Had to Quit Being a Firefighter, But Became a Fighter for Patients
State: New Jersey
Congressional District: NJ02
Blood Disease, Gastrointestinal Disease, Immune Disease, Mental Health, Migraine, Neurological Disease, Rare Disease
Issues and Challenges
Jen has encountered: Rare / Underserved Disease, Gaslighting, Invisible Illness, Medical Devices, Step-Therapy / Fail First
(Disclaimer: In the story we will be taking a deep dive into the different conditions that impact my life, their origins, the procedures/devices I required to improve my quality of life, and also the impact of the trauma and complications I encountered during treatment. There will be a trigger warning directly before any talk of sensitive topics. I would also like to state that while I do have extensive medical knowledge, every patient is different and no two journeys are the same. The ideas in this story should not be taken as a substitute for medical advice. Thank you for reading!)
October 3rd, 2016. I still remember that brisk autumn day like it was yesterday. The morning air was cool and crisp as I left for my shift in a local suburban town. I had been both excited and nervous since I had finally earned the right to drive my squad’s rigs. My partner drove for the first half of the shift and after lunch, it would be my turn, but when we stopped to grab food I realized I couldn’t read the menu. Suddenly my vision was becoming blurry. I had been suffering from terrible migraines and other odd symptoms for several months, but my doctors couldn’t explain anything at that time. I had always been sick as a child, so I just figured I would get over it at some point as I had always seemed to. But this was different. My vision was slipping away and there was nothing I could do.
I left work early and immediately went for an emergency appointment with my eye doctor. She told me very calmly 'Your optic nerves are completely inflamed, it’s called Frank Papilledema. I’m operating under the suspicion that you have Pseudo-tumor Cerebi. It’s a rare condition that can cause headaches and blindness due to the build-up of cerebrospinal fluid (CSF is a water-like fluid that surrounds and cushions the brain. Too much or too little of this fluid can cause severe and even fatal complications). You need to get to the emergency room immediately or the damage could be permanent. A lumbar puncture (spinal tap) can help drain the fluid and relieve the pressure on your brain; effectively restoring your vision'.
Now, when I first heard the term “Pseudo-tumor” the condition didn’t sound that scary, or even dangerous. The name denoting a “fake tumor” seemed so benign in itself, so I was fairly calm when I got to the ER. It sounded like it would be a simple fix, but I was wrong. Before I continue I would like to note that the proper and more commonly used name for this condition is Idiopathic Intracranial Hypertension (IIH for short), due to the benign nature of the original name.
At the hospital, most of the residents and attending physicians made a big fuss over wanting to peer into my eyes and see my optic nerves. It was rare to see Papilledema, especially as severe as mine. I let all of them look because for one moment I finally felt like everyone believed me when I said I was sick. However one doctor, after checking my optic nerves, was under the impression that we were dealing with optic neuritis that would be indicative of Multiple Sclerosis. From there on, my team decided to ignore my ophthalmologist’s notes. This was a grave error because in those notes she had stated that if my pressure were to increase any more, I could run into complications and even more damage to my optic nerves. At this point, I was admitted, and placed on steroids to treat MS.
Sadly, steroids are one of the worst things you can give a patient with IIH. I remember laying in my hospital bed, having a conversation with my mother and a friend of mine who had come to visit. I was speaking and then I paused mid-sentence. All of a sudden, I was unable to speak or comprehend what was being said to me. My body felt weirdly heavy on one side, and my head was killing me. I was suffering from a TIA (Transient Ischemic Attack aka mini-stroke). I was then taken for an emergency lumbar puncture and my opening pressure was 26 (anything over 20 is indicative of raised intracranial pressure, and since I’m a much smaller individual it’s even more pertinent).
Realizing their error, my team had begun to panic. I was subsequently diagnosed with IIH and flown via helicopter to a hospital in the city that was better equipped to care for me. I was told I needed emergency surgery. Specifically, I needed a shunt placed that would continuously drain the fluid building on my brain. Unfortunately, the hospital in the city was not so easily convinced. With IIH being such a rare condition, research and literature being so limited, and also there being a stigma surrounding patients with IIH; I wasn’t taken seriously.
