“Advocacy without

research is not credible.

Research without

advocacy is not relevant.”

Albert Einstein

WINTER 2008

L O O S E CONNECTIONS

e d n f . o r g

TABLE OF CONTENTS

WINTER 2008

ADVOCACY

Letter From the Board .........2

EDNF: From 2008 to 2009 ..3

Feature Articles ............... 4-6

From the Editor’s Desk .......5

Remembering Why I Write

Poetry ..................................9

And Your Little Dog Does,

Too! ....................................18

MEDICAL

Ophthalmology MRG ........10

Rescue of Migratory Defects

Of EDS Fibroblasts ...........11

MEMBERSHIP & NEWS

Board of Directors ...............2

Submissions Guidelines ......3

Donors ...............................14

Membership Form .............19

2009 EDNF COMMUNICATIONS THEME Advocacy: active support of a cause or course of action.

— Collins Essential English Dictionary 2nd Edition 2006 © HarperCollins Publishers 2004, 2006)

ADVOCACY IS:Speaking up for ourselves or for others;An attempt to change HOW THINGS WORK in order to change HOW THINGS ARE.

Reasons for taking charge of your situation and becoming a Self-Advocate:

In order for others to know that you disagree with them or that your rights have been disregarded, you need to — TELL THEM!!You are the expert on your life situation and what you think, feel and need. Don’t wait for someone else to speak out on your behalf; it may never happen!The people who have the authority to make decisions in your situation will think everything is OK if you don’t speak up for yourself.By speaking out, you may in fact be helping a lot of other people in the same or similar situations.You have a responsibility to yourself to take care of yourself. Speaking out will help you to keep your self-respect and dignity.— From “How to Become a Self-Advocate,” by Vedna McGill and Dorothy McKenna; funded by 

Status of Women Canada at http://womanspace.ca. 

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I am only one, but still I am one. I cannot do everything, but still I can do something; and because I cannot do everything, I will not refuse to do something that I can do.

— Helen Keller

Advocacy is the pursuit of influencing outcomes — including public-policy and resource allocation decisions within political, economic, and social systems and institutions — that directly affect people’s lives. (Cohen, 2001)

Therefore, advocacy can be seen as a deliberate process of speaking out on issues of concern in order to exert some influence on behalf of ideas or persons…. Advocacy groups are different from political parties which “seek to influence government policy by governing.” They are “any organization that seeks to influence government policy, but not to govern.” This definition includes social movements, sometimes network of organizations which are also focused on encouraging social change. Social movements try to either influence governments or, like the environmental movement, to influence people’s ideas or actions.

— From Wikipedia, the free encyclopedia

L O O S E CONNECTIONS

PUBLISHERS INDEX

Published Quarterly by

Ehlers-Danlos

National Foundation

FOUNDER

Nancy Hanna Rogowski

1957 – 1995

President & CEO

Cindy Lauren

Board of Directors

Robin Coppi

Michele Darwin

Richard Goldenhersh

Jeanne Kingsbury

Cindy Lauren

Charlotte Mecum

Professional Advisory

Network

Patrick Agnew, DPM

Peter Byers, MD

Edith Cheng, MD

Joseph Coselli, MD, FACC

Joseph Ernest III, MD

Clair Francomano, MD

Tamison Jewett, MD

Mark Lavallee, MD

Howard Levy, MD, PhD

Nazli McDonnell, MD, PhD

Dianna Milewicz, MD, PhD

Anna Mitchell, MD, PhD

Raman Mitra, MD, PhD

Linda Neumann-Potash, RN, MN

Terry Olson, PT

Mary F. Otterson, MD, MS

Melanie Pepin, MS, CSG

Elizabeth Russell, MD

Ulrike Schwarze, MD

Karen Sparrow, PhD

Brad Tinkle, MD, PhD

Mike Yergler, MD

Editor

Barbara Goldenhersh, PhD

Graphics/Type

Mark C. Martino

PAGE TWO • WINTER 2008

AS the year comes to a close I reflect back on all that has been happening at EDNF and am filled

with pride. The year began with a flurry of activity as we worked together to host the 17th Learning Conference in Houston. The first EDNF Kids & Teens program was an overwhelming success and I am proud to report that many of the friendships formed between our younger members continue today. These are the relationships that will enable our children to become spokespeople for EDNF.

The new MRG series including the Pain Management, Dentistry and Ophthalmology MRGs are allowing EDNF to educate the medical professionals with a renewed commitment to reach the medical schools and specialists with the most up-to-date information available to us from the greatest minds in research and treatment of EDS. The Pediatrics One-Sheet is successfully allowing us to begin a new initiative of educating the physicians who care for well children to look for the physical characteristics of hypermobile joints and skin/bruising issues that could be warning signs for an inherited disorder of the connective tissue like EDS. If we can educate physicians to keep an eye on these characteristics then we can look forward to more conversations with parents and more referrals to geneticists that can lead to early diagnosis.

As we begin 2009 with the theme of Advocacy, we can count on even more presence in Washington, D.C. Relationships with the Coalition for Skin Diseases (CSD) and the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), in addition to NORD and GenTAC, are strategically putting EDNF in the best possible place both individually and collaboratively with other

organizations working towards the same goals.

I was privileged to participate in a radio program on Sirius XM’s Doctor  Radio that we believe will be the first in many opportunities for EDNF in the media. The hour-long discussion with Dr. Claire Francomano and Dr. Stephen Honig (Director of the Osteoporosis Center at the Hospital for Joint Diseases) enabled EDNF to reach a nationwide listening audience and discuss EDS. Callers with questions came from across the country, including Rhode Island, Illinois, California and even Canada. The program was very well received. We were able to touch on the general definition of Ehlers-Danlos syndrome and the warning signs as well as some of the challenges in diagnosis and long-term concerns. Sixty minutes was not enough, but it did allow us to touch on the genetic component, some of the concerns related to bone and cardiac health as well as symptomatic treatment and special monitoring concerns for the vascular type of EDS.

A call to participate in membership was widely answered and we are seeing our membership grow which is the key to our future. Please remember that all that we accomplish at EDNF requires funds to produce and distribute. Membership and donations are what sustain us. Every dollar makes a difference and no donation is too small. Help us spread the word and share in our excitement at all we have accomplished and all that is in store for us in the future. We are making a difference and we will continue every day with our positive attitudes and proactive energy to make a difference of the lives of everyone with EDS.

Michele DarwinEDNF Board of Directors, Chair 

LETTER FROM THE BOARD OF DIRECTORS“§ positive attitude may not solve all your problems, but it will annoy enough people to make it worth the effort.” — Herm Albright

GUIDELINES FOR SUBMISSIONSTO LOOSE CONNECTIONS

For text documents, use Helvetica or Times of at least 10 points (preferably 12 to 16) in size, so our editors can easily read your document and prepare it for publication. Attach the text document in either Word (.doc) or Rich Text Format (.rtf) to an e-mail sent to editor@ednf.org that also tells us how to reach you for more information.

For photographs, attach them to an e-mail to editor@ednf.org; send three to five images that are at least 2400 x 3000 pixels (300 dpi resolution & 8 x 10 inches) and without compression beyond that per-formed by the camera when it was taken. In the e-mail, please identify the event or cause for the photographs, including any relevant iden-tification (persons involved, date, photographer’s name if needed) and how to reach you for more information.

Following are the deadlines for the next year of four issues; special arrangements can be made with the editors before these dates, but not afterwards.

PUBLISHED ON

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COLUMNS DUE

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Text articles, photographs, or any other submissions to Loose Connections are accepted only on condition that publication of that material is not under copyright or other restrictions on its publication. Ehlers-Danlos National Foundation reserves all and final editorial privileges, including the right to choose not to print a submitted story; submissions may be edited at the discretion of the editorial staff.

The opinions expressed in Loose Connections are those of the contributors, authors, or advertisers, and do not necessarily reflect the views of Ehlers-Danlos National Foundation, Inc., the editorial staff, Professional Advisory Network, or the Board of Directors.

