Ehlers-Danlos Syndrome -> Tammy's Story

Orthopediatic Outlook - Stormy Skies Ahead

2011-09-03T05:49:00.000-07:00

I am still processing the results of my latest orthopedic appointment emotionally but mentally know what has to happen. The little kid inside me, however, swings back and forth between, "this needs to happen," and, "NO!" I'm about to send my emotional self to her room to finish out the tantrum.

The latest appointment was to go over the findings of a series of x-rays. There is a little osteoarthritis setting into my shoulder; not surprised. My short leg is 14mm shorter than my long leg; hasn't changed. My spine has become unstable. WHAT?!? Yes, unstable. After over 2 decades of stability it is collapsing. Gravity is no longer my friend. I am an adult staring into the abyss of corrective spinal surgery. I hated hearing the words Harrington Rods spoken aloud after so many years of believing I escaped their cold surgical steel clutches.

But. mentally, I knew this was coming. I could feel it in the increasing pain and unidentified anxiety. The strange "you are too young for this" diagnosis of bursitis just 3 weeks ago that prompted me to demand a new spinal evaluation. The knowledge that gravity works and it was only a matter of time before the curves increased. The nurse who laughingly refused to remeasure my height last year when it was 1/2 inch shorter than I knew it should be. I knew. But I refused to accept.

Now, I have no choice. My curvatures are each over 60 degrees. This is no longer a case of avoiding surgery but needing to embrace it before my spine kills me. Some of my internal organs are in danger of being damaged. There is a potential I will break a rib on my hip bone - good thing I have strong bones! Straightening my spine will also eliminate pain, correct problems with my hips and shoulder, and on the petty side I'll be MUCH taller (at least 3 inches) and no longer be 5 pounds overweight for my height. Hey, I have to find a ray of sunshine in this somewhere :)

Pain Management Program

2011-08-27T12:59:00.000-07:00

This spring, at my specialist's recommendation, I enrolled in a pain management program through my local hospital's wellness center. My requirements were that it would be non-invasive and limited pharmaceuticals - I admit I was very surprised to find that at a traditional hospital!

The program was supposed to start with an informational meeting, but due to time constraints and because I want to keep working and not threaten my employment status with frequent absenteeism I started with an orthopedic evaluation and physical therapy. The evaluation found bursitis in one hip which was causing a new onset of pain and weakness and, when my shoulder was moved the way he wanted, limited mobility in my previously "worked on" shoulder. Of course, if allowed to move the shoulder "my" way it has hypermobility. PT has focused on these two and not, as I wanted, all-over body strengthening to help manage the constant discomfort.

I still haven't had the informational meeting with the head of the program, but found out last week that was due to a hiking disaster she experienced and the program is slowly changing hands. Thus, I am fighting for my needs with the feeling of little support other than my family.

I pushed and was sent to a second orthopedist who is re-evaluating my short leg and scoliosis. If these have changed at all (which is likely, it has been about a decade since last evaluated but at that point both had been stable for about 15 years) then they could be the cause of the bursitis, limited shoulder mobility, and possibly other areas of pain. Won't know more about this until early September.

My goal with all of this is to reduce the amount of daily pain and numerous subluxations/dislocations I experience. I want to be able to keep on top of it and not be constantly surprised by new problems. I love my career, but know EDS is slowly making it difficult to work everyday and be effective at what I do. My hope was for the pain management program to help me organize it all and figure out who and what would help the best, but so far it is, yet again, me figuring it all out with almost no knowledgeable help from the medical community. This is extremely frustrating and I sometimes want to just give up, but I am still too stuburn to admit I am disabled.

Summer is My Favorite Season

2010-06-10T11:07:00.000-07:00

I always look forward to summer. The flowers are blooming, trees fully leaved, and the days nice and warm. Perhaps it is the warmth I look forward to the most.

