Our Teddy Bear's Journey

Theodore was born with renal failure. This is his story.

Just like Old Times. Sort of.

We saw Otolaryngology yesterday. Yes she does want to scope him. Which I knew she would and I’m not happy but I’m not unhappy. It’s one of those “I don’t want to but it’s probably a good idea” things. Those are a bit harder for me than the “do this or he’ll die” surgeries, though, that’s for sure.

So because of his heart defect, he has to have an in person appointment with anesthesia. It’ll take 45 minutes, they said. I have no idea what they’re going to do. We never had to do it before because we didn’t know about his heart. Harumph.

So, on the schedule between now and April, we have:

  • pre op
  • this procedure
  • post op followup
  • derm appointment
  • transplant clinic
  • Echo and cardiology annual appointment

Yep, 6 trips in  3 months. It’s almost like old times. But it’s still not *every* week, like we did for about 18 months, so it’s hard to complain too much.

But it’s not like old times – before his transplant, he could not be NPO safely. He had to be admitted for an IV if he had to be NPO. This procedure will hopefully be outpatient. :)

So they consider it an “operation.” It’s more anesthesia than he gets for GI scopes, more than you get for ear tubes, but not as much as he got for any of the big surgeries. She’ll basically stick a camera down his windpipe and into his bronchial tubes and see what there is to see. If there’s anything obviously wrong that can be fixed easily, she’ll fix it. If there’s anything obviously wrong that can’t be fixed easily, we’ll schedule another OR date and take care of it. Chances are good she’ll find nothing, and I think I offended her when I said that that was what I was expecting… but it’s just that I’m used to doing lots of invasive testing and STILL HAVING NO ANSWERS.

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Well, it’s not reflux

Teddy was admitted for 24 hours right after Christmas for his pH study. They put a fancy tube down his nose all the way to the top of his stomach and it measured if anything was coming back up, and if anything was, whether it was acid.

He was scheduled for sedation for this, despite the fact that the clinic nurse and I had discussed and agreed when it was scheduled that he didn’t need it. So I refused. They balked. They weren’t sure they’d ever placed a pH probe on a 4 year old without sedation. Would the doctor even agree to this? I held fast, and they eventually relented. It was fine, even though the resident who actually placed the probe had so obviously never done it before. He was fine.

It was a super boring stay, but since no doctors really needed to see us for any reason, we didn’t have to hang around our room much. There was a big snow and ice storm so there were hardly any clinic patients walking around, so we ran up and down the deserted hallways. Derm had a billion cancellations so they agreed to see us while we were there about Teddy’s weird skin nonsense. (Folliculitis infected with staph or strep.)


So we got a call a week or so later. It’s not reflux. He has no reflux. At all.

So… the vomiting, the coughing, the gagging, the increase in coughing and gagging in the mornings, the stridor and the croup…  well, we ruled out the easy and obvious cause. We see ENT again on Thursday to discuss next steps, but I’m 99% certain she’s going to recommend scoping his trachea and I don’t wanna. I mean, we’ll do it because that’s the next step, and the only step really, but I don’t wanna.

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Croup Croup Croup Croup Croup

Well, we’re continuing to chip away (or at least TRYING to chip away) at Teddy’s “other” issues. Persistent diarrhea and vomiting have been back-burnered for the time being. 

Next up: croup. Teddy gets croup ridiculously frequently and it’s usually serious enough to require an ER trip – retractions, low o2, inability to lay down… all the symptoms they say “if your kid has one or more of these with croup, go to the ER.” So far, we’ve nailed down only the fact that this shouldn’t be happening. :)

Last month, we saw ENT for their thoughts. His trachea is slightly small for his age (or was at the time of his last surgery 2 years ago) but not overly small. Not small enough to be causing this.

ENT thinks it could be 1 of 3 things:
– damage from intubation (though no difficulty has ever been noted with intubating him – but he was last intubated under her supervision so there may be a hint of bias there)
– something growing in there (cyst or nodule)
– damage from reflux

She wanted to scope his trachea but I said, hey let’s talk to GI and pursue the reflux thing first, since that’s easiest and I’m in favor of easy.