EDNF does not endorse any products.

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L O O S E CONNECTIONSWINTER 2008 • PAGE THREE

EDNF: FROM 2008 TO 2009

It has been a strong but challenging year for EDNF. Our volunteers are really taking EDS awareness to the

forefront. It is due to their commitment and passion that we have been able to make these strides forward, creating a recognizable brand, one that immediately conveys EDNF ’s commitment to responsible, accessible and reliable information. They have further made some serious advancements in addressing the overall legal and legislative areas which will directly impact our members. These may not seem as critical as physician education, but have just as dramatic an effect once a diagnosis of EDS is achieved.

Please join us in thanking those who have made such important efforts on behalf of the entire EDS community.

§dvocacy: CSD Lobbying Day, Washington DC – Angel DenardiNIAMS/NIH visit – Michele DarwinMedicare Disability Waiting Period Coalition – Angel DenardiGroup to remove Medicare Caps for Chronically Ill – Angel DenardiSigned on to the Medically Fragile Children’s Act – Angel DenardiGenetic Nondiscrimination Act Signed CSD Vaseline Notepad project – Michele Darwin

Communication:Year Three for The Hinge – Barbara GoldenhershYear Two for the quarterly PAN Hinge – Barbara Goldenhersh, Claire Blanca, Elise MakhoulMRG, Loose Connections, updated wallet info card, web design – Mark MartinoPediatrics One-Sheet, Kids & Teens – Michele DarwinMRG mailing project – John Paul II High School, Plano, TexasModerator of web Boards, Doctors listing project – Liza Sauls

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Doctor Radio, Sirius XM Radio – Michele Darwin & Claire FrancomanoTriage query correspondence – Sue JenkinsEDS Survey – Stacey Le FevreIssues and Management of Joint Hypermobility manual – Dr. Brad Tinkle

Meetings & Conferences:Houston Conference – Conference chair, Michele Hegler, and committeeAmerican Assoc. of Family Physicians meeting – Greater San Diego GroupKids & Teens Program – Michele DarwinEDS Training Day – EDS Advocates of Northern CaliforniaLocal Group Liaison – Troy Winkels

Fundraising: “Triple It!”second year – Angel donorSeventh Calvary Award – Elliott & Sandy ChackEDS Sabal Foundation Golf Tournament – Jeanne KingsburyMarin Human Race – EDS Advocates of Northern California

This is an overview of the many ways that EDNF members are making a vital and crucial difference for themselves and for all who come behind them. Each and every person who becomes aware of EDS adds to our united voice. There are so many important activities like this that take place every day: sometimes in small groups and sometimes just one-to-one. But each event is part of the tide of more information and respect for EDS. With this momentum and the help and partnership of all of us, we can reach more, educate more, show more about EDS and what has to be done.2009 Going forward, we have some very exciting plans coming up for the next year. It’s important to remember that we

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(continued)

L O O S E CONNECTIONSPAGE FOUR • WINTER 2008

must confront the reality of EDS every day: yesterday, today and tomorrow. In the midst of all the turmoil of current events, we have to remember how EDS can define our lives and our future. The only way change can be made is with your help and participation, so be part of the change you seek, deserve and demand. Below are a few of the big projects on tap for 2009, and we invite you to join us as we continue to make strides towards greater awareness and understanding of EDS for patients, families, doctors and colleagues. Onward!

An expansion of the One-Sheet, for adults — a whole-body perspective for physicians to chart in achieving the best diagnosis. An expanded MRG project addressing Vascular EDS — an overview MRG and more in-depth attendant booklet. An exemplary committee of experts has been engaged to develop this information.The MRG project will continue its publication schedule.A premier fundraising event is being planned for May Awareness month.

Cynthia LaurenEDNF President & CEO 

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MeRRIAM-WebSteR defines advocacy as: the act or process of advocating

or supporting a cause or proposal. I might add to that definition the word “willingness” because, as I have discovered, all advocacy really is, is the willingness to find your voice and speak out on behalf of your chosen cause. The hows and whys are important too, but without willingness it’s just useless information.

Those of us with EDS have a built-in cause. We may not have chosen this cause, but it’s ours just the same. Finding your voice to speak out on legislation that impacts the EDS population is easy. If your own struggles are not inspiration enough, simply visit EDNF’s on-line message board and there you will find all the inspiration you need in the stories of your fellow EDSers.

So, in the spirit of full disclosure I must divulge how I got involved with advocacy in the first place. I mean, I’m practically a hermit, how do I get myself into these things?

Well, that’s easy, it was by accident. You see, when first asked by Maggie Buckley to help out with the EDNF Advocacy Committee, I said, “Sure! I’d love to do it.” All the while I was really thinking about the awareness aspect of EDS in general, not about politics, legislation and all that other “mumbo jumbo” I had managed to avoid understanding all these years.

Without willingness there is no advocacy, without advocacy there is no change.I had no idea who to advocate to or what, specifically, to advocate about.

When given the opportunity, I can talk about EDS all day long. I can be passionate about it and — on a good day — even articulate. I wondered, though, if my representatives would listen. Did it matter how passionate I was if no one cared what I was talking about? Then it occurred to me that of course they didn’t care, and it didn’t even matter that they didn’t care.

Is it even practical to ask someone to care about something they don’t understand?

What mattered is that I cared and, if I did my job, eventually they might care too.

So, although I have learned about advocacy through “trial by fire” and continue to learn all the time, the most important thing I can share is that it doesn’t take a particular skill to advocate. Sure, there are right ways and wrong ways to get your message heard, and some learning that takes about ten minutes and a little bit of practice, but all you need to know is that if you have the passion and the willingness you are already an advocate.

To join the EDNF advocacy team just drop me a line to: nmconnect@yahoo.com.

§ngel DenardiEDNF Member Volunteer 

WHAT DOES EDS ADVOCACY MEAN?

(continued from previus page)

The purpose

of life is a life

of purpose.— Robert Byrne

L O O S E CONNECTIONSWINTER 2008 • PAGE FIVE

It has been more than a month since the 2008 Conference, and I have finally been able to find time

to just sit and think about all that has happened. The Conference was great and I met a lot of new friends there. It was so good to see the people that I had met before again. The seminars were wonderful this year. As I sit here collecting my thoughts I can’t help but remember the lady from Wisconsin who said, “I look around and we all have EDS. I can’t help but wonder who won’t be here next time.” I think that sometime between all the seminars and the gala and excitement of being there we all had that thought and just didn’t say it.

The memorial room at the conference was done so well and lots of us visited it, so we all know what might happen.

AFTERMATH OF A WONDERFUL CONFERENCEEven I made a short visit and found it brought back memories that I wasn’t prepared to handle at the time. When this lady made that statement it was easy to see that no one really wanted to talk about it, the subject was dropped pretty quickly. Now, as I sit here writing this, that very same lady is in the hospital in ICU fighting for her own life. Shortly after she was admitted to the hospital I received a text message from her that said, “Fighting to see all of us outside these walls.” The doctors have said that most people would not have survived this long. This all tells me that she is not only fighting for her life, but is fighting for all of us, just to see everyone one more time.

I also remember the young lady from Alabama whose family had to sacrifice

so much to just save enough money for her to be at the conference. She has EDS, too, and is pregnant; she wanted to attend the seminar about EDS and pregnancy, to learn all she could before giving birth to her new baby.

October 1, 2008 Laurie’s conditions never improved. She passed away today at 5:55 p.m. She will be greatly missed.

Congratulations to Stephanie and her new baby girl, Lauren Elizabeth, who was born today at 2:03 p.m.

Troy WinklesEDNF Local Group Liaison 

From the Editor’s Desk Barbara Goldenhersh, PhD

WelCOMe to a new a d d i t i o n f o r L o o s e Connections. This ongoing

column will look at what has happened in and around EDNF and what is to come. It is from the perspective of a busy volunteer struggling as we all do with the many manifestations of EDS. Hopefully this column will provide you with insight into recent events and brief views into the future.

