Assessment of the Use of Non-Pharmacological Methods for Managing Depression in Patients with Myotonic Dystrophy (DM) and Facioscapulohumeral Muscular Dystrophy (FSHD)

Background: Myotonic dystrophy (DM) and facioscapulohumeral muscular dystrophy (FSHD) are two types of muscular dystrophies with multi-system manifestations. Purpose: The purpose of this study was to determine 1: the prevalence of depression in patients with myotonic dystrophy (DM) and facioscapulohumeral muscular dystrophy (FSHD) and 2: which non-pharmacological methods DM and FSHD patients are using to manage their depression. Methods: A survey was conducted using the online system, Qualtrics. The voluntary and anonymous survey was emailed to 1,205 eligible patients from the National Registry for DM and FSHD Patients and Family Members at the University of Rochester. Participants were at least 18 years old, a member of the registry, and diagnosed with DM or FSHD. The 65 question survey included questions on basic demographic information, depression diagnosis, medication use and effectiveness, and non-pharmacological management. Surveys were collected between May 2017 and August 2017 and the responses were analyzed and compared to the general population. The study was approved by the St. John Fisher College Institutional Review Board and the Registry Scientific Advisory Committee. Results: Of the 1,205 surveys that were sent, 466 patients responded. A total of 46 percent of patients had DM (DM1 30 percent, n=138/460 and DM2 16 percent, n=75/460) and 48 percent (n=223/460) of respondents had FSHD. Of the study respondents, 34 percent (n=150/436) reported being diagnosed with depression, while 8 percent (n=24/294) feel depressed, but haven’t been diagnosed. Non-pharmacological techniques used by patients who were diagnosed with depression or feel that they are depressed included: exercise (33 percent, n=57/150), relaxation techniques once per week (51 percent, n=50/98), and visiting a counselor or therapist once per week (11 percent, n=4/37). The most common type of relaxation technique used was meditation (52 percent, n=77/147) followed by yoga (18 percent, n=24/147). In conclusion, 32 percent (n=34/107) stated that relaxation techniques helped them, and 49 percent (n=52/107) stated that relaxation techniques may have helped them. Conclusion: Patients with both DM and FSHD have been diagnosed with depression. To manage their depression, and similar to what occurs in the general population, DM and FSHD patients are using a combination of both pharmacologic and non-pharmacologic strategies. DM and FSHD patients also believe that these non-pharmacologic methods, which include exercise, counseling, and relaxation techniques are helpful in their managing depression

Analyzing Pain Medication Use and Adherence in Patients with Myotonic Dystrophy and Facioscapulohumeral Dystrophy

Objectives: Myotonic dystrophy (DM) and facioscapulohumeral dystrophy (FSHD) are two of the most common muscular dystrophies in adults. It has been reported that patients with these two disorders may suffer from pain and inadequate pain management. The purpose of this study is to analyze the current use of pain medications and develop a survey to assess pain medication use and adherence in this patient population. Methods: Patients registered in the National Registry for DM and FSHD at the University of Rochester were surveyed on pain medication use and the most significant problem of their disease. After analysis of these surveys, an additional survey was created that contained questions specifically on pain medication use, adherence, and general questions about the patients’ pain. Questions for the survey were devised from previous studies on medication adherence, questionnaires on medication adherence and beliefs, and questions from the Brief Pain Inventory. Results: In the first survey, pain medication was used by 34% of the survey respondents (n=519/1527). Specifically, NSAIDs were used by 23.5% and opioids were used by 4% of survey respondents. In a separate survey, pain was reported as the most burdensome problem by 8% of patients (n= 27/355). This was greater than problems related with balance (n=10/355), fatigue (n=20/355), or gastrointestinal distress (n=8/355). Conclusion: Despite the low reported response that pain was the most significant problem associated with their disease, the prevalence of pain medication use indicates that further study into the impact of pain and pain medication use in this patient population is warranted. Specifically, investigating questions about adherence, use, and beliefs toward pain medication will lead to an increased understanding of pain and its treatment in this patient population. This knowledge can lead to the ability of pharmacists to optimize pain management and to reduce adverse reactions to pain medications

Course-Level Curriculum Map Pilot Program

Objective: To pilot a newly developed course level curriculum map that documents course learning outcomes, corresponding teaching activities, assessments, student achievement on assessments, and evidence-based changes made subsequently at the course level. Method: The newly developed course level curriculum map was piloted in twelve courses involving nine faculty members. Faculty either retroactively recorded data or utilized the map during the semester. At the conclusion of the pilot period, a focus group was conducted during which seven of the faculty members shared experiences and recommendations. Focus group comments were recorded by three people. Recorders’ notes were compared for accuracy and completeness. Notes were analyzed using ATLAS.ti, a qualitative research program. Results: Qualitative analysis of focus group notes yielded four principal comment codes: learning outcomes, changing activities, documenting changes, and changing assessments. These four main codes document that faculty believe work is needed on writing better course learning outcomes, creating more appropriately matched course activities, better documenting data-driven changes that occur at the course level, and developing appropriate assessments. Implications: Faculty comments support school-wide adoption of course level curriculum mapping. They also suggest a need to provide faculty development on writing precise learning outcomes and mapping them to specific classroom activities to support outcomes achievement. Additionally, curricular changes made at the course level need to be carefully documented and linked to appropriate evidence derived from assessments. The value of the map for inclusion in the dossier for promotion and tenure was also noted

