Dr. Diana Driscoll spoke to doctors and patients at the Ehlers-Danlos National Foundation Meeting (EDNF) in Houston, Texas August 1st and 2nd. Her topic was “Your Eyes and Ehlers-Danlos Syndrome”. Dr. Driscoll, herself is an Ehlers-Danlos Patient, therefore her lecture extended beyond the ocular effects of Ehlers-Danlos Syndrome (EDS) and included some of the treatments and techniques that she has found to have increased her functionality.
The National EDNF Meeting is held every other year and is presented as an opportunity for patients with EDS, doctors and researchers to unite in their quest for answers to this debilitating condition.
Ehlers-Danlos affects multiple body systems requiring a multidisciplinary team of doctors to assist patients. Physicians of many specialties were present as speakers, patients and attendees.
About Ehlers-Danlos Syndrome
Ehlers-Danlos Syndrome is an inherited connective tissue disorder affecting our body’s production of collagen used to make connective tissue. Connective tissue is the glue that holds our body together making up approximately 80% of our body weight. The most common symptoms of EDS affect the joints and skin.
Skin symptoms are characterized by thin, soft, velvety skin that bruises easily and stretches. Tearing of the skin is common along with slow wound healing. Scars are often look like “cigarette paper”.
Joint symptoms include hyperextensible joints (joints that extend beyond their normal range of motion). People with hyperextensible joints have often been called “double jointed”. Because the ligaments and tendons are “stretchy” the joints dislocate or become subluxated easily. The premature onset of osteoarthritis is common and is often difficult to identify in an X-ray.
Some of the more common ocular symptoms of EDS are keratoconus, angioid streaks, and myopia. EDS patients are not candidates for LASIK. At Total Eye Care we are keenly aware of the potential complication that can result from any corneal surgeries performed on Ehlers-Danlos patients.
One of the most debilitating complications 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, body temperature, blood sugar regulation, hormonal/endocrine imbalances and our sleep cycle.
Postural Orthostatic Tachycardia causes a person’s heart to race when they stand still. When a normal person stands, their heart rate may go up a few beats per minute and stabilize. A POTS patient’s heart rate will go up over 30 beats per minute. If their body can not compensate, a POTS patient will often faint or shake uncontrollably. A tilt table is used to test for POTS.
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’s ability to regulate our bodies.
The classification system for Ehlers-Danlos Syndrome consists of 6 types; Classical, Hypermobile, Vascular, Kyphoscoliosis, Arthrochalasia, Dermatosparaxis. The prevalence of EDS is approximately 1 in 3000. EDS affects both males and females and is not specific regarding race or ethnicity.
Ehlers-Danlos Syndrome is a genetic disorder, however many patients, such as Dr. Driscoll, are unaffected until the symptoms are triggered by a virus.
A copy of the lecture slides for “Your Eyes and Ehlers-Danlos Syndrome“ is available in PDF form as is a handout for patients to give to their eye doctor before their eye exam.
This entry has been cross posted on The Total Eye Care Website.
19 responses so far ↓
Fiona // August 11, 2008 at 10:11 pm |
Thank you so much for posting the lecture slides of your talk. I had no idea that so many of my eye problems were EDS-related! My eye doctor had never heard of EDS but was willing to Google it and make an educated guess that he should be looking for lens issues. I will certainly bring the handout to him next time.
Dr. Diana Driscoll To Speak Before the Ehlers-Danlos National Foundation National Meeting « The Eye Doc Blog // August 12, 2008 at 10:28 am |
[...] UPDATE: Visit the post EDNF conference entry for the lecture notes and a handout for eye doctors. [...]
Effie Goodall // October 9, 2008 at 7:28 am |
Dr. Driscoll. I am so glad that you posted your lecture slides and your personal information. I think that I have the kind of POTs that you do. I get lots of faint spells and fatigue is hard to endure. I have seen a sleep doc and I ask him if I could try my own little test on used his oximeter going from sitting to standing my heart rate increased by over 30 beats.
Most recently my GP sent me to a neurologist because I have abnormal tracking and saccades in my eyes. The neurologist is sending me to a neuralopthamologist to evaluate further. I had a horizontal MRI that came out normal. The NP at the neurologist’s office said that a vertical MRI would not have changed my MRI results. What are your thoughts? What could abnormal saccades and tracking mean? I have Classic EDS. Thank you again for being so helpful and transparent regarding your illness. I sincerely wish you the best and have the most appreciation for you.
Warmly,
Effie Goodall
Diana Driscoll // October 10, 2008 at 3:31 pm |
Hi Effie,
It is so difficult to give you an answer on your tracking and saccades without getting a chance to check your records. What is the neurologist’s reasons for it, if he can’t see anything on the MRI? I’m assuming this is a new symptom/sign for you? I wouldn’t let this just drop. Ask until you get an answer. If you can email me, I’ll send you a link to the latest article on vertical MRI’s with cranio-cervical instability. Are you having any Chiari symptoms? Any anterior brainstem symptoms? Any blepharoclonus? (If you don’t know, that’s OK. Just ask the neurologist. It may send him running to his books, though!). It’s important to keep seeking answers for abnormal results like that.
