5/25/18

Disclosures

Ø Program Manager ImagingDMD Studies

Physical Therapy Ø Sub-Investigator ImagingDMD Studies
University of Florida
Ø Instructor MedBridge Educational Courses

Claudia Senesac, PT PhD, PCS

University of Florida
Peggy Sedlacek, PT, MS
Children’s of Alabama

Pre Symptomatic
Observations Commonly Reported
Stage 1 • Pre-Symptomatic Difficulty noted with:
Climbing Stairs Getting up from the Floor-Partial Gowers

Stage 2 • Early Ambulatory Sign

Jumping Running: slow, no flight phase

Stage 3 • Late Ambulatory-Transitional Toe Walking Frequent Falling

Weakness Leg Cramps

Stage 4 • Early Non-ambulatory Clumsiness Fatigue

Large Calf M uscles M uscle Tightness-Hamstrings/HC

Stage 5 • Late Non-ambulatory Head lag on pull to sit- difficulty lifting head
from supine
Decreased score on BSID III
Bailey Scale of Infant D evelopm ent
(Connolly et al, 2013)

Delayed M otor Skills Delayed Speech

Begin A Regular Routine Stretching

Start Early- Begin a Routine
Make it Enjoyable…..Make it Fun!!!!

Concentration Areas Stretching Ø May help to maintain length of the

muscle over time
Heel Cords Incline Standing Board Ø May improve comfort and positioning in
bed
Hamstrings Long Sitting
Ø Improve hygiene
Hip Flexors Prone Lying Ø May improve fit of shoes
Ø May improve hand manipulation
IT Band Side Lying Stretch
Ø Improves Well Being
Consider Initiation of Night Braces Consider Behavior Ø Improves Circulation

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Important areas of focus for Stretching STRETCHING IN THE BEGINNING

In The Beginning
• Stretching heel cords
• Stretching hamstring muscles
• Stretching hip flexors

Hamstring stretch Heel Cord Stretch

Hip Flexor stretch

Important Areas to Add for Stretching ADDITIONAL STRETCHES

Addition to stretching program Stretching forearm pronators

• Stretching forearm pronators

• Stretching elbow

Stretching elbow flexors

Stretching Resources for Parents Pre-Symptomatic

Encourage Recreational Activities
• PPMD Website
Stretches for Duchenne Muscular Dystrophy (CD)-View online
http://www.parentprojectmd.org/site/PageServer?pagename=Care_resources_materials
Instructional Photographs -View online
http://www.parentprojectmd.org/site/DocServer/Sep_11_DMD_Book_Stretches1.pdf?docID=11703

• YouTube
Stretches for Duchenne Muscular Dystrophy-YouTube Video https://www.youtube.com/watch?v=6eHLt3KAOtg

• CINRG Website
Amazon.com

StretchOUT Stretch Instruction and Workout http://www.cinrgresearch.org/stretchvideo2/index.cfm

stjohn.ca

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Early Ambulatory Common Measures

Clinic vs. Clinical Trial

Ø Often just receiving diagnosis Ø Manual Muscle test

Ø Elementary School
Ø Initiating Steroids
Ø Difficulty keeping up with
peers
Ø Increased falling
Ø Occasional leg pain or
cramping
Ø ROM (tightness may begin to
develop or worsen)
Ø Myometry
Ø Range of Motion
Ø Goniometry
Ø Postural and Gait assessment
Ø Timed Performance Tests
Ø North Star Ambulatory Assessment
Ø 6 minute walk test
nytimes.com
Ø PUL
Ø Brooks Scale- UE
Ø Vignos Scale- LE

Why Are Assessments So Important Therapist’s Role

Encourage, Instruct, Educate Families and Child, Be
available to Consult
• Provide evidence for status and change in
status Ø Maximize Range of Motion and Gross Motor
Skills
• Provide feedback for change in medication or Ø Safety First
treatment Ø Families should be instructed in
Ø STRETCHING-Keep building a routine
• Demonstrate pattern of progression Ø Safe activities for play, recreation
• Predict need for intervention Ø Maximize Balance and Coordination
• Loss of ambulation: Equipment, home access, Ø Maintain Overall Fitness
transportation, transfer teaching, support Ø Consult with School: Adaptations/Modifications
• Help explain status to parents and others Ø DON’T OVER FATIGUE-Rest periods are “OK”

Night Splints- Often Recommended

How To Fit Everything In *Consider Goals

Ø Make stretching part of the daily

routine ØMaintains prolonged
Ø Brushing teeth
Ø During homework position for success
stretch (6+hours/night)
Ø Shown to be effective {Scott et al 1981, Hyde et al 2000)
Ø Active assistive stretching- get child
involved ØNight vs Day use
Ø Equipment that might help ØIncreased padding
improve/maintain ROM
Ø Night splints ØShoes vs no shoes
Ø Standing wedge
Ø Prone wedge

