UNDERSTANDING “IIH” – FAQ’S

“(the cranium is) Just like carry-on luggage, only so much can fit…one pair of socks to many, and the seams start to strain.”

I’ve only lived with IIH for a few years – much less time than many, many others I encounter in support forums.  Lately I’ve taken notice of newly diagnosed patients who seem to be as perplexed at the lack of information and explanation of their condition as I remember being in 2017 when I was told “Nobody knows nothing about IIH.” (paraphrase). 

Judging by the bewildered posts of new members of our auspicious group, there hasn’t been much change.

What follows is the beginning of a “Frequently Asked Questions” section of Shuntwhisperer.  The information here is a combination of 1) my personal experience, observation of others with IIH/PTC/ETC, 2) published research that is fairly abundant for a disorder that seems to amount to a Black Hole of Understanding, 3) readily accepted tenets of human physiology, and 4) the babblings and savante-garde notions of a guy with a hole in his head and a tube in his brain.

Fair warning, take it for what its worth ; )

In all seriousness, I pray this helps those seeking answers, and as always, it’s not about me being right, it’s about getting others to think and talk about IIH in the hopes that this dialog will spark understanding and improved quality of life for everyone. 

Thus, with the timelessness of the inspired dialog of Shakespearean-trained actor Patrick Stewart,

“ENGAGE!”

 

FREQUENTLY ASKED QUESTIONS ABOUT IIH

Short Answers First, Technomedical Stuff Second

WHAT IS “INTRACRANIAL HYPERTENSION”?

Short Answer:

“Intracranial Hypertension”, or “IIH”,  is one name for a group of complex disorders in which an individual develops physical signs and symptoms caused by abnormally high pressures of the fluids in and around the brain.* 

 

Geek Stuff:

The adult human brain is enclosed in a protective bony compartment of the skull known as the cranium.  The main contents of the cranium are the brain, connective tissue (meninges), and two fluids: blood and cerebrospinal fluid (CSF).

Because the cranium is rigid and has a fixed volume, net accumulations of either fluid will increase the pressure inside the cranium (Intracranial Pressure, or ICP).

“Intracranial Hypertension” results when an individual’s tolerance for normal pressure variations becomes compromised, especially high pressures.  These pressures can affect the brain itself, becoming stressed with predictable results   It is referred to by several abbreviations which likely represent individual variations of the same “disorder”:

  • IIH – “Idiopathic (‘of unknown origin’) Intracranial Hypertension
  • NPH- “Normal Pressure Hydrocephalus” –  A very misleading term that is contradictory and would only apply to true conditions of hydrocephalus, a condition where Cerebrospinal Fluid becomes trapped due to developmental or traumatic factors, allowing the very slow forming CSF to accumulate in isolated areas under significant pressure, causing deformation and/or damage to the brain tissue
  • BNPH- “Benign Normal Pressure Hydrocephalus” – Another extremely misleading term, as with “NPH”, along with the fact it is anything but “benign” (harmless)
  • PTC – “Pseudotumor Cerebri” – literally, “false brain tumor”, one of the earliest names given to this class of disorders due to the fact that a patient’s symptoms usually mimic those of a brain tumor, but upon CT/MRI, no tumor is evident.

I consider these “different” diagnoses to be representations of a similar disorder, expressing itself differently in individual patients due to their particular physiology.   Consequently, I refer to these disorders as Intracranial Pressure Dysregulation Disorders, or ICPDDs.

427420290-cerebellar-vermis-metencefalon-cerebral-hemisfere-brain-lobe
The Brain and its supporting anatomical structures are encased in a rigid bony protective compartment of the skull known as the cranium.  Just like carry-luggage, only so much can fit inside.  One pair of socks too many, and the seams start to strain.

*Growths of soft tissue such as tumors and cysts may also increase ICP with similar presentation; however these conditions fall into different diagnostic and treatment categories.  For the purposes of discussing the ICPDDs, the focus will be on blood and cerebrospinal fluid, assuming there are no abnormalities of the soft tissues in the cranium, including the brain itself. 

 

WHAT IS THE SOURCE OF “INTRACRANIAL PRESSURE”?

Short Answer:

Intracranial Pressure, or ICP, is the created by high volumes of blood being pumped into an extensive network of blood vessels that support the brain. (This is the foundational principal of Intracranial Pressure Dynamics, hence my appellation “Cerebrovascular ICP Dominance Principal”).**

Geek Stuff:

The brain requires more energy than any other organ in the human body.  That energy is delivered by blood containing oxygen and glucose.  Blood also carries away metabolic waste from brain cells (neurons).

