Loose Connections (November 1997)

Loose Connections This Issue Is Devoted To The Topics Of EDS And Pain Vol. XII, Number 4 The Official Communications Link Of The Ehlers–Danlos National Foundation 6399 Wilshire Blvd. Suite 510 Los Angeles, California 90048

November, 1997 (213) 651–3038

Chronic Pain is a Manifestation of the Ehlers–Danlos Syndrome Anubha Sacheti, Judy Szemere, Bruce Bernstein, Triantafyllos Tafas, Neil Schechter, Petros Tsipouras “Reprinted by permission of Elsevier Science, Inc. Journal of Pain and Symptom Management, Vol. 14, No. 2, pp. 88–93. Copyright 1997 by the U.S. Cancer Pain Relief Committee.”

Department of Pediatrics, University of Connecticut Health Center, Farmington CT (A.S., J.S., B.B., N.S., P.T.), Center for Children’s Health and Development, Saint Francis Hospital and Medical Center, Hartford CT (B.B., N.S.), Department of Biology, University of Athens, Greece (T.T.).

AS and JS contributed equally to this paper.

Corresponding Author: Petros Tsipouras, MD Department of Pediatrics University of Connecticut Health Center 263 Farmington Avenue Farmington, CT 06030

In This Issue...

Chronic Pain is a Manifestation of the Ehlers–Danlos Syndrome ..................... 1 MAJOR Fundraising Event ................. 2 Notes From Nancy ............................... 2 The Chair’s Connection ........................ 3 Manuscript Guidelines .......................... 3 The Executive Element ......................... 4 Branch News ..................................... 5–6 Wish List ............................................... 9 Learning To Manage Fibromyalgia Syndrome ..................... 10 Membership Dues ............................... 13 Branch Bylaws Committee Update .... 13 Guidelines to Select a Pain Unit ......... 14 LettersTo The Editor ........................... 15 Memorials and Honorariums .............. 16 Donations ............................................ 17 1998 Learning Conference Update ..... 17 The International Ehlers–Danlos Syndrome Network (I.E.D.S.N.) ........ 17 Back Issues .......................................... 18 Membership Form ............................... 19

Abstract The Ehlers–Danlos syndrome (EDS) is a group of heritable systemic disorders of connective tissue manifesting joint hypermobility, skin extensibility and tissue fragility. Although the presence of pain has been documented in the various types of the EDS its natural history, distribution and management have not been defined. We conducted a semi–structured interview in 51 individuals affected with different types of EDS. The overwhelming majority of individuals reported chronic pain of early onset involving several joints and refractory to a variety of pharmacologic and physical interventions. Our data shows that chronic pain is a common manifestation of EDS.

impossible to determine the exact type of EDS in a substantial proportion of affected individuals [Beighton et al., 1988]. The variable degree of clinical expression probably accounts for the underdiagnosis of EDS and hence the lack of accurate figures of prevalence for the various types. EDS type III is the most common form of the disorder presenting with generalized joint hypermobility frequently complicated by recurrent joint dislocations [Beighton, 1993].

Affected individuals are often unable to ambulate because of joint instability. EDS types I and II whose cardinal manifestation are skin fragility and generalized joint hypermobility are the best recognized forms of the disorder [Beighton, 1993]. EDS type IV Key Words: Ehlers–Danlos syndrome, is an uncommon type of EDS but also one chronic pain, heritable disorders of associated with the highest morbidity and connective tissue, natural history, analgesics. reduced life expectancy [Beighton, 1993].

EDS type IV is associated with spontaneous rupture of internal organs, i.e. arteries, Introduction intestine, uterus and to a lesser degree with skin and joint manifestations.

The Ehlers–Danlos syndrome (EDS) is a group of heritable disorders of connective Although the presence of pain has been tissue characterized by varying degrees of documented, its contribution to the overall joint hypermobility, skin extensibility, and morbidity associated with the disorder has tissue fragility [Beighton, 1993]. Pain is a never been assessed, nor has its scope and frequent yet poorly characterized clinical intensity been formally investigated. The finding in EDS [Beighton, 1993]. Nine impetus for the present study was provided by different types of EDS have been described, the findings of Lumley et al. [1994] who each clinical type distinguished from the others depending on what system is primarily reported that pain significantly affected the psychosocial functioning of individuals with involved. Most of the types of EDS are EDS. The present study indicates that chronic, inherited as an autosomal dominant trait, frequently debilitating pain of early onset and although X–linked and autosomal recessive diverse distribution is a constant feature in types have been described [Beighton, 1993]. most individuals affected with different types The diagnosis of EDS is clinical. Although diagnostic criteria have been promulgated for of EDS. The presence of pain in association each of the different types of EDS, it is Continued Continued on on page page 8.7 Views expressed herein are only those of the authors, and should not be construed to represent the opinions or policies of the Ehlers–Danlos National Foundation and it’s elected officials.

L oose Connections Published quarterly by the

Ehlers–Danlos National Foundation — Founder — Nancy Hanna Rogowski 1958 – 1995

Executive Director Linda Neumann–Potash, R.N., M.N.

President Nancy Regas, R.N., M.S., M.F.C.C.

Vice President Karen Skrocki Czerpak, R. N.

Mark your calendars and plan on attending if you can. The EDNF is hosting it’s first major fundraising event, “Comic Connections” with HAR VEY KORMAN (yes, you read his name HARVEY correctly!) and YARMY’S ARMY on Sunday, June 14, 1998 at the Olympic Collection in Los Angeles, California. This black tie comedy night will feature Harvey Korman and many other well known comedians.

The Foundation is indebted to Harvey Korman and wishes to extend our gratitude to him for the time and dedication he has given to this project.

The purpose of this event is not only to raise funds for the EDNF, but we are also seeking more exposure for EDS as well as the Foundation nation wide. More details to follow in future issues of Loose Connections and on .ednf .or g our new web site: http://www http://www.ednf .ednf.or .org

VP for Patient Advocacy Susan Stephenson, R.N., B.S.N., C.C.R.N.

Secretary Meryl B. Brutman, M.P.H.

Notes From Nancy

Treasurer

Nancy L. Regas, RN, MS, MFCC — President, EDNF

Harold Goldstein

Board of Directors Darlene A. Clarke, R.N., M.S.N., Chair Meryl B. Brutman, M.P.H. Maggie Buckley Karen Skrocki Czerpak, R.N. Harold Goldstein Linda Neumann–Potash, R.N., M.N. Nancy Regas, R.N., M.S., M.F.C.C.

Medical Advisory Board Petros Tsipouras, M.D., Chair Patrick Agnew, D.P.M. Robin Bennett, M.S. William Cole, M.D. Mark Evans, M.D. Richard Wenstrup, M.D.

Medical Consultants Pat Aulicino, M.D. Peter Beighton, M.D., Ph.D. Peter Byers, M.D. Sheldon Pinnell, M.D. F. Michael Pope, M.D. Catherine A. Stolle, Ph.D. Alan Weinberger, M.D.

Editors Darlene A. Clarke, R.N., M.S.N. Karen Skrocki Czerpak, R.N. Harold Goldstein Linda Neumann–Potash, R.N., M.N.

Designer & Publisher Keith G. Clarke (kclarke@en.com)

This newsletter has focused on a topic that most people would just as soon never have to confront; PAIN. Pain can be an aggravating, limiting, overwhelming entity that is confined to the person enduring it. Because it cannot be seen, touched or heard, but only experienced by its owner, it lends itself to misunderstanding by others. Pain can be easily dismissed by others because they cannot feel it exactly like its recipient. It can also be exaggerated by its owner because it cannot be officially challenged. Those opposite ends of the spectrum create an antagonistic relationship which contradicts the goal of health. How then can we live with pain since it doesn’t seem to be going away? Do we really want pain to disappear forever?

It is important to understand the function of pain. Pain is an alarm that goes off internally to signal its owner that adjustments need to be made somehow. All too often we ignore the signal at its first sounding causing it to get louder and more intense. Finally when we try to do something, it is too little too late. We may even try to hush the sound without understanding its cry hence we are surprised when it returns with greater force or greater frequency. Sometimes comprehending its meaning in our lives comes with relative ease if we stop and really listen; other times, however, it takes much investigation and the assistance of medical personnel who are willing to listen with us.

That leads to my next point. If in fact you are one of those persons who needs to listen, the first step comes in the acceptance of the owner’s definition. All too often our lack of understanding leads to a feeling of powerlessness; therefore we resort to what is easier by saying to the owner that the pain shouldn’t, couldn’t possibly be there and if it is there, it must be the owner’s fault. This creates a defensive posture with the owner of the pain, and then, time is wasted on justifying the pain instead of trying to interpret and respond to it.

The game begins. The owner of the pain is asking, “Do they believe me?” While the health care providers are asking, “Do we believe the storyteller?” Not only is time wasted, but the chances of building a relationship based on trust is greatly damaged.

Pain is an inescapable part of life. Without it we would be placed at greater risk because we would never know when the boundaries between safety and danger are crossed. We would never know when the body is crying for help. So in that case, pain is a necessary part of our existence. The challenge to discover how best to interpret it and how to shut off the alarm when we have heeded its warning. There is no one in this life who has eluded pain from their life’s journey. The task at hand remains to hone our listening skills whether we be the owner of the pain or the helper. We never know when our bell will ring.

Warmly, Nancy L. Regas

November, 1997 Ehlers–Danlos National Foundation — Page 2

Manuscript Guidelines for...

The Chair’s Connection Darlene A. Clarke, R.N., M.S.N., Chair, Board of Directors Fall is quickly slipping away and Winter is rapidly approaching. As the seasons undergo change, so has the EDNF. It is with extreme pleasure that I announce the most recent change for the Foundation. We now have the addition of Maggie Buckley to the Board of Directors. Many of you will recall seeing her name linked in Loose Connections to the Northern California Branch where she is currently the President, and as a member of the EDNF Branch Bylaws Committee. Maggie is no stranger to the Foundation, she has been an active member since 1991 and is always helping in whatever way she can. Not only has Maggie founded the Northern California Branch and served on the Branch Bylaws Committee, she brings considerable expertise, skill and knowledge to the Board and the Foundation.

Maggie received a B.A. in Applied Social Relations and her M.B.A. in Accounting. She is currently employed as a Business Manager and Controller. She oversees all the accounting and reporting functions for a corporation in California. Other significant accomplishments Maggie has achieved include: leading two organizations through the steps of Strategic Planning, identifying and administering training budgets, and overseeing the conversion and implementation of accounting software for two different employers. Additionally, she served two years as a fundraising volunteer for the American Cancer Society. These are just a sampling of some of the skills and talents Maggie is bringing to the Board of Directors as well as the Foundation.

Please join me in welcoming Maggie to the EDNF Board of Directors. With the assistance of people like Maggie, we will truly find a brighter day for individuals with EDS. Respectfully, Darlene

Loose Connections 1.

Manuscripts should be voluntary contributions submitted for the exclusive attention of Loose Connections.

2.

The submitted manuscripts should be written in a clear and concise manner. The author(s) should write in a style appropriate for lay audience. The content of the manuscript must focus on Ehlers–Danlos Syndrome, complications of EDS, current research on EDS, or the day to day issues of living and coping with EDS.

3.

Manuscripts should be submitted on a 3.5" disc in WordPerfect 5.1 or higher or an IBM compatible word processor. If this is not possible, the manuscript must be typed double spaced.

