When we returned from India, I was unable to get into my neuro-ophthalmologist until the end of January. My retinologist was able to fit me in and she did a few more tests. During this visit, I had an eye angiogram in addition to the OCT and vision acuity exams. This is were they inject dye into your veins and take images of the blood vessels of the eye. My results during this appointment were that I had healthy blood vessels of the eye; however, she was concerned that I continued to have progressive symptoms and wanted to refer my to a retinologist at UCLA. I was able to get an appointment in the middle of February for the UCLA retinologist.
Also, the attending physician at Mayo Clinic called and spoke to me about my symptoms both previous and new since my appointment and recommended a few additional tests. Ultimately, I saw my primary care provider and she ordered a CT scan from my head to my pelvis to look for any potential cancers or other abnormalities that could cause an eye response from a systemic problem.
On December 23rd, I received a call from her that I had a nodule/mass in the lining of my lung (called the pleura) near the diaphragm as well as several borderline enlarged neck lymph nodes. Being in healthcare, I knew this location of the lung nodule was more concerning than other locations regardless of size. Also, lung cancer was one of the most common causes of paraneoplastic retinopathy. Obviously, this news ruined the holiday for us as we processed the "what ifs" and worried about getting seen quickly.
I was able to get a referral to a pulmonologist at a cancer center before the new year. Typically, the recommendation would be to get a repeat CT scan in 3 months to monitor growth; however, they were able to review a chest x-ray that I had earlier in the year (to rule out active TB) and a previous CT of the abdomen and the nodule was not present at either time. For that reason, the pulmonologist ordered a PET scan to look for activity in the lesion that would indicate certain types of cancer. Luckily, my insurance covered the exam and got the prior authorization quickly.
More to come on the PET scan results and next appointments.
Earlier in the year, we scheduled a trip in November to see my husband's family in India (my step-daughter lives there so we go yearly); however, we debated whether to go for months due to the situation with my eyes. We had already cancelled our personal vacation to the Maldives that we had added onto our visit to see his family (since we were able to receive a near full refund) but were unable to receive much of a refund for the India trip. Ultimately, since Mayo clinic gave me no answers, treatments, or next steps, we decided to go. Moreover, since my father-in-law is a physician, we knew I could receive care if absolutely needed. Knowing that whatever I was experiencing with my eyes was likely rare, my husband thought seeing a few specialists in India may be beneficial since the physicians there see such a high number of patients in comparison to the USA.
While there, I ended up seeing two neurologists. The first one did some neurologic exams which were mostly about equal strength and evaluations for movement disorders. As expected, these were absolutely normal. Also, he reviewed some of my testing from the USA and thought that perhaps my enlarged blind spot was due to a lack of focus during the exam. I attempted to tell him that the visual field test checks the position of my eye and focus every 15 seconds during the exam and scores it. I had perfect focus throughout the test and it was reproducible at different clinics and different time periods. Ultimately, this physician stated that I was just too "stressed". If you know me personally, you may have just laughed at this statement. At work and in multiple other situations, I am often looked towards to provide a calm and rationale presence. Oh well, another unsuccessful visit.
The second neurologist came to my in-laws family house and was very thorough in his physical exam and questions. While he wasn't able to determine a cause based on his exam, he did recommend a number of testing options to help rule out a number of additional causes that weren't previously considered. Many of these recommendations, we ended up doing when we returned to the USA. More on these results in a future post.
Finally, we visited my in-laws spiritual advisor, also referred to as a swami. Each time we visit India, we do go to the temple and perform rituals to bring good fortune to our family. This time the focus was on me and my eye health. First, I was weighed in relation to jaggery (a type of cane sugar), which was purchased as an offering to the temple. Then, I was to do 14 circles around a portion of the temple and give an offering to the gods each time we did a circle. From there, I was to stare at one of the Hindu gods and was asked to close my eyes. Swami then poured water into his hands and splashed the water onto my eyes. While I used splashed as the descriptive word, it would be better described as throwing the water at my eyes with shockingly good aim and force. This occurred two additional times with a Malayalam (one of many Indian languages) prayer or mantra (repeated words). Afterwards, my husband and mother-in-law were told that my eyes would heal. Swami speaks limited English so the majority of his instructions are translated through my family since I do not speak Malayalam despite multiple attempts.
