Our Teddy Bear's Journey

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

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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PTLD and Tonsils

When Teddy’s nephrologist said a few months ago that she wanted to take out his tonsils at some point because they were large (not because they were enlarged, or growing, or anything like that – just because they were large) and they make her nervous, I was kind of not on board with that. Her concern is PTLD, which I’ve blogged about before. It’s the stuff that haunts my nightmares. They feel Teddy might be at a greater risk for PTLD because of his congenital immunodeficiency, and of course peds transplant recipients are at a higher risk to start with. PTLD can often start in the tonsils, so they (and his lymph nodes) are something we monitor pretty closely.

I sat down to do some research on PTLD and enlarged tonsils and here’s what I found:

http://www.newswise.com/articles/enlarged-tonsils-marker-for-post-transplant-lymphoproliferative-disorder

The goal of this study was “to recognize adenotonsillar enlargement in those children at risk for PTLD, thus allowing for earlier diagnosis and treatment of this organ-threatening and life-threatening disorder.”

Conclusions: Adenotonsillar enlargement in the post-transplant population appears to have greater significance, as a marker for EBV hyperplasia and PTLD. When identified either by symptoms or physical exam, plans for adenotonsillectomy should be emphasized. Unlike healthy children who may be observed for regression, pathologic examination of adenotonsillar tissue is necessary to identify early lymphoproliferative disorders.

So… enlarged tonsils are more significant in post-transplant kids, and really can’t just be observed.

http://ndt.oxfordjournals.org/content/25/7/2089.full

Gist of the first page is that PTLD can be difficult to diagnose because there are so many forms.  “in children, the lymph nodes of the Waldeyer’s ring and tonsils are very common target organs.”  Honestly, I didn’t stick with this article all the way through.

http://www.drninashapiro.com/wp-content/uploads/2011/02/shappdf17.pdf

This was an interesting paper, though the survey included is of little use to us because Teddy is still a mostly nonverbal, nonpotty trained toddler who doesn’t eat orally.

Really, knowing that a biopsy is critically important to early identification of PTLD, and early detection is critical to treatment, is enough for me. It goes against my “God put those parts in there for a reason” ethic, but the reasons for proceeding are compelling.

Now with EBV active in his system, and the tonsils larger than they have been, I totally understand and agree with the plan to get them out asap.

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What’s Working

I feel like I spend a lot of time talking about the bumps and not enough time talking about what’s working. Because it’s the things that aren’t working that occupy my time. But I need to focus a bit more on what IS working. So, let’s talk about what *is* working post-transplant.

– Motor skills. Teddy’s made HUGE progress in motor skills. I mean, he’s walking. He’s almost running. He climbs a wee bit. He still falls down quite a bit, but he’s making great progress.

– Erythropoietin. Teddy’s failed kidneys didn’t make erythropoietin. It’s a hormone that your kidneys make to tell your body to make red blood cells. Kidneys that aren’t working don’t make the hormone. So Teddy received weekly shots of a synthetic version of the hormone. We needed to continue the shots for a month or so after transplant, but were able to stop by about 6 weeks post. Yay! The Epogen really hurts going in, so we were very happy to leave this injection behind. Teddy’s awesome new kidney, Lefty, is making all the erythropoietin he needs.

– Iron storage. Teddy’s never been good at iron. He had to have iron infusions for a while. Post-transplant, he’s been on the max dose of elemental iron for his weight. Last week, we stopped the iron (to try to improve some of his other labs that are bad). This week, his hemoglobin was still right in the target range. Wahoo!

– Calcitrol. Calcitriol’s another hormone made in your kidneys (and your liver, to a much lesser extent). Vitamin D (the vitamin) can’t be used in your body. Your body converts it to the hormone Calcitriol. In your kidneys. (I had this link rattling around in my bookmarks – it talks about Vit D and how it becomes hormonally active.) You can give a person with kidney failure all the sunlight and cod liver oil that you want, but if their kidneys can’t convert the vitamin D to the hormone calcitriol, it won’t do one bit of good. So, Teddy had to take a synthetic version of the hormone calcitriol. (Notably, until the very end before his transplant, when things were really getting out of control, he never needed actual Vitamin D. His Vitamin D level was fine. He had plenty. His body just couldn’t use it, because it was unable to convert the vitamin into the hormone.) Teddy’s awesome new kidney is happily converting the Vitamin D into the useable form, the hormone calcitriol.

– Bone Health. Bone health is kind of complicated. Vitamin D (actually, the active form Calcitriol), Calcium, Parathyroid hormone, phosphorous, and a few other things all play a role. When you have a growing baby whose body can’t convert Vit D into a useable form, who has calcium levels that are variably wonky, who has very high Parathyroid hormone, who pees out his phosphorous… you have a growing baby who is growing kind of crappy bones. Many kidney babies get renal rickets – a form of rickets found only in the renal population. Teddy did not have issues anywhere near that bad – in fact, his bones are pretty OK. But NOW his bones have no reason to not be growing straight and strong. 🙂 (This is a reasonably decent discussion of the role these things play in bone health. I’ve seen better, but I can’t find it.)

