Kathleen R. Bogart

Living With Moebius Syndrome: Adjustment, Social Competence, and Satisfaction With Life

CONTEXT Moebius syndrome is a rare congenital condition that results in bilateral facial paralysis. Several studies have reported social interaction and adjustment problems in people with Moebius syndrome and other facial movement disorders, presumably resulting from lack of facial expression. OBJECTIVE To determine whether adults with Moebius syndrome experience increased anxiety and depression and/or decreased social competence and satisfaction with life compared with people without facial movement disorders. DESIGN Internet-based quasi-experimental study with comparison group. PARTICIPANTS Thirty-seven adults with Moebius syndrome recruited through the United States-based Moebius Syndrome Foundation newsletter and Web site and 37 age- and gender-matched control participants recruited through a university participant database. MEASURES Anxiety and depression, social competence, satisfaction with life, ability to express emotion facially, and questions about Moebius syndrome symptoms. RESULTS People with Moebius syndrome reported significantly lower social competence than the matched control group and normative data but did not differ significantly from the control group or norms in anxiety, depression, or satisfaction with life. In people with Moebius syndrome, degree of facial expression impairment was not significantly related to the adjustment variables. CONCLUSIONS Many people with Moebius syndrome are better adjusted than previous research suggests, despite their difficulties with social interaction. To enhance interaction, people with Moebius syndrome could compensate for the lack of facial expression with alternative expressive channels.

Compensatory expressive behavior for facial paralysis: adaptation to congenital or acquired disability

OBJECTIVE Although there has been little research on the adaptive behavior of people with congenital compared to acquired disability, there is reason to predict that people with congenital conditions may be better adapted because they have lived with their conditions for their entire lives (Smart, 2008). We examined whether people with congenital facial paralysis (FP), compared to people with acquired FP, compensate more for impoverished facial expression by using alternative channels of expression (i.e., voice and body). RESEARCH METHOD/DESIGN Participants with congenital (n = 13) and acquired (n = 14) FP were videotaped while recalling emotional events. MAIN OUTCOME MEASURES Expressive verbal behavior was measured using the Linguistic Inquiry Word Count (Pennebaker, Booth, & Francis, 2007). Nonverbal behavior and FP severity were rated by trained coders. RESULTS People with congenital FP, compared to acquired FP, used more compensatory expressive verbal and nonverbal behavior in their language, voices, and bodies. The extent of FP severity had little effect on compensatory expressivity. CONCLUSIONS/IMPLICATIONS This study provides the first behavioral evidence that people with congenital FP use more adaptations to express themselves than people with acquired FP. These behaviors could inform social functioning interventions for people with FP.

Social Interaction Experiences of Adults with Moebius Syndrome: A Focus Group

This focus group study explored the social interaction experiences and strategies of 12 adults with Moebius Syndrome, a rare congenital condition characterized by facial paralysis. Content analysis revealed five themes of social functioning: social engagement/disengagement; resilience/sensitivity; social support/stigma; being understood/misunderstood; and public awareness/lack of awareness of Moebius Syndrome. Participants used compensatory expressive strategies such as vocal tone, gestures, and humor. The combination of being unable to express oneself with the face, having a facial difference, and having a rare disease is particularly stigmatizing. Increasing public awareness and developing social skills programs for people with facial paralysis could facilitate social functioning.

Is Apathy a Valid and Meaningful Symptom or Syndrome in Parkinson's Disease? A Critical Review

OBJECTIVE To review the nearly 30 papers suggesting that apathy may occur frequently in Parkinsons disease (PD) and that it may be a symptom or syndrome that is separate from depression. METHOD Literature review. RESULTS The review revealed three possible explanations for the high rates of apathy found in PD. First, there is much interest in an endogenous explanation of apathy because the basal ganglia and dopamine are implicated in both PD and apathy. Researchers have suggested links between apathy, dopamine depletion, and basal ganglia dysfunction in PD. Second, apathy in PD may be exogenous, resulting from disability and activity restriction. Third, apathy findings are inflated due to conceptual problems and methodological confounds. Indeed, apathy may be consistently confounded with symptoms of PD, including expressive masking, depression, disability, and cognitive decline. CONCLUSION Because apathy has not yet been found to relate to meaningful patient outcomes, and it appears that other factors such as depression and cognition are more strongly related to quality of life than apathy, there is not enough evidence to conclude that apathy is a clinically meaningful syndrome in PD. The role of PD in motivation is of theoretical and practical interest and deserves further research.

