That is the kind of day Kendall had yesterday. It was really and truly awesome.
She was awake for almost 5 straight hours without crashing or melting down too bad. I really don’t think that has ever happened in her whole life! It gave me hope that maybe we CAN get out of the house more this summer without having to rush home for naps! She didn’t need any oxygen (or rather, she didn’t turn colors or do anything else to indicate that she needed it, so I didn’t lug a tank around with us!) I got to see her face all day with no grippies/tape/tubes on it – I didn’t realize that it had been a few weeks since I had!
She was trying to stabilize herself more when cruising along furniture and even took on quick faltering half-step to get from one piece of furniture to another! She learned “duck” in speech therapy, went up to a harder level of beads to string in OT – i mean – really, it was a SPECTACULAR day!
I hope we get to string a few more like that together, and maybe even a couple weeks like that!
I know so many of you are waiting for the update on the cardiology appointment from Tuesday, so, here goes. First – a link to what the main issue is: Pulmonary Hypertension.
A few weeks ago Dr. A recommended that we get in with her cardiologist quicker than our scheduled follow up (in October) in order to more effectively rule out whether we were dealing with PH or not. Given the fact that Kendall has dealt with respiratory symptoms, the open PDA (heart defect), and feeding/growth problems from day one, reading about the symptoms and effects of PH was, understandably, a little scary in that it all made sense. And is a scary thing in itself to think that Kendall’s heart could be working much harder than any of us could even imagine – and what the long term ramifications of all that might mean.
So – I was apprehensive for tuesday’s appointment. Have been for a few weeks, but just couldn’t bring myself to breathe life into those words here. Or even out loud really. I was worried that there WAS a reason she was struggling so much without oxygen, and I was worried that there WASN’T a reason she was struggling so much. And I guess – as much as you can find a happy middle of the road with something like heart issues – we found out that there WAS and WASN’T.
Dr. T (heart doc) reviewed the ECG (getting hooked up to a bunch of electrodes placed all over the body) and the EKG (heart ultrasound) from this visit and compared them to last year’s tests. Her PDA (extra valve in the heart that all developing fetuses have that is supposed to close with a few hours after birth to allow proper blood flow) is still open. It was open last year at almost 6 months, and it has not changed in size at all in one year. In essence, she will likely never be able to close it on her own – although he did say that he HAS seen a few spontaneous closures over the years. Because of this open extra valve, she is having some blood flow backwards and get re-circulated in her system that has NOT gone to her lungs and gotten oxygenated, as all your blood should. This un-oxygenated blood is part of what we’re seeing when she has her “off-color” times/days.
So there is the reason there IS a problem.
BUT – here’s where there ISN’T a problem – she right now doesn’t appear to be in a bad state of hypertension (extra pressure in her heart/lungs). He feels that she probably suffers from “intermittent” pulmonary hypertension, probably during times of extra stress on her body. Which, given her body’s reactions to things, means basically anytime she is slightly off her baseline: any illness, too hot, too cold, retaining fluid, being slightly dehydrated – any of those times can put extra stress on her heart.
The good news is that if it is truly just intermittent, she will probably not even need medication. The super great news is that right now, we don’t even have to talk about surgery to close the hole. Those were my two big worries – more meds and surgery. HOWEVER, that doesn’t mean she is totally out of the woods. Part of his deciding factor in saying no surgery right now is that she has such a hard time with anesthesia (she reacted to one GA with something called “malignant hyperthermia”, meaning she needs lots of crazy precautions taken anytime she is under GA. blah blah blah its a big pain in the booty for everyone involved, mostly her, period the end.) Anyways – the bottom line is that since she is not really failing to thrive anymore, and the heart defect doesn’t appear to be THE MAIN issue that she deals with with all the other issues, its not worth putting her under GA for JUST that. If she has to go under for anything else, we will probably have a discussion about adding the PDA closure to that. It’s a relatively simple procedure as far as heart surgeries go, its really just a matter of her handling the anesthesia.
And even with the meds, it doesn’t mean that she won’t EVER need them. In fact we have a pretty short leash with trying to get her respiratory issues under control before he wants to start the meds. And i know that sounds like a tall order for miss wrench-in-the-plans, but what he means is that if she is still having some of her issues with dropping her sats, higher HR’s, blue colors, breathing too hard/fast over the next week, we will know that she isn’t just dealing with a random virus/illness right now, and that it is time for the meds. So in true Terra (all or nothing) fashion – I took her right off the O2 to ENSURE that we can hit that one week mark! No seriously – I went based on the fact that everything else seemed to be going so well, why not see how she did without the O2. And yesterday she did great.
Last nite when I got her up to get her ready for bed (hook her up to all her tubes and wires and give meds and such), she was crank-o-matic. She seemed to be in pretty bad pain, but she is just losing the ibuprofen or tylenol right back out her G-tube venting bag, so that wasn’t going to be super-helpful. I am assuming at this point that it is related to something with her gut function – since last Saturday when she vomited up some mostly undigested french fries from 12 hours earlier, she has not been real hungry acting at all, and in fact has screamed at me when i tried to give her food. VERY unlike miss K4, but she seems otherwise to be holding her own, so maybe it is just a “normal” picky toddler thing. Of course, in typical K4 fashion, there are other factors playing into it – namely her tummy is hugely distended and is hardly relieved by venting (typically when her tummy is huge its just a lot of air that once we get the air bubbles out, her tummy goes down), as well as what is coming out the other end is just indicative that things aren’t actually moving like they should. A fellow SN mom suggested to me that it might be the overgrowth in her tummy acting up again – and that wouldn’t surprise me really. It’s just that there’s so many theories and factors going into one little body – it’s hard to say. Bottom line – she is going on about the last day she can probably tolerate having less than half her normal intake (of calories – whether that is food she eats or formula she drinks/gets tubed in). And then she will find herself trying to walk around with a backpack strapped on and food running continuously.
Luckily, she has her 18 month “well-child” checkup with Dr. Natalie tomorrow, so I am sure that we can come up with any other thoughts on what may be going on. She is definitely not acting “UTI’-ish” like she was last weekend – so either the double dosing of abx all week helped keep the bugs under control (and probably resulted in the crazy tummy issues we see now), OR – it was never UTI’ish to begin with, and is instead just some other viral silliness throwing her precariously balanced system offline. Either way – I am sure she will tip one way or the other over the next couple of days and then we’ll have our answer.
But for now, we will take yesterday’s spectacularness. And hope that we continue on that path for today. And tomorrow. And maybe even the day after that…
Hope you are all having a spectacular day!