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Balance, Proprioception and the Aging Hemophilia Population

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1 Balance, Proprioception and the Aging Hemophilia Population
Great Plains Regional Hemophilia Providers Meeting Balance, Proprioception and the Aging Hemophilia Population Bruno UK Steiner, PT,MT The Anatomical Works 4/24/12



4 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

5 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% Speaker’s Notes: Hemarthrosis is bleeding into a joint. Joint bleeding is the most common type of bleeding episode. The most commonly affected joints are the knees, elbows, and ankles but bleeding can occur in shoulders, hips, wrists or any other joint in the body. A target joint is repeated bleeding into a joint and often leads to hemophilic arthropathy. Knees 44% Hemophilic Arthropathy Ankle 15% Source: World Federation of Hemophilia. Facts and Figures Monograph Series

6 End-stage joint arthropathy
Destruction of cartilage Narrowing joint space Subchondral cysts Collapse and sclerosis

7 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….

8 Deterioration of joint position sense
deteriorated proprioception and balance in: standing, walking positional transfers

9 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

10 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

11 Proprioceptive Mechanoreceptors
Located in joint structures Located in muscle to transduce stretch of the muscle Located cutaneously



14 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

15 Type II Mechanoreceptor: Pacinian Corpuscle
Thought to respond to high velocity changes in joint position. found in skin and joint structures

16 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

17 Type IV Mechanoreceptors: Free Nerve Endings

18 Dorsal Spinocerebellar Tract
Mechanoreceptors conveys proprioceptive information to the cerebellum for further coordination and processing

19 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














33 Hemarthrosis Knees >50% of bleeds Elbow, ankles, shoulders, wrists

34 Intra-articular bleeding

35 Muscle Bleeding Signs and Symptoms Vague ache or pain Heat Swelling
Inability/unwillingness to move muscle Tightness of skin Speaker’s Notes: Muscle bleeding is the second most frequent site of bleeding. Any area of the body can be affected but frequently the extremities are involved. Large muscle bleeding can lead to nerve compression (i.e. iliopsoas, thigh - see picture). Significant blood loss can occur in large muscles before appreciable swelling is evident. Therefore, it is important to monitor hemogloblin levels. Bleeding into small muscles can also cause compartment syndrome (i.e. forearm, calf). Signs and symptoms of muscle bleeding may include: Vague ache or pain Heat Swelling Inability/unwillingness to move muscle Tightness of skin Source: Butler . Basic Concepts of Hemophilia 2001; 3; 12.

36 Courtesy Ollie Edmunds MD

37 Courtesy Ollie Edmunds MD

38 Courtesy Ollie Edmunds MD

39 Courtesy Ollie Edmunds MD

40 Courtesy Ollie Edmunds MD

41 Courtesy Ollie Edmunds MD

42 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

43 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

44 20 PWH and 20 controls (mean age 39.4)
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.”

45 A decrease in proprioception
Why does this all matter? A decrease in proprioception increases the risk of falls in People with Hemophilia

46 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

47 Fall Prevention is where Physical Therapists can have a great impact in the management of PWH

48 The Physical Therapist’s Role
Acute versus sub-acute management and treatment

49 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

50 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!

51 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

52 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

53 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

54 Proprioceptive Re-education
Balancing exercises Functional transfers Single leg standing progression to greater levels of difficulty and balance duration

55 Benefits of Resistive and Proprioceptive Training
Importance of resistance training for haemophilia patients1 increasing muscle strength decreasing the frequency and severity of bleeding episodes and associated pain 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

56 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

57 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.

58 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

59 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

60 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

61 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

62 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 = ) maximum knee pain (P = ) physical function (P = ) stiffness (P = ) compared to the baseline Brismee et al. Clin Rehabil. 2007

63 A word on assistive devices

64 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!

65 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?

66 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

67 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.

68 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.

69 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.

70 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

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