L O O S E CONNECTIONS

The Magazine About Living With EDS Winter 2010

2011 Conference Announcement ................................................................................................... 2

From the Editor’s Desk Barbara Goldenhersh, PhD ........................................................................... 3

Cancer Treatment Advice for an EDSer ........................................................................................... 5

What I Wish I Had Known Sara E. Strecker ...................................................................................... 7

When to See a Doctor for Neck Pain Johns Hopkins Health Alert ..................................................... 8

Ideas to Live by: Helpful Hints for a New Year Ali Ruffolo .............................................................. 9

How Water Therapy Can Soothe Your Back Johns Hopkins Health Alert ........................................ 10

2010 Donors to EDNF .................................................................................................................... 11

Fibromyalgia & EDS: The Hypermobility Connection Amy Bianco .............................................. 20

Ehlers-Danlos Syndrome & IOM Report On Vitamin D & Calcium ............................................. 24

IOM Report on Vitamin D & Calcium Press Release ..................................................................... 26

Publisher’s Index/Staff and Credits ................................................................................................ 29

2011 Learning ConferenceHilton Baltimore Baltimore, Maryland July 21-23

SAve thE dATEJulY 21 - 23, 2011

EDNF is pleased to announce the

2011 Learning ConferenceHilton Baltimore401 West Pratt Street, Baltimore, Maryland

The hotel is ideally located in Baltimore’s Inner Harbor, where you’ll find superior shopping, dining, and entertainment. The Hilton Baltimore is adjacent to Camden Yards (Orioles’ Stadium) and just minutes away are the Hippodrome Theatre, University of Maryland, Johns Hopkins and M & T Bank Stadium, home of the Baltimore Ravens. For your comfort and enjoyment, the Hilton Baltimore features a heated indoor lap pool, sauna, whirlpool and gym overlooking Camden Yards.

Stay tuned for details on the agenda and this year’s exceptional physician line up!

Click to see 3D tours.Click to see hotel information.Click to see MapQuest.

WINTER 2010PAGE 3

FROM THE EDITOR’S DESK

IN medical schools teaching Western medi-cine, doctors are taught the adage, “If you

hear hoofbeats, think horses, not zebras.” That is, look for the simple answer to a medical prob-lem, that is the more likely answer. But there are many patients here to say that there are definitely zebras in this world and they deserve the best medical care as well as anyone. In my own story, I have Ehlers-Danlos syndrome. It is considered rare but current research is showing that there are far more with EDS than ever con-sidered before, many not diagnosed and misdiagnosed.

In my case, though I had many serious symptoms from in-fancy on, not a single doctor connected the dots. Doctors did not ask questions when I was unable to walk at 18 months and the X-rays showed my hips were not connected to my legs. My parents were told I would never walk although I did at 2-1/2. Dermatologists always mentioned my rare skin but none thought it important enough to look it up in the index of the medical book on their shelf; velvet skin would have taken them to Ehlers-Danlos syndrome.

I was 59 years of age by the time I was diagnosed in 2003. Since then, I have been on a mission to educate the medical community on this connective tissue disorder which involves great pain, bruising and fragile skin issues, fragile organs and exhaustion, among other issues. I continue to work with Ehlers-Danlos National Foundation (EDNF) to improve understanding, treatment and management of this disorder.

“I am again diagnosed

with something that

might be thought of

as a zebra disease.”

And now I am again diagnosed with something that might be thought of as a zebra disease and thus often missed in diagnosis. Thinking back, I noted there was a sharp pain in my right breast during Labor Day weekend. I ignored that as pain has been an issue always and I tend to note but ignore most. Rosh Hashona and Yom Kippur brought a time of introspection as it always does, as Jews everywhere consider how they can improve their caring of others, their possibilities for doing additional charitable

acts and good deeds and determine ways in which they may have fallen short of these things in the past year. It is a time when the book of life is opened for our introspection and closed with a prayer for another

year of life. Saturday evening ended this most holy time and it was with great shock that on Monday I was diagnosed with inflammatory breast cancer, a rare, extremely fast growing form of cancer which had apparently begun less than three weeks before but already had my breast extremely hot, greatly swollen, discolored and painful, my right arm swollen and the lymph nodes involved. 

Inflammatory breast cancer is found it 2% of all breast cancers. It is not seen in a mammogram or ultrasound. An MRI is required to see it and should be called for when a breast has major changes; a biopsy is required for a definitive diagnosis. My right breast began to get larger when I gained ten pounds suddenly. l noticed a great deal of swelling in the breast, distinctive heat coming off the breast, extreme heaviness of the breast and the color heliotrope (purple).

WINTER 2010PAGE 4

I was lucky that my internist and breast surgeon, after taking an X-ray to rule out something else, recognized it as cancer and told me I could not wait for treatment of this type of cancer. Many others are not so lucky. Problems with the breast are often misunderstood to be a breast infection. By the time the antibiotics are completed and no improvement has been seen, this fast growing cancer will have spread quickly to the bones and organs or the body. It is essential that, if you see drastic changes to your breasts which happen rapidly, make certain that doctors look further than the typical infections.

This form of cancer must be treated immediately and aggressively. I am undergoing six months of chemotherapy followed by a complete mastectomy, then six months of radiation treatments. Needless to say I have moments which swerve back and forth through both highs and lows, and I expect to have many more.

I am still trying to take in all that I learned from my various oncologists and to make sense of it all. The fight my body is undergoing seems to require all my energy, and includes an amazingly high level of pain which I cannot get under control to this point. I am going to have to use enough medication to at least mediate the continuous torture, something I have always avoided before but I am finally beyond the point where I can go on without medication. And yes, the answer is that cancer pain — I understand that it hits about 50% of cancer cases — is definitely worse than EDS pain. I would never have believed that there was something worse!

I really have been concerned by the vascular complications of my breast. Mine definitely does not resemble the photo online that shows a red, inflamed breast. In my case, the color is purple and the veins are swollen and distended, appearing like small aneurysms. My discussions with those who had been through this assured

me of the uniqueness of my condition; my talks with medical friends assured me that I needed to follow up on the problem. The oncologist feels that this may well be related to the bruising problems of EDS and feels that even if a vein busts, it should not spread the cancer. And better yet, the breast is beginning to soften, which means the chemo is doing what it is supposed to, melting the cancer so it can be removed.

The oncologist is very clear and knowledgeable but spares nothing. I will be having approximately 6-1/2 weeks of radiation at fifteen minutes per dose. I have ten infected nodes which will have to be excised when the mastectomy is done. The oncologist’s concern in relation to my EDS was clear. He told me that he would not do radiation if it were at all possible. Since he will be radiating the nodes which reside beneath the collar bone, the breast bone and the underarm, he will be radiating along the major arteries and veins. His concern is in regard to future aneurysms. He feels that the more fragile nature of the vascular system of someone with Classical EDS may well thin out those veins and thus make them susceptible to rupture in the future.

Thinking of “ologists”, one hates to have more of them than close friends or even family members. Mine are definitely nearing that. The ologists have each received all information I have on EDS and are truly discussing what it means to their issues. They also asked me to contact some of our great EDS doctors for suggestions such as surgery needs, and some of them were kind enough to provide answers, which are provided below in the event they can prove helpful to others.

It has become my signature to wear clothing with zebra designs throughout the EDNF Learning Conferences: I am a zebra and want others to know we are there and should not be ashamed of it. I received a great pink zebra;

WINTER 2010PAGE 5

cancer patients can be zebras, too. I am taking it with me to chemo to build awareness of medical zebras for all I come in contact with.

This cancer was discovered right after Yom Kippur, when one hopes to be written in the book of life. I was frightened by the timing of the diagnosis until my daughter shared what a friend suggested to her. The cancer had begun the year before; God may well have been letting me know quickly that it was there so that I can face it and make this year yet another worth living. It is with that outlook that I now face my year of heavy fighting to beat this cancer and continue the mission I set out for myself to make a difference.

I want to take a moment to thank each and every one who has lovingly signed my guestbook, sent cards, emails, books, hats, helpful items to make my life more bearable. I greatly appreciate each and every one of you, but my frequent exhaustion has made it difficult for me to personally thank each of you. Please understand and know that all you have done has meant a great deal to me! I thank you for the love you have shown and the prayers I have received.

