Lillian's Heart Journey

2nd Heart Transplant Anniversary

noreply@blogger.com (Andrew) — Sun, 24 Mar 2013 17:55:00 +0000

Lillian 2 years to the hour after being in surgery for her transplant prep.

Today is a somewhat emotional day for us today as it is Lillian's 2nd anniversary for getting her heart transplant. We are always excited today to celebrate the life she was given, but our thoughts always return to our donor family and their gift today. We are thankful as Lillian has done extremely well post transplant, with only a few minor issues that are still bothering us. For the most part she is a happy little girl who loves playing with her sisters.

Of the issues still affecting us, the largest would be getting med levels right. Despite our best efforts, Lillian's Cyclosporine levels are continually low, and stay that way no matter how much we raise her dose. This is probably due in part to the... cleansing side effects of some of her other medicine, which is a two part battle we are always fighting. Thankfully, it is about as small of an issue that a transplant patient can have. We also find that her extremities are extremely cold to the touch most of the time, but it appears that this is likely just something that will always affect her.

Lillian's favorite activity - dress up

Last year, I had biopsy results to share, which will have to come later this year as Lillian's biopsy was postponed due to the entire family spending all of February with one of at least 5 distinct colds. We will have her biopsy in early April, leaving us with another week or so to stress and worry about it. Given the concern last year about a tight aorta, there is a decent chance that this will need to be ballooned during her biopsy.

Despite our worries for her biopsy, we feel that Lillian is doing extremely well, and we are eternally grateful for the life she was given. We always consider today to be a bigger day than her birthday, because without today, she wouldn't have a birthday at all. We have our donor angel to thank for that.

12/28/2012

3/08/2013

3/24/2013

A Wild Post Appears

noreply@blogger.com (Andrew) — Wed, 05 Sep 2012 04:56:00 +0000

August 31st

I feel that it is high time for a new post on this blog. I have neglected it for some time for various reasons (buying a house, Lillian doing totally fine), but seeing as it has been almost half a year since I last posted, I figured it was high time for a status update and another picture dump.

April 3rd

Health wise, Lillian has been doing pretty good overall since my last update. We've had a couple flus and colds; as well as huge fluctuations with lab levels, but this is fairly standard stuff post transplant. One item of concern is recent labs indicating decreasing kidney function, but that is likely due to Lillian not drinking enough. Even a never ending supply of chocolate milk doesn't appear to be enough, so we might have to resort to less desirable options like soda. At this point though, both the calories and fluids of that would be appreciated, as she is still chronically underweight.


April 6th - the concept of an Easter egg hunt is overwhelming

Developmentally, we've still a little behind. Lillian's way behind Annabelle in speech (at this age Annabelle was holding fully coherent conversations with us), only just now getting to the point of making attempts at words. Her attempts are still crude, but fully understandable to us. Her vocabulary has recently hugely improved though, so we're hopeful that it's a good sign of things to come. She also got her first sentence out last week - "Ailah, no!", indicating great things to come.

April 7th

Even though her speech mechanics are still quite rudimentary, her custom vocabulary is quite entertaining. For example, while Lillian still does not say "yes", she has a very enthusiastic "I dooo! I dooo! I dooo!" that takes it's place, and hearing it shouted loudly is always entertaining. Dada is still a word for just about everything though. I would estimate her vocabulary to be ~50 words at this point.

April 7th

In other areas, she appears to be catching up. She is a social player at this point, but needs to learn how to share more. She loves playing singing games like "Row Row Row Your Boat" and "Patta-Cake", and has a secret handshake with me that she loves to do at every point she can. She is an enthusiastic reader, although she rarely has the patience to sit through a book that she brings you. With her sisters, it is non stop running and giggling and general revelry. Even when they are playing nice though, there's usually a look in her eyes that indicates she's trying to do something naughty to get a reaction out of them and us.

