Amyotrophic Lateral Sclerosis (ALS): A Case Study: Difference between revisions

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== Client Characteristics ==
== Client Characteristics ==
[[File:Soccer-ball.jpg|alt=A soccer player kicking a soccer ball in the field|thumb]]
A 24-year-old R-hand dominant Caucasian male named Jared Miller. Jared lives with his parents—Jane and James Miller—and his sister Jaya in Perth, Ontario. Jared was previously an athlete on his high school soccer team. After completing his undergraduate degree in Kinesiology at [https://www.queensu.ca/ Queen's University], Jared hoped to obtain a doctorate degree in Dentistry. However, Jared was not able to pursue this career aspiration after being diagnosed with ALS 6-months ago. He first noticed changes in muscle strength, after falling several times on the soccer field, and expressing a general feeling of weakness. Since the onset of these initial symptoms, Jared has been experiencing ongoing muscle weakness, cramping and associated pain, and thus was referred to physiotherapy by a neurologist. Despite having been diagnosed with ALS, Jared has no comorbid conditions. (4)  
A 24-year-old R-hand dominant Caucasian male named Jared Miller. Jared lives with his parents—Jane and James Miller—and his sister Jaya in Perth, Ontario. Jared was previously an athlete on his high school soccer team. After completing his undergraduate degree in Kinesiology at [https://www.queensu.ca/ Queen's University], Jared hoped to obtain a doctorate degree in Dentistry. However, Jared was not able to pursue this career aspiration after being diagnosed with ALS 6-months ago. He first noticed changes in muscle strength, after falling several times on the soccer field, and expressing a general feeling of weakness. Since the onset of these initial symptoms, Jared has been experiencing ongoing muscle weakness, cramping and associated pain, and thus was referred to physiotherapy by a neurologist. Despite having been diagnosed with ALS, Jared has no comorbid conditions. (4)  


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=== History of Present Illness ===
=== History of Present Illness ===
One year ago, the patient noticed general weakness and cramping in his legs while playing soccer, which led to numerous falls on the field over several games. He spoke with his family doctor after 2 weeks of frequent falling and cramping. His family doctor referred him to a neurologist in Ottawa. Jared was diagnosed with ALS 6 months ago by his neurologist, on November 10, 2021. Treatment to date has been Riluzole medication prescribed by his doctor.  
One year ago, the patient noticed general weakness and cramping in his legs while playing soccer, which led to numerous falls on the field over several games. He spoke with his family doctor after 2 weeks of frequent falling and cramping. His family doctor referred him to a neurologist in Ottawa. Jared was diagnosed with ALS 6 months ago by his neurologist, on November 10, 2021. Treatment to date has been Riluzole medication prescribed by his doctor.  
 
[[File:Screen Shot 2021-05-13 at 8.24.27 PM.png|alt=A pain diagram showing human body from the front and back. Achy pain represented by arrows on the front of both thighs and outside front of both calves. Stabbing/sharp pain, represented by oblique lines, located on back of calves.|left|thumb]]
'''Pain:'''                                                                                                                                                                                                                                                Site: calves, quads                                                                                                                                                                                                                  Radiation: not radiating                                                                                                                                                                                                              Nature: deep cramping                                                                                                                                                                                                    Periodicity: intermittent                                                                                                                                                                                                  Duration:  noticed it last 30 days                                                                                                                                                                                  Intensity: 4/10                                                                                                                                                                                                                        Quality: sharp , cramping                                                                                                                                                                                          Aggravating factors: walking upstairs, running, walking distances >100 m                                                                                          Alleviating factors: massage, rest
'''Pain:'''                                                                                                                                                                                                                                                Site: calves, quads                                                                                                                                                                                                                  Radiation: not radiating                                                                                                                                                                                                              Nature: deep cramping                                                                                                                                                                                                    Periodicity: intermittent                                                                                                                                                                                                  Duration:  noticed it last 30 days                                                                                                                                                                                  Intensity: 4/10                                                                                                                                                                                                                        Quality: sharp , cramping                                                                                                                                                                                          Aggravating factors: walking upstairs, running, walking distances >100 m                                                                                          Alleviating factors: massage, rest