Handwritten manuscripts will be automatically rejected.

4.

Fancy type fonts, italic, bold and underlines are not to be utilized. We will convert them to our printing style.

5.

Manuscripts/discs should be sent to: The Ehlers–Danlos National Foundation 6399 Wilshire Blvd., Suite 510 Los Angeles, CA 90048

6.

A 100 word abstract should be included that stimulates readers’ interest in the topic and states what the readers will learn or how they will be better off after reading the article.

7.

Include a title/author biography page. The authors’ biographic information includes: name, credentials, position, professional affiliation, city and state. Example: Thomas Smith, M.D., Professor, Department of Pediatrics, Case Western Reserve University, Cleveland, OH.

8.

Tables and figures should be placed at the end of the manuscript after the references. Tables must be numbered consecutively with Arabic numbers and have a title at the top. Figures and tables must be cited in numerical order in the text.

9.

Number pages consecutively centered at the bottom of each page.

Do not justify the right margin. Do not use running headers or footers.

10. Subdivide the manuscript into main sections by inserting subheads in the text. Subheads should be succinct and meaningful.

We’re On The Internet See Our Brand New Web Site http://www.ednf.org Contact The Foundation At Our E–Mail Address: loosejoint@aol.com

11. References are placed at the end of the manuscript. References are cited consecutively by number and listed in citation order in the reference list.

12. Written permission must be obtained from a) the holder of copyrighted material used in the manuscript; and b) individuals mentioned in the narrative or acknowledgment. Letters of permission must be submitted to the publisher of Loose Connections before publication of the manuscript.

13. If you request that your 3.5 disc or original manuscript be returned after publication, enclose a self–addressed envelope or manuscript–sized envelope with sufficient postage affixed.

14. Surveys submitted for publication in Loose Connections must include a letter showing IRB (Institutional Review Board) approval from the researcher’s associated institution.

15. Loose Connections reserves the right to edit all manuscripts to its style and space requirements and to clarify the presentation if necessary.

November, 1997 Ehlers–Danlos National Foundation — Page 3

The Executive Element Linda Neumann–Potash, RN, MN The Editorial Board of Loose Connections decided several months ago that the focus of this issue would be on pain and EDS. Pain, whether acute, chronic or a combination of the two is something that affects nearly everyone with EDS. One of the most difficult components of dealing with chronic pain is being able to accurately communicate pain to your physician or other health care professional.

Pain is very subjective, therefore only the person who is experiencing the pain knows what it really feels like. It’s difficult at best, to convey pain that is located in only one part of the body. Describing the type, location and intensity of pain in EDS is complicated by the fact that more than one site is usually involved. One may have a combination ranging from no pain to severe pain depending on the location.

Now that I’ve listed several reasons why describing pain may be difficult, I thought I’d give some practical tips on how to talk to your physician about pain. While I’m not a physician or a pain management expert, my experience comes from 12 years as a nurse, as well as living with chronic pain on a daily basis.

First and foremost, you need to have a plan or agenda for each office visit and then listen to what your physician is asking. Decide before hand when during the visit are you going to talk about your pain. Timing is extremely important. Saying, “by the way, I’m in a lot of pain” as your physician stands up and is about to leave the room, is probably not the best time to bring up the subject. Make a list beforehand of things to discuss with your physician, prioritize the list before your appointment and bring it with you.

Preparation and timing are not the only skills you will need when discussing pain with your physician. You must also be able to accurately rate the pain you are experiencing.

The second point I made is to listen to what your physician is asking. A very common question, physicians ask in relation to pain is; “On a scale of 0–10, with 0 being no pain and 10 being the worst pain imaginable, what level is your pain?” This question gives two distinct boundaries, “O” is no pain and “10” is the worst pain you can imagine. Therefore your answer must be within this scale. If you are in severe pain and answer that your pain is a level 12, I can almost guarantee that your physician will not take you seriously. If 10 is the worst pain imaginable, answering 12

The next time you go to the physician, take these drawings with you. It provides a visual way to describe the pain you are having over a period of time. For me, it’s much easier to discuss my pain using this drawing rather than trying to explain it. Using the body outline drawing is great for kids who are in pain. Look how difficult it is for us as adults to describe pain, it’s much more difficult for a child. This works well for children over 7 but it even works for some younger children.

The important thing is to let the child pick the colors to represent the pain.

There are many other tools available to assist you.

Your task is to figure out what works makes no sense and invalidates your best for you. I realize that for many it’s response. Remember to stay within the scale, difficult to discuss pain with your physician. and pay attention to the parameters. Some Keep in mind that once you find the way that physicians use a scale of 0–5 or a scale of 0– works best for you it will more than likely 100. While rating your pain will be helpful to lead to effective pain management. your physician, it does not address multiple pain sites in the body.

A component of coping and living with EDS is learning to deal with your pain. An important I mentioned earlier that often more than one key aspect of pain management is to move past site is involved and the intensity may differ the point of focusing all of your energy on pain. depending on the site. Using a scale such as It is imperative that you learn to accurately 0–10 does not take into account different learn to communicate the pain you experience intensities of pain. I prefer to use a to your physician. The “Eland Body Outline modification of the “Eland Body Outline Pain Tool” is one such method that will help Pain tool.” Using a body outline allows you you achieve this goal. Keep in mind that there to show the intensity of pain in different are many ways to treat pain, and often times it locations throughout your body. You will need a body outline on a piece of paper and 4 takes a combination of approaches. Do not be discouraged if the first approach does not different color markers, crayons, or pencils. manage your pain – employ another method.

You can draw a body outline freehand, or Eventually, an effective method of pain control Xerox one out of a coloring book, or other will be discovered and you will find that there material. Make sure you have two outlines, is life beyond EDS pain! one of the front of the body and one of the back. Next choose your 4 colors. Pick one Sincerely, color to represent “no pain”, one color to Linda represent “mild pain”, one color to represent “moderate pain” and one to represent “severe pain”. Make sure you indicate on the top of The Board of Directors the paper what intensity of pain is represented by what color. The next step is to would like to extend a fill in the outline using the 4 colors BIG THANK YOU to: indicating the intensity of pain you are Theresa Wong having at the time. Write the date and time La Pointique on the page and any other information you want to include.

International Ltd. The body outline tool gives you and your physician a visual description of the pain you are experiencing at the time. If your pain intensity changes throughout the day, try doing the drawing at several intervals. You can also use the tool before and after you take pain medication, relaxation therapy or any other intervention that you use to decease your pain.

Turwila WA For donating a 8' X 10' Pro Display Back–Wall Booth to be used for presentations at professional conferences. This beautiful display provides us with a professional image at these very important events.

November, 1997 Ehlers–Danlos National Foundation — Page 4

— Branch News —

By Harold Goldstein, Director, Local Branch Organization On September 26th, the Board of Directors issued charters to two new Branches, MINNEAPOLIS–ST. PAUL and WASHINGTON METROPOLITAN AREA, bringing the total number of chartered Branches to 20.

At the same meeting, the Board of Directors took other actions affecting Branches: it approved a set of standard Bylaws for Branches, drafted by a committee consisting mostly of people active in Branches; it appointed as Educational Co-Directors for the Foundation Karen Czerpak, R.N., and Darlene Clarke, R.N., M.S.N. Karen will have the responsibility of acquiring copies of current publications on EDS suitable for member education or outreach to health professionals, and also to acquire a library of taped lectures by speakers at Branch meetings, to be made available to other Branches. Darlene will have the responsibility for developing educational publications for healthcare professionals, members and the general public. The Board also called upon any Branches that have conducted successful fundraising to share their experience by writing brief articles for Loose Connections on how they did it.

Here’s where Branches are being organized, and their Branch presidents or volunteer organizers. (Chartered Branches are shown in BOLD and PURPLE CAPS.)

AZ

Phoenix: The group is trying to set up a meeting, and asks any members in the area who are interested to call Debbie Krueger, (602) 978–1016.

Tucson: Robin Forsyth, (602) 579–8351.

CA

SOUTHERN CALIFORNIA BRANCH: A meeting of the Branch will be held on November 23rd at Sherman Oaks Hospital. Active support groups meet in Santa Barbara and the San Fernando Valley. Shari Gamson: (818) 893–3937 or Rich Scholl (805) 683–8874.

NORTHERN CALIFORNIA BRANCH: On November 15th a dinner and silent auction will be held at the Basque Cultural Center to raise funds for scholarships to attend EDNF’s Learning Conference in Tampa, FL next summer. Maggie Buckley, President (510) 947–2358.

DC

WASHINGTON METROPOLITAN BRANCH: At the September 7th meeting, the Branch planned its program for the fall and the kinds of speakers it would like to hear from. Marilyn Della Badia, President, (703) 924–1220.

FL

TAMPA BAY AREA BRANCH: The golf tournament was rained out, but rescheduled for early October. The Branch has been busy planning for the national EDNF conference which it will host next summer. The Branch’s lively newsletter, Tampa Bay Connections, prints recipes as well as fund-raising ideas. Peggy Rocha Snuggs, President, (813) 949–1585.

Southeast Florida: Colleen Butcher, (561) 283–9499.

GA

ATLANTA BRANCH: A meeting on September 27th was devoted to support group activity and planning future activity. At a meeting on November 22nd, the group will hear a report from April Leaman , President, who will attend the Baltimore Human Genetics meeting; the group is interested in getting information on the new nosology for EDS to doctors in the community. Contact Kathy Parrish, Secretary, (770) 338–9570.

IL

CHICAGO BRANCH: A call to the local Arthritis Association produced three speakers for the Branch’s October 4th meeting: Dr. Carey Dachman, a rheumatologist, Dr. David Wakely, a psychologist, and Robert Hayden, a certified acupuncturist. Pat Damler, Branch President, has run a one–woman professional outreach program, lecturing on EDS to staffs of the University of Illinois Family Practice Clinic and the Swedish– American Hospital, at Rockford. On January 3rd, Torsten Muehl, a physical therapist, will talk to the Branch at Alexian Brothers Hospital, Elk Grove Village. Pat’s phone is (815) 568–6216.

ME

MAINE BRANCH: The Branch will meet the second Sunday of every even–numbered month. At the October 12th meeting, the plan was to videotape the members telling what the EDNF means to them, for showing at the EDNF fundraising event planned for next year in Los Angeles. Samantha Paine–Paradis, President, (207) 442–8779.

MD

BALTIMORE BRANCH: Branch members will staff the EDNF’s booth at the National Human Genetics Conference to be held in Baltimore, October 29th to November 1st. At the December meeting, the Branch will review its work for the year and plan ahead. Michelle Adams, (410) 879–0548.

MA MI MN

Boston: Plans were under way for a November meeting at press time. Robin Neas, (617) 767–4553.

Detroit: Marianne Hoppel, (248) 363–2755.

MINNEAPOLIS–ST. PAUL BRANCH: At its October 7th meeting, the Branch discussed how to get brochures for lay people on EDS that doctors can hand to patients. Jackie Collins, President, (612) 934–4420.

MO

ST. LOUIS BRANCH: Janet Dunn, President, (314) 645–4114.

Continued On Page 6

November, 1997 Ehlers–Danlos National Foundation — Page 5

— Branch News — NB NH NJ

Omaha: First meeting will be held in January. Cheri Lynne Woodward, (402) 451–8905.

