Living with a diagnosis of pseudotumor cerebri/idiopathic intercranial hypertension

After being diagnosed with this disease in October of 2008, I was surprised and dismayed to find so little information on Pseudotumor cerebri.  Sure you can find medical reports and statistics, but the human side has been lost.  Nowadays there is a lot more information out there then there was then.  Reason maybe being it's rarity, less than 1 in a 100,000.  Although these numbers have grown in just the two and a half years I have had it.  Another reason may be the fact they still do not know the cause.  

It is primarily found in women of a child bearing age who are obese, although I personally am not over weight.  There are also young men and women and older men who are getting the disease, although the numbers are considerably lower, they exist non the less.  It has many stages and treatments.  I would like to touch more on the daily living of the disease and the ways I have found to alleviate some of the symptoms.  Maybe give you a heads up of what to expect in the months and years to come.

I started to notice a headache one day in October, I had awoke with it and could never really seem to shake it throughout the day.  The next day it was still there and getting worse it seemed.  Not being one for having headaches and being pregnant I put if out on stress.  The days that followed it continually got worse, I was puking every morning and I literally felt like my head was going to explode.  It wasn't like any headache I had ever had, it was a pressure like  your head was in a vice.  When I finally went to the emergency room, I had not been able to keep anything down for days and I just wanted someone to screw a hole in my head to relieve the pressure.

The first hospital I went to was CMAC of Charleston, WV.  I have no qualms putting their name out there as a place not to go for this disease, maybe their treatment would be better than their diagnosis skills.  But alas I will never put myself in their hands again.

I won't describe the horrid treatment and unethical behavior, however I will touch on the mis-diagnosis or maybe lack there of.  First I was told it may be menegitis, then was given a lumbar puncture.  The position of this procedure is imperative to the proper diagnosis, which is lying on your side.  They were trying to give me one hunched over with an ever growing belly.  After about an hour of failed attempts and Oops I keep hitting bone, I was pretty much done.  I was given a half-hearted talk about checking cultures, which was pretty much bs and sent home.

I felt a bit of relief from the headache, the seven failed attempts had produced a small amount of fluid thus the relief.  In hind sight the pain caused to my spine from repeated puncture may have also lessened the pain in comparison stand point.  However the next day I awoke to the same pain.  Being literally scared to go to another doctor, due to treatment at aforementioned hospital, I stayed home another week in pain.  I couldn't eat, sleep, just lied there with my eyes closed.  I have never been a baby for pain, but the most I have ever felt in my life and I have birthed two children.  So i know, the pain is very real and excruciating.

In desperation I went to another hospital after two failed attempts at others.  I went to Cabell in Huntington, Wv, hands down the best, although I may be a little biased.  I was given a cat scan first to look for tumors, as this is what all of my symptoms would indicate.  Seeing nothing there, they gave me another lumbar puncture.  Although usually being accompanied with Valium due to the fact of false high pressure with anxiety, I was pregnant and was unable to go that route.  They basically have to take a pressure sample of your spinal fluid through your back, although still not quite sure how that one figures out.  My pressure was high, although I could have told them that and the diagnosis was made.  

The first time I heard Tumor and cerebri which I knew was the head, I was scared.  I had  a child growing inside of me and I felt like I was just handed a death sentence.  The doctor, I wish I could remember her name, explained a bit of what it was.  I was told to look up the disease when I got home and urged to stay away from forums.  She also put me on Acetazolamide three times a day. 

So after getting the diagnosis, a visit to a neurologist will be the next step of your journey. It shouldn't take long for this to occur, my visit was actually 3 days later. There he will surely do some blood work to check your levels, depending on the medication prescribed, will debate what they are actually going to be looking for. Mine being Diamox, my doctor checks my protein levels for liver function approximately every 3 months.

When in the neurologist office, they will give you a battery of test. To me, it is almost like a sobriety test. They will check your reflexes, coordination and legs for swelling. I believe for another side effect of the disease. These are all painless test that usually in whole take about 20-30 minutes. Your doctor will also want to check your optic nerve, which entails the light close to the eyeball while you stare off in one spot. They actually have to dilate the eyes a bit and look behind the pupil. Of course there will be questions as well about side effects , nausea and sight or vision problems. You must understand that although the pain is in your head, the real danger is the pressure in your eyes, kind of like glaucoma. Although I am sure death would be an end if uncontrollable, the main thing to worry about from what I have researched is widening blind spots, Papilledema, or worse case scenario vision loss and blindness. Hasan Ercan at Cabell hospital in Huntington, Wv is who I use, saying this to simple add he is wonderful!

Next will be a visit with an Opthamologist, this one will take a few weeks from my memory. In my experience, this visit will not be quite as pleasant, not really painful but uncomfortable none the less. They will first dilate your eyes with a yellow mixture, then they will check the pressure of your eyes. This will be done by a nurse in which you will rest your chin on a strap and they will blow air very close to your eyeball. I have a tendency to pull back when things comes towards my eyes, so I actually have to have another nurse hold my head in place. After a period of time in a room waiting for your eyes to dialate, you will be taken in to another room. They will then do a field study, which will entail looking in to a screen with one eye covered. You will be told to push a button every time you see a light speck. This will gauge your peripheral vision and any loss there of. They will then do the other eye the same way, in my experience it is usually 10-15 minutes for each eye. After that will be another machine you stick your eye up to, where they will take a picture of your optic nerve to look for any permanent damage there. Next, you will see the actually doctor you are there to see, he will talk to you for a moment while going over your results. Hopefully you will not get any bad news. If you do, you go to the next step of the disease, you have made it this far you can do it.