Heat helps my joints feel better. The lack of shivering also gives my muscles a chance to heal from the damage of winter. I can exercise more because I feel better, which, in turn, makes me feel even better.

I haven't had a major joint episode since my elbow last year, though my left shoulder gave me a scare about a month ago. Luckily, since it is getting warmer, I can exercise the joint more and strengthen it before I have a full dislocation.

I'm looking forward to my next visit with my EDS specialist. I have several new oddities that I want to discus with him to find out if they are EDS related. One is an occasional extreme headache, not a migraine but seriously painful, that is occurring about once every 2 months. The other is a bizarre hive-like rash that will occasionally appear and disappear a few hours later. I am wondering if my skin allergies are related to EDS (since my skin is very much a result of EDS) and if there is anything I can do besides live in a hypoallergenic bubble :)

Now, to go enjoy exercising in the warming weather and get these joints of mine ready for next winter!

Elbow Dislocation

2009-04-29T04:24:00.001-07:00

Ouch!

I've dislocated ankles, wrists and even a hip before. My mom even referred to one shoulder as "permanently dislocated." But this is the first time it has ever hurt THIS much!!

Looks like what happened is I weakened the elbow doing yard work, such is the nature of spring. Then I made the mistake of reaching for something on the table and was hit with an amazing wave of pain! I iced it, rested it, checked it out and knew it had dislocated and managed to reset it a day later, but the pain wouldn't go away.

After another day I called my Dr, and he sent me to an orthopedist. The ortho found that I had reset the elbow but managed to seriously pull the muscles and tendons around it until the elbow was so weak it keeps slipping - hence the continual pain.

He wrapped it and had me measured for yet another brace. This time it is a compression brace, from the same company that makes the shoulder brace I have had wonderful luck with. I'll wear the brace (once it arrives) for several weeks so the elbow can start healing, then begin yet another set of strengthening exercises. At least it wasn't too serious (I was afraid I was finally facing surgery and pins - pushed that one off a while longer :-) )

Taking Winter One Day at a Time

2009-02-06T16:42:00.000-08:00

I wish I could say that things are getting easier. At least it seems that EDS is getting more manageable.

I think the biggest help has been in the area of pain management. If I can keep my pain under control then I don't seem to have as many episodes of clumsiness or exhaustion. What is seeming to help the most are little things that I wouldn't have expected to make such a big impact.

For example, keeping a pair of thin knit gloves around and remembering to wear them whenever I go out (even if just to take out the trash). For longer excursions outside or even driving I have found that Isotoner gloves are amazingly comfortable, keep my hands warm and even help stabilize the loose finger joints. Now, if I could just find a pair of gloves that allow me to operate the latch on my dog's leash!

Another thing that is helping is to remember to rest. Sounds silly, but the little change of stopping and lying down for 30 minutes instead of pushing through until I complete something works wonders! I am only able to do this on the weekends, but it helps enough right now that my pushing through the work week isn't leaving me as worn out by Saturday as it used to.

Another Fall

2008-06-04T11:00:00.000-07:00

I fell again yesterday. This time I landed on the inside of my left knee and managed to damage not only that knee but the ankle, hip, left hand and elbow. I thought I didn't hurt too much this morning, but became light-headed at work and was sent home by the nurse. I see my doctor in about a half an hour to see if I did more than just bruise and to figure out what to do about the pain.

Wow, I hurt! I braced my ankle to try to stabilize my knee and hip (since my latest new knee brace slips after less than an hour of wear). I am limping a bit straighter, at least. If I sit too long my back hurts. If I stand my leg, especially the knee, hurts. Lying down causes all of the joints wrenched in the fall to throb. Any suggestions?

I have also started writing about EDS (as well as other topics). I had this need to spread the word, and was accepted as a writer for an online magazine called Suite101. You can read my EDS articles (yes! I'm a published author!) here:

I'm Still Around

2008-03-04T13:43:00.001-08:00

Winter is an interesting time. I find that days and weeks literally disappear. I realized today that it is March, and am not really sure where January and February went! Much of it is due to keeping busy with children and the seemingly endless array of winter storms, but I know now that pain is a major factor.

