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Winter of Disjointedness

I haven’t written an update in over 7 months, which boggles my mind. It was the same amount of time between my two previous blog entries, too, so I’m not doing a very good job of keeping my friends and family up to date, but I find the speed at which our lives zoom by very unnerving.

Yesterday, my husband and I were marveling that it had been over 2 years since I stopped doing weekly immunoglobulin infusions and IV fluids. For so long, it was our routine, dictating the days he worked, the days we cleaned, what I ate, when I showered… Then we stopped and lapsed into a no-structure schedule and I’ve definitely gone downhill since then, but can I blame the lack of IVIG? I don’t know. I guess partly. 

It has been incredibly beneficial to me to track the big changes over the past 12 years – it’s helpful for my mother, too, who needs to take notes to remember the big diagnoses and events, let alone the nuances of ever-changing chronic conditions. 

And that – “ever-changing” – is my biggest hurdle. It’s hard to call my illness chronic when it seems to jump from one new acute problem to another, the blindsiding surprises causing a constant low-level wary vigilance that takes an incredibly heavy toll on us both, as we try to anticipate any crisis and create contingency plans for every situation.

Looking back on the last 7 months, two big things stand out – one good, one bad.

The good is that I finally figured out, after many months of scary bowel, blood pressure and heart issues that were truly life-altering for both of us (I couldn’t be left alone in the mornings), that I had developed reactions to Miralax (polyethylene glycol, like Macrogol), which had been my safe bowel medication for years (I’ve failed all of the prescriptions I’ve tried thus far).

It took an elimination of almost every single other thing I ingest to figure it out. I was scared to eat or drink anything, not knowing if, many hours later, all hell would break loose and cause hemodynamic instability that needed medical attention. But it was the Miralax, which I would take halfway through each night’s sleep. 

Since stopping Miralax, I have had fewer than 5 serious bowel episodes that caused pelvic floor spasms. They had been happening daily and I can’t overstate the difference in my quality of life without this constant threat and fear. 

My first endocrinologist told me: “With new symptoms, always look first at the medications you take.” Over and over, I have failed to remember this advice, like a few years ago when I was in an extreme hyperthyroid state – breathless, palpitations, not sleeping, thinking I’d developed anxiety because of the “beehive in my chest” – and it took 3 months before it dawned on me that it was from increasing my thyroid medication. Those symptoms were gone immediately after I stopped taking (temporarily) those hormones.

So, take heed, friends: never assume a medication is benign just because you’ve taken it for ages with no issues.

The bad standout thing from the last 7 months is a massive increase in EDS instability problems. I have a few areas of my body that have been “going out” repeatedly for years, but usually resolve after a few days or weeks: my neck, right clavicle, right thumb and one spot in my thoracic spine. But, back in December, I developed back-hip-SI joint instability that caused repeated injuries with normal, innocuous movements, like sitting up in bed.

I got hip x-rays and a lumbar MRI, which didn’t show the herniated disc that the doctor suspected. I started physical therapy, but a sneeze one night in February, and daring to sit on the loo the next morning caused an injury that landed me in the emergency room.

Something ripped (my physical therapist thinks a ligament or tendon attached to my sacrum) and I couldn’t get off the floor and I couldn’t empty my bladder.

The EMT that came to my house was married to a woman with EDS and he understood everything that was happening to me, which was incredibly reassuring, as the back pain in that moment made it difficult to explain that no “event” or accident had caused the injury and that my blood pressure is normally this low and that I didn’t want any medications because of reactions and please be extra careful when transferring me from clamshell to gurney to bed etc.

In the ER, I wound up having to create a huge fuss, begging for/demanding a catheter because “I am going to jam this pen into my abdomen to drain my bladder.” The pain eclipsed anything I’ve felt before. Bless the EMTs again – they could have left me in the hospital hallway, but they insisted I get a room before they left. It took 2 nurses way too long to get a foley catheter in, while holding me down with my legs splayed back, making my hip/back injury much worse, but, eventually they were able to drain 1200 ml of urine, noting that it is normal to feel the need to pee with about 150-200 ml.

The rest of the hospital visit was a debacle. They ordered another, redundant lumbar MRI, even though I repeatedly asked for a hip/SI joint MRI. They ordered a kidney CT scan, even though I asked for an ultrasound (so much radiation in my life!). They didn’t secure the bladder catheter to my leg and the balloon got pulled out accidentally, which is one of the worst pains I’ve ever felt. They told me my bloodwork looked fine, even though my potassium was too low. They couldn’t get my IV line to stop filling with blood (the doctor actually came in, said, “that’s weird,” clamped it off and left (?!), so I unclamped it and got medical tape out of my bag to secure it myself in a way that allowed the saline to flow). And they gave me fentanyl, which didn’t touch the back pain, but caused one of the worst migraines of my life that went on for days and I thought I might have to go back to the ER to break that pain!

But, worst of all, I couldn’t be in a seated position in the week afterwards and bearing down slightly (which I have to do even to pass gas and urine) flared the pain in my flank, so I couldn’t do my daily enemas, which meant I couldn’t eat. It became very obvious that something like this – a different structural issue – would probably be the thing that pushes me into bowel surgery. 

