Insights From the Transition to Competency-Based Medical Education in Neurology Programs.

Canadian neurology residency programs recently transitioned to Competency-Based Medical Education (CBME). Iterative evaluation is required to optimize CBME implementation. This study aimed to examine the variability and challenges in uptake of CBME in neurology residency programs and identify its benefits and pitfalls. Neurology residents and faculty participated in respective anonymous surveys. Common barriers to uptake were identified from both perspectives. Orientation to CBME was adequate, but workload was increased and contributed to burnout for faculty and residents. It is premature to draw conclusions regarding benefits of CBME. Future research considerations include standardization of entrustment scales and reduction of stakeholder burden.

Getting airtime: Exploring how patients shape the stories they tell health practitioners.

INTRODUCTION: Effective communication during health encounters is known to decrease patient complaints, increase patient adherence and optimise health outcomes. While the aim of patient-centred care is to find common ground, health practitioners tend to drive the encounter, often interrupting patients within the first minute of the clinical conversation. Optimal care for people with chronic illnesses requires individuals to interact with health practitioners regarding their health concerns, but given these constraints, we know little about how patients strategise conversations with their care providers. This understanding may further our efforts to educate health practitioners and trainees to learn and practice patient-centred care. METHODS: A constructivist grounded theory approach with iterative data collection and analysis was used to explore the processes patients use to present and shape their stories for interactions with health practitioners. Twenty-one patients (n = 16 female; 5 male) representing a variety of chronic illnesses participated in semi-structured interviews. Using the constant comparative method of analysis, salient themes were ascertained. RESULTS: Patients engage in extensive strategic preparations for productive health encounters. From the data, we identified four related elements comprising patients' process of planning, preparing, and strategising for health encounters: deciding to go, organising to get airtime, rehearsing a game plan, and anticipating external forces. By focusing on the extensive preparatory work patients engage in, our study expands the dimensions of how we understand illness-related work. Assembling personal health information, gathering disease information and achieving equanimity represent the dimensions of this 'health interaction work'. CONCLUSION: The work patients engage in for health encounters is noteworthy yet often invisible. And work that is unseen may also be undervalued. Acknowledging, illuminating and valuing patients' preparatory work for health encounters add to how we understand patient-centred care, and this offers new targets for us to effectively teach and deliver it.

Survey of Canadian Myotonic Dystrophy Patients' Access to Computer Technology.

BACKGROUND: Myotonic dystrophy type 1 is an autosomal dominant condition affecting distal hand strength, energy, and cognition. Increasingly, patients and families are seeking information online. An online neuromuscular patient portal under development can help patients access resources and interact with each other regardless of location. It is unknown how individuals living with myotonic dystrophy interact with technology and whether barriers to access exist. We aimed to characterize technology use among participants with myotonic dystrophy and to determine whether there is interest in a patient portal. METHODS: Surveys were mailed to 156 participants with myotonic dystrophy type 1 registered with the Canadian Neuromuscular Disease Registry. RESULTS: Seventy-five participants (60% female) responded; almost half were younger than 46 years. Most (84%) used the internet; almost half of the responders (47%) used social media. The complexity and cost of technology were commonly cited reasons not to use technology. The majority of responders (76%) were interested in a myotonic dystrophy patient portal. CONCLUSIONS: Patients in a Canada-wide registry of myotonic dystrophy have access to and use technology such as computers and mobile phones. These patients expressed interest in a portal that would provide them with an opportunity to network with others with myotonic dystrophy and to access information about the disease.

Evolving Motivations: Patients' and Caregivers' Perceptions About Seeking Myotonic Dystrophy (DM1) and Huntington's Disease Care.

Patient-centered care provision is challenging under ideal circumstances; myotonic dystrophy (DM1) and Huntington's disease (HD) are examples of chronic, progressive health conditions that may challenge its limits. If we can understand how care unfolds in these conditions, health care providers may be better equipped to address patients' needs. Constructivist grounded theory informed data collection and analysis. Fourteen patients with DM1 or HD, and 10 caregivers participated in semistructured interviews. Constant comparative analysis was used to identify themes. Participants attended clinic to seek expert information and social support. Medical management, altruism, and support provided the motivation. However, motivations evolved, with clinic becoming more important for caregivers as patients deteriorated. Clinic was conceptualized as a "safe space" to actively participate in health care and research. In the absence of disease-halting or curative treatments, participants perceived that they derived a therapeutic benefit from seeking care and from engaging in education and advocacy.

