Awaiting biopsy results

Ananya just had her biopsy. Its always disconcerting to let her go for these procedures - I hope she and I never get used to it. The results will be back this evening. Her output from the abdominal drain (JP drain) is the same - it just fills more slowly. I don't know what that means. She's only getting 6 hours of IV fluids now - and gets 50% of what she puts out in the JP drain back in the form of albumin. I continue to hope the longer our stay here extends.. that no other complications develop - like line infections, pneumonias, kidney failure etc. Normally the central lines stay in ~ 3 weeks...we're nearing that mark without an endpoint in sight. Boy, I can't wait to get out of here, and stay out...

Still hoping for a drought

Ananya has been on steroids for 6 days now. She seems so much better than the girl who last thursday had a belly the size of africa and was lying in bed listless, not even able to turn her head from one side to the other. Her belly has shrunk a significant amount, one of the drains in her belly stopped draining, and she's eating, laughing and playing with us. (The steroids make her a little cranky).
That's the good news. Unfortunately the amount of fluid out of her abdomen (the ascites) while diminished is still significant. Enough for the transplant docs to think she may not be responding completely to the steroids, and that there may still be some rejection left to go after. So she's scheduled for a liver biopsy tommorrow to see. If there's rejection - she'll get a t-cell antibody treatment - thymoglobulin or OKT3. Both have reasonable side effects so I hope the biopsy looks good, and that by tommorrow the ascites has reduced to nothing. I know, I know - wishful thinking.

Interestingly it was briefly mentioned to us that Ananya being of Indian origin may have something to do with the rejection she's had. One of the tranpslant docs. looked back at the last 4 transplants done here which involved a caucasian donor and indian recipient. All of them had rejection to a significant degree. 4's a pretty small number but interesting none the less.

So here's to hoping Ananya's belly dries up soon..

New Attending, New Plan

So Starting monday we got a new attending surgeon. What are attendings? Attendings are the senior most physicians in charge of you or your loved one's care. They are the - in the words of our eloquent president - 'The Deciders'. All decisions are supposed to be run by them before anything happens, because they are responsible for your care. There are typically residents, fellows, physician assistants, nurse practitioners as part of the team - but all of them are acting on the attendings behalf.

So the center Ananya had a liver transplant at has 4 pediatric transplant surgeons. Each of the four take turns taking care of the patients in the intensive care unit and on the regular floors. Every week a different attending is on 'service' taking primary care of patients.

This rotating model of service is what you find at a lot of hospitals. So If I see Dr. Jones who is part of a 5 physician group - and I happen to get admitted to the hospital for a pneumonia - I may not necessarily see Dr. Jones - I'll see the physician who's turn it is that particular week to see the hospital patients for the group.

A system that makes sense, right? Well a little side effect if you have the misfortune of being in the hospital long enough is that each particular physician may have a different plan for you. So for example, on Sunday Ananya was thought to be in need of more fluid to jumpstart the kidneys. On Monday, the new attending makes a plan to take fluid off to jumpstart the kidneys. I think it can be a little disconcerting for patients and families when plans shift 180 degrees - b/c it suggest uncertainty among the physicians taking care of you, and that someone's wrong. Of course the standard answer I think we as physicians would give if queried about why the plan is so different than our colleagues (we seldom are asked) is to say that the patients clinical status changes on a day to day basis. So Ananya could have had too little fluid on Sunday, and have too much fluid on Monday - so everyone's right. And so everyone's happy that the patients attempt at clearing the mist has been thwarted.

