Hello my faithful friends. Here I am, breaking into The Eye Doc Blog yet again. I may need to consider having one devoted solely to this subject, don’t you think? Meanwhile, I wanted to reach out to you, first to thank you for your amazing stories of courage as we all help each other put the pieces of the proverbial puzzle together. It was also time to give you another update and a wonderful source of information. Thanks, Babe (that is Dr. Rich to you all. ahem) for posting this.
I’d like to address our Ehlers-Danlos friends (known and unknown), those suffering from POTS (postural orthostatic tachycardia syndrome) and those dealing with Multiple Sclerosis (even if the diagnosis is not certain). Research is on the cutting edge of science in these areas and because ‘I have donated my body to science while still using it’ (gotta love a pioneer, right?), I’ve jumped at the chance to have the veins in my neck and head tested for blockages or narrowed areas (stenoses) and poor flow. This condition is known as CCSVI (chronic cerebrospinal vascular insufficiency) and I was recently tested for it (BINGO!) and treated.
I don’t show my veins to just anyone, but for you, dear friends, I’ll give you a peek (hopefully you have a strong stomach). On your left, the large “garden hose” is my internal jugular vein (too large because the valve at the base was lousy), and on your right are my “Ramen noodles”. Somewhere in that mess is a tiny internal jugular vein, and most of the other noodles are “collaterals” – an attempt by my body to drain fluid from my brain my making its own route out of there! I had that jugular vein ballooned, and the bad valve ballooned. Another vein was functional only when I looked to the left. It was not treated. (Fascinatingly, I knew before the procedure that if I lay on my back I feel as if I’m suffocating unless I tip my head to the left.)
The venogram procedure for CCSVI is something I knew I needed to have before there was even a procedure (follow that one!). I knew there was too much pressure in my head, but I wasn’t sure what to do about it except for taking Diamox to relieve some pressure (which DID help). By comparing notes with many of you, I was convinced that our high pressure was confined (mainly) to the head and neck area – an interesting puzzle.
After the procedure, I felt better, even the same day. As a doctor, I am well aware of the “placebo effect” and I try to look as objectively at my condition as possible. I also know that I need to give it time to be sure the placebo effect is unrelated.
Knowing that, however, I was aware that I could think more clearly, multi-task, I had very little mental and physical fatigue, and strangely, I immediately began to make my own body heat (I’ve been hypothermic for years). I had a bit of a crash 5 days later (which I was told to expect, but no one knows why that happens to many of us). I’m climbing back out, though. Other symptoms haven’t changed, so I’ll keep you posted.
Meanwhile, I must thank Dr. Clair Francomano, who has tirelessly given of herself to research Ehlers-Danlos Syndrome. She is a famous geneticist and has always been THE expert on Ehlers-Danlos and has made some discoveries about our bodies that continue to amaze me. Her knowledge base extends beyond EDS research, and you have likely heard her speak or come across some of her research studies (her C.V. is approximately 34 pages long). Beyond knowledge and the much needed intellectual curiosity that a rare disease requires, Dr. Francomano is consistently empathetic towards her patients. I don’t know how she continues to be so incredibly caring to all of us, but we are all blessed to have her in our corner.
I am also incredibly grateful to my Interventional Radiologist, Dr. Gooding. It was not only his skill and experience that impressed me. Amazingly, he listened carefully to what I had to say (a feat in itself, as my husband will attest), and my procedure was painless, but fruitful. Dr. Rich and I knew he would be our choice for our children’s treatment, should the angioplasty be necessary.
And while I’m thanking people, I must say that the report I received from Dr. Haacke was simply amazing (it is about 60 pages long). The level of care and detail he puts into his reports will astound you, and it left little doubt in my mind that we were spot-on with CCSVI running in our family.
I would like to close by mentioning that many M.S. patients may have undiagnosed Ehlers-Danlos Syndrome, and I would recommend that all M.S. patients are screened for EDS. 90% of all Ehlers-Danlos patients are never diagnosed, but they go on to develop M.S. And finally, if you have a diagnosis of “chronic fatigue syndrome” or (heaven forbid) “fibromyalgia”, may I kindly suggest those are symptoms, not diagnoses? Again, Ehlers-Danlos is something to consider.
