Friday, June 05, 2009

Ehlers-Danlos Syndrome: A Reference Librarian Looks at Consumer Health Reference Sources

This is Ehlers-Danlos Syndrome Awareness Month. EDS is a group of connective tissue disorders which may, depending on the type and severity, make a person super flexible, reduce mobility, cause constant pain, or even threaten life. Basic to all is that a person's collagen is defective, resulting in stretched skin and dislocated joints. The Ehlers-Danlos National Foundation website is a good starting point for learning about the condition.

A good website from which to learn about the life of a person with EDS is LibrarianInBlack. Last week Sarah Houghton-Jan wrote about her life with EDS. As a dedicated librarian intent on educating readers, she embedded two videos about other EDS cases and photos showing hyperelastic skin and joints. She also included a link to the Ehlers-Danlos Support Group. You can also look through the recent posts and archives of LibrarianInBlack to see what an active person Sarah is despite her condition.

The following is a collection of EDS resources available in public libraries (many at the Downers Grove Public Library). Readers will notice that there is disagreement in the EDS literature as to how many types of the syndrome exist. No one source tells the whole story. Persons with EDS will, of course, seek many sources to help them understand their prognosis. Friends and family are urged to take time to also explore many resources.

Missing from the list are book-length EDS memoirs. I found none through our local library system catalog, Worldcat, or Amazon.

Reference Books

Dorland's Illustrated Medical Dictionary, 31st ed., 2007 - A user has to look at two entries to see all that the dictionary says about about EDS. On page 603 is an entry "Ehlers-Danlos Syndrome" which provides a pronunciation and the names of the two dermatologists for whom the syndrome is named, Edvard Ehler of Denmark, 1863-1937 and Henri Alexandre Danlos of France, 1844-1912. For more information, readers have to turn to page 1854 and look under "syndromes." Dorland's describes six types of EDS (down from ten) and includes a photo of hyperextensible skin.

American Medical Association Family Medical Guide, 4th ed., 2004. - This AMA guide has about a page of information about EDS under the heading of "Autosomal Dominant Disorders." Across pages 969-70 are paragraphs about symptoms, diagnosis, and treatment. Near the end is a stern warning to parents to reduce injuries by preventing their children from "showing off" their unusual flexibility.

Dictionary of Syndromes and Inherited Disorders by Patricia Gilbert, 3rd ed., 2000 - Gilbert says that an alternate name for the syndrome is "joint laxity." On pages 102-5, she describes population incidence of EDS, genetic causation, syndrome characteristics, and case management. She emphasizes the need for good dental care.

Current Medical Diagnosis and Treatment by Stephen J. McPhee and Maxine A. Papadakis, 2009 ed. - CMDT does not have a separate entry for EDS, but it is mentioned in entries for mitral valve prolapse (p. 301), cardiac involvement in miscellaneous systemic disease (p. 373), cardiac patient and pregnancy (p. 374), and thoracic aortic aneurysms (p. 414).

Harrison's Principles of Internal Medicine, 17th ed., 2008 - Harrison's includes about two large pages of professional level content about EDS, pages 2465-67. A chart explains eight types of EDS. The syndrome is also mentioned in entries about skin manifestations of internal disease (p. 335), bleeding (p. 366), valvular heart disease (p. 1472), cardiac manifestations of systemic diseases (p. 1499), aortic aneurysms (p. 1563), stroke (p. 2519), and osteoporosis (p. 2400).

Gale Encyclopedia of Medicine, 3rd ed., 2006 - Like other resources, the encyclopedia describes many types of EDS and explains family genetic patterns. Friends and family may appreciate the prognosis section, which describes the challenges of living with EDS. A total of five pages is included in the encyclopedia. Entries in two other Gale titles, Gale Encyclopedia of Children's Health and Gale Encyclopedia of Genetic Disorders, 2nd ed., resemble this entry. The latter, however, also mentions EDS in entries for other syndromes.

Genetic Disorders Sourcebook, 3rd ed., 2004 - Only two brief pages about EDS. A bit disappointing.

Encyclopedia of Genetic Disorders and Birth Defects by James Wynbrandt and Mark D. Ludman, 3rd ed., 2008 - The authors note that EDS experts have proposed that the official number of types of EDS be reduced to six, but they then describe ten types anyway. Two full pages of data.

Encyclopedia of Skin and Skin Disorders by Carol Turkington and Jeffrey S. Dover 3rd ed., 2007 - The authors focus on skin in their entry about EDS but also describe the overall condition of the EDS patient on pages 125-26.