Manchester: Tom and Tina Roe, (603) 624–7947.

CENTRAL NEW JERSEY BRANCH: The Branch has established these goals: mutual emotional support and friendship, and development of a local network of medical professionals familiar with EDS. Next meeting will be November 9th in East Brunswick. Lisa Schoenberg, President, (732) 254–1288.

SOUTHERN NEW JERSEY BRANCH: Cathy Bowen, President, (609) 625–7975.

NY

NEW YORK METROPOLITAN AREA BRANCH: On October 4th, the Branch had its second dinner in an Italian restaurant. La dolce vita? Not at all. Look for Kim’s upcoming article in Loose Connections, “The Effect of Marinara Sauce on Collagen.” City Bank in mid–town Manhattan is giving the Branch the use of a computer room where members will be taught how to search Medline and how to tap into various EDS websites. The Branch mails meeting notices, and also runs a “phone tree” four days before each meeting to remind all members; this boosts attendance. Kim Christensen, Co–President, (914) 632–7264.

WESTERN NEW YORK BRANCH: The mailing list of health professionals is up to over 800, but must wait until the new Nosology is published to include accurate, current EDS information. At a meeting October 24th at Crossroads House, Batavia, NY the group showed a film on the Human Genome Project and held a support group session. Lou Van Wert, President, (716) 688–2756.

NC

PIEDMONT TRIAD BRANCH: On November 22nd at the Wesley Long Community Hospital in Greensboro, Deborah Larrimore, a licensed massage therapist, will demonstrate procedures patients can do themselves to alleviate pain. A holiday dinner is planned for December 27th. The Branch has discussed future activities, including speaking on EDS at a meeting of the local nurses’ association, speaking at a State meeting of social workers to show that children with bruises are not necessarily “abused children”, getting onto a morning TV program and holding a fundraising activity. Charlotte Mecum, President, (910) 722–5879.

Western North Carolina: Hannah Dickson, (704) 253–1323.

Raleigh–Durham–Chapel Hill: Jules Leggett, (919) 722–9443.

OH

NORTHEAST OHIO BRANCH: On September 13th, the Branch held a social and took a historic tour of Cleveland on Lolly the Trolly. The October 4th meeting was devoted to a discussion of coping with EDS. They’ll have their annual holiday social on December 6th at SW General Health Center . The Branch raises funds through the sale of sweatshirts, polo shirts, T–shirts (all with the Branch’s logo embroidered), various artifacts engraved with the Branch’s logo, as well as nuts and candies for the holidays. Darlene Clarke, President, (440) 888–7317.

OR PA

Portland: Shulamit Levine, (503) 775–0058.

RI TX

Providence: Sue Kozlow would appreciate signs of interest, (401) 233–2046.

PHILADELPHIA BRANCH: The Branch’s newsletter is “The Bugsquasher.” Want to know why? Call Roberta Kroll, President, (215) 794–8043.

Dallas: Sarah Tovar, (972) 625–9142.

Amarillo: A meeting is planned for November 1st. Connie Nieto, (806) 293–7361.

Odessa–Midland: Shawanda Cox, (915) 385–1628.

VA WA WI

RICHMOND BRANCH: Kim Hayes, President, (804) 739– 0739.

SEATTLE BRANCH: On December 6th, Dr. Jonathan Wright will speak on nutrition at the Burien Library.

Barbara Uggen, President, (253) 529–4861.

MILWAUKEE BRANCH: On October 21st, Jennifer Feltz of the YMCA described programs offered at the “Y” for people with disabilities. On November 18th, Dr. Montavon was due to speak on alternative pain management methods. All meetings are at Children’s Hospital. Lynn Sanders, President, (414) 679–9682.

For Information About Local Branches & Support Groups Your organizer would appreciate a call from you expressing your interest. If you would like to set up a branch in your area, write or call: Harold Goldstein, Director, Local Branch Organization, 4701 Willard Ave., Apt 934 Chevy Chase, MD 20815 (301) 656–2053

November, 1997 Ehlers–Danlos National Foundation — Page 6

Chronic Pain... from page 1 with the absence of a systematic approach for The participants ranged in age from 9 to 70 substantially smaller number of painful years with a mean of 34, but only 6 were less locations as compared to individuals with chronic pain management impacts dramatically on the well being of affected than 20 years of age. For the purpose of this EDS type I and type III, the small sample individuals and, as a previous study study we relied on patients* reports of their size precludes meaningful statistical analysis. suggested, influences their social interactions. previously determined diagnosis. Fifteen of The “mean intensity” rating, calculated for each individual as the mean of the Methods Data were collected in semi– Table 1 structured interviews with an intensity ratings for those locations opportunistic sampling of individuals affected, was 5.3 for the total sample Sample Demographics drawn from either the University of and similar for all types. Forty–three Gender Number with Number (%) Age (Mean [range]) (Female/Male) chronic pain (%) (84%) individuals indicated that their Connecticut Heritable Disorders of Connective Tissue Clinic or attendants pain had become worse during the Total Sample 51 (100) 34.0 ± 12.7 [9 - 70] 42/9 46 (90.2) of the Annual Meeting of the Ehlers– course of their life (Table 2).

Type I 9 (17.6) 27.4 ± 14.1 [9 - 46] 7/2 6 (67.8) Danlos National Foundation held Type II 4 (7.8) 38.7 ± 25.6 [24 - 62] 3/1 4 (100) August 15–18, 1995 in Cincinnati.

Respondents identified a total of 13 Type III 28 (54.9) 36.5 ± 12.3 [14 - 70] 26/2 28 (100) Interviews were conducted by two of us principal locations of their pain, Type IV 7 (13.7) 33.9 ± 9.2 [15 - 42] 3/4 5 (71.4) (AS in the General Clinical Research ranging from 22 individuals (43%) JHS 1 (2.4) 24.0 1/0 1 (100) Center of the University of Connecticut noting elbow pain to 41 (80%) Unknown 2 (3.9) 26.5 ± 9.2 [20 - 33] 2/0 2 (100) Health Center in Farmington and JS in reporting pain in the shoulders at one JHS: Joint Hypermobility Syndrome Cincinnati). The length of interviews time or another. Similar numbers of ranged from 20 to 60 minutes and in individuals reported pain in their hands addition to recording age, sex, and the type of the participants were interviewed in (38, 75%), knees( 36, 71%), and spine (34, 67%). Interestingly, almost half of the EDS, three major topics were explored: Connecticut and the remainder were interviewed in Cincinnati. The patients patients reported frequent headaches and a 1) The experience of chronic pain was interviewed in Connecticut had been third had stomach aches. Approximately 70% previously evaluated by one of us (PT). described by a series of questions of all patients reported continuous pain in concerning intensity, quality, time of Twenty–eight (55%) of those interviewed their lower extremities, ankles, feet, toes, and stated that they were affected with EDS Type hips. The description of pain qualities onset and general progression for each III. Although there are no accurate figures suggested a pattern of significant distress, location identified (modeled on the described primarily as aching, sharp, McGill–Melzack Pain Questionnaire about the relative frequency of each EDS [Melzak, 1975]). Intensity was assessed type the previous number reflects the throbbing or burning. for each location on a 10 point analogue distribution observed by one of us (PT) in the clinic. The remaining 23 were distributed The locations noted and the time of onset scale with 0 = no pain and 10 = worst during the life cycle for the 45 adults are pain imaginable. Respondents were between Types I, II, IV and Joint Hypermobility Syndrome, a phenotypically shown in Figure I. Approximately 50% of asked to describe the quality of each similar disorder. Two individuals were pain with adjectives. A list of adjectives individuals reported onset of pain in most such as throbbing, burning, tingling, etc. affected with a form of EDS which could not locations in adulthood, although pain in shoulders, knees, ankles, feet and toes began was given. be typed. earlier. However, forty of the forty–five (89%) adults remembered chronic pain 2) Functional impacts of chronic pain were Nature of Pain beginning in at least one location with onset elicited with a 5 item checklist (sleep, in childhood or adolescence. sexual functioning, social relations, Forty–six of the 51 individuals interviewed physical activity, school or job indicated they had chronic pain over the last The percentages of individuals experiencing functioning).

6 months or longer (Table 1). Of the adults only two (2/45) felt they were free of chronic dysfunction in various areas are presented in Figure 2. Sleep and physical activity were 3) Strategies utilized to control pain were pain. most frequently noted (70%) with sexual elicited with an eight item checklist activity the least, although still substantial (medication, narcotics, physical therapy, Individuals’ reports of pain locations, heat/cold, massage, bandages/splits/ intensity, and course are summarized in Table (45%). Only 6 respondents (11.8%) noted no braces, exercises, other).

2. The mean number of pain locations for all dysfunction related to their pain.

51 respondents was 8.0. Although individuals The strategy for coding the data for purposes with EDS type IV seemed to have a Coping Strategies of descriptive analysis included Table 2 categorization of specific pain locations, Respondents identified more than 20 Chronic Pain Experience pain quality, coping strategies and onset major strategies for coping with pain.

Mean intensity of Number of locations Progressive periods (childhood, adolescence, Figure 3 shows the utilization (number locations affected Mean ± SD (range) Pain (%) adulthood).

Mean ± SD (range) who use or tried) of the 7 most frequent strategies and their efficacy for the total Total Sample 8.0 ± 3.3 (0 – 14) 5.3 ± 1.5 (1.0 – 8.6) 43 / 51 (84.3) Results sample. Additional strategies less Type I 8.8 ± 3.6 (1 – 12) 5.2 ± 3.6 (1.0 – 8.6) 7 / 9 (77.8) Sample Characteristics frequently noted included distraction, Type II 6.8 ± 3.3 (3 – 10) 4.3 ± 2.0 (3.2 – 8.0) 4 / 4 (100.0) diet, TENS, rest, water bed and pillow, Type III 8.9 ± 2.6 (5 – 14) 5.3 ± 1.4 (2.3 – 8.0) 24 / 26 (85.7) Demographics of the sample are craniosacral therapy, shiatsu, energy Type IV 4.3 ± 3.6 (0 – 11) 5.7 ± 1.1 (4.4 – 7.4) 5 / 7 (71.4) provided in Table 1. The majority (42/ work, smoking, and use of the 51) of those interviewed were women.

November, 1997 Ehlers–Danlos National Foundation — Page 7

Chronic Pain... from page 7 Alexander Technique. These were used in less than 5% of the cases. Eighty–eight percent of the respondents had taken pain medications, and 51% had taken narcotics.

an attempt to cope more effectively with day–to–day pain associated with this condition. The need to use multiple coping strategies suggests that no one approach is uniformly successful.

