Economic Burden of Sickle Cell Disease among Children and Adults.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 958-958 ◽  
Author(s):  
Abraham G. Hartzema ◽  
Teresa L. Kauf ◽  
Thomas D. Coates ◽  
Liu Huazhi ◽  
Nakita Mody-Patel

Abstract Background: The economic burden of sickle cell disease (SCD) has not been determined. Cost-of-care estimates improve health care planning, inform research priorities, and contribute to an understanding of the cost-effectiveness of new SCD treatments. The objectives of this study were examine resource utilization in the Florida Medicaid population; to estimate the cost of medical care for SCD patients; and to examine the proportion of medical care costs attributable to SCD versus other morbidities. Methods: Pediatric (0 – 19 years; y) and adult (> 20 y) patients with a diagnosis of SCD between 1/2001–12/2005 were identified from Florida Medicaid claims. Patients with at least 1 inpatient or 2 outpatient SCD claims at least 30 days apart and ≥6 Medicaid-eligible months, not necessarily consecutive, were included in the analysis. Medicaid administrative data consist of medical and pharmacy utilization and reimbursement claims for medically-needy and low-income recipients. Resource utilization and costs were estimated using paid claims for hospitalizations, emergency room visits, outpatient visits, pharmacy claims, and other care (blood transfusions, hydroxyurea, iron chelation, serum ferritin tests). Claims containing a primary or secondary ICD-9 code indicating SCD were considered “SCD-related”; all other claims were classified as “non-SCD-related”. Individuals aged >65 y and/or dually eligible for Medicare or other health insurance were excluded. Annualized costs were estimated by multiplying mean cost per patient-month by 12. All costs were inflated to 2005 US$ using the medical care component of the Consumer Price Index. Results: A total of 4,294 individuals met study inclusion criteria. Mean age at first claim was 14.5 y (range: 0–64 y; SD 13.2); 55.8% were female; 79.1% African-American. Mean SCD-related costs for pediatric (0–9 y) and adolescent (10–19 y) patients were estimated at $5,292 and $11,508 per year, respectively. Overall, 66% of total medical costs were related to SCD. The distribution of SCD-related costs was bimodal, with the youngest (0–9 y) and oldest (50–64 y) having the lowest proportion of SCD-related costs (53.7% and 35.3%, respectively). Non-SCD-related costs increased with age, ranging from $4,548 annually for ages 0–9 to $16,656 for ages 50–64. While SCD accounted for the largest proportion of total medical costs for patients 10–19 y (77.27%; $11,508), absolute SCD costs were highest for patients 30–39 y (67.1%; $21,792). Cost distributions were similar for males and females. Hospitalizations accounted for 80.5% of all SCD-related costs; emergency room visits, 3.2%; office visits, 0.9%; outpatient drugs, 3.6%; other medical care, 11.7%. Total discounted medical costs (3%) for a SCD patient from birth through age 45 were estimated at $448,460, with 67% of costs related to SCD. Conclusion: Our results suggest that the burden of SCD is substantial. Moreover, non-SCD-related medical costs for SCD patients are considerably higher than expenditures reported for the general US population. While our results reflect the payer’s perspective, the clinical, humanistic, and economic burden of care on individual patients also is likely high. Interventions designed to prevent SCD complications and keep patients out of the hospital may reduce the significant economic burden of the disease.

2015 ◽  
Vol 31 (2) ◽  
pp. 265-275 ◽  
Author(s):  
Silvia Maria de Macedo Barbosa ◽  
Sylvia Costa Lima Farhat ◽  
Lourdes Conceição Martins ◽  
Luiz Alberto Amador Pereira ◽  
Paulo Hilário Nascimento Saldiva ◽  
...  

