"I know everyone says to trust your gut, so I’ll say trust your headache. Your pain is real, and ‘borderline-ish’ does not serve anyone."

Doctor: “You need to go to the emergency department.”

Me: “Right now? Am I ok to drive? Do I need to call someone?”

Doctor: “You don’t need an ambulance, but go there soon. They can help you with your headache there.”

Concerning, but not urgent. This would soon become the theme of the next 6 months and beyond. Welcome to my IIH patient journey.

December 2022

Taking a step back, this headache and various other symptoms had been interrupting my life at random for months already. Unlike my old friend, migraine, with its reliably rapid onset and short duration, this headache was all over the place. It caused a spectrum of pain from dull to crushing to sharp, and in different parts of my head. When I laid down, it felt as if all of the blood was rushing to my head. When I was upright, it felt like I was wearing a tight hat. Even my scalp hurt to touch. My vision blurred when I bent down to tie my shoes. I had pain behind my eyes, a whooshing sound in my left ear, and pain in my neck and shoulders. What the heck was this?

March 2023

Under normal circumstances, I might have seen a doctor sooner. I’d just gotten a divorce several months prior, and before that, I experienced three significant deaths in my family in a short timeframe. At that time, everything was upside down. I jokingly googled “Can you sprain your brain?” I knew that grief could cause physical ailments, so, maybe? I took the headaches as a signal to slow down, which I did. However, at some point, the occasional headaches grew more frequent until they merged into one constant, unrelenting main event that upstaged everything else in my life.

April 2023

Back to the doctor telling me to go to the emergency department. It had been a couple of weeks of this non-stop headache, and I’d been to the doctor twice. Nothing we tried was working. NSAIDs, caffeine, electrolytes, NSAID injection, steroid injection, two migraine medications, more powerful NSAIDs. So, I drove myself to the ER.

Ten hours, one blood draw, and one CT scanUses several X-ray images and computer processing to create cross sectional images. Click the term to read more later, they’d ruled out anything serious (I had not sprained my brain). I was admitted to the hospital for observation and more tests. I received a lumbar punctureA procedure where a needle is placed in the lower part of the spine (the lumbar spine) to access cerebrospinal fluid. Click the term to read more (LP), an MRI/MRV, and an eye exam from an ophthalmologistA specialist in the branch of medicine concerned with the study and treatment of disorders and diseases of the eye. Click the term to read more. The LP revealed an opening pressure of 23, and there was no papilledemaSwelling of the optic nerve that carries visual signals from the eye to the brain. Click the term to read more (optic nerve swelling) present during the eye exam. The imaging revealed partial empty sella, optic nerve tortuosity with prominence of cerebrospinal fluidFluid that is made by specialized cells in the ventricles of the brain. Click the term to read more (CSFFluid that is made by specialized cells in the ventricles of the brain. Click the term to read more), and stenosis of the lateral segments of the bilateral transverse sinuses, consistent with underlying IIH. Sella refers to the container for your pituitary glandAlso known as the “master gland” it is the area of the brain that manages and makes a large portion of your hormones. Click the term to read more, which appears “empty” on imaging when CSF pressure has flattened the pituitary gland. The last part about the stenosis stood out to me as interesting, but I would be told over and over again that it was not.

Later, a neurologistMedical doctor who diagnoses and treats nonsurgical issues related to the brain and nervous system.  Click the term to read more stopped by to tell me that IdiopathicThe term idiopathic is used when there is no detectable reason for something. Click the term to read more Intracranial Hypertension (IIH) used to be called Pseudotumor Cerebri, and, while I probably don’t have it, I’m a woman with a high enough BMIA value derived from the mass (weight) and height of a person. A healthy range is between 18.5-24.9, overweight is defined as 25-29.9 and obese… Click the term to read more to fit the description of who gets IIH. He added that the other criteria for diagnosis are papilledema and an opening pressure of 25 or higher, so I was ‘borderline-ish.’ He said that losing weight would likely relieve my symptoms. To add to the confusion, he believed that what I was having was a migraine, though I assured him it was not a migraine, and thought I should repeat the lumbar puncture in a few months to be sure. I asked, “Do I or don’t I have IIH?” He said it was too close to call. I asked about the bilateral transverse sinus stenosisNarrowing of the transverse sinus of the brain. It is a likely source of intracranial hypertension seen in patients with IIH. Click the term to read more. He said it was common and not a concern.