You may be asking how my eyes did during this month since the majority of the discussion has been on the appointments. During November, my eyes progressively and substantially worsened. During this time, the floaters went from a few at a time to hundreds. I begin to see near constant clouds (or what could be described as fog or smoke) floating through my vision. The scariest thing that began to happen was that lights would appear to flicker on and off. My vision would go totally black and disappear for just a moment but this started happening multiple times a day. Furthermore, I began to have a spot in my vision which was moving and present in both eyes at the same location. When not focusing on it, it looked as though the spot was blinking, but with focus it was squiggly lines moving from the outside of a circle to the inside of the circle. This spot was most evident when looking at either the sky or a body of water but was always constantly there. Needless to say, my eyes were not improving and I began to worry that I would completely lose my vision if we couldn't figure out the cause. Since I am nearly deaf in one ear (which has been present since I was a child) and I use my vision to read lips to supplement my hearing, this truly terrified me. It felt as though if I lost my vision, I would lose a majority of my hearing at the same time.
After a few strings were pulled, I was able to get an appointment with the neuro-ophthalmology department to see one of their fellows on November 1st, 2019. During this appointment, I had brought an entire list of dates and things that I began to observe with my vision as well as explained to the best of my ability what I was seeing. As you would imagine, describing abnormalities in vision is not all that easy, but I did the best as I could.
Throughout the appointment, the fellow had great empathy but seemed to be focusing on symptoms that actually didn't bother me not did I bring up. For example, prior to my lasik surgery, I had extensive halos around lights at night but since I had that surgery in 2015, this was significantly reduced. So when she asked about halos, I stated yes but not bothersome, but this became the focus of the appointment rather than the loss in left peripheral vision, flashing lights, fog or clouds rolling through my vision, and extensive new floaters.
Nonetheless, they performed a few of the diagnostic examinations that I have had in the past including the OCT and visual field tests. My OCT was still normal with some difficulty obtaining the photo in the left eye as usual. My visual field test had an enlarged blind spot on the left as it had a few weeks prior. Furthermore, there were a few areas of nonspecific blind spots. After they performed these, they decided to look at the cornea (transparent part of the eye) closer to ensure that my lasik flap did not fail. With this exam, my lasik flap was normal but I had a few abrasions due to dry eye on my cornea.
After these examinations, the fellow saw me one additional time that day and more or less stated they had no explanation for what I was seeing. Perhaps my dry eyes were causing my blurring and if it got worse to come back. I asked a number of questions to redirect the conversation and advocate for myself, but this got me no closer to answers or further care. Needless to say, the worsening was why I was already there and which was an insult to the two physicians who had referred me to this department for more testing.
I was visibly upset when I left but as soon as I told my dad who what the outcome was, I broke down in tears. Ultimately, I bought a flight home the next day since I wouldn't be getting any further care. My brother-in-law wanted to try to see if I could somehow be seen again or contact patient services. I declined since it was an extremely disappointing experience and for the first time during the process of losing vision, I felt as though someone thought I was exaggerating or making up what I was seeing.
That evening around 7pm, the fellow called me after she discussed my case with her attending physician and he had recommended more testing as it could be a disease called AZOOR. They asked if I could stay to have the testing done the next week. Although I had already bought my flight home and it was less than 24 hours before the flight (so couldn't cancel for a refund or rebook), I decided to stay since they wanted to do 1 of the 2 testing that my Las Vegas doctor had sent me to get completed. Interestingly, my grandmother has AZOOR and has gone essentially blind in one eye. This disease is not thought to be genetic but often occurs in young females.
The next week, I had a manual visual field test performed which still showed an enlarged blind spot. As well a specialized OCT looking at a different layer of the eye. This specialized OCT didn't show any obvious abnormalities other than a mild thinning of the optic nerve on the left. The final diagnostic exam I had was an ERG (electroretinogram) which is like a EKG for the eye where they look at the function rather than the structure of the eye. This exam takes a few hours and is relatively uncomfortable, but manageable. This was one of the two exams why my Vegas doctor sent me to a tertiary center. Ultimately, the results showed no obvious defects (more on this on a later date...)
