key: cord-1001418-mphesjii authors: Eichinger, Katy; Lewis, Leann; Dilek, Nuran; Higgs, Kiley; Walker, Michaela; Palmer, David; Cooley, John M.; Johnson, Nicholas; Tawil, Rabi; Statland, Jeffrey title: A patient‐focused survey to assess the effects of the COVID‐19 pandemic and social guidelines on people with muscular dystrophy date: 2021-06-25 journal: Muscle Nerve DOI: 10.1002/mus.27349 sha: 908450324f0aa2f1136e06ca5c2492c0a3884bd0 doc_id: 1001418 cord_uid: mphesjii INTRODUCTION/AIMS: In this study, we examined the social and health impacts of the coronavirus disease 2019 (COVID‐19) pandemic and social guidelines on people with muscular dystrophies. METHODS: A prospective de‐identified electronic survey was distributed to adults with self‐reported facioscapulohumeral muscular dystrophy (FSHD), myotonic dystrophy (DM), and limb‐girdle muscular dystrophy (LGMD) enrolled in national registries or with patient advocacy groups. The COVID‐19 Impact Survey was developed by muscular dystrophy experts in association with patient collaborators and advocacy groups. The Perceived Stress Scale was used to measure perceived stress. RESULTS: Respondents (n = 774: 56% FSHD; 35% DM, and 9% LGMD) were mostly women and middle‐aged (range 19–87 y). Rates of COVID‐19 infections were low (<1%), compliance with local social distancing guidelines and policies high (98%). Major challenges reported during the pandemic included: obtaining treatment (40%), managing stress (37%), social distancing (36%), and obtaining essentials (34%). The majority reported a slight worsening in their disease state. Respondents reported moderate stress levels (stress score = 15.4; range = 0–35), with higher stress levels reported by women and those under age 30 y. Three‐quarters of participants who participated in telemedicine visits were satisfied with the encounters; however, most reported a preference for in‐person visits. DISCUSSION: People with muscular dystrophy reported moderate stress and challenges during the COVID‐19 pandemic. Interventions such as exercise and stress‐coping strategies, including strategies specific to women or individuals <30 y, may be important. Further investigation is needed into the role of telemedicine in the care of individuals with muscular dystrophy. Data were summarized by responses to each question: frequencies, means, and SDs. Associations between discrete or continuous data elements were explored using Pearson correlations. 10 Differences in perceived stress between groups based on clinical diagnosis, gender, age categories, or ambulatory status was performed by analysis of variance using Tukey's method for multiple comparisons, or T-test. Where there were differences by diagnosis, an analysis of covariance was performed to adjust for potential demographic differences. Statistical analysis was performed using SPSS version 26 (IBM, Armonk, NY). For this exploratory survey, all statistical testing was two-sided and a P-value <.05 is significant. Seven hundred and seventy-four adults with MD residing in the United States responded to the survey (Table 1) . Respondents were approximately equally divided between the sexes, with the exception of those with DM, who were predominantly women. The majority of the participants reported genetic confirmation and most individuals reported living with other people. Approximately a third reported using a wheelchair either part-time or full-time. Social distancing (95%), avoiding crowds larger than 10 (86%), and wearing masks (84%) were reported by the majority of participants as social strategies. Participants reported stay-at-home orders less frequently (78%). Ninety-eight percent of the participants reported adhering to the local social distancing recommendations that were in place in their state. The most frequently reported challenges during the COVID-19 pandemic were obtaining treatment, managing stress, social distancing, and obtaining essentials (groceries, medications, etc) ( Figure 1A ). Other challenges reported by respondents included lack of access to exercise, lack of access to mental health services, isolation, and change in work (ie, working from home, working more/less hours). Figure 1B summarizes the challenges experienced due to social distancing with the most frequently reported being loneliness and performing daily tasks. The mean PSS score was 15.4, indicating moderate stress. Individuals with DM, women, and individuals younger than 30 y of age reported significantly higher levels of stress (Table 2) . However, when adjusted for sex, the difference in stress scores between disease conditions did not remain. There was a low, significant, negative correlation between lower PSS scores and ability to manage stress (r = À0.47; P < .0001). There was also a low, significant correlation between higher PSS scores and individuals who reported higher pain levels (r = 0.30; P < .0001). The majority of participants (88%) reported that they have been able to manage their stress and have done so most frequently by resting and sleeping, exercising, and meditating. Other ways respondents Over half of the individuals reported that their muscle disease did not change while a third reported a slight worsening of their muscle T A B L E 1 Patient characteristics disease. Forty-three percent of individuals reported that their exercise had decreased. The majority of individuals reported pain with 47% reporting that their pain had worsened during the pandemic. A small percentage of individuals (2.2%) reported that their condition had improved during the pandemic. Of these individuals, 82% reported exercising with 71% reporting increasing their exercise; 67% reported improvement in their pain; and all reported being able to manage their stress. In contrast, a similar percentage reported that their muscle disease was much worse. Of these individuals, 61% reported exercising with half reporting a decrease in exercise; the majority of these individuals reported that their pain had increased, and only 44% reported that they were able to manage their stress. Individuals reported interruptions in healthcare delivery with two-thirds reporting canceled or rescheduled healthcare visits. The majority of these visits were primary care appointments, followed by neurology and therapy (physical or occupational therapy). Fortytwo percent of participants reported that they had participated in a telemedicine appointment via telephone calls or video conferencing platforms. Participants reported a mean satisfaction rating of 75.7 (0 = not satisfied; 100 = completely satisfied), which was consistent across platforms and diseases. A small percentage of individuals (10%) reported that telemedicine