This has been an exciting year — the addition of new Medical Resource Guides, a radio interview on Sirius XM Radio, influence through EDNF on many of the issues so important to our membership. They include participation in NIAMS, input on requested changes on social security disability, committee to lessen the Medicare waiting period, push for care act in congress, participation in NORD drive to eliminate insurance caps for long term and chronic illness, the medically fragile children’s act,

facilitation of genetic research through GenTAC and NIA, and so much more.

None of the above would happen if EDNF did not exist and proactively maintain relationships with policy-makers, advocacy committees and groups. It is not simply a small group of people who make this happen, it is the very existence of the totality of our organization. The strength of our growing membership will help us continue to influence in so many ways the future care, treatment and understanding of those of us with EDS.

As I look toward the upcoming year and consider future communications, it appears natural that the theme for 2009 must be ADVOCACY. And as we look to advocate for EDNF, it is up to each of us to step up when we can to influence our care and treatment through advocacy.

How do we individually participate as advocates? We accomplish this through the act of influencing a person or group’s attitude toward the issues that affect us. And so, we must speak out and advocate for change!

We must consider how tough it is to lobby for the dollars, attention and research when the medical world still thinks we are “rare” and “don’t hurt.” We must convince pharmaceutical companies who don’t see a disorder that can make them millions from a pill. We must realize that media doesn’t want to see the average middle-aged EDS housewife and clumsy kids when they get more viewers with a guy who pulls his skin over his face.

Achieving our goals through advocacy will take the active participation of all of us.

Speak out! educate! Advocate eDNF/eDS! ■■■

L O O S E CONNECTIONS

At social events I used to find that question awkward to answer because I no longer

work outside the home, but the labels “homemaker” or “on disability” don’t fit comfortably. Now I introduce myself as a Volunteer Patient Advocate. Though I am comfortable with that label, others have a variety of images that they project on me relating to the healthcare industry. I understand that perception because I used to share that perception.

Healthcare professionals, especially doctors, nurses and pharmacists have the inside track on getting pain relief, right? Well, I used to think so. Then a doctor friend asked me to teach her how to be her Patient Advocate. That was the first time anybody had labeled me a Patient Advocate. Until that moment I had always thought of healthcare providers as Patient Advocates who consult one another to determine the best course of treatment for a particular patient. From my limited view, I believed that

they were the ones who did all of the research, ordered the right tests, administered treatment and wrote up the right paperwork to see that costs were covered. But here was this doctor friend of mine challenging my beliefs at the same time that she was telling me I had a skill that she valued. When I asked her what made her, a pediatrician, think that I could improve the situation, she answered, “You are a great Patient Advocate and I’ve seen you get results for yourself and others with doctors and legislators. I need you to help my doctor see me as

“WHAT DO YOU DO?”an informed patient instead of as an overreacting woman doctor.”

Since then I have given a lot of thought to what the job description for a Patient Advocate might look like. I have found Patient Advocates who are volunteers as well as those that are paid for the work. Some of them have earned degrees in business, a high school diploma or even degrees in healthcare related fields. Some have sales, performance, management or record keeping experience, but few have all of these skills. There are many strengths which are applicable, but not every Patient Advocate has these traits: multi-lingual, old, young, parent, spouse, friend, tech savvy, great writing skills, physical strength, physical endurance, coping with a disability, having overcome discrimination, etc. So there is no universal job description for a Patient Advocate.

Maybe there shouldn’t be a job description for a Patient Advocate, as many of the attributes that make them successful are intangible and immeasurable. The things good advocates have in common are that they are well informed on an issue or condition, passionate about their message and persistent in delivering that message in order to effect change. The targeted change for the Patient Advocates may have many forms: patient care, communication, education, awareness, fundraising, research support, legislation. The results of the efforts of successful Patient Advocates are sometimes big news:

creation of California’s Legislative Task Force on Fibromyalgia in 2008; the Governor of Wyoming signing a proclamation declaring September as Pain Awareness Month;

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the Montana Pain Initiative’s two-day pain summit with nearly 200 participants;passage of the Military and Veterans Pain Care Policy Acts; the Dallas Morning News 2007 story profiling how teenager Nikki Darwin copes with the painful consequences of Ehlers-Danlos syndrome;the Grand Junction Journal 2008 story detailing how Janet Kelleher overcame her Ehlers-Danlos syndrome challenges and walked away with the lead role in a local production of Reckless;the Wall Street Journal 2007 article about Barbara Calder’s difficulty getting treated for symptoms of Ehlers-Danlos syndrome despite having health insurance coverage.

Other successes of Patient Advocates are celebrated with far less fanfare:

the doctor who prepared a presentation on the Beighton Scale and Brighton Criteria as tools for diagnosing hypermobility symptoms for his clinic partners and staff;the grocery store clerk who learns to keep a pain diary which identifies patterns in pain spikes that his doctor is able to address effectively;the thousands of people who have signed up with the American Pain Foundation to receive legislative and media links so that they may click on links to quickly give input to elected officials on healthcare legislation;the Chicago mom who regularly checks ResearchAmerica.org to find out more about how her Congressional Representatives stand on healthcare related legislation;the woman in San Francisco who accompanies her sister to

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PAGE SIX • WINTER 2008

I introduce myself as a Volunteer Patient Advocate.

L O O S E CONNECTIONS

the emergency room to make sure every person coming into contact with her sister is aware of the potential for bruising and dislocations associated with Ehlers-Danlos syndrome;or, my doctor friend who learned how to prepare for an appointment by focusing on one issue at a time, which led to a diagnosis and treatment plan that returned her to full-time work in her specialty field.

The potential for being a volunteer Patient Advocate is within each of us. Ask yourself, “What have I done today to improve the quality of life of people with Ehlers-Danlos syndrome?” Ask yourself how your skills and situation may be activated to raise public awareness, provide practical information or promote research. Add your voice to others. Write a letter of introduction to your Senator, Congressional Representative or State Legislator asking them to keep your personal story (or that of a family member or friend) in mind as they make voting decisions. By taking any action that helps to remove barriers and increase access to effective pain management for even one person, we all win. After all, even healthcare providers and legislators are patients some of the time.

Maggie BuckleyEDNF Volunteer Patient Advocate 

Maggie Buckley  is a Volunteer Patient Advocate affiliated with the American Pain  Foundation,  the  Ehlers-Danlos National Foundation and the National Fibromyalgia  Association.  Prior  to  a disabling  injury  in  1998,  she worked as a Business Manager, Controller and Management  Consultant  utilizing  an undergraduate  degree  in  Social Work and an MBA in accounting. She lives in Northern California with her husband of 20+ years and their dog.

• ONe of the items most frequently requested by EDNF members is the

provision of a doctor referral list. For the last few years, names had been collected and noted via the media sections of the message boards, or noted as members discussed their doctors in thousands of posts. Last year, a section of the website was established called “Physician Directory” and the information posted all in one area.

This initial grouping of the names and contact information was a rough collection of doctors with varying levels of EDS awareness and expertise. While some are known experts in research, treatment and varying aspects of EDS, others were simply people who received a brochure at a non-EDS medical conference or an EDS CD-ROM through their hospital or medical school. The initial listing also has had a few search issues, making it arduous to search by specific parameters.

The volunteers compiling and monitoring the list are working to make the search features more

user-friendly. They will also be contacting doctors, to verify their information and willingness to accept EDS patients. We are also looking into the possibility of a rating or comment system. A submission form link will be added, where members may quickly send a new name with complete contact information, or submit change of addresses for those that have moved.

To enable this list to grow and make doctors available in more areas, we need your help! Please check the listings for any doctors you use now, and if they are already included, please check that their contact information is still current. If you see a doctor that is not listed, need to change an entry, or would simply like to comment on the treatment you received from a doctor, please email the information to EDSdrs@aol.com or send in the form below.

