ALBERT

Description: Menorrhagia is a common clinical problem among reproductive age women and annually 5% of reproductive age women seek medical attention, usually with gynecologists and other primary care physicians, for this symptom. Since underlying bleeding disorders are common in women presenting with menorrhagia, and referral for comprehensive hemostatic testing of substantial numbers of women with otherwise unexplained menorrhagia is problematic from the public health and cost perspective, a short, easy-to-administer screening tool comprised of 8 questions for identifying women with menorrhagia for hemostatic evaluation was previously developed (Am J Ob Gyn2008;198:e1–163e8). In the present study, the validity of the screening tool was evaluated in a multi-site, prospectively recruited cohort of women with menorrhagia. 232 women with menorrhagia age 18 and older with a pictorial blood assessment chart (PBAC) score 〉 100 were recruited from 5 US centers as potential subjects for a prospective cross-over study for evaluation of intranasal DDAVP versus tranexamic acid. All subjects underwent comprehensive laboratory testing for bleeding disorders, including VWF, platelet aggregation/ATP release, and factor assays. Study participants were administered a questionnaire which included the 8 screening tool questions in 4 categories, including history of duration and severity of menorrhagia, anemia treatment, excessive bleeding with hemostatic challenges, and family diagnosis of bleeding disorder. A screening tool was considered positive if there was a positive response for any of the questions in the four categories. Sensitivity of the screening tool with 95% confidence interval was calculated for bleeding disorders and also separately for low VWF (ristocetin cofactor 〈 50%), and platelet function defects.217 women with menorrhagia including 78% white and 16% black women with complete data were evaluated. In this population, a positive screening tool had a sensitivity of 89% (95% CI, 83–93) for bleeding disorders, 89% for platelet function defects (95% CI, 82–94), and 73% for low VWF (95% CI, 39–94). The sensitivity for bleeding disorders was 87% (95% CI, 79–92) among white women and 94% (95% CI, 79–99) among black women. Adding a PBAC score 〉 185 increased the sensitivity of the screening tool for bleeding disorders to 95% (95% CI, 90–98). Using a multi-site US population of adult women with menorrhagia, this study confirms the benefit of a short screening tool to assist primary care physicians in the selection of women with menorrhagia to refer for comprehensive hemostatic testing and evaluation. population of adult women with menorrhagia, this study confirms the benefit of a short screening tool to assist primary care physicians in the selection of women with menorrhagia to refer for comprehensive hemostatic testing and evaluation.

Description: Background: Numeracy, defined as the ability to handle basic probability and numerical concepts including computation, estimation, logic, and problem solving, is an under-recognized component of health literacy. Numeracy has been shown to influence performance of health tasks in non-hemophilia populations. Little is known about numeracy in the hemophilia population. Since hemophilia treatment requires understanding of numerical concepts to manage factor replacement, it is likely that numeracy also influences performance of health tasks by patients with hemophilia. A greater understanding of numeracy status and the characteristics influencing numeracy in the hemophilia population may allow healthcare providers to better influence health task performance. The objective of this study is to explore numeracy in the hemophilia population using two different tests of numeracy and to evaluate characteristics that are associated with low numeracy. Methods: Using a cross-sectional design, adults with moderate or severe hemophilia A or B who spoke and read English were enrolled at their annual visit at the Emory/Children's Health Care of Atlanta Hemophilia Treatment Center (HTC). Numeracy was measured using the validated Schwartz Woloshin (SW) test requiring answers in words and the unvalidated stick figure test requiring answers using images. Subjects were considered numerate with the SW numeracy test if all three questions were answered correctly or with the stick figure numeracy test if all four questions were answered correctly. Demographic and socioeconomic characteristics collected included age, race, ethnicity, household income (more or less than $50,000), level of education completed (more or less than completion of college), and duration of time followed at this HTC. Clinical information including type and severity of hemophilia, history of viral infections, history of depression, and use of chronic medication were abstracted from the medical records. Descriptive statistics of each variable and bivariate associations between numerate status and each dependent variable were calculated. Multivariable modeling using logistic regression was performed using the validated SW numeracy test as the dependent variable. Results: Of 91 enrolled participants with complete data, all were men. Most had hemophilia A [n=82 (90%)] and severe disease [69 (76%)]. Median age was 34 years [interquartile range (IQR) 18]. Sixty-three (69%) were Caucasian; 5 (6%) were Hispanic; 55 (61%) reported income of

