with Sickle Cell Disease (SCD): Still a (High-)Risk Constellation?

2021 ◽  
Vol 11 (9) ◽  
pp. 870
Author(s):  
Pia Proske ◽  
Laura Distelmaier ◽  
Carmen Aramayo-Singelmann ◽  
Nikolaos Koliastas ◽  
Antonella Iannaccone ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Neonatal Outcomes ◽  
University Hospital ◽  
High Rate ◽  
Successful Outcome ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Significant Difference ◽  
Tract Infections

Background: This monocentric study conducted at the University Hospital of Essen aims to describe maternal and fetal/neonatal outcomes in sickle cell disease (SCD) documented between 1996 to 2021 (N = 53), reflecting the largest monocentric analysis carried out in Germany. Methods/Results: 46 pregnancies in 22 patients were followed. None of the patients died. In total, 35% (11/31) of pregnancies were preterm. 15 pregnancies in eight patients were conceived on hydroxycarbamide (HC), of which nine had a successful outcome and three were terminated prematurely. There was no difference regarding the rate of spontaneous abortions in patients receiving HC compared to HC-naive patients prior to conception. In patients other than HbS/C disease, pregnancies were complicated by vaso-occlusive crises (VOCs)/acute pain crises (APCs) (96%, 23/24); acute chest syndrome (ACS) (13%, 3/24), transfusion demand (79%, 19/24), urinary tract infections (UTIs) (42%, 10/24) and thromboembolic events (8%, 2/24). In HbS/C patients complications included: VOCs/APCs (43%, 3/7; ACS: 14%, 1/7), transfusion demand (14%, 1/7), and UTIs (14%, 1/7). Independent of preterm deliveries, a significant difference with respect to neonatal growth in favor of neonates from HbS/C mothers was observed. Conclusion: Our data support the results of previous studies, highlighting the high rate of maternal and fetal/neonatal complications in pregnant SCD patients.

Whole Blood Tissue Factor Procoagulant Activity Is Elevated in Patients With Sickle Cell Disease

Blood ◽  
1998 ◽  
Vol 91 (11) ◽  
pp. 4216-4223 ◽  
Author(s):  
Nigel S. Key ◽  
Arne Slungaard ◽  
Luke Dandelet ◽  
Stephen C. Nelson ◽  
Christopher Moertel ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Tissue Factor ◽  
Mononuclear Cell ◽  
Sickle Cell ◽  
Whole Blood ◽  
Procoagulant Activity ◽  
Cell Disease ◽  
Blood Samples ◽  
Significant Difference ◽  
Whole Blood Samples

Abstract We developed a simple assay for the measurement of tissue factor procoagulant activity (TF PCA) in whole blood samples that avoids the need for mononuclear cell isolation. This method combines convenience of sample collection and processing with a high degree of sensitivity and specificity for TF. Using this method, we have determined that TF PCA is detectable in whole blood samples from normal individuals, which is itself a novel observation. Essentially all PCA could be shown to be localized in the mononuclear cell fraction of blood. Compared with controls, whole blood TF levels were significantly (P &lt; .000001) elevated in patients with sickle cell disease (SCD), regardless of the subtype of hemoglobinopathy (SS or SC disease). No significant difference in TF PCA was observed between patients in pain crisis compared with those in steady-state disease. Because TF functions as cofactor in the proteolytic conversion of FVII to FVIIa in vitro, it was expected that an increase in circulating TF PCA would lead to an increased in vivo generation of FVIIa. On the contrary, FVIIa levels were actually decreased in the plasma of patients with SCD. Plasma TF pathway inhibitor (TFPI) antigen levels were normal in SCD patients, suggesting that accelerated clearance of FVIIa by the TFPI pathway was not responsible for the reduced FVIIa levels. We propose that elevated levels of circulating TF PCA may play an important role in triggering the activation of coagulation known to occur in patients with SCD. Because TF is the principal cellular ligand for FVIIa, it is possible that increased binding to TF accounts for the diminished plasma FVIIa levels.

