Quick update

Some progress today. Ailah will be discharged today if she passes the car seat challenge but would have to return tomorrow for an evaluation. If she has jaundice, she would be re-admitted. If she doesn't pass the car seat challenge she will have to stay but we would push for a transfer to Children's at that point.

We also got some labs back for Lillian, one of her anti rejection meds (mycophenolate mofitil or CellCept) was 2 to 4 times the target we have, so that is starting to look like the culprit. We would like it to be at 2 to 4, but she was at 8 on the 18th. It is likely that the result that we took yesterday will be even higher. Even with a potential cause, we still need to get another result back before we take her out of isolation. Assuming a c diff culture comes back negative, we can take her out of isolation. If it is positive, we have two possible causes.

Big Day - New Surprises and Re-Admission

Big day today! Kathryn and I welcomed a new addition to our family. Ailah (pronounced eye-lah) Guinevere Boer was born at 6:14 this morning, weighing 6 pounds, 13.2 ounces and measuring 19 inches long. She joins big sisters Lillian (who you all know so well now) and Annabelle. Strange to think of how Lillian is a big sister now, but she is. Life should be even more interesting now. Unfortunately though, not all news from today is good news.

Kathryn's labor with Ailah was extremely quick. She woke me up at 4:45AM, and we got to the hospital around 5:30AM, and Ailah was born at 6:14AM, so not exactly a slow process. The labor was so quick that the things that happen during normal labor, such as having the fluid emptied from the lungs, couldn't happen. For several factors, ranging from potentially swallowing fluid during birth to not having a chance to get it out, Ailah was born with fluid in her lungs. Since she is also technically a preemie, the UW has decided to keep her for 24 hours at least, after which we will evaluate where we are with her.

In terms of concerns we have - oxygen saturation. With fluid in her lungs, it makes it harder for Ailah to send oxygen to her body and they've been running on the low side, low to mid 80s. We wouldn't expect a baby to be at 100 yet, but she should be closer. Add to that a low starting blood sugar, and there was minor cause for concern. Right now we are at a crossroads... assuming that the fluid gets better, we should have her home before the end of the weekend. If it doesn't, they could decide to diagnose her with pneumonia (I have no idea behind the reasoning for this, she does not have an infection) and take her to the NICU. If this occurs, I will probably push for a transfer to Children's because...

Lillian has been readmitted at Children's. Shortly after Ailah was born, Lillian started throwing up repeatedly and constantly. Between 6:30 and 12:30ish, she threw up approximately 6 times. It's not abnormal for Lillian to throw up if she has a full stomach and bowels, but we would expect a one-and-done situation from that, and that didn't happen. She kept throwing up even after she had nothing left in her stomach and started throwing up a yellow substance and then just spit. The more she threw up, the more upset she got (understandably of course), and it got to the point that we were worried enough to take her from Children's.

Once at Children's, Lillian continued to get worse. While her and I were waiting to be seen from 1 to 1:45, she threw up an additional 7 times and was miserable. Clearly dehydrated, she was admitted. Kathryn and I had originally thought that it was a poor reaction to the soap we had to wash her pacifier with at the hospital, but Children's was thinking that it was either a poor reaction to her meds or potentially an infection. For now, we have started to run tests (ruled out rotavirus so far) and have dropped all meds except for one of the anti rejection meds in an attempt to find out what is going on. Aside from ruling out rotavirus, we don't really know what's up. Since infection is the most likely suspect, we are in isolation for now, but other ideas have been thrown around like nec (unlikely considering the sudden onset of symptoms) to a milk intolerance (which makes total sense now that it's been suggested, especially in light of her absolutely losing it every time we offer a bottle). Right now we get to wait for more labs and tests and see if the purging of her system continues.

Sorry if I rambled/made no sense, it's been a long day. Not often that you have two kids in two different hospitals. More pictures now of Ailah for now

During an late false alarm last week, Annabelle demonstrates the proper way to sleep comfortably. It's no wonder so many people have back problems...

Annabelle tries to take a picture of Ailah.