Myotonic Dystrophy and Huntington's Disease Care: "We Like to Think We're Making a Difference".

BACKGROUND: Patient-centered care for individuals with myotonic dystrophy (DM1) and Huntington's disease (HD)-chronic, progressive, and life-limiting neurological conditions-may be challenged by patients' cognitive and behavioral impairments. However, no research has explored health care providers' (HCPs') perspectives about patient-centered care provision for these patients along their disease trajectory. METHODS: Constructivist grounded theory informed the iterative data collection and analysis process. Eleven DM1 or HD HCPs participated in semistructured interviews, and three stages of coding were used to analyze their interview transcripts. Codes were collapsed into themes and categories. RESULTS: Three categories including an evolving care approach, fluid roles, and making a difference were identified. Participants described that their clinical care approach evolved depending on the patient's disease stage and caregivers' degree of involvement. HCPs described that their main goal was to provide hope to patients and caregivers through medical management, crisis prevention, support, and advocacy. Despite the lack of curative treatments, HCPs perceived that patients benefited from ongoing clinical care provided by proactive clinicians. CONCLUSIONS: Providing care for individuals with DM1 and HD is a balancing act. HCPs must strike a balance between (1) the frustrations and rewards of patient-centered care provision, (2) addressing symptoms and preventing and managing crises while focusing on patients' and caregivers' quality of life concerns, and (3) advocating for patients while addressing caregivers' needs. This raises important questions: Is patient-centered care possible for patients with cognitive decline? Does chronic neurological care need to evolve to better address patients' and caregivers' complex needs?

Pompe Disease: Diagnosis and Management. Evidence-Based Guidelines from a Canadian Expert Panel.

Pompe disease is a lysosomal storage disorder caused by a deficiency of the enzyme acid alpha-glucosidase. Patients have skeletal muscle and respiratory weakness with or without cardiomyopathy. The objective of our review was to systematically evaluate the quality of evidence from the literature to formulate evidence-based guidelines for the diagnosis and management of patients with Pompe disease. The literature review was conducted using published literature, clinical trials, cohort studies and systematic reviews. Cardinal treatment decisions produced seven management guidelines and were assigned a GRADE classification based on the quality of evidence in the published literature. In addition, six recommendations were made based on best clinical practices but with insufficient data to form a guideline. Studying outcomes in rare diseases is challenging due to the small number of patients, but this is in particular the reason why we believe that informed treatment decisions need to consider the quality of the evidence.

Intravenous immunoglobulin response in treatment-naïve chronic inflammatory demyelinating polyradiculoneuropathy.

OBJECTIVE: There is no consensus on which treatment should be used preferentially in individual patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Patients unlikely to respond to intravenous immunoglobulin (IVIg) could be prescribed corticosteroids first to avoid high cost and a delayed treatment response. We investigated which factors determined a response to IVIg. METHODS: Treatment-naïve patients with CIDP initially treated with at least one full course of IVIg (2 g/kg) at one of two neuromuscular disease centres were included. Patients fulfilled the European Federation of Neurological Societies/Peripheral Nerve Society clinical criteria for CIDP. Significant improvement following IVIg was defined as an improvement (≥ 1 grade) on the modified Rankin scale. Difference in weakness between arms and legs was defined as ≥ 2 grades on the Medical Research Council scale between ankle dorsiflexion and wrist extension. Clinical predictors with a p value <0.15 in univariate analysis were analysed in multivariate logistic regression. RESULTS: Of a total of 281 patients, 214 patients (76%) improved. In univariate analysis, the presence of pain, other autoimmune disease, difference in weakness between arms and legs, and a myelin-associated glycoprotein negative IgM monoclonal gammopathy of undetermined significance were associated with no response to IVIg. In multivariate analysis no pain (p=0.018) and no difference in weakness between arms and legs (p=0.048) were independently associated with IVIg response. Of IVIg non-responders, 66% improved with plasma exchange and 58% with corticosteroids. CONCLUSIONS: IVIg is a very effective first-line treatment. Patients with CIDP presenting with pain or a difference in weakness between arms and legs are less likely to respond to IVIg.

Truths and misinformation: a qualitative exploration of myotonic dystrophy.

