Comprehensive Care Center Research Articles

Successful use of buprenorphine-naloxone medication-assisted program to treat concurrent pain and opioid addiction after cancer therapy.

Cancer pain is often treated with opioids, a therapeutic regimen that can become a challenge in patients with an opioid use disorder (OUD). While use of the buprenorphine-naloxone combination is an effective medication-assisted treatment (MAT) for OUD, its use in pain patients with OUD has been controversial due to concerns that co-administration of buprenorphine can reduce or block analge-sia and precipitate opioid withdrawal in those patients requiring full opioid agonists. Data on its use in cancer pain patients are lack-ing. In this case series, the authors explore the frequency of buprenorphine-naloxone use and its outcomes in patients in a Compre-hensive Care Center (CCC) Pain Registry. OUD was deduced from an International Classification of Diseases (ICD-10) diagnostic code for opioid-related disorders recorded in the electronic medical records. Of 2,320 chronic cancer pain patients, 125 patients had ICD-10 code for opioid-related disorders, and 43 had a diagnosis of opioid abuse of whom 11 received buprenorphine-naloxone combina-tions. Eight patients on 18 (6-24) mg per day of buprenorphine-naloxone remained in therapy for 4 (2-7) years without opioid abuse relapse. This assessment was based on clinician's notes, the Prescription Monitoring Program, random urine drug screening, and the absence of Urgent Care Center visits for opioid withdrawal or overdose. When short-term opioids were administered for acute pain, these patients were able to taper down and stop them quickly without an opioid abuse relapse. Buprenorphine-naloxone was effec-tive as the sole analgesic in selected patients. Given its success at the CCC, buprenorphine-naloxone should be made available and strongly considered as a treatment for patients suffering from OUD during and following cancer treatment and when cancer pain re-duces or resolves.

Security amidst stigma: Exploring HIV and sexual minority stressors through an attachment-based psychotherapy group.

Despite strides in HIV prevention and lesbian, gay, bisexual, and transgender care, comprehensive care centers are of critical importance for lesbian, gay, bisexual, and transgender communities and people with HIV/AIDS who continue to contend with intersecting stigmas and chronic minority stressors. Building on the integrated attachment and sexual minority stress model, we discuss these themes by highlighting a group vignette from an urban psychiatric clinic that has provided affirmative psychotherapy to marginalized communities affected by HIV/AIDS for over 2 decades. The authors have rotated at the clinic as cofacilitators of a weekly, process-oriented group for sexual minority men who are HIV positive or are affected by HIV. In this article, we provide a theoretical foundation for HIV-affirming group psychotherapy and clinical integration of minority stress and attachment-based interventions. Group psychotherapy provides a rare opportunity to bond an often-isolated community by evoking factors of universality, cohesiveness, and catharsis. It simultaneously enables us to confront individual existential concerns with serostatus disclosure, grief, and feelings of victimization, as well as challenge internalized stigma and rejection sensitivity. We apply these issues to a verbatim clinical exchange, analyzing attachment-related themes and issues pertaining to minority stress and stigma, as well as discuss group mechanisms for attachment interventions. (PsycINFO Database Record (c) 2020 APA, all rights reserved).

A single centre retrospective study of low dose prophylaxis with extended half-life factor IX for severe haemophilia B.

Extended half-life factor IX concentrates (EHL-FIX) can be administered weekly to prevent bleeding for persons with severe haemophilia B. We report the experience of a large UK haemophilia comprehensive care centre using low dose EHL-FIX for persons with severe haemophilia B. The low doses used in real world are approximately half of the doses used in clinical trials. We aim to assess the efficacy and safety of low dose EHL-FIX. Data from a cohort of 13 patients who were switched from standard half-life factor IX (SHL-FIX) to Alprolix® (mean dose 31.5IU/kg) and seven patients who switched from standard half-life factor IX to Idelvion® (mean dose 20.2IU/kg) were included. The median annualized bleeding rate was similar for SHL-FIX (median 3, interquartile range [IQR] 1-5) and EHL-FIX (median 3, IQR 1-5.25). Quality of life scores, measured using the European Quality of Life 5 Dimensions assessment were similar for SHL-FIX (median 0.76, IQR: 0.63-0.84) and EHL-FIX (median 0.79, IQR: 0.58-0.88). This study shows that EHL-FIX given at low doses can be effective for prevention of bleeding for persons with severe haemophilia B.

Associated Factors of Self-injury Among Adolescents with Autism Spectrum Disorder in a Community and Residential Treatment Setting.

