Design and Development of a Community-Based, Interdisciplinary, Collaborative Dementia Care Program.

OBJECTIVE: To describe the design, development, and baseline characteristics of enrollees of a home-based, interdisciplinary, dyadic, pilot dementia care program. DESIGN: Single-arm, dementia care intervention in partnership with primary care providers delivered by Health Coaches to persons with dementia and caregiver "dyads" and supervised by an interdisciplinary team. SETTING: Home- and virtual-based dyad support. PARTICIPANTS: Persons with mild cognitive impairment or dementia diagnosis and/or who were prescribed antidementia medications; had an identified caregiver willing to participate; were under the care of a partner primary care provider; and had health insurance through the affiliated accountable care organization (Banner Health Network). INTERVENTION: Provision of personalized dementia education and support in the home or virtually by Health Coaches supported by an interdisciplinary team. MEASUREMENTS: Cognition, function, mood, and behavior of persons with dementia; caregiver stress and program satisfaction; primary care provider satisfaction. RESULTS: Served dyads from three primary care clinics with a total of 87 dyads enrolled between December 2018 and June 2020. CONCLUSION: A pilot Dementia Care Partners demonstrated feasibility and suggested acceptability, and high satisfaction among primary care providers and caregivers.

Leg Ulcers: A Report in Patients with Hemoglobin E Beta Thalassemia and Review of the Literature in Severe Beta Thalassemia.

BACKGROUND: Leg ulcers are a frequent complication in patients with the inherited hemoglobin disorders. In thalassemia, the literature is limited, and factors associated with the development of leg ulcers in hemoglobin E (HbE) beta thalassemia, the most common form of severe beta-thalassemia worldwide, have not previously been reported. METHODS: We reviewed all available medical records of patients with HbE beta thalassemia to document the onset of leg ulcers at the 2 largest treatment centers in Sri Lanka. We reviewed the literature to identify studies reporting outcomes of interventions for ulcers in severe thalassemia. RESULTS: Of a total of 255 actively registered patients with HbE thalassemia in the 2 centers, 196 patient charts were evaluable. A leg ulcer with a documented date of onset was recorded in 45 (22%) of 196 evaluable patients, aged (mean ± SEM) 22.2 ± 1.4 years. Most had been irregularly transfused; steady-state hemoglobin was 6.4 ± 0.2 g/dL. Treatment achieving healing in 17 patients included transfusions, antibiotics, oral zinc, wound toileting, and skin grafting. CONCLUSION: Leg ulcers may be more common in HbE beta thalassemia than in other forms of thalassemia. A systematic approach to treatment will be needed to document the prevalence and factors placing such patients at risk for leg ulcers. Controlled trials to evaluate the optimal treatment of this common complication are indicated.

Pitfalls in the Diagnosis of ß-Thalassemia Intermedia.

We present case histories of three patients who had ß-thalassemia (ß-thal) trait with 'unusual severity' managed as ß-thal intermedia (ß-TI) where the basis of disease severity could not be explained with routine hematological and genetic investigations. The clinical diagnosis of 'thalassemia intermedia' was justifiable as they had a ß-thal mutation and disease severity that did not fit in with either ß-thal trait or with ß-thal major (ß-TM). As mutations of α, ß, and γ genes could not explain the unusual severity of the disease, further analysis with next-generation sequencing (NGS) for red cell diseases was carried out, which led to the diagnosis of coexisting membranopathies. This case series highlights the inherent difficulty in the diagnosis of ß-TI with certainty in some patients where the genetic basis is not clear-cut.

Transfusion-transmitted hepatitis C: A cluster of cases in transfusion-dependent thalassaemia patients in Sri Lanka.

