Niki Naeve

Note: The next few entries will be very detailed information about my tethered cord experience, which will be utterly boring to most people.  However, I’ve received quite a few messages from fellow “warriors” with questions about diagnosis and treatment of tethered cord, the exact surgical process and recovery process – so this is for them. 

The Plan, beginning March 11, 2024:

Day 1: Fly to Boston.  Pick up (an electric) car and drive it to Providence. 

Day 2: Shop at Whole Foods and make a big vat of paleo-friendly chicken and veggie soup, freeze portions ahead for easy eating later.  Get plenty food for breakfasts and snacks for both of us.

Day 3:  Surgery.

Days 4-6:  Still in hospital?  Back to Airbnb?  Won’t know until we know.

Day 15: Drive rental car back to Logan airport.  Use wheelchair in all airports. In Denver, take arranged ride home.  Lay on couch, ice, go to bed, repeat.

Of course the plan threatened to unravel nearly from the start.  It would have been helpful if we’d had instructions regarding where to get our car before landing, goggle-eyed, at the capacious Logan International airport. Eventually we followed people out to a bus stop and figured we’d ask the driver where they were going.  There turned out to be no access to the driver, but other passengers we squeezed in with confirmed we were heading to the rental car area.  Whew!  I t was crowded and lots of luggage had to be lugged; it was clear right away this would not be a good situation for me on the way home.  So, we modified the plan.  New plan: Colin will drop me at the airport (with my backpack stacked onto wheeled luggage I can handle.)  Then he’ll drop rental car and take the bus back.  I’ll check in (three seats for me, one for him) and secure wheelchair ride.

So you aren’t as confused as we were:  https://www.massport.com/logan-airport/getting-to-logan/car-rentals

Our Airbnb had a Tesla charger.  I’d called Dollar several times to try to establish if whatever we picked up would be compatible (or come with an adapter,) and was told that we’d probably  get something which would work, but we’d have to confirm by calling the location desk… which of course is utterly unreachable.  We figured, worst case scenario we’d buy an extension cord and charge verrrry slooowlyy using a regular outdoor outlet.  More on that, later.

At the rental car pickup location, several things happened.  When we arrived, it was already after 10pm, so we were ready to be on our way.  Let me summarize as succinctly as I can: There’s a 90 minute wait for cars right now, we suggest you try another company.  Trying others, we learn that others have the same problem.  Back in line, now there’s a three hour wait.  Your time starts when you get this slip of paper into the hands of someone with Dollar in the garage.  We make our way to the garage.  Downstairs garage tells us Dollar is upstairs, upstairs tells us it’s downstairs.  Round and round with luggage we went.

Then there’s the extra driver issue…I’d forgotten Dollar charges for any extra driver, domestic partners, married, whatever – there’s a charge.  For this trip this is really silly, because while I reserved the car, I will never be driving it.  But it’s too late to change the renter and have Colin reserve it.  I find on line that if you join “Dollar Express Rewards,” you can bypass this annoying charge.  I have plenty time waiting for the car, so I join.  (The phone app for this is obnoxious – join using a laptop at home if you’re going to.  There is also wi-fi at the rental car hub.) 

Alas, the guy at the desk says he can’t add the membership to the reservation, but I may be able to do it on line, or the national phone line surely can.  The next day I did call the national line, (there’s always about a 20 minute wait) and they said this can only be done at a physical location. 

For the record, companies like Enterprise do not charge for an extra driver.

Finally, I set Colin down on a bench with the luggage and go in search of the right place in the garage on my own.  It’s in the opposite direction from where we’d originally set out.  Our car is waiting for us.  Had been, since we arrived!!!  Turns out there’s no wait for electric vehicles.  The garage just never told the desk, so the desk never told us.  We could have been on our way an hour ago.  We open the car.  Under a panel in the trunk Colin quickly found what we hoped was a converter which would work at our Airbnb.  Finally, we’re on our way.

At that time of night there wasn’t much difference between toll road and non-toll drive times to Providence, and we had chosen not to pay the daily fee for unlimited toll access, which meant if we passed through one we’d be charged $10 for the day plus whatever tolls (usually a couple bucks.)  We broke out the snacks and I tried to navigate while Colin figured out the ins and outs of the first EV either of us had ever driven. 

It was a Kia Niro.  The dash controls were a mystery at first.  The same panel which controls the radio controls the heating and cooling, you just have to know how to toggle it. There’s a round knob which you use to select drive vs. reverse, etc.  Of course there’s no gas gauge – miles left on your charge are displayed, but can change wildly based on how you’re driving and how cold it is outside.  There’s a learning curve,  it’s past 11pm, and we’re navigating dark backstreets in Boston.

