It's Been A While

Aurelia continues to grow and thrive and is doing fantastically well, but I am getting ahead of myself. Our last update was in July, just after her heart surgery. She recovered well and we were home a week after the surgery, though there were a few difficulites along the way. She is a quick healer though. We were not supposed to give her belly time for a full two weeks after her surgery, but that didn't stop her from giving herself belly time. Prior to the surgery, she had been working on crawling (a very slow form of it that includes lots of rolling), and she wasn't willing to let a little thing like open heart surgery get in the way of her practice! The doctors told us that she would self-regulate and if it hurt her to be on her belly, she wouldn't do it. Well, either she's one tough cookie - or it really didn't hurt. She spent as much time as possible on her belly!

We were relieved that we did not have to turn the distractors in her jaw any more. That finished just prior to her heart surgery. Now the plan was to wait and allow her jawbone to heal. We returned to CHOP in late August for a follow-up sleep study and I was happy that we were able to go home the next day (quite different than the previous one that ended with a 2-week stay in the hospital!). We later got the results of the sleep study that still showed mild-moderate sleep apnea, but it was GREATLY improved. Which means that the mandibular distractors worked and that the pain and frustration of moving her jaw was worth it. There was a chance that she would have gone through all of that and it wouldn't have worked. We were ecstatic to hear that the procedure was a success! Aurelia was scheduled to get the distractors taken out in September, just 2 days before her first birthday.

While we waited for that surgery date, she continued with her weekly therapies. She has a Physical Therapist (Miss Marjorie) and a Developmental Therapist (Miss Lynne), who she was been working with since February. In July, we added Occupational Therapy (with Miss Judi), which she also gets weekly. She makes improvements daily. In July, while she was in the hospital, she began sitting up, unassisted. And now, just a couple of months later, she is army crawling and is able to get anywhere that she wants to go. Over the last week or so, she began to get herself into a sitting position from a laying position, all by herself! Aurelia really is one determined little girl, which is wonderful to see such fierce strength and will. Madison is truly her biggest cheerleader ~ she jumps and shouts out "you did it Aurelia! I'm so proud of you!" each time that Aurelia sits up!

Another addition to Aurelia's daily routine has been the introduction to FOOD. Yes, actual baby food! In August, she had an appointment with the feeding team and while they are cautious, they are allowing us to give Aurelia an ounce of stage-2 baby food one time a day. The fear is that she is a risk for aspiration, so we have to be very careful. Also, because her core isn't that strong yet, she tires out very quickly. An ounce of food isn't a whole lot, but it is about all that she can handle at this point in time. The introduction of food has been a slow one. Aurelia had been grabbing at our plates/forks/anything left close by for months. And she brings everything to her mouth, but as we discovered when we first gave her baby food, she didn't quite know what to do with the food when she got it into her mouth. Like any baby getting their first tastes, she spit most of it out. She is extremely enthusiastic though and grabs the spoon from us with almost every bite. From the first time that I gave her some applesauce, she has tried to feed herself. And I'm happy to say that now, nearly 3 1/2 months later, most of the food actually gets swallowed. AND, she's been enjoying little star puffs, so she is slowly getting the idea of how food and her mouth are supposed to work. We will reevaluate all of this once she gets her cleft palate repaired. We will likely have to start all over because she'll have a whole new mouth, which will work a bit differently. In the meantime, we soldier on with trying different tastes and different textures. In fact, we just added a FOURTH weekly therapy. She will now be seeing Miss Cindy, a Speech Therapist, on a weekly basis too. It is great to have so many services available to us! In PA, she gets all of these therapies at our house (until age 3), which is so covenient. I try to be home for all of them, but it is great that she can still have therapy at home when I can't be there (it is good for our new nanny, Kaitlyn, to see what goes on in the therapies too).

On to the surgeries... As I mentioned above, Aurelia was supposed to get the distractors out on September 21st. But, our little girl caught a cold and it was decided to postpone the surgery. No need to put any additional risks on the surgery. We had a to wait a full month, but on October 24th, the distractors FINALLY came out! Aurelia healed quickly and we were only in the hospital for one night. What a miracle worker she is!

And now we are anticipating her FINAL surgery, cleft palate repair, for next week, November 9th. This will be surgery #12 and we are so excited that it will soon be over. Again, it should be a one night hospital stay. Some kids stay longer, but since Aurelia is primarilly tube-fed, we don't have to worry about getting her back on an eating schedule. I'm sure that her mouth will be sore. In fact, one of our therapists noticed this week that Aurelia's tongue seems to be tied, so there may be an additional part to her surgery. I called her plastic surgeon, a really great guy named Dr. Jesse Taylor, and he'll take a look at her when we come in for the surgery and decide whether or not he can do that repair at that time too. I still can't believe that none of us noticed her tongue before because it is now so obvious to me. Either way, we are very hopeful that once her cleft is repaired, we'll be able to increase her oral feedings.