The hallmarks of sickle cell disease are anemia and vasculopathy. The aim of this study was to assess the association between air pollution and children's emergency room visits of sickle cell patients. We adopted a case-crossover design. Daily counts of children's and adolescents' sickle cell disease emergency room visits from the pediatric emergency unit in São Paulo, Brazil, were evaluated from September 1999 to December 2004, matching by temperature, humidity and controlling for day of the week. Interquartile range increases of the four-day moving averages of PM10, NO2, SO2, CO, and O3 were associated with increases of 18.9% (95%CI: 11.2-26.5), 19% (95%CI: 8.3-29.6), 14.4% (95%CI: 6.5-22.4), 16,5% (95%CI: 8.9-24.0), and 9.8% (95%CI: 1.1-18.6) in total sickle cell emergency room visits, respectively. When the analyses were stratified by pain, PM10 was found to be 40.3% higher than in sickle cell patients without pain symptoms. Exposure to air pollution can affect the cardiovascular health of children and may promote a significant health burden in a sensitive group.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 2177-2177
Author(s):  
Francis Vekeman ◽  
Medha Sasane ◽  
Wendy Y Cheng ◽  
Agnihotram V Ramanakumar ◽  
Jonathan Fortier ◽  
...  

Abstract Introduction: While adherence to iron chelation therapy (ICT) is critical for successful iron overload (IO) treatment among patients with sickle cell disease (SCD), published data indicate it is often suboptimal. Deferoxamine (DFO) and Deferasirox (DFX) are ICTs indicated for the treatment of chronic IO in patients with SCD. Lack of patient adherence may impact patient outcomes and increase cost of care. This study evaluated the economic burden of ICT non-adherence in patients with SCD from the state Medicaid program’s perspective. Methods: Patients with SCD were identified from Florida, Iowa, Kansas, Mississippi, Missouri, and New Jersey (1997-2013) state Medicaid programs. Patients were required to have ≥1 ICD-9 diagnosis code for SCD (282.6), ≥1 prescription for DFO or DFX, and ≥6 months of continuous enrollment prior to the 1st DFO/DFX prescription (index date), which was defined as the baseline period. Adherence was estimated using the medication possession ratio (MPR), defined as the sum of the days of medication supply divided by the number of days between 1st and last prescription fill plus the days of supply of the last fill; a threshold of ≥0.80 was used to define optimal adherence. All-cause and SCD-specific resource utilization per-patient-per-month (PPPM) was assessed using cumulative rates, accounting for all visits observed, and compared between adherent and non-adherent patients using cumulative rate ratios (CRR). All-cause and SCD-specific healthcare costs were computed using mean cost PPPM. Regression models adjusting for baseline characteristics were used to assess resource utilization and cost differences between adherent and non-adherent patients. Results: A total of 846 eligible patients with SCD were included with 77 in DFO-only, 686 in DFX-only), and 83 in DFO/DFX switch cohort. Mean (SD) MPR was 0.68 (0.27) for DFO-only patients and 0.75 (0.26) for DFX-only patients (p<0.05). Among all users of ICT, 409 (48.3%) were considered adherent. Adherent patients were slightly younger (19 vs. 21 years, p=0.003) than non-adherent patients. Rates of transfusions were comparable between the two groups (mean [SD] transfusions PPPM, adherent: 0.41 [0.47]; non-adherent: 0.40 [0.54], p=0.456) at baseline. The adjusted rate of all-cause IP visits PPPM was lower in adherent versus non-adherent patients (CRR=0.87 [95% CI: 0.83, 0.91]; p<0.001). The adjusted rates of all-cause outpatient (OP) visits (1.10 [1.08, 1.13], p<0.001) and ER visits (1.06 [1.01, 1.10], p=0.010) PPPM of adherent patients were higher in adherent patients than those in non-adherent patients. A similar trend was observed in SCD-specific resource utilization except for rates of ER visits, which were similar between cohorts. From cost perspective, total all-cause and SCD-specific costs were lower in adherent versus non-adherent patients primarily due to lower IP costs (Table 1). SCD-specific ER and OP costs were similar in both cohorts. All-cause pharmacy costs were higher in adherent versus non-adherent patients. Conclusion: Published studies have reported low adherence to ICT, and a similar trend was found in this study. Adherent patients were observed to have less frequent hospitalizations and lower overall and SCD-specific IP costs compared to non-adherent patients. It should be noted that the rate of OP visits was higher in the adherent patients compared to non-adherent patients suggesting that adherent patients may be more closely monitored potentially resulting in better overall patient management and fewer hospitalizations. Additional analyses are needed to explore differences between adherent and non-adherent patients. Table 1 Costs PPPM Adherent patients (N=409) [A] Non-adherent patients (N=437) [B] Adjusted cost difference[A] – [B] P -value All-cause, mean [SD] $4,766 [$4,388] $5,304 [$4,725] -$724 0.072 Inpatient $1,911 [$3,647] $2,996 [$4,439] -$947 0.016 Emergency room $27 [$87] $40 [$88] -$203 0.104 Outpatient $580 [$697] $485 [$617] $49 0.500 Pharmacy $2,248 [$1,949] $1,783 [$1,449] $432 0.004 Pharmacy without ICT $215 [$482] $274 [$544] -$50 0.192 SCD-specific, mean [SD] $2,237 [$3,679] $3,116 [$4,301] -$952 0.0160 Inpatient $1,776 [$3,546] $2,782 [$4,268] -$855 0.0160 Emergency room $18 [$63] $28 [$69] -$199 0.1200 Outpatient $443 [$658] $306 [$548] $105 0.1120 Disclosures Vekeman: Novartis Pharmaceuticals: Research Funding. Sasane:Novartis Pharmaceuticals: Employment. Cheng:Novartis Pharmaceuticals: Research Funding. Ramanakumar:Novartis Pharmaceuticals: Research Funding. Fortier:Novartis Pharmaceuticals: Research Funding. Duh:Novartis Pharmaceuticals: Research Funding. Paley:Novartis Pharma: Employment. Adams-Graves:Novartis Pharmaceuticals: Consultancy, Honoraria, Speakers Bureau.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4861-4861
Author(s):  
Samip Master ◽  
Abhishek Patel ◽  
Richard Preston Mansour

