SPRING 2007 “The best way to predict the future is to create it.”
Peter F. Drucker
L O O S E CONNECTIONS
(1909-2005)
SHARING OUR GOLDEN THREAD STORIES
TABLE OF CONTENTS
We introduce you to Ellen Smith. Her biography can be found on page two, with more of her pieces in issues to come. We believe they will speak to you as they did to us.
SPRING 2007
What Is EDS?
FEATURES
By Ellen Smith (10/29/2006)
EDS Every Day is a Surprise Each Day you Slip Energy is Depleted Seriously Everyone Dreads needing Scooters Easily you Dislocate and Sublux Equilibrium Desperately Slips Episodes of Divorce and Separations are common Effective Daily Strengthening is needed Efficient Diagnosis is Stressed Employ Demeanor Sheathe to protect from judgment Empty Dampened Spirits Enormous Dreams for Solutions are helpful Evaluate Desperate Situations Emphasize Digestive Supplements Escape Dangerous Situations Each Daughter and Son has a 50% of having it Education is Desperately Suggested Educate, Debate, Scholars needed EDS Ehlers Danlos Syndrome Everyone Dreads the Sacrifice Each Desires Simplicity E D S . . . easy to describe, sad to live with
Is the Switch On or Off?
By Ellen Smith (10/25/2006)
On — Light Freedom Carefree Happy Dreams Future
Golden Thread Story..... 1, 2 Studies in Sleep................. 4 Making the Most of Your Doctor’s Visit.................... 6
MEDICAL Ehlers-Danlos Syndrome, Hypermobility Type........... 9 Update on the EDS Dog Model 2006..................... 11
Off — Pain Disorientation Discouragement Loss Uncertainty Chronic illness
MEMBERSHIP & NEWS
So, what is it today? Is it on or off? If only we could have control I would switch to the on position forever
Local Groups................... 14
EDNF Partnerships........... 3 Sports................................ 5 Reviews......................... 7, 8 Kids & Teens................... 12 News......................... 12, 13 Membership Form........... 15 COMING NEXT ISSUE: Understanding the Social Security Disability Application Process
www.ednf.org
L O O S E CONNECTIONS PAGE TWO • SPRING 2007
PUBLISHERS INDEX
SHARING OUR GOLDEN THREAD STORIES (cont’d.)
Published Quarterly by Ehlers-Danlos National Foundation
Laugh With Me
Ellen Smith was suddenly visited by her Creative Muse last year and continues to write prodigiously. She shares her thoughts, her fears, her concerns on having EDS.
FOUNDER Nancy Hanna Rogowski 1957 – 1995
Inside me is a balloon that is filled Do you wonder what I have filled it with? There is passion for life There is compassion towards others There is love that is beyond words, Love towards my husband, my children, family and friends There is so much in this balloon that I have enough to cope, So, please learn to laugh with me
went to the reservoir in October and decided to try to block out all the stresses: missing teaching again from over a fourmonth recuperation, extreme waves of exhaustion and throat and chest pressure from the surgery and sarcoidosis, one let down after another. I tried to block it all out and just enjoy the moment with my dogs. It was then that I suddenly had this weird experience — this poem came into my head and I felt compelled to sit down and write the words out. Keep in mind I don’t choose to read poetry; I gravitate to reading history (I’m an eighth-grade Social Studies teacher), yet this need was there to express these ideas coming out of my emotions. A very odd and exciting experience.
President & CEO Cindy Lauren Board of Directors Richard Blouse Robin Coppi Richard Goldenhersh Terri Keyes Jeanne Kingsbury Cindy Lauren Charlotte Mecum David Specht 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 Nazli McDonnell, MD, PhD Dianna Milewics, MD, PhD Anna Mitchell, MD, PhD Raman Mitra, MD, PhD Linda Neumann-Potash, RN Terry Olson, PT Melanie Pepin, MS, CGC John Reeves, II, PhD Elizabeth Russell, MD Ulrike Schwarz, MD Mike Yergler, MD Editor Barbara Goldenhersh Layout & Graphics Mark C. Martino 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.
By Ellen Smith (1/28/2007)
As I walk with my leg braces trying not to catch the inside bars between my knees to prevent falls, Laugh with me At least I have these braces to help me I still experience the privilege of walking As I try to adjust to my collar that goes from waist up, Laugh with me At least I have one last option to try to prevent more instability of the neck The last resort is surgery I would rather not have to learn to walk again and have the neck Fused So, laugh with me as I learn to tolerate this As I announce my next surgical stabilization Laugh with me At least it gives me hope for better quality of life And someday, Laugh with me when I look into your eyes knowing you well But I forget your name Laugh with me, please Know it’s the disease, not the love I have towards you. So, laugh with me as I progress My life is good For I have that filled balloon, It helps me cope
I
I am 56 years old and wasn’t diagnosed until 2004, after needing a second bladder repair just two years after the first. That smart doctor suspected EDS and sent me off to the geneticist before doing the second, and then nineteen days later, the third bladder repair. I have since seen three geneticists: one in Rhode Island, one at Tufts University and recently Dr. Claire Francomano. Since my diagnosis, I have read and researched constantly. The EDNF web site provided me a great deal of material to review and allowed me to learn from others through the message boards. Moreover, through this, I have realized I must set a goal to at least make things better for all of those with EDS, including my four sons, each of whom has a 50 percent chance of having inherited EDS. I finally became part of the NIH research and met with Dr. Nazli McDonnell in April this year; she suggested seeing Dr. Francomano. For a long time, I knew something was really wrong, but looked good to everyone else, as always. Strange things kept happening despite eating well, exercising consistently, and being happy with my life. My muse has remained with me and I have written over thirty poems since I began this recent journey. I have now resigned my teaching position and will continue my journey, accepting the muse as long as it remains with me, and working to make a difference in the future for all of us who share this disorder. ■■■
L O O S E CONNECTIONS SPRING 2007 • PAGE THREE
VASCULAR COMPLICATIONS? EDNF PARTNERS WITH GENTAC
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eginning in December 2006, EDNF entered a research partnership with the Genetic Alliance, the group representing over 600 organizations that are working together to create quality educational resources, build research networks across the U.S. and to educate the policy-makers in the halls of Congress about the prevalence and need for research in genetic disorders. There is a dedicated registry, Registry of Genetically Triggered Thoracic Aneurysms, or GenTac, that is being started at five centers in the U.S.: Johns Hopkins, University of Texas, Cornell University, University of Oregon and University of Pennsylvania. This registry will begin with tissue samples from about 4,000 patients and the goal is for each center to accrue about 200 patients per year. Enrollment will begin this spring once the final application form and institutional review board approval is completed.
Patients with vascular EDS as well as other EDSers with aortic dilation will be included. Other diagnoses that will participate include Marfan, Loeys-Dietz, Turner, non-specific connective tissue disorders with aortic involvement and familial aortic aneurysm. Vascular EDS patients that have certain genetic mutations but without aortic dilation or dissection will be included, as well as both hypermobile and classical EDSers with aortic complications. This will be an important opportunity for patients to advance research and understanding of these related conditions. Nazli McDonnell, M.D., Ph.D. attended the steering committee meeting as an essential voice for the EDS community; she supports this two-year program and its follow up. We will keep you informed as developments occur, and we urge you to share this with your ■■■ doctors and family.
EDNF ANNOUNCES PARTNERSHIP WITH
NATIONAL TISSUE BANK
L
ong considered the “Holy Grail” of fundraising and health advocacy, research is where many people wish to see their donor dollars go. Research into a cure or treatments to offset the often drastic and chronic impacts of Ehlers-Danlos Syndrome has been especially challenged by the complexity of the disease, the many different manifestations and the lack of awareness in the healthcare community of this urgent need.
Different types of human tissues and organs are needed for research:
Unlike Marfan or Osteogenisis Imperfecta, there is much more than a single gene to identify and examine in the currently defined subtypes of EDS; we are finding that rather than a specific delineation among subtypes of EDS, there appears to be much overlap and a continuum of symptoms that defies easy categorization. How then can this problem be approached?
