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.  I have experienced everything I am conveying here with complete honesty and sincerity.

Forget what you  think you know and start by understanding 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.

It just doesn’t show on the outside.  No bloody bones protruding.  Just the person telling you “I don’t feel well!” and acting strangely, which probably alienates you.  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.  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.

To say that pressure on the brain is a problem is understatement.  The brain 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; in extreme cases, the brain and its associated structures such as optic nerves can be physically damaged by prolonged high pressures, sometimes irreversibly.

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): litereally, 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).  At some point, it was noted these patients exhibited abnormally high ICPs when tested by lumbar puncture.   A short list of 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, 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.

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.  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 to varying degrees, 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.

Give them love; give them space.  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

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

14 thoughts on “A Caregiver’s Thumbnail Guide to “Intracranial Hypertension”

  1. hello, I was diagnosed with IIH. I would like to translate your entry into Polish and put it on my blog. Can I? Of course, I will write that You wrote it and I will give a link to the original.

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    1. Please feel free to translate and share this guide; I’m humbled that you feel it is worthwhile to share. I’ve been away from my site several weeks wrestling with trying to reassemble my life and dealing with my grief, and praying for guidance. I believe those prayers have been answered, and as such I hope to bring new information to ShuntWhisperer and unite our community. I would welcome your help and suggestions as well; this is not about me; it’s about helping everyone who has this disorder as a service to God. Prayers and comfort to you, Kruzelosia, please stay in touch.

      Wes

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  2. Please contact me! I am currently working on getting my data together so that my NS and NO can possibly present to DR. Wilson… dont give up hope!!!!

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  3. I have no direct experience, but in an appointment with my Neurosurgeon yesterday he mentioned some of his patients had done just that – moved to Arizona – with good results. More stable weather, less humidity.

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    1. Yvette, your question has sparked a new post; it touched on a set of factors that I’m investigating for connections to factors that relate to your question. Look for “Yvette’s Question” under “Topics”, and remember, there is always hope.

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  4. Hello my name is Joanna Isiah and I recently had an lp my opening pressure showed 25 I had relief for 2 hours and then all my pressure came back. I am so worried and need some advice I do not know what to do at this point? I go back next month to see my neurologist im just afraid that my choice may be the wrong choice. Is a shunt something I really need? I feel like I’m getting worse how do I know when’s the right time? Any advice you have I’m all ears at this point thank you.

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  5. Joanna: I apologize for not getting back to you sooner; I’m in the process of moving and thus have been neglectful of checking for responses and comments; I’ve remedied that so that now I will get immediate notifications when someone posts a comment. Now, on to your situation.

    You had an LP with an OP of 25; your relief lasted only 2 hours. When the LP was performed, did the procedure also drain a large portion of CSF? 25-30 cc? Or was the procedure merely to check your pressure? If a Large Volume LP was performed, you should have enjoyed 36-48 hours of relief before your CSF was replenished. A mere 2 hours of relief leads me to wonder if no CSF was removed, or if return of your symptoms was due to overdrainage. If you feel comfortable posting your symptoms here, your case, like mine, can serve as a learning experience. If you’re not comfortable with that, I will send you my contact info and we can converse privately.

    First of all, IMHO pressures measured during an LP are important, but not as important as your symptoms and findings on MRIs and CTs. The pressure values used in medecine to determine “high” or “low” Intracranial Pressure (ICPs) are arbitrary; they have no basis in science. Furthermore, there is a growing body of evidence that in patients with ICPDDs such as IIH, these pressures vary greatly day to day, even during the day. These variations are likely normal, but for some reason, ICPDD/IIH/PTC patients can’t tolerate the changes. I know with certainty that air density changes chiefly related to atmospheric pressure affect ICPs, either directly or through physiologic response. It is a poorly researched are, but essentially all ICPDD patients have exaggerated response to changes in air pressure, and I believe this exaggerated response to be a symptom of ICPDDs.

