Impact of a Regional Pediatric Hematology/Oncology Fellowship Program in Guatemala.

PURPOSE: This study aimed to describe and assess the regional experience of a pediatric hematology/oncology fellowship program based in Guatemala. METHODS: The Unidad Nacional de Oncología Pediátrica (UNOP) in Guatemala City, Guatemala, is the only hospital in Central America dedicated exclusively to childhood and adolescent cancer. To address the regional need for specialists, a fellowship program in pediatric hematology/oncology was launched in 2003. The UNOP fellowship program comprises 3 years of training. Although the program is based at UNOP, it also includes rotations locally and internationally to enhance clinical exposure. The curriculum is based on international standards to cover clinical expertise, research, professionalism, communication, and health advocacy. Trainees are selected according to country or facility-level need for pediatric hematologists/oncologists, with a plan for them to be hired immediately after completing their training. RESULTS: Forty physicians from 10 countries in Latin America have completed training. In addition, there are currently 13 fellows from five countries in training. Of the graduates, 39 (98%) are now practicing in pediatric hematology/oncology in Latin America. Moreover, many of them have leadership positions within their institutions and participate in research, advocacy, and policy making. Graduates from the UNOP program contribute to institutions by providing care for an increasing number of patients with pediatric cancer. The UNOP program is the first pediatric hematology/oncology fellowship program in the world to be accredited by Accreditation Council for Graduate Medical Education-International, an international body accrediting clinical training programs. CONCLUSION: The UNOP program has trained specialists to increase the available care for children with cancer in Latin America. This regional approach to specialist training can maximize resources and serve as a model for other programs and regions.

Preventing Job Burnout: Could Workplace Support Protect Maternal and Child Health Professionals Who Are Doing Public Health Equity Work?

PURPOSE: To assess the potential of workplace support to protect public health equity workers against job burnout and to identify key workplace support components. DESCRIPTION: This mixed-methods, explanatory sequential study analyzed survey and interview data collected between August 2020 and June 2021. Participants included governmental and non-governmental public health employees whose programs largely focus on Maternal and Child Health populations and who reported that their jobs involved working to reduce health inequities ("equity work"). Regression analysis tested the effect of emotional labor on job burnout, and whether workplace support modified that effect. Qualitative analysis of interview transcripts explored possible components of needed workplace support. ASSESSMENT: Emotional labor was positively associated with job burnout (p < .001), and there was a significant negative interaction between emotional labor and workplace support, meaning workplace support appeared to reduce the effect of emotional labor on burnout (p = .036). Qualitative analysis identified four support components: peer-to-peer mentoring connections, workplace accommodations, engaged and empathetic supervision, and mental health resources. CONCLUSION: Workplace support is associated with reduced job burnout for public health equity workers, especially those whose jobs involve high levels of emotional labor. Few public health employers are providing needed emotional supports for their equity workers, but certain supports appear to be helpful in reducing job burnout.

Understanding the Emotional Labor of Public Health Equity Work: a Mixed Methods Study.

BACKGROUND: Rectifying historic race-based health inequities depends on a resilient public health workforce to implement change and dismantle systemic racism in varied organizations and community contexts. Yet, public health equity workers may be vulnerable to job burnout because personal investment in the continual struggle against inequality exacts an emotional toll. Our study sought to quantify the presence of emotional labor in public health equity work and better understand its dimensions. METHODS: We conducted a mixed methods study of public health equity workers focused on maternal and child health in the USA. Participants completed a survey on the emotional demands of their public health equity work. A subset of survey respondents was interviewed to gain a better understanding of the emotional toll and support received to cope. RESULTS: Public health equity work was found to involve high levels of emotional labor (M = 5.61, range = 1-7). A positive association was noted between personal efficacy (i.e., belief in one's ability to do equity work well) and increased job satisfaction. However, burnout increased when equity workers did not receive adequate support for their emotional labor. Qualitative analysis revealed eight themes depicting the emotional burden, benefits and drawbacks, and coping strategies of public health equity work. CONCLUSIONS: Public health equity workers report high degrees of emotional labor and inadequate workplace support to cope with the demands. In our study, workplace support was associated with higher job satisfaction and lower burnout. Research is urgently needed to develop and scale an effective model to support public health equity workers.

Validity and reliability of the Colorado Adult Joint Assessment Scale in adults with moderate-severe hemophilia A.