More investigation is clearly necessary to study the origin of pain and the efficacy of specific intervention in these individuals. In the interim, some basic principles of pain management could be extrapolated from other chronic diseases. Certainly a pain Discussion problem list should be a part of their medical record [Portenoy, A number of points emerge 1988]. The various types of pain clearly from the interviews of that individuals with EDS individuals with EDS. First, and experience may have different most striking, is the fact that origins, different intensities, and moderate to severe pain is a run different courses. A detailed common every day occurrence pain problem list will allow for a for essentially all of them. In more thorough understanding and addition, the pain associated with tracking of each type of pain and EDS starts early in life and intervention for it. A pain evolves over time. The assessment technique that is overwhelming majority of developmentally appropriate patients feel that their pain has should be taught to the patient and gotten worse. Despite these used routinely [Paige and Cioffi, observations, we were unable to 1993]. From the pharmacological find any articles reviewing the point of view, non–steroidal anti– management of pain associated inflammatory agents (NSAIDs) with EDS. would appear to have a major role, especially if the pain is of The pain problems associated Fig. 1 Onset and distribution of chronic pain in individuals 20 years of age and older (N=45). inflammatory origin. It should be with EDS are complex and not noted that the chronic use of these uniform. Most patients affected agents is frequently associated with with EDS have pain in several locations. gastrointestinal, renal, and hematologic The origins of these pains are probably consequences; therefore, they should be quite variable. Some pains are likely monitored if used chronically. Particular secondary to frequent dislocations/ caution should be exercised in individuals subluxations, some from repeated soft affected with EDS type IV who are tissue injury, or multiple surgical particularly prone to bruising and bleeding. operations and resultant nerve injury.

For moderate to severe pain, an NSAID in conjunction with a weak opioid usually in a Our limited sample size precludes statistical fixed combination is often used [Acute Pain analysis of differences in pain patterns Management Guideline Panel, 1992]. This associated with various types of EDS. It approach, in chronic non–malignant pain, is appears that the number of pain locations considered controversial [Turk, et al. 1994]. and pain intensity are similar across the Fig. 2 Impact of chronic pain on functioning (N=51) If used however, care should be taken so different types of EDS with the exception of that the NSAID and/or acetaminophen individuals affected with EDS type IV do not reach toxic levels in individuals who identified fewer pain locations.

Fig. 3 Strategies used or tried for coping with pain (N=51) who take large numbers of these pills This could reflect the relative paucity daily. In those instances, a separate of joint hypermobility in that EDS NSAID or acetaminophen can be type. given in conjunction with codeine or oxycodon. Opioids, either short acting This life–long history of discomfort or long acting, should be considered has compelled many people to for severe pain. They should be explore numerous coping strategies. monitored for side effects, in Most patients have taken some type particular constipation, which can of medication and over half the cause extreme discomfort. Therefore, sample have used opioids. A host of any individual on chronic opioids physical interventions were also used should take prophylactic laxatives. A by patients with EDS. Physical newer drug, tramadol (Ultramä), therapy has been tried by many, which is an opioid analgesic may be a particularly for pain in the shoulders, valuable alternative to opioids for spine, knees, and hips. Massage, use some individuals with EDS in chronic of heat or cold, and chiropractic pain because it lacks some of the side manipulation are frequently used in effects traditionally associated with opioids [Sunshine, 1992]. Finally, November, 1997 Ehlers–Danlos National Foundation — Page 8

tricyclic antidepressants may be of benefit.

These agents may help the patient sleep better at night and have some analgesic activity particularly against neuropathic pain.

Neuropathic pain arises from nerve injury and is often opioid resistant.

Non–pharmacologic approaches, such as physical therapy and exercise may be warranted but may also be quite traumatic and stressful for this group of patients. Physical therapy taking place in water (hydrotherapy), however, may be less damaging to joints and more enjoyable. Behavioral and cognitive coping techniques may also be extremely valuable. These include hypnosis, breathing, meditation, visual imagery, and other forms of distraction.

Obvious limitations of our study are the small sample size, the over–representation of women in our sample, the potential self– selection bias and lack of a control group.

These sampling issues stem from our reliance on the participants of the annual meeting of the Ehlers–Danlos National Foundation, our decision to utilize an interview approach and our inability to identify an appropriate age– matched control population. These limitations notwithstanding, our findings are congruent with our clinical experience.

In summary, our data reveals 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.

Extrapolation from the literature on other

diseases allows us a starting point from which to develop a clinical pain management algorithm. EDS should be considered in the differential diagnosis of chronic musculoskeletal pain. Clearly, further research is necessary to identify the most humane way to manage the devastating effects of this symptom in individuals with EDS.

Acknowledgments This paper is dedicated to the late Nancy H. Rogowski, the founder of the Ehlers–Danlos National Foundation whose selfless dedication to her cause touched the lives of many people. The authors are grateful to all individuals affected with Ehlers–Danlos syndrome who participated in this study. We are grateful to Dr. Richard J. Wenstrup, Cincinnati, for his comments. This work was supported in part by a General Clinical Research Center grant (NCRR–NIH MO1– RR–06192) to the University of Connecticut Health Center and also by the Ehlers–Danlos National Foundation.

References Acute Pain Management Guideline Panel (1992): Acute Pain Management: Operative or Medical Procedures and Trauma. AHCPR Pub. No. 92–0032. Rockville, MD: Agency for Health Care Policy and Research, Public Health Service, U.S. Department of Health and Human Services.

The EDNF is steadily growing and evolving as an organization. In an attempt to meet our ever increasing demands, the Foundation is in need of office equipment.

Below is our “Wish List” for specific items that we need, but aren’t in a position to purchase at this time.

If you either have the means or ability to donate this equipment, it would be greatly appreciated. Together... with YOUR help, we will find a brighter day.

w/ Windows 95, FAX/Modem & CD ROM

Beighton P. The Ehlers–Danlos syndromes.

In Beighton P (Ed): “McKusick’s Heritable Disorders of Connective Tissue, 5th edition.”

St. Louis: Mosby, 1993:189–257.

Lumley MA, Jordan M, Rubenstein R, Tsipouras P, Evans MI. Psychosocial functioning in the Ehlers–Danlos syndrome.

Am J Med Genet 1994;53:149–152.

Melzack R. McGill pain questionnaire: major properties and scoring methods. Pain 1975;1:277–299.

Paige D, Cioffi AM. Pain Assessment and Measurement. In RS Sinatra, AH Hord, B Ginsberg, LM Preble (eds) Acute Pain: Mechanisms and Management. St. Louis: Mosby–Yearbook, 1992.

Portenoy R. Practical aspects of pain control in cancer. CA 1988;38:327–52.

Sunshine A, Olson NZ, Zighelboim I, DeCastro A, Minn FL. Analgesic oral efficacy of tramadol hydrochloride in post– operative pain. Clin Pharmacol Ther 1992;51:740–746.

Turk DC, Brody MC, Okifuji EA. Physicians attitudes and practices regarding the long term prescribing of opioids for non–cancer Beighton P, de Paepe A, Danks D, Finidori G, pain. Pain 1994;59:201–208.

Gedde–Dahl T, Goodman R, Hall JG, Hollister DW, Horton W, McKusick VA, Opitz JM, Pope FM, Pyeritz RE, Rimoin DL,

Wish List

Pentium PC

Sillence D, Spranger JW, Thompson E, Tsipouras P, Viljoen D, Winship I, Young I. International nosology of heritable disorders of connective tissue, Berlin, 1986. Am J Med Genet 1988;29:581–594.

Two Plain Paper FAXs,

Your donation is tax deductible and is sincerely appreciated.

Some thoughts about EDS

by Harm Lok (Netherlands) Breaking down my body, loosing joint after joint, It never can affect my belief and take away my dreams about walking over mountains, and flying though the sky When I close my eyes, my world is the same as yours.

Thinking over a world without struggle in any way.

Even my body is not perfect, when pain is heavy, I always look to bright sides around.

And when seemed to disappear, I close my eyes, and when I open my eyes I know: Life, even with EDS is valuable to live.

Harm

November, 1997 Ehlers–Danlos National Foundation — Page 9

Learning To Manage Fibromyalgia Syndrome Darlene A. Clarke, R.N., M.S.N., Chair, Board of Directors; President & Founder of the Northeast Ohio Branch of the EDNF. Darlene has Hypermobility Type EDS and Fibromyalgia Syndrome.

Fibromyalgia can run in families, possibly suggesting an inherited predisposition. To date, the cause of Fibromyalgia is not known.

It may lie dormant until it is triggered by an injury/trauma, stress, or a sleep disturbance.

There are other theories as to what triggers Fibromyalgia. One theory is that the syndrome is caused by an infectious agent such as the influenza virus. Other suspected causes include extreme physical deconditioning of the nervous system brought on by a lack of exercise, and changes in muscle metabolism that can result in decreased blood flow to the muscles with fatigue and decreased strength the end results.4 More recent research points It’s morning and as you awake you realize you to two key chemicals of the Central Nervous are exhausted and you haven’t even left the System that facilitate the regulation of pain bed yet. The thought crosses your mind that messages transmitted to our brain. These two for as tired as you are, you may as well have chemicals are substance P and serotonin. never gone to bed. You barely have enough Substance P begins the pain–signal process energy to get out of bed and care for yourself, following tissue injury. Serotonin reduces the let alone being able to care for the children, or intensity of pain signals in the transmission of go to work or school. As you struggle to get pain and is very important in sleep regulation. out of bed, you realize that your body aches Low levels of serotonin and elevated levels of all over and you feel as if you are a 100 years substance P have been found in individuals old. You are stiff and can hardly move. What with Fibromyalgia. These findings support the is wrong with you? After seeking one or more theories that individuals with Fibromyalgia medical evaluations, you finally get the have unusually high pain intensity messages answer. Your physician informs you that you transmitted to their brain as well as have Fibromyalgia syndrome. Such a big word deficiencies in the inhibition of pain.5 One that sounds ominous and must mean you have clear fact remains: more research is needed on some dreaded problem. You have never even Fibromyalgia. Despite not being able to heard the term before. So, what is Fibromyalgia identify the exact cause of Fibromyalgia, syndrome? individuals present with similar signs and symptoms.

Fibromyalgia syndrome is a common form of chronic, generalized muscular pain and Signs and Symptoms fatigue. In some cases, the pain is so intense that it can be incapacitating.1 Despite that Probably the most frustrating aspect of having Fibromyalgia does not result in serious, long– Fibromyalgia syndrome, as in EDS, is that you term target organ damage, it does have a “look healthy.” Compounded by the fact that negative impact on the quality of life similar test results are “normal,” it is difficult to to that of rheumatoid arthritis.2 To effectively convince others that something is wrong with manage and cope with Fibromyalgia you. Every X–ray and blood test your syndrome, it is imperative that one has a clear physician ordered has come back within understanding of Fibromyalgia, its signs and normal limits. You embark down the path of symptoms, triggering factors and how it is self–doubt and frustration – is this pain real, treated and managed. or is it all in my head? Why is it so difficult to put a label to the signs and symptoms of The term “Fibromyalgia” can be broken down Fibromyalgia? into fibro (fibros tissue), my (muscles), and algia (pain and tenderness). In Fibromyalgia Fibromyalgia is frequently misunderstood and there is pain in the muscles and fibrous confusing because the symptoms seen in this connective tissues which comprise ligaments syndrome are also found in other conditions and tendons. Therefore, while Fibromyalgia such as rheumatoid arthritis and lupus. feels like a joint disease, it affects muscles and Women are affected more than men and the their attachments to bone and joint deformity does not ensue.3 Individuals with EDS are often also diagnosed with Fibromyalgia syndrome. Fibromyalgia is a common and often disabling disorder whose cause remains obscure. Fibromyalgia frequently resembles other disorders such as rheumatoid arthritis and lupus. Individuals commonly complain of widespread muscle pain and tenderness, as well as fatigue and exhaustion after minimal exertion. A characteristically high “tender point” is evident. For individuals diagnosed with Fibromyalgia, learning to effectively cope with this disorder is essential to one’s emotional and physical health.

symptoms usually occur first when an individual is between 20 and 40 years old.1 However, it must be noted that Fibromyalgia can sometimes affect children and the elderly.4 Recent research studies have established guidelines for diagnosing Fibromyalgia based on the presence of certain signs and symptoms.

