I had to wait over a year, but I finally made it. In July 2018, I tried to make my first appointment with Dr. Systrom and the earliest I could get in was April of 2019. A whole 9 months away! Yes, he had a long wait list.
You can check what that first appointment was like here, but essentially I completed 3 tests/labs within a few days of that appointment and had to be scheduled for the iCPET as the final, tell-all test. The earliest appointment I could get was nearly 4 months out (July 30, 2019).
Waiting for this appointment was kind of like torture. I was counting down the months, to weeks, to days. As the appointment got closer, I would become more anxious because I felt like Dr. Systrom was my last hope.
Dr. Systrom seemed to be the only doctor who could help me at the time. He had predictions of what was ailing me, but I didn’t know if they would find what they were looking for and/if I would respond well to the their available chronic fatigue syndrome treatment. I tried my best to be optimistic about the whole thing, but it’s hard when you have a scarcity mindset about your illness.
On the day of the appointment, it almost didn’t feel real because I had been anticipating it for so long. Anxiously, we arrived 30 minutes early for my 8:30 appointment and the waited. I first gave them a urine sample and then waited for another 45 minutes before starting the whole procedure.
Shortly after she prepped me, I signed the consent form for the catheters and they moved me to room #2.
Catheter Insertion
Talk about intimidating. The room was sterile, white with a table in the middle, large fluoroscopy machine, huge monitors, and small metal tables with torture devices (just kidding, just syringes and catheters).
They had me lay in the table like a one armed scare crow (left arm out for catheter placement), head turned the the left, facing the large monitor. In the left corner of the screen was my name and my variable heart rate. 85, 90, 95. The more I thought about what was about to happen, the higher my heart rate would jump. Time to meditate. They swabbed down my neck and arm with a blue sterilizer and covered me with surgical drapes.
Dr. Systrom said that it would feel like a bee sting, and would hurt no more than a drilling at the dentists. I’ve never had a cavity, so I can’t vouch for that, but he was pretty accurate with his analogy. I had been dreading the insertion of the catheters for days, but I was able to get through it fairly easily.
They put a catheter inside my heart!
Overseen by the Director of Pulmonary Vascular Disease, Dr. Aaron Waxman, started off by placing the pulmonary artery catheter by inserting it through the jugular. Of course they told me they were going to start and not to be nervous, but then my heart rate jumped up to 135bpm. They joked about how telling someone not to be nervous never works. I joked back that I was not nervous, just excited. 😉
They injected me with lidocaine, which you definitely feel at first. The needle is so fine that it is not excruciatingly painful, but a bit uncomfortable. It definitely feels like a very long bee sting. This takes them about a few seconds to do, while they ask if you are still able to feel the needle. When you no longer feel the needle, they know that they have injected you with enough lidocaine. At this point they insert the catheter into the pulmonary artery.
If you’re as anxious as I am, I definitely recommend some breathing exercises to get through the catheter insertion. Prior to the appointment, my mind was expecting the worst, but it was much easier than I had imagined. I mean, just imagine that you are awake and that there is something inside of your heart! It sounds like it probably would be painful, right? Well I promise you it’s not painful like you might image it to be. It just feels a little funky.
They warned me that the insertion into the heart would cause a “tickle”. I did not feel any pain when they inserted the catheter into my heart, but did experience the “tickle” which was a series of fluttering pulses, which I could kind of feel in my throat.
Distal Radial Artery Catheter
After the pulmonary catheter was placed, the distal radial artery catheter was pretty much a breeze. The pain was similar minus the heart tickle. Overall, I probably was in the cath lab for about 20 minutes, from prep to insertion.
Overall my experience with Dr. Waxman and his team was superb. They made me feel very comfortable, they were wicked friendly, made some good jokes, and also allowed me do a little photo shoot.
Here I am with Dr. Aaron Waxman post catheter insertion. He is wheeling me over to Dr. Systroms lab room for iCPET.
Going in for iCPET (Invasive Cardio Pulmonary Exercise Testing)
Ok, so the hard and most dreaded part was over. Dr. Systrom greeted me, and without me saying anything, he reassured me that he believed me and my illness and most importantly, that I was not “making it up”. That was nice to hear. He proceeded to tell me what I was in for with iCPET and that they would have some answers for me in just a few minutes. This was an instant relief. I didn’t realize they were able to report back to me immediately.
There were also 4 awesome tech ladies in the room who then proceeded with the test. They hooked me up with a 12-lead electrocardiogram to measure heart function, got me on the bike, hooked me up wit a nose plug and mouth piece, which were designed to measure exchange of gases and oxygen uptake. Definitely not the cutest look…
For 9 minutes, I biked with the greater resistance at each minute. At each minute mark they collected blood samples from both catheters. Considering how deconditioned I was, and that I actually have some physically problems, this test got pretty challenging during the last few minutes! Imagine trying to bike up an extremely steep hill… drunk!
