Three particular informational sources have played a key role in making sense of disparate symptoms:
The Driscoll Theory by Dr. Diana Driscoll. Although primarily focused on Ehlers-Danlos/POTS patients and the particular Intracranial Pressure Dysregulatory Disorder these patients often experience – Intracranial Hypotension – especially her observations about the carotid triangle, vagus tone, and cerebrospinal pooling/blockage due to “brain sag”.
Adrenal Fatigue Syndrome by endocrinologist Dr. Michael Lam, MD and Dorine Lam, RD, MS, MPH. A well written book describing endocrine system interactions as a result of long term, high level stress, how that system begins to become dysregulated, and the hormonal consequences thereof, as well as approaches in dealing them.
Multiple Research Papers that have documented the presence of hormonal receptors in the cells that produce CSF. In a word, many of these hormones become dysregulated in Adrenal Fatigue/Failure. Each in its unique way contributes to increases in blood pressure, CSF production – or, as in the case of aldosterone – is responsible for both. This means increased blood pressure & therefore increased Cerebrovascular Dynamics as well as increased CSF production. The perfect storm for development of elevated ICP, and if not controlled, an ICPDD in patients with predisposing factors. Some receptors are estrogen/progesterone sensitive, possibly explaining why patients are more commonly female. Further, looking back to Dr. Lam and Dorine Lam’s body of work, it will be interesting to compare the upswing in ICPDD diagnosis with the increase in estrogen-like compounds in everything from food supply to dental fillings.
All these fell in my lap within a two week period in December 2018 while I was hunkeringS down waiting for a shunt revision, and to my surprise, they did not lead me to what I was looking for: why I developed IIH.
Well, sort of.
They actually led me to a conclusion that I cannot emphasize enough, hence the big letters here:
“The Secondary Acquired Adult Onset form of the “disorder” known as IIH is actually a complex symptom of a dysregulated endocrine system, including “Adrenal Fatigue”, which is most commonly caused by chronic stress. Therefore, I name CHRONIC STRESS to be the main cause of altered/elevated ICP and the onset of ICPDDs, and the ultimate reason I developed an ICPDD.”
Shuntwhisperer’s Neuroendocrine Theory of IIH, Dec 2108
But wait, there’s more:
“Successful treatment of ICPDDs must necessarily involve endocrine function AND CAPACITY* before any ICPDD can be successfully treated. To treat only the ICPDD is treating only the symptom; without lifestyle/dietary changes and endocrine support can possibly condemn a patient to unnecessary prolongation of their ICPDD.”
Shuntwhisperer Neuroendocrine Theory of IIH Corollary #1 Jan 2019
And just when you didn’t think it could not get ANY better:
“Elevated ICPs cause deformation and damage to key portions of the brain controlling the endocrine regulation, especially the HPA Axis. The HPA axis must be well regulated to maintain ICP equilibrium. THEREFORE: TREATMENT OF ICPDDS WITHOUT TREATMENT OF ASSOCIATED ENDOCRINE DYSREGULATION WILL BE SUBOPTIMAL.”
Shuntwhisperer’s Neuroendocrine Theory of IIH Corollary #2 Jan 2019
In other words, endocrine dysregulation that leads to an ICPDD, left untreated, can cause the ICPDD to persist, and more corollaries are coming.
But don’t take my word for it.
Take an expert’s. I swear, I did not rip this off, but Dr. Lam’s book led me to the same conclusion:
Welcome to The Shunt Whisperer. I’m a 57 year old professional forced into retirement and disability as the result of a sports injury over 10 years ago. I was treated with a VP shunt in May 2017 as treatment for an Intracranial Pressure Spectrum Disorder (ICPSD). In my case, it the initial diagnosis was Idiopathic Intracranial Hypertension, the result of a neck injury. At least that’s when the symptoms that seem to be relieved by the shunt appeared. This purpose of this site is to tell the story of what having a shunt has been like, insights I’ve had as a shunt patient with medical training, and where I think shunt treatment needs to go. I want to help others understand their condition better and hopefully live a better quality of life. Having a hole in your head and a tube in your brain isn’t much help if you can’t get out of bed or engage in activities that bring you satisfaction.
Nothing herein is intended to be critical. ICPSDs have only recently been recognized as real, explaining a handful of conditions that have confounded medicine for over 20 years. The reality is that right now, there are only a handful of specialists, chiefly neurosurgeons and neurologists, at a few centers around the nation, that are willing to step in and treat this condition.
The reality of ICPSD treatment is this: it is in the early stages. Methods and devices are evolving to meet the need of an adult population with an ICPSD. Basically, diagnosis of an ICPSD means too much blood and/or cerebrospinal fluid is being retained in our central nervous system (brain and spinal cord). Excess fluid leads to excess pressure which can damage the brain directly including the optic nerves/auditory nerves, as well as stress parts of the brain that control our bodies’ functions including blood pressure, body temperature, weight, sleep cycles, and others, and is linked to conditions such as fibromyalgia and dementias.
My experience thus far has been the equivalent of Alice falling down the Rabbit Hole. I’m told I fall in what is believed to be 20% of patients who develop secondary symptoms after shunting. The experience has not been without positive results, however, including a significant drop in a severe and debilitating chronic “fibromyalgia” pain, as well as an improvement in my physical function. Understand that I have been dealing with symptoms that were relieved with the shunt in March 2017 since a neck injury in mid 2007. There is no doubt the shunt helped me, no doubt it was the best option at the time. It hasn’t been perfect, but after six months of living with it, I believe I know a few things that can help.
It’s my hope that telling my story helps somebody else be more informed and less surprised/confused by their ICPSD and it’s treatment. Don’t use this information to make major health decisions without consultation with your physician. It’s just here to give a little more understanding into this condition and treatment from someone who has and is currently undergoing it. I have a fear that if I don’t put this information out there, it might not get to somebody who needs it. It takes a lot of my available energy to do this; as such, it may take a while between additions and updates.
*soft tissue tumors can also occupy space inside the skull and potentially raise ICP but are considered a separate causative mechanism from those that are considered in ICPDDs
The cause of these imbalances can be either genetic/developmental (primary) or related to trauma (secondary). They are closely interlinked and may both be present to a certain degree.
One example of CSF Imbalance would be non-communicating hydrocephalus. CSF is produced from arterial blood in small hollow areas of the brain called ventricles. Ventricles are normally interconnected with one another and the space around the brain. CSF circulates through these areas passively due to the pulsation of blood vessels and also likely movement of the body. If one or more of these ventricles does not communicate with the rest of the system, the CSF it produces accumulates and displaces the brain outward.
Another exmaple of CSF Imbalance illustrates the interrelationship between CSF and Blood dynamics: obstructed veins may not absorb enough CSF out of the skull to prevent a net accumulation of CSF, as well as leading to this example of:
Cerebrovascular Dynamic Imbalance: would be Chronic Cerebrovascular Venous Insufficiency, or CCVVI. In simple terms, damage or constriction (stenosis) of one or more of the veins that drains blood from the brain compromises the ability of blood to leave the brain. At a certain critical level of blood flow and pressure, blood begins to accumulate in the brain as it is pumped through arteries at a greater volume than it can drain. This accumulation of blood causes the thin-walled veins to swell, which in turn pushes on brain tissue. Brain tissue may become displaced as a result, being pushed into areas occupied by CSF. Since an intact skull is rigid and does not expand, the pressure of the CSF becomes pressurized (Newtons’s Third Law) while simultaneously trapping brain tissue between a vise of swelling veins. As focal areas of brain tissue that control specific physiologic functions become stressed, their functions become altered. This pressure on brain tissue is likely the cause of symptoms associated with IIH.
Communication over the internet can be challenging. Not like talking face to face; it’s easy to misunderstand what is being said, or why somebody is saying it. That said, I want to lead with this: ShuntWhisperer is just my story for about my ICPDD for whatever it’s worth. ICPDDs and their treatment are poorly understood, doctors do the best they can with what is available now. I think I’ve stumbled on to something…interesting… for no other reason than weird coincidence, and I don’t believe in coincidence. Maybe this story it will help somebody, maybe it will spark conversation or thought, but it is absolutely, positively not intended to point fingers or place blame. Despite the challenges of the last year, I would not change anything. I’m just trying to make the most of it for the possible benefit of anyone who finds my experience useful in their journey, both patient and doctor. I try to keep this as light as possible; any humor is just that, never intended to be irreverent. The fact is I’m still here to type this because of the efforts of my neurosurgeon and the Grace of God.
Now, we return to our irregularly unscheduled post:
Is it seriously this “simple”?
So, last year, after I received my shunt, I had a pretty wild ride to put things mildly. Despite all of it, I’m seriously grateful for everything. There seem to be so many unknowns in ICPDDs; all through my experience I’ve tried to learn as much as possible about what is going on in my head and pass that information along. It seems invaluable to me, and I pass it along not for my benefit, but in the hopes that it helps somebody else, and maybe fills in a small part of the overall puzzle.
My Biggest Issue: wild swings in my ICP, with a distinct trend towards overdrainage. The higher the shunt was adjusted, the worse it got. I never felt like I had enough CSF/ICP/MysteryFluid in my head. Ever.
