Overwhelmed and waiting for official diagnosis

[Melodious21]

Hello everyone. I’m new to this site and so thankful to have found it! I’ve been busy reading as much as I can on here to better understand what’s going on with me and how to proceed.

A little (or a lot!) about me… I’ll be 38 this month and have been dealing with worsening health problems since childhood. I started having headaches/migraines as a teenager that got progressively worse until they were everyday by age 22. I’ve tried every migraine medication/treatment out there with no relief. I was diagnosed with fibromyalgia as well at that time, but in 2020 I found out that I actually have Ehlers Danlos (hEDS), along with craniocervical instability and POTS. Before that I was being treated for mold toxicity but my symptoms haven’t cleared up even as I’ve slowly gotten rid of the mold in my system.

Other than the daily and extremely debilitating headaches, and all over body pain from the hEDS, my other symptoms include: light sensitivity, intense pain in my ears (primarily the left, which is the side my pain is generally worse on); hearing loss in my right ear; TMJ pain and jaw popping; facial and sinus pain; nerve/muscle pain and numbness down my neck and arms with worse pain turning my head to the left; a painful popping feeling inside my throat if I swallow with my head turned; dizziness/vertigo; chronic sore throats and hoarseness; pain with talking, smiling, chewing; difficulty swallowing and often feeling like there’s something stuck in my throat; dry mouth; nausea; dysautonomia; heart palpitations/occasional chest pain; chronic fatigue; brain fog; and MCAS. Honestly, I could go on but this already sounds ridiculous!

I don’t yet have an official ES diagnosis but last year I got a CT myleogram to look for a CSF leak and after the blood patches didn’t resolve my symptoms, my doctor who ordered the myleogram, who recently learned about ES, noticed that both of my styloids are over 3cm. He’s now ordered more imaging to better see what’s going on and has sent a referral to Dr. Joshua Schindler at OHSU in Portland, Oregon where I live. He brought up my elongated styloids before my recent blood patch at Cedars Sinai in LA, but wanted to see how that went first. Before my latest blood patch and being referred to Dr. Schindler, I tried calling and calling Dr. Andrew Patel’s office but they never answered their phone or returned any of my messages. Maybe they ignored me because I didn’t have a referral but what medical office never even answers their phone?

It sounds like Dr. Schindler could be a good local option for me but I’m worried about how long it might take to see him because I’ve had bad experiences in the past trying to get into see doctors at OHSU.

I noticed another option in Portland is Dr. Baber Khatib but it looks like he only does intra-oral surgeries and I’m not sure that he’s done that many yet.

How do you decide if intra-oral or external surgery is best? It seems like there are pluses and minuses to both. I’ve been leaning towards external because it sounds more effective and I worry about being able to eat and infections after intra-oral given my negative experience with both of these things happening after a sinus surgery I had. (Side note: I told my ENT who did that surgery about all of my symptoms and she never brought up ES to me). I also have a lot anxiety about surgery in general because with past surgeries I’ve really struggled with recovery and often feel worse rather than better. I’m guessing because of my EDS. Have others here with EDS had successful surgeries?

I’ve also seen a lot about Dr. Samji on here and I’m wondering if it’s worth traveling to see someone more experienced like him. Should I be trying to see more than one doctor for different opinions and to hopefully speed up the process?

I’m just very overwhelmed in general right now. I’ve been stuck in a particularly bad flare up since the beginning of January where I haven’t been able to work, drive, or see friends. I barely make it out of bed most days. My poor husband is really overwhelmed trying to care for me while he simultaneously works 60+ hours a week and we have no family anywhere close to us to help. My parents have offered to have me stay with them in New York if I can find a doctor there, but the options there don’t look great. Thankfully they would also be willing to come stay with me if/when I have surgery. But what to do to manage the pain and keep going in the meantime is incredibly overwhelming. Do others here experience prolonged flare ups when the pain is much worse? Has anything helped you out of it?