Anyhow, the new transplant surgeon had a bevy of new ideas - Ananya needed lasix to get fluid off, and we should get an x-ray to follow some fluid that was noted in the lungs. I questioned him to make sure I understood why he thought lasix would help - (a diuretic) - b/c if she didn't have enough fluid - a diuretic would make kidney function worse. Ultimately he said the prograf causes the kidney to clamp down, and that little kids, unlike adults just didn't have the capacity to deal with a lot of fluid. So while you or I could pee out 5 extra liters of fluid babies kidneys need a little help. So fine, I accepted. The X-ray was a harder sell There is good data to suggest cumulative radiation dose in kids will result in a higher rate of cancer later on. Now one x-ray is like a drop of water in terms of radiation dose - but I'm thinking of total aggregate dose she may get in the next 5 years - so I absolutely refuse to have her get studies which won't change the teams management. The attending said he just wanted to follow the lung fluid that was noted prior. I didn't need an x-ray to tell me her lungs had less fluid - she had gone from needing 2 liters of oxygen and saturating 93% to being a 100% on no oxygen. In addition her lung exam certainly ruled out a significant effusion. But I was interested to see whether the right upper lobe of her lung that had been collapsed after intubation was now open - so I agreed to the x-ray.

The x-ray result came back first- the lung fields looked great - no fluid...but the right upper lobe was still collapsed. Bummer. So more chest PT, and some breathing treatments to try to break up junk that may still be in there. After ananya got the lasix she peed like she hadn't peed in a while, and it lasted well after the lasix effect should have passed - the labs the next day did show an improved creatinine from .4 down to .3. Yay!

Ananya's mood improves!

Oh I almost forgot - Ananya is returning to the baby I remember. Happy, cheerful, playful, wilful - which is great to see. For a while she was this listless baby that just layed there and stared. She's still v. deconditioned which surprises the surgeons a little I think. Prior to the transplant she was walking with help, now she won't really even sit up on her own. Her legs are also much skinnier than they were before - so she's going to need some major physical therapy. But honestly, I am surprised at how backward Ishani went functionally. Hopefully it won't take her too long to physically return to herself as well. As a parent - its hard to be patient. Once we get out of the hospital, I'll edit some of these posts with pictures..

Ascites, kidney function and prograf

So last I left off, we had (hopefully) discovered why Ananya was leaking so much fluid from her belly (ascites) - the biopsy done Friday revealed rejection. Steroids were started in the hope of tamping the rejection down, and we were in a wait and see pattern.

Over the weekend Ananya's ascites did definitely get better, though she continues to still have a large amount of ascites. I really really hope that this means that this rejection she's having is steroid responsive. Ideally the ascites would be completely gone, but I would be a lot more worried if the amount of ascites she was having hadn't changed. My other worry (as I had noted in the last post) was supporting her without complications through this episode of massive ascites. The huge leakage of fluid from the vascular system into the belly that occurs makes fluid management difficult. Ananya is not able to drink enough fluid to keep up with the large losses of fluid into the belly. Since the fluid isn't in the blood, the kidneys don't see as much volume and begin to complain - they 'complain' by appropriately not making much urine. The kidney thinks you're in the desert b/c its not seeing any fluid and goes into massive fluid preservation mode. Of course, the truth is the body has a huge amount of fluid - its just in the belly and not in the vascular system. This is called total body volume overload but reduced effective arterial blood volume. Eventually the kidney will start to show some wear and damage. The usual marker used for kidney function is Creatinine. Ishani's creatinine which had been hanging at .2 - .3 went to .4. Her Blood urea nitrogen (BUN) also sky rocketed from a low of 4 to 21!. The ratio of BUN to creatinine is a poor man's measure of dehydration. A high ratio indicates dehydration. Now things are not as easy as this b/c there are multiple other confounders. She is on an antibiotic called Bactrim that has been shown to raise creatinine and the steroids she started in massive doses by itself raises the BUN. So what's going on?

