Occupational Therapy for Neurodegenerative Conditions

• Neurodegenerative conditions are chronic and potentially progressive, requiring individuals to cope with disability and the threat of functional loss.
• These conditions involve mechanisms that attack the peripheral and central nervous systems.
• Occupational therapy consistently focuses on participation in valued occupations to enhance the quality of life.
• The nature, type, intensity, and context of occupational participation are individual to each patient.
• It's important for OTs to maintain objectivity when treating individuals whose disease course leads to increasing disability and/or death.
• Hospital admissions typically follow major relapses/exacerbations or deterioration of the disease.
• Inpatient rehabilitation lasts one to several weeks, with daily OT to address changing symptoms.
• Home OT/Outpatient services involve 45-60 minute weekly/biweekly sessions to maximize independence at home and minimize the effect of symptoms.
• Long-term care focuses on adjusting to changing symptoms, ensuring maintenance of function, controlling equipment, and developing palliative care plans.

OT Evaluation

• Gather information about roles/occupations, history of the disease, previous and current management strategies, and current concerns/challenges.
• Be sensitive to individuals’ changing function and fear of the unknown.
• Address areas clients may not naturally mention, such as fatigue, depression, sexual function, and cognitive concerns.

Sensorimotor Functions

• Assess postural control and balance, ROM/muscle strength/tone, gross and fine motor coordination, and skin integrity.
• Paralysis/weakness/spasticity/rigidity may be present.

Cognitive/Perceptual Functions

• Evaluate foundation visual and cognitive skills, focusing on primary and then executive cognitive skills.

Psychosocial Functions

• Consider coping mechanisms, support systems, and adaptation/modification to occupational roles.

General Considerations

• Focus on safe, functional, and comfortable maintenance/participation/independence in chosen tasks/roles with adaptations, planning, technologies, and resources.
• Reduce the effects of the disease, such as fatigue and pain, to improve quality of life.
• Reassess regularly and reorder priorities as things change.
• Intervention approaches are typically compensatory, not restorative, except to ameliorate the effects of deconditioning.
• Family involvement and education are essential for the client, family, and the OT.
• Adopt an interdisciplinary team and collaborative efforts, using palliative, preventative, and compensatory approaches.

ALS: Amyotrophic Lateral Sclerosis

• Late-onset, neurodegenerative disease affecting both upper motor neurons (UMN) and lower motor neurons (LMN).
• Incidence rate is 2.7 per 100,000 people, with onset between ages 40-70 years, more common in men.
• Etiology is unknown; genetic and environmental factors may play a role.
• Median survival rate after diagnosis is 20 to 48 months.
• Death usually occurs 2-5 years after onset; 10-20% survive > 10 years.
• Typically presents as limb onset (UMN and LMN signs) or bulbar onset (speech and swallowing difficulties).
• Motor tract disorder (UMN): weakness, spasticity, hyperreflexia.
• Motor neuron disorder (LMN): weakness or atrophy in extremities, cervical extensor weakness, fasciculations, muscle cramps, loss of reflexes.
• Bulbar involvement affects cranial nerves, leading to muscle atrophy, initially focal and asymmetrical, gradually widespread.
• Early symptoms: wasting of thenar/hypothenar eminences, finger extension weakness, shoulders, falling, and ambulation/bed mobility problems.
• Primary symptoms: motor dysfunction (weakness, spasticity, dysphagia).
• Up to 50% develop cognitive or behavioral difficulties; 13% experience frontotemporal dementia.
• Vision, bowel/bladder control, and sensation are commonly spared.
• Relatively fast progression leads to QOL issues, depression, loss of control, and hopelessness.
• Often leads to early withdrawal from work and economic instability.
• ALS has 6 stages; stages 1-3 are ambulatory, and stages 4-6 are wheelchair dependent.

ALS Medical management

• Diagnosis involves neuro exams, EMG, nerve conduction velocity tests, MRI, blood/urinalysis, spinal tap, and muscle biopsies.
• Riluzole (pill) and Edaravone (IV infusion) are two FDA-approved drugs that can slow the course of the disease and reduce ADL difficulties.
• Medications manage symptoms like spasticity, anxiety, depression, and insomnia.
• Assisted ventilation, tracheostomies, and gastrostomies may be needed for eating/breathing.

ALS: OT Assessment

• Use the ALS Functional Rating Scale-Revised, ADL/IADL, and Mobility assessments to determine functional abilities.
• Assess UE abilities with FMC tests (Purdue, 9-hole, timed tests) and ROM/MMT to track changes.
• Consider fatigue and QOL using the Multidimensional Fatigue Inventory.
• Evaluate mobility, seating, positioning, communication, swallowing, and social participation needs.

