Gender Differences in Hospitalized Patients with Sickle Cell Disease (SCD): 1990–2002.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 2256-2256
Author(s):  
Carlton Haywood ◽  
Sophie Lanzkron
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Multivariate Analyses ◽  
Private Insurance ◽  
Adult Males ◽  
Inclusion Criteria ◽  
Cell Disease ◽  
Adult Females ◽  
The Us ◽  
Univariate Analyses

Abstract We show in a companion abstract that there has been no decrease in overall admissions for adults with sickle cell anemia (SCA) in the US for the period 1990–2002. The objective of this study was to further describe the characteristics of all adult and pediatric inpatients with sickle cell disease (SCD) in the US for this period. Methods: We examined data from the National Hospital Discharge Survey (NHDS) for the period 1990–2002. The NHDS is a nationally representative survey of non-federal short-stay hospitals in the U.S. which allows for the estimation of inpatient utilization data. Inclusion criteria for our analysis consisted of the following ICD-9 codes: 282.61 or 282.62 (classified as SS), and 282.60, 282.63, 282.64, and 282.69 (classified as Other/Not Specified) found in any one of seven possible patient diagnostic records. Gender and SCD genotype differences in mean length of stay (LOS) were assessed using univariate analyses, and multivariate analyses adjusting for insurance status, age, hospital bedsize, time, and region of the country. Analyses were conducted for the population in the aggregate, and stratified by adult/pediatric status (18+ years for adults, 0–17 for pediatric). Results: There were 13,221 records, which met our inclusion criteria resulting in an estimate of 1,079,523 SCD discharges over the 13 year period. (83,040 discharges/year) More than half of the adult discharges were for females, while less than half of the pediatric discharges were for females (55% vs. 44%, p < 0.0001). A greater percentage of adult than pediatric discharges were coded as SS (81% vs. 74%, p < 0.0001). Among adults, patients coded as SS were younger than patients with an Other/Not Specified SCD code (30.6 vs. 35, p < 0.001). The reverse was true for pediatric cases, where patients coded as SS were slightly older than patients coded as Other/Not Specified SCD (9.6 vs. 7.7, p < 0.001). In univariate analyses of LOS data, adult females with SS had longer mean LOS than adult males with SS (7.27 days vs. 6.29 days, p = 0.001). There were no differences in mean LOS for adult females and adult males with Other/Not Specified SCD codes (p = 0.160). Adult males with SS and adult males with Other/Not Specified SCD codes did not differ in their mean LOS (p = 0.850), while adult females with SS experienced significantly longer mean LOS than adult females with Other/Not Specified SCD codes (7.27 days vs. 5.45 days, p < 0.001). Among the pediatric cases, females with SS experienced longer LOS than females with Other/Not Specified SCD codes (4.57 days vs. 3.22 days, p < 0.001). Adult females continued to experience nearly a full day longer mean LOS than adult males in multivariate analyses (β = 0.83, p = 0.006). Among adults, 27% of men had medicare compared to 19% of the women and 17% of men compared to 25% of women had private insurance. (p < 0.0001) Conclusions: Our analysis of the characteristics of inpatients with SCD demonstrates that adult females with SCD account for a higher percentage of inpatient visits than adult males with SCD. Females with SS experience longer mean inpatient LOS than females without SS and males with or without SS, regardless of age. The reasons for these differences are unknown, but warrant further study. Of the hosptilized adults more women had private insurance than men. Whether this impacts on likelihood of admissions needs closer examination. A better understanding of patient characteristics will help guide interventions that will limit barriers to care for this patient population.

scholarly journals COVID Symptoms and COVID Anxiety in Sickle Cell Disease

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 16-17
Author(s):  
Wally R Smith ◽  
Benjamin Jaworowski ◽  
Shirley Johnson ◽  
Thokozeni Lipato ◽  
Daniel M Sop
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Research Funding ◽  
Telephone Survey ◽  
Cell Disease ◽  
Weighted Mean ◽  
The Us ◽  
Associated Symptoms ◽  
At Home

