Author: David Kominsky

Apparently, it’s been quite a long time since we’ve posted. I’ve been posting some updates on my Facebook page, but this blog has been awfully quiet. So this will serve as a brief recap to last month or so, as well as a bit about where we are now.

Some things have changed rather significantly. Eva no longer has a gallbladder. Unfortunately, while that dealt with one type of pain, there was another pain we were hoping it would address that it didn’t. The surgery itself went well (on December 24th), though the healing was more painful than either Eva or I anticipated. It was a laparoscopic procedure and I think we both had the sense that it was therefore “no big deal.” It remained major surgery, minor only in comparison to having it done conventionally (or whatever you call non-laparoscopic surgery). Eva was fully debilitated for ten days.

At the end of the ten days, Eva got suddenly quite sick. On January 5, the nurse came in the morning (9:00 AM) to draw labs and take vitals. Her vitals were very slightly off for her (think 120 over 65 rather than 102 over 62). By 1:00 PM, Eva had spiked a fever (100.0, which is a lot for her), her blood pressure was way up, her she was shaking, sweating and shivering. By 4:00 PM, our primary care doctor wanted Eva to go to the hospital via ambulance. This led to 4 days in the hospital, treating for suspected sepsis and working to get Eva’s pain and diarrhea under control. On the 9th, she was finally ready to return home, albeit with some new bile salts, and a new suggestion for what was causing the pain that the removal of the gallbladder was supposed to cure but didn’t.

The pain which had been tentatively ascribed to the gallbladder is now being seen as muscular skeletal pain along the rib cage. This diagnosis was not due to the hospitalist, but due to a visit by a GI who came by. She saw where Eva was hurting, had her move a little, and said, “this isn’t GI pain.” This is opening up some new treatment options, like lidocaine patches.

The bile salts, whatever those are, seem to be dealing with the diarrhea and are also lessening the intestinal pain. While that is good, it isn’t enough of a lessening for Eva to be able to return to tube-feeds rather than IV feeding (though, as I type this, she is setting up to try a trickle feed to see how it goes, since the dietician asked her to give it a shot yesterday).

All of this pain, new (muscular-skeletal) and old (intestinal), has been taking a huge toll on Eva. For the last two months or so, she has been slipping into a depression. While the usual answer for that would be medication, Eva has medication-resistant depression. What works for her is transcranial magnetic stimulation (TMS). The downside to that is that it is an intensive process: 18 minutes a day, five days a week for five or six weeks, in person, at a doctors office. We were set to start this process on Monday of this week, but insurance hasn’t yet rendered a determination on coverage. So we are still waiting, with Eva feeling crushed in spirit and body at the moment.

Fundamentally, Eva (and I, to a lesser degree), are needing to redefine what normal looks like (again). The baseline expectation that we hold keeps resetting ever downwards. Quality of life slips lower each time something that seemed “temporary” begins to feel like it might be the new “normal”. While gastroparesis is not typically a degenerative disease, it is also, in some ways, not so much a diagnosis as a symptom. And we remain unclear what it might be a symptom of.

We are moving forward. Eva is looking into getting pain-blocks for the muscular-skeletal pain. Eva has an appointment with her Naturopath GI in Portland (telehealth) to look more closely at root causes. And we are waiting on the TMS. But all in all, things around here feel pretty bleak.

First the headline: nothing has gotten any better.

Maybe a month ago or so Eva developed a new pain (because that was exactly what she needed). It is in the upper right quadrant of the abdomen. There is good news associated with this: apparently there is exactly one thing that causes pain there: the gallbladder.

There’s also another another new symptom that seems gallbladder related: intense pain for 10 – 15 minutes accompanied by discharge of a cola- colored fluid in Eva’s vent bag (normally the discharge is a bright yellow).

Following an ultrasound, it was confirmed that there are some issues with Eva’s gallbladder: sludge, stones, and a 6 mm polyp. The polyp is apparently a long-standing thing, and therefore not worrisome in the way it might otherwise be. Stones and sludge are not inherently problematic, unless they are causing pain. Which they are. So we see a surgeon to see what he thinks.

We saw the surgeon yesterday. He thinks Eva is complex. With someone else, he would be giving 97% to 99% odds that removing the gallbladder would fix that pain. With Eva, he feels like it is a toss-up, but there’s no other treatment. With some trepidation, Eva is going to go ahead and schedule surgery to remove the gallbladder. Actually, she has scheduled a call with a scheduler to schedule the surgery. Streamlined, this process is not.

