loose connections, february 1998

The Revised Ehlers–Danlos Syndrome NosologyCompiled By Darlene A. Clarke, R.N., M.S.N.,

Karen Skrocki Czerpak, R.N. &Linda Neumann–Potash, R.N., M.N.

The Facts AboutEhlers–Danlos

Syndrome

Vol. XIII, Number 1 The Official Communications Link Of The Ehlers–Danlos National Foundation February, 19986399 Wilshire Blvd. Suite 510 Los Angeles, California 90048 (213) 651–3038

Loose ConnectionsLoose Connections

Editors Note: As many of you are aware, theEDS Nosology paper is still “in press” withthe American Journal of Medical Geneticsand has not been published to date.Therefore, the EDNF has not been able toprovide complete information on the revisedNosology. In an attempt to update ourmembers as much as possible at this time, theEditors of Loose Connections decided toprint the Foundation’s two new EDSbrochures as the lead article for this issue.The following brochures were revised inJanuary of 1998 to reflect the revisedNosolgy. Please know that as soon as theEDS New Nosology article is published, theFoundation will request permission from thepublisher to reprint the article for ourmembers.

In This Issue...The Revised Ehlers–DanlosSyndrome Nosology ........................... 1Notes From Nancy .............................. 2EDNF’S Comic Connectionwith Harvey Korman .......................... 2The Chair’s Connection ...................... 3The Executive Element ....................... 4Branch News ................................ 5 & 6Wish List ............................................. 7A Very Special Thank You .................. 7Manuscript Guidelines ....................... 7Eighth NationalLearning Conference ........................ 10EDS In NIH Research Project .......... 10Letters To The Editor ........................ 11Memorials and Honorariums ............ 12Donations .......................................... 12Non-Bowl, Bowl–A–Thon ............... 13Back Issues ....................................... 14Membership Form ............................ 15

Ehlers–Danlos syndrome (EDS) is aheterogeneous group of heritable connectivetissue disorders, characterized by articular(joint) hypermobility, skin extensibility andtissue fragility. There are six major types ofEDS. The different types of EDS areclassified according to their manifestationsof signs and symptoms. Each type of EDS isa distinct disorder that “runs true” in afamily. This means that an individual withVascular Type EDS will not have a child withClassical Type EDS. Individuals with EDShave a defect in their connective tissue, thetissue which provides support to many bodyparts such as the skin, muscles andligaments. The fragile skin and unstablejoints found in EDS are the result of faultycollagen. Collagen is a protein which acts asa “glue” in the body, adding strength andelasticity to connective tissue.

Symptoms

Clinical manifestations of EDS are mostoften skin and joint related and may include:

Skin: soft velvet–like skin; variable skinhyperextensibility; fragile skin that tears orbruises easily (bruising may be severe);severe scarring; slow and poor woundhealing; development of molluscoid

Views expressed herein are only those of the authoopinions or policies of the Ehlers–Danlos Nat

pseudotumors (fleshy lesions associated withscars over pressure areas).

Joints: joint hypermobility; loose/unstablejoints which are prone to frequentdislocations and/or subluxations; joint pain;hyperextensible joints (they move beyond thejoint’s normal range); early onset ofosteoarthritis.

Miscellaneous/Less Common: chronic,early onset, debilitating musculoskeletal pain(usually associated with the HypermobilityType); arterial/intestinal/uterine fragility orrupture (usually associated with the VascularType); Scoliosis at birth and scleral fragility(associated with the Kyphoscoliosis Type);poor muscle tone (associated with theArthrochalasia Type); mitral valve prolapse;and gum disease.

Prevalence

At this time, research statistics of EDS showthe prevalence as 1 in 5,000 to 1 in 10,000. Itis known to affect both males and females ofall racial and ethnic backgrounds.

Hereditary PatternsThe two known inheritance patterns for EDSinclude autosomal dominant and autosomalrecessive. Specifics regarding geneticinheritance may be found in another EDNFinformational brochure. Regardless of theinheritance pattern, we have no choice inwhich genes we pass on to our children.

Continued on page 8.Continued on page 8

rs, and should not be construed to represent theional Foundation and it’s elected officials.

http://www.ednf.org

The EDNF’s first major fundraising event,“Comic Connections” with Harvey Kormanand Yarmy’s Army is really taking shape. Theevent will be held on Sunday, June 14, 1998 atthe Olympic Collection in Los Angeles,California. This black tie comedy night willfeature Harvey Korman and many other wellknown comedians. The cost of each ticket toattend this star studded event is $200.00 perperson. The Honorary Committee for this eventis forming with Harvey Korman as the Chair.The following individuals have agreed to serve

on the Honorary Committee, notably, MelBrooks, Carol Burnett, Tim Conway, TedDanson, Budd Friedman, NormanGreenbaum, Buz Kohan, Tommy Lasorda,Hal Linden, Peter Marshall, Tom Poston, VinScully, Steve Soboroff, Alan Thicke, SaulTurteltaub and Dick Van Patten. This eventhas been a dream of the EDNF Board ofDirectors for many years. It almost seemsunbelievable that it is really going to happen.But thanks to the generosity and compassion ofHarvey Korman, this is soon to be a reality.

LooseConnectionsPublished Quarterly By The

Ehlers–DanlosNational Foundation

— Founder —

Nancy Hanna Rogowski1958 – 1995

Executive DirectorLinda Neumann–Potash, R.N., M.N.

PresidentNancy Regas, R.N., M.S., M.F.C.C.

Vice PresidentKaren Skrocki Czerpak, R. N.

VP for Patient AdvocacySusan Stephenson, R.N., B.S.N., C.C.R.N.

SecretaryMaggie Buckley

TreasurerHarold Goldstein

Board of DirectorsDarlene A. Clarke, R.N., M.S.N., Chair

Maggie BuckleyKaren Skrocki Czerpak, R.N.

Harold GoldsteinNancy Regas, R.N., M.S., M.F.C.C.

MedicalAdvisory Board

Petros Tsipouras, M.D., ChairPatrick Agnew, D.P.M.Robin Bennett, M.S.William Cole, M.D.Mark Evans, M.D.

Richard Wenstrup, M.D.

Medical ConsultantsPat 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.

EditorsDarlene A. Clarke, R.N., M.S.N.

Karen Skrocki Czerpak, R.N.Linda Neumann–Potash, R.N., M.N.

Publisher Keith G. Clarke

to” [such wisdom]; “do you want yourmommy?” [she knew what really mattered]The patient nodded. The five year old marcheddown the hall of the Intensive Care Unit andsaid, she wants her mommy. She found themother and pulled her into the room.

As I watched the story, tears streamed downmy face because the hope was so powerful ittraveled across the air waves; it will and candefeat any trial or tribulation. In both cases, theparents believed and gave their children hope,and the children gave hope right back. It iscontagious.

So if there is any new year’s resolution youmake, promise yourself to never give up hopeand to live for the wonderful bounty in life. Ihave personally learned, time and time again,there is no substitute and there is nothingbetter. Maybe it’s time to recall the words toour song; they were written to provide hopeand to serve as a reminder of that hope:

We are searching for tomorrow, but we’reblinded by today,

The winds of fear may blow us down, but thedreams and hope remain.

