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Connecting the dots with EDS, POTS,
MCAS, GI, Neurological and
Physiotherapy in managing pain in
EDS
Pradeep Chopra, MD
Pain Medicine
Assistant Professor (Clinical)
Brown Medical School.
USA
Email: snapa102@gmail.com
1
Pradeep Chopra, MD
Disclosure and disclaimer
• I have no actual or potential conflict of interest in relation to this
presentation or program.
• I have no financial interest in any of the products mentioned in this
presentation
• This presentation will discuss “off-label” uses of medications
• Discussions in this presentation are for a general information purposes
only. Please discuss with your physician your own particular treatment.
This presentation or discussion is NOT meant to take the place of your
doctor.
2
Pradeep Chopra, MD
Disclosure
• Training and Fellowship, Harvard Medical school
• Weird Pain Medicine specialist
• Assistant Professor – Brown Medical School, Rhode Island, USA
• Special interest in complex pain conditions
Pradeep Chopra, MD 3
Connecting the dots
• EDS is a complex condition
• This presentation is about connecting the dots between all the
symptoms that come with having EDS.
Pradeep Chopra, MD 4
EDS
Cranio-
cervical
instability
MCAS
Abdominal
pain
Chiari
POTS
Tethered
Cord
syndrome
Joint
instability
Autoimmun
e
dysfunction
Pradeep Chopra, MD 5
Cranio-
cervical
instability
MCAS
Abdominal
pain
Chiari
POTS
Tethered
Cord
syndrome
Autoimmun
e
dysfunction
Joint
instability
EDS
Pradeep Chopra, MD 6
Three things to know in EDS
1. EDS is not a disease. It is a form of the human body.
2. Soft connective tissue
3. Poor joint position sense (proprioception)
4. Co-existing conditions (Mast cell activation, POTS, Chiari etc.,)
Pradeep Chopra, MD 7
Pradeep Chopra, MD
Pain in EDS by body regions
Pradeep Chopra, MD 8
Pradeep Chopra, MD
Common causes of headaches in EDS
1. Migraines
2. Chiari malformation
3. Cervicogenic Headaches – from muscles
4. Temporo Mandibular joint dysfunction (TMJ)
5. PoTS / Dysautonomia
7. Spontaneous CSF (Cerebrospinal) leak – low pressure inside
the head
8. Cranio Cervical Instability (Instability of the neck and head)
9. Idiopathic Intracranial Hypertension (raised pressure inside
the head)
Pradeep Chopra, MD 9
“My head feels too heavy to
hold up”
Pradeep Chopra, MD 10
Chin poking forward position
Pradeep Chopra, MD 11
https://www.braceability.com/blogs/articles/text-neck-how-
smartphones-hurt-your-spine
Pradeep Chopra, MD 12
Pradeep Chopra, MD
Neck pain and headaches
• A common cause of neck pain is posture, using a smart phone
(“Texting neck”)
• 'Chin poking forward position'
• Before looking at other reasons, correct this first
• If there are other reasons like cervical instability, Chiari
malformation etc – these need to be addressed
Pradeep Chopra, MD 13
Pradeep Chopra, MD
Pain from a poor posture
Pradeep Chopra, MD 14
Pradeep Chopra, MD
Pain from a poor posture
Pradeep Chopra, MD 15
1
6
Pradeep Chopra, MD
Pradeep Chopra, MD
Common reasons for poor posture in
EDS
• Vision – Blurry vision. Usually intermittent
• Postural Orthostatic Intolerance (POTS)
• Laxity of spinal ligaments
• Instability of the head on the neck ( Cranio Cervical instability)
Pradeep Chopra, MD 17
Managing neck pain and headaches
from poor posture
• Place index finger in front of chin and push back head gently till
ears are in line with shoulders
• Large monitor, sit well balanced while working on a computer
• Manage POTS
Pradeep Chopra, MD 18
“My whole head hurts,
I see double,
I can feel my heart beat in my
ears, ”
Pradeep Chopra, MD
19
Headaches – Idiopathic Intracranial
Hypertension (IIH)
• Raised pressure inside the head
• Vision problems – double vision, sensitivity to light
• Ringing in the ears which pulses (pulsatile tinnitus)
• Maybe because of narrowing of blood flow (venous sinus stenosis)
• Diagnosis: spinal tap, eye exam, MR venography
• Treatment: medicines to decrease fluid pressure in the head, shunt to
drain excess fluid, stent
Pradeep Chopra, MD 20
“My headache gets worse when
I stand and it almost goes away
when I lie down”
Pradeep Chopra, MD
21
Low pressure inside the head - Spontaneous
CSF leak
Spontaneous CSF leak
• Headaches get worse being upright
• Almost resolve with lying supine
• Headaches worse by the end of the day
• Severe headache all day in long standing cases
• Nausea and vomiting
• Tinnitus – change in CSF pressure can cause change in inner ear
pressure
• Neck pain
23
Pradeep Chopra, MD
Differential diagnosis
CSF leak POTS
Headache + +
Headache worse
upright
+ +
Headache
better supine
+ + Not significantly better
Neck pain + +
Fatigue + +
Nausea + +
Orthostatics - + Heart rate greater than
30BPM with no change in BP
Dizziness - +
POTS: Postural Orthostatic Tachycardia Syndrome
24
Pradeep Chopra, MD
CSF leak - managements
• Increase PO fluids and caffeine
• Abdominal binder
• Epidural blood patch – high volume. May need multiple patches
• Directed epidural fibrin glue
• Surgical repair
Schievink WI, Gordon OK, Tourje J: Connective Tissue Disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial
hypotension: A prospective study. Neurosurgery 54:65-71, 2004
25
Pradeep Chopra, MD
“My headache gets worse when
I stand and it almost goes away
when I lie down”
Spontaneous CSF leak
26
Pradeep Chopra, MD
“My headache gets
worse when I cough,
I have tingling in my
hands and feet,
I have difficulty
swallowing”
Pradeep Chopra, MD
27
Pradeep Chopra, MD
Chiari Malformation
28
Pradeep Chopra, MD
Chiari malformation
Pradeep Chopra, MD 29
http://www.craniofacial.vcu.edu/conditions/chiari.html
Chiari malformation
http://www.craniofacial.vcu.edu/conditions/chiari.html
Pradeep Chopra, MD
Small posterior fossa
pushes the brainstem
and cerebellar tonsils
through the foramen
magnum into the spinal
canal
30
Symptoms of Chiari Malformation
• COMMON FEATURES
• Pressure headaches in the back of the
head (suboccipital)
• Neck pain
• Balance problems
• Numbness or paresthesia’s to arms or
legs
• Dizziness
• Difficulty swallowing
• Poor Hand co-ordination
• Headaches made worse by coughing or
straining
• Ringing or buzzing in the ears
• Hearing loss
• Nausea, vomiting
• Muscle weakness
• Vision problems
31
Pradeep Chopra, MD
Chiari Malformation and EDS
A diagnosis of Chiari Malformation depends on:
1.Good history and physical examination
2.Upright MRI of the neck (cranial settling in the upright position)
Pradeep Chopra, MD
32
Cranial Settling in EDS- Deformative
stresses on the brain stem, lower cranial
nerves, spinal cord
Clivo-axial angle normal 1400
33
Pradeep Chopra, MD
Chiari malformation
• The herniation causes obstruction to CSF flow.
• Empty Sella syndrome – flattening of the pituitary gland and resulting
hormonal changes.
• There may be an association between Chiari, Craniocervical instability
and Tethered cord syndrome in EDS
34
Pradeep Chopra, MD
“My headache gets worse
when I cough,
I have tingling in my hands
and feet,
I have difficulty swallowing”
Chiari Malformation
35
Pradeep Chopra, MD
Neck pain
3
6
Pradeep Chopra, MD
Cranio Cervical instability in EDS
• The neck is stabilized by ligaments
• Laxity of the ligaments causes the joints in the neck to move more
• Excessive movement of the joints in the neck causes cranio cervical
instability
Pradeep Chopra, MD 37
Remember a lot of these symptoms overlap with other
conditions
Cranio Cervical instability (CCI
• Neck pain / stiffness • Poor vision
• Headaches • Anxiety
• Dizziness • Lightheaded
• Paresthesia to face • Poor balance
• Fatigue • Difficulty swallowing
• Poor sleep
• Nausea
Pradeep Chopra, MD 38
Imaging for Cranio Cervical Instability
•Need functional imaging technology
•Static pictures are not helpful
•Functional computerized tomography (fCT scan)
•Flexion.
•Rotate neck left 90 degrees.
•Rotate neck right 90 degrees.
Pradeep Chopra, MD 39
Cranio Cervical instability in EDS – MRI scan
findings
• These measurements have to specifically asked for when getting an
MRI.
1. Clivo-axial angle (normal 140 to 160 degrees)
2. Harris Measurement (instability if > 12mm)
3. Grabb, Mapstone and Oakes measurement (> 9mm suggests high
risk of ventral brainstem compression)
Pradeep Chopra, MD 40
Pradeep Chopra, MD
Cranio Cervical Instability management
•Mild to moderate:
•Neck muscles strengthening exercises
•Hard cervical collar (Vista Aspen collar)
•Severe Instability:
•Surgical fusion
Pradeep Chopra, MD 41
Vista® MultiPost Therapy Collar – an
improved design
4
2
Pradeep Chopra, MD
Barrie – Lieou syndrome
• Cervical instability pushing on the
autonomic nerves
• Usually triggered by a whiplash
type injury
• Difficulty swallowing
• Tongue numbness
• Blurred vision
• Tinnitus
• Dizziness
• Neck pain
• Headaches
43
Ross Hauser, MD, Caring Medical
Soft neck collars are useless
“neck warmers”
Pradeep Chopra, MD
44
TMJ Pain
Temporo Mandibular Joint
Dysfunction
Pradeep Chopra, MD
Pradeep Chopra, MD
Temporo-Mandibular joint pain
Pradeep Chopra, MD 46
Pradeep Chopra, MD
TMJ Pain
• Very closely related to neck issues
• Clicking noises
• Clenching, grinding
• Pain with chewing
• Difficulty opening mouth wide (eating an apple)
• Jaw locking up
Pradeep Chopra, MD 47
Pradeep Chopra, MD
Dental issues in Ehlers Danlos Syndromes
• Teeth: weak and thin enamel, prone to cavities
• Gums: periodontal gum weakness, gingivitis, easy bleeding,
delayed healing after surgery, tissue breakdown after surgery
(tooth extraction), gum recession and pocketing
• Poor tooth stability, crowding of teeth
• Very high incidence of gingivitis – this increases inflammatory
reactions in the body (higher diabetes, higher arthritis etc)
Pradeep Chopra, MD 48
Livionex® - an excellent dental gel for EDS and MCAS
• No salicyclates
• Free of gluten, sugar, chemicals,
color dyes
• No harsh abrasives or detergents
• Helps rebuild enamel and
disrupts plaque formation
• Helps repair connective tissue to
close pockets
Pradeep Chopra, MD 49
Numbing medicine injections
• Commonly used numbing medicine injections (Lidocaine) may be
ineffective
• Carbocaine (Mepivicaine) works well in EDS
5
0
Pradeep Chopra, MD
Upper back pain
Pradeep Chopra, MD 51
Upper back pain in EDS
• Usually poor posture
• Shoulder instability
• Rib subluxation
• Doing a repetitive task with hands or arms (typing, vacuuming) -
Repetitive Strain Injury
• In women, it can be the weight of the breast tissue dragging the
upper torso forward and the muscles of the upper back attempting to
stabilize the torso
5
2
Pradeep Chopra, MD
Pradeep Chopra, MD
Upper back and Shoulder pain in women
with EDS
• Sports bra with:
• Helps upper back, shoulder and Thoracic outlet pain
• racer back (cross straps).
• Wide straps.
• Front closure
• Proper fitting – recommend getting it done
professionally.
Pradeep Chopra, MD 53
Pradeep Chopra, MD
High Racer back – Posture bra
2-51
5
4
Pradeep Chopra, MD
Compression garments
55
www.dmorthotics.com or
Pam Cowans -
p.cowans@dmorthotics.com
Pradeep Chopra, MD
Joint support compression. cw-x (check
Zappos.com)
56
Pradeep Chopra, MD
Pradeep Chopra, MD
Spinal Instability
• The spine is made up of multiple joints – held together by
ligaments and muscles.
