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Copy of AMYOTROPHIC LATERAL SCLEROSIS

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Transcript of Copy of AMYOTROPHIC LATERAL SCLEROSIS

AMYOTROPHIC LATERAL SCLEROSIS (ALS)
REFERENCES
WHAT IS ALS?
The Model of Human Occupation
CASE STUDY
Sarah is a 46 year old married mother of 2 teenage daughters. She previously worked as an administration assistant in a busy law firm. Sarah is an active youthful women, who cooks for her family on a daily basis, enjoys gardening, knitting, and playing tennis at her local club twice a week. Sarah is also responsible for driving her daughters to and from their extra curricular activities.
Whilst at work, Sarah has recently been experiencing a loss of dexterity in her fingers while typing, noticing a decline in her efficiency and productivity, along with a slight slur in her speech when taking phone calls.
When at home Sarah has experienced difficulties when cooking. She complains of finding it hard to open jars and holding utensils as well as lifting heavy pots/pans from the stove.
Sarah's main OPI issue is upper limb function.
Amyotrophic lateral sclerosis
is a
motor neuron disease
that is a progressive condition caused by degeneration of the motor neurons and damage to the motor neuron system. This is the most common degenerative disease of the motor neuron system. Degeneration leads to wasting and weakness of the skeletal muscles, which decreases control over limbs movement.

The neuron areas affected occurs at the anterior horn cells of the spinal cord and corticospinal tracts, both upper and lower motor neurons. Diagnosis occurs through the linkages made between physical symptoms and signs, blood testing and cancellation of other conditions that present with similar symptoms.

Aeitiology

• Cause is unknown and is in most cases sporadic. Some unproven theories include trauma, exposure to toxins and heavy manual activity or exercise
• Amyotrophic lateral sclerosis has a chance of being inherited from an autosomal dominant inheritance (only 5-10% cases)

Treatment
There are no curative medications and treatments for amyotrophic lateral sclerosis. Rilutek is a drug that slows the process of degeneration throughout the body. There are however medications to treat symptoms present with ALS.

Prognosis
The prognosis of the amyotrophic lateral sclerosis varies between individuals. It is however a rapidly progressive disease with a prognosis of 2-5. The median survival length of time from onset of symptoms is 3.5 years.
Neuron damage eventually leads to degeneration of the muscles controlling the tongue, larynx, pharynx and muscles of the respiratory system. A build up of carbon dioxide in the lungs from ventilator failure is in most cases the cause of death from ALS as the body is unable to expel and instead retains CO2 in the body.

Support
Family and professional supports can provide an essential supportive environment for coping with ALS. Respite can be provided to families who choose to care for those affected with ALS. Professional help in the home setting to care for the client can provide benefits to both clients and families to help relieve stress, maintain patient comfort and eliminate the need for inconveniencing travel.


ASSESSMENTS
Occupational Therapy
Occupational Therapists work in a multidisciplinary team with other allied health professionals in order to focus on and extend expert advice to address all of the client’s significant issues. The role of an OT is to holistically assess the client and determine what their meaningful occupations are in order to determine what their occupational performance issues may be and maximise the client’s functional ability to facilitate independence.
ALLIED HEALTH TEAM
Other allied health professionals that may be involved with a person who has ALS includes:
-Physiotherapist:
Assess in detail and plan interventions that focus on the client’s physical abilities, including mobility status, transfers ability, balance, range of movement, endurance and muscle strength.
-Speech Therapist:
Assess and plan interventions that focus on the client’s swallowing and communication abilities. May assist client’s with saliva management and communication strategies as condition progresses.
-Dietician:
Determine an appropriate diet for the client to ensure adequate nutrition is maintained. Factors that may affect this include the client’s ability to swallow, chew and status of the digestive tract.
-Social Worker:
Assess the client’s current social situation. This may involve family and friend supports, finances, other health issues, determining whether the client requires a guardian or power of attorney and other social aspects that are the foundations of the client’s life.
-Nursing Staff:
Review and attend to any current medical needs the client may have, such as medications, continence and monitoring the client’s vital signs.
Together, allied health professionals liaise with each other to ensure that other professionals are aware of essential information regarding the client’s functional abilities and work as a team toward a common goal of increasing the client’s independence and quality of life.