OBJECTIVES: To report the clinical and virologic epidemiology of a recent epidemic of hepatitis C in thalassaemia patients in Sri Lanka. BACKGROUND: Transfusion-dependent thalassaemia patients remain at risk for hepatitis C virus (HCV). Here, we report a cluster of recent HCV infections in Sri Lankan thalassaemia patients and examine the phylogenetic relationship of viral sequences. METHODS: We conducted two prospective cross-sectional surveys of 513 patients in four Sri Lankan thalassaemia centres in 2014/2015 and re-surveyed one centre in 2016. We screened for anti-HCV antibodies using the CTK Biotech enzyme-linked immunosorbent assay (ELISA) kits and confirmed active infection by reverse transcription-polymerase chain reaction (RT-PCR) for HCV-RNA. HCV genomes were sequenced by unbiased target enrichment. RESULTS: Anti-HCV antibodies were found in 116/513 (22.6%) of patients initially tested. Active hepatitis C infection was found in 26 patients with no cases of active hepatitis B infection. Of 26 patients with HCV, two were infected with genotype 1(a), and the rest had 3(a). In a single centre (Ragama), 122 patients (120 new cases and two previously tested, but negative) were retested for anti-HCV antibodies. 32/122 (26.2%) patients were seropositive. Twenty-three (23/122; 18.8%) of these new cases were confirmed by HCV PCR (all genotype 3[a]). CONCLUSION: There is a significant cluster of recent HCV cases in multiply transfused thalassaemia patients in several centres in Sri Lanka. Most of the viruses shared a close phylogenetic relationship. The results are consistent with recent continuing transfusion-transmitted HCV infection. Routine surveillance for HCV of chronically transfused patients is required irrespective of screening of blood products.

Marriage patterns in Sri Lanka and the prevalence of parental consanguinity in patients with ß-thalassaemia: a cross-sectional descriptive analysis.

Consanguineous marriages potentially play an important role in the transmission of ß-thalassaemia in many communities. This study aimed to determine the rate and socio-demographic associations of consanguineous marriages and to assess the influence on the prevalence of ß-thalassaemia in Sri Lanka. Three marriage registrars from each district of Sri Lanka were randomly selected to prospectively collect data on all couples who registered their marriage during a 6-month period starting 1st July 2009. Separately, the parents of patients with ß-thalassaemia were interviewed to identify consanguinity. A total of 5255 marriages were recorded from 22 districts. The average age at marriage was 27.3 (±6.1) years for males and 24.1 (±5.7) years for females. A majority (71%) of marriages were 'love' marriages, except in the Moor community where 84% were 'arranged' marriages. Overall, the national consanguinity rate was 7.4%. It was significantly higher among ethnic Tamils (22.4%) compared with Sinhalese (3.8%) and Moors (3.2%) (p < 0.001). Consanguinity rates were also higher in 'arranged' as opposed to 'love' marriages (11.7% vs 5.6%, p < 0.001). In patients with ß-thalassaemia, the overall consanguinity rate was 14.5%; it was highest among Tamils (44%) and lowest among Sinhalese (12%). Parental consanguinity among patients with ß-thalassaemia was double the national average. Although consanguinity is not the major factor in the transmission of the disease in the country, emphasis should be given to this significant practice when conducting ß-thalassaemia prevention and awareness campaigns, especially in high-prevalence communities.

Female pediatric and adolescent genitalia trauma: a retrospective analysis of the National Trauma Data Bank.

PURPOSE: Pediatric genitalia injury represents 0.6% of all pediatric trauma. It is crucial for providers to understand whether pediatric patients are at risk for violent mechanisms, such as rape, assault, or other abuse. Therefore, we sought to perform a large database analysis of pediatric and adolescent female genitalia trauma, comparing mechanisms of injury (i.e., sexual abuse) and need for operative intervention between adolescent and pediatric cohorts. METHODS: The National Trauma Data Bank was queried (years 2007-2015) for female patients ≤ 16 years old with external genitalia (vaginal or vulvar) trauma. Two groups were compared: pediatrics (< 12 years old) and adolescents (12-16 years old). RESULTS: Out of 303,992 female patients, 3206 (1.1%) were identified to have genitalia trauma with the majority being pediatric patients (92.1%) and with injury to the vagina (62.6%). Pediatric patients with vaginal injury were less likely to be victims of rape (4.1% vs. 17.3%, p < 0.001) and assault (2.1% vs. 7.2%, p < 0.001) but more likely to be victims of other abuse (9.5% vs. 3.4%, p = 0.003). More of the adolescent patients with vaginal trauma required repair (58.7% vs. 43.2%, p < 0.001). Pediatric patients with injury to the vulva were less likely to be victims of rape (0.7% vs. 2.8%, p = 0.01) and motor vehicle accidents (4.2% vs. 11.0%, p < 0.001). CONCLUSION: Genitalia trauma occurs in 1.1% of pediatric and adolescent trauma cases with the vagina being more commonly injured compared to the vulva. Adolescent patients with vaginal injuries were more likely to be victims of rape and assault and required repair more often, while those with vulvar injuries were more likely due to motor vehicle accidents. Health care providers must be aware of these at-risk populations and the differences between them to identify female victims of violence and provide resources to assist with recovery.

