Tag Archives: constipation

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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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Halloween Update Litany

I’m going to try to write something. An update of sorts. Not necessarily because today is exactly nine years since this illness stepped into my body and started controlling the trajectory of my life, but more because it is a quiet Saturday and I can’t call any clinics and I don’t have any medical appointments. It’s Halloween, but we’re completely ignoring it this year. It’s a beautiful day, but I woke up after five hours sleep with bad brain symptoms, so I’m not up for going outside or calling a family member or washing my bed clothes, which are in dire need. And I’m just so tapped out on research right now. Endless, endless research into treatments and specialists and ways to bankrupt ourselves on nifty devices that might miraculously give a reprieve from symptoms or plateau my decline in functioning.

I often don’t write — even if I have the time and energy — because I feel like I want to express something meaningful and express it beautifully, or at least express it well. Express it in a way that others might identify with it or even be moved by it. Or, if not meaningful or moving, I’d like to be able to write something informative. But that takes more mental energy and creativity. I always find a reason not to tap into the emotions that are necessary to write deeply and thoughtfully. I stay sane with distraction, coasting along a wave of TV shows and dog cuddles, trying not to look into the depths below. I’m finding distraction harder this year.

After five years of a slow, but fairly steady increase in functioning, I’ve gone downhill. Not because my dog died or because wildfire smoke was choking us for weeks or because I can’t see my family and my one friend who kept me sane by visiting regularly. And not because of the emotional toll of the pandemic and the rage and heartbreak caused by the political strife in the world. That’s all just icing on the distress cake. The actual bulk of my cake is made of pain, exhaustion, reactions, and failing organs and bones, with thin, bitter layers of isolation and future worries between the tiers of sponge. It’s a really unpalatable cake.

When I first met my friend Jak over at Mast Cells & Collagen Behaving Badly, she had been through ME, then she was dealing with mast cell disease and her body had started to have problems from EDS (Ehlers-Danlos Syndrome, a connective tissue disorder). Most people I’ve met with this illness trifecta started having mast cell reactions after ME hit them. I was the other way around. I’d been dealing with angioedema since I was teenager and I first went into anaphylaxis in 2001. ME hit a decade later. When I met Jak, I didn’t have an EDS diagnosis and, when I first got it, I ignored it and decided it wasn’t true. I remembered, though, that Jak had said, “I could have told you that. With some of your symptoms, it seems obvious.” Her pain and subluxations didn’t start in earnest until she was in her 40s and dealing with peri-menapause, so she cautioned me that EDS could raise its head in the future. No, no, I’m not hypermobile, I said and I ignored it. Well, there’s no ignoring it now.

Last year, I went back to the top EDS doctor here in Seattle and told him I hadn’t believed his diagnosis and could we start from scratch, work me up again, see if he truly thought I had EDS? He smiled (good doctor), he agreed (did another physical exam), he reiterated that I had EDS and showed me what my body is not meant to do. He also diagnosed thoracic outlet syndrome (TOS). For all my research, there are so many odd things about my body that I barely notice. It’s like whack-a-mole — I’m just trying to push down whatever the most concerning thing is on any given day. What do I care if I can’t hang my clothes up or hold my phone to my face without losing circulation through my arms and having my hands go numb? It’s really not important in the grand scheme of this illness. You adapt. So when the doctor asked me if I felt anything as he manipulated my arm, I said, “Nothing. Except there’s no blood flowing and I have pins and needles.” He smiled gently again. “That’s not nothing. That’s what I’m looking for.” It took a very long time for blood to come back into my forearm and hand after whatever he did and I had electric zaps for hours. That was a year ago and I still haven’t read about TOS or whether those symptoms are normal or what to do about them. I don’t really care right now because I’m too busy trying to whack bigger, louder moles.

