Amyotrophic Lateral Sclerosis: A Spinal-Onset Case Study: Difference between revisions
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== Abstract == | == Abstract == | ||
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive neurological | Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive neurological disorder that affects voluntary muscle movement. Specifically, upper and lower motor neurons are destroyed which results in various devastating symptoms. Patients die on average 3-5 years after symptoms onset due to respiratory failure. The current cause and cure remain unknown. In this case study, | ||
== Introduction == | == Introduction == | ||
Revision as of 00:43, 12 May 2023
Original Editor -Hannah Perreault
Top Contributors - Jessie Fleming
Abstract[edit | edit source]
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive neurological disorder that affects voluntary muscle movement. Specifically, upper and lower motor neurons are destroyed which results in various devastating symptoms. Patients die on average 3-5 years after symptoms onset due to respiratory failure. The current cause and cure remain unknown. In this case study,
Introduction[edit | edit source]
Client Characterstics[edit | edit source]
Examination Findings[edit | edit source]
Clinical Hypothesis[edit | edit source]
Intervention[edit | edit source]
Outcome & Referrals[edit | edit source]
Multidisciplinary teams are vital in the management of ALS, due to the progressive and widespread effect of the disorder on multiple body systems[1]. Thus, it is important to view patients with a holistic lens, taking into consideration how aspects aside from physical body systems (ie. Musculoskeletal, respiratory, etc.) can be greatly impacted, especially in conditions such as ALS. Accordingly, in addition to seeing a physical therapist, Lou would benefit from being referred to other health care professionals including a respiratory therapist (RT), occupational therapist (OT), and dietitian, among others, to address both physical and cognitive complications associated with the condition[1].
One of the major complications of ALS is respiratory insufficiency, including respiratory muscle weakness, ventilatory failure, and a decline in vital capacity [1]. This decline in respiratory functioning can progress to complications including truncated speech, dyspnea, fatigue, orthopnea, and inefficient breathing mechanics, and is the most frequent cause of death in ALS [1] [2]. Moreover, respiratory insufficiency is a strong predictor of quality of life due to its interference with routine and leisure daily activities. Thus, an important referral for Lou is to an RT. The importance of RTs in the management of ALS has been highlighted in various studies. For example, non-invasive ventilation (NIV), now considered the standard of care for respiratory-related complications in ALS, is a highly effective but under-utilized management strategy[2][3]. Its use has been associated with significant improvements in quality of life and survival rates and has added benefits when implemented early on, reinforcing the importance of Lou having an RT as part of his care team [4]. Studies have found patients to be more compliant with NIV when an RT is part of the multidisciplinary clinic they attend, due to enhanced patient and caregiver education on its use and importance and the ability of the therapist to make individualized machine setting adjustments for patients[5]. In addition to implementing interventions such as NIV, RTs are important in the continual monitoring of respiratory function, such as evaluating forced vital capacity and vital capacity, to assess objective changes in status as well as patient-reported subjective symptoms [6]. Thus, it seems reasonable to suggest that an important referral for Lou is to an RT, to minimize the lethal effects of respiratory dysfunction that are seen in ALS and maximize his quality of life for as long as possible.
Since there is currently no cure for ALS and it has a progressive course, OTs can serve as another key addition to the team involved in the care of individuals living with ALS [1]. An OT can help Lou maintain as much independence as possible for as long as possible by helping him find compensatory strategies for completing ADLs and leisure activities, modifying his home and work environment, and optimizing his use of and access to assistive devices. For example, an OT can help fit and educate Lou on using a robotic-assisted glove (RAG) device to help moderate the weakness he experiences, which is especially prevalent in his distal extremities[7]. Such a device will allow Lou to continue to use his hands in a natural manner, helping him retain functional independence and the ability to participate in hobbies such as painting[7]. Having an OT within the multidisciplinary care team can also aid in setting practical and client-centered goals, which may enhance autonomy and feelings of self-control, with the hopes of mitigating Lou’s feelings of depression[8]. Interestingly, RAG has also been found to induce neuroplastic changes in sensorimotor pathways and enhance subjective perceptions of performance in daily tasks in patients with ALS, lending further support for the beneficial effects of an OT [7].