Fondly,
Diana
Jilly // October 29, 2008 at 1:15 am |
I have EDS III and POTS and now have developed monocular diplopia. My eye Dr. worked for 2 hours today w. various tests and she has referred me to a specialist. I will give him your handout.
Thank you.
Jilly // November 19, 2008 at 2:50 am |
^^^
Macular pucker. (I’m 41.)
Wendy K. // January 7, 2009 at 4:48 pm |
Hi Dr. Driscoll,
I just wanted to write you a quick thank you on posting this info. for patients with ehlers danlos. However my nephew (son) has not been officially diagnosed yet (already know for sure in my heart) this info. was very helpful for the optometrist. He had never seen a patient to date with it so this was/will be his first. He was very receptive to your information. I have posted a link to your info. on my son’s website. If you get a chance, i would like you to visit it. We have a lot of info. on there and if you would like further info. on medical/family photos just email me and i will send you a link to that as well. I am pushing that info. on any doctor who will have it to better understand the disease.
Wendy K.
serena // January 14, 2009 at 6:15 am |
firstly thank you for this v informative blog!!!
keratoconus- does/can the corneal cone shapedness come and go? vary from day to day?
bf (who to me shows signs of ’several’ connective tissue disorders) the other day had the cone shapedness as pronounced as in the pictures in your slideshow- next day it was back to normal…????
with thanks
serena (EDShypermobility/autonomic dysfunction)
RAD // January 14, 2009 at 11:06 am |
Thanks for the nice comments. Regarding your keratoconus question. I would not expect a keratoconic cornea to change so rapidly that it goes from normal to showing the pronounced protusion, called Munson’s sign, that rapidly. I would recommend that you have your boyfriend see an eye doctor and he or she could take topographical corneal photos and corneal thickness measurements (pachymetry). These measurements would be the way to monitor that type of change. Follow this link for a more comprehensive review of keratoconus. Please feel free to write back if you have more questions.
Kelly // February 10, 2009 at 10:20 pm |
I was just diagnosed with Ehlers-Danlos today and have a question. My eyes used to be very blue, but have changed color over the past two years. My husband has noticed it, too. They now look almost green, or like there is more yellow/brown that with the blue looks green. Is this possibly related to the EDS? Is this even possible at all? My primary care doctor looked at me like I was crazy for even thinking they have changed, but they have.
RAD // February 12, 2009 at 2:43 pm |
Congratulations on getting a diagnosis, many people struggle for years trying to find out what is wrong. Regarding the change in the color of your eyes. I am not aware of any reports that EDS has changed the iris color. I would recommend you take close up photos of both eyes and repeat this periodically, possibly every 3 months. Your eye doctor could also help you with this. I can not think of any reason why EDS would cause a change in eye color. However, so many things about EDS are unknown it merits further study and follow up.
David // March 21, 2009 at 4:13 pm |
I have just been diagnosed with EDS III and have recently been looking into LASIK surgery. I’ve never had an issues with other types of surgeries/healing in the past. How real is the risk here?
David // March 21, 2009 at 4:29 pm |
I forgot to mention above that I have seen two clinics prior to the EDS diagnosis. One cleared me without question. The other mentioned I have thinner corneas and I had to get some yellow deposits checked out. A consultation/check with an independent ophthalmic surgeon said they were insignificant.
I really would like to get LASIK done as I couldn’t ever get on with contacts and I find glasses very uncomfortable above the ears and across the bridge of the nose.
David // March 21, 2009 at 4:31 pm |
Further to above, both clinics use Wavefront technology.
RAD // March 23, 2009 at 5:48 pm |
David, a number of things concern me here, not the least of which are thin corneas. I would encourage you to see a doctor that is familiar with all of the the refractive options, including laser refractive surgeries, lens replacement surgeries, contact lenses, glasses etc. You only have two eyes and if you should have to fix a bad LASIK/PRK outcome you will wish you had exhausted all options.
Ehlers-Danlos And Your Eyes // August 25, 2009 at 4:38 am |
[...] recently came across a great blog post on The Eye Doc Blog that talked about Dr Diana Discoll’s address on the ocular effects of Ehlers-Danlos Syndrome [...]
Kim // September 18, 2009 at 5:40 pm |
I was just diagnosed with EDS-hypermobility . Took a year from when my Rheum. suspected it. Tmjd diagnosis at 20 but not too many problems except Proprioception srewed up my short game in golf. The problems with EDS accelerated after pregnancy. Had C-def for 28 days early in pregnancy followed by 1st hiatal hernia. Could this virus have triggered the EDS?
RAD // September 19, 2009 at 5:09 am |
Congrats on finally finding an answer. As far as can a virus cause EDS, not that I’m aware of, however the research into EDS is relatively thin at this point. A virus has been known to trigger autonomic nervous system dysfunction in EDS patients. Many patients with EDS get POTS (postural orthostatic tachycardia syndrome) which is a type of autonomic dysfunction.
RAD // September 19, 2009 at 5:48 am |
My prior answer was before morning coffee. Many women notice that EDS gets worse during pregnancy, this is because their bodies increase the amount of relaxin released, a hormone that loosens the joints, making delivery of the baby easier. Unfortunately, it will make all of their joints more flexible. After pregnancy the relaxin levels go down.