  • Big Numbers Alert: The adult brain has an estimated 100 billion cells called neurons.  Getting blood to each neuron requires a vast and intricate network of blood vessels estimated to be up to 100,000 miles in total length.  The majority of the vessels are so small that blood cells must pass through one at a time.

Blood enters the brain through arteries under high pressure (Average Mean Arterial Pressure of 90 mm Hg) and volume (750-1000cc per minute).  Resistance to the flow of viscous blood (~1.8 times “thicker” than water) through the network of vessels creates pressure in the vessels.  This pressure is transferred from the blood vessels to the brain tissue and ultimately into cerebrospinal fluid in and around the brain and spinal cord, creating what is termed “Intracranial Pressure.”

**Shortly after arriving at and naming this principal “Monro-Kellie 2.0” in Millennial TechnoEvolutionary Credit to the Monro-Kellie Principal, I discovered a cheeky neurosurgeon of “Her Majesty’s Royal College of Medicine, London” had published a paper on this very subject in 2016.  His name is Wilson.  Dr. Mark Wilson.  His publication:

Monro-Kellie 2.0: The Dynamic Vascular and Venous Pathophysiological Components of Intracranial Pressure

This publication, accurate and well written, was obviously composed by MI6 as cover for “Dr. Agent” Wilson.  Subterfuge aside, it does make both Agent…excuse me, Dr. Wilson and myself seem like a pair of bright bulbs.  Nonetheless,  a pair of very stiff upper lipped chaps suggested I change the name by which I referred to this principal, in the name of the Queen…and my kneecaps.

And I do favor mine very, very dry, and of course…

Shaken…not stirred.

XE3_00992_XL
“Dr.” Mark Wilson, Neurosurgeon

 

WHAT IS “NORMAL” INTRACRANIAL PRESSURE (ICP)?

Short Answer:

Intracranial Pressure is not a fixed, unchanging value.   ICP is dynamic by virtue of its origin in the volume and pressure of blood flowing into the brain, which is in turn inside of the rigid cranium with a limited amount of available space.  Factors affecting systemic blood pressure and an individual’s response/reaction to those factors, will cause ICP to vary as well.  “Normal” ICP is a patient-specific range of pressure determined by individual physiology.  Thus, for each of us, “Normal ICP” is any pressure that allows healthy & asymptomatic brain function.

Geek Stuff: 

While scientific studies establish an average of 110 mm of water (defined as the pressure at the base of a water column 1mm in diameter and 110mm tall), the truth is that “normal” ICP varies from person to person, again for reasons of individual physiology.  Furthermore, ICP varies in an individual over periods from as few as a few minutes to as long as years as dynamics of blood flow and CSF production fluctuate.

All aspects of ICP are dependent on the fact that blood is the only fluid that enters the cranium/brain.  Therefore the pressure and flow of that blood directly affect ICP, and hence the fact that an individual patient’s ICP varies within a personal range and is not a static value.

Finally, the brain itself ensures it receives adequate nutrient-rich blood, controlling blood flow through heart rate/contraction, as well as other critical factors including systemic blood pressure, available oxygen in the air (partial pressure of oxygen), a patient’s lung health (presence/absence of fibrosis, COPD, smoker, and other factors that affect gas exchange with blood in the lungs), health of the blood (anemia, hemoglobin, age/size of blood cells), as well as environmental factors including barometric pressure (and hence altitude), heat, humidity…the list goes on.

In a word, any condition that potentially hinders a person’s brain from getting adequate oxygen in the smallest quantity of blood possible, or which makes their tolerance for ICP fluctuations more narrow, or changes the dynamics of those fluctuations to higher ranges for longer periods potentially predisposes them to developing an ICPDD.

Mayfield-Clinic-Pic
Chiari Malformations are essentially brains too big for the available cranium…or vice versa.  The result is less available volume to allow a patient to tolerate ICP fluctations, not to mention the potential interruption of CSF circulation between the cranium and spinal cord, aka “corking” (credit to Dr. Diana Driscoll and her ongoing body of work, “The Driscoll Theory”)

See FAQ Topic “HOW DOES ICP BECOME ELEVATED?” (coming soon to a url near you! 7/30/2019)

 

WHAT CAUSES ICP TO BECOME ELEVATED?

(That was fast!)

Short answer: too many socks in the carry-on luggage.

The adult cranium is a rigid bony case and does not expand or contract. After the bony plates of the skull fuse in adulthood, it has a defined available volume.  If one or more of the contents of the cranium accumulates without a matching decrease in the other contents, the pressure within the cranium predictably increases.*  For the purposes of discussion of ICPDDs, blood and cerebrospinal fluid (CSF) will be the only cranial contents considered to affect ICP.  Growths of soft tissue such as tumors and cysts can also increase ICP, however these conditions generally fall into different diagnoses and treatments.

