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
试验速览
- 阶段
- 不适用
- 发起方
- 入组人数
- 8
- 试验地点
- 2
- 主要终点
- Change of Forced Vital Capacity (FVC) from baseline at Week 6, Week 12, Week 16
研究概览
简要总结
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.
详细描述
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.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Health Services Research
- 盲法
- None
入排标准
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Newly diagnosed patients at ALS Centre Moscow (consecutive sampling)
- •Diagnosis of probable or definite ALS by the revised El Escorial criteria confirmed by neurologist at ALS Moscow Centre prior to screening for enrollment.
- •Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised (ALSFRS-R) bulbar subscore ≥ 9, but ≤ 11, where bulbar score=the sum of ALSFRS-R questions 1-3 (maximum score of 12)
- •Forced vital capacity (FVC) greater than 60%
- •Unimpaired cognition as evidenced by Edinburgh Cognitive and Behavioural ALS Screen (ECAS) cut-off scores adjusted for age and education
- •Able to consent to treatment
- •Native speakers of Russian
排除标准
- •Tracheostomy or mechanical ventilation
- •Diaphragmatic pacer
- •Significant concurrent respiratory disease
- •Allergies to barium
- •Receiving any other experimental treatment for dysarthria, dysphagia, dystussia and dyspnoea for the duration of the study
- •Receiving any other music therapy treatment for the duration of the study
研究组 & 干预措施
Music therapy protocol
Each participant received 12 home-based music therapy treatment sessions over 6-week time period.
干预措施: Music therapy (Procedure)
结局指标
主要结局
Change of Forced Vital Capacity (FVC) from baseline at Week 6, Week 12, Week 16
时间窗: Throughout the field phase of the study (16 weeks)
FVC is a standard spirometry test which measures the volume of air that can forcibly be blown out after full inspiration.
Change of Peak Cough Flow (PCF) from baseline at Week 6, Week 12, Week 16
时间窗: Throughout the field phase of the study (16 weeks)
PCF is a measure of cough effectiveness, portable peak flow meter was used.
Change of Maximal Inspiratory Pressure (MIP) from baseline at Week 6, Week 12, Week 16
时间窗: Throughout the field phase of the study (16 weeks)
MIP is the inspiratory pressure generated against a completely occluded airway; used to evaluate inspiratory respiratory muscle strength.
Change of Maximal Expiratory Pressure (MEP) from baseline at Week 6, Week 12, Week 16
时间窗: Throughout the field phase of the study (16 weeks)
MIP is a measure of the strength of respiratory muscles, obtained by having the patient exhale as strongly as possible against a mouthpiece.
Change of Center for Neurologic Study Bulbar Function Scale (CNS-BFS) Swallowing subscore from baseline at Week 6, Week 12, Week 16
时间窗: Throughout the field phase of the study (16 weeks)
The Center for Neurologic Study Bulbar Function Scale (CNS-BFS) consists of three domains (swallowing, speech, and salivation), each of which is assessed with a 7-item, self report questionnaire. Each question is scored from "1" (does not apply) to "5" (applies most of the time). Swallowing domain subscore can range from "7" (best outcome) to "35" (worst outcome).
Change of Center for Neurologic Study Bulbar Function Scale (CNS-BFS) Speech subscore from baseline at Week 6, Week 12, Week 16
时间窗: Throughout the field phase of the study (16 weeks)
The Center for Neurologic Study Bulbar Function Scale (CNS-BFS) consists of three domains (swallowing, speech, and salivation), each of which is assessed with a 7-item, self report questionnaire. Each question is scored from "1" (does not apply) to "5" (applies most of the time). Speech domain subscore can range from "7" (best outcome) to "35" (worst outcome).
Phenomenological analysis of treatment sessions documentation
时间窗: Throughout the therapy phase (6 weeks)
Analysis of narrative accounts of music therapy sessions submitted by the therapist
Phenomenological analysis of participant semi-structured interviews post-treatment
时间窗: Week 13
Analysis of participant's answers to open questions in regards to impressions of music therapy treatment
Change in video fluoroscopic swallowing study (VFSS) results from baseline at Week 6, Week 12
时间窗: Throughout the field phase of the study (16 weeks)
VFSS is an x-ray-based method of evaluating a person's swallowing ability.
Change in acoustic assessment parameters of recorded voice from baseline at Week 6, Week 12, Week 16
时间窗: Throughout the field phase of the study (16 weeks)
Machine-analyzed set of structured voice samples.
Change in Visual Analogue Scale for current perceived ease of respiration from pre-treatment to post-treatment
时间窗: Throughout the therapy phase (6 weeks)
Visual Analogue Scale (VAS) for current perceived ease of respiration is a one question 10-point scale administered at the beginning and at the end of every music therapy session to assess short-term change in current perceived ease of respiration. The outcome can range from "1" (very difficult) to "10" (very easy).
Change of Visual Analogue Scale for current perceived ease of speech from pre-treatment to post-treatment
时间窗: Throughout the therapy phase (6 weeks)
Visual Analogue Scale (VAS) for current perceived ease of speech is a one question 10-point scale administered at the beginning and at the end of every music therapy session to assess short-term change in current perceived ease of speech. The outcome can range from "1" (very difficult) to "10" (very easy).
Phenomenological analysis of participant semi-structured interviews pre-treatment
时间窗: Week 5
Analysis of participant's answers to open questions in regards to expectations for music therapy treatment
Phenomenological analysis of caregiver semi-structured interview pre-treatment
时间窗: Week 5
Analysis of main caregiver's answers to open questions in regards to expectations for music therapy treatment
Phenomenological analysis of caregiver semi-structured interview post-treatment
时间窗: Week 13
Analysis of main caregiver's answers to open questions in regards to impressions of music therapy treatment
次要结局
未报告次要终点
研究者
Alisa Apreleva
Principal Investigator
Anglia Ruskin University