Download Balance, Proprioception and the Aging Hemophilia

Great Plains Regional Hemophilia Providers Meeting
Balance, Proprioception and the
Aging Hemophilia Population
Bruno UK Steiner, PT,MT
The Anatomical Works
4/24/12
https://www2a.cdc.gov/ncbddd/htcweb/UDC_Report
People with Hemophilia are maturing
• They will gradually exhibit challenges and
diseases of aging that we all ultimately face
whether they are orthopedic, neurological,
circulatory, organic.
• In some cases, the challenges will be greater
for the Person with hemophilia ie. greater
incidence of osteoporosis, arthritic changes.
• A greater incidence of falls, which can be
catastrophic for this clientele
Hemarthrosis
(Joint Bleeding)
• Most common site of bleeding
• Most frequently affected joints:
– Knees, elbows and ankles
Shoulder 8%
Elbow 25%
• Target joint
– Repeated bleeding in the same joint
Hip 5%
Knees 44%
Ankle 15%
Hemophilic Arthropathy
Source: World Federation of Hemophilia. Facts and Figures Monograph Series. 1998.
End-stage joint arthropathy
– Destruction of
cartilage
– Narrowing joint
space
– Subchondral cysts
– Collapse and
sclerosis
Hemophilic arthropathy might be
similar to osteoarthritis
Valentino, JTH, 2000
• Important implication for a community PT (we
know how to treat OA)
• Both result in
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Structural and functional failure of synovial joints
Loss and erosion of articular cartilage
Alteration of subchondral bone
Synovial inflammation
Pain and disability
Severe decrease in ROM, strength, function
And….
Deterioration of joint position sense
• deteriorated proprioception and balance in:
– standing,
– walking
– positional transfers
Proprioception
• Is the body’s sense/awareness of position and
movement
• It is how our CNS monitors movement and
coordinates postural/motion adjustment
• Involves peripheral mechanoreceptors: which
sense deformational, velocity and positional
change in joint and related tissues
• Relays info to the cerebellum and cerebral
cortex for further processing
Proprioceptive Mechanoreceptors
• Nerve endings which are part of the PNS
• Provide continuous afferent flow of nerve
impulses to the CNS (Cerebellum, Thalamus,
Cortex via the spinal cord)
• Classified Type I, II, III, IV
• Described in many tissues of the locomotor
system: Cruciate and Collateral ligaments,
Menisci, Joint capsules, Tendons, Tendon
Sheaths, and Aponeurosis.
McCray, 2005
Proprioceptive Mechanoreceptors
• Located in joint structures
• Located in muscle to transduce stretch of the
muscle
• Located cutaneously
Type I Mechanoreceptor: Ruffini’s Corpuscle
• Located in the deep layers of the skin, ligaments, joint structures
• Registers mechanical deformation within joints, angle change, with specificity
of up to 2 degrees
Type II
Mechanoreceptor:
Pacinian Corpuscle
• Thought to respond
to high velocity
changes in joint
position.
• found in skin and
joint structures
Type III Mechanoreceptor:
Golgi Tendon Organ
• Neurotendinous stretch
receptors
• Helps regulate the force of
muscle contractions
• Monitors muscle force
through the entire
physiological range of motion
• Affects the timing of the
transitions between the stance
and swing phases of walking
Type IV Mechanoreceptors: Free
Nerve Endings
Dorsal Spinocerebellar Tract
• Mechanoreceptors conveys proprioceptive
information to the cerebellum for further coordination
and processing
Dorsal ColumnMedial
Lemniscal
Pathway
• Information from
Mechanoreceptors
are transmitted to the
Medulla Oblongata
• From M.O. to the
Thalamus and
ultimately relayed to
the Cerebral Cortex
Hemarthrosis
• Knees >50% of bleeds
• Elbow, ankles,
shoulders, wrists
Intra-articular bleeding
Muscle Bleeding
Signs and Symptoms
• Vague ache or pain
• Heat
• Swelling
• Inability/unwillingness
to move muscle
• Tightness of skin
Source: Butler . Basic Concepts of Hemophilia 2001; 3; 12.