When you hurt and, like me, become very good at ignoring it your days become a blur. You do what you have to do and occasionally realize that the world is continuing around you. From time to time I realize it is happening, but feel helpless to do anything about it. Even resting can be a challenge when lying down becomes uncomfortable after about 8 hours.

With EDS if you fall or even just slip then you are risking soft tissue damage. One doctor described it to me as microscopic tears in the muscles and ligaments. Once this small damage occurs the pain can set in, and without lots of sleep and rest your tissue is going to take longer to heal. What a catch 22 that to heal you must rest but resting is so uncomfortable you must get up and move, thus leading to potentially more damage! Let alone the fact that your collagen is genetically damaged and it takes longer for your body to heal (even with rest).

Laughing at the irony is sometimes my only defense.

Yoga Therapy

2007-10-15T07:14:00.000-07:00

Some of you might think I am a bit crazy but I am really enjoying yoga therapy! I did not know what to expect, but now that I am in the midst of it I really like it.

Most people know yoga as stretching. I knew it as stretching, strength, and breathing. I now know it has a meditation and healing side as well. My therapist, Mary, is trained in using the Chakras for healing. Chakras are the seven energy points formed by our own energy (or conciousness) according to ancient teachings. She is helping me to balance my Chakras through meditation, mantras, and relaxation. If you want an idea about all of this, try this youtube link for a video of Yogi Bhajan: http://www.youtube.com/watch?v=3gG2jBJgkK4 .

I am, today, 18 days into a 40 day cycle. So far I am not experiencing less pain but am finding that I am managing it better. I am more relaxed and more aware of what is going on around me, especially more aware of what triggers the discomfort. The breathing exercises really have helped - especially when my shoulder brace broke and several days later I was in so much pain I went to the hospital. I find that I am now more inclined to slow down and relax instead of becoming tense and upset. I know this is just a start and there is much more for me to learn and try, but if all I get out of the therapy is the ability to manage the discomfort and pain without resorting to pain killers, then it is definitely worth it!!

Physical Therapy

2007-09-16T09:07:00.000-07:00

I went through 6 weeks of physical therapy for my major joints (shoulders, hips, knees, elbows). I had detectable improvement in most joints - including my ankles. My PTs and I decided to brace one shoulder and one knee since no matter what we tried I could not keep my shoulder in place nor my knee from bending backwards.

The braces are interesting to live with. The shoulder brace is designed for stroke patients who have lost the use of an arm and need to keep from subluxing the shoulder. It works really well, and even (almost!) eliminated the tension and pain from my neck and shoulder blade! Problem is, since I can still move my arm, it has started to rip. The knee brace has special straps we adjusted to keep my knee from bending backwards. It was interesting to watch me walk for the first 4 weeks of use; I felt like I was learning to walk again.

Though the braced joints are strengthening, I have had problems with my skin. If a brace slips and pinches, then I have bruising that not only hurts under the brace, but seem to not want to heal. On two separate days the heat index climbed past 100 F and I developed a heat rash under both braces. I have started going "brace free" on Sundays to try to promote healing of my skin because in both areas it is now red and blotchy and not wanting to heal. Realizing that the rashes are also itchy and when braced you cannot reach the skin to scratch . . . I've started looking for other options.

I could continue with PT, but it is impossible to go while holding my job. I would have to take a day off twice each week for a half hour PT session. Since I have participated in Yoga on and off since college I know that it is not just about flexibility but many forms also promote strength. I started calling around to find a studio that had an instructor who held afternoon or evening classes and would be comfortable working with my challenges. One of my PTs suggested a studio his wife recently started at, which is actually located between my work and home. The head of the studio had me speak with one of her guest instructors who is a Yoga Therapist. I had not previously heard of a Yoga Therapist, but called and was impressed. This woman specializes in using Yoga to heal the body and believes, with the help of her colleague who is a doctor who specializes in naturopathy, that she can help further strengthen my joints and significantly reduce the pain and fatigue. I've agreed, and will have my initial session next week.