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Late Ambulatory-Transitional Why Predicting Loss of

A Time of Change Ambulation is Important
Ø Limited community mobility • The impact on families is huge and life
Ø Increased risk of falls changing
Ø Increased fatigue • Discussion: start early and repeat often
Ø Consider power W/C purchase • Equipment often takes 6 months to receive
Ø Needs change: across life span
Ø Provider(s), location of services change • Home modifications are costly and stressful
with needs, and frequency • Transportation becomes challenging
Ø Continual assessment of status and • Transfers are challenging
needs
Ø Goals are unique and challenging
ocregister.com
• School needs time to prepare (hoyer,
based on individual and family classroom location, evacuation plan, etc).

Fractures
Fracture? Now what?
Ø Most often in the lower extremities
Ø Generally in boys older than 9
years
Ø Falls while ambulating
Ø Falls from wheelchair (seat belts!!)
Ø May result in loss of ambulation
Ø Boys need to avoid immobilization
Ø Families need to contact their
specialists
James et al, 2015; Subasi et al, 2016 Abstract MDA Clinical Conference
Consult “NEW” Care Guidelines www.thelancet.com or www.parentprojectmd.org

Remobilization After Fracture Early Non-Ambulatory

Ø Family education!!! Ø Increased tightness
Ø Mat Exercise Ø Encourage activity Ø Disuse atrophy
Ø Aquatic activities Ø Increased weight gain
Ø Aquatic Therapy
Ø Ergometers – Ø Loss of functional
Ø Body Weight Supported TM active assist independence

Ø Up and Free walker- May be an Ø Assistance for Ø Changes in

Transfers community
option www.litegait.com
Ø Equipment needs participation
Ø Introduce weight bearing ASAP changing Ø Initiation of BiPAP/
Cough Assist
Ø Work on Balance reactions wcpo.com
Ø May increase risk of
scoliosis

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Late Non-Ambulatory Daily Routines Changing

Ø May have increase c/o Pain Ø Transfers-more dependent
Ø Continue LE stretching
Ø Maximal Assistance
Ø Include UE stretching
Ø Limited UE Function
Ø Sustained positioning
Ø Respiratory Support
Ø ¼ - ¾ Prone lying if tolerated
Ø Reliance on Technology
Ø Side Lying
Ø Decreased Participation Ø Standing frames
Ø Decreased Employment Options Ø Elevating leg rests
Ø May need to Employ Caregivers Ø Reclining in WC
CINRG: StretchOut Video

www.easystandevlov.com

Assistive Technology MedTrade Expo

Exciting and Changing all the Time Largest Medical Equipment
Ø Blue tooth devices
Ø Siri/Dragon Speak
Tradeshow and Conference
Ø Environmental control systems
Ø Google home & Amazon Echo
Ø Computers / tablets / smart phones Spring: Las Vegas February 2018-look
Ø Alternative keyboards/ touch pad at 2019 schedule for west coast dates
screens Fall: Atlanta October 2018
Ø Electronic pointing devices
https://www.medtrade.com/
Ø Voice assist (amplifiers)
Ø Glassouse glassouse.com
Ø TouchTapSwipe guide
Ø (dmdpathfinders.org.uk)

Historically
Ø Exercise recommendations have been
based on rodent studies that induced
Exercise injury
Ø Resultant recommendations were:
exercise may cause damage- exercise
with caution
Ø Current studies suggest
Ø Exercise may be beneficial- BUT questions
remain
Ø Age?
Ø How much?
Ø What type?

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Exercise Exercise
What do we know…..
Ø Staying active is KEY
Ø No exercise leads to muscle atrophy Ø Stretching may help to maintain ROM allowing activity and
participation
Ø Important for bone health
Ø Age appropriate recreational activities as opposed to “resistive”
Ø Important for self-esteem strengthening regimes
Ø Too much exercise increases muscle breakdown Ø Concentric low load or isometric versus eccentric high load - stay
Ø Rhabdomyolysis submaximal-add power if needed/energy conservation
Ø Younger boys benefit from exercise more than older Ø Don’t overdo!! Build in Rest Periods
boys * however most of the research is with this group Ø Self modulation
Ø Boys with DMD are 40% less active than age Ø Structured breaks
matched peers (McDonald, 2000) ** supported by
other studies Ø Fatigue is REAL, Differences in endurance
Ø Incorporate balance and coordination skills