In the face of this myriad of data - one thing was clear - Ishani was simply not making wet diapers. She would have the barest smidgeon of wetness - when she used to have 6-8 v. wet diapers in the past. That was enough to make my plea to the team that she was still dry and may need some extra volume. Sure enough, after receiving extra volume in the afternoon on sunday, Ananya had a nice wet diaper. and continued to have a couple more wet diapers. Now why should I be so concerned? Well Prograf - which is the immune suppressive medicine Ananya will be on for the rest of her life has as a complication renal dysfunction. With older generations of drugs as many as 70% of kids would manifest some renal dysfunction over the long term. The amount of renal dysfunction is related to where your kidney function starts with and the total dose of immune medicine (prograf) you recieve. So I want to make sure the team is on top of making sure she doesn't come out of this massive ascites episode with some renal dysfunction thats significant in the long run. Being an adult MD I'm still not clear about what the significance of a Cr. rise from .2 to .4 is, but I am concerned if she's making small amounts of urine- b/c it suggest a kidney under stress. After all this fluid, etc. what happened to her kidney function - Her Cr. stayed at .4 and her BUN that had made a steady march up came down from 21 to 17. More importantly her wet diapers continue. So I'll continue to research the relevance of the Cr of .4 in a 1 year old, but am a little mollified about the urine output and a BUN going in the right direction.

I'm still hoping this ascites vanishes down to nothing soon....

complications post transplant

Ananya developed massive ascites about 4 days after the liver transplant. Ascites is basically fluid that accumulates in the abdominal cavity. After transplant some fluid is expected - not the amount Ananya had. She weighs 18 pounds and was having about 2 pounds of fluid leaking into her abdomen daily. After transplant 2 drains are left in the abdomen. The main reason is to allow early diagnosis of operative complications like bile leak or bleeding. In this case we escaped those complications but did get a relatively uncommon complication of massive ascites.
Massive ascites is usually a manifestation of end stage liver disease and is not a pretty sight - the belly gets massively distended with fluid and the short term fix is to use a needle
to drain the fluid. Ananya had some drains already so she just kept pouring out of them. Along with this she stopped making urine b/c so much fluid was going into the abdomen. Also her fluid status became difficult to manage - she needed fluid to replace everything being lost in the belly but giving her more fluid means more fluid goes in the belly, the lungs - basically every where but where the fluid needs to be. Ananya was listless, tired and looked awful which made me terrified. The primary caretakers on the floor are physician assistants who are the eyes and ears of the transplant surgeons. They are v. Good but its a tad disconcerting feeling that your daughter is decompensating and there are no doctors around. Suffice it to say, I was highly stressed and wanted decisions made now by a doc before my daughter got worse. Too many times in hospitals I see bad things happen to patients b/c no intervention is made when the patient first starts to demonstrate warning signs. So as my daughters oxygen levels are dropping probably b/c of fluids we're giving her the solution IS NOT to just put her on oxygen.
When the transplant team did get up here Ananya was looking a little better but still cruddy. The team said that ascites sometimes just happens and it would get better on its own. Actually massive ascites does not happen frequently - docs would rather pretend to know what's going on than admit they're not sure. I was not sure if we would be able to support her through this b/c managing fluid status is so difficult and its not difficult to send someone into kidney or respiratory failure. So when told that rejection was a possibility, I inquired about empiric treatment of rejection (w/ steroids) followed by a biopsy later in the day. If the biopsy definitely ruled out rejection the steroids could be stopped. They said it was pretty low prob that it was rejection w/ liver #'s looking so good. So the biopsy happened the next day, ishani continued to leak out but seemed to be holding her own. (still looking cruddy) the results came back at 5 pm the same day - it was rejection. That was yesterday - she's now on steroids and we're hoping she responds to it......