More later!

Update

2007-06-28T13:57:00.000-07:00

It has been a while, but I now have some more information. Unfortunately answers will probably never be available due to how little is known about EDS.

It is now believed that my sister either had Vascular or Classical. I present with either Classical or Hypermobile. My test for Vascular came back as negative, but there is a 1 to 2 percent chance of error. This leads my geneticist to believe I most likely have Classical since types run true in a family. Also, Classical can present differently in family members - with stronger manifestations in different affected areas. That would allow my sister to have more vascular symptoms and myself more flexibility. Problem is there is currently no way to genetically test for Classical and, until another family member has a diagnosis, no one else to whom I can be compared. So, we decided to err on the side of caution and list me as EDS-Vascular (just in case that 1 to 2 percent chance has hit me).

What does this mean? I am ordering a new ID bracelet and will notify my local EMS services of my condition since I will need special treatment in a trauma situation. I will have an echocardiogram every few years. I have been instructed for specific vascular complications to watch for - especially pain in my chest or between my shoulders - and to get myself immediately to an Emergency Department if it happens. I have to work on strengthening both my heart and my muscles. Yea for physical therapy! Since I am still considered young and my heart shows no complications I am to push myself so that, if complications arise, I will be better able to survive. My children show mild signs and we have decided not to diagnose them unless these signs and symptoms are more persistent.

Well, that's it in a nutshell. I'll be posting less frequently now but will still use this blog to post anything interesting or newsworthy about my progress.

Spring Is Here!

2007-04-27T12:47:00.000-07:00

With the warmer weather I have been feeling much less pain. Tree has suggested that it might be the warmth, but today's storm front did not lower the temperature much (still near 50 C) and my joints are now sore. I cannot tell about the muscle groups since I was feeling better with the spring weather and went out and worked in the gardens; so my muscles were sore before the rain began.

I am starting to wonder if some of the joint discomfort/pain might be arthritis. Osteoarthritis is common with EDS, but I am still considered quite young for it to develop. Mom developed it in her early 40s, so it might not be too far fetched. I'll have to remember to ask at my next appointment.

Good News, Not-So-Good News, and Strange

2007-04-14T12:27:00.000-07:00

I'll start with the Good News: my type of EDS is NOT Vascular, at least we are 99% certain. The genetic tests are negative. The echo last week shows a normal heart (yea!). That leaves us with Hypermobile. When you score a 9/9 on a test where a 5/9 shows hypermobility, well, not too many questions left there . . .

The Not-So-Good News: I have been really sore and tired with the weather systems this past week. The annoying but somewhat weak storm on Wednesday/Thursday just made me tired. The Nor' Easter on it's way in for tomorrow is exhausting. My legs, feet, and hands hurt. My neck, shoulders, and hips keep popping. Luckily my arms are not statically throbbing, but if you poke me in the bicep I'll either cry or hit my head on the ceiling from the physical shock of pain.

We also know that one of the kids most likely has EDS (though we suspect both do). Nuala had her well-child visit yesterday and my nurse practitioner agreed to check some areas that are not normal for a pre-schooler. Her feet are flattening and she has a mild spinal curvature - both of which are indicators of EDS Hypermobile in young children (who, by nature, are flexible and difficult to test for hypermobility). She'll be meeting my chiropractor next week.

