My October 2018 was less Pumpkin spice latte’s, Autumn leaves and Fall winery visits and more fun house mirrors, Jumanjj drums and Rocky running up the steps in the city of Philadelphia. Those three things were my introduction to the rare disease, known as Pseudo tumor cerebri-false brain tumor or the current favored term by Doctors, idiopathic intracranial hypertension. Here is a brief description of it from the Mayo Clinic website-
Pseudo tumor cerebri occurs when the pressure in our skull (intracranial pressure) increases for no obvious reason.
Symptoms mimic those of a brain tumor, but no tumor is present. Pseudotumor cerebri can occur in children and adults, but it’s most common in women of childbearing age who are obese.
When no underlying cause for the increased intracranial pressure can be discovered, pseudotumor cerebri may also be called idiopathic intracranial hypertension.
The increased intracranial pressure associated with pseudotumor cerebri can cause swelling of the optic nerve and result in vision loss. Medications often can reduce this pressure, but in some cases, surgery is necessary.
Pseudotumor cerebri signs and symptoms may include:
Moderate to severe headaches that may originate behind your eyes and worsen with eye movement
Ringing in the ears that pulses in time with your heartbeat (pulsatile tinnitus)
Nausea, vomiting or dizziness
Blurred or dimmed vision
Brief episodes of blindness, lasting only a few seconds and affecting one or both eyes (visual obscurations)
Difficulty seeing to the side
Double vision (diplopia)
Seeing light flashes (photopsia)
Neck, shoulder or back pain
May clinic official website
Fun house Mirrors
For me, IIH was subtle in it’s arrival. I had some brief moments when I would get up and all of a sudden my vision would go black. But it was quick and I rationalized that I got up to soon or was perhaps fatigued. I could still see well enough to work and other than some quick black out’s and an increased sensitivity to light, it was incredibly minor visually until it wasn’t.
One Saturday, I was driving Jojo back to University and I was having trouble understanding what was going on visually on the right side of the road, half way into the trip. It was Fall, so there were grass cuttings in the shoulder of the road. For me, these grass cuttings kept moving to the center of the road and then would move back out which didn’t make sense at all because it wasn’t a windy day. The center line of the road, it also was no longer two lines, but was four. Things were beginning to double now too. I kept asking Jojo about it, but I wasn’t making any sense to him because to him none of this was occurring to him visually. I started to panic now and pulled off the road and asked Jojo to drive the rest of the way. When we got to his University I called Tom and asked him to come get me. I didn’t feel safe to drive home. The next day I called my Optometrist and asked for an appointment. I knew that that office tested for glaucoma and could tell if a cataract was forming, so I thought he could give me a brief look and tell me if I needed to see an Ophthalmologist.
Once at the Optometrist office, I was feeling hopeful that this riddle could be answered. He tested my vision by looking at my eyes, giving me basic vision tests and asking me to describe to him exactly what happened. After examination and our discussion, his diagnosis for me was ocular migraine. He informed me that ocular migraines were fleeting, could be very frightening but didn’t happen that often and that I should follow up with a Neurologist if it happened again but to work on stress reduction, sleep and nutrition to help prevent a re-occurrence. Feeling relieved, I left his office mildly hopeful. I bought some Excedrin Migraine pills, resolved to get more sleep each night, filled my grocery cart with vegetables and fruits and upped my self care and stress management game. Maybe this was all just a big wake up call to take better care of myself and I’d look back at this trip to the fun house mirror room as a health scare that lead me to some healthy changes.
I didn’t drive for two weeks. I was still too nervous to do so even with new attention to better health and a diagnosis of ocular migraines. I was still having some small black outs if I got up too quickly and sometimes everything would just get blurry and out of focus on the computer screen, but never very long and seemed to resolve if I steadied myself and if I changed my angle in viewing the screen. Eventually though I had to drive. Tom wasn’t free that day to help me and I had an appointment with Jojo I couldn’t miss. First half hour, it was okay. But then once I got on the freeway and had five lanes, traffic cones, concrete walls on the far lanes, it all started up again , the double vision and blurriness. It was clear to me now , I could not will this away with healthy living and medication for migraines. Either I had a serious mental health problem that was causing me to hallucinate or I had a serious vision problem. Regardless, I had to deal with it if I ever wanted to feel safe behind a steering wheel again.
