The Biggest Milestone Yet - Lillian's First Birthday

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!

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

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

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

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

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!)