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.


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:

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Hey, @MsBearshark - I have been keeping this post open in a window since I saw it wanting to respond. I’m so sorry that you’re going through so much and having such a rough time of things. I’m sorry that the styloidectomy with Hepworth hasn’t helped, and that you developed mts and nutcracker. Do you think that you developed them somehow as a result of surgery? Although to have been so severe post surgery, I’m guessing that you already had it ? Is there any logic in that at all? I’ve been seeing Darlene at Dr Spencer’s office, and she is also Wanting to go in and do a venogram on me and place a stent if warranted. I had to say no to the stent because of a recent nickel allergy finding. But she’s going to send me out for more specific metal testing including where they actually place the stent materials directly on your forearm. On the MCAS topic, I see Dr Melamed’s colleague, Dr Sarid. They are fantastic there aren’t they? I’m so curious about what Dr Melamed said to you about the link between compression and mast cell activation syndrome or other immuno issues. I wish you had gotten an answer as well. I don’t know if you are on Zolaire injections yet, if not that might be something to discuss with Dr Melamed. Dr Sarid started me on them some time ago. He’s thrown the kitchen sink at me hoping that it will help with surgery recovery. I can tell that I’m tolerating some things better now but it’s not as big of a difference as I wish it would be. But Zolaire is pretty good stuff. Also the drug ketotifen. Dr S said that he has seen more and more evidence of what an excellent and really simple medication this is for people that have mast cell activation syndrome. It has to be compounded in the us because we’re crazy here but seems to be worth it. Here’s to a more successful and longer lasting outcome for you next month.


Hi @akc , thanks for checking in. I ended up going through with my second styloidectomy last weds and am currently recovering from that. I’ve heard conflicting things about the connection between hEDS, MCAS, POTS, IJV compressions, MTS and Nutcracker Syndrome (all of which I have to some degree). Dr. Melamed seems to think that all of this could be caused by vascular inflammation stemming from MCAS, but I haven’t had a positive response to any of the antihistamine medicines he’s given me. I’m also a little confused why he hasn’t tried me on some kind of systemic anti-inflammatory drug if inflammation really is the cause. Dr. Melamed also keeps saying we need to “find my triggers”, but I already know what they are. I’m triggered by exercise, exertion, heart rate rise, bending over, or laying flat. That keeps getting ignored when I bring it up. However, I did start taking Cromolyn Sodium, and that has gotten rid of flares of full body aches and helped quite a bit with getting some real sleep. He prescribed Ketotifin, but my insurance won’t cover any compounded drugs so I haven’t tried that yet. They’re working on insurance approval for Xolair.

That said, after my first styloidectomy Dr. Hepworth put me on Eliquis. The MTS symptoms started after that, although my head pain, fatigue and brain fog has always been worst around my period, and I had some pelvic pain prior to surgery, it was just dramatically worse afterwards. I’ve been on Eliquis since 12/22, and in speaking with Dr. Hepworth prior to surgery last weds, he mentioned that there’s some evidence coming out of Eliquis potentially causing MCAS and aggravating hEDS symptoms. He told me not to restart any blood thinners after this surgery, unless symptoms return. I didn’t get any more info from him at the time since there was a lot going on pre-op, but I hope to talk with Dr. Hepworth more about this Eliquis/MCAS connection at my followup with him in a few weeks. The timing does seem to track for that side of my symptoms, although it’s hard to remember exactly what popped up when at this point.

It’s very frustrating that these issues aren’t really being studied as a whole, at least not yet. When you search PubMed there are a few articles linking these issues in patient populations, but I haven’t found anything that gives any answer as to which is the cause and which syndromes are the effect.

In any case, I’m hopeful that this latest surgery might be my last. It’s hard to tell yet, as there’s still quite a bit of swelling, but fingers crossed that once blood flow is fully restored through my jugulars, I’ll be able to get back to living life to it’s fullest.


I hope this for you too! I’m glad you’ve got the second surgery behind you now & wonder if stopping Eliquis might cause your MCAS symptoms to disappear. Wouldn’t that be a simple fix!!