BACKGROUND: The Colorado Adult Joint Assessment Scale (CAJAS) is designed to assess joint health in adults with hemophilia. The CAJAS comprises nine items (swelling, muscle atrophy, axial deformity, crepitus, range of motion, contracture, instability, strength, gait) and assesses six joints. OBJECTIVE: To assess CAJAS content validity and psychometric properties. PATIENTS/METHODS: Data were obtained from the Trial to Evaluate the Effect of Secondary Prophylaxis With rFVIII Therapy in Severe Hemophilia A Adult and/or Adolescent Subjects Compared to That of Episodic Treatment (SPINART) study and a separate CAJAS validation study. CAJAS assessments in SPINART were performed by physical therapists (PTs) from the United States, Romania, Bulgaria, and Argentina. In the validation study, content validity was assessed from interviews with six PTs at three US hemophilia centers; cultural equivalence was assessed with seven non-US PTs from SPINART. Reliability data were collected from 30 subjects at four US centers. Test-retest reliability was evaluated by having the same PT perform CAJAS examinations at two visits, 7-10 days apart. Inter-rater reliability was assessed by comparing CAJAS scores of two different PTs performing separate examinations of the same patient several hours apart at the same visit. Psychometric properties were assessed using SPINART and validation study data. RESULTS: The CAJAS demonstrated good content validity. Test-retest reliability was high (intraclass correlation coefficient, 0.98), as was inter-rater reliability (intraclass correlation coefficient, 0.88). Internal consistency reliability was strong (α = .90). The CAJAS demonstrated good convergent/divergent validity, known-groups validity, and ability to detect change. CONCLUSIONS: The CAJAS is a valid and reliable measure of joint health in adults with moderate-severe hemophilia and is appropriate for use in clinical practice.

Self-management of heart failure in dementia and cognitive impairment: a systematic review.

BACKGROUND: The cornerstone of effective management in heart failure (HF) is the ability to self-care. Aims include i) To determine factors influencing self-care in HF patients with cognitive impairment (CI) and ii) to determine the influence of cognitive domains on self-care in patients with HF and CI. METHODS: MEDLINE, CINAHL, EMBASE, EBSCOHost, PsychINFO, ProQuest Research Library, Health Technology Assessment Database, The Cochrane Library, Web of Science and Scopus databases were systematically searched. Original research describing the relationship between cognition and HF self-care in community-dwelling older persons with dementia/CI in English, published in a peer-reviewed journal from 1stJanuary(2000)-22ndMarch(2016) was identified. Study and population characteristics, data sources, self-care processes, methods of cognitive assessment, cognitive domains affected, study outcomes, impact of impairment, and other risk factors of self-care impairment were abstracted by two reviewers. RESULTS: Of 10,688 studies identified, 14 met the inclusion criteria. Patients with HF and CI ranged from 14 to 73%. Where reported, self-care maintenance adequacy ranged from 50 to 61%; self-care management adequacy ranged from 14 to 36% and self-care confidence adequacy ranged from 0 to 44% on the Self-care of Heart Failure Index (SCHFI). All but one study predicted poor self-care ability according to poor outcome on cognitive testing. Additionally, specific cognitive domain deficits impaired self-care. Subjects with lower cognitive scores were less likely to seek assistance while subjects with depression had poor self-care abilities. CONCLUSIONS: Clinicians must consider the type and severity of impairments in cognitive domains to tailor management. Awareness of depression, self-confidence and support access may modulate self-care ability.

Differences in thrombotic risk factors in black and white women with adverse pregnancy outcome.

INTRODUCTION: Black women have an increased risk of adverse pregnancy outcomes and the characteristics of thrombotic risk factors in this population are unknown. The objective of this study was to examine the racial differences in thrombotic risk factors among women with adverse pregnancy outcomes. METHODS: Uniform data were collected in women with adverse pregnancy outcomes (pregnancy losses, intrauterine growth restriction (IUGR), prematurity, placental abruption and preeclampsia) referred to Thrombosis Network Centers funded by the Centers for Disease Control and Prevention (CDC). RESULTS: Among 343 white and 66 black women seen for adverse pregnancy outcomes, protein S and antithrombin deficiencies were more common in black women. The prevalence of diagnosed thrombophilia was higher among whites compared to blacks largely due to Factor V Leiden mutation. The prevalence of a personal history of venous thromboembolism (VTE) did not differ significantly by race. A family history of VTE, thrombophilia, and stroke or myocardial infarction (MI) was higher among whites. Black women had a higher body mass index, and a higher prevalence of hypertension, while the prevalence of sickle cell disease was approximately 27 fold higher compared to the general US black population. CONCLUSIONS: Thrombotic risk factors differ significantly in white and black women with adverse pregnancy outcomes. Such differences highlight the importance of considering race separately when assessing thrombotic risk factors for adverse pregnancy outcomes.