Look at that hair! Early indication is a brunette, we'll see if that stays that way though.

Doctor's Visit Update

It appears my previous post about Lillian's Dr visit has disappeared into the ether, never to be seen again. Said doctor's appointment went very well for Lillian. Her BNP is down to 87 (from as high as 1900 in the past), and her last DR-17 levels were 24 (and they aren't even reported as positive under 20, so this is about as low as she can go). Her Cyclosporine levels were a little low at 199 (we want her at 250 to 300), but we simply think that she's outgrown her dose and it's time to go up. Dr. Kemna said her ECHO looks great, so it's about as good of a checkup that we could ask for.

In terms of things we still need to do - eating, eating, eating! At the risk of sounding like a broken record, Lillian continues to have issues eating, refusing to take a bottle at all right now. Also, in the near future, we hope to take her off her prednisone, a steroid she's on currently. Taking this off allows us to also take off a whole host of othermeds, reducing her total from 9ish to 4ish (I forget the exact numbers at the moment, and I'm in a bit of a rush)! That would be an awesome step to make and it's looking like we'll get to do it ahead of schedule.

The Elusive New Post

First off, sorry about no new posts in such a long time. Turns out having a infant with special needs at home on top of a hyperactive 3 year old consumes a lot of your time, especially when working 50 to 60 hours a week. For those of you still checking on the blog regularly or how want to keep up with it without having to check it every day, I'd highly recommend subscribing at this point. You can either do it through Google's follow blog feature (which to be honest is my prefered method because it lets me know how many people are reading still) or via RSS/Atom, which you can follow by clicking here (RSS) or here (Atom). I've also added handy subscribe by email buttons to the side under followers, and a widget for subscribing via RSS. Are you going "wha?" when I say RSS? You're in luck, because I've gone ahead and Googled something for you, which you can find here. Why should you subscribe? Well, I'd imagine with the event that's going to be happening soon that I will have even less time to update the blog. By subscribing, you get notified when I do update, rather than have to check constantly. *edit* in the process, I forgot to actually change the feed, so if you clicked on anything but the text links above, you got the old feed. This has been rectified, so you should be good to go now.

 Lillian's at home experience has been interesting. Kathryn and I always expected her to be quiet due to being in the hospital for such a long time. However, it looks like our expectations were for naught. Lillian is just as, and in many cases louder and more vocal than Annabelle was at a comparable age.
If you know Annabelle, you know this is no small feat on Lillian's part, and means that any hopes of having quiet time are now lost for what feels like eternity. But if Lillian is even a quarter as funny and quirky as Annabelle is, it will be well worth it.

Even though we couldn't go out for Easter, we still got the girls dressed up in their best dresses. Getting a picture of them both looking at the Camera is another proposition though. I've settled for at least one kid in the picture looking and smiling/not making a horrendous face.

Annabelle decided Lillian's exersaucer needed a couple stuffed animals

In terms of actual medical updates: Lillian continues to have massive issues feeding and is regressing at this point. Even before the transplant she would eat a little, but now she refuses to take any formula by mouth. We have to gravity bolus every ounce she takes which gets old since she's also refusing food of just about any type, and pukes what little she does accept. My gut feeling is this is related to Cyclosporine induced nausea, about which we have little we can do.

On other fronts, our most recent clinic appointment (last Wednesday) revealed a potential issue. While Kathryn's recollection of the issue is inconsistent to say the least, it appeared that Lillian's left ventricle was shrinking. You might remember that this is the ventricle that has caused us so many issues in the first place, so we are puzzled by that development. In terms of action, there's not much we can do now except to watch it, and it will be re-evaluated in a couple hours at her clinic appointment today. Further updates when I have them (hopefully not almost a month this time!)

A Quick Note on the Backend

I'm changing some things on the back end to make it easier for people to keep up to date on the blog. Part of that means changing the feed address, so if you're currently subscribed via RSS/Atom, you should come to the main blog page to update your subscription. If you don't know what RSS/Atom means, don't worry, I haven't changed anything for you. You can also follow the new feed by clicking here.

