From the initial visit with my immunologist, she suspected I had POTS. I did too. In the interim, she recommended I have intravenous infusions of Lactated Ringer 1-2 liters and increase to 2 times per week. However, it took me a while to find a knowledgeable cardiologist willing totreat me for POTS. Eventually, I found one through my local Ehlers-Danlos Syndrome Facebook support group. The closest support group to me is the Raleigh Ehlers-Danlos (EDS) support group. Joining a support group with others who have EDS, MCAS, & POTS has been an invaluable asset for me. Not only have I found the Ehlers-Danlos Society Facebook support group helpful, joining a local group has been one of the best decisions I have made that has helped me navigate my chronic illnesses.
A Brief Overview of Postural Orthostatic Tachycardia Syndrome
A tidbit about POTS, the acronym stands for Postural Orthostatic Tachycardia Syndrome. POTS is a form of dysautonomia. Dysautonomia is a disease of the autonomic nervous system. So if you think of the word automatic in relation to your body, think of the part of your body that works automatically. For example, you don’t have to tell your heart to beat, it does it consistently automatically. Or you don’t have to tell your eyes to blink when an object gets too close to your eyes. Or better yet, turn tell your stomach to turn gastric juices acid to begin the process of digesting your food. When this automatic process malfunctions, it’s called dysautonomia. Two things to keep in mind about POTS:
- The symptoms of POTS are mostly associated with standing.
- The diagnosis of POTS requires an increase of your heart rate up to 30 beats per minute or more after standing up or 120 bpm or higher within 10 minutes of standing without a drop in your blood pressure.
POTS is not a disease, rather a collection of symptoms that presents itself due to a known or unknown cause. Symptoms can vary in affected in individuals. However, there is a frame of symptoms associated with POTS, these symptoms manifest themselves when a person stands up. The International Society of Dysautonomia provides information about POTS including symptoms, diagnostic criteria, and diseases associated with POTS.
What Are My Symptoms of POTS
My heart rate rises when I stand up. The longer I stand the worse I feel. Depending on the time of month, I feel dizzy, lightheaded & I can feel my chest beating very fast. Other times I can stand longer before feeling any symptoms. At it’s worst I feel dizziness, lightheaded, nausea, shakiness and my legs become very weak like a soggy french fry. I have trouble regulating my body temperature. I am extremely sensitive to temperature fluctuations, even if it’s a degree too hot or too cold. This is called heat & cold intolerance. If I do not sit down immediately, my mast cell starts to degranulate and I will most have an anaphylactic reaction. To decrease the symptoms of POTS some physicians will order weekly IV infusions. My cardiologist increased my infusions to 1-2 liters 3 to 4 times a week. I have found these infusions very helpful.
Issues That Complicated Finding A Physician To Insert The Port Cath
What is a port catheter? An implanted device that allows access to a large vein to administer IV fluids, medications and nutrition. The port can also be used for blood draws. If you are like me and you receive IV therapy, eventually your veins start to wear out and form scar tissues. Once, I stuck myself 15 times unsuccessful to start an IV. Implanted ports makes it much easier to access a vein. They are not without risks though, so it’s a decision that has to be weighed carefully. After 3 months of IV therapy, I was done. It’s time for a port.
The initial doctor I was referred to did not return our request for a consultation. Usually when your referring physician requests a port placement the process is simple & quick. For me, it’s not as simple. I’ve always been an overachiever, why stop now?
In my case, my mast cells are extremely rouge & rebellious, at least that’s what I imagine they are doing in my body. Prior to any procedure, I have to be start a medication protocol 24 hours in advance to negate any potential reaction. I usually can not check in and sit in the waiting because of my sensitivity to fragrances. The staff needs to be informed about sensitivity to hand sanitizer, therefore they must wash their hands. I make it a point to educate the staff on my symptoms of anaphylaxis. My reactions do not present as most typical anaphylactic reaction.
I have names for my pesky mast cells depending on what trigger causes them to degranulate. Juvenile mast cells reacts to common hospital items such as adhesives, hand sanitizer, & ultrasound gel. Miss Sensitive mast cells reacts to fragrances. Mr. Passive Aggressive reactions to emotional triggers: nervousness, anxiety, anger, surprises (even good surprises), intimacy, tears or physical pressure. Millenial mast cell reacts to heat & cold intolerance, sudden increase or decrease in heart rate. The narcissistic mast cell reacts to foods: all fruits,vegetables except cassava, lettuce, & sweet potatoes, grains except rice, all nuts & seeds except sunflower flower seeds. Last but not least THE NEEDY mast cell reacts to unknown triggers & at complete random times.