Barbara Goldenhersh, PhDEditor-in-Chief

Cancer Treatment Advice for an EDSer“I have a surgeon who tells me that I will have chemo and then will operate doing a full mastectomy and removing affected lymph glands, etc. She requested that I ask of my esteemed medical team for some information on surgery for those of us with EDS. Thank you for your past and future work with those of us struggling with EDS and your help in my dealing with this latest hurdle.”

…Maintaining a positive attitude throughout treatment really does improve your prognosis.

As you know, the classical type of EDS increases tissue fragility. The most likely complication would be delayed wound healing or wound dehiscence (reopening) after the surgery. I generally advise surgeons to perhaps use a few more sutures than usual and to leave them in 150% as long as usual (so, if sutures would ordinarily come out in seven days, instead wait until day ten or eleven). Very importantly, your surgeon should NOT try to pull the sutures any tighter than usual, since there is also risk

of the sutures pulling right through the tissue being sutured.

Along the same lines, there is some increased risk of tissue tearing during the surgery. The only precaution would be to not pull or push anything any harder than necessary, and to just be prepared for such potential complications.

Significant bleeding during or after surgery is not usually a serious problem, but does happen in some patients. Usually it responds to the same medicines typically used for von Willebrand disease (ddAVP; Amicar).

All of the above comments represent my general advice for EDS-CL patients. Your personal history is an important part of the risk assessment, too. Any surgical complications that happened in the past are a bit more likely to occur in this surgery, too. By the same token, if you’ve had one or more major surgeries without soft tissue fragility, wound healing problems or clinically significant bleeding, then perhaps those specific problems are a bit

WINTER 2010PAGE 6

less likely for you (but no guarantees — so the surgeon needs to be prepared for any/all).

Separate from all of this is the issue of breast reconstruction. Good for you for looking at potential positives in this situation. However, I encourage you to talk with your surgeon about the potential risks of the various different reconstruction options. Not being a surgeon, I can’t give you advice about how intricate and complicated the different procedures are, but I’m sure there are differences. Unfortunately, you may be faced with a situation in which some of the newer techniques — like flap rotations of living tissue — might carry greater risk of complication when considering your EDS and soft tissue fragility. You might want to consider choosing the simplest operation, even if it might not be as good cosmetically as a more involved reconstruction.

Howard Levy, MD, PhD

Healing is obviously an issue but it will heal. [Your doctor] only needs to be conservative in her closure and should have the excisions well-opposed and in layers if possible. Biggest problem is opening of the wound not the underlying soft tissue so if sutured well, and left in place longer, good likelihood this will be okay.

Brad Tinkle, MD, PhD

I would suggest leaving sutures in place longer than the average person as the healing is slower, and she should close the incision with extra sutures. spot test her for tape allergies before surgery.

Linda Simmons

I don’t think there is anything special to say about this. Only that wound healing may take longer than average; might put a few extra stitches in and leave them a little longer than they would ordinarily do. If he/she thinks about putting a drain in after surgery, same issues would apply.

Clair Francomano, MD

As far as the EDS is concerned, because the tissues are less robust than normal, the three most important issues from the surgical point of view, are:

• extra gentle handling of the tissues is needed during surgery

• ensuring good hemostasis (control of bleeding) and

• because healing is slower than normal, using additional sutures (stitches) and allowing them to remain in place for longer.

Rodney Grahame CBE, MD, FRCP, FACP, FRSA

As for EDS classical and breast surgery, did a little Pub Med search which came up blank for the most part. Bruise and bleeding may be more than the non-affected individual. It always seems that way to me ( I have classical EDS too, quite severe, actually). Good news, in this particular surgery they tie off or use electrocautery to control bleeding!!

 Mark Lavallee, MD, CSCS, FACSM

WINTER 2010PAGE 7

WHEN I was little no one knew that my hypermobility was actually Ehlers-

Danlos Syndrome. At this point in life, I’ve known that I’ve been hypermobile for 20 years; but I’ve been diagnosed with EDS for only five years. I had the pleasure of recently attending an IEP (Individualized Education Program) meeting for a wonderful girl named Karen with EDS. I participated in a medical advisory capacity. In simple terms, an IEP is a meeting that discusses how the school can allocate resources in order to help a child with a disability perform to his or her full potential.

They had these meetings when I was in school, but not for me. I had no diagnosis, and there-fore nothing was done because from the school’s perspective nothing was wrong. I had a medical excuse for gym when I hurt myself, which was often, and just came to terms with the fact that I would never be physically fit. I didn’t know I could get a second set of books so my back didn’t hurt carrying them from class to class. I didn’t know that I could have a locker closer to my classes instead of across the building, or that extra time on tests would help with hand fatigue.

I spent a lot of time before this meeting trying to figure out what would be good modifications for someone with EDS to have. And I spent a lot of time grappling with the fact that I would have given almost anything in my youth to be physically fit. I vividly remember being bent over on the soccer field, panting, knowing

WHAT I WISH I HAD KNOWN

that I would never run that last quarter mile in the two minutes I had left in order to “pass.” I remember quiet tears falling down my face in second grade because my hand hurt so much and the teacher wouldn’t let me take a break from the timed writing. When I tore my ACL (anterior cruciate ligament) during a kickball game in fourth grade, I thought I was a failure

because I couldn’t do these things that came so easily to everyone else. It wasn’t a matter of effort; physically I could not perform these tasks. Looking back now, I realize that this was my body screaming at me that

something was wrong. But, other than knowing I was strange or different or weird, there were no answers. One thing I do remember though was that I just wanted to be like everyone else.

The following is a list I complied for Karen’s IEP. EDNF all ready provides a wonderful booklet which acts as a guide to having a child with EDS but these were a few additional items we came up with for Karen’s meeting and I felt I should share them with you.

• Chair cushions, not just for the EDSer but as a reward for other people in the class;

• Permission to get up and walk around the classroom during the day;

• Longer passing periods and extra bath-room breaks;

• A second set of books for home;

“I thought I was a failure

because I couldn’t do

these things that came so

easily to everyone else.”

WINTER 2010PAGE 8

• Lockers close to the classroom, or if the school is large, two lockers;

• The ability to carry water and to snack throughout the day;

• Physical activities are not graded based on skill;

• Participation in gym can be done in a dif-ferent capacity, i.e. scorekeeper;

• Extra testing time or computer-based tests;

• Scribes and note-takers for tests and class.

This would have made life easier for me, and I hope that my suggestions make Karen’s success in school much less of a struggle. I, personally, encourage early diagnosis. Perhaps if I had known what I know now, I wouldn’t have done such physical damage trying to be “normal.”

Sara E. Strecker

THE cervical spine, located in the neck, is subject to many of the same problems that

plague the lower back — muscle strains and spasms, disk degeneration and denervation, and spinal stenosis. About 10 to 15% of people experience neck pain at any given time. Most neck pain is short lived and gets better on its own or with simple self-care measures. But sometimes neck pain is a red flag for a more serious problem.

The neck supports the weight of the head, which can weigh 10 lbs or more. Although the head should be centered over the spine when we sit or stand upright, countless activities — such as sitting at a computer, reading, watching television, or eating — cause people to slump, rounding their upper back and tilting their head forward. This posture can lead to muscle strain or spasm in the neck. Muscle strain or spasm can also arise from constantly looking over or under one’s glasses, having poor sleeping posture, or taking extended car trips.

Many cases of neck pain get better on their own. If neck pain is linked to a recent recreational or work-related activity, the pain will likely decrease within two weeks, and treatment from a doctor is usually unnecessary. However, you

JOHNS HOPKINS HEALTH ALERT: When to See a Doctor for Neck Painshould see a doctor if your neck pain follows a serious injury or is accompanied by the following:

• headaches, fever, or weight loss

• pain that worsens at night

• difficulty walking, clumsiness, or weakness

• pain, numbness, or tingling in your fin-gers, arms, or legs

• problems with bladder, bowel, or sexual function

• discomfort or pressure in your chest

• severe pain over a bone that might indi-cate a fracture or injury to a ligament

• osteoporosis or extended corticosteroid treatment (increases your risk of fracture)

Also see your doctor if your pain lasts longer than two weeks. Waiting too long before seeing the doctor for neck pain decreases the likelihood that the pain will completely resolve.

WINTER 2010PAGE 9

AS this year ends and we usher in 2011, here are some tips to help live comfortably

with EDS.