The depression has really set in from the prolonged pain and inability to function. I feel like I’m trapped in my own body and living the same painful day over and over. Even though I am generally a fairly happy and optimistic person, I feel like I’m losing hope and my ability to keep going. It is at least reassuring to see that some people are finding relief from their surgeries. I guess I’m just afraid to get my hopes up again like I did with the blood patches. I’m not sure how much more defeat and debilitating pain I can take.

Thank you for taking the time to read this long post! The isolation I’ve been experiencing lately and lack of understanding from the mostly healthy people in my life has been a lot on top of everything else.

[Jules]

Hi & welcome to the site!
Sounds like you’ve had a really tough journey, & dealing with EDS, CCI & POTS as well is rough…
There have been quite a few mentions of these in the discussions; hopefully others with experience of these will chip in, but it seems that surgery recovery can be tough & there can be complications but surgery can be worth it.
Re Intra-oral surgery vs external, the consensus is that external is better- better visibility for surgeons, more of the styloid can be removed, less infection risk etc, although members have had good outcomes with intra-oral.
Dr Samji is very experienced & has done many successful surgeries, so would be worth seeing if you don’t get anywhere with local doctors… @Snapple2020 has EDS & had surgery with Dr Samji. Although if you have vascular ES he’s not the most experienced with that.
There’s been a discussion recently about NYC doctors, here’s a link:
NYC surgeons? - Symptoms and Treatments / Doctor Information - Living with Eagle
Whether they do online appts to save you travelling I’m not sure.
Flare ups are common, there’s info in the Newbies Guide Section about treatments for the pain, but briefly heat or ice can help, lidocaine/ steroid injections into the styloid area sometimes work temporarily, or lidocaine patches, one member’s just mentioned TENS helping, sleeping propped up a bit can help. Nerve pain meds like Gabapentin or Amitriptyline help if you get neuralgia caused by ES.
So sorry that the patches didn’t help, but if pressure on the veins is causing the leaks then removing the styloids is the best next step…
It’s so hard when others don’t understand, & it sounds as if there’s alot of pressure on your husband too, so hopefully connecting with others on here will help. Sending you a hug

[Snapple2020]

Melodious,
WOW, your list reads like my daughter (age 28) and my life (now age 66). My neck issues started at age 19 after a whiplash accident, another whiplash a few years later. Ive been suffering from chronic headaches and neck pain ( as well as worsening TMJ) ever since. I saw chiropractors, PT, OT, massage, acupuncture, cranial sacral practitioners of every kind. In my late 30’s some orthopedic docs (so cal area) told me I was “hyper mobile” and my neck was too loose as well as gave me a fibromyalgia diagnosis. I had several years on and off of proliferon injections. I cant say the prolo helped me a great deal. I was desperate and willing to try anything. A pain doc I see is trying to talk me into more prolo but I have resisted since I am about injectioned out. I did have some specialists at UCLA many years ago say they thought all the prolo injections caused scar tissue nodules in my neck. This is has not been able to be picked up on any imaging but this doesn’t surprise me. I (or my daughter) have so many of the same issues as you, I could write forever. We should probably talk. I have been dealing with the positional dizziness and ear pain myself for the past 2 years and been through significant evaluations for that. Thats a chapter in itself. Many chapters to share but long history of torn ligaments, nerve compression and scar tissue in various parts of my body (primarily hands/wrists,elbow). Rotator cuff tears and more recently diagnosed with thoracic outlet syndrome. I have had probably 15+ surgeries in my lifetime. lets talk about your past surgeries and the problems you had. I have PTSD from some of the recent surgeries I had.