BACKGROUND: Myotonic dystrophy (DM1) is an autosomal dominant, progressive, and multisystem condition that impacts affected individuals physically, socially, and emotionally. Understanding individuals' perceptions of their disease is critical to ensuring appropriate information, education, and counseling. METHODS: We conducted a content analysis of findings from a larger study that used a novel, qualitative research approach called photovoice to explore nine patients' experiences of living with DM1. Participants took pictures that illustrated barriers or facilitators to living with DM1; their photographs then formed the basis of semistructured interviews. Transcripts were analyzed and, among themes, we identified one titled "DM1 truths and misinformation" that described participants' disease knowledge. Analysis revealed four categories within this broader theme: "the physical and emotional cost of DM1," "managing my DM1," "genetics and me" and "patients as advocates and educators." RESULTS: Findings showed that DM1 participants had good core knowledge with respect to their disease and its implications. However, each participant held as fact fragments of misinformation that shaped decision-making and pointed to a clear need for strategies to mitigate variable interpretation of health information. CONCLUSIONS: We conclude that there is a need for increased education and awareness about symptoms, genetic information and treatment strategies for patients, their family members, and health care providers.

Non-dystrophic myotonia: prospective study of objective and patient reported outcomes.

Non-dystrophic myotonias are rare diseases caused by mutations in skeletal muscle chloride and sodium ion channels with considerable phenotypic overlap between diseases. Few prospective studies have evaluated the sensitivity of symptoms and signs of myotonia in a large cohort of patients. We performed a prospective observational study of 95 participants with definite or clinically suspected non-dystrophic myotonia recruited from six sites in the USA, UK and Canada between March 2006 and March 2009. We used the common infrastructure and data elements provided by the NIH-funded Rare Disease Clinical Research Network. Outcomes included a standardized symptom interview and physical exam; the Short Form-36 and the Individualized Neuromuscular Quality of Life instruments; electrophysiological short and prolonged exercise tests; manual muscle testing; and a modified get-up-and-go test. Thirty-two participants had chloride channel mutations, 34 had sodium channel mutations, nine had myotonic dystrophy type 2, one had myotonic dystrophy type 1, and 17 had no identified mutation. Phenotype comparisons were restricted to those with sodium channel mutations, chloride channel mutations, and myotonic dystrophy type 2. Muscle stiffness was the most prominent symptom overall, seen in 66.7% to 100% of participants. In comparison with chloride channel mutations, participants with sodium mutations had an earlier age of onset of stiffness (5 years versus 10 years), frequent eye closure myotonia (73.5% versus 25%), more impairment on the Individualized Neuromuscular Quality of Life summary score (20.0 versus 9.44), and paradoxical eye closure myotonia (50% versus 0%). Handgrip myotonia was seen in three-quarters of participants, with warm up of myotonia in 75% chloride channel mutations, but also 35.3% of sodium channel mutations. The short exercise test showed ≥10% decrement in the compound muscle action potential amplitude in 59.3% of chloride channel participants compared with 27.6% of sodium channel participants, which increased post-cooling to 57.6% in sodium channel mutations. In evaluation of patients with clinical and electrical myotonia, despite considerable phenotypic overlap, the presence of eye closure myotonia, paradoxical myotonia, and an increase in short exercise test sensitivity post-cooling suggest sodium channel mutations. Outcomes designed to measure stiffness or the electrophysiological correlates of stiffness may prove useful for future clinical trials, regardless of underlying mutation, and include patient-reported stiffness, bedside manoeuvres to evaluate myotonia, muscle specific quality of life instruments and short exercise testing.

Exploring frontline faculty perspectives after a curriculum change.

CONTEXT: Curriculum renewal is an essential and continual process for undergraduate medical education programmes. Although there is substantial literature on the critical role of leadership in successful curricular change, the voices of frontline faculty teachers implementing such change have not been explored. We aimed not only to examine and understand the perceptions of faculty members as they face curriculum change, but also to explore the influences on their engagement with change. METHODS: We used a constructivist grounded approach in this exploratory study. Sixteen faculty members teaching in the pre-clinical years were interviewed on their perspectives on a recent curricular change in the undergraduate medical programme at a single Canadian medical school. Constant comparative analysis was conducted to identify recurring themes. RESULTS: Faculty teachers' engagement with curriculum change was influenced by three critical tensions during three phases of the change: (i) tension between individual and institutional values, which was prominent as change was being introduced; (ii) tension between drivers of change and restrainers of change, which was prominent as change was being enacted, and (iii) tension between perceived gains and perceived losses, which was prominent as teachers reflected on change once implemented. CONCLUSIONS: We propose a model of faculty engagement with curricular change that elucidates the need to consider individual experiences and motivations within the broader context of the institutional culture of medical schools. Importantly, if individual and institutional values are misaligned, barriers to change outweigh facilitators, or perceived losses prevail; subsequently faculty teachers' engagement may be threatened, exposing the medical education programme to risk.