Rudolph's Pediatrics, 21st ed., 2003 - This pediatrics medical guide describes the clinical features of six types of EDS and emphasizes injury prevention (shin guards, knee pads, braces). Surgical correction is also discussed.

Newspaper and Magazine Articles

"Kids' Illnesses Spark Battle with State: A California Mom Spent Years Trying to Figure Out What Made Her Children Sick, Then Almost Lost Them," Chicago Tribune, March 20, 2008, p. 4 - A story about how a pattern of bruises and injuries in her children nearly led to abuse charges against a mother whose children have EDS.

"A Life with Purpose," Swimming World, May 2005, pp. 29-30 - A profile of Nancy Burpee, a champion swimmer who has had EDS for 40 years.

"Coping with Livelong Pain," Toronto Star, April 30, 2009, p. U10 - A story about how a pain clinic is helping a mother and her two daughter, all of whom have Ehlers-Danlos syndrome.

"A Mother's Burden," Santa Fe New Mexican, August 8, 2007, p. D1 - A story about a mother and three daughters with EDS. Tells how use of braces reduced pain and injuries in the children.

"After a Multitude of Tests, an Answer from Grandmother's Memory," New York Times, August 24, 2004, p. E5 - A story about the difficulty of diagnosing EDS in infants and toddlers.


Ehlers-Danlos National Foundation - Sarah says that this is the website with the most useful information.

Ehlers-Danlos Support Group - This British site has a message board and a section with advice for living with EDS.

Mayo Clinic - The Mayo Clinic website includes a basic definition, symptoms, causes, complications, treatment, and advice for coping with the syndrome.

Medline Plus - This site from the National Library of Medicine and National Institute of Health tells us that EDS is also called "cutis elastica." The EDS page includes links to articles from nonprofit organizations and medical journals.

National Organization for Rare Disorders - Basic information on EDS, identifying eight types. The site has a list of recommended websites.

NLM Gateway - Search this website to find basic information, medical journal articles, and clinical trials that may be recruiting participants.

Final Note

My abbreviated notes about these resources, of course, oversimplify what they contain, but together they suggest the vast amount of information on the topic. Ironically, there is still much that is not known or understood, and being a rare condition, as Sarah explains, EDS is not a priority in medical research. I hope that you spend a bit of time with these resources to comprehend the uncertainty of the EDS experience and the hand dealt to people with the syndrome.


elise said...

LeftPaw Press published Issues and Management of Joint Hypermobility- A Guide for the Ehlers-Danlos Syndrome Hypermobility Type and the Hypermobility Syndrome by Dr. Brad T. Tinkle (Paperback and hard cover) at the end of 2008.
It's an excellent resource that both patients and the medical team can understand. The book is relatively inexpensive so I hope folks consider donating a copy to their favorite local library!

(can be found on Amazon as well)

Yes, Sarah rocks! Thanks for the post!
~From an EDSer.

Sarah Houghton-Jan said...

Thank you for this Rick. You have just given more help than you know to a huge group of people with EDS. Gooooooo reference!

Saravanan said...

Here is some additional information about the "genetics" of this condition that was written by our Genetic Counselor and other genetic professionals: I hope it helps. Thanks, AccessDNAbu

Terri said...

Hi .....I have Ehlers Danlos hypermobility.....I have a body full of Titanium and it works extrememly well and as pain free as I could possibly hope.....I have steel pins in every toe and the last two on each foot are sewen together. I have had 7 knee surgeries which includes replacement when I finally hit 55. I have had both hips replaced and anal prolapse surgery, and hysterectomy. I had 2 tough pregnancies and one was 72 hrs which of course did a lot of damage. The other was a C section after being induced and 9 hours of hard labor only to be at 4 Cm. That boy, as a man has already had 2 shoulder surgeries. His wife in involved in a breakthrough method of pain management and she is an RN, called MELT. Take a look at it and the book is on line. I't s amazing. His wife also has EDS. She has been in excruciating pain
all of her life. A dancer, baton twirler, actress, RN, Yoga instructor, Pilates instructor.....nothing worked until MELT.
She is now getting certified to train others. Take a look at it..
I have not had the shoulder problems however one of my shoulders is very noticeably lower than the other, in fact one side of my body is.
Salon Pas patches have been a Godsend. you can get them anywhere and they are cheap. Only get the white ones.
Also Fit Flop shoes, on Amazon....have a wobble board in them. fixes our imbalance! I wear NOTHING but Fit Flops!