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Physical Therapy for Hypermobility Spectrum Disorder

2/23/18

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PT for Hypermobility Spectrum Disorders

A Zebra Among Us: Recognition & Management of

Hypermobility Spectrum Disorders Combined Sections Meeting 2018

New Orleans, LA, February 21-24, 2018 LeslieNRussek,PT,DPT,PhD,OCS

ClarksonUniversity,Canton-PotsdamHospital,Potsdam,NYStephanieSaboPT,MPT

Cincinna:Children’sHospitalMedicalCenter,Cincinna:,OHJaneSimmondsDProf,MCSP,MACP,SFHEA

GreatOrmondIns:tuteofChildHealth,UniversityCollegeLondon,London,UKHeatherPurdin,MS,PT,CMPT

GoodHealthPhysicalTherapy&Wellness,Portland,OR

1

Disclosures The speakers have no financial or other conflicts of interest.

PT for Hypermobility Spectrum Disorders 2

Affiliations [email protected] [email protected] [email protected] [email protected]


Learning Objectives Attheendofthiscourse,parDcipantswillbeableto:

1.  Describetypicalclinicalpresenta:onofpa:entswithhypermobilityspectrumdisorder(HSD)throughthelifespan,includingpediatric,adolescent,andadultpa:entswithinthecontextoftheICFmodel.

2.  Applythe2017Interna:onalClassifica:ontoiden:fyHypermobilitySpectrumDisordersandhEDS.

3.  Proposeevidence-basedapproachesforphysicaltherapymanagementforindividualswithhypermobilityspectrumdisorders.

4.  Recognizecommonchallengesandpi]allsworkingwiththesecomplexpa:ents.


Outline •  25min:Introduc:ontoEDS,hEDS,HSD(LRussek)•  25min:Pediatriccase(StephanieSabo,presentedbyL.Russek)

–  Exam,evalua:on,motordelays,motorcontrolissues,bracing•  25min:Adolescentcase(JaneSimmonds)

–  Exam,evalua:on,stra:fiedmanagement,POTS,fa:gue,GIproblems,psychosocialissues

•  25min:Adultcase(HeatherPurdin)–  Exam,evalua:on,chronicpain,MCAD,

•  20min:QA(panel)


Many Types of EDS •  Hypermobile (III): Loose joints, joint pain. Most common. •  Classical (I & II): Velvety, stretchy, fragile skin. Common. •  Vascular (IV): Possible arterial/organ rupture. Most serious. •  Kyphoscoliosis: Joint laxity, muscle hypotonia,

developmental delay. Severe functional loss over time. •  Arthrochalasia (VII): Congenital hip dislocation, lax joints. •  Dermatosparaxis (VII): Severe skin fragility & bruising.

•  Malfait, 2017



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Vascular Ehlers-Danlos Syndrome

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

7

Classical Ehlers-Danlos Syndrome


Many Names of Hypermobility •  Generalizedjointhypermobility/laxity(GJH)

–  Mightormightnothavepainorsymptoms•  Jointhypermobilitysyndrome(JHS)&hypermobilitysyndrome

(HMS):termsodenusedbyrheumatologists•  Ehlers-DanlosSyndrome–hypermobilitytype(EDS–HTortypeIII)

terminologyusedbygene:cists•  NewlyproposedterminologyhypermobileEhlers-DanlosSyndrome

(hEDS)andHypermobilitySpectrumDisorder(HSD)•  Wewillrefertoitas‘hypermobility’or‘HSD’


Hypermobility Spectrum Disorder

Individual

JointLaxity

GJH

EDS

hEDS

Asymptomatic Mild Symptoms Severe Symptoms

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

Localized-HSD Generalized-HSD Peripheral-HSD Historical-HSD


Prevalence of EDS •  HSDisthemostcommonsystemicinheritedconnec:ve:ssue

disorderinhumans(Tinkle,2017)–  intheUKprevalenceofHSDassociatedwithchronicwidespreadpain

orseverelydisablingpain(Mulvey,2013)isalmostashighasfibromyalgia(Fayaz,2016)and100:meshigherthanrheumatoidarthri:s.(Humphreys,2013)

•  Affects~10millionpeopleintheU.S.(Tinkle,2017)–  30xnumberofTHA/yr;60xnumberofACLreconstruc:ons/yr

•  HSDinmusculoskeletalhealthcare:30-55%

•  InOmaniwomen,probablynotthishighintheUS.(Clark,2011)•  80-90%ofallEDSishEDS(Tinkle,2017)


Pathophysiology •  Autosomaldominantconnec:ve:ssuedisorder•  Thereisnogene:cmarkerknownforhEDS

–  IncontrasttomostoftheotherformsofEDS•  Heterogeneouspathophysiology

–  TenascinXabnormality?–  TypeIIIcollagenabnormality?–  Exactconnec:ve:ssueabnormalityisunknown–  (Tinkle,2017;Malfait,2017)



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Common Signs & Symptoms •  Musculoskeletal: joint hypermobility, subluxations/dislocations, sprains,

muscle spasm, TMD, flat feet, finger deformities, arthralgia, myalgia, fractures, pain, proprioceptive deficits, kinesiophobia

•  Integumentary: stretchy skin, easy bruising, atrophic scarring, poor wound healing, frequent hernias

•  Cardiovascular: dysautonomia, postural orthostatic tachycardia syndrome Other: gastritis, IBS, incontinence, developmental delay, poor coordination, anxiety, mast cell activation, organ prolapse

•  Disability due to pain, fatigue, anxiety, depression (Castori, 2011; Colombi, 2015; Scheper, 2016; Tinkle, 2017)


Major Comorbidities •  Chronic pain: fibromyalgia, myofascial pain, OA, TMD

–  Hyperalgesia (Scheper, 2017)

•  Developmental delay in children •  Dysautonomia: POTS, thermoregulation, gut, sexual

dysfunction •  Mast Cell Activation Disorder: systemic inflammation •  Gastrointestinal disorders: GERD, IBS, malabsorption

syndrome (Tinkle, 2017)

•  Tethered cord syndrome (Henderson, 2017)


HSD Through the Life Span 1.  Hypermobile phase

–  Hypermobile joints –  Clumsiness/motor delay –  Constipation/diarrhea –  Abdominal hernias

2.  Pain phase –  Chronic musculoskeletal pain –  Strains, sprains, dislocations –  Unrefreshing sleep –  Chronic fatigue –  Memory/cognitive problems –  Gastric reflux, abdominal pain –  Paresthesias –  Tachycardia –  Incontinence/UTI

3.  Stiffness phase –  Widespread pain –  Fatigue –  Tendinosis/tendon rupture –  Chronic gastritis –  Stiffness

–  (Castori, 2011; Tinkle, 2017)


Evolving Diagnostic Criteria •  Un:l2017:

–  BeightonScale(Beighton,1973)mostodenusedforGJH•  Carter-Wilkinson(Carter,1964)•  Rotés/HospitaldelMar(Bulbena,1992)

–  VillafrancheClassificaDonusedmostlybygene:cistsforchildren:EDS-HT(Beighton,1998)

–  BrightonCriteriausedmostlybyrheumatologistsforadults:JHS(Grahame,1998)

•  NewcriteriaforhEDS:“The 2017 international classification of the Ehlers-Danlos syndromes.”(Malfait,2017)

•  NoclearguidelinesregardingHSD;Mustexcludeotherpoten:aldiagnoses


2017 hEDS Diagnostic Criteria Mustmeetall3criteria:

1.  Generalizedjointhypermobility

2.  Featuresofheritableconnec:ve:ssuedisorder•  Mustmeet≥2of3categories,A-C

3.  Absenceofexclusioncriteria•  (Malfait,2017)


BeightonScore≥5/9

•  2:Bend5thfingerback>90°•  2:Touchthumbtoforearm•  2:Elbowhyperextension>10°•  2:Kneehyperextension>10°•  1:Palmstofloor,kneesstraight

•  Prepubescent≥6/9•  Pubescent-50years≥5/9•  Over50years≥4/9

Pts with limited ROM for cause, add 1 point if ≥2/5 on the 5-Item Questionnaire: 1.  Can you now (or could you ever) place your hands flat on the floor without

bending your knees? 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? 4.  As a child or teenager, did your shoulder or kneecap dislocate on more than

one occasion? 5.  Do you consider yourself “double-jointed”?

