EHLERS-DANLOS SYNDROME PHYSICAL THERAPY AND...

PHYSICAL THERAPY AND EHLERS-DANLOS SYNDROME

From a Structural Approach to a Process Approach

Jan Dommerholt, PT, DPT, DAAPM

ONLY 10%of physicians referring EDS-HT patients to rheumatology clinics realized that their joint hypermobility was the underlying cause of their patient’s pain

Adib et al. Joint hypermobility syndrome in childhood. A not so benign multisystem disorder? Rheumatology. 2005

PHYSICAL THERAPY

A lack of awareness of the condition amongst health professionals, patients and wider societyPalmer S, Terry R, Rimes KA, Clark C, Simmonds J, Horwood J, Physiotherapy management of joint hypermobility syndrome - a focus group study of patient and health professional perspectives

Physiotherapy (2015) http://dx.doi.org/10.1016/j.physio.2015.05.001

SUBJECTIVE HEALTH COMPLAINTS

The complaints reported were Musculoskeletal (98%) Pseudoneurological (96%) Gastrointestinal (94%) Allergic (73%) and Influenza-like (58%)Maenad S, Assmus J & Berglund B. International Journal of Nursing Studies 48 (2011) 720–724

EDS-HT: A CHARACTERIZATION OF THE PATIENT’S LIVED EXPERIENCE

Joints (99%) Cardiovascular system (96%) Gastrointestinal system (96%) Skin (95%) Neurological/psychological manifestations (88%) Genitourinary system (67%)

Murray B et al. EDS-‐HT: A characterization of the patients’ lived experience. AJOMG. 2013

Physical therapy is generally accepted as an efficient treatment for some musculoskeletal

complications of EDS

Keer R, Simmonds J. 2011. Joint protection and physical rehabilitation of the adult with hypermobility syndrome. Curr Opin Rheumatol 23: 131–136

Physical therapy has limits especially concerning time spent (by both the practitioner and the

patient), inter-operator variability, and long-term efficacy

Castori M et al.2012. Management of pain and fatigue in the joint hypermobility syndrome (a.k.a. Ehlers–Danlos syndrome, hypermobility type): Principles and proposal for a multidisciplinary approach. Am J

Med Genet Part A 158A:2055–2070.

900 females and 46 males

81% (755/942) had received exercise advice from a physiotherapist

77% (701/940) agreed or strongly agreed that exercise was important for management

ATTITUDES, BELIEFS AND BEHAVIOURS TOWARDS EXERCISE AMONGST INDIVIDUALS WITH JOINT HYPERMOBILITY SYNDROME/

EHLERS DANLOS SYNDROME – HYPERMOBILITY TYPE J. Simmonds, M. Cairns, N. Ninis, W. Lever, Q. Aziz, A. Hakim

Swimming 28% (261/946)

Walking 24% (233/946) and

Pilates 22% (221/496)

ATTITUDES, BELIEFS AND BEHAVIOURS TOWARDS EXERCISE AMONGST INDIVIDUALS WITH JOINT HYPERMOBILITY SYNDROME/

EHLERS DANLOS SYNDROME – HYPERMOBILITY TYPE J. Simmonds, M. Cairns, N. Ninis, W. Lever, Q. Aziz, A. Hakim

Diagnosis and subsequent referral to physiotherapy services is often difficult and convoluted

Referral was often for acute single joint injury, failing to recognize the long-term multi-joint nature of the condition

PHYSICAL THERAPY

Palmer S, Terry R, Rimes KA, Clark C, Simmonds J, Horwood J, Physiotherapy management of joint hypermobility syndrome - a focus group study of patient and health professional perspectives


Health professionals and patients felt that if left undiagnosed, EDS was more difficult to treat because of its chronic nature

When EDS was treated by health professionals with knowledge of the condition, patients reported satisfactory outcomes

PHYSICAL THERAPY

Palmer S, Terry R, Rimes KA, Clark C, Simmonds J, Horwood J, Physiotherapy management of joint hypermobility syndrome - a focus group study of patient and health professional perspectives