Because nutritional concerns are common among those with ALS, another beneficial referral for Lou is to a dietitian[9]. In the management of ALS, dietitians can improve the nutritional status of patients by providing accurate information and education on calorically sufficient diets, valuable supplements, food modifications to address chewing/swallowing difficulties, possible tube feeding methods, and overall safety of consuming and handling food[9]. Having Lou see a dietitian will help mitigate the progressively worsening factors that contribute to poor nutrition in individuals with ALS, and prevent complications such as excessive weight loss, muscle mass and strength loss, malnutrition, dehydration, choking, and aspiration[9][2]. Likewise, due to Lou’s hypertension, having a dietitian on his care team can help him make optimal decisions about the foods he consumes to ensure he feels as healthy as possible for as long as possible, while still enjoying what he likes.
Referral Letter:
*Please note: this sample referral letter is directed toward Mr. Gherig's GP, for a formal referral to a respiratory therapist (RT).
To Dr. QueensU,
After a comprehensive physiotherapy assessment of Mr. Lou Gherig, whom you diagnosed with ALS, it is highly advised that he receive a referral to a respiratory therapist as well. Mr. Gherig would greatly benefit from seeing a respiratory therapist to assess, monitor, and mitigate the adverse effects of respiratory insufficiency that are starting to appear and will continue to worsen rapidly. Having Mr. Gherig see a respiratory therapist will allow me (lead physiotherapist) and the rest of the multidisciplinary care team to focus on our specialities to ensure he receives the best possible care for every aspect of his condition.
Thank you for your time and consideration.
Sincerely,
PT858
https://www.youtube.com/watch?v=qNFk9cJqcF8
Discussion[edit | edit source]
Self-study questions[edit | edit source]
References[edit | edit source]
- ↑ 1.0 1.1 1.2 1.3 1.4 O’Sullivan B. S, Schmitz J. T, & Fulk D. G, editors. Physical Rehabilitation. 7th-ed. Philadelphia: F. A. Davis Company, 2019.
- ↑ 2.0 2.1 2.2 Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, et al. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009;73(15):1218–26.
- ↑ Bourke SC, Bullock RE, Williams TL, Shaw PJ, Gibson GJ. Noninvasive ventilation in ALS: Indications and effect on quality of life. Neurology. 2003;61(2):171–7.
- ↑ Lechtzin N, Scott Y, Busse AM, Clawson LL, Kimball R, Wiener CM. Early use of non-invasive ventilation prolongs survival in subjects with ALS. Amyotrophic lateral sclerosis. 2007;8(3):185–8.
- ↑ Kareus SA, Kagebein S, Rudnicki SA. The importance of a respiratory therapist in the ALS clinic. Amyotrophic lateral sclerosis. 2008;9(3):173–6.
- ↑ Andersen PM, Abrahams S, Borasio GD, de Carvalho M, Chio A, Van Damme P, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS) - revised report of an EFNS task force. European journal of neurology. Received 16 November 2010 Accepted 12 July 2011. 2012;19(3):360–75.
- ↑ 7.0 7.1 7.2 Yamakawa I, Yamada A, Sonoda Y, Wakita K, Nishioka T, Harada Y, et al. Occupational therapy using a robotic-assisted glove ameliorates finger dexterity and modulates functional connectivity in amyotrophic lateral sclerosis. Journal of clinical neuroscience. 2023;107:144–9.
- ↑ Arbesman M, Sheard K. Systematic review of the effectiveness of occupational therapy-related interventions for people with amyotrophic lateral sclerosis. The American journal of occupational therapy. 2014;68(1):20–6.
- ↑ 9.0 9.1 9.2 Guillemin L, Hofstede J, Anderson T, Walkeden H, Schellenberg K, Kang E, et al. Perceptions and experiences of nutrition interventions in individuals with amyotrophic lateral sclerosis (ALS) and their caregivers. Canadian journal of dietetic practice and research. 2022;83(4):193–7.