Like I said, none of these are particularly painful, however your eyes will feel strained and sore. The dilation will obscure your vision markedly and light will be very uncomfortable. Having another person to drive you home is optimal, however I have always driven myself home after these visits. Usually, with no pressure on eyes and everything looking good, you can expect to have these appointments every 6 months or so. If you are not during all of these test and at least every 6 months you are jeopardizing your health and your eye sight in particular. Find a specialist that you are comfortable with, but will also go through all of the steps that are needed.

You may also be sent to your local hospital to get a MRI-V.  This will be to look at the vessels in your brain to see if there is any areas of concern.  I would consider taking a couple of Ibuprofen before you go, the test will last 45-60 minutes.  You will have to answer a few question  about any metal you may have in your body, old tattoos, etc.   After changing in to a gown, you will get in to a large circular machine on a rolling tray.  The first part is around 30-40 minutes, you will have to remain perfectly still and a loud piercing sound will come from the machine.  Second part you are rolled out and dyes will be injected.  Back in you go for another 10-15 minutes or so.  

Some will have to get emergency and/or periodic lumbar punctures. From your side of it, I am sure you have already had one since that is the tell tale test. Again, find a capable person to do this, I have had good ones and bad ones and there is no need for any extra pain. Do not be afraid or embarrassed to speak up if the local is not deep enough in, or if the pain is too much. Usually a Valium may be given through an Iv, if you are pregnant a pain medication may be given through an Iv.

A stressful situation will cause headaches due to the raised cerebrospinal pressure which may lead to a emergency room visit. Know your hospitals and always stick to one you trust. I have had one of these episodes in the past and was given a pain medication through an Iv. Some doctors will opt for a lumbar puncture, although if you are not privy to these on a regular basis and no vision difficulties are present, you can always request the less invasive treatment. This is your body and your health, research your disease as much as you can, be proactive. I also recommend keeping a notebook of questions for your doctor, the average time spent with your actually doctor is 15 minutes, so make the most of it. Thoughts and questions will go through your head the months in between and I have found it is good to have them all written down.

First and foremost take control of your health, know what the steps of the disease are and where you are in it. I personally would be in the second phase of the disease as far as medical intervention, although I have researched the progression to keep myself aware and cautious. The steps are as followed.

1. In some cases the first lumbar puncture will alleviate the excess pressure to end their symptoms.

2. Oral diuretics-acetazolamide (Diamox) --Furosemide

3.Optic nerve sheath decompression. This is primarily used for vision loss and eye pressure. This is a surgical intervention to increase blood flow to optic nerve

4.Cerebrospinal Fluid Shunt. This is the last resort technique, it is usually very effective in short term relief. However long term the statistics are high for further surgeries.

There are several other forms of medical interventions that may occur and they are coming up with more as the disease becomes more common. If you are at the earlier stages of the disease there is some things you can do to try to stunt to progress or in some cases put it in remission for good.

If you have any excess body fat that is possible to shed, it is imperative that you do what you can to do so. Extra weight brings extra pressure, this is usually why most of the cases are over weight or obese women. More than not, cases spring up at a time of weight gain. This is your best chance of putting the syndrome in remission and hopefully for good. Last time I checked it was 1 in 3 will experience remission with a severe enough weight loss.

For pain relief, in some cases migraine medication will be prescribed. OTC ibuprofen and/or tylenol can be used for dull minor headaches. However please keep in mind that taking OTCs more than twice a week will cause residual headaches. I have found that wrapping a scarf tightly around my temples for about 15 minutes or so, can do wonders sometimes.

Try to change your diet to lower your salt intake, this will help stop water retention. I have also read articles recommending to lower your fluid intake as well, I will neither confirm or deny. I worked with a specialist from Arizona named Dr. Foster who wanted to use the concept she used for migraine patients on my condition. The basis was a diet that was low in adrenaline foods such as citrus, cutting out most premade food to relieve yourself of a lot of chemicals. Paired with at least 30 minutes of exercise daily to raise your seretonin levels and magnesium. I will admit this is a hard diet to stick to but if followed strictly I believe you could really find a benefit in this as far as your headache and your anxiety levels. I found results in as little as 2 days. In adding all the whole foods and cutting processed foods, you will also see a very quick slim down of your figure. I have provided her link below because I do believe this will benefit you.

I hope that this was helpful for you in one way.  To either let you know that you are not alone or to give you a useful tip or two.  There will be times waking up that you just wish the headache wasn't dull and throbbing  or you don't have the nausea.  Just take that to drive you to the weight loss you may need to put this in to remission.  If you have done this and you still have symptoms, just take it day by day.  Every day try to make it better, by lowering your stress and being mindful of the affects of what we put in our bodies.  I urge you to constantly look for new information on this disease and learn anything new that may help you.  My best wishes to you.