Living with Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

I’m getting pretty familiar with the white walls of a doctor’s office and the piercing sound of silence as I wait to discuss what’s wrong with me. The past year has been focused on my physical health which is funny because before, it was always focused on my mental health. The two go hand in hand in the end. 

I was diagnosed with PCOS (poly-cystic ovarian syndrome) a little over a year ago. I was struggling with being able to lose weight, feeling exhausted no matter how much sleep I got, and rupturing cysts. When the migraines started, I just thought it was another symptom of the PCOS and my out of whack hormones. I was very wrong.

I went for a routine eye checkup at VisionWorks to get new glasses in January of 2024. I hadn’t been in forever and wanted new glasses, knowing my vision had gotten worse in the past two years I hadn’t been. Everything was great until the eye scan. The optometrist’s brow furrowed as she did a double-take at the screen and muttered “uhm, that’s not right”. Of course I asked what she meant to which she replied, “I’m not sure, I’m seeing some swelling on your optic nerve, I’m going to have to get a second opinion”. She told me someone would reach out to schedule another appointment soon and sent me on my way without any further explanation. I wouldn’t hear the words “pseudotumor cerebri” until 2 months later. In between this time I still struggled with debilitating symptoms and even took a trip to the clinic for what I determined to be vertigo after I was so dizzy I fell out of bed and the shower that morning. I was so confused as to what was happening to me. 

In March I went into the Indiana Cataract and Laser Eye Institute for the first time where they dilated my eyes and ran a series of tests. Surrounded in a room full of elderly, one of the nurses told me I was a “breath of fresh air around here” and I believed her. The optometrist there told me that my optic nerve was swollen and asked me if I had been experiencing any migraines. I told him about the weekly headaches and several migraines that caused me to get sick, lose vision, and felt like my head was going to explode. He told me that he suspected I had a condition known as pseudotumor cerebri (aka idiopathic intracranial hypertension), a condition that acts as if there is a brain tumor when there isn’t one there. He wanted me to come back for a field vision test, have a MRI to rule out a REAL tumor, get set up with a neurologist, and have a spinal tap to confirm the diagnosis. 

Afterward I scoured the internet for more information on idiopathic intracranial hypertension (IIH). This condition causes issues in the reabsorption of spinal fluid in the body. In turn, this causes swelling and pressure on the brain and optic nerve. If left untreated, this condition ultimately leads to blindness. One of the major symptoms is debilitating headaches and migraines as the spinal fluid crushes the brain and optic nerve. Other symptoms are ear ringing, sensitivity to light and sound, fatigue, brain fog and cognitive difficulties, weight gain, neck and back pain, dizziness, vomiting, depression and anxiety. All of which I experience. This condition is extremely rare, affecting every 1 in 100,000 people, and the cause is unknown.

After we ruled out the possibility of a brain tumor with a MRI, I was set up with a neurologist who referred me to have a spinal tap or lumbar puncture. She also started me on a low dose of Diamox to attempt lowering the pressure. The anesthesiologist was amazing and asked why someone as young as me was there to see him. He stuck a long needle in my spine and pulled out spinal fluid to be tested for pressure and CSF analysis. He told me he could feel the pressure as it was coming out, “It’s shooting out of there quick”. Following the procedure I was perfectly comfortable and only had to lay down for about an hour before I was released to go home. This was done the week before my birthday (June 2nd), right before we planned on going to Michigan for a few days to celebrate Me. This week (and a half) after the spinal tap was hell. I thought I would be able to go back to work but I was wrong again. I spent the whole week on the couch with just enough PTO to be able to still go on my extended weekend birthday trip to Michigan. I was in less pain by the trip but still unable to enjoy myself to the fullest due to limited mobility and worsening headaches. 

At this point, my diagnosis was confirmed with an opening pressure of 27. My dosage of Diamox was basically quadrupled after the span of 4 months as well as trying out the only other medication used for this condition. Nothing worked. I was still having increasing headaches and migraines, especially as the atmospheric pressure fluctuated rapidly with the seasons and weather. It was a miserable existence. One medication, the Topamax, even triggered manic symptoms in my Bipolar 1 diagnosis. I went to my neurologist and optometrist several times during this span of time as I progressively got worse. All while I was still trying to keep up with work, graduate school, and being a good mom and girlfriend. Around the end of September my neurologist had me go in again to see if my optic nerve was still swollen, if it was she wanted to refer me to neurosurgery as medication wasn’t working for me.