Pain The most common complaint from patients with Fibromyalgia is that they have diffuse “pain all over” – almost like the flu. Although in rarer cases, the pain is quite severe and disabling. More often, the symptoms often begin insidiously and may wax and wane in severity. In almost all cases, individuals complain of some degree of pain continuously. The pain is most often worse at “tender points” specifically located on the body. Fibromyalgia type pain generally is worse in the morning and late evening and often is associated with stiffness. Muscle groups that are used repetitively result in an increase in the level of pain you experience.

Fatigue and Sleep Disturbances Have you ever awoke after sleeping and felt refreshed and rejuvenated? Most likely your answer would be “no.” Immense fatigue is frequently the debilitating aspect of Fibromyalgia. The fatigue may be exhibited as an overall tiredness and lack of energy, or as muscular fatigue and a lack of endurance. In either case, it can be difficult to perform activities of daily living such as caring for self or others, performing household chores, going shopping, or to function effectively at work.

Keep in mind that it takes a tremendous amount of energy to deal with having chronic pain and this too can contribute to an individual’s fatigue.

Approximately 90% of individuals with Fibromyalgia complain of moderate to severe fatigue coupled with a lack of energy or the type of exhaustion that is found with a lack of sleep. In most cases, individuals wake up feeling tired even after sleeping all night.

While their minds may be rested, their bodies feel as if they never went to sleep. Many are aware that their sleep has become lighter with frequent awakenings throughout the night.

Scientific studies reveal that individuals with Fibromyalgia have abnormal sleep patterns Continued

Views expressed herein are only those of the authors, and should not be construed to represent the opinions or policies of the Ehlers–Danlos National Foundation and it’s elected officials.

November, 1997 Ehlers–Danlos National Foundation — Page 10

Ergonomics also plays an important role at home. It’s Low Cervical: often difficult for someone bilateral at C5 – C7 with Fibromyalgia to push a vacuum cleaner, wash walls, Trapezius: Second Rib: paint or prepare meals. These bilateral at the bilateral at the midpoint of the second tasks require the use of upper border cosochondral shoulder muscles to maintain junctions arms in extension at some Supraspinatus: level in front of our body. bilateral above the Greater What generally happens is scapular spine trochanter: Central Nervous System near the medial bilateral, that an ache begins in the Symptoms border posterior to the neck, shoulders and upper trochanteric As in other chronic illnesses, prominence back. The ache rapidly turns Gluteal: bilateral mood and mental changes into pain that elevates in upper outer Lateral quandrants of can occur in individuals with intensity as it slowly radiates Knees: bilateral epicondyle: buttocks in Fibromyalgia. Many at the medial fat bilateral, 2 cm up and down the back. You anterior fold of pad proximal to distal to the individuals feel “blue” or muscle feel as if you can’t maintain the joint line epicondyles “down,” although only about your arms in this position 25% are clinically depressed. any longer, and you quickly In most cases, depression return your arms to your and/or anxiety tends to sides as you wait for the pain follow the onset of time your physician palpated these 18 “tender to slowly ebb away. Most, if not all of you, Fibromyalgia symptoms and may be the result points.” Did you say “ouch,” flinch, make a can relate to this all too familiar type of pain. of Fibromyalgia and not the cause of it.3 facial grimace, attempt to withdraw away from your doctor or even jump? These are the Emotional Factors People with Fibromyalgia may also experience usual responses elicited by an individual with numbness and tingling in their hands, arms, Fibromyalgia. Frequently, patients are shocked Stress is something that each of us has to deal face, feet and legs. These symptoms can also with on a daily basis. Unfortunately, by the level of pain they experience when be found in other disorders and usually require these areas are palpated. Now that you know emotional stress is a prominent factor numerous tests before the diagnosis of aggravating Fibromyalgia. We are faced with how Fibromyalgia is diagnosed, let’s look at Fibromyalgia is reached by a physician. stressful situations at home, work, school, the common triggering factors of driving your car on the highway etc. It isn’t Fibromyalgia.

Other Problems difficult to identify situations that aggravate your Fibromyalgia symptoms – that’s the easy Common Triggers of Muscular as well as migraine headaches are part of the equation. The trick is to avoid or Fibromyalgia common in Fibromyalgia. Abdominal attempt to modify any stressful situation.

Three common factors seem to trigger a flare bloating, pain, alternating diarrhea and in Fibromyalgia symptoms, physical factors, constipation are also commonly seen. They It is extremely difficult to deal with those resemble irritable bowel syndrome or “spastic emotional factors and environmental factors. situations in which any stressor is unrelenting.

Keep in mind that your Fibromyalgia can colon” in nature. Similar bladder spasms and These types of situations can present become aggravated by these factors alone or irritability results in urinary urgency and themselves in the home with family members frequency. The skin and circulatory system are any combination of these factors. or in the workplace with coworkers or sensitive to moisture and temperature changes supervisors. In these situations, one must look resulting in temporary changes in skin color.2,3 Physical Factors at not only the circumstance, but how you

and are deficient in Stage 4 sleep – the deepest stage.1,3,5 It is important to note that a secondary Fibromyalgia syndrome can occur with any connective tissue disease (EDS included) and may not necessarily be related to sleep disturbances.6

Figure 1

Occiput: bilateral at the suboccipital muscle insertions

Several physical factors can aggravate your Fibromyalgia. Infections are one type of physical factor that can produce the most Currently, no definitive laboratory tests exist profound exacerbation of Fibromyalgia to make the diagnosis of Fibromyalgia. Rather, symptoms. The second type of physical the diagnosis is made by a physician after factors deal with ergonomics. The positions obtaining the individual’s medical history and we maintain our bodies in has a direct performing a complete physical examination. correlation to the pain associated in Fibromyalgia. For instance, do you have a job A hallmark of Fibromyalgia syndrome is that requires you use repetitive movements reduced pain thresholds at designated tender all day? Are you required to work in one spot 2 points, demonstrated upon palpation. with your arms elevated above your work According to the American College of surface? Do you need to remain in one Rheumatology, the diagnostic criteria for Fibromyalgia includes: a). widespread diffuse position (i.e., standing, sitting or driving) for prolonged amounts time? If you answered pain that has been present for at least three months and b). pain that is present in 11 of the “yes” to any of the above questions, you will be able to relate to the pain that results from 18 bilateral tender point sites (see figure 1) maintaining your muscles in a sustained when 4 kg of force is applied by digital isometric position for any length of time.

1,2,7 palpation. Recall your response the first

Clinical Diagnosis

react to the stressor. Ultimately, it comes down to dividing these stressful situations into two categories: those that you have control over and those you have absolutely no control over.

In circumstances that you have no control over, it is imperative that you learn to alter your response to those particular situations.

Save your energy for those battles that you can emerge victorious.

Environmental Factors How many times have you been able to predict the approaching weather without having looked at a weather map? Probably too numerous to count. You feel like you are a human barometer with the ability to predict weather changes before they occur. Most

November, 1997 Ehlers–Danlos National Foundation — Page 11

Continued

likely you have found that when the barometric pressure drops, it is followed by either rain, cold, snow, and fog – all of which aggravate your symptoms.

Many individuals with Fibromyalgia report that certain types of artificial lighting, cold drafts or irritating noises often result in a flare of their muscle pain. When was the last time that you were able to enter a pool that wasn’t heated? Just a guess, but it was probably many years ago. Most individuals with Fibromyalgia do not tolerate swimming in cold or cool water. We are usually found in the heated pools that others say are “too hot to swim in” or in hot tubs. The goal is to determine what factors aggravate your Fibromyalgia and either eliminate them if possible or at least modify them. Now that you know what factors can trigger your Fibromyalgia, let’s look at how it is treated and managed.

medications such as acetaminophen, aspirin or ibuprofen to relieve pain and reduce stiffness.

If you are currently on a nonsteroidal anti– inflammatory drug to help manage your EDS, your physician will likely instruct you to take acetaminophen, not ibuprofen or aspirin, to control your pain. Narcotics, cortisone derivatives and tranquilizers are avoided because of their ineffectiveness and potential side–effects.2,3,7,8 To improve Stage 4 sleep, your physician may prescribe low doses of tricyclic antidepressants (Elavil, Sinequan) or the muscle relaxant cyclobenzaprine (Flexeril).

These drugs are structurally similar and they block the re–uptake of the neurotransmitters that regulate deep Stage 4 sleep.3,8 These medications carry the risk of potential side– effects which include: drowsiness, weight gain, dry mouth and constipation to name a few. Do not be discouraged if you do not respond to the first medication(s) prescribed.

Your doctor may need to adjust dosages or change drugs to achieve an improvement.

Always practice proper body mechanics and apply ergonomic principles at home, work and at rest. These techniques will prevent injury and reduce flare–ups of Fibromyalgia symptoms.

Your doctor may prescribe various physical therapy treatments. Possible treatments include: massage, heat or ice applications, ultrasound or whirlpool therapy and microvoltage electrical stimulation. These are all passive physical therapy techniques with varying results from individual to individual.

These treatments are usually prescribed for flare–ups and are employed for short periods of time.3,8

Taking Control of Fibromyalgia

You can engage in several actions that will help you gain control of your Fibromyalgia.

First and foremost, listen to your body.

Identify those triggers that aggravate your Treatment and Management symptoms and then avoid or at least minimize Since Fibromyalgia’s definitive cause is not them. Get your stress level under control by known, current treatments are focused on practicing relaxation techniques. Relaxation relieving painful symptoms, not necessarily techniques are primarily effective for chronic Individuals with Fibromyalgia should practice curing them. Additionally, the treatment good sleep hygiene to promote sleep. Bedtime pain and have many benefits. Relaxation program prescribed by your physician will be rituals can relax you in preparation of sleep. techniques enable you to reduce anxiety, pain individualized to address your specific needs. These bedtime rituals can include: taking a and stress, reduce muscle tension pain and relieve the helplessness and depression warm bath or shower, reading a novel, Numerous treatments have been employed associated with chronic pain. Despite having watching a relaxing television program or over the years for Fibromyalgia. No one listening to soothing music which can promote chronic pain and fatigue, learn to develop a method has been entirely successful, but positive attitude. Remember that if you have a sleep. Attempt to follow a regular sleep several methods will be prescribed by your constant negative attitude, your Fibromyalgia schedule and go to bed at the same time each physician in an attempt to control your day. Sleep eight hours nightly if possible. Avoid symptoms will exacerbate. Look for the good symptoms. A sense of overwhelming relief in every situation – it’s there, but you may alcohol, caffeine and tobacco before retiring – comes with the validation of your symptoms need to look closely. they will disturb your sleep. Also abstain from and a diagnosis. Just knowing the name of taking midday naps. your condition will not be enough though.