Doctor Appointments Today

Lillian had a couple doctor appointments earlier today, and another one a bit later from now. The first ones didn't go fantastic, she's lost quite a bit of weight (we think because we're weaning the steroids), so we hope to see that improve soon. Lillian's DR-17 antibody levels have also gone up, to the mid 50s. While that isn't much higher than the level she had at discharge (44), the trend isn't in the way we want. She is going to get the levels redrawn at the later appointment today, and hopefully they will look a little better, as the numbers we have so far are from Monday.

Adjusting to Home Life

I know there are many people who have been waiting for this post for a while now, so I apologize about it's delay. Setting back into normal routines and incorporating new ones for Lillian has been stressful and time consuming, but totally, absolutely worth it. With the exception of one issue which I'll go into later, Lillian has been thriving at home, and so has Annabelle. Annabelle has been so excited to have Lillian home that she has been running around telling people about how we brought Lillian home because she got a new heart.

We've also seen quite the changes in Lillian since arriving at home too. At the hospital, Lillian was extraordinarily quiet and almost never made normal baby noises, the exact opposite of Annabelle. Those of you who know Annabelle know that she is not a silent child by any stretch of the imagination, so we had expected Lillian to at least be similarly noisy. But from our time at the hospital, it looked like this would trait would not carry over to Lillian, as she always seemed reserved and quiet. Granted, when you grow up at the hospital, this could be expected, but Annabelle was always in our room putting on some sort of show or making some enormous racket for Lillian (and the nurses), so it was a surprise to see Lillian so quiet. However, Lillian's made a complete turn around at home. She has taken up Annabelle's old standby of yelling/grunting/shouting/screaming at the top of her lungs for no particular reason other than to make noise, something Kathryn and I welcome and enjoy. Annabelle's personality is so spontaneous and funny (to the point that as soon as I can think of a decent name, I'm starting a Twitter account dedicated to Annabelle's one liners), that we would be thrilled to see Lillian have even a shade of the joy and vitality that Annabelle has.

However, not all of the Lillian's changes at home have been good. We've been struggling to get Lillian to eat orally even after transplant. We had high hopes that the transplant would solve or at least help Lillian's feeding issues, and for a brief time, there was hope in that regard, but she came down from that high point pretty quickly. By the time we were discharged, Lillian was back down to eating only about half a bottle a feed, and even then, requiring a song and dance from us to even convince her to eat. At home, she has refused to take a single bottle at all, and we've had to bolus all her food. This is likely related to the formula change that we did upon discharge, something we have no option about since the formula she was on at the hospital is exclusively available to them (Enfamil, the brand, doesn't even mention it on their website). We're already feeling like we're at our wits end and will likely have to feed her via NG tube for much longer than we had hoped, prolonging the need for the tube. Of course, the fact that she's never allowed to get hungry doesn't help either, as we *have* to feed her every 4 hours.

Besides the feeding issues though, Lillian is overall doing very well. She seems happy to be home, although she certainly has her moments. Some are 'roid rage moments, moments where she clenches her fists, furrows her brow and grunts like she's going to go to war; while others are simply her being a baby. Annabelle is certainly helping her adjust though.

I do think it is harder for Kathryn and I to adjust at this point though. We've been going to Seattle Children's for the past 6 months, to the point that it was our new normal to be there every day. It certainly has been a radical change for us specifically. Children's was in many ways our home, and we loved all the staff members there to the point that they felt like family (especially our main day nurses; Greg (or Greggy as Annabelle called him), Kelly, and Courtney). Many other nurses deserved to be named too, in no particular order (sorry if I spell your names wrong, your name tags are impossible to read) - Jamie, Kara, Kaitlynn, Tonya, Jenn, Sofan, Suzie, Annie, Sam, Angie, Jessica... more names than I can even list, there have been so many of you who have cared for Lillian, granted Annabelle her ridiculous requests and laughed during one of Annabelle's *many* flights from our room. In some senses I feel like I am experiencing a form of Stockholm Syndrome, although I would never describe any of you as keeping us hostages. But we would be lying if we said that we didn't miss you all too. You made our stay tolerable and even at times enjoyable; laughed at our dumb jokes and insane family; our waiting time seem short; supported us beyond simply caring for Lillian and showed us hospitality far above what you were called to do. We feel so blessed to have had all of you as nurses and caregivers, rooting for Lillian and us the entire way. I would write DAISY award recommendations for all of you, but that would then defeat the purpose. Thank you so much for the care and support you have given our family, we would have never made it through this without you (doctors/surgeons/unit coordinators, I haven't forgotten you either, but this paragraph is insane at this point). It is my honest opinion that you're all shining examples of humanity, a yard stick by which other people should aspire to even be judged against. Thank you a million times over!