What is iCPET measuring?
During iCPET, they sample blood for the pulmonary artery port, as well as the distal radial artery for these blood gases: oxygen, carbon dioxide, pH, and lactate; along with a variety of bio-markers as part of their research to determine diseases in the plasma.
Arterial and mixed venous blood sampling is taken at rest, every minute of exercise, at peak exercise, and then one hour after recovery from exercise. They are also measuring pressures at several different ports along the pulmonary artery catheter and distal radial catheter.
Some of the problems they determine from iCPET include:
- Pulmonary Hypertension – This could occur, but this is not very prevalent in ME/CFS
- A variety of Heart Failure Preserved Ejection Fraction (HFpEF) – This is exercise induced heart failure that is most common in older patients. This is just apparent during exercise and not at rest. Without exercise, they are not able to see pressures elevated as a function of the left heart not relaxing.
- Internally Preload Failure – Dr. Systrom exclaims this as “ubiquitous” with all patients clinically diagnosed with ME/CFS.
- Impaired Systemic Oxygen Extraction – Abnormality in systemic oxygen utilization and uptake by the exercising skeletal muscles. Impaired oxygen extraction at peak exercise may be either caused by Vascular Dysregulations or Intrinsic Mitochondrial dysfunction in the limb skeletal muscle.
What is Preload Failure?
As described by Dr. Systrom, preload failure as an “unwillingness of the big veins in the legs, abdomen, and the pelvis to squeeze down and physically push blood up to the right heart to prime the pump”. What they find is that the right heart has low and inadequate filling pressures and blood volume.
A more technical description of preload failure is low biventricular filling pressures during exercise is caused by unexplained dyspnea.
It is hypothesized that small fiber polyneuropathy may be the underlying mechanism behind the exertional intolerance of preload failure and ME/CFS.
So How Did I Do?
Immediately after I finished biking, they had me lay horizontally in a reclining chair. At this point, I was extremely out of breath, dizzy, shaking, nauseated, and slightly disoriented. They monitored me to see my rate of recovery, which they told me was mild to moderate.
I definitely did not look very flattering, laying there mouth breathing as Dr. Systrom stood over me explaining the results of the test. What he told me was all gibberish to me at the time, but essentially he said that I did have preload failure and that there was impairment in systemic oxygen extraction.
Can I just saw how on point Dr. Systrom was? At my first appointment he reviewed my health records and just by talking to me predicted everything that he had just diagnosed. I am so thankful that I found him, for I probably would not have been able to document otherwise that I actually have a psychical illness. (Yes, NOT psychosomatic!)
iCPET Blood Results
For all of you who love the details, here are my blood tests taken at each minute from the pulmonary and radial catheter. I do not have knowledge in interpreting these types of tests, but have highlighted the figures that were outside of the standard range. What did stand out to me the most was lactate increase, pO2 and sO2.
(click image to expand)
Finishing up the appointment
After iCPET, they brought me back to the first room where the nurse hooked me up to and IV and took their final blood samples about an hour later. At this point, I had been at the hospital for 7 hours and it felt like the lidocaine was slightly wearing off. My family was able to come see me at this point, but turning my head, talking, or coughing was uncomfortable. I could definitely feel the catheter in my heart.
Starting off the day, I was in a good mood and able to make small talk with my providers, but after iCPEt, I was completely wiped out. I had no life in me to even make small talk with the providers.
Finally, Charlie Lee, my PA, came in to remove the catheters and explained that I had a secondary diagnosis of dysautonomia.
What is Dyautonomia?
Dysautonomia or Autonomic Dysfunction is impairment of the autonomic nervous system (ANS). The ANS is responsible for automatic functions like heart rate, blood pressure, breathing, digestion, etc. In this case, the nerves had reduced signaling to the veins, resulting in poor squeezing of those veins. Low blood pressure and poor blood return to the heart would then affect the pressures coming back to the right atrium. Thus when less blood is entering the heart, suboptimal blood amounts are leaving the heart, resulting in symptoms.
