PainChampions (September 15, 2016)

PainChampions September 15, 2016 September is Pain Awareness Month, and for the next year — through Pain Awareness Month 2017 — The 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, doesn’t 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.

I’m 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 aren’t 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 on—which means the problems can be widespread, in a wide range of severities, and maybe even show up in places one wouldn’t think are related unless the underlying collagen is considered. The Society’s web site, ehlers-danlos.com, is an ever-growing and improving source of EDS information.

PainChampions

September 15, 2016

Page 2

Probably the most damaging misconception about Ehlers-Danlos has been the idea that EDS don’t result in pain. I’m not sure how that started. Perhaps the first cases were coincidentally part of the minority that don’t 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 don’t 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 Society’s 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 EhlersDanlos 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 EDS…these 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-tosevere 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 Society’s 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 shouldn’t 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 I’ve ever seen. And so it’s 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 B.C.

Hippocrates (the “Father of Western Medicine”) notices Nomads and Scythians have lax joints and multiple scars.

1657

Job Janszoon van Meekeren (Dutch surgeon) presents a Spanish sailor known for being able to stretch the skin on his chest out to arm’s length.

1892

Alexandr Nicolaevich Chernogubov presents a 17 year old boy with recurrent joint dislocations; cutaneous nodules; hyperextensible and fragile skin; and multiple scars from minor injuries. Chernogubov diagnoses an abnormality in the boy’s connective tissues.

1899

Edvard Ehlers presents a 21 year old law student with a history of late walking and frequent knee subluxations; 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.

1988

The Berlin nosology of Heritable Connective Tissue Disorders is published, representing the consensus of opinions from geneticists, and defining syndromes (including the numbered system of Ehlers-Danlos syndromes) based on phenotype.

1997

The Villefranche nosology is published, re-examining the diagnosis of Ehlers-Danlos syndrome and resulting in a 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.