The stigma that currently surrounds IIH is that it’s a relatively benign and self-limiting condition with an easy treatment. Most physicians also believe that only overweight women of childbearing age develop IIH and that the only way to cure it is for the patient to lose mass amounts of weight. Please keep in mind that I was 5’0, 139lbs, and had just spent the past six months undergoing intense physical training to achieve my Firefighter 1 certification. So, I was quite baffled when I woke up in the other hospital and was chastised for being “overweight”. They pulled another lumbar puncture, prescribed medications, gave me pamphlets on diets and losing weight, and sent me on my way.
Just to briefly explain the science of why lumbar punctures are utilized in this scenario: Think of the body as nothing more than glorified plumbing, everything is connected. When there is a narrowing or kink in the tubing, fluid backs up and pools in one location; adding stress and pressure to other pipes. The brain and spinal cavity are directly connected. A lumbar puncture essentially drains the excess fluid from the spinal cavity, therefore, relieving the pressure on the brain. However, this method is archaic and ineffective. The body replenishes CSF every 6-8 hours, so only a constant drain can truly provide relief. The other issue with lumbar punctures is that they are highly invasive, excruciatingly painful, and can cause permanent nerve damage. I went on to have 53 performed in the past 6 years and sustained severe nerve damage to my back and legs. During the time I was being treated with LPs, I was also placed on a series of diuretic medications that help to force the excess fluid out of the body in an attempt to alleviate the pressure more consistently (Diamox and Lasix). But one of the primary issues with these medications is that when overprescribed, as they often are, they can cause severe symptoms and heavy damage to patients’ kidneys. I was still trying to keep working at the time, but the more my dosage was increased the more unbearable it became to be active. I eventually gave in and applied for disability benefits, but that is a different story for another time.
My mother grew restless in our search for answers. Being a nurse, she knew I was suffering and that my condition was more dangerous than most doctors presumed. She began researching and discovered a specialty hospital with a Cerebrospinal Fluid Disease Center and felt an overwhelming twinge of hope upon discovering that they were revolutionizing a new procedure that could restore the proper flow of CSF in my brain, alleviate my symptoms, and potentially restore my vision. We met with the doctors and I was officially a qualified candidate due to the stenosis of my right transverse sinus in my brain. The surgery itself was a less invasive angiogram. The stent was placed without incident and upon waking up, I truly could see better. Sadly it didn’t last.
Due to the severity of my intracranial pressure, the stent simply couldn’t hold. I even tried having a second one placed, but it just couldn’t withstand the pressure. At this point, I had no other choice than to opt for the shunt. I’ll never forget the way my mother cried and begged for other options. She knew the risks better than anyone. Her mother had died due to a shunt malfunction that nobody noticed. As tough as it was to have that thought in the back of my mind, I had to move forward. I wanted my life back, and at the time, I didn’t care what it would cost me. But before I would be a candidate, I would need to undergo ICP monitoring. While lumbar punctures are good for reading pressures in an emergency setting, they only provide a quick look or a snapshot in time of what your pressure is doing. ICP monitoring, while highly invasive, is the gold standard due to its in-depth look at every variable in the activity level of CSF for one specific patient. The process takes three days and the surveillance is 24/7. The procedure itself involves drilling a small burr hole into the skull and implanting a short wire and sensor, which is secured with a bolt, into the intracranial space to deliver real-time reads of intracranial pressure. There is even a built-in component that releases a numbing agent upon removal so that the patient does not feel pain during extraction. I personally have a love/hate relationship with this procedure, but at this stage in my journey, it showed my doctors just how much I was truly suffering. A few months later, I was prepped and on the table for shunt placement.
