Dear Doctors, About Self Care

Dear Doctors,

Do me a favor. Either really believe in caregiver self-care, or just stop talking about it.

And by “really believe in” it, I mean “actually help it happen.”

I feel like it’s important to note at this juncture that you might THINK that you’re helping. You might feel like a friendly reminder “don’t forget to take care of yourself!” is helping. You might feel like “use some of that respite time for a date night!” is helping. But these things aren’t helping. These things are nagging. Or, alternatively, these things are about as useful as saying “hey, why don’t you move to Mars?”

Oftentimes, the end result of these types of comments is the parent/caregiver just feels even crappier, because they KNOW they should be taking better care of themselves, and you’ve just reminded them that YOU KNOW they’re failing at this. And parents don’t like to think their child’s doctors see them as a failure at anything.

So either help, or just shut up.

How can you actually help? Well, for starters, if you know you don’t plan to actually offer practical help, then don’t bring it up. Do you plan to find me a trustworthy babysitter or respite person who is comfortable with Gtube feeds and medications and severe ADHD and developmental delays? And the money to pay for both them as well as the evening out? No? Then why bring it up? When you bring it up, it makes me feel like I have to justify myself to you. “Oh, well, it isn’t really that easy…” and then it turns into a conversation of me trying to convince you that it’s actually not that easy. But the thing is, this is really actually NONE of your business. I actually shouldn’t HAVE TO try to talk you into understanding that the real life, on the ground experiences of your patients are different from your own personal experiences.

Secondly, if you really have a commitment to caregiver self-care, take a minute to assess all the things you’re asking your patients’ caregivers to do. And then think through the things that all the child’s other doctors are asking them to do. And then look at that list. Really look at it. And then consider if, in exchange for a bit of sanity, or some time to eat lunch every day, you could let something go. Nothing you can let go? How can you get community supports in place to help?

Thirdly, listen to the parents of your patients. If they’ve made a well thought out decision to drop something from their radar, LISTEN TO THEM. Why did they decide to let that thing go? Are they overlooking important information that they need to know to make a better decision? Are there compromises to be reached here? Is there something ELSE you could back down on so the parent feels able to bring increased focus to the area they had previously dropped? If the thing the parent has dropped feels vitally important to you, the doctor, then it’s time for a conversation, but the conversation needs to be more than just “this is important, so do it.” You need to look at the child and parents as whole people.

Your patients’ parents are more than just unskilled labor existing solely to support your patient.

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Immunology, also titled Weekly Home Infusions Until the End of Time

This update isn’t going to be as clear as most of them. Because Immunology is super hard, turns out, and I don’t fully understand all of it.

So we saw immunology at the request of Transplant. We’ve seen Immunology in the past – when he was having all those blood infections from his central lines, and then right before and right after transplant – and they said he had some immune deficiencies but some IVIG should take care of it, get him through the post-transplant period, and he should be fine.

And he was, for the most part. In fact, he had low IgG at that time, and now he doesn’t.

But then the CMV kept coming back. All the respiratory infections. Bunch of other infections. All mild, nothing super big, but just constant. Which I had assumed was just… normal. But when you make a list of all the infections, it’s a really big list. Big enough that Transplant was shocked when we really listed them all out. And then include the mystery fevers and the UTIs that aren’t UTIs… it’s a big list.

So back to Immunology.

They ran some labs. And I don’t understand most of them. I mean, they’re all numbers and random letters. And Google is absolutely no help. I can Google the individual things, for the most part, but putting them together to make some sort of picture that makes sense…. nope. Can’t do that.

The one thing I could definitely tell on my own (and later was explained by Immunology) was that he has issues with his NK Cells. To call it NK Cell Deficiency, this has to be the person’s only immune issue, and that’s not the case here, but I’m still hiding in the NKD groups anyway, because I don’t know where else to go. Regardless, as of the present time, it seems evident that not only does he have too few NK cells, but the ones that he does have do NOTHING. The actual result was “virtually no activity.”

NK Cells are the cells that are supposed to respond quickly to viral infections and cancerous cells. Teddy’s just can’t be bothered. I’ve been looking for a link to put here of what happens when your NK cells don’t work, but I could find basically nothing. Because basically nobody really knows much. Whoohoo. In fact, most articles start by acknowledging that, essentially, we don’t know much about NK cells but we know they’re important based on looking at people whose NK cells are either too few or too lazy. There’s this, but it’s just a lot of long words to say, “well, who the heck knows, anyway…” and then a bunch of majorly depressing shorter words about all the things that destroy the people who have this.

