Physical Therapy for Hypermobility 2/23/18 Spectrum Disorder

A Zebra Among Us: Disclosures Recognition & Management of The speakers have no financial or other conflicts of interest. Hypermobility Spectrum Disorders Combined Sections Meeting 2018 New Orleans, LA, February 21-24, 2018 Leslie N Russek, PT, DPT, PhD, OCS Clarkson University, Canton-Potsdam Hospital, Potsdam, NY Stephanie Sabo PT, MPT Cincinna: Children’s Hospital Medical Center, Cincinna:, OH Jane Simmonds DProf, MCSP, MACP, SFHEA Great Ormond Ins:tute of Child Health, University College London, London, UK Heather Purdin, MS, PT, CMPT Good Health Physical Therapy & Wellness, Portland, OR

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Affiliations Learning Objectives At the end of this course, parDcipants will be able to: 1. Describe typical clinical presenta:on of pa:ents with hypermobility spectrum disorder (HSD) through the lifespan, including pediatric, adolescent, and adult pa:ents within the context of the ICF model. [email protected] [email protected] 2. Apply the 2017 Interna:onal Classifica:on to iden:fy Hypermobility Spectrum Disorders and hEDS. 3. Propose evidence-based approaches for physical therapy management for individuals with hypermobility spectrum disorders. 4. Recognize common challenges and pi]alls working with these complex [email protected] [email protected] pa:ents.

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Outline Many Types of EDS • Hypermobile (III): Loose joints, joint pain. Most common. • 25 min: Introduc:on to EDS, hEDS, HSD (L Russek) • 25 min: Pediatric case (Stephanie Sabo, presented by L. Russek) • Classical (I & II): Velvety, stretchy, fragile skin. Common. – Exam, evalua:on, motor delays, motor control issues, bracing • Vascular (IV): Possible arterial/organ rupture. Most serious. • 25 min: Adolescent case (Jane Simmonds) • Kyphoscoliosis: Joint laxity, muscle hypotonia, – Exam, evalua:on, stra:fied management, POTS, fa:gue, GI problems, developmental delay. Severe functional loss over time. psychosocial issues • 25 min: Adult case (Heather Purdin) • Arthrochalasia (VII): Congenital hip dislocation, lax joints. – Exam, evalua:on, chronic pain, MCAD, • Dermatosparaxis (VII): Severe skin fragility & bruising. • 20 min: QA (panel) • Malfait, 2017

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Classical Ehlers-Danlos Vascular Ehlers-Danlos Syndrome Syndrome

www.ehlersdanlosnetwork.org/vascular.html PT for Hypermobility Spectrum Disorders 7 PT for Hypermobility Spectrum Disorders 8

Many Names of Hypermobility Hypermobility Spectrum Disorder • Generalized joint hypermobility/laxity (GJH) Asymptomatic Mild Symptoms Severe Symptoms – Might or might not have pain or symptoms • Joint hypermobility syndrome (JHS) & hypermobility syndrome Localized-HSD Generalized-HSD (HMS): terms oden used by rheumatologists Peripheral-HSD Individual Historical-HSD EDS • Ehlers-Danlos Syndrome – hypermobility type (EDS – HT or type III) Joint Laxity terminology used by gene:cists

• Newly proposed terminology hypermobile Ehlers-Danlos Syndrome hEDS (hEDS) and Hypermobility Spectrum Disorder (HSD) GJH

• We will refer to it as ‘hypermobility’ or ‘HSD’ GJH = (asymptomatic) joint hypermobility; EDS = Ehlers-Danlos Syndrome; hEDS = hypermobile EDS Castori, 2017 PT for Hypermobility Spectrum Disorders 9 PT for Hypermobility Spectrum Disorders 10

Prevalence of EDS Pathophysiology • HSD is the most common systemic inherited connec:ve :ssue disorder in humans (Tinkle, 2017) • Autosomal dominant connec:ve :ssue disorder – in the UK prevalence of HSD associated with chronic widespread pain • There is no gene:c marker known for hEDS or severely disabling pain (Mulvey, 2013) is almost as high as fibromyalgia (Fayaz, 2016) and 100 :mes higher than rheumatoid arthri:s.(Humphreys, 2013) – In contrast to most of the other forms of EDS • Affects ~10 million people in the U.S. (Tinkle, 2017) • Heterogeneous pathophysiology – 30x number of THA/yr; 60x number of ACL reconstruc:ons/yr – Tenascin X abnormality? • HSD in musculoskeletal healthcare: 30-55% – Type III collagen abnormality? • In Omani women, probably not this high in the US. (Clark, 2011) – • 80-90% of all EDS is hEDS (Tinkle, 2017) Exact connec:ve :ssue abnormality is unknown – (Tinkle, 2017; Malfait, 2017)

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Common Signs & Symptoms Major Comorbidities • Musculoskeletal: joint hypermobility, subluxations/dislocations, sprains, • Chronic pain: fibromyalgia, myofascial pain, OA, TMD muscle spasm, TMD, flat feet, finger deformities, arthralgia, myalgia, – Hyperalgesia (Scheper, 2017) fractures, pain, proprioceptive deficits, kinesiophobia • Developmental delay in children • Integumentary: stretchy skin, easy bruising, atrophic scarring, poor wound • Dysautonomia: POTS, thermoregulation, gut, sexual healing, frequent hernias dysfunction • Cardiovascular: dysautonomia, postural orthostatic tachycardia syndrome • Mast Cell Activation Disorder: systemic inflammation Other: gastritis, IBS, incontinence, developmental delay, poor coordination, anxiety, mast cell activation, organ prolapse • Gastrointestinal disorders: GERD, IBS, malabsorption syndrome (Tinkle, 2017) • Disability due to pain, fatigue, anxiety, depression (Castori, 2011; Colombi, 2015; Scheper, 2016; Tinkle, 2017) • Tethered cord syndrome (Henderson, 2017)

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HSD Through the Life Span Evolving Diagnostic Criteria 1. Hypermobile phase 3. Stiffness phase – Hypermobile joints – Widespread pain • Un:l 2017: – Clumsiness/motor delay – Fatigue – Beighton Scale (Beighton, 1973) most oden used for GJH – Constipation/diarrhea – Tendinosis/tendon rupture • Carter-Wilkinson (Carter, 1964) – Abdominal hernias – Chronic gastritis • Rotés/Hospital del Mar (Bulbena, 1992) 2. Pain phase – Stiffness – Chronic musculoskeletal pain – (Castori, 2011; Tinkle, 2017) – Villafranche ClassificaDon used mostly by gene:cists for children: EDS-HT – Strains, sprains, dislocations (Beighton, 1998) – Unrefreshing sleep – Brighton Criteria used mostly by rheumatologists for adults: JHS – Chronic fatigue (Grahame, 1998) – Memory/cognitive problems – Gastric reflux, abdominal pain • New criteria for hEDS: “The 2017 international classification of the Ehlers- – Paresthesias Danlos syndromes.” (Malfait, 2017) – Tachycardia – Incontinence/UTI • No clear guidelines regarding HSD; Must exclude other poten:al diagnoses

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2017 hEDS Diagnostic Criteria 1: Generalized Joint Hypermobility

Must meet all 3 criteria: 1. Generalized joint hypermobility Beighton Score ≥5/9 (Malfait, 2017; diagram from Juul-Kristensen) • 2: Bend 5th finger back >90° 2. Features of heritable connec:ve :ssue disorder • 2: Touch thumb to forearm Pts with limited ROM for cause, add 1 point if ≥2/5 on the 5-Item Questionnaire: • 2: Elbow hyperextension >10° 1. Can you now (or could you ever) place your hands flat on the floor without • Must meet ≥ 2 of 3 categories, A-C • 2: Knee hyperextension >10° bending your knees? • 1: Palms to floor, knees straight 2. Can you now (or could you ever) bend your thumb to touch your forearm? 3. As a child, did you amuse your friends by contorting your body into strange shapes or could you do the splits? 3. Absence of exclusion criteria • Prepubescent ≥6/9 4. As a child or teenager, did your shoulder or kneecap dislocate on more than • Pubescent-50 years ≥5/9 one occasion? • (Malfait, 2017) • Over 50 years ≥4/9 5. Do you consider yourself “double-jointed”?

