1. Moving Naturally with Hypermobility
June 18, 2015

2. Outline Review of Hypermobility Syndrome / EDS
Affects on the musculoskeletal system
Role of Exercise
Role of Sleep
Taking stress off of the joints
Moving properly with hypermobility
Resources for patients

3. Christopher Gnip, DPT, PT
Graduated from Washington University in St Louis in 2006 with a doctorate in Physical Therapy
Practicing in metro DC since 2006
Manages several clinics for Physiotherapy Associates
Interested and started learning more about hypermobility syndromes in 2010
Collected research and presented poster at National PT Conference in Feb 2015
Still needs to learn a lot more about these syndromes (We all do!!)
Not only a practitioner that treats hypermobility, but also a client 

4. Causes of Hypermobility Syndromes / Ehlers Danlos Syndrome
Primary reason is a collagen deficiency / mutation
Collagen is a protein found in most connective tissues throughout the body
Collagen is the ‘glue’ that keeps connective tissues strong
Due to the deficiency, there is a laxity in most connective tissues throughout the body
Not only affects muscles, tendons, and ligaments, but can also affect other vessels, such as blood and lymphatic vessels
Hypermobility Type
Type III) Most common type
Genetic Information: To date, no distinctive biochemical collagen finding has been identified by researchers. Inherited in an autosomal dominant manner.
Classical Type
(Type I & II) Occasional internal organ fragility
Genetic Information: In some families type V collagen is affected (abnormal electrophoretic mobility of proa1(V) or proa2(V) chains) as well as type I. Inherited in an autosomal dominant or recessive manner.
Vascular Type
(Type IV) Fragility of arteries, intestines & other internal organs
Genetic Information: Caused by structural defects in the proa1(III) chain of collagen type III, encoded by the COL3A1 gene. Inherited in an autosomal dominant manner. VEDS incidence is estimated at one in 250,000; particularly serious because of possible arterial or organ rupture. See EDNF’s Vascular EDS Medical Resource Guide and Clinic Reference Manual: Vascular Type.
Kyphoscoliosis, Type
(Type VI) Very rare; kyphoscoliosis, hypotonia, fragility of eyes and arteries
Genetic Information: Result of a deficiency of lysyl hydroxylase (procollagen-lysine 5-dioxygenase, or PLOD), which is a collagen-modifying enzyme. Inherited in an autosomal recessive manner. Can be diagnosed through urine test.
Arthrochalasia,Type
(Type VII-A&B) Very rare
Genetic Information: Caused by mutations leading to deficient processing of the amino-terminal end of proa1(I) [type A] or proa2(I)[type B] chains of collagen type I. Inherited in an autosomal dominant manner. Can also be diagnosed by skin biopsy.
Dermatosparaxis Type

5. Hypermobility Syndrome: ways to assess
Beighton Scale and Brighton Criteria
Beighton Scale
Assesses laxity in elbows, knees, thumbs, fingers and low back
4 / 9 is a major criteria for diagnosis
Will usually score less as we age

6. Beighton Scale- Measures

7. Beighton Scale- Measures

8. Beighton Scale- Measures

9. Diagnosing Requirements for Diagnosis Brighton Criteria Major Criteria
Beighton Score of 4 or more
Joint pain affecting 4 or more joints and lasting more than 3 months
Minor criteria
Beighton score of 1, 2, or 3
Back pain or pain in one to three joints lasting more than 3 months, or spinal spondylosis, spondylolysis, or spondylolisthesis
Dislocation of more than one joint, or of one joint more than once; 3 or more soft-tissue problems (e.g., tendonitis, bursitis)
Tall, thin body shape; Skin hyper-extensibility, stretch marks, thin skin, or abnormal scarring
Drooping eyelids, nearsightedness; Varicose veins, hernia, or uterine or rectal prolapsed
Mitral valve prolapse
Requirements for Diagnosis
2 major criteria
1 major and two minor criteria
4 minor criteria
2 minor criteria and a clearly affected first-degree relative

10. Effects of Joint Hypermobility Syndrome
Cardiovascular Issues
Gastro-Intestinal Issues
Joint Laxity
Anxiety
Autonomic Nervous System Disturbances
Joint Hypermobility Syndrome
Musculo-tendinous Issues
Chronic Pain
Poor Sleep
Chronic Fatigue
POTS
Breathing Difficulty
Mast Cell Issues

11. Affects on the musculoskeletal system
Due to the collagen defect, muscles and tendons have a decreased capacity to contract as effectively, have a greater chance of getting strained, and take longer to heal than normal tissue
Increased risk of joint subluxations and dislocations
Muscles and tendons though have to work extra for sufferers of joint hypermobility due to the overall joint laxity
Muscle activation is even more important with joint hypermobility due to lack of static stability

12. Affects on the musculoskeletal system, cont…
Poor posture, joint irritation and muscle strains can lead to:
Neck pain / headaches
TMJ disorder
Shoulder pain
Hand/Elbow/Wrist pain
Low back pain
Pelvic floor dysfunctions
Patello-femoral pain syndromes
Ankle / foot pain

13. Life is a journey, and we need to adjust the tuner along the way….

14. Unfortunately, there’s usually more than one tuner….

15. Ways to tune your life Get help- you’re not in this alone
Go to an MD or PT who understands hypermobility syndrome
Assess your posture and daily activities
Exercising
Might need to modify or change what you’re doing, but you need to keep moving
Moving correctly is key…Victor will focus on this in a little bit
Eat right!
Get more sleep
Don’t give up!!!