I didn’t get official diagnosis of EDS until my daughter was diagnosed with POTS in 2007 and I began the deep dive into the POTS research and learned of the EDS link from other POTS moms. This after a 7 year journey to find the diagnosis. Even Children’s hospital in LA could diagnosis the POTS but that was many years ago. My daughter has been disabled from the POTS since age 13, has MCAS, EDS and has rarely showed signs of improvement. She did a 10 day inpatient study at Vanderbilt where they discovered she was hypovolemic - low blood volume, not uncommon in POTS. We have tried every drug out there not of which have helped and the side effects not worth it. Cromolyn for the IBS prescribed by POTS doc (also for MCAS) has been one of the few drugs to help and with no side effects. She gets 1L a week saline IV infusion. Between 2 POTS docs and about 18 years we have perfected this as really the only thing that helps her POTS symptoms somewhat stable.

I am curious who diagnosed with the EDS and the POTS as well as CCI and how you are bing treated for that. There is a really good CCI / EDS doc on the east coast. I lived 20 years in southern CA and have seen docs at UCLA, USC and Cedar Sinai and familiar with those facilities and had a variety of good and bad experiences.
I lived in Central Oregon for 10 years and familiar with OSHU. I fired my docs there. My daughter saw a POTS doc in Portland (not OSHU) and didn’t think much of her. I also have flown her to Boston’s Children Hospital along with Vanderbilt Dysautonomia Center and honestly do not think much of OSHU. Personally, if you have complex medical issues, I would don’t recommend going there. Sorry I am a bit of a medical snob.
I now live in Seattle and have flown to LA or Chicago trying to deal with my ear pain, dizziness and mild hearing loss. I don’t have a high opinion of the medical profession in this area except a few. Even my PCP says I (as well as my daughter) am too complex of a patient for him to manage. I do however had a great hand/elbow surgeon who is also an EDS specialist. He has given me some good referrals. He is on the international consortium of EDS specialists.

Personally, if you have a CFS leak that is unresolved, I would use caution before choosing an ES surgeon right away. I totally recommend traveling to get the most experienced surgeon and in my opinion Washington & Oregon is not the place to find one. I thought there was one down at UCLA? Although I like Dr. Samji, his draw back is that he doesn’t really deal with vascular issues and I wished that had been more thoroughly evaluated. Given the complexity of you medical conditions, I would suggest being at a major medical center and not an outpatient surgery center. Have you considered Dr. Hepworth in Denver? He works with a vascular surgeon. Maybe between all our brains around here, we can come up with some more doc suggestions.

I personally chose to have external ES surgery. Dr. Samji gives you some good nerve blocks and prednisone post op and I had very little pain issues. I will say my TMJ issues did kick up though. I am having some reoccurring pain issues that were present before Eagles surgery. I knew going into ES surgery it was unlikely it would solve all y neck and facial pain issues. I had hoped the ES would take care of some of the issues but honestly I think it is the neck instability, possible nerve compression and likely issues within the scalenes in the neck. I also suspect not all the pieces of my calcified ligaments were removed and pressing on nerves.

Ive been to several pain centers (Cedar Sinai) and (UCLA) not to mention a few others. I recently saw a new oral facial pain doc at University of WA. He claims not many centers taking their approach. They are doing lidocaine trigger point injections in the jaw, neck and temples. I recently had something similar in my chest, bracial plexus and scalenes and some of it was helpful. They also do “dry needling”.
As for intense pain flairs…I have had good success with botox injections (also mixed with steroids and nerve blocks) in my temples, occipital area, other neck areas and below the ears close to styloid. Pain killers barely take the edge off.
I have found that regular massage, trigger point injections and cranial sacral therapy to also be the most beneficial but needs to be done weekly to get me out of a bad pain flair. When I was being seen at the Cedar Sinai Pain center, I was given a drug called pamelor to help block the pain path to the brain. In my case it worked like a charm but caused me to gain weight.

I have a friend in medicine in SF. She says the best docs are on the east coast. In my experience she is partially right. I always check out docs CV’s to see where they have trained. There are few docs on west coast that have much knowledge of EDS and Ive come to learn this has been a major stumbling block to many docs I see.

Message me privately and we can chat some more. I have walked this path for many years and had my down days as well. I struggle to watch my daughter go thru even worse but I never give up. I know you are overwhelmed but you are not alone. we are all here to help prop you up and point in the right direction to get the help you need.