visits would be better if they were easier to access and use and if there was audio/visual improvement. The majority of individuals (74.7%) who had experienced both in-person and telemedicine healthcare appointments preferred in-person appointments. Thirteen percent of the participants reported participating in research studies and half of these participants reported that the study had been affected by the pandemic. Participants reported delayed or canceled study visits and/or modifications to study procedures. Despite these challenges, 94% reported that their willingness remained unchanged, or they would be more likely to participate in future research studies. This study revealed that the COVID-19 pandemic and associated guidelines had an impact across social and medical needs of people with MD, and that the vast majority of individuals adhered to local F I G U R E 1 Challenges faced during the COVID-19 pandemic. A, The most common challenges identified were consistent across muscular dystrophies, and included obtaining treatment, social distancing, obtaining essentials, and stress management. B, Looking closer at challenges related to social distancing the most common were feeling alone and performing daily tasks social guidelines. The MD care community has published proposed consensus guidelines for treatments of MD during the COVID-19 pandemic, and they recognized a need for better understanding of the impact of the pandemic and the associated social guidelines on MD and encouraged collaborative treatment decisions with patients, caregivers, and physicians. 11, 12 Individuals with MD experienced challenges related to accessing care and essentials as well as managing stress. The number of individuals who reported that their disease improved during the pandemic was very small; therefore, findings need to be interpreted with caution. However, those who reported that their disease improved during this period were those who participated in exercise, whose pain had improved, and who were able to manage their stress. Conversely, in the small sample of individuals who reported significant worsening of their disease during this period, the majority did not exercise or reduced their exercise, and reported worsening pain and had difficulty managing stress. Studies have shown an association between increased stress and decreased physical activity; therefore, it is not surprising to see an association between increased exercise and improved sense of health. 13 T A B L E 2 Differences in perceived stress by diagnosis, sex, age, and ambulatory status Individuals under the age of 30 y reported significantly more stress than those between the ages of 30 and 60 (3.6; P = .01; CI = 0.72-6.5); and those who were over 60 y of age (6.7; P < .0001; CI = 4.0-9.8). Additionally, individuals between the ages of 30 and 60 y reported significantly more stress than individuals over 60 y of age (3.2; P < .0001; CI = 1.9-4.6). Individuals reported access to care as a major challenge due to 14, 15 Also similar, we found higher stress levels in women and those under 30 y of age. One explanation for this difference may be women are more likely than men to disrupt their daily lives by following social distancing guidelines, staying home from work, and being the primary caregivers of children and sick or elderly family members. 16 The Research has become a main priority for the MD community, as transformational gene-based therapies are currently being approved for inherited neuromuscular diseases. 18 The effect of the pandemic on the willingness of people with MD to participate in research was unknown. Despite delayed or canceled study visits, and modified study procedures during the pandemic, 19,20 study participants have showed great flexibility during this time, and it is encouraging that these experiences have not decreased their likelihood for participating in future research. There are several limitations to this study. We designed this study in response to the pandemic and therefore we do not have data on how these individuals would have responded to these questions prior to the pandemic. Additionally, we did not collect data on a nonmuscular dystrophy sample for comparison and, therefore, could only compare our findings with other reports in the literature. We are also limited by bias that may affect the decision to participate in patient registries or respond to surveys, which may select for more or less affected individuals, individuals without cognitive involvement (DM) or for older individuals who have time to participate in registries. We cannot eliminate the possibility that someone would be in more than one registry and may have responded to the survey twice; although we included a question to minimize this possibility. L.L., N.D., K.H., M.W., D.P., and J.C. report no disclosures. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines. The data that support the findings of this study are available from the corresponding author upon reasonable request. Nicholas Johnson https://orcid.org/0000-0002-3917-4257 Jeffrey Statland https://orcid.org/0000-0003-0790-5315 Introducing the health act: Expanding medicare reimbursement COVID-19: prevention and control measures in community Global telemedicine implementation and integration within health systems to fight the COVID-19 pandemic: a call to action Centers for Disease Control and Prevention. Communities, schools, workplaces, and events Research electronic data capture (REDCap): a metadata-driven methodology and workflow process for providing translational research informatics support A global measure of perceived stress Perceived stress in a probability sample of the United States Who's stressed? Distributions of psychological stress in the United States in probability samples from 1983, 2006 and Perceived stress scale Applied Statistics for the Behavioral Sciences Spinal muscular atrophy care in the COVID-19 pandemic era The care of patients with Duchenne, Becker, and other muscular dystrophies in the COVID-19 pandemic The effects of stress on physical activity and exercise Risk factors of critical & mortal COVID-19 cases: a systematic literature review and meta-analysis Anxiety, worry and perceived stress in the world due to the COVID-19 pandemic Is there a widening gender gap in coronavirus stress Great expectations: virus-mediated gene therapy in neurological disorders Coronavirus Pandemic Brings Hundreds Of U.S. Clinical Trials To A Halt Suspended studies and virtual lab meetings: How the COVID-19 pandemic is affecting epilepsy researchers Additional supporting information may be found online in the Supporting Information section at the end of this article.