Courtesy Ollie Edmunds MD
Courtesy Ollie Edmunds MD
Courtesy Ollie Edmunds MD
Courtesy Ollie Edmunds MD
Courtesy Ollie Edmunds MD
Courtesy Ollie Edmunds MD
Deterioration of Joint Position Sense
Skinner, Barrack, J Electromyogr Kinesiol 1991 Sep;1(3):180-90
• Joint position sense in the normal and
pathological knee joint: Conclusions
– Structural damage (ACL disruption, arthritis,total knee
replacement) as well as aging cause deterioration of Joint
position sense
– Total knee replacement and arthritic change cause the
greatest deterioration
– Reconstruction of ligamentous structures and/or
rehabilitation appears to restore joint position sense to a
near normal level
Furthermore aging appears to decrease the number of
mechanoreceptors responsible for proprioception or
joint position sense
• Decrease in the number of mechanoreceptors in rabbit ACL:
the effects of aging.
Aydog, Korkusuz et al, Knee Surg Traumatol Arthrosc 2006 April
– Researchers conclude that aging results in both diminished
numbers and changed morphology of mechanoreceptors
Balance dysfunctions in adults with Haemophilia
Fearn, Hill et al, Haemophilia (2010)
• 20 PWH and 20 controls (mean age 39.4)
• Impairment of balance in PWH compared with
controls
• Recommendations made:
– “clinicians should include assessments of balance
and related measures when reviewing adults with
haemophilia.”
Why does this all matter?
A decrease in proprioception
increases the risk of falls in
People with Hemophilia
A Fall can have a big impact on the
lifestyle of a PWH
• Often require immobilization and factor
product
• Sometimes hospitalization
• Sometimes a permanent reduction in their
mobility
• Furthermore, fear of falling can limit
confidence and restrict lifestyle choices
Fearn, Hill et al. Haemophilia 2010
Fall Prevention
is where Physical Therapists can
have a great impact in the
management of PWH
The Physical Therapist’s Role
Acute versus sub-acute management
and treatment
The Acute Patient
• RICE, clotting factor
• Focus on damage containment, decreasing
swelling, pain, tissue tension
• Assess nerve entrapment, compartment
syndromes and neurovascular compromise
• Loading a bleeding joint results in progressive
joint damage
• Must prevent continued synovial membrane
microtrauma and mechanical impingement
(can result in repeated bleeding)
Mulvany, 2003
Sub-Acute/Chronic Rehabilitation
• Treatment must be individualized to meet the
patients needs…
• The patient may need to infuse pre-therapy to
reduce bleed risk
• Must focus on fall prevention!
PTs need to Assess:
• Strength, ROM of the affected extremity
• Resultant joint hypomobility/stiffness
– assess whether due to joint deformity, joint or
myofascial contracture
• Balance/proprioception in standing as well as
gait
• Function/Transfers:
– sit to stand, stand to sit, bed mobility
PT Assessment cont’d
• Get a sense of the patient’s joint/ tissue
irritability to guide the treatment approach
and intensity
– Treatment should progress as per patient’s
tolerance levels (pain and muscle fatigue must be
considered in tailoring any exercise regimen)
• Assess use of Gait assistive devices
PT Sub-acute/chronic treatment
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Soft tissue mobilization
Joint mobilization
Stretching
Casting
Splinting
• Resistance training
• Low impact, mid range (avoidance of extremes of range, and
explosive movt’s)
• Orthotics and assistive devices/wheeled mobility
Proprioceptive Re-education
• Balancing exercises
• Functional transfers
• Single leg standing progression to greater
levels of difficulty and balance duration
Benefits of Resistive and
Proprioceptive Training
1. Importance of resistance training for haemophilia patients1
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increasing muscle strength
decreasing the frequency and severity of bleeding episodes and
associated pain
2. Tailored home exercise program targeting balance,
strengthening and walking2
– positive physical outcomes including improved balance
and mobility
1Tiktinsky
et al Haemophilia 2002Hill,
2Fearn et al Haemophilia 2010
But balance training has to be ongoing
Evidence of detraining after 12-week home-based exercise
programs designed to reduce fall risk factors in older people
recently discharged from hospital
Vogler et al Arch Phys Med Rehabil 2012 April 11
– Conclude that balance improvements and fall risk reductions
associated with the program were partially to totally lost after
cessation of the intervention
– These significant detraining effects suggest that sustained adherence
to falls prevention exercise programs is required to reduce fall risk
Proprioception as a Way of Life
for the Maturing Hemophilia
Population
• Should have exercise session at least twice a
week
• Should be a lifelong practice
• You don’t use it, you lose it
– This goes for the entire aging population, not just
for PWH.