Thank you everyone for your love and support, and I hope that I’ve been able to give you some help also as we travel this journey together.
A gentle hug,
Dr. Diana
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Thank you soo much for postiing this on CCSVI. It is so awesome when a Doctor can validate CCSVI!! Sorry that your validation is first hand though. I just about cried when i read how you tilted your head to compensate for the lack of blood flow. I did this for years!!! I also had excruciating pain in my mastoid bone and base of skull when I lay down and turned my head to the right or the left. Then I was tested and treated for CCSVI. It has been almost 1 year for me post angio and I just had my jugulars Dopplered and they are still flowing, but I was told and knew from experience like you, that when lying down I could not turn my head to the far right without going numb and tingly and wanting to pass out. The Doppler just confirmed that as when Im lying down the right side goes totally blocked when I turn my head that way but interestingly when I am standing it is the best source of blood flow. Thank you for talking about CCSVI!!!!
Hi Ginger,
Thanks for chiming in! I am so glad to hear that you’ve had some relief from the CCSVI treatment.
The positional symptoms you described are HUGE for many of us, but most doctors hear about it and give you that stare that is on the edge of “I think this lady may be crazy, but I’m not entirely sure” look. Right? Doctors love objective reports, and seeing how the results of the venogram dovetail with positional symptoms is the kind of finding that will encourage the doctors to believe this is real.
I had gotten to the point (prior to CCSVI), of knowing that many of our symptoms were positional, but until technology could catch up with our needs (an MRI that could walk around with us, for example), it would be impossible to prove. We now have some proof. How great is that?!
BTW, wearing a soft cervical collar at night helps me a fair amount. I believe it keeps me from bending my neck into those weird positions that cut off the drainage. I am fairly flexible (Ehlers-Danlos), so I’m not certain if it would help you. They are so inexpensive, though, and you can usually buy one at a drug store, so you may want to try it. Our intracranial pressures are greatest at about 2 a.m., so night time is a critical time for us to try to keep the flow going!
One other thing — did you try tipping the head of your bed up a couple of inches? Because many of us (certainly me) have bidirectional flow in our veins, gravity can encourage the flow to go down, even during the night time. If you try it, please let me know how it goes!
Thanks so much, Ginger,
Warmly,
Dr. Diana
Looks to me that blood was draining from your head just fine. Was there any evidence that your intracranial pressure was increased? Was any attempt made to measure it? If not, you have been deceived. How much did you pay for this charlatanry?
“CCSVI” is junk science and “liberation” is a scam.
Thanks for commenting. There are a number of studies underway. Skepticism is healthy component to the practice of medicine. However, to proclaim CCSVI as charlatanry is premature. Yes we don’t have multi million dollar double blind studies to irrefutable proclaim CCSVI as the end all be all treatment for MS, however preliminary results for the treatment of CCSVI look very promising. Those that have been through years of suffering are not willing to wait to have traditional medicine bless their treatment. Early study results will be made public at the first meeting of the International Society of Neurovascular Disease in Bologna, Italy March 14-15, 2011. If a trip accross the pond is not for you I look forward to seeing you at the 2011 CCSVI CONFERENCE to be held in San Diego May 14, 2011 where a presentation of the current study results will also be made.
Thank you for popping on the site! It is only through corroboration and the sharing of experiences with open minds will we ever be able to advance in medicine. It seems that you have a closed mind so early in the game here, however , and I would not be so quick to dismiss much of Dr. Zamboni’s findings.
Did you read that I have Ehlers-Danlos Syndrome and Postural Orthostatic Tachycardia Syndrome (viral induced)? One look at the current MRI should not be reason to discount the possibly high ICP. When my symptoms began with flushing (after ruling out mast cell disease, we came to learn that flushing is a sign of sudden ICP increase), I developed occipital headaches that radiated down my neck and into my shoulders (another sign of high ICP). Taking Florinef multiplied my headache by about 100 in 4 days (it increases blood volume) and I got overnight relief from Diamox (which decreases the production of CSF and has a mild diuretic effect), I knew without a doubt that my suspicions of high intracranial pressure were correct. I had also developed dementia and aphasia that recovered (about 90% of the way) after 6 months on Diamox.