The CNDR: collaborating to translate new therapies for Canadians.

BACKGROUND: Patient registries represent an important method of organizing "real world" patient information for clinical and research purposes. Registries can facilitate clinical trial planning and recruitment and are particularly useful in this regard for uncommon and rare diseases. Neuromuscular diseases (NMDs) are individually rare but in aggregate have a significant prevalence. In Canada, information on NMDs is lacking. Barriers to performing Canadian multicentre NMD research exist which can be overcome by a comprehensive and collaborative NMD registry. METHODS: We describe the objectives, design, feasibility and initial recruitment results for the Canadian Neuromuscular Disease Registry (CNDR). RESULTS: The CNDR is a clinic-based registry which launched nationally in June 2011, incorporates paediatric and adult neuromuscular clinics in British Columbia, Alberta, Ontario, Quebec, New Brunswick and Nova Scotia and, as of December 2012, has recruited 1161 patients from 12 provinces and territories. Complete medical datasets have been captured on 460 "index disease" patients. Another 618 "non-index" patients have been recruited with capture of physician-confirmed diagnosis and contact information. We have demonstrated the feasibility of blended clinic and central office-based recruitment. "Index disease" patients recruited at the time of writing include 253 with Duchenne and Becker muscular dystrophy, 161 with myotonic dystrophy, and 71 with ALS. CONCLUSIONS: The CNDR is a new nationwide registry of patients with NMDs that represents an important advance in Canadian neuromuscular disease research capacity. It provides an innovative platform for organizing patient information to facilitate clinical research and to expedite translation of recent laboratory findings into human studies.

Decomposition-based quantitative electromyography in the evaluation of muscular dystrophy severity.

INTRODUCTION: Electromyography is useful in the diagnosis of myopathies, but its utility in determining disease severity requires further investigation. In this study we aimed to determine whether decomposition-based quantitative electromyography (DQEMG) could indicate the severity of involvement in a cohort of patients with muscular dystrophies (MDs). METHODS: Fifteen patients with facioscapulohumeral (FSHD), limb-girdle (LGMD), and Becker (BMD) muscular dystrophy, and 7 healthy controls, participated in this investigation. Knee extensor isometric strength differentiated the "more severe" and "less severe" MD groups. The vastus lateralis (VL), biceps brachii (BB), and tibialis anterior (TA) muscle groups were investigated using DQEMG. RESULTS: All muscles from the MD group showed changes in mean MUP (motor unit potential) AAR (area-to-amplitude ratio), and turns, compared with controls (P < 0.05). More severely affected muscles (VL and BB) also had shortened mean MUP durations compared with controls (P < 0.01). CONCLUSIONS: DQEMG was capable of indicating the severity of MD involvement, as changes in MUP morphology reflected the progressive nature of the disease.

A quantitative measure of handgrip myotonia in non-dystrophic myotonia.

INTRODUCTION: Non-dystrophic myotonia (NDM) is characterized by myotonia without muscle wasting. A standardized quantitative myotonia assessment (QMA) is important for clinical trials. METHODS: Myotonia was assessed in 91 individuals enrolled in a natural history study using a commercially available computerized handgrip myometer and automated software. Average peak force and 90% to 5% relaxation times were compared with historical normal controls studied with identical methods. RESULTS: Thirty subjects had chloride channel mutations, 31 had sodium channel mutations, 6 had DM2 mutations, and 24 had no identified mutation. Chloride channel mutations were associated with prolonged first handgrip relaxation times and warm-up on subsequent handgrips. Sodium channel mutations were associated with prolonged first handgrip relaxation times and paradoxical myotonia or warm-up, depending on underlying mutations. DM2 subjects had normal relaxation times but decreased peak force. Sample size estimates are provided for clinical trial planning. CONCLUSION: QMA is an automated, non-invasive technique for evaluating myotonia in NDM.

Mexiletine for symptoms and signs of myotonia in nondystrophic myotonia: a randomized controlled trial.

