SATURDAY
We left Long Island on Saturday afternoon after my best friend's first baby shower, which I wouldn't miss for anything in the world. We were a little late in heading out, because we cooked for for me to bring with us, since I can't safely eat at any old restaurant with all of my weird allergies lately.
I had what I call a "POTS breathing attack" in the car around 8 PM. What triggers these attacks, I have no idea, but it becomes very difficult for me to breath and I get really dizzy and twitchy feeling - like I'm being smothered and injected with pure caffeine at the same time. Normally, I lay down and try to distract myself from how uncomfortable I am, but that's pretty hard to do when you are in a car going 80 mph on a winding, bumpy road. For some reason, long car rides seem to make my symptoms worse, so pulling over and letting it pass wouldn't help. I would probably have another attack as soon as we started driving again. The only thing that seems to help these breathing attacks is my albuterol inhaler or an albuterol nebulizer treatment, although it doesn't feel like an asthma attack and the docs in the ER usually don't hear any wheezing, so I have to argue with them to give me the albuterol nebulizer for an "off label" use since you are only supposed to get it if you have wheezing.
We made it about half-way across PA on Route 80 by 10 PM and decided to find a side-of-the-highway hotel. First we stopped at a Holiday Inn, but when I went into the room, it smelled like brand new carpet and paint... mmmm, nothing like the smell of fresh Volatile Organic Compounds in the morning! The windows in the room didn't open, so we just couldn't stay here. I checked out other rooms, but the entire hotel was in the process of being painted and re-carpeted, so this was an asthma attack waiting to happen. The front desk was nice enough to refund out money so we could find another hotel. Just up the block there was a Comfort Inn, and there was no fresh paint or carpet anywhere in sight - it was just run down enough to fit my breathing needs. The parking lot was filled with rusty pick-ups and a racing team truck, and there was a disproportionate number of middle aged fat guys with mullets hanging out in the lobby. Thankfully, I am not allergic to mullets (although I wish I was). Anyway, we woke up as early as we could on Sunday morning and headed out for Cleveland.
SUNDAY
It was a pretty uneventful drive on Sunday. There were some strange road signs in western PA and Ohio along the way. We saw a sign for the Jersey Shore when we were about 10 miles from the Ohio border - that was pretty random considering we were in the middle of nowhere. There were also signs on Route 80 in western PA that said "Buckle Up, Next Million Miles." I bet the Department of Transportation guy who coined that term thought he was pretty clever. My husband most enjoyed the "70 mph Speed Limit" signs when we first hit the Ohio border. He decided that meant he could go 90 mph, instead of his normal 75 mph. Did I mention I live with Speedy Gonzales?
The first thing we did when we hit Cleveland around 3 PM was search out the Rock and Roll Hall of Fame. Yup, there it is. I was surprised that it is right on Lake Erie, since they don't show that in any of the photos. We didn't go inside because they close at 5:30 PM and it's $22 per person. If I'm shelling out $66, we are going to be there more than an hour or two. Maybe later in the week.
Then we checked into the Embassy Suites hotel. They gave us a room on the 13th floor. I didn't realize we were staying in a skyscraper, which kind of freaked me out. Ever since 9/11, I have been really afraid of tall buildings. This actually started a few days after 9/11, when my family and I went to volunteer at Ground Zero with the American Red Cross. We were stationed in the large Marriott right next to "the pile" and we were cooking and serving industrial sized portions of food for the Ground Zero workers. I was walking kind of fast carrying a large tray of brownies in the kitchen, and I tripped and almost fell down an open elevator shaft. I have never been so spooked in my whole life. The elevator doors had been pried open and broken when they were looking for people, but apparently a beam from the Trader Center had plunged through the roof of the Marriott and damaged the elevators. There was nothing besides a one-inch strip of yellow police tape between me and a 20+ story plunge down an elevator shaft. And no one had warned the volunteers about the elevator shaft - perhaps because they didn't know about it. As you can imagine, things were a bit disorganized and chaotic down there in the first few weeks following 9/11. Anyways, that's what triggered my fear staying in tall buildings. I highly doubt Al Qaeda is planning an attack on Cleveland, but I was still not happy about staying on the 13th floor. So the front desk was nice enough to move us down tot he 5th floor, which is the lowest floor they have guest rooms on. OK, but I'm still not thrilled about it.
