NEUROENDOCRINE THEORY FROM AN ENDOCRINOLOGIST – AN EXPERT SPEAKS

Three particular informational sources have played a key role in making sense of disparate symptoms:

  1. The Driscoll Theory by Dr. Diana Driscoll.  Although primarily focused on Ehlers-Danlos/POTS patients and the particular Intracranial Pressure Dysregulatory Disorder these patients often experience – Intracranial Hypotension – especially her observations about the carotid triangle, vagus tone, and cerebrospinal pooling/blockage due to “brain sag”.
  2. Adrenal Fatigue Syndrome by endocrinologist Dr. Michael Lam, MD and Dorine Lam, RD, MS, MPH.  A well written book describing endocrine system interactions as a result of long term, high level stress, how that system begins to become dysregulated, and the hormonal consequences thereof, as well as approaches in dealing them.
  3. Multiple Research Papers that have documented the presence of hormonal receptors in the cells that produce CSF.  In a word, many of these hormones become dysregulated in Adrenal Fatigue/Failure.  Each in its unique way contributes to increases in blood pressure, CSF production – or, as in the case of aldosterone – is responsible for both.  This means increased blood pressure & therefore increased Cerebrovascular Dynamics as well as increased CSF production.  The perfect storm for development of elevated ICP, and if not controlled, an ICPDD in patients with predisposing factors.  Some receptors are estrogen/progesterone sensitive, possibly explaining why patients are more commonly female.   Further,  looking back to Dr. Lam and Dorine Lam’s body of work, it will be interesting to compare the upswing in ICPDD diagnosis with the increase in estrogen-like compounds in everything from food supply to dental fillings.

All these fell in my lap within a two week period in December 2018 while I was hunkeringS down waiting for a shunt revision, and to my surprise, they did not lead me to what I was looking for: why I developed IIH.

Well, sort of.

They actually led me to a conclusion that I cannot emphasize enough, hence the big letters here:

“The Secondary Acquired Adult Onset form of the “disorder” known as IIH is actually a complex symptom of a dysregulated endocrine system, including “Adrenal Fatigue”, which is most commonly caused by chronic stress.  Therefore, I name CHRONIC STRESS to be the main cause of altered/elevated ICP and the onset of ICPDDs, and the ultimate reason I developed an ICPDD.”

Shuntwhisperer’s Neuroendocrine Theory of IIH, Dec 2108

But wait, there’s more:

“Successful treatment of ICPDDs must necessarily involve endocrine function AND CAPACITY* before any ICPDD can be successfully treated.  To treat only the ICPDD is treating only the symptom; without lifestyle/dietary changes and endocrine support can possibly condemn a patient to unnecessary prolongation of their ICPDD.”

Shuntwhisperer Neuroendocrine Theory of IIH Corollary #1 Jan 2019

And just when you didn’t think it could not get ANY better:

“Elevated ICPs cause deformation and damage to key portions of the brain controlling the endocrine regulation, especially the HPA Axis.  The HPA axis must be well regulated to maintain ICP equilibrium.  THEREFORE: TREATMENT OF ICPDDS WITHOUT TREATMENT OF ASSOCIATED ENDOCRINE DYSREGULATION WILL BE SUBOPTIMAL.”

Shuntwhisperer’s Neuroendocrine Theory of IIH Corollary #2 Jan 2019

In other words, endocrine dysregulation that leads to an ICPDD, left untreated, can cause the ICPDD to persist, and more corollaries are coming.

But don’t take my word for it.

Take an expert’s.  I swear, I did not rip this off, but Dr. Lam’s book led me to the same conclusion:

NeuroEndoMetabolic Component Dominance: Stages of Stress

Been a long hot summer day.  I miss Trina.  Leaving you to it.

