When it's not one thing, it's another!

So, I got to be strapped to a table and treated like a lab rat a couple weeks ago.

I completed the autonomic testing with Dr. Barboi. It was pretty horrible! Going without my medications for 24 hours was the hardest part. I could barely be upright or stay conscious during that time, and I was absolutely miserable. My medications clearly make a huge difference in my quality of life and level of functioning.

The testing had 4 parts, all terrible for me in their own way. None of these tests would even feel like a strain on a healthy person, but they definitely showed my body's weaknesses.

First was the QSART sweat test. They applied a solution to several spots on my skin, then sent a low level of electricity to them. It was rather painful for me, but ended up being the easiest of the tests.

Next, we did a deep breathing test. You just take slow deep breaths for a couple of minutes, and they watch how your vitals react. I learned many years ago that I can't do any type of controlled breathing- for meditation or yoga- or I pass out. And that's what happened! Even though I was lying down, I came in and out of consciousness a few times. To be fair, I was nearly passed out before this test, so it didn't take much.

After that was the valsalva test. I had to exhale really hard for 15 seconds through a tube. Unfortunately, this action triggered my hemicrania facial pain to spike to a level 10. It really didn't come back down to below an 8 the rest of that day. Any type of exertion or straining triggers my pain, so this was absolutely horrible for me. I cried in pain for a bit afterward.

But, we weren't done yet! Finally was the tilt table test. I've had two TTTs before, which confirmed my diagnosis of POTS. So I knew what to expect. I was strapped to the table and tilted up to an almost upright position. Then, I just had to wait there while they watched my body's reaction. I got my usual hot flashes, nausea, vertigo, and lightheadedness. Luckily, this only went on for about 7-8 minutes before Dr. Barboi said he had seen all he needed.

I was then able to take my medication and drink water to help me recover. I felt significantly improved after a short time, although still pretty out of it and in severe pain. They wheeled me into the next room for my consultation with the doctor. He said the testing showed that I have another new diagnosis. Seriously!?! I was correct that at some point, my POTS has evolved into Hyperadrenergic POTS.

My basic understanding of the condition is this: I still have trouble with vasoconstriction, thanks to my stretchy EDS blood vessels. So, I still have problems pumping oxygen to my brain a lot of the time. But my body has started over-reacting to this problem with a surge of sympathetic activity. Epinephrine and norepinephrine are released. My body goes into fight-or-flight mode, causing heart rate and blood pressure elevation, hot flashes, enlarged pupils, tremors, shortness of breath, anxiety, and headache.

There are any number of triggers for this hyperadrenergic state; mainly being upright, exertion, stress, and any change my body has to cope with. This has been a major problem for me since my surgery in 2013. Any trauma or change in my medication or routine cause me to have trouble.

So, I'm not surprised to have a new diagnosis, and I always like to have an explanation for my symptoms. I also like when the testing proves my self-diagnosis correct. But, I am a bit exasperated to have another rare diagnosis to add to the list.

This definitely explains why I feel incredibly anxious the day after a restless night. My body really relies on quality sleep to maintain homeostasis. It also probably explains my body's reaction to new medications and dosage changes recently.

My well-being is a very delicate thing, and I get thrown off very easily. This is generally a lifelong condition, and can be progressive.

For treatment, we are starting with a new medication to manage my postural tachycardia- Ivabradine. We're starting with a small dose, so we don't overwhelm my system. Unfortunately, my insurance would not cover this medication (No surprise. It's fairly new in the US, and only approved to treat heart failure.) After looking into patient assistance programs, it became clear I needed to order it internationally. It's been ordered, but will take some time to arrive. I expect to be able to try it out in the next couple of weeks.

The doctor had other treatment ideas for me to try in the future, including adjusting my psychotropic medications. I'm pretty reluctant to try this, since I know my mental health is quite delicate as well, but I'm sure I will try it sometime when I feel stable enough.

With so many rare diagnoses, I'm always dealing with a number of problems. I never know when I wake up each day which symptoms will give me the most trouble.

My head has finally given me a little break from the relentless pain. I still have an ongoing low level headache with lots of sensitivities that trigger more severe pain, but it hasn't been my most prominent symptom most days. I'm very grateful for the break! It just doesn't always feel like one. My body never really gives me a break!

Currently, I'm dealing with a major mast cell reaction to a couple of mosquito bites. I've had to take two different extra anti-histamines every 2-3 hours just to not scratch all of my skin off. It has definitely interfered with my sleep.

I am constantly trying to avoid exposure to mast cell triggers. Mosquitoes would be a great one to avoid! But also all types of scents, smoke, and fumes. I have a new mask that definitely helps a bit. But still have to avoid known triggers.

I've also been having various joint problems. The most difficult one has been my shoulders, which don't want to stay in the socket. I've been experimenting with different mattress toppers to try to find something that allows me to lie on my sides to sleep. I'm waiting for a new, 4 inch memory foam mattress topper to try to help.