Background Even before the US upswing of the current COVID pandemic, the number of sickle cell disease (SCD) patients coming to hospitals and EDs appeared to fall drastically. This happened despite SCD patients having often been heavy utilizers of the ED and hospital for their iconic vaso-occlusive crises (VOC). Though ambulatory SCD clinics quick converted largely to telehealth in order to comply with stay-at-home orders designed to suppress person-to-person transmission, some SCD patients appeared to avoid care, delay care, or refuse doctors' invitations for care. Presumably patients did so out of COVID fears, but this has not been confirmed in the literature. Further, whether these patients had COVID symptoms but stayed at home has not been studied. As part of quality improvement (QI) to conduct COVID surveillance in an adult sickle cell program, we sought to explain and predict SCD health care utilization patterns we were observing, as well as to determine urgent physical and mental health needs of patients who appeared to be avoiding care. Methods Fifteen staff in the Adult Sickle Cell Medical Home at Virginia Commonwealth University, a large urban academic medical center, conducted a telephone survey ("wellness check"was used when we talked to patients) of all known adults with SCD over 19 days in 2020. A staff member confirmed the patient had SCD, asked permission to proceed, then asked about symptoms consistent with COVID-19. At the end of the telephone survey, respondents wer invited to complete an email survey of sickle cell and COVID-19 utilization attitudes (19-33 items, depending on the response pattern, either drawn from the National Health Interview Survey, from the Adult Sickle Cell Quality of Life Measurement quality of care survey, or drafted by the authors), the Sickle Cell Stress Survey-Adult (SCSS-A, a 10-item previously validated survey), and anxiety and depression (PHQ9 of the PRIME-MD). Results Of 622 adults approached by phone call, 353 responded to the following yes/no screening questions regarding the prior 14 days: fever over 100 F 0/353 (0.00%); cough 3/353(0.01%); difficulty breathing 0/353(0.00%); unexplained shortness of breath 2/353(0.01%); sore throat 2/353 (0.01%); unexplained muscle soreness 2/353(0.01%);contact with anyone who tested positive for COVID-19 2/353(0.01%); testing for COVID 19 6/353(0.02%). For QI purposes, we set a threshold of three or more COVID-associated symptoms or the presence of fever as criteria requiring intense telephone or in-person staff monitoring for the following week. Only three patients met criteria. A total of 219/353 had email surveys sent. Of 63 patients (28.8%) who returned email surveys by June 10, 2020, 35.9% had already managed a "pain attack" at home 4 or more times in the prior 12 months, and 45.5% of these said their bad ER experiences were very or somewhat important in that decision. In the prior 14 days, although 30/64 reported a crisis for at least one day, only 4/64 had visited the Emergency Department for pain. On a 0-10 scale, 21/61 patients endorsed "0" for worry that they would be COVID-infected by going for medical care (weighted mean 3.9), but 18/59 endorsed "10" for worry they were more at risk of COVID because of SCD (weighted mean 6.31), and 22/60 endorsed "10" for worry they would fare worse than others if COVID infected (weighted mean 6.97). Many patients forwent "needed" care (16/62) or delayed "needed" care by at least a day (36/61). Eleven patients met criteria for moderately severe to severe depression on the PHQ-9, and 28/63 somewhat or strongly agreed with the statement "death is always on the back of my mind" on the SCSS-A. Conclusions In adolescents and adults with SCD, many were already reticent to come to the ED for pain, but a significant portion reported delays or avoidance of needed care during the early stages of the US COVID pandemic, and few reported using the ED despite over half reporting at least one crisis day in 14. Patients nonetheless reported very few COVID-associated symptoms. Fears of COVID infection/susceptibility may limit visits for needed sickle cell care among adults. Acknowledgements: Mica Ferlis RN, FNP, Caitlin McManus, RN, FNP, Emily Sushko, RN, FNP, Justin West, RN, Kate Osborne, RN, Stefani Vaughan-Sams, Marla Brannon, BS, Nakeiya Williams, BS Disclosures Smith: GlycoMimetics, Inc.: Consultancy; Emmaeus Pharmaceuticals, Inc.: Consultancy; Novartis, Inc.: Consultancy, Other: Investigator, Research Funding; Global Blood Therapeutics, Inc.: Consultancy, Research Funding; Shire, Inc.: Other: Investigator, Research Funding; NHLBI: Research Funding; Patient-Centered Outcomes Research Institute: Other: Investigator, Research Funding; Health Resources and Services Administration: Other: Investigator, Research Funding; Incyte: Other: Investigator; Pfizer: Consultancy; Ironwood: Consultancy; Novo Nordisk: Consultancy; Imara: Research Funding; Shire: Research Funding.