Variation in hospitalizations and hospital length of stay in children with vaso-occlusive crises in sickle cell disease

2005 ◽  
Vol 44 (2) ◽  
pp. 182-186 ◽  
Author(s):  
Julie A. Panepinto ◽  
David C. Brousseau ◽  
Cheryl A. Hillery ◽  
J. Paul Scott
Keyword(s):  
Sickle Cell Disease ◽  
Length Of Stay ◽  
Sickle Cell ◽  
Hospital Length ◽  
Hospital Length Of Stay ◽  
Cell Disease

Cell Free Fetal and Total DNA Levels in Pregnancies at Risk of Sickle Cell Disease and Significant Ethnic Variation.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3791-3791
Author(s):  
Ageliki Gerovassili ◽  
Kypros H. Nicolaides ◽  
Swee Lay Thein ◽  
David Rees
Keyword(s):  
At Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Globin Gene ◽  
Cell Disease ◽  
Maternal Weight ◽  
Chromosome 11 ◽  
Significant Difference ◽  
African Caribbean

Abstract Cell free (cf) DNA in maternal circulation is increasingly investigated in pregnancy. We aimed to determine if sickle cell trait women had quantitative differences of cfDNA with controls and if there was an ethnic difference between the cfDNA levels of Northern European and African/African-Caribbean populations. Non-invasive prenatal diagnosis through quantification of fetal and total cfDNA was tested in 33 pregnant women at risk of carrying a fetus affected with sickle cell disease and 124 control pregnancies. Fetal cfDNA assays were based on two Y chromosome specific markers (SRY and DYS14) and total cfDNA was based on the β-globin gene on chromosome 11. Maternal age (MA), gestation age (GA), fetal sex, storage time prior to extraction, maternal weight and body mass index (BMI) before pregnancy were compared to the fetal and total cfDNA levels. No significant difference in the fetal or total cfDNA levels was found between any of the control pregnancies and the sickle cell trait mothers carrying HbAA, HbAS and HbSS fetuses. However, higher levels of total cfDNA, but lower fetal cfDNA levels were observed in the African/African-Caribbean population compared with the Nothern Europeans. A significant variation in cfDNA was found between ethnic groups, which should be taken under consideration in future studies measuring cfDNA.

Impact of Hydroxyurea on Thrombin Antithrombin Complex and Vaso-Occlusive Crisis in Pediatric Sickle Cell Disease.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5525-5525
Author(s):  
Mohsen Saleh Elalfy ◽  
Ashraf M. Abdelmonem ◽  
Soha Youssef ◽  
Heba Ismail
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Coagulation System ◽  
P Value ◽  
Cell Disease ◽  
Significant Difference ◽  
Healthy Control ◽  
Pain Crisis

Abstract Background: Several studies suggest that increased activity of the coagulation system may be important in the pathogenesis of vascular occlusion in sickle cell disease. Hydroxyurea (HU) has been shown to reduce the frequency of vaso-occlusive manifestations in both adults and children with sickle cell disease (SCD). Aim: To analyze the effect of HU on Thrombin-Antithrombin (TAT) as a marker of thrombin generation and hypercoagualbility in SCD and to find out the relation between TAT level and vaso-occusive crisis. Subjects and Method: we evaluated 37 child with sickle cell hemoglobinopathy (mean age 10.92±5.39 years) and 15 normal control children (mean age 9.75±6.34 years). Informed consent was obtained from patients and/or guardians and study approval by local IRB was obtained. Twenty-two patients (59.5%) were on HU, 15 (41.5%) patients did not receive HU, 7 (46.7%) of them were transfusion dependant. TAT assay was done in vitro using a sandwich enzyme immunoassay. Results: Mean patients’ age at institution of HU was 8.54± 3.85 years with median treatment duration of 4.5 years. Causes for initiating HU therapy were frequent blood transfusion in 11 patients (50%), frequent pain crisis (≥ 3/year) in 9 patients (41%), severe anemia and parents refusing blood transfusion in 1 patient (4.5%) and stroke in another patient (4.5%). HU dose was 20.82±4.95 mg/kg/day. We measured TAT in all patients and compared them to healthy control. There was significant difference in TAT level in sickle cell patients (198.86±185.7) compared to healthy control 2.91±0.94, [P value < 0.0001]. When the level of TAT was compared between the HU and non-HU groups we found that patients on HU had statistically significant lower TAT level (172.36 vs.225.37) [P=0.039]. There was also a significant negative correlation between HU dose and TAT level (p=0.03). A significant positive correlation between number of vaso-occlusive crisis/year [P=0.03], frequency of pain crisis/year [P=0.04], duration of pain crisis [P=0.03] and TAT level was observed. Conclusion: Hydroxyurea has significant inhibitory effect on thrombogenesis in sickle cell patients, which may be another mechanism for reducing vaso-occlusive crisis. Sickle cell children with higher TAT level had more frequent and severe vaso-occlusive crisis.