After my hospital stay, I was in worse pain than when I’d arrived, probably because none of this made sense. In the coming days and weeks, my family and friends couldn’t understand what was happening. How could doctors just let me stay in pain? What are the next steps Everyone wanted to know how they could help, but I didn’t even know. I needed a guide at this point, quite literally. By now, my vision was blurry all of the time, and it was impossible to work.

I learned, on my own, that IIH involves either an overproduction of CSF, or venous outflow issue or both. And, it was a rare condition. Not getting it treated could lead to permanent vision loss. I was worried about keeping my brain safe and wanted to protect my vision. How was this not urgent? I wasn’t willing to accept ‘borderline-ish’ as a diagnosis for something this debilitating.

I was privileged to take a medical leave from my job. My kind coworkers covered my workload, and my managers and benefits team set everything up for my leave. I was also fortunate to have family nearby to chauffeur me around, bring me meals, take care of some yard work, and help me set up a spreadsheet to keep track of phone calls to make. I was unprepared to ask for help, and had to learn.

I reached out to my primary care physician in tears. “Help, I’m completely lost and confused.” She explained:

• The neurologist should be the captain of my IIH team, ordering imaging and labs and managing any medications.
• The neuro-ophthalmologistMedical doctor who specializes in neurological conditions that affect vision. They can diagnose and treat conditions related to the optic nerve. Click the term to read more would monitor my vision and work closely with the neurologist.
• The endocrinologist would evaluate the empty sella to ensure my pituitary gland was ok.
• This seemed so simple, yet unknowable before. I’m a user experience designer, and the majority of my work is research, lots of which involves talking to people. I’m trained to ask questions to learn the most, but even when it’s second nature, that critical skill vanishes when overwhelmed and in pain.

The remainder of April was spent contacting specialists who were in-network for my insurance to grant me an urgent appointment (urgent = within a few weeks). Most often I’d hear, “that’s concerning, but not urgent.” Every office had a different way of gatekeeping the most precious and scarce commodity of all: the 15 minute appointment 4 months from now. Some needed a referral faxed from my primary care doctor, even though I had a PPO that allowed me to see specialists without one. Some needed the doctor to review my scans before they would agree that it was “urgent enough” which took weeks. I was in so much pain that I just needed someone, anyone to see me. I thought that once I saw a specialist, someone would get it, they’d know what to do, and I’d be on my way to treatment. It took weeks to get everything booked, and then several more weeks waiting for the appointments. It couldn’t get worse, could it?

Over the next couple of months:

• A neurologist insisted that I did not have IIH and wanted to give me an occipital nerve block; I declined and never went back.
• Another neurologist believed it was a migraine.
• An ophthalmologist noted slight papilledema and wanted to know, “What’s your plan for losing the weight?”
• A neuro-ophthalmologist, whose clinic sees IIH patients every week, did not see papilledema. He stated “I’m not concerned about your stenosis; lots of people have it” after I told him I was concerned. I asked if he wanted to take a look at the scans themselves, and he declined.

June 2023

At this point, I was beyond distressed. I’d spent two months of medical leave getting nowhere and knew I’d need to return to work soon. I spent most days learning as much as I could about IIH and stenosis and looking for stories like mine. I scoured social media, podcasts, PubMed, and YouTube. I found community and validation in IIH Hub and the /iih subreddit. There were many people like me, in pain, not being heard, and having their life put on hold. I was furious for all of us.

