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. - PowerPoint PPT Presentation
Balance, Proprioception and the Aging Hemophilia Population
Balance, Proprioception and the Aging Hemophilia PopulationBruno UK Steiner, PT,MTThe Anatomical Works
4/24/12Great Plains Regional Hemophilia Providers Meeting
https://www2a.cdc.gov/ncbddd/htcweb/UDC_ReportPeople with Hemophilia are maturingThey 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 ArthropathyHemarthrosis (Joint Bleeding)Most common site of bleedingMost frequently affected joints:Knees, elbows and anklesTarget 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. 1998.5Speakers 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.
End-stage joint arthropathyDestruction of cartilageNarrowing joint spaceSubchondral cystsCollapse and sclerosis
6Hemophilic arthropathy might be similar to osteoarthritis Valentino, JTH, 2000Important implication for a community PT (we know how to treat OA)Both result in Structural and functional failure of synovial jointsLoss and erosion of articular cartilageAlteration of subchondral boneSynovial inflammationPain and disabilitySevere decrease in ROM, strength, functionAnd.Deterioration of joint position sense
deteriorated proprioception and balance in:standing, walking positional transfers
ProprioceptionIs the bodys sense/awareness of position and movementIt is how our CNS monitors movement and coordinates postural/motion adjustmentInvolves peripheral mechanoreceptors: which sense deformational, velocity and positional change in joint and related tissuesRelays info to the cerebellum and cerebral cortex for further processing
Proprioceptive MechanoreceptorsNerve 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, IVDescribed in many tissues of the locomotor system: Cruciate and Collateral ligaments, Menisci, Joint capsules, Tendons, Tendon Sheaths, and Aponeurosis. McCray, 2005Proprioceptive MechanoreceptorsLocated in joint structuresLocated in muscle to transduce stretch of the muscleLocated cutaneously
Type I Mechanoreceptor: Ruffinis 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
HemarthrosisKnees >50% of bleedsElbow, ankles, shoulders, wrists
34Muscle BleedingSigns and SymptomsVague ache or painHeatSwellingInability/unwillingness to move muscleTightness of skin
Source: Butler . Basic Concepts of Hemophilia 2001; 3; 12.
35Speakers 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 painHeatSwellingInability/unwillingness to move muscleTightness of skin
Courtesy Ollie Edmunds MDCourtesy Ollie Edmunds MDCourtesy Ollie Edmunds MDCourtesy Ollie Edmunds MDCourtesy Ollie Edmunds MDCourtesy Ollie Edmunds MDDeterioration of Joint Position Sense Skinner, Barrack, J Electromyogr Kinesiol 1991 Sep;1(3):180-90Joint 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 deteriorationReconstruction 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 senseDecrease 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 controlsRecommendations 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 HemophiliaWhy does this all matter?A Fall can have a big impact on the lifestyle of a PWHOften require immobilization and factor productSometimes hospitalizationSometimes a permanent reduction in their mobilityFurthermore, fear of falling can limit confidence and restrict lifestyle choicesFearn, Hill et al. Haemophilia 2010Fall Preventionis where Physical Therapists can have a great impact in the management of PWHThe Physical Therapists RoleAcute versus sub-acute management and treatment
The Acute PatientRICE, clotting factorFocus on damage containment, decreasing swelling, pain, tissue tensionAssess nerve entrapment, compartment syndromes and neurovascular compromiseLoading a bleeding joint results in progressive joint damageMust prevent continued synovial membrane microtrauma and mechanical impingement (can result in repeated bleeding)Mulvany, 2003Sub-Acute/Chronic Rehabilitation
Treatment must be individualized to meet the patients needsThe patient may need to infuse pre-therapy to reduce bleed riskMust focus on fall prevention!PTs need to Assess:Strength, ROM of the affected extremityResultant joint hypomobility/stiffness assess whether due to joint deformity, joint or myofascial contractureBalance/proprioception in standing as well as gaitFunction/Transfers: sit to stand, stand to sit, bed mobility
PT Assessment contdGet a sense of the patients joint/ tissue irritability to guide the treatment approach and intensity Treatment should progress as per patients tolerance levels (pain and muscle fatigue must be considered in tailoring any exercise regimen)Assess use of Gait assistive devicesPT Sub-acute/chronic treatmentSoft tissue mobilizationJoint mobilizationStretchingCasting SplintingResistance training Low impact, mid range (avoidance of extremes of range, and explosive movts)Orthotics and assistive devices/wheeled mobility
Proprioceptive Re-educationBalancing exercisesFunctional transfersSingle leg standing progression to greater levels of difficulty and balance durationBenefits of Resistive and Proprioceptive TrainingImportance of resistance training for haemophilia patients1 increasing muscle strengthdecreasing the frequency and severity of bleeding episodes and associated pain Tailored home exercise program targeting balance, strengthening and walking2positive 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 interventionThese 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 PopulationShould have exercise session at least twice a weekShould be a lifelong practiceYou dont use it, you lose it This goes for the entire aging population, not just for PWH. Physical activity and exerciseIncrease joint circulationNutrition to articular cartilageStrengthen musclesImprove joint stabilityPreserve/improve joint function and ROMWeight loss/maintenanceRelieved pressure on weight bearing joints
Role of Exercise