PainChampions

September 15, 2016

September is Pain Awareness Month, and for the next yearthrough Pain Awareness
Month 2017The Ehlers-Danlos Society is partnering with The Pain Community to
foster awareness of pain in association with Ehlers-Danlos syndromes (EDS), how
we can approach treating pain, and along the way, we hope for some advocacy
opportunities, too. Each month, we and The Pain Community will share new articles,
and continue the conversation.

Our project is called Pain Champions. Living with chronic Ehlers-Danlos pain is much
like living as an athletic champion. Your life is dedicated to training, learning how to
get better results from the programs you undertake. Every day efforts lead to better
outcomes. Victories are measured most often against your own record; getting better
at a sport, or at managing your pain, doesnt have to involve competition against
anyone else. And we hope to help each other find new ways of becoming the best
champions we can be.

Im going to talk a little bit about Ehlers-Danlos and pain. There will be more to come
in subsequent posts, but an overview might be a helpful beginning.

For those of you who arent aware, Ehlers-Danlos syndromes are a group of
connective tissue disorders which produce a spectrum of complex problems across
multiple systems of the body. Most EDS are a distinct problem in making or using
one of the types of collagen. Collagen is found throughout the body, and is what the
body uses to provide strength and elasticity to tissue. Normal collagen is a strong
protein that allows tissue to be stretched but not beyond its limit, and then safely
returns it to normal. With a badly-constructed or processed collagen, some of the
tissue in the EDS-affected body can be pulled beyond normal limits which causes
damage. Collagen is the most abundant protein in the body and can be found almost
anywhere, in skin, muscles, tendons and ligaments, blood vessels, organs, gums,
eyes, and so onwhich means the problems can be widespread, in a wide range of
severities, and maybe even show up in places one wouldnt think are related unless
the underlying collagen is considered. The Societys web site, ehlers-danlos.com, is an
ever-growing and improving source of EDS information.
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Probably the most damaging misconception about Ehlers-Danlos has been the idea
that EDS dont result in pain. Im not sure how that started. Perhaps the first cases
were coincidentally part of the minority that dont report pain. Maybe it was because
the first case studies were all men, and men appear to report pain less frequently. For
whatever reason, you can still encounter doctors today who will tell you that you dont
have pain because of Ehlers-Danlos. Nothing could be further from the truth.

The very year the Villefranche nosology was published and EDS were clearly defined
as diagnoses, an important study was released, Chronic Pain is a Manifestation of
the Ehlers-Danlos Syndrome (Sacheti et al., 1997). The full study was reprinted in the
Societys magazine, Loose Connections; you can find the PDF through http://issuu.
com/markmartino8/docs/lc_1997-12.

The most important single point was that nine out of ten people with Ehlers-
Danlos have chronic pain, and the authors state outright, Chronic pain is a common
manifestation of EDS. Their findings included:

90% of EDS population has chronic pain for more than six months
84% feel it worsened over their lifetime
89% had chronic pain before adulthood
88% have taken pain medication
51% have taken narcotics

The authors reported, The various types of pain that individuals with EDS experience
may have different origins, different intensities, and run different courses. Our data
reveal that individuals with EDS experience frequent and severe pain through much
of their lives. These problems have been unrecognized previously in the published
literature. Because EDS is relatively rare, no systematic study of pain in this population
or its relief has been performed. Clearly, further research is necessary to identify the
most humane way to manage the devastating effects of this symptom in individuals
with EDS.

So about 60 years after EDS were named, we have not only the first comprehensive
criteria for diagnosis, but also a clear statement that most with EDS experience severe,
continual pain, and a passionate call for treatment.

Confirmation and expansion of those results were published in Pain in Ehlers-

PainChampions September 15, 2016 Page 3

Danlos Syndrome is Common, Severe, and Associated with Functional Impairment

(Voermans et al., J Pain Symptom Manage. 2010 Sep;40(3):370-8). A group of 273
people with EDS were evaluated. Most patients, 92%, reported chronic pain lasting for
more than one year; 84% reported a gradual increase of pain. Half of the patients with
pain were awake during the night because of pain, and of that half, 93% were awake
at least two hours. Impairment in performing daily activities was reported by 87%,
with 55% of them considered moderate-to-severely impaired.

