EHLERS-DANLOS SYNDROME EDS AND PAIN MANAGEMENT PAIN MANAGEMENT

Collagen is the most abundant

protein in the human body. Medical Resource Guide
It provides the structural strength in most human
tissue, including the heart and blood vessels, eyes
and skin, cartilage and bone.
What happens when this basic
building block is flawed?
When muscles, ligaments, tendons and even
large organs are built with structurally defective
collagen there is systemic weakness and instability
evident throughout the body.
There is Ehlers-Danlos Syndrome.
PERCENT OF EDS POPULATION
Excessively mobile joints, chronic pain and skin
softness characterize Ehlers-Danlos syndrome 90% CHRONIC PAIN +6 MONTHS
(EDS). At least six types of EDS have been iden- 84% WORSENED OVER LIFETIME
tified; clinical manifestations vary according to 89% CHRONIC PAIN PREADULTHOOD
type and may also include poor wound healing
with atrophic scars, easy bruising, chronic pain 88% TAKEN PAIN MEDS

and generalized connective tissue fragility. Each 51% TAKEN NARCOTICS

type is thought to involve a unique defect in STATISTICS FROM Chronic Pain is a Manifestation of the Ehlers-Danlos Syndrome
(Sacheti et al, 1997); full text available at ednf.org
connective tissue, although not all of the genes
responsible for causing EDS have been found.
Within each family the type of EDS runs true, but
individual family members may vary in clinical
severity and manifestations.
As a group of genetic disorders of connective
tissue, the estimated prevalence of EDS is 1 in
5,000. It is known to affect men and women
of all racial and ethnic backgrounds.
Chronic pain is a well-established and
cardinal manifestation of EDS and it
is common for pain to be out of pro-
portion to physical and radiologi-

1760 OLD MEADOW ROAD

MCLEAN, VIRGINIA 22102

cal findings. The etiology of EDS

MRG
pain is not clearly understood, but

Supported by a grant from

some of the likely causes include
muscle spasm (tender points are

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sometimes present) and degen-

SUITE 500
erative arthritis; neuropathic
pain is also common.
MULTI-MODAL MANAGEMENT FOR PAIN IN EDS TOLERANCE, DEPENDENCE & ADDICTION EHLERS-DANLOS NATIONAL FOUNDATION
INFREQUENT PAIN EVENTS CHRONIC PAIN Tolerance is not addiction. Pain Management Medical Resource Guide brings you
highlights of information found at ednf.org, available
(less than four days per week) (constant & disturbing pain) whenever and however you need it.
The capacity to endure continued subjection to something, Pain Management Medical Resource Guide and all its
ANALGESICS MANAGE ACUTE EVENTS esp. a drug; diminution in the body’s response to a drug contents are copyright © 2007 Ehlers-Danlos National
May be ineffective as single agents or Treat acute events quickly and aggressively to defend against
after continued use. (From New Oxford American Dictionary 2007) Foundation. Ehlers-Danlos National Foundation is a
require excessive doses central neuronal plasticity (“learned” chronic pain)
501 (c) (3) non-profit organization dedicated to raising
if needed ANALGESICS FOR CHRONIC PAIN Physical dependence is not addiction. awareness of EDS.
Short-acting opioids Use combinations of medicines on a schedule to keep pain
Muscle relaxants under control PAIN MANAGEMENT M.R.G. ADVISORY PANEL
if needed A state of adaptation that often includes tolerance and is Mark Lavallee, MD, CSCS, FACSM Nazli B. McDonnell, MD, PhD
PHYSICAL THERAPY
Short-acting opioids (breakthrough pain) manifested by a drug class specific withdrawal syndrome Sports Medicine Institute Research/Genetics
Reconditioning Memorial Hospital & National Institute of Aging
Long-acting opioids (baseline pain; see at bottom) that can be produced by abrupt cessation…. Physical
Exercise (gradual toning for stability, not strength 1) Health System National Institutes of Health
Flare-up management by: PREVENT RECURRING INJURY dependence on and tolerance to prescribed drugs do not South Bend, Indiana Baltimore, Maryland
Distraction techniques Hypermobile joints and fragile connective tissues endure chronic constitute sufficient evidence of psychoactive substance
acute injury Howard P. Levy, MD, PhD Melanie Pepin, MS, CGC
Trigger-point/myofascial therapy use disorder or addiction. They are normal responses that Division of General Internal Genetic Counselor
Heat/cold application Bracing may be helpful (maintain toning exercise) often occur with the persistent use of certain medications. Medicine & McKusick-Nathans Collagen Diagnostic Laboratory
Ultrasonic/electrical stimulation Orthopedic surgery should be avoided (From “Definitions related to the medical use of opioids: Institute of Genetic Medicine University of Washington
Almost never provides long-term improvement Evolution towards universal agreement”; Savage et al., Journal Johns Hopkins University Seattle, Washington
PSYCHOLOGICAL THERAPY Added risk of soft tissue fragility and poor wound healing, esp. of Pain and Symptom Management, 2003, 26:655-67) Baltimore, Maryland
Relaxation Classical & Vascular EDS
Stress management OCCUPATIONAL THERAPY
The choice may be simple: dependence PAIN MANAGEMENT MEDICAL RESOURCE GUIDE
(incl. Mindfullness-Based Stress Reduction) on drugs or dependence on pain.
Group therapy
Delay disability and encourage activity as long as possible
Body mechanics/ergonomics
IS SUPPORTED BY A GRANT FROM
Cognitive restructuring (incl. hypnosis) Intervention in workplace and work simplification
Pacing skills The fact or condition of being addicted to a particular
PSYCHIATRIC THERAPY substance, thing, or activity, when a person has lost control
Medication for mood/pain NEUROPATHIC PAIN over and continues use even when such use is doing them
CONTRIBUTING PROBLEMS