Roads on the East Coast, similarly, are a mystery.  Nothing is straight.  Everything is under construction.  We plotted a course going North of Boston to go South to Providence in order to avoid tolls, and took several wrong turns.  I’d say if you’re not a confident driver/navigator or equivalent team, just take car services, or at least decide to pay for toll roads, which are much more straightforward.  Thankfully Colin and I are pretty experienced travelers, have GPS and a well-honed shorthand for communicating on the fly.  Snacks help.  We made it to the Airbnb.

The next day, we navigated our way to Whole Foods in Providence and (as best we could estimate) bought groceries for two weeks.  There are plenty other local grocery store options, but I find Whole Foods (aka “Whole Paycheck”) is an easy one-stop shop for those with special dietary needs.   I’d already decided I was going to press the “easy” button for many things these coming weeks, even if it cost a bit more.

I came home and made a big pot of chicken and veggie soup.  I’d brought some “Better Than Bullion” broth, which mankes any soup tasty,  as well as an array of spices packed in teeny containers, an invaluable trick we’ve used in travel for decades.  I had also pre-packed several Tokyo Joe containers and used those to freeze portions for future consuming.  

I reviewed the information from Dr. Klinge’s office and planned out the route to the hospital, parking, and the special disinfecting showers I’d need to do before surgery.  I packed a separate bag to have with me in prep (keeping lip balm, tissue, my phone, glasses and a few other convenience items, taking out valuables like credit cards and cash) and several bags for Colin to load into the room (pillows, a blanket, clip on phone holder, snacks, water.)

One thing I appreciated in Dr. Klinge’s instructions is the departure from the usual restriction of water after midnight the night before surgery.   (Food is the same as usual – don’t eat.)   I was allowed to get up and drink water during the night.  So I set an alarm and drank a full glass of water (with some electrolytes) around 4am.  This should supposedly help those of us with difficult veins in surgery prep, and make us less uncomfortable.

 If you’re not familiar with The Bendy Bodies Podcast, it’s worth checking out.  The host is an anesthesiologist who has  EDS.  She has pushed back on this after midnight water restriction, knowing that many of us struggle with POTS, and dehydration can lead to a nightmare of deflated veins the morning of surgery.  About 75% of Dr. Klinge’s tethered cord patients have some form of EDS, so thankfully the team is fully prepared for our special needs, like how we are positioned and moved during surgery, our predisposition for MCAS and POTSs, and how we process anesthesia.  Check out Dr. Bluestein’s episode on preparing for surgery here:  https://www.youtube.com/watch?v=b_Ls8OWKxU0

Alas, despite my diligent intake of water, there was still the usual difficulty finding a vein.  I’m not gonna lie, I may have used the F word once or twice times during the procedure, during which time Dr. Klinge happened to enter the curtained enclosure for our pre-surgical chat.   She kindly noted, “This is obviously quite painful for her,”  then after hovering in a slightly motherly way for a moment, waiting for a non-agonized window to speak with me, (or perhaps trying to think of something more comforting to say,) apologized that she had to be on her way to her first surgery, and took her leave.

Finally the anesthesiologist came in and aimed for the cubital vein, in the crook of my left elbow.  This tends to be the easiest access in many bodies, but I let him know that’s my least favorite spot for an overnight stay.  He assured me they’d find a more comfortable one once I was under (apparently veins tend to plump up once you’re down) and he’d remove the cubital.  Alas, though it wasn’t used, that hole stayed in my arm the entire stay, the nurses making the logical point that if my other access failed, this would be a good backup and save me an additional poke.  It was only uncomfortable when I bent that arm, but was overall just a minor annoyance for a couple days.

I’d been looking forward to this moment for weeks.  The moment when anesthesia kicks in and there is nothing left for me to do, consciously.  It was time for Dr. Klinge and her team to do their magic. Someone turned a switch and it got dark, fast. 

I once asked an anesthesiologist if people dream when they’re under, and he replied that the state of being anesthetized is really more akin to being in a coma than falling asleep.  So, being fully out is generally a dreamless and painless experience where time just seems to skip to when you wake up.  Twilight sedation can be less time-jumpy, and some people remember some experiences.  Because EDSers tend to process anesthesia differently than others, we can be prone to those scary experiences where anesthesia does not work properly.  This happened to me during my cardiac ablation, done long before I had an EDS diagnosis, a traumatic experience of being strapped to a table and feeling every time the electrophysiologist burned my heart, back and shoulder in spasm.  Thankfully, EMDR for PTSD really helped my brain cope with that experience.

Still, many anesthesiologists aren’t aware of our needs.  A few years ago when I was being prepped for a different surgery, one rolled his eyes condescendingly and said, “Where did you read that?”  So it’s a real comfort that Klinge’s team is on it.

For the medically curious, this is the best video I’ve found of the actual surgical process – scroll down until you see a video.  It’s short, filmed clearly and well edited!  https://link.springer.com/article/10.1007/s00701-023-05568-9?fbclid=IwAR3vDipC7-5Le_RssRFHxKBGqALYMlOPysly3DeDtacIXQE5GsL0iX5iXNo 

Colin was particularly impressed with the fact that during my surgery someone stimulated nerves with electricity to make sure they were what they thought they were, so a nerve to, say, my leg wasn’t accidentally cut instead of the filum terminale.  We picture a Frankenstin-like experience where someone zaps the open nerve in my back and my leg jerks. 

Another interesting aspect of Dr. Klinge’s surgery is that she not only cuts the filum, but removes it completely and sends part of it to the lab.  (I’ve heard Dr. Pang cuts, but does not remove it.)  Dr. Klinge believes that removing the filum in its entirely reduces chances of re-tethering.  Currently the re-tether rate is about 5%, and there’s little data about exactly how or why some people re-tether and some do not.  Some theorize it may be more likely to happen if you over-do physical activity or don’t have your MCAS (mast cell disorder) under control.  It usually happens around the 6 month mark, and I’m told that would feel like symptoms which disappeared suddenly recur with a vengeance.  Fingers crossed, I won’t have to feel that.

A note about MCAS –  I heard Dr. Maitland speak at an EDS conference in NY.  This thing she said stuck with me: “The mast cells disrupt the collagen, the collagen disrupts the connective tissue; the connective tissue disrupts everything.”  Many EDSers seem to have mast cell over-activation, if not a formal diagnosis of MCAS.  Indeed, I’ve heard a rumor there’s a study coming out in mice in which MCAS and EDS seem to be inextricably linked, perhaps carried on the same gene or set of genes.  So if you have tethered cord and haven’t investigated for EDS and MCAS, that’s a direction you may want to look.

Colin says Dr. Klinge spoke with him after my surgery.  She said the character of the filum was “supportive of the diagnosis.”  Seems that means it was kind of tough, presenting some difficulty removing it.  I picture someone pulling this thing like a tough worm out of my lower back.  Lab analysis also indicated “congested vasculature.”  No one seems to know what that means for sure, but those in the know are suspicious this could support the idea that my Renal Nutcracker Syndrome has created collateral veins along the spine, which could be causing some of my paraspinal pain. 

Speaking of vascular compression syndromes, no one knows how tethered cord surgery may affect them.  There’s just zero data. When I told Dr. Klinge that before investigating tethered cord, I’d been planning to have MALS surgery next, she said that she’d done TC surgery on a few patients with a MALS pain pattern like mine (front to back, just to the right of the diaphragm) and they’d had mixed results.  One or two had that pain clear up entirely post surgery.  One or two experienced some relief.  One or two felt no relief and went on to have MALS surgery next.

Anatomically, I can see how surgery for TC could make abdominal compressions better or worse.  I’d imagine it could open up some visceral space, which could be good for some compressions.  But I can also see how it might cause the renal vein to stretch, which could exacerbate symptoms of Renal Nutcracker Syndrome. 

A “Nutcracker friend” of mine has experience in medical research and we’ve discussed creating a survey for tethered cord surgical patients.  Dr. Klinge’s office has responded favorably to the idea of a voluntary set of questions her surgical patients could respond to about compression syndromes, before and perhaps a year after surgery.  The idea would be to see if any patterns emerge about how these syndromes respond.  While we recognize it could not be 100% scientific (because these people will not be medically screened for compressions by a research team) we figure it could point in a direction, which could encourage more research.

Next thing, I’m waking up in recovery.  I must have drifted in and out of consciousness, because I don’t remember much until Colin showed up, and he says that was several hours.  A nurse popped in and out of my vision, checking vitals, speaking soothing words.   I recall that he was funny.   Several of us were held there because there was “no room in the inn.”  Apparently, there was a shortage of patient rooms available, and rumor had it we may be staying in recovery for the night. 

Colin was told guests aren’t usually allowed in recovery, but when dinner arrived they let him in to feed me, possibly because they were shorthanded, (not to mention feeding a person while they’re flat on their back is best done by an intimate acquaintance,) and possibly because at this point they thought I’d be in there for the night, anyway. 