A Transgender Health Care Panel Discussion in a Required Diversity Course

OBJECTIVES: To examine the impact of a panel discussion on transgender health care on first-year (P1) pharmacy students\u27 knowledge and understanding of transgender experiences in an Introduction to Diversity course. DESIGN: The panel consisted of both transgender males and females. After panelists shared their healthcare experiences, students asked them questions in a moderated setting. Students completed evaluations on the presentation and learning outcomes. They also wrote a self-reflection paper on the experience. ASSESSMENT: Ninety-one percent of students agreed that they could describe methods for showing respect to a transgender patient and 91.0% evaluated the usefulness of the presentation to be very good or excellent. Qualitative analysis (phenomenological study) was conducted on the self-reflection papers and revealed 7 major themes. CONCLUSION: First-year students reported that they found the panel discussion to be eye opening and relevant to their pharmacy career. Our panel may serve as model for other pharmacy schools to implement

The Need for Transgender Health Content in the Pharmacy Curriculum

The article, “The Need for Transgender Health Content in the Pharmacy Curriculum,” addresses transgender patients and how they often have complex medical, psychological, and social concerns. According to the article, these patients may not only need to learn to manage complicated medication regimens, which can have significant side effects, but may also face many barriers to treatment. Some of those barriers include difficulties with insurance, fear of discrimination, lack of support, and a mistrust of the healthcare system. Given the important role that medications play in maintaining their quality of life, the article suggests that pharmacists are in a unique position to not only improve the healthcare that transgender patients receive, but also to improve their perceptions of the system. The article also talks about how current literature shows that little has been done to educate pharmacists on the specific needs of the transgender patient. Parkhill and Mathews say that the likelihood that pharmacy students will encounter transgender patients in their pharmacies will increase as more transgender individuals begin to live their lives authentically. And for that reason, they are recognizing the critical need to increase pharmacy student awareness of the healthcare issues facing this population. The article concludes that by increasing education and awareness of the barriers to healthcare that transgender patients face, students will receive the training required to care for their patients in a culturally competent way

Medication adherence in patients with myotonic dystrophy and facioscapulohumeral muscular dystrophy

Myotonic dystrophy (DM) and facioscapulohumeral muscular dystrophy (FSHD) are the two most common adult muscular dystrophies and have progressive and often disabling manifestations. Higher levels of medication adherence lead to better health outcomes, especially important to patients with DM and FSHD because of their multisystem manifestations and complexity of care. However, medication adherence has not previously been studied in a large cohort of DM type 1 (DM1), DM type 2 (DM2), and FSHD patients. The purpose of our study was to survey medication adherence and disease manifestations in patients enrolled in the NIH-supported National DM and FSHD Registry. The study was completed by 110 DM1, 49 DM2, and 193 FSHD patients. Notable comorbidities were hypertension in FSHD (44 %) and DM2 (37 %), gastroesophageal reflux disease in DM1 (24 %) and DM2 (31 %) and arrhythmias (29 %) and thyroid disease (20 %) in DM1. Each group reported high levels of adherence based on regimen complexity, medication costs, health literacy, side effect profile, and their beliefs about treatment. Only dysphagia in DM1 was reported to significantly impact medication adherence. Approximately 35 % of study patients reported polypharmacy (taking 6 or more medications). Of the patients with polypharmacy, the DM1 cohort was significantly younger (mean 55.0 years) compared to DM2 (59.0 years) and FSHD (63.2 years), and had shorter disease duration (mean 26 years) compared to FSHD (26.8 years) and DM2 (34.8 years). Future research is needed to assess techniques to ease pill swallowing in DM1 and to monitor polypharmacy and potential drug interactions in DM and FSHD

Implementing a Substances of Abuse Outreach Program in the Rochester City School District.

Objective: This program aims to provide substances of abuse education to local high school students. Methods: An outreach program, modeled after the National Institute on Drug Abuse instructional materials, was developed by student pharmacists and faculty for delivery to the Rochester City School District. Strict regulations prevented admittance into any classrooms until the program was presented to all health teachers at a district-wide meeting. Approval was given to begin working in the classrooms in April 2011. The program was first adopted in two health education classes at Edison Tech (May 2011). Information was presented to students using lecture, small group discussion, and printed materials. The topic covered, marijuana, was based on the teacher\u27s preference. A formative assessment was used to address student perceptions of the program due to the small number of students in each of the classes (n = 5–7). The teacher of record in the classroom conducted the assessment interviews. Results: Student feedback included comments such as, “We liked them and appreciate the time they took to come in,” “I didn\u27t know about pharm parties” and, “I learned a lot”. Feedback from the teacher also reflected the positive impact of the program in his classroom: “There is a perception that the outside community does not care about urbanites and this program at the very root reverses that dynamic very clearly. My hope is that we can develop an ongoing relationship.” Conclusion: The timing of program approval by the school district impeded broad implementation for spring 2011; however, plans are in place for several schools in spring 2012. The opportunity to pilot the program with Edison Tech was beneficial and provided valuable feedback for improvement. Student pharmacists are in a unique position to provide this education and are viewed as approachable and non-threatening to high school–aged students

Transposon Insertion Sequencing Elucidates Novel Gene Involvement in Susceptibility and Resistance to Phages T4 and T7 in Escherichia coli O157.