CONTEXT: Nondystrophic myotonias (NDMs) are rare diseases caused by mutations in skeletal muscle ion channels. Patients experience delayed muscle relaxation causing functionally limiting stiffness and pain. Mexiletine-induced sodium channel blockade reduced myotonia in small studies; however, as is common in rare diseases, larger studies of safety and efficacy have not previously been considered feasible. OBJECTIVE: To determine the effects of mexiletine for symptoms and signs of myotonia in patients with NDMs. DESIGN, SETTING, AND PARTICIPANTS: A randomized, double-blind, placebo-controlled 2-period crossover study at 7 neuromuscular referral centers in 4 countries of 59 patients with NDMs conducted between December 23, 2008, and March 30, 2011, as part of the National Institutes of Health-funded Rare Disease Clinical Research Network. INTERVENTION: Oral 200-mg mexiletine or placebo capsules 3 times daily for 4 weeks, followed by the opposite intervention for 4 weeks, with 1-week washout in between. MAIN OUTCOME MEASURES: Patient-reported severity score of stiffness recorded on an interactive voice response (IVR) diary (scale of 1 = minimal to 9 = worst ever experienced). Secondary end points included IVR-reported changes in pain, weakness, and tiredness; clinical myotonia assessment; quantitative measure of handgrip myotonia; and Individualized Neuromuscular Quality of Life summary quality of life score (INQOL-QOL, percentage of maximal detrimental impact). RESULTS: Mexiletine significantly improved patient-reported severity score stiffness on the IVR diary. Because of a statistically significant interaction between treatment and period for this outcome, primary end point is presented by period (period 1 means were 2.53 for mexiletine and 4.21 for placebo; difference, -1.68; 95% CI, -2.66 to -0.706; P < .001; period 2 means were 1.60 for mexiletine and 5.27 for placebo; difference, -3.68; 95% CI, -3.85 to -0.139; P = .04). Mexiletine improved the INQOL-QOL score (mexiletine, 14.0 vs placebo, 16.7; difference, -2.69; 95% CI, -4.07 to -1.30; P < .001) and decreased handgrip myotonia on clinical examination (mexiletine, 0.164 seconds vs placebo, 0.494 seconds; difference, -0.330; 95% CI, -0.633 to -0.142; P < .001). The most common adverse effect was gastrointestinal (9 mexiletine and 1 placebo). Two participants experienced transient cardiac effects that did not require stopping the study (1 in each group). One serious adverse event was determined to be not study related. CONCLUSION: In this preliminary study of patients with NDMs, the use of mexiletine compared with placebo resulted in improved patient-reported stiffness over 4 weeks of treatment, despite some concern about the maintenance of blinding. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00832000.

Common epigenetic changes of D4Z4 in contraction-dependent and contraction-independent FSHD.

Facioscapulohumeral muscular dystrophy (FSHD), caused by partial deletion of the D4Z4 macrosatellite repeat on chromosome 4q, has a complex genetic and epigenetic etiology. To develop FSHD, D4Z4 contraction needs to occur on a specific genetic background. Only contractions associated with the 4qA161 haplotype cause FSHD. In addition, contraction of the D4Z4 repeat in FSHD patients is associated with significant D4Z4 hypomethylation. To date, however, the methylation status of contracted repeats on nonpathogenic haplotypes has not been studied. We have performed a detailed methylation study of the D4Z4 repeat on chromosome 4q and on a highly homologous repeat on chromosome 10q. We show that patients with a D4Z4 deletion (FSHD1) have D4Z4-restricted hypomethylation. Importantly, controls with a D4Z4 contraction on a nonpathogenic chromosome 4q haplotype or on chromosome 10q also demonstrate hypomethylation. In 15 FSHD families without D4Z4 contractions but with at least one 4qA161 haplotype (FSHD2), we observed D4Z4-restricted hypomethylation on chromosomes 4q and 10q. This finding implies that a genetic defect resulting in D4Z4 hypomethylation underlies FSHD2. In conclusion, we describe two ways to develop FSHD: (1) contraction-dependent or (2) contraction-independent D4Z4 hypomethylation on the 4qA161 subtelomere.

Using Electronic Health Record Data to Assess Residents' Clinical Performance in the Workplace: The Good, the Bad, and the Unthinkable.