Until November, when thing began to change…
I was using the Whisperer Method to monitor blood flow into my head. The numbers I got were deadly accurate both in how they related to my SOTD (Symptomology Of The Day). The Cerebral Perfusion Coefficient (a figure that gave me a relative idea of how much blood was flowing into my brain along with the static pressure in the blood vessels) numbers stayed within a fixed range. On the low end of the scale I considered myself “underperfused”, that is, not enough blood going into my brain to make enough CSF to keep up with normal losses along with…some other loss of CSF, someplace. I thought it might be through my shunt, but I don’t think so now. On the high side of the scale I felt like my head was going to burst, my shunt felt physically swollen, but perversely, I still felt as if I had no CSF in my head. The “no CSF” feeling is one that I was well accustomed to as it was the same set of overdrainage symptoms for which I was hospitalized in June 2017, 3 months after getting my shunt.
The Deadly Accuracy of the numbers and symptom correlation held up until the end of November, when I noticed numbers and symptoms starting to diverge a little. Then a bit more. This divergence continued up until Christmas day, the last “BAD” day I had according to the old pattern. Suddenly the numbers and symptoms no longer seemed to match. I had no answer.
From Christmas on, suddenly the wild instability rapidly diminished. Extremes of high and low ICPs became closer together. No more apparent swelling of my shunt which is
made largely of rigid materials incapable of expansion. It was as if either a blockage in my shunt had been cleared, or a leak in my head had been closed off. Curiouser (TheWhisperers Unabridged Dictionary), the Cerebral Perfusion Numbers started climbing to new highs, up to 20% higher, without the usual symptoms high ICP symptoms of feeling like my head was going to explode. January into March I actually felt pretty stable, still with bad days, but the best I had felt since getting the shunt. This left me scratching my head, and that’s where I found The Answer To It All.
I’ve never been accused of being the sharpest knife or having a full picnic basket, etc. A little slow at times. So, a few days ago, I’m rubbing my head where the shunt catheter goes into my skull. A slightly larger than dime sized hole created in my skull to allow the catheter to be poked into the hollow area in my brain. No jokes about how easy it should be to hit a hollow area in my brain, please. I’m delicate…
The skin over this hole, a.k.a. “craniotomy”, is slightly indented, normal for a surgical wound. What I noticed is that I no longer noticed it anymore, because it was no longer changingshape. All last year it changed contour with my ICP. Sometimes it was like it was being sucked/pressed into my skull, other times, it was almost as if it was being pushed out under conditions of high ICP. It had become a sign I used to make note of in recording what was going on with my disorder, but now it was no longer changing….
Then, slowly, reality crept up on me. I like to blame the fact that I have a tube in my brain for all these issues of slow comprehension; rather convenient really. Never mind the fact I was slow before I got the shunt, but now I can point to it and say “See? I have a tube in my brain. I’m slow for a reason”, which sounds infinitely better than “I’ve always been slow.”
It was about the hole in my skull; a hole in bone. A dime-ish sized hole was made in my skull, exposing the dura underneath.
The dura is carefully opened, and a 3D guidance technique, a catheter over a needle, sort of like an IV catheter for the brain, was then inserted through the into the immense hollow area in my brain to drain CSF, after which the dura was sutured around the catheter and, my scalp repositioned and stapled shut. Joking aside, I have always appreciated the precision of this particular aspect of the procedure. I had employed guidance techniques generated from 3D scans to place dental implants with extreme precision; the shunt cath placement is another level of precision entirely.
I joke that placing a shunt is not unlike poking a straw into the foil hole of a juice box. No adult can put a straw in a juice box as well as a kid, who with a deft poke can stick that straw through the foil like a kung-fu master, creating an almost perfect hole to match the straw. Perfect enough to suck juice through the straw, but we all know what happens if you squeeze the box with the straw plugged: juice seeps out around the straw, and the ratio of juice-leakage-to-squeeze is directly dependent on the cost of the shirt that is being stained, and inversely dependent on the time since same shirt was laundered.
Doubling factor if dry cleaning is involved (see: Whisperer’s Laws of Life 301).
If I have failed in my wordsmithing to create a clear mental image of this condition in your head, let me invite you to get a juice box, a bag of Capri Sun, a Corona Beer Pouch
(see: Whisperer’s Intellectual Property Item 526), poke the straw through the foil (or get a child to do it for you, except for the Beer Pouch, of course), plug the end of the straw and squeeze the box/bag of juice. See where it leaks – between the foil and the straw, right where the straw goes through the foil. Except in my case, it was not juice, but CSF, leaking around the catheter where it penetrated the dura. There is no magic seal here, and if ICP exceeds the pressure/flow settings of the shunt system, here is a place where CSF can leak.
I’ve been crafting, honing, polishing this for 3 days now, and each time I think about it, I keep wondering how many other shunt patients this might be a factor for, with any type of shunt, VP or LP. The good news is there is a way to mitigate this leakage, or at least reduce the period of time it occurs through a simple surgical method to encourage faster growth of bone into the craniotomy.
As a dentist in my Previous Life, I was intimately familiar with the tissue of Bone. Drilled a lot of holes in bone, grafted a lot of bone, moved bone from one place to another, bone, bone, bone, all day long, bone. But, it paid the bills. I know about bone in the human body, how it reacts to injury, how it heals – or doesn’t. Without the fancy “bone bandage” known as a guided tissue membrane, bone may never fill in a defect completely, and if it does, it takes much longer. A hole in the skull heals from the sides in and from the bottom up, over top of the dura, which by the way makes a dandy guided tissue membrane, at least on the brain side. The issue here is the scalp tissue is a source of soft tissue that fills the hole in the skull well before bone has a chance.
Short story: I didn’t get the fancy bandage for whatever reason. I got a hole “stabbed” (actually carefully incised and then sutured, but for the sake of sensationalism…) in my dura (I wonder now if a child was brought into the operating room to do that part), a catheter inserted into my brain (with great care an precision), the latter hooked up to the shunt valve, and my scalp flopped back into place and stapled as the neurosurgeon asked the charge nurse if he still had time to make the late morning round of golf while the kid that poked the catheter into my brain sucked on a juice box under his surgical mask while sitting on a stool in the corner. In all seriousness, I can’t find a thing that says that shunt craniotomies should be grafted over; maybe they should be.
Bottom Line: March 2017 until I’m guessing November-ish 2017, I had a CSF leak that mucked up the attempts to control my ICP. Ironically, whenever the shunt was “turned up” to a higher pressure setting, it made the problem worse. Nothing sealed the dura to the tube. CSF seeped between the tube and the dura, under the surgical flap, finding its way under my scalp, along the tube and around the shunt body, and likely farther. In surgery the phenomenon is called tunneling, and the leakage of CSF around the catheter created what is known as a pseudomeningocele. Finally in November a stage of healing of the craniotomy was reached that mitigated significant leaks. The timing is right for the size of the hole. However, since to guided tissue membrane/Bone Bandage was used, there is also a good chance the hole isn’t filled with bone, but with a thick scar tissue that may eventually turn to bone. Whatever is in the hole, my head isn’t leaking from an unwanted place anymore.
Google the term “Intracranial Hypotension”(not “hypertension”) . It’s a real thing. Usually caused by an unwanted, unpredicted leak in the dura. I believe this was a large part of the reason for my “wild ride” I went on after my shunt last year. My concern now is who else may be suffering from it or may potentially suffer from it; possibly by posting this, a surgeon may be persuaded to add 10 minutes to the procedure and graft a VP shunt: I don’t know about LP shunts.
This theory also explains a new phenomenon: the return in the last six weeks of my pre-shunt symptoms that were associated with high ICPs, the reason I got the shunt in the first place. Not as bad yet, but getting there. Fibro pain coming back, other bad stuff, BUT: there is a clear silver lining. Regaining proper ICP balance may be as simple as turning my shunt down a notch. As I write this I’m awaiting a CT to check my meninges, and I’ll post the results here.
(edit to add 5/22/18: those symptoms are definitely back. Time to check in with the neurosurgeon.)
The silver lining here is that I’ve gotten this far and have avoided at least one procedure that would likely have made things worse. Early on last August when I was still having overdrainage symptoms despite having a shunt set at 200mm H2O PLUS a 250mm shunt assist, my neurosurgeon graciously offered to revise me to a higher pressure shunt valve. I declined that offer because my personal situation was not amenable to having the surgery at that time, and I believed I could hold on until another option became available. In retrospect, that higher pressure shunt would have likely made me worse. I consulted with other neurosurgeons for their opinions, but I was never satisfied with their explanations based on nothing better than “gut feeling” and the personal records I had kept. Now I believe I have that explanation, and I’m hopeful that a simple pressure adjustment in my shunt will restore some or all of the reduction in fibro pain, stop the return of the exercise intolerance which is again threatening to leave me bedridden for days after engaging in any physical exertion, and the sleep cycle disorders/insomnia which is back. It is also my hope that this story helps somebody else, or sparks a thought process somewhere in the minds of doctors wrestling with a disorder that has so many unknowns attached. I was told by one surgeon I was the “Unicorn” of this disorder (!); if so, at least now I know from whenst my horn grew…
Bottom Line: without a good seal, CSF can and does leak around shunt catheters. Pseudomeningoceles are a known postoperative possibility with shunts, but their frequency of occurrence is controversial. Secret or not, the answer may be a few added minutes to a shunt placement and a few hundred bucks for a bone bandage. The bandage keeps soft tissue from the skin flap out of the craniotomy, encouraging more rapid and predictable bone healing around the shunt catheter. Bone grafting material made from the patient’s own blood (Platelet Rich Fibrin), can be used to further seal the craniotomy before the bandage is placed and everything sewn/stapled shunt. This is just an opinion I toss out there as a Disabled Dentist With a Hole In My Head.
