TPTA Meeting 2018 Kim Carter PT NCS Janna Knickerbocker OT BCPR Objectives State the classical presentation of ALS IBM LGMD and SMA and how this impacts OT and PT treatment interventions ID: 780446
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Slide1
Preservation, Prevention, and Participation in Progressive Neuromuscular Disease
TPTA
Meeting 2018
Kim Carter, PT, NCS
Janna Knickerbocker, OT, BCPR
Slide2Objectives
State the classical presentation of ALS, IBM, LGMD,
and SMA
and
how this impacts OT and PT treatment interventions
State the role of PT and OT in the treatment of patients with neuromuscular disease
Discuss the latest research in exercise in patients with neuromuscular disease
Slide3Clinics: One stop
MDA clinic
Clients attend biannually or quarterly
MD-neurologist (and residents), PT, OT, SW, ATP, RT, CPO, MDA rep
ALS clinic
Clients may attend quarterly or monthly depending on progression of the disease
MD-neurologist (and residents), PT, OT, ST, RT, SW, ATP, CPO, ALS rep, dietician
Slide4Role of PT and OT
Preservation of Function
Prevention of Secondary Complications
Maximizing Participation
Slide5How do we do this?
Preserve ROM
Prevent functional decline
Screen for other disciplines
Increase safety with all mobility and ADL’s to prevent falls
Education
Assess for assistive devices
Splinting/bracing
Promote cardiovascular fitness
Support or strengthen existing musculature
Slide6Amyotrophic Lateral Sclerosis
Slide7ALS-Classical presentation
Disease that destroys the motor neurons (UMN and LMN)
Avg. age of onset 45-69 years Avg. age of onset 45-69 years Incidence is 2/100,000 and Military veterans are twice as likely to develop ALS
Life expectancy is 3-5 years from onset of symptoms
Usually persistent weakness occurs on one side of the body then spreads to the other side of the body.
Diagnosis is a rule out procedure that includes: evidence of UMN and LMN symptoms, EMG, MRI, muscle biopsy, blood test
Slide8Stages of ALS
Early stage
Muscle weakness may be limited to a single body region or mild symptoms may affect more than one region
Muscle cramping or twitching, atrophy
The patient may be independent with ADLs and walking but performance is suboptimal due to fatigue, poor balance, tripping while walking, slurred words, and weak grip
May see cognitive decline (frontotemporal degeneration)
Slide9Stages of ALS
Middle stages
Symptoms become more widespread
Unused muscles may cause contractures
Weakness in swallowing muscles can cause choking
Weakness in breathing muscles causes impaired endurance with functional activities
Slide10Stages of ALS
Late stages
Most voluntary muscles are paralyzed
Respiratory muscles are compromised, will probably use some form of mechanical support
Mobility is limited and caregivers are needed around the clock
Eating may not be possible and may receive nutrition through feeding tube
May need to use some type of communication device
Slide11Stages of ALS
In the end, most deaths occur due to respiratory failure
Hospice Care will help with pain control
Slide12Role of OT and PT
Preserve ROM
Upper Extremity
Shoulders for hygiene
Subluxation
Wrist and Fingers
Ankles
Bracing options
Hands
Resting hand
Long
opponens
Ankles
Neck
Slide13Role of OT and PT
Prevent functional decline
Energy conservation
Introduce adaptive equipment before they need it
Screen for other disciplines
Ask about swallowing and breathing
Slide14Adaptive Equipment
Equipment
Feeding
Desk support
MAS and feeders
Built up handles
Long
opponens
Positioning
Fastener aids and clothing changes
Bed mobility
Under mattress rails
Leg lifts
Hoyer lifts –eventually
Environmental control units
Slide15Personal Assistance Services
Hired caregiver to assist in BADL’s and IADL’s
Tennessee uses Choices program through Medicaid
https://www.tn.gov/tenncare/long-term-services-supports/choices.html
Done well services increase quality of life
Better if client has a choice in caregiver
Relationship between client and PAS critical
PAS must be dependable
Also helps the caregiver
OT’s role
Assertiveness training
Use of adaptive equipment and technology
Training on techniques
Use of activity adaptations and environmental modifications
Slide16Role of PT and OT
Increase safety with all mobility and ADL’s to decrease falls
Assessing balance and endurance
Caregiver and patient education
Bed mobility and Transfers
Fall Recovery
Ordering adaptive equipment before it is needed
Sliding board, increasing height of surfaces
Home modifications
Slide17Role of PT and OT
Education
On progression of disease
Support group
Prevention of pressure sores
Positioning
Assess for assistive devices
MD/ALS closet
Power wheelchair evaluation before the patient needs it
Slide18Wheelchair Evaluations
Client’s hesitation
Long process so start early (3-6 months)
Become familiar with the language
Focus on MRADL’s
Consider number of falls and respiratory support in justification
Slide19When is a PWC indicated?