The Strange: Questions that have been brought up by the testing results. How could one sister have had Vascular and the other Hypermobile since the types run true in a family? Do my parents each have EDS? Are my parents related? (the answer to this is No, at least not within the past 300 years)

I now know that my mother and surviving sister do have heart conditions (which might indicate Vascular) but this has not been pursued with EDS in mind. My maternal grandfather also had the same problem as my mother. My father is not unusually flexible, considering other family members, though his brothers and sister are. My paternal grandfather also has had joint and heart problems, as well as being flexible, but has not been overly forward with sharing this information. If Hypermobile has a prevalence of 1 in 5,000 to 1 in 10,000 people, and Vascular is 1 in 50,000 to 1 in 100,000 (according to the EDNF), then what is the chance of two unrelated partners having one of each? I'm too tired to figure this one out.

Not much to do now but get that physical therapy appointment, watch my children, and wait for my next genetic's appointment. Perhaps I'd better get my eyes checked as well.

First Appointment

2007-04-02T15:06:00.000-07:00

Today's appointment went as expected. I have Ehlers-Danlos Syndrome, most likely the hypermobility type. This is unusual since my sister presented with so many of the symptoms of the vascular type. Consequently, I also approved the tests to check my collagen. There is no test (yet) that is associated with the hypermobility type, but some are available for the others. Hopefully my insurance company decides to cover this, which is likely since it is being used as a diagnostic test. If insurance does not pick up the bill I am looking at $2,000 to $3,000 in medical costs. A risk we are willing to take.

I also am being scheduled for follow-up appointments. One for an echocardiogram to see if there are problems with the valves in my heart. One with a physical therapist to start me with exercise routines that will strengthen and support my deteriorating joints. And, of course, a follow up to look at the results of the tests and echocardiogram.

And on to the next level . . .

Preparing for Tomorrow

2007-04-01T13:22:00.000-07:00

My much anticipated appointment is tomorrow. I spoke with another genetic counselor, Jillian, a few days ago. She has me gathering additional information to add to the medical records they already have and also prepared me for some of what will happen at the appointment.

I filled out a sheet of family information so I had all that with me. This required time on the phone with my parents to answer questions about them and my grandparents that I did not previously know.

I have collected photographs. Primarily these are of my sister so they have visuals of the prominent facial features that help identify the vascular type of EDS. I also have several of my other sister and myself, plus my children and my sister's. I am thinking about bringing one of my parents as well, just in case.

At the appointment I know I will go through a flexibility test. Jillian also mentioned skin features that will be examined, such as stretchiness and how my skin forms scars. She also mentioned that since I have never had an echocardiogram that I may need to have one scheduled; this is to check for mitral valve prolapse which I have discovered is also a common problem with EDS.

More soon . . .

Anatomy of a Fall

2007-03-25T11:51:00.000-07:00

This fall was nothing unusual, even typical for me. What is different is I am now hyper-aware of how my body responds to everything.

I was trying to get one of our cats into the carrier for a vet visit on Thursday afternoon. Not only did she escape but I instinctually spun around to catch her. My left foot never moved even though the rest of me did. It was as if my ankle was a ball and socket joint! When I put my right foot down my left ankle, followed by the left knee, gave out and down I went. I couldn't stand back up until I popped my ankle. What was interesting is that my joints (hips, knees, ankles, and areas of my feet and toes) hurt but not as much as the muscles. My back did not even bother me much, and all discomfort was gone after a good night's sleep. My legs took longer; only easing up the throbbing in the muscles and soreness in the joints as of yesterday evening.

I need to find a way to deal with the discomfort I am having other than being stoic. I've given up on acetaminophen. Since two extra strength capsules make me light-headed I am leery of taking more, but those two do nothing for the muscle or joint discomfort. Warmth helps, but not completely and not when there is a major atmospheric weather change. Cold doesn't help at all and sometimes makes it worse. Alcohol makes me forget I hurt, but I can still tell the discomfort is there. Chiropractic helps with my spine, but unrealistic for everyday unless I convince Tree to become a chiropractor. Aspirin and ibuprofen both have dangerous results for me or I would try those. I am on the lookout for more ideas to try since it is not like this is actually painful and I don't want to take chances with pharmaceuticals.