He also has impaired T-cell function. That is from the immune suppression, probably. The “probably” part makes me giggle a little. Like I said, immunology is hard. (A study published in 2015 says, “But the immunoregulatory mechanism of FK506 is still largely unknown.” (FK506 = Tacrolimus, one of the two immune suppressants T takes.) So we’re doing this to him on purpose. Probably.

Then he has antibody deficiency. To determine this, they look at whether he responds to and maintains a response to the immunizations he’s gotten. He hasn’t. We’d previously determined that the MMR was essentially a waste of time (man, remember all the bellyaching I did over that one? Those two, really? So close together like that to squeeze them in before transplant? Shoulda skipped it.). I can’t remember which other ones we’ve checked, either around transplant time or more recently, but every one we’ve checked has come back as not immune. Not immune despite, in some cases, extra doses. More reading here.

And then there’s the rest of the effects of immune suppression.

So I don’t think this is the end of the story for T immunologically. I don’t know if having two or three things wrong with his immune system means there’s one over-arching immunologic diagnosis… it’s certainly not specified in the clinic notes and is one of my questions for our next immunology appointment. It’s also possible (I want to say likely, but we’re not going there) that the results from Genetics will shed some light on this.


So. Treatment.

Teddy started SCIG and will continue it until the end of time.

Subcutaneous Immunoglobulin. It’s an infusion of immunoglobulins that goes in under your skin. Once a week, Teddy’s going to get an infusion that will last for an hour or so. The medicine goes under his skin in a big lump and then is slowly absorbed over the next 24 hours or so. We did the first one last week at UI and it was fine. We’ll either go back again this week or we’ll start doing them at home this week. We’re still waiting to hear one way or the other. (Because, you know, we don’t have anything ELSE on our schedule, right?) This is what he’s getting. We’ll do this every week for all time.

And it’s fine. I hate it. I hate it a lot. I super hate it. I hate it. We may not see any improvement in infections for SIX MONTHS!! I hate that, too. We have to do this every week. EVERY WEEK. But it’s fine. I mean, I’ve given him injections. Daily and weekly injections. This is better than that. Right?

It’s better than dialysis. Dialysis was nightly. And it was awful. This should be OK. But it’s just one more thing, you know? One more thing. One more thing for us to have to do at home. One more hour and a half of his life every week that we have to devote to medical things.  78 hours a year. One more thing I have to do to him. One more thing I have to force on him. One more time I get to be his nurse AND his mom all at the same time. One more thing.

I don’t want to be misunderstood here. I’m happy to do it. I would do anything he needed. Gladly.

But I’m so tired of one more thing.

Dear Doctors,

I’m considering making a series of Dear Doctors posts. Dear Doctors, Here’s Some Things You Might Not Realize From A Patient Perspective.

Here’s installment #1.

Dear Doctors,

Why is everything so very very hard? I truly don’t understand it. When I call and say, “Our insurance won’t reimburse very well for the prescription at this particular concentration, but if you change the concentration to this other option, they’ll reimburse it fine. Can you please write a new prescription for this other concentration?” this should be a simple transaction. Nurse gets message, relays need to doctor. Doctor says, “sure, that seems simple enough.” Doctor writes new rx, nurse returns call and says, “ok, it’s ready to pick up any time today before 4,” and then there actually is someone available at the clinic until 4 to enable pickup. That is SO EASY. It’s not hard. It isn’t. Until YOU make it hard.

Until your nurse calls and argues that there’s no reason not to fill it as written. Until your nurse calls and doesn’t care about reimbursement rates. Until your nurse calls the pharmacy and countermands my instructions to the pharmacy. Until your nurse refuses to ask you to please just do this because it’ll take 5 minutes. Until it takes three more phone calls. Until there isn’t anyone at the clinic to hand me the prescription when I show up during the time I was told because they’re all at lunch.

Then it’s hard.

And that doesn’t seem like a big deal, right? So it was a few phone calls and an inconvenience in having to wait around at the clinic. NBD, right? Except those were only a handful of healthcare-related phone calls I had to make that day. Except that day wasn’t the day I had respite, and just leaving my house to pick up a prescription is a lot harder than it is for you with your regular kids. Except I had only slept about 3 hours the night before, and the night before that, and the night before that going back 6 years. Except while I was on the phone, my child – your patient – was opening the front door again and running down the sidewalk because I wasn’t paying attention. Except I had to use the time I was hoping to actually eat some lunch to drive over and pick up the prescription. I skipped lunch that day, again. Except I had just spent the last hour managing medications and getting food started for my kiddo, and had been looking forward to actually getting something accomplished when I got the call from the pharmacy explaining that your nurse had told them she wouldn’t ask you to change the prescription. Except, except, except.

