What is Ehlers-Danlos Syndrome? Intro for Fibro Patients Fibro/CFS Support Group Meeting September 20, 2014 Portland, OR USA Jan Groh, speaker Http://ohtwist.wordpress.com 1

Who am I? (Jan Groh) 47 yo Hypermobile EDS Patient, diagnosed Feb 14, 2012 after major onset “cascade” left me wheelchair bound Took 25 years to get diagnosed after being written off as just a depressed woman since 18. Dxed with CFS in 1993 I have been observing over 6000 EDS patients daily online via social forums, where I've gathered so much info - trying to share with doctors and the world to help all to recognize sooner Led OR EDS group 2012/2013 inc “mini” Conference Working on a book as we speak I've yet to meet a fibro patient IRL who doesn't pass Brighton DX Criteria for HEDS! 2

Can you spot young Jan with EDS? Think small! Mini Me (Jan age 11) Can you spot young Jan with EDS? Think small! (Doctors can note the fine lordosis in lower back, plus very bendy elbows. Knees suprisingly straight!) 3

I could have been a 4th “Ross Sister” as a child.... very bendy! The Ross Sisters in “Solid Potato Salad” number I could have been a 4th “Ross Sister” as a child.... very bendy!

What is Fibromyalgia? Poorly defined / hard to diagnose / somewhat subjective Characterized by widespread pain & fatigue Poor sleep Headaches Depression & anxiety Multiple Chemical Sensitivity Much more... a lot like a mild Ehlers patient ;)

What is Ehlers-Danlos Syndrome? A collection of genetic collagen defects that is NOT RARE, it’s RARELY DIAGNOSED!* * depending on who’s talking :) It’s a Heritable Disorder of Connective Tissue (HDTC) It presents in an almost mind-numbing array of ways & rates (as individual as fingerprints) “If you can't connect the issues, think connective tissues!” - unknown 6

Think zebras, not horses when you hear hoofbeats! BUT! Castori et al cite up to 2% may have some form of EDS now Also often “triggered” like fibromyalgia...

The 6 Main Types of Ehlers-Danlos Syndrome There is a LOT of cross-over between types, it’s not always easy to distinguish! Please consult a trained medical geneticist if you suspect any type of EDS! There are single tissue markers for these 5 rare types: Dermatosparaxis (DEDS / VII c ) – very rare, skin sign Arthrochalasia (AEDS / VII a, b) – rare, bilateral hip disloc. Kyphoscoliotic (KEDS / VI) – rare, severe scoliosis Vascular (VEDS / IV) – rare, can be lethal from arterial & organ ruptures, thin non-stretchy skin, very visible veins Classical (CEDS / I & II) – semi-rare, skin signs, scars Last but not least! No single easy tissue marker test yet for: Hypermobile (HEDS/ III) – common?, joint pain/hypermobility Use BRIGHTON DIAGNOSTIC CRITERIA (not Beighton!) to DX! 8

Beighton vs Brighton Professor Beighton developed the 9 pt epidemiological field test for hypermobility over 20 years ago. Was not meant to be used to diagnose! Use to suspect EDS/HMS only. It misses many patients who are no longer or never were bendy! The Brighton Diagnostic Criteria were adopted in 1999 in Brighton England for this very reason. They help to find non-bendy zebras as well as bendy ones! 9

You could say we got “Bright” in Brighton England! (All due respect to the good Professor Beighton) 10

The Brighton (not Beighton The Brighton (not Beighton!) Diagnostic Criteria Use to diagnose BJHS (aka JHS/HMS/EDS III/HEDS) Major Criteria (2) A Beighton score of 4/9 or greater (either currently or historically) Arthralgia for longer than 3 months in 4 or more joints (joint pain) Minor Criteria (8) A Beighton score of 1, 2 or 3/9 (0, 1, 2 or 3 if aged 50+) Arthralgia (joint pain) > 3 months in one to three joints or back pain (> 3 months), spondylosis, spondylolysis/spondylolisthesis. Dislocation/subluxation in more than one joint, or in one joint on more than one occasion. Soft tissue rheumatism. > 3 lesions (e.g. epicondylitis, tenosynovitis, bursitis). Marfanoid habitus (tall, slim, span/height ratio >1.03, upper: lower segment ratio less than 0.89, arachnodactyly [positive Steinberg/wrist signs]. Abnormal skin: striae (stripes), hyperextensibility (stretchy), thin skin, papyraceous scarring. Eye signs: drooping eyelids or myopia or antimongoloid slant (almond shaped). Varicose veins or hernia or uterine/rectal prolapse. The BJHS is diagnosed in the presence two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative. How you can have EDS without being bendy! 11