Again, this list is 100% member driven, so your continued efforts to educate more doctors is only half the mission! Once you have them educated, please remember to get them listed, so others may be helped. ■■■

HELP US HELP YOU:PHYSICIAN DIRECTORY

WINTER 2008 • PAGE SEVEN

DOCTOR’S NAME

SPECIALTY

ADDRESS

CITY/STATE/ZIP

PHONE NO.

FAX NO.

PHYSICIAN DIRECTORY RECOMMENDATION

MAIL TO: EDNF, 3200 WILSHIRE BLVD, STE 1601 SOUTH TOWERLOS ANGELES CA 99010, OR FAX TO (213) 427-0057

L O O S E CONNECTIONS

I AM so fortunate to have a terrific network of doctors here in Cincinnati, Ohio, but it did

not start out that way. Seven years ago when living in Toledo, I began having some strange symptoms. It started with my hands and feet burning. Then, I began having wrist and finger pain. It went from there to subluxing joints, widespread pain in my joints and muscles and extreme fatigue. I went to my primary care physician. She took some blood work which came back as a positive antinuclear antibody (ANA). She said I have a connective tissue disorder. The doctor sent me to the only rheumatologist on my health care plan. He took some x-rays and family history. His diagnosis was lupus and fibromyalgia. I went on some medication for lupus and guaifenesin (was used because of its uricosuric effect) for fibromyalgia treatment. Neither of these treatments were helpful, to say the least.

I ended up moving to Cincinnati for another teaching position. I went through doctor after doctor to find out what exactly was wrong. One of the many doctors was a

DOCTOR BRAD TINKLE:A COMPLETE DOCTOR SUCCESS FOR EDS!

rheumatologist. He did not think I had lupus but just fibromyalgia. I was treated as if I had fibromyalgia for months with no relief. I tried walking miles a day to supposedly “cure” the pain, but I ended up with my knees, ankles and hips constantly subluxing. I then tried swimming which left me with shoulder, elbow and wrist subluxing. Finally, my doctor noticed hypermobility in my joints. He had just taken a seminar on Ehlers-Danlos syndrome. He told me to see the geneticist at Cincinnati Children’s Hospital Medical Center. The geneticist was a specialist in EDS.

I made an appointment with Dr. Richard Wenstrup, the geneticist, Leah Hochstetter, the genetic counselor, and Dr. Brad Tinkle, the resident. They did a thorough examination which included: family history, a skin biopsy, assessed joint hypermobility using the Beighton scale, reviewed my symptoms and recent x-rays, MRI, echocardiography and blood work. The diagnosis was Ehlers-Danlos syndrome hypermobility type (formally known as type III). Dr. Wenstrup left his

position at the hospital but left a very competent, compassionate, understanding individual, and a leading expert in connective tissue disorders in his place.

Dr. Brad Tinkle now serves as director of the Skeletal Dysplasia Center, co-director of the Marfan/Ehlers-Danlos syndrome clinic, as well as director of the Connective Tissue Clinic. Dr. Tinkle has been my doctor for over four years. He has been such a support, very knowledgeable about EDS, connects and lends a hand with my other seven specialist doctors, helps with my disability paperwork, and always is available for consul-tation. He works very hard to inform the public about EDS. He has written a book, a best seller in my opinion, on Ehlers-Danlos syndrome called Issues and Management of Joint Hypermobility: A Guide for the Ehlers-Danlos Syndrome, Hypermobility Type and the Hypermobile Syndrome. The book completely explains EDS in an understandable language. It also has many references and websites for further information. Dr. Tinkle is also on the medical board of advisors for the Ehlers-Danlos National Foundation.

I recently went to an EDS social/support meeting set up by Dr. Brad Tinkle. It was an eye-opener. There are many of us EDSers out there, but not all of us have competent doctors or doctors who even know about or believe in Ehlers-Danlos syndrome. I feel very blessed that I have a wonderful network of doctors and an EDS expert who help make my life a little less difficult.

§ngela GriswoldEDNF Member 

PAGE EIGHT • WINTER 2008

Life LessonsIn the meantime, we can look clearly at our lives and the life around us, and Live. Before we start crying and praying to the Universe to take away our Trials and Tribulations, we might more closely examine what it has given us. Maybe the “good” things are tests, possibly rather difficult ones at that, and the “bad” things are gifts to help us grow: problems to solve, situations to learn to avoid,

habits to change, conditions to accept, lessons to learn, things to transform -all opportunities to find Wisdom, Happiness, and Truth.

From The Te of Piglet by Benjamin Hoff

L O O S E CONNECTIONSWINTER 2008 • PAGE NINE

I ReMeMbeR, as a kid, the feeling of riding my trusty ole bike, Holding tightly to handlebars,

as easy as pumping pedals for power.Balancing, fighting wind wobble, keeps it together, speeding down-hill, leaning in as a compacted cannonball.Relying on it like the promise of a best friend protecting my heart,While wind whip rushes into my ears filled with velocity.We pass the world, finally ahead of it, exhilarated, as one speeding bike-body until…The chain pops like my joints give way.And we come crashing down hard, exploding, cryingFrom pain-yes, but also from having been betrayed by a bicycle-body again.

As a young adult with EDS,The chain popped more frequently to disrupt my rides.Not just of steep, daredevil hills, but of mundane ones, too, like around my block and fun ones lost:Family outings on flat packed, gravel trails made for that sort of thing, but not for me.

Now as an older adult with EDS I can’t ride a bike at allAnd sometimes can’t walk without a cane, brace, or chair with wheels to roll me again.At least it’s electric (Boogie-Woogie) and I’ve learned to control it some with modification and humor.But miss being on the verge of almost loosing it, and still winning, in the end, with my bicycle-body again.

Shuddering, I recall the feel of chains ker-chunking, beneath me, inside of me, extending themselvesMisaligned from proper sprockets, too far removed as promises broken: my rebellious collagen no longer secures me, but makes me afraid to lean into anything anymore.

So it’s yard sale time. I scan my garage looking for items suitable for a drag to the curb and potential bargaining with customers.And instead I haggle with myself, counting memories stored, but covered in a thick film of dusty years gone by… …when I was more able bodied and could actually use this stuff… the bike I can’t ride, a kayak I can’t propel, and a fly fishing rod I learned to love before my back and shoulders gave way.And even swim gear. How I loved to swim: relaxing laps repeated by stronger windmill arms. Pressing on even when they ached,

REMEMBERINGWHY I WRITE POETRY

I knew I’d hit pain’s saturation point untilI’d glide more easily, almost effortlessly, in water, with water, moving through life itself as a stream of molecules at peace. And go on forever if I wanted, but now I water walk.It’s not the same, but the closest thing that comes to defying gravity pushing in.

And so I consider, but can not bring myself to sell dusty garage statues, and to justify the space they take.I’ll tell you that my wish is to pass on an inheritance to my kids —Besides the hand-me-down jeans — no I mean genes — of a hereditary connective tissue disorder.

But it’s years before they could use this stuff, if they ever will and really I’m holding onto unrealistic dreams for myself, still not ready to let them go. Will I ever?

Because I’m still the kid wobbling on a bicycle, wanting desperately to go all out, leaning in, and feel the rush of life whizzing byOr the swimmer-pushing beyond exhaustion’s brink releasing myself from pain to move effortlessly and easily again.

But I can’t.And the best I can do is explain it to you from a new body —Body of work, a body of poems, built from nouns and verbs as bones,Lines as limbs secure me now and then reaching out, in an attempt to descend a hill successfully, share messages and visions of hope, new meaning, and fresher perspective.Compounds and conjunctions now for joints I stand back up, a-lit with adjectives, dappled by the light and guidance of punctuation.Making sense, strikes a chord, my own gears turning once more,To express myself fast, free, and fearless, at least in words able to keep upright in the wobble, all the way past bottom, and beyond, gliding out, back to control again, with heart-pounding accomplishment and satisfaction, even in Loose Connections.That’s OK with poetry since stanzas with imagery and metaphor should be hyper-extended.