Vein squeezing happens under 2 circumstances; standing and exertion. When standing, gravity works against the veins lowering overall blood pressure. This can lead to dizziness, lightheartedness, and passing out. With exertion, the body needs more blood flow to meet the increased needs of the body, meaning the veins would need to squeeze more. This does not happen with dysautonomia, and as a result, these are some common symptoms:
- shortness of breath
- fatigue
- Lightheaded/dizzy/fainting
- Chest pain
- Racing heart
- Fluctuating high/low heart rate
- Fluctuating high/low blood pressure
- Nerve pain
- Mild ankle swelling
Dysautonomia & Chronic Fatigue Syndrome Treatment
Thankfully Dr. Systrom has been able to determine a treatment that has been effective for the majority of his patients with ME/CFS. I was prescribed Mestinon, also known as Pyridostigmine. This is an off label use of the medication, which is typically used to treat myasthenia gravis. Mestinon is a cholinergic muscle strengthener. As explained by my PA, the nerves and muscles fail to communicate properly. Mestinon helps improve communication by preventing the breakdown of neurotransmitter, acytelcholine. When acytelcholine is available, this improves the function of nerves that interact with slow-twitch muscle fibers. These are the exact fibers that are not getting enough oxygen in patients with ME/CFS. In addition, Mestinon also promotes greater constriction of the veins, promoting greater blood flow back to the heart during peak exercise.
Dr. Systrom had me start by taking half a tablet (30mg) three times a day with food for 2 weeks. Following the first 2 weeks, I will increase the Mestinon to 60mg, 3 times a day. They did say that I may see improvements over weeks to months, depending on severity. So far after 1 week, I have not noticed any real changes.
In addition to medication, I was also prescribed these non-medication options:
- Increase salt intake to a least 2 g per day and increase fluid intake to a least 68 oz per day: Sodium and fluid intake must be done together. Sodium attracts keeps fluids in the veins, helping blood return to the heart.
- Recumbent exercise: This exercise is suppose to be done in a more horizontal position in order to optimize blood flow, vs upright exercise, where blood flow will be fighting with gravity. The suggested exercises include swimming, using a recumbent stationary bike, or a rowing machine. The goal is to work your way up to exercising 30 minutes a day, 5 days a week. This would be done gradually over a 6-12 month period, which will help train your nervous system to increase vein squeezing. – Considering I did not want to pay for a gym membership just to bike for a few minutes a day, I bought these inexpensive bike on amazon. It costs as much as I would spend on 3 month gym membership, and I don’t leave my house to use it.
- Raise the head of the bed 20-30 degrees: This sleeping angle will keep gravity resisting vein blood flow, forcing the nervous system to constantly stimulate vein squeezing to counteract gravity during sleep. This helps prevents you from feeling sluggish in the morning.
- Compression socks: These can possibly increase blood pressure in the legs and reduce leg swelling.
- Physical maneuvers, like squatting, can increase vein blood pressure and alleviate mild lightheartedness.
- Avoid symptom triggers: including overexertion in hot weather or standing up quickly.
Overall Experience
Overall, I am super thankful I found Dr. Systrom. It has been a long journey to find a doctor, who is not only able to diagnosis me, but believed me. A majority of doctors did basic blood tests, found nothing, and then dismissed me as a hypochondriac. Each test lead to a dead end, which led doctors to believe nothing was wrong with me. They would quickly give up and just refer me to see a therapist.
The entire team associated with Dr. Systrom and Dr. Waxman were attentive and professional. Not once did I feel as if they were being condescending or judgmental. They understand the struggle of the patients who have yet to find proper care and diagnosis by other lack-luster medical professionals.
It was a hell of a long wait, but one hundred percent worth it. I now have a true diagnosis, physical proof, and a doctor who is on board. So far, I have been doing treatment for 1 week, but I will be writing a follow up within the next few weeks to give you an update on how treatment is going. I look forward to feeling better and what this treatment may mean for ME/CFS!!
If you have any questions about the iCPET or anything else ME/CFS related, please leave me a comment below or shoot me a personal email at sarah@mentalolympian.com
1 Month: Mestinon for ME/CFS
After trialing Mestinon for 1 month, these are my results so far...
Resources
“David M. Systrom, MD – Brigham and Women’s Hospital“. physiciandirectory.brighamandwomens.org.
“Announcing SMCI’s new ME/CFS research program at Brigham’s and Women Hospital“. Solve ME/CFS Initiative.
“The Exercise Response to Pharmacologic Cholinergic Stimulation in Preload Failure – Full Text View – ClinicalTrials.gov“. clinicaltrials.gov.
Oldham, William M.; Lewis, Gregory D.; Opotowsky, Alexander R.; Waxman, Aaron B.; Systrom, David M. (Mar 2016). “Unexplained Exertional Dyspnea Caused by Low Ventricular Filling Pressures: Results from Clinical Invasive Cardiopulmonary Exercise Testing“. Pulmonary Circulation. 6 (1): 55–62. doi:10.1086/685054. ISSN 2045-8932. PMC 4860548 Freely accessible. PMID 27162614.
