The Driscoll Theory and how it might help us…

I have been meaning to post about the Driscoll Theory for a while now and just haven’t gotten around to it. However, I think it could make a huge difference in my daughter’s life and the lives of others, so I feel led to get the word out.

Dr. Diana Driscoll is an optometrist who also suffers from EDS and POTS (and other junk). She has used her medical knowledge to piece together information about her own condition (and her childrens’) and has shared that with the rest of us. I will attempt to summarize her theory, as I understand it, but I encourage everyone to go to Dr. Diana’s web site and check it out for yourself. (Go to the articles and handouts section of her website and download the article. You can also watch a video where she explains part of the theory in person.)

Anyway, many people with EDS also have ugly neurological symptoms – headaches, dizziness, fainting, tachydardia, light and sound sensitivity, nausea, fatigue, brain fog, aphasia, blurred vision, anxiety, and on and on. Many of those symptoms are POTS symptoms. Many of those symptoms are Cranial instability and Cranial settling symptoms. Also, there are some EDS patients who develop MS, strangely enough.

POTS is experienced by about 1/2 of EDS patients -in fact, orthostatic intolerance is a minor diagnostic criteria for diagnosing EDS. The theory is that the blood vessels are ‘floppy’ due to the defective connective tissue and aren’t able to act on the brain’s signals, resulting in orthostatic intolerance. That assumption could be true in some cases. However, Cranial Instability symptoms definitely overlap with POTS symptoms – when the brain stem is being ‘squished’ the body just doesn’t work quite right.

In the last 10 years, the Chiari Institute has discovered a definite link between EDS patients and Chiari/ Cranial Instability/ Tethered Cord, explaining many of the neurological/POTS symptoms EDS patients experience. In fact, the Chiari Institute automatically screens their patients for EDS now. Instead of being ‘impossible’ to have both EDS and Chiari (there technically is a 1 in 25 million chance of having both), the stats now tell us that 1 out of 15 Chiari patients also have EDS. This is because a Chiari Malformation can be acquired, not just congenital, through cranial instability, a tethered cord pulling the brain down, lumbar punctures, etc.  The processes created by defective collagen can result in a Chiari Malformation or, at the very least, crowding of the hind brain which result in the Cerebrospinal fluid being blocked, causing a plethora of symptoms. This blockage of CSF flow is believe to be the main culprit for all of the symptoms experienced.

The treatment for POTS is to drink lots of water, increase salt intake and  medication if the home remedies don’t work. POTS symptoms are extremely debilitating and don’t always respond to treatment, particularly in EDS patients.The treatment for Cranial Instability is cervical fusion. The treatment for Chiari is decompression surgery. Unfortunately, these surgeries often do not have ideal outcomes for EDS patients – EDS folks are always more complicated and often get only temporary relief from these serious surgeries.

This is where the Driscoll Theory comes in. Basically, she has found a correlation between low levels of high intercranial pressure and EDS patients who eventually develop POTS. The thought is that CSF builds up above the brain and the pressure actually pushes the brain down, creating the POTS and Chiari symptoms, in general making the person feel horrible and wreaking havoc in their lives. This CSF blockage just gets worse over time and will eventually result in brain damage – or even MS. Lumbar punctures would only make the symptoms worse. Decompression surgery and even fusion would only make it worse.

The awesome news is that a simple medication called Diamox can immediately start improving the symptoms. It is a mild diurectic that reduces the intercranial pressure and lets the brain float properly. The poor results EDS patients often get with the neurosurgeries I mentioned above can easily be explained by the Driscoll Theory – it could radically change the course of treatment for EDS patients experiencing these symptoms. Dr. Diana’s initial study found a connection between EDS patients who have this symptomology and who had externally communicated hydrocephalus as infants. This has traditionally been considered a benign condition and not investigated but it could be the clue to intervening earlier in these childrens’ lives – before they experience such debilitating symptoms. She is doing an additional study to further investigate this connection.

This new information gives me so much hope that we can avoid surgery for my daughter; I can’t say how excited I am by this prospect. Trying not to get too excited but I now actually have hope – I really don’t love the idea of my baby having several neurosurgeries. I really, really don’t love the idea of those surgeries failing and doing nothing to help her symptoms! I was resigned to surgery, but now I have hope that it won’t be necessary and, if it is, we can only do what is absolutely necessary instead of blindly going forward.

Now, I know I am vastly oversimplifying this theory – there are components I didn’t even mention. It also includes Mast Cell Disorder and CCSVI and other stuff that complicate EDS. I have picked out the parts that apply to us the most. I really don’t know about CCSVI in regards to my daughter; my gut tells me no. I am mostly convinced at this point that she could have Mast Cell Disorder. (Mast Cells are created in the connective tissue. That right there is enough to convince me!) That treatment is quite simple as well – zyrtec or zantac – so I will be investigating that option for my daughter. There is information on the EDNF website about a study regarding EDS, POTS, and Mast Cell Disorder, so this is an amazing time for EDS research!

So, with the Driscoll Theory in hand, we will go to the Headache Clinic at Cincinnati Children’s and politely ask if we can try Diamox to see if it helps. I figure it is a quite benign medication, so, if she is going to be put on any meds, why not Diamox? Something that could help us avoid surgery all together or even temporarily would be amazing. Heck, I will even take something that just makes Em’s headache better for a few minutes. She has no life right now due to her headache and the thought of a simple little pill restoring her life, well, that would be an absolute miracle!

I encourage anyone with EDS to go to Dr. Diana’s web site and check out this theory and consider if it applies to them. I don’t think Diamox would make any difference for someone with ‘regular’ POTS but for EDSers, it is definitely worth thinking about.

Click here to read about our success with the Driscoll Theory: The Driscoll Theory and how it helped us…