Being in hospitals too long is bad for you

So last I posted Ananya had a fever which was a little bit of a bummer. She got started on some heavy duty antibiotics given her recent surgery and immunosuppression, and risk factors like being on the ventilator for 3.5 days. Interestingly the v. early infections after transplant are probably not related to being immunosuppressed - typically those bugs are bigger players later in the game - I assume b/c it takes some time to whack down your immune system. I was a little peeved Ananya still had her foley catheter (bladder catheter) still in. All these lines and tubes going into the body are great sources of infection - so the sooner those can come out safely the better. The foley had really outlived its usefulness...and I had asked the day before and got some bulls*&*t answer from the nurse about monitoring output. Its surprising to see (now that I'm on the other side) how much the nurses act as gatekeepers of information. There is constant censoring of information going on between the nurses and doctors - the nurses v. much shade their presentations of the patients. Anyway, nurses are probably the most important part of the healthcare team - but if you don't like the answer you're getting from a RN - don't be afraid to kick it up the doctor - just do it in a way that avoids upsetting the nurse..
So I asked about the foley catheter this morning when the transplant team was rounding and they were similarly excited to get the foley out. I also came to find out that Ananya's nurse for the day also was taking care of another post liver transplant kid next door. Which ordinarily would be fine - except this particular poor kid was growing out some super resistant bacteria. I let the team know that I was not happy about the nursing assigments - they said they'd see what they could do but emphasized to the nurse in the meantime about gloves/gowns/hygiene. So by evening shift the assignments had changed, but a last minute switch by the RNs again left us with a nurse covering Ananya and the kid with super resistant bugs. That soured my mood quite a bit. If Ananya does get the same super resistant bug that the other kid has - I may have a cow. One thing is always painfully clear - no one cares more about the welfare of your loved one than you. Seems simple but I think there's a sense when you go to a hospital the caregivers do everything they can. The sad truth is that for the large part of the team - you represent a job...folks will do the best they can - as long as they're not too inconvenienced. So our RN will work her tail off - but the system fails her. you could blame the charge nurse, but she's probably leaned on by administration to optimize patient-nurse rations - so in the end its always the suits/money counters who are to blame. Health care is simply not an industry that does well when the bottom line is the bottom line.

Ok, ok, enough editorializing. Ananya's fever responds to tylenol - it seemed to break early this am - without tylenol. hopefully it stays down. Maybe its due to tat collapsed right upper lobe that we need to open up with some good chest physical therapy...Otherwise liver numbers look good, ultrasounds i'm told of the liver look good as well. which makes me happy despite the little day to day frustrations.

Fever

So Ananya has a fever. Her transplant was Tuesday night - She spiked Saturday early morning about 2:00 am. After extubation she's been doing these worrisome episodes where she breathes really really fast (tachypnea) about 60-80. Earlier this afternoon the team thought she was a little anxious and gave her some fentanyl/ativan which she responded to beautifully. My wife woke me up at about 1:30 am to say that Ishani was getting a cxr b/c she was working so hard - when I came down and saw her - she really did look like she was working hard - nasal flaring - some belly breaths, I listened to her lungs and she sounded junky and she was tachycardic to the 170's - so I did some chest thumping (chest pt) and when I was doing it thought she was a little warm. The Nurse said her axillary temp had been ok 37. something (anything over 38 (100.4) is considered a temp in hospitals) but when we got a rectal temp she was 39.1 (102.4). So that certainly explains the tachycardia and tachypnea. We're giving her some tylenol and she'll need some big gun antibiotics to cover hospital bugs. Her CXR looked unchanged from before - so hopefully its not in the lungs ( though that RUL still remains collapsed). Typically this early post transplant line and catheter (she has a central line - a large iv going into her jugular vein and a catheter in her bladder (foley)) related infections would predominate. though about 20% of these infections could be viral. Her belly is pretty soft and her output from the drains she has in her belly (JP drains) hasn't changed so I doubt she has anything brewing in her belly. The surgeons make lots of connections - one connection that may be prone to leakage and is a more serious complication is when the connection made between the donor livers bile duct and the recipients intestine breaks down. This would result in bile leaking into her abdominal cavity - which is one of the main reasons surgeons leave drains in the cavity. All right, more later! Keeping our fingers crossed that this is a minor bump....