I have dural ectasia of the cervical area, though not below, my ear drums hurt when the Diamox begins to wear off, and I developed CSF rhinorrhea. My doctors/researchers agreed that a lumbar puncture would not be a good idea, as most EDS (classic) patients do not heal well when their dura is punctured, and even after what seems to be a successful blood patch, the puncture site reopens even years later. Much like normal pressure hydrocephalus, our suspicions are that the pressure surges periodically, causing the damage (brain atrophy is apparent when compared to my MRI from 5 years ago). I can’t tell you how many EDS patients show “normal or low” opening pressures, yet have high ICP and respond well to treatment.
I also had every symptom of Chiari Malformation in addition to symptoms of anterior brainstem damage (as in a retroflexed odontoid) without imaging to indicate either condition. This is when we began to consider the extra pressure collecting in the cisterns (especially in the cerebromedullary cistern near the foramen magnum) and possibly in the 3rd ventricle, causing the hypothalamus function to deteriorate.
My thoughts are of course seen through the eyes of an EDS patient, an eye doctor, a POTS patient and a soon-to-be M.S. patient (did you know that M.S. patients are 10-11 times more likely to have EDS than the general population?) And heaven knows our vessels (and connective tissue elsewhere) are affected in dramatic ways.
The MRI you see was taken after I had been taking Diamox for about a year, yet the radiologist agrees that there is an excessive amount of CSF. You would be interested to see that many EDS patients develop left ventricular diastolic dysfuction, including myself, that cannot be attributable to high blood pressure. Many of our symptoms (including short-term memory deficits and loss of executive function) may be attributable to decreased cerebral blood flow, secondary to the resulting cerebrovascular resistance when venous outflow is inhibited. As we know, cerebral blood flow is equal to the cerebral perfusion pressure divided by the cerebrovascular resistance. (Is this why vasoconstrictors and abdominal binders ease our symptoms?).
As far as the money goes, I feel it is an investment in research, if not for me directly, then for our children (collectively).
There are many things to consider here and I don’t believe we are a “one size fits all” population. We need great minds on this. We need healthy skepticism and the offering of alternative thoughts. We don’t need closed minds and we certainly don’t need unsympathetic dismissal. Seeing doctors who dismiss us without exhibiting the intellectual curiosity needed to understand the complex, is where our money and time is a total waste.
WOW!! So many of the ehlers/POTS/ chiari people continue to have complications despite the repeated fusions,lumbar punctures, this would begin to make some kinda sense to it all.
Hi Linda,
Dr. Diana
Thank you for commenting! I completely agree. There are so many similarities between Ehlers-Danlos, POTS, M.S., Chiari, Normal Pressure Hydrocephalus (among a few others), yet medicine still does not fully understand any of these conditions and their sequela.
When I was diagnosed with Ehlers-Danlos, I learned that 40-70% of us develop POTS, but no one knew why! I’ll discuss it further in another post, but for our family (with viral-induced POTS), the neurosurgeon explained that the CSF was no longer absorbed (through the arachnoid villi properly). Taking Diamox was a HUGE help, and completing the drainage with CCSVI will likely be the other piece of the puzzle for us.
Now we can look toward neuroprotective agents to take care of the brain cells that are not permanently damaged, and fortunately, many are responding over time.
It’s a journey, Linda, but to have some answers for fellow sufferers is wonderful.
I look forward to, well, looking forward!
I would like to add that intracranial pressure does not have to high to cause damage to the brain. Normal pressure hydrocephalus (NPH) can damage the brain just as much as increased intracranial pressure. NPH has been associated with Parkinson’s and Alzheimer’s disease. It is not the pressure but the increase in CSF volume that does the damage. In this regard, EDS patients often have underdevelopment of the face, vault and base of the skull called craniosynostosis, dysostosis and craniocervical syndromes. The smaller size (hypoplasia) of the skull as well as smaller openings in the base for the passage of the cord, blood vessels and nerves restricts CSF flow in and out of the brain. This can overwhelm the drainage system and cause an increase in CSF volume. It can also cause CSF jets in the brain. Forensic evidence shows that CSF jets are strong enough to scour holes in the roof of the hard bones of the skull. They can also damage the brain and cause turbulance in circulation that makes that makes it more difficult to get adequate oxygen and nutrition to the cells. It also slows down waste removal.
