Transcript Ehlers Danlos Syndrome

Ehlers Danlos Syndrome
Heather Purdin Goodell, PT
Heather Purdin Goodell, M.S., P.T.
• Graduate of Duke University 1995 with BS in
BioPsychoSocial Psychology, Health Psych, and
Neuropsych
• Honors Thesis on Pain Behaviors in Children
• Master’s Degree in Physical Therapy Duke University
1997
• Special Initiatives award for “enhancing awareness of
cultural diversity in our program and profession”
• Private practice owner with mission of providing
holistic care and large portion of patients having
chronic pain
• Personal experience with HEDS
Common Orthopedic Problems
• TMJ dysfunction present
in > 70%
• Neck pain/tension
headaches/unstable
segments/herniations
• Shoulder
sublux/dislocate/multidir
ectional instability and
tendonitis
• Elbow tendonitis
• Early onset OA in hands
and feet
• Rib subluxation repeated
at certain levels
• Low Back Pain – unstable
segments, disc herniation
• Hip dislocation
• Patellofemoral syndrome
• Flat feet, “plantar
fasciitis”
• Migraines
Common Neuromuscular problems
• Poor proprioception (sense of position) leads to
increased incidence of injuries, clumsiness
• Pediatrics: Late walker, no crawling
• Poor balance – possibly greater issue for
geriatrics as other factors begin affecting balance
• Reduced sensation/muscle weakness
• Increased peripheral neuropathy
• Increased pain sensitivity/reactivity possibly
associated with larger amygdalas and smaller
anterior cingulate and parietal lobes
Common Biochemical Problems
• Hyperadrenergia – too much adrenaline may be
associated with low blood pressure, possible
mechanical cause at adrenals or at receptors or
due to different brain make up, psychological
response can lead to a vicious feedback loop
• Adrenaline ↔ Panic/overactivity
• Chronic exposure to adrenaline makes for
“jumpiness”, go-getter personality, “go ‘til you
drop” mentality = poor pacing and cycle of too
much then too little activity
• Chronic Fatigue, Adrenal Fatigue, Fibromyalgia
Treatment – BioPsychoSocial Approach
• “The best management program should
include drugs, physical therapy, cognitivebehavioral therapy, and adherence to a series
of lifestyle recommendations.” Castori 2012
Biological = medicine & mechanics
• Medicines to treat neurotransmitter issues:
- SSRIs
- *SNRIs – seratonin and norepinepherine re-uptake inhibitor
and at higher doses dopamine
- Blood pressure meds to increase BP (salt and water intake
natural treatment), for decreased BP (clonidine inhibits
sympathetic outflow and causes vasoconstriction) – see the
dysautonomia information network/POTS What to do
- Beta blockers have adrenaline buffering effect – low doses
- Valium and Ativan for acute episodes and to assist sleep
- Other sleep aides – melatonin over the counter 1-5 mg
- Naturopathic: Serriphos, Relax Max, adrenal support
Inflammation increases pain sensitivity
Medicine to treat connective tissue
issues
• Vitamin C (children 2-4000mg) and E – assist
with collagen generation
• Bone/joint support: Calcium Citrate 1000mg +
D3 880mg, glucosamine and chondroitin
• Energy – B complex, Cerefolin NAC also helps
memory and mental clarity
• Others: vit K, y-linolenic acid, pycnogenol
mangesium, zinc, methly sulfonyl methane,
silica
• Long term effect of pills on Kidneys, Liver?
Biological: Mechanics = PT
• Discharge goal is to teach neutral joint
mechanics at all joints for all activities. – Learn
to be your own PT
• Where to start? Is it the loosest or the tightest
link that is the biggest problem? Beware of
the tight one.
• Start at the biggest complaint & relieve the
most pain quickly and if this isn’t the tightest
or loosest link then go there next.
Pain leads to inactivity & inactivity
leads to tissue failure
• Affects on Bone – after 12
weeks immobilization bone
hardness is reduced 55-60%
• Connective tissue –
immobilization→less water,
altered collagen &
glycosaminoglycans → ↑
space between collagen
fibres, reduced elasticity,
more brittle, ***capsule
and ligaments fail at lower
loads*** -- Keer and
Grahame
• Nerve – neural reflex causes
muscle atrophy from joint
damage and immobilization
• Muscle – decreased fiber
size, altered sarcomere
alignment, ↓ mass
– Reduced #, size, function of
mitochondria
– Reduced oxidative
capacity/increased
fatiguability
– Most in 1st 5-7 days immobile
– Atrophy also from reflex
inhibition due to pain, fear of
pain, injury and inflammation
Key Components to PT Program
• Education about condition, pacing, self
treatment, diet (protein and water), rest, sleep,
Explain Pain, stress management, ergonomics
• Proprioception, coordination and kinaesthesia
• Core stability endurance and strength
• Global Muscle strength and endurance
• Controlled flexibility
• Cardiovascular fitness
• Relaxation and breathing
Mechanics of Functional Movements –
easy to get quick change in pain
• Sit to stand
• Posture in sitting or standing
• Sleeping posture, surface (memory foam
helps), use props/supports
• Mechanics of specific functions
– Reaching and neutral shoulder (stabilize shoulder
blade and shoulder before moving arm)
– Engaging muscles before moving (think before you
move)
Bracing and Taping
• Multidirectional
instability of the _____
• Hips, Shoulders, SI,
Knees, Ribs, Fingers
• Learning to self manage
painful areas as they
arise
• Brace for the activity –
think prevention!
