Aimee M. Verrall

Efficacy of a Telephone-Delivered Self-Management Intervention for Persons With Multiple Sclerosis: A Randomized Controlled Trial With a One-Year Follow-Up

OBJECTIVE To evaluate the efficacy of a telephone-delivered self-management intervention for fatigue, pain, and depression in adults with multiple sclerosis (MS). DESIGN Single-center, randomized (1:1), single-blind (outcome assessors), parallel-group trial with a primary endpoint of posttreatment (9-11 wk postrandomization) and long-term follow-up at 6 and 12 months. SETTING Telephone-delivered across the United States. PARTICIPANTS Adults with MS (N=163) with fatigue, chronic pain, and/or moderate depressive symptoms (age range, 25-76 y). INTERVENTIONS Eight-week individual telephone-delivered self-management intervention (T-SM) (n=75) versus an 8-week individual telephone-delivered MS education intervention (T-ED) (n=88). MAIN OUTCOME MEASURES The primary outcome was the proportion who achieved a ≥50% decrease in 1 or more symptoms-fatigue impact, pain interference, and/or depression severity. Secondary outcomes included continuous measures of pain, fatigue impact, depression, self-efficacy, activation, health-related quality of life, resilience, and affect. RESULTS For our primary outcome, 58% of those in the T-SM group and 46% of those in the T-ED group had a ≥50% reduction in 1 or more symptoms; this difference was not statistically significant (odds ratio, 1.50; 95% confidence interval, .77-2.93; P=.238). Participants in both groups significantly improved from baseline to posttreatment in primary and secondary outcome measures (P<.05). T-SM participants reported significantly higher treatment satisfaction and therapeutic alliance and greater improvements in activation, positive affect, and social roles. Improvements were generally maintained at 6 and 12 months. CONCLUSIONS Both interventions resulted in short- and long-term, clinically meaningful benefits. The study demonstrated that the telephone is an effective method for engaging participants in care and extending the reach of rehabilitation for individuals with MS.

Body mass index and waist circumference in persons aging with muscular dystrophy, multiple sclerosis, post-polio syndrome, and spinal cord injury

BACKGROUND Body mass index (BMI) and waist circumference (WC) are well-understood in the general population, but are not adequately understood among persons with disabilities. OBJECTIVE To describe and compare BMI and WC among individuals with muscular dystrophy (MD), multiple sclerosis (MS), post-polio syndrome (PPS), and spinal cord injury (SCI). BMI scores were also compared to normative data of the U.S. population, with consideration for age, sex, and mobility limitations. METHODS Persons with MD (n = 339), MS (n = 597), PPS (n = 443), and SCI (n = 488) completed postal surveys that included self-reported BMI and WC data. NHANES data were used to compare the current sample with a representative US sample. RESULTS Participants with PPS had higher BMI than participants with MD, MS, and SCI. In addition, participants with MS had significantly higher BMI relative to participants with SCI. BMI was significantly positively associated with age, years since diagnosis, mobility, and interactions of some of these factors. Relative to the general population, BMI was lower in MD, MS, and SCI across age groups, as well as in men with PPS and women ages 60-74 years with PPS. No significant differences were identified between MD, MS, PPS, and SCI in WC. CONCLUSIONS The presence of group differences in BMI and absence of group differences in WC suggests that BMI may not accurately represent health risk in SCI, MD, and possibly MS, because of biasing elements of the conditions, such as changes in body composition and mobility limitations.

Quality of Life Instruments in Ménière's Disease†‡

Objective: Evaluate the sensitivity of quality of life (QoL) instruments over time in patients with Ménières disease using general, symptom‐specific, and disease‐specific QoL instruments.