Self-injurious behavior (SIB) occurs in up to 50% of individuals with autism. As one of the most serious conditions in individuals with developmental disabilities, SIB affects the individual and his or her family in multiple contexts. A systematic analysis of factors most commonly associated with SIB could inform the development of individualized intervention strategies. The current study examined factors related to SIB in an analysis of client records of 145 children with autism in a comprehensive care center. Predictor variables included age, gender, the Adaptive Behavior Composite, sensory processing, aggression, stereotypies, irritability, adaptive skills, and medical conditions. Age, irritability, and the Adaptive Behavior Composite were found to significantly predict SIB.

Pulmonary arterial capacitance predicts outcomes in patients with pulmonary hypertension independent of race/ethnicity, sex, and etiology.

Pulmonary arterial capacitance (PAC) is a strong hemodynamic predictor of outcomes in patients with pulmonary hypertension (PH). Its value across subgroups of race/ethnicity, sex, and PH etiologies is unclear. We hypothesized that the association of PAC with outcomes would not vary across World Health Organization (WHO) PH group, race/ethnicity, or sex. We performed a retrospective study in patients with PH diagnosed and managed at the Pulmonary Hypertension Comprehensive Care Center of a tertiary care hospital (n=270). Demographic, diagnostic, treatment, and outcome data were extracted from the electronic medical record. Cox proportional hazards models were used to model time from right heart catheterization to event in univariate and multivariable models. Our primary outcome was all-cause mortality and our secondary outcome was PH hospitalization. The median age of the cohort was 56 years (±14.6), and 67% were female. In multivariable Cox models adjusted for significant covariates, decreased PAC remained independently and significantly associated with both all-cause mortality (p=0.029) and hospitalization for PH (p=0.010). No significant interactions were observed between PAC and race, sex, or WHO group. Hispanic patients exhibited a significant independent association with increased hospitalizations (p=0.030), and there was a trend toward increased all-cause mortality in African Americans. WHO group 2PH was associated with more frequent hospitalization (p=0.004). Decreased PAC is significantly associated with mortality and hospitalization in PH patients independent of race, sex, and PH subgroups. Further investigation is required to characterize the effects and determinants of racial disparities in PH.

Perceptions and attitudes towards gym use and physical activity in young men with haemophilia

Abstract Introduction A growing body of evidence supports the recommendation of both physiotherapy and physical activity in people with haemophilia. Physical benefits include increasing strength and flexibility and reducing the risks of osteoporosis, arthropathy, and intramuscular and joint bleeds; social benefits have also been observed. However, anecdotal evidence suggests that people with haemophilia may still be averse to engaging with physical activity due to fears of causing bleeding, joint pain and joint damage. Methodology Qualitative interviews were conducted with young men with haemophilia treated at comprehensive care centres in London, to explore and identify the reasons behind risk-averse behaviours towards exercise and physical activity. The interview questions were designed to prompt discussion and capture opinions relating to participants’ physical activity and gym membership/use, and the degree to which their haemophilia impacts on both. Results Ten participants were interviewed. Preferred activities were variable, with five participants describing themselves as very physically active, three moderately active, and two reporting little physical activity; four described themselves as ‘not gym-confident’. Seven participants described themselves as highly or moderately motivated to undertake physical activity, with motivations including weight loss and getting fit for summer holidays. However, there was some anxiety around weight-bearing exercise due to the fear of pain or injury. All participants had been exposed to personal trainers (PTs) and recognised the importance of being properly introduced to training equipment but felt that PTs were too expensive for them. The majority of participants reported sports-related injuries and self-perceived limitations on activity due to their personal/individual experience of living with haemophlia. Physiotherapists were often the first point of contact for advice and support on safe physical activity. All participants recognised the benefits of physical activity and had been encouraged in this by their physiotherapists. Conclusion Young men with haemophilia are keen to use the gym as part of their personal fitness regimens. The ongoing safety concerns of health care professionals warrants further research.

Clinical problems and surgical interventions in inherited factor VII deficiency.

Aim:Factor VII deficiency is one of the hereditary coagulation disorders that has autosomal reccessive inheritance and is observed relatively frequently (1/500 000). It is clinically heterogeneous, and may be asymptomatic or lead to life-threatening bleeding. Thus, there is no correlation between FVII activity and clinical findings. Plasma-derived and recombinant FVII concentrates are currently used for treatment. In countries where access to these products is lacking, fresh frozen plasma and prothrombin complex concentrates are also used, though they contain low amounts of factor FVII. In this study, we present the clinical properties, treatments, and surgical interventions used in patients followed up in our clinic with a diagnosis of factor FVII deficiency.Material and Methods:Patients who were diagnosed as FVII deficiency in Division of Pediatric Hematology and Oncology between July 1997 and July 2018, were included in the study. The patients’ demographic characteristics, symptoms at presentation, PT, aPTT, and FVII values, types of bleeding, and treatments and surgical interventions used, were recorded. The bleedings observed in the patients were classified by severity as asymptomatic, minor, and major.Results:A total of 18 patients (7 girls and 11 boys) with a mean age of 9.64±9.63 years were included in the study. The mean follow-up time was found as 78.06±54.4 months. When the bleedings were classified clinically, no bleeding was observed in eight patients (44.4%). The factor FVII level was found as <10% in three of these eight asymptomatic patients and above 20% in the others. Minor bleeding was observed in nine patients (50%) and major bleeding was observed in one patient. When the patients were classified as asymptomatic and symptomatic, there was no significant difference between the two groups in terms of FVII level (p=0.57). A total of 21 surgical interventions were performed in 14 (78%) of 18 patients who were being followed up.Conclusion:FVII deficiency has a very wide spectrum both clinically and in terms of approach to surgical interventions. Therefore, patients with factor FVII deficiency should be followed up and treated by comprehensive care centers with close collaboration of multiple disciplines.