True to my earlier words, I can't name everyone in this picture, although I recognize everyone in this picture. I apologize for not knowing all of your names, but it simply isn't possible for me. Plus I have the memory of a goldfish. From left to right, unknown, although we saw you constantly, Courtney, Greg, Kara, unknown, but again, saw you constantly, Jessica, and the cleaning lady who always took the time to make Lillian's day brighter, to the point where Lillian would burst out laughing when Lillian saw her. A photo we will forever cherish.

Going Home

Almost Ready to Leave

Lillian has had her PICC line removed. It served us well, lasting since October, well past the length that most lines last. Now we just need the ECHO rejection scores and if they are good we will be heading home in the next couple hours.

The Start of a New Beginning - Preparing to go Home

Pudding!

We've made some good progress in the last couple days, to the point that I'll cut straight to the point and say that barring any terrible lab results or turns for the worse, Lillian will be coming home this Thursday. In terms of the actual progress we've made: Lillian's DR17 levels have dropped to 44, an improvement from the 57 they were at late last week. That number is still higher than we like, but with the trend in the right direction (the first time she tested positive for the antibodies to the DR17 antigen she was at 68, then she dropped to 57 last week and 44 yesterday). Hopefully that number will continue to drop, but right now it is enough for us to begin the discharge process.

Besides the HLA antibody tests, Lillian also gets two rejections scores (they use 2 different methodologies to generate the scores, hence how they can be different) via electrocardiogram. These scores (I have no context for them besides the numbers) were 2 and 4 last time she had an ECHO, but the results we got today from the ECHO yesterday were 2 and 2, so another improvement in that regard.

In terms of the actual discharge process: right now, Kathryn is beginning her 48 hour stay with Lillian. This is basically a change for the hospital to test us with having Lillian's care transferred to us. This policy is good, as it's been over 5 months since we were solely responsible for Lillian's care. We're also going to be running extensive blood tests tomorrow to test her Valganciclovir (anti viral targeting the CMV virus so far contained within the heart), as well as getting another HLA antibody sometime over the next days. She will have lab draws on Thursday, after which we will pull her thigh PICC line. It's going to be weird seeing that go, as that line has been in since October 24th, 2010. Stable and reliable for use.

On the homeward stretch now! Hopefully this one sticks a little bit more than the last one.

Some Mild Improvements

Sorry about the lack of posts recently. We are still at the hospital for now, but we are seeing some improvements in the signs of rejection that Lillian was showing earlier. First of all, the HLA antibody DR 17 came back at 57 today, down from 68 eariler in the week. The levels are still higher than we'd like (ideally she wouldn't even test positive for them), but the trend is in the direction that we need it to be. Lillian has another HLA antibody test on Monday, the results of which will likely determine when we go home.

Her echo rejection scores also were down to 2 and 4, although the 4 is the less reliable test so that is also a good sign. Her BNP is up to 300 today though, but her Cyclosporine levels were also high (300, we shoot for 200 to 250) so that is likely the cause. With any luck the test results will keep improving and we will be home soon.

Little to Report

Lillian only had an ECHO today, so I am unable to report on the HLA DR17 antibody levels yet. This would give us perhaps the clearest view of potential rejection. However, in terms of her ECHO, we have a slight increase in her rejection scores, from 3 and 2 (they use two different methodologies, hence the two numbers) to 4 and 4. This is not a score we want higher, but we generally don't consider scores under 5 rejection. The trend isn't what we would like though. On other fronts, Lillian's BNP was down to the mid 100s, which is a good sign.