The p.H63D allele of the HFE gene protects against low iron stores in Sri Lanka.

In hereditary hemochromatosis, iron overload is associated with homozygosity for the p.C282Y mutation. A second mutation, p.H63D, occurs at significant frequencies in Europe, North Africa, the Middle East and Asia. Early studies in Sri Lanka indicated that the variant had arisen independently, suggesting that it had been the subject of selective pressure. However, its role in iron absorption is unclear. In a survey of 7526 Sri Lankan secondary school students, we determined hemoglobin genotype and measured red cell indices, serum ferritin, transferrin receptor, iron zinc protoporphyrin and hepcidin. These variables were compared according to the presence or absence of the p.H63D variant in a subset of 1313 students for whom DNA samples were available. Students were classified as having low red cell indices if they had an MCV <80 fl and/or MCH <27 pg. Hetero and/or homozygosity for the p.H63D variant was more common in students with normal than low red cell indices (16.4% and 11.9% respectively; p = 0.019). Iron biomarkers and red cell indices were greater in children with the p.H63D variant than in normal and this was statistically significant for MCV (p = 0.046). Our findings suggest that selective pressure by mild iron deficiency contributes to the high frequencies of the p.H63D variant.

Interprofessional Education Among Occupational Therapy Programs: Faculty Perceptions of Challenges and Opportunities.

OBJECTIVE: We investigated occupational therapy faculty beliefs about and perceptions of interprofessional education (IPE) and to identify differences in faculty positions on IPE between programs affiliated with an on-campus academic health care center (AHC) and programs not affiliated with an on-campus AHC. METHOD: Online surveys were distributed by email to 1,466 faculty at programs accredited by the Accreditation Council for Occupational Therapy Education. The results were described using descriptive statistics and cross-tabulations. RESULTS: Faculty responses supported the need for IPE. Ethics was ranked as the most important IPE competency among both the AHC and the non-AHC groups. IPE was more commonly included in the curriculum of programs with an on-campus AHC than in the curriculum of those without an AHC. CONCLUSION: The majority of occupational therapy faculty supported the need for IPE; however, many reported limitations with faculty or time constraints as barriers to IPE.

Haemoglobin variants, iron status and anaemia in Sri Lankan adolescents with low red cell indices: A cross sectional survey.

Iron deficiency complicates the use of red cell indices to screen for carriers of haemoglobin variants in many populations. In a cross sectional survey of 7526 secondary school students from 25 districts of Sri Lanka, 1963 (26.0%) students had low red cell indices. Iron deficiency, identified by low serum ferritin, was the major identifiable cause occurring in 550/1806 (30.5%) students. Low red cell indices occurred in iron-replete students with alpha-thalassaemia including those with single alpha-globin gene deletions. Anaemia and low red cell indices were also common in beta-thalassaemia trait. An unexpected finding was that low red cell indices occurred in 713 iron-replete students with a normal haemoglobin genotype. It is common practice to prescribe iron supplements to individuals with low red cell indices. Since low red cell indices were a feature of all forms of α thalassaemia and also of iron deficiency, in areas where both conditions are common, such as Sri Lanka, it is imperative to differentiate between the two, to allow targeted administration of iron supplements and avoid the possible deleterious effects of increased iron availability in iron replete individuals with low red cell indices due to other causes such as α thalassaemia.

Health Behaviors and Preventive Healthcare Utilization Among African-American Attendees at a Faith-Based Public Health Conference: Healthy Churches 2020.

Unhealthy eating habits and physical inactivity along with lack of access to quality healthcare contribute to the marked health disparities in chronic diseases among African-Americans. Faith-based public health conferences offer a potential opportunity to improve health literacy and change health behaviors through health promotion within this population, thereby reducing health disparities. This study examined the self-reported health behaviors and preventive healthcare utilization patterns of 77 participants at a predominantly African-American faith-based public health conference, Healthy Churches 2020. A self-administered questionnaire was distributed to a sample of attendees to assess their health behaviors (diet and physical activity), preventive healthcare utilization (annual healthcare provider visits), and health-promoting activities at their places of worship. The results indicate that attendees of a faith-based public health conference have adequate preventive healthcare utilization, but suboptimal healthy behaviors. Our findings support the need for ongoing health-promoting activities with an emphasis on diet and physical activity among this population.

