Lifelong impact of ENPP1 Deficiency and the early onset form of ABCC6 Deficiency from patient or caregiver perspective.

The ectonucleotide pyrophosphatase/phosphodiesterase family member 1 (ENPP1) and ATP-binding cassette subfamily C member 6 (ABCC6) proteins play a prominent role in inhibiting ectopic calcification and arterial stenosis. Patients with ENPP1 Deficiency or infant onset ABCC6 Deficiency often present with pathological calcification, narrowed blood vessels, multiorgan dysfunction and high infant mortality. The heterogenous presentation and progression is well documented. Our objective was to characterize how these morbidities lead to burden of illness and poor quality of life across ages from the patient/caregiver perspective. Patients/caregivers were interviewed via phone using Institutional Review Board-approved questionnaires. Patient-reported outcomes were collected via validated instruments. Thirty-one caregivers and 7 patients participated: infant onset ABCC6 Deficiency, n = 6 (infants/children); ENPP1 Deficiency, n = 32 (13 infants, 12 children, 7 adults). ENPP1 and ABCC6-deficient children aged <8 years and aged 8-18 years reported poor school functioning (0.69 vs 0.72 effect size, respectively) and poor physical health (0.88 vs 1, respectively). In the total ENPP1 cohort, 72% (23/32) reported bone/joint pain and/or mobility/fatigue issues. Three of seven ENPP1-deficient adults reported moderate to severe pain (>4), as measured by the Brief Pain Inventory (BPI), that interfered with daily activities despite pain medication. Top reported burdens for caregivers of infants with ABCC6/ENPP1 Deficiencies included heart-related issues and hospitalizations. Treatment/medications, and hearing loss were the highest burdens reported by caregivers/families of the pediatric ENPP1 Deficiency cohort, whereas adults reported bone/joint pain and mobility impairment as the greatest burdens. Individuals with ENPP1 Deficiency or infant onset ABCC6 Deficiency experience lifelong morbidity causing substantial physical and emotional burden to patients/caregivers.

Musculoskeletal Comorbidities and Quality of Life in ENPP1-Deficient Adults and the Response of Enthesopathy to Enzyme Replacement Therapy in Murine Models.

Ectonucleotide pyrophosphatase/phosphodiesterase 1 (ENPP1) deficiency leads to cardiovascular calcification in infancy, fibroblast growth factor 23 (FGF23)-mediated hypophosphatemic rickets in childhood, and osteomalacia in adulthood. Excessive enthesis mineralization and cervical spine fusion have been previously reported in patients with biallelic ENPP1 deficiency, but their effect on quality of life is unknown. We describe additional musculoskeletal complications in patients with ENPP1 deficiency, namely osteoarthritis and interosseous membrane ossification, and for the first time evaluate health-related quality of life (HRQoL) in patients with this disease, both subjectively via narrative report, and objectively via the Brief Pain Inventory-Short Form, and a Patient Reported Outcome Measurement Information System Physical Function (PROMIS PF) short form. Residual pain, similar in magnitude to that identified in adult patients with X-linked hypophosphatemia, was experienced by the majority of patients despite use of analgesic medications. Impairment in physical function varied from mild to severe. To assess murine ENPP1 deficiency for the presence of enthesopathy, and for the potential response to enzyme replacement therapy, we maintained Enpp1asj/asj mice on regular chow for 23 weeks and treated cohorts with either vehicle or a long-acting form of recombinant ENPP1. Enpp1asj/asj mice treated with vehicle exhibited robust calcification throughout their Achilles tendons, whereas two-thirds of those treated with ENPP1 enzyme replacement exhibited complete or partial suppression of the Achilles tendon calcification. Our combined results document that musculoskeletal complications are a significant source of morbidity in biallelic ENPP1 deficiency, a phenotype which is closely recapitulated in Enpp1asj/asj mice. Finally, we show that a long-acting form of recombinant ENPP1 prevents the development of enthesis calcification at the relatively modest dose of 0.3 mg/kg per week, suggesting that suppression of enthesopathy may be attainable upon dose escalation. © 2021 American Society for Bone and Mineral Research (ASBMR). This article has been contributed to by US Government employees and their work is in the public domain in the USA.

Student attitudes toward a web-based interprofessional education package.