scholarly journals Utility of Procalcitonin in Differentiating Acute Chest Syndrome from Vaso-Occlusive Crisis in Sickle Cell Disease

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 10-11
Author(s):  
Satish Maharaj ◽  
Simone Chang ◽  
Karan Seegobin ◽  
Marwan Shaikh ◽  
Kamila I. Cisak
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Complete Blood Count ◽  
Conflicts Of Interest ◽  
Statistical Significance ◽  
Acute Chest Syndrome ◽  
Adult Males ◽  
Cell Disease ◽  
Unequal Variances ◽  
Recorded Data

Background: Acute chest syndrome (ACS) frequently complicates sickle cell disease (SCD) and is a leading cause of hospitalization and mortality. Many factors have been implicated in ACS, including infections, thrombosis, fat and pulmonary emboli. However, a clear etiology is not defined in 50% of the cases and ACS is considered a clinical endpoint for different pathogenic processes (Vichinsky et al 2000). The non-specific nature of ACS makes diagnostic tests challenging, and there are no serum tests clinical used to aid diagnosis. Procalcitonin (PCT) is a prohormone of calcitonin and serum PCT rises within hours of an inflammatory stimulus. PCT has clinical utility as a marker of severe systemic inflammation, infection, and sepsis (Becker et al. 2008). Few studies have evaluated PCT as a biomarker for ACS in patients presenting with vaso-occlusive crises (VOC). Two studies have reported no difference in PCT (Biemond et al. 2018 and Stankovic et al 2011), while one study reported higher PCT between ACS and VOC (Patel et al 2014). Methods: We retrospectively reviewed 106 patients with SCD who presented to the emergency department with fever and painful crises during 2015-2019. The patients were divided into two categories based on discharge diagnoses - patients with VOC only (n=88) and patients with ACS (n=18). Inclusion criteria for both groups were patients with SCD, 17 years and older and PCT measurement on presentation. Exclusion criteria were defined as patients who had received empiric antibiotics prior to PCT testing. Data collected on presentation included genotype, age, gender, complete blood count, PCT, creatinine, total bilirubin and hydroxyurea use. Length of stay was recorded. Data was analyzed between the two groups using descriptive statistics and accounting for unequal variances, withp-value set at 0.05 for significance. Results: Demographics and clinical characteristics are summarized in Table 1 (Figure). The sample included primarily adult males (77%), with about two-thirds on hydroxyurea. Genotype HbSS (73.6%) was most prevalent followed by HbSC (22.6%) and HbSβ (3.8%). The ACS group had a higher percentage of HbSS, lower use of hydroxyurea and higher mean bilirubin. Mean PCT for the ACS group was 0.52 ng/mL (range, 0.05-2.04), compared to 0.31 ng/mL (range, 0.02-6.82) in the VOC group; withp=0.084. ROC analysis showed a PCT&gt;0.5ng/mL had 39% sensitivity and 85% specificity for ACS in this sample. Conclusion: In this sample, PCT on presentation was higher in those with ACS compared to VOC, but this difference did not achieve statistical significance. Further study in a larger population would be useful to evaluate this finding. Disclosures No relevant conflicts of interest to declare.