The evolutionary and clinical implications of the uneven distribution of the frequency of the inherited haemoglobin variants over short geographical distances.

Studies of the frequency of heterozygous carriers for common inherited diseases of haemoglobin in over 7500 adolescent children in 25 districts in Sri Lanka have disclosed a highly significant variation over very short geographical distances. A further analysis of these findings, including their relationship to the past frequency and distribution of malaria, climatic variation, altitude, ethnic origin and consanguinity rates, have provided evidence regarding the evolutionary basis for the variable distribution of these conditions over short distances. It is likely that the complex interplay between malaria and the environment, together with related ethnic and social issues, exists in many countries across the tropical belt. Hence, these observations emphasise the importance of micromapping heterozygote distributions in high-frequency countries in order to define their true burden and the facilities required for the prevention and management of the homozygous and compound heterozygous disorders that result from their interaction.

Hepcidin is suppressed by erythropoiesis in hemoglobin E ß-thalassemia and ß-thalassemia trait.

Hemoglobin E (HbE) ß-thalassemia is the most common severe thalassemia syndrome across Asia, and millions of people are carriers. Clinical heterogeneity in HbE ß-thalassemia is incompletely explained by genotype, and the interaction of phenotypic variation with hepcidin is unknown. The effect of thalassemia carriage on hepcidin is also unknown, but it could be relevant for iron supplementation programs aimed at combating anemia. In 62 of 69 Sri Lankan patients with HbE ß-thalassemia with moderate or severe phenotype, hepcidin was suppressed, and overall hepcidin inversely correlated with iron accumulation. On segregating by phenotype, there were no differences in hepcidin, erythropoiesis, or hemoglobin between severe or moderate disease, but multiple linear regression showed that erythropoiesis inversely correlated with hepcidin only in severe phenotypes. In moderate disease, no independent predictors of hepcidin were identifiable; nevertheless, the low hepcidin levels indicate a significant risk for iron overload. In a population survey of Sri Lankan schoolchildren, ß-thalassemia (but not HbE) trait was associated with increased erythropoiesis and mildly suppressed hepcidin, suggesting an enhanced propensity to accumulate iron. In summary, the influence of erythropoiesis on hepcidin suppression associates with phenotypic disease variation and pathogenesis in HbE ß-thalassemia and indicates that the epidemiology of ß-thalassemia trait requires consideration when planning public health iron interventions.

Hepcidin detects iron deficiency in Sri Lankan adolescents with a high burden of hemoglobinopathy: A diagnostic test accuracy study.

Anemia affects over 800 million women and children globally. Measurement of hepcidin as an index of iron status shows promise, but its diagnostic performance where hemoglobinopathies are prevalent is unclear. We evaluated the performance of hepcidin as a diagnostic test of iron deficiency in adolescents across Sri Lanka. We selected 2273 samples from a nationally representative cross-sectional study of 7526 secondary schoolchildren across Sri Lanka and analyzed associations between hepcidin and participant characteristics, iron indices, inflammatory markers, and hemoglobinopathy states. We evaluated the diagnostic accuracy of hepcidin as a test for iron deficiency with estimation of the AUCROC , sensitivity/specificity at each hepcidin cutoff, and calculation of the Youden Index to find the optimal threshold. Hepcidin was associated with ferritin, sTfR, and hemoglobin. The AUCROC for hepcidin as a test of iron deficiency was 0.78; hepcidin outperformed Hb and sTfR. The Youden index-predicted cutoff to detect iron deficiency (3.2 ng/mL) was similar to thresholds previously identified to predict iron utilization and identify deficiency in African populations. Neither age, sex, nor α- or ß-thalassemia trait affected diagnostic properties of hepcidin. Hepcidin pre-screening would prevent most iron-replete thalassemia carriers from receiving iron whilst still ensuring most iron deficient children were supplemented. Our data indicate that the physiological relationship between hepcidin and iron status transcends specific populations. Measurement of hepcidin in individuals or populations could establish the need for iron interventions. Am. J. Hematol. 92:196-203, 2017. © 2016 Wiley Periodicals, Inc.

