How Can Physical Therapy Help My EDS/HSD

Symptoms?Leslie Russek, PT, DPT, PhD, OCS

Clarkson University, Physical Therapy Dept.Canton-Potsdam Hospital, Physical Therapy Dept.

A Checklist of potential physical therapy interventions For EDS/HSD is available on my web page:

https://webspace.clarkson.edu/~Lrussek/hsd.html

1

Objectives1. List complaints PT may be able to address2. Outline a process for PT to evaluate you and

determine a plan of care3. Describe treatment approaches PT may use4. Describe the role of PT in long-term

management of EDS/HSD5. Explain how your PT should empower YOU to

manage your EDS/HSD signs and symptoms

2

Complaints PT may be able to address• Pain: localized or widespread, including headaches• Joint instability, subluxations or dislocations• Functional limitations: gross or fine motor• Developmental delay and clumsiness• Fatigue, sleep disturbance• Postural Orthostatic Tachycardia Syndrome (POTS)• Incontinence, vaginal or pelvic pain• (Anxiety and depression)• (Gastrointestinal problems, gastroparesis)• (Immune issues)

3

Why Physical Therapy?

4

• PTs are experts in the movement system• Some approaches are are best provided by PT:

o Exercise, neuromuscular re-education, body mechanics, posture, ergonomics, manual therapy, modalities, braces, assistive devices…

• Most PTs are skilled in:o Pain management, pain neuroscience educationo Application of behavioral approaches to functional

activities: e.g. pacing, sleep hygiene…• You may develop a strong therapeutic relationship

with your PT, discuss problems & solutions• Some PTs have advanced training: e.g., women’s

health, visceral mobilization, etc.

Complaints PT may be able to address• Pain: localized or widespread, including headaches• Joint instability, subluxations or dislocations• Functional limitations: gross or fine motor• Developmental delay and clumsiness• Fatigue, sleep disturbance• Postural Orthostatic Tachycardia Syndrome (POTS)• Incontinence, vaginal or pelvic pain• (Anxiety and depression)• (Gastrointestinal problems, gastroparesis)• (Immune issues)

5

Pain Management1. Assessment of pain type, source, perpetuation2. Education about prevention and management3. Exercise to improve quality of movement,

endurance, enhance natural pain-decreasing neural pathways

4. Manual therapy for alignment, tissue healing5. Taping/bracing/orthotics for alignment & quality

of movement6. Modalities for pain and inflammation

(Engelbert, 2017, Chopra, 2017)6

Pain Assessment• Use a biopsychosocial

approach• Look for contributing

factors as well as signs, symptoms, & involved tissues

7

CENTRAL SENSITIZATION: Central nervous system becomes hyper-responsive; widespread allodynia

Inflammation ➔ nociception and SENSITIZATION

DEEP SOMATIC NOCICEPTION: muscle/trigger points, ligament, tendon, bursae, fascia: referred pain, other symptoms; dull cramping or aching, poorly localized.

DEEP VISCERAL NOCICEPTION:Many ‘silent nociceptors’.

Types of Pain

NEUROGENIC INFLAMMATION

Biopsychosocial Pain Assessment• Identify causal, aggravating & perpetuating factorso Stressors affecting sensitive, stressed, or injured tissues

• (Nociception, inflammation)o Referred pain from musculoskeletal or visceral tissues

• (Nociception, inflammation, activation of silent nociceptors)

o Sensitive nerves, neurogenic inflammation, neuroplasticity• (Neuropathic pain, peripheral and central sensitization,

inflammation, neurogenic inflammation)

o Emotional, psychological and environmental factors• (Central sensitization, neurogenic inflammation)

9

Physical Stresses to Tissues1. Is there an imbalance between lax joints and tight

muscles? 2. Does poor posture, alignment or gravity stress

joints/muscles? 3. Are body mechanics stretching or stressing

joints/muscles?4. Is poor proprioception or motor control leading to

instability? 5. What is causing muscle trigger points?

10

Referred Pain

• Visceral and deep musculoskeletal tissues can refer pain

• Pain referral can irritate tissues at the referral site through neurogenic inflammation

• This can cause nociception and tissue damage at the referral site.