After all the diagnostic exams were completed, I meet with the fellow again and she offered reassurance. She stated it was unlikely but not impossible that they are missing something that is subtle and subclinical at this time. I was to contact them if there was worsening or new symptoms, but she was optimistic that I would improve.
Since I continue to write and tell my story, you can probably guess my vision didn't improve or even stabilize for that matter.
I was fortunate to be referred to a large tertiary center, which was Mayo Clinic in Rochester MN. My first appointments were scheduled with the neurology department. During this appointment, the neurologist who specializes in idiopathic intracranial hypertension was not convinced my eye issues were related to the results on the lumbar puncture and MRI; however, to ensure this he ordered a repeat lumbar puncture and a MRV (magnetic resonance venography, which is the same techniques as a MRI but the dye allows them to see the veins of the brain better). During this fist visit with neurology, he highly recommended seeing neuro-ophthalmology and he saw that the request from my Vegas physician was denied with no rationale so he re-requested for that department to see me. Furthermore, he tested my neurologic responses and I had no neurologic defects.
The next day, I had my MRV which was a similar experience to my previous two MRIs. And the following the day, I had the lumbar puncture preformed. This time during the lumbar puncture, they had me lie on my side in the fetal position while they took the fluid from my spine. Additionally, I was to continue to lie down for 20 - 30 minutes after completing to ensure that the spinal fluid redistributed. With these changes, I had no headache!
Meanwhile, through a handful of connections (old colleagues but mostly my brother-in-law who works for Mayo Clinic), I was finally able to get an appointment at the end of the week with neuro-ophthalmology department. Initially, it sounded like I would have to fly back a few weeks to months later to be seen.
After the MRV and lumbar puncture were completed, I saw the neurologist for my second appointment with him. My opening pressure during the lumbar puncture was at the upper limit of normal, but not elevated and there were no abnormalities on the cell counts. Interestingly, my MRV showed extensive signs of intracranial hypertension with nearly attenuated transverse venous sinus (nearly collapsed blood vessels on the back of my brain). So again, the results were incongruent.
Furthermore, my neurologist who is specialized in intracranial hypertension had no idea what this meant other than at the time, I did not have intracranial hypertension. He stated he thought about my case for an entire evening (and likely through the weekend), because it didn't fully make sense. He stated that I may be at risk for developing intracranial hypertension at sometime in my lifetime or perhaps I just had more spinal fluid volume causing the changes on MRI and MRV. Also, he was adamant that if they ever wanted to do neurosurgery for this, that I come back to see him at that time. To that end, I do need to get my eyes checked for a swollen optic nerves (papilledema) every 6 months to monitor for intracranial hypertension for the rest of my life since I have those changes on imaging.
Ultimately, my neurology appointments were helpful as they ruled out a condition that was questionable and I didn't need neurosurgery!!! On the other hand, it was disappointing to not know what was causing my vision loss.
Next up, I will describe my neuro-ophthalmology appointments at Mayo Clinic.
When evidence of intracranial hypertension was found on my MRI, my neuro-ophthalmologist ordered a stat lumbar puncture. With not driving, not knowing the date of the procedure and my husband out of town, I had a lot of anxiety how I was going to get to this procedure and back. To that end, outpatient stat means something very different than inpatient stat and it was ordered on Thursday and not performed until Monday when my husband was back so there was no reason to worry.
A lumbar puncture is also referred to as a spinal tap and a needle is inserted into your spinal fluid via your low back to test the fluid. This procedure was done by using an X-ray to find a good position. Unfortunately, I have scoliosis and needed to be an a relatively awkward position for them to get the needle in easily. Luckily, there were no issues once they found the correct position. Normally, you would wait 15 - 30 min after they removed the fluid for it to redistribute along your spinal cord and in your brain. Since this was an outpatient setting, they put the bandage on, gave me instructions on managing the injection site, and sent me on my way less than 5 minutes later.
I ended up having the worst headache of my life (and I have had viral meningitis and migraines in the past). For the first 2 days, I couldn't lift my head off of my pillow without my head pounding. The next day, I could sit up but no quick movements. Each day it slowly slowly got better, but it took me a complete week to be back to my baseline.
