As many of you may know Dr. Diana Driscoll is on medical disability due to the autonomic nervous system complications from Ehlers-Danlos Syndrome and has not seen patients in the office since August of 2004. In the course of her research trying to find what was making her ill she accumulated considerable knowledge in Ehlers-Danlos and the complications associated with it. Dr. Driscoll has been asked to share her knowledge at the Ehlers-Danlos National Foundation Meeting in Houston July 31st - Aug 2.
Ehlers-Danlos Syndrome is a connective tissue disorder that affects multiple systems and organs. EDS, as it is called by patients that have it, has numerous different types. The most common types of EDS are classical and hypermobile. Another, rarer type called the vascular form, is the most dangerous because it involves weakening of the blood vessels which may rupture (an aneurysm), which is often fatal.
The most common symptoms of EDS are hyper-flexible joints (people sometimes refer to this as “being double Jointed”) that are flexible beyond the normal range of movement, skin that is very stretchy, soft, bruises easily and is sometimes fragile (tears easily). EDS patients often complain of joint pain that may be mistaken for arthritis, yet the x-rays are not consistent with arthritis.
The most debilitating complication of EDS is a type of dysautonomia called Postural Orthostatic Tachycardia Syndrome or POTS. Dysautonomia is the result of our autonomic nervous system ceasing to function properly. Our autonomic nervous system regulates functions that our body does automatically such as digestion, breathing, heart rate, blood pressure etc. It is estimated that 40 to 70% of EDS patients suffer from POTS. Many patients with POTS don’t know that they have EDS and are often mis-diagnosed with fibromyalgia or chronic fatigue syndrome. It is thought that autonomic dysfunction is caused by instability of the cervical spine thus interfering with the brain stem.
The medical literature reports that the incidence of EDS is approximately 1 in 10-14,000, however doctors report seeing it clinically more frequently. It is hypothesized that once doctors are made more aware of how to diagnose EDS that the reported incidence will be more like 1 in 3-5000 people.
Once doctors become more aware of how to identify patients with EDS I believe that the incidence reported in the general public will rise markedly. I have personally identified numerous patients in our practice that have EDS and concur with the incidence of 1 in 3-5000. I, in fact, would not be surprised if it was indeed more common than 1 in 3000.
Registration for the Ehlers-Danlos National Foundation 2008 Meeting is available at the EDNF website.
UPDATE: Visit the post EDNF conference entry for the lecture notes and a handout for eye doctors.
5 responses so far ↓
Diana // July 4, 2008 at 2:09 pm
I wanted to take a moment to thank everyone who has continued to keep me and our children in their thoughts and prayers through this journey. I am grateful that my husband (Dr. Rich) is able to help so many others with a diagnosis, especially those who have suffered for years without a doctor’s understanding.
For those wonderful folks who continue to ask how I am doing, I wanted to report that I have improved to the point that I am able to work some (although I’ll never again be able to practice optometry). I am involved in hotel development, and as long as the work is on my body’s own schedule, I’m truly enjoying it! A tremor, inability to stand beyond a few minutes, positional neck issues, balance problems, and incredible fatigue has slowed me down, but makes me that much more motivated to make a difference in the world, and to work as long as my body will allow!
Blessings to you all,
Dr. Diana
Sherry Wermuth // July 17, 2008 at 10:33 pm
Diana walks as brave a walk as anyone I know. She stays incredibly positive despite the fact that she cannot care for her children and home to any significant degree, and certainly less than her conscientious heart wishes to. She faithfully expresses her gratitude for her husband’s unconditional love and double-duty at home, and she faces each day with a courage that defies her suffering. Her daily battle with intense pain in the midst of a frightening and degenerative condition is nothing short of valiant. To see her is to behold a beaming and constant smile that says she is going to be joyful in the face of grave circumstances. I am changed when I spend time with her because I witness her choice to be courageous and grateful. This, in spite of the fact that she watches her young children suffer from the same debilitating diseases.
Thank you, Diana, for your “Yes” to life and love.
linda simmons // August 12, 2008 at 8:14 am
I am soo sorry we missed out on your lecture in houston, there was so much to see.My dtg. the 15 yo in the w/c sat up tooo long fri. and took a dive off the couch face first, she was fine,the BP was low and pulse fast.She is EDIII, 9/9 on beighton scale,S/P fusion,decompression, and reflux sx.We now know POTS is also a major problem for her, its only taken 4 yrs to figure that out.We are waiting to see a specialist for the tilt table test,she goes to school 1/2 day and has a aide with her at school.Do you know anything else we could try?She is on salt pills and forced fluids, atenolol 50mg for tachycardia. LindainFla
simmonr@bellsouth.net
Dr. Diana // August 12, 2008 at 3:39 pm
Hi Linda,
I must first tell you that most everyone I know with POTS does worse ON the B-blockers! They slow the heart, but usually make us feel much worse overall. Please download the lecture notes and I think you’ll find some handy hints there. We are all short on Magnesium, so I’d add that right away (in the evening), and try an abdominal binder when she wants to be more active (you can get them at the drug store). Huge help for me! Is she wearing a cervical collar? I wear one when I’m active, in the car, or just feel sick… xanax is a big help for me, as is Cymbalta (talk to your doctor first!). I also couldn’t get through the day without a Sudafed! Again, please discuss any vasoconstrictors with your doctor, and let me know if you have any questions on any of the suggestions I have in my notes. Hang in, you are not alone on this journey,
Warmly,
Dr. Diana
linda simmons // September 30, 2008 at 5:58 pm
Thank you for the response its so funny Lisa and I were talking about weaning off the atenolol. She takes suafed just cause she feels stuffy and I guess it makes her feel better, she wears her cervical collar in the car for safety her Dad wanted her to stop today and she said she wasn’t taking it off.We see Dr Grubb’s NP in Oct.
Thanks, Feel good
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