ONLY ABOUT 4% OF PHYSICAL THERAPISTS ADMIT LIKING THE MANAGEMENT OF PATIENTS WITH PERSISTENT (CHRONIC)

PAIN

Wolff MS et al 1991 Physical Therapy 71:207-‐214

POSTURE CORRECTION

Perceived asymmetry, imbalances, misalignments, or postural deviations are usually normal biological variations and not pathology, even in many cases of EDS

The cause of many common musculoskeletal and pain complaints cannot be explained solely by biomechanics, structure or posture

LACK OF VALIDITY OF MOST COMMON ORTHOPEDIC TESTS

The majority of orthopedic tests used in physical therapy and medicine lack diagnostic validity, i.e.,

Most tests for the shoulder (gleno-humeral joint) lack sensitivity and specificity

There are no reliable tests for the position of the sacroiliac joint

The validity of a clinical test can be defined as the extent to which the test measures the intended construct

Symptoms of Pain Do Not Correlate with Rotator Cuff Tear Severity A Cross-‐Sectional Study of 393 Patients with a Symptomatic Atraumatic Full-‐

Thickness Rotator Cuff Tear Dunn et al. JBJS 2014

The anatomical findings are not associated with the level of pain

LACK OF VALIDITY OF SACRO-ILIAC JOINT TESTS

Tests for sacroiliac joint (SIJ) symmetry of motion and palpation fail to achieve meaningful reliability

Numerous studies have examined the validity of palpatory and motion symmetry tests and have consistently found it to be limited

Asymmetrical positions are likely to be the product of local muscular forces producing strain on the pelvis, which give the illusion of SIJ positional faults

LACK OF VALIDITY OF MOST COMMON ORTHOPEDIC TESTS

Most tests when considered independently lack sufficient diagnostic accuracy to determine the appropriate clinical course of action

Clustering of tests may provide improved diagnostic accuracy

Abnormal Findings in Asymptomatic Subjects

Of 1211 asymptomatic subjects in their 20s:

73.3% of males 78.0% of females

had bulging discs

Nakashima, H, Yukawa, Y, Suda, K, Yamagata, M, Ueta, T & Kato, F, 2015. Abnormal findings on magnetic resonance images of the cervical spines in 1211 asymptomatic subjects. Spine (Phila Pa 1976), 40, 392-398

JOINT MANIPULATION

Many of our patients with EDS-HT have received joint manipulations to “correct stiff spinal segments”

It is, however, questionable whether EDS-HT patients ever need spinal manipulations

NO HANDS-ON PHYSICAL THERAPY??

If the goal of hands-on physical therapy is to correct asymmetry, imbalances, misalignments, or postural deviations, its value is questionable

ERGONOMICS

The best ergonomic chair is only as good as the behavior of the person who sits in that chair

ERGONOMICS

Ergonomic modifications are often indicated, but they are not the silver bullet to solve all problems

RING SPLINTS

CHRONIC PAIN

Dutch study:

92% reported chronic pain

87% of those with pain were disabledVoermans et al: Pain in Ehlers-Danlos Syndrome is common, severe, and associated

with functional impairment. J Pain Symptom Manage. 2010 40(3):370-8

“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”

Maillard & Payne; Physiotherapy and occupational therapy in the hypermobile child. In: Hakim, Keer & Grahame: Hypermobility, Fibromyalgia and Chronic Pain. Churchill Livingstone, Elsevier, 2010

“…. the pain will ease but only when the muscles are strong and fit and are protecting the joints more fully, and when the child is functioning normally both biomechanically and generally….

…. the pain is the last thing to improve and only does so slowly…..