    Knowing what symptoms you have that were relieved helps determining the state of your personal disorder. Findings such as Empty Sella/Partial Empty Sella, Optic Nerve damage, unusual dimensions or size ratio of the ventricles of your brain are among the important findings, or signs. Gait disturbance/difficulty walking, vision problems, tinnitus, sudden weight change (usually gain) are among the usual neurologic symptoms in ICPDD (Intracranial Pressure Dysregulatory Disorders).

    Advice: Having followed my personal disorder for 14 months since receiving a VP shunt, I can say with certainty that my condition is related to cerebrovascular imbalance: the veins that drain blood from my brain cannot keep up with the arterial blood flow into my brain above a certain pressure and blood flow. This causes blood to back up in the blood vessels in the brain, causing the brain itself to expand. There is very limited space inside the skull, and as the brain expands, pressure is exerted on the CSF both inside and around the brain. This means that the increased CSF pressure is a symptom, not a cause of my particular disorder, and my VP shunt is draining away precious CSF. This leads to what I call the CSF Paradox: Neuro Docs tell me that they do not know exactly what CSF does, BUT it is VERY important, likely cleansing the brain of dead material that accumulates in certain dementias. Then, they turn around and give us Diamox to reduce CSF or shunts to drain it, sometimes using two shunts, which only aggravates the underlying problem. I hope to have a visual presentation on this within a month; I’ve been testing the logic and theory in myself and others, and so far it’s holding up.

    RULE OF MEDICAL TREATMENT: KNOW THE CAUSE BEFORE TREATING. This rule is getting a little bent at the moment because ICPDDs seem to be poorly understood, and devices and methods for treatment are limited, mostly using approaches to treat hydrocephalus in children. Hydrocephalus is a genetic condition in children that allows CSF to accumulate without normal reabsorption, and is an entirely different disorder than ICPDDs seen in adults. Very few neurologists/neurosurgeons actually intervene and perform treatment; the best I know is Dr. Kenneth Liu at Penn State Neurological Services in Hershey, PA. I was told by another neurosurgeon in December 2017 that 90% of neuro docs either don’t believe in IIH or don’t want to deal with it, 7-8% use a conservative approach with drugs like Diamox that can have very unpleasant side effects, and only 1-2% actually do something. Those surgeons, while receiving criticism from others docs that advocate a conservative approach or no approach, are the ones who have a better understanding of treating ICPDDs than the ones that sit on the sidelines.

    FOR YOU: I don’t know where you are in your disorder, but look forward to hearing more from you, and if I have any recommendations, it would be my privilege to pass them on to you. When you see your neurosurgeon I recommend ask for an angiogram to look for any evidence of kinks, blockages, or stenosis of any veins that drain blood from your brain. This goes back to attempting to find the true cause of your problem before undergoing any treatment. Listen to your neurosurgeon’s opinion, but don’t feel pressured to do anything without a chance to think about it (unless it is a true emergency). If you are having severe symptoms such as damage to your optic nerves or daily, constant migraines, it may be necessary to try some of the medications or have a shunt placement to maintain your health while understanding and treatment of ICPDDs evolves. This is not an easy disorder to deal with, but always, always know there are many who suffer from it and are ready to support you.

    LAST: ShuntWhisperer has always been about telling my story for the sake of others like yourself, who face a disorder with serious consequences but for some reason is poorly understood. I went into treatment believing that my shunt would give me the relief I received from my LP, and while I did get an enormous reprieve from my fibro pain and exercise intolerance, I also ended up with a hellish swing in emotions and moods as a result of the change in my CSF dynamics. That said, as terrible and awful as my last year has been (and it has been indescribably horrific), I count myself fortunate to have been put on this path; I have been able to make observations that I hope can help others, but I do not believe everyone has the same type or degree of disorder. This next part is crucial: When I was dealing with the adverse effects of the shunt, I would lay in bed and recite the Lord’s Prayer over and over; I held my wife’s hand, and I kept a vision in my mind from childhood of feeding lambs with a bottle with my grandmother in my mind’s eye. Don’t place your faith in doctors, because they will ultimately fail you. Don’t place your faith in me; this site is my service to God, but I’m just a man, or as I joke, “a disabled dentist with a hole in his head.” Place your faith in God, pray, ask others to pray for you. If everyone here prayed for each other, it would make a difference. Establish a solid foundation, a lighthouse that you can always look to if the disorder becomes difficult to manage.