In any case the technology is here now to measure blood and CSF flow in the brain.
Hi Dr. Flanagan,
Thank you for commenting. You are spot on! For most Ehlers-Danlos patients, we have anatomical and skeletal variations (I like to call them improvements – ha) that may set us up for this. I have a small head and likely the small foramen magnum to go with it. Most every EDS patient has mid-face hypoplasia and a high palate. We also have lower ears with eustachian tubes that angle upward fairly sharply (we sound like a bunch of freaks!). But it is all fairly subtle to the untrained eye.
Because you have studied cranial anatomy for decades, though, you would probably see us coming!
I have quite a few EDS patient/friends who develop syrinx (I’m assuming from the “jets” as you described?) Many of these syrinx would come and go, amazingly.
I am so happy to see that technology is finally catching up to our needs! I’m so happy to know that your great mind is on this, Dr. Flanagan!
Warmly,
Dr. Diana
Dr. Diana,
Wow, thank you for sharing your story and information! My health “story” is very similar to yours: Probably EDS (neuro did the genetic tests for the types that have tests, which were negative, which leaves that I probably have hyper-flexibility type 3), POTS ( have tachycardia, fainting, dizziness, low BP, run high heart rate even at resting, yada, yada, all very autonomic disorder type stuff). Also, my ECHO showed: diastolic dysfunction; mildly abnormal relaxation w/o significant increase of left atrial pressure, Right ventricle at upper limits of normal, Mild pulmonary hypertension.
I had to go to the ER for a horrible POTS attack in October. The only thing that worked was 6 bags of saline IV fluid… yet I was not dehydrated. ER doctor, neuro and cardio all concluded that I must have low blood volume and therefore keeping it up is the way to treat (they also RX pressure stockings, but my hands are too weak from my neurological stuff to put them on myself and I don’t feel any difference when they are on than not on) me, so I am on fludrocortisone .2 a day (I’m not sure how much it is helping, I still have days with dizziness, tachacardia, etc. but nothing as bad as the huge attack that put me in the ER).
What I find fascinating about your story is that you mention your headaches got MUCH worse on the florinef… because my “migraines” have gone from 6 or so a month to 15-20 a month! My only treatment is Excedrine, if I catch it in time. No one has made any connection with my increase in headaches with the fludrocortisone! I will ask my dr. about that!
Also, my grandmother had glaucoma (and Alzheimer’s) and my dad has low pressure glaucoma. Incidentally, my dad’s brother, my uncle, has Parkinson’s. So much of this seems family related!
I also have been worked up for mast cell disease and a very invasive surgical muscle biopsy so we could test for mitochondrial disease (negative but showed mild neruomuscular disease – totally useless), no lesions on MRI for brain or spine (but all my symptoms point to MS). Haven’t had a spinal tap yet, but after reading what you wrote, glad we haven’t tried it!
I found out about CCSVI and there was a big informational meeting in Seattle (I live in the Portland,OR area) the end of January and Arizona Doppler Services flew in to offer Zamboni CCSVI scans, so I went up for that and had 4 of the 5 Zamboni criteria (which kind of shocked me). I am scheduled for the “liberation” procedure at Vascular Access Center Seattle in April.
Thanks again for you post. Hopefully I will get away from these headaches soon!
Hi Jeri,
Isn’t it amazing how our stories align, yet with “twists”? I also evaluated myself (ha) for mast cell disease, and tried the mitochondrial repair stuff, to no avail.
Jeri, are your headaches ever in the back of your head, going down your neck and radiating out to the top of your shoulders in a kind of “angel wing” pattern? Or are they strictly migraines that affect your entire head?