(Malfait, 2017; diagram from Juul-Kristensen)

1: Generalized Joint Hypermobility



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2: Features of Heritable Connective Tissue Disorder

•  Musthave≥2ofthefollowing3categories:A.  Systemicmanifesta:ons

•  ≥5of12op:onsB.  FamilyhistoryC.  Musculoskeletalcomplica:ons

•  ≥1of3op:ons


2A: Systemic Manifestations i.  Unusuallysod/velvetyskinii.  Mildskinhyperextensibility(forearm)iii.  Unexplainedstraiae/stretchmarksiv.  Bilateralpapulesofheelv.  Recurrent/mul:pleabdominalherniasvi.  Atrophicscarringin≥2sitesvii.  Pelvicfloor,rectal,uterineprolapseviii.  Dentalcrowdingorhigh,narrowpalateix.  ArachnodactylybilateralSteinbergorWalkersignx.  Armspan/height≥1.05xi.  Mitralvalveprolapsemildorgreaterxii.  Aor:crootdila:onMeetsSystemicManifestaDonsifYESto≥5items


2A: Systemic Manifestations i.  Unusuallysodorvelvetyskinii.  Mildskinhyperextensibility(>1.5cmonvolar,non-

dominantforearm)iii.  Unexplainedstraiae/stretchmarksinany�or

prepubertal�w/osignificantweightchange


2A: Systemic Manifestations iv.  Bilateralpiezogenicpapulesofheel*v.  Recurrentormul:pleabdominalhernias(umbilical,

inguinal,crural;nothiatalhernia)*Piezogenic papules are sub-cutaneous fat herniations through the fascia; they may appear as white nodules only with weight bearing Malfait, 2017


vi.  Atrophicscarringinvolvingatleast2sites(notlikeclassicalEDS)

Malfait, 2017 Hypermobile EDS, Castori, 2015


Hypermobile EDS Classical EDS 2A: Systemic Manifestations 2A: Systemic Manifestations

vii.  Pelvicfloor,rectal,and/oruterineprolapseinchildren,�,nulliparous�w/omorbidobesity

viii. Dentalcrowdingandhighornarrowpalateix.  Bilateralarachnodactyly

–  Bilateral Steinberg OR bilateral Walker sign



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2A: Systemic Manifestations x.  Armspan/height*≥1.05xi.  Mitralvalveprolapsemildorgreaterxii.  Aor:crootdila:on,z-score>+2

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


2B: Family History •  1stdegreerela:veindependentlymeetsdiagnos:ccriteriaforhEDS


2C: Musculoskeletal Complications 1.  Pain≥2limbs,recurringdailyforatleast3months2.  Chronicwidespreadpainfor≥3months3.  Recurrentjointdisloca:onsorfrankjointinstabilityin

absenceoftrauma(aorb)a.  3+atrauma:cdisloca:onsofsamejointOR2+disloca:onsof2

differentjointsatdifferent:mesb.  Medicalconfirma:onofjointinstabilityat2+jointsnotrelatedto

trauma

•  Ifyesto≥1item(andnotduetootherconnecDveDssuedisorder,e.g.

lupus,RA),thenposiDveformusculoskeletalcomplicaDons


2: Features of Heritable Connective Tissue Disorder

•  Insummary,tomeetthecriterion:FeaturesofheritableconnecDveDssuedisorder

•  Musthave≥2ofthefollowing3categories:

A.  Systemicmanifesta:onsB.  FamilyhistoryC.  Musculoskeletalcomplica:ons(notexplainedbyanother

connec:ve:ssuedisorder)


3: Absence of Exclusion Criteria TomeetthisCriterion,all3ofthefollowingmustbeABSENT:

1.  Unusualskinfragility(shouldpromptconsidera:onofothertypesofEDS)

2.  Otherheritableoracquiredconnec:ve:ssuedisorder(e.g,lupusorRA)

3.  Neuromusculardisordersthatmaycausejointhypermobilitybymeansofhypotoniaorconnec:ve:ssuelaxity(e.g.,Marfan,otherEDS,OI,etc.)


Patient Examination •  Useabiopsychosocialapproach

•  Lookforcontribu:ngfactorsaswellassigns,symptoms,&involved:ssues



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International Classification of Functioning Disability and Health


Engelbert , 2017; Pacey, 2014 PhD thesis


Gastrointestinal

Dysautonomia

Fatigue

Psychological

Pain

Urogenital

Cardiovascular Neuromusculoskeletal

Symptom Profile Ninis, 2015


Where To Start? Focus on patient’s primary activity & participation restrictions Engelbert, 2017

•  Startatthebiggestcomplaint&worktowardsmallerissues•  Lookforthekeystructures/problemsthatarecausingthe

pa:ent’sdeficits


Principles of the Subjective Exam •  Thorough,biopsychosocialinterview•  Qualityoflifeassessment•  Psychosocialassessmenttoolsasindicated(e.g.,depression,anxiety,etc.)

•  Iden:fyhabitsandlifestylechoicesthatcontributetoprimarycomplaints–  E.g.,sleephygiene,postures,ac:vi:es

•  Whathashelped/harmedinthepast–  Avoidiatrogenicinjuries(Bovet,2012)


Outcome Measures •  BristolImpactofHypermobility–  Theonlycondi:on-specific–  55ques:ons–  ~10mintocomplete–  Validated–  Reliability,MCIDnotyetdetermined

–  (Palmer,2017)



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What is Different in HSD? •  TissuesinHSDaremorefragile(Tinkle,2017)•  Maybedamagedbymoresubtlestressesorcontribu:ngfactors

(“theprincessandthepea”)–  Areslowertoheal(Tinkle,2017)

•  Gravitycanhaveabigimpact•  Abnormalpainprocessing/hyperalgesia(Chopra,2017;Scheper,2017)•  Mul:plecomorbidi:escancompoundproblems

–  E.g.,Poorcoordina:on�microtrauma–  Fa:gueorpain�decondi:oning–  POTS:anxiety�muscletriggerpoints–  MCAD�excessiveinflammatoryresponse


Principles of the Physical Exam •  Iden:fy:ssuescausingsymptoms•  Iden:fystressorsaffec:ngthose:ssues

1.  Isthereanimbalancebetweenlaxjointsand:ghtmuscles?2.  Doespoorposture,alignmentorgravitystressjoints/muscles?3.  Arebodymechanicsstretchingorstressingjoints/muscles?4.  Ispoorpropriocep:onormotorcontrolleadingtoinstability?5.  Whatiscausingmuscletriggerpoints?

•  POTStest,ifappropriate


Interventions •  PaDenteducaDon!!!•  Exercise(appropriate)•  Painmanagement,focusingonself-care•  Assis:veandortho:cdevices?•  Manualtherapy?