I was holding out very little hope for a positive experience, and still had a few weeks before seeing the neurologist #3. In the meantime, I had a routine appointment previously scheduled with my OB/GYN. I explained my recent health fiasco, and to my surprise, she was familiar with IIH. She believed I had it, and was empathetic to the fact that I’d gone this long without any medication to treat it. She prescribed a low dose of Diamox (AcetazolamideMedication used to treat glaucoma, epilepsy, altitude sickness, periodic paralysis, idiopathic intracranial hypertension, urine alkalinization, and heart failure. Click the term to read more) to try until I could see the neurologist. It worked, mostly. For the first time in nearly 3 months, I had a break from the constant headache. Talk about a plot twist.

Once I saw the third neurologist, things began to look up. He reviewed all the hospital notes, listened to me carefully, and agreed that this was not a migraine – it was IIH. He increased my Diamox dosage and I got more relief from the pressure and most of the pain.

Unfortunately, the medication made me exhausted, nauseous, and uninterested in food.* The little food I did eat gave me the worst acid reflux of my life. Also, I felt dehydrated no matter how much water I drank. But, less of a headache. This was potentially as good as it could get for now.

*Spoiler alert: I went on to lose about 10% of my body weight, which has had zero impact on the frequency or intensity of my symptoms.

July 2023

By mid-summer, I’d used up my medical leave and it was time to return to work. Unfortunately, I was still a patient who was a long way from relief and having my life back. I had several appointments a month, some of which required blood work or imaging. I work from home, and have full flexibility in my schedule, yet this was still a very challenging time for me.

One of these appointments was a repeat of my MRI. One of the findings was a filling defect involving the confluence of sinuses. I remember MyChart flagging this as a ‘Yellow Non-Critical Significant result.’ Very on-brand for my IIH.

My neurologist ordered a CTV to look into this more, and based on those findings, referred me to a neurosurgeonMedical doctor who diagnoses and treats surgical issues related to the brain, spine, and nervous system. Click the term to read more. I was scheduled quickly for a consult and soon our conversation was centered around the stenosis. Yes, that stenosis that everyone else was not interested in. The next step would be to find out if it could be repaired with a venous stent. Some surgeons perform a separate diagnostic angiogram to measure the pressure gradient of the left and right transverse sinus stenosis, and then place the stent(s) in a separate procedure. His approach would combine the diagnostic and treatment with stent(s), if needed, in one general anesthesia event. I felt validated and cared for, and thought I could return to a better quality of life soon. This was a huge win in so many ways. We scheduled the surgery, and I made arrangements at work and at home.

August 2023

My surgery was scheduled on a Monday. The Friday before, the hospital called. I was expecting them to let me know my arrival time and other details, but they were calling to say that my insurance company had denied the pre-authorization for the surgery. I dropped everything to make several phone calls. I learned that the insurance company had requested more information from my surgeon and team the week prior, which they provided. Despite all of the supporting evidence they provided as to why I needed the surgery, the insurance company decided that because it involved a venous stent, it was experimental, and they denied the pre-authorization.

Although my surgeon and team filed an expedited appeal, it wouldn’t be resolved in time, so the surgery was canceled. The despair I’d felt earlier in this process was nothing compared to this. I was gutted.

Not only did the expedited appeal get denied, but the subsequent appeal we filed also got denied. We waited all of August and well into September to find that out. I enlisted the help of a patient advocateTo self-advocate in your health is to communicate your needs Click the term to read more to navigate my next steps, but we had very few options available to us. I now know that once an appeal is filed, we lose the right to a peer to peer review, which might have helped us. This isn’t spelled out anywhere, and since we didn’t know to ask, we weren’t told.

September 2023

I decided to take a new approach and move all of my neuro care to a university hospital, which again, I am privileged to have as an option. I felt that the more advocates I had within a larger network, the better my chance at getting my insurance to cover the care I need.