April 9th

Her personality is really coming into bloom at this point. By looks she's a fairly faint red-head, but has the full attitude of one. Overall she's happy and friendly, but she has an ornery streak a mile wide, and is just as much of a troll as her sisters are. If we so much as dare to cross her, she not only pouts but tries to retaliate with sneaky slapping and general mischievousness (in once such instance, grandma pissed her off for who knows what, and two hours later during lunch, Lillian looked grandma in the eye as she slowly pushed her full plate off the table). She will be a handful for sure when she's older, but we will gladly take her strong, fighting personality, and attribute her success in part to it.

Good progress so far, more than we had ever expected. Here's hoping the rest of the year continues to treat her well.If any Children's staff are still reading, know that we still love you guys, and in some ways missing seeing you all.

April 14th

May 5th

June 8th

July 3rd - pop its did not last long

July 6th

July 13th

July 27th - a late first trip to the beach

August 10th - chilling at the pool in Walla Walla

Lillian's Heart Birthday and First Biopsy

noreply@blogger.com (Andrew) — Sat, 24 Mar 2012 19:57:00 +0000

Today is a huge milestone for us - Lillian's one year anniversary! We are thrilled that Lillian has done so well over this first year. Looking back, we never in our wildest imaginations thought that she would be doing as well today as she is. But we're thankful to be wrong on this one! To anyone who sees her on the street, she looks like a completely normal (if slightly hairy) child, and you would need to see her rapidly fading scars to tell otherwise. I guess the only thing we could wish for at this point is for her to stop communicating via high pitched screams and start talking. But we're certainly happy with the progress she's made. It's hard to believe that this is how she looked a year ago today (at the same time this post was... er... posted).

7-24-11 @ 2:33 PM

vs today:

She's made a staggering amount of progress in the year, matching a normal child's growth more closely than the expected growth of a transplant patient, which is a huge accomplishment. Lillian loves being at home now, and spends as much time as possible making messes (her favorite game is dump the bucket) and playing with her sisters.

Part of the post transplant treatment involves yearly heart tissue biopsies as this is the only way to actually check for rejection. Lillian had her biopsy last week, and I'm thrilled to say she passed with flying colors and no rejection. We do have some concern about her aorta getting a tad smaller around the graft points, but this is typical and just something we will be watching at this point. Overall, we're very pleased.

While today is a very happy day for us, we're also tempered out of concern for our donor family today. A year ago, they gave us a gift that changed our lives, but one that only came at a time of terrible loss for them. While we have not heard from our donor family yet, and at this point don't expect that we will; we are eternally grateful for them, and hope that the idea of their child saving several others helps them with the pain of this time. In thanks to them, please enjoy again Lillian's video covering her hospital stay.

Finally, I think a big picture dump covering her first year is in order. Some you've seen before (especially if you're friends with me on Facebook), but I can't think of a better way to show how she's grown than to show you pictures of her.

3-24-11 - Up early in the morning waiting for transplant.

In the back waiting to go to surgery

A surgical assistant taking Lillian back

3-28-11 4 days post transplant and not in a fantastic mood

3-31-11 - 7 days post transplant and much happier

4-2-11 - Out of ICU and sitting up in a surgical ward

4-14-11 - Discharge day, Lillian with some of her main nurses

Finally! Going home!

4-16-11 - Lillian has been home for 2 days and is starting to get used to it

4-19-11 - Lillian's first (home) sink bath

5-28-11 - Back in the hospital due to a nasty flu/C Diff

6-9-11 - Look at me, finally a supported stander!

6-19-11 - Lillian being goofy

7-2-11 - One of Lillian's first experiences with the sun (delayed due to sensitivity to sun caused by meds). She reacts like any good ginger should!

Enjoying a bath (now one of her favorite things) later that day

7-4-11 - The girls all dressed up for the 4th.

7-23-11 - One of our first big milestones - Lillian's first birthday!

8-6-11 - Lillian playing with cousin Robert

8-8-11 - Back in the bath again (we do bathe her more often than the pictures would suggest)

8-11-11 - Lillian with one of her cardiologists, Dr. Matthew Files. As always, this is one of my favorite pictures that I've ever taken.