Description: Background: Acute chest syndrome (ACS) is one of the most feared complications of sickle cell disease. This syndrome is defined by a clinical constellation of symptoms including fever, infiltrate on lung imaging and hypoxia. ACS is a well-known complication to occur early in the hospitalization of patients for sickle cell vaso-occlusive pain crises (VOP). It is hard to differentiate from pneumonia and its rate of occurrence has not been well characterized. Management of ACS includes exchange or simple transfusion, depending on the severity of its presentation, along with critical care support and pain control. Early recognition of this clinical syndrome can lead to better management and outcomes for patients with sickle cell disease. Methods: The Emory University Georgia Comprehensive Sickle Cell Center at Grady Health System has 1073 sickle cell patients and provides a 24/7 acute care unit (ACU) for the evaluation of patients presenting with pain crises. We conducted a retrospective review of all the ACS cases reported at the Center for the last 5 years. Results: The number of sickle cell patient-visits in the ACC was 4409. 30 cases of ACS were confirmed on retrospective review. The rate of ACS was 6.8 per 1000 patient visits. The median age was 39, and the patients' phenotypes 89% SS and 11% SC. Of the patients evaluated, 18% were smokers, 43% were taking hydroxyurea and 89% were taking folic acid. Seventy five percent of the patients were successfully managed with supportive care only, while the remaining 25% required red cell exchanges. On admission, the median white cell count (WBC), platelet count, LDH and reticulocyte count were increased above their upper limits of normal. Eighteen percent of the patients had a d-dimer checked and the median was well above the range of normal (6097). Table 1 shows the patient characteristics and data. Discussion: The rate of ACS over the last 5 years at our sickle cell center is well below historic reports of ACS. We believe that several factors explain our observation. Because our 24/7 ACU is staffed with providers who know their patients' histories and treatment plans and are experienced in the management of VOP, we postulate that our comprehensive management approach involving the rapid and appropriate initiation of intravenous fluids, the administration of pain medications along with antibiotics, and the use of anti-inflammatory medications contribute to the truncation of the sickle cell-related inflammatory state and the prevention of serious complications such as ACS. The fact that inflammatory markers (WBC, Platelet count, LDH) were initially elevated is to be expected in the setting of VOP. What is remarkable is the high percentage of good patient outcomes associated with our comprehensive management approach. This retrospective report should be validated in a prospective study that compares the results of the management of all acute care VOP visits delivered in a 24/7 ACU with the management of acute care VOP delivered in a general emergency room. Such a study should include the collection of laboratory and clinical data that is typically abnormal in patients with VOP. If a significantly decreased rate of ACS rate is observed when 24/7 ACU care is provided, then the institution of 24/7 acute care units would be warranted on a national level to improve the survival of patients with sickle cell disease. Table 1. Characteristics of patients studied Age median (range) 39 (19-58) Gender Females 14/ males 14 Genotype n (%) SC 3 (11%)/ SS 25 (89%) Smoking (%) 18 % Hydrea use (%) 43% Folic acid use (%) 89% Management n (%) Exchanged 7 (25%)/ supportive 21 (75%) Hemoglobin median (range) 10.4 (4.9-11.1) White count median (range) 18.6 (6.1-47) Platelet median (range) 492 (79-646) Reticuolcyte count median (range) 300.6 (151-597) LDH median (range) 677 (238-2287) D-dimer % patients/ median (range) 18%/ 6097 (609-33370) Ferritin % patients/ median (range) 11%/ 2844 (335.2-6060) Total/direct bilirubin median (range) 3.7/1.3 (1-19/0.2-4.9) Creatinine median (range) 1.3 (0.5-3.5) AST/ALT median (range) 59/49 (21-110/12-118) Disclosures No relevant conflicts of interest to declare.