(Engelbert,2017;ClinicalGuidelines;Chopra,2017)


Patient Education EducateandempowerthepaDent/family

•  Paineduca:on&self-management•  Bodymechanics/ergonomics

•  Ortho:cs,braces,&splintsif/whenneeded•  Appropriateexercise/ac:vity•  Sleephygiene&fa:guemanagement•  POTSself-management•  Psychological&socialwellness,relaxa:onstrategies•  Dietandfluidmanagement•  Otherissues:GIdysfunc:on,MCAD,incon:nence,etc.Refertootherprofessionalsasappropriate(Engelbert,2017;Chopra,2017)


Exercise Prescription •  Propriocep:on,stabiliza:on,motorcontrol&coordina:on

•  Strengthening•  Appropriatestretching,stabilizingasneeded•  Cardiovascularcondi:oning

–  “GradedExerciseTherapy”•  (POTS-specificexercise)(Engelbert,2017;Palmer,2014)


Pain Management (in Clinic) •  Physiologicalquie:ng•  Biofeedback•  TENS(trialforhomedevice)•  Manualtherapy•  Dryneedling(iflegalinyourstate)•  Shi<topa=entself-management

–  Avoidextensiveuseofmodali=esintheclinic(Chopra,2017;Engelbert,2017)



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Medications •  Li|ledefini:veresearchevidenceformedica:ons•  NSAIDsfortrueinflamma:on

–  NSAIDsmayslow:ssuehealing,aggravateGIsymptoms•  Tricyclics,an:-seizure,SNRImedsforneuropathicpain•  Topicalanalgesicsandan:-inflammatorymedica:ons•  Acetaminophen•  Cau:ons:

–  Opiatesforshorttermuseonly–  Musclerelaxersmayaggravateinstability


Chopra, 2017 Tinkle, 2017

Barriers To Treatment •  Nega:vepastexperienceswithPT(Bovet,2016)•  Iatrogenicinjuries(Bovet,2016)•  Barrierstodoingexercise:(Simmonds,2017)

– Fear– Pain– Fa:gue


Applying Evidence-Based Practice

•  Althoughmoreresearchisavailablenow,manyques:onsaboutop:maltreatmentremainunanswered

•  Wethereforerelyontheprinciplesofevidence-basedprac:ce


Review •  Hypermobility-relatedcomplaintsarecommon

–  Hypermobilityaffectsmostbodysystems•  The2017diagnos:ccriteriaforhEDSarerestric:ve

–  ConsiderHypermobilitySpectrumDisorders•  PTexamina:onshouldbebiopsychosocial(ICFmodel)

–  Accountfor:ssuefragility–  Lookforcontribu:ngfactors

•  PTmanagementshouldaddresscontribu:ngfactorsPT for Hypermobility Spectrum Disorders 46

Case Examples •  Theremainderofthissessionwillbethroughcaseexamples–  Pediatriccase:StephanieSabo,presentedbyL.Russek

•  Addressesassessment,motordelays,motorcontrolissues,bracing–  Adolescentcase:JaneSimmonds

•  Addressesassessment,stra:fiedmanagement,POTS,fa:gue,GIproblems,psychosocialissues

–  Adultcase:HeatherPurdin•  Addressesassessment,chronicpain,MCAD


Managing Pediatric Patients with HSD

Case and slides provided by Stephanie Sabo, PT, MPT



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Common Presentation in Children

•  Difficultywithprolongedwalkingorstanding•  Legpainworseineveningoratnightandwith/aderac:vity•  Legpainsymptomsrelievedbyrubbingorheat•  Historyof“growingpains”•  Difficultysi~nginchairatschool,figi:ng•  Trips,falls,beinglabeled“clumsy”(Bernie,2011;Adib,2005)•  Grossmotordelays(Tirosh1991,Davidovitch1994,Bernie2011)•  Coordina:ondifficul:es(Kirby2007)


Developmental Condition vs. Hypermobility in Young Children?

•  Standardizedmotorcompetencytestsarenotadequate–  Donotcapturethefullimpactofjointhypermobilityonmotorfunc:onandqualityoflife

–  Qualityofmovementandcompensa:onsshouldbethefocusoftheassessment

–  Childrencanodenachieveindividualmotoritems,butnotefficientlyorrepe::vely

–  (Remvig,2011)PT for Hypermobility Spectrum Disorders 50

Prevalence

•  34-35%inschoolagechildren•  (Arroyo,1988;Remvig,2011;Junge,2013)

•  NOgenderdifferencefoundat10y/o

•  (Remvig,2011)

•  Postpubertalgenderdifferences•  (Quatman,2008)


Additional Hx: Prior Therapies •  Itisimportanttoaskaboutwhetherthechildhashadpriortherapyand

whetheriswasperceivedashelpful–  Some:mespriortherapyreportedtobeofli|lebenefitandworsenedsymptoms–  Askifhypermobilitybeingtargetedinpriortherapies;ifhypermobilitywasnot

recognized,lesslikelythattherapywassuccessful•  Authorssuggestthefollowingpoten:alreasonsforpreviouslyfailed

therapies:–  Techniquesbeingtooaggressive–  Dura:ontoshort–  Frequencytohigh–  Failingtoappreciatefullscopeofinterven:onsrequired

(Keer, 2003; Hakim 2003)


Referral Sources •  Pediatrics•  Rheumatology•  Gene:cs•  DevelopmentalPediatrics•  Orthopedics•  PainManagement


Referral Sources •  TheEDS/hypermobilitypopula:ondoesn’tfitanyspecialtyarea

perfectly•  Jointhypermobilityand/oritsimpactonfunc:onmaybeiden:fied

duringtreatmentofanothercondi:on,soitishelpfultoscreen•  Children:

–  Mayormaynotcomewiththisdiagnosisonthereferral–  Mayhaveseenmanyphysiciansorservicesmul:ple:meslookingfor

answers•  Pa:entandfamilyodenexpressfrustra:onswithdelayindiagnosis

orlackofexplana:onforsymptoms.



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•  MotherwithhEDS•  Over2-hourdrivefromtherapy•  Parentsseparated•  Limitedinvolvementofbiologicalfather

IMPAIRMENTS PARTICIPATION

PERSONAL FACTORS ENVIRONMENT

•  Paininankles&knees,7/10-10/10–  Worsewithac:vity&atnight

•  Headaches•  Poorposture•  Poorbalance•  Abnormalgait•  Jointhypermobility•  Muscle:ghtness:HS,heelcords•  Poormotorcontrol&motorskills

•  Difficultywalking>20minutes•  Difficultysi~nginchairatschool

•  Troubleatschool•  Troublewithsocialfunc:on,playingwithfriends

Tommy,age4

•  Anxious•  A|en:ondeficit/hyperac:vitydisorder

•  Self-imageas“clumsy”anduncoordinated

I want to play just like all the

other kids

ACTIVITY


Tommy: Initial Presentation •  4yearoldmalereferredtoPTbyPediatricRheumatologist

–  Onsetofsymptoms~1yearago,withincreasedcomplaintsinthelast6months

–  Motherwithbehavioranda|en:onconcerns:“doesn’teversits:ll”

–  SenttoRheumatologybyprimarycarephysician–  6monthslaterseenbyGene:cs(perRheumatologyReferral)

•  Motherwithhypermobility/EDS

Insert subject


Tommy: Initial Presentation •  Paininanklesandknees2-3x/week

–  7/10onNumericRa:ngScale(NRS)–  increaseswithphysicalac:vity–  increasesinevening/night

•  Upto10/10onNRS;motherhastakenhimtoemergencydepartmentforpain

•  Bilateralhipandkneexrays-normal•  Usesrubbingandmedica:onforrelief(e.g.,overthecounter

TylenolorMotrin)•  Pa:entalsohascomplaintsofheadaches

Insert subject


Tommy: Findings •  Posture:

–  Shouldersforward/rounded,increasedlordosis/anteriorpelvic:lt,genurecurvatum,calcanealvalgusandpesplanus,genuvalgum,externalrota:onatfeet

•  Gait:–  Decreasedkneeflexion,overprona:onofrearfoot-midfoot,audiblefootslap,

ERatfeet•  ROM:

–  HipER85o,HipIR70o,KneeExtension+8o,AnkleDorsiflexion30o,Ankleinversion60o

•  Flexibility:–  Hamstrings-40o;heelcords20o


Measuring Flexibility •  Assessmuscleflexibility

–  Differen:atemuscleflexibilityandjointmobility

•  Isolatemusclelengthvs.jointlaxity–  Reinforceneedtostrengthenmusclesaroundloosejoints

•  Muscularimbalances–  Commonlyseelimita:onsinhamstringsandgastrocnemiusmusclegroups


Tommy: Findings •  Singlelimbstance:right:4-5seconds;led:4-5seconds

–  Notedunsteadiness,withlateraltrunklean,trunkflexion,increasedanklestrategies,andadduc:onofoppositeLE

•  Bridgeinhooklyingfor3secondsx3reps–  Notedunsteadinessanddecreasedeccentriccontroluponlowering

•  Miniwallsqua~ngfor10secondsx3reps–  Noteddifficultywithmiddlerangecontrol:pushingkneeintogenu

recurvatumandpelvisforwardintoAPTwithoutcueing•  Modifiedheelraises:singleUEsupportfor3secondsx3reps

–  Notedtoini:allycompletethroughfullankleplantarflexionbutlockingoutankles;whencompletedinmiddlerange,demonstratedunsteadinessandsteppingstrategies



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Measuring Strength •  Assesscorestrength

–  Qualityofmovementisveryimportant:focusonac:va:onofkeymusclegroupsthroughhips,coreandpelvis•  Forexample:bridgingmayini:atethroughtrunkextensionandlackcoreac:va:on

–  Under-u:liza:onofkeyposturalmusclesiscommon-Transverseabdominus,mul:fidus,gluteals,serratusanterior

–  Co-contrac:onisodenlacking/decreased–  (Falkerslev,2013;Jensen,2013;Grahame,2009)


Common Strength Deficits

•  Pts have difficulty maintaining stable, neutral postures and effectively controlling movement

•  Efficient use of proper positions to accomplish tasks is not natural

•  Pts often initially demonstrate a full crab walk position when asked to perform a bridge

•  Even athletic patients can have difficulty sustaining a neutral core with simple bridging


Strength

•  Assessextremitystrengthusingfunc:onalstrengthtes:ng(nomanualmuscletes:ng):–  Alltes:ngshouldbedoneinneutraljointposi:ons,where

strengthandenduranceareodendecreased–  Avoidposi:onsatendofjointrange

•  lockedjointsareassociatedwithligamentousdependency–  Assessthequalityofmuscleco-contrac:on–  Lookformuscleimbalances

•  Oden,keyposturalmusclesareveryweakandnotproperlyu:lized


Tommy: Ankle Strength

• With heel raises, Tommy positions ankles in end range to help stabilize

• He is unable to maintain a static position


Tommy: Leg Strength Wall sits: •  Note wide base of support with genu

valgum and increased pronation/ calcaneal valgum and knee flexion

•  He has muscle endurance limitations and genu recurvatum with knee extension

•  He has overall poor quality of movement and poor mid range control


Balance and Proprioception •  AssessbalanceandpropriocepDon

–  Observecompensatorypa|ernsofmovement–  Assessforalteredawarenessofbodyposi:oninspace(common)

•  Mul:plestudiesconfirmpropriocep:vedeficits:–  Lowerlimbpropriocep:onisdecreasedinBenignJointHypermobilitySyndrome

–  Decreasedpropriocep:oninUEjoints(fingers)(Smith,2013;Rombaut,2010;Fatoye,2009;Sahin2008;Schubert-Hjalmarsson,2012)



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Balance and Proprioception

Single leg stand: •  Observe duration of

single limb stance AND the quality of the movement


Balance and Proprioception

Devia:onstolookfor•  Lateral trunk lean (gluteus medius weakness) •  Unsteadiness •  Trendelenburg (hip drop) •  Trunk flexion •  Adduction of opposite leg using other limb for stability •  Increased ankle strategies •  Pronation through midfoot •  Locking out knee •  Internal rotation of stance limb


Tommy: Intervention/Treatment Plan •  FrequencyforPTservices:recommendedfollowupvisit

~4-6weeksaderevalua:on;however,pa:entwaslosttofollowupfor6monthsduetofamilyhavingsocialconcerns

•  EducaDon:jointprotec:on,ac:vitymodifica:on,sodjoints•  OrthoDcs:fi|edwithortho:csatevalua:on•  HEP:bridging,resistedhipabduc:on,squats,passive

hamstringstretch•  Treatments:completedHEP4x/weekfromevalua:onun:l

6monthfollowupvisit


Basic Principles of Intervention •  Beginlowlevelforbaseline

–  Odenneedtobeginingravityeliminatedposi:onsand/oruseisometrics

•  Progressatslowrate•  Considerthewholeperson

–  Notjustonejointata:me–  Nonmusculoskeletalmanifesta:ons

•  Customizetreatmentbasedonindividualneedsandgoals (Celletti, 2013)


Joint Protection •  Posturalre-educa:onandawareness

–  Reducesstressonmusclesandjoints–  Reducepainandfa:gueover:me

•  Focusonawarenessofneutraljointposi:ons.Avoid:–  Kneeandelbowhyperextension–  Anteriorpelvic:lt/hiphanging(forwardand

laterally)–  Wsi~ng–  Roundedshoulderandforwardhead (Rombaut, 2012)


Joint Protection

•  Pa:entsshouldbeinstructedhowtoavoidoverstretchingjointsandtoreducemechanicalstressonjoints–  Forexample:individualscanprac:cefindingandmaintaininga“sodknee”

posi:on,withkneesneutralorveryslightlyflexed

•  Lackofposturalmuscleac:va:onreinforcesposturaldeficits•  Avoidexcessivejointmovement,whichcouldleadtotears

andruptureofthesod:ssuessurroundingthejoint•  (Rombaut,2012)



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Controlled Stretching •  Paya%en(ontotechnique:

–  Stabilizesurroundingjointstostretchspecific:ghtmuscleswithoutstressingotherstructures

•  Contrastpa:ent’sini:alposi:onforstandinghamstringstretch(top)withperformanceAFTERinstruc:oninproperform(bo|om)


Orthoses: Summary of Evidence •  Thereislimited,inconclusiveevidence,evenasitrelatestothegeneral

“flatfeet”diagnosisinthepediatricpopula:on•  Systema:creviewsfindthatstudiesusedifferentpa:entpopula:onsand

widelyvaryingtypeoforthoses (Evans,2011)•  Noinforma:ontoconsistentlysupportusingortho:csornot•  Planusfootinearlylifeisassociatedwithadultdegenera:vejointdisease

andinterven:onisindicated (Gross,2011)•  Anecdotalexperiencewithuseofortho:csinthehypermobilepopula:on

isveryposi:ve (LocalCCHconsensus,2016)


Minimal Control Orthotics

•  Providesomesupporttopronated(flat)feet

•  Canreducestressonalllowerextremityjoints

•  Customizable

Cascade DAFO Fast Fit http://cascadedafo.com

Vasyli orthotics www.vasylimedical.com


Models of Therapy •  ConsultaDve:Emphasisisonhomeexerciseprogramsthatareperiodicallyupdatedbythetherapist