I hope to write more next week and try to keep this blog a bit more active with entries. I will also add some photos later, but I really wanted to get you caught up on what has been happening since July. As I said, our girl is growing and thriving and doing really well. We are so proud of the work that she is doing and with how far she has come.

Needed a break

Hi folks. Sorry for the delay in posting these last few days, but I needed a break from writing. It's been a stressful day or two and I just didn't have the wherewithal to sit down at the computer and type something out. Aurelia is stable and in a similar place to where she was a few days ago. She still is terribly gassy, has needed medicine to help move her bowels, and is still watched closely for the amount she urinates. Her fevers spiked a few nights in a row, but last night it was not quite so dramatic a rise.

In terms of pain Aurelia is on oxycodone and tylenol, if she needs the additional relief. Sleep has been difficult for her, in part because of the gas, but also because she is so nosy. Our girl has to inspect everything going on in and everyone entering the pod. We hope to be out of intensive care soon so we have a shot at a private room. Her recovery should go well there.

Aurelia's breathing is still assisted by 1/4 liter of oxygen, but that is what she came in on, and she is a terribly difficult ween. We hope to start weening her off morphine and diazepam again soon, and maybe her oxygen, too. She has to relax, more, though, and breathe deeply for that to happen.

Not much to report other than that. Jillian went home for a night and returned with my in-laws and Madison as we approach this next phase in the step-down unit. I'll be returning to Gettysburg for a few days to be with the cats tomorrow morning sometime, I think. Well, thanks as always for all your support and prayers. Hope you are all well!

PS - I fixed the comments - everyone should be able to comment now.

THUNK...OW!!!!

That's right, folks, that's the sound I make when the other shoe hits me square in the noggin. So far the other shoe appears to be something light, a ballet slipper perhaps, as opposed to a steel-toed work boot, but this is still a bit of a disheartening morning for me.

After a lovely afternoon visit yesterday from Grandma and Grandpa Dittrich, Aurelia endured a difficult evening. Her belly swelled substantially, she had trouble peeing, pooping, and she became magnificently gassy. Her pain medications failed to alleviate much discomfort and sleep eluded her for much of the night. With two fever spikes up to 101.8 we guaranteed our stay here in the CICU for at least another day.

The team took a urine analysis, blood analysis, and cultures, looking for infections that might be lurking somewhere unseen. This morning the urine analysis came back clean and Aurelia's white blood cell count is reasonable. Thankfully her fever is down after some ibuprofen and she moved her bowels and peed substantially a little while ago.

Currently, our best guess is that her intestinal track failed to start up to full speed after her surgery yesterday (remember that comment I made about her kidney seeming to have started up?), which meant that she had limited function, but not enough to tolerate full feeds. So all day yesterday, when we ran full feeds, Aurelia's body failed to fully process her food. This resulted in the gas, constipation, lack of urine, and painful belly swelling. To help her the team stopped last night's overnight feed and we will start a Pedialite feed later today to see if things are back up and running. We will keep you all posted.

Oh good news! I forgot to mention that pulmonary no longer believes Aurelia needs her inhalers. Yay!!!!

Just a little fluid

Good news continues unabated this morning as Aurelia's improvement continues. All of her tubes and wires, other than the IV, are out and gone, her normal feeds have resumed, other than morphine and diazepam she is off pain medication, and her breathing has not gotten any worse. Our only concern thus far is a stubborn bit of fluid in her lungs. We have started chest PT and increased her diuretics in an attempt to drain her lungs more so we can ween her off oxygen.

A liberal estimate has us leaving the intensive care unit today and maybe moving into the step-down unit tomorrow. I cannot believe Aurelia is doing so well! I don't want to jinx all this progress, but I confess to feeling a bit of disbelief regarding her progress. Never has anything like this been so smooth for us, so I keep looking up for the proverbial shoe, or anvil, or whatever, to drop. I sincerely hope it doesn't, but have a hard time believing it won't!

We have a few questions for doctors on rounds today, when they make their way to Aurelia:
1. How long do we have to do wound care behind her ears?
2. Now that her tracheomalacia resolved itself, do we still need her inhalers?
3. Can we get a swallow study while we are here?
4. Can audiology follow up with Aurelia while she is here, too?