Revision as of 03:14, 14 May 2021

Abstract[edit | edit source]

Introduction[edit | edit source]

Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease that involves the neurons responsible for controlling voluntary muscle movement. ALS most commonly appears between the ages of 55 to 75, but has the potential to impact individuals within any age group. In ALS, both the upper motor neurons and lower motor neurons degenerate, and therefore, stop sending signals to the muscles. This results in weakening of the muscles, twitching—known as fasciculations—and muscle wasting. The early symptoms usually involve muscle weakness, and eventually progresses to the inability to speak, eat, move, or breathe. Respiratory failure is the major cause of death in individuals with ALS, and usually occurs within 3 to 5 years from the onset of symptoms. (11). Approximately 70% of patients with ALS present with limb-onset ALS—weakness in the arms and legs—and the remaining patients present with bulbar-onset ALS, which manifests with dysarthria—motor speech disorder—and dysphagia—difficulty swallowing (12).  

The clinical presentation of ALS is not homogenous. There is considerable variability in the phenotypical expression of ALS, which is demonstrated throughout the literature. Jamorzik et. Al (2015) discusses a 68-year-old man who first presented with dysarthria in July 2012, and several months later began experiencing generalized fasciculations. A year later, the patient’s speech became more slurred, but he did not present with muscular atrophy or weakness. Clinical evaluations supported by electrophysiological and biochemical testing led to the diagnosis of bulbar-onset ALS (12). Conversely, Venizelos, Park & Fisher (2011) presented a case of a 57-year-old Caucasian male who began experiencing unexplained bilateral lower extremity weakness and fasciculations. Within five months, his lower extremity weakness progressed until he was unable to stand independently, and he also presented with muscular atrophy in the upper extremities, specifically in the intrinsic hand muscles. Despite being diagnosed with multiple other diseases, including chronic acquired demyelinated polyneuropathy (CIPD), the patient was later diagnosed with ALS and died one year after symptom onset (17). In addition, Majmudar, Wu, and Paganoni (2014) discussed a case involving a 45-year-old female patient who presented with an early-stage of ALS. The patient first began experiencing right leg and arm weakness, fatigue, and suffered from several falls while playing with her kids. The patient expressed having difficulty with activities involving fine motor control, such as cutting food and doing up buttons on clothing (22).  

Although there is no cure for ALS, riluzole is a medication that has been shown to be moderately effective at prolonging life by two to three months (15). Moreover, physiotherapy in conjunction with other forms of healthcare have been shown to be beneficial for symptom management, optimizing functional independence, and increasing quality of life in those with ALS; management for ALS should be tailored to the individual to meet their individual needs and goals (21). Majmudar et al. (2014) suggested that exercise, energy conservation techniques, stretching, and range of motion (ROM), as well as assistive devices for gait, may be appropriate for early-stage ALS. However, the individual should be monitored for signs of overexertion, excessive fatigue, and any increase in pain (22). Tsitkanou et al. (2019) demonstrated that both endurance and resistance training have an advantageous impact on quality of life, however, do not extend life expectancy (28).  

The purpose of this report is to create a realistic picture of a young individual diagnosed with early-stage ALS. A fictional case study will be used to illustrate the progressive nature of the disease, as well as outline the process of a physiotherapy assessment and likely findings for patients with ALS. Physiotherapy interventions for ALS, supported by the work of other healthcare professionals, focuses on maximizing patients’ mobility, respiratory function, independence, and quality of life. (9). Therefore, this report will also outline various physiotherapy interventions that have been successful for improving quality of life in patients with ALS. This case study was developed by integrating evidence-based research and clinical findings, for the purpose of educating other healthcare professionals on ALS and informing clinical decision making.

