Home-Based Music Therapy to Support Bulbar and Respiratory Functions of Persons with Early and Mid-Stage Amyotrophic Lateral Sclerosis—Protocol and Results from a Feasibility Study
Respiratory failure, malnutrition, aspiration pneumonia, and dehydration are the precursors to mortality in ALS. Loss of natural communication is considered one of the worst aspects of ALS. This first study to test the feasibility of a music therapy protocol for bulbar and respiratory rehabilitation in ALS employs a mixed-methods case study series design with repeated measures. Newly diagnosed patients meeting the inclusion criteria were invited to participate, until the desired sample size (n = 8) was achieved. The protocol was delivered to participants in their homes twice weekly for six weeks. Individualised exercise sets for independent practice were provided. Feasibility data (recruitment, retention, adherence, tolerability, self-motivation and personal impressions) were collected. Bulbar and respiratory changes were objectively measured. Results. A high recruitment rate (100%), a high retention rate (87.5%) and high mean adherence to treatment (95.4%) provide evidence for the feasibility of the study protocol. The treatment was well tolerated. Mean adherence to the suggested independent exercise routine was 53%. The outcome measurements to evaluate the therapy-induced change in bulbar and respiratory functions were defined. Findings suggest that the protocol is safe to use in early- and mid-stage ALS and that music therapy was beneficial for the participants’ bulbar and respiratory functions. Mean trends suggesting that these functions were sustained or improved during the treatment period were observed for most outcome parameters: Maximal Inspiratory Pressure, Maximal Expiratory Pressure, Peak Expiratory Flow, the Center for Neurologic Study—Bulbar Function Scale speech and swallowing subscales, Maximum Phonation Time, Maximum Repetition Rate—Alternating, Maximum Repetition Rate—Sequential, Jitter, Shimmer, NHR, Speaking rate, Speech–pause ratio, Pause frequency, hypernasality level, Time-to-Laryngeal Vestibule Closure, Maximum Pharyngeal Constriction Area, Peak Position of the Hyoid Bone, Total Pharyngeal Residue C24area. Conclusion. The suggested design and protocol are feasible for a larger study, with some modifications, including aerodynamic measure of nasalance, abbreviated voice sampling and psychological screening.
We are excited to let you know that our article titled “Home-based music therapy to support bulbar and respiratory functions of persons with early and mid-stage amyotrophic lateral sclerosis – protocol and results from a feasibility study” has been accepted for publication in a peer-reviewed, open access “Brain Sciences” journal. The link will follow soon!
This study would not be possible without the access to clinical and laboratory resources provided by the study host organization, ALS Centre Moscow (Moscow, Russia), and the tireless support from the administration, the volunteers and the medical team of the Centre, including research assistant Dr. Marina Bialik, research assistant, music therapist Maria Pakosh (Ilchenko), research nurses Yana Batmanova, Sergey Korneychuk and Daria Puzanok, administrative director Anna Kasianova, coordinator Andrey Prokofiev, music therapist Ruslan Semenov, volunteer coordinator Ksenia Nazarova, and the medical director of the study Dr. Lev Brylev, who also served as a field supervisor for this study.
The author of this thesis is immensely grateful to all the external expert advisors who have helped to move this research forward: Dr. Richard Sloan at West Dorset MND Association (UK); Natalia Semina at Rehabilitation centre “Aprel” (Moscow, Russia); Dr. Jordan Green, CCC-SLP, at MGH Institute of Health Professions (Boston, USA); Dr. Emily Plowman, CCC-SLP, at the University of Florida (USA); Dr. Yana Yunusova, CCC-SLP, at the University of Toronto; Anastasia Beltukova, music therapist, at “Turmalin” (Moscow, Russia); Tina Georgievskaya, vocal coach (Moscow, Russia); Alex Sherman at the Neurological Clinical Research Institute at Massachusetts General Hospital (Boston, USA); Prof. Alfredo Raglio at University of Pavia (Italy); Victoria Edwards, SLT, Siofra Mulkerrin, SLT, and the MND team at Arthur Rank Hospice (Cambridge, UK); Dr. Kirill Gorbachev at Buyanov Moscow City Clinical Hospital (Moscow, Russia) and Dr. Vera Fominykh at Institute of Higher Nervous Activity and Neuropsychology (Moscow, Russia), Terri Handler and Dr. Matvey Lukashev at ALS TDI (Cambridge, MA, USA).
Indispensable for the research process was direct help with the research design and pseudonymized data analysis from Jufen Zhang, statistician from School of Medicine, Anglia Ruskin University (Chelmsford, UK), Zhanna Bottaeva, SLP, at Clinical and Research Institute of Emergency Pediatric Surgery and Trauma (CRIEPST); Dr. Ashley Waito, CCC-SLP, researcher at Bulbar Function Lab, Sunnybrook Research Institute (Toronto, Canada), Dr. Marziye Eshghi, CCC-SLP, researcher at Speech and Feeding Disorders Lab, Massachusetts General Hospital (Boston, MA, USA), Fyodor Soykin, IT specialist at Canopy Education Inc (Cambridge, MA, USA), Daria Ivanchenko, logopedist (Moscow, Russia), and Ekaterina Bruno, CCC-SLP (Dallas, USA).
A heart felt thank you to Dr. Alexander Street, music therapist and the first academic supervisor for this research project at Anglia Ruskin University, who provided continuous advice and support during all the stages of this research, and to Prof. Jörg Fachner, the second academic supervisor for this project, who helped to define the research subject and to formulate the study aims and design.
Above all, the author is grateful to all the research participants and their caregivers, as well as to Maria Gostintseva, Andrey Demidov, Pavel Andreev, Victoria Petrova, Sergey Borisenko, Anthony Carbajal and many other persons living with ALS, to ALS caregivers, volunteers and care specialists who have inspired this research.
This study was generously sponsored by The Stephen Hawking Foundation (UK), BOSE Corporation (USA), Remedywave (USA) and crowdfunding donations through Palliative Care Initiative (USA). Thank you.
Music therapy is evidence-based professional clinical application of music and its elements to improve psychological, emotional, cognitive, physical and social health and wellbeing of human individuals and communities (Davis, Gfeller, Thaut, & American Music Therapy Association, 2008), (Pedersen & Wigram 2002), (Wheeler, 2015), (Hanser, 2018).
“The new approach involves injecting shRNA—an artificial RNA molecule capable of silencing or turning off a targeted gene—that is delivered to cells via a harmless adeno-associated virus. In the new research, single injections of the shRNA-carrying virus were placed at two sites in the spinal cord of adult mice expressing an ALS-causing mutation of the SOD1 gene, either just before disease onset or when the animals had begun showing symptoms”.
“In adult mice already displaying ALS-like symptoms, the injection effectively blocked further disease progression and degeneration of motor neurons”.