• Athletic shoes: Walking challenges many with EDS. To help ease the aches on knees and legs, opt for comfort over style. It is wise to invest in a durable pair that supports your ankles and keeps you steady on your feet. Sporting goods stores offer a warranty to replace them within a year, no matter the reason. The small price, usually between five and fifteen dollars, is a good investment. Keep your receipt and remember to trade them in before the year is up. You will receive either a new pair or a credit for the price paid.

• Hand and foot warmers: These are available at Dick’s, Gander, Wal-Mart and others. For those living in cold climates or who simply live with cold hands, these nifty packets fit either in a glove or pockets, shoes or boots. Keeping hands and feet warm will help with dexterity and avoid the discomfort brought by being cold. The price is between fifty cents to two dollars.

• Socks: Socks with rubber nubs on the bottom are wonderful for walking around the house. The rubber nubs help avoid falls, plus your feet will stay warm!

• Heating pad: Keep a heating pad in the two rooms used the most, usually the bedroom and family room. For aches and pain when ice or medications are not enough, try a heating pad.

• Epsom salts: Soaking in a warm tub with these help joint aches and muscle soreness.

IDEAS TO LIVE BY: HELPFUL HINTS FOR A NEW YEAR

For those living with POTS or other issues, please remember to get up slowly from a warm bath.

• Pill boxes: A seven-day pill box is small enough to take with you when you are out.

• Ice packs: Keep a bag of ice ready in the freezer. Fill a half-gallon baggie with water, lay flat until frozen, and use as needed. They’re great for headaches, neck or back pain. Smaller ones are good for sprains or strains.

• Ready-to-eat meals: Keep frozen meals, such as Stouffer’s or Lean Cuisine, in the freezer for dinner or lunch. Soups or stews are other great choices. Save these for the days when taking the time and energy to stand up and cook is not an option. They come in individual or family sizes.

• Snack: EDS can present gastrointestinal challenges. Jello, pudding, yogurts, soft fruits, crackers and are handy ready to eat and usually easy on the stomach. Nutrition is important for healing and avoiding injury.

• Drinks: Liquid nutrition such as Ensure or Boost is readily available, in store brands as well. It tastes best when cold.

• Friends: Find a blog or support group online. EDNF has message boards and a Facebook page. Shared information, reaching out on the bad days, and knowing there are others who understand, when some cannot, is priceless!

• Knowledge: Inform yourself. Catch up on the EDNF website. If you are informed and learn ways to manage pain, discomfort, mobility and other issues, you take control. There

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is much comfort is knowing you are not useless or moderated by EDS.

• Family: There are good days and bad days. For me, a hug any time of day from my children, my mom, my husband, or a friend is instant comfort!

• Blankets: Wrap up in a favorite blanket for the couch or day beds. Do you have one that is well washed? The softness and feeling of soft fabric is wonderful physical comfort. I keep one upstairs and one downstairs.

• Loose pants: As wintertime is here, yoga, sweat, or athletic pants are loose, comfortable and still keep you warm. Restrictive clothing is not comfortable!

• Attitude: Perspective is powerful! EDS will try to run your life. Do not let it! Instead, adopt the attitude of: “I will manage my life and symptoms and not allow myself to be discouraged.” Attitude affects the body physically, mentally, and emotionally. You are your own source of comfort when you are armed with a positive attitude.

Living with Ehlers-Danlos means finding help where we can. The smallest of comforts and anything to take charge and help manage our symptoms are valuable tools.

Please share your tips on the EDNF website or Facebook page.

Ali Ruffolo

JOHNS HOPKINS HEALTH ALERT: How Water Therapy Can Soothe Your BackThe health benefits of water therapy have been acknowledged throughout history to help treat various ailments, including muscle pain. Today, we also use water therapy to manage various musculoskeletal conditions, including low back pain. Working out in water can increase your cardiovascular fitness, improve your flexibility, and help you relax. If you have back problems, aquatic exercise can also help to:

• Stabilize your lower back. Water naturally resists when you move. Exercising against this resistance, with the right pushing or pulling motions, gently develops muscle strength in your back, abdomen, and hips. When you target these core muscles, you help stabilize your posture and support your lower back. The deeper you immerse your body, the greater the resistance and the more challenging the water workout will be.

• Minimize pressure on your spine, joints, and muscles. Because water is buoyant, it sup-

ports your weight, thereby minimizing the pressure placed on your spine, joints, and muscles. This results in a number of benefits. First, the reduced stress can help alleviate pain and decrease the chances of further aggravating your back. Second, you can move through the water with relative ease, which enables you to stretch your back muscles and improve your range of motion. Third, you're able to perform movements in water that may be too difficult or painful to carry out on a hard surface during land-based exercises.

• Soothe and relax your muscles. Even without exercising, being in warm water as is generally recommended for people recovering from a back injury can relieve pain, reduce swelling, and relax your back muscles. In addition, the soothing, warm water environment may motivate you to exercise more consistently and, in turn, enable you to achieve better results from your exercise routine.

WINTER 2010PAGE 11

EHLERS-DANLOS NATIONAL FOUNDATION GRATEFULLY ACKNOWLEDGES THE INVALUABLE SUPPORT OF OUR DONORS IN 2010.

Marilyn AdelsteinIn memory of Selma Lebow, my very special forever friend

Alabama GroupBrian AlexanderMary Alice Troxel

In memory of Ed Koslosky, Jr.Lorraine & Daryl Amdur

Primrose School “dress down” dayAmerica’s Charities Distribution AccountJanet Anderson

In memory of Ruth Ann CooperKim Anderson Melinda AndersonThomas & Ann Marie ArgentieriAdrienne & Warren Armstrong

In memory of Ed Koslosky, Jr.AT&T United Way / Employee Giving CampaignUplifting Athletes Inc.Atlanta Area Support Group Conference

Scholarship ProjectIn memory of Dr. Audrey Lowe, Glenn Ross, Dr. April Leaman

Bettie & Albert BacarisseIn memory of Ed Koslosky, Jr.

Pam BadikIn memory of Tomas Badik

Margaret BaerIn memory of Ed Koslosky, Jr.

Katie BairPhyllis Baker

In memory of Ruth Ann CooperEmily BallBarbara BanksCatherine Barber & Patricia Swanson

In memory of Lucille HoffmanWanda BarlowElayne Pamela BaumgartMarvin Bellin, M.D.Elizabeth Belter

A

B

Alyson BenisonAlyson Benison’s Firstgiving Page

Frank BenisonIan BenisonRichard BenisonWanda Bennewitz

In memory of Glenn RossMari Beth Krause

In memory of Ruth Ann CooperThe Biomat CompanyBrian Weston BlamiresThe Blanchard FoundationJeffrey Michael Blilie

On behalf of Haidyn BlilieJoyce BlountLynn Bodner

In memory of Glenn Davis Ross – A life worth living and an inspiration to others!

Carissa BokelbergShari BorekAuntie Joie Bostwick

In name of Cathy, In memory of Heather Leigh Hawkins aka Lcy J

Mary BotknechtJessica BowenJo BraleyFiona BremnerCarole BrownDavid Brown

In honor of the memory of Nick ThorntonSteven BrownellSteven BrownerBeverly BroylesRobert BryantCraig & Jeanne BrysonMaggie BuckleyMary Budke

In memory of Ruth Ann CooperLeeann Burks

In memory of Ruth Ann CooperStacy Burks

In memory of Ruth Ann CooperKaylene ButlerPeter Byers

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EHLERS-DANLOS NATIONAL FOUNDATION GRATEFULLY ACKNOWLEDGES THE INVALUABLE SUPPORT OF OUR DONORS IN 2010.

State of California Calmoseptine, Inc.Julie Cartwright

In honor of all children and their parents who struggle with any conditions considered less than ‘normal’ in 2010

Acacia Nicole Brooke CavnessCenter Brunswick United Methodist

Church Bell ChoirIn memory of Lucille Hoffman

Jeff ChaceEliot & Sandra ChackGisele ChaghouriPaula Charlton

In memory of Ruth Ann CooperTrevor ChaulkElaine Cherry

In memory of my beloved friend, Selma Lebow, who passed away on September 4, 2010

Lori A. ChlubiskiKathleen & Bruno CiaralliMichelle CicchiniStephanie ClarkHarriet CohenMartha Colbert

In memory of Selma Lebow and in honor of her grandson Jonathan Corn

Michael ColonBeth E. Colvin

Glenn Davis Ross – May his memory live forever in the hearts of those who loved him

Pamela CombsIn memory of Brian Jenkins

Community Foundation For Southeast MichiganJoseph Conda

In memory of Ruth Ann CooperRichard Connell

In memory of Ruth Ann CooperDavid & B.B. Connor

In memory of Ed Koslosky, Jr.Carol Cook

In memory of Tyson MooreRobert & Janus Kay Cook

In memory of Brian JenkinsThe Christie Cookie Company

C Mark CooperIn memory of Ruth Ann Cooper

Debbie CostiganAlice Cox

In honor of our nephew Ed.Marjorie Crawford

In memory Edward Koslosky, Jr.