Description: Background: Distress can affect a patient's ability to cope with and manage disease. Patients may feel uncomfortable initiating conversations about distress with their provider. Systematic screening for distress in patients with bleeding disorders may help identify distressed patients and guide treatment services planning. Objectives: Describe prevalence of distress in adult patients with bleeding disorders and the specific problems, health characteristics, and behaviors most associated with distress in this population. Methods: Patients who attended the Emory Comprehensive Bleeding Disorder Clinic for a regularly scheduled visit between January 1st, 2012 through February 28th, 2014 and who completed a distress screen, pain screen, and clinic questionnaire during the visit were evaluated cross-sectionally. Distress was measured by the National Comprehensive Cancer Network Distress Management Tool which allowed patients to rate recent distress on a 0-10 point scale and also asked patients to identify from a checklist whether any of 38 specific issues among 5 different categories were recently problematic. A distress rating of 5 or more was categorized as high distress, 1-4 as mild/moderate distress, and 0 as no distress. Pain was measured by the Brief Pain Inventory Short Form which asked patients to rate their various pain types on 0-10 point scales. Patients reported current behavioral and demographic information including employment status, alcohol, and tobacco use on the clinic questionnaire. Depressive symptoms were measured on a 0-6 point scale using the Patient Health Questionnaire-2 screening tool. Primary diagnosis, age, prophylaxis use, and HIV and HCV status were taken from medical records. Unadjusted logistic regression models to identify predictors of high distress were used. Adjusted logistic regression models that controlled for possible confounders were used to examine whether diagnosis and employment were associated with high distress. In the adjusted model examining employment, because depression and pain were collinear, we ran two models; one including depression and one including pain. Results: Of the 168 patients who formed the cohort, most were male (69%) and most had hemophilia as the primary diagnosis (67%). Nearly three-quarters identified as White race with one-quarter Black or African-American. Average patient age was 36 years and ranged from 18 to 83 years. HCV and HIV prevalence in the cohort were 35% and 13%, respectively. High distress prevalence (distress rating ≥ 5) was 31.6% (Figure 1). Patients who reported at least one emotional concern from the problem checklist were more likely to report high distress (Figure 2). In unadjusted analyses, patients who were older, unemployed or disabled, required assistance in daily life activities, reported opioid use, used crutches, had higher depressive symptoms, exercised less, and who reported high pain levels were more likely to report high distress. Diagnosis, gender, race, alcohol consumption, tobacco use, prophylaxis use, and HIV and HCV status were not associated with high distress. Unemployment, disability, higher depression symptoms, and high pain were associated with high distress in multivariate models (Table 1). Conclusions: Nearly one-third of patients with bleeding disorders reported high distress while an additional 40% reported mild/moderate distress. Further study is needed to determine if high distress impacts clinical outcomes of patients with bleeding disorders as has been demonstrated in other chronic disorders. Table 1 Multivariate analysis results Adjusted Odds Ratios for High Distress (95% CI) Predictor Model 1 (n=148) Model 2 (n=126) Model 3 (n=137) Diagnosis Severe/moderate hemophilia 1.0 Mild hemophilia 1.91 (0.53 6.85) Other bleeding disorders 1.02 (0.28 3.74) Employment Work full-time 1.0 1.0 Student, part-time, retired, homemaker, other 0.51 (0.14 1.86) 0.29 (0.07 1.18) Unemployed 6.15 (1.33 28.44) 5.49 (1.09 27.82) Disabled 3.41 (1.08 10.78) 2.37 (0.64 8.87) Age 1.02 (0.99 1.05) 1.02 (0.99 1.05) 1.02 (0.99 1.06) Pain Score 0 (no pain) 1.0 1-4 (low pain) 1.12 (0.34 3.73) 5-10 (high pain) 4.85 (1.29 18.33) Depression score 2.61 (1.78 3.83) 2.29 (1.52 3.45) All models also adjusted for race and HIV/HCV positivity Figure 1 Figure 1. Figure 2 Figure 2. Disclosures No relevant conflicts of interest to declare.

Description: Background: Health literacy (HL) is the degree to which individuals have the capacity to obtain, process, and understand basic health information and services needed to make appropriate health decisions. Numeracy, a component of HL, is defined as the ability to handle numerical concepts. Both low HL and low numeracy have been associated with poor adherence to treatment regimens in a variety of chronic diseases. For the management of hemophilia, a chronic bleeding disorder, adherence to treatment regimens can be difficult and requires completion of multiple complex tasks. We hypothesized that lower HL and lower numeracy are associated with decreased adherence to treatment in persons with hemophilia (PWH). A secondary aim of this study was to evaluate other demographic and clinical characteristics that may be associated with adherence. Methods: In a cross-sectional study, adult PWH completed questionnaires to assess the main outcome variable, adherence measured by the validated hemophilia regimen treatment adherence scale (VERITAS) and the primary independent variables, HL as measured by the shortened Test of Functional Health Literacy in Adults (sTOFHLA) and numeracy as measured by the Schwartz-Woloshin questions. Other potential independent or confounding variables including general demographic and clinical information, the General Self-Efficacy (GSE) Scale, the Wake Forest Physician Trust Scale (WFPTS), and the Haem-A-QoL were also collected. Analysis proceeded from descriptive