9-23-11 - 6 months post transplant

10-23-11 - I'm always thankful for Lillian's willingness to sit still and pose for a photo, especially since Annabelle refuses to ever sit still.

10-28-11 - At a friends for an early Halloween party in one of Mom's funky outfits.

11-17-11 - Another classic pose


11-19-11

11-22-11 - More of Lillian's playful side

11-30-11 - Look at how that scar has healed

12-9-11 - With little sister Ailah

12-21-11 - It's safe to say pudding is only after fruit snacks on Lillian's list of favorite things

12-23-11 - 9 months post transplant with an early Christmas present

1-13-12 - Lillian's first snow day

1-21-12 - Lillian has consistently awesome post shower hair

2-2-12

2-3-12 - Lillian enjoying the winter sun in our yard

Back with cousin Robert again

2-24-12 - Gogurt, yet another of Lillian's favorite things

3-11-12 - At the aquarium with Annabelle. This was exciting for Lillian to say the least, who threw a fit if we spent less than 20 minutes at each and every exhibit/tank

It's been a interesting first year post transplant. Here's hoping for health and adventure in her second year!

Snow Days

noreply@blogger.com (Kathryn) — Thu, 26 Jan 2012 01:28:00 +0000

Not much to report other then that Lils will have her first biopsy on march 16. Depending on the results of that she will either stay for the night at the hospital or go home the very same day. Other than that, Lillian got to play in the snow for the first time last week in Seattle's once-per-year snowfall. She had seen snow previously, but only from the window of a hospital room at Children's.

Christmas 2011

noreply@blogger.com (Kathryn) — Sun, 22 Jan 2012 22:17:00 +0000

This is late, but the girls had a Christmas to remember, visiting with family, having way too many sweets and finally ending with a magic show, courtesy of Joel. We went a little overboard as it was Lillian’s first Christmas home and Ailah’s first Christmas, but the girls had an awesome time, and so did we as a result.

Christmas Eve was Lillian’s 9 month anniversary of transplant. It was very emotional for us as a family. We are so every thankful to our donor family, words cannot express… All Christmas Eve I thought of them and prayed for them.

I know we posted Lillian's video some time ago, but we finally wrote to our donor family last month telling them about Lillian. We settled on a short letter as the longer drafts just didn't feel right. Hopefully someday we will know who our donor angel was.

Lillian with Aunt Kim

Lillian and Annabelle with Aunt Katie

Triple photobomb family picture

The girls with Great Grandpa Boer

Annabelle getting her gift from Aunt Katie and Joel

Annabelle and Katrina

Halloween and Other Pictures

noreply@blogger.com (Andrew) — Mon, 07 Nov 2011 00:17:00 +0000

Mainly pictures today, I'm finally getting some Halloween pictures up, esp since the costumes are so cute. Lillian and the other girls are still doing great, although with a newfound love of candy and trick or treating. Figure next big update will be after the next clinic appointment when we can hopefully put Lillian on twice a day meds and get some more sleep!

Lillian petting a friend's goat

Lillian's Story - Preparing to Contact our Donor Family and an Update

noreply@blogger.com (Andrew) — Fri, 28 Oct 2011 01:34:00 +0000

First of all, I owe all the visitors of this blog (of which there are apparently quite a bit still) an apology for a lack of updates. Lillian has been progressing very well over the past two months, but there is still little to post about. I will at least try to keep pictures updated more frequently though, even if the distance between milestones for Lillian starts to spread out a bit more. I wanted to say thank you to those who still check in on Lillian on a daily basis despite the lack of news, it is extremely encouraging to see you still reading this, and that will hopefully be motivation for me to make more frequent (but shorter) posts.

Kathryn and I feel that an appropriate amount of time has passed at this point that we have begun preparations to contact Lillian's donor family. This is not a task that we take lightly, as we need to balance giving them time to mourn without going so long that they think we have forgotten about them. That of course couldn't be further from the truth; Kathryn and I think about and pray for our donor family on a daily basis, as Lillian is a living reminder of them. But we also understand why they might not want to talk to us immediately or at all, and we wish first of all to be respectful of them and their wishes.