It is my fervent hope that this clicks with ShuntWhisperer fans (both of you), maybe some docs, and that maybe, just maybe, addressing this small issue will make the post treatment course of other ICPDD patients and VP shunt recipients in general better. As always, prayers to all. Questions can now be sent via email to email@example.com, and I am have setting a Twitter Feed specifically for ShuntWhisperer.
The Shunt Whisperer
May 19, 2018
Missing my wife in my life more and more every day: I love you, baby.
I received a question from Yvette on ShuntWhisperer:
“Does moving to a sunnier, dry climate at higher elevation help? Or maybe to the coast to help alleviate the barometric pressure? Or is there no escape?”
Short answer: Yes. Heck yes, there is escape. I started doing something similar last summer, moving to higher altitudes/lower barometric pressures to escape the oppressive heat/humidity of Central Virginia, conditions that were robbing of my ability to function. I continue to do so as needed, finding that driving to and staying in an areas a mere 1000 ft higher with a barometric pressure 40 or more millibars lower changed my condition like flipping a switch. It was uncanny.
Let me elaborate on why certain areas might be beneficial for ICPDD sufferers. Most of us are puppets to changes in barometric pressure that accompany normal weather changes. Any neurosurgeon/neurologist that tells you that barometric pressure cannot possibly affect ICP is ignorant in a a dangerous fashion, IMHO. Barometric pressure changes are very significant; as an example, here in Virginia, the barometer changes 20-30 mb (millibars) every 7-10 days as weather fronts pass through. Looking at the significance of that change, we find that ONE millibar is the equivalent of TEN mm of water; thus, a change of 30 millibars exerts an increase on tissues exposed to air pressure of 300 mm H20. Now, look at the area of tissues exposed to this pressure: skin and the lining of the lungs are the chief areas. The average person has a surface are of skin of 15-20 square feet. The lungs, however, have a huge surface area of tissue, 80-100 square YARDS, translating to 720-900 square feet; add in the surface area of the skin, and we can safely assume an affected body surface area of up to 1000 square feet. That barometric pressure change of 300mm water per square inch from a mere weather phenomenon converts to a “mere” 0.428 lb/square inch, or 61.6 lb/square foot (!). Apply that to the area of the skin and lungs, and this change in weather causes a change in pressure on skin and lungs of…1000 square feet of tissue filled with blood vessels being pressed against the body with a force of 0.428 lb/square inch, 61.6 total lbs/square foot, and:
61,600 lbs of total pressure exerted on the 3000 square feet of skin and lungs. At the high end. Variable of skin area to body volume enter, but this is significant. It raises intra-abdominal and intra-thoracic pressures, condition known to decrease vascular drainiage from the skull; ICP increases and Starling Resistor functions become factors…all of it bad for ICPDD patients.
Ultimately, I believe these changes affect the dynamics of blood sequestration inside long bones and the skull. Blood and other fluids are pushed from the vessel in the skin and lungs and end up trapped in areas where atmospheric pressure doesn’t reach. Teeth as well, if you want to be technically accurate. Is this the mechanism that affects our symptoms? Not certain, but it is a good argument, because almost every ICPDD patient relates that their ICP varies directly with changes in barometric pressure. Shunted patients have a different sent of symptoms than non-shunted patients; increases in barometric pressure cause increased CSF flow out of my shunt.
Now let’s throw in another wrinkle: atmospheric infrasound. Severe weather systems produce very low frequency pressure waves that can travel hundreds of miles at hundreds of miles an hour. Infrasound is known to be deleterious to physical and mental human physiology. Last summer, after I got my shunt, I had just begun to make the connection between barometric pressure and my symptoms, but I also noticed something else: there were times when I would feel badly when there was a severe storm approaching two to three days out. This was well before any changes in the barometer associated with these fronts occurred. I remarked to Trina there was something about these storms, especially those with severe thunderstorms and tornadoes that was beating me down, and it wasn’t barometric pressure or heat or anything I could put finger on. It was worse after my shunt for some reason. Then I happened across and article on Atmospheric Infrasound and Associated Effects on Human Physiology. The study set up elaborate devices to measure “sound” too low in frequency to hear, but recognized to affect blood flow, cognition, heart rhythm – pretty much everything in the human body to one degree or another. Effects were chiefly psychologic, creating feelings of despair, depression, hallucinations, and more. Changes in heart rhythm were notedFurther, athletic and cognitive performance fell off dramatically as this infrasound became stronger. I believed this was the source of my peculiar response to storms two days away.
Often, there was always a storm two days away. But it was the bad ones carrying thunderstorms and tornadic activity that beat me down the worst.
Recently I moved to a location in the isolation of the mountains. Paradise, to be sure, but at the elevation and location, a lot of winds. Stronger winds tended to make me feel poorly. Then, in mid-April, the strangest, strongest confluence of weather factors came together to leave me so sick and weak I couldn’t get out of the chair I was sitting in. I remember strapping on my CPAP mask and turning on my oxygen generator at 5:30 pm. I woke up at 7 am. The storm system had dumped 6 inches of rain in our area and I’m told brought epic lighting. I don’t remember a bit of it, and I love a good lightning show. My house has a metal roof, and I don’t remember any sound of rain on the tin roof. The storm spawned several tornadoes to the south. I was essentially passed out in my chair with an oxygen mask on. I managed to screenshot the system before I passed out:
Back to Yvette’s question about moving to an area that might be more suitable to persons susceptible to mere changes in the weather. The answer is a definite yes, and IMHO probably and worthy of taking a couple of weeks vacation to such a place. Such areas in the United States are going to be confined to areas west of what is known as the “tornado line”, where cool dry air moving east from the Rocky Mountains collides with warm moist air being pushed up out of the Gulf of Mexico, spawning thunderstorms of epic proportions and of course, tornados. I had researched where the most stable weather areas in the states were, and the southwest fit the bill perfectly, most especially Arizona with its Medical Marijuana program. I lost Trina before we ever had the chance to go to Concho for a few weeks to see if improvements in our symptoms would make the move and change in lifestyle worthwhile. New Mexico and Nevada also seem to be areas of opportunity, with changes in barometric pressure a mere 5-10 mb every two weeks as opposed to the average 20-30 every 7-10 day cycle we were seeing here in Virginia. As far as coastal areas are concerned, I’d say it depends on whether a person can tolerate the higher barometric pressures seen at sea level, and if the coastal area is in a stable area with regards to weather. In short, ICPDD patients all have different phsyiologies; shunts/stents further complicate the picture. As far as I’m concerned, I’m ready to go live anyplace that works, even if it means being a migrant nomad, sleeping on a cot in the back of machine shop, pushing a broom and emptying trash in exchange for a degree of improved physical comfort.
My ICPDD has taken everything from me except for my life. Some days I sincerely wonder if that is the day that something pops/kinks/blocks/clusterforks and I’m off to maybe see my sweetheart again. Until then, I’ll continue putting my experience and research here. Right now I’m extremely interested in infrasound, and a quirk in its properties that would allow merely wearing headphones with music to cancel it out. Not sure yet, but right now a mix of Jack White/Lynyrd Skynyrd/Gangstagrass beat in my tinnitus ridden ears, and I feel as good as I usually feel.
I’m going to head to Eastern AZ later this summer once I work out the AirBnB and how to get there on the cheap. Maybe FedEx myself in a large box. At any rate, Yvette, Yes, moving to a different climate, probably dryer, cooler, higher in elevation, with boring weather patterns, does seem to help ICPDD patients. Dr. Kenneth Liu at Penn State Neurologic Services in Hershey, PA, told me at an appointment earlier this week that some of his patients have relocated to AZ for its climate, with satisfactory result.
More will come. The more I research the topic of infrasound, the more convinced I become of it’s significance to ICPDD patients. I told another contact I was at a point where I need to start a MindMap, as I believe I may have found another piece to the puzzle of ICPDDs vs. Weather, a piece called density altitude. All this is little more that intuition and gut feeling trying it interpret what I’m personally experiencing. In the meantime, I’d like to ask for prayers as I leave the house where Trina and I lived for a different place, picking through the pieces of our life together cut short by her ICPDD. Prayers and comfort to all, and prayers to our doctors for insight, compassion, and vision as they seek to treat a condition that seems completely elusive and not as rare as it represented to be.
In service to God, in memory of Trina; may these words help others.
You’re always in my heart, sweetheart. I miss you every moment.
If you are the caregiver, spouse, partner, or friend of someone who has been diagnosed with what is currently referred to as “Intracranial Hypertension” or “Pseudotumor Cerebri”, it is important that you understand what this disorder is and the effects it may cause in the patient.
I speak as both an IIH patient with a VP shunt and the caregiver to my wife. Yes, we both were diagnosed with IIH. We both followed what I refer to as the Neuroendocrine Theory of IIH; tragically, before she could be treated, she succumbed to they physiologic and psychologic effects of her disorder despite my pleading with physicians to help her. I have experienced everything I am conveying here with complete honesty and sincerity.
Forget what you think you know about “Intracranial Hypertension”. “IIH” patients are very ill. Cancer Ill, in my opinion . We’re talking about a disorder that damages the physical structure of the brain. Very, very serious. Don’t let anyone tell you otherwise.