[An aside: I understand that this downhill progression in mobility and upward trajectory in pain is probably due to my tethered spinal cord, for which I have so far refused surgery, but, no, I’m still not convinced that that operation would make things better. I will write about this some other time.]

During this time, I had an urgent appointment with Dr. McDreamy, my colorectal surgeon (who really is dreamy) to figure out next steps in case the injury remained bad enough that I couldn’t eat/poop normally (ha – as in, my “normal”) for an extended period of time. He said that he would be available whenever I was ready and we could do a colostomy bag. Music to my ears. The most reassuring discussion I’ve ever had with a doctor. He said I could try botox in my anal sphincter and a sacral nerve stimulator first, if I wanted (though, they won’t fix my prolapse issues, so they are not long-term answers). 

I haven’t made those appointments yet. As usual, I don’t want to rock this shitty, broken, leaky boat in case it makes things even worse. I’ve been concentrating on the most pressing concern, which is trying to heal this injury, but it’s discouragingly stubborn and I have no medications to help, aside from CBD balm and lidocaine patches. It’s a moving target, from left hip to SI joint to lumbar spine to thoracic paraspinal muscles and back again.

I have to be aware of every change in position: I am wearing an SI belt and lumbar brace all day, but sitting, turning, reaching, and bending can all cause re-injury. I have 4 physical therapists(!) and I’m doing daily exercises, but they are tiny, basic isometric movements that always cause worsening pain, so it’s slow-going.

I’m disabled to the point that I haven’t gone for a scooter dog walk in 8 weeks and I’ve also had massively increased exhaustion and weakness in the last month, so everything feels impossible. Getting out of the house with Penny is the only thing that keeps me sane and this has really taken a toll. But, even if my normal energy returns, my big mobility scooter with good suspension is just too jostley on a body held together with jello and rebar. 

In a post 15 months ago, I said my plan of action consisted of:

  • Getting calories in and out (I’m doing an adequate job of calories, but not nutrition. I’m eating about a third of my protein needs and not nearly enough vegetables for my vitamin requirements)
  • Keep trying bowel medications (I’m currently managing with only Mag O7)
  • Pelvic floor physical therapy (I’m now in regular and counterstrain PT)
  • Get a “small bowel follow through” study (didn’t do)
  • Get a mesenteric CT angiogram (didn’t do)
  • Talk to my surgeon about a stoma (done!)
  • Trial a fentanyl patch (done!)

My updated plan is:

  • Better nutrition
  • Get immunoglobulin infusions back
  • Trial painkillers, muscle relaxants, bowel meds and more
  • Religiously do PT exercises
  • Get a small bowel follow through
  • Try anal sphincter botox
  • Come to terms with the fact that I can’t put off bowel and spinal surgery forever. And we’re not getting any younger.
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On The Road Again!

I haven’t had spare anything (time, energy, bandwidth) in about 20 weeks. Wow, 5 months — for this long, it’s been one event after another. And January through March was a haze of bare-survival after my husband’s knee replacement surgery and the resulting toll on my body. (His body bounced back pretty well, thankfully.)

In April, before our trip to Joshua Tree, it was weeks of careful packing, plus dealing with repeated dental visits (a successful amalgam removal and crown placement sans anesthetic).

We were gone almost 6 weeks for the California trip and it was not a good time for various reasons that I will one day write about. Mostly hell from constant colon crises.

Right after we got home, my Mum came to visit from Ireland for 6 weeks and I tried to focus all of my extra energy on her. It was wonderful and sorely needed.

As soon as she left, my Dad came to visit — also wonderful and sorely needed, as I hadn’t seen him in the flesh in 4 years — but it was more taxing than I anticipated because it was only 5 days, so, in order to see him and my sister as much as I wanted to, I had to rearrange my usual careful schedule.

Then, as soon as he left, we started packing to get back on the road again to Salt Lake City to see Dr. Pace and Dr. Maitland.

This is why my lengthy to-try list of medications never happens. This is why I haven’t gotten IVIG infusions back on schedule. There hasn’t been any time in the last 5 months that I wanted to risk terrible side effects.In Joshua Tree, even though I brought a suitcase of supplies, thinking it’d be an ideal time to try things and get infusions back because my husband was there with me every day, he explicitly asked me not to do anything different that could make things worse. It was a bad month.

Friends may remember that back in June we were in Vegas in 110° heat, on the way to the Metrodora institute in Utah and, due to unstable health issues, we decided to turn around and go back the way we came to get home to Seattle as quickly as possible. I loved arriving home. I NEVER love arriving home. Seattle has been wonderful this summer, especially after the desert magic was so diminished for me this year.

So, now we’re again on the road to the Metrodora institute and, the upside of everything is, back in June, we were going to be paying out of pocket to see Dr. Laura Pace (a neurogastroenterologist who *might* be able to help with my complex bowel-dysautonomia issues – see here and here), but between then and now, they’ve joined my insurance and all of the providers at the clinic are covered, so I’m seeing Dr. Anne Maitland, too (a mast cell specialist – see here and here) and anyone else they want to throw my way.