Just open up your hearts and minds, younever know what you might find;

Together we will find a way,And help to make tomorrow,A Brighter Day

With Hope,Nancy

Notes From NancyNancy L. Regas, RN, MS, MFCC — President, EDNF

A new year has begun and we are one stepcloser to the turn of the century. A constant thattranscends these milestones is hope. Webster’sCollegiate Dictionary says that hope is toexpect with desire. It is the belief that there issomething better, something to reach for,dreams can and will come true; finally, it putsdefinition into our lives. Without hope, there isno living, there is only existing.

There isn’t a single person that can say theirlives are perfect. In our human struggles, wecling to the belief that things and people canchange and grow. In our growth comesfulfillment and the attainment of goals. Withouthope, why should we reach for the stars, whyshould we try?

I saw a tremendous story on a news programabout a three year old girl who needed a fiveorgan transplant. They asked the mother, Howcan you go on? The mother’s reply was simple,How can I not? Look at the smile on her face.She doesn’t even know how sick she is, she justloves life. They traveled the country andeventually got the transplants. They met a fiveyear girl who had had the same surgery, whichwas successful. She told the reporter, “This is alucky world. I wouldn’t be alive without theoperation.” It was so clear and so simple forher; children just know and unquestionablybelieve. When the five year old visited her newfriend in the hospital after surgery, the nursestold the little girl to open her eyes to see herguest. With tubes everywhere, attached toeverything, she shook her head no. The fiveyear old spoke, “That’s okay, you don’t have

February, 1998 Ehlers–Danlos National Foundation —

EDNF’S

Comic Connection with

Harvey Korman

The Chair’s ConnectionDarlene A. Clarke, R.N., M.S.N., Chair, Board of Directors

I have been asked repeatedly by friends, family and members ofthe Northeast Ohio Branch the following questions: “What isalternative medicine?” “Can it help someone with EDS?” As aproduct of “Traditional Western Medicine”, I knew that I did nothave the answer to either question. Therefore, I decided to answerthe first question in an attempt to educate myself as well as otherEDNF members. Before I proceed, I must stipulate that neither theEDNF, the EDNF medical advisory board, nor myself personallyadvocates using alternative medicine in place of traditionalmedicine to manage EDS. Alternative medicine used in conjunctionwith traditional/conventional medicine may prove beneficial inhelping to manage your EDS. This is an informed decision for youand your current physician to make. So, what is alternativecomplementary medicine?

Alternative complementary medicine is known as a holisticapproach to medicine. This means that physicians approach diseaseand illness by simultaneously focusing on the “whole” person(body, mind and soul). Alternative complementary medicineincludes treatments that are non-traumatic to the body - some ofwhich have been documented in many cultures for more than 2,000years. The goals of alternative complementary medicine are tomanage, cure (when possible), or reduce the pain and severity ofdisease conditions. These goals are accomplished by protecting,promoting, or modifying normal body functioning without the useof drugs which are considered “xenobi-otic” or foreign to thehuman body. A new trend is slowly emerging in medicine today.Some physicians who can prescribe medications (M.D.’s andD.O.’s) are gradually supplementing their traditional medicalpractice with alternative complementary options to provideoptimum patient health care and treatments.

It is estimated that over 200 alternative therapies are currentlybeing used in the U.S. today. It is important to keep in mind thatwhat we Americans and Western medicine practitioners identify asalternative medicine is literally traditional medicine for almost 80%of the world. That doesn’t make one form of medicine right orwrong - although, it is worth investigating all of your healthcareoptions whether you are ill or are just trying to maintain anoptimum level of health. Let’s take a look at possible alternativecomplementary therapies that might be prescribed if you visited aphysician who practices alternative medicine.

Prescribing physicians offering alternative medicine in conjunctionwith traditional medicine will often prescribe alternative therapiessuch as: osteopathic manipulation therapy, Chiropracticmanipulation, acupuncture, energy work, massage, tai chi, andvitamin and nutrition therapy to assist the body in its quest forhomeostasis (inner balance). Alternative complementary medicineis now receiving serious consideration by patients and physiciansalike. Medications and surgery are sometimes weighed verycarefully alongside the benefits of alternative medicine as acomplementary or primary procedure choice by patients andphysicians.

Most insurance companies are not currently reimbursing the cost ofmany alternative medicine treatments and therapies. It is worthnoting though that positive treatment results and patient demand is

facilitating the reevaluation of thishealthcare insurance practice. Despite thelack of reimbursement by insurancecompanies, many patients are continuingto employ alternative complementarytherapies. These patients generally assumean active role in their healthcare plan andtake responsibility for their treatmentchoices. Research estimates that at least61 million Americans use some form ofalternative or complementary therapy for existing problems or forpreventative purposes. How did these individuals choose analternative or complementary care physician?

The best advice one can give on how to choose an alternativecomplementary care physician is to use the same criteria that youused to choose your physician trained in Western medicine. You mustcarefully look at the physician’s qualifications. Specifically ascertainhis or her alternative medicine knowledge, experience in alternativemedicine, educational preparation, certification and diagnosticabilities. Interview the prospective physician either in person or onthe telephone (you have the right to do this). It is extremely importantto determine how much he or she knows about EDS and itscomplications. If you choose an alternative complementary medicinephysician, what procedures/tests might you expect to be done prior toany form of treatment?

Several of the diagnostic tests that will be done are the same as whenyou see your primary care “traditional medicine” physician - somewill be different though. When you visit a physician operatedalternative medicine clinic, you will have a thorough diagnosticwork-up including: blood work, live blood cell analysis, urinalysis,infra-red imaging, hair analysis, digestive tract evaluation andbiological aging markers. These tests will be completed beforeappropriate therapy is prescribed.

Alternative complementary medicine is now recognized as a viabletreatment option that may be explored as a second opinion to diseasecentered treatments and to surgical procedures. It also hasdemonstrated merit as a life-long preventative health care practice,particularly for individuals wishing to improve the quality of theirlives. Alternative complementary medicine may or may not be anappropriate choice for you. The information presented only gives youa basic understanding of alternative complementary medicine. Thedecision to further explore this form of medicine is totally up to you.If you decide that alternative complementary medicine might be anoption for you, you need to conduct more research on this subject.Always be informed of your healthcare options, success rates andpotential complications before agreeing to any form of medicaltreatment - traditional or alternative.

Respectfully,Darlene

Reference:

Mrklas, T. (1997, Fall/Winter). What is alternative medicine? ChronicPain Solutions, p. 14.


The Executive ElementLinda Neumann–Potash, RN, MN

A mission statement. How important is it? It isabsolutely essential for any organizationbecause it provides direction and purpose.Have you taken the time to read the missionstatement of the EDNF? We have printed it onevery issue of Loose Connections for the last10 years. It states “The Ehlers-DanlosNational Foundation was created in 1985 inan effort to provide emotional support andupdated information to those who suffer fromthe disorder. In addition, EDNF serves as avital informational link to and from themedical community.” We recently rang in theNew Year, typically a time for most people toreflect on the year they’ve had and to plan anddream about the future. It is the same for mein my job as Executive Director of the EDNF.I looked back and have planned with ourmission statement in mind.