Individual counseling Common in EDS, also called neuralgia (different from usual
nociceptive pain); described as numbness, tingling, burning,
SLEEP THERAPY electrical, hot/cold
Sleep disturbance is common in EDS (contributes Incl. disease/dysfunction of peripheral nerves (numbness,
to poor pain recovery; incl. apnea) weakness), nerve injury, axonal loss
Tricyclic antidepressants (TCAs) Antidepressants (TCAs/SNRIs)
or others harm. (From New Oxford American Dictionary 2007)
The chance of addiction is very low when these medications
are taken as directed by a doctor and used for pain.
Purdue Pharma L.P., a privately held pharmaceutical company
founded by physicians, is focused on the needs of patients.
Together with Purdue Products L.P. and other independent
associated companies, Purdue is dedicated to finding, developing,

COMPLEMENTARY/ALTERNATIVE
Antiseizure medications
Topicals (capsaicin)
URGENT INFORMATION on Vascular EDS and bringing to market new medicines and related products that
promote health and healing. We are known for our pioneering
Meditation ARTERIAL RUPTURE IS THE MOST research on persistent pain, a principal cause of human suffering.
COMMON CAUSE OF SUDDEN DEATH.
Acupuncture
Massage
NON-PAIN PATHOPHYSIOLOGY Partners Against Pain®
Additional genetic factors/disabilities • Arterial or intestinal rupture commonly presents as acute
Yoga Side effects from treatments, incl. overuse abdominal or flank pain that can be diffuse or localized. Keeping you informed about new
MAINTAIN GENERAL HEALTH
or ineffectiveness of analgesics or • Spontaneous arterial rupture is most likely to occur developments in pain management.
short-acting opioids
Avoid weight extremes & smoking2 in a person’s twenties or thirties, but can occur at any In 1993, Purdue created Partners Against Pain® (PAP)
point in life. to forge an informational alliance among physicians,
1The key is slow improvement in muscle tone, not strength. Tone is the LONG-ACTING OPIOIDS (methadone, morphine, • Cerebral arterial rupture may present with altered mental
degree of muscle contraction at rest; strength is voluntary force that can oxycodone, fentanyl; oral/transdermal/pump) nurses, pharmacists, hospice personnel, pain experts,
status and be mistaken for drug overdose.
be invoked at will. Build tone with non-resistance exercise that gradually Tolerance for pain-killing builds up readily, but not to side patients, and caregivers in the fight to ease cancer and
• Mid-size arteries are
increases repetitions. Avoid hyperextension, resistance and impact. Elastic effects incl. GI dysmotility & reduced consciousness non-cancer pain. Purdue sponsors the award-winning
bands may make things worse because they combine increased resistance commonly involved.
website — www.partnersagainstpain.com — an
with joint hyperextension. Toning is a life-long strategy; if exercises are
SURGICAL PAIN INTERVENTION Arterial, intestinal, or uterine invaluable resource that provides pain information,
stopped, pain usually recurs. (Howard Levy, MD, PhD)
Injection (trigger point, nerve root) fragility or rupture usually arise assessment tools, and support 24 hours a day.
2Quit smoking (pain, depression and addiction are all modulated by the
same nicotinic and muscarinic receptors). Maintain normal weight; obesity Implantable nerve stimulators in EDS Vascular type, but should
The content of this publication is provided by EDNF and has
or thinness seem to present problems. (Mark Lavallee, MD, CSCS, FACSM) Medication pumps (subarachnoid cavity) be investigated for any EDS type. not been created or modified by Purdue in any manner.

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