Looking at blood and CSF, it becomes apparent that blood is the dominant, driving force behind ICP:

  • Blood is the only fluid to enter the cranium, at a rate of nearly one liter per minute at rest, a figure that represents approximately 20% of cardiac output.  This is because the brain demands enormous quantities of oxygen and glucose to function, and actively regulates the amount of blood it receives in real time.
  • By contrast, Cerebrospinal Fluid (CSF) is a by-product of brain function.  It is “distilled” by osmosis in specialized cells lining small hollow (normally) interconnected areas within the brain known as ventricles, as well as by similar tissue along the outside of the brain.  It is produced at a mere 0.35cc/minute from arterial blood, and as such accounts for only 0.0004% of the fluid dynamics within the cranium, with blood accounting for 99.9996%.
  • Blood and CSF are both present in the cranium in approximately equal volumes of 150 cc.  The blood volume enters and exits at approximately 6-7 times per minute; CSF is produced and replaced approximately every 7 hours.
  • CSF flow passively in and around the brain, following pressure gradients from areas of production to areas where it is absorbed by veins and lymphatic tissue.  This passive circulation is aided by pulsations of blood vessels in the brain as well as physical movement of the body.

ICP becomes elevated if blood and or ICP accumulate at a greater rate than they are removed from the cranium.  Due to the high flow rate of blood into the brain, even the smallest deficit in drainage of blood from the brain becomes a potential source for rapid elevations of ICP; this is known as (Chronic) Cerebrospinal Venous Insufficiency, or CCSVI, but could more accurately be termed Cerebrovascular Outflow Insufficiency, Acute and/or Chronic.  This is likely the source of elevated ICP in patients who do not have isolated pockets of non-draining CSF (“Non-Communicating Hydrocephalus”); however, Cerebrovascular (Blood) Dynamics and Cerebrospinal Fluid (CSF) Dynamics are inextricably linked due to the enclosed nature of the cranium**.

NIagara Falls
Cerebrovascular ICP Dynamics
frozen waterfall
CSF Dyamics

 

*Monro-Kellie Doctrine

**”Monro-Kellie 2.0, The Pathophysiology of Cerebrovascular Dynamics in Intracranial Pressure”, Dr. Agent Mark Wilson, Royal College of Medicine Branch, MI6

 

 

 

 

 

SHUNTWHISPERER 2.0 – NEW PERSPECTIVES, NEW THEORIES, BETTER OUTCOMES AND QUALITY OF LIFE

“…My last post was in late 2018 regarding an epiphany I had been given regarding a theory about how a person develops acquired adult IIH. Of course, right at that point, the creaking, weak, bottom fell out from under me.

Again….”

Note:  I need to acknowledge something to a person who reached out to me for help.  This person believed they were developing an infection of their shunt and were desperate.  Due to my own recent challenges, I didn’t open that email until a week later, and then due to some strange glitch, lost it entirely.  To that person, I am deeply sorry, and I pray you were able to avail yourself of the ER in time.  FWIW, I just went through the same problem 7 weeks post-revision.  I post my email contact, ShuntWhisperer@ShuntWhisperer.com to help answer questions if possible, but I am not a neurologist or neurosurgeon, and if anyone should find themselves in and emergency situation (infection of the shunt tract qualifies), get to your physician or ER first, then email me if you like.  FWIW, I am making it a point to check my email every morning at the very least as long as I am able.

 

Greetings to all. 

It’s been a minute, to be sure…more like 4 months.

My last post was in late 2018 regarding an epiphany I had been given regarding a theory about how a person develops acquired adult IIH.  Of course, right at that point, the creaking, weak, bottom fell out from under me.

Again. 

I had to devote what little functional capacity I had to managing this new challenge; making new posts here was just not possible.  February was a very, very, very (etc.) difficult month.  Most days I felt like I a lone person in a dark void above a bottomless abyss, clinging by my fingernails to my spiritual, almost tangible touchstone, Jesus Christ.  I can say with complete certainty He is the only reason I am still here to right this story.

The Lord works in His own way(s).  In my case, Dr. Kenneth Liu of Penn State Neurological Services, the neurosurgeon who saved my life by placing my first VP Shunt two years ago, revised my shunt and repaired the inexplicably persistent hole/CSF leak associated with the craniotomy (surgically created hole in my head).  I referenced this problem in the post “Juice Boxes and My Brain…Who Knew?). I can now say with a weird sense of satisfaction that I now have a metal plate in my head.  A small one, but nonetheless, a metal plate.

Cool.