Maillard & Payne; Physiotherapy and occupational therapy in the hypermobile child. In: Hakim, Keer & Grahame: Hypermobility, Fibromyalgia and Chronic Pain. Churchill Livingstone, Elsevier, 2010

PHYSICAL THERAPY FOR PAIN MANAGEMENT

During a pain experience, multiple areas of the brain are activated at exactly the same time

The most common areas associated with the pain neuromatrix are the anterior cingulate, primary sensory cortex, thalamus, anterior insula, and the prefrontal and posterior parietal cortices

Louw A: Treating the Brain in Chronic Pain. In: Fernández de las Peñas, C, J. Cleland and J Dommerholt: Manual Therapy for Musculoskeletal Pain Syndromes – An Evidenced and Clinical-Informed Approach. Churchill Livingstone (Elsevier), 2016

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HOMUNCULUS


In chronic pain these areas of the brain are likely to be ‘enslaved’ by pain

Exercise or specific movements can be difficult, since the motor cortex is being utilized as part of the pain neuromatrix



Patients have been accused of malingering, or being lazy or not motivated

Yet from a neuroscience perspective there is a justified reason for their difficulty with these exercises



Traditionally, clinicians have either followed a top-down (cognitive) or a bottom-up approach (such as manual therapy) to treat pain The two approaches are not mutually exclusive and clinicians are therefore urged to consider a combination of the two


COMBINATION

TOP-DOWN: therapeutic neuroscience education (TNE)

BOTTOM-UP: manual therapy and exercise

HANDS-ON PHYSICAL THERAPY??

If the goal of hands-on physical therapy is to compliment “therapeutic neuroscience education”, hands-on therapy can be very useful

Manual therapy

Soft tissue mobilizations

Dry needling

Exercise

HANDS-ON PHYSICAL THERAPY??

Therapeutic neuroscience education works best if combined with movement-based therapies such as manual therapy and exercise

Feeding the brain information ‘from both ends’


WHAT DOES THE RESEARCH SAY ABOUT PT, EDS AND EXERCISE?

Not much…….

Rombaut et al. Arch Phys Med Rehabil 2011;92:1106-‐12

Positive outcome:

Only 33.9% of the patients who underwent surgery

Only 63.4% of patients in physical therapy

2001 titles were identified Four articles met the inclusion criteria, comprising one controlled trial, one comparative trial and two cohort studies All studies found clinical improvements over time

Physiotherapy. 2014;100(3):220-227

No convincing evidence that exercise was better than control or that joint-specific and generalized exercise differed in effectiveness

Physiotherapy. 2014;100(3):220-227

Internal Focus

vs

External Focus

COMMON PT HOME EXERCISE PROGRAM

AROM shld push-ups at wall

Stand facing wall, about 12-18 inches away

Place hands on wall at shoulder height

Slowly bend elbows, bringing face to wall

Push back to start position and repeat

Perform 1 set of 5 repetitions, twice a day

Perform 1 repetition every 4 seconds

COMMON PT HOME EXERCISE PROGRAM

AROM knee step ups

Stand with involved leg up on step

Shift weight over knee

Step up slowly

Step back down leading with involved leg

Repeat

Special Instructions:

Do not push off with trailing foot. This can be done by keeping ball of foot of the trailing foot lifted up.

Perform 1 set of 5 repetitions, twice a day

Perform 1 repetition every 4 seconds

Lie on your left side with your knees bent and place a ball between your knees

Press your left toes slightly into the wall

Inhale through your nose and gently slide your right leg forward without letting your trunk rotate forward

Exhale and gently push your left leg up into the ball

Inhale again and slide your right leg forward further

Exhale and squeeze your left leg into the ball

Repeat this sequence until you have taken a total of three breaths in and out

Boyle, KL, 2011. Managing a female patient with left low back pain and sacroiliac joint pain with therapeutic exercise: a case report. Physiother Can, 63, 154-163.