My leg pain has gotten much worse. I can’t stand in the kitchen to cook as long as I could before and, anytime I do, I have to wear compression stockings and a back brace, but still need to go to the couch and lie down with my feet up after a short while, groaning with the effort. My neck and back have gotten worse. Something in my thoracic spine keeps going out and my lower back and tailbone have a constant steady ache. My neck always feels unstable, so I move it very gingerly, but it also always feels like rebar, so I try not to immobilize it. I pull a different shoulder or neck muscle seemingly every day, usually from thrashing around in bed (unfortunately, not in a fun way, not when I’m conscious). I’m currently ignoring a jaw ache and maybe a cracked tooth from clamping in my sleep and the fact that my eyes never stop burning and are sore when I move them. My left patella keeps shifting out of place and it’s agony when it happens, so I’ve been doing a deep-dive into knee braces and kinesiology taping. I broke my wrist and finger this summer when Penny lunged at an off-leash dog and snapped my hand behind my mobility scooter seat. I’m still wearing a cast or a brace a full three months later and my right hand, which picked up the slack when I couldn’t use my left, has developed instability in the wrist and a subluxing thumb. When my endocrinologist heard about my fractures, she said we needed an appointment asap because she is very concerned that my osteoporosis has progressed. She’ll probably suggest I take more drugs. 🙄

And really none of this is that important because it all pales in comparison to my bowel hell. Bowhell.

Warning: lots of talk about poop and toilets ahead. Enemas barely work anymore. To have a bowel movement, I have to use a liter of water and massage my abdomen for sometimes hours each day. And “massage” sounds delicate. It’s not. I often worry I’m going to rupture something with my squeezing. With my broken wrist and finger, I couldn’t manage to press my abdomen properly for weeks and my bowels suffered. I couldn’t evacuate effectively, which meant I couldn’t eat enough and I didn’t sleep properly. Everything has a cascade effect.

Compounding my bathroom issues is how difficult it is to sit on a toilet. I lose circulation in my legs very quickly (even with a Squatty Potty) and it is incredibly painful on my neck and back. My doctor asked me when my neck was the most painful and I realised it is sitting on the toilet because I have no support for my spine and nothing to lean back on. I’ve resorted to using a hard neck brace (only sometimes — sometimes it makes it worse) and putting a chair in front of me to lean my forehead against, but, even so, after I’m done, I have to lie flat on the floor and, if I have the energy, use heat, traction and ice to help the spinal pain. I was never conscious of just how much I need to support my neck until two years ago when my mother was visiting and I’d made enough improvements energy-wise to go to the opera. Wow, three hours sitting in a short-backed chair was excruciating. I was almost in tears. I was dizzy, my heart rate was high, my legs were losing circulation (I’m short, so I was using my backpack as a foot stool) and I could not hold my head up.

So, sitting is an ordeal. And shitting is an ordeal.

I have a long history of vasovagal collapse from abdominal pain. In my twenties, it happened with the onset of my period because of severe dysmenorrhea. Shockingly, when I got sick, my cramps virtually disappeared. But they’re baaacck! And my period often likes to come three times in one month, so this cramping and inflammation, coupled with random pelvic floor spasms, coupled with colon pain has been a lot. Last June, I sat up in bed one morning and some deep part of my lower abdomen spasmed and I immediately went into a vasovagal episode. My heart rate went so low, that I was having trouble breathing. My blood pressure dropped, too, but the main problem was the bradycardia. I was shaking all over and trying not to black out, but after about 20 minutes, I had to call the paramedics. Before they even got here, the pain abruptly ended and, instantly, my heart rate came up and I could breathe again. (I told them not to come inside because of covid and I gave myself IV fluids at home. I’ve dealt with this before, emergency rooms really can’t help.)

From that day forward, every day for six weeks, I was in an acute bowel pain crisis. I couldn’t seem to eat anything that didn’t contribute to the pain across my transverse colon, I lost weight, I wept each evening, I slept poorly, my attention was never not on this organ that was constantly yelling at me that something was wrong. I wound up getting a CT scan (a big deal during covid and when I’ve had so much radiation in my life) and blood tests because I thought: what if this is life-threatening? I was spooked by a fellow EDSer’s emergency surgery for a ruptured bowel and resulting colostomy bag, but I was even more concerned about the possibility of an elemental liquid diet or a feeding tube. I’ve gone to great lengths to keep a varied diet, not only because food is my one joy besides dogs, but also because I know so many people who never got foods back after strict and prolonged eliminations. And feeding tubes — I never want tubes of any sort stuck in my body, too many complications. It’s the reason I’m still doing weekly peripheral IVs after five years, rather than getting a port or PICC (I don’t know anyone else who has come close to tapping veins for this length of time).