This surgery resulted in an immediate, marked improvement, but still with instability, though not nearly as severe.  There was still a small but noticeable  CSF leak.  At 5 weeks that last leak suddenly stopped, and it was as if my brain had suddenly been switched back “ON”.  Most notable was that extreme sensitivity to certain triggers, especially barometric pressure, was either drastically reduced or eliminated altogether.   Before the revision surgery, I could not stay at my new retreat in the mountains at a modest 2200’ elevation if the barometer dropped below 940 mb.  I would become bedridden with fatigue, pain, and ‘brain fog’.  I would be forced to leave the mountain for lower elevations, and I began to be able to predict at which turn in the road I would start to feel improvement.  I would stay at a lower altitude for a week, and when I felt better, I returned, only to have the scenario play out over again within the next 7-10 days.  Was this real, or was it some perverse trick of my psyche?  I decided to find out, and with a prescription from Dr. Liu, I bought a home hyperbaric chamber.  Expensive, but it was the only way I knew to answer the question about the effect of barometric pressure on my personal form of this disorder.

I’ll go into more detail about the chamber later; for now I’ll just say I ran a series of controlled experiments.  The results were undeniable: climbing in the hyperbaric chamber and simply increasing the pressure inside to 30mb above local pressure reduced or eliminated by pain and feelings of fatigue.  However, no matter how long I stayed in the chamber, once I exited, those symptoms returned in 1-6 hours depending on local conditions, so there was no doubt that this small change in barometric pressure was enough to drastically affect my well being.

I also noticed something else: when I got out of the chamber, I could feel my shunt flowing.  If you have a shunt, you know what I mean.  More importantly, I noticed that swelling developed along the shunt components under my scalp starting at the craniotomy and eventually proceeding back to behind my ear.   I interpreted this as a CSF leak.  The catheter that connects to the shunt valve is a surgical silicone; human tissue does not adhere to this material.  I also discovered these catheters are further treated make them even more resistant to the adhesion of cells in order to prevent stray bits of protein and brain tissue from adhering to the inside of the catheter and eventually blocking it; this treatment is intended to prolong the life of the shunt.  In my case, bone had failed to fill back in the craniotomy, leaving a path for CSF to leak between the outside of the catheter and the dura and along the catheter and shunt under my scalp.  This resulted in overdrainage of CSF as it was drained not only from inside my brain as intended, but from outside the brain as well.  This caused me to experience intracranial hypotension, and was a truly miserable test of my personal belief that a more appropriate term for this disorder is Intracranial Pressure Dsyregulatory Disorder (ICPDD). 

I took this information to Dr. Liu and ultimately the decision was made to revise the shunt and repair the craniotomy.  Now that this unwanted leak has been stopped I have experienced increasingly stable improvement of my disorder, including greater functional capacity and dramatically reduced ‘fibro’ pain.  I still have challenges from the effects of almost 2 years of wild swings in my ICP, including physical deconditioning.  Spring weather patterns here have been brutal, with at least 4 “bomb-grade” storms (defined as a weather system with at least a 24 mb drop in barometric pressure in 24 hours) in the last month.  Prior to the March surgery, these systems invariably laid me up for at least three days; now, their effects on my disorder are greatly diminished, and seem to be continuing to diminish with each passing day.  Only one recent freak set of back-to-back storms really hit me hard, but in that case there was also a potentially serious late postop complication that I was fortunate to recognize and address before it got out of control.

All this preamble brings us to now.  ShuntWhisperer started as merely a “this is my story, maybe it will help you” type of blog.  Over the course of two years, I’ve learned more as I’ve continued research and experienced new challenges that while very difficult, contributed to a better understanding of my disorder.  The structure of the original site was never intended to present this material of this depth in a coherent and orderly manner.  I never expected it to be more than a few pages, but it has blossomed well beyond that.  Thus I will be  restructuring the site “on the run” as ShuntWhisperer 2.0.  It is intended to be concise, presented in lay language to the degree possible, and accompanied by references to existing research, with a reference section for physicians.  Please don’t expect a visually dazzling site; I simply don’t have the time to post anything but pertinent information.   Also, the original ShuntWhisperer site and its contents will still be available, accessible from the new landing page.

SW 2.0 is still intended to be based on my personal experiences and research.  I can’t claim anything I post applies to any other person with a disorder of altered intracranial pressure, although there are certain immutable laws of human physiology that lead me to suspect that is indeed the case; I just can’t make that claim.  I’m not sponsored, I don’t get paid for this, and don’t care to receive any credit for any good that comes of what I post.  I am doing this in Service to God in an attempt to help others, as well as in memory of my wife Trina.  My contact email is Shuntwhisperer@shuntwhisperer.com for anyone who has a question, feedback, or suggestion. 