11

Visceral Referral Patterns

Trigger Point Referral Patterns

Referred Pain: Headaches

12

• Many headaches are caused by musculoskeletal problems in the neck or head

• Trigger points• Joint problems in the

neck• Temporomandibular

disorders(picture from Travell & Simons)

Neurological Changes

13

Neuroplasticity• Changes in brain structure or

connections due to painPeripheral & central sensitization: • Increased reactivity of neurons in

peripheral or central nervous system

• Occurs due to inflammation, psychological factors (e.g. stress or anxiety), being sedentary

Neurogenic inflammation• Sensory nerves can fire backwards

and release inflammatory chemicals at their peripheral receptors.

• Can cause nociception and tissue damage in those tissues

Picture: http://www.nationalpain.com/central-sensitization/

Psychosocial Aspects of Pain

14

The pain experience is affected by:• Physiologic response to actual

or potential tissue damage• Previous pain experience• Beliefs/attitudes about pain• Coping style• Emotions• Family, social, and cultural

background• Interaction between sensory

input and brain processing

http://www.painxchange.com.au/images/BiopsychosocialPain.png

Pain Management: Education

15

• Factors contributing to/perpetuating tissue stress• Preventing and managing nerve sensitivity

o Pain neuroscience education & “Explain Pain”

• Addressing psychosocial factors aggravating pain• Self-management using exercise, TENS, heat, ice,

topical rubs, relaxation, mindfulness meditation, etc.• Use of braces, splints, orthotics, assistive technology,

environmental modification

Pain Management: Exercise

16

• To address contributing factors: posture, muscle tightness/weakness, trigger points, coordination problems, non-muscle tissue weakness.

• Neuromuscular re-education for motor control training, muscle recruitment, balance, relaxation.

• Aerobic exercise to restore normal pain inhibitory pathways and improve endurance.

• Neuromuscular re-education and exercise to address kinesiophobia (fear of movement) (Kernan, 2007)

Pain Management: Exercise“Exercise-induced analgesia”• Regular exercise activates nerve pathways from the brain,

stopping nerves from transmitting nociceptive information • Improves descending pain control• Decreases hypersensitivity of nerves• This process does not work properly in people who

are deconditioned/sedentary • A single bout of exercise may increase pain• Regular exercise restores proper function of exercise-induced

analgesiaLima LV, Abner TSS, Sluka KA. Does exercise increase or decrease pain? Central mechanisms underlying these two phenomena. J Physiol. 2017;595(13):4141-4150.

17

Not All Exercises Are Appropriate

18

• For exercise to be helpful and not harmful, it must be:o The correct exercise (for you, now)o Done correctly (proper motor control)

o At the correct dose (intensity, time/reps)o Not overstressing other joints or muscles

• There is no protocol appropriate for everyone with EDS/HSD

Doing Exercises Correctly

19

• External feedback seems to improve accuracy, learning and retention compared to internal feedback. (Lauber, 2014; Lohse, 2014)

• Using laser targets, biofeedback, etc.

• See Jan Dommerholt’s presentation

www.optp.com/SenMoCOR-System

www.optp.com/STABILIZER-Pressure-Biofeedback?kw=stabilizerwww.amazon.com

Pain Management: Manual Therapy

20

• Goals:o Decrease spasm, trigger points, adhesions

o Restore proper tissue alignment and mobility

o Calm peripheral or central nervous systemo Decrease pain

• Options: massage, trigger point release, soft tissue mobilization, myofascial release, joint mobilizations, instrument-assisted, dry needling

Pain Management: Taping

21

Kinesio/Rock taping

• Improve tissue alignment, position sense, stability, fluid movement in tissues

McConnell taping• Improve tissue

alignment, stability, proprioception

http://www.ktss.us/what

https://americanpostureinstitute.com/the-ultimate-guide-to-posture-taping/

http://www.beantownphysio.com/pt-tip/archive/mcconnell-taping.html

Pain Management: Modalities

22

• Options: Heat, ice, TENS (conventional or acupuncture-like), ultrasound/phonophoresis, laser, iontophoresis, shock-wave therapy, etc.

