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Ehlers-Danlos National Foundation
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By the way, May is also Mental Health Month.
“It’s all in your head.” If you’re living with chronic pain, this is probably the most frustrating thing someone could say to you
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#EDSAwareness Of the six types categorized for diagnosis in 1997, Hypermobility Type EDS (HEDS) is the most prevalent. Unfortunately, the genetic cause has not yet been determined for HEDS. "EDS, Hypermobility type, may be the most common heritable disorder of connective tissue." (Dr. H.P. Levy, cited below)

Diagnostic Information: Joint hypermobility is the dominant clinical manifestation. Generalized joint hypermobility that affects large (elbows, knees) and small (fingers and toes) joints is evident in HEDS. Recurring joint subluxations and dislocations are common occurrences. Certain joints, such as the shoulder, patella, and temporomandibular joint dislocate frequently. The skin involvement (smooth velvety skin with or without hyperextensibility) as well as bruising tendencies in HEDS are present but quite variable in severity.  Chronic pain is a well-established and cardinal manifestation of HEDS and it is common for pain to be out of proportion to physical and radiological findings. Musculoskeletal pain is early onset, chronic and may be debilitating. The origin of the pain is not clearly understood, but some of the likely causes include muscle spasm (tender points are sometimes present) and degenerative arthritis; neuropathic pain is also common. http://www.ednf.org/hypermobility-type

Remember, though, that diagnostic criteria are used only to distinguish HEDS from other connective tissue disorders; it isn't required that all criteria be present for diagnosis, and HEDS can have many more effects on the body. For a list of major and minor criteria, see www.ednf.org/nosology.

Effects may include — more detailed information on how these areas are affected will be covered later this month:
("Ehlers-Danlos Syndrome, Hypermobility Type" by Howard P. Levy, MD, PhD on http://www.ncbi.nlm.nih.gov/books/NBK1279/

• skin manifestations

• joint instability (subluxations and dislocations everywhere, including the spine; sprains; tendinitis and bursitis)
• osteoarthritis
• osteoporosis
• vitamin D deficiency

• chronic pain (including myofascial, neuropathic, and osteoarthritic)
• fatigue and sleep disturbance
• headaches, especially migraines

• easy bruising
• mildly prolonged bleeding from the gums
• bleeding time may be prolonged (no consistent coagulation factor abnormalities have been reported; may be due to capillary and soft tissue fragility)

• functional bowel disorders in one- to two-thirds of individuals with HEDS and Classic EDS
• GERD and gastritis despite treatment
• early satiety and delayed gastric emptying\• irritable bowel syndrome

• autonomic dysfunction
• possible aortic root dilation (usually stable, and of a mild degree in 11-33% of HEDS and CEDS)

• high, narrow palate
• dental crowding
• periodontal disease

• pregnancy may be complicated

• delayed onset and/or resistance to local anesthesia
• poor balance
• possible association with Chiari malformation

HEDS has an autosomal dominant (http://www.nlm.nih.gov/medlineplus/ency/article/002049.htm) inheritance pattern. The proportion of de novo cases is unknown. Each child of a person with HEDS has a 50% chance of inheriting it.
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EDNF's Resource Guide PDFs http://ednf.org/resource-guides include: the Ehlers-Danlos, Dentistry, Ophthalmology, Pain Management, and Vascular Medical Resource Guides; the Clinical Reference Manual: Vascular Type; and the ER and Surgery Information Booklet. #EDSAwareness
The guides below were created by members of our Professional Advisory Network and are for your use free of charge. You can also view these on the right hand side of the page.  If you would like to help off-set the costs of creating these guides please consider making a donation to EDNF. Medical Resource Guide Dentistry Resource Guide
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Welcome to ‪#EDSAwareness‬ Month!

Ehlers-Danlos syndromes are a group of connective tissue gene disorders that produce a spectrum of complex problems across multiple systems of the body. Each type of EDS is defined as a distinct problem in making or using one of the types of collagen. Collagen is found throughout the body. Ehlers-Danlos syndromes come down to a structural problem.

An analogy: If you build a house with bad materials, say half the necessary wood or soft aluminum nails, you know you'll have problems. Some problems are more likely to show up than others, but because those materials were used everywhere and aren't necessarily visible, you can be surprised by a problem.

It's the same thing with EDS and collagen. The collagen a person with EDS is built with is not structured the way it should be. Collagen is what the body uses to provide strength and elasticity to tissue; normal collagen is a strong protein that allows tissue to be stretched but not beyond its limit, and then safely returns it to normal. WIth a badly built or processed collagen, some of the tissue in the EDS-affected body can be pulled beyond normal limits which causes damage. Collagen is the most abundant protein in the body and can be found almost anywhere, in skin, muscles, tendons and ligaments, blood vessels, organs, gums, eyes, and so on.