The major problem faced by “IIH” patients: it just doesn’t show on the outside. No bloody bones protruding, no disease process that is well understood and routinely treated. Just the person telling you “I don’t feel well!” and acting strangely, which may alienates you and others. Most “Normals” don’t know how to take this, or pull away from what is simply a very ill person who is hurting – or worse. This is a subjective disorder surrounded by controversy in the medical field. In late 2017, a neurosurgeon offered this opinion: “if you had 100 neurosurgeons/neurologists, 50 of them would deny this condition exists. Of the remaining 40 would acknowledge there was a problem but offer no treatment. Of the remaining 10, 7 or 8 would treat the disorder with medications and lumbar punctures; only 2 of the original 100 would offer any direct (surgical) intervention. Plus, nobody is sure of what causes the disorder to develop.
Hopefully this Guide gives you an idea of what is really going on.
Start with the term “Intracranial Hypertension”; it’s misleading. This is not “hypertension” like “high blood pressure”. This is increased pressure of the two fluids, blood and cerebrospinal fluid (CSF) inside the skull. A more accurate term I use is Intracranial Pressure Dysregulation Disorder (ICPDD). The basic outline:
The brain and spinal cord are encased in a tissue sac called the dura. They are further encased and protected in a hard shell of the skull (cranium) and the spine. There is a limited amount of space for the contents of the brain, spinal cord, and two fluids; this space is essentially watertight and airtight, with minimal extra room for anything else. ICPDD patients experience a disruption in the balance of fluids flowing in and out of the brain/dura. The patient’s normal physiology that regulates this balance is disrupted. The end result is an abnormal accumulation of either or both fluids inside the very confined space of the skull and spine. This results in increased pressure in that very confined space. The brain is literally squeezed in a liquid vise in the confines of the skull. Evidence of this is seen when the optic nerve(s) are visibly damaged by fluid pressure, threatening eyesight. Other patients have deformation and damage to their pituitary gland, potentially crippling a critical endocrine system and leading to pain, fatigue, and intolerance to exercise and exertion.
To say that pressure on the brain is a problem is understatement. The brain is an organic computer with the consistency of firm tofu. It’s mostly fat and water. It is divided into areas that control nearly all of the functions of a person’s body such as temperature regulation, energy, blood pressure, and thyroid function to name only a few. Additionally, areas of the brain control memory, mood, thinking ability (cognitive function), and sleep cycles. Increased I pressure on these areas understandably causes alteration of the function(s) those areas control; yet, in my experience, the response of physicians has been to tell me I’m “Hypervigilant” (pay too much attention to the symptoms of my disorder; these symptoms frequently keep me bedridden, have cost me my livelihood, financial stability, by wife…so, yeah, I sorta pay attention to them). Other labels are “psychosomatic”, “Munchausen-esque”, and “hysterical”, the irony of which would be hysterical if it wasn’t so tragic. All while telling me there is little understanding of “IIH”. Probably the most epic experience was when I was seeking emergent care from a neurologist; as I vacillated between misery an agony on one side of the desk, the neurologist read my MRI report and said “well, your MRI doesn’t show any changes in your brain structure, so I don’t believe your symptoms are due to your Intracranial Hypertension, if you actually have it. Besides, I can’t take your account of your symptoms into consideration because you’re the patient. I have to rely on test results.”
True story. More common than I could have ever believed.
The result in a patient with an ICPDD is a group of symptoms that would be expected to mimic damage to the brain, which is exactly what is happening . This is the basis for the origin of another term for an ICPDD, Pseudotumor Cerebri (PTC): literally, False Brain Tumor. Doctors treating the earliest ICPDD patient noted them to exhibit symptoms that would normally be associated with a brain tumor, but no such tumor can be found on MRI or CT scan). Referring to my experience with the neurologist who told me I couldn’t be sick because my MRI “looked good”: changes in brain structure don’t happen overnight. It takes time, and are preceeded by symptoms of increased intracranial pressure, some listed below. A short list of these possible symptoms include:
Memory Difficulty: searching for words
Cognitive Difficulty: performing simple math, taking longer than normal to process information
Weight Gain (often thought to be the cause of ICPDDs, may actually be a symptom of increased pressure on an area of the brain that controls metabolism
Difficulty walking (Gait Disturbance)
Intolerance to hot/cold
Vision problems due to pressure and/or pressure-related damage to the optic nerve.
Anhedonism, or lack of interest in pleasure activities, including sex
Ringing in the ears (tinnitus)
Insomnia, which if prolonged, causes further difficulties associated with sleep deprivation
Fibromyalgia Pain/Exercise Intolerance
Increased risk of Hemorrhagic Stroke due to increased pressure in the blood vessels of the brain*
Sensitivity to light/Intolerance to light/photophobia*
*These symptoms were suggested by readers to be added to this “short list”; a reminder that symptoms vary among ICPDD patients; more significantly, this is an example of my hope that by sharing our personal understanding of these disorders based on our actual experience as ICPDD patients will advance and improve treatments more quickly. #CrowdHealing (before anyone asks, yeah, the Twitterfeed is coming…)
These are but a few of the more common symptoms There are many more. Try this exercise. Buy a block of firm tofu; Its about the consistency of the brain. Squeeze it, release it. See how much pressure is required to leave a dent. How much is required to make tear in the surface, to deform it beyond its ability to return to original shape. Now imagine each damaged area as controlling a part of your loved one’s body. That is literally what is happening in their skulls as cerebrospinal fluid both expands the brain from within and presses on the brain in the space around it. Blood swells the vessels of the brain and further adds to the pressure that has no release because of the watertight, airtight manner that the Central Nervous System is enclosed.
Further, changes in conditions such as barometric pressure and temperature (aka weather) can cause aggravation of the symptoms. Certain foods or activities can worsen symptoms as well. Each patient has both commonalities and unique individual consideration. Perversely, the medications used to treat increased ICPs can cause some of the symptoms of ICP.
Your loved one will experience fear at being betrayed by their own brain as well. They may look “normal” on the outside, but inside, the most critical organ in their body is being subjected to conditions that can cause them to seem to be a different person. And since the brain is the CPU for the functions of the entire body, their miseries are not confined to “headaches”.
What they need from you is unconditional love. Drop everything else, give them the support they need. I’ve been on both ends, a patient, and a caregiver. The frustration, the fear is paralyzing when you’re the patient wondering what is happening. The tendency is to dismiss the patient because there are no bloody bones sticking out anywhere, but trust me, that would actually be a blessing because it would likely get more attention.
Causes of ICPDDs are controversial, although recently, after 18 months of research, I have a working theory of how I developed IIH. In my case I can pinpoint the day and the event that started mine. Same for my wife. Incidents of trauma to the neck with later aggravation. For others, it can be prolonged use of certain drugs such as birth control or antibiotics. If you’re reading this, you’re likely a male in your early to mid 40s, as the most common ICPDD patients present as female, in their 40s, considered overweight by the medical profession. That weight is often blamed for the disease, but don’t be so easily swayed. Remember that block of tofu? Imagine if you squeezed too hard on the part that controls metabolism…most ladies say their weight “just came on” in a short period of time with no change in their eating or activity habits. Unfortunately the extra weight becomes an issue, and losing the weight helps most patients significatly, but don’t blame the weight or the patient for the disorder.
And after being diagnosed with an ICPDD? Consider this pull quote from this publication:
“…pseudotumor can cause chronic disabling headaches and visual complications. Therapy is sub-optimal, symptomatic, insufficient, and often complicated by side effects…”
Pseudotumor Cerebri and Ciprofloxacin: A Case Report
Treatments for ICPDDs currently consist of drugs that often have aforementioned significant side effects, or international surgeries to relieve pressure on the brain such as shunts or venous stents. All options are difficult and life changing. You need to love the person, support them, understand they can’t control what is happening to them, and are frustrated at not being able to do what the used to do, want to do, or what other people want them to do.
More information can be found here on ShuntWhisperer. Com, as well as the Intracranial Hypertension Research Foundation (IHRFoundation.org), the National Organization of Rare Disorders (RareDiseases.org), and others that can be found under search terms such as “Intracranial Hypertension Research”.
The bottom line is that your loved one is suffering from a disorder that is literally squeezing their brain, the organ that not only controls their bodies, but also houses their personality, the invisible pattern of electrical activity that is them, that is their soul. Put yourself in that position for a moment, or longer, and imagine feeling like an unseen force is pulling the strings and you’re the puppet forced to respond…or not.
As if this isn’t enough of a challenge, the current “Opioid Crisis” is making pain associated with “IIH” an inconvenient symptoms to treat. Some physicians even go so far as to tell patients their pain is no big deal, just live with it. “I’d rather see you in pain than give you a prescription for a narcotic” is a phrase I’ve heard on more than one occasion. What I have found, however, is that the pain created by pressure on the pain centers of the brain responded best to small doses of acetozolamide; maybe this is an answer for someone else reading this.
What to do for a loved one who has suddenly and seemingly inexplicably lost their ability to function, work, be a parent, partner, or engage in leisure activities? Simple: Give them love, unconditional love. Give them space to be sick. Give them support. Give yourself space, find support for yourself. This is a serious disorder, every bit as serious as cancer, but not nearly as well understood, and currently, IMHO, not yet nearly as well managed. Hopefully this will improve in the near future. God’s Blessings to all.