PURPOSE: Novel approaches are required to meet assessment demands and cultivate authentic feedback in competency-based medical education. One potential source of data to help meet these demands is the electronic health record (EHR). However, the literature offers limited guidance regarding how EHR data could be used to support workplace teaching and learning. Furthermore, given its sheer volume and availability, there exists a risk of exploiting the educational potential of EHR data. This qualitative study examined how EHR data might be effectively integrated and used to support meaningful assessments of residents' clinical performance. METHOD: Following constructivist grounded theory, using both purposive and theoretical sampling, in 2016-2017 the authors conducted individual interviews with 11 clinical teaching faculty and 10 senior residents across 12 postgraduate specialties within the Schulich School of Medicine and Dentistry at Western University. Constant comparative inductive analysis was conducted. RESULTS: Analysis identified key issues related to affordances and challenges of using EHRs to assess resident performance. These include the nature of EHR data; the potential of using EHR data for assessment; and the dangers of using EHR data for assessment. Findings offer considerations for using EHR data to assess resident performance in appropriate and meaningful ways. CONCLUSIONS: EHR data have potential to support formative assessment practices and guide feedback discussions with residents, but evaluators must take context into account. The EHR was not designed with the purpose of assessing resident performance; therefore, adoption and use of these data for educational purposes require careful thought, consideration, and care.

Phenotype of combined Duchenne and facioscapulohumeral muscular dystrophy.

This case report describes a young boy with concomitant genetically-confirmed Duchenne muscular dystrophy and facioscapulohumeral muscular dystrophy with a novel dystrophin mutation in exon 6 and a D4Z4 fragment of 31 kb. This child presented with a more severe phenotype than expected for either individual disease process and underscores the role for thorough diagnostic investigation in identifying atypical clinical presentations.

Approach to the Patient With HyperCKemia.

PURPOSE OF REVIEW: Neurologists commonly receive consultation requests regarding the evaluation of patients with an elevated serum creatine kinase (CK), a condition known as hyperCKemia. This article outlines an approach to the history and examination of patients with hyperCKemia in order to narrow the localization and differential of an elevated CK and guide possible next steps. This article aims to help clinicians identify treatable or reversible etiologies as well as those that will change management. RECENT FINDINGS: An unrevealing patient history (assessing for acquired and hereditary etiologies) in an otherwise neurologically intact individual who has a normal nerve conduction study and EMG predicts that the likelihood of diagnosing the patient after further investigations will be quite low. After a comprehensive workup, a positive diagnosis is made in approximately 25% of cases of hyperCKemia. SUMMARY: The best predictors for added diagnostic yield with further testing in hyperCKemia are a higher level of CK and a younger age; the presence of weakness increases the likelihood of a specific cause other than idiopathic or familial hyperCKemia. Many etiologies do not yet have treatments that alter clinical outcomes, and, even in the absence of a specific diagnosis, good communication with patients and primary care providers remains essential to ensure longitudinal surveillance with expectant management for potential consequences. Many patients with hyperCKemia of uncertain etiology, however, will not develop significant muscle disease on longitudinal follow-up.

Hard to Swallow: A Phenomenological Exploration of the Experience of Caring for Individuals With Myotonic Dystrophy and Dysphagia.

PURPOSE: Myotonic dystrophy (DM1), a genetic, multisystemic disorder, is the most prevalent adult form of muscular dystrophy. Dysphagia is a common symptom that may be difficult to diagnose and treat and can be associated with increased morbidity and mortality. Preexisting cognitive impairment or apathy, both well described in the DM1 literature, may contribute to management challenges. Caregivers may become important for managing a family member's swallowing dysfunction. Although clinicians place great importance on swallowing difficulties, it is unknown how dysphagia impacts patients and their caregivers. Therefore, the purpose of this study was to explore the experiences of caregivers living with those with DM1and dysphagia. METHODS: An interpretive phenomenological approach was used to study the lived experience of six caregivers for individuals with DM1 and dysphagia. Audio-taped semistructured interviews were used for data collection, and data were analyzed using van Manen's steps for phenomenological analysis. FINDINGS: Despite the potential for dysphagia to cause morbidity and mortality in individuals with DM1, caregivers did not describe this as a problematic symptom. Instead, they highlighted more debilitating symptoms like fatigue or weakness and discussed the caregiving experience. Themes pertaining to participants' lived body, lived relationality, lived time, and lived space were identified. CONCLUSIONS: Healthcare providers need to balance issues of clinical concern with those that are important for individuals and their family members. Assessments of caregiver knowledge and burden at each clinic visit may be warranted.