When we look for rejection, we look at several categories, including her clinical appearance (awesome), BNP levels (improving), antibody levels (worrysome to say the least) and ECHO rejection scores (getting worse). So a mixed bag on that front. The HLA antigen test tomorrow will be telling.

Lillian got her drainage tube sutures taken out this morning. 2 look great, but one looks like it reopened at best and is possibly infected at worst. We are putting special dressings on it for now, and hopefully it won't turn into anything.

First Signs of Rejection

Mixed day today. On one hand, Lillian had a fantastic day. She ate her entire bottles, she had (and absolutely loved) baby food, and even got her pacer wires out. She's extremely happy, and from the outside, she looks perfect. On the other hand, we have a giant cloud over our heads in terms of the first signs that Lillian's rejecting - and they're strong ones too. This is going to get pretty detailed (and that's about the only picture for today), so for all you skimmers, here's the TL:DR version: Lillian gets labs several times a week to check for signs of rejections. Today one of those labs went from "none" to "medium to high". It's only one lab on one day, but it's a really strong sign. Her body is generating a specific type of immune cell designed to attack one of seven unique targets on her donor heart. These cells appeared in massive numbers out of the blue, indicating that these targets (known as antigens) were present earlier, either through a blood transfusion or the tissue graft she has from her Norwood. This sudden appearance is especially telling - it indicates that she was exposed to this target before, thus her body has already developed the cells. In effect, she has effectively been immunized, only this time what she's been immunized against is her new heart.

Long version:
A blood test drawn last night indicates that Lillian has tested positive for an antibody that targets a specific antigen on the donor heart. For those who aren't in medicine, antigens are molecules or substances that the body sees and tries to kill. It does this by producing antibodies for that antigen. Each antibody is specifically targeted for a unique antigen, and will only attack things with that antigen. This process is what happens when you are vaccinated: you're given a weak/killed version of a virus that your body can identify and kill easily, along the way producing antibodies that target that virus. Then, if you were to ever get that virus again, your body would immediately swarm it with antibodies and be done with it.

Before I proceed any further, I need to give some background. This is basically a science lesson that I'm learning too as I'm typing this so I could be wrong at points (if I am, please correct me, I don't want to give out wrong information.).

from Wikipedia

Every normal human, as part of our chromosomes has something known as the major histocompatibility complex (i.e., a section of genes that plays a large part in your immune system). For humans, this resides in a tiny subsection of chromosome 6, and is called the human leukocyte antigen. We classify these antigens into several types, split into two groups. MHC class 1 are antigens which "display" a sub-string of amino acids on the inside of a cell. Class 1 generally targets viral antigens and works by attracting CD8 positive T-Cells (also known as killer T-Cells) which then kill the cells. MHC class 2 antigens (note that I'm not sure why we call these antigens, or why we say HLA antigens, as that's technically redundant) present antigens from the outside of the cell to T-lymphocytes. These cells stimulate helper cells (called T-helper cells) which in turn induce B-cell production, which in turn (it's about to end, trust me) produce antibodies specific to the antigen. HLA antigens A, B and C are all MHC class 1, while the HLA antigens DP, DM, DOA,DOB,DQ, & DR are all MHC class 2 antigens (there are further sub categories, but that's deep enough on this front)

And back to Lillian. Lillian is tested against the A*, Cw*, B*, DRB1*, DR, and DQB1* types (my understanding of this is murky, my source sheet simply says HLA typing, so I don't actually know what we're testing for). We then simply watch her levels of these antigens, in the form of say 51,53 (her level for DR) or 07,45 (her level for B*). In turn, the donor heart has at least 7 HLA antigens that we've identified, so we specifically look for antibodies for those antigens. Up until now (both before the transplant and after) the tests for these antibodies have been negative (which is good), but today, we saw that she has tested positive for antibodies to the donors DR17 antigen. While I don't have context for the number (it could be number of antibodies per some unit of blood, or some percentage), the number associated with the positive result is 68, which we classify as a medium to high level of antibodies *for that specific antigen*.