My new neurologist admitted that she did not have many IIH patients, but promised to remove blockers for me wherever she could. And she delivered on it by connecting me with the interventional radiology (IR) and the neuro-interventional team.

December 2023

At my first appointment with IR, the goal was to determine if I was a candidate for a stent. Thanks to my previous workup, we had only a few more tests left: blood work, another MRV, and then a diagnostic angiogram. I warned my team about the insurance company denying the pre-authorization, but they felt confident that their approach and the data from the angiogram would build a solid case for the medical necessity.

I love science and data, but I can’t sugar-coat it: the 2.5 hour long angiogram was a harrowing, and at times, painful, experience. My IR team was caring and responsive, but since heavy sedation interferes with the pressure measurement, the goal was to use as little as possible to keep patients comfortable during this procedure. I was much more aware than I wanted to be, but I did get to view all of the vasculature in my brain, which was amazing.

The results? The criteria for a stent is a pressure gradient higher than 8. On my non-dominant sinus, it’s a 9, and on the dominant sinus, it’s a 10. And with that, we scheduled the stent surgery for January 17, 2024.

January 2024

Because of what I’d been through in August with my insurance company, I knew to check early for pre-authorization activity in my patient portal. Sure enough, two weeks ahead of surgery, the insurance company was asking for “proof of medical necessity.” I had an awful feeling about this. My neuro-interventional surgeon and his nurse practitioner provided everything that was requested. Denied.

Next, they requested a peer-to-peer review, which was scheduled less than 24 hours before the surgery, on January 16. At this point, I was already e-checked in, and had my arrival time of 5:30 AM. I’d been on dual antiplatelets for 2 weeks prior, and had driven the 40 miles each way to the hospital lab twice in one week to check that my blood was ready for surgery. I’d been masking up for weeks and had managed to avoid the colds and flus and COVID circulating that time of year. I’d made arrangements for the second time to be off work and put life on hold for the next two weeks. My mom had her snacks and reading material packed for the next day at the hospital. Everything and everyone was ready.

My neuro-interventional surgeon presented a strong case for the review. The neurosurgeon “peer” who did not perform neurointerventional procedures, determined that my surgery wasn’t medically necessary. The surgery was canceled.

My team requested a second peer to peer review, this time asking specifically for a neuro-interventional surgeon. Instead of responding, the insurance company asked for more documentation. The team responded to all of their requests right away, and have spent hours on the phone following up on the status of the request. No updates. The remainder of January and February was spent waiting.

March 2024

We recently learned that the insurance company “received no such request” and we had to submit the request all over again. The process is seemingly designed to confuse, delay, deflect, and push patients and their practitioners to either give up or appeal.

As I write this near the end of March, we still don’t have a response to our request. I consulted with the hospital’s financial services department, a patient advocate, and two insurance agents to see if there were other options so that I could have the surgery. I was told to continue pursuing peer-to-peer reviews, or appeal. Or pay out of pocket and assume a debt larger than my mortgage. And a range of other options in between that involve many more steps and lower odds of success. All for a surgery that many other carriers cover without issue.

Despite the roadblocks I’m facing with my insurance company, I think about how much longer it would have taken to understand what was happening with my health if I’d listened to the doctors I met in the beginning. I know everyone says to trust your gut, so I’ll say trust your headache. Your pain is real, and ‘borderline-ish’ does not serve anyone. I’m glad I trusted myself to push back when I wasn’t being heard. I’m glad that I still trusted other doctors to help me, without making assumptions as to whether or not they could help. There’s always going to be someone out there who can connect the dots, or give you the missing piece of the puzzle when you need it, you just have to keep asking.

If you would like to hear more about Nicole’s IIH Journey, please attend our upcoming IIH Community Chat as we discuss what it is like to navigate through the healthcare system. This chat session is open to everyone in the IIH Community (patients, caregivers, and physicians) – register here.