We promise to provide emotional support toour members. Have we done it? I think so.Although we have not had a national learningconference since we last met in Cincinnati, westill have other ways to provide you with asense of support. The list of chartered localbranches continues to grow. We now have 21chartered Branches and more are on thehorizon. This allows our members to meet ona regular basis and share experiences with oneanother. If you are not being served by a localbranch and would like to start one in yourarea, you are welcome to contact HaroldGoldstein, Director, Local BranchOrganization. His address and phone numberare listed at the end of each issue’s BranchNews section. If you live in an area notserviced by a local branch, we can provideyou with the name of other EDNF members,so that you may communicate with someonewho knows what life with EDS is like. Finally,for those of you who have been waiting forwhat seemed an eternity, we will be havingour eighth national learning conference thisJuly, in Tampa, Florida. For those of you whoare newly diagnosed or new to theFoundation, I strongly recommend attendingthis conference. It is a very powerfulexperience. You will meet many other peoplewith EDS and learn more in a few days thanyou could ever think possible. Of course, forthose of you who have already been to aconference, I need not sell it to you. Youalready know the benefits of meeting andsharing with other people in a similarsituation. Some describe it as the one weekout of the year that they feel normal.

We promise to provide updated information tothose who suffer from EDS. Have we donethis over the past year? Most definitely. Loose

Connections is our primary source fordisseminating information. I am sure that allwould agree that Loose Connections hascontinued to improve both in format and incontent over the last year. Thanks to the hardwork of Keith Clarke, an EDNF member whograciously volunteers his time and talent topublish Loose Connections, we are able tokeep you well informed. We continue to seekan editor for Loose Connections; that is,someone who will look to the future to securetimely articles and manage the wholeenterprise of putting together an issue filledwith medical information as well as ideas forliving and coping with EDS. In the nearfuture, we will have brand new brochures onsubjects such as types of EDS and the Factsabout EDS based on the new Nosology. Thebrochures will be a good way for you toinform your health care providers about EDS.

We promise to serve as a vital informationallink to and from the medical community.Have we done this? The answer to this is aresounding yes. This has been a very excitingyear for us. In June, EDNF and the EDSSupport Group of the UK, co-sponsored theNosology meeting in Villefranche, France.Five physician experts on EDS from aroundthe world gathered in France to discuss therevision of the classification of EDS. Theresulting Nosology paper will be published inthe American Journal of Medical Genetics inthe near future. The classification of EDStypes has been simplified, each defined bymajor and minor criteria. Preliminarybrochures have been printed based on the newNosology. We have purchased a booth andhave disseminated our new information at theAmerican Society of Human Genetics last fallin Baltimore and will be doing the same thisFebruary at the Joint Clinical Geneticsconference in Los Angeles. We distributedover 2000 brochures in Baltimore toresearchers, geneticists, nurses and genetic

counselors. We plan to continue this annually.We will also be targeting other specialtygroups such as orthopedic surgeons andrheumatologists by attending their yearlyconferences and distributing our information.Hopefully, more people will become awarenot only of our existence, but also of our newinformation. Our goal is to help facilitateearlier diagnosis of EDS. In addition, we nowhave a website full of information on EDS.Many articles from past issues of LooseConnections may be found there. It is linkedto other related websites such as those of localbranches and those of EDS support groupsaround the world. Many people have foundout about our existence through theinformation superhighway. Please take sometime to visit the website if you haven’t alreadydone so and invite your health care providersto do the same. Once again, we have KeithClarke to thank for setting this up for us. Ourweb site address is: http://www.ednf.org

So, in my assessment, 1997 looks like it was agood year. We hope to make 1998 an evenbetter year. On June 14th, we will hold our firstmajor fund raising event. It is our ComicConnections event, hosted by Harvey Korman.In addition to Harvey as the host, many othershave agreed to serve on the honorarycommittee, notably, Mel Brooks, CarolBurnett, Tim Conway, Ted Danson, BuddFriedman, Norman Greenbaum, Buz Kohan,Tommy Lasorda, Hal Linden, Peter Marshall,Tom Poston, Vin Scully, Steve Soboroff, AlanThicke, Saul Turteltaub, and Dick Van Patten.This event has been a dream of everyone onthe board of directors for many years. Italmost seems unbelievable that it is reallygoing to happen. But thanks to the generosityand compassion of Harvey Korman, this issoon to be a reality. It is a tribute to him thatso many others answered his request to serveon the honorary committee. With this event,we hope to not only raise necessary funds, butalso to raise awareness of EDS.

The Board of Directors meets via telephoneconferencing every other month . We arestriving to continue the dream of NancyRogowski. We believe that we are doing agood job, but we realize that much more needsto be accomplished. If you feel that you have atalent or resources that would help our efforts,please contact me. EDNF was built by oneperson, volunteering her time and talents andcontinues to run by people volunteering theirtime and talents. We welcome your feedbackas well as your support. You now know ourmission statement. Our goal is to improve thelives of those affected by EDS throughsupport, education and, research.


Continued On Page 6

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MA

GAIL

FL

CA

AZ

DC

MD

— Branch News —


By Harold Goldstein, Director, Local Branch Organization

An outfit from the Texas Panhandle area around Amarillo came loping in to town carrying an application for acharter that would make them our 21st chartered branch.

The Internal Revenue Service has notified us that the tax-exempt status of the national organization has beenextended to the first 20 chartered branches, those whose names are printed in capital letters in the summary below.

Phoenix: An organizing meeting is planned for late in February. Christine Phillips, (602) 465-0502.

Tucson: The group held a meeting and elected officers. Robin Forsyth, (520) 579-8351.

GREATER LOS ANGELES BRANCH: At its November meeting the branch elected new officers. Believingthat its area was too big to serve members conveniently, it decided to ask the Board of Directors to change itscharter to Greater Los Angeles Branch. Meetings will be held every second month, alternating between the SanFernando Valley and Central Los Angeles. First meeting for 1998 was set for January 11, with Linda Neumann-Potash presenting a brief overview of the new EDS Nosology. Goals for the year were also established. Severalmembers of the Branch have volunteered to assist with the EDNF booth at the 5th Annual Clinical GeneticsConference to be held in Los Angeles. On March 8th, Cheryl Hood, C.P.O., will speak on “Bracing and OrthoticOptions for People with EDS.” On May 17th, Linda Neumann-Potash will speak on the new diagnostic criteria forEDS. Shari Gamson, President, (818) 893-3937.

San Diego: Toni Owen, (619) 274-0472

NORTHERN CALIFORNIA BRANCH: The branch’s exhibit at the Abilities Expo in San Mateo was visitedby many people, and there was much interest in a list and demonstration of “EDS-friendly products” by StayceCurry, R.N. The branch also ran a successful dinner gala and silent auction to raise money for scholarships toattend the Tampa Learning Conference. The meeting on February 21 will feature a discussion of skin care. At theApril 18 meeting Patti Gladfelter will return, discussing physical therapy, and conducting brief individualsessions with members. Maggie Buckley, President, (510) 947-2358.

Santa Barbara: This is a support group that meets irregularly. Rich Scholl, (805) 683-8874.

WASHINGTON METROPOLITAN AREA: A meeting to take care of organizational chores and to set anagenda for the year, including topics for speakers, was scheduled for February 1. Marilyn Della Badia, President,(703) 924-1220.

TAMPA BAY AREA: At the November meeting, the branch decided to skip a Learning Day for 1998 andconcentrate on the national EDNF Conference, on which members are working hard. Peggy Rocha Snuggs,President, (813) 949-1585.

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

Orlando: Pam Williams, (352) 735-2735.

ATLANTA: Kathy Parrish, Secretary, (770) 338-9570.

CHICAGO: Branch is raising funds by “Shop N’ Share” days at local stores, at which the branch gets apercentage of the amounts of members’ purchases. Local dues were set at $20 per year for each family. PatDamler, President, (815) 568-6216.