•  Intermediate:1-3x/wk•  Intensive:Frequentsessionsoverashortperiodof:me


Consultative Model •  Emphasisonhomeprogrammingandselfmanagement(odenusedinini:alstages)–  Homeexerciseprogramestablishedatini:alsession–  Educa:onwithchildandcaregivers:posture,jointprotec:on…–  Ortho:cs,ifindicated

•  Followupplanestablished–  Individualized,emphasizingselfmanagement–  Frequencyoftherapyisdeterminedattheevalua:on–  Follow-upoden4to6weekslater


Intensive Treatment Model •  Mul:disciplinaryprogram,including

OT,PT,MD,Psychology,ChildLife–  Seekstoprovideknowledge,aswellas

aphysicalboost–  Providestoolsforselfmanagement–  ProvidessocialInterac:ons–  Par:cipantsbecometheirownexperts–  Facilitateshomeadherenceand

lifelongmanagement–  IncreasesindependencewithHEP (Bathen, 2013; Birt, 2013)



2/23/18


Example Intensive Model •  2-weekprogram•  Par:cipantsgroupedbygenderandage•  AllwithEDSand/orjointhypermobilitycondi:ons•  Benefits:

–  IntensiveexposuretoPTempowersparentsandchildren–  Par:cipantsgainknowledge,skillsandconfidencetoadaptexercisesandself-

managesymptoms

•  Adolescentsfacedthemostdifficul:eswithfinding:me,privacyandmo:va:ontodotheexerciseprogramathome


Intensive Model (2 week program) •  Programstarted2011

•  Outcomemeasurespreandpost:–  PedsQL,COPM,TampaScaleKinesiophobia

•  Psychologyusescogni:vebehavioraltherapyandgroupsessions

•  MusicincorporatedinexercisesessionsandwithChildLife

•  ChildLifehelpspa:entswritele|ersofencouragementtothenextgroupcomingintotheprogram

•  HEPbooksareconstructedwithpicturesofthepa:entdoingtheexercises

•  (Celle~,2013;Castori,2012)

PT

MD

Child Life

Aqua-tics PSY

OT



Outcome Measures •  CanadianOccupa:onalPerformanceMeasure(COPM)

•  PediatricQualityofLifeMeasure(PedsQL)•  TampaScaleforKinesiophobia(TSK)


Tommy: Outcome At 6 months: •  Leg pain decreased from

daily to 1-2x/month •  Improved quality of

movements •  Improved ability to sustain

hold counts •  Decreased postural

compensations


Summary of Pediatric Case •  Hypermobilepa:entsareinmostpediatriccaseloads•  Func:onalimpactofjointhypermobilitypainandfa:gueissignificant

andisodenunder-appreciated•  Standardtherapyfrequentlyfailsthesepa:ents•  Specific,targetedjointstabiliza:on,neuromusculartrainingand

educa:onareneeded•  Lowintensityandslowprogressionofinterven:onisindicated•  Livescanbechanged!



2/23/18


Thanks To: Stephanie Sabo PT, MPT

Physical Therapist II [email protected]

•  Cincinnati Children's Hospital Medical Center Joint Hypermobility Team 2014. “Identification and Management of Pediatric Joint Hypermobility” CCHMC EBDM Website Guideline 43 pages 1-22.

Available at https://www.cincinnatichildrens.org


Managing Adolescents with HSD and HEDS Jane Simmonds DProf, MCSP, MACP, SFHEA

Outline of Adolescent Case

•  Consider issues which arise in adolescence

•  Stratified approach to management Hypermobility Spectrum Disorders and Hypermobile Ehlers Danlos Syndrome

•  Case study

•  Plans for research


Reflect on the key physiological and psychological changes during adolescence 1. Hormonal changes

2. Growth – Peak Height Velocity

3. Developing independence Seminal study (Kirk,1967) described three quarters of patients developed symptoms prior to age 15 years Hypermobile adolescents 2 times more likely to develop musculoskeletal problems than non hypermobile counterpart. Risk increases 12 fold if overweight (Tobias, 2013)


DS

Joint Laxity

hEDS

Asymptomatic Mild Signs Significant Signs & Symptoms & Symptoms

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

GJH

(Castori, 2017)


SPECTRUM

SIMPLE/ACUTE INTERMEDIATE COMPLEX/CHRONIC



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STRATIFIED MANAGEMENT

SIMPLE/ EARLY

Episode of acute musculoskeletal injury, sprains, dislocation, subluxations

enthesopathies

(Pacey 2010; Smith 2005)



SIMPLE/ EARLY


enthesopathies

(Pacey, 2010; Smith, 2005)



SIMPLE/ EARLY


enthesopathies

SIMPLE/ EARLY Ice, electrotherapy, tape, support, exercise,

screen, education – rehabilitate and prevent

PT for Hypermobility Spectrum Disorders 93 PT for Hypermobility Spectrum Disorders 94


SIMPLE/ EARLY


enthesopathies



INTERMEDIATE Recurrent episodes, series of episodes at

different sites, deconditioning, some central/ peripheral sensitization, mild

systemic conditions



SIMPLE/ EARLY


enthesopathies





systemic conditions

INTERMEDIATE Physiotherapy modalities have temporary

effect, no effect or exacerbate Modified / adapted approach

Functional Restoration

(Engelbert 2017; Scheper 2017)



2/23/18


Subjective Assessment

Listen and develop the therapeutic

alliance

Listen to the history carefully……. Explore expectations with young person and parent Identify problems – prioritize –  Pain – local/ general/ acute/ chronic (sensitization) –  Joint instability – subluxations, dislocations, clicking –  Fatigue – sleep, fluid, diet –  Anxiety - Low mood/ depression –  Gastrointestinal dysmotility –  Dysautonomia – Postural Tachycardia Syndrome (POTS) –  Gynaecology and bladder problems –  Allergies

Explore impact –  Physical activity/ Sport/ Hobbies Physical Education –  Social, School, General health –  Family history and thorough developmental history **



Identify barriers to

rehabilitation





Identify personal strengths and

interests to drive rehabilitation




Objective Assessment Careful active and passive joint range and muscle length Functional assessment * Posture and gait – compensatory patterns Sit to stand/ squat – gluteal, quadriceps Single leg dip Heel raise – tibialis posterior Balance – Single leg / Y Balance Test / Hop/ Jump Repositioning tests – proprioception/ kinaesthetic Strength/ activation (careful testing* - through range) Test for POTS (standing test…refer on)

Observe Carefully


Dysautonomia/ Postural Tachycardia Syndrome (POTS)

Near syncope on standing Venous pooling with colour changes Tachycardia –standing and changing position Can result in massive anxiety Excessive heart rate on exercise Hyperventilation Heat intolerance Nausea Mathias (2011); Kizilbash (2014)


Dysautonomia/ Postural Tachycardia Syndrome (POTS)

Near syncope on standing Venous pooling with colour changes Tachycardia –standing and changing position Can result in massive anxiety Excessive heart rate on exercise Hyperventilation Heat intolerance Nausea

Mechanisms Illness

Hormonal Deconditioning Hypermobility Mathias (2011); Kizilbash (2014)



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Chair

Walking

Chair

Artefact corrected heart rate


Autonomic Testing Typical response POTS patient

Tilt Test Rise of ≥30 BPM adults Rise of ≥ 40 BPM in adolescents

Time Time Mathias (2011); Raj (2013); Kizilbash (2014)


Autonomic Testing Typical response POTS patient

Time 10 Minute Standing Test – Alternative to Tilt Test

Time

Tilt Test Rise of ≥30 BPM adults Rise of ≥ 40 BPM in adolescents

Mathias (2011); Raj (2013); Kizilbash (2014)


