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…

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

  1. Thank you for writing this article. Perfect words to help my loved ones understand what’s happening in me. Although I rarely complain, it’s always a struggle to be “me.”

  2. Thank you so much for this well written article. As a sufferer of PTC one of the hardest battles is to explain this condition in a way that gives the listener a clearer understanding of the trials and pain the patient has to endure. I can see it in the eyes of people I love as well as hear it in their tone when speaking about why I am not who I once was. Many blaming the “new me” on my personal daily life choices. That I lack interest in relationships. That I’m not really sick and am hiding what’s really my problem. To be told “it’s not like you have cancer” or “but you did this and that just last week” in an attempt to dismiss the condition as anything more than just a headache and I’m trying to get out of responsibilities. I wish so badly that I could just clean my whole house and not feel like my eyeballs are popping out and I’m having a stroke. To be able to see even half as well as I used to. A healthy person cannot tell a sick person how they feel anymore than a rich person can tell a poor person what it is really like to suffer financial hardships. This article, among others, have truly helped me and my family. Thanks you!

    1. Malory: your comment is one of about a dozen that just showed up in my WordPress Inbox, some tech glitch, so apologies for not getting back to you sooner. It is so frustrating to feel so bad and seemingly not be able to get help. You are so right that there is a disconnect between those who are well and those who have an invisible illness. I’ve been dealing with this issue for 11 years; friends and family alike dismissed both Trina and I as malingering addicts – some as they toked away on fat joints. It took me getting a hole drilled in my head and Trina to become so damaged and broken that she was driven to suicide before some of them began to understand. It’s easy to be angry, but honestly I’m so grateful for renewed relationships – its so tragic they came at such a price. I truly pray you find the support & relief you need, and again I apologize for the delayed response.

  3. I have suffered with this illness since I was 13 years old. My parents were my warriors and kept me fighting what I felt was a losing battle. Obviously as a 46 year old woman I now know I was wrong about some parts but not others. For years I had been told I was being over dramatic and making things up. My parents believed me because they lived it with me. The outside world was another story. Things started to come together once visual fields tests spinal taps confirmed I was in danger of losing the sight I had left, and my life. I’ve fought a long battle and have to admit with a you can do anything attitude I’ve beaten the odds. I’m still suffering and will be until the illness takes me from this earth. I just wanted to thank you for putting into words what I have never been able to do and have the proof to back it up. I have been told that I’m the longest living survivor on record with the least amount of disabilities or at least the most independent. I don’t know about all that nor do I care. I just really want to that you for doing what I haven’t had the strength and/or patience to do.

    Dr. Stacey Landau-White

    1. Dr. White, first let me say you are amongst a dozen or so comments from February 2018 that just popped into my inbox today. Your story is an inspiration to say the least. I’ve been fighting this since 2007 but was only diagnosed in 2017. Some aspects of my lifelong health and information from my mother about my birth make me think I’ve had some component all of my life as well, but only very ill since 2007. Many others here tell similar stories of having their symptoms dismissed because of a lack of broken/bloody bones protruding from somewhere in their bodies. A similar issue exists for patients in chronic pain. I thank you for your kind words; it is feedback from others such as yourself that give us all strength. Prayers to you and thank you for your comment.

  4. When I found this article I cried. I cried that someone understood me. I cried that I had a resource to help people understand how I feel. Thank you for your beautiful words!

    1. Thanks for your comment, E. The fact that most patients are female is an enormous factor; linking the reason(s) is the challenge for ultimately preventing as well as early, appropriate intervention as opposed to current protocols that leap to CSF reduction/diversion with apparent regard to the degree CSF is the cause or merely a symptom of increased ICP.

  5. 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.

    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

  6. 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.

    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.

  7. 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.

  8. 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.

  9. 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!

    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.

      1. Yvette- It has become very clear that with the progression of closure of my craniotomy, an unwanted CSF around the proximal catheter that is inserted into my brain is rapidly being plugged up. My shunt, previously turned up to its maximum, along with the shunt assist/anti-siphon device, have an enormous combined system pressure. Predictably, my high ICP symptoms are returning. Temporarily without benefit of a neurosurgeon, I asked my PCP to prescribe acetazolamide. Based on a conversation with another Shuntwhisperer reader, I started cautiously at 1/4 tablet 4 time daily to assess my sensitivity to this drug. Turns out I am VERY sensitive to it, feeling the effects of the first dose within 30 minutes.