Nevertheless, the interventional radiologist did not return our request for consultation despite multiple calls from my referring physican. I was truly disappointed because he was recommended as the best by my local EDS Facebook support group.
My cardiologist knew of another EDS versed interventional radiologist so he referred me to him. They called me right away to book the consultation. I always do my research on every physician before seeing them for the 1st time. And I was very concerned about the comments I heard about him. I decided, I needed to find another physician.
The search goes on. To help me out, my gynecologist referred me to an interventional radiologist she was familiar with. I was ecstatic when I finally had my initial telehealth consult with him. He had concerns about my multiple anaphylactic reactions to adhesives, hand sanitizer and fragrance which was very understandable. I was able to assure him that if we follow my pre-procedure medication protocol, we can successfully implant the port cath. We agreed to a trial run of the of the Ekg leads & surgical tape.
At home, I took the necessary medications. My husband and I call it pre-gaming before a procedure. Before applying the EKG leads, I started an IV infusion of Lactated Ringers with Benadryl and Famotidine. Then, I applied the EKG leads 15 minutes after I started an infusionSolu-Medrol. Initially, I felt a bit woozy but that cleared up in 15 minutes. Afterwards, I had a very mild case of itching on my face and legs. 2 hours later, I had no symptoms. After I no longer had no symptoms, I applied the surgical tape to the back of my hand. I went to bed, the next day I was still in the clear. I was very excited.
The interventional radiologist and I had 1 more consult to finalize our plans for the port insertion into the left upper region of my chest above my breast. I had a great degree of respect for this doctor because he was honest with me about his trepidations. I am not in denial that I come with a lot of liability. His apprehension was that the procedure would be performed in a non-hospital outpatient setting. And suppose he encountered a life threatening problem, he wouldl not have the necessary medical intervention right at his disposal. I explained to him that I preferred an out-patient setting because the benefits of a smaller setting outweighed a larger facility. I have found that a smaller outpatient setting gives me a better degree of control due to a smaller staff and less potential to be exposed mast cell triggers. Our plan was to have me come in as the 1st patient of the day, that, check-in from my car ( I think I mentioned that to him), I would change as quickly as possible and ushered into the operating room. If at any point I am not he felt I was not tolerating the insertion well, he would stop the port placement. Also, we agreed to schedule the procedure after my menses before ovulation because I would be less relative. At the conclusion our discussion, he informed me his scheduler would give me a call.
It felt surreal, like a pinch me moment. I thanked the doctor profusely. I waited the rest of the day but no call from the scheduler. Our consult occurred on a Friday morning. Monday morning , I took the initiative to call. A very pleasant woman answered the phone, eager to schedule me & make adjustments to have me be there at 7:15am. She told me to hold on, she had to clarify one more thing. 3 minutes later, she was back on the line, her voice had changed. She assured me she would give me a call back. She almost sounded fearful, concerned and teary. Later that day, I received a call from a woman, whom I suspected was the office manager. The doctor did inform me he met with his practicing partner and the office manager to discuss my case; if all 3 felt comfortable taking me on as a patient. She started asking me questions I already discussed with the doctor. First she asked what allergies I, then confirmed I was allergic to adhesives. Later she stated tell me you what other allergies do you have?I was confused but I answered. Not for nothing, I am not the kind of patient you forget. Then she started to say, I don’t think we can do it here. We have to check you in, then you will be in the preoperative room, you can’t bring your filter and we don’t use a hepa filter her in this building, the nurse will have to start her own IV she can not use mine. I said you can check me in from my car; I already drove to your office & picked up the EKG leads & surgical tape the doctor will be using. I tested it at home with no issues, the transition to the operating room should be no issue if I am the first patient and that I discussed this issue with the physician performing the procedure, he was comfortable with that.. I didn’t have to bring my own air filter, it’s not a deal breaker, just an extra precaution. She had an objection for every statement I made. She replied no we can’t check you in from the car we don’t do that, we use adhesives here and the catheter is latex… Really?
It didn’t matter what I said, she had already made up her mind. I was deeply hurt. I hung up. I felt like a freak of nature, an anomaly because of all the medical baggage I have. I would have rather had the physician call me & said he couldn’t perform the procedure himself. I have to imagine it was out if his control.
It took me a day to get my bearing again. I reached out to the 1st interventional radiologist again. I was told, I’d have to start the referral process all over again. I was fine with it. I also called the office of the interventional radiologist who had bad reviews. I’m hoping my experience would be different from what I read. One of his patients did say he was phenomenal.
The plot thickens…I will continue to update this situation. Do you have a port? If so what was your experience ?