• Spinal instability with reflex muscle spasms may happen
at any level
5
7
Pradeep Chopra, MD
Pradeep Chopra, MD
Spinal Instability
• Thoracic spine – subluxations where the ribs meet the spine
(costo—vertebral joints)
• Lumbar spine – subluxations of the facet joints.
• Sacroiliac joint pain (SI Joint) – maybe more from uneven
posture or pain from joints in the legs
• Kyphosis (spine poking backwards), scoliosis (spine sideways)
• Maybe a symptom of Tethered Cord syndrome
Pradeep Chopra, MD 58
Back pain can be from unstable joints in the
spine, causing muscles to spasm.
Pradeep Chopra, MD 59
Spinal pain
• If the pain is from the joints in the spine – postural
correction, compression garments, muscle
strengthening
• Steroid injections not very helpful but can be used
in very select cases
Pradeep Chopra, MD
Good support for the SI joint and lower lumbar region
Brace for lumbar and Sacroiliac joint
– Lumbo Lady
Pradeep Chopra, MD 61
Tethered Cord Syndrome
Pradeep Chopra, MD 62
Tethered Cord Syndrome (TCS)
http://javid1985.hubpages.com/hub/Tethered-Spinal-Cord-Syndrome-What-
You-Need-To-Know Pradeep Chopra, MD 63
Clinical symptoms
• Low back pain
• Neurogenic bladder
• Leg weakness and sensory loss
• Musculoskeletal abnormalities
• Toe walking
• Heel walking
6
4
Pradeep Chopra, MD
Neurogenic bladder
• Bladder symptoms (Neurogenic bladder)
• Increased (decreased) frequency,
• Urgency
• Sense of incomplete emptying of the bladder
• Incontinence
Pradeep Chopra, MD 65
Pain from Ribs in EDS
6
6
Pradeep Chopra, MD
Breathing and rib pain
• Patients with EDS often have “Air Hunger” – a feeling of having
forgotten to take a breath
• Pain from ribs
• All the tests for heart and lungs are normal.
• Each rib has 3 joints in the back 6
7
Pradeep Chopra, MD
6
8
Pradeep Chopra, MD
Breathing and rib pain
• Loss of proprioception from the ribs, muscles of breathing and
diaphragm gives a feeling of not having taken a full breath in or full
breath out
• Similar to the uncoordinated movement of the rest of the joints in the
body
• Some of the muscles of breathing are also part of the lower back
Pradeep Chopra, MD
Exercise to help with chest wall pain (and
lower back)
• Book or sand bag should be over
the belly button
• Lie on your back
• Breathe in and breathe out for
20 minutes a day – lifting the
book up
70
http://www.normalbreathing.com/
Diaphragm release exercise
• Lie on your stomach on the floor
• Place a 1.5 inch rubber ball under your belly button
• Just lie comfortably in this position for 20 minutes.
• If you get uncomfortable before a minute, use a smaller ball.
7
1
Pradeep Chopra, MD
Chest wall pain and rib subluxations
• Singing (high and low notes), wind instrument like a flute or recorder
• May help with chest muscle and rib pain and strengthening lower
back
• Rib subluxations happen with uncoordinated breathing (remember
poor proprioception), and poor posture
Pradeep Chopra, MD
Pradeep Chopra, MD
Pain in the arms in EDS
7
3
Pradeep Chopra, MD
Pradeep Chopra, MD
Pain in arms
• Shoulder joint subluxations, dislocation
• Thoracic outlet syndrome
• Elbows: Tendonitis, bursitis, hyperextension
• Wrist and fingers: subluxations, muscle pain, tendonitis
Pradeep Chopra, MD 74
Pradeep Chopra, MD
Shoulder pain
• Laxity of the shoulder joint causes the muscles (rotator cuff)
around the shoulder to spasm
• Thoracic Outlet syndrome.
Pradeep Chopra, MD 75
Pradeep Chopra, MD
Pain patterns in Thoracic Outlet syndrome
Pradeep Chopra, MD 76
Pradeep Chopra, MD
Thoracic Outlet Syndrome
Pradeep Chopra, MD 77
Pradeep Chopra, MD
Thoracic Outlet Syndrome
•Thoracic Outlet exercises
•Kinesio taping
•Stabilize shoulder
•surgery
Pradeep Chopra, MD 78
Pradeep Chopra, MD
Kineseo Taping for shoulder joint pain
79
Pradeep Chopra, MD
Pradeep Chopra, MD
The EDS way of holding a pen
• Poor proprioception makes patients
grip a pen with as many fingers as
possible
• They hold the pen very tight and press
down hard on paper (poor haptic
feedback)
• Puts abnormal pressure on the
muscles and joints of the hand and
wrist
80
Pradeep Chopra, MD
• Dense foam padding (Ableware®) or
wrap a foam padded tape – for
pens, tooth brush, forks, knives
• Compression half finger gloves
• Brace for unstable joints
81
Pradeep Chopra, MD
Splints for fingers
82
Splinting and braces in general
• Braces maintain joint in neutral position
• Avoid hyper – extension
• Braces help with joint position awareness (proprioception)
• Start using them gradually
• Gradually decrease their use as you gain strength
Pradeep Chopra, MD 83
Do braces make your muscles weaker?
• No, they do not cause muscle atrophy
• It’s a common misconception
• Braces are not tight enough to stop muscles from moving
• In fact, braces stabilize joints so the muscles can move the joints more
efficiently.
Pradeep Chopra, MD 84
Legs
Pradeep Chopra, MD 85
Pain in lower half of the body
• Pay attention to feet and ankles.
• If the feet and ankles are unstable, they make
• The knees even more unstable, which then
• Makes the hips unstable, which then
• Throws the pelvis off – Sacroiliac joint pain, lumbar pain
Pradeep Chopra, MD 86
87
Pradeep Chopra, MD
Flexible flat feet – predominantly in the
forefoot
Pradeep Chopra, MD 88
Pronation
Pradeep Chopra, MD 89
http://www.integrativepersonaltraining.co
m/blog/run-flat-feet-knee-pain/
Pradeep Chopra, MD
90
Pradeep Chopra, MD
Pradeep Chopra, MD
The feet in EDS
• Barefoot walking, where safe and comfortable – helps with
conditioning of muscles under natural loads
• Repeated rising on tip toes – strengthens the muscles in foot and with
proprioception
• Ankle raises by lifting heel (not leaning forward)
• Descend in a slow controlled way
91
Pradeep Chopra, MD
Pradeep Chopra, MD
Footwear - shoes
• Extremely important to wear proper footwear
• Help with unstable ankles, hypermobile feet
• Cushioned mid sole
• Good, strong heel counter provides stability
• Fastenings should be over the mid-sole for better support
• Sneakers !!
92
Pradeep Chopra, MD
Pedag
www.pedagusa.com
Pradeep Chopra, MD 93
Pradeep Chopra, MD
Ankle brace to stabilize the ankle joint
94
Pradeep Chopra, MD
Pradeep Chopra, MD
Knees
2-114
95
Pradeep Chopra, MD
Pradeep Chopra, MD
Patella is stabilized muscles of the thigh
2-115
96
Pradeep Chopra, MD
Hyperextension at the knees
Pradeep Chopra, MD 97
Kinesio taping – EDS knee
• A combination of two strips of 25
cm in length and 2.5cm in width
along the collateral ligament (sides
of the knee) using 50% tape
tension applied distally (furthest)
to proximal, a horizontal tape
below the patella 25 cm in length
and 2.5 cm in width applied with
25% tension and lastly a Y tape 30
cm in length and 5cm in width cut
with 5cm in initial base applied
laterally to the patella with no tape
tension.
Pradeep Chopra, MD 98
Ther Adv Musculoskelet Dis. 2015 Feb; 7(1): 3–10. doi: 10.1177/1759720X14564561 PMCID: PMC4314299 The effects of
neuromuscular taping on gait walking strategy in a patient with joint hypermobility syndrome/Ehlers–Danlos syndrome
hypermobility type Filippo Camerota, Manuela Galli, Veronica Cimolin,corresponding author Claudia Celletti, Andrea Ancillao,
David Blow, and Giorgio Albertini Author information
Pradeep Chopra, MD
Treatment options for knee pain in EDS
• Stabilize the feet and ankles, first
• Strengthen muscles around the knee
• Knee brace
• Two straps above knee
• Two straps below knee
• Patella stabilizer
• Metal strut to prevent hyperextension
Pradeep Chopra, MD
Wrap around knee brace - Gripper
Pradeep Chopra, MD 100
Pradeep Chopra, MD
A missed cause of leg pain – the
proximal tibio fibular joint
101
Pradeep Chopra, MD
Pradeep Chopra, MD
Knee pain – often missed cause
• Site of pain from the proximal
Tibiofibular joint
• It can inflame the peroneal
nerve which causes pain down
the side of the leg and even foot
drop
102
Proximal Tibio-Fibular joint
Pradeep Chopra, MD 103
Proximal Tibio-Fibular joint
Pradeep Chopra, MD 104
Tendonitis and bursitis
105
Pradeep Chopra, MD
Pradeep Chopra, MD
Tendonitis and bursitis in EDS
106
Courtesy http://www.ateevia.com/bursitis.aspx
Misaligned bones and
tendons
Subluxed
bones
Pradeep Chopra, MD
Tendonitis and bursitis in EDS
• Treatment lies in correcting the underlying problem –
•correct bracing to align the joints
•Correct posture (especially standing)
•Avoid repetitive use of joint
•Maintain proper balance
107
Pradeep Chopra, MD
Sacroiliac joint pain
• Very common cause of low back pain
• Usually around the lower back, radiates into the buttock region and may even go
down the legs (but not below the knee)
• Groin - occasionally
108
Pradeep Chopra, MD
109
Pradeep Chopra, MD
• Leg length discrepancy
110
Pradeep Chopra, MD
LumboTrain® Lady –
a good brace for SIJ
Pradeep Chopra, MD 111
Dysautonomia / POTS (Postural Orthostatic
Tachycardia Syndrome)
112
Pradeep Chopra, MD
Dysautonomia
• This is a fancy name for a bunch of medical conditions that affect the
nerves that control automatic functions in our body.
• “Dysfunction of Autonomic Nervous System”
• PoTS – Postural Orthostatic Tachycardia Syndrome
• Its one of the most common types seen in EDS
Pradeep Chopra, MD 113
Postural Orthostatic Tachycardia syndrome
(PoTS) - Symptoms
• Fainting, dizziness
• Heart racing (Palpitations)
• Fatigue
• Headaches
• Difficulty maintaining body temperature
• Poor concentration “brain fog”
• Feeling of constant anxiety
• Stomach pain
• Wake up feeling tired
114
Pradeep Chopra, MD
PoTS
• Patients with EDS pool excessive blood to their lower extremities,
• This triggers an increased activity in the same nervous system that
controls ‘fight or flight’
Pradeep Chopra, MD 115
POTS - Postural Orthostatic Tachycardia syndrome
Dysautonomia International for more information and high salt diet recipes.
http://www.dysautonomiainternational.org/
• EDS Awareness
https://www.chronicpainpartners.com/
116
Pradeep Chopra, MD
PoTS
• The constant feeling of dizziness makes patients feel unstable
• The laxity of the joints makes the muscles tighten reflexly
• This constant imbalance of muscles worsens their pain and fatigue
117
Pradeep Chopra, MD
Anxiety in EDS
•Patients with EDS and PoTS (Dysautonomia) are often
over diagnosed to have anxiety
•Symptoms of undiagnosed palpitations, fatigue,
dizziness, chronic pain are attributed to ‘anxiety’.
•In most cases its PoTS– its because of excessive
activity of the flight or fight nerves to control blood
pressure.
Pradeep Chopra, MD
118
POTS - Postural Orthostatic Tachycardia syndrome
• Increase in heart rate by 30 beats/min within 10 minutes of standing
• heart rate of 120 beats / min within the first 10 minutes of standing
• No significant change in blood pressure
• Syncope or almost syncope (fainting)
• In children an increase of 40 beats/minute
Pradeep Chopra, MD 119
POTS - tests
• Orthostatics – Measure blood pressure and heart rate:
• after lying supine for 5 minutes
• standing up - immediately
• Standing for 10 minutes
• Q-sweat test
• Tilt Table Test
Pradeep Chopra, MD 120
Sample readings in POTS
Lying flat for 5 minutes 110/66 82
Standing, immediately 100/68 115
Standing for 10 minutes 112/65 108
Pradeep Chopra, MD 121
PoTS - treatment
• Increase salt by mouth (salt pills: http://saltstick.com/vitassiumpage/)
• Increase electrolyte oral fluids
• Trial of ‘Banana bag oral rehydration fluid https://www.bananabagdrink.com
or Gatorade
• Compression tights up to thighs.
