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  • 1
    Online Resource
    Online Resource
    American Society of Hematology ; 2019
    In:  Blood Vol. 134, No. Supplement_1 ( 2019-11-13), p. 4841-4841
    In: Blood, American Society of Hematology, Vol. 134, No. Supplement_1 ( 2019-11-13), p. 4841-4841
    Abstract: Introduction Sickle cell disease (SCD) is a hereditary red blood cell disorder characterized by severe anemia, acute and painful vaso-occlusive crises and progressive organ failure. Success of health management of children with SCD is highly contingent on caregivers' capabilities. Caregivers of SCD affected children must perform a variety of tasks including communication with healthcare providers, reading and understanding of health information, interpretation of acute symptoms and administration of medication and complex decision making with regard to treatment options. A construct which describes the knowledge and competences of persons to meet the complex demands of health is 'health literacy' (HL) (1, 2). The measurement and assessment of HL is of growing importance due to expected and reported relationships between inadequate HL and health outcomes (3-5). In addition, caregivers with higher HL levels feel more confident in their ability to perform caregiving tasks, e.g. have a higher self-efficacy, often associated with higher quality of life (6, 7). Information on literacy levels and the relationship between HL, disease knowledge and self-efficacy may guide interventions in comprehensive SCD care. The aims of this study were to: (a) gain insight into levels of HL in caregivers of children with SCD using objective and subjective measures and to (b) assess the relationship between HL, caregivers self-efficacy' in communication with healthcare professionals and knowledge of SCD on different topics related to the illness. This abstract reports preliminary results. Methods In this cross-sectional, observational study, we included caregivers of children with SCD who attended the outpatient clinic of the Sickle Cell Comprehensive Care Center in the Erasmus Medical Center for a routine follow-up visit. Caregivers included in the study had to be able to communicate verbally in Dutch with professionals. HL was measured using the Short Assessment of Health Literacy in Dutch (SAHL-D) (8, 9); self-reported HL was evaluated by the Set of Brief Screening Questions (SBSQ) (10, 11). Self-efficacy was measured using the Perceived Self-Efficacy in Caregiver-Patient Interactions (PECPI) scale (12). Knowledge of SCD was assessed using a structured 13-item questionnaire (SCD-K) based on information and education provided in our clinic. Since data were not-normally distributed, they were analyzed using non-parametric statistics. Results To date, a total of 42 caregivers were included. Study inclusion will continue until at least 75 caregivers have been included. Demographics are presented in Table 1. Caregivers were mainly the child's mother (81.0%) often the single head of household (66.7%). The mean age of caregivers was 34.4 years. Educational level ranged from never attended school to post college Almost a quarter (23.8%) of caregivers was born in the Netherlands, others the rest were non-western migrants (76.2%). 78.6% of caregivers had low HL according to the SAHL-D. Caregivers with lower HL were more likely to have lower education (p=0.012) and to have been born outside the Netherlands (p=0.002). Only four caregivers (9.5%) reported having difficulties in understanding and applying health information (measured by SBSQ). The correlation between the SAHL-D and the SBSQ scores was weak (r=0.39). Mean scores on the SBSQ and PECPI were high, indicating that caregivers perceived their abilities for self-efficacy and their ability to read and understand medical information as quite high. Responses to individual SCD-K items however suggest large knowledge deficits: only 64.3% of caregivers knew which temperature is considered as fever [ 〉 38.0 °C or 〉 38.5 °C] and only 14.3% was aware which risk factors are able to provoke sickle cell crises. The relationship between literacy status and item responses on SCD-K assessment scale was also examined. Caregivers with low literacy scored significantly lower on almost every individual item. Discussion Inadequate HL is highly prevalent in caregivers of children with SCD. Not being born in the Netherlands and lower education levels are strong predictors of low HL. Our study suggests that healthcare professionals should be attentive to possible low HL. In addition, these results underline that health information should be tailored to the HL skills and specific context of patients and their families. Disclosures Cnossen: NWO: Other: Governmental grants , ZonMW, Innovation fund and Nationale Wetenschapsagenda 2018; Roche: Other: Travel Grants; Takeda: Other: Travel Grants, Research Funding; Shire: Other: Travel Grants, Research Funding; Baxter: Other: Travel Grants, Research Funding; Sobi: Research Funding; CSL Behring: Other: Travel Grants, Research Funding; Nordic Pharma: Research Funding; Novo Nordisk: Research Funding; Pfizer: Other: Travel Grants, Research Funding; Bayer: Other: Travel Grants, Research Funding.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    RVK:
    RVK:
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2019
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 2
    Online Resource
    Online Resource
    American Society of Hematology ; 2019
