Alberto Friedmann, MS American College of Sport Medicine Exercise Treatment of Non-Accident Related Chronic Ankle Instability in Ehlers-Danlos Syndrome Alberto Friedmann, MS American College of Sport Medicine

Ankle Injuries in Sport: Common Debilitating Poorly Rehabilitated Reocurring Functional Instability

Chronic instability is not limited to injuries: Genetic and neuromuscular disorders result in instability without trauma. Hereditary Disorders of Connective Tissue (HDCT) Ehlers-Danlos Syndrome Marfan’s Syndrome Osteogenesis Imperfecta Benign Hypermobility Syndrome (Mild EDS Hypermobility – Type III)

Hereditary Disorders of Connective Tissue: Result of defective collagen (glue) Elastin and collagen are weak Tissue is frail and lax Fibrillins are weak and tear

Up to 70% show the same symptoms as those with frequent ankle sprains.

Unable to perform (0 points) Able to Perform (1 Point) “Hypermobility is defined as an abnormally increased range of joint motion due to excessive laxity of the constraining soft tissues” (Everman, 1998). Clinical Maneuver Unable to perform (0 points) Able to Perform (1 Point) Apposition of Thumb to Forearm Right Left 1 Extension of fifth finger beyond 90 degrees Extension of elbow beyond 10 degrees Extension of knee beyond 10 degrees Forward flexion of trunk, legs straight, palms touching the floor Total Beighton Score (0-9)

Functional Ankle Instability Inability to use the ankle for daily activity: Walking Standing Bathing Getting out of a chair Basic balancing Functional instability of the ankle is the most common residual disabilities after an acute ankle sprain.

Ankle Rehabilitative Therapies Strengthening (pronation) Balance Proprioception Caused by nerve-fiber damage Traditionally low in hypermobile subjects “Recent studies have demonstrated reduced proprioceptive sensation in the joints of subjects who have hypermobility syndrome. Such findings have led to speculation that impaired sensory feedback contributes to excessive joint trauma in effected individuals” (Everman, 1998).

Most common trauma: Talofibular Joint Calcaneofibular Joint Other trauma – often seen in hypermobile subjects: Talocrural Joint Talocalcaneal Joint Talocaneonavicular Joint Calcaneocuboid Joint

Traditional rehabilitation therapies: Balance Boards Theraband resistance Open and Closed Kinetic Chain Peroneal reaction treatment Joint proprioception treatment

Traditional means of rehabilitaion: Resistance training Reactive Neuromuscular training Proprioceptive training

Study purpose was to determine the effects of exercise on non-accident-related chronic ankle instability, particularly in subjects who have Ehlers-Danlos Syndrome or Benign Hypermobility Syndrome. The study looked at multi-range stability in the talocrural, talocalcaneal, talocaneonavicular and calcaneocuboid joints .

Hindfoot Talocrural Tibiofibular Talocalcanear (Subtalar) Midfoot Talocalcaneonavicular Cuneonavicular Cuboideonavicular Intercuneiform Cuneocuboid Calcaneocuboid

Participants: Direct Observation Volunteers Hypermobility Syndrome Five or higher on Beighton’s Scale 18-older No recent ankle trauma No severe ankle trauma

Procedures: Eight-Week Exercise Program Three days of exercise per week Traditional Rehabilitation Therapies Resistance Training Reactive Neuromuscular Proprioception/Balance Active stabilization exercises

Measures: Range of Motion Functional Strength Stability Proprioception Neurological Reflex Study start, after four weeks, study end (total of three measures)

Range of Motion: Standard Goniometer Anatomical Neutral Dorsiflexion – 200 Plantar Flexion – 500 Inversion – 50 Eversion - 50

Monthly Training Regimen Exercise Mon. Tue. Wed. Thur. Friday Sat. Sun. Resistance Training X Reactive Neuromuscular Training Proprioception Exercises Active Stabilization Exercises

Resistance Exercises Leg Press Calf Raise Knee Extensor Knee Flexor Two sets of 12 repetitions at 60% max. Resistance increased by 10% at week four Third set added at week seven

Reactive Neuromuscular Training Used resistance tubing: Uniplanar Anterior Weight Shift Uniplanar Posterior Weight Shift Uniplanar Medial Weight Shift Uniplanar Lateral Weight Shift

Proprioceptive Exercises One-foot standing balance One-foot standing balance with hip flexion One-foot standing balance using weights in diagonal pattern One-foot standing balance while playing catch Exercises on balance board

Active Stabilization Exercises Used a step-stool measuring 16” x 16” x 8” (40.6cm x 40.6cm x 20.3cm Forward step-up on stool Lateral step-up on stool Two-foot hop-up on stool Two-foot lateral hop-up on stool One-foot hop-up on stool One-foot lateral hop-up on stool Two-foot jump-over stool Two-foot lateral jump-over stool One-foot jump-over stool One-foot lateral jump-over stool

Functionality Plantar Flexion Dorsiflexion Inversion Eversion Measured on a scale 0-15: 0 Non functional 0-4 Functionally Poor 5-9 Functionally Fair 10-15 Functional

Functionality After four weeks: After eight weeks: Overall increase in functional strength Decrease in pain Decrease in joint popping After eight weeks: All participants fully functional in all tests Virtual elimination of pain Elimination of joint popping

Quality of Life Functional Strength Less = lower exercise = atrophy = less functionality More = more exercise = hypertrophy = more functionality = Independence= Higher Quality of Life

Quality of Life Pain More = depression = unwillingness to exercise = atrophy = more pain Less = Better outlook = social activities = social exercise = Less medication = Higher Quality of Life

Quality of Life Proprioception Less = Poor balance = unwillingness to exercise = Higher risk of injury = Nerve fiber damage = Decreased proprioception More = Better balance = feeling of ability = exercise adherence = lower risk of injury = Increased independence = Higher quality of life

Change in Range of Motion, Active

Change in Range of Motion, Passive

Overall Change in Range of Motion

Reduction in ROM Overall decrease in both passive and active indicates: Hypertrophy in tendons and ligaments as well as muscle tissue. Hypermobile joints can be strengthened and stabilized before laxity leads to injury. Proper, supervised exercise is of benefit to this population

Inversion Changes Inversion injuries: Common Reoccur Difficult to rehabilitate

Participants showed: Decreased Range of Motion Decreased Pain and Joint Popping Increased Balance and Proprioception Increased Daily Functioning

Implications Rehabilitation of other joints for this special population Shoulder Capsule Hip Socket Interphalangeal Joints Metacarpophalangeal Joints

Implications EDS patients, and possibly patients with other hypermobility syndromes, could be treated in multiple joints prior to disruptive injuries or the need for surgery due to joint hyperlaxity. Injury and surgery is more damaging and dangerous for this population than the average person.

Implications Study participants showed primary gains during the initial four weeks of study intervention Primary increases in range of motion occurred during the final four weeks of study intervention Therefore, it is possible that a four-week intervention followed by maintenance would be as, if not more, successful

Future Research Studies involving a larger population and studies involving multiple-joint treatments Long-term effects of exercise on children with Hereditary Disorders of Connective Tissue Four-week versus Eight-week programs Animal studies involving muscle, tendon and ligament tensile strength, elasticity and plasticity