Update on treatment regimens: prophylaxis versus on-demand therapy.

Prophylaxis, or the routine scheduled replacement of clotting factor concentrate in patients with hemophilia, greatly decreases episodes of joint hemorrhage and effectively prevents the development of chronic joint arthropathy and disability. Despite clear evidence of its effectiveness and the fact that it is recommended by international authorities, prophylaxis still is not widely accepted in the hemophilia community. In the United States, approximately half of children with severe hemophilia A and one third of those with hemophilia B are on prophylactic regimens. Factors limiting acceptance include the need for venous access, factor availability, poor acceptance of injection therapy, safety concerns, cost, and a perceived lack of need. Questions remaining to be answered include the reasons for inter-individual variability in bleeding patterns, predictors for and reversibility of joint damage, and the optimal regimen for prophylaxis. A need exists for validated and standardized outcome measures in future research, as well as for research into factors that affect families' adherence to prophylactic regimens for their children.

Advances in care of children with hemophilia.

Care for children with severe hemophilia has moved from pediatric hospital wards and rehabilitation services to the home, school, and community. Advances in hemophilia are due largely to the development of specialized hemophilia treatment centers, which created a system of comprehensive care and focused healthcare efforts on prevention and education. Parallel advances in coagulation resulted in identification of clotting factors VIII and IX, elucidation of the protein molecular and biochemical structures and functions, sequencing of their respective genes and transfer of the human genes for production of proteins by recombinant technology, and development of gene therapy. The tragedy of the human immunodeficiency virus and hepatitis C raised awareness in patients as well as healthcare providers of the vulnerability of blood products to viral contamination and spurred progress in science leading to viral inactivation of purified proteins. Concomitantly, physicians treating bleeding episodes in the clinic investigated pharmacokinetics and pharmacoeconomics of various strategies of clotting factor replacement. The observation that trough factor levels as low as 1 to 2% were adequate to prevent most bleeding episodes led to current prophylactic regimens that allow boys to participate fully in school and community activities while factor concentrate is infused at home on a regular schedule. Currently, children with hemophilia look forward to a normal life expectancy and excellent health-related quality of life. Physician and community partnerships through research and advocacy societies have accelerated clinical advancements as well as extension of treatment to developing countries. The future of hemophilia promises a cure with gene therapy. Given the past accomplishments in hemophilia, a long-term solution to replacement of the genetically deficient protein lies on the horizon.

32P Radiosynoviorthesis in children with hemophilia.

BACKGROUND: This study was performed to prospectively evaluate the safety, efficacy, and cost of injecting P-colloid into joints of children with hemophilia and synovitis to decrease the rate of joint bleeding. PATIENTS AND METHODS: Eligibility included a diagnosis of hemophilia, history of more than six hemorrhages into a joint within a 6-month period, and evidence of synovitis by objective imaging. With written, informed consent, 0.25 to 1.0 mCi of P-colloid was injected into the problem joints. Safety was monitored by external beta-scanning and physical assessment. Efficacy was determined by analysis of the change in joint hemorrhage frequency from 6 months before and up to 96 months after the injection using a signed-rank test. Physical assessment and pain assessment were analyzed similarly using values obtained within 1 week before and 6 months after the radiosynoviorthesis. Cost was modeled using charges from the authors' institution in relation to existing alternative therapies. RESULTS: One hundred injections were given into 91 joints in 59 children. Seven children had high-titer neutralizing antibodies to factor VIII or IX. Nine children were infected with HIV. Joints injected included 44 ankles, 19 knees, 27 elbows, and 1 shoulder. Nine joints required reinjection. All children showed a significant decrease in bleeding rate (P < 0.0001) and pain (P = 0.03), with improved physical function (P = 0.02). In one child acute lymphocytic leukemia developed, but it was judged unrelated to the two P injections that he had received 3 and 10 months before the leukemia diagnosis. There were no cases of bleeding, infection, or inflammation caused by the injection. Cost was substantially less than medical and surgical alternatives. CONCLUSIONS: Radiosynoviorthesis is effective in limiting the frequency of joint hemorrhage, decreasing pain and improving function in children with hemophilia. However, long-term safety studies are needed.