Experiments using bacteriophage (phage) to infect bacterial strains have helped define some basic genetic concepts in microbiology, but our understanding of the complexity of bacterium-phage interactions is still limited. As the global threat of antibiotic resistance continues to increase, phage therapy has reemerged as an attractive alternative or supplement to treating antibiotic-resistant bacterial infections. Further, the long-used method of phage typing to classify bacterial strains is being replaced by molecular genetic techniques. Thus, there is a growing need for a complete understanding of the precise molecular mechanisms underpinning phage-bacterium interactions to optimize phage therapy for the clinic as well as for retrospectively interpreting phage typing data on the molecular level. In this study, a genomics-based fitness assay (TraDIS) was used to identify all host genes involved in phage susceptibility and resistance for a T4 phage infecting Shiga-toxigenic Escherichia coli O157. The TraDIS results identified both established and previously unidentified genes involved in phage infection, and a subset were confirmed by site-directed mutagenesis and phenotypic testing of 14 T4 and 2 T7 phages. For the first time, the entire sap operon was implicated in phage susceptibility and, conversely, the stringent starvation protein A gene (sspA) was shown to provide phage resistance. Identifying genes involved in phage infection and replication should facilitate the selection of bespoke phage combinations to target specific bacterial pathogens.IMPORTANCE Antibiotic resistance has diminished treatment options for many common bacterial infections. Phage therapy is an alternative option that was once popularly used across Europe to kill bacteria within humans. Phage therapy acts by using highly specific viruses (called phages) that infect and lyse certain bacterial species to treat the infection. Whole-genome sequencing has allowed modernization of the investigations into phage-bacterium interactions. Here, using E. coli O157 and T4 bacteriophage as a model, we have exploited a genome-wide fitness assay to investigate all genes involved in defining phage resistance or susceptibility. This knowledge of the genetic determinants of phage resistance and susceptibility can be used to design bespoke phage combinations targeted to specific bacterial infections for successful infection eradication.This work was supported by the Wellcome Trust (grant number WT098051). A.K.C. and C.J.B. were supported by the Medical Research Council (grant number G1100100/1). D.L.G. and A.S.L. were supported by BBSRC (UKRI) funding (programme number P013740)

A global data-driven census of Salmonella small proteins and their potential functions in bacterial virulence.

Small proteins are an emerging class of gene products with diverse roles in bacterial physiology. However, a full understanding of their importance has been hampered by insufficient genome annotations and a lack of comprehensive characterization in microbes other than Escherichia coli. We have taken an integrative approach to accelerate the discovery of small proteins and their putative virulence-associated functions in Salmonella Typhimurium. We merged the annotated small proteome of Salmonella with new small proteins predicted with in silico and experimental approaches. We then exploited existing and newly generated global datasets that provide information on small open reading frame expression during infection of epithelial cells (dual RNA-seq), contribution to bacterial fitness inside macrophages (Transposon-directed insertion sequencing), and potential engagement in molecular interactions (Grad-seq). This integrative approach suggested a new role for the small protein MgrB beyond its known function in regulating PhoQ. We demonstrate a virulence and motility defect of a Salmonella ΔmgrB mutant and reveal an effect of MgrB in regulating the Salmonella transcriptome and proteome under infection-relevant conditions. Our study highlights the power of interpreting available 'omics' datasets with a focus on small proteins, and may serve as a blueprint for a data integration-based survey of small proteins in diverse bacteria

Phylogeographical analysis of the dominant multidrug-resistant H58 clade of Salmonella Typhi identifies inter- and intracontinental transmission events.

The emergence of multidrug-resistant (MDR) typhoid is a major global health threat affecting many countries where the disease is endemic. Here whole-genome sequence analysis of 1,832 Salmonella enterica serovar Typhi (S. Typhi) identifies a single dominant MDR lineage, H58, that has emerged and spread throughout Asia and Africa over the last 30 years. Our analysis identifies numerous transmissions of H58, including multiple transfers from Asia to Africa and an ongoing, unrecognized MDR epidemic within Africa itself. Notably, our analysis indicates that H58 lineages are displacing antibiotic-sensitive isolates, transforming the global population structure of this pathogen. H58 isolates can harbor a complex MDR element residing either on transmissible IncHI1 plasmids or within multiple chromosomal integration sites. We also identify new mutations that define the H58 lineage. This phylogeographical analysis provides a framework to facilitate global management of MDR typhoid and is applicable to similar MDR lineages emerging in other bacterial species