Week one on RTA meds - still pinching myself.

I am still pinching myself that we have a diagnosis. I am so grateful that the reason for JAM's failure to thrive has been discovered.  It will take a while for his system to regulate and stabilize, but we are already starting to see some benefits. Given that RTA could be life threatening or cause rickets, we are so very happy that this kidney disease was diagnosed so early (relatively speaking) in his life.

Down sides:

While we are in the early stages of this, he will have to get blood tests regularly to see what the meds are doing to his Ph levels. First test comes next week. Then it's every few weeks until he's at a good level, then at least every three months for the first year on meds.  That said, it's better than if he had diabetes, so all told, not too bad. Too bad that he is a very very difficult poke. In order to get a good draw on the first try, we have to drive to the children's hospital 45 minutes away. Even then, they struggle to get blood on the first try. [Last time we had a blood draw the phlebotomist took one look at J, remembered him from the previous draw, and immediately called for backup.]

While I thought solving his weight gain issues would gain us a free pass to take out his g-tube finally (he hasn't really needed it for over six months), the nephrologist recommends we keep it in, at least until we are sure he'll take the drug orally. He mentioned that he knew a kid who had a g-tube given to him specifically for RTA -- he wouldn't tolerate the drug orally, and they had to give it to him somehow, so they put him through surgery, gave him a g-tube, and administered the drug through the g-tube. I thought this was extreme until I learned how much medicine J has to take daily. It's only one medicine, but he gets a lot of it three times a day, at least for now. There's no sneaking it in to one swallow, because too much has to be given for that. We haven't even tried to give it orally yet. It's super sweet (smelling at least), and so I could see how this salt-loving boy would utterly reject it, even if we tried to hide it in applesauce or some other yummy treat. We'll wait until he's a bit better at eating and until things are stable with his Ph levels before we try giving him the drug orally. And then we'll wait several months more before we remove his feeding tube. (Sigh.) It's okay, it's kind of nice to have as a back-up.

How he's tolerating it:

At first the drug seemed to make him nauseous. Either that or he had a headache (a distinct possibility since E and I were both fighting a bug that gave us a huge headache). So maybe it wasn't the drug. But he wasn't feeling that great.

Now he's doing a lot better.  He's still more irritable than normal, but he's turning a corner. We're only five days in, but we're starting to see small changes.

Up sides:

The biggest has been in his gut. [Okay, TMI warning here -- you may want to skip this paragraph.] He would never ever have normal bowel movements. He'd go to both extremes -- sometimes at the same time. Theoretically (from an RTA standpoint) this might be because muscle weakness impacts bowel function, as your bowels are one big muscle. Coordinating that muscle when you are weak is hard. Plus, he has extra scarring from his NEC (bowel surgery). This issue has resolved itself already. It's bizarre. We kept waiting for constipation to hit again, but it hasn't. So, we know his muscles are  now working better. He's stronger.  Maybe we can stop being followed by GI in the next year.

His breathing seems slightly improved. His energy level as well. Not enough to know for sure that it's better, but he's going in the right direction.

All in all, I'm thrilled.

I still haven't found any good resources for RTA. And I have no idea why J has it. A friend says you can get it from sniffing too much glue (there's some truth to this). Not sure where Jonathan's gotten all that glue to sniff, maybe he's been hiding under his crib mattress.

Update on Failure to Thrive - RTA.

Remember this post from about a month ago?  How I was wishing I could change the Ph in my boy's blood and get him to suddenly and magically start gaining weight, stop vomiting, have regular bowel movements, stop having his arms buckle underneath him when crawling, and stop being lethargic?

Well, turns out we can change his Ph.

His blood is too acidic. He has renal tubular acidosis. It's rare. Even among all my doctor and nurse friends I don't know anyone (other than the nephrologist) who knows anyone who has been diagnosed with RTA.

But he has it. And we can do something about it. Finally, an answer.

We are rejoicing.

He isn't tolerating the medicine well yet, but we're hopeful.

Downside: there are lots more blood tests in his future. Poor babe.

Becoming the patient - How being a preemie parent changed my perspective on healthcare.

Being a preemie parent -- or the parent of any medically fragile, medically complex child -- changes your perspective on things.

As of yesterday morning we'd been to the ER (emergency room) a total of three times this summer. Twice for J's severe illnesses, once for his older sister's broken arm.  That's more than the previous three years combined.

Yesterday we added one more ER visit and I became the patient. It was a dumb injury. I tripped on an uneven sidewalk. I was carrying JAM. I protected his head and body with my arms, so I didn't have anything to stop my fall. My knee fell on another piece of uneven concrete that acted like a knife, cutting a 5 cm gap in my knee. I knew immediately that J was scared and I was more badly injured than just a scrape I could walk off. Thankfully, my brother had just turned the corner down the same street and saw us fall. He acted as a first responder and took me to the ER, my sister-in-law took the other kids home, including an utterly unharmed Jonathan. I did my job well. My baby was safe. (I will wear my scar as a badge of honor.)