We have no reason to believe that this will fix the intestinal pain that prevents her from using her J-tube for feeding (though it would be lovely if it did), but suspect it will fix the additional pain (which is mainly around a 7 on a 10-point scale) associated with the gallbladder. And less pain is better (not our family motto, but it might be in the running). Also, it seems like doctors would like to get the “extraneous” pain dealt with before focusing on the intestinal issues more closely (in part because no one has any more good ideas on the intestinal pain).

Speaking of intestinal pain and getting more data: we met with an interventional GI this week about the colonoscopy and small bowel enterography mentioned in my last post. The key question was how to do the colonoscopy prep. For those who have done a colonoscopy prep, little more needs saying. For those who haven’t, there exist two options for colonoscopy prep: high-volume and low volume. High volume colonoscopy prep involved drinking 4 liters of a solution that flushes everything in your GI tract out your anus. By the end, you are putting out clear liquid, and therefore when they stick a cameral up your butt, they can see pink flesh with no other matter to block the view. It’s about as wonderful as it sounds. Needless to say, getting 4 liters of anything into Eva is a non-starter.

But then there’s the low-volume prep: that’s only 2 liters. With the same result. Which unfortunately, is also 1.75 liters more than is really imaginable for Eva. Also, the GI says that people with slow motility (e.g., those with gastroparesis) tend to do better with the high-volume prep because it is harder, not easier, to flush everything through the system.

The end result is that a colonoscopy is not a good option for Eva. We did ask what she thought might be an option to try to get more information about her intestines. The only option she could come up with was surgically going in and taking a look, which she felt was rather a big deal. We agreed. So for the moment, we are going to wait and see what symptoms remain following the removal of the gallbladder.

Step by step, we keep moving forward. But this is really a lot less fun than it looks from the outside.

This is David writing. Eva is too distracted by pain to feel like writing.

I suppose that first paragraph could be the entire blog post. But I’ll add details.

We still don’t know what’s wrong. We know it isn’t c. diff. Multiple stool tests have confirmed that. And there is no evidence of a UTI. But Eva’s white blood cell count has been gradually rising, suggesting an infection somewhere in the system. Unless it’s just being caused by stress.

There is, as mentioned above, a lot of pain. Some days are better than others. Some days are worse. They aren’t ever good.

As I mentioned last time, the GI wants to do a special MRI called enterography. This would give a close look at Eva’s intestines. Unfortunately, to do this, Eva would need to get 1500 ml of contrast into her digestive tract. Now, we have a fairly clear idea of how much we can put into Eva’s intestines, depending on whether the contrast acts more like her formula or more like water.

If it acts like formula, Eva can pump it in at 3.5 ml/hour. In which case it would take 428 hours to get it in. Obviously a non-starter. If it acts more like water (or exactly like water) we might get it in at the rate at which she can push water flushes, roughly 60 ml/15 minutes. Which would take more than 6 hours. And assumes that Eva can maintain that rate for more than 2 syringes of 60 ml each, which is untested. And it assumes it doesn’t start pushing stuff out the other end, which seems like a really bad assumption.

All in all, it seems unlikely that this is going to work at all.

After pointing all this out to various doctors, we are now working on a variety of other options.

An abdominal CT scan, which is looking at Eva’s liver in particular. Her liver enzymes have been elevated for a while, and people aren’t delighted with that. Eva cut out one pain med mid-week and we will see if her labs look better Monday (we should have gotten a first look with Thursday’s labs, but they seem to have gotten lost in the maze that is Quest Diagnostics). The CT scan should show any physical abnormalities there.

A colonoscopy with small bowel enteroscopy has been ordered because if you can’t see it with an MRI, you can always go in with a camera and take a look. This is looking, again, for physical features that might lead to pain and trouble feeding enterally.

Meanwhile, Eva is not only getting full nutrition from TPN (IV nutrition) she finds herself somewhat dehydrated, despite receiving 2.25 liters of fluid with the TPN. So we are now infusing another liter of saline either daily or every other day depending on how Eva feels. This also falls into the “somewhat weird” category.

In summary, we still don’t know what’s going on, Eva’s miserable, and completely over being a medical mystery.

Once again, this David writing for Eva.

A week on from the last post, we have some clarity on where we are going, but none on what’s going on. I’ll start with the news, and then get to the philosophical ramblings alluded to in the title.

As you may recall, Eva is/was down to 3.5 or 5.0 ml/hr on her feed (which is about 10% of what it should be). We had two plans for improving that, and two hypotheses about what was going on.

Plan 1 was to supplement her feed with Prostat, a protein concentrate, through her tube. This was not successful. It traveled through her system very quickly. And unpleasantly.