Walter & Bari Jo DavisKate DeFranciscoMick & Anne Dembrosky

In memory of Lucille HoffmanShelly DenesCrystal Deppert

In memory of Ruth Ann CooperTed & Ann Doege

In memory of Ms. Sandra HableAlesia & John Joseph DolanPenny J. Doss

In honor of our son Joshua that died and Karsten, his son, who is dealing with vascular type

Shirley & George DownsDeborah Ann DrazninGershie DubinskyMandi DugganPatricia Duncan

In honor of Catherine HerbesGaye N. DurstRachel Dworkin

Cathleen & Walter EagenRona & Tim Egner

In memory of Michael D. GainesGraham EisenhauerJim EisenhauerNancy EisenhauerKathy Embree

In memory of Ruth Ann CooperEnglesby Intermediate School PTO

May Activity NightEnterprise Holdings

In memory of Lorraine Earnhart

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EHLERS-DANLOS NATIONAL FOUNDATION GRATEFULLY ACKNOWLEDGES THE INVALUABLE SUPPORT OF OUR DONORS IN 2010.

Ruth & Family Espinosa-BaroneEDNF WNY 2010 Branch Contribution

Lauren & David Ewald

Fred FalknerIn memory of Glenn Ross

Timothy FeeneyStacey FeldmanErica FelinsSeymour & Darlene Ferley

In memory of Tomas BadikAlvin l Feterman

In honor of Vanessa BalterWayne FeuerMillie FingermanRichard & Barbara FischerAndrew FisherBarbara FisherHelaine FisherLorne FisherMerle FisherJanis Flavin

In memory of Selma LebowMargaret FooteRonni FordCarol J. FosterRobert & Cynthia Freiling

Shari GamsonARF Annual Resource Fund

Viviana Garcia Linda Garlisch

In memory of Ruth Ann CooperClarice Gauf

In honor of Selma, an amazing mother & grandmother of the London Family. We are thinking of the Londons in this sorrowful time.

David & Dalit GellerFrederick GiddyDr. Judy Giddy-KingMeghan Gilleylen

Andrea GinsburgFor the refuah shleima of Natalie Ginsburg

Barbara & Richard GoldenhershRichard & Lucille Goldsmith

Primrose School “dress down” dayAviva GoldsteinElaine Goodman

In loving memory of my very dear friend, Selma Lebow

Ruth GoodsteinSharri GordonCheryl Gowie Professor R. GrahameAndrew GrodnerJack GurevitchBrenda Guyton

Diane HackettIn memory of Meghan Hackett

John HackettIn memory of Meghan Hackett

Christine Louise HagmeierMarla Hallmark

In memory of Ruth Ann CooperAndrea HalpernJames N. Halpin

In memory of Glenn RossR.T. HandScott HarrisMarion HebdonBrian HeckerJan HempsteadMaryAnne HensonBrooke Herrman

In memory of Ruth Ann CooperBillie Hester & Family

In memory of Ruth Ann CooperHeather HirschelDebra HoepkerMargaret Hoerr

In memory of Ruth Ann Cooper

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WINTER 2010PAGE 14

EHLERS-DANLOS NATIONAL FOUNDATION GRATEFULLY ACKNOWLEDGES THE INVALUABLE SUPPORT OF OUR DONORS IN 2010.

Donald Hoffman & Co-Workers at the NATO SEASPARROW Surface Missile System Project OfficeIn memory of Lucille Hoffman

Robin HollowayLyla & David Homan

In memory of Ruth Ann CooperTracy HorstmannEdward Horvath

In memory of Ryan HanelineDaniel Houston

In memory of Ruth Ann CooperLynda HovingMargery Howard

In memory of Ruth Ann Cooper

IBM Employee Services CenterEllen IcocheaiGive.com Holding LLCIPRO

In memory of Thomas JaksonITW Foundation

Stephanie JaffeIn memory of your beloved brother, Glenn

Dianna JamesIn memory of Ruth Ann Cooper

Heather JefferyMelissa JeraceStephen & Catherine JeronisVenetta Ikya JohnsonJohnson & Johnson Family of CompaniesBryan Johnston

In memory of Ruth Ann CooperJP Morgan Chase & Co.

On-going payroll deduction (Jeffrey Judd)

Kaiser Permanente Community Giving CampaignKaiser Permanente Employee Giving Campaign – Nancy Mahoney

Rachel KarwowskiNancy Kaszynski

In memory of Lawrence Prenatt, Sr.Seth Addison KaufmanSandra Kay BarrGeorge & Elayne KeehfussW. Keith Jones

Swim-a-thonBarbara KellerPat KellyRose Kelly

In memory of Ruth Ann CooperTerri Keyes

Conference Scholarship Project – In memory of Grace Berardini

Gary KingJeanne Kingsbury

Doctors’ Meeting / EducationAlford & Nettie Kinsey

In memory of Ed Koslosky, Jr.Randi KlecklerPatricia Klesel

In memory of Ed Koslosky, Jr.Linda Knuppel

In memory of Ruth Ann CooperCarol KopolowElaine Kotler

Alyson Benison’s Firstgiving PageLinda KrinnTanuja Kulkarni

La Grange Roadrunner Square Dance ClubIn memory of Ed Koslosky, Jr.

Dong LamTorryn LamMark LaneThomas Langan

In memory of Glenn David Ross

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EHLERS-DANLOS NATIONAL FOUNDATION GRATEFULLY ACKNOWLEDGES THE INVALUABLE SUPPORT OF OUR DONORS IN 2010.

Ross Langan McKendreeIn memory of Glenn Davis Ross

Maurene LangeIn memory of Edward Koslosky, Jr.

Richard LangworthIn memory of our dear friend’s sister, Selma Lebow

Frances LannonKim Lee

In memory of Carol MackAmy Leesman

In memory of Ruth Ann CooperRandee LefkowLeft Paw Press / Lauren OriginalsDalia LehmanLemonade Pi, Inc.Heather LeonardKathleen LerchJoann Lero

In memory of Edward Koslosky, Jr.Brenna LevinMarjolaine LewisLexisNexis Cares

Matching Gift ProgramLinda Lieberman

In memory of Glenn David RossM. Lintner

In memory of Tyson MooreNancy Lowery

In memory of Ed Koslosky, Jr.

Nancy MahoneyElisabeth MakhoulRichard MalenfantSuzanne Malhenzie

In honor of friends with EDS; the neighborhood children raised this money by holding a lemonade stand

Eliyahu ManManchester Valley High School

Movie Night FundraiserGladys Manheim

In Memory of Glen RossLauren Marani

In memory of Patrick Clancy

Kathy MartinIn memory of Glenn Ross

Marc & Szeman MaslowDaniel Materna

In honor of a real good guy and a great friend, Jeremy Semanoff

Susan MathesonChristmas gift to her nephew and his family

Joan (Joni) McClellandIn memory of Mildred M. McClelland

Nancy McGheeJeannee McKinley Jones\Robert McMillan

In memory of Nicholas ThorntonMedco Employee Giving CampaignParker Medical Associates LLCMarcia Meeker

In memory of Ruth Ann CooperStacy Mendelsohn

With heartfelt sympathy, in memory of SelmaGerald & Delora Meuleners

In memory of Ryan PalmersheimMidwest Central Community Unit

Schools District No. 191In memory of Ruth Ann Cooper

Kiran MilunskyConnie Montgomery

In memory of Ruth Ann CooperJulie Moore

In memory of Ruth Ann CooperSusan Moore

In honor of a childhood friend, Tyson Moore; may he and his family find peace

Gretchen MorrisIn memory of Ruth Ann Cooper

Beverley MoscovitchPaul Mostinki & Lana AnkinKimberly Muhlhauser

In memory of Anat, a great friend who will never be forgotten.