Eat healthy and avoid fat in your diet. Excess Your physician and healthcare team will weight is detrimental to your body and can Exercise is a key element in Fibromyalgia educate you and your family about management. Your physician will recommend aggravate your symptoms. Eating properly Fibromyalgia. Specifically, you’ll be informed that you slowly increase your aerobic fitness also maintains your health and avoids illness. about your prognosis, possible causative Keep in mind that an illness (i.e., cold, flu, with structured, low impact activities such as factors, the aspects of treatment and specific swimming, water aerobics, walking, or riding bacterial infection etc.) can be a triggering body mechanics techniques. This education factor for your symptoms. a stationary bicycle.2,3,7,8 Research has will prove invaluable to you. If you are taught indicated that aerobic exercise improves sleep, Educate yourself, family and healthcare about what to expect and how to deal with enhances endorphin release and has providers about Fibromyalgia syndrome. flare–ups, you will be able to control your antidepressant effects.2 It is important to Remember that knowledge is a powerful Fibromyalgia. The goal of education is to check with your physician to ensure that the weapon in coping with any chronic illness. moderate the effects of Fibromyalgia on your exercise program you have chosen is not life. Only then will Fibromyalgia not become contraindicated in your specific type of EDS. Knowledge also helps you to take control of your Fibromyalgia and reduce the your entire life.

Remember to always stretch your muscles exacerbation of its symptoms. before engaging in any aerobic activity.

Medications may be prescribed by your Increase your exercise program gradually to Lastly, seek support from others with physician to control pain and improve deep reduce the chance of injury. As you continue Fibromyalgia. Check with your physician, Stage 4 sleep. If pain is prominent, your to exercise, you’ll notice that you feel better, healthcare institution, and within your local doctor may prescribe a low dose of an have more endurance and experience a community for a Fibromyalgia support group. analgesic or nonsteroidal anti–inflammatory reduction in Fibromyalgia symptoms. It’s It is extremely beneficial to receive support drug, although most nonsteroidal anti– worth the possible pain and fatigue felt and understanding from others who have the inflammatory drugs are marginally beneficial initially to achieve these end–results. same type of problems from Fibromyalgia as and carry the risk of side–effects that you do. Connecting with a support group also outweigh their benefits. Therefore, you will be most likely instructed to use over the counter Continued November, 1997 Ehlers–Danlos National Foundation — Page 12

helps you and your family to cope with having a chronic illness. And who knows, maybe someday you will have the opportunity to offer support to someone else just diagnosed with Fibromyalgia. I guarantee it will be one of the most gratifying and fulfilling experiences of your life.

Conclusion Receiving a diagnosis of Fibromyalgia syndrome is an important first step in managing this condition. It is not the last step though! It is imperative that you have a clear understanding of Fibromyalgia, its signs and symptoms, triggering factors and treatments.

Learning to effectively cope with this disorder is essential to one’s emotional and physical health. It is only then that you will be able to manage your Fibromyalgia and prevent it from becoming your whole life.

While there isn’t a cure for Fibromyalgia, there are treatments that are effective in managing its symptoms. There is life after finding out you have Fibromyalgia.

Tomorrow will be better and holds the possibility of a cure!

References 1

Fan, P.T., & Blanton, M.E. (1992). Clinical features and diagnosis of Fibromyalgia.

Journal of Musculoskeletal Medicine, 9(4), 24–42.

2

Wilke, W.S. (1996). Fibromyalgia: Recognizing and addressing the multiple interrelated factors. Postgraduate Medicine, 100(1), 153–170.

3

Arthritis Foundation, Inc. (1995) Fibromyalgia Syndrome, 1–12.

Darlene A. Clarke, R.N., M.S.N., Chair, Branch Bylaws Committee Bylaws Committee Members: Kim Christensen, Co–President, New York City Branch; Darlene Clarke, President, Northeast Ohio Branch; Peggy Rocha Snuggs, President, Tampa Bay Area Branch; Lynn Sanders, President, Milwaukee Branch; Maggie Buckley, President, San Francisco Bay Area Branch; Shari Gamson, Southern California Branch, Linda Neumann–Potash, Executive Director; & Harold Goldstein, Director, Local Branch Organization.

The Bylaws Committee completed our draft of standardized Bylaws during our August 17th meeting. We met one final time on September 9th to ensure accuracy of our draft. We then had the Bylaws reviewed again by Brian Glick, who is an attorney.

Brian ensured that the Bylaws as written, did not jeopardize the tax–exempt status of the EDNF and its subsequent Branches.

Finally, the Branch Bylaws draft was reviewed at the September 26th Board of Directors meeting. I am pleased to announce that the Bylaws were unanimously adopted by the Board. All chartered Branches will be receiving their copy of the Bylaws. When a new Branch is chartered, they will receive a copy of the Bylaws. More than likely, you will need to

submit a copy of these Bylaws to the appropriate governmental agency in your state when you register as a charitable, not– for–profit organization.

I want to personally commend all of the Committee members who willingly gave so much of their time, energy and expertise to the development of the standardized Branch Bylaws. Each member had a unique perspective, thereby ensuring that each Branch could easily adopt the final set of Bylaws. The blending of the skills and talents of the Committee members enabled us to achieve our goals for the completion and adoption of the Bylaws. This truly was a great group of individuals to work with on this project. On behalf of the Board of Directors, I would also like to take this opportunity to extend a special thank–you to Brian Glick. Without his expertise, input and guidance, we would not have been able to complete this project as quickly as we did.

The Committee was extremely grateful that Brian was always there when we needed clarification on an article or to answer our endless questions (both of which were often). To the entire Committee and to Brian Glick, I would like to say that we couldn’t have done it without each of you!

— Membership Dues —

4

Dunkin, M.A. (1993, September–October).

Fibromyalgia: Out of the Closet. Arthritis Today, pp. 24–28.

5

Dunkin, M.A. (1997, September–October).

Fibromyalgia: Syndrome of the ’90s. Arthritis Today, pp. 41–47.

6

Ignatavicius, D.D., Workman, M.L., & Mishler, M.A. (1995). Medical–Surgical Nursing (2nd ed.). Philadelphia: W.B. Saunders Company.

7

Goldberg, D.L. (1990) Fibromyalgia and chronic fatigue syndrome: Are they the same?

Journal of Musculoskeletal Medicine, 7(5), 19–28.

8

EDNF Branch Bylaws Committee Update

Sherman, C. (1992) Managing fibromyalgia with exercise. The Physician and Sports Medicine, 20(10), 166–172.

Each of you is personally aware that the cost of almost everything continues to escalate.

This holds true for the EDNF and its services. Therefore, in order to meet the current needs of our members and to ensure the continued services of the Foundation, the EDNF membership dues will reflect an increase that will be effective on January 1, 1998. The increase will be as follows: Individual Membership .................... $25.00 Family Membership ......................... $30.00 Canada & Mexico: Individual Membership .................... $30.00 Family Membership ......................... $35.00 International: Individual Membership .................... $35.00 Family Membership ......................... $40.00

The EDNF is now equipped to accept credit card payment for membership dues. This will be particularly helpful to our international members.

The decision to increase membership dues was not made lightly. The cost of printing, shipping, and postage to name only a few, have increased dramatically in the past several years. Currently, the membership dues do not even cover the expense of publishing, shipping and mailing Loose Connections on a quarterly basis to you. Please keep in mind that membership dues have not been increased in 8 years! We realize that some members will not have the economic resources to maintain their membership. If this is your case, please notify the Executive Director in writing and accommodations will be made. Thank–you in advance for your understanding in this matter.

November, 1997 Ehlers–Danlos National Foundation — Page 13

Guidelines to Help Select a Pain Unit Reprinted with permission from the American Chronic Pain Association Many who suffer from chronic pain have been thorough a number of different treatments. They have tried physical therapy, biofeedback, surgery, medications, counseling, and other treatment modalities without much success. They find that the pain affects virtually every aspect of their everyday lives: work, home, school, and social activities are all controlled by the pain.

If you want to regain control of your life, it is important to learn how to cope with chronic pain. Although your pain may never go away, it is possible to reduce pain levels and, more importantly improve quality of life. To do so, you may need a multidiscplinary approach to chronic pain. While many of you have tried almost every available medical intervention without great success, sometimes these therapies are most effective when performed together in a controlled setting. To successfully regain control of your life, you must have all the necessary ingredients of pain management in the right quality. It is possible to live with chronic pain.

A multidiscplinary pain program can provide you with the necessary skills, medical intervention, and direction to effectively cope with chronic pain. The following information will tell you how to go about locating a pain management program in your area, what to look for in a well defined pain program, and what other issues to consider.

Learning something about the people who run the program: Try to meet several of the staff members to get a sense of the people you will be dealing with while on the unit.

The program should have complete medical staff trained in pain management techniques including: Physician (may be a neurologist, psychiatrist, physiatrist, or anesthesiologist but should have expertise in pain management).

Registered Nurse, Psychiatrist or psychologist.

Physical Therapist and Occupational Therapist.

Biofeedback Therapist and Family Counselor.

Vocational Counselor.

Personnel trained in pain management intervention.

Make sure the program includes most of the following features: Biofeedback Group Therapy Counseling Occupational Therapy Family Counseling Assertiveness Training TENS Units Regional anesthesia (nerve blocks) Physical Therapy (exercise and body mechanics) Relaxation Training & Stress Management Educational Program Covering Medications & Other Aspects of Pain & its Management Aftercare (follow–up support once you have left the unit)

Multidisciplinary Pain Management Units Make sure you locate a legitimate program.

Hospitals and rehabilitation centers are most likely to offer comprehensive treatment than are “stand alone” programs.

Facilities that offer pain management should include several specific components listed below: The Commission on Accreditation of Rehabilitation Facilities (telephone: (800) 444–8991) can provide you with a listing of accredited pain programs in your area (your health insurance may require that the unit be CARF accredited in order for you to receive reimbursement). You can call the American Pain Society, a group of health care providers, at (847) 375-4715 for additional information about pain units in your area.

Choose a good program that is convenient for you and your family: Most pain management programs are part of a hospital or rehabilitation center. The program should be housed in a separate unit designed for pain management.

Many pain management programs do not offer inpatient care. Choosing a program close to your home will enable you to commute to the program each day.

• • •

•

• • •

•

Be sure your family can be involved in your care: Family members should be required to be involved in your treatment.

The program should provide special education sessions for family members.

Joint counseling for you and your family should also be available.

Also consider these additional factors: What services will your insurance company reimburse, and what will you be expected to cover?

What is the unit’s physical setup (it is in a patient care area or in an area by itself)?

What is the program’s length of stay?

Is the program inpatient or outpatient (when going through medication detoxification, inpatient is recommended) If you choose an out–of–town unit, can your family be involved in your care?

Do you understand what is required of you during your stay (length of time you will be on the unit, responsibility to take care of personal needs, etc.)?

• Does the unit provide any type of job retraining?

• Make sure that, before accepting you, the unit reviews your previous medical record and gives you a complete physical evaluation to be sure you can participate in the program.

• Your personal physician can refer you to the unit, but many programs also accept self referral.

• Obtain copies of your recent medical records to prevent duplicate testing.

• Before you enter the unit, check with your insurance company to see what type of benefits it provides for pain management.

• Try to talk with both present and past program participants to get their feedback about their stay on the unit.

Pain management can make a significant difference in your life; however, you must realize that much of what you gain from your stay will be up to you. Treatment is designed to help you get out of the patient role and back to being a person. The program should help to restore your ability to function and to enjoy life. It will be up to you to become actively involved in the program if you expect to regain control of your life. Pain programs are difficult, but the benefits can improve your life–styles!