ALS: OT Intervention

• Early intervention focuses on individual barriers to function.
• Later intervention focuses on the physical environment, assistive technologies, and supports.
• Optimize strength and ROM with home exercise programs.
• Maintain function in ADL and IADL using assistive or adaptive devices.
• Decrease fatigue with splints and orthotics.
• Manage pain and energy using joint protection, energy conservation, pain management, and work simplification techniques.
• Optimize safety, assess positioning, perform safe transfers, and maintain skin integrity.
• Enable communication through AAC and assess and manage dysphagia.
• Identify and obtain equipment such as hospital beds and lifts and modify the environment to enhance safety, participation, and comfort.
• Address emotional needs, caregiver burden, and QOL.
• Encourage daily stretching, AROM, strengthening, endurance, and home breathing programs to maintain functional level and minimize secondary complications.

ALS Movement Programs

• Neck collars, U-cuffs, foot-drop splints, canes/walkers, wheelchairs, raised toilet seats, shower chairs, grab bars, transfer boards, and slip-on shoes are examples of equipment and assistive tech.
• Smart home technologies include lighting, call-alert systems, motion sensors, remote/keyless door entry/unlocking systems, and voice activation.
• Aquatic therapy, buttonhooks, dressing sticks, LH reaching tools, and E-stim for hand function may not be helpful or have insufficient evidence.
• Avoid slingback/sling seats.
• Limb onset ALS presents with UMN and LMN signs, while bulbar onset ALS presents with speech and swallowing difficulties.

PD: Parkinson’s Disease

• Progressive and variable condition most common in later adult years, with onset typically between 65-70 years.
• Incidence is approximately 1% in people > 60 years, slightly more common in men.
• Etiology involves hereditary and environmental factors.
• Pathogenesis involves the loss of dopaminergic neurons of the substantia nigra, affecting the striatum, thalamus, and motor cortex.
• The course of the disease is determined by the distribution of symptoms, functional implications, presence of tremor, postural stability, mobility, and effectiveness of medication.
• Cardinal signs include tremor (resting), rigidity (cogwheel), akinesia/bradykinesia (slowed movement), and postural instability.
• Other symptoms include cognitive impairment, dementia (15-20%), depression, apathy, sensory symptoms, weight loss, and sleep disturbance.
• Oral motor changes, swallowing issues, speaking difficulty, festinating gait (freezing/start stop), and urinary and sexual dysfunction are also observed.

Parkinsons disease stages

• Hoehn & Yahr scale has 5 stages, while Bradley’s scale has 3 stages.
• Stage 1: unilateral symptoms, little/no functional problems, resting tremor.
• Stage 2: midline or bilateral symptoms, balance OK, mild problems with trunk mobility & postural reflexes.
• Stage 3: postural instability, mild-mod functional problems.
• Stage 4: increasing postural instability but able to walk; problems with ADL; decreased dexterity.
• Stage 5: confined to wheelchair or bed.

Medical Management

• Levodopa and carbidopa (Sinemet, Atamet) are used to compensate for dopamine loss.
• OT interventions should account for fluctuations in medication effectiveness throughout the day to maximize therapeutic benefit.
• Surgical procedures include pallidotomy, thalamotomy, and deep brain stimulation (DBS) for advanced stages.
• Social consequences include impacted handwriting, softened voice, reduced interest in leisure/social activities, and limited facial expression/hand gesturing.
• Individuals and families may experience guilt, despair, and anger as caregiving needs increase.
• Costs include medications, wheeled mobility, accessibility modifications, self-care and safety equipment, and in-home and caregiver supports.
• Early retirement is common, with people stopping work 5-6 years earlier than the general population.

Medical management

• Identify occupational performance problems related to mobility, fine motor activities, fatigue, work, safety, swallowing/mealtimes, sexual activity, sleep, affect/mood, cognition, and movement like bradykinesia and postural instability.
• The Parkinson’s Disease Questionnaire-39 (PDQ-39) is a 39-item screening tool with 8 domains: mobility, ADL, emotional well-being, stigma, social support, cognition, communication, and bodily discomfort.
• LSVT BIG and LOUD involve high-amplitude movements and voice amplitude training, respectively.
• https://www.youtube.com/watch?v=pHUagjNMRAE
• https://www.youtube.com/watch?v=6GRmxRRjnmg

Interventions

• Balance retraining and maintenance programs, such as Tai Chi & Qi Gong, can be successful.
• https://www.youtube.com/watch?v=gNIdxYjGVV8
• Compensatory approaches, like weighted items and cuffs, can be implemented using equipment.
• Activity analysis, modifications, energy conservation, work simplification, and pacing can be used
• Weighted eating utensils may be useful
• Prevent deconditioning and lack of activity
• Educate patients on the importance of timing activities to synchronize with optimal medication effectiveness.
• Enhance/modify leisure activities to encourage participation, and decrease isolation
• Smart devices, electronic tablets, and electronic aids to daily living (EADLs) can serve as communication aids.
• Voice Interventions: LSVT LOUD focuses on volume, pitch, & annunciation
• Enlarge felt-tip pens, take rests, and complete HEP for facial movement to improve communication skills.
• Optimize movement initiation by using self-cueing techniques and by maintaining an uncluttered path.