    In:  Blood Vol. 134, No. Supplement_1 ( 2019-11-13), p. 1030-1030
    In: Blood, American Society of Hematology, Vol. 134, No. Supplement_1 ( 2019-11-13), p. 1030-1030
    Abstract: Introduction Sickle cell disease (SCD) is an autosomally, recessive inherited hemoglobinopathy and multisystem disorder characterized by ongoing hemolytic anemia, episodes of vaso-occlusion and progressive organ failure with ultimately a shortened life expectancy. Despite intensive comprehensive care and improved rates of morbidity and mortality, SCD care is still marked by high utilization of medical resources. Until now, most cost-of care studies have focused on one or two care categories, such as hospitalizations and physician visits [1-4]. Also, few studies have evaluated healthcare expenditures exclusively in children. Estimating cost-of care is important as it can ensure sufficient allocation of resources. In addition, SCD expenditures can be used to raise awareness of disease severity and serve as an incentive for prevention and management of disease complications. Primary aims of this study were to (a) investigate the overall cost of healthcare for pediatric SCD patients and to (b) estimate major cost drivers. Methods All pediatric SCD patients visiting the Erasmus University Medical Center-Sophia Children's Hospital for routine or emergency care from January 1st to December 31st 2017 with a diagnosis of SCD were included. Retrospective data of this cohort were analyzed for 24 months during January 1st 2015 to December 1st 2016. Patients were grouped into four age categories; (A) 0-12 months, (B) 1-5 years, (C) 5-13 years and (D) 13-19 years. For patients born before January 1st 2015, each individual contributed two years of follow-up time. As some children were born during one of the two years during the study time period, the weighted average was calculated based on the time patients were potentially able to make costs. Healthcare utilization of included patients was based upon data from two main sources. The clinical SCD standard treatment guideline was used to determine the expected resource use of routine comprehensive care (planned elective care) and the Erasmus University Medical Center financial claims database was used to estimate real-world resource use associated with acute and inpatient care (additional care). The included items for the SCD guideline and financial claims database per cost category are summarized in Table 1. Results A total of 125 patients were analyzed. The mean age was 8.1 years (SD: 5 years) on December 31st 2015 and 9.9 year (SD: 5 years) on December 31st 2016. Expenditures for the 125 children with SCD averaged €4285.09 (SD: €820.36) per child per year. The majority (49%) of costs was associated with standard treatment (i.e. prophylactic antibiotics); 23% with diagnostics; 19% with inpatient hospital care and 9% with outpatients visits. Annual average costs per patient per age group are depicted in Figure 1. Total expenditures for children with SCD increased per age group, ranging from €2962 (category A), €3726 (category B), and €4087 (category C) to €5890 (category D). This was mostly explained by increases in admission costs. Discussion Although healthcare utilization and costs of pediatric SCD patients have been studied previously [5-7], studies from Europe comprehensive care centers are scarce and have been mostly based on only one aspect of care such as hospitalizations costs [2] . To our knowledge, this is the first study combining standard treatment costs with real world resource use. The total annual coast of healthcare for children with SCD, including inpatient care, outpatient care, diagnostics and treatment averaged €4285.09 per patient per year. This is much lower when compared to costs of healthcare for pediatric patients with SCD reported in other studies. Kauf et al. calculated total costs of healthcare for SCD patients aged 0-9 years to be $10.704 [7] . In addition, inpatient care accounts for a relatively small part of total costs in our study. This finding has been inline with previous research where comprehensive care has been suggested as a means of reducing costs associated with SCD care [8]. A comprehensive, multidisciplinary approach is necessary to address the physical, mental and social needs of each child and their family. Comprehensive care, with effective management in the outpatient setting is able to prevent hospital admission, and is essential for delivery of high quality cost-effective care in SCD. Disclosures Cnossen: Pfizer: Other: Travel Grants, Research Funding; Bayer: Other: Travel Grants, Research Funding; Novo Nordisk: Research Funding; Nordic Pharma: Research Funding; CSL Behring: Other: Travel Grants, Research Funding; Sobi: Research Funding; Baxter: Other: Travel Grants, Research Funding; Shire: Other: Travel Grants, Research Funding; Takeda: Other: Travel Grants, Research Funding; Roche: Other: Travel Grants; NWO: Other: Governmental grants , ZonMW, Innovation fund and Nationale Wetenschapsagenda 2018.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    RVK:
    RVK:
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2019
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
    Location Call Number Limitation Availability
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  • 3
    Online Resource
    Online Resource
    American Society of Hematology ; 2019
    In:  Blood Vol. 134, No. Supplement_1 ( 2019-11-13), p. 4835-4835