Except our life is HARD. Life is so hard, every day. We are still really just surviving. And have been for six years. And I don’t have the kind of stamina I used to. And when YOUR office makes things so much harder than they need to be… it’s just HARD. I need a new word.

It didn’t need to be so hard. You let it be hard. You made it hard. You could have made it easy, but you CHOSE hard. I don’t know why you would treat patients – your customers – like that.

I would challenge care providers – especially those who care for children with a lot going on – to ask their patients if their office is doing anything that makes life harder than it needs to.

Some of those things won’t be able to be changed. Our pediatrician’s office lets you come in for vaccine only visits only at a certain time every day – a time that’s horribly inconvenient and hard for us. But that’s what works best for their work flow and I totally get that. But some of the things you can change. LISTEN to your patients. Consider their feedback. I’m not talking about the post-visit satisfaction surveys. I’m talking about calling parents a few days before their visit and telling them you want to discuss this, or emailing them a few days after their visit. Honestly seek their feedback. Make sure they feel safe to tell you. And LISTEN. Ask if they have suggestions for improvement. Talk about their ideas to your staff. See where you can make improvements for your families – tiny things can make such a huge difference in the lives of medical families.

June is super full of doctors

If you’re familiar with the musical Carousel, and the song June is Bustin’ Out All Over, sing that to the tune. June is super full of doctors!

SUPER full.

Not really June, though. More like the last half of June. That’s really all we’re doing last week and this week and part of next week. Just doctors.

So we had one Iowa City Day last week (got another one coming up). Transplant clinic and then Neuropsych. Highlights:

Labs look good, except the things that look bad. The things that look bad are from known, transient causes, and will hopefully straighten out on their own. We met with nutrition, who I liked way more than last time. She has more families with blended diets now, she’s a bit older now (I mean, it’s been a few years), she wasn’t as full of fresh-out-of-college advice. She gave us the GREAT news that because the bulk of T’s diet is gtube and very healthy, he can have as much oral salt as he wants. Which is awesome because 3 months ago, his nephrologist asked us to cut oral salt. And Teddy’s oral foods are basically all salt based. We switched to low salt chips and he stopped eating them. Whoohoo!

But she wants us to increase calories. Dramatically. And I already increased dramatically. I’m already not getting them all in on a daily basis because he’s so very very puky with very low volume tolerance, and we’re restricted by meds, too. (meds + food = puke, every time. Food has to be separated from meds by at least 45 minutes. Meds are at 9 and 9:30 am and 9 pm. Food can’t start any sooner than 10 am and that’s pushing it. Can’t go any later than 8 pm. Unless he’s up early and I can get food in him at 7, but no later than 7. Or if I want to feed him at 10 pm and risk that he wakes up during the night to vomit. *OMG I thought this would be easier post transplant and the fact that it isn’t is a major source of frustration and bitterness for me.*

He got a renal ultrasound the day before, for his urology appointment later this week, and nephrology peeked at it and confirmed how very very pretty his pretty pretty kidney is.

Then his urine came back all icky. So we’re going to do a 24 hour catch to screen for kidney stones and also we dropped more urine off this morning to double check for UTI.

We had HOURS between appointments and the plan had been to burn off some energy at the playground, but it was POURING rain, so we ate ice cream for lunch and played go fish before going to the waiting room for neuropsych about an hour early and playing with their toys and puzzles. The doctor came out about a half hour early and said she was back from lunch and ready to go if we were, so whoohoo.

That appointment both went well and was harder than anticipated. It wasn’t the “OK, NOW I know what’s going on with him!” that I had expected. Last year, the story was that he was too inattentive to really be doing his best for their tests, but if we had him on meds that worked for this year, it should be pretty revealing. And it was sort of, but not so much in other ways. I’m dancing around this a little, but it was both revealing and still not revealing enough. But evidently this was the visit where we finally wrote down a new diagnosis, but with the understanding that further diagnoses are definitely still pending.

I don’t know. I’ll write more about this later maybe. I’m still mulling.

So last week was Case Manager, Ultrasound, Iowa City, BHIS a few other things. This week is BHIS, therapy, dentist, some sort of screening appointment for a potential new psychiatrist, urology, Dr. Noble (ugh), a few other things. Next week, we cap off our insanity with another full (3 appointment) day in Iowa City, where we meet with the people who decide our fate. Not really. But it feels like a heavy, destiny-influencing day. Genetics. Immunology. Aerodigestive.