When taking on this project, we were aware that ALS is a very challenging condition, and much research is needed in order to fully understand the cause of the disease and develop a cure. However, an additional challenge we faced was developing a physiotherapy treatment plan that targeted the multitude of impairments experienced by those with ALS. This progressive disease can lead to impairments in balance, gait, dexterity, strength, and so on. Therefore, the requirement for a treatment plan to tackle the immense number of symptoms, as well as work to achieve patient specific goals, adds to the complexity of the disorder.  

Client Characteristics[edit | edit source]

A soccer player kicking a soccer ball in the field

A 24-year-old R-hand dominant Caucasian male named Jared Miller. Jared lives with his parents—Jane and James Miller—and his sister Jaya in Perth, Ontario. Jared was previously an athlete on his high school soccer team. After completing his undergraduate degree in Kinesiology at Queen's University, Jared hoped to obtain a doctorate degree in Dentistry. However, Jared was not able to pursue this career aspiration after being diagnosed with ALS 6-months ago. He first noticed changes in muscle strength, after falling several times on the soccer field, and expressing a general feeling of weakness. Since the onset of these initial symptoms, Jared has been experiencing ongoing muscle weakness, cramping and associated pain, and thus was referred to physiotherapy by a neurologist. Despite having been diagnosed with ALS, Jared has no comorbid conditions. (4)

Examination Findings[edit | edit source]

Subjective[edit | edit source]

Chief Concern[edit | edit source]

Feeling general weakness, cramping, and 4/10 pain in arms and legs recently, with multiple recent falls which is limiting participation in work, hiking and navigating his environment.  

Patient Profile[edit | edit source]

24-year-old male with ALS. DOB: 1996/02/05

During the initial assessment the patient was friendly yet appeared to be saddened and of low spirit. Jared has done an abundance of research on ALS and was very focused on the poor prognosis associated with the disease. The patient reports feeling defeated due to a lack of available treatments for ALS and feeling as if he was a burden to his mother. Additionally, the patient communicated feelings as if his dreams were taken away from him. He explained that he stopped playing soccer as a result of increased cramping and weakness in his legs, as well as having difficulty breathing while running on the field. Jared stated that he misses both the physical and social aspect of playing soccer. His parents have noticed changes in Jared’s voice, but reported that Jared’s friends and other family members haven’t noticed much change. Jared explained that his body image has been impacted since his diagnosis, as he has seen noticeable changes in his muscle mass. With that said, Jared expressed being motivated to start physiotherapy. Jared hopes to improve his lower extremity strength and balance, in order to go on leisurely hikes with his parents.

History of Present Illness[edit | edit source]

One year ago, the patient noticed general weakness and cramping in his legs while playing soccer, which led to numerous falls on the field over several games. He spoke with his family doctor after 2 weeks of frequent falling and cramping. His family doctor referred him to a neurologist in Ottawa. Jared was diagnosed with ALS 6 months ago by his neurologist, on November 10, 2021. Treatment to date has been Riluzole medication prescribed by his doctor.

A pain diagram showing human body from the front and back. Achy pain represented by arrows on the front of both thighs and outside front of both calves. Stabbing/sharp pain, represented by oblique lines, located on back of calves.

Pain: Site: calves, quads   Radiation: not radiating   Nature: deep cramping Periodicity: intermittent   Duration:  noticed it last 30 days   Intensity: 4/10 Quality: sharp , cramping   Aggravating factors: walking upstairs, running, walking distances >100 m   Alleviating factors: massage, rest

Past medical history[edit | edit source]

No significant past medical history. Patient had tonsillectomy at age 10.  

Medications[edit | edit source]

Riluzole, 100mg orally/day (8), Acetaminophen as needed.

Health Habits[edit | edit source]

Patient does not smoke cigarettes, drink alcohol, or do recreational drugs.

Family history[edit | edit source]

No known family history of ALS. Patient’s father has asthma. Mother has Type II diabetes.

Social history[edit | edit source]

Lives in a 3-storey home, with mom, dad, and younger sister. Enjoys playing and watching sports, and hiking with family. Patient meets up with friends every weekend to watch the soccer game. The patient used to work at Independent Grocer in the grocery department, which included tasks of stocking shelves and unloading skids. He left this job 3 months ago on medical leave, as it was challenging to lift heavy objects and have the control to place small objects onto the shelves.