Assisted bicycle training delays functional deterioration in boys with
Duchenne muscular dystrophy: the randomized controlled trial "no use is
disuse".
Jansen M, van Alfen N, Geurts AC, de Groot IJ
Different types of upper extremity exercise training in Duchenne muscular
dystrophy: effects on functional performance, strength, endurance, and
ambulation.
Alemdaroglu I, Karaduman A, Yilmaz ÖT, Topaloğlu H

Arm and Leg Ergometer Ex-n-Flex (active assist) • Subjects = 24 boys ages 8-12 y/o, ambulatory
• 24 boys age 8-12 years (amb and non-amb) •
• Training at 50% max, 40 minutes, 3 day/week • Study group n=12 * assistive UE ex/PT-Arm
Ergometer
• 24 weeks – 40 minutes per session, 3x/week x 8 weeks
Assisted bicycle training – positive effects on subjects’ muscular endurance,
• Delays functional deterioration in boys with DMD performance of ADL’s arm function, ambulation
status- NO significant change in muscular strength
– Outcomes remained stable (MFM and Assisted 6min M O T O m e d V iv a 2 M o v e m e n t T h e ra p y
S y s te m s ;

cycling test) • Control group n=12 *strengthening ROM ex/Family

R E C K -T e c h n ik G m b H & C o . K G , G e rm a n y )

• No serious adverse events – 40 minutes per session, 5x/week x 8 weeks

• Safe and feasible – Improved grip strength and endurance only
• May decline the deterioration due to disuse – Improved NSAA score
• Summary-Both groups improved to varying levels Muscle Nerve. 2015 May;51(5):697-705. doi:
Neurorehabil Neural Repair. 2013 Nov-Dec;27(9):816-
27. doi: 10.1177/1545968313496326. Epub 2013 Jul 24. 10.1002/mus.24451. Epub 2015 Mar 5.

Pilot Study for Mild to Moderate-Intensity Resistance Exercise in Boys

with Duchenne Muscular Dystrophy Resources in Alabama
DJ Lott, KD Cooke, HJ Park, BM Black, SC Forbes, BJ Byrne, GA Walter, K Vandenborne

Subjects: 8 ambulatory boys w/DMD (8.3 + 0.7yr)

Safety Measures: 48 hr before/after
• Therapy services at
Ø T 2 weighted MRI Children’s and Beyond
Ø Pain assessment • Community resources
Ø Clinic Exam & CK levels
• Education support
Protocol: 4 exercises-Isometric maximal voluntary contraction (MVC)
Ø Quads and Hams at 2 different angles
• Equipment resources
• Recreation
Ø Isometric exercise-Mild level (30%MVC) n=4
Ø Isometric exercise-Moderate level (50%MVC) n=4
• Participation
Example of protocol
Exercise Hamstrings- 30 and 60 degrees
Exercise Quadriceps- 30 and 60 degrees
Preliminary Results indicate that exercise is feasible and safe and may be beneficial

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Therapy Services at Children’s and Community Resources

Beyond
• Outpatient COA PT & OT: 205-638-9645; • Children’s Rehabilitation Services: Assist with
205-638-6289 wheelchair costs; Back up manual wheelchair; Home assessment and
modifications; Transfer equipment; Bathing equipment; Service coordination
• Outpatient PT: Night splinting; Equipment referrals– wheelchairs, (Muscle Shoals, Huntsville, Gadsden, Anniston, Birmingham/Homewood; Tuscaloosa; Selma;
patient lifts, standers, bathing ; Stretching; Positioning; Home modifications; School Montgomery; Opelika; Jackson; Andalusia; Dothan) www.rehab.alabama.gov
consultation-Plan for college mobility
• ADRS at Lakeshore: Adaptive Driving Training; Assistive
• Outpatient OT: Hand splinting; Stretching; ADL’s; Adaptive equipment; Technology; Employability Development, Vocational training - Phone: (205) 870-
Mobile Arm Supports; Gaming controllers; W heelchair adaptations (head control, 5999
joystick adaptations, Voice activated system, Assistive technology; Home
modifications recommendations; School strategies; Sleeping positions; Plan for
college needs • Teen Transition Clinic: To assist adolescents and young
adults in their planning, for transition to adult life are eligible to participate in
• Hippotherapy: Special Equestrians ((205) 987-W HOA)-One on One the clinic; www.rehab.alabama.gov
therapy using the horse