Charlene SwobodaEDNF MemberArtist/Poet (I can not help it!) living with EDS 

L O O S E CONNECTIONS

OPHTHALMOLOGY MRGNEWEST MEDICAL RESOURCE GUIDE RELEASED

the Ophthalmology  Medical R esource   Guide i s t h e newest EDNF publication

in the Medical Resource Guide (MRG) series. This MRG includes information about Ophthalmology and EDS, Baseline Eye Exam Recommendations for EDS, Signs and Symptoms for the EDS Patient, including when to seek emergency help, and Kyphoscoliosis Type.

The MRGs focus on a specialty and present an overview of the current medical information on EDS available in that specialty. For EDSers, the MRGs are an excellent way to introduce yourself to a new doctor, but have also proved to be personally useful and relevant — and

new warnings for Vascular EDS have been posted on EDNF’s emergency web pages in the past year as a direct result of MRG research and contri-butions by many members of EDNF’s Professional Advisory Network.

A free copy of the Ophthalmology MRG is available to EDNF members who mail a S.A.S.E. (self-addressed, stamped envelope) to:

Ehlers-Danlos National Foundation3200 Wilshire BlvdSuite 1601, South TowerLos Angeles CA 90010

Copies are also available through EDNF’s on-line store. ■■■

EDNF Reminder:We are often the educated ones on EDS. Go to your doctors prepared. Bring the information provided in the EDNF publications, articles, etc. and share them with a positive attitude. The medical community does listen when we offer professional materials in a helpful and respectful manner.

PAGE TEN • WINTER 2008

CAROTID-CAVERNOUS FISTULA: LIFE-THREATENING EMERGENCY

Emergency consideration should be given to any Vascular EDS patient who becomes aware of redness, pain and prominence of one or both eyes and the sound of pulsations in their head: this can be a manifestation of a life-threatening carotid-cavernous fistula.

In this emergency condition, high pressure blood from the internal carotid artery can pass directly into veins behind the eye, which shunts blood inappropriately into the tissue around the eyes and into the eye itself, thereby causing the presenting symptoms.

The greater risk is that the high pressure blood will leak out of the confines of the blood vessels and that could be life-threatening.

It is absolutely critical to seek immediate hospital-based medical attention, and to inform emergency medical staff of the patient’s Vascular EDS and the risk of a carotid-cavernous fistula.

NEW Vascular EDS Emergency Information

L O O S E CONNECTIONS

This  is  extracted  with  permission  from the  full  paper,  published  in  Journal of Investigative Dermatology  of August, 2008;128(8):1915-9. We urge you to read the complete text, available at www.ednf.org (Medical Professionals section under Medical Reports and Findings). To  respond, write editor@ednf.org. — Ed.

MutAtIONS in the genes encoding for type V collagen have been found in the

classical type of Ehlers-Danlos syndrome (EDS); the most common mutations lead to a non-functional COL5A1 allele. We characterized three skin fibroblast strains derived from patients affected by classical EDS caused by COL5A1 haploinsufficiency. As a typical clinical hallmark of EDS is the impaired wound healing, we analyzed the repair capability of fibroblasts in a monolayer wound. The mutant fibroblast strains were unable to move into the scraped area showing a marked delay in wound repair. In all the EDS strains, type V collagen was absent in the extracellular space, causing lack of fibronectin fibrillar network and impairing the expression of a2b1 and a5b1 integrins. (Integrins are cell surface receptors that interact with the extracellular matrix (ECM) and mediate various intracellular signals.)

The abnormal integrin pattern inhibited cell migration, whereas the migratory capability remarkably improved in the presence of exogenous type.

Ehlers-Danlos syndrome (EDS) refers to a group of rare inherited connective tissue disorders characterized by joint hypermobility, hyperextensible, elastic and fragile skin, tissue fragility, and impaired wound healing (Steinmann et al., 2002). In about half of the patients with a clinical diagnosis of classical EDS, there are mutations in either

COL5A1 or COL5A2 gene leading to a non-functional COL5A1 (Schwarze et al., 2000; Malfait et al., 2005).

Type V collagen is a minor component of tissues containing type I collagen, such as skin, bone, cornea, and blood vessels while playing a regulatory role in the extracellular matrix (ECM) assembly and contributing to the development of functional connective tissues (Chanut-Delalande et al., 2004; Roulet et al., 2007).

The pathogenic mechanism leading to classical EDS phenotype is poorly understood. Examined matr ix deposition in long-term cultures showed total collagen synthesis was reduced, and significantly fewer fibrils were organized as compared to control cells. Integrins expression is at least partly controlled by the matrix components and are involved in cell movement as well as in angiogenesis (Angiogenesis is a physiological

process involving the growth of new blood vessels from pre-existing vessels) and wound repair. By binding to a5b1 integrin it improves wound healing.

In this study, we investigated three COL5A1 haploinsufficient EDS fibroblast strains to evaluate their behavior in a wounding assay and their adequacy as tools for testing the effect on EDS cells migration of potential repairing therapeutics. We present evidence that the lack of a fibrillar matrix and an abnormal expression

pattern of integrins in these cells delay wound repair, which is restored by the treatment with exogenous type V collagen, but not with IGFBP-1.

Molecular analysis of EDS fibroblasts and ECM organization

Among the skin biopsies from patients affected by EDS routinely examined in our laboratories, we selected three fibroblast strains derived from children. In these probands, all the major diagnostic criteria for classical EDS were fulfilled and the clinical histories indicated a comparable phenotypic expression. In all patients, the electro-phoretic mobility of types I and III collagen was normal. In some patients, the amount of a1(V) chain associated to the cell layer was decreased, as seen by the lowering of the a1(V)/type I collagen ratio, whereas in P-32, a1(V) chain was undetectable The COL5A1 null allele test was informative for all three patients with at least one polymorphic marker. As previously observed for missense mutations, the fibrillar network of type V collagen was missing.

The results reported here highlight the pivotal role of type V collagen in dermal matrix organization and may contribute to explain the molecular mechanisms resulting from the deficiency of type V collagen and leading to classical EDS clinical phenotype. The different forms of type V collagen may have specific biological functions.

Wound assay in Ehlers-Danlos syndrome

Fibroblasts delayed wound healing. At time 0, immediately after wounding and washing of the scraped cells, the

RESCUE OF MIGRATORY DEFECTS OF EHLERS-DANLOSSYNDROME FIBROBLASTS IN VITRO BY TYPE V COLLAGEN BUT NOT INSULIN-LIKE BINDING PROTEIN-1BY SIMONA VIGLIO, NICOLETTA ZOPPI, ANTONELLA SANGALLI, ANGELO GALLANTI, SERGIO BARLATI, MONICA MOTTES, MARINA COLOMBI & MAURIZIA VALLI

WINTER 2008 • PAGE ELEVEN

(continued)

The results highlight the pivotal role of type V collagen in dermal matrix organization.

L O O S E CONNECTIONSPAGE TWELVE • WINTER 2008

EDS ON SIRIUSSATELLITE RADIO

ON October 29, 2008, Ehlers-Danlos syndrome took to the airwaves of Sirius

XM Satellite Radio. Doctor  Radio is heard continually on Sirius XM Channel 114 and presents medical information and real-life stories; doctors from New York University’s Langone Medical Center and guests talk with listeners who call with medical questions.

“EDNF was approached by Sirius’ Doctor  Radio to do a one-hour segment on Ehlers-Danlos syndrome,” said Michele Darwin, Chair of EDNF’s Board of Directors. Moderated by Dr. Roshini Rajapaksa, the session guests were Ms. Darwin, EDNF PAN member Dr. Clair Francomano, and Dr. Stephen Honig, Director of The Osteoporosis Center at The Hospital for Joint Diseases.

“I found the whole experience to be extremely positive,” Ms. Darwin said. “The host and producer were very helpful and committed to a fair and informative program that was an excellent format for us to raise awareness of Ehlers-Danlos sydrome to a national audience. Dr. Francomano did a wonderful job of speaking of the genetic component in a way that was easy for all to understand and we were able to cover many important topics, although sixty minutes were not long enough.”