• Can be helpful to temporarily decrease pain or inflammation, or improve tissue mobility

• Benefit is not maintained unless tissues are used during the period of benefit, through active interventions such as exercise or function

• Regular home use of heat, ice, TENS may decrease pain enough to improve function and exercise tolerance

Pain Management Patient Resources

23

• 5-minute video about chronic pain neuroscience: https://www.youtube.com/watch?v=RWMKucuejIs

• UC Davis pamphlet: search “U.C. Davis pain self-management plan” has multiple modules that let you pick what area might be most helpful for you now.

• American Chronic Pain Association: https://www.theacpa.org

• On-line pain self-management: https://www.liveplanbe.ca has questionnaires to customize information and suggestions for issues that you face

Complaints PT may be able to address• Pain: localized or widespread, including headaches• Joint instability, subluxations or dislocations• Functional limitations: gross or fine motor• Developmental delay and clumsiness• Fatigue, sleep disturbance• Postural Orthostatic Tachycardia Syndrome (POTS)• Incontinence, vaginal or pelvic pain• (Anxiety and depression)• (Gastrointestinal problems, gastroparesis)• (Immune issues)

24

Joint Instability• Potential causes:

o Joint laxityo Traumao Muscle weakness, poor motor controlo Poor position sense or body awarenesso Excessive stress from tight muscleso Excessive stress from habits, postures or

activities◦ (but stresses may be really mild)

25

Cartoon from: https://darrengoossens.wordpress.com/category/comic/page/8/

Joint Instability: Education• Address contributing factors: posture, body

mechanics, ergonomics, joint protection• Use of braces, splints, taping, assistive

technology, environmental modification.o Generally recommended for acute flares, return to

function, controversial for long-term use(Engelbert, 2017)

26

Joint Instability: Neuromuscular Re-ed

27

• Improve body awareness (Engelbert, 2017)

o Intrinsic feedback: proprioception, body awareness, tactile feedback (compressive clothing or taping)

o External/augmented feedback: laser, pressure biofeedback, wobble boards, virtual reality, Wii

◦ External focus seems to result in better retention and transfer of motor skills than internal focus

o (Lauber, 2014; Lohse, 2014)

Complaints PT may be able to address• Pain: localized or widespread, including headaches• Joint instability, subluxations or dislocations• Functional limitations: gross or fine motor• Developmental delay and clumsiness• Fatigue, sleep disturbance• Postural Orthostatic Tachycardia Syndrome (POTS)• Incontinence, vaginal or pelvic pain• (Anxiety and depression)• (Gastrointestinal problems, gastroparesis)• (Immune issues)

28

Decreased Function: Manage Pain• Assessment of pain type, source, contributing

factors• Education about:

o Musculoskeletal, neurological, and psychosocial contributing factors

o Self-management of paino Orthotics, braces, environmental modifications

• Exercise to address contributing factors, motor control/coordination, aerobic conditioning

• Manual therapy, taping, modalities29

Decreased Function: Fear of Movement

30

• People with EDS/HSD may be afraid of movement (kinesiophobia) due to fear of pain, injury or instability

• Decreased activity leads to decreased muscle tone, aerobic capacity, and strength, making pain and injury more likely

• Fear of movement is also linked to fatigue, perhaps through decreased activity and deconditioning. (Celletti, 2013)

• Fear of movement is a common reason people with EDS/HSD do not exercise. (Simmonds, 2017)

Decreased Function: Fear of Movement

31

• PT should address this fear through gradually progressed activity and exerciseo Best if integrated with a behavioral approach

• PT should not aggravate this fear by increasing pain (Perrot, 2018)

• Good communication and partnership with a PT knowledgeable about EDS/HSD can help patients exercise more successfully (Simmonds, 2017)

Complaints PT may be able to address• Pain: localized or widespread, including headaches• Joint instability, subluxations or dislocations• Functional limitations: gross or fine motor• Developmental delay and clumsiness• Fatigue, sleep disturbance• Postural Orthostatic Tachycardia Syndrome (POTS)• Incontinence, vaginal or pelvic pain• (Anxiety and depression)• (Gastrointestinal problems, gastroparesis)• (Immune issues)

32

Developmental Delay in Children• Education about contributing factors • Neuromuscular re-education (as for joint

instability)• Therapeutic exercise for functional stability,

especially in mid-range (but not excluding end range)