Today we saw GI. GI… sort of listened to me, but I think had decided to do a PH Probe even before she walked in, which is fine since that’s basically what I wanted to do anyway, but I guess I thought we’d talk about it a little first. She seemed to think it was significant that we took him off the PPI but he’s had croup both on and off the PPI, and coughing both on and off, and vomiting both on and off. The PPI has never seemed to make a bit of difference, even on a dose 3x as much as you’d typically take. Either it’s not reflux, or the PPI isn’t the right drug for him.

So we’re going to do a Ph Probe, which means a day inpatient. That’ll tell us if he’s still refluxing or not and then we can go from there.

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Croup again, and bonus mystery fevers

I realized that I haven’t updated for a while, whoops!

Teddy had a quick hospital stay in August (or maybe early September) with… Croup!  Yes, that winter illness that he gets year round because he’s special that way. :)

In keeping with his standard, he went to sleep with no fever, no runny nose, no signs of a virus at all. And he woke up a few hours later in respiratory distress. This time it was pretty scary bad – he would not lay down, his color was terrible, he tried to vomit but couldn’t. He had stridor (wheezing sound when you try to breathe) and retractions around his ribs and up by his neck (skin goes in instead of out when you try to breathe in). He was drooling. By the time I got fully awake, I threw the pulse ox on him while I got some shoes on and his o2 saturation was around 84. That’s pretty bad. Bad.

We drove to the ER and the nurse checked his o2, it was 82, and we skipped the rest of triage and ran back to a room while she paged respiratory therapy and then started treatment without waiting for the respiratory therapist to come. It was pretty bad.

Poor kid. Two or three treatments later, he finally was able to sleep again, but the ER doctor let us know that there was no chance they were letting us go home. He considered letting us stay in the ER, but thought it likely that he’d need another treatment since he still wasn’t doing fantastic.

So we had our first in-town admission. It was interesting. I’m used to every third person we see knowing Teddy on sight, lol. Nobody there knew us. Nobody there knew anything about kidneys. Or kidney transplants. They never got his meds from the pharmacy (I had Randy bring them from home). Phlebotomy didn’t come up on time for his Prograf level (which is a drug trough and must be drawn before the next dose is given, which must be done at 9).  But the kids and Randy could come hang out with us, which was also weird.

They let us go home the next evening.

But that was his 9th time getting croup – 4 times warranting ER trips, and 5 times treating at home with prednisolone (that we have specifically for this purpose) in less than a year. That’s a lot. That’s a lot even for a transplant kid.

So, we get to add ENT to our team. :)

Hopefully they’ll have an opinion on what the deal is and why he keeps getting croup and what we can do to stop it. A theory’s been floated by nephrology that it’s not even croup, that it’s something that mimics croup, but I suspect we’d need Dr. House to figure it out, lol. I think if it was structural abnormalities, it would have been noticed during one of the 20 odd times he’s been intubated, but maybe not? Who knows.

In the meantime, Teddy’s had Hand Foot and Mouth disease (remarkably mild – or else misdiagnosed, which is a definite possibility) and now mystery low grade fevers that come and go for the last few weeks. Bleh.

And an added extra bonus: I’m now first aid/CPR/AED certified, so if you collapse and lose consciousness, look me up.

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Feeding Therapy – new place

We ended our family’s services at ChildServe in the spring. We’d been unhappy there for a while, but staying was easier than leaving, until we found out that they had no real plans in place to address the spread of contagious illness among their patients. (they neither monitor nor alert immune compromised families when other patients have, for example, chicken pox.)  Not kosher.

We just started back up at Blank, and so far, I’ve been impressed.

ChildServe focused solely on introducing new foods. But none of the success we had with him trying (putting in his mouth and spitting right back out) new foods at ChildServe ever translated to success at home. He didn’t add any new foods to his diet.