Background: Acute pain crises from vaso occlusive episodes are major cause of morbidity, hospitalization and emergency room visits for patients with sickle cell disease. Major causes of acute painful crises have been attributed to dehydration, infections and fever. Weather change has also been attributed as one of the major cause of acute pain crises by physicians and patients. Data on effects of environmental changes on acute pain crises in patients with sickle cell disease is mixed. Here we report our investigation to see if number of emergency room visits for adult patients with SCD were associated with environmental changes. Method: At our hematology clinic we take care of around 300 adult patients with SCD and we have on average around 11,000 emergency room visit per years from these patients. Electronically from EHR (Electronic health Record), we pulled out data on number of emergency room visit every day for adult patient with SCD in year 2015.We also gathered data on daily environmental changes from a national weather website for the year 2015 and collected data on daily temperature changes, humidity and precipitation/rain in our area. We did analysis to see if there was any correlation between the weather changes and emergency room visit. Results: As shown in graphs below, there was no correlation between the number of ER visits and weather changes in adult patient with SCD. Conclusion: Our analysis showed that there was no correlation between number of ER visit for adult patient with SCD and weather changes like -temperature, humidity and precipitation/rain. ER Visits and Precipitation ER visits, temperature and humidity Figure 1 Figure 1. Figure 2 Figure 2. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5582-5582
Author(s):  
Jigar Rajpura ◽  
Joseph Thomas