UNLABELLED: The objective of this study was to examine undergraduate health science students' attitudes toward interprofessional learning using a web-based interprofessional education package. METHODS: A before and after study of student attitudes toward a web-based interprofessional education package was conducted using the 17-item Readiness for Interprofessional Learning Scale (RIPLS). Students completed the RIPLS before and after the educational package. RESULTS: Forty-six students commenced the study with 2 students failing to complete. The group contained mostly females with a mean age of 22.6 yrs. A statistically significant change was found for RIPLS subscale one, "shared learning," p=0.035. CONCLUSIONS: This study demonstrated that a web-based interprofessional education package did appear to have an impact on undergraduate students' attitudes toward interprofessional learning.

Factor structure of the Communicator Styles Measure (CSM) when used with undergraduate health science students.

BACKGROUND: It is important for educators to consider the communication skills of students enrolled in health science programmes. To date, research into this area is limited, and having measures that are valid and reliable would assist educators and researchers to complete high-quality investigations. PURPOSE: The purpose of this study was to investigate the factor structure of Communicator Styles Measure. METHOD: Data from the Communicator Styles Measure completed by 860 undergraduate health science students enrolled in eight different courses at an Australian university (response rate of 59%) were analysed using principal component analysis with varimax rotation and a cluster analysis using elementary linkage analysis. The Communicator Styles Measure is a self-report scale consisting of 40 items designed to assess ten communication styles and one's perception of his/her ability to communicate. RESULTS: Communicator Styles Measure items loaded onto five new viable factors labelled personable, energetic, confident, open and confronting. Six items of the original 40 from the Communicator Styles Measure did not load onto any factor and were therefore considered redundant. CONCLUSION: The original factor structure proposed by the Communicator Styles Measure's author was not supported, which calls into question its construct validity. However, the five new factors identified in this study may be useful for researchers and educators when assessing the communication skills of students and practitioners. Further investigation into the construct validity and reliability of the five new Communicator Styles Measure factors is recommended.

Levels of empathy in undergraduate nursing students.

Empathy and absence of prejudice and stigma are instrumental in facilitating effective nurse-patient relations. This study assessed empathy levels and regard for specific medical conditions in undergraduate nursing students. A cross-sectional study was undertaken using paper-based versions of the Jefferson Scale of Physician Empathy (JSPE) and Medical Condition Regard Scale (MCRS), along with a brief set of demographic questions. Participants reported good empathy levels on JSPE. Attitudes towards intellectual disability, chronic pain, acute mental illness and terminal illness rated well on MCRS. Attitudes towards substance abuse, however, were lower. There were no significant differences between age groups, gender or year level of study. Overall results of this study were positive. Nursing students demonstrated acceptable empathy levels. Attitudes towards patients who abuse substances highlight an area that needs both further exploration and addressing. Attitudes towards mental health diagnoses were particularly favourable given that these often attract stigma and negative attitudes.

Predictors of empathy in health science students.

UNLABELLED: The significance of both empathy and effective communication as key components in the provision of health care services is widely acknowledged. It is important, therefore, to promote those communication styles which facilitate an empathetic understanding among health science students. OBJECTIVE: To explores whether listening and communication styles are predictive of empathy among health science students. METHODS: A cross-sectional study of 860 undergraduate health science students (response rate, 59%) using paper-based versions of the Jefferson Scale of Physician Empathy-Health Professional Version, Listening Styles Profile, Communicator Styles Measure, and a brief demographic questionnaire. Two stepwise linear regression analyses were completed using the empathy construct as the dependent/criterion variable and listening and communication styles as the two sets of independent/predictor variables. RESULTS: As there was a statistically significant difference in empathy between males and females, gender was controlled for in both regression models. In first model, the People and Time listening styles were found to be predictive of empathy, accounting for 20.3% of the total variance. In the second model, both the Friendly and Relaxed communication styles were predictive of empathy, accounting for 9.7% of the total variance. CONCLUSION: The findings indicate that People and Time listening styles and the Friendly and Relaxed communication styles were significant predictors of empathy in health science students. The findings suggest that promoting effective communication among health science students may improve their ability to empathize.

Levels of empathy in undergraduate midwifery students: an Australian cross-sectional study.