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2: Features of Heritable Connective 2A: Systemic Manifestations Tissue Disorder i. Unusually sod/velvety skin ii. Mild skin hyperextensibility (forearm) • Must have ≥ 2 of the following 3 categories: iii. Unexplained straiae/stretch marks iv. Bilateral papules of heel A. Systemic manifesta:ons v. Recurrent/mul:ple abdominal hernias • ≥5 of 12 op:ons vi. Atrophic scarring in ≥ 2 sites vii. Pelvic floor, rectal, uterine prolapse B. Family history viii. Dental crowding or high, narrow palate ix. Arachnodactyly bilateral Steinberg or Walker sign C. Musculoskeletal complica:ons x. Arm span/height ≥ 1.05 • ≥1 of 3 op:ons xi. Mitral valve prolapse mild or greater xii. Aor:c root dila:on Meets Systemic ManifestaDons if YES to ≥ 5 items

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2A: Systemic Manifestations 2A: Systemic Manifestations

i. Unusually sod or velvety skin iv. Bilateral piezogenic papules of heel * ii. Mild skin hyperextensibility (>1.5 cm on volar, non- dominant forearm) v. Recurrent or mul:ple abdominal hernias (umbilical, iii. Unexplained straiae/stretch marks in any or inguinal, crural; not hiatal hernia) prepubertal w/o significant weight change *Piezogenic papules are sub- cutaneous fat herniations through the fascia; they may appear as white nodules only with weight bearing Malfait, 2017

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2A: Systemic Manifestations 2A: Systemic Manifestations Hypermobile EDS Classical EDS vi. Atrophic scarring involving vii. Pelvic floor, rectal, and/or uterine prolapse in at least 2 sites (not like children, , nulliparous w/o morbid obesity classical EDS) viii. Dental crowding and high or narrow palate ix. Bilateral arachnodactyly – Bilateral Steinberg OR bilateral Walker sign

Hypermobile EDS, Castori, 2015 Malfait, 2017 PT for Hypermobility Spectrum Disorders 23 PT for Hypermobility Spectrum Disorders 24

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2A: Systemic Manifestations 2B: Family History x. Arm span/height* ≥ 1.05 • 1st degree rela:ve independently meets xi. Mitral valve prolapse mild or greater diagnos:c criteria for hEDS xii. Aor:c root dila:on, z-score >+2

*Arm span is from tip of middle finger to tip of middle finger

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2: Features of Heritable Connective 2C: Musculoskeletal Complications Tissue Disorder 1. Pain ≥ 2 limbs, recurring daily for at least 3 months 2. Chronic widespread pain for ≥ 3 months • In summary, to meet the criterion: Features of 3. Recurrent joint disloca:ons or frank joint instability in absence of trauma (a or b) heritable connecDve Dssue disorder a. 3+ atrauma:c disloca:ons of same joint OR 2+ disloca:ons of 2 different joints at different :mes • Must have ≥ 2 of the following 3 categories: b. Medical confirma:on of joint instability at 2+ joints not related to A. Systemic manifesta:ons trauma B. Family history • If yes to ≥ 1 item (and not due to other connecDve Dssue disorder, e.g. lupus, RA), then posiDve for musculoskeletal complicaDons C. Musculoskeletal complica:ons (not explained by another connec:ve :ssue disorder)

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3: Absence of Exclusion Criteria Patient Examination

To meet this Criterion, all 3 of the following must be ABSENT: • Use a biopsychosocial 1. Unusual skin fragility (should prompt considera:on of other types of EDS) approach 2. Other heritable or acquired connec:ve :ssue disorder (e.g, • Look for contribu:ng lupus or RA) factors as well as 3. Neuromuscular disorders that may cause joint hypermobility signs, symptoms, & by means of hypotonia or connec:ve :ssue laxity (e.g., involved :ssues Marfan, other EDS, OI, etc.)

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International Classification of Functioning Disability and Health

Engelbert , 2017; Pacey, 2014 PhD thesis

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Fatigue Pain Dysautonomia Where To Start? Focus on patient’s primary activity & participation Neuromusculoskeletal Cardiovascular restrictions Engelbert, 2017

Symptom Profile • Start at the biggest complaint & work toward smaller issues Ninis, 2015 • Look for the key structures/problems that are causing the Urogenital Gastrointestinal pa:ent’s deficits Psychological

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Principles of the Subjective Exam Outcome Measures • Thorough, biopsychosocial interview • Bristol Impact of • Quality of life assessment Hypermobility • Psychosocial assessment tools as indicated (e.g., – The only condi:on-specific depression, anxiety, etc.) – 55 ques:ons • Iden:fy habits and lifestyle choices that contribute to – ~10 min to complete primary complaints – Validated – E.g., sleep hygiene, postures, ac:vi:es – Reliability, MCID not yet • What has helped/harmed in the past determined – Avoid iatrogenic injuries (Bovet, 2012) – (Palmer, 2017)

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What is Different in HSD? Principles of the Physical Exam • Tissues in HSD are more fragile (Tinkle, 2017) • May be damaged by more subtle stresses or contribu:ng factors • Iden:fy :ssues causing symptoms (“the princess and the pea”) • Iden:fy stressors affec:ng those :ssues – (Tinkle, 2017) Are slower to heal 1. Is there an imbalance between lax joints and :ght muscles? • Gravity can have a big impact 2. Does poor posture, alignment or gravity stress joints/muscles? • Abnormal pain processing/hyperalgesia (Chopra, 2017; Scheper, 2017) • Mul:ple comorbidi:es can compound problems 3. Are body mechanics stretching or stressing joints/muscles? – E.g., Poor coordina:on microtrauma 4. Is poor propriocep:on or motor control leading to instability? – Fa:gue or pain decondi:oning 5. What is causing muscle trigger points? – POTS: anxiety muscle trigger points • POTS test, if appropriate – MCAD excessive inflammatory response

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Interventions Patient Education Educate and empower the paDent/family • PaDent educaDon !!! • Pain educa:on & self-management • Body mechanics/ergonomics • Exercise (appropriate) • Ortho:cs, braces, & splints if/when needed • Appropriate exercise/ac:vity • Pain management, focusing on self-care • Sleep hygiene & fa:gue management • POTS self-management • Assis:ve and ortho:c devices? • Psychological & social wellness, relaxa:on strategies • Diet and fluid management • Manual therapy? • Other issues: GI dysfunc:on, MCAD, incon:nence, etc. (Engelbert, 2017; Clinical Guidelines; Chopra, 2017) Refer to other professionals as appropriate (Engelbert, 2017; Chopra, 2017)

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Exercise Prescription Pain Management (in Clinic)

