Thank you @Jules - yes a hard surgery to recover from and C2 can be damaged or sacrificed which is a trade off - numbness for pain. I hope it does improve but anything accepted for unbearable headaches and ear pain that used to leave me writhing and crying on the floor.
I think we all reach a point where surgery and side effects therefrom are the only option. Fear of the unknown is normal, I was petrified and surgery was long and complicated with a large blood loss. But hey, it’s behind me now and so far, only post op surgical pain
I’ve had both C1 transverse processes shaved during styloidectomies/decompression and afterward have had no trouble whatsoever with any kind of instability. Reading articles is one thing, but discussing the subject with your surgeon is the best thing to do.
I’m so, so HAPPY for you, @Mcwelly! You had a long & grueling journey before getting to this point. What a relief to know your terrible symptoms have subsided. You’ll have new challenges w/ the C1/2 fusion but I bet they’ll pale in comparison to the pain you dealt with for so long.
I’d take numbness over pain any day but agree with Jules that your present numbness may gradually disappear over the next months. Maybe just getting that hard collar off from around your neck will help.
I can share that I have hEDS and have significant amount of neck instability and having had 2 whiplashed accidents in the past didnt help. Ive been dealing with it for decades. I had scan and eval for CCI and in the big scheme considered stable although my C2 on the right is borderline on the outer limits of normal. I didn’t even consider a C1 shave making me more unstable. Given my compression level, that had the priority over everything else. I just had revision and IJV decompression last week that included a C1 shave on left side. I had some significant compression and other stuff going on in my neck and happy to have this behind me. There is always a risk with any surgery and I considered this low in my case.
Hello. I also consulted with Dr. Kamran Aghayev, and I’m actually scheduled to have surgery with him next month. After speaking with him, I feel very confident—he’s incredibly knowledgeable, experienced, and took the time to explain everything in detail, especially regarding the importance of the C1 resection. Hope it was a good decision
He gave me surgery quite quickly earlier this week.
I reached back out to him this tuesday after a lul of time after diagnosis and Bassam said he could take me next week.
The money was transferred today, with confirmation to my bank.
I am now scheduled to undergo styloidectomy and C1 shave on wednesday the 6th of May.
Thanks for well wishes. Im feeling a bit fuzzy brained and alot of head pressure, a little TMJ but minor, generally numb in my neck and feels thick. I did have a sinus retention cyst removed too so my sinuses/ears are a bit off. We had a long day of travel Tuesday and Im tired but overall feeling ok, I tend to push myself. The MA at my post-op Tuesday said I was doing better after surgery than she had ever seen. IE: so functional. I laughed because when you have had a bunch of kids, and you are sick, you rise to the occasion because you don’t have a choice. I should probably lay down and ice my neck but I am trying to catch up after being a week away. I was just reading my OP report. The work on left side was extensive. About 80% compressed w C1 shave on left. “jugular vein decorticated all the way down to clavicle”, scar tissue banding and hypertrophy of lymphatics all thru left side of neck. The jugular vein was tightly constrained by fibrotic bands around it much like shrink wrapping…The bulky lymphatics (or lymph garbage) on right were overlaying the jugular, carotid and vagus nerve and removed. There was some scar tissue from previous styloidectomy. Did need to clip more off at skull base by C1 shave. Dr. H suspects this lymph stuff may be caused by long term chronic Lyme which I am going to have more testing for. Likely the long term MCAS causing constant inflammation for decades contributes to this. I am now treating the MCAS which I was not aware of before. I had my daughter US and CT scan while there and unfortunately she too has significant bilateral IJV compression with a C1 shave needed on one side. I looked at her scan last night and it appears it is 90% compressed at C1. This is not the end of the road unfortunately.
I’m glad you’re doing as well as you are, @Snapple2020, but I’m very sorry to hear about your daughter’s situation. I’m glad there’s an answer, or at least a partial answer, to what’s causing her severe POTS symptoms. I hope that can be taken care of this year for her. It would be so great for her to feel more normal which is also what I’m praying for, for you. Step by step, you’re getting things figured out & being able to deal with them. I’m thankful for that.
Thank you @Isaiah_40_31 - definitely numbness over pain any day! Funny little things I had like the blocking of my eustaschian tube on left side, which I had for years, has gone, tinnitus too, pain. So far no infections since op . It’s always difficult to know whether styloids were cause or if the ES symptoms came on because of c1/2 involvement and luckily this seems to have helped. I’m so grateful for all your support on here, the source of reliable signposting, information helped me get to the answers and help, thank you so much!
