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Ehlers-Danlos, Exercise &

Rehabilitation

Mark E. Lavallee, M.D, C.S.C.S, F.A.C.S.M.

Director, York Hospital Sports Medicine Fellowship

Past Chair, EDNF, Professional Advisory Council

Ass’t Clin.Professor, Penn State Univ., College of Medicine

Adjunct Clin. Professor, Drexel University, School of Medicine

Chairman, USA Weightlifting, Sports Medicine Society

Team Physician, Gettysburg College

York, Pennsylvania

EDNF National Conference August 15th, 2015

Disclosures

No conflicts of interests

All patients have given permission for their images/stories to be used in this talk.

Not Weightlifting Injuries

Not Football Injuries

Not all hyperflexible athletes have a

connective tissue disorder!

Not all patients with connective tissue

disorders are pathological flexible!!

Do EDS Patients Exercise?

1440 questionaires

205 responses

Response rate 13.8%

196 questionaires were included

Respondants: – 160 female

– 38 male

Lavallee ME, Johnson P, EDNF, vol 2,

2000.

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Ehlers – Danlos Syndrome Activity Questionnaire Please fill out this form for each family member who has EDS BASIC INFORMATION Age in years: __________ Gender: Female \ Male

State of residence?____________________________

Type of EDS? Classical Hypermobile Vascular Other Unknown

(Type I & II) (Type III) (Type IV) (I don’t know what type)

What age were you diagnosed with EDS (in years)?____________________ ACTIVITY INFORMATION Amount of exercise? None 1/year

1/month couple times /month couple times /week daily

Type of exercise?__________________________________ How long exercising? 5 minutes 10 min 15 min

30 min 45 min > one hour other_______

Highest level of education? High School College Graduate Doctoral other______________

Did/Do you participate in sports / athletics in high school, college, professional, recreational or other? Yes / No

If Yes What sports? _________________________________________ Position played?_______________________________________ Length of participation?________________________________ Have you every had an injury playing sports? Yes / No If Yes Type of injury?_______________________________________ Did you require surgery because of the injury? Yes / No DISABILITY INFORMATION Are you employed? Yes / No If yes, Occupation?__________________________________ Do you require special accommodations at work? Yes / No If No, Do you consider yourself disabled? Yes / No What is your current perceived level of disability? Scale 1 – 10 (1 none, 10 need total

help) 1 2 3 4 5 6 7 8 9 10 None some help needed

totally dependent on help Are you receiving financial assistance? (i.e. disability, SSI, Social Security, etc.)

Yes / No Do you require assistance? wheelchair \ crutches \ scooter \ braces \ other

____________________________ Number of major surgeries you’ve had? none 1 2 3 4 5 6 7 8

9 10 or greater OTHER INFORMATION Have you been diagnosed with any other associated medical condition (listed below)? Endometriosis Fibromyalgia Chronic Fatigue Syndrome Dysparunia

Asthma Amenorrhea Osteoporosis Problems with wound healing

Fibrocystic Breast Disease Do you take supplements? Glucosamine \ Chondrotin \ Vitamin C \ Creatine \

other_______________________ Do you seek alternative forms of medical therapy? Yes \ No If yes: Massage \ Chiropractic \ Osteopathic (D.O.) \

Homeopathy \ Acupuncture \ other____________________ Please mail or fax by March 15, 2000 to:

Name & Address (optional) Mark E. Lavallee, M.D. Director, Sports Medicine, Memorial FP Residency Sports Medicine Institute 111 W. Jefferson, Suite 100 South Bend, IN 46601 Tel. No. 219-289-4SMI Fax No. 219-239-6461

Type of Respondents

– 22 (11.6%) Unknown

– 14 (7.1%) Vascular

– 50 (25.5%) Classical

– 103(52.6%)Hypermobile

– Lavallee ME, Johnson P, EDNF, vol 2, 2000.

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How Active are EDS patients?