– Pee. Teddy’s never had trouble peeing, but he pee was “poor quality.” Yes, his pee was criticized for being subpar. But now he has awesome pee. His awesome new kidney is able to hold on to essential nutrients like sodium, phosphorous, etc., that his old kidneys couldn’t hang on to.

– Filtering Blood. This is of course the big one. Teddy has a kidney that actually filters his blood. It takes the waste products out and keeps the good stuff in. Throughout ALL of the bumps Teddy’s had since transplant, this one has remained rock solid. His kidney has always, always done its main job of cleaning the blood. So, so amazing. Go, Lefty!

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PTLD (or, last week’s Scary Talk)

PTLD scares me the most out of all of the potential post-transplant complications. That and all the other types of cancer he could get (for example, he’s at a much higher risk of skin cancer).

PTLD is Post Transplant Lymphoproliferative Disorder. It’s a type of cancer specific to transplant patients, frequently associated with Epstein-Barr Virus infection. Pediatric patients are at a much higher risk than adult patients (partly or possibly primarily because many peds transplant patients have not been exposed to Epstein-Barr Virus at the time of their transplant, but receive a kidney from an EBV-positive donor, or are exposed to EBV some time in their lives while immunosuppressed).

Then, evidently, because of his hypogammaglobulinemia (basically, parts of his immune system have been pretty low ever since we started looking at them) and possible functional antibody deficiency, he’s actually at an even higher risk for developing PTLD. Yay, him! 🙂

Mostly, we just monitor him closely for symptoms of EBV, and I’m supposed to get very familiar with all of his lymph nodes, and we’ll watch his tonsils closely, and a few other things, and if he gets it, the treatment is to reduce immunosuppression and hope it goes away. 🙂  I need to note – chances of him developing PTLD are still fairly low, in the grand scheme of things. I can’t quantify it – frankly, every study I’ve read has been unable to quantify it even in the general transplant population, let alone factoring in his immune issues.

But it freaks me the H out.

And it’s one of those transplant things that most people just aren’t aware of. Getting a transplant is a GREAT thing, and there are obviously more benefits than drawbacks (I mean, being alive and having a greater than average risk for cancers still beats not being alive, right?), but there are still a lot of scary stuff that come along with it.

 

If you want to read more about PTLD: here, here, and here.

More about hyopgammaglobulinemia: here, here, and here.

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Medical Advice and Being Careful Where You Get It From

So I feel like this goes without saying, but I don’t think it does. Please don’t take medical advice from a blog. Not this blog, not any blog.

I try to always give sources when I’m making statements of fact about things, or when I’m discussing decisions I made, if I made them using medical studies or websites or book, I give the sources so you can do your own research and draw your own conclusions. (and if I ever don’t, please ask!)

I also do a lot of reading of blogs of other medical needs moms, and blogs on health in general, and it’s just appalling to me the amount of bad advice that’s out there, or authoritative statements made with nothing given to back those statements up.

If someone says “X is safe for everyone except if you have liver disease,” don’t just say, “well, my kid doesn’t have liver disease, it must be ok!” Use your internet to work for you – a quick Google of “X counterindications” might show that, in fact, X is not safe if you have liver disease, or kidney disease, or a seizure disorder. Or it could cause miscarriage. Then you’ll be super glad you took the 2 minutes it took me to perform that particular internet search, right?

If someone says “hand sanitizer is evil because it contains triclosan and triclosan is evil,” don’t just take their word for it. Again, a less than 2 minute internet search yielded the information that the FDA doesn’t allow triclosan to be used in leave-on products like hand sanitizer.

If someone says “don’t take fish oil if you’re taking immunosuppressants!” don’t just believe them, unless they publish a source for this statement. (Because studies available at the NIH website directly contradict this.) At the same time, if they said “absolutely take fish oil when you’re on immunosuppressants!” don’t just believe that, either!!

Most of us want our care providers to practice evidence-based medicine. But *you* have a part to play, too – you have to form evidence-based opinions. You cannot base your opinions on what you read on someone’s blog. Even if that person acts like an authority. Even if that person says they’ve done all the research. Even if you’re busy and tired and you think, “hey, this person says they’ve done all the research, I’ll just take their word for it.” Did they provide their sources? If so, look at them. If not, then you have no real reason to believe them in the first place. Be skeptical!! This is your or your child’s health we’re talking about!

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Common Post-Transplant Questions Answered #3

3. Is he all better?

Yes and no.