The ShuntWhisperer

July 11, 2019

 

Stressed-Out-1024x614

 

 

 

 

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

How Intracranial Pressure Becomes Elevated: Part 2, Short and Sweet

Intracranial Hypertension is the elevation of pressure  of fluids and tissue inside the fixed volume of the rigid intact skull.   This elevation of pressure has two basic mechanisms*:

  • Cerebrospinal Fluid Dynamic Imbalance: A net accumulation of of Cerebrospinal Fluid (CSF).
  • Cerebrovascular Dynamic Imbalance: A net accumulation of blood in the brain caused when veins are not capable of draining the volume of blood pumped into the brain by the heart (Monro-Kellie 2.0, Dr. Mark Wilson, Royal College of London, 2016)

*soft tissue tumors can also occupy space inside the skull and potentially raise ICP but are considered a separate causative mechanism from those that are considered in ICPDDs

The cause of these imbalances can be either genetic/developmental (primary) or related to trauma (secondary).  They are closely interlinked and may both be present to a certain degree.

One example of CSF Imbalance would be non-communicating hydrocephalus.  CSF is produced from arterial blood in small hollow areas of the brain called ventricles.  Ventricles are normally interconnected with one another and the space around the brain.   CSF circulates through these areas passively due to the pulsation of blood vessels and also likely movement of the body.  If one or more of these ventricles does not communicate with the rest of the system, the CSF it produces accumulates and displaces the brain outward.

Another exmaple of CSF Imbalance illustrates the interrelationship between CSF and Blood dynamics:  obstructed veins may not absorb enough CSF out of the skull to prevent a net accumulation of CSF, as well as leading to this example of:

Cerebrovascular Dynamic Imbalance: would be Chronic Cerebrovascular Venous Insufficiency, or CCVVI.  In simple terms, damage or constriction (stenosis) of one or more of the veins that drains blood from the brain compromises the ability of blood to leave the brain.  At a certain critical level of blood flow and pressure, blood begins to accumulate in the brain as it is pumped through arteries at a greater volume than it can drain.  This accumulation of blood causes the thin-walled veins to swell, which in turn pushes on brain tissue.  Brain tissue may become displaced as a result, being pushed into areas occupied by CSF.  Since an intact skull is rigid and does not expand, the pressure of the CSF becomes pressurized (Newtons’s Third Law)  while simultaneously trapping brain tissue between a vise of swelling veins.    As focal areas of brain tissue that control specific physiologic functions become stressed, their functions become altered.  This pressure on brain tissue is likely the cause of symptoms associated with IIH.

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…

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

“You’re Fat, and That’s Why You Have IIH. Lose weight and you’ll be better.”

“… what if the weight gain is not the cause of IIH, but instead a symptom?

Ever heard this before?  Stick with me.  I’m on your side.

If you’re visiting this page, statistics predict you’re likely a female, 40-ish, with a “high Body Mass Index” (a number that decides if you’re fat).

Chances are, you underwent a rapid weight gain (40+ pounds) in a relatively short period (4-6 months) for no reason that you could identify.

No secret addiction to double cheeseburgers.  No nightly pints/quarts/gallons of Ben&Jerry’s.

You just started gaining weight.  Somewhere along the line you might have noticed a headache, vision problems, ringing in your ears, or any of a number of other symptoms that ultimately resulted in a diagnosis of an Intracranial Pressure Dysregulation Disorder (IIH/PTC/NPH/etcPH).

And the first thing your doctor does is tell you it’s all because you got fat.

Makes you feel really good about yourself, right?  Especially when loosing the weight requires drastic measures like the South Beach Rice Kernel Diet (where you run to South Beach from where ever you live, eat a kernel of rice for a meal, and then run home.  Daily.)

Trouble is, when you feel like hammered crap, it’s sort of hard to exercise.  My wife Trina and I shared the same PCP.  She had gained weight because she was in so much pain she could rarely get out of bed.  He told her at an appointment over the summer to loose 20 lbs.

“How?” she asked:”I hurt so bad I can’t get out of bed, you won’t treat my pain, and I only eat one small meal a day (truth)?”

