Chronic Illness Warrior
Updated: Jan 12
What is going on? This part of my story up until today, mostly just the facts of the past year, as complete as I can remember......
~March 2018: I couldn’t ignore it anymore. I could not work out without feeling light headed, sometimes feeling as if I would pass out and with sudden increased headaches. I starting planning only short burst HIIT workouts from 3 minutes of activity, then 2….then only 1. I WAS SCARED TO WORK OUT. I didn't want to admit something was really wrong. I wanted it to just pass.
~April 2018: I got a referral for ENT due to chronic nasal sinus issues and allergies not being relieved with Claritin. When going over my notes the doctor saw that I claimed “unilateral pulsatile tinnitus that can be stopped when I press on the side of my neck”. He said, “I know you are here for allergies and sinus but we need to talk about that. Have you had an MRI for it?” – I didn’t remember that I had one in 2010, so I said no. He then explained, “One out of 4 people who have unilateral pulsatile tinnitus have a physical cause. It can be something like an aneurysm, you need a neurology consultation.”
While I waited for my referral ...
~May2018: Headaches (front behind my eyes, back of head on the right side, and down neck) became more and more noticeable, increasing in frequency and duration. Note: Like a frog in a pot, I likely never noticed that I had daily headaches for years. First because I ate so poorly and drank alcohol, a headache may have been normal and passed off as due to one of those factors. It wasn’t until it elevated to the point where the pressure came down the base of my skull making is difficult to turn my neck (much like whiplash) that I knew there was much more to the headaches than just a headache. (it hit it’s all time high in July, pre-medication)
~June 4, 2018: MRI/V/A revealed the left sinuses of my brain were missing. Ok – those are my words. The medical terminology was, “Impression: 1. Dominant right transverse sinus. Possible stenosis at the junction of the transverse and sigmoid sinus. This can be developmental or be secondary to increased intracranial pressure such as idiopathic intracranial hypertension/pseudotumor which can be associated with tinnitus. 2. Hypoplastic left transverse sinus. 3. Otherwise unremarkable noncontrast examination. In general, contrast enhanced MR venography along with noncontrast examination is a better and more complete evaluation. If symptoms persist, a contrast enhanced brain MR venogram could be performed. 4. Empty Sella Note: The radiologist noted possible stenosis, possible association with IH/PTC, and empty sella.
June 26, 2018: Post MRI follow up and First appointment with neurologist A. This was the first time in 41 years that I encountered a doctor I did not believe was actually in her profession to heal people or to be an advocate for her patients. From the moment she walked in, I felt like a client.
Neurologist A walked in with a red snakeskin looking oversized purse as her doctor tool bag and plopped it down on the desk like she was annoyed just to be there. It only went downhill from there. Without discussing my concerns, she read off the computer checklist and decided I must have just had three different types of headaches. Migraine, sinus/tension and neck stiffness. She hadn’t even put a stethoscope to my body yet and already prescribed three different medications. When pressed to order a lumbar puncture, one of the first tests recommended to diagnose or dismiss Intracranial Hypertension/IH (Pseudotumor Cerebri), she said, “I believe a lumbar puncture is invasive and unnecessary”. To which I replied, “I believe signing up for 60 years of medication when there are tests to rule out other, possibly fixable, problems is irresponsible”. She relented and referred me to interventional radiology to request a lumbar puncture. I filled the prescriptions (Topamax, Xanaflex and eh something else) and began my medication routine.
July 13th, 2018: First appointment with Interventional Radiologist A – he was great. He ordered a lumbar puncture. And went on to explain more about IH than the previous docs had. He also suggested that I may have had the hypoplastic transverse sinus my whole life but that the symptoms likely exacerbated with trauma like a head injury or pregnancy. (Note: This will come into play later when I explain how the dots connect back to 2009 in a future post.)
During the month of July my HEADACHES GOT WORSE. BY THE TIME WE LEFT FOR PARIS I WAS ALMOST COMPLETELY UNABLE TO TURN MY NECK AT ALL.