As always, prayers to all for comfort, support, and a better quality of life.

 

Wes

The ShuntWhisperer

April 26, 2019

 

Today’s storm and rain has passed; it’s a gorgeous spring day on the mountain, and I’m going to take a break to enjoy it, wishing Trina was with me.

“But ask the animals, and they will teach you, or the birds in the sky, and they will tell you; or speak to the earth, and it will teach you, or let the fish in the sea inform you.  Which of all these does not know that the handoff the Lord has done this?” 

           Job 12: 7-9

 

Welcome To The Shunt Whisperer 2.0

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.

Prayers and Blessings to everyone,

The Shunt Whisperer

October 6, 2017

Juice Boxes And My Brain: Who Knew?

“…March 2017 until I’m guessing November-ish 2017, I had a CSF leak that mucked up the attempts to control my ICP…”

 

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.

#CROWDHEALING.

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

Sophysa polaris
Propaganda Pic of My Shunt. The “Reservoir” is thick silicone, the rest inflexible plastic/metal. My baby.

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 changing shape.  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.

9569
Rendering of how the shunt is placed into the brain. Gruesome Reality.

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.

endoscopic-third-ventriculostomy-lg
Catheter inserted into ventricle in brain. Catheter is placed over a rigid metal guide tube which is withdrawn after the catheter is properly placed.

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

Corona Juice Pouch
Too much time on my hands ; ), but a great idea nonetheless.

(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.

Now:

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 whispers@shuntwhisperer.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.

 

A Caregiver’s Thumbnail Guide to “Intracranial Hypertension”

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
  • Mood Alteration/swings
  • 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
  • Fatigue
  • Increased risk of Hemorrhagic Stroke due to increased pressure in the blood vessels of the brain*
  • Nausea/Vomiting*
  • 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

Fernando, et. Al

https://fqresearch.org/pdf_files/cipro_and_pseudotumor_cerebri.pdf

I know this to be the truth all too well.

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.

 

The ShuntWhisperer

February 3, 2018, revised May 1, 2019

Thinking of you every day, baby.  I miss you so much…

Defining Your Disorder: Quality of Life by the Numbers

“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.”

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:

1)  Weight

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.

4)  Ambient Temperature

5)  Pain Level according to the pain scale

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:

 

QUALITY OF LIFE/FUNCTIONAL CAPACITY

COMFORT INDEX SCALE

ICPDD (Intracranial Pressure Dysregulation Disorder)

copyright 2018 ShuntWhisperer Media

 

 

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.

Welcome; let’s get to work…

“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…”

Welcome to ShuntWhisperer.com.

It’s gonna be a bumpy ride.

I started this site after being treated with a VP shunt for an Intracranial Pressure Dysregulation Disorder (ICPDD).   For whatever reason, the result of the shunt treatment was nothing like what I expected it to be.

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?

I know.

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.IMG_0023 (2) - Copy - Copy

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 Shunt Whisperer

December 26, 2017

A Christmas Story: Barometers, Blood, and Swollen Brains…now with Pictures and Proof!!!

THE WEATHER AND MY IIH/ICPDD:

WHAT I KNOW…

 

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 2/1/2017 – 3/31/2017, COURTESY WWW.WUNDERGROUND.COM

Screenshot-2017-12-23 Weather History for Charlottesville, VA Weather Underground

 

BAROMETRIC PRESSURE GRAPH 2/16/2017, DAY OF DIAGNOSTIC LP – OPENING PRESSURE 17mm H2O,  CLOSING PRESSURE 16mm

Screenshot-2017-12-23 Weather History for Charlottesville, VA Weather Underground(2)

BAROMETRIC PRESSURE GRAPH 3/23/2017, DAY OF VP SHUNT PLACEMENT – “YOUR CSF SHOT ACROSS THE ROOM.”

Screenshot-2017-12-23 Weather History for Charlottesville, VA Weather Underground(1)

 

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.IMG-6505

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.

IMG-5031

EMERGENCY RELIEF OPTIONS

…Everything here is based on the concept of reducing the demand for blood flow to the head beyond normal, and attempting to maximize the very tricky nature of how veins drain blood from our brain….

Reading about so many fellow ICPDD patients with terrible headaches, pain, when their pressures are high.  Questions about “How can I make this stop?!”.   Short of proper diagnosis and medical intervention, relief from symptoisms of high intracranial pressures may not be completely possible, but I know from personal experience that certain things help me enough to make things at least bearable.  I don’t know if they will help you or not, and as such, I’m opening this page up to comments so everyone can learn from everyone else.  This is the first time I’ve done this on ShuntWhisperer.   I pray it helps because I know the terrible toll that incessant pressure and pain can have on a human being.  One of my goals when I started this site was group data mining to help direct opinions and treatment of this disorder.   Achieving meaningful results through that goal start here.  I’m tired of seeing so many people suffer.  I’ll say up front, I don’t have a clue if this will help anyone else.  It may be the hardest I’ve worked on something that has little value, but I have to try.  This list is far from comprehensive and will be added to over time, but it’s a start.

