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).