While at the Conference in Hamilton in Sept 2015, the following posters were on display. They were manned by students who were available to explain each one. Following is their summary explanation of each poster.
The experience of living with visible differences from a rare and unpredictable disease: Body image in scleroderma
Lisa R. Jewett, Vanessa C. Delisle, Dr. Sandra Peláez, Dr. Vanessa L. Malcarne, Dr. Marie Hudson, & Dr. Brett D. Thombs
Scleroderma is a rare, chronic disease often resulting in unique appearance changes that may pose challenges to the social and psychological lives of individuals with the condition. To date, research on body image and appearance changes in scleroderma has been largely limited to studies using questionnaires; however, a more detailed approach is needed to better understand the effects of the appearance chances from the disease. The present study aimed to explore the relationship between the physical appearance changes experienced by women with scleroderma and various aspects of their daily lives.
In order to answer this question, face-to-face, Skype, or telephone interviews were conducted with 18 women with scleroderma (12 from the Canadian Scleroderma Research Group Registry and six from the Scleroderma Society of Canada). Interview data were transcribed verbatim and analyzed to examine common themes and subthemes.
Preliminary findings revealed three main themes highlighting the relationship between appearance changes and various aspects of daily life, in terms of avoidance behaviours, beliefs about the self, and social relationships. Further subthemes related to these overall themes included avoiding images of the self, avoiding others, and hiding appearance changes; experiencing lowered self-esteem, difficulties with relationships and intimacy, and fear of the appearance changes getting worse; as well as having to manage the reactions of other people, including staring or negative comments.
The physical appearance changes experienced by women with scleroderma have diverse effects on their behaviours, beliefs about the self, and interactions with others. These findings represent an important step towards a more in-depth understanding of the experiences of women living with a rare, chronic, and disfiguring medical condition. Future studies should explore the specific needs of women with scleroderma based on the relationships found in the present study in order to better understand the types of support that might be useful to address these areas.
The Scleroderma Support Group Survey:
Understanding people’s experiences with scleroderma support groups
Vanessa C Delisle, MSc; Stephanie T Gumuchian, BSc; Vanessa L Malcarne, PhD; Ghassan El-Baalbaki, PhD; Sandra Pelaez, PhD; Brett D Thombs, PhD; & the Scleroderma Support Group Team*
*Members of the Scleroderma Support Group Team include: L. Dyas; S. Elrod; C. Fortune; K. Gottesman; M. Richard; R. Riggs; M. Sauve; I. Sevsek; and N. Stephens.
Peer-led support groups play a central role in the lives of many people with scleroderma. There is no published research, however, on the factors that may encourage or discourage people with scleroderma from attending scleroderma support groups or the training and support needs of facilitators of these groups. As such, the aim of the present study was to fill gaps in knowledge about scleroderma support group participation and facilitation. To do this, adults with scleroderma were recruited through the Scleroderma Society of Canada and the Scleroderma Foundation and invited to partake in an anonymous online survey. Separate surveys were administered to (1) scleroderma support group leaders, (2) people with scleroderma who attend support groups, and (3) people with scleroderma who do not attend support groups. The Support Group Leaders Survey assessed the training and support needs of current group leaders. The second survey, the Support Group Members Survey, assessed the reasons why people join and continue to attend support groups, as well as the organizational factors that they consider important for a successful support group. The third and final survey, the Support Group Non-attenders Survey, assessed the factors that influence people’s decisions to not attend support groups. Overall, 80 support group leaders, 171 support group members, and 242 people with scleroderma who do not attend support groups completed the surveys. The results of the Support Group Leaders Survey suggest that current leaders may struggle with managing complex group dynamics, as well as publicizing the group and recruiting group members. The results of the Support Group Members Survey suggest that the most common reasons people with scleroderma join and continue to attend support groups are to receive emotional and practical support, and to get to know others with the disease. Lastly, according to the results of the Support Group Non-attenders Survey, the most common reasons that people with scleroderma do not attend support groups include: already having enough support, availability and accessibility issues, and discomfort seeing others with scleroderma. These findings are important, as they will inform the development of a training program for support groups leaders, which will be disseminated by the Scleroderma Society of Canada and the Scleroderma Foundation and will ultimately improve access to support groups and the ability of support groups to meet members’ needs on a sustained basis.