I don’t even know where to start. Let’s call this post “We’re missing something.”

We’re missing something.

At least one something.

Here’s a general update:

  • Croup. Teddy continues to get croup monthly. His regular Otolaryngologist decided that he’s above her pay grade at this point, so had us see aerodigestive at UI. (and we want to see aerodigestive at Cincinatti – and I think we’re still headed that direction – but I don’t regret going ahead and seeing them at UI.) That doctor did an in-office scope on Teddy and discovered a few things. a) his left nostril dead ends. That seems like a big thing to miss for 6 years, eh? That’s going to be a new thing to tackle but not just at the moment.   b) his airway is just awful looking. Very angry tissue.  c) he didn’t see any stenosis.

    So what the heck? Now he has no stenosis? Did he outgrow it? Maybe. But there goes the “he has subglottic stenosis so that’s causing the croup” theory.  (which means the “he probably needs surgery to correct this” treatment plan is also out the window for the time being, which makes me happy.)

    He thinks his airway looks like severe reflux. Even though reflux was ruled out by a pH probe. Even though he’s been on reflux meds before with no noticeable difference. Even though, even though, even though. And this isn’t the first time we’ve heard it, and it was even a theory I had. Even after the pH probe (because they aren’t 100%). So I’m on board with this as a working theory. We’re trying a PPI again and we’ll see. He said to do it for 2 months and if he hasn’t had croup in that time, we can figure it’s working, but I’m assuming that we’re also going to do another scope. In the meantime, after 10 days on the PPI, he got croup and ended up back in the hospital, so….

  • Transplant is concerned that he’s getting sick that often – and then I told them that this doesn’t even include when he’s sick and doesn’t get croup. I don’t even keep track of that. We’ve been working with a new therapist for about 8 months now and she said this week that she has not seen him NOT sick – and she comes weekly or every other week. He’s sick ALL THE TIME. People want to know why we never go places and do things, it’s because Teddy’s ALWAYS SICK. They’re concerned, so they had us see immunology. Immunology basically wanted to run a bunch of labs, but based on the labs they’d run the last time they saw him (pre transplant when he kept getting line infections), they had an idea of what they’d see and had worked out a treatment plan that we’d proceed with when the labs came back looking as expected. The labs have come back. They’re not as expected. I don’t have the first idea what they mean, because immunology labs are HARD, man. But some things are WAAAAY of kilter.
  • Genetics. Meanwhile, I requested to see genetics again. Last time we saw them, he had far fewer diagnoses and they were really just looking at liver things, or liver/kidney things, mostly to rule out big things that could affect transplant. They never did any general overview stuff. He has SO MANY diagnoses right now, it’s hard not to wonder if something doesn’t tie a lot of things together. I have a few theories from Dr Google, and I think they’re good theories. We’ll see what they say. Microarray came back normal.
  • Weight/eating. He recently started a new med that has, as a side effect, appetite suppression. He has stopped eating by mouth almost entirely, and he’s lost a not insignificant amount of weight. We’re trying to stop that weight trend by increasing tube feeds, but I think we have to increase them more than I realize, or else something else is going on.
  • CMV He continues to have elevated CMV levels. He’s been on a treatment dose of Valcyte for a month now and the level’s finally gone down. He’ll be on treatment dose for another month, then down to a prophylactic dose for a while. Again. Me = not a Valcyte fan.
  • There’s just something else. Something. He’s struggling. And I don’t know what it is, what we’re missing, how we’re missing it. I’m hoping Genetics, neuropsych, immunology, and Aerodigestive can suss this out. If it doesn’t happen on its own, and if nobody has any major breakthroughs on their own, I’m going to ask if everyone – neuropsych, transplant, genetics, aerodigestive/ent, immunology, and either letters from or conference calls with OT and possibly his BHIS worker – can get in a room together and share ideas. We have so many pieces. So many. How do they fit together?

Feeding Tube Related Issues

So, Teddy has been complaining of pain from his button since fall. When we were in Iowa City for another appointment, I asked if someone from GI could pop over and measure his stoma to see if he needed to size up his button, figuring that would be an easy answer. And as it turned out, his button was quite small and he needed to size up quite a bit.

But the pain didn’t stop. So fast forward to January and I asked again if someone from GI could see us for an actual appointment this time, while we were in town for another appointment. So the same doctor saw us.