[Isaiah_40_31]

It definitely sounds like you have vascular symptoms @Melodious21. Dr. Hepworth in Denver should be your go to. He deals w/ very complex vascular ES cases which often show up in people w/ EDS/CCI. He is also masterful w/ CSF leak repair. As Jules mentioned, getting pressure off the vascular tissues will reduce pressure w/in your skull which in turn allows those blood patches to do their job & not fail.

[Melodious21]

Thank you for your responses and support everyone! I appreciate your offer to connect more with me on all of this @Snapple2020. It definitely sounds like we have a lot of similar things going on! I’m sorry to hear both you and your daughter are similarly struggling with all of this. It has been really challenging to find the proper care in the Portland area for all of these complicated conditions. Unfortunately, my symptoms and financial constraints make traveling that much harder as well. Financially this has all been tough since I barely have an income from going on disability at such a young age (the benefits are really low since I was never healthy enough to work much). These days having a one income household is quite impossible, so my retired parents have done what they can to help with my healthcare costs, but that obviously has some major limits.

Just to clarify a little more about the possible CSF leak, they never did not actually find a leak on imaging. But given my EDS, symptoms, the perineurial cysts they found along my spine, and the fact that the headaches started after I fell backwards off the roof of my treehouse when I was 13, they proceeded with the blood patches to see if they would help. I was told that many leaks don’t show up on imaging so the blood patches can be diagnostic as well. Since they didn’t resolve my symptoms, they’re now thinking a leak is less likely. But based off of what I’m reading, I’m now wondering if the ES has complicated things to the point of a patch on its own not being enough.

It is sounding like Dr. Hepworth might be the ideal option for me, but I’m not sure if it will be possible to see him given the potential cost. I have a Medicare Advantage plan through Regence Blue Cross and was told by them that they will cover out of state doctors if they are in the Medicare Advantage BCBS PPO network. He doesn’t come up when I used BCBS doctor search though, so I’m not too hopeful that he’ll be covered. Are there any other doctors who are good with vascular ES that would be worth looking into?

[Melodious21]

Disregard what I said about Dr. Hepworth not taking my insurance. I realized I needed to change the location to Colorado when I was using the doctor search and it looks like he is in fact in my network. Headache brain can be tough to work through! I’ll start doing my research into him but I believe I read a post on here that said he doesn’t offer any televisits, which might make it tricky to get set up with him. Ideally, It will be tough to afford the extra travel expenses if I have to go there for more than just the surgeries. Definitely worth looking into though if Dr. Schindler doesn’t end up working out here.

[Isaiah_40_31]

It’s true that Dr. Hepworth requires an in person initial appt w/ his NP - Alison Love. After that all pre -surgery appts are with him via Zoom or in person so only one trip to CO is required prior to surgery. That said, your initial visit will also need to include whatever imaging & vascular testing he orders in CO as he has a particular testing protocol for people w/ vascular ES.

Vascular ES requires special surgical care. If Dr. Schindler is at all reluctant about ordering the appropriate testing ie dynamic venogram/angiogram to check blood flow velocities through your IJVs & ICAs, or about discussing his surgical approach if you do have vascular compression, do not consider him for surgery.

Vascular ES can only be dealt w/ via external surgery. The styloids can’t be cut back short enough to free up the IJVs, in particular, via intraoral surgery.

A couple of forum members, who are Dr. Hepworth’s patients & live near Denver, have offered their hospitality to forum members who come from out of the area for appts at Dr. Hep’s ofc or for surgery. If that would make a trip to CO more doable for you, I will pass along their screen names.

