Published Apr 19, 2013
umcRN, BSN, RN
867 Posts
Have a kiddo right now whose a little bit of a mystery and wondering if anyone else has ever seen something like this. Kiddo has been pretty sick for a few months, is now s/p a heart transplant, was finally on an upward curve a few weeks ago but recently blood pressure bottomed out. Labs are all normal, no signs of infection, nothing growing in cultures however requiring high doses of epi, vaso and dopa to maintain barely acceptable blood pressure (have not been able to rule out rejection at this time as too unstable for cath). However despite being so labile with blood pressures kiddo seems to be feeling so much better. Is awake, alert, answers questions (still slightly sedated and vented), asks for toys (speaks some sign language), the other day I had him sitting up in a chair coloring! Then he wanted to play with my phone and was pushing buttons and "making calls".
We're all scratching our heads a little bit and now trying to get him towards extubating and hoping the BP issues resolve themselves...
Any thoughts or similar experiences?
NotReady4PrimeTime, RN
5 Articles; 7,358 Posts
Very odd... but he's obviously perfusing his brain!
Double-Helix, BSN, RN
3,377 Posts
What other medications is he taking? Which immunosuppresive? Have the doses of his pre-transplant medications been adjusted? How long after transplant did this blood pressure issue start? He certainly doesn't seem septic. Has he had an echo to evaluate cardiac function?
As an aside, this kid is trached, right? I'm scratching my head trying to figure our how a kid could be sitting up and coloring while happily accepting intubation.
brithoover
244 Posts
We had a kid in onco whose pressure bottomed out but was sitting up talking happy as ever. Mom refused to have have her assessed by a doc because she said she was fine. Ended up she was septic (even though all cultures were negative). Went to ICU and died 2 days later.
Oh no, this kiddo was not trached, nasally intubated, never once has tried to pull the tube out (although is restrained if someone isn't sitting right there). Yes, sitting up in a tumble foam chair, coloring & playing with play dough.
Pt is now about 2 months out from transplant. As far as when the pressure issues started, he was septic shortly after surgery and it took a long time to get over that (5weeks), however recently pt had been off pressors for >48 hours when BP started going downhill again. No changes to meds (is on tacro, vangancyclovir, prednisolone, amphotericin - prior infection was fungal, sildenafil, methadone, ativan, precedex and a few other minor meds I can't think of at the moment). All cultures NTD though was treated with broad spectrum abx for about 5 days.
Pt is now actually extubated as of a few days ago and we are weaning pressors slowly as he still appears quite sensitive to them. However when I left the other day he was down to "just" 12 of dopa (off epi & vasopressin).
Still scratching our heads, no idea why he slumped so quickly and worry that it will happen again. Heart function is "great" per echo but can you call it great if you're on so much vasoactive support? One thing he has not had is a cath, he has never been stable enough and no one wants to take a fungal infected child and start shoving catheters into their heart. Blood tests do not reveal rejection.
He has definitely been keeping his brain well perfused through all of this (kid doesn't miss a beat!) although I wish he'd have perfused some other vital organs down south (kidneys).
As an update to this, kiddo couldn't last 12 hours without his beloved vasopressin, and then epi (as well as a total of 30ml/kg fluid), but still waves at anyone who walks by, blows kisses and begs for his favorite book to be read for the one thousandth time (not exaggerating, I have it memorized now)
He sounds like a VAD candidate. We'd have either a Berlin or a Heart Ware in him by now.
Post transplant? and await transplant #2?
Yes indeedy! Although there's a chance that with time on a Berlin he could recover enough function to come off. Seen it once with a kiddie who had chemotherapy-related cardiomyopathy and once in a kiddie post transplant - similar story. I don't think that's an option with Heart Ware though because of how the device is implanted.
Sadly I don't think my hospital is quite there yet with the berlins. We've only been doing them for about a year or so and only as bridge to transplant. Haven't taken anyone off yet. As it is the kid is already going back on the list for a kidney.
We're grasping at a new straw now. Sildenafil toxicity. Anyone heard of it? It's not dialyzed off and it's renally cleared. He's been on it for months, now wondering if that's causing the continued hypotension. Like I said, grasping for straws but would be an "easy" fix.
I suppose sildenafil could be the culprit, but it's actually mostly metabolized by the liver and largely excreted in feces. Severe renal dysfunction on its own wouldn't necessarily cause toxicity. Is his pulmonary hypertension so severe that he needs high doses? (Side note: The FDA has issued a black-box warning for using sildenafil for pediatric patients with pulmonary hypertension but the increased risk of mortality is related to the PAH itself. http://www.fda.gov/Drugs/DrugSafety/ucm317123.htm One of our attendings is a world-class expert in pediatric PAH and he's disputing their findings. He's a firm believer and has used the IV form in select patients.)
Back to Berlins... we've been implanting them for 8 years (24 in total) and explanted 3 of them over the years. If I recall correctly we've only had 2 or 3 fatalities. The biggest cause of morbidity is coagulopathy.