Sickle Cell Disease Mortality in the United States: Age at Death and Contributing Causes.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 81-81 ◽  
Author(s):  
Carlton Haywood ◽  
Sophie Lanzkron
Keyword(s):  
Sickle Cell Disease ◽  
Cause Of Death ◽  
Sickle Cell ◽  
Causes Of Death ◽  
College Education ◽  
Age At Death ◽  
Cell Disease ◽  
Time Period ◽  
Icd 10 ◽  
The U.S

Abstract BACKGROUND: In the early 1990’s, the Cooperative Study of Sickle Cell Disease (CSSCD) estimated a median life expectancy of 42 years for males, and 48 years for females with sickle cell anemia. We used death certificate data from the late 1990’s and early 2000’s to examine age at death and contributing causes of death for persons with sickle cell disease (SCD). METHODS: We used the National Center for Health Statistics Multiple Cause of Death (MCOD) files to examine age at death and contributing causes of death for persons in the U.S. with SCD during the years 1999 to 2004. The MCOD files contain data from all death certificates filed in the U.S. Each observation in the data has listed an underlying (primary) cause of death, as well as up to 20 conditions thought to contribute to the death. We used ICD-10 codes D570-D578 to identify all deaths attributed to SCD during the time period under study. Records with the ICD-10 code for sickle cell trait (D573) were excluded from further analyses. We used the Clinical Classification Software provided by the Healthcare Cost and Utilization Project to collapse all listed ICD-10 codes into smaller categories. Analyses of age at death were conducted using t-tests, median tests, ANOVA, and multiple linear regression as appropriate. RESULTS: From 1999 to 2004, there were 4553 deaths in the U.S. attributed to SCD (mean = 759/yr, sd = 42.6). SCD was listed as the primary cause in 65% of the deaths. 95% of the deaths were attributed to HbSS disease, and approximately 1% of the deaths were attributed to double heterozygous sickle cell disorders (SC/SD/SE/Thal). 50.4% of the deaths were among males. 64% of the decedents had a high school education or less. 54% of the decedents lived in the South. 68% of the decedents died as inpatients in a hospital. The mean age at death for the time period was 38.2 years (sd = 15.6). There was no change in the mean age at death during the time period. Females were older than males at death (39.4 vs. 36.9, p < 0.0001). Those with HbSS were younger than those with a double heterozygous disorder (38 vs. 47, p < 0.02). Having SCD listed as the primary cause of death was associated with younger age at death (36.8 vs. 40.7, p < 0.0001). Decedents with at least some college education were older at death than those with high school educations or less (40.9 vs. 37.0 p < 0.0001). There were no regional differences in mean age at death. In a multivariate model of age at death with the predictors gender, region, education, and whether or not SCD was listed as the primary cause of death, being female and having some college education remained associated with older age at death, while having SCD listed as the primary cause of death remained associated with younger age at death. Septicemia, pulmonary heart disease, liver disease and renal failure were among the top contributing causes of death for adults, while septicemia, acute cerebrovascular disease and pneumonia were among the top contributing causes of death for kids. CONCLUSIONS: Persons dying from SCD during 1999 to 2004 experienced ages at death that are not improved over those reported by the CSSCD, suggesting the continued need for societal efforts aimed at improving the quality of care for SCD, especially among adults with the condition. Educational attainment is associated with age at death among the SCD population, though it is not possible from the cross-sectional nature of this data to determine the causal directionality of this association.

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