Title:
Emotional wellbeing in those with chronic illness due to a genetic mutation in valosin-containing protein

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Jones, Mallory
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Abstract
Valosin-containing protein (VCP) is a ubiquitous protein and a member of the ATPases Associated with a variety of cellular Activities (AAA proteins) superfamily. VCP has many functions in the cell including protein quality control, membrane fusion, and maintenance of the cell cycle and apoptosis. Mutations of the gene encoding VCP lead to a rare but devastating disorder with several pathologies including inclusion body myopathy, Paget’s disease of bone, ALS, and frontotemporal dementia. People who develop VCP Disease often feel disenfranchised with their diagnosis, being unable to access appropriate treatments or support systems. Another complication of its rare nature is that this disease is frequently misdiagnosed, due in large part to lack of awareness. Advocacy programs like Cure VCP Disease, Inc. seek to educate caregivers, patients, and medical professionals about the disease and advocate for increased research and resources devoted to its cure. These groups are an integral facet of the rare disease community, and it is imperative that the scientific community bolster and encourage the work of these groups. This review will examine existing literature concerning VCP Disease and will present what is currently known of this multisystem proteinopathy through the lens of patient advocacy. Unpublished and deidentified testimonials of several patients and caregivers will be presented to emphasize the importance of patient advocacy work. Additionally, grip strength data obtained using a dynamometer is analyzed in an effort to identify factors for prediction of symptom progression.
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Date Issued
2023-01-18
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Text
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Undergraduate Thesis
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