Sunday, August 23, 2009

My Story

Idiopathic Intracranial Hypertension (IIH) or Pseudotumor Cerebri (PTC)....what is it? Prior to my diagnosis I had never heard of it. Nobody in my family had it. I had no friends who had been diagnosed with it. To me, it was all a foreign language. My family members and friends were equally confused...and I think to some extent still are. In fact, I think it is fair to say that many of the medical professionals I come across are confused as well....some who have never heard of it. So, let me define it for you here....

Intracranial Hypertension is a condition in which the body either produces too much cerebrospinal fluid pressure or doesn't absorb it well, resulting in increased cerebrospinal fluid pressure. It is likely due to high pressure caused by the buildup or poor absorption of cerebrospinal fluid in the subarachnoid space surrounding the brain, but the reason for the buildup or poor absorption are also unknown. The disorder is most common in women between the ages of 20 and 50. And at one time, being overweight seemed to be a contributing factor. "Seemed" being the operative word, as the subject of weight and it's impact on IIH is still being researched. There are many people in the world who are over 500 pounds who do not have IIH and there are very skinny people who do have the disease. There have been patients who have been overweight and dropped thier BMI range down to "normal" levels and still had IIH. Unfortunately, this disease affects men and children as well, of all races, skinny and fat, short and tall. It knows no bounds. IIH affects us each differently. Some of us will have success with medications and some of us will require surgeries. Some of us will go into remission, only to have the disease return months or years later. Others will go into remission and be lucky enough never to see the disease rear it's ugly head again. And, then again, there will be some who will be disabled for a lifetime. No amount of medication, lumbar punctures, surgeries or dietary changes will make a difference. At least, not yet. We hope with more research and funding for a better day.

Symptoms of Intracranial Hypertension are: headache, sometimes daily, sometimes severe, not relieved by medication; hearing loss; impaired vision or eventual blindness; memory problems; nausea; pain behind the eyes; pulsating intracranial noises ("swooshing" sound or heartbeat); shoulder, back and/or neck pain; tinnitus; and vomiting. In addition, patients have complained of swelling behind the ears; swollen lymph nodes; night sweats; muscle spasms; abscence seizures; insomnia; depression; mood swings; and dizzy spells...although not all of these latter complaints have been thoroughly researched enough to be included in the definition of IIH.

Diagnosis: *A lumbar puncture (or spinal tap) is considered the gold standard when it comes to an IH diagnosis. This test measures the pressure of the cerebrospinal fluid that circulates in the sub-arachnoid space, which surrounds the brain and the spinal cord. When intracranial pressure is high, that pressure is transmitted through out the entire sub-arachnoid space. Diagnosis is usually confirmed after a spinal tap reveals a high opening CSF pressure.

*For adults:Normal pressure readings are generally below 200mm/H2O. Borderline high pressure readings are between 200-250mm/H2O.Anything above 250mm/H2O is considered a high pressure reading.

*For young children:Anything above 200 mm/H2O is considered a high pressure reading.

Once the spinal tap is taken and the opening pressure is taken the fluid is checked for abnormalities. Patients with IIH have normal cerebral spinal fluid (CSF) findings. In addition, patients with IIH will also have the following for diagnosis:

  • Normal CAT scans and MRI's
  • No sign of brain injury (No paralysis/muscle damage, etc.)
  • Has signs and symptoms of increased intracranial pressure such as headache and/or papilledema (swelling of the optic nerve behind the eye)
  • Patient is awake and alert
  • Has no other cause for increased intracranial pressure

Treatment: Now that I have given a thorough, and respectfully, a rather boring definition of this disorder let me say here that the incidence of disease for the general population is only 1 in 100, 000. So, by all standards this disease is considered rare. Therefore, we do not have a popular celebrity speaking on our behalf to find a cure. No pharmaceutical companies have stepped up to the plate to find new medications. The same treatments that were used 100 years ago to treat this disease are still be used today. Those treatments consist of medications which were created for other diseases (i.e. glaucoma/mountain sickness) and as such give IIH patients horrible side effects.