• • • •
• Blood samples; • Discarded surgical specimens; • Tissues and organs donated after death.
How to become a research tissue donor: Tell people in your family; Discuss with your doctors and genetic counselors; Contact and notify EDNF; Contact NDRI at 1-800-222-6374 or raredisease@ndriresource. org.
Can anyone donate tissues for research? EDNF has partnered with the National Disease Research Interchange (NRDI) to allow EDS patients the opportunity to donate tissue Yes. and organs for research on the syndrome. The lack of these tissue samples has hampered researchers as they search for the genetic How do the donations reach the scientists? causes of, and a path to treatment for, the different types of EDS. NDRI has a very large database of The Tissue Bank will receive, with strict scientific protocols, donated researchers in the world who are seeking blood, tissue, bone and organs from donors and make those cell human tissues. NDRI matches donors with lines available to the over 100 scientists in the U.S. studying these appropriate requests and then sends the disorders. This is especially meaningful because the previous belief tissues directly to that researcher. Your personal details remain that EDS is rare may well be disproved — that in fact, it is not that strictly confidential and no donor information is given to the rare, and is part of a wider spectrum of genetic and connective tissue scientists. syndromes. Close to 90 percent of rare diseases have a genetic cause, so donated tissues are critical to the studies that will reveal NDRI follows strict governmental regulations and guidelines molecular triggers and allow opportunities to engineer intervention. regarding donor consent and confidentiality; tissue samples are Moreover, research into one disease has frequently led to discoveries only provided to approved biomedical research. in related fields and breakthroughs in treating other maladies. This is just one of many such opportunities being brought to the EDS It is important that you notify EDNF of your wish to donate as we community through the work of EDNF. work with NDRI to facilitate this procedure. ■■■
L O O S E CONNECTIONS PAGE FOUR • SPRING 2007
YOU CAN MAKE STUDIES IN SLEEP FOR A DIFFERENCE! SUGGESTIONS A BETTER NIGHT’S SLEEP We encourage you to add your voice to our efforts: send your U.S. Senators and Congressperson your personal story of dealing with Ehlers-Danlos Syndrome and encourage them to support federally funded biomedical research. TIPS FOR WRITING TO CONGRESS More and more people are using e-mail and faxes to communicate their concerns and increasingly the offices of elected officials prefer electronic communications for constituent contact. • State your purpose for writing in the first sentence • Be courteous • Include personal information about why this issue matters to you • Address only one issue in each letter or email HOW TO ADDRESS YOUR CORRESPONDENCE To a Senator: The Honorable [Full Name] United States Senate Washington DC 20510 Dear Senator [Lastname]:
To a Representative: The Honorable [Full Name] United States House of Representatives Washington, DC 20510 Dear Mr./Ms./Mrs. [Lastname]:
BY BARBARA GOLDENHERSH
Sleep disturbance seems to be one of our most common problems as EDSers, so we begin “Studies in Sleep,” an occasional column to look at the issue. If you have your own sure tricks to fall asleep — or stay asleep — send them to editor@ednf.org for future editions.
breath for a count of seven, and breathe out slowly for a count of seven.
One hour before bedtime, call someone you care about and say goodnight.
Sample Visualization
Pay attention to your senses as your body relaxes into the bed. Try imagining yourself as a stick of butter in a warm pan; sense that your body is melting have a great deal of difficulty into the bed like the butter relaxes into with sleep, and have discovered a pool of liquid. some techniques that could help you, too. I share them here. Imagine a beautiful, serene scene, perhaps a favorite place you have been, During the evening, one or two hours or one you wish you could see. Take in before bedtime, take time to write a list the scene with every sense: hear the of things to do the next day; include sounds, imagine what it looks like, things such as possible actions you can how it smells, tastes, feels. Let yourself take to solve problems, errands and relax into sleep. phone calls to make. Let that be your time to consider those things that need planning or worry: get them down on paper and out of your mind.
I
As you prepare for bed, think of three I am on a beach … things that went well that day and why they went well; allow yourself to feel My toes are wiggling into the sand at the water’s edge … fulfilled and satisfied with your day.
I feel the warmth of the sun on my face, the coolness of the breeze, the smell of the ocean, the sounds of the waves and the seagulls. I bend down to brush my hand into the water as a wave splashes up and Relaxation Techniques I taste the moistness of the air as I breath deeply and relax into the joy of the day. I In typical strategies, the first step is see a shell and lift it to my ear; I listen to usually to physically tighten muscles sound of the ocean as the waves flow and and then let them go. With connective the sense of the ocean I hear in the shell … tissue problems, this can cause unintended joint movement, pinched Fill in your own details; perhaps you nerves, and other pain. A different are in New Zealand, on a boat or in a method is needed. forest, a mountaintop or on a cloud. Remember what relaxes you and find Take three deep, cleansing breaths your own textures, your own images using the following technique: breathe and let yourself find a safe, calm center in slowly for a count of seven, hold your of sleep. ■■■ Once you get into bed, use a relaxation and visualization technique. I have adapted some for use by those with connective tissue disorders.
L O O S E CONNECTIONS SPRING 2007 • PAGE FIVE
SPORTS VEDS? HOW ABOUT
RUNNING A TRIATHLON? BY THEA HYNES
A
ll my life I have been involved in some form of sports. At the age of ten I began riding and showing horses. Everything for the most part (dressage, cross-country jumping and stadium jumping) until the past five years when I concentrated on dressage because my hips were so bad it was unsafe to be jumping and dislocating at the same time.
Farewell Edzel
riding five miles, just three days after being allowed to work out again. The next week it all hit the fan.
The hip was great, but I was diagnosed with diverticulitis with perforations after a fortypound weight loss for which I spent sixteen days in the hospital and had an emergency temporary colostomy. This was followed I was having my hip replaced in June of by eight days of intense antibiotics that did 2006 (my fortieth operation) and since I not work. was side-lined from the horses, my doctor suggested I try swimming and biking prior Eight weeks later I was scheduled to go back to the operation to strengthen my muscles in to the hospital to have the colostomy and help with recovery. After four days at reversed, but during pre-screening tests the the gym, I got bored until I came across doctors noticed I had developed a fistula information on the web about triathlons. from my colon to my bladder, so I have six Being as goal-oriented as I am and not more weeks of waiting. The hardest part being able to ride, I thought, “What a great off all of this was not being able to work way to make sure I keep working out.” All I out — other than my walking two miles a had to add to the mix was some running. day and going a few steps more every day. OK, all you EDSers out there, I hear you The reversal colostomy should be done cringing, but think about it: I need both by Thanksgiving and I plan to go back hips replaced and both knees replaced; I in training by mid-January. My plans for dislocate when I sleep, and a sneeze can put 2007 if all goes well include the Danskin me in the hospital with aortic dissections; Women’s Triathlon at Walt Disney World in yes, I am VEDS. In reality, what did I have May; in June the Hyannis Sprint Triathlon, to lose? whereI hope to take 30 minutes off my 2006 time; and in September, my first halfSo after endless laps in the local hotel pool Olympic-distance triathlon. and swimming in Boston harbor for openwater training, dodging Boston traffic on All this gears up to: my first and last my bike, and hitting the pavement, both marathon, in Dublin, Ireland at the end literally and figuratively, I competed in my of October — I admit I plan on doing a bit first sprint triathlon in Hyannis, Maine. of walking for that one. As I mentioned My two goals were first, to just finish before, what have I got to lose? Everyone and second, to do it under two hours. My sees the difference between when I work official time was one hour, 57 minutes for a out and not; it shows both physically and quarter-mile swim, ten-mile bike ride and emotionally. Last spring when I started this, 3.6-mile run(I admit I walked a bit, but I I could not run 100 yards without wanting finished). By the way, I am also 42 years to call 911. But my best friend, a runner, old. told me something I remember with each step I take: “Just go from telephone pole to Sixteen days after the triathlon I had my left pole, end of the pool to end of the pool, or hip replaced. Both my doctor and I agreed driveway to driveway and sooner or later that it was thanks to the triathlon training you will hit the finish line.” that I had one of the strongest and fastest recovery for this operation. Six weeks after I may be one of the slowest on the course, the operation I was back on the my bike I may not have the best form, but I will always “TRI.” ■■■
Edzel has been the friendly voice answering the phone at EDNF for more than two years. Edzel came to California from Maryland, where he went to school after leaving the Philippines. He began working at the EDNF office in November, 2004. Edzel’s help has been a large part of the growth and success we have enjoyed. Edzel lost his dad this past spring, and he returned to the Philippines to help his mom. We will miss him. In a website posting, Edzel said goodbye to the Foundation in February:
This foundation has been a part of me and will always be. My prayers and hope to find a better way of living, a cure and widespread awareness will always be with you all. Will miss you and with much gratitude, Elizalde “Edzel” Lejano
L O O S E CONNECTIONS PAGE SIX • SPRING 2007
MAKING THE MOST
OF YOUR DOCTOR’S VISIT BY TROY WINKELS
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octors spend an average of only a few minutes with each patient for a routine examination. This doesn’t include the time spent outside the examination room reviewing charts and records, of course, but there are only a few minutes when you actually see the doctor, and this can be confusing and frustrating. Here are a few suggestions that may help to relieve some of the stress and worry associated with your next visit. This might even improve your quality of care by helping your doctor better understand your symptoms and conditions. Before your visit, make notes. Write down a list of things you wish to tell the doctor. This keeps you from forgetting something important when you’re under the stress of the visit. Make a list of all the prescriptions you’re taking, including any over-the-counter drugs, nutritional supplements and
vitamins. The information may be in of information. One final thing I would your chart, yes, but it’s not easy to find, add, bring a list of the names of all your and my doctor has asked many times. doctors and their specialties. It can make communication much easier when you Before you leave the exam room, go don’t have to remember their names and over your list to be sure you covered what they do. everything. This benefits both you and your doctor by keeping the discussion At every doctor’s visit I always make sure focused on the purpose of your visit. they have all my insurance information, too. On those visits when I really feel Never feel shy or too embarrassed to ask bad, it keeps me from having forms to fill questions. Remember that doctors are out in the waiting room. only human and may not realize they haven’t explained something well, or Your doctor visits would also be great put it in terms that make sense. Don’t times to wear your Zebra shirt and hesitate in saying you don’t understand. wrist-bands, something I feel I should mention. If the doctor asks questions, be as honest as possible, even if they might We all have busy lives and of course, be a little embarrassing. Withholding so does your doctor. Any advanced the truth could affect your quality of care preparation you do will always strengthen and might lead to an incorrect or missed your role as a partner in your health diagnosis. care. A good doctor will encourage your desire to understand as much as Always keep in mind that the doctor’s possible about yourself and welcome nurse or assistant can be a great source your participation. ■■■
NOTES FOR YOUR DOCTOR This page is part of our continuing focus on this year’s communications theme, “Educating the Health Care Community: Teaching the Doctors.” After reading Mr. Winkels’ column, the editors also had some advice. 1. Always bring new information to leave with your health care professional — a research article you find on the web site or in Loose Connections, or a brochure, the MRG, even one of the CDs. Share what EDNF has to offer, and show your health care professional that you are working to learn. Select items that inform them about your particular EDS type when you can. 2. I keep on computer four documents that I always have with me when I go to a doctor. I print out two copies, one set for me, one to leave with my doctor or his office. a. There is an on-going list of my prescriptions that includes the brand and drug names, dosages, and prescription information. It’s easy to mark off which prescriptions I
need refilled each visit, plus each doctor sees exactly the same information. b. I type in my notes for visits with my doctors, too. I come home after the visit and add any information, including test results, in my computer document and save it. c. Some time ago, I subscribed to Medic Alert. For each doctor’s visit, I also come armed with my one-page list of Medic Alert emergency instructions and treatment information, which includes how to reach my doctors and therapists. This is what I keep in my wallet for emergency use, too. d. To keep my memory and medical records consistent, I carry a one-page medical history on myself that starts with my most recent diagnoses and moves backward. It lists what was diagnosed in which year, along with a short list of what treatments are underway and/or been tried. Both the history and the Medic Alert page have been amazingly useful, particularly when I’m faced with a substitute doctor or a brand-new volume of my medical chart that’s empty.
“The eyes cannot see what the mind does not know, but once the mind knows, the eyes cannot help but see it.”
L O O S E CONNECTIONS SPRING 2007 • PAGE SEVEN
REVIEWS
BOOK
HYPERMOBILITY SYNDROME
RECOGNITION AND MANAGEMENT FOR PHYSIOTHERAPISTS BY ROSEMARY KEER & RODNEY GRAHAME, Reviewed by Janet Sayer While intended for those treating individuals diagnosed with Hypermobility Syndrome, this book would be useful for any hypermobile person, EDSer or not Published in 2003 by Butterworth Heinemann, ISBN 0-7506-5390-6.
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hen I was referred to a physiotherapist, my doctor wrote in his letter that my hypermobility and subsequent joint pain would be ‘an interesting challenge’ for her. As the authors state in the Preface, “physiotherapy forms the mainstay of treatment, yet the majority of physiotherapists shy away from treating such patients because, as they report, they are at a loss to know how to treat them.” This excellent book is obviously aimed at informing and improving the knowledge and skills of physiotherapists in treating the hypermobile patient. It also offers valuable insights for people with joint hypermobility or those who work with hypermobile people (such as sports trainers or workplace ergonomic assessors). The first two chapters detail the clinical features and common complications of Hypermobility Syndrome, and introduce the range of disorders and diagnostic criteria of connective tissue disorders. I learned a new word here, kinesiphobia — a fear of pain induced by movement leading to disuse and deconditioning, something I certainly understand!
greater degree of disability in later life. Early diagnosis clearly should be a priority, both from the experience of my and family and of others, as discussed on the EDNF site. Chapter 6 discusses physiotherapy assessment of the hypermobile adult and therefore is aimed at the physiotherapist reader. Chapter 7 is really useful from a patient perspective, addressing often-discussed issues such as whether to stretch. I know we like stretching because it can alleviate pain, but this chapter explains the need for the stretch to be both controlled and even. It also addresses the reason why sustaining some of the postures we are able to do is harmful. Useful advice is given regarding lifestyle adaptations such as sleeping, carrying, clothing and footwear, travelling and even coping with a family and children. I found Chapter 8 particularly relevant to me. It is about rehabilitation, fitness, sports and performance for individuals with joint hypermobility. I run a yearly half-marathon to raise money for Cancer Research because my Dad died of cancer. I know that exercise is important, but I also know that I have to be very careful. From my own experience I have learned that everything depends upon pain and movement, which can be unpredictable. I employ a Personal Trainer who is brilliant at taking an individual and
Early diagnosis clearly should be a priority. Chapter 3 examines the overall management of joint hypermobility. Local anaesthetics are included in that they are less effective for hypermobile patients. (I have a new young dentist, who was very complaisant when he knew I had EDS: “Oh yes we were taught all about that, and don’t worry, I’m going to give you a horse-grade local.” I didn’t feel a thing, thankfully after years of suffering!) I particularly like the emphasis on every person having their own “unique variation of the condition and no one understands its nuances better than the individual themselves.” The focus of Chapters 4 and 5 are hypermobility in children and the management of hypermobile adolescents, which includes addressing psychosocial issues. The message in these chapters is the need for a clear diagnosis and treatment program, in order to avoid the development of a “chronic pain cycle” and a
holistic approach to fitness; Craig is now very knowledgeable about EDS and certainly looks after me. He came to some of my physiotherapy sessions to learn about what would help me. I certainly recommend that if several professionals are involved in your care, they meet each other to discuss what will help.
that challenges unhelpful beliefs. It points out trigger points and describes useful self-help strategies for them. The final chapter is told from our viewpoint, giving a patient’s perspective. Throughout the book there are case studies, which also serve to increase the reader’s identification with hypermobile patients, and enhance the physiotherapist’s journey to increased knowledge. This book should be essential reading for physiotherapists working with hypermobile patients. I suggest we make our physiotherapists aware of its availability. I felt three points were most important: • If you see a physiotherapist, make sure they are knowledgeable about EDS. Remember you are unique, and the symptoms you experience are valid and unique to you. Be sure you get the care you deserve; you’re worth it! • Facilitate a multidisciplinary involvement: get all the people involved in your care together, so they can share knowledge to get the best from you. Do this for your children, too: sports teachers and coaches should see the physiotherapist to learn what will work best. Activity is important, as well as inclusion on a psychosocial level. • Learn as much as you can about EDS to enable yourself. This last is still difficult for me. When I was first diagnosed, I went into complete denial, and Craig would say that this is still the case at times. No, I don’t want EDS and will continue to fight it, but not by denying it; I’ll fight it best ■■■ by learning.