We felt that the best way to start would be to make a video explaining Lillian's story and introducing her to them. The concepts for this video (and indeed our letter as well) have been in our heads for some time, so it is cathartic in many ways to finally commit them to a tangible medium. We hope that they will find some solace in seeing Lillian walking (yes, she has learned a new trick since the last time I posted!), smiling, laughing and playing with her sisters and cousins. While we can never truly put ourselves in their shoes, I honestly believe that if I was put in the same situation as them, that this is exactly what I would want to see. It is little more than a slideshow to be honest; Lillian has gotten quick enough that she's very difficult to photograph now, but it is a visual journey through Lillian's life to this point through our eyes; of her struggles and triumphs, her milestones both positive and life threatening. I hope most of all that it allows the viewer a window into our feelings - the blind fear when she was born; the quiet exhaustion of late nights listening to machines keeping her alive run; the near hopelessness that we felt when there seemed to be no light at the end of the tunnel. But I also hope that you see that we never gave up hope for Lillian, the excitement to come into the hospital every day and see her, whether it was lying and paralyzed or sitting up and giggling. In many ways the feelings were both positive and negative at the same time; such as the feeling of seeing the end of the tunnel on transplant day mixed with the deepest sorrow for our donor family. One final note - please watch this video with sound, the songs that play are the ones that I listened to before giving Lillian to the surgeons; unsure of if I would see her again. They are in many ways her songs.

Now that you've watched the video, I wanted to point out something that we stumbled upon during the creation of the video. While we were making the video and running the test runs, it was striking to see the dramatic difference in the technical quality of the photos both before and after transplant. This is entirely due to the generous gift of a DSLR from a family member, but I found it a fitting metaphor to see the dramatically improved lighting and subdued noise in these pictures that matches how we saw our outlook for Lillian brightened and we came out of the darkest parts of her life during the same time period. There are other subtexts as well, both accidental and intentional that I won't ruin at this time and will let you discover on your own.

Now, to Lillian today. As you read above and saw in the video, Lillian is now walking quite well. She didn't take to it as quickly as Annabelle did, but now that she has, she has been walking around our house with a look on her face that says "Oh yeah, I'm the boss now!" She's still a little unsteady and walks bowlegged, but she's quick and more comfortable every day. And she relishes the fact that Annabelle treats Lillian walking towards her as "Lillian's coming to get me", much to Lillian's enjoyment.

We've also made major strides on eating. While we're still trying to pack the pounds on her (in part by feeding her whole milk mixed with heavy whipping cream), she has gotten slightly less picky and we've discovered a love for beef and other meats that has been extremely useful in getting her to eat. Still struggling though to figure out how to get around her aversions to anything slimy or at a temperature slightly above lukewarm, but she is eating a better and more varied diet (for her at least, any nutritionist would shoot us for giving her straight whipping cream with strawberry flavor; but it's good for her specifically).

On the numbers front - Lillian's labs came back (today in fact) better than they ever have been. Her Cyclosporine levels are slightly on the low side but within range, and those can be attributed to her weight gain (she's around 21.3 now). But her BNP levels are lower than they've ever been (37), and her kidneys are in phenomenal shape due to the amount of liquid she's been drinking. All in all, we're very happy with the numbers, but we will probably change the Cyclosporine dose next visit, hopefully to twice a day if we can finally get her to cross 22 pounds.

In other small news, Lillian has discovered the wonder of a hot shower, we can't even take one now with her hearing it from across the house and doing everything she can to get in, clothes or not. She is getting more vocal on a daily basis (although she is still not talking; she is behind Annabelle, but that is not entirely surprising considering) and we hope she'll start talking soon as her yelling has gotten... shrill. She is in just about every way a normal child who is a teensy bit behind in speech and has to take medicine 3 times a day. Kathryn and I are more than happy with making it this far.

