My Cleveland Clinic Trip
Back in January 2019, I finally received my hypermobility and MCAS diagnoses. The provider I saw for hypermobility also had given me a differential diagnosis of Postural Orthostatic Tachycardia Syndrome (POTS). Over the summer this year, I was able to get a virtual appointment with the POTS clinic at Cleveland Clinic. Last week, I went in for a day of testing.
It was… an experience. Honestly, I’m feeling less like this was the best step for me.
The Background
That differential diagnosis came from a provider who routinely works with hypermobile / EDS folks and sees POTS regularly. According to him, my symptoms met the POTS diagnosis. He didn’t want to put me through all the testing in case we could get things under control without medication, etc. For a while, the tips he gave me about drinking more water, wearing compression socks, and increasing my salt intake worked.
As time went on, that wasn’t the case any longer. I’m someone who needs regular check-ins on this stuff. With COVID-19 and then having moved, I haven’t had that for a while. Add in having just left a job where self-care was preached without leaving much room or time for it, and things got bad. My POTS symptoms have been getting worse and worse. So, now that I’m settled in Ohio, I reached out to the Cleveland Clinic to get set up with their POTS clinic.
My initial virtual appointment was at 6:45 AM, which was rough. Still, the nurse practitioner I saw was really nice. I felt heard and seen. We set up three tests. Since I live about 3 hours south of Cleveland, I got all the tests set up to take place in one day. This allowed my partner and I to drive in the night before. It also made things a little easier as I basically had to stop every single one of my medications for at least two days (save 2 of them) for two of the tests.
The Tests
The Tilt Table Test
A tilt table test is the gold standard of testing for POTS and similar conditions. You start by laying down on a table. An IV is started while you’re laying down, just in case additional medications need to be administered to produce results or for another reason. This involves having an automated blood pressure cuff on one arm, electrodes all over your body, and another automated BP system on two fingers on the opposite hand to the arm BP cuff.
After a while, the techs get a baseline blood pressure and pulse. Then, the table raises you to a 30-degree incline while measuring how your blood pressure and pulse react. After some time, you’re then adjusted to a 70-degree incline and generally have to stand there for 30 minutes. Following this, you’re readjusted back to laying down fully while your body’s reaction is measured. Throughout this experience, you’re also asked to share and rate any symptoms that pop up for you.
This is a test that requires that you have a driver with you, as you will be barred from driving for the rest of the day.
My Experience
I’ve been very nervous about this test for the months leading up to this. I had nightmares the night before about just projectile vomiting or getting sick in other ways on the table during incline shifts. The techs that I met were wonderful and very sweet. They worked to walk me through everything that was about to happen and did a great job of aftercare for me before releasing me back to my partner.
The test itself though? PHEW! That was difficult to go through. By about halfway through the 70-degree standing portion of the test, my feet, ankles, and right hand (where the finger BP cuff was) were all completely numb. Literally, by the time I’d hit 5 minutes left, I felt about ready to fall down. Good thing they strap you onto the bed!!
Skin Biopsy
The skin biopsy taken during this day-long testing situation was to rule out small fiber neuropathy. The skin biopsy was performed in an exam room by a provider and a nurse. The test area is cleaned with alcohol and then lidocaine is injected into two spots – one near the ankle and the other on the outer thigh of the same leg. Within moments, they are able to use a hole-punch biopsy tool to take a small piece of skin (3mm in diameter; 2 mm deep) from each spot. They bandage you up and send you home with some post-procedure care instructions.
You’re generally instructed to shower after 24 hours with the bandages on. This helps to protect the site but also loosens up the adhesive for easier bandage removal. Following that, the instructions were to use a regular bandaid and change it daily, up until the sites began to form scabs. After that point, no additional bandaging is needed and the wounds can heal uncovered.
My Experience
They had to shave these parts of my legs, which was oddly gender-affirming! Y’all, I am SO hairy now.
The most painful part of this entire procedure was honestly just getting that lidocaine injection. It’s been a while since I had a lidocaine injection, even though I used to regularly have to get them in my upper back and neck. Even then, those were really focused on getting deep into the muscles and knots that were causing pain as opposed to skin-deep work. The lidocaine here wore off more quickly, due to not being as deep. Without having taken a lot of my medications due to the other tests, I hit some pretty high pain levels once the lidocaine cleared out. A nap on the car ride back home after taking meds helped immensely.
It’s been a week now, meaning I have finally built up a scab at each biopsy site and can stop bandaging over them. I’m also allowed to take baths and long showers again, which is great.
QSART
The QSART (quantitative sudomotor axon reflex test) is a way to measure how well the nerves that control your sweating are operating. The provider will clean up three spots on one of your legs and one spot on your wrist. These spots are generally on the same side of the body (AKA if using the left leg, they will also use the left arm). The tech will place electrodes onto each of these clean spots. Then, they fill the space here with acetylcholine, a chemical that helps to encourage sweating. The electrodes receive electrical stimulation to also help your sweat glands produce, well, sweat! The tech will measure the amount of sweat produced during this test.
Since this involves electrical stimulation, it’s important to know that this will not necessarily be a comfortable test to undergo.
This test may be done not only for possible POTS and dysautonomia patients, but also for those with suspected peripheral neuropathy, pain disorders such as Complex Regional Pain Syndrome (CRPS), and issues that affect various bodily functions that are supposed to be more automated such as breathing.