Prediction of outcomes in stroke patients from radiological findings – A hospital-based retrospective cohort study from a comprehensive stroke care center in Kerala, India

Introduction: Functional outcome after stroke can be improved by early and accurate diagnosis with the help of imaging techniques such as computed tomography (CT) and magnetic resonance imaging. Alberta Stroke Program Early CT Score (ASPECTS) calculated from the CT image infarct areas of middle cerebral artery (MCA) circulation is supposed to be a reliable outcome predictor. Methodology: A retrospective cohort study was conducted in the Comprehensive Stroke Care Center for a period of 6 months. All adult patients (18–90 years) who had visited the hospital from January 2014 to December 2017 were enrolled for the study. Patients with MCA strokes were only included in the study since the ASPECTS is defined for this territory. The data were gathered from the electronic format of the patient information system in the institution and were entered in a structured proforma specifically designed for the study. ASPECTS was calculated from the CT findings of the patient. The modified Rankin Scale (mRS) scores were calculated for every patient at four different time points. The collected data were compiled in a Microsoft Excel sheet and were analyzed using IBM Statistical Package for Social Sciences (SPSS version 21). The summary statistics for categorical variables were reported using frequency and percentage and continuous variables as mean (standard deviation). We reported the association of the ASPECTS with mRS at different time points using the Chi-Square test. Results: A total of 132 adult patients were enrolled in the study. The mean age of the study population was 60.72 (±13.99) years, and among them, 93 (70.45%) were males. Majority of the patients were nonvegetarians (99.92%). The most prevalent risk factor found was hypertension (62.1%). The most common symptom presented by the patients was facial palsy (28%). When the ASPECTS was calculated for 132 patients, 62 patients fell in the high ASPECTS category (8–10), 45 patients had a moderate ASPECTS (5–7), and 25 had low ASPECTS (0–4). mRS scores calculated for four different time points showed a decline in disability in most of the patients. ASPECT and mRS had a negative association signifying, as the ASPECTS increases the number of functional disability decreases. Conclusions: Our study results show that ASPECTS can be a definite outcome predictor in MCA stroke patients.

False Positive Tuberculosis Cases (Xpert MTB/RIF Assay) Among People Living With HIV Attending Bahati Comprehensive Care Centre Nairobi, Kenya

The introduction of GeneXpert MTB/RIF assay has impacted positively in tuberculosis diagnosis, providing a rapid way of identifying tuberculosis patients in high burden, low income countries. However Mycobacterium tuberculosis (MTB) detection in previously treated patients, which may be due to old deoxyribonucleic acid or active disease, still remains a diagnostic dilemma for diagnosis of tuberculosis. A retrospective cohort study was conducted among consenting patients with signs and symptoms of tuberculosis attending Bahati comprehensive care centers. A total of three hundred and forty six patients were sampled and their sputa collected, subsequently laboratory analysis was carried out for detection and culture of Mycobacterium tuberculosis. Seventy seven (22%) sputa had Mycobacterium tuberculosis detected on Xpert MTB/RIF assay sputa from these patients with bacteriologically confirmed pulmonary tuberculosis were subjected to culture on Mycobacterium Growth Indicator Tube (MGIT) media. Detection of Mycobacterium tuberculosis on Xpert MTB/RIF assay with no isolation of growth on culture indicated a false positive tuberculosis diagnosis. Out of 77 isolates subjected for culture a total of 0(0%) and 5(7.5%); P=0.484, isoniazid preventive therapy and non- isoniazid preventive therapy patients had false positive tuberculosis cases, while 0(0%) and 5(25%); P=0.001 new and retreatment patient’s had false positive tuberculosis. Our study concluded that there was no significant association between isoniazid preventive therapy and tuberculosis false positivity but there was significant association between patient treatment status and tuberculosis false positivity. Previously treated tuberculosis patients were significantly associated with false positivity, this call for clinicians to exercise caution when interpreting results from previously treated tuberculosis patients.