If you need further information, please call the American Chronic Pain Association’s national office at (916) 632–0922.

•

Editors Note: Since this article was originally published, more information about organizations mentioned has changed or been updated.

American Chronic Pain Association P.O. 850 Rocklin, CA, 95677 American Pain Society 4700 W. Lake Avenue Glenview, IL 60025 (Tel) 847/375-4715 (Fax) 847/375-4777 (E-mail) info@ampainsoc.org (Website) http://www.ampainsoc.org/ The Commission on Accreditation of Rehabilitation Facilities 891 E. Grant Road Tucson, AZ 85712 (520) 325-1044 Voice/TDD FAX (520) 318-1129 (E-mail) webmaster@carf.org (Website) http://www.carf.org/

November, 1997 Ehlers–Danlos National Foundation — Page 14

Letters To The Editor Dear Editor:

Dear Editor,

I have a need to get feedback from others on successes and failures with repair and reconstructions of ligaments in the knee. I have a very complicated problem with my knee. I have deteriorated cartilage, a torn meniscus, stretched anterior crucial ligament and a torn posterior crucial ligament along with chronic quadriceps weakness due to the tear. I have been advised that there is no successful surgery to date for the posterior cruciate ligament that would stabilize my knee, especially given the fact that I have EDS. I am wearing an excellent brace which does help but is becoming quite confining and aggravating when I am constantly being stopped by the general public, friend and foe, to talk with me about my knee which can’t simply be explained only to then hear about their knee problems from a skiing accident of the like. The knee brace is not helping as much as it used to either and I just feel there has to be something that can be done to help the pain and to stabilize it with surgery. If there is anyone in the foundation who has had a similar problem and tried some solutions down the surgery avenue, could you please write in to respond on the outcome, who did your surgery, and the method of repair. I really appreciate the wealth of information that is learned from the newsletter and other’s input.

In the issue Loose Connections dated August 1997 in the letters to the editor section on page 14, there was a mother asking for help for her daughter who has bruises on her shins and knees. My son and I have the same problem. I have found a product it’s called DERMABLEND. It’s a cream that comes in all kinds of shades.

You can find the shade which matches the color of your skin to cover up the bruises. During the summer when the color of your skin gets darker from the sun, you can get a little darker shade. Demablend is waterproof. It is sold at Macy’s or other fine stores at cosmetic counters.

Sometimes drugstores carry it also. The cost is about $12.00.

Thanks for your help.

K.K. , St. Louis, MO Dear Loose Connections, My name is Laurie and I’m writing to you about my son Joe. He is 2 & 1/2 years old, has a head full of blond curls and loves baseball.

He is thought to have a type of collagen disorder and although no one can put a name to it several have mentioned Ehlers–Danlos syndrome VII. He has very flexible joints, saggy skin on his face and arms, normal bruising, normal wound healing and has been in a cast or a brace for dislocated hips since he was 3 months old and now I’ve been told that it is unlikely that any additional improvement of his acetabulum will occur. Surgery has been suggested to improve the size and shape of his acetabulum in order to promote continued hip growth.

My question is, are there any other ED patients out there that have similar stories about hip dislocation and acetabular deformities?

Our surgeon has one other experience with E.D. patients. Are there any special risks? Any precautions to be taken? Are the outcomes good? Is there a specialist in the U.S. for E.D. patients with orthopaedic problems?

If you have any information that could be helpful, please e–mail me at Meehane@HiWAAY.net or write to: Laurie B. Meehan 802 Lenlock Dr. Huntsville AL 35802 I’m scared. I want to give my son a chance to have a normal life without braces, but I don’t want to take away his ability to make a homerun, even if he wobbles in to home plate.

Thank you.

When I was a kid I struggled with the same “ashamed” of my legs problem. I didn’t want it to show anymore. And you are right, the world is so hard for anyone who looks different than the “normal” people do. So when I found this product I felt free for the first time in many years. I am now 37 years old and have come to the point that I don’t care as much anymore that people stare or that people say something about my legs. But everyday is still a struggle. Now my son who is 7 years old has to go through this same problem; that he cannot do certain activities and that people stare at him because of the bruises on his legs. I haven’t applied the Dermablemd on his legs yet. I will only do this for him if he asks for it. I don’t want him to feel ashamed having those bruises.

I really hope this product will help your daughter to find some freedom on those hot summer days so she can be a kid again.

Thank you, Dineke Glasser

Special thank you to the following individuals and organizations for their fundraising activities on behalf of EDNF: Evening Star Grandmother’s Club Charter #1021, Brooklyn, NY Executive Call Center, Annual Employee Outing, Manchester, NH Susan Dion, Life Jam ’97, Huntington, MA Tampa Bay Branch, 2nd Annual Golf Tournament

Special thank you to the following organizations for their support with Loose Connections and the Web Site: Leigh at St. Genesis (Internet Services) Sue at ExchangeNet (Internet Services) Jeff at Cowgill Printing Joan and Alan at J. P. Graphics Gary and Rick at Northern Ohio Printing Jim and Debbie at Premier Impressions

Views expressed herein are only those of the authors, and should not be construed to represent the opinions or policies of the Ehlers–Danlos National Foundation and it’s elected officials.

November, 1997 Ehlers–Danlos National Foundation — Page 15

— Memorials and Honorariums — In Memory of Jordan David Specht David and Diane Specht and Family Specht Reality, Inc. Paul D. Akins III Auto Sales Todd and Teresa Alderfer Arthur and Beverly Altemose Catherine Balsavage Barnet Bank – South Cape Coral The Basile Corporation Adam and Pauline Benfield Berks County Bank Berks County Legion League Bethlehm and Pennllyn Pikes William and Margaret Bickel Birdsboro American Legion Sr. Babe Ruth Coaches Parents Assoc.

Janet and James Borgman Boyertown Midget Mite Football Assoc., Inc. Brumbach Associates, Inc. Cassel–Miller Real Estate, Inc. Central Montgomery County Assoc. of Realtors, Inc. Century 21 Harris and Henry A.L. Coffman & Sons Barbara and James Corum, Jr. Mary S. Cressman Anita Curtis James and Regina Custer Custom Cut, Gary Martin Lewin and Daryl Deery Richard and Shannon Delp Edward and Tierra Dobry Mrs. Thelam Douglas ERA Philadelphia Broker Council Mr. & Mrs. William Filler John Gemberling Ellen and Herbert Gieseler Gilbertsville Elementary School Robert and Jean Grim John Hagmann Tammy Hoffman Insurance Innovators, Inc. Christine and John Jones Robert and Joyce Jones Mildred S. Kelley Brian Kelly Mary and Frank Kolarz Emilie S. Kurtz Ronald and Phyliis Leidy Patricia Leinbach Kerry and Lesley Leister George I. Lent Gregory and Gail Levengood Raymond and Janet Leyfret, Jr. Joan A. Lucidi

Walter and Jean MacFarland III William and Brenda Marion Edward and Shirley Martin Mark and Loreen Martin Terry and Damon Meadows David and Donna Minner Victoria Morici Robert and Ingrid Moses Lois A. Motko Dorothy L. Moyer Patricia Moyer National Penn Bancshares, Inc. Sharon O’Melia Howard Michael and Gail Ondo Optimist Club of Boyertown Owl’s Sports Club Jesse and Barbara Painter Panos Papantoniou Doris Parrish Vincent and Laura Pettine Timothy and Stephanie Phillips Phoenixville Federal Savings Professional Pharmacy & Convalescent Products, Inc. The Prudential Select Properties The Realty Group, Inc. Oliver and Shirley Reed Frances and Emil Reh Melvin and Shirley Reitnour Sue Richard Gerald and Elaine Richards John and Nancy Rinker, Jr John and Susan Ruthkowski James and Paula Scheffey Mrs. Barbara Schreiber and Family John Sheridan Jr. Thomas and Trudy Siak Fancis and Anthony Shreves Donaled Smale Mark and Lisa Smith Richard and Eloise Smith Royal Spatz Donald and Sheila Specht John Stetler Bev Tornetta Tornetta Realty Corp Walace and Mary Van Dyke Joseph and Linda Voytilla Louise Wagner Norma and Edgar Walls, Jr. Vicki Waycock Wayne and Kathleen Weidner William and Patricia Wilkins Christopher and Carlyn Yerkes Norman and Betty Yocom Larry and Sandra Zentz

"LIFE"

by David Daniel Bowen

In Memory of Trevor Mursch

His name tells how it is for him He wonders, whether he should go on But he feels so strong on reaching his goal

Mr. & Mrs. Curtis Liles, III Donalyn Hernandez

In Memory of Eric J. Espinosa Dr. Susan Ristow

There are many things that happen to him but he just pushes on The goal is like a shining light, that is blinding him The only way of stopping the light is to reach the goal

Wishing a Speedy Recovery to Dr. Michael Goodman Joan and Herbert Majower Selma and Samuel Gordon

In Memory of My Family Members who had EDS Type IV; Something happens and he thinks Judith Simmons Dion, about stopping Lisa Dion Pickford, but the way is Michelle Dion, and now drawn for him Roger Pickford, Jr. And he knows after getting through this has helped him see the light even brighter now

Susan E. Dion

In Memory of Robert Berliner

The way he feels now he can do

Shirley Burkom

"Anything"

In Memory of Anna Schneider

June 7th, 1996 David died July 8th,1996

Josef F. Schneider Madeleine Got Rodney and Marlene McGowan Wolf and Ute Schneider Mona Brown Gary and Veronica Brown Louise and A.G. Hagen Catherine Girden

In Loving –Memory of David Daniel Bowen III "Our Hero" You were "Courage & Strength" You were exceptional.......

David, Catherine & Melissa Bowen

In Honor of Rona Majower

In Loving Memory of Jordan David Specht

Muriel Goodfriend

"Our Hero" You were "Courage & Strength" You were exceptional.......

David, Catherine & Melissa Bowen

In Honor of Darlene Clarke who works tirelessly for the Foundation

In Loving Memory of a Very Special Mother, Lucile Hurtubise Anne Hurtubise

November, 1997 Ehlers–Danlos National Foundation — Page 16

The Center for Oral and Maxillofacial Surgery Margaret Clarke Michael B. and Mary Ellen Feigi Michael S. and Gloria Feigi Greg and Shari McDonald Mr. & Mrs. Cosmo Midea Dr. Thomas P. Murphy Ron, Robbin and Rachel Ricci

Donations

The International Ehlers–Danlos Syndrome Network (I.E.D.S.N.)

10/96 through 9/97 Patron Donors ($ 250 – $ 499) Judith and Marvin Bellin Barbara Bergfelder William Buckley Lorraine Earnhart Mrs. Meg Harmon Pam and Dick Malenfant Sally Pinkerstaff, M.D. The Rup Family Harley Rutstein

Founded in June 1997. The purpose of this network is to promote cooperation between Ehlers–Danlos Syndrome Organizations worldwide. All organizations represented in the Network are autonomous and the Network will offer the opportunity to work collectively when there is a need.