Physical activity and exercise
– Increase joint circulation
• Nutrition to articular cartilage
– Strengthen muscles
– Improve joint stability
– Preserve/improve joint function and ROM
– Weight loss/maintenance
• Relieved pressure on weight bearing joints
Role of Exercise and Physical Activity
on Hemophilic Arthropathy
Various exercises include:
• Aerobic exercise
– Walking
– Aquatic/swimming
– Biking
• Strength/resistance training
– May stabilize joints
– Improved walking ability, disability and pain in elderly with OA (FAST
study)
– Isometric training
• Balance and flexibility
– Stretching (tai chi, yoga)
– Improved flexibility of muscles and tendons around affected joints
• Eases pain
• Improves balance
Forsyth et al. Haemophilia 2011
Recommended Activities
• Low impact, mid range (avoidance of extremes
of range)
• Swimming
• Resistance training
• Tai chi (or Tai Chi like): a martial art with
profound benefits
Tai Chi
• Using all muscles/joints (big and small)
• Using smooth motion with wide range of
motion but no hyperextension
• Isometric, concentric and eccentric exercises
• Never incorporates extreme movements
– there will be no stresses or strains causing
hemarthroses or muscle bleeds
– Smooth, slow, gradual loading and unloading of
joint and muscle: no explosive movt’s
– A truly choreographed neurophysiological
workout
Group and home-based tai chi in elderly
subjects with knee osteoarthritis
• Randomized clinical trial
• 41 adults (70 +/- 9.2 years) with knee osteoarthritis
• 6 weeks of group tai chi sessions (40 min) TIW,
followed by another 6 weeks of home-based tai chi
training
• Significant improvements in
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mean overall knee pain (P = 0.0078)
maximum knee pain (P = 0.0035)
physical function (P = 0.0075)
stiffness (P = 0.0206) compared to the baseline
Brismee et al. Clin Rehabil. 2007
A word on assistive devices
A Word on Gait Assistive Devices:
Use of Cane
• A cane should be used with the hand on the opposing
side of the affected knee/ankle
- with right knee/ankle arthropathy, use cane in the
left hand
- right heel strike should accompany left cane strike
• Cane height should be measured to the crease of the
wrist… consider use of bicycle glove if pressure is an
issue.
• … There are always exceptions!
Exceptions: Use of Canes
• Case 1.
- Right sided LE arthropathy (knee/ankle) and left
sided UE arthropathy (elbow/shoulder)
- Which side for the cane?
• Case 2.
- Patient has right sided knee OA and uses the
cane on his right. When trying to train the use on
his left, his balance and use of the cane is
precarious at best.
- What do you do?
Maybe Assess the use of a Roller
Walker
• Handle height should be to the crease of the
wrist.
– Typically, people have them a little or much too
high, resulting in shoulder and elbow pain.
• Appropriate ambulatory assistive devices
should be considered proprioceptive training
equipment
Adaptive/assistive considerations
– Consult Occupational Therapy
Use of different bath grab bar configurations following
a balance perturbation
Guitard, Sveistrup et al Ottawa, Canada, Assist Technol 2011 Winter;23(4):205-15
• Vertically oriented bars appear to be favored
• Recommends use of vertical grab bars in the bath to
promote safety
• Additional bars may be needed to ensure safety during
stand to sit and sit to stand phases of bath transfer.
Cautionary notes/suggestions for the
multidisciplinary Team
• Communicate with community PT (provide
insight and information for this special
clientele)
• Verify whether your patient is engaged in
balance training program, encourage these
types of activity
• PT care should be individualized.
– Ideally, the therapist should not work on too many
patients at once.
Manual Physical Therapy
• Specific gently administered soft tissue
manipulation and joint mobilization
• Effective for contractures and marked
myofascial and joint tightness
• May progress clients to greater muscle and
connective tissue length. Moderate
improvement in ROM may improve function
and pain considerably.
Other considerations
• The importance of a good working
relationship between therapist and client
• Consultation with PT for other orthopedic
conditions that normally crop up
– SI, spinal, myofascial pain and strains/sprains
nerve root irritation etc.
• If need be, patient may benefit and progress
with a change in therapist