[Melodious21]

@Isaiah_40_31 thank you so much for this additional information! Luckily, I am already scheduled for the dynamic CT angiogram/venogram in a week so I’ll have that imaging before I see anyone, but it makes sense that Dr. Hepworth would likely do his own imaging anyway. I went through that at Cedars Sinai with the CSF leak search. It sounds like it’s definitely wise to get a referral to Dr. Hepworth as well since I know that will take some time and I’m apprehensive to put all of my eggs in one basket with Dr. Schindler here, even though I see he has treated at least one other member here with vascular ES. I would definitely love to connect with more with people who are in Dr. Hepworth’s area, at the very least to hear about their experiences with him and his team. It really warms my heart to hear how much people are supporting people on here. I’m already feeling less alone in this. Thank you all again for helping me navigate this! And I’ll let you know how the imaging turns out!

[Isaiah_40_31]

@Melodious21 - I’m so glad you’ve got the angio/venogram testing scheduled. That is a giant step forward. I didn’t realize Dr. Schindler had worked w/ a VES patient. I’m very interested to know what you learn from him about his surgical approach & what type of care he takes to make sure compressed vascular tissues are decompressed during surgery.

We have MANY members who’ve seen Dr. Hepworth for ES surgery. You can use the magnifying glass icon to search his name & discussions where he’s mentioned will come up. I’m sure you’ve read a bunch of them already. The two members I was referring to are @MsBearshark & @nnaeve. Both have some ongoing issues post op that Dr. Hepworth is working on them with. He does not drop the ball when symptoms persist post op.

I’m so glad you found this forum & are feeling encouraged by being among your “peers” here. We are all so thankful for the support we’ve received from forum members here during our searches for answers & solutions to ES.

[Snapple2020]

It would probably be helpful to get the official POTS diagnosis, as well as EDS along with CCI. We used to live in Bend, OR and luckily have 2 excellent providers who at a minimum can provide formal diagnosis for two of them.

1. Dr. Michael Feldman…although he is a nephrologist, he is one of the Oregon POTS guru’s. He’s a great doc and very patient friendly.
   Michael E. Feldman, MD - Nephrology / HBOT

2. EDS Geneticist: Central Oregon Clinical Genetics Center, Osvaldo Schirripa, M.D., M.S.
   541-678-5417. You may have to make several calls to get thru to him. He’s a rare bird that knows EDS on the west coast and he is in your backyard.

3. Dr. Fraser Henderson (CCI/EDS specialist on east coast - Maryland) https://www.metropolitanneurosurgery.org/

I do know there is a radiology group in Portland (Open Advanced MRI?) that does a special rotational MRI that Dr. Henderson requires before seeing you as a patient. I spoke to them on the phone when I was searching out an upright MRI and they told me about several docs in the Portland area that order this specialized scan. I was surprised to find this in Portland area. I think I was lucky to have a radiology tech answer the phone and she gave me alot of info. Ive tried to find the order form for this specialized scan from them as I considered driving down to get it and contacting the docs they told me whom are using it. It might be worth a call to see what docs in Portland use it and do CCI evaluations? I recently had it done in Seattle.

4. As for medicare…I was a state volunteer at one time. Although I know $$$ is tight, if you have been on an MA plan for less that a year (from starting on medicare), I suggest switching out to a supplement if you can. I have been on both and generally speaking MA plans are limited to care only within your state and do not travel. This may vary from state to state or the policy so I suggest you confirm this with Blue Cross you can go to Dr. Hepworth. If your insurance card has a little suitcase in the lower right corner, this indicates you can travel. Supplements allow for care anywhere in the US. MA plans are run like an HMO (even if they claim it is a PPO) and have many limitations such as requirement for pre-authorizations such as for MRI’s,etc. With a Medicare supplement, they are not required. I was on BC PPO MA plan and I personally found it to be a nightmare. They refused to pay for specialized medications among other things costing me $$$ out of pocket - they drug me thru the mud wasting time appealing when I was in serious pain. Their so called dental supplement was a joke and again very limited - I found their PPO dentists to not have a very high level of expertise. If you have a good dentist already who takes it, you are lucky. With a supplement although it costs more, I don’t have to bang my head against a wall trying to get things authorized or get referrals. If you have complex medical needs and need to travel, a supplement is preferred. Unfortunately bumping up to a supplement is not easy from MA (after 1 year trial period) and normally requires a medical evaluation. Insurance can deny it. If you move to another state (sometimes another county), then you can bump up to supplement and it is guaranteed issue.