Disability identity predicts lower anxiety and depression in multiple sclerosis.

PURPOSE/OBJECTIVE Disability identity involves affirming ones status as a person with a disability and incorporating this group membership into ones identity. It is associated with well-being in populations of people with disabilities, but its relationship with depression and anxiety in multiple sclerosis (MS) has yet to be examined. It was hypothesized that disability identity would predict lower depression and anxiety above and beyond the effects of demographic and disability variables. RESEARCH METHOD/DESIGN One hundred and six participants with MS completed a cross-sectional Internet survey measuring disability identity, depression and anxiety, activities of daily living performance, MS duration, and demographic variables. Inclusion criteria were as follows: self-reported diagnosis of MS, self-reported impairment in walking, age 18 or older, living in the United States, and English fluency. RESULTS Hierarchical regressions revealed that disability identity was a unique predictor of lower depression and anxiety. Older age and higher activities of daily living performance also predicted lower depression and anxiety. CONCLUSIONS/IMPLICATIONS Stronger disability identity predicts lower psychological distress in MS. Clinical implications include supporting patient disability identity by encouraging involvement in disability support and advocacy groups. Future research should examine psychosocial interventions to affirm identity.

The unfairness of it all: Exploring the role of injustice appraisals in rehabilitation outcomes.

OBJECTIVE A fundamental principle of rehabilitation psychology is that individual appraisals of the social and physical environment-including injury itself-have profound consequences for coping and adjustment. When core assumptions of a just and predictable world are violated and accompanied by ostensibly undeserved suffering and loss, perceptions of injustice can arise. Given the role of appraisal processes in adjustment to disability, mounting empirical support, and absence of targeted interventions, the current article considers perceptions of injustice regarding personal injury/disability as a fundamental appraisal affecting rehabilitation outcomes. RESEARCH METHOD The authors review theory underpinning the relevance of injustice appraisals and critically examine existing literature regarding the impact of perceived injustice and related constructs (i.e., attribution of blame, anger, and belief in a just world) on adjustment following injury. RESULTS The authors bring attention to perceptions of injustice regarding personal injury/disability as a fundamental appraisal affecting rehabilitation outcomes. Dimensions of the social environment that have not received substantial attention in current research on condition-related injustice appraisals are highlighted. IMPLICATIONS Perceived injustice is a potentially central appraisal process to physical and psychological outcomes in the context of rehabilitation. Research regarding the role of perceived injustice, related constructs, and potential social/environmental modulators of injustice perception is still in its infancy. Guided buy its foundational principles, the field of rehabilitation psychology can broaden and shape inquiry regarding perceived injustice. This article aims to guide future research, offer concepts for key areas of discourse, and consider potential interventions in the rehabilitation psychology domain.

“People are all about appearances”: A focus group of teenagers with Moebius Syndrome

This focus group study examined the social experiences of 10 adolescents aged 12–17 years with Moebius Syndrome, a rare condition involving congenital facial paralysis. Content analysis revealed five themes: social engagement/disengagement, resilience/sensitivity, social support/stigma, being understood/misunderstood, and public awareness/lack of awareness of Moebius Syndrome. Compared to previous research on adults with Moebius Syndrome, adolescents were more likely to experience bullying and social strain. Like adults, teenagers displayed resilience and communication skills. Greater public awareness is key to reducing the stigma associated with this condition, and social skills programs should be developed to support those who struggle with social functioning.