    In: Blood, American Society of Hematology, Vol. 134, No. Supplement_1 ( 2019-11-13), p. 4835-4835
    Abstract: Introduction Sickle cell disease (SCD) is a severe inherited red blood cell disorder with high morbidity requiring integrated comprehensive care. There are not many chronic diseases that primarily affect those from an often impoverished, minority background. Care delivery is complicated by disparities in healthcare regarding access. Cultural mismatches between providers and families also play a role. Healthcare access can be defined as the degree of fit between patients and the healthcare system (1, 2), and is described by four overlapping dimensions: physical accessibility, affordability, information accessibility and non-discrimination (3, 4). This qualitative study focuses on the challenges regarding the right to healthcare for children and adolescents with SCD and their families. The aims of this study were to (a) map the various perspectives and initiatives of SCD healthcare professionals, while focusing on existing best practices and lessons learned and (b) to provide practical recommendations relevant for high-income countries to improve accessibility of healthcare in SCD. Methods This qualitative study consisted of semi-structured, in-depth interviews which were conducted from February 1st to May 1st 2019 with SCD healthcare professionals, including (pediatric) hematologists, nurses, and psychosocial staff. Participants were affiliated with the SCORE (Sickle Cell Outcome REsearch) consortium which includes all SCD comprehensive care centers in the Netherlands (Fig 1). We identified eligible participants within SCORE and recruited participants using a combination of maximum variation and snowball sampling. Interviews occurred privately and were audio-recorded. The audio-recordings were transcribed verbatim and analyzed using the NVivoâ qualitative analysis software. Results Twenty-four healthcare professionals from different clinical sites participated in the study (Fig 1). Transcripts were coded into the four dimensions of health care access. Content analyses of the four dimensions in turn, revealed seven themes associated with best practices on topics related to improving accessibility of health care for children and adolescent SCD patients and their families (Fig 2). These themes included: 1) Cutting invisible costs: addressing the financial burden of a child with SCD; 2) Same-day appointments: reducing the amount of hospital visits; 3) Specialized and shared care: bridging the gap; 4) Optimizing methods of verbal and written communication: enabling mutual understanding between patients and healthcare professionals; 5) Building strong digital connections: improving the use of e-health while watching out for pitfalls; 6) Tools for patient empowerment and resilience: being aware of (self) stigmatization; and 7) Changing society: towards compassion and public awareness. Discussion The United Nations Convention on The Rights of the Child is the most widely signed and ratified human rights treaty and safeguards the right of the child to the enjoyment of the highest attainable standard of health. This right must be interpreted broadly, paying attention to all factors that may affect the realization of this right (5). Equity of access to healthcare for all patient populations requires the appreciation of intersecting vulnerabilities. This qualitative study provides best practices and solution-based recommendations from a grassroots-level to improve access to healthcare for children and adolescents with SCD and their families. Healthcare professionals experience a unique combination of intersecting vulnerabilities that characterize SCD patients and hampers their overall access to necessary healthcare. The seven key recommendations address these obstacles across all four dimensions of accessibility. Holistic implementation would positively impact the access to the highest attainable standard of healthcare for pediatric SCD patients and their families. These recommendations are built on local best practices that deserve wider implementation. They are especially relevant in the organization of SCD care, but all physicians will recognize these experiences with regard to healthcare in vulnerable patient populations in general. Disclosures Cnossen: Pfizer: Other: Travel Grants, Research Funding; Bayer: Other: Travel Grants, Research Funding; Novo Nordisk: Research Funding; Nordic Pharma: Research Funding; CSL Behring: Other: Travel Grants, Research Funding; Sobi: Research Funding; Baxter: Other: Travel Grants, Research Funding; Shire: Other: Travel Grants, Research Funding; Takeda: Other: Travel Grants, Research Funding; Roche: Other: Travel Grants; NWO: Other: Governmental grants , ZonMW, Innovation fund and Nationale Wetenschapsagenda 2018.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    RVK:
    RVK:
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2019
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 4
    Online Resource
    Online Resource
    American Society of Hematology ; 2017
    In:  Blood Advances Vol. 1, No. 26 ( 2017-12-12), p. 2491-2502
    In: Blood Advances, American Society of Hematology, Vol. 1, No. 26 ( 2017-12-12), p. 2491-2502
    Abstract: Free histones, not nucleosomes, are cytotoxic and are degraded by FSAP in serum to protect against cytotoxicity. Free histone H3 was not detectable in sera of septic baboons and patients with meningococcal sepsis.