The admitting nurse asked to look at the injury. I lifted the towel. She looked shocked and immediately covered up my wound again. Yes, I know, that there is nearly entirely exposed kneecap. That's how I knew I needed to go to the ER. That one isn't going to heal itself. She told me it was one of the worst lacerations she'd seen. I thought "no, this isn't THAT bad. Looks pretty clean to me. Besides, I'm not in any danger here. It just hurts."

Yep, being a preemie parent changed me. I'm a bit tougher. After all, it's a knee. It's not NEC.

Ways being a preemie parent changed my experience in the ER:

1. They asked me my date of birth at registration. I gave Jonathan's DoB and then realized I was older than two.

2. It ain't a "medical emergency" unless you need to be sedated or put on oxygen. This here was just an inconvenience.

3. I automatically interpret numbers on the machines, it's second nature now. "Oh good. My blood pressure is pretty close to normal and my blood oxygen only a little lower than usual, but still in a normal range, so I am actually handling this fall pretty well. Guess I'm not going into much shock."

4. I understand the medical wards better. I knew why they got me in fast - I was the scariest one in the waiting room - and I suspected that if the child with burns had come in ten seconds earlier, my wait would have been about eight minutes longer. And I wouldn't have minded a bit.

5. In general I have a lot more patience and am a lot less demanding -- I know what to expect now.  They're not going to give me sprite until they see if I need surgery, so there's no sense in asking until I'm no longer NPO.

6. At the same time, I also know when I need to ask questions and how to answer their questions.  I feel like I'm part of the medical team now, from the moment I walk in. Being dramatic doesn't do anyone good. Unless you are afraid you're bleeding out on the sidewalk with only your infant around. Then, by all means, one should scream as loud as possible (and I did). But once they're attending to you? Keep to the facts and be honest about how you're feeling.

7. I notice cleanliness. Yes, nurses, I saw when you didn't put on hand sanitizer as you walked in the room. I also notice when nurses put on gloves and then rummage around opening and shutting eight different drawers trying to get the bandage for my hand ready. Yes, I know you're protecting yourself -- but if you'd first gotten out the bandage and set it aside and THEN put on the gloves, you'd be helping protect ME, too. I don't think I would have noticed before. Yes, I know this isn't a sterile procedure, but we're in the ER, and there are lots of germs around. And this here is an open wound. (They were a lot more careful and absolutely sterile when dealing with my knee -- as that was a surgery and not just a band-aid.)

8. I've seen a lot of medical procedures over the last two years. And the short of it is, I'm used to blood and needles. As such, I thought it was kinda fun to watch myself get stitched up with two layers of stitches. It's all super interesting, once you get over the shock.

The one thing that hasn't changed: I still make really bad jokes when I'm in pain. I inherited that from my father.

"This wound is 5 cm."
"Oh, good, I'm half way to having this baby then."

...

Really?  I really thought THAT was an appropriate response?

-----------------------

For those of you with medically complex kids: how has parenting changed your perspective on medicine and doctor's offices?

Thriving - of growing a garden and growing a child

Exploring sand

J is happy. He loves his sisters, books, balls and sandboxes. He's growing taller and looks more like a toddler. He is getting healthy after a round of a tummy bug and has snapped back into being his normal toddler self.

But his energy level is and always has been low. He used to quake when he woke up from low blood sugar. He has finally -- after eight months of trying -- gained a pound. But one pound in eight months isn't so great for a one year old. Sometimes he'll be crawling and his arms will buckle beneath him. He's weak.

They say this is "failure to thrive," and I did and do hate that term. But maybe they're right.

I had a garden last year. It was on clay, alkaline soil. I tried to plant blueberries, and if you have read this blog from the beginning, you remember what a disaster that was. No matter what I would do, my garden would not thrive.

Check out my garden this year.

I've never been able to grow squash. My kale has always been wimpy. Tomatoes and peas worked, but barely. And I've never attempted kohlrabi, knowing it wouldn't work in my shady, clay soil.

So what changed?

I moved. I got a new garden, one with sandy-to-loamy soil. One where plants can thrive and the Ph levels are perfect (or easily amendable).

And suddenly plants that would die or barely hang on before started growing like crazy.  I love my new garden.




















Jonathan got a new early intervention teacher. She met him for the first time last week. Her sage advice to me? We've got to get him gaining weight. If he doesn't have the energy level to sustain a normal toddler lifestyle, he'll continue to fall behind on gross motor skills and overall development.

No, duh.

Least inspirational speech ever.

He loves to eat.

I wish I could get new, better soil for Jonathan. I wish there were a way that he would suddenly be able to start to thrive, to grow, to gain weight.  I want answers because I want him to become all that he can be.

I learned early on that planting peppers outside before the last frost was a recipe for short, small, probably dead pepper plants. Wait until it's warmer, until they can grow well.  Otherwise your crop will be decimated.

Jonathan didn't wait until it was time. We're so glad he hung on, but I had hoped that by now the
"thriving" part of toddlerhood would have started.