So the problems resulting from being built out of a protein that doesn't behave the way it should can be widespread, in a wide range of severities, and maybe even show up in places one wouldn't think are related until it is remembered that collagen is used there, too.

http://www.ednf.org/what-eds
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Thank you to everyone who uses Amazon Smile for EDNF! Mother's Day is Sunday, May 10. While you're shopping, please consider using Amazon Smile http://amzn.to/1QKrkfU to have Amazon donate to Ehlers-Danlos National Foundation.
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Have some beauty in your life; distract yourself, and visit the Rijkmuseum. Free online. https://www.rijksmuseum.nl/en/explore-the-collection
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"Comments on the draft National Pain Strategy must be received no later than 5 p.m. on May 20th, 2015. This document includes objectives and plans related to key areas of pain and pain care, including professional education and training, public education and communication, service delivery and reimbursement, prevention and care, disparities, and population research."

http://iprcc.nih.gov/National_Pain_Strategy/Public_Comment_NPS_Draft.htm

The draft in PDF is available: http://iprcc.nih.gov/docs/DraftHHSNationalPainStrategy.pdf
DEPARTMENT OF HEALTH AND HUMAN SERVICES. National Institutes of Health. Solicitation of Written Comments on Draft National Pain Strategy. ACTION: Notice https://www.federalregister.gov/a/2015-07626. SUMMARY: The National Institute of Neurological Disorders and Stroke (NINDS) Office of Pain ...
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‪#‎EDSAwareness‬ Classical Type EDS (CEDS) is the second most prevalent of the six types categorized for diagnosis in 1997.

Diagnostic Information: Marked skin hyperextensibility (stretchy) with widened atrophic scars and joint hypermobility are found in the CEDS. The skin manifestations range in severity from mild to severe expression. The skin is smooth and velvety with the evidence of tissue fragility and easy bruisability. Examples of tissue extensibility and fragility include hiatal hernia, anal prolapse in childhood and cervical insufficiency. Hernias may be a post-operative complication. Scars are found mostly over pressure points such as the knees, elbows, forehead and chin. Molluscoid pseudo tumors (calcified hematomas) associated with scars are frequently found over pressure points such as the elbows, and spheroids (fat containing cysts) are usually found the on the forearms and shins. Complications of joint hypermobility include sprains, dislocations/subluxations and pes planus (flat foot) to name a few. Recurrent joint subluxations are common in the shoulder, patella and temporomandibular joints. Muscle hypotonia and delayed gross motor development may also be evident. http://www.ednf.org/classical-type

CEDS most often originates from COL5A1 and COL5A2 genes. (http://www.ednf.org/eds-diagnosis).

Effects on the body may include: 
(adapted from "Ehlers-Danlos Syndrome, Classic Type" by Fransiska Malfait, MD, PhD, Richard Wenstrup, MD, and Anne De Paepe, MD, PhD, available on http://www.ncbi.nlm.nih.gov/books/NBK1244/)

• skin: fragile (splitting after minor trauma, particularly shins, forehead, chin); delayed wound healing
• scars stretch after primary healing, then widening (papyraceous)
• molluscoid pseudotumors, subcutaneous spheroids, piezogenic papules, acrocyanosis, chillblains (see bottom for definitions)

• tissue fragility
• cervical insufficiency in pregnancy
• hernia

• joint hypermobility complications
• chronic pain

• muscular hypotonia causing delayed motor development, problems with walking, and mild motor disturbance
• fatigue and muscle cramps

• easy bruising
• prolonged bleeding despite normal coagulation

• structural cardiac problems are not usual
• aortic root dilatation may be more common that expected but usually of little clinical consequence and mitral valve prolapse may occur but is rarely severe; intervention is not usually needed
• in severe form of CEDS, possibility of spontaneous rupture of large arteries with intracranial aneurysms and arteriovenous fistulae

• pregnancy carries risks for mother and newborn
• premature rupture of membranes and premature birth when either mother or baby is affected
• breech presentation is more frequent

CEDS has an autosomal dominant (http://www.nlm.nih.gov/medlineplus/ency/article/002049.htm) inheritance pattern. The proportion of de novo cases is approximately 50%, but family members might appear to be unaffected and their CEDS unrecognized. Each child of a person with CEDS has a 50% chance of inheriting it.

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Molluscoid pseudotumors: fleshy, heaped-up lesions associated with scars over pressure points such as the elbows and knees.