Some. Symptoms We See a Lot Some* Symptoms We See a Lot! * This list is not at all comprehensive – we could list many more. See website. Bones & Joints Cardio/Dysautonomia Other Chronic early joint pain Poor temp. regulation (+/-) Crowded or weak teeth Subluxations (partial disloc) Tachycardia (all variations) Flat feet (fallen arches) “Double jointed” PVC’s, head-rushes Thin/stretchy/soft skin Dislocations Early onset varicose veins Headaches Tendonitis /bursitis Spider veins IBS (wet/dry/flips) Spinal issues (all kinds) Enlarged aortic roots Trouble swallowing/dysph Weak neck, C-C settling Mitral valve prolapse Depression/anxiety Kyphosis Insomnia Easy bruising Chondromalacia (patella) Thyroid trouble (+/-) Unusual or keloid scars Sciatica Frequent urination Hearing trouble/loss Osteopoenia/-porosis Aneurysms Floaters / retinal detach. Bone spurs Easy bleeding Myopia (near sightedness) 12

Some Common EDS Comorbidities Dysautonomia/POTS Migraines Fibromyalgia Chronic Fatigue Syndrome Arthritis Diabetes Thyroid issues (+/-) Autism Spectrum Chiari Malformation Tethered Cord Cranial-Cervical Settling Mood and anxiety disorders Allergies and MCAD (masto/MCAS) Sensory Processing Disorder Insomnia /Hyperadrenergia MS, AS, RA and all other AIs Leaky Gut Syndrome Malabsorption/malnutrition Hydrocephalus (LLECH) CVID (chronic infections) Arachnoiditis Fibromuscular dysplasia

Overlap between Fibromyalgia and Ehlers-Danlos Syndrome ?? Common to both: Headaches Widespread variable chronic pain Tender / trigger points Tendonitis Chronic fatigue (not always ME) GI distress & IBS Neuralgia & neuropathy Skin sensitivity Secondary arthritis & AI disorders Insomnia, hyperadrenergia Brain fog (neuroinflammation) Depression & anxiety EDS “only”: FAMILY HISTORY Subluxations Dislocations Ruptures Tears Breaks

Perhaps it's a matter of degree on an EDS “Spectrum”? Severe enough to “see” clinically (Double allele?) Ehlers-Danlos Syndrome (Start to sublux/dislocate/tear more) Too “mild” to “see” clinically (Single allele?) Severity Fibromyalgia / BJHS/ JHS/ HMS (Chronic widespread pain, few dislocations) Time, Stress & Triggers...

E.g. “Frequent urination”: There are often BOTH bio-mechanical (X) AND bio-chemical (Y) causes to MANY of our issues! E.g. “Frequent urination”: Allergies – biochemical (Y) MCAS / Masto reactions – biochemical (Y) Tethered cord – biomechanical (X) Impinged lumbar nerve roots -biomechanical (X) UTIs (X & Y?) Solve for BOTH X & Y !!

What Can I Do About It? In consultation with your doctor you might try: Find & eliminate all allergens & triggers as best you can Get checked for nutritional deficiencies inc electrolytes & supplement as appropriate, inc C, Calc, Mag, Zinc, D3 Keep blood volume up with water and salt as needed Stay as conditioned as possible without injuring! See next Get appropriate core-strengthening “zebra-friendly” PT Manage pain and optimize sleep (helps lower pain) Eat as organic & unprocessed as possible Avoid triggers, mitigate reactions with H1/H2 blockers 17

Body work of choice as tolerated: Pilates and/or yoga (careful, do NOT go to full range!) Chiropractic with CARE (AVOID neck thrusts!!) Core-building PT with CARE (same as chiro) Alexander Technique (teaches proper Use of Self) Bowen Therapy / NST (helps re-integrate the body) Gokhale Method Feldenkrais Massage & Cranio-sacral work MELT method TENS Units/microstim

Mind and spirit are equally crucial: Good sleep and diet Cognitive and/or dialectical behavioral therapy Supportive talk therapy and a support group Energy work – acupuncture or Reiki (or both) Meditation, breathing & spiritual practice of choice Journaling – and gratitude practice & grief cslng

Got Collagen?  If not, try an exoskeleton! Talk to Heather first before investing in an exoskeleton! 20

Thank you to all my supporters! ComForcare Home Care http://www.greaterpw.comforcare.com Blanket Booster Http://www.blanketbooster.com More information: Support: http://oreds.org Information: http://ohtwist.wordpress.com EDNF: http://ednf.org EDS Support: http://www.chronicpainpartners.com EDNF Support forum: http://www.inspire.com