OPI & Occupational Objectives
Occupational performance issues associated with ALS
A person with ALS may experience difficulties in completing or participating in their daily occupations due to:
-difficulties swallowing due to saliva management
-decreased endurance and fatigue
-decreased functional mobility
-decreased fine motor ability, affecting handwriting, doing up buttons, etc
-reduced upper limb function, affecting shower/dressing, toileting, cutlery use when bringing food to mouth
-disturbed sleep due to decreased respiratory function and decreased ability to change position

Occupational Therapy Approach
As ALS is a progressive condition with no known cure, the primary goal of an OT when working with an individual with ALS is to regain and maximise functional independence as much as possible, and maximise comfort by minimising pain and facilitating both intellectually and psychologically stimulating activities.
An OT’s intervention plan should be guided by appropriate assessments and models of best practice to ensure that the client’s needs and wishes are being met. In addition to assisting the client’s functional abilities to ensure their safety in their home environment and in the community, it is essential for an OT to address the client’s meaningful occupations and what activities or tasks are most significant to them in order to gain a holistic view of the client and direct intervention to the appropriate areas in order to improve the client’s overall satisfaction and quality of life.
OT goals and objectives include:
-retraining in personal, domestic and leisure activities
-liaising with other allied health professionals such as Physio and Speech Therapists to combat physical, swallowing and communication difficulties
-education for both client and client’s family addressing strategies and techniques

ASL
Degeneration of the body on part at a time
RESOURCES FOR CLIENTS
There are many organisations and local support groups for patients and families affected.
• ALS association
• Motor Neuron Disease Association in all states of Australia
• MND Australia www.mndaust.asn.au
• MND Research Institute of Australia www.mndresearch.asn.org
• Calvary Health Care Bethlehem www.bethlehem.org.au

OT APPROACH
Sarah at this stage is experiencing disturbances with speech and upper-limb function. This is affecting her ability to effectively complete her activities including:
- Typing which is impacting on her ability to complete her work by making her slower and making more mistakes, ultimately being a more timely task
- Cooking for her family. She is having difficulty cutting items, opening jars, holding finer utensils and lifting pots/pans off the stove
- Speech impacting her ability to speak clearly at both work and home
- Dexterity, fine motor and strength in her upper limbs which impacts on her driving (controlling the steering wheel), knitting (having the dexterity to manipulate the needles and wool), tennis (holding the racquet and having enough power to hit the ball), showering, dressing, etc.

Sarah has identified the 4 most important occupational performance issues:
- Self-care
- Cooking
- Housework
- Going for walks

A standardized test that an occupational therapist could complete with Sarah to determine her functional capacities is the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALS-FRS). It is a questionnaire based scale and looks at four domains:
- Bulbar function (speech, salivation, swallowing)
- Fine motor (writing, feeding)
- Gross motor (walking, transfers)
- Respiratory insufficiency

Other interventions and assessents for Sarah:
- Functional assessments (such as shower assessments)
- Education regarding condition
- Adaptive equipment



TASK ANALYSIS

1. Collecting items: required for post-shower including towel and clean underwear, and place in arms reach from the shower. If bath mat is used post-shower, position close to the entry to shower
2. Undress: take off dirty clothing and put in appropriate area
3. Set-up: Ensure products used in shower (e.g. soap, shampoo etc.) are already in the shower recess. Turn on water while outside shower and feel for a comfortable temperature
4. Washing: Using the shampoo collect/open/pour liquid onto hands. Put onto hair and use hands to thoroughly clean hair in a gentle scrubbing motion. Rinse and repeat with conditioner. Using soap (+/-) washcloth to wash upper body, lower body and genital area. Turn off taps and get out of shower
5. Drying off: Using the towel, thoroughly dry off all areas of the body and then don clothing.

As Sarah reported having difficulties with fine motor and some fatigue the OT recommended the following to allow her to be independent as possible:
- The use of a shower stool of days when she is feeling particularly energetic so that she can conserve energy for other activities
- A hand held shower hose so when she is using a shower stool she can have better control of where the water is hitting her body rather than adjusting her body to where the water is falling
- Using a mixer tap so that it’s easier to turn the tap on/off and to control the water temperature
- Using a soap on a rope and attaching it to a solid surface so if she drops the soap with her affected fine motor skills, it’s not as difficult to pick it back up and continue washing
- The use of shampoo and conditioner with pumps rather than lids to make it easier to use


SHOWER ASSESSMENT
INTERVENTION
In order to support a
client-centred
practice and shape
a useful structure
for intervention
planning for Sarah
MOHO
Sarah’s occupations that have been affected are activities that provide meaning to her life and are essential for independence.Therefore the MOHO is essential in the therapy process as it is highly concerned with the abilities of which Sarah can participate in everyday life occupations and to achieve a state of positive adaptation after the onset of her condition.
Volition
Volition refers to the process by which a person is motivated towards and chooses what activities they engage in. Volition includes thoughts and feelings before engaging in a task.
Sarah's personal causation, values and interests are considered and analysed to give an insight into what activities motivate her, interest her and provide meaning to her to ensure the therapy approach is client-centered.
Habituation
Habituation refers to the process of organising actions into patterns or routines. In relation to Sarah her activities of daily living were habits, behaviours that involve learned ways of doing things that unfold automatically. Roles also play a vital part in habituation, Sarah’s Role as a mother, wife and a worker provides her with a sense of obligations.
Performance capacity
Performance capacity refers to the underlying mental and physical capabilities and how they are used in performance. In relation to Sarah she now has upper limb difficulties that affect her physical capabilities in tasks such as cooking, typing, answering phone calls, driving, dressing, showering, and playing tennis, knitting, speech, swallowing and communication. Knowing all the physical aspects that are deterring Sarah away from occupations is important in selecting therapy approaches.
ALS & THE MOHO
Within the MOHO these elements lead onto whether or not those tasks can be performed including three terms,

1. Participation
2. Performance
3. Skill

These elements are analysed to see whether the client is able to actually do the activities that make up their life.