These recommendations are based on conclusions I’ve arrived at through evaluation and tracking of my own personal condition.  I know that my pressure symptoms are directly related to how much blood is flowing to my brain.  Above a certain point*, the veins that drain blood from my brain can’t accommodate the amount of blood flowing in via arteries.   I have no idea if I have any CSF “imbalance”** aside from when it all drains out of my shunt when blood flow to my brain hits that critical mark.  Everything here is based on the concept of reducing the demand for blood flow to the head beyond normal, and attempting to maximize the very tricky nature of how veins drain blood from our brain.  And everyone, I’m not a doctor; I’m a patient just like you.   I’ve had a rough time after my shunt, but I’ve learned some things as a result.  I do this in service of God, praying that this information might make somebody else’s life easier.  Please don’t do anything here that your doctor has told you NOT to do.

 

Here we go:

EASY MEASURES

  1.   COOL YOUR HEAD/BODY  The brain does not like to be hot.  Heat, especially when combined with humidity, cause your body to demand more blood to run through the brain in order to carry away the heat.  Of course, heat taken from the brain is radiated away through the skin all over your body, so the more of “yourself” you can cool, the better.    Try these “easy” things first, based on where you what’s available
    1. COOL SHOWER/BATH:  Whichever you prefer, or is necessary depending on your equilibrium.  The water does not have to be cold, just cooler than your body.  Make sure to get your hair wet.   Refresh with cool water as needed.  It’s not rocket surgery.
    2. ICE TOWEL: soak two clean towels in water.  Wrap them separately in saran wrap.  Put one in the freezer and one in the refrigerator.  Wait for the towel in the freezer to get cold, maybe a little stiff, but not frozen hard.  Take it out and put the towel in the refrigerator in the freezer.   Wrap the towel you just took out of the freezer over your head, neck, and face.  Be careful that the towel is not so cold as to give you frostbite.  Leave it on for 15 minutes while reclining at about 45 degrees in a chair or against some pillows.  When that towel is no longer cool enough, rotate with the other one.  Of course, don’t leave them in the freezer long enough to turn to ice logs.
    3. FAN/WATER MIST:  Sit in front of a fan, again preferably reclined about 45 degrees. The bigger the fan, the better.  You’ll need a pump mister that puts out a fine mist.  Expose as much skin as modesty dictates, and spray your hair, face, and body with the water mist.  Let the fan evaporate the water from your skin.  Repeat as needed.  If you’re in a public place where stripping down and spraying yourself with water might be frowned upon, there are misting bottles with attached fans that you can use on your face/head/neck as privacy allows.
    4. EVAPORATIVE FABRIC HEADGEAR  I loved these thing last summer.  Go to Amazon, type in “Mission Multi-Cool”.  I have several.  I don’t necessarily endorse the mission brand; I have several of different brands, and they all seem to do what I need them to do: keep my head cool.  Get them wet, preferably with cool water, wmission scarfring them out, put them around your neck, pull them up over your face, or completely cover your head.  As the water evaporates from the fabric, an enormous amount of heat is carried away from your skin as well.  Carry a bottle of water to refresh them when they get dry.  I keep a few in the refrigerator to rotate as I use and launder them.  Even just keeping it around my neck helps, and when I can I pull it up over my face.  A must have in the ICPDD Warrior Kit

BOLDER STEPS

  1. CUT YOUR HAIR SHORT An extension of keeping your head cool.  During the summer, it was the consensus of many members of support groups for ICPDDs on Facebook that short hair helped some feel better.  Makes sense, long hair is an insulator and holds heat on your head.  I guess this decision requires weighing fashion sense with desire for comfort, and it’s a “semi-nuclear” option because you don’t know what degree of comfort you will get until you do it.  I’ve gone to a military “high and tight”, and I’ve got to say that it helps, especially when I’m using iced towels.
  2. AIR CONDITIONING AC removes humidity from the air in a room or home.  Dry air carries away moisture from your body more efficiently by allowing evaporation of sweat or the water you just spritzed on you.   I’d suggest that anyone with an ICPDD have air conditioning in at least one room,  especially the bedroom. 