• Propriocep:on, stabiliza:on, motor control & • Physiological quie:ng coordina:on • Biofeedback • Strengthening • TENS (trial for home device) • Appropriate stretching, stabilizing as needed • Manual therapy • Cardiovascular condi:oning • Dry needling (if legal in your state) – “Graded Exercise Therapy” • Shi< to pa=ent self-management • (POTS-specific exercise) (Engelbert, 2017; Palmer, 2014) – Avoid extensive use of modali=es in the clinic (Chopra, 2017; Engelbert, 2017)

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Medications Barriers To Treatment

• Li|le defini:ve research evidence for medica:ons • Nega:ve past experiences with PT (Bovet, 2016) • NSAIDs for true inflamma:on – NSAIDs may slow :ssue healing, aggravate GI symptoms • Iatrogenic injuries (Bovet, 2016) • Tricyclics, an:-seizure, SNRI meds for neuropathic pain • (Simmonds, 2017) • Topical analgesics and an:-inflammatory medica:ons Barriers to doing exercise: • Acetaminophen – Fear • Cau:ons: – Pain – Opiates for short term use only – Fa:gue – Muscle relaxers may aggravate instability Chopra, 2017 Tinkle, 2017

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Applying Evidence-Based Review Practice • Although more research is • Hypermobility-related complaints are common available now, many – Hypermobility affects most body systems ques:ons about op:mal • The 2017 diagnos:c criteria for hEDS are restric:ve treatment remain – Consider Hypermobility Spectrum Disorders unanswered • PT examina:on should be biopsychosocial (ICF model) • We therefore rely on the – Account for :ssue fragility principles of evidence- – Look for contribu:ng factors based prac:ce • PT management should address contribu:ng factors

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Case Examples Managing Pediatric Patients with HSD • The remainder of this session will be through case examples – Pediatric case: Stephanie Sabo, presented by L. Russek • Addresses assessment, motor delays, motor control issues, bracing – Adolescent case: Jane Simmonds • Addresses assessment, stra:fied management, POTS, fa:gue, GI problems, psychosocial issues – Adult case: Heather Purdin Case and slides provided by Stephanie Sabo, PT, MPT • Addresses assessment, chronic pain, MCAD

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Developmental Condition vs. Common Presentation in Children Hypermobility in Young Children? • Difficulty with prolonged walking or standing • • Leg pain worse in evening or at night and with/ader ac:vity Standardized motor competency tests are not • Leg pain symptoms relieved by rubbing or heat adequate – • History of “growing pains” Do not capture the full impact of joint hypermobility on motor func:on and quality of life • Difficulty si~ng in chair at school, figi:ng – Quality of movement and compensa:ons should be the focus • Trips, falls, being labeled “clumsy” (Bernie, 2011; Adib, 2005) of the assessment • Gross motor delays (Tirosh 1991, Davidovitch 1994, Bernie 2011) – Children can oden achieve individual motor items, but not • Coordina:on difficul:es (Kirby 2007) efficiently or repe::vely – (Remvig, 2011) PT for Hypermobility Spectrum Disorders 49 PT for Hypermobility Spectrum Disorders 50

Prevalence Additional Hx: Prior Therapies

• It is important to ask about whether the child has had prior therapy and • 34-35% in school age children whether is was perceived as helpful • (Arroyo, 1988; Remvig, 2011; Junge, 2013) – Some:mes prior therapy reported to be of li|le benefit and worsened symptoms – Ask if hypermobility being targeted in prior therapies; if hypermobility was not • NO gender difference found at 10 recognized, less likely that therapy was successful y/o • Authors suggest the following poten:al reasons for previously failed • (Remvig, 2011) therapies: – Techniques being too aggressive • Post pubertal gender differences – Dura:on to short • (Quatman, 2008) – Frequency to high – Failing to appreciate full scope of interven:ons required (Keer, 2003; Hakim 2003) PT for Hypermobility Spectrum Disorders 51 PT for Hypermobility Spectrum Disorders 52

Referral Sources Referral Sources

• Pediatrics • The EDS/hypermobility popula:on doesn’t fit any specialty area perfectly • Rheumatology • Joint hypermobility and/or its impact on func:on may be iden:fied • Gene:cs during treatment of another condi:on, so it is helpful to screen • Children: • Developmental Pediatrics – May or may not come with this diagnosis on the referral – May have seen many physicians or services mul:ple :mes looking for • Orthopedics answers • Pain Management • Pa:ent and family oden express frustra:ons with delay in diagnosis or lack of explana:on for symptoms.

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Insert subject I want to play just like all the Tommy: Initial Presentation Tommy, age 4 other kids • 4 year old male referred to PT by Pediatric Rheumatologist IMPAIRMENTS ACTIVITY PARTICIPATION – Onset of symptoms ~1 year ago, with increased complaints in • Pain in ankles & knees, 7/10-10/10 • Difficulty walking > 20 minutes • Trouble at school the last 6 months – Worse with ac:vity & at night • Difficulty si~ng in chair at • Trouble with social func:on, • Headaches school playing with friends – Mother with behavior and a|en:on concerns: “doesn’t ever sit • Poor posture • Poor balance PERSONAL FACTORS ENVIRONMENT s:ll” • Abnormal gait • Anxious • Mother with hEDS – • Joint hypermobility Sent to Rheumatology by primary care physician • • Over 2-hour drive from therapy • Muscle :ghtness: HS, heel cords A|en:on deficit/hyperac:vity disorder • Parents separated – 6 months later seen by Gene:cs (per Rheumatology Referral) • Poor motor control & motor skills • Self-image as “clumsy” and • Limited involvement of uncoordinated biological father • Mother with hypermobility/EDS

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Insert subject Tommy: Initial Presentation Tommy: Findings

• Pain in ankles and knees 2-3 x/week • Posture: – 7/10 on Numeric Ra:ng Scale (NRS) – Shoulders forward/rounded, increased lordosis/anterior pelvic :lt, genu – recurvatum, calcaneal valgus and pes planus, genu valgum, external rota:on increases with physical ac:vity at feet – increases in evening/night • Gait: • Up to 10/10 on NRS; mother has taken him to emergency – Decreased knee flexion, over prona:on of rearfoot-midfoot, audible foot slap, department for pain ER at feet • Bilateral hip and knee x rays - normal • ROM: – Hip ER 85o, Hip IR 70o, Knee Extension +8o, Ankle Dorsiflexion 30o, Ankle • Uses rubbing and medica:on for relief (e.g., over the counter o Tylenol or Motrin) inversion 60 • • Pa:ent also has complaints of headaches Flexibility: – Hamstrings -40o; heelcords 20o

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Measuring Flexibility Tommy: Findings

• Single limb stance: right: 4-5 seconds; led: 4-5 seconds • Assess muscle flexibility – Noted unsteadiness, with lateral trunk lean, trunk flexion, increased ankle – Differen:ate muscle flexibility and joint mobility strategies, and adduc:on of opposite LE • Bridge in hook lying for 3 seconds x 3 reps • Isolate muscle length vs. joint laxity – Noted unsteadiness and decreased eccentric control upon lowering • Mini wall squa~ng for 10 seconds x 3 reps – Reinforce need to strengthen muscles around loose joints – Noted difficulty with middle range control: pushing knee into genu recurvatum and pelvis forward into APT without cueing • Muscular imbalances • Modified heel raises: single UE support for 3 seconds x 3 reps – Noted to ini:ally complete through full ankle plantarflexion but locking out – Commonly see limita:ons in hamstrings and ankles; when completed in middle range, demonstrated unsteadiness and gastrocnemius muscle groups stepping strategies