Or, for those with severe papilledema...optic nerve fenestration surgery. *During surgery, a small window-like opening is made in the sheath around the optic nerve, which allows cerebrospinal fluid (CSF) to drain behind the eye and relieves optic nerve swelling. This surgery is primarily used as a tool to try and save vision, but does nothing to relieve the pain of daily headaches, neck and back pain caused from increased (CSF) squeezing the brain and spine.

Lastly, and probably most barbaric, are the neurosurgical shunts. Shunt operations are also performed when drug therapy has been unsuccessful. *Shunts may be used to control papilledema and prevent vision loss, as well as to treat headaches that have been unresponsive to any medication. A neurosurgical shunt is a surgically-implanted catheter that is used to drain CSF into another area of the body such as the abdomen. A shunt lowers intracranial pressure by removing CSF to another site, where it can be absorbed. The problem is that these shunts are used on the brain as if we were treating a plumbing problem and not a neurological one. Therefore, there are many complications. *Shunts have a checkered history with an initial 50% success rate and alternately, a high revision rate of 50%. They are the most common pediatric neurosurgical procedure and the second most common neurosurgical procedure for adults.
Unfortunately, there is no “perfect” treatment for chronic IH, especially since every person’s experience is different. While surgery certainly helps some people, it can also lead to repeat operations, which can produce serious, sometimes life-threatening complications. The most common problem with shunts is that the catheter becomes blocked and has to be replaced.

So, now you did I get here? Well, I have been asking that question myself. Sometimes, I think it may have been a lifetime in the making. The term "Idiopathic" essentially means that there is "no underlying cause. Doctors and scientists don't really know what causes IIH. But, they do believe there are some risk factors as listed below. Additionally, they have discovered a second type of IIH called Secondary IIH which unlike primary IIH has a definitive causitive source for the disease. These are listed below as well....

Risk Factors for Developing IIH:

Exogenous Substances:

  • Non-steroidal anti-inflammatory drugs (NSAIDs), tetracycline, nitrofurantoin, isotretinoin, tamoxifen, nalidixic acid, lithium, and the starting or stopping of steroids
  • amiodarone,
  • antibiotics,
  • carbidopa,
  • levodopa,
  • chlordecone,
  • corticosteroids,
  • cyclosporine,
  • danazol,
  • growth hormone,
  • indomethacin,

Systemic Diseases:

  • anemia,
  • chronic respiratory insufficiency,
  • familial Mediterranean fever,
  • hypertension,
  • multiple sclerosis,
  • polyangiitis overlap syndrome,
  • psittacosis,
  • Reye syndrome,
  • sarcoidosis,
  • infectious mononeucleosis
  • systemic lupus erythematosus…
  • Disorders of cerebral venous drainage

Secondary IIH : While pseudotumor cerebri or primary intracranial hypertension is idiopathic, secondary intracranial hypertension always has a clear cause. Possible causes include:

  • dural venous sinus thrombosis
  • kidney failure
  • leukemia
  • lupus
  • excess Vitamin A
  • growth hormone treatments
  • nasal fluticasone propionate

As the story goes my mother says that the doctors thought I had something like Leukemia when I was 3 years old. One day I just didn't wake up. I didn't come rushing out of my room, with giggles and grins. My mother finally came in to check on me and I had a horribly high fever. I was lethargic and had a rash. I believe she said I was like this for weeks.

After this, I guess my health never really was perfect. My childhood was riddled with terrible strep throat infections. One after another. (I believe I read somewhere that the streptoccocus bacteria was linked to IIH as well). I remember there was always a gaggle of nurses holding me down to keep me still in order to get that penicillin shot in my behind. In between the strep infections, I constantly had urinary tract infections and subsequent kidney infections. It seems I was always on an antibiotic for something.