Once again, I had to call home and ask to be picked up. I was unable to drive home. With motion, my eyes weren’t working together well. My peripheral vision on the right especially was not good. I scheduled an appointment with an Ophthalmologist for the following week. No nutrition improvement or supplement, no stress reduction, increased water intake or well being exercises were going to fix whatever was wrong with me physically. Whatever was happening, it was serious and was starting to limit my ability to function independently.
The eye appointment started like the earlier one. I was passing all the vision tests. Nothing looked wrong by gross examination. Maybe it was a neurological or physiological issue the Ophthalmologist thought. I knew it was not helping my case at all that I could pass a vision test at this time. Behind a steering wheel, I had blurry vision, double vision and extreme emotional PTSD but now I could read the vision lines in her office just fine(this would change as the disease progressed). I was battling to get my invisible symptoms to get taken seriously. As a final rule out for eye disease, she offered to dilate my eyes and look at my retina. She said she didn’t think she would see anything, but that it could at least rule some things out before I moved on to neurology or psychiatry. My eye’s were dilated. I waited in the waiting room and then was called back in and she looked at my retina and my optic nerve. This part to me, will always be a little bit of comic relief. Before this moment, I got the feeling that she thought I was a hypochondriac or truly did have ocular migraines and she was just indulging me to be polite and to be a good Doctor and rule everything out as a precaution. But once she looked at my optic nerves, she switched to very concerned Doctor and rushed to get her partner to look at my eyes. Next there were rushed calls to the emergency room and instructions for me to go to the emergency room asap due to the papilledema, extreme optic nerve swelling she was seeing. Now I was considered a very ill patient rather than a patient who means well, but is confused on what is going on with her body.
I did go to the ER. Actually I ended up there twice in a ten day period. My vision problems did progress beyond driving impairment. All vision became diminished making work and deciphering the world on my own impossible. When I went for a MRI in a medical building, I remember staring up at the board in the lobby with all the Doctor’s names and offices listed and not being able to read the names clearly without seeing double and seeing a gray haze over everything. I felt so discouraged and dependent on others for help since I couldn’t see on my own which floor I needed to go to. This wasn’t the last line of letters on a vision test, this was something I needed to be able to read on my own if I wanted to be able to handle life independently. My emotions were very bleak during this time.
Unfortunately It took several weeks to get an idiopathic intracranial hypertension diagnosis. Optic nerve swelling can also be a sign of Multiple Sclerosis so that was explored first, causing my symptoms to continue to worsen since MS isn’t treated with the same medications as idiopathic intracranial hypertension. This misdiagnosis also complicated the process of finding a Neurologist for me since no Neurology office had an opening for a potential MS patient for a month or so, nothing out of the ordinary for a possible case of MS . It wasn’t until I continued to lose vision and I got sicker, that one of my Ed Physician ‘s stepped in and made a lot of phone calls on my behalf to find a Neurologist who would see me immediately. Once I saw a Neurologist, the pace picked up dramatically. He ordered another MRI, a spinal TAP in the OR and started me on a high dose medication that reduces the spinal fluid my body is making. Gradually I began to get better. Another week and I was able to work. Three weeks, and with a eye patch covering my right eye I was able to drive on the highway again. No cause was found to explain why I have idiopathic intracranial hypertension. Lupus, MS, Lyme Disease, several other auto immune diseases were all tested to see if they were the cause but none were a match. I also do not have venous sinus stenosis of the brain, another cause that can be treated and cure the disease. So now, the disease is managed by medication, follow up visits to the Neurologist and monitoring of the optic nerve for swelling levels by the Ophthalmologist. I’m not cured yet but I’m also not in the fun house mirror room any longer. Woo Hoo ! This photo is a dramatization of my skull pre-medication. Humor helps….
