Dysphagia Clinical Trial
Official title:
Measuring the Effects of a Music Therapy Protocol on Respiratory and Bulbar Functions of Patients With Early and Mid-stage Amyotrophic Lateral Sclerosis: Mixed Methods Single Case Study Series
This study evaluates potential of music therapy treatment to support breathing, speech, swallow and cough of persons with amyotrophic lateral sclerosis (ALS). Music therapy is the clinical use of music and its elements to enhance human health and wellbeing. Application of music therapy principles in neurorehabilitation allow to treat cognitive, sensory, and motor dysfunctions.
ALS clinical presentation and pathophysiology
Amyotrophic lateral sclerosis (ALS) is a group of rapidly progressive fatal neurological
diseases involving the brain and spinal cord. Clinical presentation is phenotypically
heterogeneous and depends on the type of onset. The pathophysiology mechanisms behind ALS are
not clear and may include oxidative stress, protein misfolding and aggregation, skeletal
muscle dysfunction, glutamate excitotoxicity, mitochondrial dysfunction, neuroinflammation,
and apoptosis. Whilst in the past ALS was considered distinctly a disorder of the motor
system, current evidence suggests that some cognitive (ALSci) or behavioural (ALSbi)
impairment occurs in up to 50% of cases, and co-morbid dementia (ALS-FTD) occurs in
approximately 14% of patients with a new diagnosis of ALS. The notion that "pure" ALS and
"pure" FTD may present two extremes of one disease continuum is reinforced by identification
of transactive response DNA‐binding protein 43 (TDP‐43) as a major pathological substrate
underlying both diseases. Emotional lability (pseudobulbar affect), a symptom frequently
correlated with bulbar involvement in ALS, may also be confusing and disruptive, especially
when communicating with those who are not aware of the nature of the problem.
Music therapy potential in multidisciplinary model of ALS care
Considering the multiple and complex needs of people with ALS (PALS), the National Institute
for Health and Care Excellence (UK) guideline [NG42] suggests coordinated care using a clinic
based, specialist ALS multidisciplinary team approach. American Academy of Neurology
recommends multidisciplinary team (MDT) model of care where patients are seen by a
comprehensive team of health care professionals who each focus on specific health domains
including walking, breathing, speaking, eating, activities of daily living, and psychosocial
needs during one clinical visit. Multidisciplinary ALS care has been shown to increase
survival of people with PALS and to improve their mental QoL.
More rehabilitation options have to be considered for PALS, enabling them to reach their
fullest potential, delaying the disease progression and prolonging lifespan. Music therapy
(MT) is the clinical use of music and its elements to accomplish individualized health goals
within a therapeutic relationship. Music engages vast network of regions located in both
hemispheres of the brain and shares processing components with other functions, such as those
involved in language, movement, reasoning and experiencing emotions. Application of MT
principles in neurorehabilitation allow to treat cognitive, sensory, and motor dysfunctions.
Professionally trained music therapists are well equipped to provide symptomatic care for
people with neurodegenerative diseases, adapting to increasing and changing disability of
each patient as the disease progresses, whilst maintaining and developing trusting
therapeutic relationship established early in the disease course. Albeit currently underused,
MT could be one of the modalities of supportive rehabilitation in ALS.
Research on clinical MT applications for ALS is scarce and presents significant challenges
due to heterogeneity of ALS clinical presentation and progression, poor scientific
understanding of the disease mechanisms and the ethical issues of research involving
terminally ill people. It has been suggested that MT could contribute to interdisciplinary
ALS care. There is anecdotal evidence that MT increases mind-body connection, reduces
distressing physical symptoms such as dyspnoea and pain, and associated feelings of
loneliness, anxiety and sadness for patients with advanced ALS, is "pleasant and restorative"
for PALS with tracheostomy and their families , and provides families affected by ALS with
opportunities for shared meaningful activities. The first randomized controlled trial (RCT)
of music therapy effect on people living with ALS found that active MT increased
communication, improved QoL and decreased the physical symptoms of the disease for people
with ALS during hospital stay. Music-assisted relaxation may be a useful strategy to optimize
noninvasive ventilation (NIV) experience for people with ALS.