Around the time I was 9 or 10 I got my first cold sore. I suppose, I am forced to admit here that I was a long time, neverending, thumbsucker. It was a habit I never wanted to give up. As such, when I got my first cold sore on my lip, it was eternally exacerbated by my bedtime thumbsucking. By morning, that one cold sore had turned into 3 as it had spread across my lip. Three or four days later I was unrecognizable as from lip to nose I had cold sores. I guess my brother had a bout of impetego when he was a small child. My parents had been informed that the way you treat such a thing was to scrub it with hot soap and water. Without taking me to the doctor, they assumed that this terrible mess I had on my lip was what my brother had when he was small. This is when the torture began. My mother would have to hold me and my father would have to scrub the cold sores until they bled. I would scream. They thought they were doing what was right...I suppose. But, the infection spread horribly and just got worse and worse as the days drug on. Eventually, the sores were so bad and so ugly that they had to take me to the doctor and they were told that I had cold sores or (HSV1). My parents felt horrible, of course. But, due to this initial first experience....other outbreaks would always be worse. I mention this as an importance because, to this day I get these cold sores. Not as often, and certainly not as bad. But, when I do get them....I get them up inside my nose. As the arteries and nerves in the nose and sinus are so closely linked to those in the brain and (CSF), you may understand my concern about this chronic infection in my nervous system? (It's also important to note that it was about this time that my parents first noticed the swelling behind my ears.)

When I was 13 I suffered a severe case of infectious mononeucleosis. I was out of school for 3 months. My liver was swollen, I had blisters on the back of my throat resembling mold growth and I had high fevers. The good news was that after this infection it would be many years before I would ever have strep throat again!

During all of this my mother had been taking care of my maternal grandmother who had suffered multiple strokes. My aunt had been diagnosed with Rheumatoid Arthritis and she was having some health troubles of her own. My mother was suffering from debilitating migraine headaches. It wouldn't be long before I began having migraines of my own....and in fact, by the time I was 17...I was.

Initially, the migraines would only hit once a month. Right around my menstrual cycle. They were pretty predictable. Sometimes stess or lack of sleep could bring them on....but mostly, it was hormonal. I could take a couple Excederin Extra Stregnth tablets and within an hour I would have relief. By my Senior year of high school, however, I started having other problems. There were times when the Excederin wouldn't help with fact...nothing would. I started having memory problems, and vision problems (seeing spots, blurred vision, etc.) And, there were times when I would have horrible muscle pain in my neck and shoulders. I recall asking my friend Maria for massages and/or sometimes missing school due to a pulled muscle.

When I was 19, I had my first child. Maybe due to hormonal changes, lack of sleep, stress or a combination of all three, the headaches worsened. By the time I was 21, I was seeing a neurologist for the migraines. Over the years the doctors tried muscle relaxers and triptan medications typically used for migraines such as Imitrex, Axert (sp?), and Relpax. Nothing seemed to work. The only thing that ever seemed to give me a little bit of relief was the Excederin or some sort of pain medication.

By my mid 20's to late 20's I was diagnosed with Fibromyalgia. My neck and shoulder muscles hurt often, and just like in my teens I was getting pulled muscles as well. But, the diagnosis never really seemed to fit. I read up on Fibromyalgia. I didn't hurt all over my body. I didn't hurt to the touch. It was just my head, neck and back. He put me on a regimen of muscle relaxers. The thought was that if we could get the muscles to relax that suddenly, the migraines would dissipate. Well, the muscle relaxers made me too drowsy to function. As I was a single mother...I had to function. This wouldn't work. I had to stop the medication.

When I was 30, I got married. We moved two hours north from my hometown of Columbus, OH to Medina, OH. By the time I was 31, we now had three children and I had come down with the virus that cause mononeucleosis called the Epstein-Barr virus. I was sick for over a year with fevers, swollen lymph nodes, chronic fatigue and migraines. This seemed to be the turning point. Because after this illness, I had daily headaches and back muscle pain. We again saw neurologists. I was placed on preventative medications like Gabapentin and Depakote but hated the side effects (drowsiness, weight gain) so much that I stopped the drugs. They also tried Amitryptiline but that just seemed to make things worse. Often times, after trying Excederin Migraine I would find no relief and would end up in the ER hooked up to an IV with a dose of Demerol and Phenergan to relieve the pain. This would typically give me some breathing room for approximately 3 days or so before the headaches would begin again.