February 3, 2018, revised May 1, 2019
Thinking of you every day, baby. I miss you so much…
ICPDDs affect the lives of patients according to its unique set of symptoms, and how those symptoms apply to each patient. Pain may be a component, but also, so may fatigue, insomnia, mood alteration, memory, cognitive function, and more. Ultimately the ability of a person to live a “normal” life may be impacted, and this tool is intended to better give a value to a “feeling” or sum of different conditions. In my opinion, not enough attention is given to Quality of Life and Functional Capacity. I’ve lived 11 years with the fallout of a severe neck injury, surgery, and IIH, and seen my ability to function vary from being able to almost be normal to barely being able to do laundry and shop for groceries.
This scale was developed to allow a patient to assign a 0-5 value to their impression of the sum of their Quality of Life and Functional Capacity. Each of these is described in the short instruction section below. This Index is best used in a diary that 1) has regular entries 1 to 4 times a day (I use phone apps, of course), and 2) keeps track of a predetermined set of activities, medications, or condition that you notice or suspect to affect your specific condition, along with specific symptoms. Note that you get to designate what you want to keep track of. If you think the price of Bitcoin plays a role in your disorder, record it and chart it along with anything else you feel is important. Better to start with a manageable number of things to record, usually no more than 5 well chosen factors that you know to be an issue in your particular condition, believe to be an issue based on research, or just want to keep track of. Very important to record all of your factors in every entry so that any trends or connections associated with graphing your entries are noticeable and accurate. Be prepared to invest at least two weeks before any hints of a connection are revealed, and a couple of months to confirm any possible relationships.
As an example: I routinely monitor my blood pressure, the barometric pressure, pain level (using an existing standardized scale such as found here), plus notes about any activity I feel is important, but it’s the numbers that make the connections: over time, plotting graphs of hard numbers may show relationships between symptoms and certain conditions. In my case I was able to expose a direct relationship between barometric pressure and my symptoms of Intracranial Pressure, as well as some interesting trends in blood pressure metrics as well. I’ve been at it since 2017 started, beginning with just checking the weather, adding barometric pressure, then blood pressure…it was a learning experience for me, and consequently took me a while before I saw any correlations. In order to get you off to a quicker start, here are some recommendations for variables that you might begin with:
2) Blood Pressure/Pulse/MAP (Mean Arterial Pressure): I use and app called Blood Pressure Companion. Enter your blood pressure and it calculates your MAP (average pressure in your arterial system during one heartbeat)
3) Barometric Pressure: again, an app: Bar-O-Meter, uses a sensor in most smartphones to give the barometric pressure in your exact location.
6) …and last but not least: the Quality of Life/Functional Capacity Index.
Other variables may be food such as caffeine, carbonated beverages, intake of certain medications – its up to you what you put in as long as you have the time to manage the diary well to make the effort bear as much information for you and your doctor as possible. It is my sincere hope that this helps others better understand their disorder and to be able to communicate it as well as possible to their physicians. Prayers for all, without further rambling, I give you the Official ShuntWhisperer Life Index:
Purpose of this Index: This index is intended to give a simple, standardized indication of any impact your disorder is having on your life. A regular diary is kept recording vital signs and environmental factors, and this index is employed to give an indication of the your ability to perform the Basic and Necessary Activities your life requires or that you desire to perform. Over time, it may become possible to determine factors that impact your disorder and make changes/improvements to your treatment approach. It also helps physicians understand how your disorder is impacting you as an individual above and beyond the results of medical tests and examinations. Ultimately the goal is that your data can be pooled with the data of other patients and used to guide improvements in the future treatment and management of your disorder.
“Quality of Life”: The single most important metric in a person’s life. Quality of Life varies between individuals and is defined as how good or bad you feel about how you are able to fulfill activities and ability to live out your life as independently as you desire.
“Functional Capacity” – the ability to perform activities and tasks, Basic or Pleasure, without assistance
“Basic Activities” – activities related to fulfilling your basic needs to live and maintain your living space. These include but are not limited to: obtaining food/shopping; obtaining/maintaining clothing including washing, folding, and storing clothes, regular personal hygiene, and any other activity related to keeping yourself healthy, properly nourished, neatly clothed, clean, a sense of organization, and ability to maintain your living area (meet financial obligations, perform or pay for residence maintenance such as trash disposal, yardwork, housekeeping, and similar), and ability to avail yourself of ancillary services such as medical appointments for general health, dental, vision, and other similar services. Basic Activities fall within a relatively common set of obligations, varying primarily by your individual living area (house vs. apartment vs. farm), your obligation towards any dependents (children, animals, gardening), and other factors of your personal lifestyle.
Pleasure Activites: Activities that bring you enjoyment, pleasure, and relaxation and give you a sense of fulfillment, activities that your perform after you have completed your Basic Activities. Pleasure Activities can include any of this very limited list of examples: physical activity and exercise (tai chi, workout at the gym, sexual activity), pursuit of a desired goal (writing a book, coding/programming) mechanical/artistic activity, visiting friends, taking vacations. Pleasure activities are wide and varied and primarily depend on your own personal interests and desires – “The things that make you tick.”
Index Scale Description
0) You feel no symptoms or affects that you relate to your disorder. You have your complete Functional Capacity, able to perform basic activities ranging from Necessary chores to pleasure activities; your Quality of Life is not affected or reduced.
1) You begin to become aware of the symptoms that you relate to your disorder, but there is minimal to no impact on your Functional Capacity; Quality of life is not significantly affected.
2) Your symptoms require intervention. Intervention may be in the form of medication, momentary periods of rest, or time taken to perform a therapeutic procedure. Your Functional Capacity is impacted to a minimal degree; your Quality of Life is still very good but you begin to have to make choices about which activities, Necessary or Pleasure, are reduced or eliminated.
3) Symptoms require both intervention along with periods of rest. Functional Capacity is reduced up to 25% of normal; Pleasure activities begin to be sacrificed in favor of therapeutic procedures and/or rest periods required to cope with symptoms.
4) Symptoms require intervention along with significant periods of rest or bed rest in addition to therapeutic procedures. Functional activity is reduced by up to 50%; all pleasure activities are sacrificed due to lack of energy, physical limitations, and/or lack of interest.
5) Symptoms require all possible interventions and bedrest. Functional Capacity is reduced up to 100%. Basic activities are significantly impacted and if improvement in Functional Capacity is not possible without prolonged periods of rest, advanced Intervention (may include doctor’s appointment or Emergency Department visit).
Note: in addition to recording a number from the list below in your diary, add a “+” if you feel your symptoms are related to high intracranial pressure, “–“ if you feel the symptoms are related to low intracranial pressure (CSF overdrainage or cerebral underperfusion)*. Symptoms of low ICP include lightheaded/emptyheaded feeling, a headache that is worse when standing and gets better when lying down, possibly sleepiness and lethargy. Symptoms of high ICP include sensation of pressure inside the skull, increased vision disturbance, gait disturbance (difficulty walking).
*”overdrainage and underperfusion” are based on the Cerebrovascular Edema/Venous Outflow Insufficiency Theory of Intracranial Hypertension. Underperfusion refers to a condition where blood flow to the brain is not sufficient to allow adequate Cerebrospinal Fluid (CSF) to keep up with normal absorption by the body and the additional losses through an unnatural CSF leak (this includes a shunt or leak from a lumbar puncture; CSF flow through the shunt is minimal. Overdrainage is excessive leakage through a shunt pathway due to Cerebrovascular Edema as a result of Venous Outflow Insufficiency. As brain tissue swells due to undrained blood backing up in brain vessels, areas containing CSF become pressurized, This includes the subarachnoid space and ventricles of the brain. As CSF becomes pressurized by expanding brain tissue, it begins to flow heavily from a shunt system and may be very noticeable. Either condition causes lower than necessary levels of CSF in the brain along with associated symptoms of lethargy/sleepiness/cognitive reduction.
Nothing like what the neurosurgeon said it would be. “Infection”, with a shrug of the shoulders, no big deal, when I asked what my risks would be. Quite possibly what he believed. Not at all what I got.
Imagine brutal HALO free-fall onto a demon’s roller coaster that dropped off the tracks in the middle of a triple loop into a bottomless pit without safety harnesses, and that’s an outline of what I endured for four months after the shunt. And don’t get me wrong, there were some enormous positive outcomes as well, improvements in long term pain and fatigue as pressure on critical areas of my brain was relieved and I began to recover; it’s my opinion that it has kept me alive. But: Holy Acid Trip, Batman, I wish I had been prepared for the beatdown I received so I could have faced it head on, KNOWN it might be coming, instead of being sucker-punched. Sucker Punched, I believe, to everyone’s surprise, including my neurosurgeon, trying to help, frustrated at not knowing the WTF of why the treatment was going sideways.
I was a dentist for 25 years. I performed a lot of complex surgeries, sedations, cut pieces from one part of a patient’s mouth and sewed/screwed/glued it to another. I spent A LOT of time preparing my patients for their post-op experience before I ever laid a scalpel on them. Well, that level of attention didn’t seem to apply here. Turns out that ICPDDs are in their infancy in the practice of medicine, thus the information that could prepare a patient for a Lewis-Carrol-meets-Hunter-S.-Thompson-esque outcome doesn’t exist. Most PCPs don’t know what “Intracranial Hypertension” is, much less how to treat it. Even amongst NeuroDocs (neurosurgeons and neurologists), there is no consensus as to the cause of ICPDDs and how to treat them. There is even a vocal group of doctors who deny they exist (I would love to let you guys spend a couple of days in my head…).
