First of all, I need to explain EDS.

The WHY BEHIND ALL THIS: 

Ok everybody just so you know:  I have hypermobile Ehlers Danlos Syndrome (EDS or hEDS).  This is a genetic disease where your collagen-building doesn’t work well for your ligaments,  so all your life your joints are cracking around out of alignment,  so you have pain in your joints pretty much all your life.  I was lucky because the pain with disconnections didn’t begin for me until my 20s.  It also disrupts all of your spinal joints which disrupts all of your nerves.  Many EDS folks are already disabled from walking by their 20s. And yes, we LOOK FINE.  Please please do not say that to me. “But you LOOK Fine.”  Because I have had to fight being labeled as crazy patient for years until I was diagnosed (about 20 years ago) and I am tired of explaining that yes “we LOOK FINE” but we are not FINE.  I will never be FINE and pain free. This is not going to go away.   It is progressive.  AND I am so fortunate that I have felt better when exercising, so I exercised my entire life ! Since we were kids, it was swimming, skating, biking, running, hiking, later Pilates, biking, walking, swimming on into adulthood. And therefore I have relatively good cardiovascular and muscles where the nerves are still working.  OH and no, a collagen supplement does not fix this.  No supplement or correct diet will fix this and as you know my diet is f*ing  amazing GF plant-centric anti inflammatory yummy and gourmet!  Oh and why doesn’t a good Chiro fix this?  Oh I DO have a great EDS chiropractor (Dr. Curtis Turchin in Sebastopol) but it has to be someone who understands careful gentle joint alignment. And even if you had a chiro at home every minute, so many joints go out of alignment regularly that it is only a part of my usual careful health maintenance.    So I manage normally with PT, massage (Thank you forever Claudia!), Chiro, exercises, hot tub, swim, etc.  You get the picture.  

As some of you know,  my horrible maze through the inept dipshit groups of docs at Kaiser that ranged from “there is so much inflammation I have no idea what to do” to “I guess I could rip out everything from the skull to the thoracic” and “your meds are causing this” I have had to fight for a year to get care.  So, I am a bit burned about the “you look fine” when it turns out yes my HUGE SMART brain is slopping onto my spine and yes the skull is cutting off the vertebral artery and a mass of nerves. This explains the excruciating head/face/neck/shoulder pains that got so much worse a year ago.  Did I go to the ER? Of course not. I am a nurse, it’s COVID,  and I would not do that.   Do I sob, cry and scream? Not usually.  Not my North Dakota Pioneer Woman Way. 

Why are we zebras? Not only are we DAZZLING (A group of zebras is a dazzle!) but when you are in med school you hear this often: “when you hear hoof beats behind you it is usually not a zebra.”  Because medical students love the odd diseases they are reminded that it’s normally NOT a zebra but it’s a common horse or cow (run of the mill diseases).   WE ARE SPECIAL! Yes we ARE!  We ARE zebras and we have a lot of subgroups of issues that occur in our bodies including allergies (Mast cell activation) cardiovascular effects.   Luckily I do not have the vascular type of subgroup which causes ruptures of vessels.  This is extremely fortunate for me and all of you who are genetically related! 

OK, enough about the WHY except to say this: The sagginess of the ligaments means your brain can slop and fold through your skull base (Chiari Malformation) and also my skull is moving around 8MM and this cuts off the nerves as well as the arteries. (Atlanto-Axial Instability).   So.  This is not without some pressure onto all the nerves down one’s spine so its hard to stay upright (dizziness), nausea, difficulty swallowing, pain, and pressure on nerves starts to impinge abilities to move, function etc.  Also as many of you know any jiggling or pressure changes means worse pain:  car, walking, elevation changes when traveling. Also it means floating in the pool is the BEST! No pressure….sigh! 

Oh yes and the other thing people ask:  Couldn’t you just exercise more the right way and fix this? NO. Exercising carefully is great and helps, but you cannot make nerves work that don’t work anymore due to pressure on them, and those nerves connect to various muscles. Those muscles won’t move if they don’t have innervation. No matter how much you try to make them, honestly.  The nerves won’t engage those muscles so that you have control over making them work.  

Believe me if I could have fixed this I would have.  You all know me well enough to know this.  Nothing daunts me if I have the resources within or without to conquer it.  And this time it means going to my lovely EDS clinic in Maryland where Dr. Henderson (The Godfather of all EDS neurosurgeons) and my doctor Malini Naranayan works.  They focus on EDS with all of our special needs.  I cannot tell you how amazing it was to finally be HEARD and SEEN and to be treated with respect.  Love them all! NO there are not doctors on the West coast that deal with EDS effectively in this way.  I would stay close to home if I could.  Why not Mayo? Their EDS focuses on vascular. They did not invent the special surgical supports that Dr. Henderson did; and his group has published many studies to inform and train others. 

Who are the well-known people with with EDS?  Many dancers have EDS (especially ballet) but also Leah Dunham has it, also Jameela Jamil (The Good Place).  

I cannot wait to get my Skull-Thoracic surgery done..then we will have to deal with my lower back but THIS is the highest priority now! If anyone wants scholarly articles on this I have read so many of them and I will be happy to send them to you! Many of the articles were written by my doc Narayanan and Dr. Henderson.  

Thanks for listening! Mostly I deal with issues with humor and of course my HUGE SMART BRAIN is causing this but it actually is the ligament issue.  I lied about the brain being too big. Sorry.

So basically I have little choice but to have this surgery in the midst of COVID, staffing shortages, supply chain hospital shortages, you name it.  Either that or I basically have no life ahead. This is pretty much unbearable.  And I am a f*ing badass Bernhoft Bitch.  Guessing no one will ever forget that!