It may be concluded that, firstly, in most cases, MT is available for PALS at the end of
life, when natural communication and motor functions are lost or limited, that music
therapists are normally not included into multidisciplinary model of ALS care and that the
predominant type of music intervention being used for treatment is music listening. Secondly,
little research has been done to understand the effects of MT interventions conducted with
patients at earlier stages of ALS and no published research addressing the use of MT
techniques for neurorehabilitation (e.g. supporting motor, cognitive, respiratory,
swallowing, speech functions) of PALS has been found.
Role of exercise in ALS treatment
Prior to recently the exercise has been discouraged for people with ALS, the recommendation
based on the assumption that exercise can lead to muscle fatigue and, hence, increase
patient's disability. This assumption however is not supported by scientific evidence,
whilst, to the contrary, there is evidence that physical inactivity secondary to ALS may lead
to cardiovascular deconditioning, disuse weakness and consequential muscle atrophy. Existing
literature reviews support evidence for moderate exercise in ALS, whilst highlighting
importance of supervised, individualized training programs. Cochrane review concluded there
was no solid evidence to deem exercise in ALS beneficial or harmful and emphasized the need
for further research. Recent data suggests that exercise may be beneficial for cellular and
morphological adaptations in motor neuron function and thus may help to maintain motor
function. Finally, preliminary results of a new RCT suggest that strictly monitored exercise
programs reduce motor deterioration in ALS. Thus, in current clinical practice regular
moderate exercise is encouraged for people with ALS to prolong mobility and independence.
MT for management of bulbar and respiratory symptoms in ALS
Even more so than in case of the general physical exercise, there is lasting controversy in
regards to the role of exercise in management of bulbar and respiratory ALS symptoms. 93% of
people with ALS experience speech impairments and 85% present with swallowing dysfunction at
some point during the disease progression. Loss of natural communication is regarded by the
patients as one of the worst aspects of the disease. Respiratory failure has been cited as
the leading cause of death, and weakening of the respiratory function and adherence to NIV as
the leading causes of anxiety of people with ALS. Patients with dysphagia reported social
isolation, fear and decreased mental health. Dysphagia and malnutrition contribute to 25.9%
of ALS mortality and increase the risk of death by 7.7 times. Aspiration pneumonia and
dehydration have also been cited among the leading factors contributing to mortality in ALS.
Although there is lack of evidence supporting the use of strengthening exercises for
improving speech in patients with ALS, there is no evidence of such exercises being harmful.
Respiratory training may have positive effect on respiratory and swallowing functions of
people with ALS. Whilst physical and psycho-emotional needs of people with ALS that MT can
potentially attend to are many and varied, literature overview and empirical evidence suggest
that researching the role of individualized, carefully monitored MT program to support
respiratory and bulbar functions of people with ALS currently constitute the highest priority
as these functions directly affect survival.
Study aim and significance
Applied MT research in multidisciplinary clinical context allows to systematically look at
bulbar and respiratory functions support in ALS, which is important to do, since swallowing,
vocalization and breathing are tightly coordinated, and close relationship exists between
these processes, in terms of location and activation of the neurons.
This study intends to determine feasibility of a MT protocol as intervention to support
respiration, cough, swallowing and speech functions of persons with amyotrophic lateral
sclerosis (PALS). Since MT constitutes a new treatment modality for bulbar and respiratory
dysfunction in ALS, the study is focused primarily on safety and tolerability of the
treatment protocol.
Music therapy treatment protocol
Upper motor neurons and lower motor neurons deterioration in ALS results in dysarthria and
dysphagia of spastic-flaccid type, although actual presentation varies in each patient and
changes with the disease progression. Generally, lower motor neurone (LMN) involvement,
characteristic of bulbar onset, leads to flaccid presentation, whilst degeneration of upper
motor neurone (UMN), characteristic of spinal onset, results in spasticity of bulbar muscles.
Muscle relaxation and moderate exercise, as well as learning efficient breathing and voice
production techniques, voice care techniques and ALS-specific communication strategies may be
helpful, rather than rigorous strengthening oral motor exercises. The protocol serves the
following primary therapy objectives:
- to increase breath support,
- to increase muscle relaxation,
- to increase speech rate,
- to prevent / decrease hypernasality,
- to maintain swallowing coordination.