Current functional status[edit | edit source]

The patient reports difficulty performing common activities of daily living, including washing dishes, cutting food, shaving, and brushing his teeth. Jared explains that he has challenges with walking long distances and running, due to muscle cramping and difficulty with balance. However, he does not use any mobility aids to assist him. Additionally, he describes that his handwriting is impaired. Jared explains that he experienced shortness of breath during exercise, however, since his diagnosis, he has not recognized any additional breathing challenges at rest. The patient reports experiencing no changes in bowel or bladder functioning, difficulty with swallowing, and changes in cognitive function.  

Subjective outcome measures administered[edit | edit source]

  • Numeric Pain Rating Scale: a self-report measure regarding the patient’s pain experience, where the patient selects a whole number between 0-10 that best reflects their intensity of his/her pain over the last 24 hours. Jared indicates a pain score of 4 (10).  

Objective[edit | edit source]

General Observations[edit | edit source]

  • Jared has slightly stooped posture including a forward head posture while sitting and standing.
  • There is mild muscle atrophy in Jared’s hands and lower extremities including the quadriceps and calf muscles.
  • There is mild bruising on Jared’s elbows and hips due to secondary falls.
  • There is visible fasciculations of John’s quadriceps bilaterally.

Speech and Language[edit | edit source]

Jared’s parents and family physician indicated a slightly slower and hyper-nasal voice.  

Tone and Sensation[edit | edit source]

  • Sensation tested using a dermatomal pattern for the hot/cold and sharp/dull testing. No significant findings were found.  
  • To rule out combined cortical sensation impairments, testing including graphesthesia and stereognosis was completed. No significant findings were found.
  • Tone was assessed using the Modified Ashworth Spasticity Scale (7). Findings include:
    • Increased tone in global muscles.
    • UE = 1+; LE = 1

Motor Control[edit | edit source]

  • Global strength was measured using manual muscle testing (MMT). Findings include: (14)
    • Bilateral ankle dorsiflexion = 3-/5
    • Wrist extension = 3+/5
    • Hip flexors = 3+/5
    • Hip extensors = 3+/5
    • Knee extension = 3+/5
  • Grip Strength was measured using held dynamometer (HHD). Findings include:
    • Lower than average grip strength (4)
    • L = 30kg; R =35kg  
  • Active Range of Motion (AROM) was measured using goniometry
    • All AROM are WNL except:
      • Wrist Extension = 50°
      • Finger Extension (DIP, PIP, MCP) = MCP 30-45°; PIP, 0°; DIP, 20° (those are normal values from PT 852)
      • Elbow extension = 10°
      • Dorsiflexion = 0°
  • Passive Range of Motion (PROM)
    • Able to straighten out trunk/posture when asked and held it for 30 seconds then went back to stoop posture
    • PROM all WNL
  • Manual dexterity was assessed using the Purdue Pegboard Test (PPBT). (16, 17)
    • Jared’s overall dexterity was slowed bilaterally with his non-dominant (L) hand scoring slightly slower than his R hand.
      • Right hand: 9
      • Left hand: 8
      • Both hands: 10
      • Right + Left + Both hands: 27
      • Assembly: 32

Neurological Testing[edit | edit source]

  • Upper Motor Neurons (UMN)
    • Positive Babinski’s sign bilaterally
    • Positive Clonus Test bilaterally  
  • Lower Motor Neuron (LMN)
    • Increased Achilles reflex - Grade 4+
    • Increased patellar reflex - Grade 3+
    • Increased biceps reflex - Grade 3+
    • Increased triceps reflex - Grade 3+

Balance[edit | edit source]

Balance was measured using the miniBESTest for a baseline (19)

  • Jared got a total score of 17/28.
  • Subscores
    • Anticipatory: 3/6
    • Reactive Postural Control: 4/6
    • Sensory Orientation: 4/6
    • Dynamic Gait: 6/10
  • Minimal Detectable Change: 5.5

Gait[edit | edit source]

Gait was assessed using the Ranchos Los Amigos Stages of Gait.