• Abdominal binder or trial of a one size smaller swimsuit.
• Cardiology or Neurology consult for Dysautonomia/POTS.
• Consider a beta-blocker such as Metoprolol
Pradeep Chopra, MD 122
Treatment of POTS
• Exercise is the key here.
• Exercise lying down.
• Dallas protocol
• Exercise in pool – simple walking, treading water
• https://youtu.be/jbGNfuXVHwc
• https://youtu.be/oXnNL_furCw
• https://youtu.be/hB8yr1e823Q
Pradeep Chopra, MD 123
Courtesy: Dysautonomia International
Treatment of POTS
• Exercises lying down:
• https://youtu.be/jbGNfuXVHwc
• https://youtu.be/oXnNL_furCw
• https://youtu.be/hB8yr1e823Q
Pradeep Chopra, MD 124
Courtesy: Dysautonomia International
and RSDSA
Exercise in pool for EDS
• Exercises in pool:
• https://youtu.be/g7df_ek50Jk
• https://youtu.be/mVvzsFk6rPo?list=PLPS8D21t0eO-
X68fTNKk7UEkegxDqzQ3U
• This one is also good for CRPS/RSD
• https://youtu.be/wUnwbNsIk1c
Pradeep Chopra, MD 125
POTS – one last thing
• POTS can be because of other conditions such as Sjogrens syndrome,
Antiphospholipid syndrome, celiac disease, anykylosing spondylitis.
Myasthenia gravis, multiple sclerosis
• Can be seen in Autoimmune conditions where patients develop
antibodies to alpha 1, Beta 1, Beta 2 adrenergic receptors, muscarinic
3 receptors, (12)g-Acetylcholine receptors, cardiac lipid raft proteins.
• These patients may respond well to IVIg infusions.
Pradeep Chopra, MD 126
Postural Orthostatic Tachycardia syndrome
(PoTS) - Symptoms
• Fainting, dizziness
• Heart racing (Palpitations)
• Fatigue
• Headaches
• Difficulty maintaining body temperature
• Poor concentration “brain fog”
• Feeling of constant anxiety
• Stomach pain
• Wake up feeling tired
127
Pradeep Chopra, MD
Mast Cell Activation
Syndrome
MCAS
Pradeep Chopra, MD 128
Mast Cell Activation Syndrome (MCAS)
• Mast cells are a type of cell found in the blood.
• They are part of our body defense mechanism (immune system)
• When they detect an attack (infection or something that is not good
for the body) they get activated
• Once mast cells get activated they release a bunch of chemicals such
as histamine, cytokines etc
Pradeep Chopra, MD 129
Mast Cell Activation Syndrome (MCAS)
• In MCAS, mast cells are inappropriately triggered and release
excessive amount of these chemical mediators (histamine, cytokines
etc.)
• It is NOT mastocytosis (whole different condition)
Pradeep Chopra, MD 130
Mast Cell Activation Syndrome (MCAS)
• Rashes, hives, itchy
• Fatigue, tiredness
• Muscle pain
• Bone and joint pain
• Abdominal pain, bloating, nausea
• Palpitations, pre-syncope
• Flushing especially after a hot shower
• Constant feeling of having flu
Pradeep Chopra, MD 131
Dermatographism (writing on skin)
Urticaria Multiforme - Scientific Figure on ResearchGate. Available from: https://www.researchgate.net/Dermatographism-Linear-wheals-after-
writing-on-the-skin_fig4_236106893 [accessed 3 Oct, 2018]
Pradeep Chopra, MD 132
Mast Cells
Pradeep Chopra, MD 133
Red Blood Cell
Mast Cell
MCAS
134
Courtesy Ann Maitland, MD
Pradeep Chopra, MD
Mast Cell Activation Syndrome (MCAS)
• Rashes, hives, itchy
• Fatigue, tiredness
• Muscle pain
• Bone and joint pain
• Abdominal pain, bloating, nausea
• Palpitations, pre-syncope
• Flushing especially after a hot shower
• Constant feeling of having flu
Pradeep Chopra, MD 135
MCAS – Testing
• Tests for MCAS are NOT reliable. However, these test can be done:
• Urine 24 hour collection for N-methylhistamine.
• Same 24 hour collection Prostaglandin D2/F2 or 11 Beta
prostaglandin F, whichever is available.
• Serum tryptase.
• Serum Chromogranin A level
Pradeep Chopra, MD 136
MCAS - tests
• The diagnosis of MCAS depends largely on a physician who knows
MCAS and is clinical
• Laboratory tests are very difficult and unreliable.
• If a biopsy sample is taken from endoscopy then:
Please request Pathologist for the following:
• Number of Mast Cells per high power field.
• Stain for Mast Cells with CD117.
• Comment on the morphology and histology.
• Request KIT-D816V mutation analysis by PCR
Pradeep Chopra, MD 137
MCAS – treatment
• Ranitidine (Zantacâ) 150mg twice a day.
• Loratidine (Claritinâ) one tablet twice a day.
• Cetrizine (Zyrtecâ) 10mg one tablet twice a day.
• Clemastine 2mg p.o. b.i.d.
• Rupatadine 10mg p.o. b.i.d.
• Quercetin 1000 mg / day
• Consider Ketotifen.
• Consider Cromolyn oral 200mg four times a day.
• Cromolyn Nebulized.
Pradeep Chopra, MD 138
MCAS – treatment
• Avoid triggers for Mast cell activation such as NSAIDs, alcohol, opioids and
sudden temperature changes.
• Inactive ingredients (binding agents, preservatives) in pills may contribute to
MCAS.
• Consider a leukotriene inhibitor such as Singulair.
• Low histamine diet.
Pradeep Chopra, MD 139
MCAS – treatment
• Review and change possible hidden food sources and the possibility of
accidental exposure to such foods.
• Some of the usual suspects for MCAS to avoid are:
• Seasonings (except olive oil, salt).
• All dairy.
• Eggs.
• Grains except quinoa and rice.
• Avoid beef products but okay to take lamb, venison and poultry.
• Food and drug reactions are a common cause of unexplained hives and
rashes. Some of the other causes of hives may be exposure to change in
temperature (hot to cold), rubbing skin, physical exertion, pressure and direct
exposure to sunlight.
Pradeep Chopra, MD 140
MCAS – treatment
• Other methods to avoid exacerbation maybe:
• Use pollen guards in windows.
• HEPA air filters, A/C.
• Use a mask (e.g. VOG mask) while dusting or vacuuming.
• Avoid long baths or showers, rapid changes in temperature, wool, dust and cigarette
smoke.
• May use nasal saline during flare ups
Pradeep Chopra, MD 141
Abdominal Pain
Pradeep Chopra, MD 142
Abdominal pain
• Very common in EDS
• Slowing down of the intestines
• Alternating diarrhea and constipation
• PoTS, MCAS –causes nausea, acid reflux, bloating and constipation
• Prolapse
• Acid reflux from MCAS
Pradeep Chopra, MD 143
• Low FODMAP diet – for bloating, pain and diarrhea
• Treating PoTS and Mast Cell Activation Syndrome helps
• In case you are wondering:
• FODMAP - Fermentable Oligo-, Di-, Mono-saccharides And Polyols
Pradeep Chopra, MD 144
Low FODMAP diet
• https://www.kcl.ac.uk/lsm/research/divisions/dns/projects/fodmaps/faq.aspx
• There are 3 stages to the low FODMAP diet:
• Stage 1: Restriction: In this stage, you reduce your FODMAP intake by avoiding foods that
are high in FODMAPs for four to eight weeks as this period is considered long enough to
identify if symptoms will respond to a low FODMAP diet.
• Stage 2: Reintroduction: If your symptoms have improved following FODMAP restriction,
it is important to reintroduce some high FODMAP foods. This will enable you to identify
which FODMAPs you are most sensitive to, as well as how much of a high FODMAP food
triggers your symptoms.
• Stage 3: Personalization: The long term aim of a low FODMAP diet is to personalize your
diet so you only avoid foods that trigger your symptoms and you return to as normal a
diet as possible.
1
4
5
Pradeep Chopra, MD
Dysfunction of movement of intestines
• Can lead to changes in the gut microbiome (the helpful bacteria that
live in our intestines)
• Problems with movement of intestines may lead to Small Intestinal
Bacterial Overgrowth (SIBO)
Pradeep Chopra, MD 146
Small Intestinal Bacterial Overgrowth -
symptoms
• Abdominal pain/discomfort
• Bloating
• Diarrhea
• Weakness
• gassy
Pradeep Chopra, MD 147
SIBO – problems
• Weight loss
• Vitamin deficiency – A,D,E,K, B12,
• Vitamin excess – Folate
• Iron deficiency
• Low protein
Pradeep Chopra, MD 148
SIBO – testing
• Organisms in the intestine produce hydrogen and methane.
• Breath test can detect levels of hydrogen and methane
• Elevated methane levels can be treated with statins (lovastatin – red
rice yeast)
• Treat any dysautonomia
• Treat any MCAS
• Treat constipation
• Drugs for improving GI motility (Resolor, Iberogast, Mestinon,
erythromycin)
Pradeep Chopra, MD 149
SIBO – treatment
• Elevated methane levels can be treated with statins (lovastatin – red
rice yeast)
• Specific antibiotics to treat SIBO.
• Be cautious of repeating antibiotics
• Treat any dysautonomia
• Treat any MCAS
• Treat constipation
• Drugs for improving GI motility (Resolor, Iberogast, Mestinon)
Pradeep Chopra, MD 150
SIBO – treatment
• Avoid simple carbohydrates
• Think of pureed foods.
Pradeep Chopra, MD 151
Median Arcuate Ligament
Syndrome
MALS
Pradeep Chopra, MD 152
Abdominal pain – MALS
• Median Arcuate Ligament Syndrome (MALS).
• Belly pain after eating food.
• Can trigger PoTS
Pradeep Chopra, MD 153
Abdominal pain – MALS
• There is an artery that supplies blood to part of the intestine
• It is called Celiac Artery
• This artery pokes out from under a ligament called Median Arcuate
ligament
• There are 2 theories about why MALS causes pain
• The median arcuate ligament is tight causing compression of the
celiac artery
• There is a nerve in that area called Celiac ganglion which may be
getting compressed or inflamed.
Pradeep Chopra, MD 154
Pradeep Chopra, MD 155
Abdominal pain – MALS
• Treatment is surgical decompression
• If treated early, symptoms resolve
• If treated late, may need IVIg infusion
Pradeep Chopra, MD 156
Pelvic pain
157
Pradeep Chopra, MD
Pelvic pain in EDS - Dysmenorrhea (painful
menstrual periods)
• Birth control to stop periods – may help with joint laxity also
• NSAID’s (ibuprofen, naproxyn)
• Control Mast Cell Activation Syndrome
158
Pradeep Chopra, MD
Pelvic pain in EDS - Interstitial cystitis (bladder
pain)
• Maybe part of Mast Cell Activation syndrome
• Cromolyn or Ketotifen
• Palmitoyl ethanolamide (PEA)
159
Pradeep Chopra, MD
Pelvic pain in EDS – Vulvodynia (vaginal pain)
• Maybe part of Mast Cell Activation syndrome
• Benadryl douche or cream
• Cromolyn douche or cream
160
Pradeep Chopra, MD
EDS and children
1
6
1
Pradeep Chopra, MD
EDS and children
• dislocated joints,
• chronic body wide pain
• Easy bruising
• Abdominal pain
• Fatigue
• Unidentified bleeding (intracranial, abdominal)
• To the ER physicians they look suspicious of Medical Child abuse
Pradeep Chopra, MD 162
• The diagnosis of Conversion Disorder or Munchausen by Proxy are
often made by providers with little training in Psychiatry and vice
versa most psychiatrists have no training in pain conditions.
• Parents – please research the facility carefully before taking your child
there.
• The facility should have trained physicians in EDS and its
complications
• There are some hospitals that have hired “Medical Child Abuse”
specialists.