Age Range: 5-75y.o.

17% (34/196) Swam in:

– HS, College, or Receationally

26.5% (48/181) Exercised Daily

43.7% (79/181) Exercised 2-4x/wk

16.6% (30/181) Did no exercise

Lavallee ME, Johnson P, EDNF, vol 2, 2000.

What factors determine flexibility?

Genetics

Stretching

Muscular Mass

Age

COLLAGEN!!

USAW Pan Am Team July 2015, Toronto, CAN

USOTC Colorado Springs, CO -- 130 kg (286 lbs) deadlift

Strength Training

DO – Core strength

– Daily exercise

– Body weight

– Resistance Bands

– Dumbells/Barbells

– Machines

DON’T – Cross-fit

– Olympic Lifts

Kendrick Farris at 2011 Pan Am Games before 2012 Summer Olympic (London)

Bronze Medalist: Chad Vaughn 2011 77kg BW 176 C&J

Kendrick at 2015 Pan Am Games, GOLD Medal

Cardio Training

DO

–Walk (10,000)

–Swimming

–Elliptical/Stepper

–Treadmill-incline

–Stationary bike

–Ergometer

DON’T

–Distance running

4.4 mile Chesapeake Swim

Core strength/ Flexibility/Balance

DO – Thera-Ball

– Yoga

– Tai Chi

– FMS

– Ballroom Dance

– TRX

DON’T – Competitive Cheer /

Gymnastic

2014 World University Team USA in Chang Mai with 1st and 2nd place team trophies!

Just Do it!

Do not feel sorry for yourself!!

–Or I will introduce you to

US Veteran

Baby with Retinolastoma

GET OFF YOUR NARCOTICS!!

Just Do it!

Exercise EVERY day!! UNLESS – Temp > 101

– Having surgery that day

Laugh EVERY Day

Help someone less fortunate than you weekly

Just Read this Book!

You will learn to appreciate:

–Your pain

–What generations before us had to endure!

Set GOALS: – Short-term (1-3 months)

– Long-term (1-3 years)

Case #1: Hong Kong Phooey

34yo Martial Artist

– World Champion

Presents w/

– Multiple joint complaints

– Joint Pain

– multiple joint laxity/dislocations

– “loose skin”

History

Family History

–Mom some “loose joints”

–Maternal Aunt “loose jointed”

Athletic History (currently, retired)

–7 State Titles

–3 National Titles

–1 World Championship, Gold Medal

–Martial Arts Hall-of-Fame inductee

2000 Competitor of the Year.

Case 2: Stretch Armstrong

15y.o HS Wrestler

Presented w/

– R shoulder dislocation (x5)

– L shoulder pain

Exam:

– Muscular, strong

- Notable laxity when relaxed

Exam

Face Skin:

– No scars

– Hyperextensible

Hands:

– Hypermobile at:

MCP

Thumb

Shoulders:

– Auto-Sublux

Meet 9/9 Carter-Wilkerson Criteria

Case 3: Champion Swimmer

17y.o. HS varsity swimmer

– Freestyle/Butterfly

– Swims 15,000m/day

– Gone to State championship last 2 years.

– c/o b/l shoulder pain

– Skin: hyperextensible, soft

Exam

GEN: Ht 6’1” Wt 185 Ht:Armspan <1:1.05

Wrist:

Hyper flexion to 125o

Thumb to forearm

Shoulder:

Painless auto-dislocate

Abduct to 230o

Hips: hyperflexible

“Palm on floor”

Case 4: Recreational Cyclist

28y.o. cyclist who fell & struck his left chest and abdomen.

He sustained:

– Large left flank hematoma

– Left A/C separation

– Left Chest wall contusion

Initial eval. was in ER

Tests

CXR: – Aortic Aneursym?