Can the Self-Efficacy to Manage Chronic Disease Scale be used to evaluate self-efficacy in patients with scleroderma? A Scleroderma Patient- centered Intervention Network Cohort Study
Kira E Riehm, Linda Kwakkenbos, Marie-Eve Carrier, Warren Nielson, Karen Nielsen, Brett D Thombs, & the SPIN Investigators
Many people living with scleroderma have to cope with physical symptoms and disability, as well as emotional challenges related to living with a burdensome, unpredictable chronic disease. A good sense of self-efficacy, or one’s perceived confidence to perform behaviours necessary to effectively manage a health condition, is related to better health status and positive psychological outcomes. However, there is no existing measure of self-efficacy in people with scleroderma. Therefore, the goal of our study was to determine if the Self-Efficacy to Manage Chronic Disease (SEMCD) Scale is a valid measure of self-efficacy in scleroderma. The SEMCD Scale is composed of six questions that assess patients’ confidence in their ability to manage fatigue, pain, and emotional distress, to do things beyond taking medication to reduce the impact of their illness, and to carry out tasks and activities that may reduce the need to see a doctor. We included 553 patients from the Scleroderma Patient-centered Intervention Network (SPIN) Cohort in our study. Participants were mostly women who were married or living as married. On average, patients were 56 years old and had been diagnosed with scleroderma 12 years prior to participation. Statistical analyses showed that the SEMCD Scale consistently and accurately measured self-efficacy in patients with scleroderma. Furthermore, we found that higher self-efficacy was strongly related to better physical functioning and sleep quality, and less depression, anxiety, and pain. Overall, results from our study show that the SEMCD Scale is a valid measure of self-efficacy in patients with scleroderma. Using this measure will permit a better understanding of self-efficacy in patients with scleroderma.
Exploring the sources of emotional distress and management strategies among people living with scleroderma through focus groups: A Scleroderma Patient-centered Intervention Network Study
Stephanie Gumuchian, Vanessa Delisle, Sandra Peláez, Marie-Eve Carrier, Lisa Jewett, Ghassan El-Baalbaki, Linda Kwakkenbos, Brett Thombs, & Susan Bartlett
Scleroderma is a chronic connective tissue disease that has negative physical and psychological implications for patients. Currently, the majority of research that has been done on scleroderma focuses on understanding symptoms and potential treatment options. However, limited research has been conducted to specifically explore the emotionally distressing experiences, such as having feelings of depression or anxiety, or developing fears about the future, that arise as a result of living with this disease.
Three focus group discussions were conducted with a total of 22 participants in order to identify the main sources of emotional distress and coping strategies used by people with scleroderma. Thematic analysis was used to identify common themes that emerged regarding areas of emotional distress, coping strategies, and the emotional experiences. In the focus group interviews, scleroderma was described as an unpredictable and overwhelming disease, resulting in many individuals experiencing illness-related emotional distress. Core sources of emotional distress included experiencing constant pain, not understanding or knowing how to cope with depressive feelings, experiencing symptom comorbidity, and experimenting with new medications and treatments. Scleroderma participants identified experiencing feelings of frustration, fear, and stress when discussing living life with the disease. Common coping strategies were attending support groups, maintaining a positive attitude by adopting a day-to-day mindset, and learning how to manage the symptoms associated with the disease.
Results from group discussions revealed sources of distress and ways of coping that were both common to living with a chronic disease and unique to scleroderma. The findings of the present study offer an in-depth understanding of the experiences with and sources of emotional distress of women and men living with scleroderma.
Characteristics of Patients with Clinically Significant Fatigue in Scleroderma
Danielle Rice, Linda Kwakkenbos, Russell Steele, Marie Hudson, Murray Baron, Brett D. Thombs, and the Canadian Scleroderma Research Group.