The therapist at Blank has noticed, in just 2 sessions, that he has very weak muscles throughout his trunk, core, neck, face, and jaw. Now that he’s no longer really *afraid* of food (which he was for a long while, but he’s gained confidence as he’s learned how to control his gag reflex), he’s lacking the physical development to actually chew it. He can’t move his lips quite right, and he can’t move his tongue quite right. He also is still majorly lacking in confidence with movement, which we’d noticed, and she guessed correctly that he doesn’t enjoy swinging. It all points to the same problem he had when we started PT and that evidently hasn’t gotten entirely better – he doesn’t have a good sense of where his body is in space, and he doesn’t have a good sense of where stuff is in his mouth. This is why sometimes he puts entirely too much food in his mouth – he needs to fill it completely up to figure out where the food is. (but then he can’t swallow it so he spits it out.)

He also has very low confidence in himself, which shows up in ignoring you if you ask him to do something he’s not sure about.

So… we’re sticking with an every other week schedule for right now because he mostly needs to work on muscle development and that’s largely going to be on my shoulders to do at home. We’re starting with animal sounds, lalala, chewy tube, and wheelbarrow exercises. :)

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Clinic Day April 2015

We started the day with nephrology. Teddy’s turning into an absolute GIANT these days, rocking the scale at 14.3 kilos in just a diaper, and a big 37 inches. He’s up a few pounds and an inch and a half over his last appointment. :)  Fatty.

Everything looks good from a nephrology standpoint. He had a few weird things in his labs, but they’re easily explanable by his phenomenal growth.

By the time we got to cardiology, Genna was really revved up and hyper and the doctor was very patient with us. He wants to monitor Teddy’s heart every year for changes, but as it stands right now, his stenosis isn’t too bad. There’s no way to tell how fast it’ll progress, so we’ll just keep an eye on it. It’s most important to keep his blood pressure under control, which obviously we’re already trying to do with nephrology, and have recently found the dose of anti hypertensive that works well for him.

Then we saw dermatology, who said that we probably wouldn’t have trouble with his dishydrotic eczema until fall again, but that I needed to embrace the cream and not fear it. If I hop on using the prescription cream as soon as I notice redness in the fall, it shouldn’t get as bad as it got.

However, he does seem to have “regular” eczema that flares up bad when his seasonal allergies kick in. It’s worse this year than it was last year.

That’s about it.

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Lurie Children’s in Chicago for GI

Catching up a bit here. In March, we went to see a GI doctor in Chicago for more ideas on Teddy’s continued vomiting and diarrhea. We loved him!

First, the appointment started exactly on time and lasted 90 very unhurried minutes. The doctor was familiar with Teddy’s history and conversant on the details. He spoke of his other transplant patients. When he asked what Teddy ate and was told that he gets a blended diet through his gtube, he said, “ok,” rather than, “what? what is that? how do you know how many calories he gets?” and he didn’t seem stupefied by the idea that this meant that his caloric and volume intake would fluctuate on a daily basis. All awesome things.

He decided to delay any action on the diarrhea. It’s improved significantly and doesn’t seem to be causing the same issues with Prograf levels (diarrhea affects how the body absorbs his anti rejection drugs) or bicarb levels (in his blood). He still gets wicked diarrhea from time to time, but it’s not constant.

He suggested the first thing we should try with the vomiting is to feed him less food less often. So I’ve adjusted how we feed him to give him only 120 mL (which he can’t do more than that any more anyway) every 3-4 hours. I don’t start feeding him via his tube until noon because I like to give him some empty-stomach time to feel hunger and to help stimulate him to eat orally, so this means that I can no longer get all the food in during the day. So we give him some food overnight now, as well, mixed with his overnight water (800 mL).

This has significantly reduced (but NOT eliminated) the daytime vomiting, but we’ve exchanged it for occasional night vomiting. It’s still an overall reduction in vomiting, so it’s still an improvement, but I really hate waking up in the middle of the night to catch vomit.

He also suggested adding Ginger, but nephrology nixed that idea immediately.

