I had my 2nd jugular decompression surgery with Dr. Hepworth one year ago. I can’t express how grateful I am for the work Hepworth has continued to do despite skepticism from others in the medical field. I have my life back. I went from being able to walk about 2 blocks on flat ground to walking as much as I like. I went from constant head pain that would flair to excruciating with the slightest activity to mostly pain free days. I’m still not 100%, but I’d say I went from 20% functional to 90% which is live changing. Read on for a more complete story.
I started having severe headaches monthly at about age 28, but they weren’t that terrible so I just lived with it. By the time I was 30, I started to get severe head pain more frequently, often the night or day after physical exertion. I kept blaming myself for not eating right, not exercising enough, not sleeping enough etc. I changed my diet, tried to exercise frequently, cut back on stressful things in my life and nothing changed. In fact, things got worse. By the time I was 35, I started noticing other symptoms. I became gluten and dairy intolerant (severe fatigue and urgent need for the bathroom). I completely blacked out 3 times, always after a rich meal. I stopped feeling rested at all when I did sleep, and spent many nights in pain unable to rest at all. My vision would blacken and I’d get severely lightheaded changing positions. Bending over started triggering head pain. My heart started racing with the slightest exertion. My heart rate would spike just turning over in bed. Then my brain stopped working. The brain fog and fatigue were so bad that I couldn’t concentrate, couldn’t work, couldn’t socialize. It was clear something was really wrong.
Like most of you, I saw many, many doctors who all either dismissed my symptoms as “being a woman” or diagnosed me with migraines and useless labels that only described my symptoms but had no treatment. I tried treatments for migraines, but by the time I saw my 3rd migraine specialist and had tried all the typical drugs, I wasn’t very convinced my issue was migraines since I never had light or sound sensitivity or any nausea. I also didn’t respond well to most of the meds, with some causing more pain. My pain was also always bilateral and way worse laying down than standing up. No one I saw could tell me why my head pain was worse laying down, they would just dismiss that symptom. So, I’d dismiss them and try a different doctor.
Finally, I found my way to Dr. Christy Young, at least the 4th neurologist I had seen, and the first to seem even vaguely interested in getting to the bottom of my pain. She listened closely and ordered a CT scan with contrast specifically requesting evaluation for Eagles Syndrome. That came back positive, with my styloids being very long, and she referred me to Dr. Hepworth.
Dr. H diagnosed me with bilateral jugular compressions caused by calcified styloids. Apparently the length wasn’t actually an issue, but the width of the base was crushing my veins against my C1. He clearly showed this to me in my imaging, and explained in plain English why my head pain got worse lying down, with exertion etc. He also pointed out the mass of collateral veins that I had grown in my head to try and compensate for so much extra blood getting trapped there. He advised me to stop the POTS treatments I was on, as those were most likely making things worse, prescribed Plavix and sent me for a jugular ultrasound.
My ultrasound showed severe compression on both sides, with neither one appearing dominant. The flows were so similar that Dr. H and his PA disagreed on which side to start with. I let Dr. H decide, knowing that there was a chance that relieving one side wouldn’t be enough. I had my first jugular decompression surgery in December, 2022. It went great. Dr. H removed some lymph nodes in addition to the styloid, and disentangled a cranial nerve that had wrapped itself around that styloid. At that time he regularly ballooned the vein after releasing pressure, so that was done as well. He also changed my prescription from Plavix to Eliquis post surgery. I felt immediate relief from the “ram’s head” pattern of pain that had been a constant for the past few years. After the immediate swelling went down, I felt amazing. I honestly hadn’t known how bad things had gotten until I woke up a few days later well rested, full of energy and not in pain. I thought all my issues were solved.
Unfortunately, about 10 days post surgery all my symptoms slowly started coming back, other than the rams head pain. They also were slower to come on, allowing me more time to compensate and treat before things got really bad.
At my 3 month followup I mentioned to Dr. H that my periods had gotten really long, heavy and painful and that I was having pelvic pain fairly regularly. This was in addition to the POTS symptoms and head pain, fatigue, brain fog etc that had also come back. He referred me to Dr. Brooke Spencer for evaluation, and ordered a catheter venogram of my head, neck and renal veins. Head and neck came back normal other than the right IJV still being compressed, but renal came back somewhat compressed and I was diagnosed with mild Nutcracker Syndrome. Dr. Spencer ordered a MRI and found my left iliac vein to be so compressed that she couldn’t measure an opening of any kind from the imaging. She recommended immediate stenting to avoid further damage to my veins. I was extremely hesitant to have a foreign object in my body, so I put that off for awhile.