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Measuring Strength Common Strength Deficits

• Assess core strength • Pts have difficulty maintaining stable, – Quality of movement is very important: focus on ac:va:on neutral postures and effectively of key muscle groups through hips, core and pelvis controlling movement • Efficient use of proper positions to • For example: bridging may ini:ate through trunk accomplish tasks is not natural extension and lack core ac:va:on • Pts often initially demonstrate a full crab – Under-u:liza:on of key postural muscles is common walk position when asked to perform a - Transverse abdominus, mul:fidus, gluteals, serratus anterior bridge • Even athletic patients can have difficulty – Co-contrac:on is oden lacking/decreased sustaining a neutral core with simple – (Falkerslev, 2013; Jensen, 2013; Grahame, 2009) bridging

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Strength Tommy: Ankle Strength • Assess extremity strength using func:onal strength tes:ng (no manual muscle tes:ng): • With heel raises, Tommy – All tes:ng should be done in neutral joint posi:ons, where positions ankles in end strength and endurance are oden decreased range to help stabilize – Avoid posi:ons at end of joint range • locked joints are associated with ligamentous dependency • He is unable to maintain – Assess the quality of muscle co-contrac:on a static position – Look for muscle imbalances • Oden, key postural muscles are very weak and not properly u:lized

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Tommy: Leg Strength Balance and Proprioception

Wall sits: • Assess balance and propriocepDon • Note wide base of support with genu – Observe compensatory pa|erns of movement valgum and increased pronation/ – Assess for altered awareness of body posi:on in space calcaneal valgum and knee flexion (common) • He has muscle endurance limitations and genu recurvatum with knee • Mul:ple studies confirm propriocep:ve deficits: extension – Lower limb propriocep:on is decreased in Benign Joint • He has overall poor quality of Hypermobility Syndrome movement and poor mid range control – Decreased propriocep:on in UE joints (fingers) (Smith, 2013; Rombaut, 2010; Fatoye, 2009; Sahin 2008; Schubert-Hjalmarsson, 2012)

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Balance and Proprioception Balance and Proprioception Devia:ons to look for Single leg stand: • Lateral trunk lean (gluteus medius weakness) • Unsteadiness • Observe duration of • Trendelenburg (hip drop) single limb stance • Trunk flexion AND the quality of the • Adduction of opposite leg using other limb for stability • Increased ankle strategies movement • Pronation through midfoot • Locking out knee • Internal rotation of stance limb

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Tommy: Intervention/Treatment Plan Basic Principles of Intervention • Frequency for PT services: recommended follow up visit • Begin low level for baseline ~4-6 weeks ader evalua:on; however, pa:ent was lost to – Oden need to begin in gravity eliminated follow up for 6 months due to family having social concerns posi:ons and/or use isometrics • EducaDon: joint protec:on, ac:vity modifica:on, sod joints • Progress at slow rate • OrthoDcs: fi|ed with ortho:cs at evalua:on • Consider the whole person • HEP: bridging, resisted hip abduc:on, squats, passive – Not just one joint at a :me hamstring stretch – Non musculoskeletal manifesta:ons • Treatments: completed HEP 4x/week from evalua:on un:l • Customize treatment based on 6 month follow up visit individual needs and goals (Celletti, 2013)

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Joint Protection Joint Protection

• Postural re-educa:on and awareness • Pa:ents should be instructed how to avoid overstretching – Reduces stress on muscles and joints joints and to reduce mechanical stress on joints – Reduce pain and fa:gue over :me – For example: individuals can prac:ce finding and maintaining a “sod knee” • Focus on awareness of neutral joint posi:on, with knees neutral or very slightly flexed posi:ons. Avoid: • Lack of postural muscle ac:va:on reinforces postural deficits – Knee and elbow hyperextension • Avoid excessive joint movement, which could lead to tears – Anterior pelvic :lt/hip hanging (forward and laterally) and rupture of the sod :ssues surrounding the joint – W si~ng • (Rombaut, 2012) (Rombaut, 2012) – Rounded shoulder and forward head PT for Hypermobility Spectrum Disorders 71 PT for Hypermobility Spectrum Disorders 72

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Controlled Stretching Orthoses: Summary of Evidence • Pay a%en(on to technique: • There is limited, inconclusive evidence, even as it relates to the general – Stabilize surrounding joints to stretch “flat feet” diagnosis in the pediatric popula:on specific :ght muscles without stressing • Systema:c reviews find that studies use different pa:ent popula:ons and other structures widely varying type of orthoses (Evans, 2011) • Contrast pa:ent’s ini:al posi:on for • No informa:on to consistently support using ortho:cs or not standing hamstring stretch (top) with • Planus foot in early life is associated with adult degenera:ve joint disease and interven:on is indicated (Gross, 2011) performance AFTER instruc:on in • proper form (bo|om) Anecdotal experience with use of ortho:cs in the hypermobile popula:on is very posi:ve (Local CCH consensus, 2016)

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Minimal Control Orthotics Models of Therapy

• Provide some support to pronated • ConsultaDve: Emphasis is on (flat) feet home exercise programs that • Can reduce stress on all lower are periodically updated by the extremity joints therapist • Customizable • Intermediate: 1-3x/wk

Cascade DAFO Fast Fit Vasyli orthotics • Intensive: Frequent sessions http://cascadedafo.com www.vasylimedical.com over a short period of :me

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Consultative Model Intensive Treatment Model • Emphasis on home programming and self management • Mul:disciplinary program, including (oden used in ini:al stages) OT, PT, MD, Psychology, Child Life – Seeks to provide knowledge, as well as – Home exercise program established at ini:al session a physical boost – Educa:on with child and caregivers: posture, joint protec:on… – Provides tools for self management – Ortho:cs, if indicated – Provides social Interac:ons • Follow up plan established – Par:cipants become their own experts – – Individualized, emphasizing self management Facilitates home adherence and lifelong management – Frequency of therapy is determined at the evalua:on – Increases independence with HEP (Bathen, 2013; Birt, 2013) – Follow-up oden 4 to 6 weeks later

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Intensive Model (2 week program) Example Intensive Model • Program started 2011 • 2-week program • Outcome measures pre and post: • Par:cipants grouped by gender and age MD – PedsQL, COPM, Tampa Scale Kinesiophobia • • All with EDS and/or joint hypermobility condi:ons Psychology uses cogni:ve behavioral therapy Child and group sessions OT • Benefits: Life • Music incorporated in exercise sessions and – Intensive exposure to PT empowers parents and children PT with Child Life – Par:cipants gain knowledge, skills and confidence to adapt exercises and self- • Child Life helps pa:ents write le|ers of manage symptoms encouragement to the next group coming into PSY Aqua- the program • Adolescents faced the most difficul:es with finding :me, tics • HEP books are constructed with pictures of privacy and mo:va:on to do the exercise program at home the pa:ent doing the exercises • (Celle~, 2013; Castori, 2012)

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Outcome Measures • Canadian Occupa:onal Performance Measure (COPM) • Pediatric Quality of Life Measure (PedsQL) • Tampa Scale for Kinesiophobia (TSK)

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Tommy: Outcome Summary of Pediatric Case

At 6 months: • Hypermobile pa:ents are in most pediatric caseloads • Leg pain decreased from • Func:onal impact of joint hypermobility pain and fa:gue is significant daily to 1-2x/month and is oden under-appreciated • Improved quality of • Standard therapy frequently fails these pa:ents movements • Specific, targeted joint stabiliza:on, neuromuscular training and • Improved ability to sustain educa:on are needed hold counts • Low intensity and slow progression of interven:on is indicated • Decreased postural compensations • Lives can be changed!