In relation to sarah due to her loss of strength and dexterity in her hands her occupational participation has been decreased dramatically leading to poor skills further leading to decreased occupational performance.

The MOHO finally concludes with the elements of occupational identity, competence and Adaptation and when looking at a client an occupational therapist must analyse what the patient needs to work on to achieve these three characteristics. Therefore thinking about what interventions such as dexterity re-training and equipment will enable Sarah to better perform in her activities of daily living to achieve occupational identity.

Due to the nature of
Amyotrophic Lateral Sclerosis
, assessments can be very broad and cover a wide magnitude of components. The type of assessments that an Occupational Therapist will perform depends on the presentation of the disease and the difficulties the client is faced with.
A brief understanding of the client’s pre – morbid history is an important starting point.
-Personal care
-Domestic occupations
-Community occupations and
-Leisure occupations are imperative to know, along with the home set up and safety.

Current
occupational performance
should also be reviewed.
-Upper and lower limb range of movement, strength, co ordination and dexterity
-Pain, sensation
-Balance when sitting and standing
-Endurance
-Position in bed, chair/wheelchair and
-Saliva management are important to be assessed.





ALSAQ-40
Another assessment is the ALSAQ-40 questionnaire, which can be used by OT’s to assess areas of occupational performance that are important to the particular patient.
There are 40 items/questions in the long form amyotrophic lateral sclerosis PRO, the ALSAQ-40, with 5 discrete scales:
• physical mobility (10 items)
• activities of daily living and independence (10 items)
• eating and drinking (3 items)
• communication (7 items)
• emotional reactions (10 items)

Patients are asked to think about the difficulties they may have experienced during the last two weeks (e.g. I have found it difficult to feed myself). Patients are asked to indicate the frequency of each event by selecting one of 5 options: 
never/rarely/sometimes/often/always or cannot do at all.
Studies have established the ALSAQ as a sensitive, valid, reliable, acceptable and feasible tool for the assessment of quality of life in ALS patients.
A global assessment that is often used by OT’s when assessing clients with ALS is the Tuft’s Quantitative Neuromuscular Exam (TQNE). This assessment is a standardized tool that is used to measure muscle strength and pulmonary function in patients with ALS. It is used to assess the rate of the disease progression.
It consists of the following items:
- pulmonary function, timed functional activities,
- Isometric strength test using an electric strain gauge.

The test takes about 1 hour to complete all 29 components and has excellent test re test reliability. It has the ability to record both mild and severe impairments accurately, measure both upper and lower motor neuron functions, is time efficient, is inexpensive and is sensitive to small changes.
TQNE


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Dal Bello-Haas, C., Kloos, A. D. & Mitsumoto, H (1998). Physical Therapy for a Patient through the six stages of Amyotrophic lateral sclerosis. Journal of American Physical Therapy Association, Dec 1998, vol. 75, no. 12

Dirette, D.K. (2012). Progressive Neurological Disorders. In B.J. Atchison, & D.K. Dirette (Eds.), Conditions in Occupational Therapy (pp. 209-223). Baltimore: Lippincott Williams & Willkins.

Jenkinson, C., Fitzpartrick, R., Brennan, C. and Swash, M. (2000) Evidence for the validity and relevance of the Amyotrophic Lateral Sclerosis assessment questionnaire: The ALSAQ-40. 2000 Vol.1 Informa Health Care

Jenkinson,C., Harris, R., & Fitzpatrick,R. (2007). The Amyotrophic Lateral Sclerosis Assessment Questionnaire(ALSAQ-40): Evidence for a method of inputing missing data, Amyotrophic Lateral Sclerosis, 8, 90-95.

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Kielhofner, G., Forsyth, K., Kramer, J.M., Melton, J., and Dobson, E (2009).The Model of Human Occupation. In E.B. Crepeau, E.S. Cohn & B.A.B. Schell (11th eds.), Willard & Spackman’s Occupational Therapy (pp 447 - 450). Philadelphia: Lipincott Williams & Wilkins.

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HSO 202
Alannah Santomartino
Georgie McNeice
Emily McMaster
Naomi Oeldrich
Hana Burian
AMYOTROPHIC LATERAL SCLEROSIS
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