I finally got into the neurosurgeon's office during mid-October. By the beginning of October, I was no longer able to work due to my condition worsening and making me unable to perform my job duties. I decided to try filing for short term disability, knowing my surgery would be coming up at some point soon. I went to the surgeon’s office alone and he immediately told me we needed to schedule another office visit so my family could attend and hear what he had to say as well. After talking with me, he mentioned that a shunt would likely be our best option as he described the options for surgery and recommended I meet with a neuro ophthalmologist to again see if my optic nerve was swollen and do another field vision test to determine any visual damage. He also told me that they believe one cause of IIH is rapid weight gain, which I have experienced on and off since getting pregnant with my daughter. He told me that remission is possible once people reach the “miracle number” for their weight but it was easier said than done, especially with my co-occurring conditions. They told me to just focus on losing 2 pounds per week before and after the surgery and sent me on my way again. 

Another month went by and I was able to get in and see the neuro ophthalmologist. She confirmed that my optic nerve was yet again, still swollen. I knew they were swollen constantly for months because my eyes were constantly burning, watering, and so swollen they felt like gravel under my eyelids. She also noticed that I had minimal damage to my peripheral vision from the field vision test. I asked her if she agreed with having shunt surgery to remedy my condition and she agreed that it would be best to lessen my headaches and avoid potential blindness. She said she would follow up with neurosurgery and I could wait for them to call and schedule another appointment.

Again, I finally got the call and scheduled the family office visit by the end of November. I took along my boyfriend and my parents so everyone could get the spiel at once. I watched as everyone's faces dropped as they realized how serious this is going to be. I mean it is brain surgery. I hole will be drilled into my skull for them to place one end of the shunt into my brain and the other end into my stomach to allow the spinal fluid to drain. There are two types of shunt procedures he offers me. The first is the typical shunt used by most surgeons but the other is a procedure that only he and 3 other people are trained in doing. The shunt is placed deeper into the area of the brain so that the ventricles don’t collapse around it, which commonly happens and leads to shunt revisions and further brain surgeries in the future. The only downside is this procedure has a .1%-1% fatality risk in which you wouldn’t wake up from the operating table. Of course that small percentage still makes myself and my family nervous. However, it’ll be my decision to make the day of surgery. At present, I am leaning more towards deep shunting because it is so much more effective and gives me a better chance of reaching remission within the following year. It’s just the risk that makes me hesitate. However, the surgeon said that out of his hundreds and hundreds of surgeries for this condition, there have only been 15 close calls and no one has died on him yet. Impressive. But I don’t think I will fully make up my mind until they schedule the surgery. I was told yet again to wait on a call for the surgery to be scheduled as Thanksgiving was the following day. He said he would do it the next day if he could but he wasn’t sure when he had a 4 hour block for the surgery. It’s maddening to keep waiting but at least I am getting closer and closer to getting myself back from this pit that I’ve crawled into. So now I wait again for the call that will determine when I can start feeling alive again. 

The recovery is expected to be 4 weeks long in which I will have what the surgeon referred to as “movie star” treatment where I do no chores, no lifting or bending, only “walk down the red carpet”. Which is going to drive me further into madness but I’m hoping it can be a time for me to be more mindful and let myself fully recover. I have so much to do in the roughly 1-2 weeks that I’m waiting for the surgery to take place. I want to wait until my mom gets back on December 9th to hopefully schedule it if at all possible but he did say it was all my choice on when it happens. I just decorated for Christmas as my recovery will plow me through the holidays and I’m working on getting things in the house clean, organized, and put into place. I even plan on making lists and a calendar to map out the days that my friends and family members can come and help babysit me. I’m nervous that things will fall behind or not get taken care of because I’m used to doing them but I have such a great support system that I know it will all be okay in the end. I’m trying to stay optimistic and I’m focused on doing what I have to do to reach remission and be free of the chains that this condition has bound me in. More updates to come!

xoxo