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Thanks To: Stephanie Sabo PT, MPT Physical Therapist II [email protected] • Cincinnati Children's Hospital Medical Center Joint Hypermobility Team 2014. “Identification and Managing Adolescents with HSD and HEDS Management of Pediatric Joint Hypermobility” CCHMC EBDM Website Guideline 43 pages 1-22. Available at https://www.cincinnatichildrens.org Jane Simmonds DProf, MCSP, MACP, SFHEA

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Reflect on the key physiological and Outline of Adolescent Case psychological changes during adolescence

1. Hormonal changes • Consider issues which arise in adolescence 2. Growth – Peak Height Velocity • Stratified approach to management Hypermobility 3. Developing independence Spectrum Disorders and Hypermobile Ehlers Danlos Syndrome Seminal study (Kirk,1967) described three quarters of patients developed symptoms prior to age 15 years

• Case study Hypermobile adolescents 2 times more likely to develop musculoskeletal problems than non hypermobile • Plans for research counterpart. Risk increases 12 fold if overweight (Tobias, 2013)

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Asymptomatic Mild Signs Significant Signs SPECTRUM & Symptoms & Symptoms

DS SIMPLE/ACUTE INTERMEDIATE COMPLEX/CHRONIC Joint Laxity

GJH hEDS

GJH = (asymptomatic) joint hypermobility; hEDS = hypermobile EDS EDS = Ehlers-Danlos Syndrome (Castori, 2017)

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STRATIFIED MANAGEMENT STRATIFIED MANAGEMENT

SIMPLE/ EARLY SIMPLE/ EARLY Episode of acute musculoskeletal injury, Episode of acute musculoskeletal injury, sprains, dislocation, subluxations sprains, dislocation, subluxations enthesopathies enthesopathies

(Pacey 2010; Smith 2005) (Pacey, 2010; Smith, 2005)

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STRATIFIED MANAGEMENT

SIMPLE/ EARLY SIMPLE/ EARLY Episode of acute musculoskeletal injury, Ice, electrotherapy, tape, support, exercise, sprains, dislocation, subluxations screen, education – rehabilitate and enthesopathies prevent

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STRATIFIED MANAGEMENT STRATIFIED MANAGEMENT

SIMPLE/ EARLY SIMPLE/ EARLY SIMPLE/ EARLY SIMPLE/ EARLY Episode of acute musculoskeletal injury, Ice, electrotherapy, tape, support, exercise, Episode of acute musculoskeletal injury, Ice, electrotherapy, tape, support, exercise, sprains, dislocation, subluxations screen, education – rehabilitate and sprains, dislocation, subluxations screen, education – rehabilitate and enthesopathies prevent enthesopathies prevent

INTERMEDIATE INTERMEDIATE INTERMEDIATE Recurrent episodes, series of episodes at Recurrent episodes, series of episodes at Physiotherapy modalities have temporary different sites, deconditioning, some different sites, deconditioning, some effect, no effect or exacerbate central/ peripheral sensitization, mild central/ peripheral sensitization, mild Modified / adapted approach systemic conditions systemic conditions Functional Restoration

(Engelbert 2017; Scheper 2017)

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Subjective Assessment Subjective Assessment Listen to the history carefully……. Listen to the history carefully……. Explore expectations with young person and parent Explore expectations with young person and parent Identify problems – prioritize Identify problems – prioritize – Pain – local/ general/ acute/ chronic (sensitization) – Pain – local/ general/ acute/ chronic (sensitization) – Joint instability – subluxations, dislocations, clicking – Joint instability – subluxations, dislocations, clicking – Fatigue – sleep, fluid, diet – Fatigue – sleep, fluid, diet – Anxiety - Low mood/ depression Listen and develop – Anxiety - Low mood/ depression – Gastrointestinal dysmotility the therapeutic – Gastrointestinal dysmotility – Dysautonomia – Postural Tachycardia Syndrome (POTS) – Dysautonomia – Postural Tachycardia Syndrome (POTS) Identify – Gynaecology and bladder problems alliance – Gynaecology and bladder problems barriers to – Allergies – Allergies rehabilitation Explore impact Explore impact – Physical activity/ Sport/ Hobbies Physical Education – Physical activity/ Sport/ Hobbies Physical Education – Social, School, General health – Social, School, General health – Family history and thorough developmental history ** – Family history and thorough developmental history **

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Subjective Assessment Objective Assessment Listen to the history carefully……. Careful active and passive joint range and muscle length Explore expectations with young person and parent Identify problems – prioritize Functional assessment * – Pain – local/ general/ acute/ chronic (sensitization) Posture and gait – compensatory patterns – Joint instability – subluxations, dislocations, clicking Sit to stand/ squat – gluteal, quadriceps – Fatigue – sleep, fluid, diet Observe Carefully – Anxiety - Low mood/ depression Single leg dip – Gastrointestinal dysmotility Heel raise – tibialis posterior – Dysautonomia – Postural Tachycardia Syndrome (POTS) Identify personal Balance – Single leg / Y Balance Test / Hop/ Jump – Gynaecology and bladder problems strengths and – Allergies Repositioning tests – proprioception/ kinaesthetic interests to drive Explore impact rehabilitation Strength/ activation (careful testing* - through range) – Physical activity/ Sport/ Hobbies Physical Education – Social, School, General health – Family history and thorough developmental history ** Test for POTS (standing test…refer on)

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Dysautonomia/ Postural Tachycardia Dysautonomia/ Postural Tachycardia Syndrome (POTS) Syndrome (POTS) Near syncope on standing Near syncope on standing Venous pooling with colour changes Venous pooling with colour changes Tachycardia –standing and changing position Tachycardia –standing and changing position Can result in massive anxiety Can result in massive anxiety Excessive heart rate on exercise Excessive heart rate on exercise

Hyperventilation Hyperventilation Mechanisms Heat intolerance Heat intolerance Illness Hormonal Nausea Nausea Deconditioning Hypermobility Mathias (2011); Kizilbash (2014) Mathias (2011); Kizilbash (2014)

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Artefact corrected heart rate Walking Autonomic Testing

Typical response POTS patient Tilt Test

Rise of ≥30 BPM adults

Rise of ≥ 40 BPM in adolescents

Time Time Chair Mathias (2011); Raj (2013); Kizilbash (2014) Chair PT for Hypermobility Spectrum Disorders 103 PT for Hypermobility Spectrum Disorders 104

Meet Helen 15 years I want to do my Autonomic Testing exams…play sport, PMHx: Hypermobility detected in early life. Enjoyed being active. dance …..go Recurrent injuries, subluxations. Physiotherapists treated single areas. shopping PC: Never got on top of problem, injury after injury….now not coping …. Typical response POTS patient Tilt Test IMPAIRMENTS ACTIVITY PARTICIPATION

• Widespread hypermobility ++ • Able to walk for 15-20 mins • Missing school Rise of ≥30 BPM adults • Recurrent shoulder, knee • Struggling with wri:ng • Struggles with dancing, not finger subluxa:ons • Struggling to carry school bag swimming or playing netball Rise of ≥ 40 BPM in • Persistent pain and fa:gue • Struggling on public transport • Reduced social ac:vity with friends (mainly online) adolescents • Decondi:oned + – due to syncope • Anxious and low mood • Pre syncope and fast heart PERSONAL FACTORS ENVIRONMENT rate when standing (POTS) Time • Female • Mother with fibromyalgia Time • Dysmenorrhea • High achiever – A student • Younger brother – hEDS • Bloa:ng and early sa:ety • Low confidence 10 Minute Standing Test – Alternative to Tilt Test Mathias (2011); Raj (2013); Kizilbash (2014) • Lovely friends