Yes, I know they're not all using the same controllers, we ran out

Update and Tons of Pictures

noreply@blogger.com (Andrew) — Sat, 13 Aug 2011 22:28:00 +0000

Not much going on right now, which is of course, a good thing. Lillian had one small incident where she had to go to the hospital for a suspected broken finger due to her sister smashing her finger in the door, but it turned out to not even be a fracture, so she got lucky on that front. Other than that, Lillian continues to eat and take meds like a champ, and she is getting better (and faster) at crawling, pulling herself up and walking around objects (tables and such) by herself. We hope to have her walking in the next two months.

We have been slowly expanding our horizons with Lillian, going to a family wedding the other day and seeing Lillian's main cardiologist from before the heart failure, who has followed her since as well. She sees her cousins regularly, and if you can't see the scar, looks like a normal kid (so much so that when we went to the ER for the finger, we were congratulated by several staff members for having "normal people issues". Biggest problem Lillian has is the sore finger and dealing with Annabelle, who can be a little too affectionate at times.

In terms of Lillian's health, she does sleep a ton (14 to 15 hours a day) and tends to be a demon when she is tired. Her eyes also appear slightly sunken, but I think that is a side effect of the meds she is on. Finally, we were blessed by a family member with a new camera, so enjoy the great pictures below (as always, click for big version).

One of Annabelle, just to illustrate Lillian's day to day entertainment

BALL!!!!

With cousin Robert (and ball)

Ailah with her great grandma

One of Lillian's favorite toys (a slide).

Slightly fuzzy due to crazy movement, but a priceless expression

A calmer version

Chilling with Mom on the ferry

Ailah sleeping at the wedding

Lillian with Dr. Files

Lillian with her 2nd cousin Kendra

Annabelle dancing with Kendra and Katana

The Biggest Milestone Yet - Lillian's First Birthday

noreply@blogger.com (Andrew) — Sun, 24 Jul 2011 05:34:00 +0000

We've hit what I think is one of our biggest milestones yet - Lillian's first birthday. Getting a heart is the only other major contender, but this day in particular is specially because we were never sure today was going to happen. It was almost surreal seeing Lillian in that seat today, especially since I remember pictures of Annabelle that look exactly like the picture above (note that I'm having problems finding them right now, otherwise I would have posted them; I blame my shoddy picture organizing). It's a huge mental milestone for us to have hit today.

I realize as I write this that I have forgotten to post about another important milestone. I'll give you a chance to figure out what it is first: go look at the pictures of Lillian in the last post, and then look at this one. I'll post one more to help:

If you're still guessing, we've removed her feeding tube entirely. I must admit this isn't entirely with the blessing of Seattle Children's, but we reached a point about 3 or 4 days ago where Lillian simply refused to leave the tube in any more, and we had to choose between antagonizing her by placing it constantly, or just accepting the ineveitable and moving on. So the tube is gone. Lillian is eating and drinking entirely by mouth at this point, although we have to bribe her to drink with apple juice and chocolate milk. And yes, we know she is far too young for this, but when you have medical conditions as serious as Lillian, it's ok to pack on a few extra pounds at this point in her life.

It is both encouraging and difficult to write this post. On the one hand, the last remaining reminder of Lillian's condition that others see are the scars, and those will fade over time. I cannot tell you what a blessing that is, and how unreal it is at this point to be able to walk into the store and not having people walk up asking what is wrong with Lillian because they can no longer tell that anything is wrong. Now we just get looks because we're in the low 20's and we're carting 3 kids around. I can't remember the last time I was able to do that. On the other hand, as I write this, I can't help but think about a Seattle family who lost their 9 year old today due to a 15 car accident earlier in the week, another transplant family in Tennessee who are at the hospital with complications that they don't yet have a cause for, and our own transplant family, who are spending today without their child while we spend today celebrating ours. Yet another family still waits for a donor after spending over a year on the list. I would ask that you keep all 4 families I mentioned in your prayers, as they either still have reason to worry or are in one of the darkest periods in their lives. Even as we finally have a day to celebrate, we have not forgotten you, or the families that we have lost touch with, yet we know are going through similar situations. Today has been a humbling experience.

Lillian with Aunt Kim

I swear Annabelle is smiling in this picture.

