Update and Tons of Pictures

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

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.