• Orthotics, braces, environmental adaptations(Engelbert, 2017)

33http://www.otforkids.co.uk/conditions/developmental-delay.php

Complaints PT may be able to address• Pain: localized or widespread, including headaches• Joint instability, subluxations or dislocations• Functional limitations: gross or fine motor• Developmental delay and clumsiness• Fatigue, sleep disturbance• Postural Orthostatic Tachycardia Syndrome

(POTS)• Incontinence, vaginal or pelvic pain• (Anxiety and depression)• (Gastrointestinal problems, gastroparesis)• (Immune issues)

34

Fatigue and Sleep Dysfunction• Assess reasons for fatigue

o Sleep disturbance due to: ◦ Pain, anxiety, poor sleep hygiene, apnea

o Deconditioning, sedentary lifestyleo Trying to do too much, not pacing, boom/bust cycleso Stress, not being able to relax and rechargeo Autonomic disorder such as POTS or orthostatic

tachycardiao Psychological factors such as depression, grief, etc.o Dieto Medicationso Other medical conditions: MCAD, fibromyalgia, etc.

o (Hakim, 2017)35

Fatigue: Managing Poor Quality Sleep

36

• Pain interfering with sleepo Positioning for decreased paino General pain management

• Sleep hygiene

• Physiological quieting, relaxation training• Regular exercise• Good information at https://sleep.org

Fatigue: Education

37

• Pacing• Body mechanics• Assistive technology and environmental

modifications

• Excellent fatigue self-management booklet at: https://www.ncl.ac.uk/medicalsciences/research/centres/fatigue/ “CRESTA Fatigue Clinic -Managing Your Energy”

Fatigue: Managing POTS

38

• Education about: Hakim, 2017o Common triggerso Fluids, salt, compression stockingso Postural changes, muscle activationo Exercise positioning and progressiono Pacingo Adapting tasks

• Exercise: o Progression from horizontal to verticalo Graded exercise therapy

• See WWW.POTSUK.ORG for excellent info

Fatigue: Graded Exercise Therapy

39

• Many people try to do too much too fast: “Start low, go slow”• Stabilize your daily routine• Start easy, e.g., muscle stretches or relaxation exercises• Select an activity/exercise you enjoy and will do consistently

o Set a baseline that you can do 5d/wk, even on bad dayso Rest after exercise, sitting, not lying down, <30 minutes

• Increase time gradually – no more than 20%/wk• Increase intensity once you can do 30 min/day• Plan for setbacks• Source: “Graded Exercise Therapy: A self-help guide for

those with chronic fatigue syndrome/myalgicencephalomyelitis.”

Complaints PT may be able to address• Pain: localized or widespread, including headaches• Joint instability, subluxations or dislocations• Functional limitations: gross or fine motor• Developmental delay and clumsiness• Fatigue, sleep disturbance• Postural Orthostatic Tachycardia Syndrome (POTS)• Incontinence, vaginal or pelvic pain• (Anxiety and depression)• (Gastrointestinal problems, gastroparesis)• (Immune issues)

40

Incontinence• Education about voiding, fluid management,

urge inhibition, nocturia control• Pelvic floor muscle retraining, including

biofeedback

• Manual therapy to low back/pelvis/hip• TENS protocol for incontinence

(Neville, 2016)

• May need an women’s/men’s health specialist

41

Vaginal Pain• Education about self-management• Pelvic floor muscle retraining, including

biofeedback• Electrical stimulation

• Dilators(Morin, 2017)

• May need a women’s health specialist

42

Complaints PT may be able to address• Pain: localized or widespread, including headaches• Joint instability, subluxations or dislocations• Functional limitations: gross or fine motor• Developmental delay and clumsiness• Fatigue, sleep disturbance• Postural Orthostatic Tachycardia Syndrome (POTS)• Incontinence, vaginal or pelvic pain• (Anxiety and depression)• (Gastrointestinal problems, gastroparesis)• (Immune issues)

43

Anxiety• Education about EDS/HSD• Assessment for POTS, education about POTS

self-management• Neuromuscular re-education & exerciseo To decrease fear of movement (Kernan, 2007)

o To calm the nervous system• Exercise: stretching muscles, relaxation, aerobico Encourage Tai Chi, qigung, Pilates, (yoga), etc.

