Long Update

Still not much information coming in from Boston.

The GI report and manometry studies have not arrived yet. Tomas' regular GI doctor went ahead and ordered the switch from a G tube to a GJ tube based solely on my telling him that is what the Boston doctor recommended. He said, "Are you sure that is what he said?"
"Yup, not really sure about the why, but he definitely said to switch back to the GJ and add in Periactin."
All I was thinking was I am certain that IS what he said, but dear God please let the report come back saying that as well!
So Tomas got his GJ tube on the 20th and his doctor wanted me to get him to 20mls an hour over the course of a few days. He was doing just fine until this past Sunday when bile started draining from his G tube (the one that goes to the stomach). This is the same problem he had last time he was on the GJ and the reason the surgeon here in NY pulled it and put a straight G in instead. For now, the volume draining is tolerable, and hopefully with the addition of the new motility med it will stay that way. Yesterday, he was doing just fine at 25 mls so his nurse moved him up to 27, and again to 29 around 4pm. By 6pm he was feeling warm and when I took his temperature it was 100.4. That is his protocol temp for needing a blood culture. If he hits 100.4 I am supposed to get him cultured. Only problem is where can you get a blood culture done after 6pm? Right, not really wanting to go there. So  I decide to hook him up to his monitors and re temp in an hour, if it is still high I'll take him in. His heart rate alarm kept going off even though I increased his parameters to 150 and he was laying o his pillow in his crib, and his next temp was 100.2. So not good. Looking like I'll have to call GI and head down to the ER in the city. But as I am ruminating on this and breaking it to my darling husband, Tomas starts hiccuping. "Hmm..."says I, and hook up a drainage bag to his stomach while decreasing his J feeds down to 5mls/h. A few minutes later his HR is down to the 130s. Abut 20 mins later his temp is down to 99.7, a full hour later and his HR is in the 110s and temp is 99.1 and there are 50cc of green bile/bloody mucous in the drain bag. Success! We are staying home. Still waiting on a response back from GI on how to proceed. A phone message I left him this morning got muddled and he thinks Tomas has a fever and want me to get him cultured and even admit him if he is looking puny. I emailed him all the correct info and am just waiting to here back to proceed.

As far as the hematologic part goes it is quite the muddy water picture. After last Monday's lab draw at the pediatrician, Tomas' hemoglobin came back low and the pedi called the GI and hem drs. Hem/onc was noncommittal about whether to transfuse, but because Tomas had been running real fevers (101-103) off and on for a few days prior GI said to bring him in for a 48 hour rule out. I went home, packed and headed into the city for what was supposed to be a 48 hour stay (kind of like Gilligan's Island). When we get to ER his Hgb comes back critical low and he needs an urgent transfusion at 1am. For the love of heaven I do not understand why doctors play this waiting game. We got up to a room a little after the transfusion started and were still on track to leave on Wed. as long as no blood infection showed up. While waiting for things to (not) grow out the floor doctor was able to get a hold of the hematologist in Boston. The good news is that there are no signs of any myleodysplasia in Tomas' bone marrow, bad news is we don't know what is causing the neutropenia or anemia. Good news is the test for Fanconi's anemia came back negative, bad news is the pathologist found markers in the morphology consistent with Schwamann-Diamond syndrome. That test is still pending.
While waiting for his 48 hours to pass, the broviac ruptures and a repair is done at midnight (again - anyone see a pattern here?). While the surgical fellow is repairing the line he notices it has slipped out from its original placement and asks me about it. I tell him we have been following it with xrays and checkups for about a month now, but that it has slipped a bit more tonight, probably from all the messing with it. He says its no good and needs to be replaced. So Tomas goes in for surgery the next day (ahem..the one we were supposed to be going home on); line is pulled and new one is put in. The next day a doctor from the infectious disease team stops by the room to tell me that the bacteria that is growing in Tomas' urine is resistant to most antibiotics, which is concerning, and that his team wants to start following Tomas to try and figure out why he gets so many infections. Be my guest. The next day we get to go home - Friday - way more than 48 hours later.

If you are still reading you'll notice that the hospitalization was not for a bloodstream infection but for a UTI. Tomas has had 4 since July and maybe half a dozen total before that. When we lived in Dallas he saw a urologist and when we moved here they dismissed him. Over the summer some suspicious deposits were found on his kidneys during an ultrasound and kidney doctors went back and forth over whether they were stones or calcium deposits. The nephrologist ordered a urology consult and the urologist said he didn't feel Tomas needed to be followed unless he got another UTI. He's had two since that visit, and during this past admission a new renal ultrasound showed nondebateable kidney stones on both sides as well as some other minor renal issues. Which brought me back to the urologists office today.

He ordered a urodynamics study, which to the best of my understanding is a pressure measurement of bladder contractions and of flow rate through the urethral sphincter. The test will show if Tomas' UTIs are bladder based or not. If he has reflux from the bladder to the kidney it will also show. Tomas did have a VCUG (which is the basic test to show reflux) and it was negative, but the doctor said that about 15% of the time the test is wrong and that the bladder is only refluxing at high pressures - hence the reason for the urodynamics to verify. So 2 things can be seen and then fixed with medication after the study. 1. Bladder is retaining urine for too long - allowing pressure to build and then urine to reflux back up. 2. Urethral sphincter is not functioning properly and his bladder is not emptying all the way, allowing urine to sit in the bladder too long.
If the test should come out normal Tomas is in deeper trouble. So weird to say that! But if the bladder is functioning properly then the stones themselves are the root of the problem and the doctor said he would need to get them removed. The position of where they are (meat of the kidney) would make it difficult. It would involve placing a stent, a ureterscopy, and then eventual removal of the stent. I have gone through this process myself 7 times and can't even fathom my child having to go through it. The doctor is afraid the stones are colonized with bacteria (the one the ID dept. is so worried about) and that they will need to come out even if the urodynamics is abnormal. Best case scenario is abnormal study which leads to meds to make bladder work better which does flush out stones. Worst case scenario is abnormal study which leads to meds which make bladder work better but DO NOT flush out stones - in which case he would still have to go in and get them.
As for what is causing the stones he thinks it is a combination of a medication Tomas is on (Diamox) and dehydration. This is such a mess of an issue. The Diamox is to treat for intracranial hypertension. It is a diuretic and has already led to a major increase in his fluid needs. We have tried taking him off it, or even reducing his dosage, but his spinal pressure shoots right back up to the too high range every time. So other than a shunt there aren't any options - and while neuro mentioned a shunt like it was no big deal I am no where near ready to go there. I can't even begin to imagine the extra infection risk we would be adding in. Meaning Diamox is here to stay.
For dehydration, the urologist wants me to add in 8oz of free water a day. It sounds like such a small amount doesn't it? 8oz=240mls; if I split that up over 10 hours it is only 24mls/h. But remember up in the beginning of my post - that 29mls/h were causing stress responses in Tomas, imagine if I added another 24 on top of it? I honestly don't see how it is going to happen. I do think I can get another 10-15 mls in through his G port every hour (that I am awake) but that only gets us half way there. The other half will likely have to be added into his TPN, which isn't as effective since it only runs 18 hours a day; leaving six hours of too dry Tomas. At some point we may need to move him back to 24 hours but for now we will see how far we can get.

Tomorrow is the appt. with the infectious disease doctor so my brain needs to do a major dump (aka blog post) in order to make room!