Ehlers-Danlos

I am dx'd Ehlers-Danlos III.  My mother was diagnosed with fibromyalgia and my daughter clearly dx's with Ehlers-Danlos III.  So we think my mother (deceased) had  EDS III.  Amongst an array of symptoms that fit into the EDS III category, she had MVP but it wasn't diagnosed until after the birth of her 6th child and was never an issue during pregnancy or childbirth.  

My question is this, can cardiovascular problems develop as an EDS'r III ages?  

I do understand that in a study between a Cincinnati clinic and the NIH (ODonnell), that approx. 20% of a group of EDS'rs (II & III) were identified to have CV involvement.  But from the literature, I can't find if there is a risk for development of CV involvement as an EDS'r ages, more specifically an EDS III patient.   From my experience with geneticists, I've learned that sometimes there isn't solid science on a matter however there is a suspicion of something common to this group ....but this is only through observation and they relay it as so.  

Any suspicions or science on CV involvement in the aging EDS groups besides the one with expected CV problems (is that IV?)

[Lisa Kessler, MS, CGC]

Thank you for clarifying the information for me about your diagnosis of EDS.  It sounds like you are familiar with the medical literature on cardiovascular findings and EDS.  As you know, Ehlers-Danlos syndrome (EDS III), is also called the hypermobility type.  EDS is a group of connective tissue disorders that affect the skin and joints and other systems of the body as well, and some symptoms from different types do overlap.  To answer one of your questions about testing for EDS from your previous post, testing is not available for all of the types of EDS, so we do not yet have an EDS test panel.  At this point in time, some of the types of EDS such as the hypermobile type are mainly diagnosed clinically.  There is some research testing available for a protein known as tenascin X, and a deficiency in this protein has been associated with some individuals who were diagnosed with hypermobility EDS.  

Hypermobility EDS runs in families in an autosomal dominant way.  This means that someone with the condition has a 50% chance of passing it on to each child s/he has.  

As you are aware, the main symptoms for hypermobility EDS are soft velvety skin that may be mildly stretchy, joint subluxation and dislocation, chronic pain and degenerative joint disease.  There are only a few studies that look at cardiovascular findings in people with classical (types I and II), and hypermobile EDS.  One study found that people with classical and hypermobile EDS are at increased risk to have a mildly enlarged aortic root, but no long term data on prognosis are available.  Some previous research included MVP as a feature of all subtypes of EDS, but more recent research did not support this finding.  Another recent paper reported on an ongoing research study with the plans to accumulate longitudinal data on people with the classical and hypermobile types of EDS.  

There does not seem to be enough long term data to answer your question about aging with Hypermobility EDS and whether or not that increases your risk to have any problems with the heart beyond what the current studies have demonstrated.  I can understand that it may be difficult when science does not yet have the answers to these questions.   I wish you all of the best.    

[sandysandy]

Thank you so much.  Your reply cleared up what is unclear.  : )  

EDNF.org (Ehlers-Danlos National Foundation) is a great resource for anyone with EDS (medical lit posted for anyone there) and raises funds for research.  

[SueNYC]

I went to a geneticist,  He was pretty convinced that me and my daughter had EDS - never finished the panel because I was only concerned about the one type (hyper mobility, elastic skin, high arched palate, baby pictures -etc.).  Anyway, is EDS ever asociated with fibroasenomas - not really that important but I have a few - just wondering?

Susan

[SueNYC]

I went to a geneticist,  He was pretty convinced that me and my daughter had EDS - never finished the panel because I was only concerned about the one type (hyper mobility, elastic skin, high arched palate, baby pictures -etc.).  Anyway, is EDS ever asociated with fibroasenomas - not really that important but I have a few - just wondering?

Susan