Update & upcoming surgery

Hello everyone,

It’s been awhile since I checked in here, so I thought I’d write an update. I was diagnosed with Eagles Syndrome with bilateral jugular vein compression about 2 years ago. Getting the diagnosis was a lifeline, as I had spent 3 years going to multiple doctors appointments per week trying to get answers to my nearly 10 years of head pain. Dr. Hepworth was the first doctor to listen to my symptoms, look at my imaging and say, “I can see what the problem is” instead of shrugging, guessing or prescribing random drugs to see what they did to me. Unfortunately, my health picture has ballooned since then.

After making the IJV compression diagnosis, Dr. H ordered jugular ultrasounds to confirm, and had me start on Plavix, which made an immediate difference to my quality of life. So, in December 2022 I had a styloidectomy and jugular angioplasty done on my left side. For 10 days post surgery I felt better than I had in over a decade. They also switched me from Plavix to Eliquis on the day of surgery though, so no way to tell if it was surgery or drugs that had a positive effect Unfortunately, the symptom relief did not last, and everything came back, along with some new pelvic and leg symptoms.

Because of the new pelvic pain and leg swelling along with my POTS symptoms, I was referred to Dr. Brooke Spencer, who diagnosed me with severe May Thurner Syndrome (MTS) and mild Nutcracker Syndrome. Both Dr. H and Dr. S felt that although my right jugular is still very compressed, it would be best to treat the MTS first, as they’ve seen some improvement to head pressure and POTS symptoms with iliac vein stents, and leaving that untreated could lead to significantly more dangerous health problems. So, on 10/31/2023 I had a stent placed in my iliac vein.

The stent has provided significant relieve from my pelvic pain, but I’ve also started birth control pills so I don’t get a period, which also helps dramatically since all my symptoms flair with my cycle. The iliac stent also decreased my leg swelling, although it’s not completely gone since the MTS had already caused permanent damage to my veins. The stent did absolutely nothing for my head pain and fatigue. It does seem to have had a somewhat positive effect on my brain fog, and I haven’t had random racing heart rates since the procedure. I still get elevated heart rate with the slightest physical activity.

Now I’m scheduled for the right side styloidectomy and jugular angioplasty 03/06/2024.

In the meantime, I’ve been seeing Dr. Melamed who is an immunologist, and based on significantly abnormal bloodwork he’s convinced I have some kind of immune system issue having to do with Ige response and mast cells. He hasn’t been able to figure out exactly what is going on, but he’s tried me on a bunch of different antihistamine medicines to see if that’s part of the picture. The antihistamines don’t seem to have any effect, but I recently started Cromolyn Sodium, which is a Mast Cell inhibitor, and that has had a positive effect on my energy level and sleep. I also haven’t had any crazy full body flu-like aching episodes since starting the Cromolyn.

I read the article Dr. Fargen recently put out connecting IJV stenosis to IIH, POTS, MCAS, and hEDS, all of which are diagnosis I have. I also most likely have an Occult Tethered spinal Cord (OTC), but have been unable to get a real OTC evaluation due to insurance BS. All of these diagnosis and overlapping symptoms combined with the lack of understanding of the origin or relationship between these issues within the medical community has me despairing that I’ll ever get back to living my life to the fullest. I really hope the next styloidectomy will be the silver bullet that fixes everything, but even Dr. H said he expects that my jugular compression is only 60% of the problem.

Since having a positive reaction to Cromolyn, I’ve joined an MCAS forum, and everyone on there seems to think that MCAS most likely originates from mold, lyme, severe trauma or other bacterial infections, but as far as I can tell science hasn’t been able to prove that yet. Dr. Melamed seems to think that whatever is going on with my immune system could be the cause of all the vascular compressions I’m experiencing, but when I press him on how that could be, the answer isn’t very convincing, especially since 90% of the treatments he’s tried me on have had zero effect. Admittedly, I find anatomical defects far easier to understand than how our immune systems work, so maybe his answers would make more sense to someone else.

Clearly there’s some kind of connection between vascular compressions, elevated immune response, spinal issues and hypermobility, but what is it? Is mold exposure 12 years ago, with no ongoing exposure in the last 10 years possibly the culprit for everything I’m experiencing? Or is it that my spinal cord is slowly collapsing my spine and the rest of my body is trying to compensate? Or is it that blood can’t get out of my head, causing my nerves, CSF volume and immune system to go haywire?

I just wish there were better answers available to us. I wish someone would study these issues as a whole, rather than as disparate syndromes.

Thanks for listening. I’m really, really hoping that next month’s surgery is a success. I just don’t know that I have the energy to keep trying to be my own doctor on top feeling shitty all the time.

6 Likes

I’m sorry that it’s been such a long journey for you, and that you’re having more issues…good that you’re working your way through, and have good doctors on your side, but I’m sure it is exhausting for you having to do so much research & detective work yourself…I hope that your surgery with Dr Hepworth goes well and improves symptoms significantly for you. Sending you a hug & will pray that it is successful for you :hugs: :pray:

I’m also very hopeful that your second IJV decompression/styloidectomy will be highly successful & rid you of many of your current symptoms as you heal. I’m very, very sorry to read about all the vascular compressions you’ve suffered & now Mast Cell disease seems to be a problem. It would be nice if only one really tough ailment hit us at a time so it could be treated & heal up before the next “storm” hits (also it’s not ideal that a next storm might be coming :cry:).

I’m glad you’ve gotten some symptoms relief from the surgeries you’ve had & truly hope that this next one makes a significant difference in how you’re feeling. :hugs: :pray:

1 Like