Sunday night I had another one of my POTS breathing attacks, and my inhaler did not help. It was getting worse, so after about a half-hour going downhill fast, we finally called 911 and I was taken to Cleveland Clinic by ambulance. This was not the introduction to Cleveland Clinic I wanted. The ambulance EMTs wouldn't give me an albuterol nebulizer treatment "off label" because I wasn't wheezing, but as soon as I got to the ER, a respiratory therapist came to see me and he knew about POTS (what a relief!) and he gave my oxygen and an albuterol nebulizer treatment. Within 5 minutes after the treatment, my breathing was back to almost normal and I was ready to go home - but of course, you never get out of the ER in 5 minutes. We got there around 9:30 PM, and we didn't get back to the hotel until 3 AM.
MONDAY
Without much sleep due to our ER visit on Sunday night, we managed to make it to my Monday morning appointments. I met with Dr. Frederick Jaeger, a cardiologist specializing in autonomic disorders. He was very nice and he did not rush me through the appointment. He answered my questions and spent a good deal of time reviewing my past medical records - no small task since I came armed with two 3-inch binders filled with over 1000 pages of medical records and over 15 CDs of prior MRI, X-Ray and CT studies. We organized them by month, put sticky tabs on the interesting findings, and made a ten page "cheat sheet" list of every test I had since I've been sick (putting the abnormal test results in bold font) to help the Cleveland Clinic docs be able to sort through the relevant and not so relevant info.
Dr. Jaeger definitely knows his stuff. Prior to my visit he had just chaired a section of a cardiology conference on autonomic disorders. He told me the experts are thinking about renaming POTS and separating it into two different named illnesses. One would be the type of POTS more closely associated with post-viral and deconditioned patients, and the other would be the type of POTS associated with neuropathy - which is the type that I have. He didn't indicate that they have agreed upon new names for these subclasses yet, but he said something about acute pandysautonomia for my type of POTS, which is a term I have seen used by other top researchers. To breakdown that term, "acute" means it's an active process and that it came on suddenly, "pan" means widespread or throughout the whole body and "dysautonomia" means there is a dysfunction of the autonomic nervous system. So combined together, that means a sudden onset of active autonomic nervous system dysfunction spread throughout the whole body. POTS may have been easier to remember, but acute pandysautonomia is a more accurate and descriptive term than POTS is, at least for POTS that is related to autonomic neuropathy. He did not mention what they have in mind as far as renaming the post-viral or deconditioning type of POTS. I will try to find that out form him before I leave.
After I saw Dr. Jaeger, I was sent to a pulmonary consult, since my bad breathing episodes are one of my worst symptoms. They are sending me for a battery of pulmonary tests including a chest CT, spirometry, a 6 minute walk test and a cardio pulmonary stress test, which they told me is pretty much me going full blast on an exercise bike until I collapse. Sounds like fun.
Dr. Jaeger also lined me up with a neuro consult, which is probably the most likely to be able to figure out what the heck is actually causing my neuropathy. I met with Dr. Chemali, who was also super nice. He spent three hours going over my history, examining me and answering all of my questions. He did not look at his watch even once during the exam and our appointment went long after the rest of the office had closed up shop. A perfect gentleman and a very sharp doctor. He trained under Dr. Chemlinsky, who trained under Dr. Philip Lo at Mayo Clinic. Dr. Lo is the guy who "practically invented POTS" according to a friend of mine who is a doctor at Mayo Clinic. Dr. Lo invented the QSART machine and was heavily involved in writing the first major research paper in which they coined the phrase "Postural Orthostatic Tachycardia Syndrome." Before Dr. Lo's journal article, POTS had been known by numerous different names, which made it harder for researchers to track how many people had it and made it harder for practitioners to have reliable diagnostic criteria. Anyways, back to Dr. Chemali - he is sending me for neurological tests I was begging my NYC neurologists for, and the NYC docs didn't even seem to know what those tests were. This week and next, I am scheduled for a Thermoregulatory Sweat Test, a QSART test (which I already had today), a lip biopsy to look for Sjorgen's Syndrome, a stomach fat biopsy to look for amyloidosis, a foot biopsy to look for small fiber neuropathy, and a bunch of blood tests to look for genetic, autoimmune and other possible causes of my neuropathy. I had everyone one of these on my "rare diseases I want to be tested for" list I had come up with after extensive research. Dr. Chemali wants to run all of those tests, and I didn't even have to ask for them - he just said "OK, this is what we're going to do..." So happy to find a doctor ho is on the same page as me! Although he's definitely many pages ahead of me since this is his field of expertise and I'm just a newly minted medical research junkie. He also mentioned tests for some other rare diseases I haven't even heard of (my research must be slipping!) so we can rule those out too.