The Cost of Healthcare for Pediatric Patients with Sickle Cell Disease

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.

Seroprevalence of Pre-Existing Nabs Against AAV1, 2, 5, 6 and 8 in South African Hemophilia B Patient Population

Introduction Several studies have shown that the induction of antibodies by natural exposure to various AAV serotypes can compromise the subsequent use of AAV as a gene therapy vector, limiting patient eligibility for AAV-delivered therapeutics. The implications of pre-existing antibodies to AAV serotypes are very different: Levels of anti-AAV2 or anti-AAV8 neutralizing antibodies (NABs) as low as 5 have been related to a decrease or even total impairment of AAV liver transduction after systemic delivery in humans. However, successful gene transfer has been reported in patients with anti-AAV5 NABs titers up to 340 and in non-human primates with titers up to 1030. Extensive surveys on the prevalence of anti-AAV antibodies in humans have been published. Results from these studies indicate that prevalence varies dependent on serotype, and that a significant proportion of individuals develop humoral immunity against various AAV serotypes early in life, starting around 2 years of age. Furthermore, the prevalence of antibodies to different AAV serotypes has been reported to vary according to geographical location. Study Objective We performed a NABs seroprevalence study in South African hemophilia B patient population (n=44) using a panel of AAV serotypes suitable for liver targeted therapy, to determine the AAV serotypes likely to be of greatest clinical applicability for the South African hemophilia B population. Methods Forty-four hemophilia B patient serum samples were obtained from Hemophilia Comprehensive Care Center in Johannesburg (South Africa). All the patient serum samples were analyzed for the presence of NABs against AAV serotypes 1, 2, 5, 6 and 8 with the use of highly sensitive luciferase-based bioassays. The assays entail incubation of the test serum samples dilution series with an AAV1, 2, 5, 6 or 8-based reporter vector that carries the luciferase gene. This incubation allows neutralizing antibodies in the test serum to bind to the reporter vector particles. These mixtures are subsequently transferred onto Hek293T cells, where reporter vector particles can transduce cells and mediate expression of luciferase. Anti-AAV1, 2, 5, 6 or 8 NABs titers were determined by calculation of the percentage of neutralization for each sample dilution and fitting the neutralization curve with a four-parameter method. Anti-AAV1, 2, 5, 6 or 8 NABs titer (IC50) is the dilution at which antibodies inhibit Hek293T cell transduction with AAV1, 2, 5, 6 or 8-LUC by 50%. The lowest patient serum dilution used in every assay was 8 and samples were considered positive when calculated anti-AAV NAB titer was ≥8. All analytical runs included proper negative and positive controls. Results and Discussion The presence of NABs against the AAV serotypes 1, 2, 5, 6 and 8 was determined in the serum of the hemophilia B patients (Fig. 1). The highest prevalence of NABs was found to be against the AAV2 serotype, 95% (n=42/44) followed by the AAV6 serotype, 82% (n=36/44), and the AAV1 serotype 77% (n=34/44). The prevalence of NABs against AAV5 and AAV8 was lower with 66% (n=29/44) for AAV5 and 64% (n=28/44) for AAV8. The serum samples positive for anti-AAV2 NABs had a high occurrence of titers above 1030 (39%) in comparison to anti-AAV1 NABs (20%), anti-AAV5 NABs (5%) or anti-AAV8 NABs (7%).The occurrence of samples with low titers (ranging from titer of 8 to titer of 50) was the highest for anti-AAV8 NABs (32%) and for anti-AAV5 NABs (27%), followed by anti-AAV2 (18%) and anti-AAV1 (5%) (Fig.1). Currently, an anti-AAV NABs titer of 5 is used as an exclusion criteria in most of the systemic AAV-based gene therapies. When applying a similar cut-off of 8, 23% of the analyzed patients could be treated with AAV1, 5% with AAV2, 34% with AAV5, 18% with AAV6 and 36% with AAV8-based therapeutics (Fig.2). However, we have previously reported that AAV5-neutralizing antibodies do not impair the efficacy of in vivo transduction of AAV5-based vector up to a measured titer of 340 in humans and 1030 in non-human primates. Therefore, applying the cut-off of 340 or 1030, either 84% or 95% of the South African Hemophilia B patients could benefit from treatment with AAV5-based gene therapy (Fig.2). Disclosures Majowicz: uniQure N.V.: Employment. van Waes:uniQure N.V.: Employment. Timmer:uniQure N.V.: Employment. van Deventer:uniQure Biopharma B.V.: Employment. Mahlangu:Takeda: Consultancy, Honoraria, Speakers Bureau; LFB: Consultancy; NovoNordisk: Consultancy, Research Funding, Speakers Bureau; Roche: Consultancy, Research Funding, Speakers Bureau; Baxalta: Consultancy, Research Funding, Speakers Bureau; Freeline Therapeutics: Research Funding; Pfizer: Consultancy, Research Funding, Speakers Bureau; Spark: Consultancy, Speakers Bureau; Chugai: Consultancy; Biomarin: Research Funding; CSL Behring: Consultancy, Research Funding, Speakers Bureau; Novartis: Research Funding; Sanofi Genzyme: Research Funding, Speakers Bureau; Shire: Consultancy, Research Funding, Speakers Bureau; Sobi: Research Funding, Speakers Bureau; uniQure: Research Funding; World Federation of Haemophilia: Speakers Bureau. Ferreira:uniQure N.V.: Employment.