Low Fixed Dose Hydroxyurea for the Treatment of Adults with Sickle Cell Disease in Nigeria

Blood ◽  
2017 ◽  
Vol 130 (Suppl_1) ◽  
pp. 981-981
Author(s):  
Titilola S. Akingbola ◽  
Bamidele Tayo ◽  
Santosh L. Saraf ◽  
Binal N. Shah ◽  
Chinedu A Ezekekwu ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Low Dose ◽  
Adverse Outcome ◽  
Clinical Malaria ◽  
Fixed Dose ◽  
Cell Disease ◽  
Hemoglobin F ◽  
The Us

Abstract Background: The vast majority of births with sickle cell anemia occur in Africa 1 and early-life mortality, generally before age five years, is as high as 90% 2,3. Hydroxyurea was approved for sickle cell anemia by the US FDA in 1998 but is not commonly used in Africa due to fear of toxicity, lack of awareness and limited availability. Hemoglobin F is a protective factor that decreases severity of sickle cell anemia, and hydroxyurea treatment leads to an increase in hemoglobin F. In the US, hydroxyurea is typically initiated at a dose of 15 mg/kg followed by dose escalations of up to 35 mg/kg if tolerated with a goal of maximal tolerated dose and maximal response in hemoglobin F. Neutropenia and thrombocytopenia are the usual limitations to achieving maximal dose. In the landmark Multicenter Study of Hydroxyurea, the clinical response to hydroxyurea correlated strongly with a reduction in the neutrophil count as well as an increase in the fetal hemoglobin concentration as reflected in percentage of F cells. A striking decrease in pain crises was observed in the first three months of therapy, before dose escalation and before maximal increase in hemoglobin F levels 4. Furthermore, hydroxyurea in the range of 10-15.9 mg/kg/day was reportedly effective in decreasing the frequency of pain episodes in children and adolescents in Oman 5, and hydroxyurea 10 mg/kg/day decreased pain episodes in children and adults with sickle cell anemia in India 6. From these perspectives, we reasoned that a fixed dose of hydroxyurea 10 mg/kg/day is reasonable to investigate in the African setting where the safety in relationship to the resources and infectious exposures is not known. Methods: We assigned 48 sickle cell anemia patients to hydroxyurea 500 mg/day for 24 weeks to determine safety and efficacy; 28 had high-risk disease based on hemoglobin F&lt;8.6% and absence of alpha-thalassemia. We defined a clinically meaningful adverse outcome category as ≥10% of patients developing platelets &lt;50,000/uL, granulocytes &lt;500/uL, clinical malaria and/or active tuberculosis. Picking up refills every four weeks was the adherence metric. We analyzed the results on an intent-to-treat basis. Results: The median (interquartile range) age was 25 (22-27) years and the median hydroxyurea dose 9.8 (9.1-10.4) mg/kg per day. The patients complied with treatment for a median of 20 (16-24) weeks. Four (8.3%) developed a pre-specified adverse outcome: clinical malaria (N=2), thrombocytopenia in combination with malaria (N=1), pulmonary tuberculosis (N=1). During therapy the median hemoglobin increased by 9.0 g/L, mean corpuscular volume by 11.2 fL and body weight by 3.0 kg while median white blood cells declined by 2600 per uL and platelets by 127,000 per uL (P&lt;0.001). The median hemoglobin F increased from 4.1% (2.3-6.3%) at baseline (N=27) to 8.5% (6.3-12.9%) during therapy (N=24) (P&lt;0.001). Conclusion: Our results suggest that low, fixed-dose dose hydroxyurea for sickle cell anemia in Nigeria is associated with a low adverse outcome rate and with improvements in blood counts, hemoglobin F and body weight. The effects on vaso-occlusive episodes and on the risks of recrudescent tuberculosis and malaria-associated thrombocytopenia should be assessed in further studies. Acknowledgment: Supported by a grant from the Doris Duke Foundation. References 1. Williams TN, Obaro SK. Sickle cell disease and malaria morbidity: a tale with two tails. Trends Parasitol 2011;27:315-20. 2. Grosse SD, Odame I, Atrash HK, Amendah DD, Piel FB, Williams TN. Sickle cell disease in Africa: a neglected cause of early childhood mortality. Am J Prev Med 2011;41:S398-405. 3. Makani J, Cox SE, Soka D, et al. Mortality in sickle cell anemia in Africa: a prospective cohort study in Tanzania. PLoS One 2011;6:e14699. 4. Charache S, Barton FB, Moore RD, et al. Hydroxyurea and sickle cell anemia. Clinical utility of a myelosuppressive "switching" agent. The Multicenter Study of Hydroxyurea in Sickle Cell Anemia. Medicine (Baltimore) 1996;75:300-26. 5. Sharef SW, Al-Hajri M, Beshlawi I, et al. Optimizing Hydroxyurea use in children with sickle cell disease: low dose regimen is effective. Eur J Haematol 2013. 6. Patel DK, Mashon RS, Patel S, Das BS, Purohit P, Bishwal SC. Low dose hydroxyurea is effective in reducing the incidence of painful crisis and frequency of blood transfusion in sickle cell anemia patients from eastern India. Hemoglobin 2012;36:409-20. Disclosures Ezekekwu: American Society of Hematology: Other: The Visitor training program was sponsored by ASH. Hsu: AstraZeneca steering committee for HESTIA trial: Research Funding. Gordeuk: Emmaus Life Sciences: Consultancy.