I did some further evaluation including genetic testing and was diagnosed with Hypermobile Spectrum Disorder (HSD), which is essentially the same as hEDS. I also had markers for chronic inflammatory diseases and was having more and more gastrointestinal issues, so I was referred to Dr. Melamed. He did more testing and found my immune system to be in a constant state of high alert. I was diagnosed with Mast Cell Activation Disorder (MCAD). I don’t ever get anaphylactic reactions or hives etc, but apparently my body responds to pretty much everything I eat and everything in my environment with full body inflammation. Dr. M tried me on the standard antihistamines and some complement blockers with no success. Then he prescribed Cromolyn Sodium, a Mast Cell inhibitor and my fatigue and brain fog lessened. I stopped getting feverish and achy after every meal.
At about the same time I learned of the Cusack Protocol and started taking Maitake mushroom supplements. I was also seeing Dr. Randy James on Dr. H’s recommendation for Atlas Orthagonal (AO) adjustments of my atlas and C1. About 2 weeks after starting the Maitake, I started holding adjustments for days instead of hours. Around the same time I was also able to start seeing Dr. Caroline Creager for PT. She diagnosed me with CCI and started treating for that along with neurovascular outflow issues and hypermobility. The combination of the drugs, supplements, PT and AO helped quite a bit with the majority of my symptoms, but I was still only able to work about 10 hours a week at the most and had stopped almost all social activities, house chores etc. Unfortunately my pelvic pain was also getting more severe, my left leg was nearly always swollen, and I couldn’t stand for more than about 8 minutes. I saw Dr. Hepworth again and he informed me that the latest research was starting to show that abdominal compressions could be connected to POTS and the head symptoms.
So, October of 2023 I got a stent placed in my left iliac vein. My leg swelling and pelvic pain cleared up immediately. My POTS symptoms got slightly better, but didn’t go away.
Since Dr. H schedules so far out, I already had a surgery date for my right side styloidectomy on the calendar. By the time that came around at the beginning of March 2024, I had progressed quite a bit with PT and seriously considered not having the 2nd surgery. That said, I still never felt good, and I could remember those beautiful 10 days after my first surgery when I had felt normal for the first time in years. So, I went ahead with it.
Surgery went well, more lymph nodes were removed, and my vein “plumped up” as soon as the styloid was removed. This time around the recovery was a little slower and swelling was quite a bit higher. I didn’t feel like I needed pain meds, but was advised to keep taking them to keep swelling down as much as possible. I also kept seeing Dr. Creager who did lots of lymphic drainage and gentle massge to keep scar tissue from forming. About 3 weeks post surgery I realized I hadn’t had a headache at all since surgery. Then I started moving around more and discovered my POTS symptoms were nearly gone. Very, very slowly I started doing more intense PT exercises, walking farther, doing more around the house. By 3 months post op I was back to working 20 hrs per week, socializing and even starting a new hobby. It was nothing short of incredible.
I was hoping that would be the end of my medical journey and I’d be able to return to life without considering how every little thing would inpact my body, but that has only been partially true. I am fully functional for everyday activities and for that I am extremely grateful. However, I still occasionally get headaches (very minor compared to pre-decompression), and I still can’t do any serious exercise or strenuous exertion without severe fatigue and significant head pain.
Along this journey I had also considered whether I have occult tethered cord, and a local spine specialist confirmed I that I have at least mild tethering, but he didn’t think it was severe enough to do anything about. I’ve tried to get further evaluation, but haven’t been able to travel to Rhode Island nor afford paying out of pocket for full evaluation. However, Dr. H agreed to order imaging and have a look himself. He didn’t see any indication of tethering, but he did note a small white spot in the dura around L3. He said this indicates a CSF leak, and that could explain my remaining pain and exercise intolerance symptoms. I also have cysts at nearly every level of my spine, which can often indicate CSF issues. So, I’m in the process of getting evaulated by Dr. Callen for a CSF leak. So far he’s not convinced, but I’ll be doing some more imaging soon and then have to decide if I want to risk getting a blind blood patch if nothing visible shows up. We’ll see.
In the meantime, I’ve read some of the more recent medical articles linking EDS, MCAS, Vascular compressions and CSF leaks. While it’s still unclear what drives which issues, it is clear that there’s a connection and that EDS and MCAS/MCAD can weaken the dura and compromise the extracelluar matrix.
So, I’m continuing to try and treat both in the hopes that my clear up the rest of my symptoms without further surgicaly intervention. I’ve added some supplements from the Cusack Protocol, been on a treatment for SIBO and recently started getting Xolair injections all in an attempt to convince my immune system to stop freaking out.
While as I said I’m still not 100% functional and don’t have the energy nor stamina that I wish I did, I’m also 40 now, so it’s hard to say exactly how much energy I should have considering I was in my 20s when this all started. That said, I’ll take 90% functional over constant pain and feeling like I’m losing my mind any day. Hopefully the medical community will catch up soon and give us all some understanding of how all these miserable afflications relate, and in a pefect world, some kind of premanent treatment.