I am keeping my expectations low for these appointments because I’ve heard some not-great stories (mainly due to disorganization), but I’m also trying to keep an open mind. It’s much easier to do this knowing I won’t be paying thousands of dollars.

Our 25th anniversary is in a few weeks, so we’re trying to pretend that it doesn’t matter if the doctors are a crushing disappointment because we’re on a celebratory holiday. Woohoo fun!… Even though I’m nervous of what the SLC elevation might do to me + it’s currently 100° out + it’s all work and no play for my husband + I can barely walk + Penny has an abscessed carnassial molar and is on meds to get her through to surgery…
But ANNIVERSARY ROAD TRIP! 🥳🥳🥳

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Uncharted Waters

I’ve reached a new physical low and I don’t have any idea of the best direction to go from here. When it comes to my health, I don’t do well emotionally without a plan of action. I can only rest easy when I have prepared for all eventualities to the best of my ability, whether it is going for a drive in the car or thinking about major surgery. As an example, I made a will when I was 37 and, in 2011, I asked my boyfriend of 13 years to marry me so he could legally speak for me if I couldn’t speak for myself. That’s a romantic proposal.

But I’m currently blinded by pain, fear and exhaustion from constant interrupted sleep. I’ve been working so hard this past year to get the appropriate testing of my GI tract, do the hail mary treatments and line up willing surgeons for the operation that is undoubtedly needed, but… a decade into this shite, I am finally at that stage where doctors look at my records and show me the door.

One doctor said right off the bat: “Do you know what scares surgeons the most? EDS [Ehlers-Danlos Syndrome]. Do you know what scares us the second most? MCAS [Mast Cell Activation Syndrome].” She sent me on my way, unwilling to even discuss what was happening anatomically.

I had another surgeon walk into the exam room and, without any greeting whatsoever and without even looking at me, she said, “Well, you certainly are obsessed with your bowels, aren’t you?” Immediately after that, she asked for the name of my therapist. There was more and worse derogatory interactions with her, but I’d like to not get riled up tonight. My great therapist said, “You encountered one of life’s assholes, this one just happened to be a doctor.”

I’ve decided any surgeon who is a) compassionate and b) willing to help people with connective tissue disorders and idiopathic anaphylactic reactions is a bloody hero. I had no idea I would become a medical pariah. 

There was a time when I was trying to fix my immune deficiency, my hypotension, my hypothyroidism, my chronic migraines, flu-like symptoms, reactivity etc… It’s all still there and I’d give anything to “just” be dealing with those. I’d have some semblance of life; I’d not be in crisis every day. Sick, yes, but not crisis. I was once chronically, predictably, stably ill and it feels a bit perverse to long for that life again.

Five years ago, I started to have intermittent pelvic floor spasms. Some were very obviously bowel spasms and some were the muscles/ligaments of the entire pelvic floor. The latter were more severe and often triggered a vasovagal collapse where my blood pressure and heart rate would plummet and my husband would call the paramedics. I stopped a lot of things to try to control these. I stopped having periods, I stopped taking stimulant laxatives, I stopped holding my pee, I stopped eating “FODMAP” foods that can generate gas, I stopped having sex.

A year ago, the spasms started to escalate. I had one single episode in January, 2022 and, this week, I have had at least one a day, six days in a row. They are SEVERE. I never use that word lightly. They are agony and I never know how long they will last and whether they will trigger a cascade into hemodynamic instability or loss of consciousness. 

screenshot of my calendar this week

The spasms usually start in the mornings when I stand up (or sometimes just sit up in bed) and my intestines drop towards the ground, along with my bladder and uterus (which have also fallen from their normal positions). The weight and pressure this causes on my pelvic floor kicks off a spasm and it is greatly exacerbated by stool or gas in my colon, so eating anything has become scary. Terrifying, actually. Even drinking causes problems — I recently realised that my electrolyte water (TriOral rehydration solution), which I’ve been drinking day and night for two years, was causing a lot of gas production.

Gas causes more issues than anything because it gets trapped in the folds and herniations and the pressure increases to unbearable levels. The pain is blinding. And by “gas,” I really mean air because there is absolutely no smell at all. It is as if someone inserted a hose into my transverse colon and pumped it up. I’ve been eating a very limited diet for years – there really is nothing more I can cut out to try to manage gas production. It’s there even if I fast and it’s made worse by the medications I have to take for constipation like Miralax, Linzess and magnesium oxide. It’s there because I probably have SIBO, too (small intestinal bacterial overgrowth — I’ve treated it many times, but I’m not sure if it’s worked).

My small bowel is “lying on the bottom of your pelvic floor, like a hose curled in the bottom of a bucket,” one surgeon told me. “Nothing is connected to the sacrum anymore — you have complete internal rectal prolapse with intussusception of the rectosigmoid.” He drew a picture of this “intussusception” – the intestine looks like a sleeve telescoping into itself when you pull your arm out. 