• Foot orthotics
Mechanics of Stretch
• Stretch aligns collagen
fibrils and cross fibers
that develop when not
stretched
• Reduces pain
• Caution for Overstretch:
Do 80% of what you
think you can
• Initially 3-5 sec to avoid
pain response
Mechanics of Strength
• Muscle fibers become
disorganized with lack of use
and become more painful –
this is reversed with
strengthening
• Regular strengthening lowers
biochemical inflammatory
response in muscles and
lowers systemic inflammation
• Key is to start light and
progress slowly
Recommended Strength Exercise
• Symmetry
• Neutral Joints!
• Spine stability initially with
support to provide feedback
about where the body is:
i.e. lean against wall/on floor
before removing support
• Spine stability in functional
movements next
• Light weights – engaging
proper muscles is chief
concern before ↑ weight
• Pool exercises
• Ball exercises
• Balance ex
• Caution with bands that get
tighter toward end range
Strengthening – how hard to go?
• #1 rule is to protect your joints at all times
• Stay painfree whenever possible – initially the painfree zone is
very narrow and then expands as you desensitize – PTs can
shine here with knowledge of body mechanics/joint protection
• Optimal strength effect: 2-3 sets to fatigue (12-20 reps)
3x/week (American College of Sports Medicine)
• Initially – 1st 2 weeks’ gains are from neuromuscular
connection and lighter but more frequent exercise may be OK
• Repeat exercises once recover from last workout or every
other day
• 80% of what you think you can do (Keer and Graham)
• Do 50% of your maximum at first. Rate of Perceived Exertion
0-10 scale: 5/10 initially for 1st week, 6/10 2nd -4th week, with a
goal of attaining 7/10
• 2 hour recovery rule: 2 hrs later pain level < or = prior level
Physiology of Cardiovascular Exercise
• Chronic pain leads to fewer mitochondria, slower
Kreb Cycle (making use of energy)which improves
with chronic exposure to cardiovascular exercise
• Increased circulation to remove waste products,
bring oxygen and reduces need for adrenaline to
perfuse vital organs and distal regions
• Ideal: daily, outdoors, 10 minutes or greater, RPE
< 7/10
• Start cardio/walking after initial core stab training
Cardio effects on mood and pain
• When Your Body Gets
the Blues (Brown) – 10
minutes of walking
outside in the clouds
elevates mood for 1.5
hrs
• compared to chocolate
consumption, there is
no “low” afterwards
Manual Therapy – soft tissue
• Re-align collagen fibers
and release cross fibers
through myofascial
release
• Reduce guarding in
muscles and fascia –
Bowen Technique
• Rhythmic oscillations to
reduce tone to normal
Manual Therapy - Joints
• Correct alignment with
gentle techniques –use
the muscles, specific
mobilization
• Extreme caution with
Chiropractic
adjustments to be very
specific and not beyond
strength of tissues
Neurological PT
• Mobilization of the nervous system through
specific exercises to improve nerve circulation
• Exercise classes – Yoga, Thai Chi, Chi Gong, Ai Chi,
Aqua aerobics to associate movement with
meditation or positive sensation
• Breathing education – diaphragmatic breathing
increases tone in postural muscles, can assist
with reducing anxiety, adrenaline
• Meditation – guided relaxation technique to
reduce tone, reduce pain, reduce fight or flight
Women’s/Men’s Health Issues
• Uterine, bladder prolapse, rectal prolapse = minor Dx criteria
• Incontinence – pesaries are braces to support bladder/rectum
• Rectocele worsens due to constipation and straining, leads to
increase in inflammation in gut and infection of bladder, yeast
infections
• Teach proper toileting techniques – squat position to relax pelvic
floor, big belly, deep breathing
• Pelvic pain associated with involuntary guarding to gain stability
of pelvis, organs
• Chronic inflammation and infection can lead to interstitial cystitis,
vulvodynia
• Erectile Dysfunction associated with low blood pressure and
vascular insufficiency
Psycho:
• Understanding pain reduces
pain perception
• Realizing that many health
issues are linked by one
common cause reduces worry
• Patient education
• Family education
• Cognitive Behavioral Therapy
– learn to respond differently and
change your neurochemistry
– work on the doing too
much/too little cycle
– address fear of movement/fear
of permanent damage with
exercise
• Pain is Depressing
(chemically)
Social:
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PT goals to get out and be active again
PT can be a fun, social outing – socialize!
Exercise classes
Support groups
– Fibromyalgia support group
• portlandfibrocfs.com
– Ehlers Danlos Support Group
• www.oreds.org
• www.ednf.org National Foundation
• www.inspire.com International EDS Online Support
Other Providers on the Team
• Massage: Bowen technique,
Neuro integrative Therapy, MFR
• Reiki, Acupuncture, other
Naturopathic rx
• Spiritual and Religious
• Psychologist
• MD/ND for medication and
medical management, but who
is in charge?: Primary care, Pain
Doc, Physiatry, Rheumatologist,
Geneticist, Orthopedist,
Gynecologist, Cardiologist,
Opthamologist, Psychiatrist?
• Caution for Quacks- people will
spend any amount of money to
be rid of pain and waste a lot on
unproven practices, providers,
and supplements
References
• Brown, When Your Body Gets the Blues
• Butler, D and Moseley, L, Explain Pain
• M. Castori, I. Sperduti, C. Celletti, F. Camerota, and P. Grammatico,
“Symptom and joint mobility progression in the joint hypermobility
syndrome (Ehlers-Danlos syndrome, hypermobility type),” Clinical and
Experimental Rheumatology, vol. 29, pp. 998–1005, 2011.
• M. Castori, “Ehlers-Danlos Syndrome, Hypermobility Type: An
Underdiagnosed Hereditary Connective Tissue Disorder with
Mucocutaneous, Articular, and Systemic Manifestations,” ISNR
Dermatology, Volume 2012 (2012), Article ID 751768, 22 pages.
• M. Castori, S. Morlino, C. Celletti et al., “Management of pain and fatigue
in the joint hypermobility syndrome (a.k.a. Ehlers-Danlos syndrome,
hypermobility type): principles and proposal for a multidisciplinary
approach,” American Journal of Medical Genetics A, vol. 158, pp. 2055–
2070, 2012.
• A. J. Hakim and R. Grahame, “A simple questionnaire to detect
hypermobility: an adjunct to the assessment of patients with diffuse
musculoskeletal pain,” International Journal of Clinical Practice, vol. 57,
no. 3, pp. 163–166, 2003.
References Con’t
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A. J. Hakim, R. J. Keer, and R. Grahame, Hypermobility, Fibromyalgia and Chronic
Pain, Churchill Livingstone, Elsevier, Edinburgh, UK, 2010.
Keer, Rosemary and Grahame, Rodney Hypermobility Syndrome – Recognition and
Management for Physiotherapists published by Butterworth Heineman, Elsevier
Limited 2003
R. Keer and J. Simmonds, “Joint protection and physical rehabilitation of the adult
with hypermobility syndrome,” Current Opinion in Rheumatology, vol. 23, no. 2, pp.
131–136, 2011.
Knight, Isobel with Hakim, A A Guide to Living with Hypermobility Synrome:
Bending without Breaking 2010
Pocinki, Alan G, MD, PLLC Joint Hypermobility and Joint Hypermobility Syndrome
J. V. Simmonds and R. J. Keer, “Hypermobility and the hypermobility syndrome—
part 2: assessment and management of hypermobility syndrome: illustrated via
case studies,” Manual Therapy, vol. 13, no. 2, pp. e1–e11, 2008.
J. V. Simmonds and R. J. Keer, “Hypermobility and the hypermobility syndrome,”
Manual Therapy, vol. 12, no. 4, pp. 298–309, 2007.
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References Con’t
www.ednf.org
www.inspire.com
www.oreds.org
Facebook: Oregon Area Ehlers-Danlos Syndrome, Fibromyalgia Support Group
Portland
http://prettyill.com
http://medicalzebras.com
http://hypermobility.org (UK)
http://ehlers-danlos.org (UK)
http://ehlersdanlosnetwork.org
http://murraywoodfoundation.org
http://www.reumatologia-dr-bravo.cl (CL)
www.dinet.org Dysautonomia information
Mobilisation of the Nervous System – NOI group course
North American Institute or Orthopaedic and Manual Therapy (NAIOMT) courses
Heather Goodell, PT
4475 SW Scholls Ferry Rd, Suite 258
Portland, OR 97225
Ph: 503-292-5882
www.goodellpt.com
[email protected]
Questions?
EDS Hypermobile Type is a Heterogeneous Syndrome
with varying presentations and intensities. Any body
system that relies on collagen is suspect.