I am relieved and also a little nervous about getting the results from all of these tests. If everything comes back negative and I leave here without any answers, I will be emotionally devastated. But if one of these tests comes back positive, it probably means that I have a lifelong degenerative disease to look forward too - also emotionally devastating. So, even though I try to be a "glass is half full" kind of patient, I know that now matter what, I'm probably going to leave here in tears. But laying in bed at home wasn't getting me anywhere and there is always a chance that if they figure out what is causing my neuropathy, that we can tailor a treatment plan to try to slow it down or minimize the symptoms - and maybe someday they will have a "cure" for whatever rare disease this may end up being. Better to know what is coming my way that to go around confused for the rest of my life!
Stay tuned for more news in the coming days....
We left Long Island on Saturday afternoon after my best friend's first baby shower, which I wouldn't miss for anything in the world. We were a little late in heading out, because we cooked for for me to bring with us, since I can't safely eat at any old restaurant with all of my weird allergies lately.
I had what I call a "POTS breathing attack" in the car around 8 PM. What triggers these attacks, I have no idea, but it becomes very difficult for me to breath and I get really dizzy and twitchy feeling - like I'm being smothered and injected with pure caffeine at the same time. Normally, I lay down and try to distract myself from how uncomfortable I am, but that's pretty hard to do when you are in a car going 80 mph on a winding, bumpy road. For some reason, long car rides seem to make my symptoms worse, so pulling over and letting it pass wouldn't help. I would probably have another attack as soon as we started driving again. The only thing that seems to help these breathing attacks is my albuterol inhaler or an albuterol nebulizer treatment, although it doesn't feel like an asthma attack and the docs in the ER usually don't hear any wheezing, so I have to argue with them to give me the albuterol nebulizer for an "off label" use since you are only supposed to get it if you have wheezing.
We made it about half-way across PA on Route 80 by 10 PM and decided to find a side-of-the-highway hotel. First we stopped at a Holiday Inn, but when I went into the room, it smelled like brand new carpet and paint... mmmm, nothing like the smell of fresh Volatile Organic Compounds in the morning! The windows in the room didn't open, so we just couldn't stay here. I checked out other rooms, but the entire hotel was in the process of being painted and re-carpeted, so this was an asthma attack waiting to happen. The front desk was nice enough to refund out money so we could find another hotel. Just up the block there was a Comfort Inn, and there was no fresh paint or carpet anywhere in sight - it was just run down enough to fit my breathing needs. The parking lot was filled with rusty pick-ups and a racing team truck, and there was a disproportionate number of middle aged fat guys with mullets hanging out in the lobby. Thankfully, I am not allergic to mullets (although I wish I was). Anyway, we woke up as early as we could on Sunday morning and headed out for Cleveland.
SUNDAY
It was a pretty uneventful drive on Sunday. There were some strange road signs in western PA and Ohio along the way. We saw a sign for the Jersey Shore when we were about 10 miles from the Ohio border - that was pretty random considering we were in the middle of nowhere. There were also signs on Route 80 in western PA that said "Buckle Up, Next Million Miles." I bet the Department of Transportation guy who coined that term thought he was pretty clever. My husband most enjoyed the "70 mph Speed Limit" signs when we first hit the Ohio border. He decided that meant he could go 90 mph, instead of his normal 75 mph. Did I mention I live with Speedy Gonzales?