MAINE BRANCH: On February 8, Jeannie Young, Occupational Therapist, will speak on “Small JointProtection and Adaptive Equipment.” Samantha Paine-Paradis. President, (207) 442-8779.

BALTIMORE: A meeting of branch officers is scheduled for January, and a branch meeting will be held inFebruary or March. Michelle Adams, President, (410) 879-0548.

Boston: The group is working at finding a place to hold its first meeting. Robin Neas, (617) 767-4553 .

Detroit: Renee Hall, (313) 868-7086

MINNEAPOLIS-ST.PAUL: A meeting on January 6 was devoted to discussion of plans for future activity.Jackie Collins, President, (612) 934-4420.

ST. LOUIS: The branch meets on the first Thursday of every month at Uncle Bill’s Pancake House, on SouthKing’s Highway. Janet Dunn, President, (314) 645-4114.

Omaha: An organization meeting was set for January 24. Cheri Lynne Woodward, (402) 451-8905.

Manchester: An organization meeting is scheduled for February 28. Tom and Tina Roe, (603) 624-7947

SOUTHERN NEW JERSEY: The group is actively promoting information on EDS among health professionalsand the public. A branch meeting is planned for February. Cathy Bowen, President, (609) 625-7975.

CENTRAL NEW JERSEY: A meeting is planned for early April. Lisa Schoenberg, President, (732) 254-1285.

NEW YORK METROPOLITAN AREA: At its November meeting the branch discussed the problem of findingdoctors familiar with EDS, and pain management and physical therapy in treatment of EDS. Catherine Alvarez,Co-President (718) 652-2867, and Kim Christensen, Co-President, (914) 632-7264.

WESTERN NEW YORK: Branch meetings have been devoted to their project to mail information on EDS to800-1,000 health professionals in the area. The branch is considering making a video to send to medical andnursing schools. Lou VanWert, President, (716) 688-2756.

PIEDMONT TRIAD: The January 24 meeting will be devoted to business. On February 28 the branch will meetas a support group, with a leader provided by the University of North Carolina at Greensboro. (The branch hasfound that its support group sessions are more productive when led by a trained counselor.) On March 28 thespeaker will be Dr. Duncan McCall, a rheumatologist. Charlotte Mecum, President, (336) 722-5879.

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

NORTHEAST OHIO: The branch held a very successful fund-raiser in December, selling bushels of nuts andbringing in bushels of money. The annual holiday celebration in December featured lots of food, exchange ofgifts, and a raffle of gifts donated by local merchants. At the next meeting, February 14, Dr. Jane Steckler willtalk about “Coping with a Chronic Illness.” Darlene Clarke, President, (440) 888-7317.

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

PHILADELPHIA: Susan Johnson of the Education Department of Doylestown Hospital will speak to thebranch on pain management on February 7. The branch is trying to enhance its support group sessions by askingmembers to report to the group on an experience they have had, in this way focusing the discussion more sharply.Roberta Kroll, President, (215) 794-8043.

Providence: Sue Kozlow, (401) 233-2046.

Dallas: Sarah Tovar, (972) 625-9142.

TEXAS PANHANDLE: At a meeting November 1 the group elected officers and applied for a charter. ConnieNieto, (806) 293-7361.

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

SEATTLE: Meetings will be quarterly, starting in February, At each there will be a pot-luck dinner, and, usually,a speaker. Barbara Uggen, President, (253) 529-4861

MILWAUKEE: The branch goes into hibernation in the winter. Next meeting will be in March. Lynn Sanders,President, (414) 679-9682.

RI

NY

NC

OH

ORPA

NJ

VAWA

TX

NHNE

For Information About Local Branches & Support GroupsYour 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

— Branch News —MI

MO

MN

WI


ManuscriptGuidelines for...

Loose Connections1. Manuscripts should be voluntary contributions submitted for the

exclusive attention of Loose Connections.

2. The submitted manuscripts should be written in a clear andconcise manner. The author(s) should write in a style appropriatefor lay audience. The content of the manuscript must focus onEhlers–Danlos Syndrome, complications of EDS, currentresearch on EDS, or the day to day issues of living and copingwith EDS.

3. Manuscripts should be submitted on a 3.5" disc in WordPerfect5.1 or higher or an IBM compatible word processor. If this is notpossible, the manuscript must be typed double spaced.Handwritten manuscripts will be automatically rejected.

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

5. Manuscripts/discs should be sent to:The Ehlers–Danlos National Foundation6399 Wilshire Blvd., Suite 510Los 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 howthey will be better off after reading the article.

7. Include a title/author biography page. The authors’ biographicinformation includes: name, credentials, position, professionalaffiliation, city and state. Example: Thomas Smith, M.D.,Professor, Department of Pediatrics, Case Western ReserveUniversity, Cleveland, OH.

8. Tables and figures should be placed at the end of the manuscriptafter the references. Tables must be numbered consecutively withArabic numbers and have a title at the top. Figures and tablesmust 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 orfooters.

10. Subdivide the manuscript into main sections by insertingsubheads in the text. Subheads should be succinct andmeaningful.

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

12. Written permission must be obtained from a) the holder ofcopyrighted material used in the manuscript; and b) individualsmentioned in the narrative or acknowledgment. Letters ofpermission must be submitted to the publisher of LooseConnections before publication of the manuscript.

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

14. Surveys submitted for publication in Loose Connections mustinclude 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 itsstyle and space requirements and to clarify the presentation ifnecessary.

Ask A PhysicianQuestion:

“What is the difference between Joint HypermobilitySyndrome and Ehlers–Danlos Syndrome?”

Answer:

Joint Hypermobility is a relatively common manifestationamong individuals who present to rheumatologists. Insome instances, there is a positive family history as thistrait is inherited in an autosomal dominant fashion. JointHypermobility is one of the major criteria in several EDStypes. However, in order to make the diagnosis of EDS inan individual, the presence of other signs, i.e., skinextensibility or the presence of dystrophic scars isnecessary. I hope this clarification is useful.

Petros Tsipouras, MDDepartment of Pediatrics

University of Connecticut Health Center263 Farmington AvenueFarmington, CT 06030

A Very SpecialThank You

The Board of Directors would like to express their sinceregratitude to members of the Baltimore and Atlanta Brancheswho helped Linda Neumann-Potash and Darlene Clarke manthe EDNF booth at the 47th Annual Meeting of the AmericanSociety of Human Genetics in October of 1997. Theirassistance allowed us to speak individually with eachparticipant who came to our booth. We could not have done itwithout their help! The members who deserve this specialthank-you are:

Baltimore BranchMichele Adams — Teresa LancasterRandy Lancaster — Nancy Spencer

Atlanta BranchApril Leaman

The 48th Annual Meeting of the American Society of HumanGenetics will be held in Denver, CO from October 27-31,1998. If any EDNF members in the Denver area would like tovolunteer to help the EDNF man our booth, please contactLinda Neumann-Potash at the National Office. We really needyour help to make this meeting a SUCCESS!! Thank you inadvance if you can be of any assistance at this meeting.