REALLY BOLD STUFF

  1. SEEK HIGHER GROUND mountainsA temporary measure I employed in extreme situations. On several occasions this summer, weather conditions combined in a way that aggravate my ICPDD to the point of intolerablility .  Even indoors I was miserable.  Heat, Humidity, and High Barometeric Pressure were the evil triad for Trina and I.  As fortune would have it, my mother lives in the mountains 100 miles to the east at an elevation 1000 feet higher, so when weather made conditions in my head intolerable, we went to visit Mom.  Geography and elevation combined to reduce heat (approximately 10 degrees F per 1000 feet elevation) and barometric pressure (30-40 millibars, or mb. Note that this equates to 300-400 mm H2O…a very significant amount of pressure).  Unfortunately, Virginia summers make humidity almost pervasive in this area, so we could rarely escape that, but the change in elevation gave the most relief.  The downside is that my body would accomodate this new elevation after 2-3 days, so I would only do this when I was desperate.  There was also the fact that we had to eventually go back home, possibly back to the same conditions I had fled from.  Worse, after my body had accomodated to the higher latitude/lower pressure, returning to the opposite conditions was occasionally very unpleasant, but that few days of relief seemed to always give me a much needed break.

OXYGEN  Two things about this:  I’m just at the point of investigating this (see Sleep Therapy Section), AND you should talk to your doctor first about this.  The only way to find out if Oxygen Therapy will work is trying it.  Oxygen plays a major role in reducing cerebral perfusion, or blood flow to the brain.  Oxygen therapy is used to treat migraines, cluster headaches, and other headaches known to be caused by high blood flow to the brain.  Low oxygen tension in “thing” air at extreme altitudes is what triggers Acute Mountain Sickness, or Altitude Sickness, and its extreme and possibly lethal consequences, High Altitude Cerebral Edema (HACE) and High Altitude Pulmonary Edema (HAPE).  A current theory exists implicating ongoing elevated ICPs can continually trigger migraines one after another.  Oxygen is one of the most therapeutic drugs available, but needs to be used properly.  Some health conditions preclude use of concentrated oxygen, so checking with your physician is recommended.  Health Insurance may cover the cost of Oxygen Therapy; a visit to your doctor may save you a lot of cash.

Or Credit.

Or at least make a dent in that amazing “Deductible” your insurance plan has.

It is available in canisters from Amazon, convenient for use or trial purposes.  At $27.99/can, you’re paying for convenience.   As I said, I haven’t used these yet, but just hit “Buy It Now”, and I’ll get back to you in a couple of days.

Screenshot-2017-11-9 Amazon com oxygen

“OH-two”  can also be delivered by oxygen concentrator/generators, available in “portable” and “home” versions.  These devices compress atmospheric air, remove nitrogen, purify the gas, and deliver oxygen that is ~85-95% pure depending on flow and other factors.  These devices are expensive, but in the world of chronic pain that can be associated with ICPDD, they may prove to be literal lifesavers.  In literal 20/20 hindsight, I realize the one treatment I never tried for Trina was oxygen therapy, and that shoulda/coulda gnaws at me.

But, hey, I’m just a disabled dentist with a hole in my head.  What the heck do I know.

Only what I’ve learned from this incredibly disabling shunt that has both relieved a number of neuroendocrine disorders and a lot of fibro pain – at least until end of June when it and my personal physiology decided to part ways.

And that’s why we’re here.

Last thing, at least for today:

 

SLEEP THERAPY – NOT AN OPTION

snoring

           

The previous measures discussed so far are more aimed at relief of immediate symptoms.  Now we need to look at a very important interceptive/preventive measure that is aimed more at long term control of not only ICPDDs but other significant health issues as well: Sleep Disorders.  There is an intuitive link between Sleep Apnea and ICPDD, but the research at this point is limited and inconclusive.9,10

Doesn’t mean there isn’t a link, but if I had to guess…and in the end, ICPDD or no, Sleep Disorders carry their own serious issues.

I can’t emphasize this point enough.  If you have an ICPDD and have not had a Sleep Study to check for a Sleep Disorder, a critical part of your potential treatment may be overlooked.  There is a clear link between IIH and decreased levels of oxygen and increased of CO2 in the blood.  Our bodies can store a certain amount of oxygen in the tissues for use when the need is high.  There has even been some evidence that resolving sleep apnea can resolve symptoms associated with IIH10

During sleep, our bodies assume a lower level of activity.  Breathing, heart rate, blood pressure all drop.  So, to, do reserves of oxygen, especially in the presence of a sleep disorder that can range from light snoring to severe sleep apnea where breathing actually stops until CO2 levels build to the point that the patient wakes up, gasping for oxygen “rich” air – possibly several times a minute.