I will start this next part by saying that there are many different kinds of shunts. In the Hydrocephalus community, the most common types are VP (Ventriculoperitoneal) and LP (Lumboperitoneal) shunts. While their locations in the body may be different, their components are the same. Each shunt is made up of a valve, a short catheter that sits in the intracranial space to drain fluid, and a long catheter that uses the force of gravity to drain CSF and transport it to a location where it can dissolve safely and effectively. Unfortunately, shunts are a rather imperfect device and are widely known for their frequent malfunctions and complications. With such a high failure rate and a near-constant need for revisions, they are one of the most underdeveloped human-use devices on the market. Due to this fact, as well as the many rare and unfortunate complications I faced, I would go on to have 10 procedures (2 stents, 3 ICP monitors, and 5 shunt procedures) in the past 6 years. When I initially had my shunt placed (summer of 2017), I did get short-term relief from my symptoms. I was having fewer headaches, my vision was improving, and I was doing well despite the hardships I faced in the post-op period. However, like many patients, I experienced my first shunt failure within a few months after my first procedure. My valve and catheter were faulty and a revision was necessary. As a patient who was getting repeated brain surgery, I learned to expect the unexpected. But I never could’ve anticipated what would happen next, and how it would affect me 5 years later.
December of 2017 was particularly challenging for me. I went from feeling like I was getting my life back and feeling like a normal 23-year-old, to having it all slip from my grasp quicker than ever before. It was frustrating, and I was bitter. I was staying in the hospital to have my initial shunt valve replaced. I remember waking up after the first surgery of that series and actually feeling much better. I remember being so relieved, but my team noted that there was an issue with the catheter placement on my post-op scans. They were concerned that if it wasn’t fixed, I would only end up in this same situation all over again. So after a day of rest, they went back in. Waking up after that surgery was different. I felt infinitely worse for reasons I couldn’t understand. No pain medication in the world would help me. That’s when a sea of white coats nervously approached my room. The resident I recognized had his head down as he opened my door, and that’s when I knew something was horribly wrong.
(Trigger warning: The will be a vivid description of surgical complications and death in this paragraph)
“There’s been a complication, and we do not know how to respond because we’ve never seen it before. You’ve developed a cyst along your shunt tract that we cannot surgically remove, but it’s growing quickly. If we don’t find a way to fenestrate it, you will die. But you just had surgery, and there are also other complications. We can’t guarantee how you’ll come out of this, if at all. Take some time to think, call us when you decide.”
I remember sitting in silence for the longest time with a blank look on my face. I felt defeated like there was just no point to how hard I had fought to even get here. I was so angry at everything, everyone, the world… and so I kicked everyone out of my room so I could cry in private. It was the first time I had cried in months. I had been holding myself together so well because I sincerely believed I needed to be strong for everyone else. But now, I felt broken. But I couldn’t stay that way for long. I threw myself back together and read the surgical contract, pen in hand. I knew what I had to do, but I needed the strength to do it. I knew I had to fight. I knew I had to at least try. So I signed that contract with my shaky right hand, hit the call bell, gave it to the somber resident from before, and was told they’d come to get me in the morning. That night, I lie awake like a new soldier on the eve of an important battle. I was hyper-aware of everything. The sound of quick sneakers against a linoleum floor, the clicks and beeps of monitors and equipment, and the sounds of a lively city just outside my window. Sleep was an impossibility, so I resolved to just wait. In the early hours of the morning, I heard the ding of the elevator and could tell by the distinct footfalls and pace they approached that it was my doctor and that it was time. They wheeled me down to the surgical ward. I tried to crack jokes to make my mother and the transport aide smile. I’m not sure if they did it to humor me, or if I’m just that funny. I had always hoped it was the latter. We then pulled up to our stop, OR 3. It was always OR 3 for some reason, and that made me feel somewhat more at home in the darkness I was trying so hard to embrace. I gave my mother one last goodbye and to this day (even writing this) I still cry thinking about how I hugged her like it was the last hug I’d ever give. She left for the waiting room, and I left for whatever awaited me beyond the daunting metal doors of OR 3. They asked me if I could stand and walk on my own and, even though I was a little unsteady, I said yes because I wanted to take in every last moment and sensation. I regretted it for a moment when I realized the implication of what I was about to do, but I pulled from every last ounce of courage I had left and approached the surgical table. Putting myself on the table that would likely kill me was the hardest thing I’ve ever done in my entire life. As a child, I had always been terrified of death after learning about it too early, but here I was staring it in the face. They strapped me in, hooked me up to meds and monitors, and as I drifted further into the gentle embrace of sedation I remember thinking how disappointed I was with my life and how I would try harder next time. Next time was a nice thought. All of a sudden there was brightness, for a second I thought I really had died. But then there was pain, and I’d never been so happy to feel pain in my entire life because it meant that I was alive.