I blamed it on the stress of a new environment, having young children, a new marriage, a snoring husband, lack of sleep, etc. But, didn't matter....I was just miserable. The doctors were frustrated and nobody could seem to figure out what was wrong with me.

Then I started having strange symptoms. I would get tingling and numbness in my extremities. Sometimes I thought I was having a stroke. Then, when I was 35 we moved to Rochester, NY. Shortly after moving there, I had an episode that left my entire right side numb and weak.....but some of my left side too, from the knee down. I was admitted to the hospital. The doctors there diagnosed me with Hemiplegic Migraines. "Hemiplegic" meaning one-sided paralysis. So that explained the numbness on my right side. What they couldn't explain was the weakness on my left side as well. After several days in the hospital, I developed SEVERE head pain. MRI's and CAT scans came back normal. I was told that they could give me Tylenol and accused of drug seeking. Of course, I believe that as early as the age of 31 I started having IIH symptoms and this time in the hospital I was having an IIH episode misdiagnosed as Hemiplegia. Like many IIH patients, I may have gone misdiagnosed and mistreated for years. And like many IIH patients, I too, have been accused of "drug seeking." After 8 hours in a state of EXTREME pain, I finally called my husband and checked myself out of the hospital.

I started having lymph node swellings and jaw pain along with the daily headaches. Insomnia and night sweats too. I went to my OB/GYN and had blood work done to check for any hormonal imbalances or thyroid disorders. Everything came back normal. My menstrual cycles became irregular and my hair began falling out. My fatigue worsened. After a couple of years, I woke up one day with a sore throat. When I went to the doctor he said he thought it was just allergies. Only a few hours later, I had developed a lump at the front of my throat the size of a baseball. I went back to the doctor and he immediately sent me to the ENT. After some additional testing, I was diagnosed with a congenital defect called a Thyroglossal Duct Cyst. This is a condition that typically shows up in childhood....but, oddly enough, it showed up when I was 37. The cyst was surgically removed.

The lump was gone. But, my condition didn't improve. I was still having night sweats, occasional lymph node swellings, jaw pain, neck/back pain, daily headaches, memory function trouble, insomnia, chronic fatigue, irregular periods, and yes....occasionally, the front of my throat would still swell up too. I also started gaining weight. Lot's of weight. Like....40 pounds of weight. I hadn't changed my habits, but, my body was changing....quickly. (Other IIH patients have complained of this as well. They had strange symptoms and then suddenly had a large onset of weight, that they could not lose.)

But, one morning I awoke to a new symptom. A "whooshing" noise keeping time with my heartbeat in my ear. It was constant. (This, unbeknownst to me was caused from the CSF putting pressure on my blood vessels.) I started seeing a new neurologist. I would wait at times for up to 3 hours to see him. I recall one time being in such excruciating pain in his office and they forgot about me. They almost closed up the office and left me there. I ended up in the ER many times during those months. Every visit he would check my vision and if I had severe head pain I would have blurred vision. He commented several times that he saw some papilledema. Once he gave me an IV of a cocktail they call "Depakon". I don't know what is in it, but I got severely ill vomiting all night. Then he tried a Calcium Channel Blocker used to treat Hemiplegic Migraines called Verapamil....but I broke out in itchy hives and had to take Benadryl as I was allergic.

Then I started having more cognitive problems. I would smell something that smelled like an "electrical smell". I would ask those around me if something was burning but nothing could be found. Shortly thereafter I would be in the midst of a conversation and just stop midsentence or just "disappear". These were small seizures caused from the immense pressure (still undiagnosed) on my brain.