Chapter 9 is especially useful for workplace ergonomics, and assesses the work-related risks of joint hypermobility and musculoskeletal disorders. It examines vulnerability and associated risk in different work-related situations, and provides preventative measures that can be taken in the workplace. I learned here why I experience pain and immobility in my wrist and hand on cold occasions in winter months. Chapter 10 examines chronic pain and the benefit of using a cognitive behavioral approach
The Shoulder
L O O S E CONNECTIONS PAGE EIGHT • SPRING 2007
REVIEWS
MEDIA
MISLEADING “MEDICAL MYSTERIES” BY MARK C. MARTINO
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his is necessarily a long review; one can hardly report on the ABC News segment on EDS (ABC News, Primetime “Medical Mysteries” on Wednesday, January 24, 2007, from 9:40 to 10:00 p.m. EST) without also covering the reaction to the story — and once that is mentioned, one has to also comment on the actions of the producers, and try to separate their behavior from the segment itself. My views are my own, of course, and not necessarily the Foundation’s or yours. We are a very diverse group. In many ways, reviewing the ABC segment is the easiest part of this task: I think we can all agree it was sensationalist and inaccurate. At every turn of fact and phrase, the script went for extremes. A few will sufficiently illustrate what I mean: • In the opening, the narration states EDS “affects about one in 10,000 people.” This is a highly conservative estimate; Dr. McDonnell has said it is one in 5,000 to 10,000 and is likely under-diagnosed. • The narrator continues, “the odds of Turner’s special variety of the disease are astronomical” when in truth he has Classical EDS, which we know is one of the more common varieties; and while the degree of skin stretching may be quite unusual, it is not a “most extreme and rare” circumstance as claimed. • Instead of accurately reporting that Garry Turner is applying a Duragesic patch, the narrator calls it morphine, with an inflection of horror. In the end, the script even seems to admire Mr. Turner: “‘Stretch’ Turner uses his EDS to make a living.” But not once is it mentioned that Mr. Turner lives with a single-payer medical system that provides care for any damage he might do to himself while performing tricks. For an EDSer in the U.S., it is far more common to become unable to perform most jobs, unable to afford private insurance, and unable to pay for costly, frequently ineffective treatment. When an American such as Dylan Keil has pain as great as Mr. Turner’s, it is incredibly expensive
to pay for a pain doctor — if one can be found at all — and there is the expense of the medications themselves, while Mr. Turner’s are provided him relatively cheaply. This is an element frequently missing from the debate: EDS can be devastating emotionally, physically AND financially. To waste more space on the segment itself would be pointlessly negative. I now come upon the issue of what happened during the taping and editing, and to what those involved said to each other. The evidence seems clearly weighted against the producers. Over and over they were told, by Dylan’s mother and father, by Dr. McDonnell, how harmful focusing on the circus and freak-show angle would be; the producers provided assurances to everyone that that was not their primary focus. When advertising for the segment focused solely on the sideshow, Dr. McDonnell was told by letter that it was only to draw in viewers, that the segment was balanced with a third of the time going to Dylan and Cindy. Still, the story transcript posted on the ABC News website was at first titled “A Real-Life Comic Book Character: Stretchy Skin” and completely omitted Dylan and Cindy’s contributions, also reducing Dr. McDonnell to one short paragraph. Once NIH Public Relations became involved, the headline changed to “A Rare Disorder: Stretchy Skin,” which was at least more sensitive; and the content was changed to include some of the missing material from NIH. The transcript as it is now on the website seems to be differently balanced than the show as broadcast; the Keils’ contributions seem larger in print than on video (their paragraphs are shorter, making less material look longer). The producers put Dylan and Cindy through two physically exhausting days, having them repeat actions to meet demands for usable footage — but ironically the only speaking footage used was of their differing opinions of Mr. Turner, shot while they’re sitting almost motionless. Neither the Keils nor Dr. McDonnell appear at all on-line in the ABC News “Video On Demand” footage (http://abcnews. go.com/Video/playerIndex?id=2823138). The clearest display of the less-than-sterling behavior of the producers can be found in their response to the extensive criticism in the ABC News Forum. There can be no mistaking the record there, or attribution to hearsay (http://forums.go.com/abcnews/Primetime/ thread?threadID=522533). The producers write, “During the interview, on January 8, 2007, the producer repeatedly asks Dr. McDonnell about EDS patient Garry Turner and his role in a circus freak show.”
Yes, they did; and when they didn’t get an answer they liked from Dr. McDonnell, they’d ask the question again, and again, and again, until she was led into saying something they could misquote. The most usable quote the producers could find in the transcripts was her eventual half-hearted response, “I think, if a person has EDS and they choose to pursue it in a lifestyle that they can make a living out of it, it’s of course okay by me as long as they’re not suffering. So that means they may have found an adaptation that works for them.” Dr. McDonnell is clearly irritated when she goes on, “But also please understand that not every person is going to find themselves a place in a freak show, or a circus show, and take advantage of their disorder in that fashion, many of them are not going to find an adaptation. And, they face the social isolation, they face a poor quality of life, and they face all the psychological problems that come with it.” In summary, the production team was misleading beforehand, and in the end they produced an inaccurate story. We come to the reaction to the show. It became clear that EDSers needed a way to respond. Some took to the ABC forums; others swamped the small, two-person EDNF office. For days, nothing else could be accomplished. The American Pain Society became involved because of the faulty portrayal of the pain issues; they are a substantially larger organization that wanted to point out inaccuracies from the standpoint of both EDSers and others with pain. EDNF felt this was clearly a more effective response than a response initiated from EDNF alone, and it allowed members the chance to respond both as a group and with individual voices. It is far more effective for a news organization to get 2,000 differing responses than 12,000 identical responses. No one asked the Foundation for comment beforehand, when it would have been useful. EDNF found a way for members to respond individually; for the Foundation to make comments now would be probably fruitless, in my view. EDNF continues working hard to increase awareness, particularly in this Communications year of “Educating the Health Care Community: Teaching the Doctors.” I have never been prouder of being an EDSer than after reading the responses at ABC and counting how many of us directly got involved. However, the unfortunate fact remains that simply because of ABC News and the subsequent response, our (continued on page 10, column 3)
L O O S E CONNECTIONS SPRING 2007 • PAGE NINE
FUNDED BY THE NIH • DEVELOPED AT THE UNIVERSITY OF WASHINGTON, SEATTLE
EHLERS-DANLOS SYNDROME, HYPERMOBILITY TYPE BY HOWARD P. LEVY, MD, P D h
This is extracted with permission from the full paper, copyright 1999-2006 and published by the University of Washington, Seattle, last accessed in September, 2006. We urge you to read the complete text, available at www.ednf.org (Medical Professionals section under Medical Reports and Findings) or at http://www.GeneTests.org.To respond, write editor@ednf.org; always consult your doctors before making changes in your treatment. — Ed.
multiple joints is sometimes accepted in lieu of a positive Beighton score in such cases, if the family history and other minor criteria are strongly suggestive.