My umbrella diagnosis is visceroptosis – abdominal organs falling and prolapsing out of their normal positions – but there are different names depending on which organ has the issue. The ones I have are:

Enterocele: where the small intestine falls between the vaginal wall and rectum (which should be fused, but, in my case, are separated).

Sigmoidocele: essentially the same as an enterocele, only the sigmoid colon has dropped.

Rectocele: the rectum herniates forward and into the vaginal wall.

Cystocele: bladder falls. 

Uterine prolapse: uterus falls.

Perineal descent: perineum bulges downward. 

Intussusception: telescoping intestine. 

The two most profound issues I have on the MRIs are the enterocele and rectocele, above. So, as you can imagine, with both of those bagged-out intestines holding onto poop and gas, prolapsed into the same low-down space, as well as my uterus and bladder sagging, it is an unbelievable amount of pressure on my pelvic floor. The only thing that has helped marginally is lying on an adjustable bed with my head as low as possible and my legs elevated, which allows gravity to shift my intestines back towards my chest. I do this over and over throughout the day to reverse the downward pull – after standing in the kitchen, after sitting at the table or on the toilet. But it doesn’t last long and just moves the pressure under my diaphragm.

Recently, though, things have escalated. Cramps wake me up all night long and, during the day, I can hardly be upright without my pelvic floor going into a spasm. I quickly get on the floor, put cushions under my bum and moan, pant, cry, try to focus all of my attention on relaxing those muscles and easing the weight on the ligaments, while begging the universe to make it stop and vowing to never let anything pass my lips again. I’m hungry all the time.

I can’t keep doing this every day. I don’t want surgery, but I can’t avoid it. I don’t want mesh implanted in my abdomen (my immunologist says absolutely no mesh in someone with a mast cell disease), but I’m told the only other option is a colostomy bag. I’m told whatever I do will probably fail (even the bag) because I will still have slow-transit constipation and shitty connective tissue. I’m told that the spasms might not stop. That thought is very overwhelming. The spasms are more life-altering that anything else. What would the future look like trying to avoid them? Pooping into a bag, but nil by mouth? IV hydration and parenteral nutrition? I need doctors to think outside the box about how to stop the symptom escalation. Botox? Prolotherapy? Nerve stimulators? Nerve blocks? Am I going to have to live mostly on my back to stop my organs from falling?

There are Facebook support groups for everything you can think of, but not for visceroptosis caused by EDS. I’ve met two people in a similar boat. (One of them shared photos of herself in the morning and at night showing how her displaced intestines caused her abdomen to balloon out over the course of the day. I did the same thing yesterday and it is striking, but, although I share everything about myself here, I’m not sure I want to post photos of myself in my knickers. Sorry to disappoint.)

There are no doctors who really understand this particular problem and there is an incredible paucity of studies. A prolapse specialist said to me: “I wouldn’t refer you to any of the colorectal surgeons I know. They really only do routine tumor excisions or coloectomies for things like bowel cancer.”

Oh. I see. What an education I’m getting! Silly me thought that any colorectal surgeon could fix this. My problems are “higher up,” not a “simple” rectocele repair. My issues are from connective tissue laxity, not from pregnancy trauma. Not only do most colorectal surgeons not have the expertise and experience, most wouldn’t take me on, anyway, because they and their hospitals like success – they don’t want to take chances on patients with high rates of complications and surgical failures. These are uncharted waters.

My only plan of action right now is:

  • continue doing everything I can to get calories in and out.
  • keep trying and retrying bowel medications.
  • continue pelvic floor physical therapy (which feels useless – breathing exercises, bowel massages, visualisation).
  • get a “small bowel follow through” study (which is proving to be complicated – shocker! – because they don’t usually do upright x-rays and they don’t have any barium drinks that look okay for me to ingest).
  • get a mesenteric CT angiogram (which I’ve been putting off for a few years because I had a small reaction to the IV contrast last time and I’m nervous it’ll be a bigger reaction this time).
  • talk to my surgeon about a stoma (for a colostomy bag) since he won’t entertain the notion of a repair surgery without mesh.
  • trial a fentanyl patch in-office with my immunologist and then other medications to develop a post-op painkiller plan (how on earth do I get the nerve to do this??).
  • do I continue looking for other surgeons who have experience with these more complicated, “higher up” repairs and who won’t use mesh?? It’s exhausting and I really don’t think that doctor exists.

But none of that feels possible when I’m so depleted. My husband had a total knee replacement surgery a few weeks ago and the extra walking, driving and chores I was doing made it very evident that my tethered spinal cord is a major player, even though I don’t want to face it. When I’m pushed outside of my energy envelope and normal step count, I have great difficulty walking. I’m hoping now that he has turned a corner with his recovery, I’ll be able to gain some ground and see a clearer path forward.

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Bowel Answers Finally

I finally had most of the colon testing done that I needed and I think I will put off or ignore the other proposed GI tests because I have one big anatomical* problem that is at the root of all of my bowel troubles and it needs surgical intervention.