More Good News - Major Steps Forward!

noreply@blogger.com (Andrew) — Tue, 05 Jul 2011 00:30:00 +0000

It's been a while since I've had time to post, but we haven't been sitting around in that time! Lillian has made some big steps forward in the last couple weeks. The first that I will mention is eating, as you can see above. Lillian seems to have finally gotten over her aversion to oral food, and has been not only accepting solid food, but demanding it. She started with yogurt melts last week, and has progressed to the point where yesterday alone she added 7 new items to her diet (including mandarin oranges), and seems to be limited only by the lack of variety at the store and the limitation of having no teeth. She also loves graham crackers, flavored puffs, and wheels. Berries and small fruit that we can put in a baby safe sucker are also a hit.

Normally solid food alone would be enough to warrant a post, but Lillian has made more progress than that. She has also gone from begrudgingly taking small amounts of liquid via syringe to taking full on sips from a sippy cup. It's messy, and usually ends up with a 5 foot circle of milk surrounding her, but it's progress none the less. Formula is still a no, and we've all but given up on that, but we hope that if she will take whole milk and eat solids, we will get the OK to remove the feeding tube (nurses reading this - try and plant this idea subliminally in Dr. Law's head). Dr approval is the only thing we would need at this point, on the trajectory she's currently on, we could have her taking everything (meds included) orally and finally remove the feeding tube. With that gone, it would be next to impossible to tell that she isn't a normal baby/toddler, as her mannerisms and behavior otherwise are rapidly catching up to where a healthy baby would be.

But we're not done yet - Lillian has made even more progress lately. Last Saturday (yes, two days ago), Lillian finally figured out how to get over her leg and has started slowly crawling around. The mobility is limited at this point as she is slow and needs major motivation (i.e., food) to get to the point where she will even attempt it, but as she figures it out, we expect that she will soon be powering around the house with her new-found mobility. Lillian has also made major progress with standing. She is only at the point of holding onto a fixed object, but every day, she is more stable and able to stand longer before falling.

With a little more time, she'll develop the eating/drinking/crawling and will be at a normal level of development. It feels so alien to say such a thing, but hoping things go well, it seems like it is finally on the horizon. We consider ourselves lucky to say that, as many families never get to this point. It isn't easy having 3 little girls, especially since they all have personalities like Annabelle (strong and belligerent), but it is a blessing to see their bright eyes and smiles every day. For those wondering about Ailah, she seems to finally be getting over the cold Annabelle gave her weeks ago, which will hopefully help her (and us) sleep a bit better. She is already drinking more than Lillian is (8 ounces every 2 hours compared to Lillian's 5 ounces every 4 hours, plus various snacks). All 3 are developing very quickly and are already getting hard to keep up with.

Clinic Update - Removing Many Meds

noreply@blogger.com (Kathryn) — Thu, 23 Jun 2011 05:09:00 +0000

Lillian had her weekly check up today. We have started the process of taking her off Prednisone, a steroid and one of her anti rejection meds. This drug has several other drugs that are used with it, so by removing it, we can take her off of Ranitidine, Nystatin and Valcyte. That means she's down to 6 meds by the end of the week, a far shot from the 20 some other families are on. Overall, she's doing great, very happy to be home.

Still Returning to Normal

noreply@blogger.com (Kathryn) — Wed, 22 Jun 2011 05:38:00 +0000

It's been about two weeks now since Lillian was discharged from Children's and we're still getting some things back to normal. In terms of numbers, her Cyclosporine (main anti-rejection medicine) has been dangerously low since she was admitted, as low as the mid 70s at one point. Her most recent labs taken Friday are at 227, so much better. We still need to get them a bit higher, to 250 to 300, but it isn't dangerously low any more.