I hope that your recovery goes smoothly and that this surgery sorts the jugular compression :hugs: :pray: :bouquet:

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Hey, I saw a tag next to my name when I popped in for the first time in… well… months :frowning: and saw your reply to me from that long ago.
Oddly though I think that you and I have been chatting in the FB group as I was asking and you were sharing how the MTS may have sped surprisingly up either due to the change in flow from the stloidectomies or from the Eloquis.
I wanted to check in to see how the medications are helping your MCAS? I wonder what happened with my referral. everyone in our groups seems to end up with Melamed except me (I am with Dr Sarid, same practice…) Not that I am complaining though. Although you get very little time with Sarid, he is also well versed in MCAS.
I hope you got your xolaire approved. I am personally at this point doing 2 vials 4 times a day of chromlyn, the xolaire, montelukast, the 3 preventive antihistamines (supplementing with gen 1 types when needed), ketotifen, and one other that my neurologist prescribed that’s also tied to MCAS. I honestly have never noticed any impact of antihistamines (apart from once years ago when my body was attacking itself daily and I was on prescription antihistamines. It took over a month, maybe 2, but they finally worked. Still, my thought process is to throw the kitchen sink at this garbage and hope that the combination works.
Can I ask what prescription antihistamines you’re on? Sarid just has me on over the counter stuff… if there’s something better (or covered!) I’d like to ask him about switching.
Ketotifen isn’t covered at all in the US. super frustrating. I’m on it now and will stay on through surgery recovery in hopes that it will help - along with all of the rest - with a faster recovery and less inflammation.
Did you ever get anywhere with your question about systemic inflammation? I feel like that’s a given… and then you have us that have it and MCAS and are… allergic to anti-inflammatories. Just not fair…
Here’s to continued success with your recovery green_heart:


Sorry it took me awhile to get back to you. Life’s been busy now that I’m feeling good enough to work and do things!
That’s funny, but not surprising, that we’ve been chatting on FB as well. My MTS symptoms did get dramatically worse after my first styloidectomy, but they also started me on Eliquis at the same time, so it’s unclear how much of that change was from the surgery vs blood thinners.

Dr Melamed tried me on all sorts of antihistamines, but none of them had any positive effect. He did prescribe Ketotifin, but I never filled it since it wasn’t covered by insurance. I have also been approved for Xolaire, although I had to pick up a random prescription I never used in order to get that approved. However, I’m doing so much better after my 2nd styloidectomy that I’m holding off on starting any new medications for 3 months. I’m still taking Cromolyn 4x day and am on birth control to keep symptoms from flairing, but otherwise I’m just doing supplements. These issues are super complicated, but it seems like in my case the vascular compression side is what was causing the majority of my symptoms, as I’ve gotten so much relief from treating those. It’s still unclear whether those compressions were exacerbated by MCAS inflammation, we’ll have to wait and see. I’m going in for a round of blood tests with Melamed in 5 weeks to see where all my markers are. When I asked Melamed about why we didn’t try a systemic anti-inflammatory medicine, he sidestepped a bit and went into how my complement system markers are high and he’s trying to knock those down as a way of decreasing inflammation. That said, my inflammation and fluid retention has gone down dramatically since my styloidectomy. I didn’t realize how swollen my abdomen was, but now I see a major difference, and my PT is astounded by how little fluid is getting stuck throughout my body now. Funnily enough, it seems like we actually need our blood to circulate in order for the rest of our systems to function! =P

I have also stopped taking Eliquis since my surgery, and both Dr. Hepworth and Dr. Melamed acknowledged that in the hEDS population, blood thinners can exacerbate or possibly trigger MCAS symptoms, although neither of them would elaborate as I don’t think the science is quite solid on that yet. So, if you are on blood thinners, you might talk to your doctors about changing or stopping those. I know I was terrified of going off blood thinners leading up to my surgery, but it wasn’t that bad this time around. I was actually feeling good enough that I considered canceling surgery, but I’m very glad that I didn’t! My definition of “good” has definitely shifted now that I’ve had some really good days. It’s amazing what we get accustomed to when in constant pain.

I certainly agree with staying on everything that’s working for you through surgery and recovery. I’ve been cautioned to give it a full 3 months before making any changes so that my body can adjust to the surgery change completely first before trying to deal with other changes.

I hope that’s somewhat helpful. I wish we weren’t all so complicated and that this collection of issues was better understood, but I have seen a couple articles come out this year that are starting to connect the dots. Hopefully someone will figure out the driver soon!

Hugs and healing thoughts to you,



Yes, super helpful. But honestly, reading your posts since this surgery just makes me so happy. To be able to say that you are so busy with work (or just plain old LIFE)… To have come off of almost all of your medications and feel good with that. It is absolutely inspiring! Truly you made me smile from ear to ear when I read this earlier today, and again reading it now. Thank you so much for the information (I think Melamed might help guide patients more than Sarid; he’s so super knowledgeable, but, for example, he just told me to take second gen antihistamines 3x per day… said to just try different ones… but I honestly didn’t know what or how so I go by price tags when I need to get more (these meds all really add up!).

I’ll look forward to hearing of your continued progress and joy of living a more normal life. Am so glad you went for that second styloidectomy! :blue_heart: :green_heart: