Abstract
Individuals with adult-onset muscular dystrophies do not die from the disease but have progressive loss of occupational performance (OP) and quality of life. There is no consistent pathway for referral and access to rehab services. OTs are underutilized, even though the greatest need is improvement in OP. The lack of guidelines makes outcomes inconsistent. To work, gene therapies require muscle bulk. OTs may train clients in “muscle protection ” to maintain muscle, giving them a chance at a cure.
Primary Author and Speaker: Kaitlin Graf
Additional Authors and Speakers: Renuka Roche
Contributing Authors: Joseph Roche
Muscular dystrophies (MDs) are characterized by progressive loss of muscle and increasing weakness. There is no known cure for MDs. Individuals with adult-onset MDs do not die from the disease but they have progressive loss of function and quality of life (QoL). In a previous study, we found that adults with MDs had significant loss of occupational performance (OP), but these individuals did not access rehab services especially OT. Review of the literature shows no standard of rehab care for these individuals and recommendations for exercise, activity and muscle use. Therefore in this study, we wanted to understand the perspective of rehab professionals including OTs on rehab access, available reasources and intervention philosophies for this population.
We utilized a phenomenological study design with semi-structured interview. Using the steps described by Kallio, et al. (2016), we developed a semi-structured interview guide. To recruit rehab professionals who work with the MD population, we contacted the adult neuromuscular clinics and neuromuscular outpatient clinics. We recruited more professionals through the clinicians who had participated. We conducted 12 interviews with: 5 OTs, 5PTs, 1 neurologist and 1 physiatrist, across 2 US states. Interviews were completed over the phone or in person by two interviewers. They were recorded transcribed using Wreally Transcription Services. Once we had raw transcribed scripts, we (2 coders, separately) identified preliminary codes in order to describe the content, then searched for patterns / themes in codes across the different interviews, and defined the themes.
Four overarching themes emerged: the role of OT and how OT services are utilized as part of rehab teams, the use of adaptive equipment and the timing of its introduction, inconsistencies regarding muscle use in individuals with MDs, and the paucity of resources both practitioners and for clients and their families in the context of rehab. There did not appear a consistent pathway for referral and access to rehab services for adults with MD as it exists for other conditions such as stroke. OTs were utilized as consultants and mostly for recommending but not training in use of adaptive equipment or other strategies. Early introduction of adaptive equipment is often resisted by patients, reportedly because they do not want others to see them as being disabled. Improved mobility seems to improve QoL, however, both professionals and individuals are hesitant to give up “walking”. Rehab professionals note that quality of life is improved with the acceptance and use of adaptive equipment. Mirroring the literature, we found inconsistency among professionals regarding recommendations for how much activity to do and type of exercise (cardiac vs strengthening. The lack of definitive intervention guidelines and resources for practitioners seems to make intervention very variable and outcomes inconsistent.
Adults with MDs have an average lifespan but progressively declining OP and QoL. However, there is no consistent system in place across 2 US states in terms of accessing rehab as they progressively decline in function. There also appears to be a lack of guiding philosophies for interventions in this population Standardization of care would allow patients to be seen at non-neuro or MD clinics more often for ‘tune-ups’ or other functional concerns, allowing them to maintain a good quality of life.Some recent basic science studies are suggest that overuse of muscles in this population causes significant loss of muscle fibers. For gene therapies to work, there needs to be muscle bulk. OTs may train clients in what we call “muscle protection” to maintain muscle, giving them a chance at a cure.
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