Now, to clarify, we would normally expect some level of antibodies in Lillian's blood to the donor's antigens. That's simply the natural reaction of the body to the new organ, and in most cases is relatively benign with the anti rejection medicines. In some cases, the body even produces very small levels of the antibodies which act as protection to the heart, in what is known as accommodation (as opposed to rejection). What's concerning for Lillian though, is that it is too early for those low levels to occur. Normally the body will encounter the new organ's antigens over time and produce antibodies some months after transplant. But these antibodies showed up out of no where and in strong numbers... exactly the response we would expect for antibodies targeting a virus that the body has been immunized against.

On top of antibodies targeting the donor's antigens, Lillian's level of CD4 T-cells has also shot up. CD4 T-cells normally act as an amplifier for T-cell receptors, but they also have a side function of interacting directly with MHC class 2 antigens, which includes the DR17 antigen Lillian has reacted to. (as an aside, CD4 levels are one of the things that we use as an indicator of HIV infection. A normal level is in the range of 500-1200x10⁶/L, while a person would undergo treatment at 350, and be diagnosed with AIDS at 200). We would normally want these levels a little lower than average. Lillian's level (the lab result) is C 52, and while I do not know the context of that number, the medical staff at Seattle Children's considered it elevated.

In a sense, her body is gearing up for war. It is recruiting soldiers (the antibodies) and is rapidly increasing the number of targeting tools (the CD4 levels). Lillian isn't in rejection yet, but this is not exactly an encouraging development. In one sense, she would be in rejection when we notice decreased heart function as a result of the body attacking the heart, so if it comes to that we have caught it extremely early (one of the reasons for doing labs constantly).

It may seem depressing at this point, but it's important to put this in perspective too. This is one blood test which detected mid to high levels of one antibody, so she certainly isn't in rejection yet, and even if she enters it, there are things we can do about it. At the same time, the swift arrival of antibodies indicating a pre "vaccination" to the heart is extremely worrying. Lillian's heart failure doctor said that they see this occasionally. However, when asked about the result of the patients who have this sudden offset of antibodies, his response was "they reject".

Fantastic Day for Lillian

Making great progress! We were hoping Lillian's feeding would improve, and it certainly has today. Lillian took 90mL a bottle, an increase from 70mL yesterday and the most she's ever eaten in one sitting in her life. We need her at around 110mL to 120mL so we're hoping we continue to see progress on that front, but we're very pleased with how she's done so far. Regardless of how much she eats she will go home with the NG tube for meds, but eating properly will expedite the process. Since she did so good, she got a little taste of chocolate pudding today, and loved it. Hopefully this will convince her that it's worth trying other solids as well. On other fronts, she should be getting her pacer wires out whenever the surgeons have time to do it, and should probably get her PICC line out on Friday. This is probably for the better as several of the doctors have expressed a mixture of shock and disbelief that it's still in.

PUDDING!

Lillian is getting happier and happier each day, although harder and harder to entertain. It seems the hospital simply isn't entertaining enough for her at this point. Annabelle still does wonders to cheer Lillian up and make her laugh, but it's easy to tell the hospital has started to grate on Lillian. Ever since the transplant, she simply loses her mind when she needs her vitals taken, so hopefully we can end them soon or go down on the frequency. I think Lillian will benefit immensely from being home, where she can experience the full extent of Annabelle's craziness. Looking like Saturday...

Quiet Weekend For Lillian

Things have been quiet lately, hence the lack of recent posts. At this point, Lillian is pretty much stable from day to day and we rarely make changes except for her meds. On that front, her cyclosporine levels are almost where we need them so we have discontinued all of the other anti rejection meds aside from MMF (mycophenolate mofetil). She will go home on both of those meds as well as about 8 more (I'll post something in depth about that later).