Pradeep Chopra, MD 163
Behavioral
• To diagnose a child with Conversion disorder or Munchausen by Proxy
without a dedicated multidisciplinary team approach and without
concrete evidence is extremely harmful to the patient.
• To label an adult with pain as being psychological is severely harmful.
It closes all doors to correct treatment.
Pradeep Chopra, MD
164
Misdiagnosis
• Please check with other patients to get feedback before you go to a
hospital.
• If you sense that the doctors are skeptical of your complaints,
consider going somewhere else.
• When you go to an a academic hospital, chances are that you will be
seen by a junior doctor / Resident – who is the least qualified to
manage complex medical conditions.
• If you are able, choose a Concierge physician.
Pradeep Chopra, MD 165
Service Dogs - invaluable
• POTS – they can sense when their owner is having an episode of
dizziness or seizure
• EDS and pain - they protect the limb from being injured or touched
• Helps boost confidence in their owners, making them more independent
• Help with balance, call for help, open doors, switch on lights, pull
wheelchairs, anxiety,
Pradeep Chopra, MD
166
Some organizations that can help get a
service dog
• https://www.pawswithacause.org
• http://www.neads.org
• http://freedomservicedogs.org
Pradeep Chopra, MD 167
To manage pain first find out what’s broken
• It is crucial to understand the cause of the pain before deciding on
treatment options.
• For example, pain in the shoulder joint can be from:
• Dislocated shoulder joint,
• Muscle spasms
• Nerve or blood vessel impingement or damage
• All of the above
• The treatment of each of these is different
Pradeep Chopra, MD
168
Types of tissue injury in EDS
• Micro trauma – small repetitive traumas resulting in constant tissue breakdown.
Invisible tissue breakdown. This is far more common.
For example – overstretching muscles and ligaments. Vacuuming, chopping food,
dusting
• Macro trauma – a large event or trauma resulting in injury.
For example - Dislocation or fracture
169
Pradeep Chopra, MD
Macro trauma Micro trauma
170
Pradeep Chopra, MD
Principles of managing pain in EDS
• Use a mix of treatments
• For example - Knee pain due to instability
• Stabilize knee with braces
• Strengthen muscles around the knee
• Medications – NSAID’s, topical etc.
• Follow the NC10 rule (if you can get at least 10% from one
treatment, five such treatments will give you 50% relief)
Pradeep Chopra, MD
171
Joint Position sense
Proprioception
The body’s ability to sense movement of the joints and their position
Pradeep Chopra, MD 172
Proprioception (Joint Position Sense)
• Proprioception, also known as Joint Position Sense, is the ability of a
joint to
1. determine its position,
2. detect movement, and
3. sense of resistance to force
Pradeep Chopra, MD 173
Proprioception – Joint sense
• The brain constantly gets information from the joints as to the exact
position of the limbs in space.
• It helps us walk, use our arms, maintain our posture without tipping
over.
• Protects our joints from over extending and our muscles from over
stretching
• EDS – poor proprioception.
Pradeep Chopra, MD 174
Proprioception – Joint sense
• The brain uses sensors in the skin as a way of figuring out the position
of different parts of the body.
• Wearing skin tight clothes over joints and limbs will help the brain
understand where the body is
• One of the reasons that exercising in water helps is because your skin
senses contact with water
Pradeep Chopra, MD 175
Proprioception exercises
• Balance board or wobble board
• Stork standing (stand on one leg)
• Stand up paddle board (SUP)
• Sitting on exercise ball
• Exercise in water – walking, treading but avoid swimming
176
Pradeep Chopra, MD
Stand up paddle board
177
www.vimbly.com
Pradeep Chopra, MD
Compression garments
Pradeep Chopra, MD 178
Proprioception
Key concept
Pradeep Chopra, MD 179
Proprioception – Joint sense
Pradeep Chopra, MD 180
Proprioception exercises
• Juggling
• Balance board or wobble board
• Stork standing (stand on one leg)
• Stand up paddle board (SUP)
• Sitting on exercise ball
• Exercise in water – walking, treading but NO swimming
Pradeep Chopra, MD 181
Exercise in water - walking
Pradeep Chopra, MD 182
Pradeep Chopra, MD 183
Exercises for EDS
184
Pradeep Chopra, MD
Exercises for EDS
• Muscles strengthening without stressing the joints – for stabilizing
joints
• Building on proprioception – for joint protection and efficient use of
muscles.
• Exercising with fatigue
• It should be daily, even if it’s a little bit
185
Pradeep Chopra, MD
Muscle strengthening exercises
• Avoid joint loading or joint stressing
• Focus on muscle strengthening
• Avoid extreme stretching
• Start very low, really very low
• Progress slowly, really slow
186
Pradeep Chopra, MD
Keep limb movements within extreme range
of motion
187
Pradeep Chopra, MD
Keep limb movements within extreme range
of motion
188
Pradeep Chopra, MD
Aquatic therapy
• Best form of exercise in EDS
• The contact of water with the skin helps the brain move your muscles
more efficiently
• The water makes us weigh less which takes the load off the joints
allowing us to exercise freely
• Avoid swimming – it strains the joints of the neck and shoulders.
189
Pradeep Chopra, MD
Avoid swimming – injury to shoulder and neck
190
Pradeep Chopra, MD
Pradeep Chopra, MD
Neuromuscular taping
(Kinesio™/ Rock Tape ™ )
1
9
1
Pradeep Chopra, MD
Pradeep Chopra, MD
Finger print pattern
2-29
1
9
2
Pradeep Chopra, MD
Pradeep Chopra, MD
Kinesio™ taping - mechanism
• Mimics the superficial layer of skin – after 10
minutes you can not feel it
• Designed to stretch
• Porous – allows for drying easily. You can take a
shower with it on
• The adhesive is applied in a wave like pattern to
mimic the qualities of fingerprints.
1
9
3
Pradeep Chopra, MD
Pradeep Chopra, MD
Kinesio™ taping - mechanism
• The tape stimulates the sensors in the skin as we
move – improves proprioception
• Helps reduce swelling
1
9
4
Pradeep Chopra, MD
Pradeep Chopra, MD
Kinesio™ taping - uses
• Reduces pain
• Improves Proprioception
• Relaxes muscles
• Stabilizes joints
• Supports weak joints
• Reduces swelling
1
9
5
Pradeep Chopra, MD
How to prevent skin reactions to K-tape
•Cavilon cream® by 3M
•Leukotape® K
•Milk of magnesia
1
9
6
Pradeep Chopra, MD
The Feldenkrais Method
Pradeep Chopra, MD 197
The Feldenkrais Method
• It is a type of physiotherapy that helps repair impaired connections between
the brain and the body
• Patients with EDS and chronic joint conditions develop inefficient or strained
habitual movement patterns
• It uses slow repetition to teach correct and safe movements in EDS
• The movements are gentle, slow, repeated movements.
• http://www.feldenkrais.com
Pradeep Chopra, MD
198
Starting treatment - medicines and exercise
Start low, go slow
Pradeep Chopra, MD 199
Miscellaneous - Amazon
Pradeep Chopra, MD 200
Miscellaneous – Palmitoylethanolamide(PEA)
topical cream 2%
Sold as Soothamide.
Moderate relief
Pradeep Chopra, MD 201
CGRP antibodies
• CGRP – Calcitonin Gene Related Peptide
• It is a protein in the body involved in transmission of pain
• CGRP antibodies are medicines to block the effect of CGRP and
decrease pain
• In the last few months there have been 2 such CGRP antibodies on
the market and a few to be released soon.
• Currently, approved for treatment of migraines only.
• They may be effective for other pain conditions.
Pradeep Chopra, MD 202
Poor concept of exercising in pain
•No pain, no gain
Pradeep Chopra, MD 203
Poor concept of exercising in pain - baloney
•No pain, no gain
Pradeep Chopra, MD 204
Fatigue in EDS
205
Pradeep Chopra, MD
Fatigue
• EDS
• PoTS / Dysautonomia
• Mast Cell Activation Syndrome (MCAS)
• Drugs
• Pain
• Poor sleep
• Secondary Mitochondrial dysfunction
206
Pradeep Chopra, MD
Fatigue - causes
• Gastrointestinal: Median Arcuate Ligament Syndrome (MALS)
• Obstructive sleep – tracheomalacia (EDS), sleep apnea
• Hormonal - Hypothalamus - Pituitary-adrenal axis suffering from
hypoperfusion or Sheehan’s syndrome.
• Sleep apnea: Obstructive, central apnea, non-restorative sleep
• Non-restorative sleep secondary to Dysautonomia
Pradeep Chopra, MD 207
Symptoms of EDS, POTS, MCAS
208
Belly pain
Racing heart
Blurry vision
Headaches, brain fog, dizzy
Dark discoloration of feet
Tremulousness
www.potsuk.org
Pradeep Chopra, MD
Pradeep Chopra, MD
Fatigue in EDS
Non Ehlers Danlos Syndrome
• Muscles – relaxed at rest and contract
with activity
• Ligaments – tensed at rest, they
support and stabilize the body
209
Ehlers Danlos Syndrome
• Muscles – They are tensed and constantly
attempt to stabilize even at rest
• Ligaments – provide no tension and
stability
Medicinal Marijuana
Pradeep Chopra, MD 210
Medicinal marijuana
• The human body has two types of receptors – CB1 and CB2
• CB1 receptors are found in the brain
• CB2 receptors are found in the rest of the body, immune cells and glia
cells in the Central Nervous System
• Chemicals that cause inflammation in the peripheral parts of the body
are modulated by cannabinoids. Hence, cannabis applied topically are
helpful
Pradeep Chopra, MD
211
Medicinal Marijuana
• Reasonable choice to try.
• Anecdotally – works well in patients with EDS
• Higher CBD levels and lower THC levels
• Vaporizing, edibles
• Topical CBD over joints, tendons, ligaments – very helpful
• Does not affect Mast Cell Activation Syndrome (MCAS) as much as
NSAID’s and opioids
Pradeep Chopra, MD
212
Medicinal Marijuana
• Reasonable choice to try.
• Anecdotally – works well in patients with EDS
• Higher CBD levels and lower THC levels
• Vaporizing, edibles
• Topical over joints and muscles.
• Does not affect Mast Cell Activation Syndrome (MCAS) as much as
NSAID’s and opioids
Pradeep Chopra, MD
2
1
3
Daytime use
2
1
4
Pain
Sleep
Pradeep Chopra, MD
For sleep
2
1
5
Pain
Sleep
Pradeep Chopra, MD
Low Dose Naltrexone
LDN
Pradeep Chopra, MD
216
3-65
Low Dose Naltrexone (LDN)
• Competitive antagonist of opioid receptors
• Clinically used for 30 years for addiction
• Suppressive effects on the CNS glia, whch helps with Central
Sensitization
Pradeep Chopra, MD 217
3-66
Low Dose Naltrexone (LDN)
• Dose can vary anywhere between 1.75mg to 4.5mg
• May cause insomnia, mild headaches initially.
• Patients report increased physical activity, flare ups not as acute,
better tolerance to pain.
• Recommend a trial of at least 6 months
• To avoid all opioids or tramadol.
Pradeep Chopra, MD 218
Medications in EDS
• Patients with EDS may have an abnormal response to medications
• Maybe very sensitive,
• May have a poor response
• May need higher or lower doses than usual
Pradeep Chopra, MD 219
Muscle Relaxants
• Limited value in EDS
• Cyclobenzaprine (Flexeril)
• Carisoprodol (Soma)
• Tizanidine (Zanaflex)
• Baclofen
• Benzodiazipine (Valium)
Pradeep Chopra, MD 220
Acetaminophen/ Paracetamol
• One of the safest analgesic in EDS
• By itself, not a strong analgesic
• Potentiates the effects of other analgesics (NSAID’s, opioids)
Pradeep Chopra, MD 221
Non steroidal Anti-inflammatory (NSAID)
• Risks with taking it orally
• May combine with acetaminophen / paracetamol to lower dose
• Helpful with inflammatory pain
• Good value to using it topically
Pradeep Chopra, MD 222
Opioids / Narcotics
• Good analgesic
• Short term use to break the cycle of pain or muscle spasm
• In general, most patients with EDS do not respond well to opioids
• Long term use – risk of opioid hyperalgesia
Pradeep Chopra, MD 223
Adjuvants
• Anti-seizure, anti-depressents
• May use only if there is a neuropathic component to the pain
Pradeep Chopra, MD 224
Anti-seizure medicines
• Gabapentin
• Pregabalin
Pradeep Chopra, MD 225
Anti-depressants
• Tricyclic antidepressants – amitriptyline, nortriptyline, doxepin
• SNRI – duloxetine, milnacipran
• SSRI – not very helpful for pain.