  • Overall impression, Jared ambulates safely with some minimal balance deficits and decreased dorsiflexion in both feet. His step length was decreased bilaterally, which resulted in an increased double stance time. He also walked with a forward stooped posture.
    • Initial contact - decreased heel strike bilaterally
    • Loading response - normal  
    • Mid stance - normal  
    • Terminal stance - normal
    • Pre-Swing - decreased hip extension bilaterally
    • Initial Swing - Increased knee flexion bilaterally
    • Mid-Swing - decreased dorsi flexion bilaterally
    • Terminal Swing - no foot clearance bilaterally  

ALS Functional rating scale - Revised (ALSFRS-R)[edit | edit source]

ALSFRS-R is a test used to measure the functional status of individuals with ALS. The ALSFRS-R is scored using 12 activities of daily living measured using a 5-point scale. The highest score for each item is 4 which represents normal function. The lowest score for each item is 0 which represents loss of the function being assessed. The total score for the scale is 48, with a higher score indicating a higher functioning individual. (13) (20)

Jared's ALSFRS-R score during his first physiotherapy appointment (01/05/2021). The left column depicts Jared's score in each category. The right graph depicts the data compared to Jared (seen as the X).

Ventilatory Muscle Strength  [edit | edit source]

  • MIP = -102 cmH20 and MEP = 143 cmH2O

BMI  [edit | edit source]

  • 6 foot 1 in, 158 pounds --> BMI of 21 (healthy/normal weight)

Clinical Impression[edit | edit source]

Physiotherapy Diagnosis[edit | edit source]

Jared is a 26-year-old male who presents to physiotherapy following a neurologist diagnosis of early-stage Amyotrophic Lateral Sclerosis (ALS) 6 months ago (1998) (2). Jared and his parents indicated that he is currently independent in all his ADLs; however, he is having difficulties with certain ADLs requiring greater hand and finger dexterity, such as brushing his teeth. Jared has recently stopped playing recreational soccer as he was experiencing an increase in falls, difficulties with his balance, increased cramping, and weakness in his legs. Jared’s decreased grip strength and impaired hand and finger dexterity led him to take a leave from work and are currently preventing him from applying to dentistry school. Jared has a good social support system at home as he is living with his sister and parents. He expressed being motivated to start physiotherapy to continue leisurely hiking with his family and returning to work at the grocery store. Therefore, Jared would benefit from physiotherapy treatment focusing on maintaining functional independence and muscle strength, as well as education on lifestyle modification and energy conservation strategies related to ALS.

Problem List (According to the ICF Framework)[edit | edit source]

Body Structure and Function

  • Decreased grip strength in kg bilaterally.  
  • Decreased active wrist extension which influences dexterity and decreased dorsiflexion which influences ambulation.
  • Mild muscle weakness globally, with upper and lower limb atrophy most notably in: Bilateral ankle dorsiflexion = 3-/5, Wrist extension = 3+/5, Hip flexors = 3+/5, Hip extensors 3+/5, Knee extension = 3+/5.  
  • Potentially experiencing body dysmorphia since his diagnosis, as he has seen noticeable changes in his muscle mass.  
  • Demonstrated LMN hyperreflexia for upper and lower limb (Achilles 4+, patellar, triceps, biceps 3+)
  • Demonstrated UMN hyperreflexia (Babinski and clonus)  
  • At risk of respiratory compromise with further neurodegeneration


Activity

  • Decreased speed while climbing stairs due to potential pain, cramping, and fatigue.
  • Decreased dynamic and anticipatory balance and increased risk of falls according to miniBEST balance testing.
  • ALSFRS-R score = 43/48.  
  • Decreased manual dexterity as shown in Purdue pegboard test
  • Drop foot gait patterning.  


Participation

  • Unable to work due to severe muscle cramping, 4/10 pain and general upper and lower extremity weakness.  
  • Unable to hike with family on the weekends due to weakness and pain.  
  • Unable to study to become a dentist due to decreased upper limb strength and hand and finger dexterity which hinders practical ability to study dentistry.