While I celebrate fully and deeply my vision gains, there is one remaining symptoms I still struggle with daily. One of the more unusual parts of this rare disease, is the symptom of pulsatile tinnitus or what I refer to as Jumanji drum head.
Pulsatile tinnitus is a thumping or whooshing sound in one or both ears that beats in sync with your pulse or heart beat. For me this is my main symptom that I live with daily since the medication keeps my vision in check. When I’m active and moving around, the motion masks the sound but when I’m lying down and the house is quiet, Jumanji head is beating away and I have to move around to break up the sound. In the beginning, this drum beat wasn’t as strong, it was a more muffled whooshing. Now I hear it more strongly. When the first Ophthalmologist asked me if I heard a whooshing sound in my ears and head, my first thought was, “Wow, how did she know?”. It seemed like such an odd symptom, that I had never even thought to look it up or connect it to my other symptoms such as double vision and headaches.
So far, I have not been able to eliminate this symptom of the disease, but I live with it. It doesn’t prevent me from driving or working and it doesn’t keep me bed bound, so I work on minimizing it by using white noise and movement. It’s annoying more than anything.
My last show and tell for idiopathic intracranial hypertension is the boxing icon Rocky. During the early days of my illness, I remember one breakfast we had at IHOP. As I was exiting the restroom, I saw a poster for breast cancer, that had the theme of fight breast cancer and it was a woman in a boxing robe and gloves. My reaction to the poster was, “I don’t know about cancer, but I know about my sickness and this feels more like a gut punch than a fair fight between two equally trained athlete’s. I’m in my most vulnerable mental, emotional and physical state and I’m suppose to fight this like a trained super athlete?” At that moment I identified more with a Mack truck crash survivor than a fighting cancer patient.
I was dealing with severe mental fogginess, blurred vision and an inability to get a Doctor to believe that I needed medical attention now rather than later. Neurologists deal with a lot of chronic, very sick patients and it can feel impossible to get an appointment that are not months away. Fortunately, I had an Ed Doctor who helped me with this.
Loss of independence and an inability to work was also weighing on me during this time. On my walk to the OR prep room with the nurse for my spinal tap, I got so emotional I couldn’t talk or keep my head up. I was overcome with the feeling of fragility and vulnerability. Not fear of the procedure but fear of the results. What if I do have Lupus, MS or another auto immune disease? What if I have to have a brain shunt? I wanted to know, but also was scared of how my life would change because of it.
Combining the stress of getting medical care, dealing with physical loss and fears about the future, I classified myself as an easy target for any imaginary Rocky health foe out there. If we were in Las Vegas and Rocky was on stage waiting for me to enter, (imagine the iconic Rocky movie theme song playing in the background , him doing warm up moves and the crowd clapping and cheering ) I’d forfeit the match from the parking lot. Fighting back was the last thing on my mind during this time. I was just trying to survive and hoping for a better tomorrow. I didn’t have the emotional strength to pick up any health boxing gloves during this time.
While I was incapable of this, I did have a Rocky in my life who fought for me during this time. He fought when I couldn’t. My husband Tom, he kept up with the phone calls. He managed the follow ups, he made sure we got to where we needed to go. He took me to all the Doctor appointments and the Emergency Room on more than one occasion. He pressed the Doctors when he saw that I was confused or hesitant to ask. He was up to the task of getting in the imaginary health ring with Rocky and he did it many times for me. I wouldn’t be as well as I am today if he hadn’t advocated and cared for me during this time.
Reflecting back on that Fight Cancer poster, I think I would rather see the imagery expanded beyond the one patient in boxing gloves. Why not a group or at least one other person in the ring with the patient fighting alongside with you ? Illness already feels so isolating without the added pressure and expectations fighting solo brings. Even Rocky had Adrian. We all need advocates, comforters and supporters during the tough times.
Until next time friends,