When a manual wheelchair is not providing the support and function that
the patient
needs
Unable to independently propel a manual wheelchair
Fatigue or pain limits
the patient’s
ability to propel or sit in a manual wheelchair
Unable to transfer in and out of the chair
Unable to change positions in the chair
The patient is no
longer participating in activities that are important to the patient
Slide20Role of PT and OTExercise
Promote cardiovascular fitness
Strengthen existing musculature
Customized strengthening for currently unaffected musculature
Speech
Handwriting
Turning in Bed
Dyspnea
Salivation
Feeding
Walking
Orthopnea
Swallowing
Dressing
Stairs
Resp
Insuff
Slide21Exercise Research
Exercise
Resistance
Endurance
Stretching
Reps
and Duration
2 sets of 8 reps
Min
20 min
1 set of 4 reps
Frequency
2 days a week
3 days a
week
3 days a week
Falls
8
12
20
Compliance
68%
50%
80%
Slide22Role of PT and OT
Exercise
Rehabilitation in Reverse
Slide23Participation
Groenestijn
, A. C.,
Schröder
, C. D.,
Kruitwagen
‐Van Reenen, E. T., Berg, L. H. and
Visser‐Meily
, J. M. (2017), Participation restrictions in ambulatory amyotrophic lateral sclerosis patients: Physical and psychological factors. Muscle Nerve, 56: 912-918.
Conclusions
: Ambulatory ALS patients have participation
restrictions
, which may be influenced if early ALS care is directed toward lung capacity, functional mobility, fatigue, and feelings of helplessness.
Slide24Inclusion Body Myositis
Slide25Inclusion-Body MyositisClassical Presentation
Inflammatory myopathy
Inflammatory cells invade muscle tissue with resulting muscle degeneration
Usually occurs in men older than 50
Avg
age 33-66
Heart and lungs are not affected
On a biopsy, discarded cellular material (inclusion bodies) can be seen in the muscle.
Proteins fold/cause clumps (inclusion bodies) which are toxic to muscles. Toxicity causes inflammation.
Quads,
dorsiflexors
, wrist and finger muscles are affected by the disease
Slide26Role of PT and OT
Preserve ROM
Hands, wrist, dorsiflexion
Important to stretch
dorsiflexors
Prevent functional decline
Primary problem is sit to stand
Make a plan for curbs and steps at home and community
Screen for other disciplines
Occasionally they may have problems with swallowing
Be aware of comorbidities due to age
of onset
Slide27Role of PT and OT
Increase safety with all mobility and ADL’s to prevent falls
Thick cushions to help with sit to stand
Home modifications
Education
On disease progression
Assess for assistive devices
Walking sticks
Need for seat elevator on power wheelchair
Assess for adaptive equipment
Adaptive feeding and communication equipment
Slide28Other Adaptive Equipment
Make things taller
Make things easier to pick up and hold as tend to move in intrinsic plus position
Foam handles with plastic utensils
Reachers
Rolling trivets
Alternatives for writing
Bottle/Can openers
Slide29Role of PT and OTExercise
Promote cardiovascular fitness
Support or strengthen existing musculature
Research
All studies involving patients with IBM have very small sample sizes
Most studies have found that resistance training at low to moderate intensities does not increase serum creatinine kinase.
Slide30Role of PT and OTExercise
7 subjects with sporadic IBM and progressive decline for 5-9 years
12 week home exercise program
Aerobic exercise and strengthening exercise performed on alternate days
Aerobic exercise performed on stationary bike (3x/week)
Strengthening for U/LE occurred on the same day but on separate sessions. (3x/week)
Knee
ext
, flexion; Hip flex,
abd
Shldr
abd
, elbow flex,
ext
; Wrist Ext
Slide31IBM Functional Rating Scale
Slide32Quality of Life/Participation
Gibson C; Johnson NE; Eastwood E;
Heatwole
C.
Inclusion
Body Myositis: What Most Impacts Patients'
Lives.
Journal
of Clinical Neuromuscular Disease. 18(2):67-71, 2016 Dec.
CONCLUSIONS
: Identifying critical issues to patients with IBM is potentially useful for clinicians whose aim is to provide optimal care to patients with IBM.
Slide33Limb Girdle Muscular Dystrophy
Slide34Limb Girdle Muscular Dystrophy
Classical Presentation
Group of disorders that affect the muscles around the shoulder and hip girdles
Heart and lungs can be affected
Men and Women are affected equally and the disease can begin in child hood, adolescence, early or late adulthood
Progression varies from a few years to a very slow progression
Slide35Role of PT and OT
Preserve ROM
Be careful with shoulder abduction and flexion above 90 degrees due to lack of
scapulohumeral
rhythm
Positioning to promote optimal trunk alignment
Prevent functional decline
Home modifications- RAMPS
Reaching overhead will be difficult
Sit to stand is very difficult
Progression is slower leads to client learning creative compensatory movements and techniques.
Screen for other disciplines
Respiratory therapy and swallowing
Slide36Role of PT and OT
Increase safety with all mobility and ADL’s to prevent falls
Caregiver training
Client hesitation with moving to a more supportive assistive device
Education
Progression of disease
Assess for assistive devices
Exercise
Limited research on
aerobic exercise and strengthening
in this population.