Sore and Tired

2007-03-18T12:21:00.000-07:00

It has been a couple of weeks of exhaustion. I have been trying to analyze just what is going on. This is what I am currently observing:

For the past few years I become REALLY tired in the winter, especially when it is very cold or we have significant weather events (like the 12+ inches of snow yesterday). A walk to a neighbor's house can mean a nap or serious lethargy for the rest of the day and my legs will be sore for at least a day or two after. My arms do the same; not as problematic after a walk but can be so if I do something simple like hold a vent in place while Tree fastens it to the wall.

My joints also go *nuts*. Standing in place is almost impossible to achieve since almost every joint involved, from my spine to my hips to my feet, feel like they are slipping back and forth. Consequently, I look like a waving flag (or a drunk - I'll leave the image to you :-) ). Sitting down means sore hips and not only the challenge of standing back up but also not knowing which way to sway to get a wayward joint to slip back where it belongs.

As an example, while typing this post I have had to stop several times to pop my joints. I know, Mom, it is a bad habit, but useful because it makes the discomfort go away and in some cases is necessary to regain the use of that joint (or the attached limb). My hands (not finger knuckles, but the back of the hand), neck, spine, left hip, and right shoulder are frequent annoyances while typing and rarely even need gentle pressure from anything but normal movement. The worst are my elbows; I don't have to do anything but move them let out a loud, satisfying *pop*.

I wonder if my muscles are overworking in the winter to keep my joints from dislocating, thus causing the soreness and exhaustion. I do feel like I have run a few miles or played a strenuous soccer match this past week. I am eagerly awaiting my first face-to-face meeting with geneticists so I can run some of my concerns and observations past them.

Moving Forward

2007-02-28T04:24:00.000-08:00

I don't play the "waiting game" very well. My doctor's office had difficulties locating the correct group at DHMC for the appointment, so one afternoon I decided I had waited long enough and both located the group, had a phone consultation, and set my first appointment.

I spoke with a genetic counselor, Sheila. She is wonderful! She knows the syndrome well enough to both ask me many direct questions and effortlessly provide answers to mine. It already seems like I have found the "right" place. Sheila advised me to make the appointment a month out so we have time to gather medical records, mine as well as my sister's, and give the group some time to pour over these. She also understood that there would be more in my sister's records to support a diagnosis of EDS than in mine.

My sister had several dramatic medical incidents that required medical/surgical support whereas most of my symptoms are not something I would consider taking to a doctor. Until a few weeks ago I wouldn't have considered my flexibility a problem; double-jointed joints runs in the family and is just a way of life. My skin being loose; never thought to check the skin on other people and it is not loose all over, mostly on my hands and neck. My joints snapping and popping and the discomfort/dislocations that result mostly happen in the winter and in association with major storm systems; I always thought it resulted from old joint injuries and the atmospheric pressure changes - I even enjoy being a human weather station! I had been working on an excuse for the discomfort in my hands, feet, hamstrings, calfs, biceps and triceps each winter - but am now finding it is normal because of the extra support these muscles have to provide the joints in my extremities when arctic cold weather arrives.

To top off the initial phone consultation, my doctor's office found the correct place the same day I did. Sheila noticed an appointment had been made for me in Lebanon, whereas I made my appointment in Manchester (shorter drive and can be done after work). She called me immediately. I know I am in good hands.

Random Research

2007-02-18T13:45:00.000-08:00

I wanted to know if there was a connection between Marfan Syndrome and Ehlers-Danlos. What I found is that they have some similar symptoms in relation to collagen but they are caused by different genes. The genes involved with Hypermobility EDS are COL3A1 and TNXB. Interestingly, COL3A1 is reported to be the one primarily responsible for the Vascular type and TNXB with another (I think it is the Classical type, but am not sure). I found most of the information at John Hopkins University's OMIM - and, WOW, did it push my medical/genetics understanding!