I went into the shunt surgery believing I would end up with the blessed relief I had gotten from my diagnostic lumbar puncture. I actually did get a lot of relief from pain, fatigue, cognitive difficulties, and other symptoms ICPDD patient’s struggle with, but I also got the Bonus Symptoms associated with unstable intracranial pressures, Bonus Symptoms that nobody seemed to understand.
The purpose for ShuntWhisperer was initially just to tell that story. I thought it might give somebody else a reference point, a warning, an explanation should they find themselves on the same path. But then I was hit with “bitchslap #2”, sort of a Buyer’s Remorse: it was revealed at my first postop with the Nurse Practiioner that there was essentially zero understanding of what was actually going on in a human skull, no way to measure the oh-so-important ICPs without drilling a hole in my head and inserting a “bolt”: quite literally a bolt with a a pressure transducer in my skull. In an Intensive Care Unit. Then she told me there was no follow-up program for shunt patients, “just come back if you have a problem.
Nothing like treating a problem instead of preventing it. Not to mention the loss of valuable data from a pool of patients that medicine admits to having zero knowledge of/about/how/why that a follow up program, a simple questionnaire might provide.
At that point, the purpose for ShuntWhisperer.com changed. I had become a member of support groups for patients with ICPDDs, and all I saw were patients just like me. Confused, struggling, refusing to give up but without any support. And some who where actually doing well.
After graduating from dental school in 1986, I did an optional residency in a hospital. It was worth 8 years of dental school. Exposed to Medical disciplines including Emergency Medicine and Anesthesiology was priceless, but without a doubt the group I came to admire the most was Internal Medicine. Physicians with intimate knowledge of human physiology who often collaborated on cases where the patient presented with a set of symptoms without a known cause. These docs were brilliant and were not satisfied until they knew exactly what had landed a patient in the hospital, a knowledge used to treat the cause of the patient’s condition, not just the symptoms.
They became my role models.
Now, faced with what appeared to be a terrible lack of understanding of a condition for which I had just had a tube stuck into by brain to drain cerebrospinal fluid, I was literally terrified. I swallowed it down and started paying attention to the particulars of my condition.
I began to see patterns; from patterns, theories. Testing of theories. My theories resulted in methods of managing symptoms. That management led to more days of function, fewer days of feeling so horrible I could not get out of bed, watching my life dwindle away one day at a time with no explanation.
Then it happened: Inexplicably, against all odds, my Trina was diagnosed with Intracranial Hypertension.
What are the odds? A married couple with an extremely rare disorder? Outside of a care facility, support group, or other instrument that brings ICPDD patients together, few of us know another person with an ICPDD…much less be married to one. Why? What is the commonality?
Odd, no physician has ever considered it odd yet. Asked that question: why?
(pssstttt: we both had multiple, severe neck injuries that preceded the onset of symptoms).
At that point, when Trina was diagnosed, it was all bets off. She had watched me go through hell with my shunt. She was scared to death. She had migraine headaches so bad that she couldn’t get out of bed. And then, just then, at that point:
Our neurosurgeon moves. To another hospital. Another STATE. He said he’d take our records, but it would be about 3 months before he could get us in, to treat my unstable shunt, to treat Trina. And considering it took me 3 months to get a consult appointment with him and another 3 months to get treated, and that other neuros treating ICPDDs were few and far between, it seemed as if there was no choice. To his credit, he did get us in quickly and started making plans, but three months of the psychological effects of a daily migraine that left her bedridden with ice packed around her head, the physical effects of the pressure on the centers of her brain that controlled her mood, memory, body temperature, functional capacity, the literal pressure that in combination with some of the most cruel and discompassionate dismissals of suffering by so-called “healers”, all came together on one morning in October, when with no warning, no good-bye, no note, nothing, not to mention being totally, completely out of “character” with the bright, beautiful Trina I knew and loved, she put a gun to her head and ended her own suffering, a result of having to cope with this disorder and its symptoms for an absolutely inhumane period of time. Make no mistake: the person that ended their life that October morning was not the Trina I new and loved; that person had been taken by the disorder. I was doing everything I could do, firm in my belief that if we could just hang on until she could get treated, she would be better…
It’s the day after Christmas. Just recalling that moment still freezes me up. It was so unnecessary. So preventable. She was the person everyone looks for: the soul-mate, the one human being who really gets you. And this disorder took her from me.
So now, the purpose of this site, my mission, my only purpose to keep going, is to help others struggling with these disorders. To challenge doctors with my theories, right or wrong, but to at least get them TALKING TO EACH OTHER. Hoping, praying that something here sparks something in somebody’s head that leads to an advance that wouldn’t otherwise come for 10 years. Not for me; I don’t care if anyone remembers me. For Trina, to remember her; for those suffering this disorder, in service to God. In one of the creeds of the Knights Templar, the words:
”Non Nobis, Domine, Non Nobis, Sed Nomini Tuo Da Glorium”: Nothing for us, Lord, nothing for us but for the glory of thy name.
Members of Support Groups for ICPDDs frequently refer to themselves as warriors, and they are no less. I truly pray that this site becomes a “weapon” in that battle by disseminating information and pushing for change an improvements. Not to demean, not to blame; I can’t imagine the responsibilities of the neurosurgeons and neurologists who, in my altruistic sense, are wrestling with the problems of their patients. I pray for them especially, and suggest that we all do as well.
I also ask that you remember one beautiful woman named Trina, my wife, who was taken by this disease, who I hope to see again someday, and who I hold up as an example of what I hope to help prevent.
The latest installment in my ongoing narrative of the effects that barometric pressure has on my condition, and presumably, others as well. The reason I have time to do this today is, of course, a rapidly dropping barometer accompanying a strong weather front. December 23, 2017.
I was recently surprised during a visit with a new neurosurgeon to discover that he was not aware that changes in barometric pressure, or even weather, were issues for his ICPDD patients. He seemed as knowledgeable as a physician could be about this new class of disorders, and he eagerly and actively accepted patients into his care. Thus, when I brought up the issue of barometric pressure and its effects on my personal disorder, it surprised me when he related that changes in barometric pressure were not a problem for the patients he saw.
Thinking about it afterwards, I can see how that impression could be an honest error, although having conducted polls of members of various support groups I belong to, it seems that almost everyone knows there is something about weather that affects their ICPDD symptoms for better or worse. Most seem to relate impending stormy weather with a change in their symptoms, usually for the worse*. Based on the information that most ICPDD patient are given to work with, I’m sure some know that some days they are better or worse than others. Another group may associate impending weather with a change, usually worsening in their symptoms. Some have discussed changes in altitude, which translates into changes in barometric pressure, as affecting their symptoms. Perhaps patients aren’t questioned about they notice any factors that trigger their symptoms.
Then there’s me, who is so tuned in to my disorder that the neurosurgeon asked the fair question “Do you think your attention and management to your symptoms is making them worse?”
Really, a very fair question, and I was impressed with this doc’s diagnostic thought processes. My answer is “No; I only started managing my own symptoms to the degree I’m able after months of study of my own disorder. Since starting to manage those symptoms based on my study, I have more “good” days, fewer days when I can’t get out of bed, and have been able to formulate theories about ICPDDs when unfortunately, consensus amongst medical professionals is rare (note that I said “consensus”, or “agreement”; the docs I see are desperately trying to get a handle on ICPDDs. The problem is that none of them suffer from one. That’s where I’ trying to help.).
Is my attention to and management of my ICPDD symptoms making my condition worse? Only if swimming to the surface and taking a breath when you realize you’re drowning makes your “drowning disorder” worse.
Disclaimer: I don’t know that my ICPDD is the same as somebody elses. Even if the cause is the same (I believe mine is Venous Outflow Insufficiency), it’s not likely that each patient shares exactly the same physiology. BUT: there are immutable laws of physiology that apply to everyone, and as such, probably bear consideration. I don’t know if I’m 100% right, but I’m definitely onto something; I’m not trying to get credit for anything except maybe to get people talking instead of just telling us how much medicine doesn’t know about ICPDDs. Remember this as you read the following:
BAROMETRIC PRESSURE/WEATHER: For the purposes of this segment, we’ll consider them one and the same, as I know that barometric pressure changes accompanying changes in weather and changes in altitude definitely affect my symptoms in a predictable manner. Short version: increases in barometric pressure cause and increase in symptoms that I associate with increased ICPs. Before I was treated with a VP shunt, high pressure systems made my head feel like it was going to explode. Extremely low barometric pressures as seen in hurricanes and severe storm fronts very simply put me in bed, barely able to function. My best days were overcast, but not rainy days. After I was shunted, barometric pressure still affects me in the same manner, but my symptoms are different because of the shunt. That last part took me three months to figure out, but it is fact. A couple of stories:
The first one I love to tell. I hate that my surgeon had stopped ICP monitoring during shunt placement, because I (and a lot of others) would love to have know what that monitoring might have told them. The day I had my diagnostic Lumbar Puncture (LP), my opening pressure (OP) was only 17mm H2O; it took nearly an hour to drain 30 cc of CSF, whereupon my closing pressure (CP) was 16. Most would look at the OP/LP values and say “That’s too low for a diagnosis of “IIH”.” However, the truth is that the numbers currently used to designate “high” and “low” ICPs have not basis in science.** I had great relief from my symptoms of pain, cognitive difficulties, etc. Based on the positive response, my surgeon recommended that I might benefit from a shunt; on the day of that consultation, I was feeling very uncomfortable with increased symptoms I associated with increased ICPs.