Aug 27th, 2018: Lumbar puncture/LP #1 – Cold, sterile room under xray. Not a bad experience at all. He hit the nerve a few times but from other people’s reports, mine was pretty good. When it was done I was shuttled out quickly. Results from LP#1 : For a “normal” person, The Cerebral Spinal Fluid/CSF pressure, measured at lumbar puncture (LP), is 8-15 mm Hg. Some say “Average is 15”. My Neuro-ophthmolgist said anything over 20 is “high”. Most neurologists claim anything over 25 is definitively “high”. My opening pressure was 24. I say 24 is “close enough” to a neurologist’s high and is certainly over the norm.
BY SEPTEMBER IT WAS GETTING MORE AND MORE DIFFICULT TO GET OUT OF BED IN THE MORNING. AS AN OVERACHIEVER, ADVENTURE SEEKER AND HELPER OF OTHERS. I DID MY BEST TO FULFILL MY COMMITMENTS TO MY FAMILY, FRIENDS AND BUSINESS, BUT ANYTHING BEYOND THAT WAS TURNING INTO A HARD NO.
Sept 7, 2018: Lumber puncture follow up with Neurologist A. She didn’t seem to care that my lumbar puncture revealed elevated intracranial pressure. She certainly didn’t apologize for her previous bad attitude as she continued it at this visit. She did immediately prescribe me Diamox, thus in my opinion silently admitting my diagnosis of Intracranial Hypertension/IH, a rare disease. However, when Joe informed her that I am allergic to Sulfa drugs (Diamox is a Sulfa drug), she blinked at him. SHE BLINKED AT HIM as if to non-verbally say, you are an idiot, I am the doctor. She never acknowledged his concern. I basically tapped him to move on, knowing that we would never see this provider again. (Note: For anyone wondering, Diamox is a sulfa medication but it is different that sulfa antibiotics and deemed safe for me to take by Neurologist B and I have been on it since.)
I have a rare disease
Later that day, I messaged customer relations within the health system I had been a patient in to speak to the head of neurology to discuss my experience. I got a call almost immediately and received an apology. I was asked to reconsider leaving the practice completely and agreed to be reassigned to Neurologist B.
Oct 1, 2018: First appointment with Neurologist B. This doctor came into the room with a better presence than A, but his first 15 minutes were the same. Read the computer, spouted off the same info, told me meds are the answer. When he asked what I wanted from him, I almost lost it. I explained that “we (Joe and I, since he was with me through all of this) had been pushed from doctor to doctor. No one wanted to claim me and dive into what is actually going on. That we believe specialists are there to be a member of our medical team and that we are supposed to work together to solve problems”. At this point his entire demeanor changed. He turned towards us, moved closer, and began really having a conversation. He answered questions about Diamox and the Sulfa allergy and what to expect from their office. I told him we may go elsewhere for a second opinion he said, “If you choose to go for a second opinion, go to Johns Hopkins they are the experts in Intracranial Hypertension”.
AND OUR JOURNEY TO JOHNS HOPKINS BEGAN.
For the months of October and November I was on Topamax and Diamox. Between those and chiropractic, I was able to move around better but Topamax was not a good med for me!
(Note: Topamax. I will forever consider it Dopamax. Some people do well on this medication, I did not. I had slurred speech and often couldn’t find the words I wanted to speak. I often apologized midsentence because of it. I was more fatigued than ever. My irritability was at an all-time high, I didn’t know this was due to the Topamax, though, until my sister was also put on it and she had the same symptoms magnified to frightening levels. Easy to say, we both concluded the use of that medication.I didn’t make the connection until November 2018. Months after being on the med.)
BY FALL 2018, MY ABILITY TO FUNCTION LIKE I HAD JUST 9-12 MONTHS PRIOR WAS GONE. I FELT LIKE MY BODY WAS FAILING ME. ANXIETY AND DEPRESSION SET IN. I COULDN'T VACUUM THE FLOOR WITHOUT GETTING WINDED. ALONG WITH THAT CREPT IN SOME OLD BAD EMOTIONAL HABITS, LIKE NIGHT SNACKING.