The Effects of Utilization of Individualized Pain Plans in Treatment of Vaso-Occlusive Crises in the Emergency Department

Background Sickle cell disease (SCD) is an inherited blood disorder that affects millions of people worldwide, with approximately 100,000 Americans affected (Center for Disease Control, 2017). In the U.S., SCD results in over 200,000 emergency department (ED) visits annually, with pain as the most common complaint (Lanzkron S, et al 2010). At The Ohio State University (OSU) Wexner Medical Center, there is a comprehensive care center for patients with SCD. At their initial patient visit, the patient and their hematologist determine a customized pain plan to be enacted when they present to the ED in acute vaso-occlusive crisis (VOC). In January 2015, these plans were implemented to allow for more rapid treatment of pain crisis in the ED at OSU. Methods A multidisciplinary group was formed in order to accelerate the treatment of SCD patients who presented with VOC. The group's goal was to reduce the time to first opioid by utilizing individualized pain plans for each patient. This would reduce the amount of time deciding the best course of treatment. With reduction in time to first opioid, outcomes including overall length of ED stay, disposition, and length of inpatient admission were identified. Data regarding these endpoints were collected from 01/01/14 to 12/31/15. Generalized linear models were fit to compare the clinical outcomes pre and post implementation of the new protocol. Comorbidities were associated with outcomes using the same modeling technique, where univariable models were built and multi-test adjustment was performed through false-discovery rate (FDR). Results During the 2-year study period, 214 patients with SCD accumulated 2429 ED visits in total. The model estimated a 48% decrease in time to first opioid after implementation of the individualized pain plan protocol (p&lt;0.0001). There was also a decrease in the length of ED stay by 22% (p&lt;0.0001). No significant difference between the type of disposition (discharged to home, inpatient admission, eloping from ED, or leaving without being seen) was found. No difference was found between the average number of visits to the ED and the length of inpatient admission pre and post protocol. The data did reveal a 13% increase in length of ED stay in patients with comorbid kidney disease (FDR p=0.02) and a 12% increase in length of stay in the ED for patients with history of venous thromboembolism (VTE) (FDR p=0.04). Patients with history of VTE were also found to have a 39% longer hospital admission than those without (FDR p=0.05). The odds of getting discharged from the ED were 55% lower in patients with essential hypertension (FDR p=0.02) and they were 2 times more likely than patients without hypertension to be admitted to the hospital (FDR p=0.05). Conclusion Utilization of individualized pain plans for patients with sickle cell disease presenting with VOC results in a significant reduction in the amount of time to first opioid administration. Implementation of the protocol also led to a reduction in length of stay in the emergency department, however the probability of admission did not change. In examining the effect of comorbidities on clinical outcomes, patients with history of kidney disease or VTE had increased length of ED stay and those with history of VTE also had longer hospital admissions. Patients with comorbid essential hypertension were also twice as likely to be admitted to the hospital though length of inpatient admission did not change. It is possible that patients with kidney disease have worse disease as evidenced by end-organ damage due to repeated vascular insult, ischemia, and inflammation. Similarly, those with history of VTE may have higher viscosity, endothelial adhesion, and dysfunction leading to clots. Patients with comorbid essential hypertension can also be thought to have recurrent vascular damage leading to systemic hypertension. From this information, it can be concluded that utilizing individualized pain plans in SCD patients with VOC will lead to decreased time to analgesia and perhaps decrease use of healthcare resources. Disclosures Desai: Novartis: Research Funding; Pfizer: Membership on an entity's Board of Directors or advisory committees, Research Funding; University of Pittsburgh: Research Funding; Ironwood: Other: Adjudication Board; Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees, Research Funding; Potomac: Speakers Bureau.

