Exercise Treatment of Non-Accident Related Chronic Ankle Instability in Ehlers-Danlos Syndrome

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

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

3. 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)

4. 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

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

6. “Hypermobility is defined as an abnormally increased range of joint motion due to excessive laxity of the constraining soft tissues” (Everman, 1998).

7. 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.

8. 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).

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

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

11. Traditional means of rehabilitaion: • Resistance training • Reactive Neuromuscular training • Proprioceptive training

12. 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 .

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

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

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

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

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

18. Monthly Training Regimen

19. 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

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

21. 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

22. 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

23. 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

24. Functionality • After four 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

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

26. 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

27. 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

28. Change in Range of Motion, Active

29. Change in Range of Motion, Passive

30. Overall Change in Range of Motion

31. 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

32. Inversion Changes • Inversion injuries: • Common • Reoccur • Difficult to rehabilitate

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

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

35. 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.

36. 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

37. 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