Pradeep Chopra, MD 226
Suggested treatments (future)
• Oxygen (Prof Hamonet)
• Hormonal modulation (Dr. Gompel)
• Levodopa/carbidopa for muscle spasms
• EPSOM (Magnesium) salt bath
• Topical analgesics – predictable absorption
Pradeep Chopra, MD 227
Oxygen supplementation
• Anecdotal experience
• Oxygen by non-rebreather mask 3 liters to 5 liters, 20minutes per day
up to twice a day.
• May administer as needed for severe symptoms of fatigue or
dizziness.
Pradeep Chopra, MD 228
Mineral supplement for fatigue
• Anecdotal experience
• Combination of CoQ10, L-carnitine, creatinine
• Good experience with fatigue
Pradeep Chopra, MD 229
Levodopa-carbidopa
• Anecdotal experience
• Significant relief for muscle spasms and dystonia
• Low dose
Pradeep Chopra, MD 230
Thank you
Pradeep Chopra, MD
snapa102@gmail.com
Pradeep Chopra, MD 231

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  • 1. Connecting the dots with EDS, POTS, MCAS, GI, Neurological and Physiotherapy in managing pain in EDS Pradeep Chopra, MD Pain Medicine Assistant Professor (Clinical) Brown Medical School. USA Email: snapa102@gmail.com 1 Pradeep Chopra, MD
  • 2. Disclosure and disclaimer • I have no actual or potential conflict of interest in relation to this presentation or program. • I have no financial interest in any of the products mentioned in this presentation • This presentation will discuss “off-label” uses of medications • Discussions in this presentation are for a general information purposes only. Please discuss with your physician your own particular treatment. This presentation or discussion is NOT meant to take the place of your doctor. 2 Pradeep Chopra, MD
  • 3. Disclosure • Training and Fellowship, Harvard Medical school • Weird Pain Medicine specialist • Assistant Professor – Brown Medical School, Rhode Island, USA • Special interest in complex pain conditions Pradeep Chopra, MD 3
  • 4. Connecting the dots • EDS is a complex condition • This presentation is about connecting the dots between all the symptoms that come with having EDS. Pradeep Chopra, MD 4
  • 7. Three things to know in EDS 1. EDS is not a disease. It is a form of the human body. 2. Soft connective tissue 3. Poor joint position sense (proprioception) 4. Co-existing conditions (Mast cell activation, POTS, Chiari etc.,) Pradeep Chopra, MD 7
  • 8. Pradeep Chopra, MD Pain in EDS by body regions Pradeep Chopra, MD 8
  • 9. Pradeep Chopra, MD Common causes of headaches in EDS 1. Migraines 2. Chiari malformation 3. Cervicogenic Headaches – from muscles 4. Temporo Mandibular joint dysfunction (TMJ) 5. PoTS / Dysautonomia 7. Spontaneous CSF (Cerebrospinal) leak – low pressure inside the head 8. Cranio Cervical Instability (Instability of the neck and head) 9. Idiopathic Intracranial Hypertension (raised pressure inside the head) Pradeep Chopra, MD 9
  • 10. “My head feels too heavy to hold up” Pradeep Chopra, MD 10
  • 11. Chin poking forward position Pradeep Chopra, MD 11
  • 13. Pradeep Chopra, MD Neck pain and headaches • A common cause of neck pain is posture, using a smart phone (“Texting neck”) • 'Chin poking forward position' • Before looking at other reasons, correct this first • If there are other reasons like cervical instability, Chiari malformation etc – these need to be addressed Pradeep Chopra, MD 13
  • 14. Pradeep Chopra, MD Pain from a poor posture Pradeep Chopra, MD 14
  • 15. Pradeep Chopra, MD Pain from a poor posture Pradeep Chopra, MD 15
  • 17. Pradeep Chopra, MD Common reasons for poor posture in EDS • Vision – Blurry vision. Usually intermittent • Postural Orthostatic Intolerance (POTS) • Laxity of spinal ligaments • Instability of the head on the neck ( Cranio Cervical instability) Pradeep Chopra, MD 17
  • 18. Managing neck pain and headaches from poor posture • Place index finger in front of chin and push back head gently till ears are in line with shoulders • Large monitor, sit well balanced while working on a computer • Manage POTS Pradeep Chopra, MD 18
  • 19. “My whole head hurts, I see double, I can feel my heart beat in my ears, ” Pradeep Chopra, MD 19
  • 20. Headaches – Idiopathic Intracranial Hypertension (IIH) • Raised pressure inside the head • Vision problems – double vision, sensitivity to light • Ringing in the ears which pulses (pulsatile tinnitus) • Maybe because of narrowing of blood flow (venous sinus stenosis) • Diagnosis: spinal tap, eye exam, MR venography • Treatment: medicines to decrease fluid pressure in the head, shunt to drain excess fluid, stent Pradeep Chopra, MD 20
  • 21. “My headache gets worse when I stand and it almost goes away when I lie down” Pradeep Chopra, MD 21
  • 22. Low pressure inside the head - Spontaneous CSF leak
  • 23. Spontaneous CSF leak • Headaches get worse being upright • Almost resolve with lying supine • Headaches worse by the end of the day • Severe headache all day in long standing cases • Nausea and vomiting • Tinnitus – change in CSF pressure can cause change in inner ear pressure • Neck pain 23 Pradeep Chopra, MD
  • 24. Differential diagnosis CSF leak POTS Headache + + Headache worse upright + + Headache better supine + + Not significantly better Neck pain + + Fatigue + + Nausea + + Orthostatics - + Heart rate greater than 30BPM with no change in BP Dizziness - + POTS: Postural Orthostatic Tachycardia Syndrome 24 Pradeep Chopra, MD
  • 25. CSF leak - managements • Increase PO fluids and caffeine • Abdominal binder • Epidural blood patch – high volume. May need multiple patches • Directed epidural fibrin glue • Surgical repair Schievink WI, Gordon OK, Tourje J: Connective Tissue Disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: A prospective study. Neurosurgery 54:65-71, 2004 25 Pradeep Chopra, MD
  • 26. “My headache gets worse when I stand and it almost goes away when I lie down” Spontaneous CSF leak 26 Pradeep Chopra, MD
  • 27. “My headache gets worse when I cough, I have tingling in my hands and feet, I have difficulty swallowing” Pradeep Chopra, MD 27
  • 28. Pradeep Chopra, MD Chiari Malformation 28 Pradeep Chopra, MD
  • 29. Chiari malformation Pradeep Chopra, MD 29 http://www.craniofacial.vcu.edu/conditions/chiari.html
  • 30. Chiari malformation http://www.craniofacial.vcu.edu/conditions/chiari.html Pradeep Chopra, MD Small posterior fossa pushes the brainstem and cerebellar tonsils through the foramen magnum into the spinal canal 30
  • 31. Symptoms of Chiari Malformation • COMMON FEATURES • Pressure headaches in the back of the head (suboccipital) • Neck pain • Balance problems • Numbness or paresthesia’s to arms or legs • Dizziness • Difficulty swallowing • Poor Hand co-ordination • Headaches made worse by coughing or straining • Ringing or buzzing in the ears • Hearing loss • Nausea, vomiting • Muscle weakness • Vision problems 31 Pradeep Chopra, MD
  • 32. Chiari Malformation and EDS A diagnosis of Chiari Malformation depends on: 1.Good history and physical examination 2.Upright MRI of the neck (cranial settling in the upright position) Pradeep Chopra, MD 32
  • 33. Cranial Settling in EDS- Deformative stresses on the brain stem, lower cranial nerves, spinal cord Clivo-axial angle normal 1400 33 Pradeep Chopra, MD
  • 34. Chiari malformation • The herniation causes obstruction to CSF flow. • Empty Sella syndrome – flattening of the pituitary gland and resulting hormonal changes. • There may be an association between Chiari, Craniocervical instability and Tethered cord syndrome in EDS 34 Pradeep Chopra, MD
  • 35. “My headache gets worse when I cough, I have tingling in my hands and feet, I have difficulty swallowing” Chiari Malformation 35 Pradeep Chopra, MD
  • 37. Cranio Cervical instability in EDS • The neck is stabilized by ligaments • Laxity of the ligaments causes the joints in the neck to move more • Excessive movement of the joints in the neck causes cranio cervical instability Pradeep Chopra, MD 37
  • 38. Remember a lot of these symptoms overlap with other conditions Cranio Cervical instability (CCI • Neck pain / stiffness • Poor vision • Headaches • Anxiety • Dizziness • Lightheaded • Paresthesia to face • Poor balance • Fatigue • Difficulty swallowing • Poor sleep • Nausea Pradeep Chopra, MD 38
  • 39. Imaging for Cranio Cervical Instability •Need functional imaging technology •Static pictures are not helpful •Functional computerized tomography (fCT scan) •Flexion. •Rotate neck left 90 degrees. •Rotate neck right 90 degrees. Pradeep Chopra, MD 39
  • 40. Cranio Cervical instability in EDS – MRI scan findings • These measurements have to specifically asked for when getting an MRI. 1. Clivo-axial angle (normal 140 to 160 degrees) 2. Harris Measurement (instability if > 12mm) 3. Grabb, Mapstone and Oakes measurement (> 9mm suggests high risk of ventral brainstem compression) Pradeep Chopra, MD 40
  • 41. Pradeep Chopra, MD Cranio Cervical Instability management •Mild to moderate: •Neck muscles strengthening exercises •Hard cervical collar (Vista Aspen collar) •Severe Instability: •Surgical fusion Pradeep Chopra, MD 41
  • 42. Vista® MultiPost Therapy Collar – an improved design 4 2 Pradeep Chopra, MD
  • 43. Barrie – Lieou syndrome • Cervical instability pushing on the autonomic nerves • Usually triggered by a whiplash type injury • Difficulty swallowing • Tongue numbness • Blurred vision • Tinnitus • Dizziness • Neck pain • Headaches 43 Ross Hauser, MD, Caring Medical
  • 44. Soft neck collars are useless “neck warmers” Pradeep Chopra, MD 44
  • 45. TMJ Pain Temporo Mandibular Joint Dysfunction Pradeep Chopra, MD
  • 46. Pradeep Chopra, MD Temporo-Mandibular joint pain Pradeep Chopra, MD 46
  • 47. Pradeep Chopra, MD TMJ Pain • Very closely related to neck issues • Clicking noises • Clenching, grinding • Pain with chewing • Difficulty opening mouth wide (eating an apple) • Jaw locking up Pradeep Chopra, MD 47
  • 48. Pradeep Chopra, MD Dental issues in Ehlers Danlos Syndromes • Teeth: weak and thin enamel, prone to cavities • Gums: periodontal gum weakness, gingivitis, easy bleeding, delayed healing after surgery, tissue breakdown after surgery (tooth extraction), gum recession and pocketing • Poor tooth stability, crowding of teeth • Very high incidence of gingivitis – this increases inflammatory reactions in the body (higher diabetes, higher arthritis etc) Pradeep Chopra, MD 48
  • 49. Livionex® - an excellent dental gel for EDS and MCAS • No salicyclates • Free of gluten, sugar, chemicals, color dyes • No harsh abrasives or detergents • Helps rebuild enamel and disrupts plaque formation • Helps repair connective tissue to close pockets Pradeep Chopra, MD 49
  • 50. Numbing medicine injections • Commonly used numbing medicine injections (Lidocaine) may be ineffective • Carbocaine (Mepivicaine) works well in EDS 5 0 Pradeep Chopra, MD
  • 51. Upper back pain Pradeep Chopra, MD 51
  • 52. Upper back pain in EDS • Usually poor posture • Shoulder instability • Rib subluxation • Doing a repetitive task with hands or arms (typing, vacuuming) - Repetitive Strain Injury • In women, it can be the weight of the breast tissue dragging the upper torso forward and the muscles of the upper back attempting to stabilize the torso 5 2 Pradeep Chopra, MD
  • 53. Pradeep Chopra, MD Upper back and Shoulder pain in women with EDS • Sports bra with: • Helps upper back, shoulder and Thoracic outlet pain • racer back (cross straps). • Wide straps. • Front closure • Proper fitting – recommend getting it done professionally. Pradeep Chopra, MD 53
  • 54. Pradeep Chopra, MD High Racer back – Posture bra 2-51 5 4 Pradeep Chopra, MD
  • 55. Compression garments 55 www.dmorthotics.com or Pam Cowans - p.cowans@dmorthotics.com Pradeep Chopra, MD