Falls Among Adults Aging With Disability

OBJECTIVE To investigate the prevalence of and risk factors for falling among individuals aging with multiple sclerosis (MS), muscular dystrophy (MD), postpolio syndrome (PPS), and spinal cord injury (SCI). DESIGN Cross-sectional survey data from 2009 to 2010 were analyzed. We used forward logistic regression models to examine whether risk factors such as age, sex, mobility level, years since diagnosis, vision, balance, weakness, number of comorbid conditions, and physical activity could distinguish participants who reported falling from those who did not. SETTING Surveys were mailed to community-dwelling individuals who had 1 of 4 diagnoses (MS, MD, PPS, or SCI). The survey response rate was 91%. PARTICIPANTS A convenience sample of community-dwelling individuals (N=1862; age, 18-94y) with MS, MD, PPS, or SCI in the United States. INTERVENTIONS Not applicable. MAIN OUTCOME MEASURE Self-reported fall within the last 6 months. RESULTS Fall prevalence for people with MS (54%), MD (70%), PPS (55%), and SCI (40%). Across all 4 groups, fall rates peaked in middle age (45-64y) and among people with moderate mobility limitations. Seven risk factors differentiated participants who fell from those who did not: mobility level, imbalance, age, curvilinear age (age(2)), number of comorbid conditions, duration of diagnosis, and sex. The models differed across diagnostic groups. CONCLUSIONS People aging with long-term physical disabilities experience unique challenges that affect their risk of falls. A better understanding of the frequency, severity, and risk factors of falls across diagnostic groups is needed to design and implement customized, effective fall prevention and management programs for these individuals.

Effects of pain and fatigue on physical functioning and depression in persons with muscular dystrophy.

BACKGROUND Pain and fatigue are common symptoms experienced by persons with muscular dystrophy (MD). However, it is unclear from previous studies whether pain and fatigue have independent effects on physical functioning and depression, and whether age moderates the relationship of pain and fatigue with physical functioning and depression. OBJECTIVE This cross-sectional study aimed to describe the relationship of pain and fatigue to physical functioning and depression in persons 20-89 years old with MD. METHOD A convenience sample of 332 individuals with MD completed a questionnaire that included measures of physical functioning (PROMIS item bank items), depression (PHQ-9), pain intensity (0-10 NRS), and fatigue (0-10 NRS). RESULTS Pain and fatigue were each independently associated with physical functioning and depression. Depressive symptoms were most severe among middle-aged participants (45-64 years old) relative to older and younger participants. Physical functioning had a negative relationship with chronological age. CONCLUSIONS Symptoms of pain and fatigue are significantly and independently related to physical functioning and depression in persons with MD. Research is needed to determine if treatments that target both pain and fatigue in patients with MD have more beneficial effects than treatments that target only one of these symptoms.

A novel WFS1 mutation in a family with dominant low frequency sensorineural hearing loss with normal VEMP and EcochG findings.

BackgroundLow frequency sensorineural hearing loss (LFSNHL) is an uncommon clinical finding. Mutations within three different identified genes (DIAPH1, MYO7A, and WFS1) are known to cause LFSNHL. The majority of hereditary LFSNHL is associated with heterozygous mutations in the WFS1 gene (wolframin protein). The goal of this study was to use genetic analysis to determine if a small American familys hereditary LFSNHL is linked to a mutation in the WFS1 gene and to use VEMP and EcochG testing to further characterize the familys audiovestibular phenotype.MethodsThe clinical phenotype of the American family was characterized by audiologic testing, vestibular evoked myogenic potentials (VEMP), and electrocochleography (EcochG) evaluation. Genetic characterization was performed by microsatellite analysis and direct sequencing of WFS1 for mutation detection.ResultsSequence analysis of the WFS1 gene revealed a novel heterozygous mutation at c.2054G>C predicting a p.R685P amino acid substitution in wolframin. The c.2054G>C mutation segregates faithfully with hearing loss in the family and is absent in 230 control chromosomes. The p.R685 residue is located within the hydrophilic C-terminus of wolframin and is conserved across species. The VEMP and EcochG findings were normal in individuals segregating the WFS1 c.2054G>C mutation.ConclusionWe discovered a novel heterozygous missense mutation in exon 8 of WFS1 predicting a p.R685P amino acid substitution that is likely to underlie the LFSNHL phenotype in the American family. For the first time, we describe VEMP and EcochG findings for individuals segregating a heterozygous WFS1 mutation.

