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HEDS and FM

By Rosie Shiver

Carnival Side Show

Perhaps you've seen the sepia tinted old photos of the carnival side show people who put their calves in back of their necks and twisted their arms into unnatural positions.  These double-jointed loose jointed, or in today's term, Hypermobile, people were considered a curiosity. We still are.

Joint Hypermobility (JH) refers to laxity of ligaments and tendons. A subset of those with JH are actually those with Hypermobile Ehlers-Danlos Syndrome (HEDS). JH runs in families, and HEDS is definitely genetically connected.  HEDS is the form of EDS that is characterized by a defective protein that produces collagen . People with HEDS, besides the loose jointedness, also bruise easily, have extra stretchy skin, and are prone to mechanically develop Osteoarthritis at very young ages.  Dislocations and lesser subluxations of joints are common. One nice side effect is that people with HEDS don?t wrinkle...we keep velvety smooth skin into our eighth decade or beyond, without plastic surgery.

There are five other types of Ehlers-Danlos Syndrome, a few of which need special medical treatment to avoid becoming fatal. HEDS is generally considered benign, but in terms of arthritis, it isn't. JH is considered rather common in Rheumatology clinics, often without the possibility of HEDS being considered. The definitive test for Ehlers-Danlos is a skin biopsy, not the nicest of procedures. So, the loose jointedness is duly noted and more often than not, dismissed.

An Ounce of Prevention

Young people with JH or HEDS are often encouraged during physical education or by pediatricians to use their extra mobility to its fullest.  Dance, Yoga, gymnastics, ballet and even football and hockey are said to be good choices. Those of us older folks with HEDS wince at the thought.

Each time a joint shifts out of place, the ligaments snap around and the tendons are overstretched. Given that in HEDS, the replacement collagen that is grown is defective, repeated wrenchings and even dislocations of joints are prime causes of the ends of bones wearing out.  After only a few years, the defective ligaments can't hold the bones in place, and even young children begin to develop Osteoarthritis.

This can be especially worrisome in the spine, as it puts massive strain on the spinal discs. Also, because the ligaments are too thin and snappish, scoliosis is very, very common, beginning as early as elementary school.

A person with HEDS or JH should not extend any of their joints beyond 80% of the possible range of motion. Given that range of motion is an indicator of  progression of arthritis, the Rheumatologist must be made aware, in fact reminded often, that range of motion hardly applies to JH and HEDS.

Although it is tempting to show off what one can do that others can't, it must be discouraged. Every time someone with HEDS does the pretzel, they are damaging ligaments and tendons that cannot be replaced with fully functioning ones.

Glucosamine is an effective treatment to help those with Osteoarthritis maintain healthy ligaments and tendons, thereby keeping their joints stabilized. Chondrotin also has strengthening and building properties.  But as the logic of  The Three Little Pigs dictates, the building materials do matter. In HEDS, the building material ( collagen ) is defective.  This means that GS/C just gives more of the already not so good stuff, and is logically contraindicated.

The Problem of Pain

Some kinds of EDS are painful all of the time. HEDS is not. Because of the lack of pain prior to dislocation of joints, the damage is done, then the pain is felt. This can occur so often that  osteoarthritis progresses very rapidly.

In the previous decade, it was thought that EDS was inherited by only 1 of every 5,000 people. Due to greater testing and a higher level of knowledge by doctors, that number is being revised downward. A major reason for that is the greater understanding concerning  Fibromyalgia.

Fibromyalgia is a widespread chronic pain condition characterized by stiffness and pain throughout all four quadrants of the body. The typical diagnosis of FM is by tender points. If 11 of 18 specific tender points of the body cause a pain reaction, the person has FM.

The problem for those with HEDS is most tender points are areas of ligaments and tendons. Being these areas are defectively produced, the pain reaction can be reduced.

On the other hand, years of  Osteoarthritis  ( and some researchers say soft tissue rheumatism in EDS as well) produce a tendency to have chronic pain.  In the case of HEDS, FM is the cart, and HEDS is the horse. It is now thought that up to 16% of those with FM have FM due to JH or HEDS.  As with FM itself, HEDS is more often diagnosed in women.  Those of us with FM and HEDS feel strongly that both are underdiagnosed in men, as men present symptoms a bit differently. Given greater understanding of HEDS and FM, later estimates are about 1 in 2,500 for both HEDS and FM.  

It?s A Family Affair

A family medical history is important when it comes to HEDS and FM. Unfortunately for many, those who inherit from the father may not know about having HEDS because men are socialized to be macho and not discuss pain.

What can be done if you think you or your children have inherited HEDS?  A pediatric rheumatologist is best for children, but that?s often not possible in most areas of the country away from big cities. Primary Care Physicians can make an informal  diagnosis of JH or HEDS, with a referral to Physical Therapy. An Occupational Therapist is even better, in order to teach proper movement and provide orthotics to prevent injury. Some wonderful OTs even make splints, braces and other orthotic devices just for their HEDS/FM patients.

Ring splints for hands are familiar to those with Rheumatoid Arthritis, and can prevent similar deformity in the hands of those with HEDS. They are beautiful, as well. One HEDS/FMer had her engagement diamond put onto a ring splint for a wedding band. Unique and useful, both.

The preeminent organization for education and support of those with EDS is the Canadian  Ehlers-Danlos Association. Many helpful, insightful sufferers can answer questions and give guidance on prevention of injury.

The best site for combined HEDS/FM is kept by C.T. She's a young lady now at Harvard on scholarship who maintains her site for fellow sufferers, as well as heads an e-mail list for us. The e-mail list features Barbara, who edits EDS Today and Joyce, with a wealth of info on bracing and splints, as well as many others who actively discuss medications and kids with EDS and FM. It's one of the best places to be.

The addresses for these are at the bottom of this article.

Awareness is Crucial

The options for living with JH, EDS, HEDS and FM are greater now than they have ever been. Many cases of Osteoarthritis can be avoided and there are new and better ways for adapting to having these conditions. But awareness is key. It is the hope of fostering and promoting greater awareness that is the motivating factor for this article being written.

Many thanks, with much gratitude, to Krissy Jo for publishing this on Arthritis Insight. Yer the tops, K.J.!! 

Hypermobility and Fibromyalgia Webpage  ( C.T.'s site):
http://anaiis.tripod.com/hmedfm/index.html

HM-ED-FM E-Mail List:
EDS-FMS-subscribe@topica.com

CEDA:
http://www.ceda.ca

EDS Today:
http://www.uggen.net/edstoday/

Fibromyalgia Association USA:
http://www.fibromyalgiaassnusa.org/index.html

UK EDS Support Group:
http://www.atv.ndirect.co.uk/

UK  Fibromyalgia Support Group:
http://www.lewolf.com/fms.htm