The Revised Nosology from page 1

How is EDS Diagnosed

Diagnosis of EDS is based upon clinicalfindings and upon the family history. Sincemany patients do not fit neatly into one ofthe specific types of EDS, a diagnosis isoften delayed or overlooked. Specificdiagnostic tests are available for some typesof EDS in which there is a knownbiochemical defect. Sometimes, a physicianmay perform a skin biopsy to study thechemical makeup of the connective tissue.The biopsy involves removing a small pieceof skin, under local anesthesia. Physicianswho are able to diagnose EDS may includemedical geneticists, pediatricians,rheumatologists and dermatologists.

Treatment/Managementof EDS

The gaping skin wounds, which are commonin several types of EDS, should beapproached with care. Proper repair of thesewounds is necessary to prevent cosmeticdisfigurement. Surgical procedures can berisky, as fragile tissues can unexpectedlytear. Suturing may present problems for thesame reason. Excessive sun exposure shouldbe avoided by the daily use of sunscreen.One should avoid activities that cause thejoint to lock or overextend.

A physician may prescribe bracing tostabilize joints. Surgical repair of joints maybe necessary at some time. Physicians mayalso consult a physical and/or occupationaltherapist to help strengthen muscles and toteach people how to properly use andpreserve their joints. To decrease bruisingand improve wound healing, some patientshave responded to ascorbic acid (vitamin C)by taking 1 to 4 grams daily. Prior to startinga regimen such as this, it is imperative toconsult with your physician for specificrecommendations.

In general, medical intervention is limited tosymptomatic therapy. Prior to pregnancy,patients with EDS should have geneticcounseling. Children with EDS should beprovided with information about thedisorder, so they can understand why contactsports and other physically stressful activitiesshould be avoided. Children should be taughtearly on that demonstrating the unusualpositions they can maintain due to loosejoints should not be done as this may cause

Februar

early degeneration of the joints. Familymembers, teachers and friends should beprovided with information about EDS sothey can accept and assist the child asnecessary.

Prognosis

The prognosis of EDS depends on thespecific type. Life expectancy can beshortened with the Vascular Type of EDS dueto the possibility of organ and vessel rupture.Life expectancy in all other types is normal.

Reference:Beighton, P., De Paepe, A., Steinmann, B.,Tsipouras, P., & Wenstrup, R. (in press).Ehlers-Danlos Syndrome: Revised Nosology,Villefranche, 1997. American Journal ofMedical Genetics.

Types OfEhlers–Danlos

SyndromeEhlers-Danlos syndrome (EDS) is aheterogeneous group of heritable connectivetissue disorders characterized by articularhypermobility, skin extensibility and tissuefragility. Individuals with EDS have a defectin their connective tissue. It is this tissue thatprovides support to many body parts such asthe skin, muscles, ligaments and organs. Thefragile skin and unstable joints found in EDSare due to faulty collagen. Collagen is aprotein that acts like glue in the body addingstrength and elasticity to connective tissue.There are six major types of EDS. Thedifferent types of EDS are classifiedaccording to the signs and symptoms that aremanifested. Each type of EDS is a distinctdisorder that “runs true” in a family. Thismeans that an individual with Vascular TypeEDS will not have a child with ClassicalType EDS.

Classical Type(Formerly EDS Types I & II)

Marked skin hyperextensibility (stretchy)with widened atrophic scars and jointhypermobility are found in the ClassicalType of EDS. The skin manifestations rangein severity from mild to severe expression.The skin is smooth and velvety with theevidence of tissue fragility and easybruisability. Examples of tissue extensibilityand fragility include hiatal hernia, anal

y, 1998 Ehlers–Danlos National Foundation —

prolapse in childhood and cervicalinsufficiency. Hernias may be a post-operative complication. Scars are foundmostly over pressure points such as theknees, elbows, forehead and chin.Molluscoid pseudotumors (calcifiedhematomas) associated with scars arefrequently found over pressure points such asthe elbows, and spheroids (fat containingcysts) are usually found the on the forearmsand shins.

Complications of joint hypermobility includesprains, dislocations/subluxations and pesplanus (flatfoot) to name a few. Recurrentjoint subluxations are common in theshoulder, patella and temporomandibularjoints. Muscle hypotonia and delayed grossmotor development may also be evident.

Clinical Testing - Abnormal electrophoreticmobility of the proα1(V) or proα2(V) chainsof collagen type V has been detected inseveral but not all families with the ClassicalType. The Classical Type of EDS is inheritedin an autosomal dominant manner.

Hypermobility Type(Formerly EDS Type III)

Joint hypermobility is the dominant clinicalmanifestation. Generalized jointhypermobility that affects large (elbows,knees) and small (fingers and toes) joints isevident in the Hypermobility Type.Recurring joint subluxations and dislocationsare common occurrences. Certain joints,such as the shoulder, patella, andtemporomandibular joint dislocatefrequently. The skin involvement(hyperextensibility and/or smooth velvetyskin) as well as bruising tendencies in theHypermobility Type are present but variablein severity.

Chronic joint and limb pain is a commoncomplaint amongst individuals with theHypermobility Type. Skeletal X-rays arenormal. Musculoskeletal pain is early onset,chronic and may be debilitating. Theanatomical distribution is wide and tenderpoints can sometimes be elicited.

To date, no distinctive biochemical collagenfinding has been identified by researchers.The Hypermobility Type of EDS is inheritedin an autosomal dominant manner.

Vascular Type(Formerly EDS Type IV)

This type is generally regarded as the mostserious form of EDS due to the possibility ofarterial or organ rupture. The skin is usuallythin and translucent with veins being seenthrough the skin. This is most apparent overthe chest and abdomen. There are certainfacial characteristics present in some affectedindividuals. These manifestations includelarge eyes, thin nose, lobeless ears, shortstature and thin scalp hair. Also evident is adecrease in subcutaneous tissue, particularlyin the face and extremities. Minor trauma canlead to extensive bruising.

Arterial/intestinal/uterine fragility or rupturecommonly arise in this type of EDS.Spontaneous arterial rupture has a peakincidence in the third or fourth decade oflife, but may occur earlier. Midsize arteriesare commonly involved. Arterial rupture isthe most common cause of sudden death.Acute diffuse or localized abdominal orflank pain is a common presentation ofarterial or intestinal rupture. Life expectancyis shortened with a majority of individualsliving only into their forties. Pregnancies maybe complicated by intra-partum uterinerupture and pre- and postpartum arterialbleeding.

Joint hypermobility is usually limited to thedigits. Tendon and muscle rupture can occur.Talipes equinovarus (clubfoot) is frequentlyseen at birth. Other manifestations that maybe found in the Vascular Type include:acrogeria (premature aging of the skin of thehands and feet); early onset varicose veins;arteriovenous fistula (an opening between anartery and vein), carotid-cavernous fistula;pneumothorax (collapse of a lung) /pneumohemothorax (collapse of a lung witha collection of air or gas and blood); gingivalrecession and complications during and aftersurgery (i.e. wound dehiscence).

The Vascular Type of EDS is caused bystructural defects in the proα1(III) chain ofcollagen type III encodes by COL3A1. Thistype of EDS is inherited in an autosomaldominant manner. A skin biopsy candiagnose this type of EDS.

Kyphoscoliosis(Formerly EDS Type VI)

Generalized joint laxity and severe muscle

Februa

hypotonia (weak muscle tone) at birth areseen in this type of EDS. The muscularhypotonia can be very pronounced and leadsto delayed gross motor development.Individuals with the Kyphoscoliosis Typepresent with scoliosis at birth that isprogressive. The phenotype is most oftensevere, frequently resulting in the loss ofambulation in the second or third decade.Scleral fragility may lead to rupture of theocular globe after minor trauma.