Predictors of the Rowland Universal Dementia Assessment Scale (RUDAS) Performance in Adults with Sickle Cell Disease

Background: Sickle cell disease (SCD) patients are at significant risk for stroke and silent cerebral infarcts. At least 33% of adults have cognitive dysfunction. However, access to specialized assessments is limited, and there is currently an unmet need for a fast, easy to administer, screening tool for cognitive impairment in SCD. The Rowland Universal Dementia Assessment Scale (RUDAS) is a 6-item task-based questionnaire that evaluates executive function, memory, language, visual-spatial function, praxis and judgment. It has been validated in many cultures and neurocognitive diseases other than SCD. Hypothesis: Poor RUDAS performance is associated with the presence of SCD complications independent of age, socioeconomic and education factors. Methods: Study design: cross-sectional, two adult sickle cell comprehensive care centers in Canada. Inclusion criteria: out-patients ≥18 years-old; all SCD phenotypes. Exclusion criteria: inability to obtain informed consent and/or follow study instructions. Intervention: RUDAS was administered twice, 2-4 months apart, in French or English, based on the patient's preference. Survey on demographics and patient-reported outcomes (PROMIS® tools for Depression and Anxiety) were completed. Baseline characteristics, SCD complications, and laboratory results were collected. Statistical plan: t-tests, Fisher exact and chi-squared tests, for continuous and discrete variables respectively, were performed to identify possible association between RUDAS and biologic, socioeconomic, and cultural factors, SCD related complications, comorbid conditions, laboratory parameters, and use of disease-modifying therapy (Table). Associations with univariate P &lt;0.05 were included in the multiple linear regression model. Multicollinearity was assessed. Results: Of the 252 participants, 92 were from Centre Hospitalier de l'Université de Montréal in Montréal, 160 were from University Health Network in Toronto. Median age at time of survey was 31.5 years (IQR 25-44). Female to male ratio was 1.15. Sickle genotype was distributed as follows: SS 55% (N=138), SC 32% (N=80), other sickle genotypes 13% (N=34). Median RUDAS score was 26 (IQR 24-28), mean score ± standard deviation was 26.0±2.9. Suspected cognitive impairment (defined as RUDAS score &lt;23/30) was found in 12% (N=29) of the participants. On univariate analysis, RUDAS score declined significantly with age (P&lt;0.001), lower eGFR (P&lt;0.001), lower systolic blood pressure (P=0.022), and lower reticulocyte count (P=0.007), while higher level of education (P=0.012), employment and/or active enrolment in a study program (P&lt;0.001), and diagnosis of depression (P=0.009) were predictive of higher RUDAS scores (Table). Reticulocyte count, eGFR, and highest level of education remained independent predictors of RUDAS score on multiple linear regression (P=0.003, &lt;0.001, and 0.001 respectively; see Table for effect size). Center, language of administration, age and diagnosis of depression were not associated with RUDAS score on multiple regression. R2 of the model was 0.323. All variance inflation factors in the model were &lt;2.0. Conclusions: Reticulocyte count and eGFR, but not SCD genotype, being independent predictors of RUDAS suggests disease phenotype may contribute to neurocognitive decline and deserves further exploration. RUDAS does not appear to be influenced by age, language of administration, socioeconomic status, and depression, on multiple regression with mild collinearity. Interestingly, education was independently associated with RUDAS score, despite previous studies showing RUDAS was not biased by education. Recruitment is ongoing at two additional sites to further delineate these relationships and to explore the role of silent cerebral infarct in neurocognitive decline in SCD patients. RUDAS may be a promising tool to identify the patients at higher risk for cognitive impairment who may benefit from access to specialized neurocognitive, educational and social interventions. Disclosures Kuo: Agios: Consultancy; Alexion: Consultancy, Honoraria; Apellis: Consultancy; Bioverativ: Other: Data Safety Monitoring Board; Bluebird Bio: Consultancy; Celgene: Consultancy; Novartis: Consultancy, Honoraria; Pfizer: Consultancy.

Health Literacy, Self-Efficacy and Knowledge of Sickle Cell Disease Among Caregivers

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 [&gt;38.0 °C or &gt;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.

Improving Access to Healthcare for Pediatric Sickle Cell Disease Patients and Their Families

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.

Management of Major Surgical Procedures in Patients with Hemophilia: A Single-Center Experience of 49 Procedures

