PBC and hEDS/HSD … Please, I’m at a loss of what to do next. T/U

Posted by aeiou13 @aeiou13, Aug 11 1:46am

Hi! I was diagnosed with PBC in 2008, been taking Ursodial ever since. Along with that I’ve been having gastrointestinal problems, too. Now Imodium is on my list of medications along with vitamin D, gabapentin, and 2 depression meds - depression since 1998. Then, in 2010 I started having major pain and problems with my R wrist. Over the next 10 years I had ulnar shortening R, hardware removal in R ulna, proximal row carpectomy R (bones removed), finally R wrist fusion, then hardware removal again, then ulnar shortening L and a screw in L thumb base (cmc). I had X-rays, MRIs, CT scans, and LOTS of blood work. (Always neg. For RA) Over all that time I had each surgery because the cartilage was seemingly disappearing and my bones were (are) rubbing against each other. I have always had a high tolerance for pain and only did each surgery b/c the pain became unbearable. Finally another surgeon recommended seeing a Rheumatologist and at the first appointment he diagnosed me with hEDS (2021). He gave me a prescription for celebrex. My R wrist pain returned and got so much worse (b/c my cartilage was totally gone, my ulna had turned “sideways” and the protrusion was on top of my hand instead of at the “wrist.”) This past December a more specialized surgeon did the Suave-Kapanji. It has worked well and now the pain in my L has become unbearable, to the point of wearing my hard thumb and ulnar brace all the time. My surgeon had already done many tests on it and I have a lot of arthritis and more bones hitting bones. He said he would hate for me to have 2 totally fused wrists b/c I’d be unable to do even more than with just one. I’m planning to have partial fusion this fall, preferably asap b/ I essentially can’t use it; I can only use my fused wrist which is already limited.
Just to add; I have restless legs and have trouble getting to and staying asleep. Most days my feet and ankles are hurting and become “frozen” and will not bend for sometimes up to an hour. When I walk, anywhere, anytime, both knees and hip bones can randomly pop in and out. My brain fog has gotten so much worse over the past couple of years and I’m just not who my brain thinks I am anymore. It’s driving me crazy.
I did some therapy last summer with someone knowledgeable about EDS, but I could only do it for 2 months. It helped a little bit but I know I need more. However, I’m a high school science teacher who loves her job. I am 100% devoted to my students and doing anything and everything to make my class the best it can be for them; my work lives with me, I don’t “leave it at the door. Which means when I have any time at all it’s for my family.
So, to my question (just wanted to be thorough with my background):
With what feels like being in my own personal hurricane, how do I manage my intense daily pain, surgeries, relearning to use my hands after every procedure, having digestive problems so I have to take Imodium if I leave my house, my increasingly forgetful and foggy brain, and my own hip-pops everyday? I take so many medications and then still need Advil all the time. Family has suggested Lions Mane (?) and it looks great, but has so many interactions with my Rx’s. It’s getting harder to do my job with the level of perfection that I want to give them.
I know this is long, so thank you for taking the time to read it. I just happened across this website today and thought it wouldn’t hurt to ask for help, which I obviously need to do. I feel like my tire’s stuck in the mud and I can’t get out by myself. Thank you so much in advance for anything you can share. Even if it’s that you have common ailments and we aren’t each battling alone. 🙂

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I am so sorry you’re struggling with so much pain and disability. It’s amazing you are so devoted to your teaching and students and deserve a lot of credit for that under the circumstances. I seriously wonder if you do have rheumatoid arthritis. Up to 40% of RA patients have negative blood tests for it. In those cases it is diagnosed by the patterns and appearance of the joints, MRI findings, especially erosions in the joints. If you have not been thoroughly evaluated by a rheumatologist, I think that’s essential. There are many effective drugs for RA that prevent the joint destruction you’re experiencing. Hands, wrists, ankles and feet are the most common joints affected by RA. The EDS is harder to treat and certainly complicates your situation. What kind of doctors have you been working with besides the surgeons? I sure hope a good rheumatologist can help you, preserve your joints and reduce your pain. You have come to a good place to get information and emotional support!

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Hi, thank you for this. I’m seeing a Gastroenterologist and a Rheumatologist. My Rheumatologist only did the Beighton test and I believe I scored a 5/9. My Gastroenterologist has been doing my blood tests, mainly for my elevated liver function tests (LFTs), and trying thyroid, iron, celiac, and RA tests, too. All these other tests have been negative.
One of the toughest things, if I’m honest, is having to be the one who looks up information and tells the doctor what to test or look for or what I think is going on! I experienced this first with my PCP about 2 years ago when I asked what he thought about therapy with knowledge of hEDS. He said that if I think it’s something I want to try that he’d give me a referral and just to let him know who it was and he’d do the paperwork. He said he has too many patients to be able to keep up with everything for every patient so he’d take my suggestions into consideration for my health. My previous doctors/surgeons told me NOT to look up stuff on the internet because not everything is accurate.
Tasked with being a self-advocate for conditions that are rare (though I’m so thankful for this website!) is proving to be so hard! Not knowing about them leaves me not knowing what to look for or ask about - Just like my students!
I’m scheduled to see my Rheumatologist next March, so maybe I’ll send a message about wanting to look into the possibility of having RA. Thank you so very much for telling me about this! 💚

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