scholarly journals End points for sickle cell disease clinical trials: renal and cardiopulmonary, cure, and low-resource settings

2019 ◽  
Vol 3 (23) ◽  
pp. 4002-4020 ◽  
Author(s):  
Ann T. Farrell ◽  
Julie Panepinto ◽  
Ankit A. Desai ◽  
Adetola A. Kassim ◽  
Jeffrey Lebensburger ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Patient Reported Outcome ◽  
Cell Disease ◽  
End Points ◽  
Low Resource Settings ◽  
Low Resource ◽  
Patient Reported ◽  
The Us ◽  
American Society

Abstract To address the global burden of sickle cell disease and the need for novel therapies, the American Society of Hematology partnered with the US Food and Drug Administration to engage the work of 7 panels of clinicians, investigators, and patients to develop consensus recommendations for clinical trial end points. The panels conducted their work through literature reviews, assessment of available evidence, and expert judgment focusing on end points related to patient-reported outcome, pain (non–patient-reported outcomes), the brain, end-organ considerations, biomarkers, measurement of cure, and low-resource settings. This article presents the findings and recommendations of the end-organ considerations, measurement of cure, and low-resource settings panels as well as relevant findings and recommendations from the biomarkers panel.

Use of Transfusion to Treat Acute Chest In Sickle Cell Disease:California Hospital Practice Patterns

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 253-253 ◽  
Author(s):  
Susan Claster ◽  
Ellen Iverson ◽  
Sheree M Schrager ◽  
Vanessa Guzman ◽  
Julie Wolfson
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Practice Patterns ◽  
Standard Of Care ◽  
Acute Chest Syndrome ◽  
Secondary Diagnosis ◽  
Inclusion Criteria ◽  
Cell Disease ◽  
Discharge Data ◽  
Public Data

Abstract Abstract 253 Introduction: Acute chest syndrome (ACS) is a leading cause of morbidity and mortality in patients with Sickle Cell Disease (SCD). Although the NIH recommends transfusion as standard of care therapy for this diagnosis, practice patterns vary. In 2000, the National Acute Chest Study group found that 72% of patients with ACS were transfused. This study, which was perfomed at academic medical centers with sickle cell treatment programs, used specific diagnostic inclusion criteria. As ACS may be difficult to distinguish from pneumonia, recognition and treatment of the diagnosis may vary by hospital and by presence or absence of a sickle cell specialist. With access to hospital discharge data from all California hospitals seeing SCD patients, we aimed to describe transfusion practices in ACS. Methods: This retrospective cohort study uses 2005–2008 public data from the California Office of Statewide Health Planning and Development (OSHPD). Data included all inpatient and emergency department (ED) discharges from all non-federal California hospitals. Cases were selected based on discharge from or admission via an ED and the presence of a sickle cell disease ICD-9 code in the primary or a secondary diagnosis (282.60-282.69, 282.41–282.42). Further inclusion criteria included presence of ACS (517.3) or respiratory diagnoses including pneumonia, pulmonary edema, acute respiratory distress syndrome, hypoxia and respiratory failure in any primary or secondary diagnosis. Frequency of transfusion, intubation and mortality were described and chi-square tests were performed to determine the statistical significance of differences between groups of patients treated with or without transfusion as well as between groups of adults and children. Results: Between 2005–2008, 4306 ED visits by Californians with SCD were associated with either ACS or a related respiratory diagnosis and only 42.5% of these cases were associated with transfusion. Limiting the diagnosis to ACS only, 1399 cases were identified, and still only 50.4% of these cases were associated with transfusion. Among patients with ACS or a related diagnosis, the majority were admitted to the hospital (90.9%); only 59.2% of intubated cases (n=238) were transfused; and of the 92 deaths during this period, only 46.7% were associated with transfusion. Patients who were 18 and over were transfused more often than young children (43.2% vs. 35.7%; 2(1)=12.37, p<0.001). Conclusions: Despite transfusion being the standard of care for treatment of ACS as well as other sickle–related complications, many ACS cases in California are not being treated in this manner. Surprisingly, the most severely ill patients, those who were either intubated or died during the study period, had a lower transfusion frequency than the cohort as a whole. Although some mild pediatric cases may not be transfused, a minority of the total cohort was under 18 years old. Recent studies imply that patients transitioning out of pediatric SCD programs have an increased risk of dying due to ACS and other serious SCD complications. Our study suggests that underutilization of transfusion may play a role in these poor adult outcomes in SCD. These findings prompt further investigation into the role of transition to adult care and appropriate medical homes in access to quality SCD care. Disclosures: No relevant conflicts of interest to declare.