Fast forward five weeks to March 23rd: I was counting the days till the shunt; it seemed that the onset of unstable spring weather was making my ICPDD symptoms swing wildly, and I felt I was deteriorating exponentially. On the morning of the shunt surgery, I was particularly uncomfortable, my head feeling as if it was going to explode. “If this doesn’t work,” I told my neurosurgeon, “we’re going to have to scramble to find another solution.” When I woke up in postop, I was groggy, but the sensation that my head was going to explode had seeming disappeared. My surgeon came by to check on me and related that “I appeared to have had some pretty high pressures, because when (he) opened my dura for the proximal catheter of the shunt, my CSF literally shot across the room.”. Contrast that to the day of my LP, when I was actually pretty comfortable, despite feeling lousy after my angiogram. Could what I emailed to my surgeon as “Post-Angiogram Blues” been a result of the procedure, or the fact that the day between the angiogram and the LP was a near record low pressure for the area, and the lowest in the months of February and March 2017? Maybe a little of both?
Look for yourselves. These are screenshots of barometric pressure for Charlottesville, Virginia, with dates of procedures shown:
BAROMETRIC PRESSURE GRAPH 2/16/2017, DAY OF DIAGNOSTIC LP – OPENING PRESSURE 17mm H2O, CLOSING PRESSURE 16mm
BAROMETRIC PRESSURE GRAPH 3/23/2017, DAY OF VP SHUNT PLACEMENT – “YOUR CSF SHOT ACROSS THE ROOM.”
What these graphs show is that on the days of my Angiogram and LP, the area was experiencing a near record low barometric pressure. I always associated an extremely low barometer with feelings of “weakness”, leaving me bedridden. I’d further postulate that the this also explains the fact that it took nearly an hour to drain 30 cc of CSF, and that there was no significant difference in the Opening/Closing Pressures because…wait for it…
…ICPs are proportionally affected by barometric pressure. This theory is given further merit by the fact that on the day of the shunt, I was experiencing possibly the worst high pressure symptoms I’d had to that point, and the fact that I hosed the OR with my CSF, under increased pressure due to increased barometric pressure.
Why? It turns out that barometric pressure units are very significant compared to the units used to measure ICP. One millibar, or hectopascal, is the equivalent of almost 10mm H2O. Thus, the difference in barometric pressure of 28-30 mb between the days of my LP and Shunt translate into 280-300mm H2O. Normally not a problem unless you suffer from and ICPDD; now, it seems to be a big problem.
I believe this is one of those physiologic commonalities we all share. Interestingly, after I received my shunt, my symptoms changed. Now, instead of the severe pressure, I had a feeling of “lightheadedness” that I now, thru bitter experience, associate with “overdrainage”, or excessive loss of CSF. The problem now was that I felt that way 95% of the time. When the barometer was low, I still felt drained, but when the barometer rose sharply to high levels, I had a paradoxical “sensation” of pressure in my head and at the base of my skull, but still had the overdrainage symptoms. As it turns out, the barometer is still affecting me in the same manner, but because I have a shunt (“an extra hole for CSF to drain through”), my symptoms are different, but no less debilitating.
My shunt has been like trying to balance a marble on top the end of a sewing needle with the other end on a granite slab. The tendency has been to require ever-increasing pressure settings on my shunt to achieve ever shorter periods of “stability”, until in early July, the whole business went sideways and the shunt could not be turned up further. I’ve been managing my symptoms by managing my cerebral perfusion and Mean Arterial Pressures in response primarily to symptoms, and the nature of those symptoms always follow certain stimuli such as barometric pressure. Now, I believe my shunt is causing CSF Hypo-tension in the following manner:
When blood flow to my brain (cerebral perfusion) is at the low end of the scale I use to measure it, I feel very lightheaded, sleepy, weak. I know it’s due to lack of CSF because these are the same symptoms for which I was hospitalized in mid-June due to lowered a “neurologic wane/depressed state of consciousness”, symptoms that resolved with in 8 hours of turning the shunt up to its max setting. Remember, CSF is made from blood, and the amount of CSF produced and absorbed depends on the amount of blood flowing into and out of the brain. In my case, at low perfusion, I don’t make enough CSF to make up for both the normal losses of my venous outflow AND the seepage/leakage through my shunt.*** During these periods, the flexible components of my shunt are soft and shrunken as if there is not a lot of pressure in them. I believe a more appropriate term for this condition is Shunt-Dependent Underperfusion as opposed to Overdrainage.
I have a right jugular vein that has been described as between 60 and 80% stenosed at the level of C2. Venous drainage from the brain favors one side, usually the right, for reasons I don’t know. At any rate, one of the main veins that drain my brain is badly narrowed. I’ve discovered that if my cerebral perfusion exceeds a certain limit, I start todevelop sensations of pressure at the base of my skull and inside the right side of my head. My right ear turns red and swells noticeably compared to the right side (see pic). Most significantly, my shunt swells up; the catheters and antechamber become tight and enlarged, and at times the skin over the shunt is painful to touch. Why?
Simple. The pinched/narrowed jugular. At a certain point, more blood can flow into my brain than can flow out. I’ve discussed this as the “Cerebrovascular Theory of ICPDD”; blood backs up in the blood vessel of the brain, the vessels swell, displacing brain tissue and causing the brain itself to swell. The only place the brain can swell towards is where there is CSF: the ventricles inside the brain and the subarachnoid space surrounding it. Because the area inside the skull is fixed, CSF is now pressurized, blood is now more pressurized, brain tissue swells and….
…in my current condition, CSF is forced out of my shunt under high pressures, accounting for the swelling of its components. CSF is only made at ~.35cc/minute; my shunt drains at .5 cc/minute under “normal” ICP. Now, however, I have higher ICP, greater flow out of the shunt in addition to CSF absorption by normal venous pathways, and I still have a CSF deficit. This explains the feeling of pressure (due to cerebrovascular edema) as well as the “overdrained” feeling due to excessive CSF loss. This is also referred to as secondary intracranial hypotension, or iatrogenic hypotension.
This all leads me to believe that my shunt is only treating a symptom of my disorder – CSF pressurized as a result of restricted blood flow out of my brain. Maybe this is why my particular condition is so unstable. I believe the cause is the narrowed jugular, and treatment of that narrowing with a stent, then assessing its impact on my stability with the possible need to intervene by adjustment, removal, or replacment of my shunt is the next course of action. All I want for Christmas….
My first neurosurgeon told me he was confused by my symptoms. The low LP values. The apparently odd instability of my shunt treatment. I’ve actually been told (paraphrasing) that I am the “unicorn” of ICPDD cases. Here’s the thing: when I was in practice, I learned the most from the cases that fell outside of the normal curve. Cases that didn’t turn out as anticipated despite following all the accepted procedures. Those cases got under my skin, and I took it as a personal challenge to figure out what happened so I might be able to avoid putting a patient into the same situation in the future. I always became a better doctor/dentist/surgeon for the effort. That’s all I’m doing here. It’s a peculiar blessing to have a particularly challenging form of a disorder shared by others AND have the modicum of knowledge necessary to at least draw basic conclusions from observing my own condition in as scientific a fashion as possible. At first it was solely to tell the story of my treatment so that others might not be as…”surprised”…if they experienced the challenging outcomes I’ve had. Then, when my wife Trina was diagnosed (hello…rare condition…a married couple both have it…what are the odds, and more importantly, what is the reason? I believe I know….), I changed mission to find out how to alleviate her suffering and hopefully prevent her from going through what I did, possibly prevent her from getting a shunt. Tragically, help did not come fast enough. Now, having lost my career and my wife/best friend, soul mate to this…disorder…my teeth are sunk in and I will not let go until somebody somewhere listens and for the sake of everyone suffering and ICPDD and being told “medicine doesn’t know why….”. I’m grateful for everything that has been done for me up to this point, but as it turns out, it seems that the a combination of the misfortune of having a New and Exciting Medical Disorder along with the fortune of having a neurosurgeon in the right place at the right time who was at least willing to try this shunt (which I admit likely saved my life despite its unstable nature) has left me with a polar opposite bag of blessings, losses and opportunities. I miss my Leloo; Christmas will never be the same again; but as long as I have a story to tell, I’ll continue to Whisper in the hopes that the right person/people listen….
The Shunt Whisperer
December 23, 2017
ASTERISKY THINGS (Trina’s Humor):
*It is my belief based on observation and recordkeeping of my own ICPDD that ICP increases with increasing barometric pressure and vice versa. For lack of any other explanation, I believe that increasing barometric pressure compresses skin against unyielding objects such as bone and cartilage, compressing blood vessels. This blood is forced into the only structures where the atmospheric pressure changes do not affect their inner pressures. Hard structures with hollow inner cavities filled with blood vessels. The are the long bones, the teeth, and the skull. Interestingly enough, the next time somebody complains about a joint hurting when weather is changing, you can impress them with this knowledge.