Also, he wants to proceed with another scope, gave our local (well, Iowa City) GI some specific instructions to follow, and wants to review the report and the biopsies himself. He’s checking for EoE, as well as some malabsorption issues. He said it’s highly unlikely this will turn up anything, but we need to check to make sure.

Finally, he thinks it’s more than likely that Teddy has slow gastric emptying, but would prefer to solve that with changes in feeding schedule rather than drugs. There is one drug (it’s actually an allergy med) that is used for slow emptying as well as an appetite stimulant that he thought might be useful. We’re currently pondering whether to give that a try. Hey, once you’ve got 10 prescriptions, what’s one more, right?

The scope is scheduled for May in Iowa City and will be Teddy’s first time NPO without a hospital admission for IV fluids. I’m supposed to give him mostly pedialyte the day before and get his whole 1 liter in before the NPO starts, even if that means he misses most of his food calories that day.

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Blenderized Diet for Tube Fed People

I wanted to wrap up Feeding Tube Awareness week by talking about what we feed Teddy.

Most people with a feeding tube are fed a liquid formula.

I believed there was a better option.

First, we kept Teddy on breastmilk for as long as possible, and 100% breastmilk until he was 1 year old. Just like his siblings.

Then, I started him on solid foods. Just like his siblings.

Except Teddy got his food blended up into a liquidish form and put directly into his belly.

It’s entirely possible (despite what your doctors may tell you) to feed a person regular foods through their feeding tube.

Some families blend up whatever everyone else is having. I figure, I’m in 100% control of what he eats without having to worry about preferences or flavor… I’m stuffing him with super nutritious foods! He does not eat what the rest of us are eating. :) (and we eat pretty good… I just prefer Teddy to have a more nutrition-focused diet while I’m still in control of it.)

It’s not super hard. It’s not a super lot of work. It’s not dangerous. As long as it’s blended smooth, it’s not going to get stuck in the tube. There are a million reasons doctors have for not recommending a blended diet, but the fact is that most of them just aren’t familiar with it, and the unfamiliar is scary.

“What if I want to try blended foods, but our doctor won’t let us?”

I chafe a little at this question, to be honest, because your doctor doesn’t have that kind of power.

When I made the decision to do this with Teddy, both his doctors and his nutritionist had been pushing formula for months and months. I asked them what they would recommend I feed him if he were eating orally. Would they recommend I put him on formula? Or at this age (1 year), would they recommend I start feeding him CKF-appropriate foods?

Then, after the answer to that was a bunch of stuttering and stammering, I calmly explained that I had decided to start feeding him regular foods through his tube. I didn’t ask. I didn’t open the floor for discussion. I just told them this was what I was going to do. And after a year of working with me… they possibly knew better than to argue, lol.

We went through three nutritionists after that. Bam, Bam, Bam. Then we landed with Susan, our current nutritionist. We were, at that time, her only patient on a blended diet, but she was willing to learn and follow our lead. :) After a lot of initial uncertainty, she now brags about his diet to her fellow nutritionists (including our old ones, lol), and advocates for them to support other families if they show an interest in blended diets.

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Feeding Tube Awareness Week

This week is ALSO Feeding Tube Awareness Week.

I thought I’d take a moment this week to talk about Teddy’s tube.

Why does Teddy have a feeding tube?

Kidney disease often makes you feel nauseous. Perhaps because of this, or perhaps for other reasons, babies with kidney failure often don’t eat enough. Additionally, Teddy had high-output renal failure, which is a completely nonmedical way of saying that he peed out waaaay more than he should, and his body would literally pee itself into dehydration. To counter this, he required more intake than a typical baby – about twice what a typical baby would get, actually. We tried without the tube for about a month (from NICU discharge at 1 month until he was about 2 months), but during that time, he was chronically dehydrated, he was not gaining weight (and ultimately started losing weight), and our whole day revolved around Feeding The Baby. Or Trying To Feed The Baby. And meds. Forcing Meds Into My Baby. Forcing Breastmilk Into My Baby. Constantly constantly constantly stressing because he wasn’t taking in enough. When our doctor called to suggest it was time for a feeding tube, I agreed basically immediately (much to her surprise, lol). I don’t regret for a second.