    Type of Medium: Online Resource
    ISSN: 2473-9529 , 2473-9537
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2017
    detail.hit.zdb_id: 2876449-3
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  • 5
    In: Blood, American Society of Hematology, Vol. 126, No. 23 ( 2015-12-03), p. 5572-5572
    Abstract: Background: Value-based health care (VBHC) according to Porter et al. is a novel approach that aims to improve health care by identifying and systematically measuring both medical and patient-centered health care outcome. Thus, including the well-being of the patient as related to physical and mental status, social functioning, and occupational consequences. By applying generic and disease specific outcome measures, health care providers are able to benchmark their center with others in order to compare care strategies and outcome, and to make informed choices with regard to optimization of care, necessary investments and possible cost reductions. Erasmus University Medical Center aims to apply this system in 80% of the disease expert centers in the upcoming three years. SickleCell Disease (SCD) is a chronic hereditary disease characterized by severe anemia, unpredictable episodes of extreme pain, cumulative organ damage and high health care utilization rates. In addition, multiple psychosocial problems influence complexity of health care delivery. Therefore, SCD is an excellent model to explore the effects of VBHC. Our hospital has a large Comprehensive Sickle Cell Care Centre. Aim: To identify ten outcome measures in SCD patients and the validated tools to quantify these measures. This is not possible without definition of baseline patient characteristics of influence on patient outcome. Methods: In six well-prepared sessions, our multidisciplinary team, including both adult and pediatric care takers defined relevant patient outcome measures and most important patient characteristics, in live sessions and surveys in collaboration with patients and parents. Results: Ten outcome measures were identified (Table 1) and discussed with patients and parents. Eight patient characteristics were defined with effect on outcome (Table 2). Eight validated tools to quantify outcome measures were described (Table 3). Specific tools and evaluation modes will be integrated into electronic patient files (Table 3). Conclusions: We believe VBHC is a valuable strategy to optimize patient care and to facilitate informed decision making with regard to health care investments. We have made a first step by identification of patient outcome measures and important baseline patient characteristics according to VBHC methodology. The coming year, outcome will be measured, thus enabling comparisons in the near future with other (inter)national centers. Table 1. Overview of most relevant patient outcome measures Mortality Anxiety Veno-occlusive crises per year Admissions per year Pain related to veno-occlusive crises Caregiver burden Complications Quality of life Social functioning Self-efficacy Table 2. Overview of baseline patient characteristics Genotype Age Born 〈 or 〉 January 2007* Socio-economic status Hemoglobin level Communication Comorbidity Mode of treatment Treatment exclusively in University Medical Center *Neonatal screening for SCD was implemented 〉 January 1st, 2007 Table 3. Overview of tools to quantify patient outcome measures Target group Tool Patient Outcome Measures All ages Electronic patient file · Mortality · Complications · Admissions per year · Veno-occlusive crises per year · 'Door-to-needle-time Emergency Department 〉 18 yrs8-18 yrs0-4 yrs EQ-5D- 5 level version (EQ-5D-5L) Pediatric Quality of Life Inventory (PEDsQL)TNO-AZL Preschool children Quality of Life (TAPQOL) · Quality of life · Social functioning · Anxiety and insecurity 〈 18 yrs 〉 18 yrs Health Care Related Quality of Life (CAREQOL-7D) Long-term Orientation (LTO) · Caregiver burden All ages Visual Analogue Scale (VAS) · Pain related to veno-occlusive crisis 〉 18 yr Hospital Anxiety and Depression Scale (HADS) · Anxiety and insecurity All ages Sickle Cell Self-Efficacy Scale (SCSES) · Self-efficacy Disclosures Cnossen: Pfizer: Other: travel funding, Research Funding; Baxter: Other: Travel Funding, Research Funding; Bayer: Other: travel funding, Research Funding; CSL Behring: Other: travel funding, Research Funding; Novo Nordisk: Research Funding; Novartis: Research Funding.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    RVK:
    RVK:
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2015
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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