**The values currently used to decide if ICPs are “high” or “low” are arbitrary numbers plucked from 30 year old research papers. There is no scientific evidence to support them; further, to say that a value of “24” is “normal” but “25” is “high” makes no sense in this system. ICPs vary widely due to a number of factors, often with a few hours. A patient’s symptoms are a far more important indicator of a disease process; ICP values are merely a snapshot of pressure for that patient, under the conditions observed, at that moment.
***The current generation of shunts are exquisitely manufactured examples of inefficient, analog devices that were originally intended for the treatment of true hydrocephalus in children. They have no active control of valve mechanisms beyond simple springs or weights, and the ball valve design lends itself to “seepage” of fluid below specified “opening” values due to accumulations of proteins or brain cells that prevent the valve from closing fully. When they do actually “open” at designated pressure setting, the valves don’t pop open, but rather creep open to “allow” CSF to flow based on the difference in pressure on either side of the valve – ranging from a slight seep to a full-on gush, again depending on pressure. This process continues until pressure differentials are within specified limits again. Further, at this time, there is no method of non-invasively measuring Intracranial Pressures, and thus determine if a shunt is functioning or not beyond patient symptoms. While inclusion of electronics to monitor intracranial pressures is definitely possible, the challenge of using them in devices that must be subjected to MRI examination has yet to be overcome. They are the best option available if regulation of CSF pressure is necessary.
Cerebrovasular Theory of Intracranial Hypertension, Part 2: Leading to The Danger Zone
It’s really simple. Damage/squeeze (“stenosis”) the main veins that drain the brain and a situation is created where the damaged veins can’t drain blood OUT as fast as the heart pumps it IN. Blood backs up, the brain swells, and pressurized CSF. The only cause? Dunno, but interesting that Diamox, a nasty drug used to “reduce CSF production”, is actually a diuretic used to treat blood pressure. Looking at the huge torrent of blood that flows through the brain, comparing it to what amounts to a quiet pool of CSF that changes at a snails pace, CSF MADE from blood to begin with, and it begs the question: why the focus on reducing precious CSF if the cause is actually damaged veins that can’t drain blood away adequately during periods of increased blood flow to the brain? Here’s the long story:
During my “experiential residency” in the study of Intracranial Pressure Dysregulation Disorders, it has become apparent that certain areas of the brain appear to be more susceptible to increased CSF pressure. However, I don’t believe that an imbalance in CSF production and absorption is the principal cause of my particular ICPDD, but rather a symptom, the result of an imbalance in the blood flowing into the brain vs. blood flowing out of the brain. The tenets of my current Cerebrovascular Induced Intracranial Hypertension are a direct extension of Dr. Mark Wilson’s ’s publication “Monro-Kellie 2.0“, and my personal experience as patient with an Intracranial Pressure Dysregulation Disorder that my neurosurgeon tell me does not fit any of the criteria for currently recognized conditions such as IIH. These tenets are as follow; facts are derived from “Brain Facts and Figures” (https://faculty.washington.edu/chudler/facts.html):
Two Fluid Postulate: The cranium contains two fluids, blood and cerebrospinal fluid, in approximately equal volumes, ~ 150 cc 1, in patients without ICPDD and “intact” ICP Autoregulation.
Static Nature of CSF: Cerebrospinal Fluid volume is assumed to be essentially constant for this discussion This does not dismiss the possibility that a net positive or negative CSF dynamic is not a contributory factor in ICPDD. However, CSF turnover only averages .35 cc/minute1, leading to the next tenet…
Blood vs. CSF: Cerebrovascular blood flow dynamics greatly outweigh CSF dynamics. Blood flow into the brain is 15-20% of cardiac output, 750-1000 cc/minute1, meaning that CSF dynamics represent a mere .05% of the change in volume of fluid In the brain per minute. This is a known fact apparently forgotten or ignored according to Dr. Wilson.
Cerebrovascular Network: Our brains demand a lion’s share of oxygen and nutrients, provided by blood flow. These nutrients are distributed through a network of blood vessels. In order to provide each cell of the brain with oxygen/nutrients, blood vessels must be in extremely close proximity to each cell. The vascular network is extremely complex, and is estimated to comprise 100,000 miles of blood vessels, the majority of them only wide enough to allow blood cells to pass through single file, about 6um (micrometers): 6/1000s of a millimeter. Main arteries and veins into/out of the brain can be as large as an inch, then branch out continually into ever smaller vessels to provide nutrients to each of the approximately 100 billion neurons in our brains. All of this is accomplished with a mere 150 cc of blood fluid volume, which flows through this cerebrovascular network at 750-1000 cc per minute.
Volumetric Balance: to maintain equilibrium of the volume of blood in the brain, the total venous outflow capacity has to equal the total arterial inflow ***This is the critical tenet of this theory: blood flowing into the brain MUST flow out at an equal rate****
Venous Outflow Insufficiency: The 800 lb Gorilla in your head: Understanding the previous tenets, the critical nature of sufficient venous outflow becomes apparent. Arteries and veins are vastly different in nature and function. Arteries are thick walled, contain smooth muscle, and dilate/contract in response to our nervous system and biochemicals (hormones). Veins are thin walled tubes of cells; their diameter is susceptible to posture, gravity, and the actual rate of flow through them (as per Bernoulli’s Principle). Veins are susceptible to damage by trauma, posture, and anatomical factors including Body Mass Index. When Venous Outflow is insufficient to match Arterial Inflow, there is a net increase in the VOLUME of blood in the brain.
Intracranial Contents, The Volumetric Paradox, and The Physical Nature of The Brain: The adult skull is approximately 1700 cc in volume; the brain occupies 1400 cc; blood, 150 cc; csf, 150 cc. However, there is a paradox: the 1400 cc of brain volume also contains a majority of the 150 cc of bloodwithin the skull. The brain itself is mostly water, 77-78%, contained within the network of neurons that comprise the tangible portion of the brain. These neurons are chiefly lipids (10-12%), proteins (8%), and 4-5% a combination of carbohydrates, “soluble organic substances”, and inorganic salts. The consistency of the brain is about the same as firm tofu (http://www.human-memory.net/brain.html); slightly elastic, with a degree of resilience. The brain “floats” in cerebrospinal fluid that not only surrounds it, but again, a paradox: approximately 2% of the brain’s volume is hollow “ventricles” (https://www.ncbi.nlm.nih.gov/pubmed/21120804), spaces where CSF is produced and distributed through the brain and then to the space around the brain and spinal cord (subarachnoid space). Thus, the 1400 cc volume of the brain also contains about 28 cc of cerebrospinal fluid. This is the basis for the Volumetric Paradox: 15% human brain’s volume is comprised of blood and cerebrospinal fluid.
The End Game: When Venous Outflow Insufficiency meets the Volumetric Paradox: Tofu and Internal Pressure: Our brains are soft, mildly resilient structures. They require enormous amounts of oxygen and nutrients, delivered by arterial blood flow. Blood depleted of nutrients and oxygen is then drained by veins. Cerebrospinal fluid, a crucial but poorly understood component of this system, changes at a snail’s pace compared to blood flow, and thus for this discussion will be considered a static innocent bystander for what happens next: Venous Outflow Insufficiency. If, for some reason, the main veins that drain the brain (sorry, it’s been a looooonnnnnnngggggg week, I’m a little punchy, I just couldn’t help it) are unable to handle the arterial blood flowing into the brain, a serious cascade of pernicious events follow. Blood begins to back up in the brain’s cerebrovascular network. Pressure within the network increases. This pressure cause the blood vessels in the brain to become engorged and expand, similar to the way that the veins in your arm start to bulge when a tourniquet is applied in order to draw blood for testing. The next part is my speculation, but under equal pressures, it seems that thin walled, passive veins enlarge more than thick walled arteries that contain active muscle. All within the 100,000 mile network of the Cerebrovascular network. As the vessels expand, pressure is exerted outward on the tofu-textured brain. The brain matter itself is forced to expand into the only spaces available to it: the outer subarachnoid space and the inner ventricles. The cerebrospinal fluid, the innocent bystander, is now “bitch-slapped” around by this building pressure, referred to as EOMAP, or “End Organ Mean Arterial Pressure”. Newton’s 3rd Law of Physics states that every action is met with an equal and opposite reaction; in the case of Venous Outflow Insufficiency, this means that as cerebrospinal fluid becomes pressurized by brain tissue expanding due to a backup of blood in the vessels of the brain, the CSF “pushes back”, trapping brain structure between the”Devil” of the engorged and expanding blood vessels and “The Deep Blue Sea” of the now-pressurized CSF both outside AND inside of our brains. And….
Voila’: Intracranial Hypertension. By definition. Caused not by CSF, the equivalent of a small pool of still water, but rather by the sudden blockage of the torrential river of blood that flows through our brains. There is much, much more to the dynamics of this process and how the negative process feeds upon itself, but this concept is already a recognized process in an extremely acute ICPDD, Acute Mountain Disease and its associated deadly consequence of HACE, High Altitude Cerebral Edema. For the time being, understanding this process as a potential cause of ICPDDs including IIH, NPH, pick-your-alpahabetic-combination-of-Hs, is critical to understanding what happens as its result. This will be explored in the next topic on ShuntWhisperer.com, “The Danger Zones.”
This post, this topic, this site, is dedicated to the memory of my wife, Trina Haddix, who died as a result of complications related to an untreated ICPDD. Her story will be told on TrinasPage.com in the coming weeks. I miss you baby, I love you so much. I will not let you be forgotten.