Linda H MurphyIn Honor of a remarkable woman, Selma

Kenneth MusickBeata Myhill

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EHLERS-DANLOS NATIONAL FOUNDATION GRATEFULLY ACKNOWLEDGES THE INVALUABLE SUPPORT OF OUR DONORS IN 2010.

NACE International Western States Corrosion SeminarIn memory of Colleen Penley

Barbara NathansonIn memory of Glenn Ross

National Fuel Gas Company Foundation: National Fuel’s Employee Charitable Giving Program & the National Fuel Gas Company

Ouida M NaylorSabrina NeeleyLaurie NelsonWilliam & Cynthia NemceffNetwork for GoodLinda Neumann-PotashNew Hanover United Methodist ChurchJoe NiemannJulie Nieuwkoop

In memory of Ruth Ann CooperRebecca Noal

Money from T-shirt salesNonprofits Insurance Alliance CaliforniaJohn & Elizabeth Nunziato

Maureen O’NeillPeggy & Dennis O’Neill

In memory of Lawrence Prenatt, Jr.Wilma Ogden

In memory of Ruth Ann CooperTammy OsuzikVernadene Otto

In memory of Howard Otto

Mary PainterCheryl Palmersheim

In memory of Ryan PalmersheimDebbie Panoushek

In loving memory of Nick Thornton

Michele Partridge-LaneWilliam Joseph Pasquina

In memory of Selma LebowScott Paticoff

In honor of my friend Sara PtachikKeith & Kathleen PattonJohn Paul Schellenberg

In memory of Glenn RossElaine Peltin

In memory of your loving mother SelmaCarole Pennington-EhlersKathryn PeoplesJohn & Anne PerdochElayne Nancy Peters

In memory of Nicholas ThorntonJudy and Mike Pidgeon

In loving memory of Michael D. GainesPiedmont Triad BranchDanielle PillionIngrid PokrassCharlie & Janice Porter

In memory of Ed Koslosky, Jr.Sarah & Jack Puffenberger

In memory of Tyson MooreBonnie Purks

Andrea RafofskyRichard Randy Adzgery

In honor and memory of Ed Koslosky, Jr.Sherri Reifsteck

In memory of Ruth Ann CooperRobert Reiner

In memory of Anat AbramskySue Reining

In memory of Ruth Ann CooperMichelle Reynolds

In memory of Ruth Ann CooperLettice RhodesSandra Richard

In memory of Tyson MooreMaggie Ridgely

Richard J RiemenschneiderDelores Ring

In memory of Ruth Ann Cooper

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EHLERS-DANLOS NATIONAL FOUNDATION GRATEFULLY ACKNOWLEDGES THE INVALUABLE SUPPORT OF OUR DONORS IN 2010.

Linda RitchieMr. & Mrs. Cecil Rives

In memory of Ed Koslosky, Jr.Diane Robarge

In memory of Tyson MooreCleo Robinson Lamb

In memory Edward Koslosky, Jr.David & Sandra Rochelle

In memory of Ed Koslosky, JrJennifer RodgersJonathan RodisCharles & Lisa RogersMichael Rogers

This donation is on behalf of Linda Ann Rogers, who passed away on 2/2/2010 after a long battle with heart disease and EDS

Charlotte Van RosenbergIn memory of Ed Koslosky, Jr

Randy RosetteIn memory of Lucille Hoffman

Judith RossIn memory of Glen David Ross

Bonnie RothsteinNancy RuffAlicia Ryley

Bill’s memorial gift

Tim & Janine SabalAlbin M. Sabal Matching Fund/EDS Sabal FoundationStephanie SailorEnid SalzedoRonna SalzedoSimon SalzedoSan Fernando Valley BranchJoe SanchezFelice Saracino

Primrose School “dress down” dayElizabeth N. SaundersJoe Scherer

In memory of NicholasMarissa SchimmelSchoolHeartJames Schroeder

In memory of Edward Koslosky, Jr.

Evan & Lindsay SchubeDonation for Blilie Charity

Duane SchultzITW Matching Gift

Diane ScottIn memory of Ruth Ann Cooper

Gregory SeguraDave SeibertCara SemanisinPeter SerraMichael SerraoSusan M. ShannonPatricia Sheehy

Primrose School “dress down”” day Cathy ShehanEmily Ann ShermanSandra Siddall

In memory Edward Koslosky, Jr.Yoni SilbermanSilver Ring Splint CompanySusan SilvermanLinda SimmonsBeverly Singer

Deepest sympathy in the loss of your beloved SelmaTiffany SkladanDavid & Doris Smith

Primrose School “dress down” dayFamily SmithJeffrey & Mary Beth Smith

In memory of Ruth Ann CooperLori A. Smith

In Memory of Nicholas Joseph ThorntonSammey Smith

In memory of Ed Koslosky, Jr.Mr. Clarence SnyderJudith Sobel

Conference Scholarship Project: In memory of Mrs. Foote

Rosemary SoldivieroCatherine SonesLinda South

In memory of Ed Koslosky, JrSouthern New England ConnectionsSouthtowns Ear, Nose & Throat, LLC

In memory of Lawrence Prenatt Jr.Serena Speaker

In memory of Ed Koslosky, Jr.’s courageous battle with this disease.

Herbert SpiegelIn memory of our Dear Cousin Selma Lebow

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EHLERS-DANLOS NATIONAL FOUNDATION GRATEFULLY ACKNOWLEDGES THE INVALUABLE SUPPORT OF OUR DONORS IN 2010.

EWALD TractorIn memory of Ed Koslosky, Jr.

Julia Ann TrempIn memory Holly DeCair

Ronald TrempJoyce Trompeter

In memory of Ruth Ann CooperThomas P. Tumminia

In memory of Gerad Marmion

Kari UlrichUnited Synagogue Youth

Tikum Olam ProgramUnited Way of New York City Designation Acct.United Way of Southeastern PennsylvaniaUnited Way of the Bay AreaSandra UpeguiEriko Usui

Julie Vance BergIn memory of Ruth Ann Cooper

Megan VandenburgMarla Vaughn

In honor and memory of Selma LebowVehicle Donation Processing Ctr. (Joseph

Tannuzzo, Michelle Vilagi)Susan VelkyJustin Villareal

Keith James WagnerJohn WahlersMarilyn & Traylor Walker

In memory of Ed Koslosky, Jr.Vince & Kate Walker

In memory of Alice PolomisLawrence Wall

Susan B. SpitzerDave & Deborah SprecherSt. Peters Episcopal Church

In memory of George HoodDonna StalkerJean StanleySusan & Harvey Starr

Hope you had a wonderful 50th wedding anniversary celebration

State Compensation Insurance FundLaura Ringelspaugh StephensVicky Sterling

In memory of David Bridgeman, wonderful father to Sadie Bridgeman

Dr. & Mrs. Mel SternbergIn memory of Glenn Ross

Robert and Amy StewartIn memory of Glenn Ross

Nancy M. StichJennifer StillwellDebra & J. Kenneth StoutRebecca Elizabeth StrongMargery StroudSusan & Stuart Needleman

In memory of your sister and sister-in-law, Selma Lebow

Rachel Swenson

Kim TabacNaomi TaffetRichard M. Taffet

Zebra braceletsSusan S. TaffetSusan Talbott Frank

In memory of Ruth Ann CooperW.N & Myrna Thiessen

In memory of Edward Koslosky, Jr.Catherine ThrasherDr. Bradley TinkleRobin TobackMadonna Rose Tomasulo

In memory of Lawrence PrenattAlma and Dick Tomlin

In memory of Ruth Ann Cooper

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EHLERS-DANLOS NATIONAL FOUNDATION GRATEFULLY ACKNOWLEDGES THE INVALUABLE SUPPORT OF OUR DONORS IN 2010.

Kimmerly M. WilliamsDebra Woiwode

In memory of Ruth Ann CooperN. Wong

In loving memory of my dear friend, Selma Lebow; I will always treasure our friendship and memories.

Craig WoodleyIn memory of Ruth Ann Cooper

Lianna Wyeth-Charles

Luan YarnoldIn memory of Ed Koslosky, Jr.

Cherie YeltonStefanie Yurus

Jo Ann & Conrad ZwernemanIn memory of Ed Koslosky, Jr.