The first thing we did when we hit Cleveland around 3 PM was search out the Rock and Roll Hall of Fame. Yup, there it is. I was surprised that it is right on Lake Erie, since they don't show that in any of the photos. We didn't go inside because they close at 5:30 PM and it's $22 per person. If I'm shelling out $66, we are going to be there more than an hour or two. Maybe later in the week.
Then we checked into the Embassy Suites hotel. They gave us a room on the 13th floor. I didn't realize we were staying in a skyscraper, which kind of freaked me out. Ever since 9/11, I have been really afraid of tall buildings. This actually started a few days after 9/11, when my family and I went to volunteer at Ground Zero with the American Red Cross. We were stationed in the large Marriott right next to "the pile" and we were cooking and serving industrial sized portions of food for the Ground Zero workers. I was walking kind of fast carrying a large tray of brownies in the kitchen, and I tripped and almost fell down an open elevator shaft. I have never been so spooked in my whole life. The elevator doors had been pried open and broken when they were looking for people, but apparently a beam from the Trader Center had plunged through the roof of the Marriott and damaged the elevators. There was nothing besides a one-inch strip of yellow police tape between me and a 20+ story plunge down an elevator shaft. And no one had warned the volunteers about the elevator shaft - perhaps because they didn't know about it. As you can imagine, things were a bit disorganized and chaotic down there in the first few weeks following 9/11. Anyways, that's what triggered my fear staying in tall buildings. I highly doubt Al Qaeda is planning an attack on Cleveland, but I was still not happy about staying on the 13th floor. So the front desk was nice enough to move us down tot he 5th floor, which is the lowest floor they have guest rooms on. OK, but I'm still not thrilled about it.
Sunday night I had another one of my POTS breathing attacks, and my inhaler did not help. It was getting worse, so after about a half-hour going downhill fast, we finally called 911 and I was taken to Cleveland Clinic by ambulance. This was not the introduction to Cleveland Clinic I wanted. The ambulance EMTs wouldn't give me an albuterol nebulizer treatment "off label" because I wasn't wheezing, but as soon as I got to the ER, a respiratory therapist came to see me and he knew about POTS (what a relief!) and he gave my oxygen and an albuterol nebulizer treatment. Within 5 minutes after the treatment, my breathing was back to almost normal and I was ready to go home - but of course, you never get out of the ER in 5 minutes. We got there around 9:30 PM, and we didn't get back to the hotel until 3 AM.
MONDAY
Without much sleep due to our ER visit on Sunday night, we managed to make it to my Monday morning appointments. I met with Dr. Frederick Jaeger, a cardiologist specializing in autonomic disorders. He was very nice and he did not rush me through the appointment. He answered my questions and spent a good deal of time reviewing my past medical records - no small task since I came armed with two 3-inch binders filled with over 1000 pages of medical records and over 15 CDs of prior MRI, X-Ray and CT studies. We organized them by month, put sticky tabs on the interesting findings, and made a ten page "cheat sheet" list of every test I had since I've been sick (putting the abnormal test results in bold font) to help the Cleveland Clinic docs be able to sort through the relevant and not so relevant info.
Dr. Jaeger definitely knows his stuff. Prior to my visit he had just chaired a section of a cardiology conference on autonomic disorders. He told me the experts are thinking about renaming POTS and separating it into two different named illnesses. One would be the type of POTS more closely associated with post-viral and deconditioned patients, and the other would be the type of POTS associated with neuropathy - which is the type that I have. He didn't indicate that they have agreed upon new names for these subclasses yet, but he said something about acute pandysautonomia for my type of POTS, which is a term I have seen used by other top researchers. To breakdown that term, "acute" means it's an active process and that it came on suddenly, "pan" means widespread or throughout the whole body and "dysautonomia" means there is a dysfunction of the autonomic nervous system. So combined together, that means a sudden onset of active autonomic nervous system dysfunction spread throughout the whole body. POTS may have been easier to remember, but acute pandysautonomia is a more accurate and descriptive term than POTS is, at least for POTS that is related to autonomic neuropathy. He did not mention what they have in mind as far as renaming the post-viral or deconditioning type of POTS. I will try to find that out form him before I leave.