Clinical Description Skin.
of sites, duration, quality, severity, and response to therapy. The severity is typically greater than expected based on physical and radiological examination; fatigue and sleep disturbance are frequently associated. • These persons are often misdiagnosed with chronic fatigue syndrome, fibromyalgia, depression, as being a hypochondriac, and/or malingering prior to recognition of joint laxity and establishment of the correct underlying diagnosis. • Pa i n m e d i c a t i o n i s f r e q u e n t l y underprescribed, and should be tailored to the individual’s subjective symptoms, not to objective findings. • Opioids are effective for both myofascial pain and neuropathic pain. Since they are typically used at least several months, they should be sustained release with additional short-acting forms used only for breakthrough pain.
The skin is often soft, velvety and may be mildly hyperextensible. Piezogenic papules (small, reversible herniations through the underlying dermis of the heel occurring only Hypermobile Ehlers-Danlos Syndrome (EDS) on weight bearing surfaces) are frequent, but is distinguished from Classical EDS, chiefly by rarely painful. Easy bruising is quite common, milder and fewer skin and soft tissue manifes- frequently without obvious cause. tations. The diagnosis of EDS, hypermobility type is based entirely on clinical evaluation Musculoskeletal. and family history. The Beighton Scale is used for hypermobility. • Joint laxity. Subluxations (incomplete dislocations) and dislocations are common Major diagnostic criteria should all be met to and represent the major manifestation establish a diagnosis: of the condition. They may occur spontaneously or with minimal trauma • Joint hypermobility, which usually is and can be acutely painful. Reduction Gastrointestinal. confirmed by a score of five or more on often occurs spontaneously or can be the nine-point Beighton scale. accomplished by the affected individual; • Functional bowel disorders are common • Soft or velvety skin with normal or slightly medical inter vention for an acute and under recognized, affecting up to 50% increased extensibility. dislocation is not usually necessary. Its of individuals with EDS, hypermobility • Absence of skin or soft tissue fragility, pain can last for hours or days afterwards. and classic types. GERD, IBS (irritable which is suggestive of other types of EDS. Excessive joint motion is evident on bowel syndrome) and delayed emptying routine activity. Joint laxity sometimes all occur with frequency. Examination variations. improves with age. • Gastritis and reflux symptoms may require • Pain. One hypothesis is that painful intensive therapy. • Young children (five years of age or myofascial spasm occurs in response to • Upper endoscopy is indicated for resistant younger) tend to be very flexible and are chronic joint instability, with neuropathic symptoms, but frequently is normal other therefore difficult to assess. pain resulting from direct ner ve than chronic gastritis. • IBS is treated as usual with medications as needed. Tegaserod (Zelnorm®) can be very helpful for those with constipation only.
Clinical Diagnosis
These persons are often misdiagnosed with chronic fatigue syndrome, fibromyalgia, depression…prior to recognition of joint laxity and establishment of the correct underlying diagnosis. • Women are, on average, more flexible than men. • Older individuals tend to lose flexibility, and post-surgical or arthritic joints often have reduced range of motion. • Degenerative joint disease is common and occurs at a younger age than in the general population, possibly due to chronic joint instability resulting in increased mechanical stress. • A history of former joint laxity or clinical demonstration of substantial laxity in
Cardiovascular.
• Autonomic dysfunction. Approximately one-third to one-half of individuals with impingement (e.g., by subluxed vertebrae, EDS, hypermobility (and classic) type herniated discs, vertebral osteoarthritis, report atypical chest pain, palpitations or peripheral joint subluxations), and/or at rest or on exertion, and/or orthostatic from mild-to-moderate nerve compression intolerance within spasmed connective tissues. • Aortic root dilatation, usually of a mild • Chronic pain, distinct from that degree, occurs in one-quarter to oneassociated with acute dislocations or third of individuals with EDS, classic and advanced osteoarthritis, is a serious hypermobility types complication of the condition and can • Mitral valve prolapse (MVP) was be both physically and psychosocially previously considered a manifestation of disabling. It is variable in age of onset (childhood through elderly), number (continued on top of next page)
L O O S E CONNECTIONS PAGE TEN • SPRING 2007
(continued from previous page)
problematic if too much force (resistance) is applied. all types of EDS, but this has not been • Braces are useful to improve joint stability. confirmed in rigorous evaluations using Shoulders and hips present more of a modern diagnostic criteria for MVP. challenge for external bracing. Occupational • Various testing is available if problems are therapists should be consulted for ring detected. splints (to stabilize interphalangeal joints) and wrist or wrist/thumb braces. A soft Oral/Dental. neck collar, if tolerated, may help with neck pain and headaches. • High, narrow palate and dental crowding • A wheelchair or scooter may be necessary are nonspecific features of most heritable to offload stress on lower extremity joints. disorders of connective tissue. Special wheelchair customizations such as • Periodontal disease (friability, gum lightweight and/or motorized chairs, seat recession) occurs in some individuals with pads, and specialized wheels and wheel EDS, and used to be considered a unique grasps may be necessary. Crutches, canes, subtype of EDS. It is now recognized as a and walkers should be used cautiously as potential manifestation of the classic type. they put increased stress on the upper • Temporomandibular dysfunction (TMJ) extremities. due to intrinsic joint instability is common, • A waterbed, adjustable air mattress, but difficult to treat. Surgery is usually or viscoelastic foam mattress (and/or disappointing and should be a last resort. pillow) may provide increased support • Periodontal disease should be identified with improved sleep quality and less pain. and treated. Prescriptions can be obtained and cost included in Federal Income Tax Schedule Obstetric. A for medical costs. Pregnancy may be complicated by premature Activity. rupture of membranes or rapid labor and delivery (less than four hours), but this is less • Improved joint stability may be achieved likely than in the classic type. Joint laxity and by low-resistance exercise to increase pain typically increase throughout gestation, muscle tone (subconscious resting muscle
Joint hyperextension must be avoided. especially in the third trimester, as normally occurs during pregnancy in unaffected women
contraction, as opposed to voluntarily recruited muscle strength). Progress should be made by increasing repetitions, frequency, or duration, not resistance. Psychiatric. • Joint hyperextension must be avoided. Individuals with EDS, hypermobility Depression is a common complication among type usually need to be educated about all individuals with chronic pain, including the normal range of joint extension and those with EDS. No data are available on cautioned not to exceed it. mood or personality disorders independent • High resistance and high impact activity of pain among individuals with EDS. increase the risk of acute subluxation/ dislocation of joints, chronic pain, and Validation of the affected individual’s osteoarthritis. symptoms can be immensely helpful, as many • Fat writing utensils can reduce strain on with EDS have been accused of malingering or finger and hand joints. This adjustment diagnosed with primary psychiatric disorders frequently results in marked reduction of by previous physicians. pain in the index finger and at the base of the thumb.
Physical Therapy & Assistive Devices.
Surgery & Other Procedures.
• Chiropractic adjustment is not strictly contraindicated, but must be performed • Many individuals will have undergone cautiously to avoid subluxations or several orthopedic procedures prior to dislocations. Not all of these work for all diagnosis. Improvement is usually less persons with EDS. than that in patients without EDS … expect • Resistance exercise (even at moderate less than optimal results. No increased risk resistance) can exacerbate joint instability of perioperative complications. and pain. Isometric exercise can also be • Lidocaine often requires much higher dosing for surgery. ■■■
CONTINUED FROM PAGE 8
Foundation was unable to function for more than a week, even tying up EDNF volunteers across the U.S. Not only did ABC set back EDS education by nationally broadcasting faulty information, the result actively interfered with those who are trying to communicate accurate facts. Now, are we clear I have little sympathy for ABC’s mistakes, or for being called a side-show freak in front of my current doctors? Yes?