        Acetozolamide has a long half life; one dose takes 5-7 days to clear the average human metabolic process. Thus, blood concentrations will continue to rise at a given dose for 7-10 days as the drug is continually ingested more quickly than it is eliminated. The first two-three days were actually good, but on day four apparently my blood level of Diamox hit a critical level, and I started feeling “overdrained”. I had stopped taking it for about 36 hours because the lethargy and brain fog were worsening.

        Then the Great Heat Wave of The Summer of 2018 hit, and the perfect storm of diamox, altitude, and heat immediately pulled the pug on my head. I laid around like a vegetable for four days. I finally pulled myself together enough to get in the car and drive to a relative’s house located 30 miles and 800 feet lower in elevation. By the end of the 45 minute trip I was much better, within 4 hours as good as I get.

        So, the answer to how Diamox “wrecked” me was a personal sensitivity to it, its long half life, combined with other environmental variable to leave me barely able to get out of bed. I considered it a learning experience and keep it around to help “manage” symptoms until I can find a new NS. I can’t imagine being on 1 or more grams a day, and I have serious concerns about the wisdom of treating ICPDDs with CSF reduction: if CSF is such a critical substance, key in providing nutrients to the brain as well as cleansing dead cells from the brain, and if inadequate CSF is associated with dementias, blanket treatment of ICPDDs with Diamox seems a resort of convenience that some researchers fear could predispose patients to dementias.
        Unless it can be shown that an ICPDD is primarily caused by a overproduction of CSF, it seems appropriate to treat the actual cause of elevated ICP instead of a secondary symptom; CSF pressure can be the result of other factors which should be addressed where possible.

        Long winded response, but that’s the story of how Diamox “wrecked” me.

  10. 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

  11. 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.

  12. 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💚💙

    1. Have you looked into Ehlers-Danlos Syndrome? EDS people very often have IIH this is a genetic disorder. I have been treating my IIH with physical therapy and cranio-sacral for years. My Craniosacral person is a license physical therapist that spent much of her career working with traumatic brain injury and she is covered under insurance. I hope this helps.

    2. Cyndria: My heart goes out to you and and your children. There is an apparent genetic predisposition, and if you ever discover what it is, please share it with us. I briefly reviewed New Vision Therapy and Chris Duquemin and find the basic theories sound. As has likely been explained to you, there is a great deal that happens in a a space known as the Carotid Triangle of the neck; it contains the Carotid Sheath, a bundle of tissue including the Internal Carotid Artery, the primary source of blood to the brain; the internal jugular, the primary pathway of blood drainage from the brain, as well as the vagus nerve and the Omohyoid and Sternocleidastoid muscles. In one tissue compartment lies the pathway of blood in and out of the brain. Directly behind this space at the levels of C1 & C2 are lateral bony tubercles from those vertebrae; injury or malformation, even posture can compress the contents of the carotid sheath against solid bone, interfering with cerbrovascular dynamics of the brain as well as vagal nerve function.

      Decompression of this compartment by stretching, massage, and craniosacral therapy can only help. I believe I understand what New Vision is trying to accomplish. By all means, continue your efforts; I must be cautious in allowing mention of crowdfunding efforts, and I can’t endorse them, but I can approve your comment and let it be available here for others to read. May God Bless your family and your efforts, please let everyone here know what transpires; ultimately, Shuntwhisperer’s goal is sharing of information that might provide answers to others : #CROWDHEALING

  13. I really really REALLY want to thank you for writing this and posting it where we can read it. I’ve been able to share this with family and friends and it has drastically changed their understanding of this very serious illness. You have helped me beyond words can express. Just wanted to say that to you. Much love to you.

  14. Thank you for sharing the reality of living with Intracranial Hypertension. It’s a serious condition that (in my opinion) should be taken very seriously! Here’s to hoping for a cure one day <3

    1. Here’s a second to that hope. The reality is that medicine is only beginning to get “up to speed” on this class of disorders, but when the right person or group decides to dedicate their career to it, all aspects of diagnosis, treatment, management, and possibly even prevention will improve rapidly.