Subcutaneous spheroids: small, cyst-like, hard shot-like nodules, freely moveable in the subcutis over the bony prominences of the legs and arms. They occur in approximately one third of affected individuals, are numerous, and feel like hard grains of rice. X-ray reveals an outer calcified layer with a translucent core. The spheroids represent subcutaneous fat globules that have lost their blood supply, becoming fibrosed and calcified.

Piezogenic papules: small, painful, reversible herniations of underlying adipose tissue globules through the fascia into the dermis, such as on medial and lateral aspects of the feet upon standing.

Acrocyanosis: a painless disorder caused by constriction or narrowing of the small blood vessels in the skin (affecting mainly the hands) in which the affected areas turn blue and become cold and sweaty; localized swelling may also occur.

Chilblains: cold injuries, characterized by a red swollen skin that is tender, hot to the touch, and may itch; can develop in less than two hours in skin exposed to cold.
Marked skin hyperextensibility (stretchy) with widened atrophic scars and joint hypermobility are found in the Classical Type of EDS. The skin manifestations range in severity from mild to severe. The skin is smooth and velvety along with evidence of fragility and a tendency to bruise easily. Examples of tissue extensibility and fragility include hiatal hernia, anal prolapse in childhood and cervical insufficiency. Hernias may be a post-operative...
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We are not alone. EDS is genetic so odds are you're not alone in your family, and I doubt you're alone in your community. There are almost 24,000 of us here on this page. There are millions of us worldwide at the traditional incidence of 1 in 2,500 to 1 in 5,000; and if the incidence is 1 or 2 in 100 as many think now, then we are millions just in the US.

Symptoms never come alone. Treatments never come alone. We are not alone.
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When EDS were recategorized in 1997, the numbering system was simplified and replaced by a named system, in which six main types of EDS were defined for diagnosis—these major and minor diagnostic criteria are what is called a nosology, the classification of EDS. Some of the previous types were removed from the nosology (www.ednf.org/nosology), and since 1997 newer forms of EDS have been discovered that remain outside the classification. In coming ‪#‎EDSAwareness‬ days, we'll focus a bit on each categorized type of EDS and explain their criteria and some of the added symptoms. We'll also go into more depth on those symptoms, as well as some of the things that help.
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May is EDS Awareness Month!
Help us connect our stories with the world by participating in the ‪#‎EhlersDanlosStories‬ Video Contest.

How to Participate:
1. Create a video that shares part of your EDS story with the world.
2. Upload your video to YouTube with the hashtag #EhlersDanlosStories in the description.
3. To officially enter the contest, paste the YouTube link of your video on EDNF's Facebook page.

The Rules:
1. The contest runs from May 1 through May 31 at midnight.
2. The video with the most views on YouTube wins a complimentary registration to the 2015 EDNF Learning Conference in Baltimore, August 13-15.
3. EDNF Staff will select a "Staff Favorite" video which will also win a complimentary registration to the 2015 EDNF Learning Conference.
4. Videos must be one minute or shorter, and appropriate for family viewing.
5. You may enter the contest at any time during the month of May.
6. Have fun and spread awareness!

Together, we can make the world aware of Ehlers-Danlos syndrome—and the challenges so many endure.
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"There are a few experimental drugs that target the wasabi receptor, and the image lets researches see the cleft in the protein where the drugs bind. That will aid in the development of new drugs. The picture may also help researchers get to the bottom of basics mechanisms of pain." 
The structure will help researchers understand pain— and create drugs that could suppress it.
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This NPR article includes a link to the full paper in "Nature" for those who are interested in reading more about the TRPA1 receptors. http://www.npr.org/blogs/health/2015/04/08/398065961/sushi-science-a-3-d-view-of-the-bodys-wasabi-receptor
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Have them in circles
268 people
Heather Ashley's profile photo
Didier Bruno's profile photo
Cheri Parr's profile photo
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Resources for those affected by Ehlers-Danlos syndrome
Introduction
Ehlers-Danlos National Foundation creates resources for those affected by the connective tissue disorder Ehlers-Danlos syndrome. Any medical information found here should be discussed with your doctors thoroughly before taking action. Established in 1985 by Founder Nancy Hanna Rogowski (1957 - 1995), we are a 501 (c) (3) Nonprofit Organization with thousands of members across America and around the world. Our volunteers and their families are the life blood of EDNF and make a huge difference to those affected by EDS.
Contact Information
Contact info
Phone
703-506-2892
Fax
703-506-3266
Address
7918 Jones Branch Drive Suite 300 McLean VA 2210