His response veritably dripped of the compassion seen in medicine today:  “Don’t pick up the fork.”

Trina wisely let a few days elapsed before she told me this.  Had she told me while in the office, there would have been a serious discussion between the PCP and myself.  As it was, I mustered what I believed to have been a diplomatic tone of truly laudable nature, and on a follow up visit, confronted our “doctor” about this.  “I might have said something like that as a joke” was his response.  “Apologize to her.  Like you mean it.  Please.  You hurt her badly” was my response.

One of the many Medical “Bricks In Her Wall.”

BUT:  what if the weight gain is not the cause of IIH, but instead a symptom?

Our bodies are contolled primarily by a system called the HPA Axis, an interaction between the hypothalamus (“H”), pituitary (“P”), and adrenal (“A”) glands.  Of these three, the hypothalamus and pituitary are closely located in an area where CSF pressure seems to concentrate as it flows from the fourth ventricles where CSF is produce to the third ventricle where it is further distributed.  Also located in close proximity are the optic nerves.

Optic nerve damage from high ICPs are known to occur.

So is “Empty Sella”, or “Partial Empty Sella” Syndrome, where the pituitary gland becomes visibly deformed due to ICPs.

If vision can become impaired when the optic nerves are squeezed and damage, how hard is it to believe that the critical functions of the hypothalamus and pituitary gland (there are others, but stick with these for now) might not also suffer as pressurized CSF physically deforms them?

I have Partial Empty Sella.  My Pituitary looks like a Pancake-itary.  Before my shunt, I had terrible intolerance to exercise.  Exercise damages muscles, and require Human Growth Hormone to be released from the Pituitary during sleep.  Oh, yeah, sleep: the “sleep gland”, or suprachiasmatic nucleus, is a close neighbor…hmmm.  Anyway, after the shunt, wow, voila, most of my fibro pain is gone, and now, for the first time in 10 years, I can exercise without pain.  I point this out to my neurosurgeon, who dismisses it saying “you only need 5% of your pituitary to function,” to which I responded, “Define ‘Function'”.

Amazing.  Neurosurgeons and Neurologists say they don’t know anything about this disorder, but when a patient who has the disorder notices a change, instead of taking into consideration, the usual response is “there’s no evidence of that”, when the evidence is standing right in front of them.

Evidence also points to high ICPs causing hypothyroidsism and generally screwing with a very delicate endocrine system in such a way that a patient so afflicted might start to gain weight.

Yeah.  High ICPs can wreck the critical ability of your body to process and store calories as well as regulate your metabolism.

Then comes the really tough part:  once the fat is present, if feeds the problem by becoming not only a reservoir for hormones, upsetting the “normal” balance, but then produces its own hormones that further aggravate the situation.

Maybe the reason so many IIH patients have problems loosing weight.

Or sleeping well.

Or are intolerant of cold, don’t regulate their body temperature well, begin to develop blood pressure problems, any of a multitude of physiologic functions regulated by the endocrine/hormone feedback between these three glands (like I said, there are others, that govern mood, memory, and so on, right in a neat little cluster).

This research article is one of many that pop up when I Google Search using “IIH HPA Axis Dysregulation”.  It is a case presentation that pertains to the pituitary damage and the results.  The “Discussion” section merits, well…discussion.

Maybe physicians need to realize at the very least that weight in an IIH patient migh actually be a result of the IIH, not the cause, even though the excess weight can, and does, aggravate IIH.

Maybe show a little compassion, actually stop and recognize the distress this disorder causes, and realize that merely pronouncing to a patient to “Lose weight!” without consideration of whether the patient is able to actually exercise because of untreated symptoms of pain and fatigue.

Maybe move on to something more meaningful than…

“Don’t pick up the fork.”

This Topic is open to comment.  Fire away.  Prayers and comfort to all.

 

The Shunt Whisperer

Wishing for Leloo at Christmas…