The well-received session has been rerun many times since the live broadcast, and the feedback has been excellent. Ms. Darwin anticipates the success of the program will bring about other media opportunities in the near future. ■■■

(continued from previous page)

border between the scraped area and confluent monolayer was sharp in control fibroblasts, whereas in EDS cells, it was not so evident and cells appeared partly detached far beyond the scraping line. After 48 hours of incubation in the presence of low serum concentration (1%), control cells had migrated in the cellular area almost reaching confluence, whereas in EDS cells, the picture was substantially the same as at time 0: cells were very sparse, and even more disorganized than immediately after scraping and showed a clear delay in scratch repair.

IGFBP-1 stimulated the migration and scratch repair of control fibroblasts, but not of EDS strains. The inability of EDS cells to respond to IGBP-1 treatment was also confirmed in other ways. EDS cells were unable to respond to the protein and migrated even less than the untreated cells. Our previous data had also highlighted a restoration of ECM organization and integrin pattern when EDS fibroblasts were cultured in the presence of purified type V collagen. To evaluate the effect of exogenously added purified human type V collagen on scratch wound repair, fibroblasts were grown and scraped in a medium supplemented with 5 mgml_1 of type

V collagen, it remarkably improved the scrape repair of EDS fibroblasts.

Normal wound healing is a complex highly regulated process and requires several factors, fibronectin and its a5b1 integrin receptor among others (Greiling and Clark, 1997; Midwood et al., 2006). The downregulation of a5b1 in EDS cells does not trigger the correct signaling pathways. The choice of therapeutics to improve wound repair in classical EDS should therefore consider possible integrins involvement in cellular response. In fact, culturing EDS fibroblasts with purified type V collagen, which restores the ECM and the a5b1 and a2b1 integrins organi-zation, improves wound repair: the wound does not widen and the cellular migration is comparable to that of control cells.

In conclusion, this wounding assay may be conveniently used to test different molecules for their capability to rescue defective migration of cultured EDS fibroblasts. Fibroblasts from control and Classical EDS patients were plated and grown to confluence in complete medium supplemented with 5 mgml_1 type V collagen. After wounding (0 hour) with a rubber policeman, fibroblasts were allowed to migrate for 48 hours in the presence of 5 mgml_1 type V collagen. ■ ■■

L O O S E CONNECTIONSWINTER 2008 • PAGE THIRTEEN

Ehlers-Danlos National Foundation is pleased to have partnered with the hCard® to offer you a special

opportunity that will help us raise funds for our valuable programs, while offering you fantastic benefits!

Purchase the hCard® today for only $25, and 50% of your purchase will immediately benefit EDNF.

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Benefits You and EDNFEDNF Partner Phones for Good offers you a great deal on your next cellular phone, from any of America’s leading providers: AT&T, Verizon, Sprint, T-Mobile and Nextel. Not only will you have an amazing selection to choose from, and receive better pricing than traditional stores, EDNF will receive up to $50.00 (see website for details) for each purchase! When it’s time to buy your next phone, please visit Phones for Good.com.

L: LOCATION of the pain and whether it travels to other body parts.

O: OTHER associated symptoms such as nausea, numbness, or weakness.

C: CHARACTER of the pain, whether it’s throbbing, sharp, dull, or burning.

A: AGGRAVATING AND ALLEVIATING factors. What makes the pain better or worse?

T: TIMING of the pain, how long it lasts, is it constant or intermittent?

E: ENVIRONMENT where the pain occurs, for example, while working or at home.

S: SEVERITY of the pain. Use a 0 to 10 pain scale from no pain to worst ever.

“LOCATES” PAIN SCALE

L O O S E CONNECTIONS1 4

�ono�s, �emo��a�s &���bute�EDNF’s members are extremely grateful for the generosity of all our donors this year. Listings are as complete as we know as of December 8, 2008.

924 Gilman St. Project

AA.C.M.E. - N.J.Dr. Anne Acreman Sara AdamsKristin AlbyBarbara AllarJanice AllenJanice and Donald AllenLorraine and Daryl AmdurAmerican Automobile AssociationWilliam & Valerie AndersKenneth AndersonKenneth AntinJames AragonaVinginia AshenfelterAT&T United Way/Employee

Giving CampaignAtlanta Area Support Group

BPam BadikNoah BaermanSusan H Bailey and FamilyBudd and Susan BailinAlice BakerBaker, Romero & Associates

Insurance BrokersLiam BambrickPhilip BarryMarilyn BatesonMarvin and Judith BellinDolores and Robert BenedictBarbara BergfelderBess Race ElementaryArleen and Peter BestMike and Beth BiersackNatalie BirrellThe Blake FamilyThe Blanchard FoundationJeff and Lisa BliliePhyllis S. BlockTim and Christine BlueRobert and Janice BolubaszIris and Peter BonnettBernard Bovee Joanna BradleyJacalyn BrecherBristol-Myers Squibb CompanyDavid and Beth Brizel

Brothers of the Christian Schools of Manhattan College

Dianne Brown Marlene BrownellRoger Bryson

CMichael and Susan CabnetPenny CacciatoreNancy CacciatoreCalifornia Community

Foundation Endowment Account

Carnegie-Stout Library Staff Committee

John and Nancy CarrShelley and Steven CarvelCenter for Volunteer and

Nonprofit LeadershipGisele ChaghouriSarah CharamugaRich and Nancy ChenaultAnthony and Diane ChericoChicks Softball Team - SBMSASasha ChicoineMarc ChuneskyRobert and Mary Jane CipcicRoberta CipcicEunice ClausingJeanne ClevelandBob CohenBeth and Merritt ColeHelen and James ColeSandra and Lawrence ColenColfax LLCRonald and Karen CollienCommunity Foundation For

Southeastern MichiganThe Community Foundation of

Louisville Depository, Inc.Community Health Charities

New EnglandCommunity Health

Charities of CaliforniaConstellation Energy Group

Employee FundCatherine ConteySharon CohenRobin and Steve CoppiRobin Coppi and FamilyCoram, Inc.Barbara Ann CostelloDebbie CostiganPat and Kate CotterMarie CoutureAmy Lynn CreaneyM. CrossRobert Crout

Chase CrowsonKaren Cyford

DMary Daley Dallas-Fort

Worth EDS GroupDebbi DamicoMichele and Scott DarwinSyble DavidMarian DavisTim and Susan DavisDeacon Charitable FoundationEudora and Alfonso Dell’aglioDawn DeWeinSarah DewsSusan DionBarry and Karen DixMarcia J. DobbsTeresa DohertyBrian DolanTracey DonaldsonMargery DonovanTerry and Penny DossLisa and Walter DubianskyJohn and Margaret DubretJohn B. and Dorothy T. DuncanMike Dyer

EHal and Pegi EckerEDNF - Northern VA Group EDS Sabal FoundationEhlers-Danlos National

Foundation Atlanta Area Support Group

Hila ElanThe Eliot and Sandra

Chack Foundation Monicia Elrod-EricksonRuth & Family Espinosa-BaroneDavid L. and Karen J. EvansTom EwertArlene and Steve EzrolKerry and Tamera Ezrol

FCheryl FalaKathy FallonThe FarwellsEmilia and Ronald FasanoJoyce FeaselShane and Jami Feasel, Charles,

Deb, Rhonda, Chris, Virginia and Shawn Halcomb

Lillian FeingoldEllen FeldmanLeigh FellnerSteven D. FeltesSeymour Darlene Ferley

Carol FillmanPaul FlinkstromJanis FoleyMargaret FooteJanice FormanCindy FoxAnnette FriedmanBrooke FriedmanFrank FriedmanPeggy Holden FultonFunding Factory