~Oct 2018 A Neuro-Ophthmalogist confirmed Intracranial Hypertension as visualized with Papilledema (swollen optic nerves).
~Nov 2018: I submitted my paperwork and MRI CDs to Johns Hopkins to be considered as a patient in their Pseudotumor Cerebri clinic, often regarded as one of the best in the world, and also the one referred by Neurologist B. Received a FEDEX delivery confirmation two days later. But the couldn't find my package. My MRI CDs we're lost!
My MRI CDs we're lost!
~Dec 2018: Johns Hopkins still could not find my MRI discs so I got new copies andresent them.
~January 2018: I was accepted as a patient with the “best” in the PTC clinic at Johns Hopkins. Dr. Abhay Moghekar. I was given an appointment dates of February 13th and 14th, arranged for my mother to come to Baltimore from NY for the appointment because Joe could not miss an inspection at work. I was set.
~Feb 1st: Received a call that I was told February 13th and 14th but SHE REALLY MEANT March 13th and 14th! GRRRRRR. Ok.
I was told the wrong month!
After my initial disappointment, I realized the change in schedule was a blessing because Joe could go with me in March but he couldn't have in February.
-March 13, 2019: Lumbar puncture and CT scan at Johns Hopkins. OK let me tell you ---- I know it isn’t the case for everyone, but for me, Johns Hopkins was like a television hospital. The GOOD television hospitals. We got to park less than 50 yards from the door of the neurology clinic. We arrived early because we weren’t sure of traffic, and they took us in early. The lumbar puncture doc had an incredible bedside manner, explained everything thoroughly and, without using an x-ray, she didn’t hit a nerve once! After my lumbar puncture, the doc turned off the lights and let me lay there resting for a half hour, with Joe by my side the whole time. Then, I was given snacks after the CT scan! SNACKS!!
March 14, 2019: First meeting with Dr. Abhay Moghekar. He is what all doctors should aspire to be. He was personable, humble and confident. He first asked about my experience thus far and then explained what we were going to discuss. He pulled up a computerized 3d model of the brain and explained how the venous system works and what he was looking for in my CT scan. I had already researched him and researched his research, so I believed I knew what he was going to say, but I had never heard that one cause of venous stenosis can be “enlarged arachnoid granulation pushing on vein from outside”. When he got to the part about stenosis he pulled up my scan and THERE IT WAS. No further explanation needed. Severe venous stenosis. He then used the computer measuring tool and showed us that it was over 90% stenosed. Although the CT was not tuned to pick up any arachnoid granulation (that would be the brain matter, not vein fluid movement) he said that based on the shape of the stenosis, that could be the cause. --- The recommendation: Venous Sinus Stenting. He went over the risks and reward, saying this is the only option that can potentially “cure” me because it fixes the cause (the stenosis). Joe asked, “if it was your wife, what would you do”? Doc M replied, “she would get the stent”. So we chose that I would, too.
I would get a stent in my brain
Questions that needed to be answered before I go through with stenting:
1. Am I allergy to nickel?
~April 5, 2019 - confirmed I am allergic to nickel.
~ April 8, 2019 Answer as posted by Joe: Today the Drs at John’s Hopkins conferred and they let us know that they will not proceed with the surgery to place a stent in Carrie’s transverse sinus. There is currently no other option for material that is FDA approved and neither the Drs nor the hospital can take on the liability that could come from placing nickel into the head of someone with a nickel allergy. The news is a blow to all of us. Currently we are awaiting word as to whether they will continue with the planned angiogram or not. Other than that, the options are to continue treating the symptoms with drugs or to have a shunt placed. Neither of which are high on our list of “solutions.” Carrie is exploring options for stent procedures in other countries and we continue to pray. Thank you all for your support through all of this.
~April 23, 2019 Cerebral Angiogram. Angiogram went fine. My arteries are small so instead of a collagen plug a doctor had to hold my femoral artery until it clotted, which it didn't do on time so I got a really cool (not so cool) hematoma and he got to hold it longer. Then 5 hours of bed rest followed by a sudden blood pressure drop which added two more hours of bed rest. Because, well, I don't do anything easy.