Fatigue is common in scleroderma and impacts patients’ quality of life. Many studies use single–item ratings or scores on questionnaires to measure fatigue levels. These methods, however, do not necessarily identify patients with clinically significant fatigue levels that warrant investigation and treatment. A recent study found that using case-definition criteria for Cancer-Related fatigue could identify scleroderma patients with clinically significant fatigue. The aim of this study was to evaluate demographic and disease characteristics of patients with clinically significant fatigue in a large sample of patients with scleroderma. Patients with scleroderma from the Canadian Scleroderma Research Group Registry completed a telephone interview that determined whether patients met fatigue case criteria, including at least 2 weeks of significant fatigue in the preceding month, at least four of seven fatigue-related symptoms, a significant effect of fatigue on work or self care, and not primarily the consequence of a psychiatric disorder. Demographic characteristics (age, sex, education level, marital status and current smoking status) were patient-reported, and the Medsger Scleroderma Disease Severity Scale (general system, peripheral vascular, skin system, joint/tendon involvement, muscle strength, gastrointestinal tract, and lung, heart, and kidney system) and medical comorbidities were recorded by a rheumatologist. Statistical methods were used to compare demographic and disease variables among patients with and without clinically significant fatigue. Among 331 patients with scleroderma who completed the fatigue interview, 113 (34%) had clinically significant fatigue. Variables associated with fatigue case status included current smoking, general system involvement, lung involvement and number of medical comorbidities. No other factors provided useful information on determining fatigue case status. Clinically significant fatigue in patients with scleroderma is associated with smoking, general system involvement, lung involvement and medical comorbidities. Smoking, a modifiable behaviour was strongly associated with fatigue caseness, even after considering disease-related variables.
Sociodemographic and Disease Variables Associated with Fatigue in Scleroderma
Brooke Levis, Linda Kwakkenbos, Stephanie Coronado-Montoya, Marie Hudson, Murray Baron, Brett D. Thombs, and the Canadian Scleroderma Research Group
Individuals living with scleroderma describe their fatigue as an ongoing exhaustion that is not alleviated by rest. Fatigue is common among individuals living with scleroderma, highlighting the importance to better understand factors that contribute to it. The Functional Assessment of Chronic Illness Therapy - Fatigue (FACIT-F) scale is a questionnaire that can be used to measure levels of fatigue. Compared to other existing questionnaires that measure fatigue, the FACIT-F is better able to differentiate between low, medium and high levels of fatigue, which is important in scleroderma, where moderate to severe fatigue is often experienced. In this study, we investigated what factors are associated with fatigue among individuals living with scleroderma. 785 individuals enrolled in the Canadian Scleroderma Research Group (CSRG) Registry were included in the analyses. The average age of participants was 58 years, and most patients were married, non-smokers, and had limited disease subtype. Individuals living with scleroderma who were younger, had less than post-secondary education, more medical conditions and more severe muscle, gastrointestinal, lung and general system disease severity were more likely to experience more severe fatigue. Thus, we found that overall, fatigue is prevalent in scleroderma and is associated with more severe disease. This study contributes to a better understanding of fatigue in scleroderma, and will help inform the development of programs that aim to reduce fatigue.
An international collaboration to collect data and develop online interventions for scleroderma patients: the Scleroderma Patient-centered Intervention Network (SPIN)
Marie-Eve Carrier, Linda Kwakkenbos, Brett D. Thombs, and the SPIN investigators.
Conducting research in a rare disease, such as scleroderma, is challenging because it is difficult to get together a large group of people who live with the illness. To overcome this obstacle, the Scleroderma Patient-centered Intervention Network (SPIN) has formed a collaborative network of people living with scleroderma, patient organizations, researchers, and health care providers from over 30 sites in 8 countries. The aims of SPIN are to develop and test online programs that are accessible, low-cost, and can be delivered on an ongoing basis to support individuals in their efforts to cope with scleroderma.
As a first step, SPIN established its cohort in 2014, to collect online data about concerns that are important to scleroderma patients, so that the online programs can address them. In less than 2 years, SPIN has recruited almost 1000 patients from 28 centers in Canada, the US and the UK. Of this group of scleroderma patients: 85% are women, 94% are English speakers (others are French speakers), 65% are married, and 41% have diffuse scleroderma, while 58% have the limited/sine subtype. The mean age of the cohort participants is 55 years old, and the mean time since their diagnosis is 10 years. Over the next year, in addition to English and French, SPIN will also start recruiting patients who speak Spanish and Dutch.