The requirement is twenty-four hours strict horizonal rest after surgery, to prevent CSF leak.  (Dr. Creager expressed a bit of surprise it isn’t more like forty-eight, but that is Dr. Klinge’s procedure.  In the end I spent most of two days horizontal, anyway.)  I didn’t think I’d be very interested in food, but when Colin waved some chicken pot pie in front of my face, my tummy reminded me I hadn’t eaten since the night before.  And I thought maybe eating horizontally would cause reflux, but it didn’t.  So far, so good.  And for the record, the chicken pot pie was the best meal I had in the hospital.

By the time a room opened up, it was 7pm – the end of visiting hours.  So Colin ran down to the parking area and grabbed my things, leaving them with the nurses at the entrance to the wing where the patient rooms are.  I think we could probably have insisted he come into the room with me, as another patient was led in maybe an hour later and her fiancé came with her, staying for maybe fifteen minutes, but we didn’t want to be a bother.  I was a little concerned because I’d given Colin the rundown on all my stuff and where to put it.  This included:

A standard sized pillow for my head

A king sized pillow for under my knees

A fleece blanket

A zip-lock bag which I’d modified to be able to tie to the bed rails (using a hole punch and some tape) which contained things I might want during the night – glasses, lip balm, shea butter, a small flashlight, cough drops, ginger hard candies, tissues, eyedrops, airpods, sleep phones, and my phone.  This was a brilliant suggestion from another patient who’d undergone the same surgery.

A couple water bottles.  I brought my Lifestraw because I’m aware I can be sensitive to minor differences in water supplies, and it provides a robust filter.  I kept this in bed with me, along with the bag.

Chargers for phone, kindle and headphones

A gooseneck holder for my phone

A change of clothes

Adult diapers (in case I couldn’t tolerate the catheter)

A no-spill water bottle

My kindle

My sleep phones

A portable room thermometer

Electrolytes

Somehow everything ended up right where I wanted it.  Maybe they just figured it out, or maybe I bossed someone around to get it that way.  I’m not sure.  As I settled in I realized I actually had a reason for being a little anxious about where my stuff would be and who would have control over it all night.

When I was five years old, I somehow contracted a case of pneumonia severe enough to land me in the “brig.”  When I say “brig,” I mean at the hospital they put me in a crib!  I was five!  The indignity!  I watched nurses come and go through the bars like a common criminal.

At night, things got quieter and darker, and all my people left me.  A quilted, powdery pink robe with a white ruffle at the neck, which my mom had lovingly made for me, hung on a hook on the outside of the crib’s enclosure. A pink teddy bear (given to me by my mom’s friend, Linda) was tucked in beside me.  I wasn’t afraid or lonely.  I was cold. 

What to do about this dilemma?  There are no blankets in here with me.  I can’t sleep.  I’m cold.  I tried some more to sleep.  It’s no good.  I’m too cold.  I looked at the nurse call button.  Wasn’t that for emergencies only?  Is being cold an emergency?  Well, I can’t sleep.  That can’t be good, right?  Surely someone just forgot to leave a blanket in here with me.  Someone will say, “Oh, dear!  My mistake!” and bring me one.   It was then I mustered all my little five-year-old courage… and pressed the button. 

After some moments, a nurse came.  She asked me what I wanted.  “I’m cold,” I said.  “Could I have a blanket?”

The nurse looked sincerely apologetic, but the answer was no. “I’m sorry.  You have a fever and we’re trying to bring it down, so I can’t bring you a blanket.” 

Well, that can’t be right.  I waited until the nurse was long gone and assessed my options.  I could call her back, but the answer would probably be the same.  I could make a jailbreak, but that would cause a ruckus, I’m sure of it, and I would be in trouble.  I spied that robe hanging on the outside of the crib.  If I reached my tiny hands through the bars and twisted just right…I thought I could reach it.  And who’s to say it didn’t just  – fall – on me, accidentally (I seem to remember that word “accidentally” being thrown around a lot in my household at that age – like when my brother punched me, it had happened “accidentally.”) Or, who’s to say, maybe I was wearing it before and no one noticed. 

I snatched the contraband robe off its hook, laid it on top of me and snuggled my exposed neck against the softness of the pink teddy bear to keep it warm.  And promptly fell asleep.

It’s the first time I can remember ever having been “naughty,” or doing something I was pretty sure adults didn’t want me to do.  And it felt right.

Now it was time for my first night in the hospital since I was five. 

Next:  Trading Tethers?