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Fatigue Meet Helen 15 years Relevant PMHx: Hypermobility detected in early life. Enjoyed being active. Outcome Pain Dysautonomia Recurrent injuries, subluxations. Physiotherapists treated single areas. PC: Never got on top of problem, injury after injury….now not coping …. Measures

IMPAIRMENTS ACTIVITY PARTICIPATION Neuromusculoskeletal • Pain Visual Analogue Scale • Pa:ent Specific Func:onal • PedsQL Cardiovascular (VAS) Scale (PSFS) • (Physical func:on, social, • PedsQL Mul:dimensional • Goal A|ainment Scale emo:onal and school) Fa:gue Scale • Single Leg Dip • Modified Star Excursion Balance Test (Y Balance) PERSONAL FACTORS ENVIRONMENT Gastrointestinal • Tilt Test/Stand Test Urogenital • Self Efficacy Scale (VAS) • PedsQL Gastro Intes:nal • Coping Scale (VAS) Psychological Symptom Profile Symptoms and Worry Scales Ninis (2015)

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Management

• Prioritize problems • Motivate and empower – partnership • Educate young person and family on all aspects of the condition • Agree goals (short and longer term) • Pain and fatigue management – including sleep routine • Movement correction • Exercise - recondition

(Engelbert, 2017)

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Survey of 946 paDents UK Experiences of Physiotherapy Liaise with school teachers Dance teacher Coaches

(Simmonds, 2017)

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Dislocation and Splinting and Tape Subluxation Limited evidence: wrist/hand neoprene splint not effective for hand pain or writing speed (small sample) (Frohlich, 2011)

Panic = muscle spasm Position the joint Analgesic Heat - Breath – Relax - Distract Give it time Expert opinion - Judicious use ** Do your usual thing… Ice, analgesia Can be very helpful for when returning from injury and for func:on Support for a few days…carry on

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Orthotics and Footwear Management of POTS • Advice – fluids, electrolytes/salt, compression tights Cochrane Review Recommends supportive footwear and orthotics – Positioning, anti syncope manoeuvres for flexible flat feet (Evans, 2011) • Medications prescribed (Midodrine, Fludrocortizone, Beta blockade) • Respiratory physiotherapy – hyperventilation • Anxiety management – psychological support • Small meals, low carbohydrate diet and FODMAP Improved gait efficiency in young people with Developmental • Graded cardiovascular exercise and resistance training – focus on lower limbs Coordination Disorder and Hypermobility Syndrome (Morrison, 2013) – Morning exercise • Incorporating exercise to manage joint instability • Recumbent to upright exercise Expert opinion - Judicious use of orthotics or supportive footwear/ heal cup/ high tops (Mathias, 2011; Fu, 2011; George, 2013; Kizilbash, 2014)

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PREMISE FOR EXERCISE: Long term benefits of improved physical fitness PREMISE FOR EXERCISE: Long term benefits of improved physical fitness and lower limb strength counteract orthostatic intolerance" and lower limb strength counteract orthostatic intolerance" • Increase blood volume" • Increase blood volume" • Increase cardiac output" • Increase cardiac output" • Enhance vascular compression due to increased muscle mass and tone" • Enhance vascular compression due to increased muscle mass and tone" • Improve endothelilial function" • Improve endothelilial function" • Improve baro reflex function " • Improve baro reflex function " " "During exercise, people with POTS have a low stroke volume response to exercise – leads to light headedness, dizziness, dyspnoea and weakness!

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For Helen Application of Exercise • Individualise treatment based on needs and goals • Begin at a low level for baseline – Weak and poor proprioception (Ferrell 2004; Engelbert 2017) – Fear and pain (Simmonds, 2017) • Progress slowly – exercise through range (Pacey 2013) • Consider motor control (Roussel, 2009 ) and engage thought (Boudreau 2010) • Consider the whole person – kinetic chain – Not just one joint at a time – Non musculoskeletal manifestations • Hands on to teach (Simmonds, 2017) • Make it relevant and fun and include the family (Birt, 2015)

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For Helen For Helen Pilates ….. Dance

(Simmonds, 2017)

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STRATIFIED MANAGEMENT STRATIFIED MANAGEMENT

SIMPLE/ EARLY SIMPLE/ EARLY SIMPLE/ EARLY SIMPLE/ EARLY Episode of acute musculoskeletal injury, Ice, electrotherapy, tape, support, exercise, Episode of acute musculoskeletal injury, Ice, electrotherapy, tape, support, exercise, sprains, dislocation, subluxations screen, education – rehabilitate and sprains, dislocation, subluxations screen, education – rehabilitate and enthesopathies prevent enthesopathies prevent

INTERMEDIATE INTERMEDIATE INTERMEDIATE INTERMEDIATE Recurrent episodes, series of episodes at Physiotherapy modalities have temporary Recurrent episodes, series of episodes at Physiotherapy modalities have temporary different sites, deconditioning, some effect, no effect or exacerbate different sites, deconditioning, some effect, no effect or exacerbate central/ peripheral sensitization, mild Modified / adapted approach central/ peripheral sensitization, mild Modified / adapted approach systemic conditions Functional Restoration systemic conditions Functional Restoration

COMPLEX LONG TERM COMPLEX LONG TERM COMPLEX/ LONG TERM Chronic, longstanding, severe, unremitting Chronic, longstanding, severe, unremitting Multi disciplinary management programme pain with profound deconditioning/ pain with profound deconditioning/ using functional andcognitive behavioural comorbidities, disability comorbidities, disability approaches (Bathen 2014)

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Summary for Functional Rehabilitation Future Research and Education Time to listen reassurance, educate, empower (young person and family)

Condition specific education, advice and support • Understand the factors which influence development of symptoms

Give HOPE, agree GOALS, and be CREATIVE • Validate and agree outcome measures • Understand what works best for young people Treat the treatable – prioritise • Intervention trials Pain and fatigue management (young person and parent) • Education patients and professionals Improve movement – use your hands

Strengthen weak muscle groups

Improve cardiovascular fitness

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Acknowledgments

Patients and families Professor Rodney Grahame Rosemary Keer Dr Nelly Ninis Dr Hanadi Kazkaz Dr Alan Hakim Professor Christopher Mathias Colleagues in the Hypermobility Unit THANK YOU [email protected]

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Meet Mindy, I need to look after my 37 y/o home maker family BODY STRUCTURE/ FUNCTION ACTIVITY PARTICIPATION • Widespread hypermobility • Limited arm func:on due to • Struggling to care for • Allograd HS and Achilles used shoulder instability family and home Adults with HSD to stabilize R shoulder and its • Church involvement ︎ • ⬇ disloca:ng again Limited mobility Guido Daniele • Past Hx lumbar discectomy • Pain limits ADL and IADL ENVIRONMENT http://www.guidodaniele.com • Weight gain 40# since PERSONAL FACTORS • Suppor:ve husband By Heather Purdin, MS, PT, CMPT stopping pain meds last year • Sick kids/near death • Uterine Prolapse • Female experience [email protected] • Dizziness and tachycardia • Driven • Recently relocated • TMJ and rib subluxa:ons • Frustrated by medical care • Stress level high due to • Widespread hives & swelling • Kids with hEDS need support new Church assignment