GAmanda GableShari and Sherman GamsonEsther GelbardMarc GerberAlbert GershowitzAmy GersteinGibbons P.C.Frederick GiddyRobert and Judy Giddy-KingJuliana and Terrence GilheanyVal GitlinGlaxoSmithKline FoundationJennifer Steidl GlenEliot GoldbergJay A. GoldbergBarb and Rich GoldenhershRicard and Lucille GoldsmithToni Thoman GomezGood SearchVal and Diane GordyFred and Aviva GorsteinRobert and Angela GrahamLisa W. GrayGRC Wireless Greater Houston BranchGreater Houston Group of EDNFGreater Lowell Community

FoundationThe GreenesMelissa GrommeshGroup of Greater San DiegoMaria Guenther

HPaul and Randi HagermanPhyllis Pierce HaleBeth HalevySean HamiltonHampton FamilyJ.D. HamptonJames T. HanleyBarbara and Ben HansenJane HansenMeg HarmonMercedes Harkins

1 5

�ono�s, �emo��a�s &���bute�L O O S E CONNECTIONS

Barry HarrisMyrna Harris - BMMSJoy Den HartogDavid and Goldie HechlerMr. & Mrs. Brad HenkeFrancis HenricksSandra HenricksJerry T. Herring, D.D.S.Janet HertzbergBarbara HickersonSteven and Carolyn HintzRosemarie Hobart Michael and Nancy HollowayBarbara HomonAlan HorowitchHoward Supply CompanyMichael HubbardSusan and Charles HurowitzGrace Ann Hutto

IiGive.com Holding LLC

JJoan L. and Tom E. JacksonDonna JacobsenJava Joe’s Roasting Company, Inc.Denise JermanJerry and ShirleyCarol JohnsonGilbert JohnsonJohnson&Johnson Family

of CompaniesBob and Linda JonesJeanne JonesJudy, Stan, Ted and SusanKimberly JonesKimberly Sue JonesMonica JonesSara Jones Lisa Juncaj/Manhattan College

KKaiser Permanente Community

Giving CampaignRachel Ann KarwowskiAllen and Annette KatzRachel Kaufman Dean and Patricia KehlSean and Nichole KellyMark KennisonKevin and Alice KerriganMichael and Terri KeyesCaroline KimseyGrace KingChuck KloseDeborah KnowltonSheila and Bob Kohn

John KopolowSharon KorneliMatt KoschakAlicia KratochwillMonica Ellen KrauseNeil and Maureen Kurtz

LThe LadermansLoretta June LanctoBarbara LanganCharles LangelandArlene LannJoyce LarsonSheena Anne LaShombMary LaSpinaContance LavalleeGerri LeachLemonade PiMaillie McCarraher LennonPhyllis LeutzDana LevenbergJessica LevineRuth LichterMrs. Francis LinglePam and Mike LippertLisa LittsDarcy Lis-BeglaneLiz Claiborne FoundationDavid and Nell LockhartLorron Department StoreKeith W. LoweLawrence LoxtermanPearl LoxtermanLubrizol FoundationJane Lyons

MMichael and Nancy MahoneyNancy MahoneyElise and Pierre MakhoulThe Malcolm and Virginia

Crawford Charitable FundRobert and Elizabeth MaliniakChristine MalinowskiDenise MarkeyMartin and Esther MarinTherese MasseyMary & Joe Mastro and

Melanie Mastro Burch Susan MathesAdrienne MayerErin and John McDonaldMcFatter Technical CenterMr. & Mrs. William McGinnisKathleen McGuireDorothy and Ed McInerneyMcClernon

Charlotte MecumThomas and Patricia MeyersMedco Employee Giving

CampaignErik MeidlNancy MelicanJoe MenasheJeanne MezakWilma MikeskaGeorge and Silvia MikkolaCatherine Mikkola-SlackLeila MillardCheryl MillaySheryl and Scott MilsteinChristopher MinixNicole Monteforte Geri MoranKathleen MoranClaudia, Rich and

Gabrielle MoravecMorgan CountyMorgan Federal BankRoss Moskowitz Rick & Laurey MouledousDeborah Musher Esther and Hope Myers

NEric E. NagleMelissa NappiStephen NashNational Fuel Gas Distribution

CorporationKelley NelsonLinda NelsonNetwork for GoodNew Mexico Connects

EDNF GroupJoseph and Ellen NiemannJenny and Jeff NoelPatricia Nolton

OBeth OlsonSusan Oricchio and FamilyTheresa A. OrsognaThomas OshimoMarianne Kathleen Ozmun

PShelly PackerRichard Pallaria David and Ruth PardoeAlan Perry and Karen Jill BrutmanCheryl PalmersheimLois and Ralph PattonAndy and Mila PaulDavid and Jane PenceSusan Penchansky

Nancy Sue PennPerryman-Bucy weddingPfizer Matching Gifts ProgramPiedmont Triad BranchClaudia Pine-Simon and

Mickey SimonMargaret PippinAlex and Mary PissalidisJudith PlaskowKevin and Alison PoeJennifer PohlmanRichard and Susan PondMarie PradaElinor Prior Pauline PurathStanley W. and Julie

Leininger Pycior

Q Gerald and Kathleen Queenan

RMargaret Raffa Joseph RandazzoRed River Farm NetworkNancy ReelingThe Reich FamilyArlene ReinhartKristy RellaHenry and Barbara RidgelyNicholas RidgelyPaul RinkertBarbara RobertsonMary K. RochfordScott Roecker and Jen McLishZona Rosan Gary RudnitskyNoah RumpfElizabeth RussellRobin, Jeff, and Lia RutbergRuth Tupler Revocable Trust

SIris Sachs and Michael

David GinsbergRosemary SackettMichael SampsonCatherine SchafmanSan Fernando Valley

Branch of EDNFMimi SandlerFelice SarcinoJohn SarneseRachel SaylerCarla Scharf-EwertSorella Schiller Laverne SchneiderDawn Poley SchulmanKen and Joan SchultzDenise Scobee and Michel Farag

L O O S E CONNECTIONS

�ono�s, �emo��a�s &���bute�1 6

Heather SelmanPeter and Christina SerraMrs. Sue ShannonSusan M. ShannonRegan ShawDina and John SheaPatricia SheehyRita Shetzich Betty and Loeb ShustermanSilver Strong & AssociatesAndrew SilvermanHelen SilvermanScott SimmonsMargie SimonEllen and Rob SimonsJoyce SklarJoan SloanJudy Sloan and Bruce KatzDavid and Doris SmithPamela SmithSheila SmithLori Snow Leslie SnyderJudith SobelThe Soldiviero FamilySolis Martial ArtsPhyllis SpectorAnnette and Stephen SpiegelDave and Deb SprecherJean StanleyMike And Rhona SteinElaine SteinfeldJoy SteinfeldDan SteringMom and Dad SteringStewart and StevensonDon and Sue SwensonShannon Schwerdtfeger

TLloyd TakataKatie TaorminaEllen and Howard TaroffFlora TassoneDoris Horn TeeMeg ThirerMarjorie ThompsonBarbara TierneyKenneth TigarSpencer TimmTLC Legal Services DivisionJoanne E. TownsendJulia Ann TrempTuckabee Elementary SchoolGlen and Cynthia Magalian TuplerDavid and Jodi TuplerMarjorie Turetzky

UJeff UeckerJudy UmbeerUnited States TreasuryUnited Way of Central and

Northeastern ConnecticutUnited Way of King CountyUnited Way of New York City

Designation AccountUnited Way of the Bay AreaUnited Way of Special DistributionUnited Way Special

Distribution AccountMark and Kim Urban

VVaccaro FamilyStephen and Harlene ValentiMaria and Ozzie VannucciPaul and Helen VarnerVehicle Donation

Processing CenterThe Verizon Team of Randy MostyDebby Villasenor

WDebbie WaggonerAnnette WagnerHope WalkerKristin WarfieldStephen and Rona WarnerVirginia WarrenState of Washington Anne WebsterNicole WeepieRoss and Virginia WehlingMatthew and Brigid WeillerJoan and Howard WeinsteinMichael WeinstenIrene WelchWellpoint Foundation/

Peter N. BowersBarbara and Loren WentzLettie and Steve WerbelRobert and Rosetta WesterWestern CT EDS GroupMina WesthoffRhonda WestoverSusan and Tom WetjenElizabeth WheelerCharles and Irma WheelwrightLaura WhittRoy and Laura WidmerSuzanne WillisGrace WoodruffKim WoodsCathleen Wynmore

The myriad tasks of Communications Director could not be accomplished without

the help of many. A huge thank you to:

Claire BlancoLara Bloom

Maggie BuckleyMichele DarwinAngel Denardi

Ross EntredichoMags Foote

Michele HeglerDebbie Ignacio

Sue JenkinsOlivia KennertCindy Lauren

E. (Lisa) Mahkoul

Mark MartinoAli RuffaloLiza Sauls

Charlyne SwabodaLani Wilson

Troy Winkles

Those who populate our message boards

And the many doctors who inform

us, learn from us, and grow with us

This isn’t a party trick.