Slide37Role of PT and OT
Promote cardiovascular fitness
12 week program of cycling at 65% (3x a week the first 4 weeks to 5x
a week
the last 8 weeks)
increased Vo2max, did not significantly increase CK
Self reported increased walking distance
9 patients with LMGD type 2I
Support or strengthen existing musculature
Limited research on strengthening in this population. A small study explored strengthening knee flexion and extension and elbow flexion at low intensity. Findings: resistance exercise did increase strength and was tolerated.
.
Slide38Participation/Quality of Life
Bergsma
A.,et
al. Different profiles of upper limb function in four types of neuromuscular disorders.
Neuromuscular Disorders
. 2017;27:1115-1122.
Slide39Spinal Muscle Atrophy
Slide40SMA
Autosomal recessive
In
spinal muscular atrophy,
mutations
or deletions of the
SMN1
gene
(survival of motor neuron)produce
a shortage of SMN protein, which causes degeneration of motor neurons in the spinal cord
.
Most people have a copy of the SMN1 gene called the SMN2 gene but it only 10% of the protein it produces is functional
Slide41SMA
Levels
SMA 1
SMA 2
SMA 3
SMA 4
Spinraza
Children
Adults
Slide42Role of PT and OT
Preserve ROM
Prevent functional decline
Screen for other disciplines
Increase safety with all mobility and ADL’s to prevent falls
Education
Assess for assistive devices
Type 2
Type 3
Splinting/bracing
Slide43Role of PT and OT
Promote
cardiovascular
fitness
Support or strengthen existing musculature
Slide44Participation
Kruitwagen
‐van Reenen, E. T., van der Pol, L
.,
Schröder
, C. ,
Wadman
, R. I., van den Berg, L. H.,
Visser‐Meily
, J. M. and Post, M. W. (2018), Social participation of adult patients with spinal muscular atrophy: frequency, restrictions, satisfaction and correlates. Muscle Nerve.
Discussion: Motor skills, feelings of depression and fatigue are correlates of participation in daily life. This knowledge can be used to optimize care for SMA patients.
Slide45Final Thoughts
Slide46Home Modifications
Ramps
Entrances and shower
Areas that enjoy first
Convert living room/den into bedroom
Recliners for breathing and fatigue
Environmental control units
Grab bars
Slide47Most Common Reason for Referral to OT and PT
To help the patient and caregiver transition to a safer way to transfer (
esp
if using a sliding board), toilet, bathtub or car.
Bed mobility
To educate the caregiver and patient how to use a Hoyer lift
To start a safe aerobic exercise or resistance exercise program
To learn how to safely perform ROM exercises
To help the patient and caregiver transition to a safer way to walk with assistive devices
ADLS
Bracing
Home modifications
Slide48Documentation
Rehab in reverse
Skilled maintenance
Skilled services must be medically necessary and services must be skilled.
Document that you are making constant educated decisions about the patient’s care
Slide49Clinics
In Memphis
MDA- Nicole
Petrouski
901-748-3036
ALS- Beth
Bumgardner
901-854-8661
In Nashville
MD and ALS Johanna Fowler 615-832-5005
In Chattanooga
ALS Monica Bailey 423-778-2018
In Knoxville
ALS Dr.
Trudell
Cole Neuroscience Center
ETSU
ALS Courtney Andrews 423-439-4712
Slide50References
Bedlack
Richard and
Mitsumoto
, Hiroshi.
Amyotrophic Lateral Sclerosis: A patient care guide for clinicians.
New York: Demos Medical; 2013.
Johnson
LG, Collier KE, Edwards DJ, et al. Improvement in aerobic capacity & after an exercise program in sporadic inclusion body myositis.
Clin
Neuromusc
Dis
2009; 10:178 – 184.
Sveen
ML,
Jeppesen
TD,
Hauerslev
S, et al. Endurance training: An effective and safe treatment for patients with LGMD2I. Neurology. 2007;68:59–61.
http://www.apta.org/Payment/Medicare/CoverageIssues/SkilledMaintenance
/
Lora L. Clawson, Merit
Cudkowicz
, Lisa
Krivickas
, Benjamin R. Brooks, Mohammed
Sanjak
, Peggy Allred, Nazem
Atassi
, Amy Swartz, Gabrielle
Steinhorn
,
Alpa
Uchil
, Kristen M. Riley, Hong Yu, David A.
Schoenfeld
& Nicholas J. Maragakis (2018) A randomized controlled trial of resistance and endurance exercise in amyotrophic lateral sclerosis, Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 19:3-4, 250-258
Fleming-
Castaldy
, R. Self- Directed Personal Assistance Services, Participation, and Quality of Life.
SIS Quarterly Home and Community Health.
2013; 20(4)
Facts You Should Know. ALS Association. Retrieved from: http://
www.alsa.org
/about-
als
/facts-you-should-
know.html
Arbesman
, M. &
Sheard
, K. AOTA Critically Appraised Topic and Paper Series: Neurodegenerative Diseases. 2012