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Gravity affects EDS/HSD Gravity is a problem

Pre-operaDve shoulder Normal shoulder x-ray Inferior anterior subluxaDon Deltoid, upper trap, rotator cuff set subluxaDon

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Subluxed Shoulder Causes Neck/Jaw Malalignment Types of Pain Downsloped R shoulder pulls lower Set shoulder brings head neck to R and head corrects to L back to neutral • Iden:fy the type(s) of pain – Nocicep:ve: mechanical, inflammatory – Neuropathic: peripheral, (central) – Central sensi:za:on: neuroplas:city • Different pain is treated differently

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Pain Quality Medications/ Exercise Other Source Referral Intervention What is MCAD? Mechanical Localized, occurs NSAIDs? Strength/Mobility Bracing, taping, Slide from Anne Maitland’s with certain Primary MD Balance throughout Ergonomics training, presentation at 2017 EDS motions. kinetic chain Muscle setting Global Learning Conference Sensitized by Proprioception inflammation Inflammatory Burning in broad NSAIDs, Pool Modalities, Tool area not Allergy meds, MCAD Proprioceptive input assisted scraping, dermatomal, bruisy Primary MD, Allergy Mindful movement Dietary guidance

Nerve Burning in a Gabapentin, Lyrica, Nerve flossing Posture training to peripheral nerve or SNRI’s, LDN, Triptylines Mindful movement address entrapped dermatomal Primary MD, Neuro, Cardio nerves, positions of pattern, searing Nutrition? External focus slack (Chopra, 2017; Castori, 2012) Central Whole body pain, SNRI’s, triptylines, LDN Cardio Meditation Sensitization difficult to localize, Primary MD, Psych Mindful movement Breathing wind-up, allodynia 137 External focus Biofeedback PT for Hypermobility Spectrum Disorders PT for Hypermobility Spectrum Disorders https://ehlers-danlos.com/pdf/Maitland-EDNF-Vegas-Mast-Cell-Activation-Disorders-S.pdf138 .

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MCAD Presentation Mindy’s Symptoms - Hives, red rash - Brain fog - Diges:ve issues - Bladder irritability - Swelling in limbs, supraclavicular - Elevated urine histamines Slide from A. Maiteland, EDS Global Learning Conference, 2017 https://ehlers-danlos.com/pdf/Maitland-EDNF-Vegas-Mast-Cell-Activation-Disorders-S.pdf. PT for Hypermobility Spectrum Disorders 139 PT for Hypermobility Spectrum Disorders 140

Manage Mast Cell Activation

• Iden:fy triggers: – Alcohol, heat, medica:ons (NSAIDs, an:bio:cs, narco:cs), allergens – Food sensi:vi:es – Excessive exercise • Manage physical and emo:onal stress • Exercise regularly, in spite of fa:gue • Advocate for pa:ent ge~ng on Mast Cell Stabilizers and meds that block chemical mediators like an:-histamines – send pa:ent to MD with research ar:cles – (Akin, 2010; Moulderings, 2011; Seniviratne, 2017; Theoharides 2015)

PT for Hypermobility SpectrumSlide Disordersfrom A. Maiteland 141, EDS Global Learning Conference, 2017 PT for Hypermobility Spectrum Disorders 142 https://ehlers-danlos.com/pdf/Maitland-EDNF-Vegas-Mast-Cell-Activation-Disorders-S.pdf.

Dietary Advice Sources of Pain • 37% of people with IBS have hEDS • FODMAP diet may be helpful (expert opinion) • Don’t just look for symptoma:c :ssue – find the – Fructose, Oligosaccharides, Disaccharides, Monoamines, and Polyols – Bacteria thrive on FODMAP foods/dysbiosis causes MCAD cause of :ssue symptoms – Monash University, Melborne Austrialia (Fikree, 2017) – E.g.: UT TrP may be causing HA, but poor propriocep=on • Other recommenda:ons are specula:ve and DNF motor control cause UT TrP – Heidi Collins, MD diet – avoid chemicals, gluten, take supplements to improve nutri:on, reduce histamines • Chronic pain - consider – Low histamine diet (especially with MCAD) – Psychosocial factors – Avoid hard foods and excessive jaw movements (ice, gums, etc.) to avoid TMD – Stress – Avoid bladder irritant foods (e.g., coffee, citrus products) – Avoid large meals (especially of refined carbohydrates) – Childhood trauma

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HSD Through the Life Span Adverse Childhood Experience 1. Hypermobile phase 3. Stiffness phase – Hypermobile joints – Widespread pain – Clumsiness/motor delay – Fatigue • Specific adverse childhood experiences (ACEs) – Constipation/diarrhea – Tendinosis/tendon rupture – Abdominal hernias – Chronic gastritis increase risk of chronic pain 2. Pain phase – Stiffness – Verbal or sexual abuse, parental psychopathy, Early – Chronic musculoskeletal pain – (Castori, 2011; Tinkle, 2017) – Strains, sprains, dislocations parental death – Unrefreshing sleep – Chronic fatigue – Adults (Sachs-Erickson, 2017) – Memory/cognitive problems – Children and adolescents (Nelson, 2017) – Gastric reflux, abdominal pain – Paresthesias • Mechanism may be via altered HPA axis and – Tachycardia – Incontinence/UTI autonomic dysfunc:on (Elbers, 2017)

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Set Realistic Goals Mindy’s Goals • Good shoulder alignment without shoulder subluxa:on: 60% met • Pa:ent avoids disability/reverses disability • Can talk unlimited by jaw pain/locking: Met • Improved stability B hips to allow 1 hour of pain free walking with • Fewer “bad” days rest breaks: Met with pain, no pain flare with 30 minutes of walking • Self treatment strategies reduce need for • Independent with progressive HEP with self-relief for pain: Met for basic program for shoulders, neck, ribs, hips, core medical interven:on • Improved biomechanics and stability of ribcage for full, pain free • Increased self-efficacy ven:la:on: Mostly met, self treats rib malalignment • Improved mobility and reduced pain in head and neck: Partly met, needs cues to stabilize neck during arm use

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Pain Self-Management Body Mechanics • Posture and ergonomics at school/work/home • Techniques to decrease pain: – Pain neuroscience educa:on (e.g., “Explain Pain”) • Sleeping posture, surface, support – Cogni:ve behavioral therapy – Engaging muscles before moving – Relaxa:on – Assis:ve devices (e.g., pens, tools, etc.) – Self-care with heat, ice, TENS • Techniques to decrease injury – Joint protec:on strategies – Bracing/splin:ng, etc.

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Biomechanical Differences Biomechanical Differences • Shoulder se~ng • Ankle protec:on – Cue up instead of down – Bed sheets can sublux talus anteriorly – Ball back in socket, not just scapulae – cue upside down “G” – • Biceps long head subluxa:on Si~ng on ankles – Reduce ER and abduc:on posi:ons • Patellofemoral instability • Hip posterior sea:ng – Typical issues Osgood Schla|er’s, tracking issues – No hip flexor use without glut use or • Elbow – ulnar nerve subluxa:on usually at 90+ – Alter posi:on of exercise to assist stability/posterior sea:ng degrees such as in long si~ng • Wrist – carpal subluxa:ons, rota:ons

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Biomechanical Differences Exercise: Do No Harm! • Craniocervical instability – Oden just long hold into mobiliza:on direc:on is enough to • Research shows that many pa:ents with hEDS normalize have nega:ve past physical therapy • Spine Instabili:es – Spondylolisthesis experiences due to: – Retrolisthesis • Rib se~ng – Iatrogenic joint injuries – ½ inhale before liding – If depressed ribs, inhale with ac:vity – Unmet rehabilita:on needs – If elevated ribs, exhale with ac:vity » (Bovet, 2016)

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Exercise: Do No Harm! Exercise: Do No Harm!