• Manual therapy, massage (Pederson, 2018)

44

Gastroparesis/Constipation• Education about

gastroparesis in EDS/HSD• Aerobic exercise• Trigger point management

through self-care or manual therapy?

• Abdominal propulsive massage?

Harrington & Haskvitz, Phys Ther. 2006;86:1511-19)

• Visceral mobilization (additional training needed)

45

Inflammation• Education about inflammation, neurogenic

inflammation, role of stress• Education about Mast Cell Activation Disorder• Exercise (regular aerobic exercise stimulates

immune function; Abd El-Kader, 2018)• Modalities to decrease localized inflammation

during flares: ice, non-thermal ultrasound, phonophoresis, iontophoresis

46

Summary

47

• Physical Therapy can address a range of concerns that are common in EDS/HSD

• There are a variety of treatment approacheso Education emphasizing self-managemento Exercise, neuromuscular re-educationo Orthotics/bracing/adaptive equipmento Manual therapyo Modalities

• Exercises must be the correct ones for you, done correctly, in the correct dosage

• Some approaches require specialized training

Your PT should empower

YOU to manage your

EDS/HSD signs and symptoms

References

49

1 . A b d E l - K a d e r S M , A l - S h r e e f F M . I n f l a m m a t o r y c y t o k i n e s a n d i m m u n e s y s t e m m o d u l a t i o n b y a e r o b i c v e r s u s r e s i s t e d e x e r c i s e t r a i n i n g f o r e l d e r l y . A f r H e a l t h S c i . 2 0 1 8 ; 1 8 ( 1 ) : 1 2 0 - 1 3 1 .

2 . C e l l e t t i C , C a s t o r i M , L a T o r r e G , C a m e r o t a F . E v a l u a t i o n o f k i n e s i o p h o b i a a n d i t s c o r r e l a t i o n s w i t h p a i n a n d f a t i g u e i n j o i n t h y p e r m o b i l i t y s y n d r o m e / E h l e r s -D a n l o s s y n d r o m e h y p e r m o b i l i t y t y p e . B i o m e d R e s I n t . 2 0 1 3 ; 2 0 1 3 : 5 8 0 4 6 0 .

3 . C h o p r a P , T i n k l e B , H a m o n e t C , e t a l . P a i n m a n a g e m e n t i n t h e E h l e r s - D a n l o s s y n d r o m e s . A m J M e d G e n e t C S e m i n M e d G e n e t . 2 0 1 7 ; 1 7 5 ( 1 ) : 2 1 2 - 2 1 9 .

4 . E n g e l b e r t R H H , J u u l - K r i s t e n s e n B , P a c e y V , e t a l . T h e E v i d e n c e - b a s e d r a t i o n a l e f o r p h y s i c a l t h e r a p y t r e a t m e n t o f c h i l d r e n , a d o l e s c e n t s a n d a d u l t s d i a g n o s e d w i t h j o i n t h y p e r m o b i l i t y s y n d r o m e / h y p e r m o b i l e E h l e r s D a n l o s S y n d r o m e . A m J M e d G e n e t C S e m i n M e d G e n e t . 2 0 1 7 ; 1 7 5 ( 1 ) : 1 5 8 - 1 6 7 .

5 . F o s s o C L , Q u i g l e y E M M . A C r i t i c a l R e v i e w o f t h e C u r r e n t C l i n i c a l L a n d s c a p e o f G a s t r o p a r e s i s . G a s t r o e n t e r o l H e p a t o l ( N Y ) . 2 0 1 8 ; 1 4 ( 3 ) : 1 4 0 - 1 4 5 .

6 . H a k i m A , D e W a n d e l e I , O ' C a l l a g h a n C , P o c i n k i A , R o w e P . C h r o n i c f a t i g u e i n E h l e r s - D a n l o s s y n d r o m e - H y p e r m o b i l e t y p e . A m J M e d G e n e t C S e m i n M e d G e n e t . 2 0 1 7 ; 1 7 5 ( 1 ) : 1 7 5 - 1 8 0 .