Hope this helps

[MsBearshark]

@Melodious21 I’m glad to hear you’ve found care in your area, but if you do end up needing to travel to Colorado to see Dr. Hepworth, feel free to reach out. I’d be happy to host you and/or go with you to your appointments if that’s helpful. I’ve only been seeing Dr. Hepworth for less than a year, but I’ve made more progress in that year than the 10 years prior seeing all sorts of doctors who had no idea what is wrong with me. Unfortunately my first surgery (left side styloidectomy) was not sufficient to alleviate my symptoms, so I’m still doing more testing and most likely facing more surgeries, but as @Isaiah_40_31 said, Dr. Hepworth is working with me through it all and has assured me many times that he will not stop seeing me until I feel I don’t need additional assistance.

That said, timelines with Dr. Hepworth are very long, so if you think you’ll want to see him, get a referral ASAP.

Best of luck!

[Melodious21]

@Snapple2020 Thank you for this extra info! I’ve been struggling to find a doctor who can treat the dysautonomia and POTS so I appreciate the recommendation. I do have an official EDS diagnosis from a rheumatologist at least, but he didn’t do much to help manage it. I did a digital motion X-ray for the CCI but different doctors have different opinions about those results.

I didn’t know about a supplemental plan with Medicare. I had original Medicare starting in 2014 and got my MedAdvantage plan with Regence in 2021. So it sounds like it would be difficult to get a supplemental plan? I’ll have to look into this more I guess. Insurance is such a nightmare to deal with and try to figure out. Especially with a 24/7 headache. I’ve had much more coverage and better care with Regence for the most part though. But there have been a ton of newer meds I’ve wanted to try and not been able to because they cost too much and aren’t covered. Thanks again for all the info and support!

[Melodious21]

@MsBearshark thank you so much! That is incredibly generous of you. I will definitely let you know how things play out. I’m sorry to hear that all your symptoms weren’t relieved with your first surgery but it sounds like you’re finally getting the care you need, so I’m sure that feels more hopeful. I wish you well with your treatment and progress!

I messaged my PCP today and asked for a referral to Dr. Hepworth. Is there anything special me or my PCP should know about sending a referral? It sounds like I may have to call a bunch after it’s sent to find out what’s going on with my referral maybe too? How long did you end up waiting to see his NP?

Thanks again for the support! Looking forward to hearing more about your progress!

[Isaiah_40_31]

@Melodious21 - Many of our members who have ES w/ IJV compression have had good reduction in headache symptoms by taking a blood thinner such as Plavix. This is something you could talk to your PCP about. Dr. Hepworth generally Rxes a blood thinner to his ES patients w/ IJV compression during the time they await surgery. Also, sleeping w/ your head elevated at night can be helpful. As you’ve read, we recommend buying a wedge pillow for post op head elevation during sleep while recovering from surgery. It’s not too soon to purchase one.

[MsBearshark]

@Melodious21 you will want to follow up with Dr. Hepworth’s office once your referral has gone through. They typically don’t answer the phone, but will return voicemails within a few days. I also recommend having physical CDs of all your imaging sent to the office, and follow up to make sure that gets into your file before your first appointment. I have not had any luck with virtual transfers of imaging, sometimes they can see it and sometimes not so a hard copy is the best option.

Other than that, you’ll just need to be patient as he is typically booked out at least 3 months.

I also second what @Isaiah_40_31 wrote. Blood thinners have been incredibly helpful to me in keeping my symptoms in check while I work through all the surgeries and testing to hopefully get to a real solution. I’d ask your PCP if they’d prescribe some. if they do and it helps, that is also a pretty solid indicator that compression of your veins is part of the problem.

Good luck!