Once the online programs are developed, some of the cohort participants will be offered to take part in a support program, and others will be followed up in the cohort. This will allow the SPIN team to test if the online programs are effective in improving coping with scleroderma, and if so, make them available to patients around the world through patient organizations partnering with SPIN.
Exercise Habits and Associated Factors in Scleroderma: A Scleroderma Patient-centered Intervention Network (SPIN) Cohort Study
Marleine Azar, Danielle Rice, Linda Kwakkenbos, Marie-Eve Carrier, Ian Shrier, Marie Hudson, Brett D. Thombs, and the SPIN investigators
Physical exercise has health benefits for people with and without medical conditions. Among people with chronic medical conditions, exercise can improve quality of life by reducing fatigue and disability and improved mobility, for instance. In scleroderma, aerobic and resistance exercise training programs appear to be safe and may improve muscle strength, aerobic capacity, and overall physical function. However, little is known about the exercise habits and the frequency of exercise among people with scleroderma. The objective of our study was to assess the proportion of individuals with scleroderma that exercise, the number of hours of exercise they do per week, and the types of exercise they perform. Furthermore, we explored characteristics of people that were associated with exercise. The sample consisted of adult participants with scleroderma who were enrolled in the Scleroderma Patient-centered Intervention Network (SPIN) Cohort. The study included 752 patients who completed questionnaires from March 2014 through August 2015 that included items related to exercise habits, demographics, and medical and psychological characteristics. More than half of participants reported at least some exercise. The patients who exercise reported that they exercise an average of 4.7 hours per week, which is more than what is recommended in Canadian Physical Activity guidelines. Variables that were associated with exercising included having more education, lower body mass index, not smoking, limited or sine disease subtypes, fewer symptoms of depression, less anxiety, better sleep disturbance and less fatigue. A better understanding of exercise patterns and factors associated with exercise will be helpful in designing interventions to encourage patients with scleroderma to exercise.
Measuring quality of life in scleroderma using the PROMIS-29 measure: a Scleroderma Patient-centered Intervention Network (SPIN) Cohort Study
Linda Kwakkenbos, Brett D. Thombs, Susan Bartlett, Marie Hudson, Luc Mouthon, Vanessa Malcarne, Maureen Sauvé, Dinesh Khanna, and the SPIN investigators
Scleroderma has far-reaching consequences for physical health, as well as emotional and social well-being and health-related quality of life. The Patient-Reported Outcomes Measurement Information System (PROMIS®) initiative is a cooperation designed to develop, evaluate, and standardize patient-reported outcome measures across different medical conditions. The PROMIS-29 questionnaire contains 29 items, which include four items each for domains reflecting physical function, anxiety, depression, fatigue, sleep disturbance, pain interference, and ability to perform social roles, plus a single item on pain intensity (range 0-10). In the general population, mean scores for each domain are 50. Higher scores represent more of the domain being measured (e.g., greater sleep disturbance, greater ability to perform social roles). In this study, we explored the associations of PROMIS-29 domains with scleroderma disease characteristics. Individuals with scleroderma were enrolled in the Scleroderma Patient-centered Intervention Network (SPIN) Cohort from 19 centers across Canada, the USA and the UK between July 2014 and May 2015. Upon enrolment, basic medical information was provided by the enrolling physician. In addition, SPIN Cohort participants completed the PROMIS-29 questionnaire. In total, 473 individuals with scleroderma were included in this study. Mean age was 55 years and mean disease duration was 12 years. Most participants were female (87%) with limited cutaneous scleroderma (59%). Means for the PROMIS-29 domains were: function 42.8, anxiety 51.5, depression 50.9, fatigue 55.9, sleep 51.8, roles 47.5, pain interference 55.9, and pain intensity 3.7. The involvement of the gastrointestinal tract was consistently associated with worse outcomes across PROMIS-29 domains. Other clinical variables with decrements in at least 5 domains included: more skin thickening, diffuse disease, and presence of contractures. Thus, scleroderma is associated with significant impairment in PROMIS-29 domains, with certain disease characteristics more commonly associated than others. These data inform priorities for future patient-centered research.