I’ll keep you posted and look in from time to time.
I’m glad that you’re back home, and functioning as you said…I know it’s hard as a mother, especially with their health conditions, but if you can keep icing & rest whenever possible, this will help you heal quicker…easier said than t=done though I know. It does sound like you had alot done, I hope that when the swelling goes down you’ll start to see the benefits, and I hope the testing you have done will be helpful. You’re so strong to keep seeking answers and treatment, at least the journey you’ve been on should save your daughter having to wait years for answers to her issues. I’m sorry that it sounds like she’ll need surgery too, but she’s in good hands- yours & Dr H’s! Hugs and prayers
Thank you very much. The surgery is scheduled for the end of this month. I plan to come to Istanbul for two weeks to recover a bit. I was also quickly scheduled for surgery, and God willing, everything goes well. I will keep you updated on my recovery if anyone is interested.
@GalinaGutak - I’m so glad you were scheduled for surgery quickly & yes! as @Jules said, we’d love to hear how your surgery went & how you’re doing after surgery. We’re here for you along the whole ES journey so please post anytime you need support or have information to share.
Thank you for sharing @Snapple2020 I was hoping to read an update from your surgery and how your are doing. I have been thinking of you so much! Hope all is going as expected. I wonder what your pathology will show from the lymph node removal… Dr. H is suspicious our daughter’s excessive swollen lymph node is from long term effect of mono or an autoimmune problem. Oh I hope it is not something too serious that needs treated on top of everything else she has going on! I hope for you that you don’t have some major thing going either! Do you mind sharing how you are treating for MCAS? I like to learn all I can for my girls sake.
I am so sorry to know your daughter has a severe IJV compression and am I right she has abdominal compressions also? Is there an end to the road eventually where we have done all the surgeries and treatments needed to function normally?
Hi Buckeye! I did find out that the pathology of the lympnodes are not tested for lyme as Dr. H indicated by his PA - this was disappointing. They just came back as benign. My assumption is benign for cancer. My lymphnodes were swollen too and on the left side he described the lymph tissue as shrink wrapped around the IJV. He referenced is as lymph garbage. He thought the same for me re: chromic lyme or EBV, some sort of autoimmune. He did suggest getting more lyme testing re: western blot. I know this is a controversial DX. Another rabbit hole to explore.
My daughter has been taking Cromolyn for years and I started on it several months ago for MCAS. They had me double up on it before surgery and after. I can say that after being on the Cromoyln for 4 days, my post nasal drip went away. There is alot of new data coming out on MCAS as the driver to inflammation especially in hEDS. I will likely now turn to focus more efforts on treating MCAS aggressively in myself and my daughter.
Yes, my daughter has significant IJV compression on one side and one side is anatomically very small. They say she hypoplastic vein. Iv pulled it into 3D and the IJV sizes from one side to the next is huge. Ive been too busy recovering from my surgery to dig into it very much. The PA looked at her velocities at my post-op and said yeah she needs surgery which I expected. Thats next on the list. I might need to get a specialized scan for CCI just to be sure.
I have private consults set up with a vascular surgeon who is reviewing all my chest & ab scans that show possible MALS, SMAS and US that shows Nutcracker and May-Thurner. My daughter has only has had US that indicates possible nutcrackers, luckily no May-Thurner. Ill be looking to get her specialized scans of her abdomen to check for MALS and Nutcracker. This is getting pretty complex. Im starting to get a handle on the what I need to get more definitive info on the nutcrackers which is my biggest concerns.
Is there an end on this road? God I hope so. As you know, it is alot to manage. I still have 2 other adult kids starting to show signs of problems albeit not like this daughter.
@Snapple2020 - Your plate is overloaded! I’m so glad you’re finding a path forward w/ all the things you’re looking at getting diagnosed for both yourself & your daughter. I hope for both of you that your testing doesn’t show positive for everything you listed.
I agree it’s disappointing that the lab didn’t test your lymph tissue for Lyme or EBV. I think the tissue samples are kept for a decent period of time post op. I bet you can request it’s retested for Lyme & EBV. You’d probably have to do that through Dr. Hepworth’s ofc but maybe through the hospital if it’s their lab that did the testing.