Continuity of Care for Acute Visits In Sickle Cell Disease: Do Patients Go to More Than One Site?

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 735-735
Author(s):  
Julie A. Panepinto ◽  
Pamela L Owens ◽  
Andrew Mosso ◽  
Claudia A Steiner ◽  
David C Brousseau
Keyword(s):  
Emergency Department ◽  
Quality Of Care ◽  
Sickle Cell Disease ◽  
Acute Care ◽  
Sickle Cell ◽  
Private Insurance ◽  
Cell Disease ◽  
Public Insurance ◽  
Number Of Visits

Abstract Abstract 735 Sickle cell disease is characterized by frequent and recurrent vaso-occlusive events that often require multiple acute care visits to the emergency department (ED) or hospital. Multiple visits for sickle cell disease are more common among younger adults and those with public insurance than children and older adults and those with private insurance or who are uninsured.1 It is not known, however, whether these multiple visits are made to more than one site of care which could potentially reduce the patient's quality of care. The objective of this study was to examine the continuity of acute care visits for patients with sickle cell disease, as defined by having one site of care (versus multiple sites of care). We hypothesized that children with sickle cell disease and those sickle cell disease patients with private insurance would be more likely to use one ED or hospital for their acute care, while adults with sickle cell disease and those sickle cell disease patients with public insurance would be more likely to use multiple sites of care. We conducted a retrospective cohort study using 2005 and 2006 data from the Healthcare and Cost Utilization Project State Inpatient Databases and State Emergency Department Databases. Data from eight states (AZ, CA, FL, MA, MO, NY, SC, and TN) with an encrypted patient identifier were used to examine all acute care visits for sickle cell-related diagnoses in children and adults with sickle cell disease. Our primary outcome was the proportion of patients with all acute care visits to one site. We derived a logistic regression model to examine the association between age and primary expected payer and likelihood of having a single site of care, adjusting for rurality of the patient's residence, gender, number of visits and state of residence. A total of 21,118 patients with sickle cell disease had one or more sickle cell disease -related acute care visits to the ED or hospital. There were 13,533 patients who made two or more visits. Approximately 66% of these patients (n=8,895) had public insurance as the primary expected payer. Of the 5,030 children (ages 1–17 years) with multiple visits, 77.3% went to the same site for their acute care over the two year time period. This is in contrast to the adults (n=8,503) for whom only 51.3% received all acute care at the same site. The proportion of patients who went to one site of care decreased as the number of visits made increased for both children and adults. In multivariable analyses, adolescents (10- 17 years olds) were more likely than young adults (18-30 years old) to go to one site for all acute care (adjusted odds ratio (AOR) 3.78, 95% confidence interval (CI) 3.23–4.43). Analyzing the likelihood of going to one site for all acute care by primary expected payer, uninsured patients were less likely to have one site of care compared to patients with private insurance as the expected payer, even after controlling for the number of visits. This association was especially pronounced among patients with an increased number of visits during the two year study period. When examining adults who made four acute care visits, 41.2% of those without insurance went to one site for care compared to 56.4% with private insurance and 56.5% with public insurance. In children with 4 acute care visits, 54.5% of those without insurance went to one site compared to 78.7% with private insurance and 75.2% of those with public insurance. In multivariable analysis, having public insurance and being uninsured were associated with decreased likelihoods of going to one site for all acute care (AOR 0.85, 95% CI 0.77–0.93 and AOR 0.64, 95% CI 0.55–0.74 respectively) compared to having private insurance. Young adults and patients who are uninsured or who have public insurance are more likely to go to multiple sites for their acute care compared with children and those with private insurance. Although the long-term effects of having multiple sites of acute care are unknown, it may indicate a lack of a medical home and may contribute to lower quality of care. 1. Brousseau DC, Owens PL, Mosso AL, Panepinto JA, Steiner CA. Acute Care Utilization and Rehospitalizations for Sickle Cell Disease. JAMA 2010;303(13):1288-1294. Disclosures: No relevant conflicts of interest to declare.