• Aquatic therapy: Huntsville Hospital for Women and Children – 256-

265-7952; Champion Sports Medicine- 205-671-8682

More Community Resources

Equipment
• Wheelchair vendors: Numotion (205-833-
0284); National Seating and Mobility (205-
Therapist 621-7332); Alabama Wheelchair (205-322-
3250) (especially short term rentals)
Medical Insurance
Vendor Funding • Orthotics: Biotech Limb and Brace (205-324-
7897); Hanger Orthotics (Birmingham,
Montgomery, Tuscaloosa, Mobile ; Talladega)
Family Birmingham Limb and Brace; Precision
Medical Solutions- Montgomery- 334-260-
3767; Auburn - 334-826-0078

Education Support Recreation

• ADAP services: ADAP provides legal • Lakeshore (205-313-7400) Lakeshore.org:
services to Alabamians with disabilities to protect, Activities separated by age - Swim classes; Active
promote and expand their rights. adap@ua.edu; children exercise (ACES); Mini Movers; Fish out of
• Bullying; Children’s Mental Health; Early Childhood; Water; Fresh; Archery; Track and Field
Foster care; Juvenile Justice; Special education

• Children’s of Alabama PT & • Local YMCA’s Swimming!!!!

OT – 205-638-9645 - Consult to schools Alabaster, Anniston, Atmore, Bessemer, Birmingham, Vestavia, Pelham,
Trussville, Mountain Brook, Brewton, Chilton County, Daphne,
Enterprise, Florence, Foley, Gadsden

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More Recreation More Recreation

Therapeutic Horse Back Riding: Special
Equestrians- Birmingham - (205) 987-9462-
• Miracle League Baseball: North Alabama
(mlhuntsville.com); Coastal Alabama (miracleleaguecoastalalabama.com);
www.specialequest.org; The Red Barn-Leeds Tuscaloosa (miracleleagueoftuscaloosa.com); Montgomery
(theredbarn.org) (205) 699-8204; Storybook (montgomerymiracle league.com); East Alabama (miraclefield.org); Dothan;
Over the Mountain; Gardendale
Farm- Opelika - (334) 444-5966-
www.hopeonhorseback.org; Therapeutic
Riding of Tuscaloosa- 712 355-8962 or 205
• Miracle League Soccer – available
752-7691 -www.trotusa.org; Happy Trails in some locations
Therapeutic Riding Center – New Market -
happytrailstrc.org

Participation Participation continued

• Camp ASCCA- Jackson’s
Gap, AL – (256) 825-9226
• Lakeshore: 205-313-7400- include siblings – summer and year long
and friends: Super Saturdays - fun; Camp Strive, JAWS, weekend camps – one
Adventure and Inclusion summer camps; Movie nights
week designated for DMD
• Lakeshore sports teams: Power – staff well trained-
soccer; Swim team; Bocce ball; Wheelchair horseback riding,
basketball; Wheelchair rugby swimming, canoeing,
fishing, arts and crafts,
• State campgrounds: 1-800-ALA- accessible waterslide,
PARK (252-7275); www.alapark.com zip-line, water tubing,
archery and rifle range,
mini-golf course, paved
nature trails, splash
pad…..

Educational Environment

Evac-chair
• IEP and/or 504 Plans
School Environment • Accessibility issues
Things to Consider • PE/ Field trip participation
• Safety Plans
• Equipment for transfers
• Toileting equipment

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Common Campus Modifications Common Classroom Modifications

• Extra time to get to class
– Classes close together if possible
• Individualized PE modifications
– With extended breaks as needed
– Adaptive sports (involve the whole
class for socialization)
• Use of accessible bathroom
• Key for elevator (for those still ambulating)
• Staff trained in transfers (using a lift)
• Assistance for lunch set up
• Safety evacuation plan
• Copies of PowerPoints or notes
• Extra set of books for home
• Use of laptop/tablet or scribe
• Modified homework to limit fatigue
• Extra time for standardized or timed tests
• Oral tests if writing difficult
• One-on-one aide
• Individualized and preferential seating or
desk

Transition to Higher Education What is Vocational Rehabilitation?

http://www.rehabworks.org

Resources
What is it?
• State Vocational Rehabilitation Department
STAR program Federal program designed
• Scholarships & Financial Aids to:
• https://www.mda.org/young-adults/resources • Enhance independence
of people with
• College or University Office of Student disabilities by helping
Disability Services them find and maintain
• Center for Independent Living (dependent employment
on location) Located throughout the
state by county and city
Services
• Can vary at each location
• Ranges from:
o Career Counseling
o Job Training & Placement
o Supported Employment
o Assistive Tech training &
support
o Medical & Psychological
Assessment
• STAR Program
– Ages 15-21 for students

Claudia Senesac: csenesac@phhp.ufl.edu

Peggy Sedlacek: Peggy.Sedlacek@childrensal.org
PPMD: www.parentprojectmd.org