The CT scan showed nothing except my big lunch and tampon (a mortifying radiology report: unremarkable, TAMPON, unremarkable, unremarkable, COPIOUS AMOUNT OF INGESTED MATERIAL IN STOMACH, unremarkable etc…) and the acute bowel pain eventually faded back to my regular constant ache with periodic stabbing knives and electric zaps. But it sure got my attention.

I started Motegrity, a selective serotonin type 4 (5-HT4) receptor agonist, which cost $265 for one box (bought online from Canada because my insurance balked) and then caused possibly the worst medication reaction I’ve ever had. I started Linzess, which cost $350 for one bottle and either causes nothing to happen or a full day of sharts. I’m still taking Iberogast, Miralax, BPC-157, SBI Protect, Thorne SF722, oregano oil, berberine, magnesium, digestive enzymes, betaine HCl, and probiotics… all for my bowels. I’m about to try Mestinon, LDN and Cromolyn again (okay, I take it back, the latter was actually the worst medication reaction I’ve ever had — and I’m going to try it again, which has to show my level of desperation); these are all medications that can help motility. Plus, I have a Xifaxan prescription at the ready (which I’ve already taken twice) when I’ve exhausted all of these options.

It’s a next level problem. What I mean is, there were four years in the beginning of this chronic illness when I was “just” dealing with ME and MCAS — when I could still poop! When it was “just” muscle pain, but my joints were fine and my bones felt sturdy. Unbelievably, there were years when I didn’t have brain symptoms. I had the low-level kind of brain fog that made you forget things or not be able to find words, but, in the beginning, I didn’t have the buzzing brain and eye pressure, slurring and screaming tinnitus that makes bed the only possibility, even if my body is feeling strong. These new additions take illness management to the next level.

I’m on my third gastrointestinal doctor. The first said: Miralax, papaya, probiotics. Huh? Did you even hear the part about dead colon? On a return visit, she said: Daily enemas for life. Are you fucking joking? I asked her when she would recommend a colonoscopy (back before I realised it would need anesthesia in my case). When you have bloody diarrhea, she said. Right. Okay.

The second GI doctor said: Colonoscopy and endoscopy. On a return visit, she said: COLONOSCOPY AND ENDOSCOPY. She would not talk about any other tests or interventions. I don’t want to go through that. I don’t think those procedures will show anything and, with my medication reactions, there are legitimate risks to full anesthesia, not to mention the clean out having risks because of my hypotension and hypoglycemia. I thought (and still think) that it was prudent to exhaust less invasive options first.

These two doctors were young women at the University of Washington, one touted as The Motility Expert and the other as being EDS-knowledgeable. I mention this because I would assume I would be most comfortable or have the best experience with them instead of the third GI doctor, who is an old man that made a slightly misogynist comment right out of the gates and doesn’t make much eye contact. But he has been the only one to think outside the box and marginally help me. He ran tests that nobody has ever run since I’ve been sick (I’d never had a stool sample done or celiac test!) and spent 40 minutes discussing my mast cell history before even broaching the subject of my bowels. He dismissed a colonoscopy and it felt like he’d lifted a 100-pound weight from my shoulders because I was crippled with guilt after rescheduling the procedures over and over for a year and a half.

So, because of the progression of bowhell symptoms and structural issues the last few years, I have been pursuing MRIs and neurosurgical consultations. A cine CSF (cerebral spinal fluid) flow study showed a lack of CSF in my hind brain, caused by low-lying cerebellar tonsils (LLCT). My neurosurgeon (who is experienced in dealing with EDS/MCAS/ME patients) also suspected craniocervical instability (CCI), but couldn’t recommend surgery from my MRI measurements and symptoms without first performing more tests (invasive cervical traction (ICT), where they lift up your skull with a pulley system to see if there is an improvement in symptoms, and intracranial pressure monitoring (ICP ), which is a bolt in the skull that holds a probe that measures pressure in your head while concurrently preforming a lumbar puncture). I decided not to do either of those because, as you might have guessed, I don’t like rocking the boat (with, say, a new soap, let alone invasive tests that involve holes in my skull) (oh, and travel across the country) (and covid). But I have wondered if the blocked CSF flow is contributing to or entirely causing my brain symptoms. That’s a big deal. I used to feel smart and effective.