Z Coil – Happy Feet

Listings are complete as of December 1, 2010; if anyone was overlooked, please accept our apologies and thanks. Names are typeset and alphabetized as submitted on our donor forms.

Teresa WaltonTommy & Mary Lou Wardell

In memory of Ed Koslosky, Jr.Washington University in St. LouisWaste Gas Fabricating Co., Inc.David Watkins

In memory of Timothy Van PattenNorma Watson

In memory of Ruth Ann CooperRichard Ways

In memory of Ed Koslosky, Jr.Jim & Belinda Weatherly

In memory of Edward Koslosky, Jr.Patricia Webb

In honor of Ed Koslosky, Jr.Sally H WeberLinda Weimer

In memory of Ruth Ann CooperMisty WeingerJason WeisserJanet WeissmanWilliam & Geraldine WestApril S. WestbergLinda Weyhrich

In memory of Ruth Ann CooperLaura WhittRoy & Laura Widmer

In memory of Ryan PalmersheimMartha Willard

In memory of Ruth Ann Cooper

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Y

Sherman and Teri Gamsonare proud to honor

Shari GamsonFor her leadership as the

Founder and long-time President of the San Fernando Valley (SFV) Branch of EDNF

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FIBROMYALGIA & EDS: THE HYPERMOBILITY CONNECTION

A DIAGNOSIS of fibromyalgia can come as a great relief at first. It is good to

know that your chronic widespread pain follows a recognized pattern, and that your fatigue, poor sleep quality, and cognitive fog are not harbingers of malignancy or some dreaded autoimmune disorder. But living with fibromyalgia can be confusing and deeply frustrating, because the diagnosis offers no specific biological explanation for its symptoms.

Recent work is confirming the hunches of visionary clinicians and researchers by bringing into focus a significant area of overlap between FM and an under-recognized connective tissue disorder called joint hypermobility syndrome (JHS). Hypermobility is the medical term for loose or double-jointedness, in which a person’s joints extend beyond the normal range. Many hypermobile people are asymptomatic; but when the trait is coupled with musculoskeletal pain and other complaints it becomes joint hypermobility syndrome, which many experts now agree is indistinguishable from Ehlers-Danlos syndrome, hypermobility type (EDS-HM) (Tinkle et al 2009). There are six major types of Ehlers-Danlos syndrome, all of which are characterized by defective protein synthesis in the extracellular matrix due to genetic factors. Like FM, EDS-HM is a diagnosis of exclusion, as the genetic defect that causes it has not yet been identified. EDS is not an autoimmune disorder; nor is it primarily inflammatory. It is a structural (or mechanical) disorder.

The extracellular matrix functions as the scaffolding of the body, found in the bones and connective tissue, as well as the skin, lungs, digestive and vascular systems. For the EDS-HM patient, it is helpful to think of pain as a three-layered system. At the core may be

orthopedic defects like developmental hip dysplasia, which is often mild but nonetheless contributes to malalignment. EDS-HM patients are also prone to painful spinal problems such as scoliosis, spondylolysis, and spondylolisthesis. Next, the ligaments around the joints are lax, which makes the joints unstable and prone to injury. On top of all this are the muscles and tendons, themselves disordered at a molecular level, which must overcompensate for the lax ligaments and hold everything together. It is not surprising that they are prone to repetitive strain and often will clamp down in painful trigger points, compressing nerves in turn. These trigger points tend to appear in many of the same locations as the tender points in FM.

In their new book Hypermobility, Fibromyalgia and Chronic Pain, rheumatologists Dr. Alan J. Hakim and Prof. Rodney Grahame, together with physiotherapist Rosemary Keer, have painstakingly compiled and examined the most recent evidence for a link between FM and JHS (or EDS-HM). Many, many people who are diagnosed with EDS-HM have already received a diagnosis of FM. It is not known how many FM patients are hypermobile, but there have been some suggestive studies. One small study limited to women found that 46.6% of those with FM were hypermobile compared with 28.8% of the controls (Sendur et al 2007). In another study of women the numbers were 64.2% among the FM group vs. 22% among the controls (Ofluoglu et al. 2006).

Perhaps most suggestive is the similarity in the non-musculoskeletal symptoms of EDS-HM and FM. Anxiety is a common symptom in both FM and EDS-HM. One fairly large study of hospital outpatients found that among those with panic disorders specifically there was a

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67.7% incidence of JHS, compared with 10.1% and 12.5% in two control groups (Martin-Santos et al. 1998). The same study also showed a significant positive correlation between joint laxity and somatic anxiety, which is manifested as muscular, cardiovascular, and gastrointestinal symptoms, among others. There are probably a number of reasons for this: chronic pain causes anxiety, and self-management of JHS demands constant vigilance. But recent research in a number of areas is suggesting that many of the non-musculoskeletal complaints of FM and EDS-HM patients — fatigue, poor regulation of circulation and blood pressure, irregular bowel habits, and disturbed sleep, for example — may be presentations of autonomic dysregulation.

The autonomic ner-vous system regulates those functions of the body that are usually beneath conscious control, such as heart rate, breathing, and digestion. Its dysregu-lation in FM has been well studied, and it is increasingly being in-vestigated in EDS-HM as well. A study of JHS patients found a significant preponderance of autonomic symptoms such as dizziness or lightheadedness, palpitations, heat intoler-ance — and of course, fatigue — compared to controls (Gazit et al. 2003); and the authors propose blood pooling the lower limbs as one possible mechanism. As Dr. Alan Pocinki ex-plained at this year’s annual conference of the Ehlers-Danlos National Foundation, venous pooling and low blood pressure from lax blood vessels may trigger a compensatory increase in adrenaline, which disrupts sleep, causing fa-tigue, which leads to another compensatory increase in adrenaline, in a vicious cycle that in many patients leads over years to “autonom-ic depletion.” When depleted in this way, the autonomic system “over-responds” to stresses, leading to wrenching boom and bust cycles in

energy and mood, and fluctuations in heart rate, blood pressure, and digestion.

Another possible source of autonomic dys-function is excessive mobility in the neck. In a 2008 study, FM researcher Andrew Holman reported that 71% of the FM patients and 85% of those with chronic widespread pain exhibited abutment or compression of the cervical spinal cord in extension views on MRI, leading him to speculate that chronic irritation in this region might give rise to dysautonomia, as well as to widespread, referred pain (Holman, 2008). Of course, hypermobility of the neck is a common concern among EDS-HM patients, many of whom must brace their necks, and some even

require surgery to stabilize them.

The American College of Rheumatology’s new preliminary guideline for the diagnosis of fibromyalgia (Wolfe et al. 2010) makes no mention

of hypermobility, even though it can be measured in the doctor’s office with a physical examination and history. Patients can test their own hypermobility with this simple test Drs. Hakim and Grahame have developed:

1. Can you now (or could you ever) place your hands flat on the floor in front of you without bending your knees?

2. Can you now (or could you ever) bend your thumb to touch your forearm?

3. As a child did you amuse your friends by contorting your body into strange shapes or could you do the splits?

4. As a child or teenager did your shoulder or kneecap dislocate on more than one occasion?

“Another possible

source of autonomic

dysfunction is excessive

mobility in the neck.”

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5. Do you consider yourself double-jointed?

If you answer yes to any two of these questions you have an 84% chance of being hypermobile.(Hakim AJ, Grahame R 2003)

It is estimated that 10 to 15% of the population is hypermobile (Beighton, 1989), but among those who suffer from chronic widespread pain the figure is surely much higher. Dr. Grahame reports that an astonishing 45% of routine general rheumatology referrals are hypermobile, and estimates that “for every patient in England with [B]JHS fortunate enough to be correctly diagnosed by a rheumatologist, there are nineteen others who are not, and whose hypermobility passes unheeded, undiagnosed and, presumably, untreated” (Grahame, 2008).

This is indeed tragic, as Dr Grahame goes on to say, for a number of reasons. Accurate differential diagnosis of EDS-HM is crucial because it is closely related to other heritable disorders of connective tissue (HDCT), such as the vascular type of EDS or the Marfan syndrome, which carry the risk of potentially lethal complications like aortic dissection or spontaneous organ rupture. The classical type of EDS, which can be difficult to distinguish from the hypermobility type, is characterized by fragile skin, which leads to poor wound healing, and complicates surgery. Even though these types of HDCT can be diagnosed molecularly, it is important to remember that the symptomatology among them and EDS-HM is not discrete. Thus it is routine for all HDCT patients to be given a baseline echocardiogram, and many physicians recommend that they be monitored for premature osteoporosis, eye problems, and other serious complications. Furthermore, misdiagnosis leads to mistreatment; procedures that may be straightforward for “normomobile” people can be disastrous for the hypermobile, and push them even further down the wrong diagnostic path.