*My surgeon always says ‘anatomic,’ but the internet tells me there’s no difference between anatomical and anatomic, so I’m sticking with the former (unless I need to write about ‘an anatomic bomb’ because that just sounds cooler). 

This week, I had four back-to-back appointments at the hospital and – for anyone in my area – every aspect of this visit to Virginia Mason was so much better than the last decade of overall experiences at Swedish or the University of Washington. Everything was streamlined and on time (even when I was late to my first appointment–total calm kindness greeted me); every clinic was in the same building; everyone I asked to wear an N95 did so with no issues; nobody read my lengthy records and insinuated anything negative or dismissed me for being too complicated or too young; everyone knew what Ehlers-Danlos was and they weren’t scared of it; and, most importantly, everyone was kind and didn’t rush me and answered all my questions. 

I had a barium fluoroscopic defecography, an anorectal manometry and internal exams plus consults with both the colorectal and urogynaecological surgeons, separately. 

The results from these appointments, along with the info gathered from my own daily hellish routine and other testing from this past year (colonoscopy, MRI defecography, abdominal CT, two mesenteric vascular duplex ultrasounds (one with breathing protocols), Genova Diagnostics stool test, urodynamic (bladder) testing, pessary trial, splinting trial, pelvic floor physical therapy, exams by a prolapse specialist and another colorectal surgeon, diet changes) have finally given us a very good idea of what is happening. And–shocker!–fiber, papaya and probiotics aren’t the answers to my problems. Neither are Miralax, Linzess, Motegrity and all the other medications that have been thrown at me (although, I’ll have to continue to use them for life). 

In the interest of transparency, education and destigmatization, I’d like to tell you exactly what I go through every day in order to defecate, but I’m going to save that for another time (you’re welcome). Instead, I’m going to tell you exactly what is happening to me anatomically because everyone has an asshole, so this shouldn’t be taboo.

My big, bad problem is an enterocele. An enterocele is a prolapse or hernia of the small intestine. The back of the vaginal wall and the front of the rectal wall should be fused, but mine have separated and my intestines–probably the small bowel, but it’s not quite clear which part–have dropped into that space, obstructing defecation. I also have a severe rectocele and less important sigmoidocele, cystocele and uterine prolapse. Everything is collapsing and falling. I also have intussusception of the rectum, which means it is telescoping into itself when I bear down. If you google this, it says it is very rare and life-threatening, but mine is happening every day, whenever I have a bowel movement and, so far, hasn’t caused a complete blockage and hasn’t telescoped itself outside my body. However, that is the normal course of things – it is probable that one day my small bowel will fall out of my anus and that will be a surgical emergency. Or my uterus or bladder might fall out of my vagina. These things have been happening to women forever and no one talks about it. Thank dog for online support groups – I can’t hate facebook when it has connected me to others going through this craziness.

Prolapses, both internal and external, are almost always caused by childbirth – usually in women who have had multiple pregnancies. Mine is caused by the poop babies I’ve carried around my whole life (chronic constipation) coupled with Ehlers-Danlos Syndrome (EDS), a disorder which affects the integrity and strength of the connective tissue throughout the body. In other words, the walls of my intestines aren’t strong, so they herniated under the pressure of poop. My pelvic muscles are affected, too, and have become lax and atrophied, so they’re not holding anything up and in place. 

I have found all of my testing incredibly fascinating (although, brutal because of the way my body pays me back for any intervention). The other day, I got to watch the barium defecography on the screen–I got to see how my anatomy moved as I pooped and ask the radiologist questions in real time. There was a huge pouch of barium mash (they literally squirt instant mashed potatoes up your bum) that descended anteriorly across the base of my pelvic floor when I bore down. I asked if it was my rectum and the doctor said no, it wasn’t meant to be there at all – that was the rectocele (or maybe enterocele–I need to get the imaging disc and look more closely). I couldn’t believe it – I wish I’d had the foresight to take a photo. It was upright and vertical and, when I pushed, it just splooged forward into a horizontal cavity that shouldn’t be there. By “it” I mean my intestine, which was outlined by the barium.

I told the tech that if she ever had another nervous patient (I had never had barium before and didn’t know if my body would react to it), tell them they get to watch the x-rays and talk to the radiologist as the test is performed. I would have been sprinting into the exam room, if I had known that!

The surgeon said this is not a functional problem with digestion and motility and there was no point in doing a Sitz Marker Study (or Smart Pill) because it would give false information. It measures how long it takes for markers to move through your GI tract, but they wouldn’t move due to the enterocele and rectocele clogging up the pass, rather than a problem with the migrating motor complex.

He said an upper barium study and/or endoscopy (my GI doctor wanted both) might be warranted if I had reflux or problems with swallowing, but I don’t. I was diagnosed with “gastroparesis” (food won’t leave my stomach), but it’s only a problem when my bowels are backed up. He thought I might have a hiatal hernia because they often go along with pelvic prolapses, but since the symptoms aren’t bad, he’s not concerned.