Benefactor ($500 – $999) Kathleen Barger Mary Bryant and Family Brent & Collen Butcher Grandmother’s Club # 1021 Kendall Door & Hardware, Inc & Kendall Corporation Stephen and Charlene Nash Erika and Tibor Neumann

Silver Benefactor ($ 1,000 – $ 2,499) Dr. & Mrs. Herbert Brizel Mr. & Mrs. Ed Brutman Marian Jandecka Mr. and Mrs. John Richardson Glen and Susan Schafer

The initial meeting of the I.E.D.S.N. was held in June 1997 in Villefranche–Sur–Mer, France. Delegates attended from Belgium, Denmark, France, Germany, Italy, Norway, Sweden, United Kingdom, and the United States. The Foundation of this organization was established during this first historic meeting. The focus will initially consist of sharing resources between participating groups. A long term goal will be the support of EDS research worldwide. The First International EDS Conference is scheduled for the summer of 2001. Listed below are the Charter Member Organizations and the contact person for each organization:

Australia Australian EDS Support Group Vicki Tate, Coordinator Upper Orara Road Karangi Via 2450 Coffs Harbour, N.S.W. Australia Telephone: 066–538115

Platinum Benefactor ($ 2,500 – $4,999) Anonymous

Gold Benefactor ($ 5,000 or more) Harold Goldstein Susan Stephenson, Ph.D.

France Association Francaise des Syndromes d’Ehlers– Danlos Marie Helene Boucand, Director 12 Alle des Lilas 83400 Hyeres France Telephone/Fax: 04–94–23–36–40

Germany Deutsche Ehlers–Danlos Initiative e. V. Brigit Martin Grafensteinstr #36 96052 Bamberg Germany Telephone: 95131289

Italy Associazone Italiana per la Sindrome di Ehlers– Danlos (A.I.S.E.D.) Dr. Kathryn Dyne Dipartimento di Biochimica Via Taramelli 3B 27100 Pavia Italy Telephone: +39 382 507231/222 Fax: + 39 382 423108 E–Mail: dbioc@unipv.it

New Zealand New Zealand Ehlers–Danlos Syndrome Support Group Janette Longshaw 46 Waiohiki Rd. RD3 Napier Hawkes Bay New Zealand Telephone: 06 844 3115

Norway Ehlers–Danlos Syndrome Support Group Norway Grete Mikalsen, Secretary Agm Boltsvei 52 0664 Oslo, Norway Telephone: 47 22 53 15 32 E–Mail: grete.mikalsen@os.telia.no

Belgium

1998 Learning Conference Update Darlene A. Clarke, R.N., M.S.N., Chair, Board of Directors Under the direction of Peggy Rocha Snuggs, the 8th National Learning Conference is taking shape. As you probably already know, the conference will be held in Tampa, Florida. The date has been narrowed down to either the last week in July or the first week in August. Peggy has been busy securing medical experts to address the participants regarding various aspects of EDS. She is also selecting hotel accommodations for conference participants. We hope to see all of you in Tampa next summer! Look for more detailed information on the conference in the next edition of Loose Connections.

Belgium Marfan/EDS Support Group Claudine Waelput Antwerpsesteenweg 783 B–9040 St. Amandsberg Belgium Telephone: 9 228 61 16

Canada Canadian Ehlers–Danlos Association (CEDA) Jill Douglas–Hand, R.N. President and Founder 183 Charlton Ave Thornhill Ontario Canada L4J 6E9 Telephone: (905) 761–7552 Fax: 905) 761–7567 E–Mail: pghand@shaw.wave.ca Web Site: http://www.interlog.com/~ceda

Sweden Ehlers–Danlos –Syndrom Riksforbund Britta Berglund Bjorkvagen 77 S–147 33 Tumba, Sweden Telephone: 46 8 530 394 33 E–Mail: eds@swedenmail.com or Britta.berglund@swipmet.se

United Kingdom Ehlers–Danlos Support Group Mrs. Valerie Burrows, Founder 1 Chandler Close Richmond, North Yorks DL 10 5QQ UK Telephone/Fax: +44 01748 823867 E–Mail: EDS_UK@compuserve.com Web Site: http://ourworld.compuserve.com/ homepages/eds_uk/

United States

Denmark The Danish Ehlers–Danlos Society Betina Winther Boserup, Chairperson Eskildsvej 12 2990 Nivaa Denmark Telephone/Fax: +45 49149632

Ehlers–Danlos National Foundation Linda Neumann–Potash RN, MN, Executive Director 6399 Wilshire Blvd. Suite 510 Los Angeles, CA 90048 (213) 651–3038 — FAX: (213) 651–1366 E–Mail: loosejoint@aol.com Web Site: http://www.ednf.org

November, 1997 Ehlers–Danlos National Foundation — Page 17

Ehlers–Danlos National Foundation — Back Issues — 6399 Wilshire Blvd. Suite 510 Los Angeles, CA 90048 (213) 651–3038 Check Your Library For Missing Back Issues Of Loose Connections & Articles Newsletters Cost: $2.50 each or 5 for $10.00 – price includes postage and handling. For Canadian orders, please add $3.00, and for all other countries outside the United States, please add $5.00 to the cost of past issues requested which will cover shipping and handling charges.

1997, Volume 12, Number 3, “Managed Care – A Basic Map For Navigating Your Insurance”

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1993, Volume 8, Number 4, Number Payment “Dental Manifestations and Considerations In Treating Patients With Ehlers–Danlos Syndrome”_______ _______

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1993, Volume 8, Number 3, “What’s Wrong With This Patient?”

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1997, Volume 12, Number 1, “Ergonomics: Recognition and Evaluation of Risk Factors and Potential Stressors (Part 3)” _______ _______

1993, Volume 8, Number 2, “Gastrointestinal Considerations in People Suffering From Ehlers–Danlos Syndrome”

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1996, Volume 11, Number 4, “Ergonomics: Recognition and Evaluation of Risk Factors and Potential Stressors (Part 2)” _______ _______

1993, Volume 8, Number 1, “Chronic Pain Management Treatment Facilities”

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1996, Volume 11, Number 3, “Ergonomics: Recognition and Evaluation of Risk Factors and Potential Stressors (Part 1)” _______ _______

1992, Volume 7, Number 4, “Perspectives on Pain History and Current Status”

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1996, Volume 11, Number 2, “The Medical Partnership: How to Work as a Team With Your Doctor”

1992, Volume 7, Number 3, “Use of Mesh to Prevent Recurrence of Hernias”

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1991, Volume 6, Number 4, “Ehlers–Danlos Syndrome Type VI”

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1991, Volume 6, Number 3, “Ehlers–Danlos Syndrome Type III and Pregnancy”

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1990, Volume 6, Number 1, “Passport to Seattle”

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1990, Volume 5, Number 4, “The Emergency Room and Ehlers–Danlos Syndrome”

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1997, Volume 12, Number 2, “The Role of the Rheumatologists in Ehlers–Danlos Syndrome”

1995, Volume 10, Number 1, “Mandibular Joint, Orthodontic and Dental Findings In EDS”

Number

Payment

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_______ _______

1994, Volume 9, Number 3, “Genetic Information and Health Insurance” 1994, Volume 9, Number 2, “Heritable Disorders of Connective Tissue and Disability and Chronic Disease In Childhood” 1994, Volume 9, Number 1, “Official Launch of the Ehlers–Danlos Syndrome Database Manager”

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_______ _______

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Total Number of Back Issues Ordered:

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Total Amount of Payment Enclosed: Please make payment to the “Ehlers–Danlos National Foundation.”

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Ehlers–Danlos National Foundation — Membership Form — 6399 Wilshire Blvd. Suite 510 Los Angeles, CA 90048 (213) 651–3038 PLEASE PRINT and send a change of address, if applicable Name: __________________________________________________________________ Date: _____________________________________ Address: ________________________________________________________________ Sex: F: ❏ M: ❏ Birthdate: ____________________ City: __________________________________ State: _______ Zip: _______________ E–mail Address: ____________________________ Telephone Number: (______) _______________________________________________ Fax Number: (______) _______________________ How did you hear about the foundation?: _________________________________________________________________________________ Are you or a family member diagnosed with Ehlers–Danlos Syndrome? .......................... Yes: ❏ No: ❏ Who: _________________________ If yes, do you know what type?: ___________________________ Are you a new member to Ehlers–Danlos National Foundation? ....................................... Yes: ❏ No: ❏ Renewal? ........ Yes: ❏ No: ❏ NEW! ❏ Check here if you’d like to receive Loose Connections in Adobe® PDF Format over the Internet. (E–Mail Address Required) I am interested in membership in the foundation and have enclosed my check which is payable in U. S. FUNDS to:

“Ehlers–Danlos National Foundation” ❏ Individual Membership ........................................................ $25.00 ❏ Family Membership ............................................................. $30.00

❏ Corporate Membership ....................................................... $ 50.00

Canada & Mexico ❏ Individual Membership ........................................................ $30.00

❏ Patron Donor ...................................................................... $250.00 ❏ Benefactor Donor ............................................................ $500.00+

❏ Family Membership ............................................................. $35.00 International:

❏ Corporate Sponsorship ..................................................... $____.__ ❏ Additional Donation ......................................................... $____.__

Donor Program

❏ Individual Membership ........................................................ $35.00 ❏ Family Membership ............................................................. $40.00 Please take a few minutes to list problems or ideas that you would like to see covered in future issues of “Loose Connections”. The Ehlers— Danlos National Foundation was created to help everyone and your ideas and thoughts are very important to us. _______________________ __________________________________________________________________________________________________________________ __________________________________________________________________________________________________________________ If you are interested in corresponding with other families, please sign the release below. Please note that only your name, address and phone number will be released and ONLY to other members. All other information you provide to the foundation will remain strictly confidential.

You are not obligated to sign this release. ❏ Check here if you’d like the name and address of three other members in your area to communicate with. ❏ Check here if you want to be notified about a support group in your area (Your name will be forwarded to the nearest branch).

Signature: ______________________________________________________________________

The EDNF is now equipped to accept credit card payment (Discover, VISA & Mastercard Only).

This will be particularly helpful to our international members.

Method of Payment :

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Check

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Money Order

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Mastercard

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VISA

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Discover

Credit Card #:

Signature: ______________________________________________________________________ Expiration Date: _____________________ The EDNF does not lend, sell or trade its membership list to any individual or organization.

The EDNF is a not for prof it corporation. Your donation is ta x deduct ible and is sincerely appreciated.

November, 1997 Ehlers–Danlos National Foundation — Page 19

Loose Connections Ehlers–Danlos National Foundation 6399 Wilshire Blvd. Suite 510 Los Angeles, CA 90048

ADDRESS CORRECTION REQUESTED

Ehlers–Danlos Syndrome (EDS) is a group of heritable disorders of the connective tissue often characterized by hyperextensible skin, hypermobile joints, easy bruisability of the skin, and a bleeding diathesis. EDS is named for two physicians (Ehlers and Danlos) who described forms of the condition in the early 1900s. At least six forms of Ehlers–Danlos Syndrome have been described, which are not gradations in severity, but represent distinct disorders which “run true” in a family.

The Ehlers–Danlos National Foundation (EDNF) was created in 1985 in an effort to provide emotional support and updated information to those who suffer from the disorder. In addition, EDNF serves as a vital informational link to and from the medical community. Loose Connections, the official communications link of EDNF, is published on a quarterly basis. Subscription information may be obtained by writing the Foundation.

6399 Wilshire Blvd. Suite 510 Los Angeles, California 90048 Phone: (213) 651–3038 — FAX: (213) 651–1366 Web Site: www.ednf.org — E–Mail: loosejoint@aol.com