Sometimes I wish we’d started off with the tube right away… but I’m ultimately glad we tried it without first. We tried. We failed. But we tried.


Why doesn’t Teddy eat orally now? Isn’t everything “fixed” since his transplant?

Teddy never really ate orally. He continued to use a bottle part time for a few months after his gtube was put in, but ultimately came to rely on the tube more and more. Ultimately, I don’t remember when but it was well before he turned 1, he stopped eating orally altogether, and actually developed a pretty intense oral aversion. Kissing him on the lips caused him to gag. Putting toys to his mouth caused him to gag.

He was also vomiting basically continuously during this time. (He’s had very very few days in his life where he hasn’t vomited – and most of the days with no vomits have been days he’s been on TPN – IV nutrition – while letting his gut rest.)

So… Oral Aversion.   Weak Mouth Muscles.     No recollection of how to handle food in his mouth.     Food is legitimately scary to him.

He’s made a LOT of progress since transplant. He is feeling better. He WANTS to eat. He has mostly figured out HOW to eat. He doesn’t gag any more. He puts everything in his mouth. (toys, I mean) But some textures and flavors of foods are still just too much. And some/many foods, he just can’t physically chew and move around in his mouth.

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Congenital Heart Defect Awareness Week

This is CHD Awareness Week.

Why, you might wonder, is Sarah putting this on Teddy’s blog?

That’s right. Remember that routine echocardiogram in January? We didn’t find heart wall thickening like we were looking for. We found a heart defect instead.

Teddy has a heart defect called Bicuspid Aortic Valve. (Sometimes it’s called Bicuspid Aortic Disease, BAD, lol.) Your aortic valve (between your heart and your aorta – the giant artery leading from your heart to the rest of your body) is supposed to be tricuspid – with three flaps. Teddy’s has two flaps. So it doesn’t open all the way (making the valve area narrower) and it doesn’t close all the way, either.

Associated with this, he also has stenosis (narrowing/hardening) of his artery, as well as regurgitation (blood flows backwards). It all means that his heart has to work a lot harder than it should.

There’s also a risk of other effects such as aneurysms in the aorta, in the brain, and in the renal veins, as well as renal cysts (which I find interesting). They think that BAV might be related to an underlying connective tissue disorder, as well. And the risk of aneurysms, as well as aortic dissection, isn’t necessarily related to the degree of complications from the valve. (In other words, a REALLY bad valve doesn’t seem to be any worse than a just sort of bad valve.) Another complication is infective endocarditis (infection of the lining of your heart), which is an interesting one because being a transplant recipient puts him at a higher risk of endocarditis,  as well.

This is a relatively common heart defect. The BIG problem is if you have this defect and don’t know it – a good chunk of athletes who keel over suddenly, for example, are found to have BAV or related issues. If you do know you have it, you can monitor for aneurysm development (at least in the aorta and renal veins), and for the stenosis or valve function to get bad enough to require surgery – hopefully BEFORE something happens.

Chances are good that Teddy will need open heart surgery at some point in his life. Chances are also good that this won’t be during his childhood. Most likely not until he’s a teenager or later.

We won’t see cardiology until April, at which point we should have more information. I think it’s a good sign that they didn’t need to see us immediately. :)

And because I like to give people more information than they probably want, here are a few scholarly articles on the subject:

This is a general overview of Bicuspid Aortic Disease, with a few pictures.


And this:

BAV occurs in 1% to 2% of the population, compared with 0.8% for all other forms of congenital cardiac disease combined. Given that serious complications will develop in ≥33% of patients with BAV, the bicuspid valve may be responsible for more deaths and morbidity than the combined effects of all the other congenital heart defects.7 Although patients with BAV may go undetected or without clinical consequences for a lifetime, the vast majority will require some intervention, most often surgery. The important clinical consequences of BAV disease are valvular stenosis, regurgitation, infective endocarditis, and aortic complications such as dilation and dissection.




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