Abstract Introduction Clinical manifestations of Sickle Cell Disease (SCD) range from frequent fatigue and anemia to severe complications involving multiple organs. We estimated all cause health care resource utilization in individuals with SCD as compared to individuals without SCD. Methods Data from the Truven multi-state Medicaid claims database were used for all analyses. Sample inclusion criteria was being enrolled in covered health plan from January 1, 2012 through December 31, 2012. A cohort of individuals with SCD was identified based on having at least one inpatient or two or more outpatient claims with ICD-9-CM codes for sickle-cell thalassemia without crisis (282.41), sickle-cell thalassemia with crisis (282.42), Sickle-cell disease, unspecified (282.60), Sickle-cell-S disease without crisis (282.61), Sickle-Cell-S disease with crisis (282.62), Sickle-cell-C disease without crisis (282.63), Sickle-cell-C disease with crisis (282.64), other sickle-cell disease without crisis (282.68), or other sickle-cell disease with crisis (282.69). From the continuously enrolled individuals, a cohort of individuals without SCD was identified by matching individuals with SCD one-to-one on age, gender, and ethnicity. Means and 95 percent confidence intervals were calculated for, number of hospitalizations, hospital days, long term care visits, long term care days, emergency room visits, and outpatient visits. Wilcoxon Mann-Whitney test was employed to assess differences in health care resource utilizations in individuals with and without SCD. Results Among 5,136,748 individuals covered in the study period, 8,652 individuals with SCD were identified and matched to 8,652 individuals without SCD. Majorities of the study sample were black (74%), female (54%), and under 24 years old (64%). Mean annual hospitalizations among individuals with SCD were 1.39 (95 percent C.I.: 1.3 to 1.4, <0.001) as compared to 0.10 (95 percent C.I.: 0.09 to 0.1, p<0.001) hospitalizations in persons without SCD. Mean annual outpatient visits in individuals with SCD were 24.7 (95 percent C.I.: 23.9 to 25.5, p<0.001) as compared to 14.18 (95 percent C.I.: 13.4 to 14.9, p<0.001) outpatient visits in persons without SCD, and, mean annual emergency room visits in individuals with SCD were 3.71 (95 percent C.I.: 3.5 to 3.9, p<0.001) as compared to 0.89 (95 percent C.I.: 0.8 to 0.9, p<0.001) emergency room visits in persons without SCD. Conclusions SCD was associated with higher annual hospitalizations, hospital days, outpatient visits and emergency room visits as compared to individuals without SCD. Disclosures Rajpura: Pfizer Inc.: Research Funding. Thomas:Pfizer Inc.: Research Funding.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 1893-1893
Author(s):  
Sara Weiss ◽  
Sejean Yang ◽  
Shu Zhang ◽  
Mandy David ◽  
Sophie M. Lanzkron ◽  
...  

Abstract Introduction Individuals with Sickle Cell Disease (SCD) require regular, and specialized treatment to manage their health. The COVID-19 pandemic disrupted in person medical visits for all individuals, with a rapid transition to telemedicine to provide medical care. Emerging data shows that the use of telemedicine may provide easier access to care and remove barriers to clinic attendance and improve access to appropriate medical care. Objective The purpose of this study was to use qualitative methods to understand the patients' experiences with telemedicine, identify patient preferences for type of appointment, and possible suggestions to improve telemedicine care. Methods Patients from the Johns Hopkins Sickle Cell Center for Adults who had at least one telemedicine visit were invited to participate in a semi structured interview via zoom meeting or telephone. The interview asked participants about their satisfaction with telemedicine care, barriers to telemedicine, benefits and risks of telemedicine and possible telemedicine improvements. Interviews were recorded, transcribed and coded by two independent raters using thematic analyses to understand the experiences of telemedicine during the COVID-19 pandemic. Results Overall, 30 adults with SCD who had at least one telemedicine visit were invited to participate and completed their interview (mean age 41 years ± xx, 67% female, 93% Black/African American, 3% Multi-Race, 3% Other). "...I can't ignore the convenience of not having to worry about transportation ... that there's nothing to stop me from getting there." During a SCD pain crisis it can it challenging to move and receive treatment as one participant reported "Maybe sometimes I might have pain...then moving around makes it difficult. So, getting in the car and finding somebody to drive you to a hospital or to whatever clinic would be difficult". Being able to access specialized SCD care even while in pain is important. Having the option of either having telemedicine or in person visits was important to SCD patients "I could treat my crisis here at home. I don't have to go to the emergency room for it. So, if I can see my doctor in the tele-visit appointment and it's going to be constantly every day ... And when it's getting worse, then I could go to the emergency room more if needed. If it's not needed, I don't even need to go". Another emerging theme amongst participants was despite the benefits from telemedicine, they also wanted to continue having in-person visits when they needed. SCD participants felt due to their SCD they still needed to see their doctor in person but it did not have to be for every visit "Well, I think telemedicine, for me, can be used in a setting where there's no such an emergency. Like if I'm having a routine exam, I don't mind having the telemedicine. But if ... I'm not feeling well ... I don't want to be having a telemedicine". SCD participants felt they needed a physical exam periodically. "The only thing I didn't like about it was if I'm having some discomfort or some pain... there was no way for the physician to physically examine me". Along with the lack of physical exam, there were concerns about the lack of vital signs "... the drawbacks would be the lack of the vitals being taken or there's not the personal touch and stuff". Conclusion The COVID-19 pandemic has presented many obstacles for patients to receive care. People living with SCD found telemedicine to be a positive tool to receive treatment. Patients reported the desire to continue with telemedicine even after the COVID -19 pandemic. Telemedicine allows for more accessibility for a group of individuals who already have numerous barriers to treatment. Future research can seek to identify the impact that telemedicine has on no-show rates, health care utilization, and the impact telemedicine has on patient reported quality of life. Disclosures Lanzkron: Teva: Current holder of individual stocks in a privately-held company; Shire: Research Funding; GBT: Research Funding; CSL Behring: Research Funding; Novo Nordisk: Consultancy; Bluebird Bio: Consultancy; Pfizer: Current holder of individual stocks in a privately-held company; Imara: Research Funding; Novartis: Research Funding.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1723-1723
Author(s):  
Jennifer Alison Busse ◽  
Kranthi Nandan Seelaboyina ◽  
Grace Malonga ◽  
Thomas Moulton