Tissue fragility including atrophic scars andeasy bruising may be seen in theKyphoscoliosis Type. Spontaneous arterialrupture can occur. Other findings may include:marfanoid habitus (Marfan like features);microcornea (abnormally small cornea); andradiologically considerable osteopenia(diminished amount of bone tissue).

Kyphoscoliosis Type EDS is the result of adeficiency of lysyl hydroxylase (PLOD),which is a collagen-modifying enzyme. Thistype of EDS is inherited in an autosomalrecessive manner. Kyphoscoliosis Type canbe diagnosed through a urine test.

Arthrochalasia Type(Formerly EDS Type VIIB)

Congenital hip dislocation has been presentin all biochemically proven individuals withthis type of EDS. Severe generalized jointhypermobility with recurrent subluxationsare seen in individuals with this type of EDS.Other manifestations of this type mayinclude: skin hyperextensibility with easybruising; tissue fragility including atrophicscars; muscle hypotonia; kyphoscoliosis andradiologically mild osteopenia.

The Arthrochalasia Type is caused bymutations leading to deficient processing ofthe amino-terminal end of proα1(I) [type A]or proα2(I) [type B] chains of collagen typeI. It is inherited in an autosomal dominantmanner. A skin biopsy can also diagnose thistype of EDS.

Dermatosparaxis Type(Formerly EDS Type VIIC)

Individuals with Dermatosparaxis Type EDShave severe skin fragility and substantialbruising. Wound healing is not impaired andthe scars are not atrophic. The skin texture issoft and doughy. Sagging, redundant skin isevident. The redundancy of facial skin results

ry, 1998 Ehlers–Danlos National Foundation —

in an appearance resembling cutis laxa.Large hernias (umbilical, inguinal) may alsobe seen. The number of patients reportedwith this type of EDS is small.

Dermatosparaxis Type EDS is caused by adeficiency of procollagen I N-terminalpeptidase. It is inherited in a autosomalrecessive manner. A skin biopsy candiagnose this type of EDS.

Other Types Of EDS

The current EDS type V (X-linked) has beendescribed in a single family. It is a rarevariant and the molecular basis of whichremains unknown. THis was previouslyremoved from the EDS classification.

The current EDS type VIII is similar to theClassical Type except that in addition itpresents with periodontal friability. This is arare type of EDS. The existence of thissyndrome as an autonomous entity isuncertain.

The EDS type IX was previously redefinedas “Occipital Horn syndrome”, an X-linkedrecessive condition allelic to Menkessyndrome.

The current EDS type X has been describedin only one family.

The EDS type XI termed “Familial JointHypermobility syndrome” was previouslyremoved from the EDS classification. Itsrelationship to the EDS is not yet defined.

Conclusion

This simplified classification system willfacilitate an accurate diagnosis of the Ehlers-Danlos syndrome and allow a clearerdistinction of disorders that overlap withEDS. It is important to note that each type ofEDS is distinct. If you have one type of EDS,you cannot develop another type. However,individuals with the same type of EDS mayhave slightly different manifestationsbecause each of us is a unique person.

Reference:

Beighton, P., De Paepe, A., Steinmann, B.,Tsipouras, P., & Wenstrup, R. (in press).Ehlers-Danlos Syndrome: Revised Nosology,Villefranche, 1997. American Journal ofMedical Genetics.

Eighth NationalLearning Conference

Comes to Tampa, FloridaThe Eighth National Learning Conference, will be held in Tampa, Florida July28th through 31st, 1998. The host Hotel will be the Tampa Airport Hilton atMetroCenter, Room rates are just$85.00 per night, which includes acomplimentary service to and fromTampa International Airport. Allrooms are oversized, offer individualclimate control, color remote TV, alarm clock radios,coffee service, irons, full length mirrors, phones withDATA ports and voice mail. Eight rooms are accessibleincluding 2 with roll-in showers.

Amenities include the use of the Hotel shuttle within a 3 mileradius, heated swimming pool and jacuzzi, lighted tenniscourt, complete fitness center, Hemingway’s restaurant and theBay Breeze lounge with nightly complimentary hor d’oeuvres, 24hour room service, complete ADA accessibilities, no access feescharged for 1-800 and credit card calls, and an 18-hole golf courseacross the street.

Reservations should be made individually (EDNF will not be responsible forHotel reservations) prior to July 7th , by calling (813) 877-6688 or the 1-800-Hiltons number. Be sure to ask for EDNF Conference rates when you call.

Those families wishing to stay in the Bay Area will be given Conference rates forthe three day period before and after the Conference.

Registration fees (to be announced) will cover the admission to all lectures,seminars, meetings, support sessions, and optional evaluations; admissions toOpening Night Party and the Banquet on July 31st. Also included in this year’sregistration are Continental Breakfasts and 2 luncheon buffets.

Plans are still being made, but some tentative sessions and seminars includephysical therapy, water therapy, genetics, pain management, orthopedic concerns/scoliosis, podiatry, immunology, patient advocacy, and sessions for care takersand children with EDS.

There will be a general membership meeting as well as an update on the newNosology.

A new pre-conference meeting/dinner session on July 27th, will be held for arepresentative from each Branch. This session will include opportunities tointeract and share, Branch finance issues, and the election of 2 Branchrepresentatives to the Executive Board. Local Branches should plan to send arepresentative. Each Branch may hold a fund raiser and may keep 100% of theprofits for this purpose!

A complete registration packet will soon be available by calling the foundation,in the next issue of Loose Connections, and on the EDNF Homepage. Mark youcalendars and plan to join us this summer in the Sunshine State.

Don’t miss this opportunity to meet other EDNF members,while learning more about EDS.

Conference Director,Peggy Rocha Snuggs

can be reached at(813)-949-1585.


EDS in NIHResearchProject

Harold Goldstein,Board of Directors

Patients with EDS are included in aresearch project at the National Institutesof Health, described as a “natural historystudy” of connective tissue disorders.Patients are selected for inclusion in thestudy on the basis of their previousmedical records. They are given a series ofexaminations - a blood sample is taken forDNA, a skin biopsy is taken for tissueculture (for EDS patients this may help indetermining the type of EDS), an extensiveeye examination is given by a geneticophthalmologist, a heart examination isgiven with an echocardiogram (and a MRIof the chest for Marfan patients),photographs are taken of arms, legs, hands,feet, and joints, a history is taken, and thepatient meets with a genetic counselor. Inannual follow-ups, the eye exam andechocardiogram are repeated.

It is intended that this will be a long-termstudy. Several hundred patients withconnective tissue disorders will beincluded; there is no particular targetnumber for EDS patients. The study isbeing conducted in the National HumanGenome Research Institute of the NationalInstitutes of Health, under the direction ofDr. Claire Francomano.

For more information, contactElizabeth Garabedian at:

(301) 435-2443,or write care of:

Medical Genetics Branch, NHGRIBuilding 10, Room 100101, MSC 1852

9000 Rockville PikeBethesda, MD 20892

Tampa, Florida

7/28 — 7/31/98

To The Great People at EDNF:

I am a member of EDNF for several years now. I have Ehlers-Danlosas so my 2 daughters. It runs on my dad’s side of the family. I askedmy younger daughter, Sarah, she is 19, for Christmas hints this yearand she wanted me to make a donation in her name to EDNF. She isa great kid, who really cares about others. I told her I’d be happy tofulfill her wish. I would like to make the donation in her honor.