Intervention[edit | edit source]

Type Intensity Frequency Time
Strength Exercises
  1. Dorsi-flexion against gravity
  2. Quads over roll
  3. Wrist flexion and extension using Theraband flexbar resistance bar
  4. Finger Flexors and Extensors using Thera web
  5. Glute bridges
  6. Wall squats
  7. Wall push-ups
  8. Banded marching
8-12 reps making sure to reach muscle fatigue

(70-80% 1RM)


2-3 sets

3x per week, with at least 48hrs rest between same muscle groups 2-3 mins rest between sets
Endurance Exercises
  1. Eliptical
  2. Arm Bike
  3. Ambulation on treadmill
Moderate intensity (able to have a conversation while working) 3x per week (every other day) 30 minutes
Inspiratory Muscle Training
  1. Yellow Thera-band around diaphragm
8-12 reps making sure to reach muscle fatigue

(70-80% 1RM)


2-3 sets

3x per week 2-3 mins rest between sets
Dexterity Training
  1. Grasping marbles and placing them in a jar, from multiple distances and heights
  2. Using clothes pins and placing them on a line
To mental / physical fatigue

2-3 sets

Recommended daily, but 3x a week minimum 30 minutes recommended, or as tolerated
Stretching

Static stretching of:

  1. Finger Flexors/Extensors
  2. Wrist Flexors/Extensors
  3. Triceps surae
  4. Tibialis Anterior
  5. Hamstrings
  6. Glutes
  7. Quadriceps
  8. Shoulders
Stretching sensation to the point of mild discomfort Recommended daily, but 3x a week minimum 30 second hold 3x a day
Ischemic Compression/Trigger point release
  1. Apply pressure slowly and progressively over the trigger point as the tension in the trigger point and the taut band subsides
Pressure into discomfort/pain (5/10 max pain) Recommended daily, but 3x a week minimum 30-60 second hold at each point
Blance
  1. Single Leg Stance
  2. Tandem Stance
  3. Foam mat/ BOSU ball 2-legged stance
  4. External Perturbations (leaning on therapist, anterior, posterior, lateral)
  5. Internal perturbations (reaching, eyes closed)
As tolerated Recommended daily, but 3x a week minimum 30 seconds hold x3 sets
Thermotherapy
  1. Heat pack
Mild warm sensation As needed 15 minutes on, 45 minutes off (or until skin is back to normal temp)
Education
  1. Provide additional information about juvenile ALS, focusing on the importance of mobility and strengthening the available motor units as the disease progresses
  2. Provide education on overuse fatigue and what to expect as the disease progresses
  3. Provide pain education
  4. Provide education on how to use a peak flow meter for self-monitoring and management
  5. Provide gait aid education as needed
N/A N/A N/A

Outcome[edit | edit source]

Jared was very motivated to follow the intervention and plans set out from his physiotherapy sessions. He understood the importance of physical activity and strengthening in order to maintain/delay the progression of ALS. After the 1st week, Jared mentioned he enjoyed the strength training, and found the stretches, massages and ischemic compressions to complement well his cramps and fatigue. He especially liked the hand specific exercises, as he found himself being more confident with his fine motor control skills.  He mentioned that his pain has become more tolerable and has only reached a maximum of 3/10 when using the VAS compared to his previous score of 4/10 before physiotherapy sessions (in both calves and quads). Although Jared was doing fine with all his exercises, we did not believe that increasing the resistance/intensity of exercises would benefit him. Increasing the intensity of exercises might negatively affect his prognosis and therefore we were cautious with progressing Jared’s interventions (29).  