Abstract Introduction Promotion of chronic illness self-management is crucial to improve outcomes of children with sickle cell disease (SCD). Improvements in patient outcomes and quality of life as well as enhancement in health literacy and patient agency are also imperative. Through an event diary developed by the Bronx Lebanon Hospital Sickle Cell Program, our pediatric SCD patients are better able to track significant health events and health care appointments. This study seeks to validate the effectiveness of the patient event diary as a tool for improvement in self management, allowing for improvement in medication and clinic visit compliance, as well as in a reduction in number of emergency room visits. Additionally, the education section at the beginning of the diary has become an integral teaching tool and reference for patients and their families. It is our hypothesis that the event diary as an educational tool, a self management tool, and a tool for providers, will lead to better management of chronic illness and improved patient outcomes. Methods We utilized laboratory and demographic data from the Bronx Lebanon Hospital Center patient electronic medical record between July 2011 and July 2013. Our sample included a population of 50 sickle cell disease patients aged 0 to 21, median age being 7 years. Sickle cell disease types included SS (64%), SC (32%), Sβ+ thalassemia and Sβ0thalassemia (4%). We used a paired, two tailed t-test to assess patients’ medication compliance on vitamin D (22 patients) and hydroxyurea (14 patients) through 25-hydroxyvitamin D (25-OHD) and MCV (mean corpuscular volume) levels, respectively. Increased MCV was used as a proxy to indicate improved medication compliance in patients on hydroxyurea. We compared patients on hydroxyurea and vitamin D six months before receiving the event diary and six months after. Additionally, we compared compliance with hematology clinic visits one year before and one year after receiving the event diary. We also compared the number emergency room visits during the year before and the year after patients received the event diary. Results 25-OHD data from six months after receiving the event diary showed significantly improved compliance with vitamin D treatment (M = 47, SD = 20) as compared to six months before receiving the event diary (M = 26, SD = 15), t(20) = 2.1, p < 0.05. Additionally, there was a statistically significant improvement in MCV levels six months after receiving the event diary (M=101.6, SD=9.6), as opposed to six months before receiving it (M=97.7, SD=5.6), t(12)=2.2, p<0.05. When looking at patient hematology clinic appointment compliance, there was an improvement, however not statistically significant, in compliance with appointments between one year before (M=33.3%, SD=41.7%) and one year after patients received the event diary (M= 27.2%, SD=30.3%), t(17)=2.1, p=0.2. Emergency department visits did decrease, however not significantly, one year before receiving the event diary (M=1.8, SD=2.5) to one year after receiving the event diary (M= 1.3, SD=1.9), t(47)=2.0, p=0.17. Conclusions These data suggest the patient event diary has significantly improved patient self-management. We believe this effect is due to enhanced understanding of disease processes and increased confidence and skills in self-management of the disease. There is improved compliance with hydroxyurea and vitamin D, as well as a trend toward improvement in clinic visit compliance and decreased emergency room visits. The patient event diary is crucial to improved patient outcomes in the pediatric SCD population, and may be useful for other pediatric populations with chronic illness. It is a significant part of our current practice and our ability to teach and empower patients and their families to manage sickle cell disease while at home. Additionally, this tool allows for better management of patients by providers. The event diary helps to elucidate difficulties in patient self-management and gives better insight into complications patients are experiencing at home. In the future, we hope to see that with improved patient knowledge of sickle cell disease and self-management facilitated by use of the event diary, there will be significantly fewer emergency room visits, fewer hospital admissions, and most important, quality of life. Disclosures: Moulton: HRSA: Research Funding; New York State Grant: Research Funding.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4988-4988
Author(s):  
Omar Abughanimeh ◽  
Steven Ebers ◽  
Mahammed Khan Suheb ◽  
Julie Eclov ◽  
Robin High ◽  
...  