Diana H., Macomb , MI

Dear Editor:

I only want to say that it’s a very very good paper which puts thefocus on the most interesting issues, even for me who live inSweden with another health care system and another insurancesystem. It’s very professional made, specially since EDNF is not abig organization.

I use to take home Loose Connections (very goodname!!) via internet and read it with Acrobat. TheSwedish EDS Forbund also have a paper - EDS-bladet - published two times every year. It’snot so good looking as Loose Connectionsbut on the other side the editioncirculation is only 400. They can usea copiator and don’t need aprinting works-house. As aprofessional journalist I should help them with the paper, but healthproblems and too early born children have stopped me from doingthat. I will see in the future.

Stefan Back, Umea, Sweden

Dear Editor,

Here is my renewal of my subscription. This past year has been avery hard one. I imagine that at almost 68 years old, I am aboutyour oldest known EDS person.

I asked some time ago about white bumps on my brain and if anyonehad an idea of what they are. I have consulted 2 neurologists in myarea and both agree they are “age bumps”. These same 2 doctorsdisagree though, on what I had last June. Whether it was a seizure(which I have no history of, nor does anyone in my family) orwhether I had a stroke (as I thought I had then and still do). Thisstroke is in the center of the back of my head which is why my motorfunctions were affected. I never “passed out” but my musclemovement and coordination were affected. I have had 2 MRI’s and 2EEG’s and since the 2nd set in October, there is no doubt that I hadanother stroke. I had one back in 1994. I have been told that theUniversity of Chicago Hospital is a good place for “brains” and planto go there in May when the Chicago weather is more beautiful.When it was 55 degrees here in Northwest Arkansas it was 7 degreesat my brother’s place in Chicago.

I am still very interested in finding out if others have the “whitebumps” on their brain as I do. The bumps are growing. In the MRItaken in October, the bumps seem to be larger that when first takenlast June. If you know of anyone else with these bumps, please letme know.

I wish you all well.

Melba Wilmoth Ray

309 Sanders Ave.

Springdale, AR 72764

Letters To The Editor


Dear E.D.N.F.,

I would like to thank you for giving me a complementarymembership this year. I am currently unemployed and struggling.This means a lot to me and I thank you for being so understanding.

Sincerely,

H.K.D.

Dear E.D.N.F., The Rugrat’s Are Coming To Town

Lisa Schoenberg's (Central NJ EDNF) daughter Thea, age 17 hasstudied nearly every type of dance, voice, acting & the violin fornumerous years. She has performed in over 20 musicals with localyouth groups, schools & performing arts camp & has donechoreography in many of them. She has taught Tap, Jazz, Hip-Hop,Stomp & Funk. Thea created a Dance Troupe at her high school, whereshe was also a Varsity cheerleader & still found time to entertain atlocal nursing homes & work P.T. at the local Dairy Queen.

She was headed to college in Sept. for a double major in Education &Dance (which requires an audition). To practice auditioning, she wentto NYC as they had just announced the new year long live NationalTour of the "Rugrats", which is made up of an extraordinary adultcast, management & technical crew. Though she is far younger & hadno professional experience, like the others, I guess she was just whothey were looking for & Thea was offered the lead role of “TommyPickles”. The babies are double casted, as the full body costumes theywear make it impossible for one person to perform an entire show.Thea wanted so much to also dance & sing in the show so, near theend of the show the mobile figures over Tommy's crib become livedancers. By this time Thea has changed costumes, the other Tommyfinishes the show, allowing Thea to become one of the dancers, atwhich time we first actually see her face.

This all came as quite a shock to all of us, but it was an opportunitythat couldn't be passed up. She is finishing her High Schoolrequirements through tutoring & a lap top & will graduate with herclass this June & will delay college for a year.

I feel that somehow this could lead to some major fund raising forthe EDNF. I was never very good at this & am looking for ideasfrom all of you. The show is opening in Wallingford Conn., on 2/6-2/10. I still don't have a complete confirmed list. The following isconfirmed & I will ask Leigh to post additional info as I get it. I dosuggest that you get tickets as quickly as possible if you want to goas they sell “like hotcackes”

2/14-2/16 .... Dayton, OH

2/17-2/18 .... Lexington, KY

2/20-2/22 .... Milwaukee, WI

2/25-3/1 ...... Atlanta, GA

3/4-3/8 ........ Phila./Camden, PA

3/10-3/12 .... Springfield, MA

3/13-3/15 .... Albany, NY

3/17-3/18 .... Huntingdon, WV.,

3/20-3/22 .... Memphis, TN

3/24-3/29 .... St. Louis, MO

4/3-4/12 ...... Radio City MusicHall N.Y.C.

Washington D.C., Massachusetts, Ohio, Michigan, Pennsylvania,Tennessee, Louisville, Chicago, Canada, Calif., Washington , Utah,Colorado, Texas & many more to come.

Any ideas or question’s, please contact me.Thanks for you help,Lisa


— Memorials and Honorariums —Donations received October 20, 1997 through January 20, 1998

Donations10/1/97 through 12/31/97

Supporters( $100 – $249)

Arthur & Beverly AltemoseKathryn S. ArnowWilliam Buckley

Center Stage CateringJan DavidsonPaul Doughty

Luba & Eugene DreyerKathy Dunn

Executive Exchange, IncMichael & Gloria Feigi

Michael & Mary Ellen FeigiAviva Goldstein

Chery & Wayne HeinmillerFrieda & Wilhelm Huber

Irving & Mae JurowJeanette & Robert KreiserEdward & Shirley MartinGreg & Shari McDonald

Mary Ann PhillipsRon & Robbin Ricci

Gabriela & Bob RamanowNancy & Louis Regas

Elizabeth RussellIrving Ryckoff

Susan & Glenn SchaferSylvia Serber

George & Alison SharpeRichard & Iris Slotkin

Alex White & Cody LyonVarooge Yerganian

Larry & Sandra ZentzRose N. Zeisel

Patron Donors($250 – $499)Alan Brutman

The Center for Oral &Maxillofacial Surgery

Ursula V. DanielJames J. Licari

Thomas P. Murphy, M.D.Tibor & Erika NeumannMr. & Mrs. Louis Rup

Jeffrey & Dorothy Samel

Silver Benefactor($1,000 – $2,499)

Nancy PotashPremier Manufacturing Corp.

Murray & Belle NathanIsabel White & Duffy White

Gold Benefactor($ 2,500 – $ 4,999)

Anonymous

Platinum Benefactor($ 5,000 or more)Harold Goldstein

In Loving Memory OfTomas Badik

Pam Badik

In Loving Memory OfWilliam Huber

Frieda & Wilhelm Huber

In Honor OfGeorge & Jo Ann’s

34th WeddingAnniversary

Ruth Carlier

In Memory OfDanny Carlier

Ruth Carlier

In Honor Of OurDaughter, Lisa

SchoenbergMr. & Mrs. Bernard Chapnick

In Honor Of MyDaughter, Sarah

Diana Hemenway

In Honor Of my sister,Meryl Brutman,My #1 SourcesOf Inspiration

Alan Brutman

In Memory OfNancy Hanna

RogowskiMr. & Mrs. David Lockhart

In Memory OfMark David ConnorStephen & Mary Ann Phillips

In Honor OfCarrie Cotter

Jan Davidson

In Memory OfJordan SpechtR.E. & Elaine Blouse

Doris Parrish

In Honor OfDarlene Clarke

Jane K. Clarke

Donald C. & Jackie Dawson(Premier Mfg. Corp.)