Lillian has continued issues with oral feeding, although we have seen some progress on that front just today. However, we don't know if that will last longer or if it has just been a good day. Time will tell. Right now, we are just re-adjusting to life on the floor, especially now that Lillian has her heart. Some changes obviously due to the transplant, including a much more complicated medication system. On the plus side, I've finally returned the pager (yes, an actual pager) that I've had since October 23rd, so it was good to see that go back. (More pictures later due to posting this from my phone).

Returning to Normalcy - Back to the Floor

Another big day in Lillian's recovery! Lillian got her arterial line out today, so she finally has her hands back, a proposition that she is thrilled with. It's hard being an 8 month old who has recently figured out how to grab things and stuff them in her mouth. With the art line out, she can do that again, as you can see above. That line being out has done wonders to improve her mood, and she's pretty much back to her former self.

Lillian's arm just after having the arterial line removed.

In other news, we learned today that Lillian's new heart is CMV positive. CMV stands for Cytomegalovirus, a virus found in anywhere between 50% to 80% of the American population (less elsewhere) and is generally benign. Most people infected with it don't even know that they have it, as the symptoms of it's infection are basically a sore throat or fever). However, CMV does present significant risk to those who are immunosuppressed like Lillian is. Since Lillian was, and still is CMV negative, we are giving her an anti-viral called Ganciclovir (pronounced gan-sci-clo-veer, brand name Cytovene (indulge me for a sec as I geek out about medical technology and medicine - Ganciclovir works by preventing the viral DNA from incorporating deoxyguanosine triphosphate, otherwise known as dGTP, a major building block of DNA strands. This prevents its DNA chain from getting longer, and (I assume) replicating any further.)), an IV only medicine which will hopefully either help prevent the virus from passing to the rest of Lillian's body or will make Lillian switch to CMV positive with relatively little incidence. If she switches to CMV positive without any major issues, the virus will remain in her T-Cells for the rest of her life (as it does for all who have it) but poses little risk. However, we need to continue the treatment with Ganciclovir as well as doing regular serological evaluations (i.e., blood work to determine if she has antibodies for CMV) to help ensure that any possible passing of the CMV virus is benign.

In a desire to prevent the brains of my non medical-personnel readership from melting, lets change gears a bit. Lillian got discharged from the CICU today! It was fairly obvious that she didn't belong in the ICU when she was doing things like in the picture above - sitting up, laughing and playing like she normally does. She's shown for quite some time that she doesn't belong there, and now that we think we have her heart rhythm issues figured out, it was time to go to the floor.

Just about ready to leave the CICU (I know you can't see Lillian, blame the bed)

She does still have the pacer wires in still just in case, but we don't think we'll need to use them again. In terms of her heart's electrophysiology, we are seeing normal P waves from her donor's sinoatrial node, as well as an occasional extra tossed in by her old SA node. In English, this means we're seeing normal rhythm again, although we do see some occasional blips here and there. However, these blips don't seem to appear to interfere with her blood pressure or her oxygen saturations, so they don't appear to be an issue for now. My guess is that they will either disappear with time or she won't even notice them, although I admit to not knowing much about her long term prognosis in that area.

Since Ganciclovir is the only medicine that requires the PICC line, the line is currently heplocked (medical jargon for a inactive line kept open with the blood thinner Heparin). This means Lillian can wear pants again! Well, maybe not everything as the line is still there, but we can certainly do shorts and more pants like things. We will probably pull the PICC line before final discharge, but for now we need it for the Ganciclovir, but only once every 12 hours. 

It is good returning to the floor again.When we first returned to the hospital for the heart failure, the CICU felt like our home, but after five months on the floor, that has all the vestiges of normality right now. It also lets us do things like sit Lillian down on a mat on the floor and play with her. Of course, the CICU still feels like family as they were only ones who cared for Lillian in the first parts of her life and in the worst parts, but the floor does have a different atmosphere. I guess in the middle of a late paragraph would be a good place to bury my thanks to the CICU staff specifically, but thanks guys and gals, you're all awesome and have helped us through an extremely trying time; caring not just for Lillian but for us as well. We owe you an incredible debt of gratitude that we will probably never be able to sufficiently repay. I just hope I never see you again unless you're visiting Lillian on the floor (which we do love and you're more than welcome to do; just walk down the hallway and you'll spot us) This time on the floor is actually easier than it was than our last time on the floor as our room is even bigger now (bigger room > better view), and Lillian is on no IV pumps at all because of the line being hep-locked. The last time she was on no IV pumps was when it looked like the Milrinone wean would work (we all know how that ended...), so this is another big milestone. It makes it so much easier to do things with her, and we won't have to drag an IV pole around with us when we go on walks in the future.