Teddy’s transplant should make LOTS of things better. We won’t know for sure right away, but it should help with growth, with development, with how he feels. Obviously, it’s adding DECADES to his expected lifespan. When his body’s not putting so much effort into staying alive, it should be able to put more effort into motor skills, language skills, and just simply energy to play.

 

In other ways, No, as difficult as it is to think about it this way, a kidney transplant is a treatment method, it is not a cure. He doesn’t get his transplant and then just go about life like any other kid. Even a year from now, when things have settled down, he’ll still have monthly clinic appointments (forever). We’ll still have to be really careful about germs (forever). We’ll have to avoid crowds, lakes, rivers, streams, some pools, ticks, sick people, some foods, and buffets (forever). He’ll have to avoid certain activities and take medication (forever). He’ll have to have his blood pressure, weight, and temp checked twice a day (forever). He’ll be watched pretty closely (forever).

He’ll still be pretty high-maintainance. Like a Ferrarri. 🙂

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Kidney Transplants

Some videos and photo shows of the process, if you’re interested:

http://www.cbsnews.com/2300-204_162-10012975.html  Slideshow with some graphic images.

http://www.hopkinsmedicine.org/kidneytransplant/stories/documentary.html   video

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Teddy Summary

Someone sent me an email, the gist of which was – I’m new to following Teddy, could you give me a summary of what’s going on with him?

haha.

I said OK, sure, I’ll put it up on the blog.

Here is a current diagnosis list

Vesicoureteric reflux  (urine flows back from his bladder to his kidneys, VUR) (Teddy has stage 4 reflux on one side, and 5 on the other. That’s the worst it can be, and then one step down from the worst. His ureters are large and twisty, rather than narrow and straight. Technically, it’s called hydroureter.)
Reflux nephropathy (kidneys damaged from the VUR)
UPJ obstruction (the kidney that isn’t too small had an obstruction at its outlet, which was fixed with T’s first surgery when he was 8 days old.)
Congenital renal dysplasia (one kidney is small and underdeveloped)
Chronic Kidney Disease, Stage V (this is the big “what’s wrong.” Everything else is either what caused this or what is caused by this. This is also known as kidney failure)
Premature Birth (36 weeks, induced for lack of fluid)
Anemia of chronic kidney failure (his kidneys don’t make the hormone that tells your body to make red blood cells)
Feeding by G-Tube (he is 100% tube fed)
Low Gammaglobulin Level (we’re still uncertain what this means)
IgA deficiency (though this is on his diagnosis list, I’m not sure if it’s true.)
High Blood Pressure (kidneys regulate blood pressure, and T was diagnosed with hypertension when he was something like 6 months old)
Hearing Loss (this has been resolved with the tubes)
Vomiting (ha ha ha)
All sorts of hyper- and hypo- things from his labs. (phosphate, calcium, etc)

 

Here are the surgeries he’s had

12/4/11 Left dismembered pyeloplasty with placement of percutaneous nephrostomy tube and circumcision.

12/7/11 Broviac placement; PD catheter placement
12/21/11 Broviac replacement (malpositioned)
2/2/12 G-tube placement, open procedure, Mic-key button
3/2 Broviac removal (infection)
3/7 Bard PowerPort placement
3/30 PowerPort replaced (malpositioned), PD Catheter replaced (malpositioned)
8/10 Tubes
9/14 PD Cath replaced
10/8 PD Cath revised, tacked down. Port removed.
11/5 PD Cath replaced.
11/14 PC Cath removed

12 surgeries before he turned 1.

Hospital Admissions:

11/26-11/29: birth (4)
3 days at home
12/2-12/23: NICU (22 days)
40 days at home
2/1-2/9: Gtube (9 days)
16 days at home
2/26-3/9: Broviac infection (13)
16 days at home
3/26-4/6: dehydration (12)
95 days at home
7/11-7/17: line infection  (enterococcus) (7)
25 days at home
8/12-8/13: pre-surg, cancelled for cdiff  (2)
22 days at home
9/5-9/18: line infection  (enterococcus and ascintobacter), PD cath replacement  (14)
13 days at home
10/2-10/13: line infection (ascintobacter), port removal, PD cath replacement  (12)
19 days at home
11/3-11/15: dehydration, then catheter replacement, then fungal peritonitis (Candida Parapsilosis), then cath removal. (13)
69 days at home

 

He’s had three bouts with C-Diff, two IVIG infusions, some 3-4 iron infusions.

Last time I saw a number, his estimated GFR (a measure of kidney function) was 8 (that’s pretty low).

That’s it in a nutshell. Happy to answer questions.