ALS-specific, individualized MT protocol was delivered to study participants in their homes
twice weekly for the duration of six weeks by the researcher, who is a board certified music
therapist and neurologic music therapist, with experience of working with this patient group
and in this setting. Facilitating music structures have been composed by the researcher to
support cueing, timing and intensity of breathing and vocalization exercises. These
structures were regularly modified to suit the unique capabilities, current individual
demands and progress of each participant. One familiar song, selected by the participant, was
used for each participant in therapeutic singing exercise closing each session.
Data collection
Assessing bulbar dysfunction in ALS presents additional challenge to a researcher, as the
existing tools may be not sensitive enough to reliably measure the change, given that ALS is
a rapidly degenerating disease and that the rate of deterioration varies greatly from patient
to patient. An array of subjective and objective measurements for bulbar and respiratory
changes are used, including standard respiratory tools (FVC, MIP, MEP, PCF),
videofluoroscopic swallow study, visual analogue scales for ease of speech and respiration,
machine analysis of recorded vocal samples, semi-structured interviews and session
descriptions, to explore the feasibility of systematic bulbar and respiratory assessment in
ALS for future research.
;
Status | Clinical Trial | Phase | |
---|---|---|---|
Active, not recruiting |
NCT03711474 -
Dysphagia Following Anterior Cervical Spine Surgery; Single Dose Steroid vs Saline (DysDexVSSal)
|
Phase 4 | |
Enrolling by invitation |
NCT04074356 -
Non-invasive Markers of Esophageal Function in Adults
|
N/A | |
Suspended |
NCT04349462 -
Post Critical Illness Dysphagia in the Intensive Care Unit
|
N/A | |
Not yet recruiting |
NCT05982977 -
Study on the Effect Mechanism of Acupuncture Combined With Swallowing Training in Oral Dysphagia of Stroke
|
N/A | |
Recruiting |
NCT03605381 -
MORbidity PRevalence Estimate In StrokE
|
||
Active, not recruiting |
NCT03455608 -
PRO-ACTIVE: Prophylactic Swallow Intervention for Patients Receiving Radiotherapy for Head and Neck Cancer
|
N/A | |
Recruiting |
NCT03682081 -
Interventions for Patients With Alzheimer's Disease and Dysphagia
|
N/A | |
Completed |
NCT05700838 -
Refining Cough Skill Training in Parkinson's Disease and Dysphagia
|
Phase 1 | |
Not yet recruiting |
NCT04064333 -
Slow-Stream Expiratory Muscle Strength Training for Veterans With Dysphagia Living in Long-term Care
|
N/A | |
Completed |
NCT02927691 -
Novel Management of Airway Protection in Parkinson's Disease: A Clinical Trial
|
Phase 2 | |
Not yet recruiting |
NCT02724761 -
Prophylactic Racemic Epinephrine in Anterior Cervical Discectomy and Fusion
|
N/A | |
Completed |
NCT01919112 -
Fostering Eating After Stroke With Transcranial Direct Current Stimulation
|
N/A | |
Completed |
NCT01370083 -
Tongue Pressure Profile Training for Dysphagia Post Stroke
|
Phase 2 | |
Withdrawn |
NCT01200147 -
Effectiveness of Rupture of Schatzki's Ring Using Biopsy Forceps Versus SIngle Dilation
|
N/A | |
Completed |
NCT01723358 -
Neuromuscular Electrical Stimulation (NMES) Treatment Technique Therapy in the Management of Young Infants With Severe Dysphagia
|
Phase 2 | |
Completed |
NCT00570557 -
Development of a Web-Based Course to Maintain Skills in Nurses Trained to Screen for Dysphagia
|
N/A | |
Recruiting |
NCT00166751 -
Sonographic Assessment of Laryngeal Elevation
|
N/A | |
Completed |
NCT01476241 -
Percutaneous Endoscopic Gastrostomy Tube Placement by Otorhinolaryngologist
|
N/A | |
Completed |
NCT00717028 -
Functional Endoscopic Evaluation of Swallowing
|
N/A | |
Recruiting |
NCT04887415 -
Respiratory Strength Training in Cardiac Surgical Patients
|
N/A |