Sensorineural hearing loss in sickle cell anaemia – a United Kingdom study

1993 ◽  
Vol 107 (9) ◽  
pp. 790-794 ◽  
Author(s):  
S. O. Ajulo ◽  
A. I. Osiname ◽  
H. M. Myatt
Keyword(s):  
Hearing Loss ◽  
Sickle Cell Disease ◽  
Sensorineural Hearing Loss ◽  
Sickle Cell ◽  
Sensorineural Hearing ◽  
Control Group ◽  
Cell Disease ◽  
The Us ◽  
The Uk ◽  
The Tropics

AbstractSensorineural hearing loss (SNHL) has been a well-documented complication of sickle cell disease in the literature from West Africa, West Indies, United States of America and the Middle East. We present a study of 52 patients with homozygous sickle cell disease and 36 control patients with haemoglobin genotype AA, matched for age and sex. Seven patients with sickle cell disease (13.5 per cent) were found to have sensorineural hearing loss i.e.>20 dB at two or more frequencies, while all the patients in the control group had normal hearing (p<0.05).Our study shows the incidence of SNHL in the UK to be similar to that reported in the US A and much lower than that found in malaria endemic areas of the tropics.We highlight the factors which we consider responsible for these differences and suggest that the crucial period in the development of SNHL in sickle cell disease may be intra-uterine or during the first few years of life. All sickle cell patients should be encouraged to have regular hearing assessment.

Utilization and Costs of Deferoxamine in Patients with Thalassemia, Sickle-Cell Disease, or Myelodysplastic Syndrome Receiving Frequent Transfusions.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5583-5583
Author(s):  
Thomas E. Delea ◽  
May Hagiwara ◽  
Simu K. Thomas ◽  
Jean-Francois Baladi ◽  
Pradyumna D. Phatak ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Drug Acquisition ◽  
Iron Chelator ◽  
Cell Disease ◽  
Total Cost ◽  
The Us ◽  
Health Insurance Claims ◽  
Using Data