16. Benefits of Exercise Controls weight – either gaining or losing weight
Helps with virtually every medical condition, including cardiovascular issues, arthritis, diabetes, stroke and chronic pain
Improves mood and decreases anxiety
Boosts energy
Helps with improved sleep
Can be fun!! Find an exercise that you enjoy doing!

18. Pain with exercise What kind of pain is it? When does the pain happen?
Ache or soreness can be OK
Burning, searing or sharp pain is not
When does the pain happen?
During the movement
Can you adjust to make it hurt less?
After the exercise
Vary the exercise if it continues to cause pain
Decreased reps, weight
Improved form / posture
Increased rest in between

19. Breaking the negative cycle of pain

20. Pain Threshold
Increased activity leads to stress on joints / tissues and make joints more prone to inflammation
Staying under this threshold can help build up strength and stability in joints and help to raise threshold

21. Pain Threshold
Goal is to raise the threshold in order to tolerate more activities, whether simple tasks such as sitting or more challenging tasks like biking, without causing increased pain
If you don’t do any activity, the pain threshold will lower and the negative cycle of pain will continue to worsen

23. Importance of sleep Time for body to heal itself
Vital for improved awareness and memory
Can help decrease chances of heart disease, diabetes and obesity
Decreased feelings of fatigue
Decreased anxiety

24. JHS and Sleep
Research has shown that during the night, there are adrenaline surges as the body tries to regulate blood pressure
These adrenaline surges leads to momentary wake cycles (a lot of us don’t even realize) that disrupts your normal sleep rhythm
Can be asleep for 8 hours but still wake up feeling tired because of all the constant disruptions while sleeping
Have you gotten a sleep study performed?
Speak to your doctor about supplements to help sleep? Beta blockers?

25. Tips for better sleeping
Positioning
With neck issues, try not to sleep on stomach
Use of pillows
If side sleeper, try to use pillow that keeps head in a level position
Use of a body pillow to keep shoulders and hips in a neutral position
Use of a contoured pillow can help
Travel pillow on long flights / trips
Practice mindfulness before going to sleep
Make bedroom place for sleep – don’t watch TV / Ipads
No caffeine or alcohol prior to going to sleep
Try to get on a consistent schedule and stick with it

26. Daily Activities / Postures
What are your normal activities that you perform on a regular basis?
Typing / Writing
Carrying a child
Cleaning / Dishes
Repetitive lifting
How do you tend to posture yourself?
Legs crossed
Knee hyperextension
Forward shoulders or head posture

27. Daily Activities / Postures
Low stress activities that are performed multiple times or for a significant amount of time can have same impact on tissues as a high impact activity performed seldom
Don’t disregard the small activities in your life and focus on the big things….often the small things are just as important
Patients with hypermobility tend to posture at their end ranges because that’s where they feel ‘most stable’, but these end range postures often puts even more stress on already lax joints and strained muscles

28. Sitting Postures

29. What’s your preferred posture?
Sitting Postures
What’s your preferred posture?

30. Evolution of bad posture

31. Role of bracing Bracing can help support joints
If it supports joints, muscles and tendons don’t have to work as hard
Bracing can cause inactivity of muscle groups, so be aware of using it for too long
Examples of bracing:
Clavicular / scapular bracing
Wrist / thumb bracing
SI belt
Knee / ankle bracing

32. POTS (Postural Orthostatic Tachycardia Syndrome)
Common in patients with hypermobility / EDS
Associated with excessive increase in heartrate upon standing
Symptoms can range from mild to severe and can affect most activities of daily living
In HJS /EDS, elasticity in blood vessels can lead to blood pooling and low blood pressure, which then causes the increased heart rate upon standing
Treatments:
increasing fluid intake to 2-3 liters per day
increasing salt consumption to 3,000 mg to 10,000 mg per day
wearing compression stockings
raising the head of the bed (to conserve blood volume)
reclined exercises such as rowing, recumbent bicycling and swimming
a healthy diet
avoiding substances and situations that worsen orthostatic symptoms
medications meant to improve symptoms

33. Mast Cell Activation Syndrome
A collection of disorders characterized by:
Accumulation of pathologic mast cells in potentially any or all organs and tissues
Aberrant release of variable subsets of mast cell mediators, leading to one or more symptoms
Has been correlated to be higher in patients with connective tissue dysfunctions
Can be a result of overreaction to environmental allergens
Can lead to multiple symptoms including skin rashes, abdominal cramping, headaches, diarrhea, and joint pain
Is some of your joint pain coming from allergens in the environment?

34. What to do next?
Realize that the body has a great ability to repair itself
Need to assess all the various stresses that we are putting the body through and assess which ones are the easiest to change
Look at your daily positions and postures, repeated movements and activities, and see if slight modifications can decrease stress on joints enough to decrease pain
Monitor your sleep and rest times and allow the body the chance to heal itself
Make sure your diet is balanced and you’re receiving the proper nutrients to help the body repair
Know that you’re not alone in this battle and that there are people who want to help you through this journey.