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Feeding Teddy part 2

You may recall my experiencing some level of frustration with our nutritionist over Teddy’s diet. Add formula. Switch to formula. Formula, formula, formula. What about food, since he’s certainly old enough for food? Sure, I can feed him food orally, but anything he eats orally is just “extra” on top of his minimum amount of breastmilk. We don’t count it.  And when I asked over and over about putting food thru the tube, the answer was somewhat the same. First, that’s crazy and second, sure, you can put some jarred baby food through his tube, she supposed, but it would be extra. It wouldn’t count.

Now, I love pumping. (ha. hahahahahahahaha.) Ok, I’m happy to pump for my Teddy Bear. Willing, at least. But I’m not going to do this forever. Also, I want Teddy to be treated like a normal kid as much as possible. And normal 1 year olds are consuming food. Whether he eats orally or not isn’t relevant. I want to move him to a blenderized whole foods diet for his daytime feeds.

I’d prefer to do this with the supervision and approval (and maybe even help?) of our nutritionist. And even though the first nutritionist moved and we have a new one, it doesn’t seem that I’m really going to get the input that I was looking for. And I got tired of waiting. I pushed on this for over 3 months. Since the day I first syringed some peas into his tube in the hospital. (ok, he was on dialysis, manual passes, and we had largely finished our training, so that would put it at… Octoberish.)

So whatever. I’ll do it without professional help.

I am not an idiot and I have managed to a) keep Teddy as healthy as can be expected  b) not kill my other two kids  c) never poison anyone. I made myself an absolutely kick ass spreadsheet (which I have tinkered with endlessly – I heart spreadsheets). I’ve come up with a bizarrely wide variety of recipes. I’m having fun.

I spent the time between October and the end of 2012 introducing Teddy to various foods one at a time like you do with regular babies, and now I’m making blends, while still introducing new foods.

I started with replacing the calories, fat, protein, and general nutritional profile of 1/8 of his daily breastmilk intake with food blends. That went pretty well.  In December, the nutritionist wanted me to give him an extra 100-200 calories every day, as well. She suggested meat, though since we had JUST cut down his protein supplement because his BUN was reaching unacceptable levels, I decided to just stick with coconut oil for an extra 120 calories every day (per T) (and no potassium).

Then I got mastitis and my supply TANKED and I got a little desperate and I bumped him to 1/4 of his daily calories coming from food. On the same day that I tried a new blend with chicken (a new food) and oatmeal (which I was iffy on whether he tolerates it). Idiotic, yes. Desperate, remember? He puked all day. He stopped pooping. His blood pressure went up. I declared myself a failure. Vomiting with kidney kids can quickly get serious, because they dehydrate so easily, so I was genuinely worried that I had actually managed to screw him up and kill off his kidney and we’d end up on hemodialysis because I was an idiot.

I backed off, moved away from chicken and oatmeal, and went back to 1/8. He pooped again a few days later, showed a FANTASTIC weight gain, and his labs on the 31st looked great. So I didn’t screw him up too terribly much.

I had PLANNED to tell everyone what I had been doing at our appt on the 31st. And then I just didn’t. I kind of just didn’t (a bit like Cinderella in Into the Woods, I decided not to decide), and I kind of decided to wait until we had a bit more experience – and success – under our belts.

Fast forward to present day, I’m regularly doing 1/4 of his daily totals as BD (half of his daytime intake). I want to move him to all breastmilk overnights and all food days, but I’m not going to rush it and I’m not going to go there without approval from our team. We’re going to hold steady where we are and continue to add more foods to his diet to give him a better nutrient profile.

Next week, I’m going to think about switching from my current method (3-4 different blends each week, switching daily) to just making one large and full of goodness blend and giving him the same thing every day. Ultimately, he’ll get just as much variety, without my having to make so many different recipes each week.

Because he can’t tolerate bolus feeds, I make Teddy’s blends in our BlendTec and I make them VERY smooth and runnier than I would if I were pushing them in. (“pushing them in” = using a syringe and pushing food into his tube.) I modify his bags following the instructions here, though I don’t use the gravity set up, I actually just use his pump. The new nutritionist was telling me he’d HAVE to tolerate bolus feeds since you can’t do BD through the pump, but that’s really not true at all. Even the instructions that came with the pump talk about how to do BD with the pump, and the Food Safety instructions she gave me talked about it, too. Just use an ice pack to keep it cool, and you’re good.

Re, our first nutritionist. Honestly, the more I think about it, the madder I am that I wasn’t more aggressive with her when Teddy was still nursing a little. I think it was ultimately a losing battle, but our nutritionist certainly didn’t help, and almost certainly hindered. Anything he nursed was “extra” and didn’t count towards his daily totals. So I had to stuff him with more breastmilk than any other kid his size would have ever eaten voluntarily, and then if he wanted to nurse on top of that, it was fine. Yeah – like he was going to want to nurse on a full stomach! Ridiculous. I mean, seriously. You could not have PLANNED to sabotage a breastfeeding relationship any more thoroughly if you were trying.

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