Avoid mechanical pain Cau:on with overuse: nerve • Stabiliza:on & motor entrapment, trigger points control are cri:cal Cau:on with changing forces Think before moving e.g., exercise bands increase • Tensile strength of :ssues resistance varies with ac:vity, menses, Allow :ssue recovery between inflammatory state, age etc. exercise bouts 2-hour rule: discomfort • Slow progression to allow should return to baseline histological changes within 2 hours

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Exercise Progression Proprioceptive Exercise

• Remember basic principles of exercise progression • Propriocep:on significantly worse in knees with BJHS (Sahin, 2008) – Propriocep:on exercises improve pain and func:on (Sahin, 2008) • Propriocep:ve ex for knees reduces pain and improves quality of life (Ferrell, 2004) – Closed Chain LE ex – bridges, squats, side lunge to s:mulate joint receptors • Neck and spine pain - Propriocep:ve ex may be less relevant than behavioral and educa:onal Rx, meta analysis (McCaskey, 2014) • Back pain reduc:on, muscle endurance and postural stability improvements with lumbar spinal stab exercise (Toprak Celenay, 2017)

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Exercise: Proprioception Exercise Progression • External focus exercises – put a laser Easier Harder on it and draw on the wall, think • • BAPS (SenMoCor System™) More propriocep:ve Less external propriocep:ve • Biofeedback (e.g., Stabilizer™) feedback feedback • Alterna:ng isometrics, dynamic – Tac:le, visual, verbal stabiliza:on – External focus, e.g. lasers • Less support • Ball exercises, balance & • More support – More challenge, e.g., unstable propriocep:on – Exercise machines surfaces • Emphasize good motor control – Against wall – Free weights or bands

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Exercise Progressions Exercise Progressions Gravity assisted stabiliza:on, Adding gravity to stabilizers No towel under the arm, set Reducing tac:le maximum tac:le feedback without challenging subluxa:on up and back, control resistance feedback/use of wall

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Exercise Progression Exercise Progressions

Easier Harder Speed/alterna:ng isometrics Against gravity is more advanced • Focus on joint stability • Focus on integra:on – Isolate leg or arm without – Coordinate core and limb spine movement movement – Isolate shoulder stabilizers – Add external challenge – Isolate core – Complex movements, e.g., Tai Chi • Visualiza:on/mind-body • Add distrac:on/mul:tasking – E.g., joint compression, Qigong

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Exercise Progression – Stabilization Central Sensitization Exercises Easier Harder • Shoulder se~ng with • Chronic Pain – unstable • Chronic Pain- longer holds, pla]orm, flowing mo:on, isometrics, longer sets, stretch feedback alternate agonist/antagonist later in session or contract/relax and stretch • Focus on body posi:on • External focus exercise – laser pointer on target/visualiza:on

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Craniocervical Flexion vs Proprioception Ex Exercise Progression

• Group 1 exercising with Stabilizer/longus coli nods Easier Harder • Mid range • Group 2 exercising with laser on head and eye and • Full Range • Short dura:on head movements to targets (external focus) • Longer dura:on • • Slow Both Groups had reduc:on in pain and neck • Fast disability index • Low impact • Add impact • • Symmetrical/bilateral Propriocep:on group > CC flexion improved • Unilateral/asymmetrical tolerance to trigger point palpa:on • RPE start at 4/10 (Galllego-Izquierdo, 2016 ) • RPE 7.5/10 ul:mate goal

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Exercise: Strengthening Cardiovascular Exercise • Strengthen stabiliza:on mm before global mm – Spine stability ex with BJHS reduced pain, improved endurance, improved postural • Chronic pain leads to decondi:oning stability (Toprak Celenay, 2016) • Possibility of increased joint s:ffness with increased strength • Exercise-induced analgesia can reduce pain – Cheerleaders increased shoulder s:ffness, decreased ant. capsule laxity (Laudner, 2013) – Increased patellar tendon s:ffness in pa:ents with cEDS (Moller, 2014) • Start cardio ader ini:al core stabiliza:on – Mechanical strain inhibits collagen breakdown (Flynn, 2010) • Need propriocep:on & motor control (Scheper, 2016) training or use machines that provide stability • Be aware of stresses on ‘incidental’ joints, e.g. – Hands gripping weights or Theraband (e.g., recumbent bike) – Wrist extension in quadruped – Spine stabiliza:on for extremity exercise • Make it fun so pa:ents s:ck with it

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Exercise: Stretching Bracing and Taping

• People with EDS can have :ght structures • Mul:direc:onal instability common • • Hips, Shoulders, SI, Knees, Ribs, Isolate stretch to proper structure Fingers, Feet… – Keep joints in proper alignment when stretching muscles • Benefit may be from propriocep:ve • Stretches may need to be gentle feedback – Only do 80% stretch and hold 3-5 sec in pa:ents with • Give pa:ent resources to self manage peripheral sensi:za:on painful areas as they arise • Teach pa:ent to use muscles to brace – This can minimize flares in sensi:zed pa:ents for the ac:vity

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Picture of Mindy’s Modified Brace Manual Therapy • Relieve mm spasm, TrP, & fascial adhesions • Decrease pain & autonomic tone • Realign joints carefully – MWM, MET, nerve mobs, grades I-IV – Stabilize nearby structures • DO NO HARM! – Do not over-mobilize

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Content may not be copied without permission of the speakers. 29 Physical Therapy for Hypermobility 2/23/18 Spectrum Disorder

Precautions and Red Flags Mindy’s outcomes • Spinal fluid increased pressure, leaks, syrinxes and headaches – • – Mindy had documented leak from nose approx. 1 cup of spinal fluid ader husband 22% increase in func:on on Care Connec:ons form pushed on her upper neck with massage which alleviated her “worst headache in • 4.5/7 global ra:ng of change on scale of -7 to +7 her life” • Craniocervical instability – is dizziness from POTS or CCI, stress, BPPV? • Worst pain reduced from 9/10 to 4-5/10, LBP 0/10 • Chiari Malforma:on – may cause increased spinal fluid pressure, ataxia especially if malalligned in upper cervical • Areas treated: Neck, jaw, ribs, shoulder, hips, LB • Tethered cord – avoid excessive nerve tension/flossing only • 21 visits over 6 months – Saddle anesthesia, difficulty walking, bowel/bladder issues, tension • Precau:ons as with a pregnant pa:ent for laxity

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Summary of Adult Case Overall Summary • Need comprehensive evalua:on • Hypermobility spectrum disorders are common • Challenge to overcome gravity • HSD involves many body systems, not just joints • Motor control is essen:al • Physical therapy is key to management • Subtle changes can be important • Program must be customized to the pa:ent • Start low, go slow! • Look for zebras!

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Content may not be copied without permission of the speakers. 30 Physical Therapy for Hypermobility Spectrum Disorders References

The whole issue of Am J Med Genet C Semin Med Genet. 2017;175(1) is devoted to EDS, including hEDS and HSD. These articles, including many cited below, are available at https://www.ehlers-danlos.com/2017-eds-international-classification/.

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