Proper diagnosis of a Sleep Disorder currently requires a “Sleep Study”, where you are taken from a familiar environment to a strange one, with a strange bed, have all sorts of wires and bands and sensors attached to you, cameras aimed at you, and told “go ahead and go to sleep”.  Home sleep studies are becoming more common; your doctor prescribes such a study, a monitor kit is mailed to you, you follow its directions at home, send the monitor back, and results return to your doctor.

However, if you have a smartphone and about $31.99, you can get a very accurate idea of any sleep disorder you have.  I need to warn you, it might scare the heck out of you.  Even if this has no effect on your ICPDD (not likely, IMHO), sleep disorders are also linked to increased blood pressure, stroke, type II diabetes, cardiovascular disease, weight gain, and other serious health disorders.  By the end of next week, you can do a self-diagnosis that you can take to your doctor.

I found this fingertip oxygen sensor on Amazon for $31.99   It records oxygen levels all night, and the results can be downloaded to a computer the next day.  It also has an alarm that can be set to go off if your blood oxygen level falls below a certain level.  My O2 level at rest is about 98%.  I set the alarm to go off at 89%; it woke me up 6 times that night.

Screenshot-2017-11-9 Amazon com CMS 50D+ Blue Finger Pulse Oximeter with USB and Sofware Sports Outdoors

As a reference, if I were supervising a sedation and the patient’s O2 level hit 94%, I would start to be alarmed.  89% is danger territory.

snorelab-record-your-snoring-logo-fit-wX-150Next tool is a phone app called SnoreLab.  It is designed specifically to record snoring, loudness, and duration.  You download and install it on your phone (last I knew it was a free app), perform the setup, hit “start” and place your phone face down next to you in bed.  Next morning you hit “stop”, and you’re presented with a graph showing when you snored, how loudly, and you have the ability to listen to see if you stop breathing…also called sleep apnea.  If you do, or if the oxygen monitor shows significant drops in oxygen levels during the night, you need to take this information to your doctor, continuing the monitoring process as many nights as you can until your appointment.

Based on the results of this oxygen monitor and SnoreLab, I recently purchased an adaptive CPAP machine.  Recent events have precluded me from putting it to use, but tonight, 11/9/2017, I’m going to start using it.   I’m also considering adding an oxygen generator.  I’ll let everyone know what the results are.  Regardless of my results, I strongly encourage anyone with and ICPDD who has not already had a sleep study to at least try this relatively inexpensive but potentially hugely important test to see if you possibly have an ICPDD.  If you do, run, do not walk to your physician with the handy printed results.  Let everyone know how what the result of any treatment is on your ICPDD via this group data mining, and maybe we can make some serious progress into treating this disorder.

There are a few things I can do that involve prescription medications,  but I am not exactly sure how to present that.  Those will require an entire page unto themselves.  There is controversy about how certain medications might affect ICPDD patients and possibly give emergency relief.  When I am comfortable presenting this, I will put it out there.  Until then,

 

The Shunt Whisperer    2015-05-24 12.05.52

Longing for Leloo

Nov. 9, 2017

 

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3707935/

http://www.acnr.co.uk/pdfs/Case%20reports/PapAJLcasestudy.pdf

10  http://jcsm.aasm.org/viewabstract.aspx?pid=28230

Let There Be No Doubt…

…about how serious Intracranial Hypertension and the group of related/associated conditions I refer 15482560-brain-pain-in-blackto as Intracranial Pressure Dysregulation Disorders (ICPDDs) are.

The toll they take on aa patient’s function and quality of life.

Or, if left untreated, the threat they pose to your life.

Ever heard the term that something “crushes your spirit”?  Guess what.  That is quite literally what ICPDDs can do.

I’m taking a brief moment from preparing the Celebration Ceremony for my wife Trina.  We were a couple that shared this rare disorder.  I know what happened to here.

In the name of trying to create a new sense of urgency in the medical community and remove any doubt that ICPDD patients need early, definitive, and meaningful intervention, I will be focusing in the near future on how elevated ICPs physically affect the brain as well as the potential results.

Trina died as a result of being afflicted with a rare disorder at a time when it is just being recognized by Medicine, when physicians who dared step in and treat her are few and far between, and resources are equally scarce.  Despite active promise of help, time just ran out for her.  Make no mistake: she was no quitter.  She was as tough as they come; she endured things that would have easily broken me.  The fact that it her condition ultimately took her from us is stark testimony to how seriously these conditons need to be viewed.

ICPDDs need to be seen in from a new point of view, and in Trina’s memory and honor, it is now my life’s work to answer this call and help all of us.  For now I mourn, but ShuntWhisperer.com is about to become very real.

May God Bless Us All.