After I saw Dr. Jaeger, I was sent to a pulmonary consult, since my bad breathing episodes are one of my worst symptoms. They are sending me for a battery of pulmonary tests including a chest CT, spirometry, a 6 minute walk test and a cardio pulmonary stress test, which they told me is pretty much me going full blast on an exercise bike until I collapse. Sounds like fun.
Dr. Jaeger also lined me up with a neuro consult, which is probably the most likely to be able to figure out what the heck is actually causing my neuropathy. I met with Dr. Chemali, who was also super nice. He spent three hours going over my history, examining me and answering all of my questions. He did not look at his watch even once during the exam and our appointment went long after the rest of the office had closed up shop. A perfect gentleman and a very sharp doctor. He trained under Dr. Chemlinsky, who trained under Dr. Philip Lo at Mayo Clinic. Dr. Lo is the guy who "practically invented POTS" according to a friend of mine who is a doctor at Mayo Clinic. Dr. Lo invented the QSART machine and was heavily involved in writing the first major research paper in which they coined the phrase "Postural Orthostatic Tachycardia Syndrome." Before Dr. Lo's journal article, POTS had been known by numerous different names, which made it harder for researchers to track how many people had it and made it harder for practitioners to have reliable diagnostic criteria. Anyways, back to Dr. Chemali - he is sending me for neurological tests I was begging my NYC neurologists for, and the NYC docs didn't even seem to know what those tests were. This week and next, I am scheduled for a Thermoregulatory Sweat Test, a QSART test (which I already had today), a lip biopsy to look for Sjorgen's Syndrome, a stomach fat biopsy to look for amyloidosis, a foot biopsy to look for small fiber neuropathy, and a bunch of blood tests to look for genetic, autoimmune and other possible causes of my neuropathy. I had everyone one of these on my "rare diseases I want to be tested for" list I had come up with after extensive research. Dr. Chemali wants to run all of those tests, and I didn't even have to ask for them - he just said "OK, this is what we're going to do..." So happy to find a doctor ho is on the same page as me! Although he's definitely many pages ahead of me since this is his field of expertise and I'm just a newly minted medical research junkie. He also mentioned tests for some other rare diseases I haven't even heard of (my research must be slipping!) so we can rule those out too.
I am relieved and also a little nervous about getting the results from all of these tests. If everything comes back negative and I leave here without any answers, I will be emotionally devastated. But if one of these tests comes back positive, it probably means that I have a lifelong degenerative disease to look forward too - also emotionally devastating. So, even though I try to be a "glass is half full" kind of patient, I know that now matter what, I'm probably going to leave here in tears. But laying in bed at home wasn't getting me anywhere and there is always a chance that if they figure out what is causing my neuropathy, that we can tailor a treatment plan to try to slow it down or minimize the symptoms - and maybe someday they will have a "cure" for whatever rare disease this may end up being. Better to know what is coming my way that to go around confused for the rest of my life!
Stay tuned for more news in the coming days....
Just letting ya know I am here with you. Please keep me updated. I am a fellow Potsie from LI. Saw an awesome doc in Cornell recently. Hopefully the CC will give you better guidelines then the other NY docs. GOOD LUCK
ReplyDeleteNot sure if I missed this, but how long did you have to wait to get an appointment with Dr. Jaeger?
ReplyDeleteDear Anonymous,
ReplyDeleteIt took me 3 months to get an appointment with Dr. Jaeger, but that is much shorter than waiting for Mayo Clinic's autonomic experts. Vanderbilt or Dr. Grubb.
Wherever you go, good luck!
I don't know if you will see this but this evening I had something similar to your breathing attack and it has never happened before. I found you via google :-p
ReplyDeleteI was just diagnosed two weeks ago so I am new to the confusing world of POTS. Any insight?