Please forget the negatives. If you can’t do it permanently, you’re simply giving ABC power over yourself , but never mind. Bear with me anyway and forgive ABC at least long enough for this point: there was good done, there are positives here. A national news magazine did a story on EDS. Yes, it is an unfortunate fact that ABCTV is a commercial enterprise, and even news divisions are now required to make money; still, somehow, despite that, and independently of EDNF’s own efforts, a story on EDS actually got done and on the air. Attention was focused on EDS for 20 minutes, and millions became aware of it, probably for the first time. The story managed to include that our joint pain can be so bad it requires morphine. I could tell the interviews with Dr. McDonnell informed some of the narration, they used her warning against doing tricks and what the consequences can be, and Mr. Turner even managed a clear explanation of how EDS disrupts tissue. Despite the carnival coverage, I’m grateful for the story at all. When I imagine what the story might have been without Dr. McDonnell’s moderating influence, I realize it could have been much worse. The effects will be beneficial, even to the Foundation, because we will make them beneficial. We have many new and returning members, and the level of member involvement has never been greater; even the number of people on the website has never been higher. Moreover, we have this controversy and publicity just before EDNF’s Annual Fund drive; there’s never been a better opportunity to get the facts — and a donation form! — to your family and friends. The funding is desperately needed for the our mission this year: to educate the medical community about EDS. Accurately, with dignity and respect. ■■■
L O O S E CONNECTIONS SPRING 2007 • PAGE ELEVEN
NATIONAL ORGANIZATION FOR RARE DISORDERS
UPDATE ON THE EDS DOG MODEL 2006 FUNDING PROVIDED BY EHLERS-DANLOS NATIONAL FOUNDATION
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he primary goal of this project was to provide a large animal (canine) model of the Ehlers-Danlos Syndrome (EDS) to mechanism of disease. A female Labrador retriever with an autosomal dominant form of EDS was donated to the Section of Medical Genetics at the Veterinary Hospital of the University of Pennsylvania for characterization and expansion of this canine model. Initial studies included the identification of the defective gene and characterization of the disease to further strengthen the EDS dog as a model for the human disorder. To this end, skin biopsy samples were obtained from each dog under local anesthesia (as is routinely done in the clinical setting for canine patients), radiographs (X-rays) of all limbs were taken, and breeding studies are still being performed to confirm the mode of inheritance. Skin biopsy samples were examined by routine microscopy and electron microscopy. The biopsy samples were also
to prevent pain while suturing the wounds does not appear to be as effective as in normal dogs. Therefore, we place the dogs under general anesthesia when suturing the wounds or when skin biopsies are obtained. Also, placing IV catheters is not as easy as it is in normal dogs: the veins frequently blow, and we have found that placing butterfly catheters and using vet wrap (a material that only sticks to itself and not to the skin or hair) to hold the butterfly in place serves our needs. We have not found any abnormalities when examining the hearts of both dogs, but we will continue to follow the heart and the greater vessels by ultrasound to rule out abnormalities that may develop. Experiments performed on skin biopsy samples (1 cm x 2.5 cm) show that the skin stretches 4 times farther than that of a normal size-matched dog. Once the skin reaches its maximum stretch, it tears at a minimum weight of about 900 g versus more than 10 kg in a normal dog. Histology and
To date, over 350 genetic diseases have been described in the dog, many of which are analogous to human genetic diseases. The number of canine models used for the study of inherited diseases is rapidly growing and dogs have been invaluable for the understanding of both disease processes and a variety of therapies. The EDS dogs offer an excellent model to use to understand disease mechanisms and ultimately to develop and test different treatment modalities. The clinical presentation of the EDS dog is more electron microscopy show abnormal collagen similar to the disease in humans than the arrangement as is to be expected. current mouse model. The dog is also a long-lived, large animal that more closely Currently, we are in the process of resembles the size of a child, making it a collaborating with Dr. Ron Minor from valuable model for preclinical trials and longCornell in New York, the world’s leading term studies. Because of the dog’s size and expert in collagen defects in animals, to long life span, more samples can be obtained find the mutation. We are also using special and pathology can be followed for a very long stains and new techniques to examine the period of time. ■■■ microscopic features of the disease in our dog model. We will continue to document disease features as they appear. We have included in our clinical evaluation protocol routine eye examinations that include pressure measurements and retinal assessment. The heart examinations are performed by Dr. Meg Sleeper, a board-certified cardiologist, and the eye examinations are performed by Dr. Andras Komaromy, a board-certified ophthalmologist.
The clinical presentation of the EDS dog is more similar to the disease in humans than the current mouse model. examined for elasticity (tensile strength) and fragility. The hearts of all dogs were examined routinely by auscultation (stethoscope), EKG, and echocardiography (ultrasound of the moving heart). While we have not found the defective gene yet, we have been able to further establish the dog as a model for the Ehlers-Danlos Syndrome in humans through the generous funds provided to us by the National Organization for Rare Disorders. Our breeding studies are highly suggestive of an autosomal dominant mode of inheritance, as one normal and two pups affected with EDS were born to an affected mother that was outcrossed to a completely unrelated sire. One puppy was so severely affected that he was stillborn. The other affected puppy is now 7 months old and shows the exact same clinical signs as her mother. The mother is pregnant again and we expect puppies in a few weeks. Both dogs have skin and joint laxity that far exceeds that of any normal dog we have in our colony. Both dogs have luxated hip joints, but do not appear to have pain associated with it. They are able to run and play like most other dogs. Other clinical features include frequent skin tears which heal fairly well, but lidocaine used
catheters and local lidocaine application. Future experi-ments are directed at developing better methods for placing IV catheters, more effective local anesthetics and understanding reasons why the skin does not react to local anesthetics as it does in normal dogs. The ultimate goal is to use the EDS dog for treatment trials that would be curative rather than just palliative. We are in the process of establishing collaborations with the research dermatologists at The Thomas Jefferson University in Philadelphia, that have been using interfering RNAs (iRNAs) in mice with EDS. The iRNAs are designed to interfere with the production of the abnormal collagen molecule, so that only the normal fibers are generated.
In summary, our dogs show not only the same clinical and pathological features as humans with EDS but they also have the same problems with medical management such as placing IV
L O O S E CONNECTIONS PAGE TWELVE • SPRING 2007
KIDS & TEENS EDNF Kids & Teens Invites Local Groups to Our 2007 Awareness Project & Photography Contest EDNF Kids & Teens are hosting a traveling art show around the country during the month of May, in honor of National EDS Awareness Month, and we need your help! Help us raise awareness in your neighborhood by reserving space at your local library, hospital lobby, city hall, doctor offices, anywhere you can display entries of this contest along with EDNF materials. This is a wonderful opportunity to work together for a great cause. Each local group will be responsible for paying the shipping charges to receive the display (which will not exceed $25.00). All photos will be mounted on acid-free mat board and ready to go. We will also include information about EDNF and a piece to be mounted with the display that explains EDNF and the importance of National Awareness Month. If you are interested in participating in this project we are making reservations now. Please contact Michele Darwin at micheledarwin@ tx.rr.com or email EDNFKidsandteens@ednf.org.
