Updated: Sep 20, 2019
Unfortunately, the title of this piece is not click bait. Just before Christmas 2013, I was sick. Really sick. Like skipped out on church on Christmas Eve because I could barely sit up sick. I felt so bad on Christmas Day that I stayed in bed the entire day. My husband was also sick, and thankfully we were both off of work that week, so we were largely able to sleep it off. The symptoms of my illness finally began to subside, but new, more peculiar symptoms replaced them. I developed the worst headache of my life. Nothing touched it. I was popping Advil into my mouth like Skittles, to no avail. A couple of days after the headache arose, I began to develop a nonstop, pulsatile tinnitus. The whooshing in my ears got so bad that I began stuffing cotton in to them in an attempt to drown out the noise.
By New Year's Eve, my headache got so bad that I begged my husband to drive me to the emergency room of our local community hospital. It was snowing pretty hard that day, but I simply could not take any more. I was examined in the emergency department, blood work was drawn, and after several hours I was sent home, "Unlikely to have meningitis", with a 10 day prescription for Tylenol 3.
I went back to work on January 2, with absolutely no improvement in my headache or the whooshing in my ears. I was on service on both January 2 and January 3, so I felt obligated to go to work, despite feeling like my head was being run over by a Mack truck, and I had the world's worst symphony playing between my ears. On Friday, January 4, I called out sick. Calling out sick for me was the equivalent of seeing a Yeti. It doesn't happen. You can imagine just how bad I felt.
I decided to try not to be a complete slouch, so I figured it would be in my best interest to do something academic, I turned on my computer and logged into my CITI training modules. While it was not the most scintillating reading, particularly with a pounding headache and the world's worst death metal symphony ringing in my ears, I set out to work on the modules. As I looked at the screen, I noticed the letters looked just a bit blurry. I figured it might be time for new glasses but I pressed on and finished.
Two days later, while indulging in NFL Wild Card Weekend, I noticed I could not focus on the numbers or lines on the field. I tried not to worry, but at the same time, I'd had the worst headache of my life for 10 days and death metal was on an endless whooshing loop in my ears. All I could do was hope it would pass. By this point my doctor spider sense should have been tingling in overdrive. I'd never experienced symptoms like this. Yet, I managed to maintain an eerie calm.
My calm ended completely on the morning of January 7, 2013. I woke up to get ready for work, planning to work through the headache and the death metal symphony. That was, until I put my glasses on. I thought I had grabbed an old pair. I looked at the frames in my hand and indeed it was the right pair. I started to freak out on the inside-I did not want to let my husband know what was going on. I was losing my sight. My glasses no longer corrected my vision. Ninety minutes later, I said goodbye to my husband. He left for work, and I sat in my driveway debating what to do. I broke into tears. I called my vice chair. When she answered the phone, I began to cry harder. I will never forget what I said next- "You have to help me. I'm going blind.”
I'm pretty sure I scared her half to death. She told me to hold tight and I'd hear something soon. I decided in a bit of poor vision related psychosis to start driving to the hospital. As I was driving, I received a call from Dr. Larry Frohman’s office. Dr. Frohman is neuro-ophthalmologist. I thought this was fitting since I had already self-diagnosed optic neuritis. The receptionist told me to come right away.
I parked in the deck, forever grateful I'd made it to work in one piece, and I headed over to the doctor's office center to be seen in the ophthalmology office. I filled out my paperwork, which took forever because I had a raging headache, I couldn't read half the words, and the death metal symphony would not quit. I called my secretary Juliette, who I considered one of my work moms, and I told her what was going on. She came to be with me. At this point I STILL hadn't told my husband what was going on, because I did not want him to worry. Juliette came and held my hand as I cried. Dr. Frohman saw me quickly, and I could tell he was very worried after the first round of tests. He sent me for visual field testing.
The visual field test made things very, very real. You stick your head inside a white box with one eye covered to figure out how much you can see with the uncovered eye. I don't fail tests, but I failed this one with flying colors. The test took entirely too long. I was supposed to click a button every time I saw a light. What I found out later was that I could only see the brightest, most central lights.
After the visual field examination, I was the most scared I had been in this experience. I knew I was going blind. I just wasn't sure why. For the first time I contemplated my future. As a pathologist, my eyes are my career. I was afraid I would never be able to work again. Never drive again. I would need someone to help me take care of myself. I'd never felt so helpless. As I sat there thinking life as I knew it was over, Dr. Frohman come in. He said, "I believe you have pseudotumor cerebri. You've got massive papilledema. You've lost your peripheral vision. You’ve lost most of your central vision and some of your color vision. We have to do an MRI to exclude a brain tumor, which I highly doubt you have." And I said, "Do what you have to do. I just don’t want to have a lumbar puncture." Dr. Frohman said," I've called the ER and they are waiting for you. Her's my phone number. Text me when the MRI is done."