Outcomes following management of squamous cell carcinoma of the scalp: A retrospective series of 235 patients treated at the Peter MacCallum Cancer Centre.

BACKGROUND: Squamous cell carcinoma of the scalp is a common clinical problem in an aging population. Despite its high incidence, little has been documented regarding treatment or outcomes. METHODS: We retrospectively analysed 235 cases treated with curative intent at Peter MaCallum Cancer Centre between 1998 and 2010. The cohort was analysed for its characteristics, management, survival and prognostic factors. RESULTS: The patients were primarily male (88%) with a median age of 79 years (range 53-98 years). There was a high proportion of immunosuppressed patients (29%) and stage T2 (48%) tumours. Management included surgery (45%), radiotherapy (28%) and surgery and adjuvant radiotherapy (26%). Median follow up from treatment was 4.5 years. Estimated 5-year overall survival (OS), disease-specific survival and progression-free survival (PFS) were 59, 94 and 51%, respectively. The 5-year cumulative incidence of local and regional relapse was 11 and 7%, respectively. There were four patients who developed distant metastases and died of their disease. Statistically significant prognostic factors identified for poor outcomes for OS and PFS were T2 stage (hazard ratio [1.7 and 2.1) and immunosuppression (HR 3.3 and 3.4). CONCLUSIONS: We conclude the presence of immunosuppression and T2 stage is prognostic for survival. Further research to establish treatment principles is warranted.

Implementation and assessment of a curriculum for bedside ultrasound training.

OBJECTIVES: This study assessed a curriculum for bedside ultrasound (US) and compared outcomes from 2 common training pathways. METHODS: The program consisted of e-learning paired with expert-led hands-on training administered to pulmonary/critical care and cardiology fellows with no prior formal training in bedside US. This "simulation-based learner" group completed a survey of attitudes and confidence before and after training, and knowledge and skills were assessed after training. The surveys and scores of the simulation-based learners were compared to the scores of "experts," who were US-trained emergency physicians, and "apprentice learners," who were intensivist physicians informally trained in bedside US on the job during fellowships. RESULTS: There was a significant difference in the self-reported level of prior training between the groups (simulation-based learners, 2.8; apprentice learners, 3.7; experts, 4.1, on a scale of 1-5 [P= .02]) but no difference in the interest level or perceived importance of bedside US. The study curriculum was successful, as shown by scores that exceeded the comparison groups in the cardiac and pulmonary courses (cardiac: simulation-based learners, 80%; apprentice learners, 73%; experts, 62% [P= .001]; pulmonary: 84%, 75%, and 72%, respectively [P =.02]). The simulation-based learners gained confidence in skills, whereas the comparison groups lost confidence after testing (P < .005); however, the simulation-based learners gained confidence in US subject areas that were not taught (abdomen [P <.002] and miscellaneous [P =.005]). CONCLUSIONS: The simulation-based learner curriculum resulted in comparable or greater knowledge and confidence in each area of US versus the comparison groups. Findings of overgeneralization of confidence highlight the importance of quality assurance and supervision in bedside US training programs.

Methemoglobinemia and ascorbate deficiency in hemoglobin E ß thalassemia: metabolic and clinical implications.

During investigations of the phenotypic diversity of hemoglobin (Hb) E ß thalassemia, a patient was encountered with persistently high levels of methemoglobin associated with a left-shift in the oxygen dissociation curve, profound ascorbate deficiency, and clinical features of scurvy; these abnormalities were corrected by treatment with vitamin C. Studies of erythropoietin production before and after treatment suggested that, as in an ascorbate-deficient murine model, the human hypoxia induction factor pathway is not totally dependent on ascorbate levels. A follow-up study of 45 patients with HbE ß thalassemia showed that methemoglobin levels were significantly increased and that there was also a significant reduction in plasma ascorbate levels. Haptoglobin levels were significantly reduced, and the high frequency of the 2.2 haptoglobin genotype may place an additional pressure on ascorbate as a free-radical scavenger in this population. There was, in addition, a highly significant correlation between methemoglobin levels, splenectomy, and factors that modify the degree of globin-chain imbalance. Because methemoglobin levels are modified by several mechanisms and may play a role in both adaptation to anemia and vascular damage, there is a strong case for its further study in other forms of thalassemia and sickle-cell anemia, particularly when splenic function is defective.