And he said that Teddy was faking pain for attention.

And we’d had such a bad day, and this was a the end of that very bad day, and I just didn’t have any “what is your actual problem, you big giant jerk” left in me that day, so I said essentially, “well, that doesn’t seem right, but fine, whatever” and left. (Honestly, the nurse brought me a free parking coupon because that is how beat down I looked. And felt.)

Anyway, so I made an appointment with a GI locally. I didn’t even care which one.  (Not Dimeo, who we’ve seen before, because evidently he won’t see you if you’ve also seen another GI doctor, and I don’t put up with that crap. Sorry, but you don’t get to boss me about how many professional opinions I get to seek.)  Anyway. I see Peds GIs at this point as completely fungible and essentially worthless, so I decided I didn’t care who we saw. We ended up with some gentleman originally from India who works in the Mercy Subspeciality Office.

Miracle of miracles, he listened to what I had to say about the pain, he asked Teddy about the pain. We discussed the possibility of referred pain from whatever’s causing it to hurt when he pees. We discussed the possibility of constipation (and ruled it out). Discussed what he felt was more likely – evidently you can develop abdominal wall pain from a Gtube that doesn’t really have any specific cause. And I can see that, I guess. It seems like the standard GI cop-out answer, but we’ll accept it as a possibility. He discussed imaging – ultrasound to look at abdominal wall for anything out of the ordinary, and another set of scopes. We’re not going to do scopes. It’s not that bad to warrant all that. But we’re going to do ultrasound. And if he needs another CT or another set of scopes for anything ELSE, then we’ll think about adding on looking at this gtube from inside if he’s still having pain. The doctor is also going to check with their surgery clinic to see if they have any thoughts, since surgery is who mostly handles Gtubes at their clinic.

It was nice to be listened to and taken seriously.

Viral Issues in Transplant Recipients

Just an informational post because the husband was having trouble coherently explaining this. 🙂

Solid organ transplant recipients obviously take immune suppressive drugs and that makes them more vulnerable to essentially everything that the immune system would usually fight. Viruses. Bacterial infections. Cancers.

A handful of viruses in particular tend to cause more trouble than others – mostly, the type that hang out in your body. A typical person is exposed to these viruses, often doesn’t develop any symptoms – or just mild cold symptoms, and then the virus lies in their body, dormant, for all time. Sometimes, even in healthy people, the viruses can be reactivated. Transplant recipients, because of the suppressed immune system, are more prone to viral reactivation. Also, if they weren’t exposed pre-transplant (obviously more common in kids than adults), their first encounter with one of these viruses can be troublesome.

This article gives a nice run down, if you’re interested in more detailed information using longer words: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3152274/

We monitor Teddy for three of these viruses on a regular basis (technically, we monitor viral load). Epstein Barr Virus (EBV), Cytomegalovirus (CMV) and BK Virus (which doesn’t actually stand for something – it’s just the initials of the first guy who ever had it).

CMV. I don’t know what to say definitively about Teddy and CMV right now because we’re not entirely sure what’s going on. It looks like his CMV levels go UP every time he has another illness. That’s not ideal, but as long as it comes back down every time, it’s better than not coming down. One of the things that was going on when he was admitted in the spring was that his CMV level was pretty high. Since then, we’ve noticed slight bumps associated with other illnesses. The risks with CMV infection are numerous. Primarily, it can cause rejection. It can also permanently damage the kidney. CMV viremia (for simplicity’s sake, “viremia” would be “high” viral load – what he had in the spring) carries with it a fairly scary mortality rate. CMV is treated by lowering immune suppression and taking a fairly nasty antiviral medication called valgancyclovir, which really does a number on your bone marrow (making you very anemic very quick, at least if you’re Teddy).

EBV. I want to say it was the first year post transplant, T got EBV for the first time. There were a lot of other things going on, and he ended up getting a tonsillectomy and a CT scan – primarily because the biggest risk factor with EBV in an immune suppressed patient is development of PTLD. PTLD – the acronym that strikes fear in the hearts of transplant moms everywhere. PTLD is a pretty aggressive cancer that transplant patients get with an awful mortality rate. It’s pretty rare… but transplant moms are so far past “rare” already – I mean, peds renal transplants are ALSO rare. So at our house, we tend to refer to EBV as “the scary cancer virus.”

BK virus. I know the LEAST about BK because we’ve never had to deal with it. BK virus primarily affects the kidney – causing permanent damage and/or graft loss.