Results of our study confirm the high prevalence of pain and add the finding that
pain is most common and most severe in patients with the hypermobility type of
EDSthese findings may indicate that pain in EDS has a compound origin: a constant
level of pain may originate in the musculoskeletal system, and additional peaks of
severe pain may be related to recurrent (sub)luxations and/or dislocations.

This is what I call chronic-acute pain in EDS. More on that in the coming weeks and
months.

[O]ur findings suggest that pain is a very common and severe symptom in this group
of EDS patients. It is related to dislocations, sleep disturbances, and moderate-to-
severe impairment in daily functioning. Therefore, treatment of pain should be a
prominent aspect of clinical management of EDS, and multidisciplinary protocols
should be developed.

The Ehlers-Danlos Societys Pain Management Medical Resource Guide puts it this
way: Chronic pain is a well-established and cardinal manifestation of EDS and it is
common for pain to be out of proportion to physical and radiological findings. The
etiology of EDS pain is not clearly understood, but some of the likely causes include
muscle spasm (tender points are sometimes present) and degenerative arthritis;
neuropathic pain is also common.

There shouldnt be any question about pain and Ehlers-Danlos anymore. There is
clear evidence that having EDS results in chronic and acute pain, and the evidence
is readily available to anyone any time. It is simply inhumane to tell people with
impossible pain to just suck it up. That unjustifiable position leads to actual physical
damage; untreated pain results in systemic changes that make chronic disease worse
(something else we will discuss in coming months).
PainChampions September 15, 2016 Page 4

As a last thought for today, Dr. Forest Tennant has said in his presentation Managing
Intractable Pain in Ehlers-Danlos, At this point in time, I put EDS in the category of
being in the top three or four most severe pain problems. A lot of people for example
think that cancer pain is the worst of pain, but let me assure you that many EDS
patients have pain far beyond any cancer patient Ive ever seen. And so its one of the
pain problems that is severe, has been very troublesome. Many physicians are afraid
of the disease and of the kind of pain that EDS patients have.

We deserve to be heard, and treated.

Mark Martino, The Ehlers-Danlos Society 400 Hippocrates (the Father of Western Medicine) notices
B.C. Nomads and Scythians have lax joints and multiple scars.

Job Janszoon van Meekeren (Dutch surgeon) presents a

1657 Spanish sailor known for being able to stretch the skin on
his chest out to arms length.

Alexandr Nicolaevich Chernogubov presents a 17 year old

boy with recurrent joint dislocations; cutaneous nodules;
1892 hyperextensible and fragile skin; and multiple scars from
minor injuries. Chernogubov diagnoses an abnormality in
the boys connective tissues.

Edvard Ehlers presents a 21 year old law student with a

history of late walking and frequent knee subluxations;
1899 many haematomata on minor trauma and discoloured
lesions on the elbows, knees, and knuckles; extensible
skin; and lax digits.

Henri-Alexandre Danlos disputes the diagnosis of a

young boy who presented with lesions on his elbows and
knees by drawing attention to his fragile and extensible
1906 skin, stating that the lesions were post traumatic pseudo
tumors rising from an inherent defect Danlos named
cutis laxa.

Frederick Parkes-Weber suggests the name

1936 Ehlers-Danlos syndrome. In Russia, the condition is
known as Chernogubov Syndrome.

The Berlin nosology of Heritable Connective Tissue

Disorders is published, representing the consensus of
1988 opinions from geneticists, and defining syndromes
(including the numbered system of Ehlers-Danlos
syndromes) based on phenotype.

The Villefranche nosology is published, re-examining the

diagnosis of Ehlers-Danlos syndrome and resulting in a
1997 revised, named system based on phenotype that begins
to integrate the molecular basis for EDS.

The New York nosology rising out of the EDS

2017 International Symposium 2016 will be published, taking
into account the research and clinical experience of 20
years.