EDS: Understanding your

pain and how to treat it

Norman Marcus, MD

Director, Clinical Muscle Pain Research

Adj Asst Prof of Anesthesiology and Neurosurgery

Weill-Cornell Medicine

It’s complicated

• Current concepts of EDS pain mechanisms and

treatments

• Why muscles cause pain in EDS

• Understanding pain mechanisms

• Functional muscle pain

• Treatments

Pain Mechanisms

Musculoskeletal Pain

Psychosocial Factors

Physical Fitness

Biomechanical Factors

Neurological Factors

Anxiety

Pain-related fear

Deconditioning

Muscle Weakness

Decreased cardiovascular

Capacity

Joint instability

Altered motor control

Connective tissue laxity: GJH

Nonphysiologicalmotor patternsProprioceptive

inacuity

CNS upregulation

Generalized hyperalgesia

Scheper MC, de Vries JE, Verbunt

J, Engelbert RH. Chronic pain in

hypermobility syndrome and

Ehlers–Danlos syndrome

(hypermobility type): it is a

challenge. Journal of pain research.

2015;8:591.

Various Treatments• Medications

– NSAIDs

– Opioids

– Antidepressants

– Sedatives/Benzos

– Cardiovascular

– Pulmonary

• Physiotherapy– Strength Training

– Massage

– Stabilization Training

– Electrotherapy

– Manual Therapy

– Aquatic Therapy

– Heat Therapy

– Stretching

• Surgery

Skeleton

Oxygen and Muscle Pain

Constricted blood vessels inhibit the flow of blood and

deliver less oxygen:

Areas of lower oxygen cause

Pain with activity

Impaired Calcium Pump/Inability to relax a part of a

contracted muscle (Trigger Points)

adapted from the Energy Crisis Hypothesis from Travell, J.

G., & Simons, D. G. (1999). Myofascial pain and

dysfunction: the trigger point manual. Vol. 2, The lower

extremities. Williams & Wilkins.

LESS OXYGEN PAIN

Decreased oxygen

When we feel pain

how does it affect us?

Hobbies

Pain

Sports

WorkSocial Life

Friends

Family

Sex

Life of Pain

Pain

how the body produces pain

Nociceptive/Pain Pathways

18

Microglia:

Trauma, Infection, Persistent Pain

Pro-inflammatory cytokines

Sickness response: Hyperalgesia

lethargy

depression

anxiety

social withdrawal

Treating Microglia

Naltrexone

– Used in overdose and heroin addiction

– Commercial doses (50-100mg)

versus

microdoses (0.1mg +)

– Effect on opiates

Mast Cell Activation Disorder

Treating Mast Cells

Medications:

• Anti-histamines

• Anti-leukotrienes

• Cannabinoid Family:

– PEA

– Medical marijuana (CBD/THC)

• 3 delivery systems

• 5 Ratios

Pain stimulates microglia

Mast cells stimulates microglia

MORE PAIN!

Opposing Mechanisms

Central Sensitization (CS)

Versus

Diffuse Noxious Inhibitory Control (DNIC)/

Conditioned Pain Modulation (CPM)

Hans Kraus, M.D.Functional Muscle Pain

• Tension/Stress

• Deficiency: Weakness and/or Stiffness

• Spasm

• Trigger Points

Animal model: Stress as a

contributing factor to back pain

Hoheisel, U, et al. Immobilization stress sensitizes rat dorsal horn neurons having input from the low back (2015). Eur J Pain. 19:861-870

Hans Kraus, M.D.Functional Muscle Pain

• Tension/Stress

• Deficiency: Weakness and/or Stiffness

• Spasm

• Trigger Points

Kraus-Weber Tests

The Y’s Way to a Healthy Back

• Taught to 300,000 patients – twice a week for

six weeks

• 12K patients studied: 80% of patients

experienced reduction and/or elimination in

back pain

• Patients with previous back surgery had an 82%

success rate

Kraus H, Nagler W, Melleby A. Evaluation of an

exercise program for back pain. Am Fam Physician.

1983;28(3):153-8.

Kraus/Marcus Lower Body Exercises

(Level 1)

Precautions

– Individualized needs

(subluxation/dislocation, strength)

– Work with providers who know EDS

– Physical therapy:

• No Isometrics!

• Use Manual Resistance

• No Passive Stretching

Hans Kraus, M.D.Functional Muscle Pain

• Tension/Stress

• Deficiency: Weakness and/or Stiffness

• Spasm

• Trigger Points

Pain-Spasm-Pain Concept

Lund HypothesisLund JP et al. The pain-adaptation model: a discussion of the relationship between chronic musculoskeletal pain

and motor activity. The Canadian Journal of Physiology and Pharmacology. 1991; 69:683–694

Graven-Nielsen, T., & Arendt-Nielsen, L. (2008). Impact of clinical and experimental pain on muscle strength and activity. Current rheumatology reports, 10(6), 475-481.

AGONIST

ANTAGONIST

Hans Kraus, M.D.Functional Muscle Pain

• Tension/Stress

• Deficiency: Weakness and/or Stiffness

• Spasm

• Trigger Points

• Tender nodular area in muscle

• Taut band

• May refer to proximal and

distal muscle

• Diagnosis made by pain to

palpation

Myofascial Trigger Points

Palpation/Pressure

• Unreliable

• Sedentary vs. Activity

• Primary Muscle/Referred Pain?

• Entheses vs. muscle tissue

Marcus, N. J., Gracely, E. J., & Keefe, K. O. (2010). A comprehensive protocol to diagnose and treat pain of

muscular origin may successfully and reliably decrease or eliminate pain in a chronic pain population. Pain

Medicine, 11(1), 25-34.

Postulate:

Muscle Stimulation Hypothesis

Stimulates nociceptors in

the entheses

Externally induced contraction

Deforms sensitized

muscle tissue

(trigger points)

Hans Kraus, M.D.Functional Muscle Pain

• Tension/Stress

• Deficiency: Weakness and/or Stiffness

• Spasm

• Trigger Points

• Muscle Pain Amenable to Injection

Injections

• Injectate DOESN’T matter!

Peloso P, Gross A, Haines T, et al. Medicinal and injection therapies for mechanical neck

disorders. Cochrane Database Syst Rev 2007;3:CD000319.

• Location,

Location,

Location!

Marcus, N. J., Shrikhande, A. A., McCarberg, B., & Gracely, E.

(2013). A preliminary study to determine if a muscle pain protocol

can produce long-term relief in chronic back pain patients. Pain

Medicine, 14(8), 1212-1221.

Post-injection Physical Therapy

• Neuromuscular electrical stimulation

• Limbering exercises

Too much of a good thing?

Photobiomodulation

(Low Level Laser Therapy)

de Freitas, L. F., & Hamblin, M. R. (2016). Proposed mechanisms of photobiomodulation or low-level light

therapy. IEEE Journal of Selected Topics in Quantum Electronics, 22(3), 348-364.

Photobiomodulation

Poster Presentation

at EDS Society Meeting

May 2016

New York, NY

Conclusion

• Muscles are a key factor in understanding EDS-

related pain

• Develop protocols to lengthen effectiveness of

pain treatments

• Prevent re-injury

• Individualize treatment