I also had a constant runny nose....constantly. It was a clear fluid coming out of my nose. I needed a tissue all of the time. It was embarrassing. I would have to get up from meetings to get a tissue, or excuse myself at lunch. Of course, this was a condition called CSF Rhinorrhea caused by high CSF pressure for long periods eventually wearing down the sinonasal bone creating a hole or a CSF leak. Again....I was still undiagnosed....and at this point, probably walking around with very dangerous pressure levels.

My mother, in the meantime, had just been diagnosed with a mild case of Multiple Sclerosis. As some, but, not all, of my symptoms, sounded a lot like MS I asked my neurologist about the possibility. He said that indeed, it could be a possibility and given the fact that my mother had been diagnosed my chances of having it had increased. An MRI and MRV were ordered. The tests came back negative. My doctor called my husband and I into the office and after another gruesome 3 hour wait, went through each of the scans with us. Laughingly he told us that, "You have MS....not." Like it was some kind of joke. He said, "You just have migraines." I knew he was wrong. I knew there was something more wrong with me. I broke down and cried. Sobbed really. He said, "What is wrong? Why are you crying?" I said, "I have been seeing you for months. I have had tests run, I have been poked, forgotten about, poisoned and yet you have done nothing to help me. Everytime we come here we wait over 3 hours to see you and you laugh at me as you tell me that I 'merely' have migraines when I know that there is something more seriously wrong." And do you want to know what he said in response? He said, "I can no longer care for you. I will no longer be your doctor."

So, I resigned. Upset and defeated I went home and looked up Hemiplegic Migraine on the internet. Maybe there is more to it than I know. As I started looking up information and medications used to treat this type of migraine I came across a medication I had never heard of before called Diamox. I clicked on the hyperlink and sure enough this medication was defined as a drug used mainly to treat mountain sickness but also to treat a disease called Pseudotumor Cerebri. Upon reading more about this disease, I jumped up and screamed for my husband in the other room. I said, "This is it!!!! I know it!!!! I'm not crazy!!!! This is what I have!!!! I have had all of these tests run except for the one defining test that counts....A lumbar puncture! I have to find a doctor that specializes in this!" I immediately began looking for a doctor in our area who knew about the disease, and I found her. But, when I called her office I was told that she was on vacation and she wasn't accepting new patients. I left my name and number anyway, and begged the secretary to ask the Dr. to reconsider. I had no idea that fate would step in.....

A few weeks later I woke up with a pulled muscle in my neck. It was very painful and I couldn't move. The doctor described the muscle as being "infected", as if something were irritating it. I was put on muscle relaxers. But, a few days later I awoke with THE WORST HEAD PAIN OF MY LIFE!!!! I have had migraines at this point for over 20 years, but this was the worst pain of my life! My husband was trying to get my girls off to school but I couldn't wait that long....he had to bring my daughter to the hospital with us. When we arrived, I told them of my symptoms and then I said, "the only test that has never been done is a spinal tap. I think I may have Pseudotumor Cerebri. Please do a tap."

A tap was opening pressure was 280, I had papilledema behind both eyes, my fluid was normal, my CAT scan and MRI was normal. It finally happened.....I had a diagnosis. I had Intracranial Hypertension. Thank God! Now I can get well.....

.....too bad that's not the way this disease works! I'll leave out all the details about how I was temporarily paralyzed and couldn't urinate or have a bowel movement on my own due to the first lumbar puncture (for almost 7 days). And, how I've had Urinary Tract Infections ever since. I will tell you that I was placed on an abnormally high dose (for my opening pressure), of 1500 mgs. of Diamox to start. Diamox lowers your fluid levels and can mess with your electrolyte levels, kidney function and potassium levels if not monitored properly. Needless to say, I wasn't being monitored. I was also taking 20 mgs. of Lasix every day (another fluid lowering drug). I couldn't move. I was very weak. I had tingling sensations all over. I couldn't take a shower because I couldn't lift a wash rag. I slept all the time. I was thirsty all the time. I remember my husband brought me some orange juice and I thought it was the best drink on the face of the planet. I slurrped it down in about .02 seconds. Those first couple of months were a nightmare. I wasn't feeling much better. I was in and out of the ER. The doctors would occasionally put me on steroids to help with inflammation, but that would just make me gain weight. During this time, I put on 15 pounds. I was miserable.