7 . H a k i m A , O ' C a l l a g h a n C , D e W a n d e l e I , S t i l e s L , P o c i n k i A , R o w e P . C a r d i o v a s c u l a r a u t o n o m i c d y s f u n c t i o n i n E h l e r s - D a n l o s s y n d r o m e - H y p e r m o b i l e t y p e . A m J M e d G e n e t C S e m i n M e d G e n e t . 2 0 1 7 ; 1 7 5 ( 1 ) : 1 6 8 - 1 7 4 .

8 . H a r r i n g t o n K L , H a s k v i t z E M . M a n a g i n g a p a t i e n t ' s c o n s t i p a t i o n w i t h p h y s i c a l t h e r a p y . P h y s T h e r . 2 0 0 6 ; 8 6 ( 1 1 ) : 1 5 1 1 - 1 5 1 9 .

9 . K e r n a n T , R a i n v i l l e J . O b s e r v e d o u t c o m e s a s s o c i a t e d w i t h a q u o t a - b a s e d e x e r c i s e a p p r o a c h o n m e a s u r e s o f k i n e s i o p h o b i a i n p a t i e n t s w i t h c h r o n i c l o w b a c k p a i n . J O r t h o p S p o r t s P h y s T h e r . 2 0 0 7 ; 3 7 ( 1 1 ) : 6 7 9 - 6 8 7 .

1 0 . L o h s e K R , J o n e s M , H e a l y A F , S h e r w o o d D E . T h e r o l e o f a t t e n t i o n i n m o t o r c o n t r o l . J E x p P s y c h o l G e n . 2 0 1 4 ; 1 4 3 ( 2 ) : 9 3 0 - 9 4 8 .

1 1 . M o r i n M , C a r r o l l M S , B e r g e r o n S . S y s t e m a t i c R e v i e w o f t h e E f f e c t i v e n e s s o f P h y s i c a l T h e r a p y M o d a l i t i e s i n W o m e n W i t h P r o v o k e d V e s t i b u l o d y n i a . S e x M e d R e v . 2 0 1 7 ; 5 ( 3 ) : 2 9 5 - 3 2 2 .

1 2 . N e v i l l e C E , B e n e c i u k J , B i s h o p M , A l a p p a t t u M . A n a l y s i s o f P h y s i c a l T h e r a p y I n t e r v e n t i o n O u t c o m e s f o r U r i n a r y I n c o n t i n e n c e i n W o m e n O l d e r T h a n 6 5 Y e a r s i n O u t p a t i e n t C l i n i c a l S e t t i n g s . T o p G e r i a t r R e h a b i l . 2 0 1 6 ; 3 2 ( 4 ) : 2 5 1 - 2 5 7 .

1 3 . P e d e r s e n K , B j o r k h e m - B e r g m a n L . T a c t i l e m a s s a g e r e d u c e s r e s c u e d o s e s f o r p a i n a n d a n x i e t y : a n o b s e r v a t i o n a l s t u d y . B M J S u p p o r t P a l l i a t C a r e . 2 0 1 8 ; 8 ( 1 ) : 3 0 - 3 3 .

1 4 . P e r r o t S , T r o u v i n A P , R o n d e a u V , e t a l . K i n e s i o p h o b i a a n d p h y s i c a l t h e r a p y - r e l a t e d p a i n i n m u s c u l o s k e l e t a l p a i n : A n a t i o n a l m u l t i c e n t e r c o h o r t s t u d y o n p a t i e n t s a n d t h e i r g e n e r a l p h y s i c i a n s . J o i n t B o n e S p i n e . 2 0 1 8 ; 8 5 ( 1 ) : 1 0 1 - 1 0 7 .

1 5 . S i m m o n d s J V , H e r b l a n d A , H a k i m A , e t a l . E x e r c i s e b e l i e f s a n d b e h a v i o u r s o f i n d i v i d u a l s w i t h J o i n t H y p e r m o b i l i t y s y n d r o m e / E h l e r s - D a n l o s s y n d r o m e -h y p e r m o b i l i t y t y p e . D i s a b i l R e h a b i l . 2 0 1 7 : 1 - 1 1 .

50