After 3 weeks of therapy, Jared’s global strength was re-assessed through MMT and grip strength, while also looking at his gait and balance. Overall, his strength was very similar to his initial assessment except for increase in DF strength to 3/5 bilaterally, wrist extensors and knee extension strength to 4-/5 bilaterally. His grip strength slightly improved to 33kg on his L and 37kg on his R hand, but it is not clinically significant (4). His gait slightly improved, as Jared displayed better foot clearance and increased DF during the swing phase, bilaterally. Finally, Jared’s balance was re-assessed by using the miniBESTest, which he scored a 18/28 (Anticipatory: 4/6, Reactive Postural Control: 4/6, Sensory Orientation: 4/6, Dynamic Gait: 6/10). Again, although it was not a clinically significant difference, there wasn’t any decrease in his balance skills. At this point of time, Jared felt slightly discouraged as there were no major overall improvements. However, we had to re-emphasize the importance of maintaining his current physical performance, and educating Jared on ALS and its prognosis, and the value of preventing the effects of ALS.  

Finally, Jared was re-assessed after 6 weeks of therapy. From the general observation, we did not find any major differences or change. As well, there were no major changes in Jared's overall AROM and PROM. We then measured various outcome measures and their results were as follows: ALSSQOL-R: 36/48, VAS: 4/10 most notably in his calves, ALSFRS-R: 44/48, MiniBEST: 17/28 (Anticipatory: 4/6, Reactive Postural Control: 4/6, Sensory Orientation: 3/6, Dynamic Gait: 6/10), Purdue Pegboard Test: overall = 31, assembly = 36 (right hand :11, left hand: 9, both hands: 11). In terms of gait, Jared displayed similar gait pattern with better overall posture (less forward stooped posture) compared to three weeks ago. However, he mentioned that the pain in his calves had forced him to take more breaks during walks. He mentioned that the pain comes on after 10 minutes of constant walking, but a 5-minute break is enough for him to continue walking before the cycle continues again. Because of this, we decided to slightly alter his endurance interventions. Since Jared was doing both the endurance exercises and participating in frequent walks with his family, we had proposed to Jared to continue his frequent walks with his family and eliminate extra endurance activities (ellipticals and walking on treadmill) on those days. That way, we hoped to reduce his general pain and control his lower extremity muscle fatigue. In terms of overall strength, there were little deviations compared to his previously assessed strength three weeks ago. His R grip strength was 37kg and L grip strength was 34kg. His MMT scores were similar, however there was an increase in strength bilaterally in his DF (3+/5) and an increase in strength bilaterally in his hip flexors and extensors (4-/5).

Although there were no significant changes in most of the results/measures compared to 6 weeks ago, having stable/consistent scores in a variety of outcome measures was seen as a positive. In other words, the interventions that were set out by PT along with the other interventions implemented by the rest of the healthcare team (neurologist, family doctor, OT, dietician, etc.), may have been effective in preventing the acute progressive nature of Jared’s ALS.

Discussion

Interdisciplinary Management of ALS[edit | edit source]

Patient-centered, multidisciplinary, comprehensive rehabilitation care is vital for individuals with amyotrophic lateral sclerosis (ALS) and has been shown to significantly improve their care and improve quality of life (Paganoni et al., 2015). Health care professionals who may be involved in the care of individuals with ALS include the patient’s family and friends, family physician, neurologist, physiotherapists, occupational therapists (OTs), registered dietitians, speech language therapists, social workers and palliative care.

All members involved in management of ALS.

Occupational Therapist[edit | edit source]

OTs play an integral role in the interdisciplinary care of individuals with ALS. There are many tasks that an OT can perform to help ALS patients maintain their maximum functional and independence level, influence their attitude and assist in changing a patient’s perspective to of more what they can do instead of what they can’t do. Although Jared is currently independent in his ADLs, he is having some difficulties with ADLs requiring hand motor dexterity. Moreover, an OT may need to fit and prescribe upper extremity bracing or adaptive devices. For example, adaptive large grip utensils may be recommended to allow individuals with ALS to maintain independence while eating or a resting hand splint may be recommended to prevent wrist and finger flexion contractures (Majmudar et al., 2014). Jared is currently able to ambulate independently; however, as his disease progresses, he will likely require the use of an Ankle-Foot Orthosis (AFO) or a gait aid which the OT can help fit and prescribe to him. As ALS progresses, Jared may need a cane, walker and eventually a wheelchair (Majmudar et al., 2014).