Background: Management of surgical procedures in people with hemophilia (PwH) has always been a major concern. Insufficient hemostatic control might be an important cause of morbidity and mortality. However, the success of surgical procedures does not only depend on appropriate replacement of the missing factor. Pre- and post-operative interventions, laboratory monitoring, care and rehabilitation of the patient are important. Therefore, surgical procedures in hemophilia patients should be performed in full-fledged hospitals capable of providing a multidisciplinary approach as a "Comprehensive Hemophilia Treatment Center". The aim of our study is to evaluate the outcomes of major surgical procedures (MSPs) among PwH who were followed at Cerrahpasa Medical Faculty. Methods: All MSPs performed on PwH between 2004 and 2017 were included. Baseline activated partial thromboplastin time (aPTT)and factor levels prior to MSP, inhibitor screening and (if any) the inhibitor titration results together with complete blood count, blood group and liver function tests were retrospectively obtained from patient files. The type of MSP, amount of factor concentrates given prior to, during and after the operation, the factor levels and aPTT results following factor replacement; complications developing during or after surgery, and information on the type of treatment modality prior to surgery (on demand vs. prophylaxis) were noted. The amount of factor concentrates administered to patients was determined in units per kilogram. Results: Of the 39 patients included in the study (37 hemophilia A and two hemophilia B) 20 were severe, 7 were moderate and 12 were mild hemophilia (Table 1). The median age at the time of MSP was 37 (20-61) years. A total of 49 MSPs were performed, two patients had 3 surgeries, six patients had 2 surgeries, and 30 patients had one surgery. Fifteen surgical procedures were performed in two, one surgical procedure was performed in three, and 33 operation procedures were performed in one operation area. There were no significant differences in complication rates between hemophilia types (A vs. B), severities (severe vs. moderate vs. mild) and number of operated regions (1 vs. &gt;1). In our study, general surgery (n=15) and orthopedic (n=10) operations were the most frequently performed MSPs (Table 2). There was no significant difference in complication rates among surgical branches. Complications were observed in a total of 6 (12%) MSPs, and one patient was deceased due to sepsis. Complication rates were 16% and 11% for MSPs done in PwH with and without inhibitors, respectively (p=non-significant). Factor consumption (U/kg) was highest in patients undergoing orthopedic surgery, followed by cardiovascular and neurosurgical operations. Factor utilization was significantly less for operations done in general surgery, urology and ear, nose and throat departments (p&lt;0.001). There was a significant positive correlation between factor consumption and duration of hospitalization (r = 0,690; p &lt;0.001). The data on the type of factor were available in 36 MSPs. Surgical procedures were carried out using plasma-derived and recombinant factor concentrates only in 26 and 5 patients, respectively. In the remaining 5, plasma-derived and recombinant factor concentrations were alternately applied. No statistically significant difference was noted with regard to the type of the factor used (plasma-derived, recombinant and plasma-derived/recombinant) and the duration of hospitalization or the complication rate. Number of days spent in the hospital were significantly higher in patients with complications than those without (16 vs. 9.5 days; p=0.003). Conclusion: The results of our retrospective study, being consistent with the current literature, clearly demonstrates that, in experienced comprehensive care centers MSPs can effectively and successfully be performed in PwH, even in those with inhibitors. Disclosures No relevant conflicts of interest to declare.

Pharmacy involvement in an oral anticancer management program: Drug acquisition and safety in an ambulatory comprehensive care center.

85 Background: Oral anticancer agents (OACA) offer benefits of convenience, flexibility and quality of life; however, similar to intravenous therapy, OACA can be prone to medication ordering errors, drug-interactions, and administration errors. Additionally, they carry unique challenges such as treatment delay due to acquisition barriers and errors related to self-administration and monitoring. An oral anticancer management program was implemented to address vulnerable aspects of the outpatient OACA medication use process. Pharmacy benefit specialists (PBS) assist with prior authorizations (PA) and drug acquisitions; while, clinical pharmacists perform medication assessments and patient education. Methods: A retrospective review of patients prescribed OACAs between January 1 - March 31, 2019 was performed. A review of all PAs facilitated by our PBS was conducted and included date of prescription, date of submission to insurance, and date of PA approval. An assessment of pharmacist interventions on the prevention of medication-related errors and toxicity was conducted and was defined by any clarification or recommendation regarding medication dose, administration instructions, drug interactions, symptom management, medication reconciliation, and/or medication monitoring. Results: A total of 567 PAs were processed. Twenty-eight PA requests were denied and 539 were approved. The average time to approval was 1.5 days (range 0-13 days). 299 of the 567 patients were prescribed a new OACA and were enrolled into our education program. Pharmacists educated 191 patients. Pharmacists intervened 175 times for the 191 patients counseled. Interventions included recommendations/clarifications for symptom management (21.5%), medication reconciliation (19.5%), drug administration (16.2%), drug interactions (13.6%), and medication monitoring (7.3%). Conclusions: Pharmacy staff can make a valuable contribution to drug acquisition and overall clinical care of patients receiving OACAs. Our PBS reduced delays in acquisition and clinical pharmacists minimized medication-related errors and toxicity.

Physiotherapy outcome measures of haemophilia acute bleed episodes: What matters to patients?