Abstract Background. Patients with thalassemia, sickle-cell disease (SCD), and myelodysplastic syndromes (MDS) receiving frequent transfusions require chelation therapy to prevent complications of iron overload. Deferoxamine (DFO) is an effective iron chelator that has been shown to reduce the morbidity and mortality associated with transfusional hemosiderosis. Data on the utilization and costs of DFO treatment are limited however. The objective of this study was to document the utilization and costs of DFO therapy in patients with transfusion-dependent anemias seen in typical clinical practice. Methods. Retrospective, observational study using data from large health insurance claims database spanning 1/97–12/04 (“study period”) and representing approximately 40 million members enrolled in &gt;70 health plans across the US. Study subjects included members meeting the following criteria: ≥1 claims with diagnosis of thalassemia (282.4x), SCD (282.6x ), or MDS (ICD-9-CM 238.7x); ≥8 claims (on different days) for a transfusion of whole blood or red cells; ≥2 claims (on different days) for DFO. Follow-up was defined as the period from the date of first DFO claim (“index date”) to end of study period, disenrollment, or 15 days after last claim for DFO, whichever occurred first. Outcomes included the number of claims for DFO and grams of DFO dispensed and the costs of DFO therapy, including costs of drug acquisition and administration. Outcomes were analyzed by qualifying diagnosis, numbers of transfusions received, and grams of DFO dispensed. Results. We identified 155 subjects who met all inclusion criteria, including 35 with thalassemia, 68 with SCD, and 52 with MDS. On average, patients received one transfusion every 3.4 weeks of follow-up. Mean DFO grams dispensed were 306 per year. MDS patients received the most transfusions but the least DFO. Only 38% of MDS patients received ≥3 g of DFO per week (≥156 g per year). Mean total DFO costs were $18,025 annually ($10,217 for drug and $7,808 for administration). Controlling for other factors, utilization of DFO was not associated with number of transfusions received; administration costs were only weakly associated with amount of DFO received. Thalassemia SCD MDS All Values are Mean±SD N 35 68 52 155 Follow-up, days 612 ± 481 420 ± 403 274 ± 336 414 ± 418 Age, years 19 ± 12 17 ± 11 63 ± 11 33 ± 24 Transfusions per year 15 ± 7 12 ± 4 24 ± 13 16 ± 10 DFO claims per year 29 ± 34 41 ± 46 30 ± 20 34 ± 37 DFO grams per year 311 ± 233 343 ± 243 223 ± 234 306 ± 241 Cost DFO acquisition, $ per year 10,287 ± 8,264 11,625 ± 8,339 7,293 ± 7,543 10,217 ± 8,207 Cost DFO administration, $ per year 7,674 ± 11,503 9,109 ± 8,177 5,403 ± 5,649 7808 ± 8,438 Total cost of DFO, $ per year 17,961 ± 17,047 20,734 ± 12,114 12,696 ± 10,886 18,025 ± 13,348 Conclusion: In this population of frequently transfused patients (mean 16 transfusions per year), utilization of DFO was low (mean &lt;1 gram per day) suggesting inadequate chelation. Costs of DFO administration were high, representing approximately 43% of the total cost of chelation.

scholarly journals Use of large scale EHR data to evaluate A1c utilization among sickle cell disease patients

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Shivani Sivasankar ◽  
An-Lin Cheng ◽  
Ira M. Lubin ◽  
Kamani Lankachandra ◽  
Mark A. Hoffman
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Large Scale ◽  
Hemoglobin A1c ◽  
Glycated Hemoglobin ◽  
The United States ◽  
Cell Disease ◽  
Healthcare Facilities ◽  
The Us ◽  
Lab Test

Abstract Background The glycated hemoglobin (A1c) test is not recommended for sickle cell disease (SCD) patients. We examine ordering patterns of diabetes-related tests for SCD patients to explore misutilization of tests among this underserved population. Methods We used de-identified electronic health record (EHR) data in the Cerner Health Facts™ (HF) data warehouse to evaluate the frequency of A1c and fructosamine tests during 2010 to 2016, for 37,151 SCD patients from 393 healthcare facilities across the United States. After excluding facilities with no A1c data, we defined three groups of facilities based on the prevalence of SCD patients with A1c test(s): adherent facilities (no SCD patients with A1c test(s)), minor non-adherent facilities, major non-adherent facilities. Results We determined that 11% of SCD patients (3927 patients) treated at 393 facilities in the US received orders for at least one A1c test. Of the 3927 SCD patients with an A1c test, only 89 patients (2.3%) received an order for a fructosamine test. At the minor non-adherent facilities, 5% of the SCD patients received an A1c test while 58% of the SCD patients at the least adherent facilities had at least one A1c test. Overall, the percent of A1c tests ordered for SCD patients between 2010 and 2016 remained similar. Conclusions Inappropriate A1c test orders among a sickle cell population is a significant quality gap. Interventions to advance adoption of professional recommendations that advocate for alternate tests, such as fructosamine, can guide clinicians in test selection to reduce this quality gap are discussed. The informatics strategy used in this work can inform other largescale analyses of lab test utilization using de-identified EHR data.

Sign in / Sign up

Export Citation Format

Share Document