My Experience
I had this done immediately following the skin biopsy. I think this was a blessing as the electrical stimulation here was incredibly uncomfortable for me. It was a burning itching, not unlike getting a tattoo but without knowing where the next zap would be. It wasn’t the worst test I’ve been through by far. That said, it was at the end of a long day without meds to help me process and handle pain.
The Results
In reviewing the tilt table results, I saw some radical changes:
As you can see, I had a hypertensive reaction to the test. This means that my blood pressure went up. My blood pressure is generally not high unless I’m incredibly stressed, though, which would make sense with wrapping up an old job, starting a new one, and being super nervous for this test. Otherwise, it’s generally been low – even with the ADHD medications that I’ve been on for a while. I did have that hypertensive crisis during top surgery, but that was an MCAS-related reaction and not something that happens regularly.
BTW: The skin biopsy results will take another week or so.
In any case, I felt like this helped to indicate something important. Then, yesterday (Dec 13), I received a message from the nurse practitioner that I saw virtually over the summer:
Notable is your Blood pressure is elevated on this exam. This can sometimes be seen with stimulant use. I would recommend that you follow up with your primary care physician in this regard or with cardiology for management of hypertension (High BP).
Otherwise the tilt table did not show Postural orthostatic tachycardia Syndrome or Orthostatic hypotension.
Because of this and from my standpoint, there is no firm basis for the prescription of medications that may be somewhat helpful for conditions like autonomic dysfunction. I would not want you to endure side effects, without clear benefit. In this scenario, patients may often benefit from optimizing nonpharmacological measures like those I provided to you on the last clinic visit. I have attached these below for your convenience.
I, then, received the same instructions about self-care that I had back in 2019:
Orthostatic Self Care
1. Make all postural changes from lying to sitting or sitting to standing slowly.
2. Drink to 2.0 -2.5 L of fluids per day. With bad symptoms, drink 500 cc of water quickly. This will result in an increased blood pressure within 5 minutes of drinking the water. The effect will last up to one hour and may improve orthostatic intolerance.
3. Increase sodium in the diet to 3 – 5 g per day. If not helpful and BP is stable, may try 5-7 g per day. IF BLOOD PRESSURE RISES OR IS RISING CUT BACK ON SALT LOADING
4. Avoid large meals which can cause low blood pressure during digestion. It is better to eat smaller meals more often than three large meals.
5. Avoid alcohol. Alcohol and cause blood to pool in the legs which may worsen low blood pressure reactions when standing. Avoid excessive caffeine intake as it may increase urine production and reduce blood volume.
6. Perform lower extremity exercises to improve strength of the leg muscles. This will help prevent blood from a pooling in the legs when standing and walking. Preferred exercises are walking, squatting or stationery bicycling.
This message was also sent with no reply possible, meaning I’ll need to start a whole new conversation to ask questions, etc., in a way that isn’t super accessible.
Sigh.
The Feels
First, it was really difficult to go through these tests. I was gaslighting myself beforehand and almost canceled these appointments a number of times. I structured leaving my previous job and starting my new one around the time I could get into the Cleveland Clinic. The week leading up to this was hell, having to drop a number of my medications and deal with not only withdrawals but increased dizziness and near-syncope due to removing the medications that help improve my symptoms. Once these tests were over, I was exhausted. Honestly, I slept a lot over the next few days. I’m still dealing with breakthrough pain. I’m also having side effects from onboarding these medications again.
I get that a tilt table test is a helpful tool, but it doesn’t capture real life. It is also a snapshot, not an end-all-be-all definitive test. For example, my biggest issues have been with larger postural changes, like from squatting to standing. This test doesn’t come close to replicating that experience. The QSART is also not fully definitive. Before I started on testosterone, I did not sweat. Like, for a long fucking time. My issues with sweating are now more intermittent and happen more over the summer. I suppose choosing to do this test in the winter hindered things.
The note about my blood pressure going up with stimulant use is accurate. That is generally how folks react to this. That said, I have not had a record of ‘high blood pressure.’ I joke about it with providers that this is one issue my family historically has had, but I’ve avoided by getting everything else. I’ve been on ADHD medications for a year and a half with no issues. I have a home BP cuff and do check my BP from time to time. Since 2017, my BP has always been wonky when I’m feeling symptoms that I know now are related to pre-syncope.
What Now?
The way this message was sent feels so… dismissive. In therapy this week, we talked about how it was bringing up a lot related to past medical trauma and providers gaslighting me about my symptoms. This provider didn’t do that directly – and I’m sure she didn’t mean to cause that reaction in me – but it is definitely bringing up related feels that are difficult to process.
I’m trying to decide if I reach out to this provider and share that there are a few things that haven’t been captured well by the clinic. Part of me wants to do this. A large part of me also recognizes that I might not like the reaction I get. I also don’t want it to come off as “why don’t you believe me???” That just feels gross. For now, I’ll sit on the fence about it.
Nevertheless, I have been able to connect with several other patients. These folks have the same symptoms as I do and have a diagnosis of POTS. They have helped validate my concerns that the understanding of POTS displayed by this provider does not match reality. They’ve also shared what has helped them and given me some ideas moving forward.
This was the first time my partner was with me for a big series of tests. I don’t know that I would have been able to get through this day without them there. I am SO lucky to have a partner who is truly a ‘partner’ with me around my health.