Around the same time as the lumbar puncture, I started having excessive exhaustion. It was persistent and some of the worst exhaustion I have experienced. One day after work, I slept 18 hours and only waking for a brief moment when my husband checked on me to ensure I was still alive. Most days, the exhaustion set in sometime during the midmorning (2 - 3 hours after waking) and often, I couldn't physically stay awake. I went from working out (high intensity interval training) three days a week or more to not being able to walk half a mile without a nap. This exhaustion continued for about a month and a half and nothing seemed to improve it. After some slow improvements and a change in meds, I began to improve a little each day. To this day, I still get overtly tired if I do too much computer work but not usually if I do anything physical.
My lumbar puncture showed I had elevated intracranial (brain) pressure and an extremely low glucose in my spinal fluid. With my neuro-ophthlamologist appointment 2 weeks after the lumbar puncture was completed and having my results, one of my other physicians prescribed a medication that can help with idiopathic intracranial hypertension, acetazolamide. This medication is a diuretic that can help in both intracranial hypertension and altitude sickness. Additionally, she referred me to Mayo Clinic neurology and ran a few more laboratory exams.
Once I saw my neuro-ophthlamalogist, my vision hadn't improved despite treating the potential intracranial hypertension for nearly 2 weeks. We discussed my results and although my brain pressure was elevated, it was considered indeterminate levels for intracranial hypertension. I had a pressure of 24 mmHg and 25 mmHg is diagnostic and 10-18 mmHg is considered normal. Also, it was unclear why my glucose was so low in my spinal fluid as it didn't follow a typical idiopathic intracranial hypertension picture. She began to doubt whether the intracranial hypertension was causing the vision issues since the pressure was not excessively high nor did the medication help. She had me stop the medication, which ended up getting restarted that weekend after my vision began to oscillate. Nonetheless, she wanted to refer me to a tertiary center which she added neuro-ophthalmology specialty to my Mayo Clinic visit. Typically, losing your vision due to intracranial hypertension is a neurologic emergency and neurosurgery should be conducted, but my picture was unclear and the risks may outweigh the benefits thus the referral was necessary.
More to come regarding my Mayo Clinic visit.
When there is sudden vision loss, the brain may be imaged. There are a number of different pathologies (causes) that they will be using imaging to rule out, but common ones include brain tumors, strokes, and multiple sclerosis. While a CT and MRI have similarities, each have advantages to be able to view different disease states. Depending on your underlying diseases and presentation, they may order one or both of these.
A CT of the brain (also called a CAT scan) uses special X-rays to get multiple views of the brain in order to get more information about the tissues and brain structure. During the CT, an X-ray beam moves in a circle around the body (or body part) to get many views. A CT can be done with or without an iodine-based contrast. CT contrast dye can be given orally and/or injected into a vein. Contrast dye can help certain structures be more evident; however, the decision if you receive contrast dye will be dependent on a number of factors including your renal function. The contrast dye is iodine-based so allergic reactions are rare but can occur.
To prepare for a CT of the brain, they will have you change into a hospital gown and remove all metal objects including piercings and jewelry. You will complete a screening tool to ensure that the CT is safe for you and there are no contraindications. If you are receiving IV contrast, they will place a peripheral (arm) IV in order to give you the contrast. You will lie on a table which will be moved into a circular machine, which is the CT machine. After you are positioned correctly in the machine, the technicians in the room will move behind a window to where the scanner controls are, but they will be able to speak to you via speakers. Once the exam begins, you will lie still for the duration of the exam and the machine will circle around you. Throughout the exam, there will be a number of whirling and clicking sounds which are normal. If they use IV contrast dye, they will either inject it at the beginning of the CT scan or midway through the exam after they have images without contrast. When it is injected, you may feel a sensation of flushing, nausea, a salty taste in your mouth, or even like you peed your pants. This will only be brief and will reside quickly. Typically, a CT of the brain takes about 15 - 30 minutes with most of this being the prep time and the actual exam only lasting around 5 minutes. Once the CT scan is complete, you will be removed from the machine and if you have an IV line, this will be removed.