The research was conducted at a UK teaching hospital and Haemophilia Comprehensive Care Centre (CCC) as part of a research programme investigating physiotherapy for acute bleed management. The aim of the study was to understand the perspectives of people with haemophilia (PWH) on validated outcome measures (OM) and whether these measures capture changes relevant to them whilst recovering from an acute bleed episode. Any person with haemophilia registered to the CCC who reported an acute bleed within the past 2years was invited to participate. Semi-structured interviews or workshops (activity-focused discussions with small groups) were conducted with PWH who had received physiotherapy treatment in the previous two years. These were used to explore opinions of PWH of commonly used outcome measures. Eight male PWH participated, mean age 61years, ranging between 39 and 71. Seven participants had severe haemophilia A and 1 had von Willebrands. Participants described numerical rating scales of pain as abstract and expressed a preference for verbal or visual descriptors. In relation to function, the men generally found haemophilia-specific OM to be more relevant. The EuroQol 5-Dimension 5-Level (EQ5D-5L) and Haemophilia and Exercise Project Test Questionnaire (HEP-Test-Q) were considered as good measures due to brevity and ability to capture relevant changes promptly. Participants in this study reported a preference for short OMs that allow them to reference their ability during the acute bleed episode in comparison with their normal function.

Performance of family planning clinics in conducting recommended HIV counseling and testing in Mombasa County, Kenya: a cross-sectional study

BackgroundA high proportion of African women utilize family planning (FP) services. Accordingly, incorporating HIV testing into FP services may strategically target the first WHO 90–90-90 goal of 90% of people living with HIV knowing their status.MethodsThe objective of this analysis was to determine the proportion of new FP clients counseled and tested for HIV, as well as correlates of HIV testing, in a random sample of 58 FP clinics in Mombasa County, Kenya. Structured interviews of FP clinic managers collected data on characteristics of FP clinics and staff. Study staff performed a 3-month review of FP registers, summarizing new client HIV testing and counseling (HTC). Because overall rates of HTC were quite low, a binary variable was created comparing clinics performing any HIV counseling and/or testing to clinics performing none. Generalized linear models were used to calculate prevalence ratios (PR) and identify correlates of HTC. Factors associated with any HTC with a p-value < 0.10 in univariate analysis were included in a multivariate analysis.ResultsOf the 58 FP clinics, 26 (45%) performed any counseling for HIV testing, and 23 (40%) performed any HIV testing. Counseling for HIV testing was conducted for 815/4389 (19%) new clients, and HIV testing was performed for 420/4389 (10%). Clinics without trained HIV testing providers uniformly did not conduct HIV counseling and/or testing (0/12 [0%]), while 27/46 (59%) of clinics with ≥1 provider performed some HTC (p < 0.001). In the subset of 46 clinics with ≥1 trained HIV testing provider, correlates of performing HTC included being a public versus non-public clinic (PR 1.70 95%CI 1.01–2.88), and having an HIV comprehensive care center (CCC) onsite (PR 2.05, 95%CI 1.04–4.06).ConclusionTrained HIV testing providers are crucial for FP clinics to perform any HTC. Approaches are needed to increase routine HTC in FP clinics including staffing changes and/or linkages with other testing services (in standalone VCT services or lab facilities) in order to improve the implementation of existing national guidelines. A future cluster randomized trial is planned to test an implementation strategy, the Systems Analysis and Improvement Approach (SAIA) to increase HTC in FP clinics.

Outcome and Prognosis Factors of Stage IV Cervical Cancer Patients: A Decade Experience

The aim of this study was to identify and compare prognostic factors, management strategies, and outcomes of very locally advanced cervical cancer (CC) (i.e., stages IVA) and metastatic CC (i.e., stages IV). A retrospective review was conducted, based on all consecutive patients treated for stage IV CC in a comprehensive cancer care center between 2004 and 2017. Sixty-eight patients were included. Performance status (PS) was ≥ 2 for 35.9%. Median age at diagnosis was 60.5. There were 24 stage IVA CC (35.3%) and 44 stage IVB CC (64.7%). Seventeen patients with stage IVB CC had only para-aortic lymph nodes metastases (38.6%), 13 had only distant metastases (29.5%) and 14 had both (31.8%). Patients with stage IVA CC experienced a radiotherapy with curative intent (n=14, 58.3%) +/- a concomitant chemotherapy, or a palliative treatment (n=10, 41.7%). Twenty-three patients with stage IVB CC received a prior chemotherapy (52.3%), eleven a primary concomitant chemoradiation (25%), and ten a palliative treatment (22.7%). The mean follow-up was 18.0 months. The 5-year overall survival was 5.1% for stages IVA (95%CI=0.7-33.9), and 10.5% for stages IVB (95%CI=3.7-29.7). In multivariate analysis, PS>1 was identified as a poor prognostic factor of disease-specific survival for stage IVA CC. PS>1 and pelvic lymph nodes involvement were identified as poor prognostic factors of overall survival and disease-specific survival for stage IVB CC. In daily clinical practice, outcomes of stages IV CC are poor. Treatment of advanced and metastatic CC remains challenging. New management strategies are needed, as well as efficient preventive strategies.