Considering what I had gone through, I had thought I’d walked away from that experience without any real complications. But as it turns out… I had also suffered a bleed somewhere in that series of surgeries which would set the stage for my future, but we’ll get to that towards the end. After my 7th procedure, I realized I was falling into this vicious cycle of feeling better and then declining and I didn’t understand why. I had a working shunt, so why was I not improving. Surgeries 8, 9, and 10 were a direct result of me asking this question repeatedly. At first, we had thought we’d found the answer in the notion that my body was sensitive to pressure fluctuations and that I needed an anti-siphoning device to provide even more settings and control over my current shunt valve. But that did very little to help me. We started to wonder if there were other causes. I knew that many things could cause fluctuations of pressure, and in the 4 years I had been sick I had developed plenty of other symptoms and issues, I just hadn’t given them much thought because my brain took up priority. But it was the summer of 2020 that would bring answers.
I had been trying to lose the weight I gained from back-to-back surgeries. But somehow all of a sudden I went into the worst stomach problems of my life and ended up with constant GI bleeds that no doctor could explain. I underwent test after test, and I was so desperate for answers. I was in the hospital more often than not, in crippling pain and unable to move. On occasion, my inflammatory markers and sed rate would come back sky high, and other times they would be stone cold. My labs were only a small part of the picture though. My pathology results from my colonoscopies were nearly inconclusive. There was deep inflammation but it didn’t fit what you’d typically see from Crohn's or Ulcerative Colitis. My doctors were scratching their heads until one day one of them asked me, “Do you ever get oral ulcers?” I took a moment to think about it. I had oral ulcers for as long as I could remember but they seemed so insignificant that I never reported them. That’s when she told me about Behçets Disease.
Behçets Disease is an ultra-rare type of Vasculitis, and it was the auto-inflammatory answer I’d been searching so desperately for. Having Behçets is like having Lupus, Crohn’s, and MS all at once. All of a sudden everything made sense. Hours of pouring over my labs from the past several years, researching genetics and family history, and documenting my day-to-day symptoms all made sense. Vasculitis was one of the answers in my mind due to the plumbing theory I mentioned previously. Vasculitis is inflammation of the blood vessels and causes changes in the blood vessel walls. The constant action of vasoconstriction and vasodilation that this inflammation causes could weaken those vital structures, causing the stenosis of the veins in my brain and effectively causing my IIH. Behçets is also known for its ability to inflame multiple body systems at once or all together at any given time. It can be caused by genetics or environmental factors. For me, it was a case of chronic Epstein-Barr virus that set the stage for Behçets. My immune system was already so delicate as a child, and suddenly every medical mystery of my life finally made sense. At first, I was at peace but then the realization set it. The gravity and magnitude of the condition I now knew I had consumed me. Things would be more complicated from here on out and I had to be ready to take the good with the bad. But as I tried to stand up and dust myself off to continue the fight, I was hit unexpectedly and fell to the ground once more.
It started internally, a rattling in my bones that couldn’t be seen. I didn’t tell anyone because they would probably think I was crazy. At this point, most people looked at me like I already was, or like I was one push away from slipping off of the deep end. But when the facial twitching started I realized I wasn’t crazy, yet I remained quiet since I knew it could potentially just be nerves reattaching from surgery. Then I developed tremors in my hands. I knew where this was going, and it was the exact one I had feared since my first procedure all those years ago. My mother would bring up the risks of developing Parkinson’s very often during my appointments, but my doctors mostly brushed it off. In their minds I was young and it would be a long time before I had to worry about such things. They said not to worry about the future, but the future had come early. After learning I had Parkinson’s, I was devastated.
Mind you, this is strictly the medical side of my story. There was so much happening in my personal life at the time. I lost so much in the all-consuming fire that was my chronic illness, that I was left with virtually nothing when the smoke cleared. People I thought I would always have by my side, fled the moment things got tough. Even family members. I learned not to get attached to anyone or anything and became virtually emotionless.