The other tests that I found fascinating and informative were:

  • The anorectal manometry was mostly normal: No evidence of nerve damage or Hirschsprung’s disease. No dyssynergia, meaning the muscles work as they should and in the correct order (rectum contracts first and then the anus relaxes). No pain, pressures were normal and sensations were okay-ish. The two abnormal findings were my anal sphincter is tight (cue the tightass jokes) and it took quite a high volume (a balloon being inflated inside my rectum) for me to have the urge to defecate. This could be because I’ve become desensitized from daily large-volume enemas for so many years or it could be because the rectocele creates more space in that area for the balloon to expand into. 
  • The abdominal CT, which showed an “enormous bladder,” four times the size it should be, and a distended sigmoid colon that was “pushed up the wazoo” (quotes by my neurosurgeon, Bolo, which his other patients will appreciate). 
  • The stool test, which I assumed would show dysbiosis, infections and metabolic imbalances, but it didn’t. It was pretty okay. 
  • The Duplex ultrasound that showed my duodenum was being compressed, which could explain the pain in a certain high-up place after I eat.
  • The colonoscopy, which showed the inside of my large bowel is lovely, with no issues. I’ve had constipation my whole life, my mother and aunt have diverticulosis and my grandmother had bowel cancer, so I assumed I’d have something wrong, but she didn’t even find a polyp. Even more interesting to me was that my cleanout in preparation for the colonoscopy was easy. I didn’t even need the second prep. I assumed I had loads of backed up stool. I thought I’d be one of those horror stories: It was found during the autopsy that she had 10 pounds of fermenting meat in her gut!

The main issue found during the colonoscopy was that it was difficult to get the scope around the bends in my colon, even using a pediatric scope, which means it is difficult for poop to get around the bends, too. My GI doctor said things were tweaked and compressed within my abdominal cavity because I am such a small person and it’s further complicated by not having a lot of fat around my organs – my colon bends at acute angles rather than soft curves because there isn’t enough fat to act as a buffer to smooth out the turns.

This is exactly how it feels, subjectively, when I’m sitting on the loo. I know I said I’d spare you this part, but one of the things I have to do is squeeze my intestines with my hands like a toothpaste tube. I have to physically push stool around those sharp angles with my fingers. 

The lack of organ fat contributes to the issue they saw on the ultrasound, too. My duodenum is compressed in between vasculature (the aorta and the superior mesenteric artery*) and the first-line treatment is to gain weight to try to bulk up the mesenteric fat pad, which will help separate the arteries enough that food can pass freely through the duodenum. 

*This is called Superior Mesenteric Artery Syndrome (SMAS), which can be very serious and life-threatening when it lets nothing pass (mine isn’t, thankfully, and we’re not even sure if the measurement was accurate, so this may not be my diagnosis). It can occur with other abdominal compression conditions, such as Median Arcuate Ligament Syndrome, Nutcracker Syndrome or May Thurner Syndrome, and seems to be more prevalent in people with EDS, mast cell disease and dysautonomia.

I didn’t believe my EDS diagnosis for years. I’m not the typical bendy person who did body-contortion party tricks as a kid and suffered subluxations my whole life. After being schooled by numerous doctors, I now believe this might be the root of all of my issues. My rheumatologist showed me what my joints aren’t meant to do, my physical therapist told me I had the most hypermobile neck she’d ever worked on, my neurosurgeon explained how a tethered spinal cord, which happens more often with connective tissue disorders, is damaging nerves and pulling my brainstem down, blocking the normal flow of cerebral spinal fluid. And, the other day in the hospital, every doctor I spoke with said, yes, the reason for all of my poop woes (and digestive, bladder and uterine woes) is shitty connective tissue that has weakened the walls of everything: fascia, vasculature, intestines. 

The bad news is these conditions will only get worse and the only thing that will help is major surgery. The colorectal and urogynecological surgeons perform the operation together to fix the prolapses, lift everything back where it should be, suture organs to the tailbone and add mesh, so your body creates scar tissue to strengthen the vaginal and rectal walls.

The really bad news is that I can’t risk mesh. Not just because of the scary outcomes you hear about in “normal” people (those mesh class action lawsuits advertised on TV), but because my immunologist warned unequivocally that with mast cell activation, my body would react to and reject the mesh, causing complications. Not to mention the much higher probability of surgical failure because of EDS – my tissues would probably just sag and drop again, sutures would fall out. I’d have to be careful with coughing, sneezing, bearing down, lifting even a light amount of weight for evermore and there would be repeated repair surgeries. I can’t imagine a life where I don’t bear down. I even have to push to pee lots of the time.

And it’s not like I would miraculously be able to have normal, formed stools and easy bowel movements. I will still be taking daily medications, I will still have the acute intestinal angles and will I be able to toothpaste tube-squeeze my guts after surgery or will that be too risky? It’s a scary proposition.

The colorectal surgeon said, without mesh, it would be a “placebo surgery.” He said there was absolutely no point. I asked about the ACE surgery that allows you to flush water from a stoma created by your appendix – kind of like an enema from above – and he said it wouldn’t help because the enterocele would still be there.