Plan 2 was to switch to an elemental formula (one which contains the nutrients in a pre-digested form). This wasn’t a failure, but doesn’t seem to have fixed anything either.

Hypothesis one: Eva had stopped tolerating her formula. Test: Switch formula. If the hypothesis is true, symptoms should ease with new formula. As seen above, in Plan 2, symptoms did not ease. This suggests that Hypothesis one is false, which is to say, the problem isn’t Eva’s formula. This is both good and bad news. Good in that we really don’t want Eva to develop intolerances to her formula every couple of years because there just aren’t that many appropriate options for her. Bad News in that it doesn’t move us any closer to a solution.

Hypothesis two: Eva had c. diff. Test: a stool sample test. Which came back negative for c. diff. and everything else. Also, her bloodwork looks good, no inflammatory markers or elevated white blood cells. This all suggests that there is no infection present.

This leaves us where we are now: we have no idea what is going on. More on that later in this post.

We do know what we are doing: Eva is going on TPN. We are hoping to start that this evening. It will be a slow ramp-up, which allows us to do this without an inpatient hospital stay. That means that Eva will not see an immediate increase in calories, but that should build steadily over a few weeks.

Now back to the title.

A little while ago, Eva and I looked at each other and agreed that we finally had a bit of a handle on her health. Not that things couldn’t and wouldn’t go wrong, but that we would recognize what was going wrong and know what to look at. For instance, if she suddenly ceased to be able to feed at her normal rate, it was either some infection (c.diff. or SIBO, for instance), or an intolerance to her formula. Obviously, in hindsight, this is hubris, the belief by mortals that they are the equals of the gods.

We are now in a place without any idea of what is going wrong with Eva, but with clear indications that something is. At this point, we do not know whether there are any tests that we should/could be running that will point us towards answers (we are communicating with Eva’s GI to try to figure that out). There is a sense of not-knowing even what questions to ask which is unfamiliar and disturbing.

Which brings us to Humility (anavah in Hebrew). As we are in the middle of the Ten Day of Repentance, humility is a featured virtue. And in knowing that one does not know, there is a deep humility. We are required to acknowledge that our knowledge is not as vast as we imagined. To admit that there are problems whose causes we lack even the language to imagine.

We are trying to treat this mystery as just another step in learning to manage Eva’s disease. But from the inside it is frustrating and scary. And Eva is doing this on around 250 calories a day.

This is David writing for/with Eva. Life has gotten adequately interesting that we felt like an update was necessary, and hard enough that it didn’t seem like it would happen if Eva had to write it.

Quick recap: Eva has been at roughly 60% nutrition (775 calories) for about the last 6 weeks. In normal times, she runs her feed at 50 ml/hour. Six weeks ago, she ceased to be able to tolerate that speed, and had to reduce to 30 or 35 ml/hour during the day and only 25 ml/hour at night. It was just too painful to go any faster. Not to say it wasn’t painful at those speeds, but it was “tolerable”. Over this period of time she dropped 15 pounds.

Tuesday evening, Eva got really sick with pain and cramping and diarrhea. Eva’s baseline pain is around a 5. It spiked up to a 9 (note that 8 is when the pain is enough to cause her to cry). Eva reduced her feed speed to until it was tolerable: 3.5 ml/hour. For reference, at 3.5 ml/hour, it would take 285 hours to get 1 liter (Eva’s theoretical goal).

Since then, Eva has been able to tolerate 5 ml/hour when she is awake and her G tube is venting, and 3.5 ml/hour at night or when she has to close off her G tube for an hour after taking her meds (so that the meds don’t flow right out the G tube. Yesterday this resulted in a whopping total of a 74 ml of formula getting into Eva’s intestines, for the 111 calories referenced in the title.

The good news is that Eva is already on IV hydration at a liter a day, so we are panicked about the lack fluids from the decreased enteral feeding. If she did not have a PICC line and IV fluids at home, we would be in the hospital already.

Why is this happening? The short answer is, we don’t know. We have hypotheses, some of which are testable, some are, at the very least, harder to test.

One possibility is that Eva has developed an intolerance to her formula. This already happened once (Summer or 2023, I believe) and she had to switch from Kate Farms to Nestle Compleat. The symptoms looked pretty similar. On the one hand, this is good news, because it is a relatively easy fix (for certain, really odd, definitions of “easy”).

By chance, we had an appointment with the dietician yesterday. She had been out for six months on maternity leave and we had been working with a substitute. Welcome back to chaos!

She suggested a new formula. We are receiving a new and different formula from Amazon today (Neocate Splash!). It is intended for toddlers and small children, and is a different type of formula: elemental. We are unclear about what that means, but the dietician seems to think it might work. We are also adding in some protein liquid straight through the tube (collagen something or other…).