IMPAIRMENTS ACTIVITY PARTICIPATION


Meet Helen 15 years

•  Abletowalkfor15-20mins•  Strugglingwithwri:ng•  Strugglingtocarryschoolbag•  Strugglingonpublictransport–duetosyncope

•  Missingschool•  Struggleswithdancing,notswimmingorplayingnetball

•  Reducedsocialac:vitywithfriends(mainlyonline)

•  Motherwithfibromyalgia•  Youngerbrother–hEDS•  Lovelyfriends

•  Female•  Highachiever–Astudent•  Lowconfidence

•  Widespreadhypermobility++•  Recurrentshoulder,kneefingersubluxa:ons

•  Persistentpainandfa:gue•  Decondi:oned+•  Anxiousandlowmood•  Presyncopeandfastheartratewhenstanding(POTS)

•  Dysmenorrhea•  Bloa:ngandearlysa:ety

PMHx: Hypermobility detected in early life. Enjoyed being active. Recurrent injuries, subluxations. Physiotherapists treated single areas. PC: Never got on top of problem, injury after injury….now not coping ….

I want to do my exams…play sport,

dance …..go shopping


Gastrointestinal

Dysautonomia

Fatigue

Psychological

Pain

Urogenital

Cardiovascular

Neuromusculoskeletal

Symptom Profile Ninis (2015)


IMPAIRMENTS ACTIVITY PARTICIPATION


Meet Helen 15 years

•  Pa:entSpecificFunc:onalScale(PSFS)

•  GoalA|ainmentScale

•  PedsQL•  (Physicalfunc:on,social,emo:onalandschool)

•  SelfEfficacyScale(VAS)•  CopingScale(VAS)

•  PainVisualAnalogueScale(VAS)

•  PedsQLMul:dimensionalFa:gueScale

•  SingleLegDip•  ModifiedStarExcursionBalanceTest(YBalance)

•  TiltTest/StandTest•  PedsQLGastroIntes:nalSymptomsandWorryScales

PMHx: Hypermobility detected in early life. Enjoyed being active. Recurrent injuries, subluxations. Physiotherapists treated single areas. PC: Never got on top of problem, injury after injury….now not coping ….

Relevant Outcome Measures



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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)


(Simmonds,2017)

Surveyof946paDentsUK

ExperiencesofPhysiotherapy


Liaise with school teachers Dance teacher Coaches


Dislocation and

Subluxation

Panic = muscle spasm Position the joint Analgesic Heat - Breath – Relax - Distract Give it time Do your usual thing… Ice, analgesia Support for a few days…carry on


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

Expertopinion-Judicioususe**Canbeveryhelpfulforwhenreturningfrominjuryandforfunc:on

Splinting and Tape



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Orthotics and Footwear

Cochrane Review Recommends supportive footwear and orthotics for flexible flat feet (Evans, 2011)

Improved gait efficiency in young people with Developmental Coordination Disorder and Hypermobility Syndrome (Morrison, 2013)

Expert opinion - Judicious use of orthotics or supportive footwear/ heal cup/ high tops


Management of POTS •  Advice – fluids, electrolytes/salt, compression tights

–  Positioning, anti syncope manoeuvres •  Medications prescribed (Midodrine, Fludrocortizone, Beta blockade)

•  Respiratory physiotherapy – hyperventilation •  Anxiety management – psychological support •  Small meals, low carbohydrate diet and FODMAP •  Graded cardiovascular exercise and resistance training – focus on lower limbs

–  Morning exercise •  Incorporating exercise to manage joint instability •  Recumbent to upright exercise

(Mathias, 2011; Fu, 2011; George, 2013; Kizilbash, 2014)


PREMISE FOR EXERCISE: Long term benefits of improved physical fitness and lower limb strength counteract orthostatic intolerance"•  Increase blood volume"•  Increase cardiac output"•  Enhance vascular compression due to increased muscle mass and tone"•  Improve endothelilial function"•  Improve baro reflex function "

"


PREMISE FOR EXERCISE: Long term benefits of improved physical fitness and lower limb strength counteract orthostatic intolerance"•  Increase blood volume"•  Increase cardiac output"•  Enhance vascular compression due to increased muscle mass and tone"•  Improve endothelilial 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!


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) •  Considermotorcontrol(Roussel,2009)andengagethought(Boudreau2010)•  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)


For Helen



2/23/18


Pilates ….. Dance

(Simmonds, 2017)

For Helen


For Helen




SIMPLE/ EARLY Episode of acute musculoskeletal injury,

sprains, dislocation, subluxations enthesopathies





systemic conditions




COMPLEX LONG TERM Chronic, longstanding, severe, unremitting

pain with profound deconditioning/ comorbidities, disability



SIMPLE/ EARLY Episode of acute musculoskeletal injury,

sprains, dislocation, subluxations enthesopathies





systemic conditions




COMPLEX LONG TERM Chronic, longstanding, severe, unremitting

pain with profound deconditioning/ comorbidities, disability

COMPLEX/ LONG TERM Multi disciplinary management programme using functional andcognitive behavioural

approaches (Bathen 2014)



2/23/18


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

Condition specific education, advice and support

Give HOPE, agree GOALS, and be CREATIVE

Treat the treatable – prioritise

Pain and fatigue management (young person and parent)

Improve movement – use your hands

Strengthen weak muscle groups

Improve cardiovascular fitness


Future Research and Education

•  Understand the factors which influence development of symptoms •  Validate and agree outcome measures •  Understand what works best for young people •  Intervention trials •  Education patients and professionals


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


[email protected]


Adults with HSD

ByHeatherPurdin,MS,PT,[email protected]

Guido Daniele http://www.guidodaniele.com

PARTICIPATION

PERSONAL FACTORS

ENVIRONMENT

•  Widespreadhypermobility•  AllogradHSandAchillesusedtostabilizeRshoulderanditsdisloca:ngagain

•  PastHxlumbardiscectomy•  Weightgain40#sincestoppingpainmedslastyear

•  UterineProlapse•  Dizzinessandtachycardia•  TMJandribsubluxa:ons•  Widespreadhives&swelling

•  Strugglingtocareforfamilyandhome

• ⬇︎Churchinvolvement

•  Female•  Driven•  Frustratedbymedicalcare•  KidswithhEDSneedsupport

•  Suppor:vehusband•  Sickkids/neardeathexperience

•  Recentlyrelocated•  StresslevelhighduetonewChurchassignment

I need to look after my

family

ACTIVITY •  Limitedarmfunc:onduetoshoulderinstability

•  Limitedmobility•  PainlimitsADLandIADL

BODY STRUCTURE/FUNCTION

132

MeetMindy,37y/ohomemaker



2/23/18


Gravity affects EDS/HSD Pre-operaDveshoulder

subluxaDonNormalshoulderx-ray


Gravity is a problem InferioranteriorsubluxaDon Deltoid,uppertrap,rotatorcuffset


Subluxed Shoulder Causes Neck/Jaw Malalignment

Downsloped R shoulder pulls lower neck to R and head corrects to L

Set shoulder brings head back to neutral


Types of Pain •  Iden:fythetype(s)ofpain

– Nocicep:ve:mechanical,inflammatory– Neuropathic:peripheral,(central)– Centralsensi:za:on:neuroplas:city

•  Differentpainistreateddifferently

136 PT for Hypermobility Spectrum Disorders

Pain Source

Quality Medications/Referral

Exercise Other Intervention

Mechanical Localized, occurs with certain motions. Sensitized by inflammation

NSAIDs? Primary MD

Strength/Mobility Balance throughout kinetic chain Proprioception

Bracing, taping, Ergonomics training, Muscle setting

Inflammatory Burning in broad area not dermatomal, bruisy

NSAIDs, Allergy meds, MCAD Primary MD, Allergy

Pool Proprioceptive input Mindful movement

Modalities, Tool assisted scraping, Dietary guidance

Nerve Burning in a peripheral nerve or dermatomal pattern, searing

Gabapentin, Lyrica, SNRI’s, LDN, Triptylines Primary MD, Neuro, Nutrition?