Springtime at Children's... and Seattle

Again, I find a picture of spring on the walk out of the hospital appropriate here. The dark clouds are passing, the sun is shining and Lillian's new life is starting to bloom. We have returned to what is a normal routine for us, but we will soon have the chance to bring Lillian somewhere foreign and alien... home. Plus, jazz hands have returned with a vengeance now.



(aren't you all amazed at how much I can write about a single day? Just wait til there's not much to post about Lillian and I start going through her historical list of medicines (it's loooooong) and start describing how they all work)

Post Transplant Day 6 - Removing Lines & Goodbye Milrinone

Slow day yesterday, hence no post. A couple things in terms of Lillian sitting up, and doing alright off her pacemaker for about an hour, but relatively minor overall. Lillian had her Milrinone turned down to .3 and ended some of the initial anti-rejection medicines (to recap again, she was given 4 or so anti rejection meds after surgery to last until the long term one, Cyclosporine, was at therapeutic levels. As she approaches that level, the other drugs will be discontinued.) At the moment, I think she's down to just the Cyclosporine and the catchy mycophenolate mofetil (also known as CellCept, which will be discontinued as well very soon) Kathryn did take this video though, so you can see how Lillian is rapidly getting back to how she was before the surgery.

Today, however, was more eventful. One of the first things that happened today is that we were able to turn off Lillian's Milrinone entirely. That's a big step for her as she has been on it for 5 months. It's an even bigger step when you remember that Milrinone was the drug that has kept her alive for the past 5 months. Without that one pump, she would have made it a couple weeks. And with Milrinone off, we don't need as many lines any more (she has 3, a PICC that has been in for over 5 months, a IJ (intra-jugular; i.e., in her neck), and a PIV in her right hand). She also has an arterial line in her left hand, but that is to monitor blood pressure and we don't give meds through it. We also had a much smaller IV pump pole.

It was also finally time to remove a line. There's no need to have 3 access lines if she's not actually on any continuous drips, so we removed the IJ line (largest infection risk). Shortly after removing the IJ line, her PIV happened to fall out as well, so she is down to just the PICC line now. This will remain in for some time as she's still receiving IV medicine, just none of them continuous at this point. Lillian certainly enjoyed having her neck and hand back though.

Lillian showed a little progress with being off her pacemaker yesterday, and showed even better progress today. She had the pacemaker on and sensing, but it never needed to activate. To clarify that a bit, when the pacemaker is sensing, we have it set to a target heart rate, in Lillian's case, 80 bpm. If she were to fall below that rate, the pacemaker would kick in and start pacing her heart keeping her at that level. Lillian was able to keep her heart rate in the 100 - 110 range all day, which is the range we need it to be. Her donor sinoatrial node also continues to improve. 

We did lose some ground on feeding today, but Lillian was extremely fussy and we switched formulas on her. It doesn't help either that we still need her to keep her calorie intake up, but she simply isn't interested in eating as often as we want her to. She is still able to take her anti rejection medicines by mouth though, so

 hopefully the lack of being willing to take a bottle was an issue for today only.

On a slightly related note, thank you to my co-workers, who sent us these beautiful flowers last night. It was quite the pleasant surprise to come home and see these resting on our doorstep. I also want to thank all of you who leave comments for us on the blog and Facebook. I know I'm really bad about responding to them, but we do read them all and love getting them. Last, a couple more pictures from the last two days. 

It's not easy to see except for Kathryn and I, but Lillian is smiling behind her binky.