But the biggest deal of all came from the neurosurgeon looking at my pelvic MRI defecography from five years ago (which I didn’t even send to him because I was only consulting him about my neck; he must have gotten it from my specialist, who was the referring physician).

“You have a large bowel,” he said.

“I’m not surprised.”

“And what have you been told about your enormous bladder?”

“My what?” He has a thick Italian accent straight out of central casting and I didn’t know there was anything abnormal about my bladder.

“Your enormous bladder. Your ENORMOUS BLADDER!”

He had to repeat it four times before I could understand what he was saying. It was pretty comical. Nobody had ever mentioned my bladder. He recommended a renal ultrasound to rule out hydronephrosis, urodynamic testing for neurogenic bladder, and a lumbar MRI to look for tethered cord. I’m sure you’ll be shocked to know, I ignored it all… until I watched online presentations by Petra Klinge, probably the top tethered cord specialist in the country, and a Q&A with Dr. Klinge and Jeffrey Greenfield where they mentioned that, although bladder symptoms are the hallmark of pediatric tethered cord, in adults it’s often bowel problems, usually constipation. Ah.

I sent my lumbar MRI (both prone and supine) to my neurosurgeon and he diagnosed “tethered cord, classic variant,” which is notoriously hard to see. In other words, as my complex disease specialist emphasized to me repeatedly, it is rare for this neurosurgeon to diagnose tethered cord before CCI from a lumbar MRI. This is the case even though they have both actively been trying to identify it early since so many of their patients have to return for a second “detethering” surgery after undergoing craniocervical fusion.

“Elizabeth, why aren’t you on a plane to New York for SFT [sectioning of the filum terminale]?” my specialist asked me.

Friends, I DON’T WANT SURGERY. EVER. No surgery, but ESPECIALLY NOT SPINAL SURGERY.

I will leave you there. We have much to discuss. This is now my focus. I need to do everything I can to manage these symptoms and to halt their progression. Meds, exercises, physical therapy, prolotherapy, I don’t know what. Right now, my plan is to plan. I’m not willing to see any healthcare practitioners in person, so it’s tricky, but it’ll be a winter of research and putting some ducks in a row.

I also have to start preparing for a what might be an inevitable surgical eventuality. I need a pain management protocol with bigger guns than paracetamol, I need to strengthen my core and my bones, I need to find muscle relaxants to which I don’t react, I need to get my blood pressure up and control my MCAS as much as possible. I need to save money. No more ignoring.

Happy Samhain, everyone. And my 9th “sickiversary” — not a happy day, but one that should be acknowledged, nonetheless.

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Need medical detectives to help with my collapses!

I need help from all the smart people here. Any brainstorming and all ideas welcomed.

This morning I had to call the paramedics because I had another “collapsing episode.” Severe abdominal pain triggered low blood pressure and a very low heart rate. I was shaking violently and having trouble breathing, the latter seemed to be caused by the discomfort in my heart. I fought very hard not to lose consciousness. I was covered in cold sweat and my vision was blacking out. But the bradycardia and trouble breathing were the worst. It felt like my heart might just slow to a stop and I would be gone. It lasted for over half an hour — an eternity while panting with my HR in the low 40s when it should have been twice as fast.

The last time this happened, my doctor ordered a heart workup, which showed nothing, really (I’ve put the results below; I don’t really know how to interpret them). What I’m trying to figure out is if there is anything I should look into besides making sure my heart is okay. I’m on BP medication (to raise it), I don’t have POTS (as in, high heart rate)… I am so wiped out today and I can’t believe I just have to ignore these episodes, shrug and move on. What my heart did today was terrifying!

If anyone wants to help me play medical detective, here is the history of these episodes (no other health history included and there was a lot, obviously).

My episodes, as best as I can describe them, are vasovagal syncope. But they’re more complicated than that. It’s not a faint and then BP recovery once I’m supine. Abdominal pain/pressure/inflammation/cramping/spasm = BP and HR crash.

The first one I remember was in 2005 on the first day of my period. I used to have excruciating dysmenorrhea and one month it caused a collapse — on the bathroom floor, cold sweats, shakes, vision blacking out, very low BP. Went to the emergency room, got IV fluids.