I've also had the usual pain in my knees, feet, and hips. Today, my right knee is the worst, but it could be something else tomorrow. I've also had a lot of pain in my hands, and some trouble with my spine and sacrum. I have a wide variety of braces that help a bit to wear as needed. I also have to be surrounded by pillows for support most of the time.

I had a flare up in my brain fog and trouble staying conscious for about a week. I had gotten a bit busy and neglected my biking a bit, which always has a major effect on how alert I feel. I've gotten back on track this week. It's just difficult, because I can't usually exercise on days that I have other things planned. So, I have to schedule several days off each week, and sometimes miss out on things in order to exercise.

I've had a couple of days where my digestive symptoms were most prominent.

It is all so frustrating. And it really is true that it's always something. So next time, instead of asking, "How are you doing?" you may as well ask, "what's giving you the most trouble today?"

At least when I'm not having too many other problems, I'm actually sleeping at night, and I don't feel too anxious or depressed most of the time. I know that this can and will change again, but I'm enjoying the relative peace, for now.

Surviving, often reluctantly

I think I have a lot to update on since last month.

Unfortunately, I had to discontinue the Indomethacin before I could reach the full daily dose. I had another adverse reaction. The kind where my chest starts burning, then it spreads throughout my torso. It's really intense, and Zantac and Tums don't seem to help it. It is still probably a GI reaction, but nothing seems to prevent it. And it is such a strong reaction that I'll do pretty much anything to avoid it.

So, I probably have the chronic headache condition called hemicrania continua, but I can't treat it. Great, right?

I've had daily head pain every day, made worse by varying triggers like scents, storms, lack of sleep, and exertion. I've pretty much been afraid to take any medication for it. My pain level has been between a 4 and a 7 most days, with some 8s. I am very grateful it hasn't been an 8 every day though. I've returned to about 3 distinct migraine days per week. Although, every day feels like I'm on the verge of a migraine. So, I never know when it will actually turn into one. My hemicrania pain is on the left side. My definite, throbbing migraines are on the right side. And some days, it just hurts all over, and I can't tease apart the different layers of headache.

I also had my appointment with Dr. Barboi last week! I was in a major flare the day I was there. I pretty much fainted while checking in, and my blood pressure was very low throughout the appointment. Dr. Barboi is wonderful. I don't know if there's much that can be improved upon in my current management of my illness, but he's going to try to help. But before he tries anything, he'd like to do a day of autonomic testing, including a tilt table test (my third). He wants to confirm that my current diagnosis for my autonomic problems is correct, so he has a good idea how my system is malfunctioning. I'm not sure it even behaves the same way every day. I have times where I seem to have a lot of hyperadrenergic symptoms with a lot of high numbers, and other times where my numbers are always really low. But I do acknowledge that the testing makes sense.

The big challenge of this testing is that you are supposed to discontinue all medication for 5 days prior to the test. He knew this would be too traumatic for my system, so he's allowing me to stop most medications only the day before and the day of the test. Even this will be a real challenge for me. I will definitely get sicker and get increased pain for at least a week afterwards. So, I'm really dreading it.

But it is really nice that doctor Barboi is close to home, and an expert in these problems. I'm so grateful he's willing to treat me and help manage things. It's also really nice that he seems to understand my body's strange over-reactions to stress and to medications. He said he could have predicted my DHE reaction, as well as the Indomethacin reaction. So, next time I want to try a new medication or treatment for one of my issues, I will definitely check with him first, to see if it's even worth trying!

Other than all of that, life has been a challenge this summer. Various things have been going wrong. Our cars both broke down the same week. Our air conditioner leaked and got the carpet in the bedroom moldy. This has triggered my allergies/mast cell symptoms to be worse, and I haven't figured out how to deal with it yet! Finally, we got a new car, and I'm allergic to the interior. My guess is that they polished the leather seats with something really smelly that I can't tolerate. I got a new mask, so I have to wear that in the car, even with the windows down the whole time. And even doing that, I'm pretty miserable in the car, so I'm avoiding our only mode of transportation, when possible. These stupid reactions are beyond frustrating, for both me and my husband, to deal with.

I haven't had to work much, so that's been nice, with all of this going on. I'm trying to get back into a routine where I get dressed every day, exercise regularly, keep the apartment neat, and actually get to the things on my to do list. It hasn't happened yet. I really haven't dealt with the insurance denial for my hospital bill yet, but it's on the list!

I'm mostly still in survival mode this whole summer. We did have a mini trip to Lake Geneva with family last weekend. It was really challenging, but a lot of fun, too! We're also hoping to plan a trip to Boston soon to meet my brand new little nephew, Lincoln!

Thanks so much for reading and following my progress. And thank you to all of my family and supporters.

I'll post again as soon as I can after my testing next week.