Cross Cultural Empathetic Behavior in Health Care Providers: A Review of 3 Countries.

This paper examines empathetic behavior in the United States, a strongly individualistic country, as contrasted with Hungary and Ethiopia, which are moderately individualistic and strongly collectivistic respectively. It suggests that empathy may have a wider than originally perceived application in diverse settings to combat factors of ethnic bias and discrimination that adversely impact health. Models that distinctly focus on the development of healthcare provider empathic care are needed to enable the needs of resource scarce regions of the world to be met, including pockets of the U.S. More investigation is warranted on how empathic behavior can positively impact health outcomes and disparities.

Alpha thalassaemia and extended alpha globin genes in Sri Lanka.

The α-globin genes were studied in nine families with unexplained hypochromic anaemia and in 167 patients with HbE ß thalassaemia in Sri Lanka. As well as the common deletion forms of α(+) thalassaemia three families from an ethnic minority were found to carry a novel form of α(0) thalassaemia, one family carried a previously reported form of α(0) thalassaemia, --(THAI), and five families had different forms of non-deletional thalassaemia. The patients with HbE ß thalassaemia who had co-inherited α thalassaemia all showed an extremely mild phenotype and reduced levels of HbF and there was a highly significant paucity of α(+) thalassaemia in these patients compared with the normal population. Extended α gene arrangements, including ααα, αααα and ααααα, occurred at a low frequency and were commoner in the more severe phenotypes of HbE ß thalassaemia. As well as emphasising the ameliorating effect of α thalassaemia on HbE ß thalassaemia the finding of a novel form of α(0) thalassaemia in an ethnic minority, together with an unexpected diversity of forms of non-deletion α thalassaemia in Sri Lanka, further emphasises the critical importance of micro-mapping populations for determining the frequency of clinically important forms of the disease.

Factors associated with sleep disturbance among older adults in inpatient rehabilitation facilities.

PURPOSE: The purpose of this article is to provide an overview of changing sleep patterns and common sleep disorders in older adults and to discuss treatment options of sleep disturbances within inpatient rehabilitation facilities (IRFs). METHODS: Through extensive review of the existing literature, common sleep disorders among older adults and several key factors that may impact sleep in older adults in inpatient rehabilitation facilities, such as behavioral and environmental factors, psychosocial and emotional factors, medical conditions, and medications were identified. FINDINGS: Current literature on the factors associated with sleep disturbance in older adults in IRFs is based on work with community-dwelling older adults and those in long-term care facilities. While interventions to address these disorders and research investigation key factors associated with sleep problems among older adults appear in the literature, there is very little work that applies these interventions within IRFs. CONCLUSIONS AND CLINICAL RELEVANCE: Research is needed to examine the impact of sleep problems on older adults in IRFs, including work that focuses on intervention trials to identify successful treatments for these problems and translate those approaches into practice.

Inside Looking Out: Updated Competency Model for Professional Rehabilitation Nursing Practice.

BACKGROUND: The original Association of Rehabilitation Nurses (ARN) Competency Model for Professional Rehabilitation Nursing published in 2016, and updated in 2020, provides a framework for rehabilitation nursing practice. AIM: This companion, but stand-alone, article to a 2022 publication further explicates and informs the updated Competency Model from inside looking out toward an increasing application for evidence-based practice (EBP). APPROACH: An eight-member 2020 ARN Task Force used an iterative process to review the original four domains and related competencies and came to consensus for the updated model. OUTCOME: This model provides revised competency role descriptors or behaviors that guide nurses practicing at different proficiency levels in various settings. CLINICAL RELEVANCE: The Competency Model for Professional Rehabilitation Nursing is a premier resource that can advance professional rehabilitation nursing and guide EBP, including evaluation, quality improvement, and research. The model describes the nurse's role on the intra/interprofessional team and fosters collaboration with other healthcare professionals to enhance the quality of life for those affected by disability and chronic illness. CONCLUSION: The domains and associated competencies of this model clarify nursing roles at different proficiency levels, and role descriptors reflect current practice, supporting advancement of the specialty practice of rehabilitation nursing well into the 21st century.