8TH CHRONIC FATIGUE CONFERENCE REPORT STAFF REPORT
I
n January, 2007 Cynthia Colabella attended the Eighth International Association for Chronic Fatigue Syndrome, Fibromyalgia and Other Related Disorders Conference, which was held at the Bahia Mar in Ft. Lauderdale, Florida, with over 600 medical and professional attendees. Physicians, journalists and others came from Japan, Sweden and other distant locales, as well as many Americans. Pharmaceutical firms were present, as well as other vendors offering information and products such as Purdue Pharma, a long-time supporter of EDNF. The Veterans Administration made a presentation about the lingering effects of the Gulf War; current pain studies were described. Patients were able to voice their opinions and ask questions. The entire seminar was taped, so anyone may purchase and then view it anytime. Call 1-800-669-8273 and order code CFS061/062. Marly Silverman, founder of PANDORA (Patient Alliance for Neuroendocrineimmune Disorders Organization for Research and Advocacy), extended an invitation to Cynthia, to learn about EDS and the similar symptoms shared between EDSers and the Conference subjects. Discussion topics included fundraising, coping and patient/doctor communications. One of the most important involved fundraising to help with awareness, especially who to target and how to go about doing so. The most important aspect of a donation is the letter of thanks to the donor, which reminds that person or company of the importance and significance of their gift; of course, everyone likes to be recognized for their efforts. The key is to be confident about your request. Don’t be afraid to ask, be sure to say thank you and let them know that they can help by telling others about what they have done. It was a great experience and Cynthia was very well received. EDNF thanks her for her promotion of EDS and extending the presence of EDNF in this professional setting. ■■■
ANNOUNCING EDNF’S BOARD OF DIRECTORS FOR 2007
Chair: David Specht Vice Chair: Richard Goldenhersh
Director of Local Groups: Charlotte Mecum
Directors: Richard Blouse
Robin Coppi Terri Keyes Jeanne Kingsbury
L O O S E CONNECTIONS SPRING 2007 • PAGE THIRTEEN
EDNF THANKS
DR. JUDY GIDDY-KING BY ROBIN COPPI
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he Board of Directors of EDNF wishes to thank Dr. Judy Giddy-King for her service on the Board. She has served for the past four years with unfailing commitment. She served as Local Group Representative, helped as Associate Director of Local Groups and as Secretary to the Board of Directors. She remains an active member of EDNF and her local group, the Western New York Branch. Group leaders will remember the great part she played in our Local Group Representative meetings at the Los Angeles and Houston National Conferences, and we all remember the wonderful conference in Buffalo, New York planned, organized and hosted by Judy and her local group. We wish her best of luck in all her endeavors. ■■■
ONE-DAY EDS SYMPOSIUM IN RICHMOND A SUCCESS BY CHARLOTTE MECUM
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n January 20, 2007, the Richmond and Central Virginia Local Group held a one-day Ehlers-Danlos Symposium for Medical Professionals and individuals in the area who have EDS. Congratulations to Diana Harris, president of the local group, for doing a fantastic job in the planning and coordinating of the event, and to the group members and all involved who worked so diligently prior to and the day of the symposium. Everything ran smoothly, in spite of some last minute unavoidable changes. The Symposium and CME and CEU credits were made possible through cooperation with The University of Virginia and Sheltering Arms Rehabilitation Hospital. Forty-four medical professionals and 65 others participated in the Symposium, held at the Collegiate School in Richmond, Virginia. Speakers included Dr. Nazli McDonnell, Principal Investigator, Ehlers-Danlos Protocol at NIH; Dr. Sanjay Desai, Orthopedic Surgeon, specializing in upper extremity problems, West End Orthopedics in Richmond; Dr. Karen Sparrow, Director of the Transitional Doctor of Physical Therapy Program and Adjunct Assistant Professor of Physical Therapy at VCU in Richmond (as well as an EDS patient herself); and Dr. Maged Hamza, Anesthesiologist and Pain Management Specialist at the VCU Medical Center in Richmond. One of the most-appreciated sessions of the day was the Doctor’s Panel, during which Symposium attendees were allowed to ask questions of the presenters. The Silver Ring Splint Co., C&S Beads and Melaleuca were vendors present at the Symposium. ■ ■ ■
Are You Interested in Becoming a Board Member?
A
ny member in good standing may serve on the Board of Directors of Ehlers-Danlos National Foundation, Inc. The Board of Directors is comprised of individuals with a variety of backgrounds and experience. The balance is based on the standard of “Time, Talent, and Treasure.” Requirements for service include:
Time: A commitment to attend at least 4 teleconferences averaging 2 hours in length. Ability and willingness to travel at least twice a year for two-day meetings. Participation on at least one EDNF committee. Talent: Board members are sought who have areas of expertise that will benefit the foundation such as legal, medical, accounting, business, or knowledge of EDS. Treasure: Board members are expected to make a financial commitment to the foundation annually, in accordance with their means. Members also cover all expenses for travel and business on behalf of the foundation. Applications will soon be available on www.ednf.org or may be requested by e-mail to staff@ednf.org. Submission of an application does not imply acceptance; applications are reviewed at regularly-scheduled quarterly meetings. Applicants will be notified after such review. The Board of Directors may request further information from the applicant. ■■■
L O O S E CONNECTIONS PAGE FOURTEEN • SPRING 2007
LOCAL GROUP REPORT
O
ur local groups are off to a great start for the New Year. The new Regional Coordinators have had their first teleconferences with the group leaders in their regions and report many activities being planned or already started by local groups. The theme for this year is Educating Medical Professionals and it is exciting to see the enthusiasm of local groups to carry this out. One local group has already held a one-day symposium for medical professionals (see page 12) and several others are hoping to hold similar events. We are planning for a busy and successful “EDS Awareness Month” in May. Each local group will be holding an awareness event of some sort, so please watch the website and The Hinge for announcements about local area activities and support them in whatever way possible. However a local group chooses to create awareness about EDS, whether through a community event, a newspaper article or TV interview, it is important that our message and facts be accurate. EDNF has prepared a Communications Guide that can be found on the web site or can be obtained from a group leader or Regional Coordinator. All local groups should consult the guide prior to an activity to spread awareness; EDNF members are welcome to consult it for public appearances when they want to be sure of their facts and the Foundation’s message. If there is not an EDNF local group in your area (listed right) and you would like to consider organizing one, please contact charlotte@ ednf.org. We can never have too many local groups; they are an essential element of EDNF being able to pursue its mission. ■ ■ ■
OUR DEEPEST GRATITUDE TO OUR DONORS This year the demands for space in Loose Connections have grown beyond our available pages, and we are unable to publish the names of EDNF donors in each issue, instead saving them for a grand thank-you listing in the Winter issue. This means little visible change to our donors, however. Be assured that donations, and memorials in particular, will still quickly receive written acknowledgments of our gratitude, and the donor lists will be available on-line at ednf.org. There were many reasons to make the change. Publication dates are necessarily so far in advance that the rolls were always out-of-date by the time they reached you. Second, there is a very welcome increase in submissions to Loose Connections, which means more information about members and by members is waiting to be printed. Also, medical knowledge about EDS is virtually exploding, with multiple papers demanding our attention each issue. And most importantly, putting the list in the last issue of the year allows us to emphasize our donors’ worth with more than a simple list of names. We hope the benefits of having more pages for EDS information are already obvious; Loose Connections will continue to grow and expand its coverage in coming issues, and we look forward to your continued participation.