I got my brain probed, my femoral artery held closed by hand, and had a momentarily frightening blood pressure issue.
~April 24, 2019 Appointment 2 with Dr. Moghekar -Number one cool thing: We were under the impression my left side transverse sinus was completely hypoplastic with no flow but my left side transverse sinus DOES have some flow, not previously noted! Could just be more sensitive test equipment, could be my new chiropractor, either way... All God. It doesn’t change much as far as way ahead but it could lead to options down the road.
We discussed options with Dr. Moghekar and there will be NO SHUNT in my near future! He was as adamant as we were that a shunt is not a good option for me at this point. (My symptoms were caught early enough and I am able to maintain a semi normal, although different that previous, semi active lifestyle with meds.)
The one way to “cure” my intracranial hypertension is still only a stent due to the transverse sinus stenosis. The FDA panel will meet this fall and make some significant decisions on whether there will be a consensus as to allowing those with nickel allergy to have stents with any percentage of nickel alloy. If not, it will prompt stent manufacturers to figure out a better, nickel free one. This could take a long time.
~April 26, 2019 A deeper look at my angiogram was performed and a mild case of another rare disease called Fibromuscular Dysplasia (FMD), which was present in my right carotid artery. By itself, this would be considered nothing to worry about and just something to pay attention to as I get older. With my IH symptoms almost exactly the same at cerebral FMD symptoms, this warrants more testing. I currently await to be scheduled for a pelvis to head CT scan of my arteries to make sure I don't have signs of FMD near my kidneys (there are no indications that it has, this is exploratory only), etc
So, I have two rare diseases
While we wait for follow on tests and medical technology advances, etc....
The current way ahead is to do everything we can to be healthy and mitigate side effects of medications.
Healthy living and regular testing is prescribe as follows: Daily multivitamins, Rehydrate drink daily, weight management etc. Quarterly neuro ophthalmologist and kidney function appointments. Annual bone density screenings.
Exercise: I can do cardio but almost nothing more. No lifting, no yoga/plyometrics, no isometrics. If we go more than a few miles, Joe will have to carry my hiking pack. I think he is up for the job.
CURRENT STATUS: January 2023
Mental/Emotional: I am CONFIDENT in my medical team at Johns Hopkins, their diagnoses, expertise and recommendation. I am SCARED and ANXIOUS over a major surgery/procedure but I am choosing to be FOCUSED on the Promise that all things are working or my good.
Energy: This is may seem like a selfish plug for my business, but I could not function without IDLife Energy and Personalized Vitamins now more than ever. Caffeine is actually recommended for relieving headaches. The b-vitamins help me focus. This disease itself truly messes with people's energy. The meds, too, cause fatigue. The lack of sleep adds insult to injury. Being emotional often keeps my mind awake late into the night. It hits from multiple angles.
Physical: I have constant pulsatile tinnitus in my right ear. It is louder than most other sounds so, although I don't have actual hearing loss, I often cannot hear out of that side. Although I do have more mobility in my neck than last summer, I have lot of stiffness and pressure radiating from the base of the skull making it hard to find comfort to sleep. Headaches are my norm. If you ask me how my head is and I say, "eh fine" that means I am likely to participate in society that day - it does not mean I actually feel "good". But I believe I will again.
Vision: My double vision is not as bad as it was before Diamox, but it is still mildly present. I may have actually had double vision for years without knowing because I believe this is how I have seen for a while not knowing it isn't "normal". The grey spot on my right eye comes and goes, typically worse at the end of the day before my next dose of Diamox. This may be the case, it may be in my head. LOL, pun not intended.
Grateful: I know this is an emotion, but it warrants its own category. My family and friends have been so supportive and flexible during this. I am truly blessed.
My continued prayer:
God, remember me? I trust You for the right doctors at the right place and the right time. So, I won’t cry too much over the set backs that come my way. I could have focused on all that went wrong, and You know there were days I did, but I prayed You would lead me and I have patience and faith that You have and You will. When my emotions get the better of me, please send someone who will point me right back to You. And.... if it is in Your will, heal me, because I know You can.