Perhaps most importantly for many patients, a diagnosis of EDS-HM narrows the cognitive frame for understanding their symptoms, making it easier to determine their limitations and adhere to treatment and management protocols. The EDS community is small and very supportive; communication among its members has saved many of us years of painful trial and error.

So if you suffer from the baffling symptoms of FM and suspect you may be hypermobile — can you touch your tongue to your nose or scratch your own back? — I urge you to seek more information about this under-appreciated condition. A good place to start is at www.hypermobility.org or www.EDNF.org. Besides the Hakim/Keer/Grahame book I also recommend Dr. Brad Tinkle’s new book Joint Hypermobility Handbook: A Guide for the Issues and Management of Ehlers-Danlos Syndrome Hypermobility Type and the Hypermobility Syndrome.

Amy BiancoMedical News Editor for Loose Connections

References

Beighton, PH: Hypermobility of Joints, 2nd Ed. (Berlin: Springer Verlag, 1989).

Gazit Y, Nahir AM, Grahame R, et al.: “Dysautonomia in the Joint Hypermobility Syndrome,” Amer J Med, 115(1):33-40, 2003.

Grahame, R: “Hypermobility: an important but often neglected area within rheumatology,” Nature.com, 2 September 2008.

Hakim AJ, Keer R, Grahame R. Hypermobility, Fibromyalgia and Chronic Pain, 1st Ed. (London: Elsevier, 2010, ISBN-13: 978-0702030055).

Hakim AJ, Grahame R: “A simple questionnaire

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to detect hypermobility: an adjunct to the assessment of patients with diffuse musculoskeletal pain.” International Journal of Clinical Practice 2003; 57:163-166.

Holman AJ: “Positional cervical spinal cord compression and fibromyalgia: a novel comorbidity with important diagnostic and treatment implications,” J Pain 9(7):613-22, 2008.

Tinkle BT, Bird HA, Grahame R, et al.: “The lack of clinical distinction between the hypermobility type of Ehlers-Danlos syndrome and the joint hypermobility syndrome (a.k.a. hypermobility syndrome),” Am J Med Genet 149A(11):2368-70, 2009.

Ofluoglu D, Gunduz OH, Kul-Panza, Guven Z: “Hypermobility in women with fibromyalgia syndrome,” Clin Rheumatol 25(3):291-3, 2006.

Martin-Santos R, Bulbena A, Porta M, et al.: “Association between the joint hypermobility syndrome and panic disorder,” Am J Psychiatry 155:1578-1583, 1998.

Sendur OF, Gurer G, Bozbas GT: “The frequency of hypermobility and its relationship with clinical findings of fibromyalgia patients,” Clin Rheumatol 26(4):485-7, 2007.

Tinkle, Brad T. MD, PhD (with various contributors): Joint Hypermobility Handbook: A Guide for the Issues & Management of Ehlers-Danlos Syndrome Hypermobility Type and the Hypermobility Syndrome (Greens Fork: Left Paw Press, 978-0-9825771-5-8, 2010)

Wolfe F, Clauw DJ, Fitzcharles MA, et al.: “The American College of Rheumatology Preliminary Diagnostic Criteria for Fibromyalgia and Measurement of Symptom Severity,” Arthritis Care & Research 62(5):600–610, 2010.

COMING SOON TO A COMPUTER NEAR YOU

EDNF is pleased to announceA new, secure members-only message board solution is in the works! We hope to launch just after the new year, and will provide all EDNF

Members with step-by-step instructions to make the transition as smooth as possible. Thank you for your patience on this matter!

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ON November 30 the Institute of Medicine released a report changing

the recommended dietary allowances for calcium and vitamin D (see their press release immediately following this story), which generated a great deal of conflicting and confusing news coverage. Loose Connections asked EDNF Professional Advisory Network member Dr. Howard Levy of Johns Hopkins University to clarify calcium and vitamin D supplementation for the EDS community.

Are measured blood levels meaningful for calcium and vitamin D?  

Blood levels for vitamin D are meaningful. The most useful is 25-hydroxy vitamin D (or 25-OH-vitamin D). Reports will often separately list D2 and D3, which indicates supplemented vs. naturally produced vitamin D, but that is likely of no clinical consequence.

Measured levels of calcium are meaningless, however. Most calcium is stored in bones and within cells, and thus can’t be measured in the blood. There are regulatory mechanisms that generally work very well to keep the calcium concentration in the blood within a fairly narrow range.

For vitamin D, below what threshold do you recommend supplementation, and generally in what amounts?  

Evidence is very good that the minimum acceptable level of 25-OH vitamin D is 20 ng/ml. Most evidence is on maintenance of bone density, but even for that, there is some debate about whether higher levels might be better. One of the points made by the IOM report is that it is too soon to claim as medical fact the

EHLERS-DANLOS SYNDROME & I.O.M. REPORT ON VITAMIN D & CALCIUM

potential benefits other than bone health that have more recently been attributed to vitamin D supplementation, because they haven’t yet been studied well enough. However, there is a big difference between insufficient evidence to prove a benefit versus evidence to prove lack of benefit. So these additional benefits represent a gray area. Improved mood, better pain control, and more energy are all potential benefits that are likely of particular interest to folks with EDS. I’m not commenting on the proposed heart disease- and/or cancer-preventing benefits, since there is even less data to support those ideas; and they aren’t related to any EDS-specific complications.  

So, if there are additional benefits to vitamin D supplementation, what is the minimum blood level required to achieve them? Expert opinion varies on how much one might need for mood/energy/pain benefits (and remember that we don’t even know for a fact that this is a real benefit). Most experts pick various levels somewhere in the 30s. Since just about everyone agrees that vitamin D toxicity (adverse effects from too much vitamin D) doesn’t happen until levels are at least above 100, I try to get everyone to a level of at least 40 ng/ml. Some of my patients prefer to get up into the 60s or higher.

In general, I prescribe 50,000 units of vitamin D (preferably D3, but any form is better than none) once per week if the blood level is below 30-35 ng/ml. Initially, I’ll prescribe a 12-week course, then recheck sometime after that. If the level is then acceptable (>40), I recommend an over-the-counter supplement of 1000-2000 units every day. If the level doesn’t reach 40 with 12 weeks of prescription supplementation, I often prescribe 50,000 units per week on an ongoing

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basis, measure levels every 3-6 months, and if/when levels get nicely above 40, then switch to 1000-2000 units per day.

The IOM report summary makes a brief mention of potential toxicity from too much vitamin D. That’s why I check levels for all of my patients, and only recommend high-dose supplementation for those who have low values. I’m prepared to caution patients to decrease their intake if levels approach or exceed 100 ng/ml. Interestingly, in the 3 to 4 years that I’ve been checking and prescribing vitamin D, I have yet to have a single patient whose level came anywhere near this.

For calcium, if my blood level is within normal range, should I supplement anyway? Should I worry about consuming too much calcium?  

For most people, a total daily calcium of 1000-1500 mg is recommended. It is hard to absorb calcium from the diet. Only a small proportion of a single large supplement is likely to be absorbed. Ideally, one should take 100 mg 10-15 times per day. Since that’s not very practical, I recommend consuming several servings per day of calcium-rich food, such as citrus and/or calcium-fortified low-fat dairy. For most people, that’ll only provide 400-600 mg per day. (Note that many foods actually provide less calcium than claimed on the label.) Most supplements provide 400-600 mg per dose, and only a portion of that will be absorbed, so I also suggest supplemental calcium twice daily to make up the difference between average nutritional intake and the overall goal.

The main benefit of calcium is to maintain bone density.  It probably doesn’t offer the various other benefits that vitamin D is now thought to possibly provide. The most common side effect of calcium supplementation is constipation, which might be perceived as a benefit for those with diarrhea-predominant irritable bowel

syndrome, but a serious problem for those with constipation-predominant IBS. Kidney stones can be a complication from way too much calcium supplementation, since excess calcium is often excreted into the urine, where it can form crystals (stones).

Does the altered skin physiology in classic or other types of EDS inhibit or enhance the absorption of vitamin D from sunlight?