  • 56. Joint support compression. cw-x (check Zappos.com) 56 Pradeep Chopra, MD
  • 57. Pradeep Chopra, MD Spinal Instability • The spine is made up of multiple joints – held together by ligaments and muscles. • Spinal instability with reflex muscle spasms may happen at any level 5 7 Pradeep Chopra, MD
  • 58. Pradeep Chopra, MD Spinal Instability • Thoracic spine – subluxations where the ribs meet the spine (costo—vertebral joints) • Lumbar spine – subluxations of the facet joints. • Sacroiliac joint pain (SI Joint) – maybe more from uneven posture or pain from joints in the legs • Kyphosis (spine poking backwards), scoliosis (spine sideways) • Maybe a symptom of Tethered Cord syndrome Pradeep Chopra, MD 58
  • 59. Back pain can be from unstable joints in the spine, causing muscles to spasm. Pradeep Chopra, MD 59
  • 60. Spinal pain • If the pain is from the joints in the spine – postural correction, compression garments, muscle strengthening • Steroid injections not very helpful but can be used in very select cases Pradeep Chopra, MD
  • 61. Good support for the SI joint and lower lumbar region Brace for lumbar and Sacroiliac joint – Lumbo Lady Pradeep Chopra, MD 61
  • 63. Tethered Cord Syndrome (TCS) http://javid1985.hubpages.com/hub/Tethered-Spinal-Cord-Syndrome-What- You-Need-To-Know Pradeep Chopra, MD 63
  • 64. Clinical symptoms • Low back pain • Neurogenic bladder • Leg weakness and sensory loss • Musculoskeletal abnormalities • Toe walking • Heel walking 6 4 Pradeep Chopra, MD
  • 65. Neurogenic bladder • Bladder symptoms (Neurogenic bladder) • Increased (decreased) frequency, • Urgency • Sense of incomplete emptying of the bladder • Incontinence Pradeep Chopra, MD 65
  • 66. Pain from Ribs in EDS 6 6 Pradeep Chopra, MD
  • 67. Breathing and rib pain • Patients with EDS often have “Air Hunger” – a feeling of having forgotten to take a breath • Pain from ribs • All the tests for heart and lungs are normal. • Each rib has 3 joints in the back 6 7 Pradeep Chopra, MD
  • 69. Breathing and rib pain • Loss of proprioception from the ribs, muscles of breathing and diaphragm gives a feeling of not having taken a full breath in or full breath out • Similar to the uncoordinated movement of the rest of the joints in the body • Some of the muscles of breathing are also part of the lower back Pradeep Chopra, MD
  • 70. Exercise to help with chest wall pain (and lower back) • Book or sand bag should be over the belly button • Lie on your back • Breathe in and breathe out for 20 minutes a day – lifting the book up 70 http://www.normalbreathing.com/
  • 71. Diaphragm release exercise • Lie on your stomach on the floor • Place a 1.5 inch rubber ball under your belly button • Just lie comfortably in this position for 20 minutes. • If you get uncomfortable before a minute, use a smaller ball. 7 1 Pradeep Chopra, MD
  • 72. Chest wall pain and rib subluxations • Singing (high and low notes), wind instrument like a flute or recorder • May help with chest muscle and rib pain and strengthening lower back • Rib subluxations happen with uncoordinated breathing (remember poor proprioception), and poor posture Pradeep Chopra, MD
  • 73. Pradeep Chopra, MD Pain in the arms in EDS 7 3 Pradeep Chopra, MD
  • 74. Pradeep Chopra, MD Pain in arms • Shoulder joint subluxations, dislocation • Thoracic outlet syndrome • Elbows: Tendonitis, bursitis, hyperextension • Wrist and fingers: subluxations, muscle pain, tendonitis Pradeep Chopra, MD 74
  • 75. Pradeep Chopra, MD Shoulder pain • Laxity of the shoulder joint causes the muscles (rotator cuff) around the shoulder to spasm • Thoracic Outlet syndrome. Pradeep Chopra, MD 75
  • 76. Pradeep Chopra, MD Pain patterns in Thoracic Outlet syndrome Pradeep Chopra, MD 76
  • 77. Pradeep Chopra, MD Thoracic Outlet Syndrome Pradeep Chopra, MD 77
  • 78. Pradeep Chopra, MD Thoracic Outlet Syndrome •Thoracic Outlet exercises •Kinesio taping •Stabilize shoulder •surgery Pradeep Chopra, MD 78
  • 79. Pradeep Chopra, MD Kineseo Taping for shoulder joint pain 79 Pradeep Chopra, MD
  • 80. Pradeep Chopra, MD The EDS way of holding a pen • Poor proprioception makes patients grip a pen with as many fingers as possible • They hold the pen very tight and press down hard on paper (poor haptic feedback) • Puts abnormal pressure on the muscles and joints of the hand and wrist 80
  • 81. Pradeep Chopra, MD • Dense foam padding (Ableware®) or wrap a foam padded tape – for pens, tooth brush, forks, knives • Compression half finger gloves • Brace for unstable joints 81
  • 82. Pradeep Chopra, MD Splints for fingers 82
  • 83. Splinting and braces in general • Braces maintain joint in neutral position • Avoid hyper – extension • Braces help with joint position awareness (proprioception) • Start using them gradually • Gradually decrease their use as you gain strength Pradeep Chopra, MD 83
  • 84. Do braces make your muscles weaker? • No, they do not cause muscle atrophy • It’s a common misconception • Braces are not tight enough to stop muscles from moving • In fact, braces stabilize joints so the muscles can move the joints more efficiently. Pradeep Chopra, MD 84
  • 86. Pain in lower half of the body • Pay attention to feet and ankles. • If the feet and ankles are unstable, they make • The knees even more unstable, which then • Makes the hips unstable, which then • Throws the pelvis off – Sacroiliac joint pain, lumbar pain Pradeep Chopra, MD 86
  • 88. Flexible flat feet – predominantly in the forefoot Pradeep Chopra, MD 88
  • 89. Pronation Pradeep Chopra, MD 89 http://www.integrativepersonaltraining.co m/blog/run-flat-feet-knee-pain/
  • 91. Pradeep Chopra, MD The feet in EDS • Barefoot walking, where safe and comfortable – helps with conditioning of muscles under natural loads • Repeated rising on tip toes – strengthens the muscles in foot and with proprioception • Ankle raises by lifting heel (not leaning forward) • Descend in a slow controlled way 91 Pradeep Chopra, MD
  • 92. Pradeep Chopra, MD Footwear - shoes • Extremely important to wear proper footwear • Help with unstable ankles, hypermobile feet • Cushioned mid sole • Good, strong heel counter provides stability • Fastenings should be over the mid-sole for better support • Sneakers !! 92 Pradeep Chopra, MD
  • 94. Pradeep Chopra, MD Ankle brace to stabilize the ankle joint 94 Pradeep Chopra, MD
  • 96. Pradeep Chopra, MD Patella is stabilized muscles of the thigh 2-115 96 Pradeep Chopra, MD
  • 97. Hyperextension at the knees Pradeep Chopra, MD 97
  • 98. Kinesio taping – EDS knee • A combination of two strips of 25 cm in length and 2.5cm in width along the collateral ligament (sides of the knee) using 50% tape tension applied distally (furthest) to proximal, a horizontal tape below the patella 25 cm in length and 2.5 cm in width applied with 25% tension and lastly a Y tape 30 cm in length and 5cm in width cut with 5cm in initial base applied laterally to the patella with no tape tension. Pradeep Chopra, MD 98 Ther Adv Musculoskelet Dis. 2015 Feb; 7(1): 3–10. doi: 10.1177/1759720X14564561 PMCID: PMC4314299 The effects of neuromuscular taping on gait walking strategy in a patient with joint hypermobility syndrome/Ehlers–Danlos syndrome hypermobility type Filippo Camerota, Manuela Galli, Veronica Cimolin,corresponding author Claudia Celletti, Andrea Ancillao, David Blow, and Giorgio Albertini Author information
  • 99. Pradeep Chopra, MD Treatment options for knee pain in EDS • Stabilize the feet and ankles, first • Strengthen muscles around the knee • Knee brace • Two straps above knee • Two straps below knee • Patella stabilizer • Metal strut to prevent hyperextension Pradeep Chopra, MD
  • 100. Wrap around knee brace - Gripper Pradeep Chopra, MD 100
  • 101. Pradeep Chopra, MD A missed cause of leg pain – the proximal tibio fibular joint 101 Pradeep Chopra, MD
  • 102. Pradeep Chopra, MD Knee pain – often missed cause • Site of pain from the proximal Tibiofibular joint • It can inflame the peroneal nerve which causes pain down the side of the leg and even foot drop 102
  • 106. Pradeep Chopra, MD Tendonitis and bursitis in EDS 106 Courtesy http://www.ateevia.com/bursitis.aspx Misaligned bones and tendons Subluxed bones Pradeep Chopra, MD
  • 107. Tendonitis and bursitis in EDS • Treatment lies in correcting the underlying problem – •correct bracing to align the joints •Correct posture (especially standing) •Avoid repetitive use of joint •Maintain proper balance 107 Pradeep Chopra, MD
  • 108. Sacroiliac joint pain • Very common cause of low back pain • Usually around the lower back, radiates into the buttock region and may even go down the legs (but not below the knee) • Groin - occasionally 108 Pradeep Chopra, MD
  • 110. • Leg length discrepancy 110 Pradeep Chopra, MD
  • 111. LumboTrain® Lady – a good brace for SIJ Pradeep Chopra, MD 111
  • 112. Dysautonomia / POTS (Postural Orthostatic Tachycardia Syndrome) 112 Pradeep Chopra, MD
  • 113. Dysautonomia • This is a fancy name for a bunch of medical conditions that affect the nerves that control automatic functions in our body. • “Dysfunction of Autonomic Nervous System” • PoTS – Postural Orthostatic Tachycardia Syndrome • Its one of the most common types seen in EDS Pradeep Chopra, MD 113
  • 114. Postural Orthostatic Tachycardia syndrome (PoTS) - Symptoms • Fainting, dizziness • Heart racing (Palpitations) • Fatigue • Headaches • Difficulty maintaining body temperature • Poor concentration “brain fog” • Feeling of constant anxiety • Stomach pain • Wake up feeling tired 114 Pradeep Chopra, MD
  • 115. PoTS • Patients with EDS pool excessive blood to their lower extremities, • This triggers an increased activity in the same nervous system that controls ‘fight or flight’ Pradeep Chopra, MD 115
  • 116. POTS - Postural Orthostatic Tachycardia syndrome Dysautonomia International for more information and high salt diet recipes. http://www.dysautonomiainternational.org/ • EDS Awareness https://www.chronicpainpartners.com/ 116 Pradeep Chopra, MD
  • 117. PoTS • The constant feeling of dizziness makes patients feel unstable • The laxity of the joints makes the muscles tighten reflexly • This constant imbalance of muscles worsens their pain and fatigue 117 Pradeep Chopra, MD
  • 118. Anxiety in EDS •Patients with EDS and PoTS (Dysautonomia) are often over diagnosed to have anxiety •Symptoms of undiagnosed palpitations, fatigue, dizziness, chronic pain are attributed to ‘anxiety’. •In most cases its PoTS– its because of excessive activity of the flight or fight nerves to control blood pressure. Pradeep Chopra, MD 118
  • 119. POTS - Postural Orthostatic Tachycardia syndrome • Increase in heart rate by 30 beats/min within 10 minutes of standing • heart rate of 120 beats / min within the first 10 minutes of standing • No significant change in blood pressure • Syncope or almost syncope (fainting) • In children an increase of 40 beats/minute Pradeep Chopra, MD 119
  • 120. POTS - tests • Orthostatics – Measure blood pressure and heart rate: • after lying supine for 5 minutes • standing up - immediately • Standing for 10 minutes • Q-sweat test • Tilt Table Test Pradeep Chopra, MD 120
  • 121. Sample readings in POTS Lying flat for 5 minutes 110/66 82 Standing, immediately 100/68 115 Standing for 10 minutes 112/65 108 Pradeep Chopra, MD 121
  • 122. PoTS - treatment • Increase salt by mouth (salt pills: http://saltstick.com/vitassiumpage/) • Increase electrolyte oral fluids • Trial of ‘Banana bag oral rehydration fluid https://www.bananabagdrink.com or Gatorade • Compression tights up to thighs. • Abdominal binder or trial of a one size smaller swimsuit. • Cardiology or Neurology consult for Dysautonomia/POTS. • Consider a beta-blocker such as Metoprolol Pradeep Chopra, MD 122