My doctor decided to up the Diamox to 2500 mgs. each day. But, now I was having horrible headaches. And they were throwing all kinds of narcotics at me....morphine, liquid morphine, toradol, vicodin, dilaudid, etc. I was working with a neurologist who knew very little about IIH. It would seem that they had overshot the therapeutic window and sent me straight into the gates of Hell. It couldn't get any worse. I made an executive decision....I stopped all the medications. I called back to the specialists office I had tried way back when, before I ended up in the hospital.....and I got in.

This brings us to today. I'm still not well, but I am better. I currently take a regimen of 100 mgs. of Wellbutrin every morning for depression. I take 20 mgs. of Lasix once each morning and once every afternoon with a Potassium supplement for therapeutic effect all day. And, I take 75 mgs. of Topamax every night at bedtime. I still have daily headaches, backaches, and neck aches but, the papilledema and "whooshing" is under control. I have had 5 lumbar punctures done in 5 months with opening pressures ranging from 250 to 330. Many days I am still on the couch with fatigue and pain and am still in the ER every couple of weeks or at the very least once a month. Dilaudid is still the only medication to take the edge off the pain. I still have insomnia and nausea. I'm not back to work and far from having a "normal" life. My doctors and I have a ways to go to figure out what will work for me to get me back to the living again. It is my goal to stay away from shunts for as long as I can.

Like many other patients with IIH it may take a very long time to find a regimen that will work to control my levels and still manage the side effects of the medications. Even once we reach a point where medications keep my pain and fluid levels down, eventually these medications will stop working. There may come a day when I have to have a shunt. I hope for a cure before that day comes. It is a relief to have a diagnosis...but, currently the diagnosis brings little relief.

In the meantime, I want to do what I can do. I want to bring awareness to this disease, in hopes that others like me won't have to wait so long for a diagnosis and will be treated with dignity and respect in hospitals and doctors offices across the nation. And, I want to help raise money for research. I can sit on the couch and whine about the limited amount of resources that are available to me to fight my pain and suffering, or I can help raise money to develop a cure. I choose the latter.

So...this is my story. It's a long one, and if you read all of it then I thank you for your commitment but mostly for your support. Please stay tuned to my post for updates on my progress. But, more importantly for updates on the progress of the development of our fundraising event next year. Hopefully, you will jump on board and help donate to the cause.

To make a donation go to:

* Excerpts taken from Intracranial Hypertension Research Foundation


  1. Holy cow, that is a life story! I'm so glad you're able to get your blog started! And let me be the first to welcome you to the wonderful world of blogging.

    Tips: if you list the full web address
    it will automatically become a link.
    And if you tag your posts (at the bottom when your are writing a new post) with a subject it makes it searchable to other people.

    Way to go! Spread the word, increase awareness, and work out your issues all in one venue!

  2. Wow, you have really been through it! I cringed when you were talking about having to scrub your cold sores as a child. Bless your heart!

    Keep up your blog. It's got to be theraputic getting it all out there and it makes the rest of us feel better knowing that we are not alone in the fight. I know I always feel better after I post on the Daily Stregnth message board because someone is always there to offer a word of support or let you know you are not crazy. Best of luck to you!


  3. I have just been diagnosed have not seen any specialist yet just discovered a week ago because i went to an retina specialist who discovered amy left eye optic nerve is swollen. Im super relived to know im not crazy because i have had migranes and well all the symptoms but never knew what was going on. However im really scared this article hit me hard all i could say while reading it was yes thats me omg im not alone.