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Great Plains Regional Hemophilia Providers Meeting. Balance, P roprioception and the Aging H emophilia P opulation. Bruno UK Steiner, PT,MT The Anatomical Works 4/24/12. HIV HCV. https://www2a.cdc.gov/ncbddd/htcweb/UDC_Report. People with Hemophilia are maturing.
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Great Plains Regional Hemophilia Providers Meeting Balance, Proprioception and the Aging Hemophilia Population Bruno UK Steiner, PT,MT The Anatomical Works 4/24/12
HIV HCV
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 • Target joint • Repeated bleeding in the same joint Shoulder 8% Elbow 25% 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 • 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 • deterioratedproprioception 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, withspecificity 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
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
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.
Deterioration of Joint Position Sense Skinner, Barrack, J ElectromyogrKinesiol 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 proprioceptionor joint position sense • Decrease in the number of mechanoreceptors in rabbit ACL: the effects of aging. Aydog, Korkusuz et al, Knee SurgTraumatolArthrosc 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.”
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. Haemophilia2010
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!