I have never heard of nor read any reports of EDS causing reduced sunlight-induced vitamin D production in the skin.

Note that one could err on the side of either too much or too little sun exposure. Too little sun lowers vitamin D levels. Too much sun increases the risk of melanoma and other skin cancers. Skin cancer can kill; vitamin D deficiency mostly just makes you feel lousy. Skin cancer requires surgery for treatment (not a fun option for anyone, but even greater risk in most types of EDS); vitamin D deficiency just requires taking a supplemental vitamin. Personally, I’d rather get less sun exposure, avoid cancer or surgery, and take a supplement for my vitamin D!

Are the GI effects of EDS likely to enhance or inhibit absorption of supplements?

Overall and for most people, there is probably no effect (although this has not been studied). With respect to vitamin D, I don’t recommend increasing sun exposure (see above), so the only alternative is oral supplementation. If usual supplementation isn’t adequate, I just gradually increase the dose or frequency until we reach our goal.

Could EDSers be considered a special subpopulation with regard to this issue? The report mentions dark-skinned people living in northern climates and people confined indoors as groups who might

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need to supplement with vitamin D. What about populations with increased risk of osteoporosis?  

Dark skin, northern climates (or, more accurately, climates closer to either the North or South pole), and indoor confinement all share the same risk factor: less vitamin D is produced in the skin because the dark pigment absorbs the UV; there’s less direct sun exposure with greater distance from the equator; and there’s less sun exposure if you spend more time indoors (or wearing clothing/sunscreen to block UV radiation). Low vitamin D adds to the osteoporosis risk for those with other risk factors, but that is no different with EDS than it

is for post-menopausal women or people with a strong family history of osteoporosis, just to cite a few other risk factors.

The main way that I would see EDS patients as a special subpopulation would be if they spend less time outdoors as a result of their EDS — essentially putting them in the same category as others confined indoors.

Is there any reason to think that EDSers would be more vulnerable to over-supplementation?

Absolutely NOT.

I.O.M. PRESS RELEASEIOM Report Sets New Dietary Intake Levels for Calcium and Vitamin D To Maintain Health and Avoid Risks Associated With Excess

MOST Americans and Canadians up to age 70 need no more than 600 international

units (IUs) of vitamin D per day to maintain health, and those 71 and older may need as much as 800 IUs, says a new report from the Institute of Medicine. The amount of calcium needed ranges, based on age, from 700 to 1,300 milligrams per day, according to the report, which updates the nutritional reference values known as Dietary Reference Intakes (DRIs) for these interrelated nutrients. 

The report’s recommendations take into account nearly 1,000 published studies as well as testimony from scientists and stakeholders. A large amount of evidence, which formed the basis of the new intake values, confirms the roles of calcium and vitamin D in promoting skeletal growth and maintenance and the amounts needed to avoid poor bone health.

The committee that wrote the report also reviewed hundreds of studies and reports on other possible health effects of vitamin D, such as protection against cancer, heart disease, autoimmune diseases, and diabetes. While these studies point to possibilities that warrant further investigation, they have yielded conflicting and mixed results and do not offer the evidence needed to confirm that vitamin D has these effects. Rigorous trials that yield consistent results are vital for reaching conclusions, as past experiences have shown. Vitamin E, for example, was believed to protect against heart disease before further studies disproved it.

“There is abundant science to confidently state how much vitamin D and calcium people need,” said committee chair Catharine Ross, professor and Dorothy Foehr Huck Chair, department of nutritional sciences, Pennsylvania State University, University Park. “We scrutinized the evidence, looking for indications of beneficial effects at all levels of intake. Amounts higher than those specified in this report are not necessary to maintain bone health.”

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The science on calcium’s role in bone health shows that 700 milligrams per day meets the needs of almost all children ages 1 through 3, and 1,000 milligrams daily is appropriate for almost all children ages 4 through 8. Adolescents ages 9 through 18 require no more than 1,300 milligrams per day. For practically all adults ages 19 through 50 and for men until age 71, 1,000 milligrams covers daily calcium needs. Women starting at age 51 and both men and women age 71 and older need no more than 1,200 milligrams per day.

As for vitamin D, 600 IUs daily meets the needs of almost everyone in the United States and Canada, although people 71 and older may require as much as 800 IUs per day because of potential physical and behavioral changes related to aging.

The majority of Americans and Canadians are getting enough vitamin D and calcium, the committee determined from reviewing national surveys of blood levels. Some adolescent girls may not get quite enough calcium, and there is a greater chance that elderly individuals may fall short of the necessary amounts of calcium and vitamin D. These individuals should increase their intake of foods containing these nutrients and possibly take a supplement.

Confusion about the amount of vitamin D necessary to ward off deficiency has arisen in recent years as tests that measure levels in patients’ blood have become widely used. The measurements of sufficiency and deficiency — the cutpoints — that clinical laboratories use to report test results have not been based on rigorous scientific studies and are not standardized. This lack of agreement means the same individual could be declared

deficient or sufficient depending on which laboratory reads the test. There may be an overestimation of the number of people with vitamin D deficiency because many labs appear to be using cutpoints that are higher than the evidence indicates are appropriate. Based on available data, almost all individuals get sufficient vitamin D when their blood levels are at or above 20 nanograms per milliliter as it is measured in America, or 50 nanomoles per liter as measured in Canada.

Although sunlight triggers the natural produc-tion of vitamin D in skin and contributes to people’s vitamin D levels, individuals’ sun ex-posure varies greatly and many people are told

to minimize their ex-posure, so the commit-tee assumed minimal sun exposure to estab-lish the DRIs. The new intake levels for vita-min D cover the needs of individuals who get little sun.

Greater amounts of food fortification and rising rates of supplement use have increased the chances that people consume high amounts of these nutrients. Getting too much calcium from dietary supplements has been associated with kidney stones, while excessive vitamin D can damage the kidneys and heart. Evidence about other possible risks associated with routine vitamin D supplementation is still tentative, and most studies have focused on very high doses taken short term rather than on routine, long-term consumption of large amounts. However, some signals suggest there are greater risks of death and chronic disease associated with long-term high vitamin D intake, which informed the committee’s conclusions about levels that consumers should not exceed.

Upper intake levels represent the upper safe boundary and should not be misunderstood

“Confusion about the

amount of vitamin D

necessary to ward off

deficiency has arisen.”

WINTER 2010PAGE 28

as amounts people need or should strive to consume. The upper intake levels for vitamin D are 2,500 IUs per day for children ages 1 through 3; 3,000 IUs daily for children 4 through 8 years old; and 4,000 IUs daily for all others. The upper intake levels for calcium are 2,500 milligrams per day from age 1 through 8; 3,000 milligrams daily from age 9 through 18; 2,500 milligrams daily from age 19 through 50; and 2,000 milligrams per day for all other age groups.

“While it is too early to make definitive statements about the risks associated with routine high doses of vitamin D and calcium, people don’t need more than the amounts established in this report,” Ross said. “Past cases such as hormone replacement therapy and high doses of beta carotene remind us that some therapies that seemed to show promise for treating or preventing health problems ultimately did not work out and even caused harm. This is why it is appropriate to approach emerging evidence about an intervention cautiously, but with an open mind.”

The new DRIs are based on much more information and higher-quality study results

than were available when the DRIs for these nutrients were first set in 1997. At that time, limitations in the evidence resulted in intake levels called Adequate Intakes, which are rougher estimations of people’s requirements than the new values. The old and new DRIs reflect different calculations and are not directly comparable.

The study was sponsored by the U.S. Department of Health and Human Services, U.S. Department of Agriculture, U.S. Department of Defense, and Health Canada. Established in 1970 under the charter of the National Academy of Sciences, the Institute of Medicine provides independent, objective, evidence-

based advice to policymakers, health professionals, the private sector, and the public.  The National Academy of Sciences, National Academy of Engineering, Institute of Medicine, and National Research

Council make up the National Academies. For more information about the Institute of Medicine, visit http://national-academies.org.

More information about the study is available at www.iom.edu/calcium.

“Upper intake levels

represent the upper safe

boundary and should

not be misunderstood.”

Acceptance of one’s life has nothing to do with resignation; it does not mean running away from the struggle. On the contrary, it means accepting it as it comes, with all the handicaps of heredity, of suffering, of psychological complexes and injustices.

Paul Tournier

WINTER 2010PAGE 29

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