Moving Naturally with Hypermobility June 18, 2015

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

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 

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

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

Beighton Scale- Measures

Beighton Scale- Measures

Beighton Scale- Measures

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

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

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

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

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

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

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!!!

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!

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

Breaking the negative cycle of pain

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

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

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

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?

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

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

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

Sitting Postures

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

Evolution of bad posture

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

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

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?

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.