Barbara Goldenhersh, PhDEDNF Communications Director

www.ednf.orgF O R M O R E I N F O R M AT I O N

L O O S E CONNECTIONSWINTER 2008 • PAGE SEVENTEEN

A total of thirty subjects were enrolled during the meeting.

Twenty-five were adults and five were children under the age of seven years (two children were present and were enrolled at the meeting; a set of three siblings were enrolled in absentia by their mother).

Two adults and three children were male, and the other 25 enrollees were female.

Twenty-four enrollees were American and six (all adults) were international.

At least 25 enrollees had VEDS confirmed by gene analysis and/or skin biopsy. One person’s genetic test is in progress following a clinical diagnosis of VEDS. Three people

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GenTAC AT EDNF CONFERENCE: A REPORThave another subtype of EDS, but have aortic dilation or aneurysm, or other significant aneurysms.

In addition, four of these people also joined Dr. Milewicz’s local study to see whether skin cells can be found in the bloodstream, or grown from cells circulating in the bloodstream.

This is the equivalent of almost seven weeks’ worth of recruitment, so it was a huge benefit to GenTAC to come to the meeting.

We would also like to say thank you to all the enrollees for joining the study. We couldn’t do this kind of research without your participation!

If you did not receive a copy of your consent form, please contact me so that

�

�

I can send you one. And please don’t forget to mail back your questionnaires; some people have returned the Form 2 and Form 3 for GenTAC, but we would love to have the rest of them back as soon as possible.

Claire NollMS, CGCCertified Genetic CounselorUniversity of Texas Health Science Center- Houston, Division of Medical Genetics6431 Fannin St., Room 6.100Houston TX 77030e-mail Claire.Noll@uth.tmc.edu 

PARTICIPATE IN RESEARCH ON EDS

Do you have a form of Ehlers-Danlos Syndrome?

Help support EDS research by participating in an on-line research study aimed at evaluating your personal experience of having EDS and the impact it has on your daily life.

My name is Stacey Le Fevre and I am the primary investigator for this research project. I was diagnosed with Ehlers-Danlos Syndrome, Hypermobile Type in 2003. I am currently working on my doctorate in clinical psychology at the University of Indianapolis and this research is being conducted in partial fulfillment of my doctoral requirements. Although research on the medical aspects of EDS has increased dramatically over the past decade, little attention has been given to how EDS affects the lives of those of us who deal with it on a daily basis. The aim of this research is to gather your collective experiences and better understand what it is like to live with the different types of EDS.

To participate you must be at least 18 years of age and have EDS. The questionnaire is 106 questions and will take approximately 30 minutes to complete. Your participation in this research study is voluntary and no identifying information will be asked. Go to the EDNF Message Boards and click on Medical Boards then Research select the topic “Online EDS Research”, click the link to the study and it will take you to the survey website. You will be asked to enter a password to enter the questionnaire (this is for test security). Please read through the information page in the beginning and check the box that you are giving your consent to participate. If at any time you have questions or concerns, please do not hesitate to contact me.

Password: EDSResearch

Thank you for your time and participation!

Sincerely,

Stacey Le FevreResearch InvestigatorUniversity of Indianapolislefevresr@uindy.edu

L O O S E CONNECTIONSPAGE EIGHTEEN • WINTER 2008

We first spotted her when she dashed in front of our car late one night. So we

did what often works with strays, opening the car door and calling, “Go for a ride?” She jumped into daughter Heather’s lap like she belonged there. We spent two months looking for her owners with no luck, so she’s been Heather’s permanent lap accessory for seven years now. Due to her tiny size and overly hairy feet when we found her, Heather christened the new addition Hobbit.

Matted and dirty, she resembled a yorkie, mixed with poodle or maltese. Any attempt at grooming her fur resulted in hideous screams…from us, as she bites hard! I’ve groomed professionally for ten years, and this tiny six-pound mutt was the worst behaved I’d seen. Our only choice to make her look — and smell — better was to shave off the long coat.

As I held up a leg to remove the matted belly fur, I felt both knees and hips pop, much like my own. I noticed that her skin-folds in many places were as long as the hair, making it hard to give her any sort of cute, fluffy haircut without performing an accidental facelift at the same time! Uh-oh; could this mean little monster have some health issues?

Piles of fur built up on the floor, until the thin, scarred body was uncovered. Instead of the Swiffer-gone-moldy look, she now sported the skinned-rat motif. Heather prefers to call it her “Kate Moss Look” due to a resemblence to the pale, overly skinny model. When people ask where we get her hair cut, it is so they can avoid that groomer. We don’t see her as ugly…but we get to explain why her health makes us choose practicality over fashion!

AND YOUR LITTLE DOG DOES TOO!Making herself at home quickly, Hobbit made it clear she needed special treatment. When she first saw my mobility scooter, she begged for a ride on my lap, so she may have been “grandma’s dog”…and if Grandma was gone, family might have allowed her to “wander off.”

Her teeth are so scarce, she needs special foods. She has more allergies than I do, and can’t eat too much or too fast or she’ll get sick. She can barely hear, she can hardly see, she tires easily on a walk and needs to be carried home. Only the softest pile of pillows will do for a bed. If the other pets try to play with her, she ends up with bruises, wide scratches and scars badly. Any jolts, missteps, jumps on or off furniture, and she’s got a limp or a joint out of place. But the issue that worried us the most was a golf ball sized hernia that hung below her belly when she first came to us!

Within days of her arrival, we had her at the vet for a checkup, and he said she had “old age issues…but she’s probably only four to five!” He found she has a heart murmer, cataracts and all her hip and knee joints are badly worn. The hernia concerned him, but he was afraid she wouldn’t heal well if she even survived the operation. Her

loose skin is as thin as tissue paper, her insides aren’t where they should be. He did find us a specialist who finally did fix it, but it is beginning to sag again four years later, and he doesn’t think it can be repaired again at her age (now over eleven).

Our wonderful vet of many years did shake his head in wonder, saying this dog knew where to run away to! Modifying a famous quote from a wicked witch, he laughed and said “Your family has EDS — and your little dog does too!”

Liza SaulsEDNF Member & Board Moderator 

PHOTO RIGHT TOP:The “Kate Moss Look”

PHOTO RIGHT: Hobbit

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WINTER 2008L O O S E CONNECTIONS

THIS EDITION OF LOOSE CONNECTIONS IS COPYRIGHT 2008 BY EHLERS-DANLOS NATIONAL FOUNDATION. PHOTOGRAPHY/ARTISTIC CREDITS: FRONT & BACK SEASON HEADERS Detail of Door © 2007 Tony Hayes; FRONT COVER & PAGE HEADERS Footway in Snowy Woods © 2007 Andrey Stenkin; PAGE NUMBER HEADERS & PAGE 12 Dallas Sky © July 2008 Mapps (Michael Mappebeck) & richardphotos (Richard Gorden); PAGE 16 Hands © 2008 Michele Darwin; PAGE 18 Photos © 2008 Liza Sauls; BACK Black-and-white reproduction of color sketch by and © 2008 Charlene Swoboda from her upcoming book Our Family’s Special Beach Trip