Balance, Proprioception and the Aging Hemophilia Population

Bruno UK Steiner, PT,MTThe Anatomical Works

4/24/12

Great Plains Regional Hemophilia Providers Meeting

HIVHCV

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

Hemophilic Arthropathy

Hemarthrosis (Joint Bleeding)

• Most common site of bleeding• Most frequently affected joints:

– Knees, elbows and ankles• Target joint

– Repeated bleeding in the same joint

Shoulder 8%

Elbow 25%

Hip 5%

Knees 44%

Ankle 15%

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 – 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 Column-Medial 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 HaemophiliaFearn, 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.”

A decrease in proprioception increases the risk of falls in People with Hemophilia

Why does this all matter?

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 Preventionis 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

• 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 – 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 Lifefor 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

• 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

Various exercises include:

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

– 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

– 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.

Adaptive/assistive considerations

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