OBJECTIVE: This exploratory study investigated empathy and attitudes towards specific medical conditions, two important aspects of the midwife-woman relationship, amongst undergraduate midwifery students at one university. METHOD: Fifty-two Bachelor of Midwifery students across three years of the course completed questionnaires, the Jefferson Scale of Physician Empathy-Health Professional (JSPE-HP) version and the Medical Condition Regard Scale (MCRS). Both the JSPE-HP and the MCRS are standardised, self-report questionnaires with established reliability and validity. FINDINGS: An overall respectable level of empathy was measured, which was shown to increase consistently and substantially with each year level of the student undergraduate program. Attitudes to selected diagnoses were very good with the exception being substance abuse, which received a significantly lower mean. CONCLUSION: The results of this study indicate that the Bachelor of Midwifery course, or studying midwifery, has a positive effect on students' empathy. Low results in participants' attitudes towards patients presenting with substance abuse raises concern that students may harbour prejudice that could impinge upon their professional practice and the way in which they care for these mothers. Hence, this aspect in particular may need further development within midwifery curriculum development. Further research around other conditions may extend on our understandings of empathy and attitudes in midwifery students and practitioners.

Practice education learning environments: the mismatch between perceived and preferred expectations of undergraduate health science students.

BACKGROUND: Practical hands-on learning opportunities are viewed as a vital component of the education of health science students, but there is a critical shortage of fieldwork placement experiences. It is therefore important that these clinical learning environments are well suited to students' perceptions and expectations. PURPOSE: To investigate how undergraduate students enrolled in health-related education programs view their clinical learning environments and specifically to compare students' perception of their 'actual' clinical learning environment to that of their 'preferred/ideal' clinical learning environment. METHOD: The Clinical Learning Environment Inventory (CLEI) was used to collect data from 548 undergraduate students (55% response rate) enrolled in all year levels of paramedics, midwifery, radiography and medical imaging, occupational therapy, pharmacy, nutrition and dietetics, physiotherapy and social work at Monash University via convenience sampling. Students were asked to rate their perception of the clinical learning environment at the completion of their placements using the CLEI. RESULTS: Satisfaction of the students enrolled in the health-related disciplines was closely linked with the five constructs measured by the CLEI: Personalization, Student Involvement, Task Orientation, Innovation, and Individualization. Significant differences were found between the student's perception of their 'actual' clinical learning environment and their 'ideal' clinical learning environment. CONCLUSION: The study highlights the importance of a supportive clinical learning environment that places emphasis on effective two-way communication. A thorough understanding of students' perceptions of their clinical learning environments is essential.

Communication styles of undergraduate health students.

BACKGROUND: Few empirical studies have been undertaken on the communication styles of specific health-related disciplines. The objective of this study is to identify the communication styles of undergraduate health students at an Australian university. METHODS: A cross-sectional study using a paper-based version of the Communicator Style Measure (CSM) was administered to a cohort of students enrolled in eight different undergraduate health-related courses. There were 1459 health students eligible for inclusion in the study. RESULTS: 860 students (response rate of 59%) participated in the study. Participants overall preferred the Friendly and Attentive communicator styles and gave least preference to the Contentious and Dominant styles. There was considerable similarity between participants from each of the health-related courses. There was no statistical difference in relation to communicator styles between the age of the participant or the year level they were enrolled in. CONCLUSION: These results show a preference for communicator styles which are facilitative of a client-centred approach, empathetic, and positive with interpersonal relationships. The lack of significant difference in communicator styles by year level further suggests that people disposed to such communicator styles are drawn to these health-related courses, rather than the specific field of study affecting their style.

Levels of empathy in undergraduate occupational therapy students.

Empathy is an important attribute for occupational therapists in establishing rapport and in better understanding their clients. However, empathy can be compromised by high workloads, personal stressors and pressures to demonstrate efficacy. Occupational therapists also work with patients from a variety of diagnostic groups. The objective of this study was to determine the extent of empathy and attitudes towards clients amongst undergraduate occupational therapy students at one Australian University. A cross-sectional study was undertaken using a written survey of the Jefferson Scale of Physician Empathy (JSPE) and the Medical Condition Regard Scale. Overall, a strong level of empathy was reported amongst students. Four medical conditions that occupational therapists work with (stroke, cerebral palsy, traumatic brain injury and depression) were held in high regard. Substance abuse, however, was held in comparatively low regard. Overall, the year of study appeared to have no significant impact on the students' empathy. Despite having a lower reported empathy level than found in health professions from other studies using the JSPE, occupational therapy students were found to have a good level of empathy. Of concern, however, was the bias reported against the medical condition of substance abuse, highlighting that the there may be a need to reinforce that patients from this diagnostic group are equally deserving of quality care irrespective of their clinical condition. Recommendations for future research include completing a longitudinal study of occupational therapy students' empathy levels and investigating the empathy levels of occupational therapists working with different client groups. Limitations of the study include the convenience sampling of occupational therapy students enrolled at one university which limits the generalizability of the results to groups of participants with similar characteristics.