Nerve flossing Mindful movement Cardio External focus

Posture training to address entrapped nerves, positions of slack

Central Sensitization

Whole body pain, difficult to localize, wind-up, allodynia

SNRI’s, triptylines, LDN Primary MD, Psych

Cardio Mindful movement External focus

Meditation Breathing Biofeedback 137


(Chopra, 2017; Castori, 2012)

What is MCAD?

PT for Hypermobility Spectrum Disorders 138 https://ehlers-danlos.com/pdf/Maitland-EDNF-Vegas-Mast-Cell-Activation-Disorders-S.pdf.

Slide from Anne Maitland’s presentation at 2017 EDS Global Learning Conference


2/23/18


MCAD Presentation


Slide from A. Maiteland, EDS Global Learning Conference, 2017 https://ehlers-danlos.com/pdf/Maitland-EDNF-Vegas-Mast-Cell-Activation-Disorders-S.pdf.

Mindy’s Symptoms -  Hives,redrash-  Brainfog-  Diges:veissues-  Bladderirritability-  Swellinginlimbs,supraclavicular

-  Elevatedurinehistamines


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

Manage Mast Cell Activation •  Iden:fytriggers:

–  Alcohol,heat,medica:ons(NSAIDs,an:bio:cs,narco:cs),allergens–  Foodsensi:vi:es–  Excessiveexercise

•  Managephysicalandemo:onalstress•  Exerciseregularly,inspiteoffa:gue•  Advocateforpa:entge~ngonMastCellStabilizersandmeds

thatblockchemicalmediatorslikean:-histamines–  sendpa:enttoMDwithresearchar:cles

–  (Akin, 2010; Moulderings, 2011; Seniviratne, 2017; Theoharides 2015)


Dietary Advice •  37%ofpeoplewithIBShavehEDS•  FODMAPdietmaybehelpful(expertopinion)

–  Fructose,Oligosaccharides,Disaccharides,Monoamines,andPolyols–  BacteriathriveonFODMAPfoods/dysbiosiscausesMCAD–  MonashUniversity,MelborneAustrialia(Fikree,2017)

•  Otherrecommenda:onsarespecula:ve–  HeidiCollins,MDdiet–avoidchemicals,gluten,takesupplementstoimprove

nutri:on,reducehistamines–  Lowhistaminediet(especiallywithMCAD)–  Avoidhardfoodsandexcessivejawmovements(ice,gums,etc.)toavoidTMD–  Avoidbladderirritantfoods(e.g.,coffee,citrusproducts)–  Avoidlargemeals(especiallyofrefinedcarbohydrates)


Sources of Pain •  Don’tjustlookforsymptoma:c:ssue–findthecauseof:ssuesymptoms–  E.g.:UTTrPmaybecausingHA,butpoorpropriocep=onandDNFmotorcontrolcauseUTTrP

•  Chronicpain-consider–  Psychosocialfactors–  Stress–  Childhoodtrauma



2/23/18


HSD Through the Life Span 1.  Hypermobile phase

–  Hypermobile joints –  Clumsiness/motor delay –  Constipation/diarrhea –  Abdominal hernias

2.  Pain phase –  Chronic musculoskeletal pain –  Strains, sprains, dislocations –  Unrefreshing sleep –  Chronic fatigue –  Memory/cognitive problems –  Gastric reflux, abdominal pain –  Paresthesias –  Tachycardia –  Incontinence/UTI

3.  Stiffness phase –  Widespread pain –  Fatigue –  Tendinosis/tendon rupture –  Chronic gastritis –  Stiffness

–  (Castori, 2011; Tinkle, 2017)


Adverse Childhood Experience •  Specificadversechildhoodexperiences(ACEs)increaseriskofchronicpain–  Verbalorsexualabuse,parentalpsychopathy,Earlyparentaldeath

–  Adults(Sachs-Erickson,2017)–  Childrenandadolescents(Nelson,2017)

•  MechanismmaybeviaalteredHPAaxisandautonomicdysfunc:on(Elbers,2017)


Set Realistic Goals •  Pa:entavoidsdisability/reversesdisability•  Fewer“bad”days•  Selftreatmentstrategiesreduceneedformedicalinterven:on

•  Increasedself-efficacy


Mindy’s Goals •  Goodshoulderalignmentwithoutshouldersubluxa:on:60%met•  Cantalkunlimitedbyjawpain/locking:Met•  ImprovedstabilityBhipstoallow1hourofpainfreewalkingwith

restbreaks:Metwithpain,nopainflarewith30minutesofwalking•  IndependentwithprogressiveHEPwithself-reliefforpain:Metfor

basicprogramforshoulders,neck,ribs,hips,core•  Improvedbiomechanicsandstabilityofribcageforfull,painfree

ven:la:on:Mostlymet,selftreatsribmalalignment•  Improvedmobilityandreducedpaininheadandneck:Partlymet,

needscuestostabilizeneckduringarmuse


Pain Self-Management •  Techniquestodecreasepain:

–  Painneuroscienceeduca:on(e.g.,“ExplainPain”)–  Cogni:vebehavioraltherapy–  Relaxa:on–  Self-carewithheat,ice,TENS

•  Techniquestodecreaseinjury–  Jointprotec:onstrategies–  Bracing/splin:ng,etc.


Body Mechanics • Postureandergonomicsatschool/work/home• Sleepingposture,surface,support– Engagingmusclesbeforemoving– Assis:vedevices(e.g.,pens,tools,etc.)



2/23/18


Biomechanical Differences •  Shoulderse~ng

–  Cueupinsteadofdown–  Ballbackinsocket,notjustscapulae–cueupsidedown“G”

•  Bicepslongheadsubluxa:on–  ReduceERandabduc:onposi:ons

•  Hipposteriorsea:ng–  Nohipflexorusewithoutglutuseor–  Alterposi:onofexercisetoassiststability/posteriorsea:ng

suchasinlongsi~ng


Biomechanical Differences •  Ankleprotec:on

–  Bedsheetscansubluxtalusanteriorly–  Si~ngonankles

•  Patellofemoralinstability–  TypicalissuesOsgoodSchla|er’s,trackingissues

•  Elbow–ulnarnervesubluxa:onusuallyat90+degrees

•  Wrist–carpalsubluxa:ons,rota:ons


Biomechanical Differences •  Craniocervicalinstability

–  Odenjustlongholdintomobiliza:ondirec:onisenoughtonormalize

•  SpineInstabili:es–  Spondylolisthesis–  Retrolisthesis

•  Ribse~ng–  ½inhalebeforeliding–  Ifdepressedribs,inhalewithac:vity–  Ifelevatedribs,exhalewithac:vity


Exercise: Do No Harm! •  Researchshowsthatmanypa:entswithhEDShavenega:vepastphysicaltherapyexperiencesdueto:

–  Iatrogenicjointinjuries

– Unmetrehabilita:onneeds»  (Bovet,2016)


Exercise: Do No Harm! Avoidmechanicalpain

•  Stabiliza:on&motorcontrolarecri:calThinkbeforemoving

•  Tensilestrengthof:ssuesvarieswithac:vity,menses,inflammatorystate,ageetc.