I said this was the “easy” solution, because best case scenario, Eva’s intestines encounter the new formula, stop protesting, and allow Eva to go back up to 35 ml/hour day, 25 ml/hour night, and she is able to start the process of increasing her feed rate at a maximum of 2 ml/hour every 3 days. At which rate she gets back to full nutrition in a month or two. Realistically, it will probably take longer than that to know whether he body accepts the formula, and the ramp-up will not start back at 35 ml/hour but at something slower, and there will be more pauses in the middle. Again, this is still best case.

Second hypothesis: Eva is sick. With something else on top of the gastroparesis. If she has some sort of infection (e.g., something like c. diff., but probably not c. diff. because we tested for that a month ago or so). This feels somewhat likely given that she has had similar symptoms from infections in the past (c. diff., SIBO). But given that it doesn’t feel to Eva like SIBO, and she recently tested negative for c. diff., it would be a new and different infection that would need tracking down.

What are our next steps (besides the new formula)? We are talking with our doctor about putting Eva on a dextrose solution for hydration. This would add some (though not a lot) of IV calories and give Eva a bit more functionality. Not a lot more functionality (it would add 175 calories per liter). But it would be better than nothing. The advantage to this is that it is truly easy. We just need to get the bags from the infusion pharmacy and could administer them exactly the same way we do the saline. Not a long-term solution, or even medium-term, but simple.

Longer-term, if things still look like this early next week, we begin to work on putting Eva back on TPN (IV nutrition). The dietician thinks that if we are willing to get Eva to the lab daily for blood draws, we could do this outpatient. Given Eva’s trauma response to being in a hospital after last year, this would be a major win. TPN is a pretty big deal as medical interventions go. We were pretty excited to move off TPN and onto saline in late winter. Nonetheless, it means Eva gets full nutrition almost instantly. If we can do this without inpatient hospital time, it is the quickest solution, but potentially takes the longest to get Eva back to normal (but at full nutrition while we work to get her back to normal).

Finally, we are doing all of this as Rosh Hashanah bears down on us. The good news (we thought) was that I have no service leading responsibilities. But it turns out I just didn’t know I had service leading responsibilities because the small lay-led congregation in the community hadn’t let me know what they wanted me to do until last night (4 days before Rosh Hashanah). To be fair to them, they thought they’d let me know already. We agreed I would do as much of it as I can prep, and assuming Eva isn’t in the hospital, I will do some form of Torah service/reading/sermon.

May the new year be a less exciting year.

This is David writing for Eva again. 

Much remains the same as the last time I posted. Eva is still in pain and nauseated. We have some new symptoms (diarrhea). We have the same feeding tube Eva has had since early August. We have blown past two appointments for getting it swapped out without getting the necessary approval from the insurance company.

Just a reminder: Eva has been in a great deal of pain since she had this tube put in on August 1, and has been unable to use it for feeding because of the pain. Instead she is now on TPN (IV nutrition). We believe the pain is being caused by this tube or how it was placed given that the pain and the placement happened at the same time. None of the imaging has shown any reason this should be true, but it also hasn’t ruled it out. 

About 6 weeks ago the GI authorized a tube swap for Eva (though this was not well communicated). It was originally scheduled for Second Day Rosh Hashanah (October 4). But first, as with everything in today’s medical system, we needed clearance from the insurer. 

Stanford is out of network, but there is a magic form that lets them perform the procedure as though they were in network. But despite a heroic push by our complex care coordinator, this form could not be completed by October 4th. The night of October 3, the procedure was pushed, rescheduled for October 24 (“hey! That’s tomorrow,” I hear you thinking). For the last three weeks our complex care coordinator has been chasing around with Stanford and Blue Shield, trying to establish where the form was and whether it had been approved. Stanford said it had been submitted. Blue Shield said they’d never seen it. Stanford couldn’t produce a copy for our complex care coordinator to show Blue Shield and push through the bureaucracy. This has been the subject of daily pushing by us and our complex care coordinator for at least 10 days.

Today, the secretary of the GI practice at Stanford sent a note through the patient portal saying, essentially, “form? What form? We never filled out a form.” Procedure was canceled again.

Eva remains in debilitating pain. The form seems not to exist yet. We have to start over from the beginning. And now Blue Shield says it is taking them a week to enter forms into their system for review, so plan extra time for that. 