It was just after noon when I arrived at the ER. I went through all the intake and was sent to one of the bays. I put on my "I no longer have any shame" hospital gown and begun to wait. I figured this might be agreed time to call someone in my sparring. At this point, most normal people would have called their spouse. I looked at my watch. It was just after two. I didn’t want him to leave work early. So, I called my sister who I knew got off work at 3:15. She came just as I was being taken for the MRI.
Usually an MRI is loud, but the death metal screeching between my ears was far worse. The headache was so excruciating that lying still was becoming a challenge, but I didn’t move because I needed this MRI to exclude a brain tumor. When I was done, I did as instructed and texted Dr. Frohman. He called me back shortly after and said," Good news! You don't have a brain tumor!" My response- "oh no.'' I knew what that meant. "Val, you need to have a lumber puncture. This is definitely pseudotumor cerebri. The lumbar puncture will likely prove it.” I had symptoms of increased intracranial pressure with no localizing neurologic signs and a normal MRI. All I needed was a high opening pressure on the lumbar puncture.
This might be a good time to point out that I hadn't had kids yet (I became pregnant with my first later that year), so I wasn't yet a two-spinal veteran. I was terrified of the possibility of a needle in my back. I knew it had to be done. Dr. Janine Greyson performed the LP. My sister held my hand, and to my surprise, the procedure was not nearly as bad as I thought it would be. My opening pressure was 280. Once the procedure was done and I was lying flat on my back for the required several hours, I finally called my husband and let him know everything that had happened. While he could not understand for the life of him why I didn't call sooner, he came right away. His sister drove him so he could drive my car home. The best thing about the LP was that it instantly cured my headache. There was no residual dural headache. For the first time in nearly two weeks, I didn't feel like my head was about to explode. The death metal symphony had finally quieted to a distant din.
It was nearly 8 pm when I was discharged from the ER. I was told to stay home for the rest of the week, and I was started on the maximum daily dose of acetazolamide. I was told it would be at least a month before I would be able to read slides again if the acetazolamide worked. If it didn't work, I would have to consider optic nerve sheath fenestration to help restore my vision.
A week later, I followed up with my ophthalmologist. My vision was beginning to improve. I went back to work but could not participate in any clinical service. I felt like I was taking up space. The acetazolamide had an awful side effect-it left a terrible metallic taste in my mouth. Additionally, anything carbonated burned in my mouth. That said, these were only minor inconveniences as that medication was my best chance at-regaining my vision. I had to leave work by 3pm each day because I could not see well enough in the dark to drive. It was a dark time in my life. But, with the love and support of family and friends, I got through it.
By the first week of February 2013, my vision was largely restored. I passed my visual field testing and was able to return to clinical service. I could see at night again. It was such a thrill to start to regain some normalcy in my life. By mid April, Dr. Frohman decided to take me off the acetazolamide. That was the last time I ever had to take the medication.
Nine months after this ordeal we conceived our first child. As pseudotumor has the highest risk of recurrence in the first trimester, I found myself back in Dr. Frohman's office. Thankfully the disease did not recur. 26 months later, we were expecting our second child. Again, I was fortunate enough to avoid recurrence.
It's now been six years since this happened. I consider myself one of the lucky ones. Many patients with pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), have to undergo optic nerve sheath fenestration because acetazolamide alone is not sufficient to control their symptoms. Many people have to remain on acetazolamide for the rest of their lives. I have been so blessed. Looking back, I had (and still have) multiple risk factors for developing IIH; I was a young black woman. I was obese. I felt like something out of a medical school textbook. I’ve struggled with my weight for most of my adult life. I continue to struggle with it. But I fight on, because in the back of my mind, I fear this could happen to me again.
I share my story, not for sympathy, but to spread awareness. While IIH is not the most common disease, I had many classic symptoms. I had the worst headache I'd ever had and it was diffuse. I had a pulsatile tinnitus-the death metal symphony as I dubbed it, was a loud whooshing sound that was constant. I could hear it in my sleep. I had significant visual loss that began in the periphery of my visual fields and spread to my central vision. I had bilateral papilledema on physical examination. I urge anyone who has any of these symptoms to please get checked out. The sooner you do, the more likely you are to save your sight.