COMPLIANCE

Of 364 patients, 73% were referred to exercise rehabilitation following cardiac surgery, 42% did not complete the program and 28% never showed up

Laustsen et al: Predictors for not completing exercise-‐based rehabilitation following cardiac surgery. Scand Cardiovasc J. 2013;47(6):344-‐51

COMPLIANCE

Up to 70% of patients do not engage in prescribed home exercise

Beinart NA: Individual and intervention-‐related factors associated with adherence to home exercise in chronic low back pain: a systematic review. Spine J. 2013;13(12):1940-‐50

An apparent conflict existed between

offering a ‘specific’ exercise program based on physical impairments and pain patterns derived from assessment, and

empowering patients to take control by undertaking an exercise program they found fun or enjoyed

Physiotherapy (2015)

INTERNAL VS EXTERNAL

Internal focus:

“Exert force with outer foot”

External focus:

“Exert force on the outer part of the plate”

Control:

No focus instructions

Wulf, G., Höß, M., & Prinz, W. (1998). Instructions for motor learning: Differential effects of internal versus external focus of attention. Journal of Motor Behavior, 30, 169-179

Wulf, G., Höß, M., & Prinz, W. (1998). Instructions for motor learning: Differential effects of internal versus external focus of attention. Journal of Motor Behavior, 30, 169-179

From Wulf G: APTA Combined Sections Meeting 2014 - Las Vegas, NV From Wulf G: APTA Combined Sections Meeting 2014 - Las Vegas, NV

Wulf, G., Weigelt, M., Poulter, D.R., & McNevin, N.H. (2003). Attentional focus on supra-postural tasks affects balance learning. Quarterly Journal of Experimental Psychology, 56, 1191-1211

From Wulf G: APTA Combined Sections Meeting 2014 - Las Vegas, NV

Wulf, G., Weigelt, M., Poulter, D.R., & McNevin, N.H. (2003). Attentional focus on supra-postural tasks affects balance learning. Quarterly Journal of Experimental Psychology, 56, 1191-1211

Examples of supra-postural tasks

The focus of attention clearly has significant effects on the accuracy with which subjects can generate force and significant effects of motor planning in force production

Human Movement Science 31 (2012) 12–25

Training with an external focus of attention leads to improved performance when no attentional focus instructions are given


An external focus of attention increases the automaticity of control


Competitive cyclists performed significantly faster during a 16.1-km competitive trial than when performing maximally, without a competitor

The improvement in performance was elicited due to a greater external distraction, deterring perceived exertion

Journal of Science and Medicine in Sport 18 (2015) 486–491

The advantages of an external focus of attention may not be immediate, but often emerge only later in practice (but the research is not consistent….)


An external focus of attention does not only improve performance, but could improve learning as well


An internal focus of attention

can hinder learning

relative to an external focusHuman Movement Science 33 (2014) 120–134

APPLIES TO

Sports performance: Golf Basketball Volleyball Soccer Dart throwing American Football Jumping

APPLIES TO

Parkinson’s disease CVA (stroke) Autism Balance in older adults Pediatrics Multiple Sclerosis

Wulf, G., Landers, M., Lewthwaite, R., & Töllner, T. (2009). External focus instructions reduce postural instability in individuals with Parkinson disease.

Physical Therapy, 89, 162-168.

MULTIPLE SCLEROSIS

MS patients with balance problems

(1) conventional balance training (control),

(2) exergame training (playing exergames on an unstable platform)

(3) single-task (ST) exercises on the unstable platform

Playing exergames on an unstable surface had superior outcomes in balance and adherence

DOES IT WORK?

Ehlers Danlos Syndrome?? POTS??

There is no research yet, that demonstrates superior outcomes

From a Structural Approach to a Process Approach

DOES IT WORK?

Eileen - Ehlers Danlos Syndrome

DOES IT WORK?

Molly - Postural Orthostatic Tachycardia Syndrome

DOES IT WORK?

Molly - Postural Orthostatic Tachycardia Syndrome

DOES IT WORK?

Cassidy - Ehlers Danlos Syndrome and Postural Orthostatic Tachycardia Syndrome

THANKS TO

EILEEN MOLLY

CASSIDY DR. MARIA ARINI, PT, DPT

EDNF OUR OTHER PATIENTS

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