statistics to bivariable associations using simple linear regression and multivariable analysis using multiple linear regression. Results: All were men (n=99); 91% had hemophilia A and 78% had severe disease. Mean age was 34 years (standard deviation (SD) 11.5). White race was reported by 69% and 6% were Hispanic. Income was reported to be ≥$50,000 in 39%; and 37% had received an undergraduate degree or higher. Mean length of time seen at the Hemophilia Treatment Center (HTC) was 16.0 years (SD 11). HIV infection was noted in 26%, and 59% were HCV positive; depression history was reported in 21%; 49% infused replacement factor prophylactically. Most of the study population (95%) had high HL; but only 23% were numerate. The mean VERITAS-Pro was 45.6 (SD 12.7) and mean VERITAS-PRN was 51.0 (SD 11.2) with a lower score indicating greater adherence. On bivariable analysis, adherence was significantly associated with depression history, GSE score, WFPTS score, and Haem-A-QoL score (p 〈 0.05). On multivariable analysis, HL score and numerate status were not significantly associated with adherence (see Table). Being on any chronic medication, longer time seen at HTC, higher physician trust, and better quality of life were significantly associated with higher adherence. Depression history was significantly associated with lower adherence. This model overall accounted for a moderate proportion of the variability of adherence between subjects (adjusted R2 = 0.296). Conclusion: In this study population, HL and numeracy were not associated with adherence. A large majority of the PWH in our study were health literate; however, the study population was limited to those who made it to their scheduled HTC appointments and were sufficiently on time to allow completion of study questionnaires which may have biased this study toward a more literate population. This study population did show that being on any chronic medication, longer time seen at the HTC, higher physician trust, and better quality of life were significantly associated with better adherence. Depression history was significantly associated with lower adherence. These factors provide a better understanding of characteristics that influence adherence and may prove important for optimizing the care of PWH. Table: Multivariable Linear Regression of the Association of Adherence with Predictors (n=91) Predictor Beta Coefficient Standard Error p-value* sTOFHLA score -0.09 0.22 0.67 Numerate 1.04 3.01 0.73 On chronic medication(s) -5.13 2.47 0.041 Depression history 6.89 3.02 0.025 Time seen at HTC -0.27 0.11 0.022 WFPTS score -0.51 0.18 0.006 Haem-A-QoL score 0.12 0.038 0.0024 *Significant if 〈 0.05 sTOFHLA: The higher the score, the better the HL; WFPTS: The higher the score, the higher the physician trust Haem-A-QoL: The lower the score, the better the quality of life Disclosures No relevant conflicts of interest to declare.

Description: Background: Sickle cell patients suffer from painful vaso-occlusive episodes (VOE) that interrupt patients' lives and productivity and lead to emergency department visits and hospitalizations and, on occasion, death. The VOE in sickle cell disease (SCD) accounts for 90% of hospital admissions for patients with SCD and constitute a financial burden on both patients and hospitals. Efforts to prevent pain crises have failed to establish a causal relationship in about 40% of reported cases. The remaining 60% of known causes of VOE are related to dehydration, febrile illness, and infections. The Emory University Sickle Cell Center at Grady Memorial hospital has been providing specialized services for SCD patients for 30 years. The center includes a 24/7 acute care unit (ACU) that is staffed by SCD providers who specialize in the management of VOE. The patients are started on intravenous narcotics and fluids within 30 minutes of presenting to the ACC. After eight hours of management, the patient is then either discharged home if the VOE is controlled or admitted to the hospital for continued management. Annually, around 3000 ACU visits are recorded with a 17% hospital admission rate. When patients are questioned on the reason for their presentation, “weather change” is frequently reported as the trigger of the VOE. A review of the literature reveals scant data to support the hypothesis that weather-related changes trigger sickle cell pain crises. Methods: In a retrospective evaluation of patient visits over the last 4 years, we identified temperature and humidity measurements for that period of time and attempted to correlate them with the frequency of ACU visits. We used the “weatherspark” website that records weather changes for the city of Atlanta because 〉 90% of our patients reside in the city. Results: The four-year review of weather data and ACU visits did not show a direct correlation between graphs of temperature and humidity, and the number of ACU visits. The annual number of visits to the ACU was 2930, 2467, 3195 and 3370 for the years 2011, 2012, 2013 and 2014 respectively. The average admission rate was 16.6% overall, and the admission rate in the years 2011, 2012, 2013 and 2014 was 12.4%, 14.9%, 19.6% and 19.6% respectively. The attached figure has an example of the year 2013 charts for temperature, humidity, sickle cell acute care visits and hospital admission graphs. Conclusion: The retrospective analysis does not reflect a direct correlation between the temperature and humidity graphs and the number of ACU visits and hospital admissions. Given the volume of data analyzed, it is unlikely that there is any correlation between temperature and humidity variations and either acute care visits or admissions. Atlanta, GA- ACU visits, Hospital admissions, Temperature and Humidity Charts for 2013: DATA from weatherspark.com Figure 1. Figure 1. Figure 2. Figure 2. Figure 3. Figure 3. Disclosures No relevant conflicts of interest to declare.