Additionally, an OT should educate individuals with ALS and their families on energy conservation and fatigue management. For example, OTs can provide education on the benefits of breaking up high-energy tasks by incorporating rest breaks into their daily activities and planning their day to prioritize the tasks that are most important to them. This type of education has shown to be effective in reducing fatigue, managing fatigue symptoms and improving different aspects such as health-related quality of life (De-Bernardi-Ojuel et al., 2021).

Finally, as ALS progresses, the OT may conduct a home assessment and provide recommendations to the family on certain modifications that they may want to make to enhance the individual’s mobility and safety. For example, a ramp may be recommended to enter the home or a stair lift if the individual with ALS lives in a multi-story home like Jared. In addition, the OT will likely give instructions to the family members and caregivers on how they can assist and support their family member with ALS with their exercises, proper positioning, how to lift and transfer the individual and how to use the assistive devices (MDA-ALS Center). Therefore, OTs play an essential role on the interdisciplinary care team of a patient with ALS, assisting in a multitude of ways as the disease progresses.

Registered Dietician  [edit | edit source]

The role of registered dieticians (RD’s) in the multidisciplinary treatment approach of ALS is a cornerstone to favourable prognosis. Increased body mass index is associated with more favourable outcomes in patients with ALS, and therefore nutritional interventions are core to the management of the neurodegenerative disease (Ning P, Yang B, Li S, Mu X, Shen Q, Hu F, Tang Y, Yang X, Xu Y)  In the context of ALS, the neurodegenerative condition has nutritional implications on body weight in the presence of dysphagia (decreased ability to swallow), in addition to the potential for increased caloric expenditure from muscular fasciculations and decreased caloric intake in the presence of mental illness such as depression (Korner et al., 2013).  RD’s are incorporated into the multidisciplinary management of ALS for their expertise and competence in conditions influencing nutritional status, including dysphasia secondary to ALS (CDO REF). In conditions such as ALS, RD’s provide swallowing assessments if dysphagia is a concern; the purpose of the assessment is to identify the risk of aspiration, developing pneumonia, and choking, and whether oral or non-oral routes of hydration and nutrition are indicated (CDO REF). The treatment interventions of a registered dietician for ALS vary depending on the physical, and nutritional status of the patient. In patients with a higher level of functioning and the absence of dysphagia, the role of a dietician may be centered around providing education on evidence-based nutritional requirements. Education provided by dieticians is individualized and addresses areas such as caloric and hydration requirements, macronutrient and micronutrient requirements, and supplementation to optimize quality of life. Dieticians will also facilitate monitoring of nutritional status to identify and address the potential for malnutrition in higher functioning people living with ALS (Rio & Cawadias, 2007). In the context of recently diagnosed and higher functioning ALS patients/clients without dysphagia such as Jared Miller in this fictitious case scenario, RD’s may provide education on nutritional guidance in the form of nutritional monitoring, meal planning, and therapeutic diets targeted for optimal nutrition. In people in the later stages of ALS diagnosis, dysphagia is commonly present and assessed by RD’s. Registered dieticians are competent to provide assessment and nutritional management for dysphagia across multiple healthcare settings (CDO REF). Following nutritional and swallowing assessments, if dysphagia and malnutrition are present in a person diagnosed with ALS, the role of a dietician within interdisciplinary patient care transitions away from primarily educational. In these cases, dieticians oversee alternative methods to oral feeding and provide nutrition through alternative feeding routes such as percutaneous endoscopic gastrostomy in which a feeding tube bypasses the esophagus and is inserted through the abdominal wall into the stomach for enteral nutrition (Rio & Cawadias, 2007). This alternative to oral feeding addresses the compromised oral route when dysphagia is present to improve or maintain nutritional status following advanced neurodegenerative disease. Maintaining nutritional status is critical to prevent weight loss and fatigue associated with ALS. RD’s are an essential component of multidisciplinary person-centered healthcare for people living with ALS in both the novel and progressed stages.

Self-study questions[edit | edit source]

References[edit | edit source]