A MRI of the brain uses a magnetic field and radio waves to get multiple views of the brain to get a 3D image of your brain. During a MRI, your body will be on a table that may move back and forth in the MRI machine which contains the magnets. A MRI can be done with or without IV contrast dye. This contrast dye is gadolinium which helps to visual blood vessels better; however, the decision if you receive contrast dye or not will depend on a few factors but primarily what structures they need to see. Since a MRI is a smaller space and is tubular, it is important to note if you are claustrophobic or not. If you are, they may give you a relaxing medication to help ease your anxiety during the exam.
To prepare for a MRI of the brain, they will have you change into a hospital gown and remove all metal objects including piercings and jewelry. You will complete a screening tool to ensure that the MRI is safe for you and there are no contraindications. If you are receiving IV contrast, they will place a peripheral (arm) IV in order to give you the contrast. You will lie on a table which will be moved into a tubular machine, which is the MRI machine. Since the sounds of the machine is loud, they may give you ear plugs or headphones to listen to music. After you are positioned correctly in the machine, the technicians in the room will move behind a window to where the scanner controls are, but they will be able to speak to you via speakers. Once the exam begins, you will lie still for the duration of the exam and the table may move back and forth a little to get images of different parts of the brain. Throughout the exam, there will be loud banging sounds which are normal. If they use IV contrast dye, they will either inject it at the beginning of the MRI scan or midway through the exam after they have images without contrast. When it is injected, you may feel a sensation of flushing, nausea, or a salty taste in your mouth. This will only be brief and will reside quickly. Typically, a MRI of the brain takes about 45 - 60 minutes with most of this being the prep time and the actual exam lasting around 30 - 45 minutes. Once the MRI scan is complete, you will be removed from the machine and if you have an IV line, this will be removed.
The optic nerve is a bundle of nerves that carry vision information from the retina to the brain thus optic nerve atrophy is a breakdown of that nerve. Ultimately, the retina can not send the impulses to the brain and this can lead to a loss of central, peripheral, and color vision and potentially blindness if the underlying condition is not addressed. Optic nerve atrophy is not a disease of in of itself, but it is a sign of a more serious condition.
Causes of Optic Nerve Atrophy
The causes of optic neuropathy are numerous. Causes include but are not limited to glaucoma, a stroke of optic nerve (anterior ischemic optic neuropathy), a tumor pressing on the optic nerve, inflammation of the nerve due to multiple sclerosis or other autoimmune conditions, infections, nutritional deficiencies, hereditary conditions, congenital defects, or medications and local radiation treatment.
Diagnosis of Optic Nerve Atrophy
Initially, your eyes will be dilated and inspected with an ophthalmoscope (in instrument) to examine the optic nerve. If optic nerve atrophy is present, the optic nerve will look pale due to a lack of blood flow to the area. Depending on what they expect the cause to be, different tests from there will be used. Often, due to the extensive differential diagnosis, a myriad of exams and laboratory tests will be conducted. This may include the aforementioned vision tests, a MRI of the brain, and blood tests may be considered depending on the likelihood of each of the potential causes.
There is no specific treatment for optic nerve atrophy since it is a sign of another disease. Once the cause is determined, that underlying condition will be addressed. Depending on the condition, vision may return back to normal or continue to progress. During this time, it is important to have regular vision exams conducted in order to monitor changes and if any sudden vision changes occur, to see the ophthalmologist as soon as possible.
During the second week of September (right after my first neuro-ophthalmology appointment), I was driving to work, which was only about a mile away, on a three lane road. I was in the middle lane and was paying extra attention due to my visual issues. I ended up slamming my brakes because I thought the car on my left was merging into my lane and was going to sideswipe my vehicle. Once I slowed to almost a stop, I noticed that the person was 100% in their lane. Luckily for me and everyone else, there was no one directly behind me.
What probably actually happened is they were coming up on the left and I was unable to see them approach. When I noticed them, they suddenly appeared from my blind spot thus they appeared to be moving to the right. Everyone was fortunate that day that I didn't harm anyone by suddenly braking on a normally busy road.
I am able to legally drive since my visual acuity is still good (multiple doctors have confirmed); however, the visual disturbances and blind spots make me hesitant to drive and are the reason since that day, I have chosen not to drive. Did I ever expect that at the age of 36 I would forgo my right to drive? Never. Was it an easy decision? Absolutely not; however, I was not willing to put others at risk due to my ego and desires.