This continued to happen once or twice a year until 2011 — and I can’t predict which months or why. It has happened when my period had nothing to do with it, but it was triggered by my bowels. I have had constipation my whole life, the last time I ever took a laxative was in 2008. The pressure/cramping caused the same low blood pressure, near-loss of consciousness result. My best friend held me up on the toilet while firemen filled my bathroom. As soon as my bowels moved and the pressure released, I recovered.

The only time I remember it not having anything to do with abdominal pain was one of the worst times. I spiked a fever and then collapsed on the front steps to my house and the medics couldn’t get a blood pressure reading at all. Went to the ER, got IV fluids.

I need to mention that all of this was pre-ME. An odd thing happened after ME destroyed my life in 2011– the dysmenorrhea stopped. I went 3 years without a collapsing episode, then had a random one in 2014 and not again until last year, once in February from random severe period cramps on the first day of menstruation (I think it might have been a burst ovarian cyst since it was so out of the norm) and once in July from a massive acute bowel spasm that was about a 9 on my pain scale.

(I should also mention that every time I went into full-blown anaphylaxis was at the start of my period. That started in 2001, four years before the collapses.)

The difference between these recent episodes (last year’s and today’s) and the pre-ME ones is it seems to be more about my heart and less about my BP. Both are low, but my BP isn’t scary-low (scary to EMTs, maybe, but not to me). I used to be able to not talk or move because I was so hypotensive, today I made it downstairs to open the door and had more awareness — was monitoring my HR and O2.

The past few days, I had a deep ache in the very bottom of my abdomen. I cannot have bowel movements and am completely enema-dependent with chronic pain and bloating, so I only noticed this pain because it was so low in my belly and deep-feeling. I kept looking for my period, thinking maybe it wasn’t my bowels, but was instead a heavy, achy uterus. When I went to sit up in bed, the pain skyrocketed, maybe my bowel spasmed like last year. I immediately started shaking and sweating, the vagal reaction happened right away. That’s my best guess — trapped gas in my very dysfunctional, SIBO-ridden bowel created pressure, pain and spasm that triggered a vasovagal response. The pain and bradycardia resolved at the same time and so did the shakes and panting, but I’ve been completely wiped out all day. I’m still in a lot of bowel pain now, 12 hours later, but it has moved up to its normal position in the upper abdominal quadrants.

So, any thoughts? With no period involvement, no actual bowel movement, and symptoms so severe that I called 911 (that is literally the SECOND since being sick — 9 years), I can’t believe I just have to ignore it and go on as usual, without answers. I mean, besides the obvious remove my bowel and remove my uterus, but I’m just not there yet.

*****

Echocardiogram:
There is mild aortic insufficiency. Mild aortic regurgitation is present. There is an eccentric jet of aortic insufficiency directed towards the septum. There is trace mitral regurgitation. Trace tricuspid regurgitation present.

30-day heart monitor:
The vast majority of episodes show normal sinus rhythm without ectopy. Some episodes of dizziness show sinus tachycardia. One PVC and one blocked PAC were observed.

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Treatment Update

Today (actually last Thursday, it took me a while to write this), I had my follow-up appointment with Dr. Kim to go over the gaggle of blood tests I had done in March. There is a lot that I am adding into my regimen, so I wanted to document it all asap before I forget everything she said.

We’re going to try hyperbaric oxygen therapy! I said it as a joke as we walked past the room with the claustrophobia chamber: “When do I get to dive?” And she thought it was actually a good idea. So, I’m going to start with a very short time (10-15 minutes) and work up to 60 minutes “at depth”, with supplemental oxygen, once a week. This is out-of-pocket, of course, and pricey at $150-$175 per 60-minute session, so I’ll try a few and see how I do.

I am starting a slow treatment for candida with Nystatin, Diflucan and Thorne SF722. Here’s the protocol:
*Nystatin on Mondays and 2 capsules a day of Thorne SF722 Tuesday through Sunday for 3 weeks.
*Then the same thing with Diflucan on Mondays for 3 weeks.
*Then Nysatin Mondays, Diflucan Thursdays and 2 SF722s on the other days for 2 months.
She didn’t mention diet and I didn’t bring it up. Yippee!