Self-Management for People with Multiple Sclerosis: Report from the First International Consensus Conference, November 15, 2010

People with multiple sclerosis (MS) must manage the day-to-day effects of the disease on their lives. Self-management interventions may be helpful in this challenge. An international, multidisciplinary consensus conference was held on November 15, 2010, by the University of Washingtons Rehabilitation Research and Training Center for Multiple Sclerosis (MS RRTC), with funding from the Consortium of Multiple Sclerosis Centers (CMSC) and the National Institute on Disability and Rehabilitation Research (NIDRR), to discuss the concept of self-management for people with MS. The specific goals of the consensus conference were as follows: 1) review the current research on self-management and related issues in chronic disability and specifically in MS; 2) review optimal research methodologies, outcome measurement tools, program planning frameworks, and dissemination strategies for self-management research; and 3) establish recommendations on the next steps necessary to develop, adapt, and test self-management interventions for people with MS. The consensus conference and this document are the initial steps toward achieving the stated goals. Participants in the consensus conference concluded that it is necessary to: 1) define an empirically based conceptual model of self-management for people with MS; 2) establish reliable and valid self-management outcome measures; 3) use best practices to validate models of self-management interventions; and 4) plan dissemination and knowledge translation of interventions once their effectiveness is established.

Bouncing back again, and again: a qualitative study of resilience in people with multiple sclerosis.

Abstract Purpose The purpose of this study was to describe the meaning of resilience, factors facilitating resilience and barriers to resilience, from the perspective of persons with multiple sclerosis (MS), their care partners and community stakeholders. Method We conducted four focus groups: two with middle-aged (36–62 years) individuals with MS [one with men (n = 6) and one with women (n = 6)], one for partners of individuals with MS (n = 11) and one with community stakeholders serving people with MS (n = 9). We asked participants to describe what resilience means to them, what factors facilitate resilience and what barriers to resilience they perceive. We analyzed the focus group transcripts for emerging themes and sub-themes. Results Participants found it difficult to generate a concise definition of resilience, but they generated evocative descriptions of the concept. Psychological adaptation, social connection, life meaning, planning and physical wellness emerged as facilitators of resilience. Resilience depletion, negative thoughts and feelings, social limitations, social stigma and physical fatigue emerged as barriers to resilience. Conclusion The unpredictable nature of MS can present unique challenges to resilient adjustment, especially during middle age. However, several factors can contribute to resilience and quality of life, and these factors are amenable to intervention. Implications for Rehabilitation Resilience is the capacity to bounce back and thrive when faced with challenges. People with MS develop resilience through psychological adaptation, social connection, life meaning, planning ahead and physical wellness. Barriers to resilience with MS include burnout, negative thoughts and feelings, social difficulties, stigma and fatigue. Interventions should address both individual and social factors that support resilience, such as promoting positive thinking, planning and engagement in meaningful activities.

Symptom profiles in individuals aging with post-polio syndrome.

nocturia, although this cutoff should be explored in larger study populations to be validated. In addition, the use of incontinence material is not only based on the presence of urinary incontinence. The results show that the presence and frequency of nocturnal incontinence was significantly higher in the group wearing incontinence material, as expected, but four residents reported that they do not experience urinary incontinence despite the use of incontinence material. These residents are subject to comorbidities and poor mobility necessitating the use of incontinence material as continence policy. The results of the SF-36 questionnaire, on which significantly lower scores concerning physical and emotional functioning and pain were found in the group wearing incontinence material, also reflect this. New or worsening incontinence even in frail, functionally and cognitively impaired nursing home residents seriously decreases quality of life. Reducing nocturnal urine volume might improve quality of life in the subgroup of individuals wearing incontinence material because of nocturnal incontinence by reducing the need for incontinence material.