    Prayers and blessings to you and your family.

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  6. Hi,
    Thank you for the helpful article. I shared it with my family. Thanks also for the weather post.

    Regarding Dr. Liu, I was curious what you mean by him “actually doing” something – do you mean shunts? Or what does he do differently from other neuros? I currently see one at Johns Hopkins but don’t feel advocated for. But I live near Hershey and would be thrilled to see someone else who comes recommended. The fact that he acknowledges weather affecting us is a great first step.

    I don’t have a shunt yet, was in remission for 2 years but now I feel my pressure pains are back and am looking for a diff doc because mine isn’t doing much since I don’t currently have optic nerve swelling. He put me on topamax but I’m afraid to try it because Diamox wrecked me before I went into remission. Just thought a second opinion could be good. Maybe Dr. Liu…thanks!

    PS: love the Shunt Whisperer name!

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    1. Sabi, I was told by another neurosurgeon in late 2017 that 90% of neurosurgeons/neurologists will not treat IIH/ICPDDs. 7-8% would supervise symptom management with drugs such as Topiramate and Topamax. only 1-2% of neuro docs who treat ICPDDs actually intervene in an attempt to either address pathologically increased pressures (shunts), or address the actual cause (stents).

      Dr. Liu is struggling to understand how to best treat ICPDDs, but he is forced to work with what is available. Nonetheless he has as deep experience in ICPDDs as to be found. If you live near Hershey, my opinion is you owe it to yourself to seek his opinion, but educate yourself and ask questions, follow your gut. Prayers and best wishes to you.

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  7. Sorry – I realized that you mentioned Dr. Liu in the post about barometric pressure and weather affecting you, in case that was confusing. I had both tabs open

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  8. Hello. This post captures vividly the struggles and suffering associated with ICP problems. All the material on your site is high-quality, both the technical information as well as the insights offered as a patient. Thank you for taking the time to write such excellent descriptions. So sorry to read about your wife’s passing. Sitting here, I have no idea what to type. What words can I offer to such a fresh and unfathomable grief, except to say how sorry I am that anyone must go through such a trial. Offering prayers for you as you journey through this valley. For as thin as my faith has become, it is, nonetheless, indestructible, having as its object the One who is everlasting, who does not fail, and in whose hands every broken strand is woven through with Love. May you know the comfort of God’s peace today.

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  9. Hello Wes I hope this day you are feeling more peace & comfort. As reminder I’m mom to 3 children with IH. My 2 girls are worse than my son. Their pressure is always over 25 even on meds. My youngest is now a walking skeleton with black bags under her eyes. My family has taken notice & are worried for her & my other 2 children’s very lives. I have met a neuro physical therapist who I wholeheartedly can help with IH & other painful illnesses/symptoms etc that go along with IH in many. He does cranial sacral & reflexology & other technique s in his massages. I have chatted with him via messenger face to face & online. I have talked with a woman in Canada who did a 2 hour treatment with her- she has IH. I set up their visit when he did volunteer work in Canada. Now thru Children’s group another IH mom has chatted with him repeatedly. We ALL believe his methods could be a huge breakthrough!! I have started a GoFundMe Cyndria Alnett siblings battle brain disease to get him here other IH mom fundraising too. Thinking if IH FOUNDATION could speak with him & fly him in he has agreed to treat for free my children & others just have to pay his flight accommodations)food &it takes 4 treatments over about 3 month span. Chris Duquemin from Channel Islands at New Vision Therapy. Jennifer from Canada said he’s amazing as he worked on her vagus? Vein from left neck to stomach etc. Maybe you can meet him & chat? Sending IH love & hugs💚💙

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