ECHO: – Large aortic arch

dilation

– nl EF

– min MVP

– no MR

Biopsy: (+) COL3A1 gene

Diagnosis: EDS Vascular type

In Conclusion

Recommendations

Consider Physical Therapy:

– Strengthening the kinetic chain

– Proprioceptive re-training

– FMS training

Prevent injury:

– Avoid contact, collision sports

– Avoid high dynamic sports

Recommendations

Encourage activity: – Low static, Low to Mild

dynamic

– May recommend sports like biking, swimming,weight-training, golf..

– Patients often excel at certain sports due to“genetic” advantage b/c of flexibility.

– Mild Strength training has show benefits in joint stabilization

Recommendations

Cardiovascular Screening

– ALL patients should get an initial ECHOCARDIOGRAM & AORTIC ULTRASOUND

Every 3-5 years for average patient

Every year for those with

– Aortic Dilatation

– Severe valvular disease

– Severe HTN

– Highly Dynamic sports

Recommendations

Wound closure issues

– Dermabond /steristrips better than sutures

Reproductive concerns

– Pregnancy

– Autosomal Dominant

Provide psychological support

Surgery as last resort.

Final thoughts

Keep EDS in differential diagnosis of the Hyperflexible Athlete.

Still as of 2014, diagnosis made by history and a thorough exam.

Be wary of sports where flexibility is important!

–Gymnastics, wrestling, swimming, figure-skating, cheerleading, dancers

Sometimes athletes despite less than optimal genetics still can succeed if they work hard enough

Thank You!

2013 US World Weightlifting Team, Wroclaw, Poland

References

Ainsworth, Aulicino, (1990) A survey of pts. w/ EDS. Loose Conections, 5(3),pp1-7.

Beighton, P. The Ehlers-danlos Syndrome. London: Heinemann Medical Books Ltd; 1970.

Beighton P, Steinmann B, Tsipouras P, & Wenstrup R, Am J Med Genetics, 1998, 77;31-37

Brown, Lehman, Peterson, “Understanding the Nature of Growing Pains,” Patient Care, April 1998, pp 63-79.

Byers, PH, Holbrook KA. Molecular basis of clinical heterogenity in the Ehlers-Dnalos Syndr. Ann NY Acad Sci: 1985;460:pp. 298.

Danlos, HA. Un cas de cutis laxa avec tumuers par contusion chronique des coudes et des genoux (xanthome juvenile pseudo-diabetique de MM Halopeau et Mace de Lepinay). Bull Soc Fr Dermatol Syphiligr. 1908;19:pp.70-72.

References

Ehlers, E. Cutis Laxa, Neigung zu Haemorrhgien in der Haut, Lockerung mehrerer Artikulationen. Dermatol Z. 1901;8 pp. 173.

Harrison: Harrison’s Principle of Internal Medicine, 12th ed, McGraw-Hill, 1991, pp. 1866-7

Jansen, LH. The structure of the connective tissue: an explanation of the symptoms of the Ehlers-Danlos Syndrome. Dermatologica. 1955;110: 108-120.

Johnson, SA. Falls HF. Ehlers-Danlos syndrome: a clinical & genetic study. Arch Dermatol Syphiligr. 1949;60: pp. 82-105.

Loose Connection, Journal of the Ehlers-Danlos National Foundation

Maltz SB, Fantus RJ, “Surgical Complication of EDS Type IV: Case Report and Lit Review,” J Trauma Injury, 2001;51 387-90.

References

Van Meekeran, J. De diatabititate extraordinaria cutis in viro quodam Hispano. In: Blasio A, ed. Observations Medico-Chirurgicae. Amsterdam, the Netherlands: Henrici & Viduae Theodori Boom:

Wenstrup R, Meyer R, Lyle J, Hoechstetter, Rose P, Levy H, Francomano, C, Genetics in Medicine 4(3) May/June 2002, p 112-117.

De Paepe A, Malfait F., “the Ehlers-Danlos syndrome, a disorder with many faces.” Clin Genet 2012: 82:pp1-11.