Did anything seem to trigger your POTS? I may have some suggestions for you…
Angioplasty helped me only after I was able to “drain my brain” of the CSF that was “stuck” there (likely from damaged arachnoid villi due to an attack by viral antibodies). My “brain drain” is Diamox. Once the CSF could get into the veins, then, whoosh, out it went! Our son, though, had to go off of Diamox, and got no relief from the angioplasty, even though his veins looked just like mine.
For me, the Diamox took care of many symptoms, and the angioplasty took care of most of the “M.S.- like” symptoms (I was numb on my left hip and my right arm and leg felt like they were dragging toddlers around). I still have orthostatic intolerance, and I’m sure the Diamox isn’t helping me with that in the short term, but perhaps it will over the long term, as I believe it’s the pressure on our brains that is the source of our problems. If not, though, I’d trade standing for being able to think any day! Speaking of standing, Jeri, did you ever try an abdominal binder (nice and tight)? I’m trying to figure out if some of us get relief from these binders because of the help it gives our hearts in getting the fluid into our brains. A big area of focus for me now is preventing congestive heart failure (yea, I’m hearing a fair number of us head in that direction, too. Ugh.)
I am collecting head circumferences (and length and weight) for kids (or adults) who are affected and have that data for the first 12-15 months of their lives. I believe I’ve found that there IS a “congenital” CCSVI evident by the increase in head circumference (much more so than length and weight) of babies before their skull sutures are closed. Yes, I agree, this pressure stuff can be congenital and can manifest in a variety of neurological disorders. I’ve have some imaging that indicates the pressure may actually push our brains onto some of our cranial nerves, causing other issues (pressure on cranial nerve 2 would cause glaucoma, for example).
Oh, Jeri, amazingly, when I got my fMRI result back, I learned that I developed a small brain lesion. I can’t tell you how many people I know have both M.S. and EDS. I think I was headed there… Yikes. Did your MRI show any brain atrophy, perchance?
Please let me (us) know how your angioplasty goes! Thank you for reaching out, and I am excited to hear from you again soon!
Dr. Diana
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Dear Doctor Diana,
This past Christmas my 57 year old sister had a carotid dissection and a massive stroke. She has had MS for the last 20 years. The doctors all say that the carotid dissection has nothing to do with the MS. I wonder if she could have the vascular form of EDS. Does this make sense to you?
Thanks for your help. Theresa
Hi Theresa,
A form of EDS is certainly possible, and all types have some vascular weakness. My main reason for doubting vascular EDS is purely because of her age. It is unusual for most vascular patients to live that long (but not impossible). Because it is generally hereditary, I wonder if others in your family show signs or symptoms of vascular EDS? I have a video on vascular EDS at Prettyill dot com. It may be of some help for you. And Theresa? I’m so sorry for your loss…
Dr. Diana
Dear Doctor Diana,
Thank you for answering my question. My mother suffered all her life from, in her words– a dizzy head. I occasionally have that now as well. I also have flat feet and a slight curvature of the spine. My sister who had the stroke has the beautiful pale skin and of course MS. She also had many ear infections as a child which have left her with a considerable hearing loss. She has an unusual mouth; I am not sure what is meant by a high palate. Notice I am using the present tense to describe my sister. We didn’t lose her. She was very disabled by her stroke, but she is recovering slowly. Thanks again. I do enjoy your videos.
Theresa
I have POTS secondary to EDS & now they think I may have MS due to these new symptoms I’m having. Is this possible?
yes that is possible. You should check out Prettyill.com. There is a lot of information on that site regarding the possible tie in between those conditions. I would highly recommend downloading Dr. Diana’s Theory there as well.
Dear Dr. Diana, I can’t tell you how thrilled I am to find this info you have discovered. For the past couple years, we have watched my beautiful 28 year old daughter ,wither away before our eyes. She has seen countless Dr’s , eventually going to Mayo clinic in phoenix. POTS was diagnosed there but she is continually worse,after reading the Driscoll Theory,I am convinced she must have EDS as does a near cousine. This was not mentioned at Mayo however . She is now being tested at her PCP in Topeka , Ks. He is now checking into the Driscoll Theory . Knowing how sick my daughter is,I have no idea how you have been do suck and able to do all this research. You,my dear, are definitely a gift from God. Will keep u posted. Love to u and yours. Judy C