Giving up the freedom to go where you want, need or to respond to situations is extremely difficult and aggravating. You can ask any elderly person who refuses to give up their license. Furthermore, if you have been to Las Vegas, you are probably aware that we are a completely car centric city. It is a sprawling metropolitan city with limited public transportation. Crossing the city using public transportation can take 2+ hours in one direction. Furthermore, we have limited bike lanes which are often shared-use with car traffic. With or without limited vision, biking for daily transportation is probably not the safest mode of transportation in Las Vegas.
In the first few months of not driving, I had moments of anger, despair, and frustration. When my husband didn't arrive when I thought he should, I would dissolve into anger instead of gratitude that he was willing to rearrange his schedule to help. To this day, 8 months later, I still struggle with this at times probably because I have always hated asking for help for any reason. Additionally, I have been driving since I was 14 years old (grew up in ND and it was legal at that time to get a license with special driving classes) and have always been on the go.
Will I ever fully accept this is my new normal? I don't know and the unknown can be scary. I renewed my drivers license so I could get the real ID and honestly, I will probably continue to renew it as long as I am able to pass the vision test just in case there is an emergency and I absolutely need to drive. Despite renewing my license, I rely on my husband, friends, family when they are in town, and Lyft or Uber when none of the aforementioned are available. Being reliant on others for daily transportation is a new experience that will likely continue to evolve.
After continued left peripheral blurriness, I was sent to one of the two neuro-ophthlamologists in Las Vegas. This doctor appointment occurs in two portions, which typically occur on two separate days. The first appointment consists of a number of visual tests and the second, the actual visit with the physician.
The vision testing included a typical vision acuity exam, color blindness exam, a depth perception exam, eye pressure test, visual field exam (Humphrey Visual Field - HCF), and an OCT (optical coherence test). Interestingly, during this appointment, there was a fire on the first floor of the building and we were evacuated. I was rescheduled later that week to complete my examinations.
During my first visit at the neuro-ophthalmologist, the majority of my initial examinations were normal at first glance; however, with testing in the future, it became evident that my visual field exam had early signs of an enlarged left blind spot. Important to note, that blind spots are normal and we have one on each eye; however, the other eye is able to see in that area so it does not cause issues. Additionally, my first OCT (which takes a picture of the retina) showed a decrease in the optic nerve thickness which was below normal at 65 microns (normal is 70-100) and significantly lower than my other eye which had a thickness of 104 microns.
During my visit with the physician, she listened carefully, took what I was experiencing seriously, and gave words of encouragement. If anyone is reading from Las Vegas or the surrounding area and would like a recommendation, please message me and I will forward you her office info. Due to the reduced optic nerve thickness, she ordered a ton of bloodwork to add to the bloodwork that I had during my hospitalization to help elucidate the cause of the optic nerve atrophy. Ultimately, they took 14 vials of blood and I was tested for the most common causes of optic nerve atrophy including: infectious, autoimmune, immune related, hematologic, metabolic, cancer, and vitamin deficiencies. As well as, a MRI of the eye orbit was ordered.
When I returned in two weeks, my eye exam showed an enlarged blind spot on the left, which with foresight was minimally evident at the first visit. For the most part the blood work was normal. I already knew that I had latent TB (tuberculosis) which was detected again (travel to India yearly), but additionally, I had a folic acid deficiency and some mild leukocytosis (indicative of inflammation) with high lymphocytes (antibodies) and eosinophils (allergy responders). Of note, latent TB just means that you have been exposed to the bacteria and are at risk for a future infection if your immune system is decreased.
The MRI of the orbit showed that I had tortuous optic nerves (zig zagged nerves) and fluid buildup around them. This indicated that the pressure in my brain (intracranial pressure) was likely too high so she ordered a stat lumbar puncture (spinal tap) in the outpatient setting. At this point in time, my physician was concerned that the TB was active in my brain causing the increased pressure and the optic nerve atrophy. The other possibility was idiopathic intracranial hypertension (also called pseudotumor cerebri), which is an increased pressure in the brain for an unknown reason but would account for the enlarged blind spot and the MRI results.
More to come on these tests, optic nerve atrophy, and the next steps.