  • 123. Treatment of POTS • Exercise is the key here. • Exercise lying down. • Dallas protocol • Exercise in pool – simple walking, treading water • https://youtu.be/jbGNfuXVHwc • https://youtu.be/oXnNL_furCw • https://youtu.be/hB8yr1e823Q Pradeep Chopra, MD 123 Courtesy: Dysautonomia International
  • 124. Treatment of POTS • Exercises lying down: • https://youtu.be/jbGNfuXVHwc • https://youtu.be/oXnNL_furCw • https://youtu.be/hB8yr1e823Q Pradeep Chopra, MD 124 Courtesy: Dysautonomia International and RSDSA
  • 125. Exercise in pool for EDS • Exercises in pool: • https://youtu.be/g7df_ek50Jk • https://youtu.be/mVvzsFk6rPo?list=PLPS8D21t0eO- X68fTNKk7UEkegxDqzQ3U • This one is also good for CRPS/RSD • https://youtu.be/wUnwbNsIk1c Pradeep Chopra, MD 125
  • 126. POTS – one last thing • POTS can be because of other conditions such as Sjogrens syndrome, Antiphospholipid syndrome, celiac disease, anykylosing spondylitis. Myasthenia gravis, multiple sclerosis • Can be seen in Autoimmune conditions where patients develop antibodies to alpha 1, Beta 1, Beta 2 adrenergic receptors, muscarinic 3 receptors, (12)g-Acetylcholine receptors, cardiac lipid raft proteins. • These patients may respond well to IVIg infusions. Pradeep Chopra, MD 126
  • 127. Postural Orthostatic Tachycardia syndrome (PoTS) - Symptoms • Fainting, dizziness • Heart racing (Palpitations) • Fatigue • Headaches • Difficulty maintaining body temperature • Poor concentration “brain fog” • Feeling of constant anxiety • Stomach pain • Wake up feeling tired 127 Pradeep Chopra, MD
  • 129. Mast Cell Activation Syndrome (MCAS) • Mast cells are a type of cell found in the blood. • They are part of our body defense mechanism (immune system) • When they detect an attack (infection or something that is not good for the body) they get activated • Once mast cells get activated they release a bunch of chemicals such as histamine, cytokines etc Pradeep Chopra, MD 129
  • 130. Mast Cell Activation Syndrome (MCAS) • In MCAS, mast cells are inappropriately triggered and release excessive amount of these chemical mediators (histamine, cytokines etc.) • It is NOT mastocytosis (whole different condition) Pradeep Chopra, MD 130
  • 131. Mast Cell Activation Syndrome (MCAS) • Rashes, hives, itchy • Fatigue, tiredness • Muscle pain • Bone and joint pain • Abdominal pain, bloating, nausea • Palpitations, pre-syncope • Flushing especially after a hot shower • Constant feeling of having flu Pradeep Chopra, MD 131
  • 132. Dermatographism (writing on skin) Urticaria Multiforme - Scientific Figure on ResearchGate. Available from: https://www.researchgate.net/Dermatographism-Linear-wheals-after- writing-on-the-skin_fig4_236106893 [accessed 3 Oct, 2018] Pradeep Chopra, MD 132
  • 133. Mast Cells Pradeep Chopra, MD 133 Red Blood Cell Mast Cell
  • 134. MCAS 134 Courtesy Ann Maitland, MD Pradeep Chopra, MD
  • 135. Mast Cell Activation Syndrome (MCAS) • Rashes, hives, itchy • Fatigue, tiredness • Muscle pain • Bone and joint pain • Abdominal pain, bloating, nausea • Palpitations, pre-syncope • Flushing especially after a hot shower • Constant feeling of having flu Pradeep Chopra, MD 135
  • 136. MCAS – Testing • Tests for MCAS are NOT reliable. However, these test can be done: • Urine 24 hour collection for N-methylhistamine. • Same 24 hour collection Prostaglandin D2/F2 or 11 Beta prostaglandin F, whichever is available. • Serum tryptase. • Serum Chromogranin A level Pradeep Chopra, MD 136
  • 137. MCAS - tests • The diagnosis of MCAS depends largely on a physician who knows MCAS and is clinical • Laboratory tests are very difficult and unreliable. • If a biopsy sample is taken from endoscopy then: Please request Pathologist for the following: • Number of Mast Cells per high power field. • Stain for Mast Cells with CD117. • Comment on the morphology and histology. • Request KIT-D816V mutation analysis by PCR Pradeep Chopra, MD 137
  • 138. MCAS – treatment • Ranitidine (Zantacâ) 150mg twice a day. • Loratidine (Claritinâ) one tablet twice a day. • Cetrizine (Zyrtecâ) 10mg one tablet twice a day. • Clemastine 2mg p.o. b.i.d. • Rupatadine 10mg p.o. b.i.d. • Quercetin 1000 mg / day • Consider Ketotifen. • Consider Cromolyn oral 200mg four times a day. • Cromolyn Nebulized. Pradeep Chopra, MD 138
  • 139. MCAS – treatment • Avoid triggers for Mast cell activation such as NSAIDs, alcohol, opioids and sudden temperature changes. • Inactive ingredients (binding agents, preservatives) in pills may contribute to MCAS. • Consider a leukotriene inhibitor such as Singulair. • Low histamine diet. Pradeep Chopra, MD 139
  • 140. MCAS – treatment • Review and change possible hidden food sources and the possibility of accidental exposure to such foods. • Some of the usual suspects for MCAS to avoid are: • Seasonings (except olive oil, salt). • All dairy. • Eggs. • Grains except quinoa and rice. • Avoid beef products but okay to take lamb, venison and poultry. • Food and drug reactions are a common cause of unexplained hives and rashes. Some of the other causes of hives may be exposure to change in temperature (hot to cold), rubbing skin, physical exertion, pressure and direct exposure to sunlight. Pradeep Chopra, MD 140
  • 141. MCAS – treatment • Other methods to avoid exacerbation maybe: • Use pollen guards in windows. • HEPA air filters, A/C. • Use a mask (e.g. VOG mask) while dusting or vacuuming. • Avoid long baths or showers, rapid changes in temperature, wool, dust and cigarette smoke. • May use nasal saline during flare ups Pradeep Chopra, MD 141
  • 143. Abdominal pain • Very common in EDS • Slowing down of the intestines • Alternating diarrhea and constipation • PoTS, MCAS –causes nausea, acid reflux, bloating and constipation • Prolapse • Acid reflux from MCAS Pradeep Chopra, MD 143
  • 144. • Low FODMAP diet – for bloating, pain and diarrhea • Treating PoTS and Mast Cell Activation Syndrome helps • In case you are wondering: • FODMAP - Fermentable Oligo-, Di-, Mono-saccharides And Polyols Pradeep Chopra, MD 144
  • 145. Low FODMAP diet • https://www.kcl.ac.uk/lsm/research/divisions/dns/projects/fodmaps/faq.aspx • There are 3 stages to the low FODMAP diet: • Stage 1: Restriction: In this stage, you reduce your FODMAP intake by avoiding foods that are high in FODMAPs for four to eight weeks as this period is considered long enough to identify if symptoms will respond to a low FODMAP diet. • Stage 2: Reintroduction: If your symptoms have improved following FODMAP restriction, it is important to reintroduce some high FODMAP foods. This will enable you to identify which FODMAPs you are most sensitive to, as well as how much of a high FODMAP food triggers your symptoms. • Stage 3: Personalization: The long term aim of a low FODMAP diet is to personalize your diet so you only avoid foods that trigger your symptoms and you return to as normal a diet as possible. 1 4 5 Pradeep Chopra, MD
  • 146. Dysfunction of movement of intestines • Can lead to changes in the gut microbiome (the helpful bacteria that live in our intestines) • Problems with movement of intestines may lead to Small Intestinal Bacterial Overgrowth (SIBO) Pradeep Chopra, MD 146
  • 147. Small Intestinal Bacterial Overgrowth - symptoms • Abdominal pain/discomfort • Bloating • Diarrhea • Weakness • gassy Pradeep Chopra, MD 147
  • 148. SIBO – problems • Weight loss • Vitamin deficiency – A,D,E,K, B12, • Vitamin excess – Folate • Iron deficiency • Low protein Pradeep Chopra, MD 148
  • 149. SIBO – testing • Organisms in the intestine produce hydrogen and methane. • Breath test can detect levels of hydrogen and methane • Elevated methane levels can be treated with statins (lovastatin – red rice yeast) • Treat any dysautonomia • Treat any MCAS • Treat constipation • Drugs for improving GI motility (Resolor, Iberogast, Mestinon, erythromycin) Pradeep Chopra, MD 149
  • 150. SIBO – treatment • Elevated methane levels can be treated with statins (lovastatin – red rice yeast) • Specific antibiotics to treat SIBO. • Be cautious of repeating antibiotics • Treat any dysautonomia • Treat any MCAS • Treat constipation • Drugs for improving GI motility (Resolor, Iberogast, Mestinon) Pradeep Chopra, MD 150
  • 151. SIBO – treatment • Avoid simple carbohydrates • Think of pureed foods. Pradeep Chopra, MD 151
  • 153. Abdominal pain – MALS • Median Arcuate Ligament Syndrome (MALS). • Belly pain after eating food. • Can trigger PoTS Pradeep Chopra, MD 153
  • 154. Abdominal pain – MALS • There is an artery that supplies blood to part of the intestine • It is called Celiac Artery • This artery pokes out from under a ligament called Median Arcuate ligament • There are 2 theories about why MALS causes pain • The median arcuate ligament is tight causing compression of the celiac artery • There is a nerve in that area called Celiac ganglion which may be getting compressed or inflamed. Pradeep Chopra, MD 154
  • 156. Abdominal pain – MALS • Treatment is surgical decompression • If treated early, symptoms resolve • If treated late, may need IVIg infusion Pradeep Chopra, MD 156
  • 158. Pelvic pain in EDS - Dysmenorrhea (painful menstrual periods) • Birth control to stop periods – may help with joint laxity also • NSAID’s (ibuprofen, naproxyn) • Control Mast Cell Activation Syndrome 158 Pradeep Chopra, MD
  • 159. Pelvic pain in EDS - Interstitial cystitis (bladder pain) • Maybe part of Mast Cell Activation syndrome • Cromolyn or Ketotifen • Palmitoyl ethanolamide (PEA) 159 Pradeep Chopra, MD
  • 160. Pelvic pain in EDS – Vulvodynia (vaginal pain) • Maybe part of Mast Cell Activation syndrome • Benadryl douche or cream • Cromolyn douche or cream 160 Pradeep Chopra, MD
  • 162. EDS and children • dislocated joints, • chronic body wide pain • Easy bruising • Abdominal pain • Fatigue • Unidentified bleeding (intracranial, abdominal) • To the ER physicians they look suspicious of Medical Child abuse Pradeep Chopra, MD 162
  • 163. • The diagnosis of Conversion Disorder or Munchausen by Proxy are often made by providers with little training in Psychiatry and vice versa most psychiatrists have no training in pain conditions. • Parents – please research the facility carefully before taking your child there. • The facility should have trained physicians in EDS and its complications • There are some hospitals that have hired “Medical Child Abuse” specialists. Pradeep Chopra, MD 163
  • 164. Behavioral • To diagnose a child with Conversion disorder or Munchausen by Proxy without a dedicated multidisciplinary team approach and without concrete evidence is extremely harmful to the patient. • To label an adult with pain as being psychological is severely harmful. It closes all doors to correct treatment. Pradeep Chopra, MD 164
  • 165. Misdiagnosis • Please check with other patients to get feedback before you go to a hospital. • If you sense that the doctors are skeptical of your complaints, consider going somewhere else. • When you go to an a academic hospital, chances are that you will be seen by a junior doctor / Resident – who is the least qualified to manage complex medical conditions. • If you are able, choose a Concierge physician. Pradeep Chopra, MD 165
  • 166. Service Dogs - invaluable • POTS – they can sense when their owner is having an episode of dizziness or seizure • EDS and pain - they protect the limb from being injured or touched • Helps boost confidence in their owners, making them more independent • Help with balance, call for help, open doors, switch on lights, pull wheelchairs, anxiety, Pradeep Chopra, MD 166