Margaret Howells

Joseph & Marilyn Vassel

In Honor OfMaggie Buckley

Sally & Chick Webb

In Honor OfMelissa Regas

Nancy, Louis & Emily Regas

Dennis & Christine Genge

Frank & Mary Cuenca

Mr. & Mrs. Bill Pappas

Drs. William & Barbara Rafaill

Mary Maher &Madelynn Rigopoulos

Jeanette Rigopoulos

In Memory OfSara Saltzman

Goldstein,Marni Goldstein

White& Callie Goldstein

Ursula Daniel

Gabriela & Bob Romanow

Herbert & Sylvia Malamud

Bernice & Ben Malamud

Beatrice Liebenberg

Leanore & Albert Hutler

Rose & James Goding

Mr. & Mrs. Ric Fleisher

Mr. & Mrs. Herman Travis

Luba & Eugene Dreyer

Kathryn Arnow

Richard & Iris Slotkin

Mr. & Mrs. Benjamin Haskel

Mr. & Mrs. Ted Seamon

Celia Levine

Michael Goldstein

Mae & Irving Jurow

Murray & Belle Nathan

Mark Levine

Harold Goldstein

Rose Zeisel

Sonya & Henry Hirschberg

Irving Ryckoff

Alex White & Cody Lyon

Mary & Gene Klaaren

Joseph & Kit Reed

Aviva Goldstein

Bruce & Irene Mackler

Jeanette & Bob Kreiser

Isabel Guy & Duffy White

George & Alison Sharpe

Peter & Theda Henle

Alfred Reifman

Eric Goldstein & Xiaorong Li

Jeanette Vosk


EDNF’s Non-Bowl, Bowl–A–ThonAnnouncing EDNF’s First Annual Non-Bowl Bowl–A–Thon. This is your opportunity to get involved and help EDNF and your localbranch raise funds without the risks of bowling. The object of this event is to get family, friends, co-workers and especially yourphysicians to sponsor you “Not toBowl”.

7 Reasons to get involved• It’s a way to get involved an help raise money and public awareness about EDS.• EDNF chartered local branches receive 40% of the proceeds when their members participate• It’s an activity that everyone can participate in• It’s a fun and easy way to raise money• Can you think of a better way to ask your physician for a donation• Earn an EDNF T-shirt and/or a tote bag for your participation• It’s an AMA approved physical activity for people affected by EDS

PrizesIf you raise $100.00, you will receive an

EDNF Tote Bag or Fanny Pack. (Colors Vary)

If you raise $200.00 you will receive anEDNF T-shirt. (T-shirts are available in S, M, L, XL and XXL)

T-shirts are available in Blue or Birch Gray.

If you raise $300.00 , you will receiveboth a T-shirt and a Tote Bag.

If you raise $400.00, you will receivea T-shirt, Fanny Pack and Tote Bag.

EDNF’s Non-Bowl, Bowl–A–ThonPlease complete the information below. If you are a member of a local chartered branch, please make sure to indicate which branch youare a member of, 40% of the money you raise will be sent to your local branch. Remember the object is to get people to sponsor you“Not toBowl”.

Name: ___________________________________________________________ Phone # _____________________________________Address: ______________________________________________________________________________________________________City: _______________________________________________ State: ______ Zip code: _____________________________________Are you a member of an EDNF Chartered Branch? ________________ Which Branch?: _____________________________________

Signature of participation, if you are under 18, a parental signature is required

_____________________________________________________________________________________________________________participant/ or parent

PLEASE USE ADDITIONAL SHEETS OF PAPER IF NECESSARY

Sponsor’s Address Amount TotalName Sponsored Paid

____________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________

EDNF is a not for profit corporation.All donations to EDNF are tax deductible and sincerely appreciated.

1997, Volume 12, Number 4, Number Payment

“Chronic Pain is a Manifestation of theEhlers–Danlos Syndrome”

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

1997, Volume 12, Number 2,“The Role of the Rheumatologist inEhlers–Danlos Syndrome” _______ _______

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



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

1995, Volume 10, Number 1,“Mandibular Joint, Orthodontic andDental Findings In EDS” _______ _______

1994, Volume 9, Number 3, “Genetic Information and Health Insurance” _______ _______

1994, Volume 9, Number 2,“Heritable Disorders of Connective Tissue andDisability and Chronic Disease In Childhood” _______ _______

1994, Volume 9, Number 1, Number Payment

“Official Launch of the Ehlers–DanlosSyndrome Database Manager” _______ _______

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

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

1993, Volume 8, Number 1,“Chronic Pain ManagementTreatment Facilities” _______ _______

1992, Volume 7, Number 4,“Perspectives on Pain Historyand Current Status” _______ _______

1992, Volume 7, Number 3,“Use of Mesh to PreventRecurrence of Hernias” _______ _______

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

1991, Volume 6, Number 3,“Ehlers–Danlos SyndromeType III and Pregnancy” _______ _______

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

1990, Volume 5, Number 4,“The Emergency Room andEhlers–Danlos Syndrome” _______ _______

Ehlers–Danlos National Foundation— Back Issues —

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(213) 651–3038

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Ehlers–Danlos National Foundation— Membership Form —

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(213) 651–3038

PLEASE PRINT and send a change of address, if applicable

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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 (500K to 750K in size)

over the Internet instead of through the Postal Service. (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

Canada & Mexico

❏ Individual Membership ........................................................ $30.00


International:❏ Individual Membership ........................................................ $35.00


Donor Program❏ Corporate Membership ....................................................... $ 50.00

❏ Patron Donor ...................................................................... $250.00

❏ Benefactor Donor ............................................................ $500.00+

❏ Corporate Sponsorship ....................................................$____.__

❏ Additional Donation .........................................................$____.__

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 andphone number will be released and ONLY to other members. All other information you provide to the foundation will remain strictlyconfidential. 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 : ❏ Check ❏ Money Order ❏ Mastercard ❏ VISA ❏ 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 tax deduct ible and is sincerely appreciated.


The Ehlers–Danlos National Foundation(EDNF) was created in 1985 in an effort toprovide emotional support and updatedinformation to those who suffer from thedisorder. In addition, EDNF serves as a vitalinformational link to and from the medicalcommunity. Loose Connections, the officialcommunications link of EDNF, is publishedon a quarterly basis. Subscriptioninformation may be obtained by writing theFoundation.

Ehlers–Danlos Syndrome (EDS) is a groupof heritable disorders of the connective tissueoften characterized by hyperextensible skin,hypermobile joints, easy bruisability of theskin, and a bleeding diathesis. EDS is namedfor two physicians (Ehlers and Danlos) whodescribed forms of the condition in the early1900s. At least six forms of Ehlers–DanlosSyndrome have been described, which are notgradations in severity, but represent distinctdisorders which “run true” in a family.

Ehlers–Danlos National Foundation

6399 Wilshire Blvd. Suite 510Los Angeles, CA 90048ADDRESS CORRECTION REQUESTED

6399 Wilshire Blvd. Suite 510Los Angeles, California 90048

Phone: (213) 651–3038 — FAX: (213) 651–1366Web Site: www.ednf.org — E–Mail: [email protected]

Loose ConnectionsLoose Connections

Together... We WillFind A Brighter Day

loose connections, february 1998

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