BY CHARLOTTE MECUM Alabama Alabama Group* • Kelly Tinnon, President, glueless@comcast.net, 256-399-4068 Arizona EDNF Action Association of AZ • Vicky Halleck, Organizer, ednfvicky@hotmail.com, 928-830-0011 Phoenix Metro Group* • Jennifer Scherr, Pres., jmgrimse@yahoo.com, 623-925-8708 California EDS Advocates of Northern CA* • Maggie Buckley, Pres., maggie_buckley@yahoo.com, 925-946-0384 Group of Greater San Diego* • Grace Berardini, Pres., gwranch@cox.net, 760-599-9478 Online Information Group* • Liza Sauls, Pres., thing1973@aol.com, 909-944-6778 San Fernando Valley Branch* - Shari Gamson, Pres., sfvbranch.ednf@gmail.com, 818-757-7355 Connecticut CT EDS Support & Awareness Group • Tara Lopes, Orgnz., yoursecretary@sbcglobal.net, 860-749-6003 Western CT EDS Support Group* • Sandra Buscher, Pres., universalm@comcast.net, 203-798-9401 Florida Central Florida Crackers Group • Cynthia Colabella, Pres., zoomzoom@thevillages.net, 352-735-1853 Emerald Coast Group* • Sue Jenkins, Pres., 1suejenkins@earthlink.net, 850-729-2882 South Florida EDS Group • Linda Simmons, Organizer, southfloridaeds@bellsouth.net, 954-434-1792 Georgia Atlanta Area Support Group* • Renee Hutchings, Pres., hutchingsrenee@hotmail.com, 404-610-0041 Indiana Michiana Branch* • Pam Malenfant, Pres., info@michianaednf.org, 269-429-8110 Kansas Mid Kansas Connection • Nathifa Love, Orgnz., luminations@gmail.com, 316-944-7520 Maryland Baltimore Metropolitan Branch* • Jill Loukides, Pres., loukides100@comcast.net, 410-757-6323 DC Metro Charter* • Sheri Collins, Pres., ipassoc@comcast.net, 301-570-5718 Massachusetts Eastern MA EDS Group • Rachel Donlan, Pres., easternmasseds@yahoo.com, 781-848-1341 Michigan Ann Arbor EDNF Group* • Katie Kerschbaum, Pres., info@a2-ednf.org, 734-741-5288 Minnesota Twin Cities EDS Support Group • Erica Sonnier, Organizer, twincitieseds@gmail.com, 651-458-4165 Missouri St. Louis Connection Group* • Stephen Bell, Pres., ehlersdanlos@charter.net, 314-322-1214 Nebraska Nebraska Group* • Linda Neumann-Potash, Pres., loosejoint@aol.com, 402-934-5908 New Jersey North Central NJ Group* • Lisa Schoenberg, Pres., lisa.cherry2sky@gmail.com, 732-566-1218 New Mexico NM Connects EDNF Group* • Angel Denardi, Pres., nmconnect@yahoo.com, 505-690-1690 New York New York City Group* • Jacqueline Cox, Pres., eds.nyc@gmail.com, 212-987-9821 Thousand Island NY EDS Group • Jo Ann Reff, Pres., crafty444@hotmail.com, 315-639-3218 Western New York Branch* • Dawn DeWein, Co-Pres., dmdewein@yahoo.com, 716-688-8291 Lynda Pixley, Co-Pres., lyndapixley@earthlink.net, 716-791-3049 North Carolina Piedmont Triad Branch* • Angie Smith, Pres., piedmont_ednf@yahoo.com, 336-694-9553 Oregon Pacific Northwest Group • Susan Burkett, Pres., sburkett@careerdirectionsnw.com, 503-234-7289 Rhode Island Southern New England Connections* • Karen Ferris, Pres., karenf30@verizon.net, 401-683-2680 Texas Dallas-Fort Worth EDS Group* • Julie Bentler, Pres., dfwedsgroup@verizon.net, 469-964-0859 Greater Austin EDS Group • Wilma Steenbergen, Pres., wilmasteenbergen@yahoo.com, 512-401-9710 Greater Houston Branch* • Michele Hegler, Pres., michele.hegler@sbcglobal.net, 281-486-5521 San Antonio EDS Group* • Irene Pierce, Pres., gizpie@sbcglobal.net, 210-392-3880 Virginia Northern Virginia Group* • Margaret Foote, Pres., ednfnorthernvirginia@comcast.net, 703-683-9637 Richmond & Central Virginia Group* • Diana Harris, Pres., dianamharris1@comcast.net, 804-272-6701 *Chartered Groups
EDNF 2007 MEMBERSHIP FORM
You can become a member online at ednf.org or by mailing or faxing this form to our office as provided below. Questions? Call us at 213-368-3800. EDNF now offers members the convenience of subscription membership. By checking the monthly box, your credit card will be debited that amount on the first of every month. Membership can still be purchased as an annual payment. Please check either annual OR monthly. Do NOT check both. Your membership will continue until you choose to cancel. Please see www.ednf.org for more information.
NAME* ADDRESS CITY STATE/ZIP
MEMBERSHIP TYPE MONTHLY AMOUNT YEARLY AMOUNT
COUNTRY E-MAIL DATE OF BIRTH TELEPHONE NO. GENDER
THREE WAYS TO BECOME A MEMBER!
Male
GENDER AGE
$ 2.50
$ 25.00
SUPPORTING
$ 5.00
$ 50.00
INTERNATIONAL
$ 4.50
$ 45.00
ACCESS
n/a
$ 15.00
TOTAL
Either mail this completed form with payment to:
Female
*Are you joining for your child? Please provide their information here. If you have more children, please list on a separate sheet. NAME
STANDARD
DATE OF BIRTH
Ehlers-Danlos National Foundation 3200 Wilshire Boulevard Suite 1601, South Tower Los Angeles, CA 90010 Or fax this completed form to EDNF at 213-427-0057.
Please provide a login name and password for online access to the member’s area of ednf.org. Password must be between 4 and 9 characters, letters and numbers only. This table is good for one EDNF account. Please print clearly. LOGIN NAME
PASSWORD
1ST CHOICE 2ND CHOICE 3RD CHOICE
Terms and Conditions: • Access level is a reduced cost membership with access to the member section of the website including one (1) EDNF login name and password. Access members will not receive mailings. Access members can download Loose Connections from the website. • Standard members will receive one (1) EDNF login name and password allowing complete access to the EDNF member area of the website. Standard members will have Loose Connections mailed to them. • Supporting members are eligible to receive two (2) EDNF login names. Please complete an application for each login name. Supporting members will have Loose Connections mailed to them. • All first time members receive a new member packet. Renewal members will not receive a packet. • If you have questions or need assistance with your login name and password, please feel free to contact us at memberservices@ednf.org or call us at 213.368.3800. • EDNF does not lend, sell or trade our membership list to any individual or organization. If you are interested in corresponding with other EDNF Families please sign below to accept the release of your contact information to those Families only:
Signature:
METHOD OF PAYMENT Note: All payments must be made in U.S. Currency drawn on a U.S. bank.
My check or money order payable to EDNF is enclosed. Please charge my: MasterCard Visa
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Card Number: Expiration Date: Name on Card: Signature: Billing Address of Cardholder: Please check if the same as member’s mailing address. Address: City: State: Zip: Country:
Presorted First-Class Mail US Postage PAID Los Angeles, CA Permit No. 3344
3200 Wilshire Boulevard Suite 1601, South Tower Los Angeles CA 90010
www.ednf.org L O O S E CONNECTIONS SPRING 2007
Write an Article. Here’s How!
We are always looking for Loose Connections articles. You can send in a contribution to our on-going columns or a suggestion for “Ask the Doctor.” And if you have an idea for an article, chances are you’d be the perfect person to write it. Loose Connections is the work of those who write it, so please: contribute by writing an article. GUIDELINES FOR CONTRIBUTIONS:
1. 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 tells us how to reach you for more information. 2. For photographs, attach to an e-mail to editor@ ednf.org three to five images that are at least 300 dpi resolution and 8 x 10 inches (2400 x 3000 pixels) and without compression beyond that performed by the camera when it was taken. In the e-mail, please identify the event or cause for the photographs, including any relevant identification (persons involved, date, photographer’s name if needed) and how to reach you for more information.
3. Following are the deadlines for 2007 issues; special arrangements can be made with the editors before these dates, but not afterwards. PUBLISHED FEATURES COLUMNS ON DUE DUE Summer Fall Winter
June 22 Sept. 7 Dec. 7
May 11 July 20 Oct. 19
May 25 Aug. 6 Nov. 5
Reviews: share with our readers the books or other media that have helped you deal with EDS or surrounding issues (for instance, chronic pain or communicating with medical professionals). Any review should include the important information about its subject; as an example, a book review should include the book’s title, its author’s name, the publisher and publication date, or a website its internet address, name of owner, and membership requirements if any. Reviews can criticize, but more, they communicate whether a Loose Connections reader should be interested in what’s being reviewed and why; reviews also should be under 900 words. “Sharing Our Golden Thread”: share your stories, the stories of how you live. Reveal what you have done to shine your golden threads, or those golden strands of your EDS loved one. Help Loose Connections strengthen our golden threads through
sharing our stories. As we blend our golden threads together, imagine what we can create! The first threads have already been read; now it is up to you. Help us weave together the story of our lives by telling the tale of your golden threads. “Golden Thread” submissions should be first person, in your own voice, and 500 words or less. “Ask the Doctor”: Do you have suggestions for questions to ask our Professional Advisory Network? Perhaps you’ve been unable to find an answer on your own, or the answers you’ve found have been confusing. Feature articles: if you have some information you think our readers need, then you should write an article around that information and send it to us. Who else will? For the first time, Loose Connections has the ability to offer ad space on a limited basis. If anyone needs information on this, then please write us, editor@ ednf.org. 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. Send us the stories you want to read!