Training in compensatory strategies enhances rapport in interactions involving people with Möbius Syndrome

In the exploratory study reported here, we tested the efficacy of an intervention designed to train teenagers with Möbius syndrome (MS) to increase the use of alternative communication strategies (e.g., gestures) to compensate for their lack of facial expressivity. Specifically, we expected the intervention to increase the level of rapport experienced in social interactions by our participants. In addition, we aimed to identify the mechanisms responsible for any such increase in rapport. In the study, five teenagers with MS interacted with three naïve participants without MS before the intervention, and with three different naïve participants without MS after the intervention. Rapport was assessed by self-report and by behavioral coders who rated videos of the interactions. Individual non-verbal behavior was assessed via behavioral coders, whereas verbal behavior was automatically extracted from the sound files. Alignment was assessed using cross recurrence quantification analysis and mixed-effects models. The results showed that observer-coded rapport was greater after the intervention, whereas self-reported rapport did not change significantly. Observer-coded gesture and expressivity increased in participants with and without MS, whereas overall linguistic alignment decreased. Fidgeting and repetitiveness of verbal behavior also decreased in both groups. In sum, the intervention may impact non-verbal and verbal behavior in participants with and without MS, increasing rapport as well as overall gesturing, while decreasing alignment.

Disability Pride Protects Self-Esteem Through the Rejection-Identification Model.

Purpose/Objective: The rejection-identification model (RIM) argues that the negative impacts of stigma, such as decreased self-esteem, may be mitigated when members of the stigmatized group choose to identify with each other rather than with the majority culture. A previously unstudied potential RIM stigma-reduction mechanism is disability pride, which views disability as a source of valuable, enriching, and positive experience. Impairment, personal, and environmental factors based on the International Classification of Functioning, Disability and Health (ICF) predict whether people will categorize themselves as disabled, but predictors of pride have received little examination. The purpose of this study was to (a) explore whether ICF factors predict disability pride, and (b) assess whether disability pride mediates a relationship between stigma and self-esteem, supporting RIM. Research Method/Design: Participants completed an Internet-based survey assessing pride, self-esteem, and ICF factors. Disability was not mentioned in recruitment materials to prevent selection biases. People who reported at least 1 impairment (n = 710) were included in analyses. Results: ICF personal and environmental factors (stigma, social support, and being a person of color), but not impairment factors, predicted disability pride. Supporting RIM, disability pride partially mediated the relationship between stigma and self-esteem. Conclusions/Implications: Disability pride is a promising way to protect self-esteem against stigma. Disability pride is still a rare phenomenon. Given that pride is associated with social support, stigma, and, to a lesser extent, ethnicity, but not impairment characteristics, interventions might focus on personal and environmental factors like these to promote pride.

Health-related quality of life among adults with diverse rare disorders

BackgroundTwenty-five to 30 million Americans live with a rare disease (RD) and share challenges unique to RD. The majority of research on RDs has focused on etiology, treatment and care, while the limited health-related quality of life (HRQL) research has been restricted to single RDs, small samples, or non-validated measures. This study reports HRQL among adults with diverse RDs, and compares their scores to those of the U.S. population and people with common chronic health conditions.MethodsWe conducted a cross-sectional survey of adults living in the U.S. diagnosed with any RD. Participants were recruited through RD organizations and completed the online survey between December 2016 and May 2017 (n = 1218). HRQL was assessed using the standardized Patient-Reported Outcomes Measurement Information System (PROMIS). RDs were classified into categories defined by Orphanet. Means and 95% confidence intervals were calculated for the main sample and for RD categories and were compared to published U.S. population norms and common chronic disease norms. Intercorrelations were conducted between HRQL, demographics, and RD experiences.ResultsWhen compared to the norms for the U.S. population and for those with common chronic diseases, mean HRQL scores were significantly poorer across all six PROMIS domains for the main sample, and were usually poorer when analyzed by sub-sets of specific RD classifications. People with rare systemic and rheumatologic, neurological, and immune diseases had the poorest HRQL. Participants had poorer HRQL if they had multiple RDs, lower income, were female, or older. Having symptoms longer was associated with worse HRQL, however, having a formal diagnosis longer was associated with better HRQL.ConclusionsThis study is the first to examine HRQL in a large, heterogeneous sample of RDs using validated measures. There is a significant disparity in HRQL among people with RD compared to the general population and people with common chronic diseases. Poor HRQL could be attributed to challenges accessing diagnoses, medical information, treatment, psychosocial support, and coping with stigma and uncertainty. As most individuals with RDs will not be cured in their lifetimes, identifying ways to improve HRQL is crucial to patient-centered care and should be a funding priority.