Take this to your PT

By Wendy Wagner, PT, MPT and Dr. Lilian Holm, PT, DPT

Dear colleague,

Your patient has been diagnosed with hypermobility spectrum disorder (HSD) or a form of Ehlers-Danlos Syndrome (likely the hypermobile type, hEDS). Please take note of these issues that may affect your assessment, treatment planning and the patient’s response to treatment during physical therapy.

ASSESSMENT

  • Collagen defect: There is suspected to be a genetic defect in the way collagen is built. For the purpose of PT, this means primary stabilizers like tendons and ligaments do not tolerate load and stretch as a typical patient.

  • Tissue fragility: Collagen is found in every tissue in the body (bone, ligament, tendon, vascular system, fascia, etc). Fragile tissues are prone to injury and may heal slower.  

  • Proprioception: Joint capsules can be stretched and likely provide poor proprioceptive input that cause patients to be inaccurate with their “safe range.”

  • Medical gaslighting: It is a common lived experience due to the wide range of body systems affected. Radiographic evidence does not always correlate with symptom presentation. The patient is the best reporter of their symptoms and response to intervention.

  • Comorbidities: Multiple comorbid conditions but most commonly postural orthostatic tachycardia (POTS), mast cell activation disorder (MCAD), GI dysfunction, anxiety and social isolation are extremely common in patients with a connective tissue disorder. Other common comorbidities include: Headaches/migraines, Chiari malformations, TMJD, SIJ dysfunction, pelvic floor dysfunction, vascular compression syndromes, depression/anxiety, and neuropathies. Their impact on the patient’s ADLs must be addressed in order for meaningful physical rehabilitation to occur. 

  • Common symptom presentation: Patients typically present with kinesiophobia, are generally deconditioned (cycle of pain<>immobility), have myofascial pain, have increased ROM (often coupled with proximal and dysfunctional muscle spasming to stabilize), experience joint instability and pain, and have central sensitization of pain (due to chronic pain that has been untreated for an extended period of time).

TREATMENT

Start low and go slow. The hypermobile patient (with their faulty connective tissue) may come to you having been pushed too hard and advanced too quickly in PT in the past. “Start low and go slow” is our plan. Expect a non-linear progression. Patients have good days and bad often as a result of their comorbidities. This is NOT your typical orthopedic patient.

  1. Let go: START with breathing and softening of the body to minimize spasming (before you move to strengthening). Patients may have poor joint position sense and likely compensatory patterns. We need to fix body mechanics first before we can begin to strengthen.  

  2. Strengthening: Once the patient is ready for strengthening, make sure feet are aligned and well supported then begin with basic postural stability (TrA, glute med, scapular stabilizers, deep neck flexors…as long as activating them doesn’t provoke symptoms), followed by strengthening of muscles surrounding proximal joints. Always start supine until they can demonstrate pelvic stabilization.

  3. Soft tissue work: Release tight muscles (likely pectorals, upper traps, SCM/scalenes and hip flexors). Tissues will not release until they trust that the proper stabilizing muscles are strong enough to protect the joint. Soft tissue work without simultaneous strengthening is a waste of time. Consider myofascial work, gentle massage and rolling vs “origin to insertion” stretching that may put too much tension on a weak ligament, tendon or joint capsule.

  4. Functional movement: Observe the patient performing ADLs and correct compensations.

  5. Ergonomics: Patients are likely spending a significant amount of time trying to rest. Ask about and correct postures that are likely to worsen symptoms (bed, couch, work station/desk, driving, sleeping)

OTHER

  • Kinesiophobia: Patients are likely anticipating pain and may easily resort to compensatory patterns. They will need careful supervision to perform exercises accurately without resorting to tension and in a pain free range.

  • Spinal instability: Upper cervical instability is common so limit cervical mobilization and any positions that put the head/neck in a dependent position (plank, prone on ball, etc) until that has been ruled out. Tethered cord is also common. 

  • Joint instability: There is an increased tendency for subluxation/dislocation of ribs, shoulders, hips, ankles, fingers, jaw, intervertebral.

  • Exercise selection: Closed chain, safe ROM, slow with few reps, stay below level of flare, isometrics, building muscle endurance.

  • Exercise prescription: Consistency is more important than intensity (few exercises but performing them daily to build a routine), start exercises in supine to minimize orthostatic intolerance and compensatory patterns, request feedback and tolerance to HEP.

  • Treatment flow/positions: Limit position changes to limit orthostatic intolerance. Start supine. Avoid loading any joints at end range (e.g. quad on extended wrists, bridging with neck flexion, ¼ kneel with rotated pelvis.

  • Pacing: Teach patients to pace their activity to maximize function. Encourage “microbreaks.”

  • Holistic Health: Encourage healthy lifestyle practices in general in order to maximize potential for tissue regeneration and energy available for rehabilitation. Careful attention to: sleep, rest, healthy food, hydration, stress management, healthy relationships/connection, movement.

  • Bracing: Bracing of unstable joints can be considered to maximize positioning and functional movement during the strengthening phase of rehabilitation and to limit pain flares that may limit progress.

“Waiting for the pain to go before returning to physical activity is not going to work, because inactivity and abnormal movement are the two most important driving forces for the pain.”

- Drs. Hakim, Keer and Grahame, 2010

“When you can’t connect the issues, think connective tissues.” - unknown

“No other condition in the history of modern medicine has been neglected in such a way as Ehlers-Danlos Syndrome.”

- Professor Rodney Grahame