So, I'm praying hard for a diagnosis. We've checked most everything. We've spent the last eight months searching for answers and we've nearly reached the end of the line. Our last hope (before we diagnose him with "wimpy white boy syndrome" - in other words, "sorry, lady, we can't do anything for your son") is acidosis.  That is to say, we hope all of this -- his fast breathing, his failure to thrive, his low energy level, chronic constipation, and the presence of kidney stones a year ago -- we hope all of this is because his Ph levels are off. If he has renal tubular acidosis, everything that is confusing about J (other than his low zinc levels) could be explained by this. If this is it, it means his kidneys aren't ridding the body of acid like they should, and it's throwing his system for a loop.


And like my new soil, acidosis is easily amended. He'd take a supplement. It would help change the ph of his body. That's it. And then he'd start to thrive. I nearly cry thinking about it. How amazing, how wonderful. If this were it?  If this were it, it would be an answer to prayer.

The nephrologist has run one set of tests. It came back confusing and inconclusive because he was still battling a tummy bug and was dehydrated.  So we're running another set of tests in a week.

Please, please be RTA.

Otherwise, we'll just be glad you hung on, and we won't expect you to produce as much as your other pepper peers.


P.S. As J's dad is quick to point out, there is one JAM oddity that cannot be explained by acidosis. That is, his PURE AWESOMENESS. Seriously, this kid is cute!

"The Gift of Presence"

Recently this article was published. I go deep here, hit the spiritual elements of our journey, and land on one solid truth: sometimes what we need more than anything else is someone to walk with us in pain and joy, in hurt and triumph.  I learned this through three simple words, spoken in to my soul. I was still pregnant, crying out to God, and more than anything else I wanted to hear, "this baby will live" or "don't worry." But I didn't hear that. Instead, my soul's ears heard a steadying and merciful, "I am here."

If you want to read more, the article, "The Gift of Presence" is here (published by Banner magazine).

You can also read more about elements of the story as published in the article in these posts from this blog:

• About crying out & giving over, when night fell
• Spiritual support on the cusp of viability
• About giving him his name, Jonathan
• Heart surgery
• Gut surgery
• Surgery just before due date
• Coming home

As I read through this article today my first thought was "ugh! a typo!"
And then I got feedback from friends, positive at that, and I thought perhaps I should re-read it.  A second reading reminded me just how blessed and surrounded we were. We were exhausted. I soaked up every ounce of those blessings and rang them out on Jonathan and then greedily looked for more blessings to soak in. Steve plodded on, focusing on the next step and acting as a strong pole for me to lean on when I crumbled, and J -- JAM knew no different. This is how life had always been. We needed and coveted your prayers and gifts, and sometimes we were sure we were completely burned out, but somehow we were sustained. Thank you. Thank you so much. Two years out almost, and it is still hard to remember, hard to believe how our world so quickly changed. But in it all, we were supported. Thanks.

Happy Birthday Virginia Apgar.

If I could have tea with anyone from the 20th century, I'd pick Virginia Apgar. Analytical and compassionate, she is my hero.

I discovered Virginia Apgar when I was sitting in on a medically themed Adventures in Ideas seminar at the University of North Carolina seven or eight years ago. In so many ways she paved the foundation for neonatal and maternal fetal medicine practices today. And she did so in an era when female medical doctors were uncommon. I wish I'd known her.

In many ways, thanks to her work, my son lives.

She saved babies with numbers.  For more on her work and my absolute adoration for her, see this recent post on Hand to Hold's blog.

Happy Birthday, Virginia Apgar. Born 105 years ago tomorrow.

No GI answers yet - but glad for summer

Jonathan is now back to his October weight. That's a loss of half a pound more. He's been pretty sick (including temps so high he ended up in the ER last weekend) so this explains some of it, but we were hoping for answers now.  No weight gain in eight months is scary in a one year old.

JAM continues to grow taller and looks more and more like a little boy every day, but he is still stuck at an easy-to-toss 15 to 16 pounds. It makes Steve's nightly romp with the kids easy and fun, but it has cause me to gain worry weight --the weight that he should have gained by now plus some.  A new gym membership and new FANTASTIC running group in town should help with both the worry and the weight on my end, but neither get to the root of JAM's sticky problems.

We had hoped that the procedure with GI would answer all our questions. It didn't. No poor absorption issues spotted. Nothing.

So we are back to confused.

In other news, he has perfected his hand clapping and has stood for up to five seconds on his own. He has even taken his first step - although it was more like a graceful fall than a step. He might just be walking by two!

And finally, I ended my day job last week. This led to a slight crisis of identity. This week I woke up to a dream where I'd just graduated from college but had no job and no idea what I wanted to do with my future. I hadn't even applied to grad school, but I thought to myself that, since I couldn't figure out what else to do with my life, maybe that's what I should be doing.

Thankfully the morning light reminded me that my job was set before me, and it was a good and worthwhile position. I even got a training manual from his new speech therapist in our new town later that same morning. She says that since he's entering the program late, I need to read chapters one through six by next Wednesday. And just like that, I'm back to school! Training how to be an advocate and teacher for JAM.

By the way, my office view couldn't be better. I get to be outside whenever the weather allows, and I can work from a sunny window inside. Plus, there's no dress code, and my solo client is one of the easiest going, cutest people in the world.  I'm blessed.  I just hope I can also bless him.