I’m increasing oral progesterone to 100mg/day (I’m at 25mg now), staying at 25mg of oral pregnenolone (uh oh, I just realised while adding this link that I’ve been swallowing my pregnenolone whole, not realising it’s sublingual… grreeaaat 😝) and changing from topical DHEA to 25mg oral.

My sex hormone binding globulin (SHBG) is high, which she said functionally lowers hormone levels. I’m going to start nettle root capsules (work up to 300mg twice a day) to bring SHBG down (not to be confused with nettle leaf, which I drink in tea every day).

I’m not anemic, but my iron is low. She wants me to add Floridix, but after reviewing the ingredients, I may just do a generic ferrous gluconate supplement for 6 months.

For sleep:
*5HTP, 75-150 mg at night (this was recommended by a friend–thank you, M–and Dr. Kim thought it was worth a shot). She says it may even interact with the 5HT4 receptors in my GI tract and help motility. 30-50 mg P5P (active vitamin B6) should be taken with 5HTP.
*Dr. Yasko recommended I get my lithium tested (she answered a quick question on Facebook, I’m not working with her) and Dr. Kim thought I could try supplementing a 20-40 mg per day without a test and see if it helps.
*Belsomra, a prescription sleep medication given to me by my sleep doctor, is still sitting on my shelf a year later and I intend to take a small nibble one of these days. It doesn’t interact with 5HTP, so I can try all the things.

For constipation, I am going to try MotilPro (work up to 3 capsules morning and noon) and a bit of iodine in the form of potassium iodide (5-20 mg 4 times per week).

She said my vitamin D at 40.4 ng/mL is actually fine and I should continue taking 4,000iu/day (I take Thorne liquid D3+K2). She bases this on my calcitriol (vitamin D 1,25) number, which is good at 48.2pg/mL, right in the middle of the range.

She’s not worried about my high cholesterol or LDL at all, so I’m going to shake off my concern about that and trust her.

She said not to worry about an Igenex lyme test or my positive bartonella test for now. She is going to treat my high mycoplasma pneumoniae eventually and she said that treatment is similar to what she’d do for tick-borne infections. I have to say, I kind of like that a reputable LLND isn’t jumping straight into Lyme testing and treatment. She’s definitely not a one-trick pony.

I’ll start antimicrobials for M. pneumoniae, CMV, HHV6 and EBV later this year when my body is stronger. She thinks it will most likely take at least 2 years to get those blood tests into the normal ranges (to the point where my immune system isn’t mounting a response against reactivated infections).

Other supplements* and prescriptions I currently take, many sporadically:

MitoCore
CoQ10/ubiquinol
Humic Acid
Thorne Trace Minerals
Thorne Riboflavin-5-phosphate
Thorne Niacel
Thorne vitamin D3+K2
Thorne B complex #6
Magnesium malate
Magnesium glycinate
Jigsaw magnesium
Potassium gluconate
Biotin
Thiamin
Vitamin A
Vitamin C
Wormwood
HCL + gentian + pepsin
Enzymedica Digest Basic
Enzymedica Digest Spectrum
Charcoal
Levothyroxine (100mcg/day)
Liothyronine (15mcg twice/day)
Prednisone (3mg), Benadryl (25mg), Zantac (10mg), fluids (sodium chloride 0.9%, 1 liter) and Gamunex-C (5g) during infusions.

*By the way, all the supplement links here are for Pure Formulas (and all brands are gluten-free, soy-free and well-regarded). I am not affiliated with them in any way and I can’t get kick-backs if you buy something from these links like lots of bloggers that make money that way (although, maybe I should look into that!). I’ve just done a lot of research and they are consistently the best for me. If you decide to order from them and you want to be a kind and selfless friend, you can use my referral code: RRKMLW or shop here. Once you complete an order (without using any of your own reward points), I get a $10 credit. 😀 I like Pure Formulas because a) free shipping with no minimum; b) 2-day shipping always if you have ShopRunner, which I do through my AmEx; c) you earn cash credits for your orders; d) you can return products you have problems with, even if opened; and e) I have contacted many supplement manufacturers to ask about recommended online retailers (because I’ve read some scary articles about knock-off supplements on Amazon) and almost all of them have told me Pure Formulas is reputable. Last thought: if you comment below with your Pure Formulas referral code I will use one whenever I order (which is often).