  15. First I would like to say thank you. Ive tried for 12 yrs to get family & friends to understand what im feeling, & most of the time it just leaves me feeling alone. So alone all the time. I do have family that TRY to understand but how can they when im.so limited on words or forming sentences. And as i read this for the first time I didnt feel alone, i felt like SOMEONE ACTUALLY UNDERSTOOD & maybe now my caregiver & family can too. I am seriously sending this to ALL my family & my caregiver. I love your name for it so much more than IH or PTC or BIH, or IIH, or SIH. I wish they xould comw up with 1 name & stick to it because how are we suppsoed to be taken seriously theres so many names NOBODY calls it the same thing or knows what to call it.
    The only thing i would add to the symptoms is that also when we cry it raises the pressures/pain & so does anything that raises your blood pressures raises your ICP as well.
    But this is seriously the best thing ivw read about IH ever & just wanted to say Thank you. Thabk you for writimg ir, Thank you for caring for your wife so much, & most of all Thank you for us who feel so alone in this fight & who are starting to doubt ourselves for showing us WE ARE NOT ALONE & PEOPLE REALLY DO UNDERSTAND & ITS NOT ALL IN OUR HEADS OR OVER DRAMATIC LIKE WE ARE ALWAYS TOLD. THANK YOU FROM THE BOTTOM OF MY HEART. I was so relieved when i read this that i couldnt help but cry

  16. After reading your post I am compelled to clarify some information that is misleading. The serum peak half life of Diamox in tablet form is 1-4 hours, extended release sequels peak at 4-6 hours. Slowing the production of CSF at the choroid plexus and acting as a diuretic is the ultimate clinical indication of Diamox use in patients with Intracranial hypertension/PTC.
    Diversion of CSF with shunt, or slowing production of CSF being produced are simply two means of intervention with the same ultimate goal of lowering ICP.
    I had been a high dose user of Diamox for four years with successful symptom management, even after stent placement. Most recently received a VP shunt, yet remain with Diamox use at lower dosing. I have never suffered any debilitating SE with Diamox use, and literally credit it with allowing me to live and continue to work with several rare illnesses.
    Intracranial hypertension is a subtype
    of hydrocephalus, both physiologically presenting as an abnormality of CSF dynamics. Ventricle enlargement the defining difference, as well as the underlying etiology of CSF blockage.
    I am now a five year rare illness patient living with Intracranial hypertension, a patient of Dr. Liu with both stents and a VP shunt. Diagnosed with Chiari four years ago and EDS as well. Chiari decompression did not resolve my elevated ICP, nor correct the alteration in CSF dynamics but knowing with certainty which came first is unclear.
    I agree there is linited knowledge regarding treatment and physiology of this disease, but we must be diligent that information being shared is clinically sound. There are designated CSF clinics to treat this illness and continued research that is ongoing. I have been a traveling gypsy for treatment, and I have never encountered any specialist referring to this illness as ICPDD. The IHR foundation is an excellent site for accurate information and updates on current research and treatment.

  17. This puts everything into perspective for me. I was just diagnosed about three weeks ago. Im about to have major brain surgery. I’m only 21. It makes me realize the changes that I’ve been seeing in myself are normal for my condition. This makes me feel like I’m not crazy, like I don’t have to have excuses for why I can’t do something. It makes me feel better about not being able to be a normal almost 22 year old. Thank you so much for writing this. It’s helping me so much.

    1. MJ: You are far from being alone in feeling confused about how you feel. Disorders that are not readily apparent seem to be frequently dismissed as unimportant or nonexistent. Further complicating matters is that unless another person has experienced your pain, it’s not likely they understand what you’re experiencing to any significant degree. This is common in all chronic illnesses, but more pronounced in “invisible” conditions such as these. I know all too well how disheartening this can feel; find all the help and support you can. Don’t apologize for feeling poorly, and try not to let your very real misery get the better if you. Find your anchor that you will need in challenging times.

      You’re young and will likely see rapid improvements in management of this class of disorders. The beauty of the Information Age is that knowledge is just a few clicks away, but only if we can sift truth from the many sources. You might consider a career in some area of Medicine where you could use your personal experience to help better understand and manage these disorders. A shining example is Dr. Diana Driscoll, whose work is showcased at PrettyIll.com.

      This last bit of advice is probably the most important I can offer: Pray. For yourself, your doctors, for everyone coping with this disorder. I have felt its power in the last year and found strength that has filled in where Medicine has not been able to. God has been my anchor through this disorder and losing Trina, and I know He will ground you as well.

      Thank you for your kind words. I’d encourage you to start a blog/site of your own. Shuntwhisperer is based on my personal experience, observations, and theory, but is far from a definitive work. My goal is to start conversation, offer information that might “click” in someone else’s mind, and just possibly, make their path a little easier. Prayers for good days and positive outcomes for us all.

      Wes
      Remembering Trina during Advent

  18. My son was recently diagnosed about 6 weeks ago. It has been a whirlwind of appointments and running here to there with no real understanding of what is going on. Thank you for this article. It has been the most informative thing that I have read. I have shared with family and close friends because I have had no idea how to explain it. Quite honestly, I was completely lost. Thank you for taking the time to educate.

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