To say we are frustrated (and that Eva is so far beyond frustrated that I don’t have words to express it), would be rather like a theologian describing God as “good” (that’s a pretty funny line for those who have studied medieval theology–if not, well, have you considered going to seminary so you get the reference? It doesn’t take more than five or six years…). Yes, we are exploring other options (can this be done truly in-network? To what degree is this a commodity procedure and to what degree does specialization matter?). Yes, the complex care coordinator is part of the frustrated we. I believe Kafka was mentioned today.

And so, we are restarting this process again tomorrow. Girding ourselves once more to battle bureaucracies that specialize in making it difficult to speak to a human (again, so grateful that the complex care coordinator does much of that navigation for us). Eva remains in pain and nauseated. And we continue to float on in winds and tides of large institutions, subject to their whims and arbitrary decisions. 

The following is Part I of the account of the last week and a half. The writer is David, because if we were waiting for Eva to be up for writing, it might be a while.

On Wednesday, May 3, Eva’s nausea ramped up to intolerable levels. She lives with constant nausea, so when she says it’s bad, it’s pretty awful. She could no longer take in liquid orally. At the time, she was still able to tolerate some jello and tiny fruit juice popsicles. 

At the same time, she was starting to vent large quantities of fluid through her G-tube (gastric-tube, i.e., from the stomach). She vented roughly 150 ml in a couple of hours. This is very unusual, but not a unique occurrence for Eva.  

By the next day, her J tube (jejunum-tube, the beginning of the small intestine) wanted in on the action and was insisting on venting small amounts of bile. This is a unique occurrence. Eva was using medical marijuana, Zofran, ginger and a scopolamine patch to try to control nausea, to no avail. 

As an aside, these events of 10 days ago are so hazy in either of our minds, that I’m looking back to text chains of conversations with our complex care coordinator to reconstruct things. It has been a long week and a half.

Eva’s current GI was unavailable for conversations on this Thursday.

On Friday, Eva’s GI is off. But we did manage to have a conversation with the on call GI at his practice, who pulled Eva off the scopolamine and put her on a dose of Ativan. 

At this point, Eva is feeling her electrolytes getting out of whack. One of the issues around gastroparesis is that the body processes salt differently and requires much more of it. Eva normally uses a packet of electrolytes for high intensity athletes daily. But she also requires regular hydration because the free water available in her formula is limited, and what she can drink is extremely limited, and she wasn’t getting enough extra in her flushes (syringes of water used to clear the j-tube every four hours or so during awake hours). This is about to become relevant. 

Saturday, things are getting worse. Eva is feeling “wonky and woozy.” Electrolytes are out of balance, she is dehydrated. We clearly need to get Eva some IV hydration. 

This seems like something simple to do. There are spas that offer IV hydration. We figure we will just show up at an urgent care around the corner and get this dealt with. No big deal. 

Big deal. Apparently urgent care doesn’t do hydration. Apparently the spas aren’t as set up to do this as their advertising claims. The complex care coordinators are not wild about us having a nurse come to the house to administer hydration because they’d kind of like testing available, just in case. This means . . . off to the hospital. 

But remember, this isn’t a big deal, we just need a bag of saline water to flow into Eva’s veins. We were originally looking to do this at an urgent care. So, instead of heading into Manhattan to a serious hospital, we head to a minor hospital in Queens (albeit one in the same medical system that we are moving our care to–NY Presbyterian). 

We arrive around two. We sit in the waiting area. For hours. Until around 5. Which sort of makes sense, because Eva doesn’t present as a particularly urgent case. Then they bring us into the treatment areas. 

We explain to a PA (physician’s assistant) what the issue is (nausea, venting through G tube and a bit through J tube, dehydration. The PA is clearly confused. We realize the PA has no idea what a GJ tube is. We educate the PA (Eva feeds through the J tube, vents through the G tube . . .).

By around 6:00, maybe, we are talking with an attending. Who isn’t quite sure what to do. She’s willing to do the saline and all, but she wants to know what normally happens when we come in for treatment. Should she be admitting Eva for in-patient care? Fun fact: this is our first time on this rodeo–we don’t know what we need. That’s why she’s the doctor. This is when we realize that there is no situation when it’s a good idea to take Eva to a less than major hospital. Nothing with Eva’s health is ever simple.

They finally get her some IV Zofran for the nausea and a hydration bag at around 7:00 PM. Five hours after we arrived at the ED (Emergency Department). 

IV Zofran is Eva’s favorite drug. Like ever. It is magic for her. It makes the nausea go away. Every time. Until now. It does nothing this time. 

By 9:00 PM the fluid has fully entered Eva’s system and the off kilter, woozy feeling has retreated. They give Eva another dose of IV Zofran and send us home. The second dose reduces the nausea to a tolerable but still pretty significant level. Eva and I are beginning to have intimations that this may turn into a “thing