I asked about a sacral nerve stimulator and he said they are usually used for nerve damage that causes incontinence issues, which I don’t have. He said the only real alternative to a mesh repair is a colectomy (with a colostomy bag). He said he wouldn’t do an ileostomy, but I can’t remember why. My immunologist suggested the same thing – go straight for the ostomy.

Mind you, removing my colon and getting a bag — even if I had a perfect surgical experience and recovery — won’t make all of my problems go away. There might still be complications with the mesh they put around the stoma to try to reduce the likelihood of a parastomal hernia (which is quite common). And I would probably have issues with scar tissue and reactions to the adhesive coverings and deodorant smells and bag emptying (which involves body positions that my spine doesn’t like to do), and your rectum still generates mucus after you get a stoma and there is leakage, both rectal and around the bag etc etc… It can still involve hours in the loo and pain and regular medical interventions. It is definitely not a cure-all or an easy road. You can’t believe the difficulties until you read patient stories. Some people’s quality of life is drastically improved – they can travel and exercise again, for example – but, of course, this wouldn’t be my case because ME/cfs is still very much the specter that keeps me mostly housebound. 

So, how long can I limp along like this and when will I have to get surgery? My doctor said, one day my small intestine will probably prolapse out of my body and I will have no choice. It could be in 7 days or 7 years, he couldn’t predict. And my daily interventions will eventually not work – more medications, more enemas with larger volumes of water, more pain, more dietary restrictions.

My quality of life from this one issue is very compromised, though my fear of surgery overrides this. I think I could probably manage to continue with my current routine for years if it weren’t for one thing: The vasovagal collapses. 

Those who know me know that these have been happening for 20 years. They started with dysmenorrhea (period pain would trigger prolonged blood pressure drops and my body would go into a sort of shock), then they started happening with bowel pain. I controlled these (or tried to) by taking nightly progesterone, so I never menstruate, and not taking any laxatives that cause cramping, like Senna or Dulcolax.

Then they started happening with pelvic floor spasms – once while using a vibrating device on my lower abdomen to try to stimulate stool/gas movement and twice from orgasms. I can stop doing those things, too (not happily), but, recently, my pelvic muscles go into spasm for no reason other than gravity dragging them down or pressure building up in my colon.

It’s not the spasms that are the problem – I can handle quite a bit of pain – it’s the subsequent collapses. They aren’t “faints” – I don’t recover by lying down and getting oxygen to my brain. In fact, they often start in the morning, after I’ve been lying down all night. My husband describes them as “catastrophic system failures.” Pale pallor, cold sweat, breathing difficulty, tunneled vision, unable to speak, bradycardia, prolonged hypotension, sometimes loss of consciousness, and often a call to 911. When an abdominal spasm occurs, if it goes on long enough and is painful enough, I will feel my body start to shake and then all the other symptoms encroach. I usually call my husband and have him on the phone until we know whether it will stop or if I will fall off the edge and need medical intervention (paramedics can’t do much besides make sure I recover, check my heart, give me IV fluids). 

I’m trying to describe this clearly, without hyperbole, but these episodes are terrifying. They are unpredictable and it feels like I’m going to die because I’m so weak and my blood pressure is so low and I can’t get a breath and everything is fading out… My specialist says it feels that way because, physiologically, it is the closest I come to death. Yikes.

Point being, if I thought these collapses could be curtailed by excising my colon, then I would be much more eager for the surgery. But nobody has ever heard of this issue. Is it vagus nerve damage? Part of dysautonomia? To do with spinal cord and brain stem? These episodes are still more frequent with hormonal fluctuations, so I’m praying that after menopause they will get better.

Maybe that’s my decision. Maybe I can hold out until after menopause – hope my organs stay inside my body and don’t rupture, hope I can keep getting food in and out with all of my exhausting interventions – and see what gets better and what gets worse after menstruation isn’t playing a part. Maybe then I’ll be able to stop taking progesterone, which might help the overall picture because it can cause constipation (in some people I’ve talked to, it paralyses the gut or stops motility entirely). I’m currently not willing to come off it and risk the angry mast cells that come with periods.

I held out a smidgen of hope that having my tethered spinal cord fixed would also solve my bowel problems and everything would get better, but it looks like there’s no getting out of this one and I have to face multiple future surgeries. Time to find some safe painkillers that don’t cause reactions! 

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Damn Your Eyes

A note about my eye exam today:

I need to update my blog more often — for myself, more than anyone else. This morning, I had my first eye exam in 7 years and I looked back here to see if I’d said anything about the last time and saw I’d had a reaction to the yellow eye drops. I had no memory of this and it saved me from possibly having another reaction today for a not-really-needed test. So, I’m going to make a note about today’s experience to remind myself in the future not to bother going to an ophthalmologist again!

My vision was 20/15 the last time I had my eyes checked. I never needed glasses at all until July of 2020, when I realised I couldn’t see my vein very well when putting in a peripheral IV catheter to do IVIG. Since then–not even 2 years–I’ve gone up 3 magnification strengths and my right eye has significantly worse vision than the left. My distance vision is a little worse, too, which I realised when my husband (who is 12 years older than I am) could make out signs on the highway that I couldn’t.