  • 167. Some organizations that can help get a service dog • https://www.pawswithacause.org • http://www.neads.org • http://freedomservicedogs.org Pradeep Chopra, MD 167
  • 168. To manage pain first find out what’s broken • It is crucial to understand the cause of the pain before deciding on treatment options. • For example, pain in the shoulder joint can be from: • Dislocated shoulder joint, • Muscle spasms • Nerve or blood vessel impingement or damage • All of the above • The treatment of each of these is different Pradeep Chopra, MD 168
  • 169. Types of tissue injury in EDS • Micro trauma – small repetitive traumas resulting in constant tissue breakdown. Invisible tissue breakdown. This is far more common. For example – overstretching muscles and ligaments. Vacuuming, chopping food, dusting • Macro trauma – a large event or trauma resulting in injury. For example - Dislocation or fracture 169 Pradeep Chopra, MD
  • 170. Macro trauma Micro trauma 170 Pradeep Chopra, MD
  • 171. Principles of managing pain in EDS • Use a mix of treatments • For example - Knee pain due to instability • Stabilize knee with braces • Strengthen muscles around the knee • Medications – NSAID’s, topical etc. • Follow the NC10 rule (if you can get at least 10% from one treatment, five such treatments will give you 50% relief) Pradeep Chopra, MD 171
  • 172. Joint Position sense Proprioception The body’s ability to sense movement of the joints and their position Pradeep Chopra, MD 172
  • 173. Proprioception (Joint Position Sense) • Proprioception, also known as Joint Position Sense, is the ability of a joint to 1. determine its position, 2. detect movement, and 3. sense of resistance to force Pradeep Chopra, MD 173
  • 174. Proprioception – Joint sense • The brain constantly gets information from the joints as to the exact position of the limbs in space. • It helps us walk, use our arms, maintain our posture without tipping over. • Protects our joints from over extending and our muscles from over stretching • EDS – poor proprioception. Pradeep Chopra, MD 174
  • 175. Proprioception – Joint sense • The brain uses sensors in the skin as a way of figuring out the position of different parts of the body. • Wearing skin tight clothes over joints and limbs will help the brain understand where the body is • One of the reasons that exercising in water helps is because your skin senses contact with water Pradeep Chopra, MD 175
  • 176. Proprioception exercises • Balance board or wobble board • Stork standing (stand on one leg) • Stand up paddle board (SUP) • Sitting on exercise ball • Exercise in water – walking, treading but avoid swimming 176 Pradeep Chopra, MD
  • 177. Stand up paddle board 177 www.vimbly.com Pradeep Chopra, MD
  • 180. Proprioception – Joint sense Pradeep Chopra, MD 180
  • 181. Proprioception exercises • Juggling • Balance board or wobble board • Stork standing (stand on one leg) • Stand up paddle board (SUP) • Sitting on exercise ball • Exercise in water – walking, treading but NO swimming Pradeep Chopra, MD 181
  • 182. Exercise in water - walking Pradeep Chopra, MD 182
  • 185. Exercises for EDS • Muscles strengthening without stressing the joints – for stabilizing joints • Building on proprioception – for joint protection and efficient use of muscles. • Exercising with fatigue • It should be daily, even if it’s a little bit 185 Pradeep Chopra, MD
  • 186. Muscle strengthening exercises • Avoid joint loading or joint stressing • Focus on muscle strengthening • Avoid extreme stretching • Start very low, really very low • Progress slowly, really slow 186 Pradeep Chopra, MD
  • 187. Keep limb movements within extreme range of motion 187 Pradeep Chopra, MD
  • 188. Keep limb movements within extreme range of motion 188 Pradeep Chopra, MD
  • 189. Aquatic therapy • Best form of exercise in EDS • The contact of water with the skin helps the brain move your muscles more efficiently • The water makes us weigh less which takes the load off the joints allowing us to exercise freely • Avoid swimming – it strains the joints of the neck and shoulders. 189 Pradeep Chopra, MD
  • 190. Avoid swimming – injury to shoulder and neck 190 Pradeep Chopra, MD
  • 191. Pradeep Chopra, MD Neuromuscular taping (Kinesio™/ Rock Tape ™ ) 1 9 1 Pradeep Chopra, MD
  • 192. Pradeep Chopra, MD Finger print pattern 2-29 1 9 2 Pradeep Chopra, MD
  • 193. Pradeep Chopra, MD Kinesio™ taping - mechanism • Mimics the superficial layer of skin – after 10 minutes you can not feel it • Designed to stretch • Porous – allows for drying easily. You can take a shower with it on • The adhesive is applied in a wave like pattern to mimic the qualities of fingerprints. 1 9 3 Pradeep Chopra, MD
  • 194. Pradeep Chopra, MD Kinesio™ taping - mechanism • The tape stimulates the sensors in the skin as we move – improves proprioception • Helps reduce swelling 1 9 4 Pradeep Chopra, MD
  • 195. Pradeep Chopra, MD Kinesio™ taping - uses • Reduces pain • Improves Proprioception • Relaxes muscles • Stabilizes joints • Supports weak joints • Reduces swelling 1 9 5 Pradeep Chopra, MD
  • 196. How to prevent skin reactions to K-tape •Cavilon cream® by 3M •Leukotape® K •Milk of magnesia 1 9 6 Pradeep Chopra, MD
  • 198. The Feldenkrais Method • It is a type of physiotherapy that helps repair impaired connections between the brain and the body • Patients with EDS and chronic joint conditions develop inefficient or strained habitual movement patterns • It uses slow repetition to teach correct and safe movements in EDS • The movements are gentle, slow, repeated movements. • http://www.feldenkrais.com Pradeep Chopra, MD 198
  • 199. Starting treatment - medicines and exercise Start low, go slow Pradeep Chopra, MD 199
  • 201. Miscellaneous – Palmitoylethanolamide(PEA) topical cream 2% Sold as Soothamide. Moderate relief Pradeep Chopra, MD 201
  • 202. CGRP antibodies • CGRP – Calcitonin Gene Related Peptide • It is a protein in the body involved in transmission of pain • CGRP antibodies are medicines to block the effect of CGRP and decrease pain • In the last few months there have been 2 such CGRP antibodies on the market and a few to be released soon. • Currently, approved for treatment of migraines only. • They may be effective for other pain conditions. Pradeep Chopra, MD 202
  • 203. Poor concept of exercising in pain •No pain, no gain Pradeep Chopra, MD 203
  • 204. Poor concept of exercising in pain - baloney •No pain, no gain Pradeep Chopra, MD 204
  • 206. Fatigue • EDS • PoTS / Dysautonomia • Mast Cell Activation Syndrome (MCAS) • Drugs • Pain • Poor sleep • Secondary Mitochondrial dysfunction 206 Pradeep Chopra, MD
  • 207. Fatigue - causes • Gastrointestinal: Median Arcuate Ligament Syndrome (MALS) • Obstructive sleep – tracheomalacia (EDS), sleep apnea • Hormonal - Hypothalamus - Pituitary-adrenal axis suffering from hypoperfusion or Sheehan’s syndrome. • Sleep apnea: Obstructive, central apnea, non-restorative sleep • Non-restorative sleep secondary to Dysautonomia Pradeep Chopra, MD 207
  • 208. Symptoms of EDS, POTS, MCAS 208 Belly pain Racing heart Blurry vision Headaches, brain fog, dizzy Dark discoloration of feet Tremulousness www.potsuk.org Pradeep Chopra, MD
  • 209. Pradeep Chopra, MD Fatigue in EDS Non Ehlers Danlos Syndrome • Muscles – relaxed at rest and contract with activity • Ligaments – tensed at rest, they support and stabilize the body 209 Ehlers Danlos Syndrome • Muscles – They are tensed and constantly attempt to stabilize even at rest • Ligaments – provide no tension and stability
  • 211. Medicinal marijuana • The human body has two types of receptors – CB1 and CB2 • CB1 receptors are found in the brain • CB2 receptors are found in the rest of the body, immune cells and glia cells in the Central Nervous System • Chemicals that cause inflammation in the peripheral parts of the body are modulated by cannabinoids. Hence, cannabis applied topically are helpful Pradeep Chopra, MD 211
  • 212. Medicinal Marijuana • Reasonable choice to try. • Anecdotally – works well in patients with EDS • Higher CBD levels and lower THC levels • Vaporizing, edibles • Topical CBD over joints, tendons, ligaments – very helpful • Does not affect Mast Cell Activation Syndrome (MCAS) as much as NSAID’s and opioids Pradeep Chopra, MD 212
  • 213. Medicinal Marijuana • Reasonable choice to try. • Anecdotally – works well in patients with EDS • Higher CBD levels and lower THC levels • Vaporizing, edibles • Topical over joints and muscles. • Does not affect Mast Cell Activation Syndrome (MCAS) as much as NSAID’s and opioids Pradeep Chopra, MD 2 1 3
  • 216. Low Dose Naltrexone LDN Pradeep Chopra, MD 216 3-65
  • 217. Low Dose Naltrexone (LDN) • Competitive antagonist of opioid receptors • Clinically used for 30 years for addiction • Suppressive effects on the CNS glia, whch helps with Central Sensitization Pradeep Chopra, MD 217 3-66
  • 218. Low Dose Naltrexone (LDN) • Dose can vary anywhere between 1.75mg to 4.5mg • May cause insomnia, mild headaches initially. • Patients report increased physical activity, flare ups not as acute, better tolerance to pain. • Recommend a trial of at least 6 months • To avoid all opioids or tramadol. Pradeep Chopra, MD 218
  • 219. Medications in EDS • Patients with EDS may have an abnormal response to medications • Maybe very sensitive, • May have a poor response • May need higher or lower doses than usual Pradeep Chopra, MD 219
  • 220. Muscle Relaxants • Limited value in EDS • Cyclobenzaprine (Flexeril) • Carisoprodol (Soma) • Tizanidine (Zanaflex) • Baclofen • Benzodiazipine (Valium) Pradeep Chopra, MD 220
  • 221. Acetaminophen/ Paracetamol • One of the safest analgesic in EDS • By itself, not a strong analgesic • Potentiates the effects of other analgesics (NSAID’s, opioids) Pradeep Chopra, MD 221
  • 222. Non steroidal Anti-inflammatory (NSAID) • Risks with taking it orally • May combine with acetaminophen / paracetamol to lower dose • Helpful with inflammatory pain • Good value to using it topically Pradeep Chopra, MD 222
  • 223. Opioids / Narcotics • Good analgesic • Short term use to break the cycle of pain or muscle spasm • In general, most patients with EDS do not respond well to opioids • Long term use – risk of opioid hyperalgesia Pradeep Chopra, MD 223
  • 224. Adjuvants • Anti-seizure, anti-depressents • May use only if there is a neuropathic component to the pain Pradeep Chopra, MD 224
  • 225. Anti-seizure medicines • Gabapentin • Pregabalin Pradeep Chopra, MD 225
  • 226. Anti-depressants • Tricyclic antidepressants – amitriptyline, nortriptyline, doxepin • SNRI – duloxetine, milnacipran • SSRI – not very helpful for pain. Pradeep Chopra, MD 226
  • 227. Suggested treatments (future) • Oxygen (Prof Hamonet) • Hormonal modulation (Dr. Gompel) • Levodopa/carbidopa for muscle spasms • EPSOM (Magnesium) salt bath • Topical analgesics – predictable absorption Pradeep Chopra, MD 227
  • 228. Oxygen supplementation • Anecdotal experience • Oxygen by non-rebreather mask 3 liters to 5 liters, 20minutes per day up to twice a day. • May administer as needed for severe symptoms of fatigue or dizziness. Pradeep Chopra, MD 228
  • 229. Mineral supplement for fatigue • Anecdotal experience • Combination of CoQ10, L-carnitine, creatinine • Good experience with fatigue Pradeep Chopra, MD 229
  • 230. Levodopa-carbidopa • Anecdotal experience • Significant relief for muscle spasms and dystonia • Low dose Pradeep Chopra, MD 230
  • 231. Thank you Pradeep Chopra, MD snapa102@gmail.com Pradeep Chopra, MD 231