I didn’t want to feel the way that I did, but having knowledge about all of my conditions and how they would impact my life was too much for me. I was doing a lot of great work as a patient advocate and as a peer support mentor, but I didn’t really have any support of my own. I desperately needed an outlet, and I found it in the most unlikely place. I had always loved gaming. I took my Nintendo Switch with me every time I was in the hospital. The ability to be transported to a far-off land and be immersed in lore and incredible stories was my saving grace. My doctors and nurses would always chuckle when they would catch me playing Skyrim or Breath of the Wild late at night. Sometimes they would even stay and tell me what games help them relax when real life becomes too much. One day my friend Ryan invited me to a group of friends he plays games with on Discord. I had never really been one for social gaming because I would get self-conscious if I wasn’t as good as other players. But this group of people was different. When I was with them I was more than my disability. They made me feel like a normal person. Slowly I came out of my shell and even learned how to love myself again. The best part is that video gaming is excellent for my hands. It’s a great way to keep my tremors at bay and maintain dexterity, and I get to play with my friends. I learned I didn’t have to shoulder all of my burdens on my own, and even learned to trust again. This was around the time I realized that I had spent the past 6 years so caught up in chasing answers and fighting to stay alive, that I had forgotten to live. I spent so much time mourning my loss of freedom, only to realize that I had been the one holding myself prisoner. But that also meant that I was the one with the key. As patients with chronic illness, we are often told to “just be positive”. But I’m here to tell you that you can both accept that your life is difficult and choose to live with rebellious hope despite that. We are human. We can’t be positive all the time, nobody can. But we do choose how we react and respond to the hardships we face.
Shifting my perspective of how I saw my life was one of the most difficult things I ever had to do. How do we get beyond the bitterness? How do we reconcile the hand we’ve been dealt? In my youth, I was always obsessed with finding my purpose. I was frustrated that a sense of purpose seemed to come so easily to those around me. During the darkest times of my journey, I would often wonder if the reason I couldn’t find mine was that maybe I wasn’t meant to have one. Maybe this was it. Nothing terrified me more than thinking my life was a waste and that this was all I would ever amount to. Especially coming from being a person whose soul existence was centered around saving lives and helping people. But over time, I learned that there was more than one way to save lives. That was how I became involved in advocacy. That was also when I realized that maybe my purpose was to survive so that I could pave the way for others through sharing my story and my experiences. I had a unique opportunity to lend my voice in support of legislation and innovations that would save people just like me, and suddenly all of my sufferings were worth it. I won’t lie to you and say that there aren’t days that I wish my life was easier. I’m only human. But I wake up every day knowing that my purpose is to save lives with my voice, and that itself is more than enough.
I have some words of wisdom for anyone who might be reading this and finds themselves in situations similar to mine.
Firstly, always trust your instincts. The human body is a miraculous thing. It will tell you what you need and when something is wrong, so listen to it. Even when the doctors gaslight you, and even when your lab results come back “normal”. Trust yourself, and remember that you are your own best advocate.
Secondly, do not try to shoulder the weight of your experiences on your own. Let people in. I waited way too long to start letting people in, and things could’ve been so much easier if I didn’t put so much pressure on myself to “be strong” and bottle it all up.
Thirdly, be kind to yourself and your body, and remember that you are only human. It’s so easy to get swept up in feelings of bitterness and insecurity, but you have to remember to appreciate the vessel that is fighting to keep you alive.
Lastly, don’t get so caught up in chasing answers, and mourning the life you had before, that you forget to live. It’s okay if it takes a while to stand up straight after being hit with the cruelty of this world. It’s okay even if it takes years, even if you have to learn how to stand all over again. Just as long as you try, just as long as you fight. Even as you feel your legs buckling with the weight of what you now carry upon your back, stand tall. Because eventually you will start to move forward again, and when you do… more will follow in your footsteps.
My Motivation and Inspiration
Not only does sharing our stories have the ability to shape the legislative policies and innovations of tomorrow, but it also holds the prospect of saving lives. Knowledge on its own is power, but sharing that knowledge is a gift only we can give.