July, 2020: The first day of the rest of my farsighted life: I had to borrow my husband’s glasses to put in my IV.

Of course, this is totally normal for people my age, but my brother started to need glasses after spending time on a submarine and had a theory that it was because he didn’t focus on anything more than about 8 feet away for so many months. I think this might hold some truth for those of us who spend so little time outside, looking at horizons and focusing on long distances.

I have a ton of eye symptoms, which bother me much more than the need for readers, but I’ve been told there is no remedy for any of it (except dry eyes). I was told in the past: “When your overall health is poor, your eye health is poor, too.” The ophthalmologist today actually said that my eye health was good, but my symptoms are probably neurological (migraines, dysautonomia etc).

Some of my eye symptoms are: floaters; blurriness; pain in orbital muscles when moving my eyes; itchy eyelashes; dry eye to the point that when I blink in the mornings, my tears feel like acid hitting my eyeballs; right eyelid spasms; light sensitivity; trouble tracking when scrolling on my phone or reading blocks of text; migraines that feel like my right eye is going to explode out of its socket (it’s silly, but I often push my eye back in with the palm of my hand because it really feels like it might pop out). I used to say that my eyes felt like I’d put grit into Vaseline and then smeared it over my eyes.

I have had radioiodine ablation on my thyroid for Graves disease (or some sort of autoimmune presentation of hyperthyroidism–endocrinologists have disagreed on whether it’s actually Graves) and was told to pay attention to eye symptoms, but the doctor today didn’t see any evidence of that being a problem. I am also positive for one of the Sjogren’s markers (carbonic anhydrase VI IgG antibodies), which ophthalmologists treat no differently than dry eye. He rattled off a bunch of things that I’ve tried in the past that have done nothing (eye drops, eye scrubs) and some I haven’t (take omega 3s, humidifier in bedroom).

He said my optic nerves, blood vessels, maculae etc were all normal. During the visual acuity test, none of the letters were sharp, but I could guess most, even though they were blurry. The doctor said my vision was considered 20/20 because I could guess 3 out of 5 of the bottom line, adding he wouldn’t be able to see any of them at all. This is the thing when you’ve had great vision your whole life–you don’t really know what is “normal” or acceptable.” I hate not being able to see the letters clearly, but being able to guess them at all, even though I was straining and it was very blurry, is still considered 20/20.

They said I am doing no damage to my eyes using the cheapo 3-pack of Costco reading glasses. It doesn’t matter what strength I use or whether I use different magnifications (I have various glasses scattered in different rooms) or whether I’m using the lenses that work for one eye, but not the other. Essentially, if you can move the phone/book closer or further away and make it work, then everything’s fine. They said I could get prescription glasses, but, if I’m getting by, there is no need. I don’t want any more energy expenditure on anything, so I’m sticking with what I’m doing. My husband was mildly alarmed by this: But if you can get prescription glasses that correct for the differences in your eyes and it makes your life better, why not? Energy is why not. It all comes down to having nothing extra to give.

For anyone with MCAS, I have had the yellow eye drops 3 times and, the last time, I had a reaction. A friend told me I could ask for a retinal scan in lieu of dilation, which I did and it was easy and only took a few minutes. Once in the past, I was told that one of my eyes had high pressure (intraocular fluid pressure) and this can be a warning for glaucoma and I should keep an eye on it (I just caught that totally unintended pun when I did a reread 🙂 ). The gold standard for checking eye pressure is the use of yellow numbing eye drops, which are used with a slit lamp and blue light. A step down from that is a handheld tonometer, which still needs numbing drops, but they don’t have the yellow dye. Lastly, there is a device called an Icare, which doesn’t need drops at all. The latter would have been my preference since I don’t know what caused the previous reaction (the dye, the numbing medication or the preservatives), but they didn’t have one at the location where I had my appointment today, so I decided to skip the pressure check, thinking/hoping I probably don’t have a problem.

Lastly, I want to note that this was BY FAR the most risky covid exposure I’ve had (or, really, just human germ exposure, in general). The rooms are tiny with no windows or ventilation and the doctor and his assistant both had to get very close to my face. Also, they cancelled my appointment last week because the ophthalmologist was out sick and I wonder — out sick with what? Even a head cold would be a deal-breaker for me. All in all, I regret the appointment because the risk of contracting a virus was very much outweighed by the chances that I have eye problems that can be remedied (I now know). But I didn’t know what was normal or should be treated and I certainly would want to know if intracranial hyper/hypotension was causing any eye damage (as seen on a CINE MRI, I have blocked cerebral spinal fluid flow in my brain because of low-lying cerebellar tonsils, which can cause intracranial pressure issues). I didn’t know if wearing shitty readers could damage my eyesight further (they can’t) and I didn’t know if having worse vision in one eye was a concern (it’s not) and I didn’t know if I could brush off all of my eye symptoms as neurological (I can). Now I know and you do, too. 🙂 Title Credit