Some other pieces I found in my random web wanderings is that collagen is the primary protein in the body. I don't remember where I read it, but I think it accounts for something like 25% of bodily proteins. It's production is linked to Vitamin C - so I will now be taking more until informed otherwise :-) . I also found that proteins are broken down and removed from the body by enzymes such as protease and bromelain. Bromelain is found in fruit such as pineapple and I am going to avoid it for a while.

Paranoid? Maybe . . .

Doctor's Visit

2007-02-16T16:56:00.000-08:00

My visit today did not provide any surprises. I did my homework ahead of time and made sure that I not only knew as much as possible about Ehlers-Danlos but I also looked into what was available at both Dartmouth-Hitchcock and Mass General. My family physician asked me about my sister, my own flexibility, and my current thoughts. He stated that he did not know much about the syndrome and would have to research it - so I mentioned that Medical Genetics at Dartmouth-Hitchcock (DHMC) states on their website that they provide services for Ehlers-Danlos. He replied that it would make more sense to refer me to them. Now I am awaiting a phone confirmation of my initial appointment.

I am more than a little nervous. Forget the potentials of testing results; I dread car rides. It will be about a 2 hour drive each way, and I find that a half-hour drive to work can sometimes leave me sore. It is amazing how the winter affects my joints; it leaves me sounding like a batch of popcorn and feeling like I've enjoyed an afternoon of soccer when all I've done is get out of bed and walk downstairs. Tree has offered to drive me, but the initial testing will be just on me and it makes more sense for him to stay with the girls. If/When the girls are involved I will definitely take him up on that offer; I can move around more as a passenger than as the driver and will want him fully involved in any decisions made concerning them.

Too much information, not enough answers.

2007-02-14T12:41:00.000-08:00

Have you ever had one event in your life become the catalyst to answering many questions? Then you discover the old saying, "for every question answered, two more questions are revealed," is true. That is where my journey begins.

My youngest sister died October 24, 2006. No apparent reason; just collapses and dies. After 3 months of speculation we are informed of her cause of death: Ehlers-Danlos Syndrome. The coroner even provided a pamphlet so we knew a bit more about the syndrome.

It took about two weeks for the shock to wear off enough for me to start researching this syndrome. I started with the Ehlers-Danlos National Foundation and still consider EDNF my primary source for information.

From the descriptions available I currently believe she inherited two forms of the syndrome, Vascular and Hypermobility. Why both? She and her daughter match the physical descriptions of the vascular type exactly. She also was extremely flexible and frequently dislocated several joints, one to the point that she required surgery to pin it in place. Add to that, her son's fingers are unable to manipulate buttons and zippers because they bend so far backwards but he has no overt characteristics of the Vascular type. Since the syndrome is "true to type," meaning that if a parent has a specific type then the child has the potential to inherit that type only, she would need to have had both genes to pass one of each type on to her children.

Then, I took a close look at myself. I, too, have very flexible fingers. I have scoliosis, subluxations and frequent dislocations. In elementary school I was commonly asked to demonstrate that I had a full split (and never took gymnastics), could bend my fingers into weird positions, pull my skin far from my body, even pop almost any joint - including my spine. It was neat then, but now I am worried. I have two young children who are also flexible, and my youngest has difficulties with her fingers dislocating.

I spoke to my chiropractor about my concerns yesterday. He had not heard of the syndrome, but looked it up in Merck's. As he read through the description he commented that it sounded similar to Marfan's Syndrome, which he does know about, except that it is a mutation in collagen (I haven't looked into this connection as of yet). He asked me to demonstrate the flexibility in my fingers and skin, then suggested that I not only find a way to get my children and myself tested (especially try to find a genetic test) but that from now on he will approach my care as if I do have Ehlers-Danlos.

I have an appointment with my family physician this Friday to discuss the possibility of having my children and me tested. I am not expecting him to know about the syndrome but hope he will be open enough to either research where/how to have this done or refer me to someone who will.

Until I have more information all I can do is speculate.