•  Slowprogressiontoallowhistologicalchanges


Exercise: Do No Harm!

Cau:onwithoveruse:nerveentrapment,triggerpointsCau:onwithchangingforces

e.g.,exercisebandsincreaseresistance

Allow:ssuerecoverybetweenexercisebouts

2-hourrule:discomfortshouldreturntobaselinewithin2hours



2/23/18


Exercise Progression •  Rememberbasicprinciplesofexerciseprogression


Proprioceptive Exercise •  Propriocep:onsignificantlyworseinkneeswithBJHS(Sahin,2008)

–  Propriocep:onexercisesimprovepainandfunc:on(Sahin,2008)•  Propriocep:veexforkneesreducespainandimprovesqualityof

life(Ferrell,2004)–  ClosedChainLEex–bridges,squats,sidelungetos:mulatejoint

receptors•  Neckandspinepain-Propriocep:veexmaybelessrelevantthan

behavioralandeduca:onalRx,metaanalysis(McCaskey,2014)

•  Backpainreduc:on,muscleenduranceandposturalstabilityimprovementswithlumbarspinalstabexercise(ToprakCelenay,2017)


Exercise: Proprioception •  Externalfocusexercises–putalaser

onitanddrawonthewall,thinkBAPS(SenMoCorSystem™)

•  Biofeedback(e.g.,Stabilizer™)•  Alterna:ngisometrics,dynamic

stabiliza:on•  Ballexercises,balance&

propriocep:on•  Emphasizegoodmotorcontrol


Exercise Progression Easier

•  Morepropriocep:vefeedback–  Tac:le,visual,verbal–  Externalfocus,e.g.lasers

•  Moresupport–  Exercisemachines–  Againstwall

Harder

•  Lessexternalpropriocep:vefeedback

•  Lesssupport–  Morechallenge,e.g.,unstable

surfaces–  Freeweightsorbands


Exercise Progressions Gravityassistedstabiliza:on,maximumtac:lefeedback

Addinggravitytostabilizerswithoutchallengingsubluxa:on


Exercise Progressions Notowelunderthearm,set

upandback,controlresistanceReducingtac:le

feedback/useofwall



2/23/18



•  Focusonjointstability–  Isolatelegorarmwithout

spinemovement–  Isolateshoulderstabilizers–  Isolatecore

•  Visualiza:on/mind-body–  E.g.,jointcompression,

Qigong

Harder

•  Focusonintegra:on–  Coordinatecoreandlimb

movement–  Addexternalchallenge–  Complexmovements,e.g.,TaiChi

•  Adddistrac:on/mul:tasking


Exercise Progressions Speed/alterna:ngisometrics Againstgravityismoreadvanced


Stabilization Exercises

•  Shoulderse~ngwithfeedback


Exercise Progression – Central Sensitization

Easier

•  ChronicPain–unstablepla]orm,flowingmo:on,alternateagonist/antagonistorcontract/relaxandstretch

•  Externalfocusexercise–laserpointerontarget/visualiza:on

Harder

•  ChronicPain-longerholds,isometrics,longersets,stretchlaterinsession

•  Focusonbodyposi:on


Craniocervical Flexion vs Proprioception Ex

•  Group1exercisingwithStabilizer/longuscolinods•  Group2exercisingwithlaseronheadandeyeandheadmovementstotargets(externalfocus)

•  BothGroupshadreduc:oninpainandneckdisabilityindex

•  Propriocep:ongroup>CCflexionimprovedtolerancetotriggerpointpalpa:on

(Galllego-Izquierdo,2016)



•  Midrange•  Shortdura:on•  Slow•  Lowimpact•  Symmetrical/bilateral •  RPEstartat4/10

Harder

•  FullRange•  Longerdura:on•  Fast•  Addimpact•  Unilateral/asymmetrical•  RPE7.5/10ul:mategoal



2/23/18


Exercise: Strengthening •  Strengthenstabiliza:onmmbeforeglobalmm

–  SpinestabilityexwithBJHSreducedpain,improvedendurance,improvedposturalstability(ToprakCelenay,2016)

•  Possibilityofincreasedjoints:ffnesswithincreasedstrength–  Cheerleadersincreasedshoulders:ffness,decreasedant.capsulelaxity(Laudner,2013)–  Increasedpatellartendons:ffnessinpa:entswithcEDS(Moller,2014)–  Mechanicalstraininhibitscollagenbreakdown(Flynn,2010)

•  Needpropriocep:on&motorcontrol(Scheper,2016)•  Beawareofstresseson‘incidental’joints,e.g.

–  HandsgrippingweightsorTheraband–  Wristextensioninquadruped–  Spinestabiliza:onforextremityexercise


Cardiovascular Exercise •  Chronicpainleadstodecondi:oning•  Exercise-inducedanalgesiacanreducepain•  Startcardioaderini:alcorestabiliza:ontrainingorusemachinesthatprovidestability(e.g.,recumbentbike)

•  Makeitfunsopa:entss:ckwithit170 PT for Hypermobility Spectrum Disorders

Exercise: Stretching •  PeoplewithEDScanhave:ghtstructures•  Isolatestretchtoproperstructure

–  Keepjointsinproperalignmentwhenstretchingmuscles

•  Stretchesmayneedtobegentle–  Onlydo80%stretchandhold3-5secinpa:entswithperipheralsensi:za:on

–  Thiscanminimizeflaresinsensi:zedpa:ents


Bracing and Taping •  Mul:direc:onalinstabilitycommon•  Hips,Shoulders,SI,Knees,Ribs,

Fingers,Feet…•  Benefitmaybefrompropriocep:ve

feedback•  Givepa:entresourcestoselfmanage

painfulareasastheyarise•  Teachpa:enttousemusclestobrace

fortheac:vity


Picture of Mindy’s Modified Brace


Manual Therapy •  Relievemmspasm,TrP,&fascialadhesions•  Decreasepain&autonomictone•  Realignjointscarefully

– MWM,MET,nervemobs,gradesI-IV– Stabilizenearbystructures

•  DONOHARM!– Donotover-mobilize



2/23/18


Precautions and Red Flags •  Spinalfluidincreasedpressure,leaks,syrinxesandheadaches–

–  Mindyhaddocumentedleakfromnoseapprox.1cupofspinalfluidaderhusbandpushedonherupperneckwithmassagewhichalleviatedher“worstheadacheinherlife”

•  Craniocervicalinstability–isdizzinessfromPOTSorCCI,stress,BPPV?•  ChiariMalforma:on–maycauseincreasedspinalfluidpressure,ataxia

especiallyifmalallignedinuppercervical•  Tetheredcord–avoidexcessivenervetension/flossingonly

–  Saddleanesthesia,difficultywalking,bowel/bladderissues,tension•  Precau:onsaswithapregnantpa:entforlaxity


Mindy’s outcomes •  22%increaseinfunc:ononCareConnec:onsform•  4.5/7globalra:ngofchangeonscaleof-7to+7•  Worstpainreducedfrom9/10to4-5/10,LBP0/10•  Areastreated:Neck,jaw,ribs,shoulder,hips,LB•  21visitsover6months


Summary of Adult Case •  Needcomprehensiveevalua:on•  Challengetoovercomegravity•  Motorcontrolisessen:al•  Subtlechangescanbeimportant•  Startlow,goslow!


Overall Summary •  Hypermobilityspectrumdisordersarecommon•  HSDinvolvesmanybodysystems,notjustjoints•  Physicaltherapyiskeytomanagement•  Programmustbecustomizedtothepa:ent•  Lookforzebras!



Physical Therapy for Hypermobility Spectrum Disorders References

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