Interventions to Optimize Safety

• Ensure home assessments and modifications, good lighting, and rehab self-cue-speech programs are in place.
• Modify Eating routine to enhance safety and participation

Motoric "Freezing"

• To manage motoric “freezing”, avoid crowds, narrow spaces, room corners, reduce distractions, avoid carrying items while walking, reduce clutter, concentrate when changing directions, and use a rhythmical beat or counting to maintain momentum.

MS: Multiple Sclerosis

• An autoimmune disorder triggered by an environmental factor or infectious agent.
• Inflammation, demyelination, and axonal transection throughout the CNS create plaques throughout brain tissue.
• Affects ~900,000 people in the US and 2.3 million worldwide.
• Onset is between 15-50 years, with peak onset at 20-30 years; women are 2-3 times more likely to be affected than men.
• Diagnosed by course, symptoms, spinal tap, and MRI.
• The four types of MS are:
  • Relapsing-remitting (RR): most common at diagnosis, with exacerbations and recovery periods.
  • Secondary progressive: occurs after RR for 10-15 years, with continued deterioration/disability.
  • Primary progressive: continuously declining from onset without clear relapses.
  • Progressive-relapsing: like RR but with continued decline between episodes.
• Symptoms: fatigue, weakness, dexterity challenges, pain, spasticity, tremor, sensory changes, cognitive changes, depression, dysphagia, dizziness, vertigo, ataxic gait, balance disturbance, visual changes, headaches, bowel & bladder problems, and sexual dysfunction. Will impact: ADL/IADL, Sleep Employment, leisure, social participation

Medical Management

• Disease-modifying therapies include interferon beta-1A (Avonex, Rebif), interferon beta-1B (Betaseron, Extavia), and glatiramer acetate (Copaxone).
• These drugs reduce the number of lesions and the frequency and severity of relapses but don’t reverse or impact long-term disability.
• Emphasis should be placed on self-management strategies like energy conservation techniques (ECT), pacing, and lifestyle changes.
• Education should be provided about the disease process and its effect on motor/cognitive processes.
• Early interventions are most effective

Medical management

• Listen for hints of cognitive difficulties.
• Inquire about dizziness, thinking problems, motivation to engage, dexterity, walking/standing/falls/balance, employment, physical & social home environment, leisure interest, bladder challenges, ADL/IADL, energy, sleeping patterns, cramping, pain, FMC, and vision changes/difficulties.
• Treatment that focuses on isolated symptoms is unlikely to be effective.

Intervention Considerations

• Emphasis should be placed on self-management strategies like energy conservation techniques (ECTs), pacing, and lifestyle changes.
• Education should be provided about the disease process and its effect on motor/cognitive processes.
• Environmental modifications should be considered to promote safety, while stretching/strength therapy enhances occupations.
• Technology use should be considered to enhance independence, and clients should be trained to balance independence with delegating and asking for help.
• Self-injectable medicines may cause harsh side effects.

Other Considerations

• Fatigue is the most common & pervasive symptom with MS (60-80%) and the primary reason for OT referrals.
• Analyzed and modified activities using assistive equipment can increase awareness of effects of body temperature on function & fatigue.
• A diary to set rhythms of activity, prioritization, and balance should be incorporated. Banking and Budgeting one's energy will maximize energy conservation efforts https://butyoudontlooksick.com/articles/written-by-christine-miserandino/the-spoon-theory/

Weakness

• Light-moderate, regular exercise can decrease fatigue/spasticity, as well as increase endurance and attention.
• Exercise has is most effective the when performed and/or tailored to functional activities

Compensation

• Utilize AE/DME to reduce strength/effort needed to manipulate items/perform
• Compensation should be achieved through compensatory and adaptive tech to reduce fatigue through effective instruction in ECT, pacing, time management, and prioritizing and delegating activities

Spasticity

• The management of tone, and weightbearing activity is important to address effectively to manage tone
• Cognitive Deficits can influence patient affect and general mental health so an important component of compensation is focus on education

Employment modifications

• Many can & should continue to work if possible rather than pursue disability benefits
• Financial independence, access to health care, maintain social network, support productive identity, health self-esteem Why do we have to be careful with heat?
• Heat exposure could trigger MS symptoms. It is generally an avoidance

Intervention

• Posture/ergonomics can be a valuable and effective intervention program with heat modalities

Evidence-Based OT Interventions for MS

• Fatigue self-management programs (online vs. F2F): positive impact on fatigue, QOL, self-efficacy
• Self-management program for spasticity reduction
• Physical Activity Programs: to improve muscle power and mobility
• Aerobic Activity programs: walking distance, endurance, QOL
• Resistance Training: to improve speed/endurance
• Nintendo Wii Fit for physical activity, balance and strength
• Emotional-regulation interventions: to improve mood, reduce depression/stress, improve self-efficacy
• Memory training: to improve memory
• Multidisciplinary rehabilitation: to improve activity levels, participation, & HR-QOL
• Home-based, Inpt Rehab, and Outpt Rehab all known to be helpful