On the other hand, some bad numbers are a bit higher. One way to check for rejection is to check for antibodies in Lillian's blood that match antigens on the donor heart. You might remember that these levels (called redundantly HLA antibodies) were one of our biggest concerns, with the DR-17 antibody levels specifically indicating an immune response to the heart. That level had been undetectable for a while, but we've seen it pop up again, at 20 (under 20 is reported as undetectable, so it is low, but back). She's also tested positive for antibodies to a new antigen though, this time DQ-2, at 41. This is likely part of her body's response to the flu, but it is worriesome if that antibody is introduced to her system, as it matches one of the donor's antigens. There's nothing we can do at this point but monitor, and it doesn't seem like an issue yet, so we're just watching it for now.

Aside from drug and antibody levels though, Lillian seems to be doing much better. She is back to her normal happy self, and seems to be thrilled to be home again. Lillian has labs and an echo tomorrow to make sure everything is still normal.

An Update on the Last Couple of Weeks

noreply@blogger.com (Andrew) — Tue, 07 Jun 2011 06:28:00 +0000

I know I've been relatively quiet in the last couple of weeks, so I apologize to those seeking updates for the silence. Turns out that having a ten month old and a new born keeps you pretty busy.

Last time I posted (over two weeks ago, yikes!), we had discovered that Lillian had a med level that was 2 to 4 times higher than it was supposed to be. At the time, we thought that was the cause of Lillian's issues, but once the rest of the family came down with flu like symptoms, it was fairly obvious that was a factor as well. Lillian managed to get good enough for Children's to discharge her after 4 days, but she was back again 2 days later (Wednesday at this point) due to worsening symptoms.

It turns out that the 1-2 punch of med levels and flu caused her to lose the lining of her stomach (we think, we would have to do a biopsy to tell for sure, and that isn't worth it). Turns out that the med that was high (CellCept/MMF) interferes with the normal growth of the stomach lining. Normally this is just something we have to live with, but at 2 to 4 times the level that it was interfering more than we would like it to, and the flu/whatever she actually had just caused it to get worse.

We ended up spending about two weeks total in the hospital, trying to get Lillian's symptoms under control and get her back up to speed. For most of the stay, she was on Pedialyte so we had to switch her back to normal food, which she couldn't digest, only causing more issues. In the end, we had to switch her back to special formula (Alimentum) which is broken down more than normal formula so it is easier to digest. We finally got her to the point where she was digesting enough for her to come home last Friday, although she continues to show some symptoms. Her demeanor however has returned to normal, so she seems to at least be feeling much better.

Annabelle immediately returning to form upon arriving at Children's

Of course, the MMF level isn't our only concern, as Lillian's lack of digestion also caused her Cyclosporine levels to dip in the 80s (we want her 250 to 300), so now she is sick due to that readjusting to normal. On the bright side, the low levels probably allowed her to fight off the bug a little better. To help her recover, we've switched her from MMF to Azathioprine for now (rejection meds with similar methods of action).

The other girls are doing fine. Ailah is healthy and eating well, and Annabelle turned 3 today. And now, pictures.

Quick update

noreply@blogger.com (Andrew) — Sat, 21 May 2011 17:59:00 +0000

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

noreply@blogger.com (Andrew) — Sat, 21 May 2011 07:10:00 +0000

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

noreply@blogger.com (Andrew) — Sat, 14 May 2011 01:06:00 +0000

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

noreply@blogger.com (Andrew) — Wed, 11 May 2011 15:27:00 +0000

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

noreply@blogger.com (Andrew) — Wed, 11 May 2011 14:51:00 +0000

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

noreply@blogger.com (Andrew) — Fri, 22 Apr 2011 17:53:00 +0000

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

noreply@blogger.com (Andrew) — Mon, 18 Apr 2011 05:52:00 +0000

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

noreply@blogger.com (Andrew) — Thu, 14 Apr 2011 22:28:00 +0000

Almost Ready to Leave

noreply@blogger.com (Andrew) — Thu, 14 Apr 2011 19:42:00 +0000

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

noreply@blogger.com (Andrew) — Wed, 13 Apr 2011 05:31:00 +0000

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

noreply@blogger.com (Andrew) — Sun, 10 Apr 2011 00:33:00 +0000

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

noreply@blogger.com (Andrew) — Thu, 07 Apr 2011 04:39:00 +0000

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.