Joseph, P.; Sanders, J.; Oaklander, A.L.; Arevalo Rodriguez, T.C.A.; Oliveira, R.; Faria Urbina, M.; Waxman, A.B.; Systrom, D.M. (May 2019). “The Pathophysiology of Chronic Fatigue Syndrome: Results from an Invasive Cardiopulmonary Exercise Laboratory“. American Journal of Respiratory and Critical Care Medicine 2019. 2019 (199): A6902. doi:10.1164/ajrccm-conference.2019.199.1_MeetingAbstracts.A6902.
Arcuri, L., Bernabeu, J., Arcuri, L., Bernabeu, J., Arcuri, L., & Bernabeu, J. (2019, June 25). Energy Crisis. Retrieved from http://protomag.com/articles/energy-crisis-0
Laboratory Tests. (n.d.). Retrieved from https://www.glowm.com/lab_text/item/3#Oxygen Saturation (SO2)
Wow, this sounds really interesting. Definately going to be researching icpet. I’m convinced there’s an answer in my body somewhere – it just needs to be found! Truly hope you’re feeling some benefits from this treatment
Yes, Its great to see what other doctors are using currently. I am hopeful about it!
Hi Sarah,
Thank you so much for posting your procedure and follow up. I have the icpet scheduled for the end of June and your post gave me a very good example of what the test involves. I met with Dr. Systrom I’m November and have my next appt in January for the tissue sample/biopsy. I’m so grateful! Started the Mestinon on my 3 rd day. Have you noticed any yet from this medication? Although I live with all the symptoms of ME/CFS the most debilitating symptoms are the flulike sickness I feel most every day now. Did you get a lot of flulike symptoms over the years.
Thank you so much!!!
Hi Patricia!
I am really glad this post was helpful to you! It can definitely help calm your nerves knowing what to expect! I was fairly nervous about the catheters, but Dr. Waxman is excellent and the procedure is quick and painless.
That’s interesting! I don’t have the flu-like symptoms daily (as I would describe it). My main issue that is most bothersome is PEM. I feel like my body is pretty sensitive to most normal activities and when I feel like I am capable of doing a little more around the house to help out, I tend to over do it and not realize it until its too late. At that point I get a flare up that consists of body aches/ numb and heavy limbs, cognitive dysfunction, dizziness, hot flashes, fatigue, etc. I don’t know if this is similar to what you experience. I generally do not feel nausea or IG symptoms unless it’s from a poor reaction due to the Mestinon.
I plan on writing a follow up this week, since I have been on the medication for 4 months now. I did have some complications, but there have been some slight improvements at this point from how I was feeling during month 1-2. At that point I was mainly bedridden and just taking a shower was exhausting. Now I am mainly housebound, and spend most of my day out of bed, sitting.
I hope in the meantime, you find some benefit with the Mestinon while you wait for your following appointments. 🙂
Ok I have just seen your previous post which answers many of my questions. Was your treatment covered by insurance if you just called directly without a doctors referral? If not can you possibly give some idea of the costs? I would consider paying if not outrageous.
Coincidentally, I had this very procedure done yesterday and went online to take another look at the research I had read about, knowing that it would make more sense to me now, and then came across this blog.
Sarah, everything you wrote was exactly what I experienced (except that Dr. Systrom wasn’t there and also he did start me on Pyridostagmine 6 months prior to this). You could have super-imposed my picture in there and it would have been MY story! Dr. Waxman and the entire staff at Brigham & Women’s was exceptional – from admitting to discharge. Each and every person introduced themselves and prepared me for what to expect every step of the way. They reduced my anxiety with their reassurances and questions aimed at getting to know me. I was able to remain pretty relaxed throughout the dreaded catheter insertions by closing my eyes and imagining “happy thoughts and places”.
Once in the exercise room, I joked about the seat on the bike and told them they should get funding for a bigger, softer seat. After all I had just been through with the catheterization procedure, I found myself laughing at how uncomfortable the seat was and also the toll it took on my jaw to keep the mouthpiece in! It was all worth the 6-month wait though!!
The only result I was given after the exercise was that I have Preload Failure. The bloodwork results were emailed to me last night but, of course, I don’t know how to read them except to see that some are out of the normal range. My Autonomic test is in 6 weeks. The most frustrating part is all the waiting but I am so grateful that someone told me about Dr. Systrom and am finally getting some answers and feeling validated rather than judged.
For anyone that might be considering this test, I would say that it is well worth it if you can get an appointment. Just know that, especially with all the Covid patients needing testing now, there is quite a wait but worth it if you need answers to your illness.
Sarah, thank you for this post and sharing your experience! I wish you all the best!