Abstract Background: Red blood cell exchange (RBCX) is an effective therapy in treatment of acute and chronic complications of sickle cell disease (SCD). It involves exchanging patient's red blood cells (RBCs) with donor RBCs to significantly lower hemoglobin S concentration without subjecting the patient to the risk of iron overload. The University of Nebraska Medical Center (UNMC) established a chronic RBCX program in November 2015, which cared for patients with multiple hemoglobinopathies. In this study, we aim to evaluate some of the outcomes of patients with SCD who joined the program. Methods: This is a retrospective study based on review of medical records of patients with sickle cell disease. We reviewed the health records of patients with SCD who were enrolled in the chronic RBCX program between 11/2015-8/2020 at UNMC. We included patients with SCD, regardless of age, who underwent RBCX in the outpatient setting during the study period. Data were collected to assess if RBCX influenced the frequency of SCD crisis, emergency room visits, hospitalizations, and other sickle cell-related complications. Results: A total of 404 sessions of exchange transfusions were performed between November 2015 and August 2020 for 21 patients with SCD. The study included 9 adults (age ≥ 18 years) and 12 children with a median age of 12 years (2-31 years). During the study period, 3 adults left the program due to relocation out of state, patient's preference, or physician's decision. Table 1 summarizes the population demographic. The most common indication for enrollment in the RBCX program was recurrent sickle cell crisis (Figure 1). The mean number of emergency room visits before enrollment in the RBCX program was 22.5 visits (2-62 visits), which reduced after enrollment to 10.4 visits (0-65 visits), with a difference in mean of 12.1 visits (P=0.0021). The mean number of hospital admissions before enrollment in the RBCX program was 13.2 admissions(0-54 admissions), which also reduced to 6.7 admissions (0-50 admissions), with a significant difference in the means equal to 6. 6 admissions (P=0.0013) (Figure 2). Thirteen patients had a baseline ferritin &gt; 500 ng/ml at enrollment; all of them had a decrease in their baseline ferritin during the study, with 4 of them achieving a new baseline &lt; 500 ng/ml. Six patients had pre-existing antibodies at enrollment due to prior alloimmunization; however, no new alloantibodies were noticed after enrollment. The patients without preexisting antibodies were transfused with Rh and Kell matched blood. The patients with pre-existing antibodies were transfused with phenotypically matched blood. Three patients became pregnant during the study period, and their pregnancies were uncomplicated except for one patient with preeclampsia resulting in early delivery. There was no reportable death, acute chest syndrome, or stroke among the patients during the study period. Conclusion Outpatient chronic RBCX demonstrated safety and feasibility in both adults and children. It also showed promising outcomes in terms of reduction of sickle cell complications, number of emergency room visits and hospitalizations. These results can provide the basis for evaluating RBCX in a prospective study to better understand changes in quality of life and clinical outcomes of patients with SCD and limited therapeutic options. Figure 1 Figure 1. Disclosures Gundabolu: Pfizer: Research Funding; Samus Therapeutics: Research Funding; BioMarin Pharmaceuticals: Consultancy; Bristol-Myers Squibb Company: Consultancy; Blueprint Medicines: Consultancy.


2018 ◽  
Vol 21 ◽  
pp. S108 ◽  
Author(s):  
J Huo ◽  
H Xiao ◽  
M Garg ◽  
C Shah ◽  
DJ Wilkie ◽  
...  

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