We also want to try and contact our home care company contact here and get a letter of medical necessity to help our application for medical assistance. We want to try and obtain a full time home care nurse for Aurelia and see if we qualify for medical assistance insurance, in the event our insurance starts to play hardball with CHOP and us. We received our first rejection for care a few weeks ago, so we are thinking the fights are beginning as she vaults way past the multi-million dollar baby level. We estimate her care to be at or beyond $2,000,000 since her birth in September. Combine that with 119 days of leave without pay for Jillian and we're thinking medical assistance is something we definitely need to have! We'll keep you all posted!

A quiet afternoon

All good news from CHOP this evening, even as chaos reigns in the pods around us. With critical emergencies proliferating in the CICU Aurelia bucked the trend, enjoying a quiet afternoon of rest and healing. Her O2 saturation is wonderful at 2 liters of oxygen, she is not maxed out on her pain medication in case she needs more, her color is good and her kidney is operating wonderfully.

That last bit, about her kidney, had us particularly concerned. When you have your heart stopped and you go on heart-lung bypass there is a risk that your other organs will shut down and have a tough time starting back up again when your heart begins beating again on its own. Given that Aurelia only has one working kidney, if that doesn't fire up and work well from the get-go then she runs a risk of fluid build up and that can be dangerous.

Regardless, Aurelia is having great fluid discharge through her foley catheter and the only fluid she's needed (besides nutrients) is some potassium, to replenish what's being lost due to her lasix. Her blood gasses have been great all afternoon, drainage from her chest tube is within acceptable limits, and even her wound care went well around her distractors. All in all, it has been a super afternoon. Thank you to everyone for all your support! Jillian and I feel tremendously blessed to have such a strong and dedicated support network of friends, family, and colleagues.

She's through!

We just received word that Aurelia has successfully underwent surgery. Dr. Spray repaired both her holes and augmented her pulmonary artery. He says that although her mitral valve appears slightly misshapen there is no leaking or backflush of blood across the valve, which is good, and it appears that whatever muscle bundles might have been in the way of her VSD repair have been cut away. Apparently, Aurelia's heart has become a little larger to compensate for the resistance placed against blood flow due to the PA band she received in October. As a result, the increase muscle tissue in her heart might have obstructed those areas the surgeons needed to access when repairing the VSDs.

The echo cardiogram taken immediately after surgery shows no leaking across the two chambers of the heart and no leaking across the valve, as I mentioned above. It also appears that her heart is successfully handling the full blood flow into and out of the lungs, and through her body. Whether or not her heart could handle pumping by itself is a concern, primarily due to the fact that her left ventricle sits right on the border line of a functioning/non-functioning ventricle (which is based on its capacity and ability to maintain adequate pressures and flow).

Currently, Aurelia is off the heart lung machine, being extubated, and undergoing an x-ray. We will be watching her carefully over the next day or two for effusion, or increased fluid around the heart, and any change in her heart's ability to handle blood flow to the lungs and body. Again, due to her heart's increased musculature the trick is going to be getting her heart to relax substantially, so that it can fill to complete capacity, and subsequently pump blood to and from the lungs to complete capacity. In the past her heart built up its strength and did not pump to capacity because of the restrictive PA band. Hopefully, though, given the medicine she is on we will see improvements over the next few days as her heart begins to fully manage the appropriate blood flow of her body.

Thank you to everyone for their prayers and well-wishes. These next 24 hours will be crucial in Aurelia's recovery, so we will keep you informed. For now Aurelia is out of the dark heart of the forest and onto the path, but the way is steep and overgrown, and fraught with briers and other dangers, so keep those prayers and positive thoughts coming.

Waiting....

God I'm so nervous. Aurelia has been in surgery for two hours now, the first being spent with anesthesia and this last having her chest open, tubes and lines placed, and getting through her scar tissue. Reports from the team are positive so far, but I cannot fathom how Aurelia endures this. I cry thinking about how strong and determined she has to be, without choice, in order to live. I would do everything and anything to take this burden from her, to let her live without this pain and trauma.

I know some people believe that young children do not remember events such as these, but I believe they do. I believe that any person, no matter how young, remembers these traumatic events and that they are imprinted, that their development as people is changed forever. I don't fear these difficulties impeding Aurelia's development and personality, but rather I think the courage and strength and happiness she shows now will only be magnified, increasing as she ages, as these traits have served her best today and so far.

When she comes back from surgery Aurelia will have a number of lines placed in her body. She will have a chest drain, an arterial line, pacing lines, foley catheter, and an IV. Her chest will hopefully be closed, packed, and covered. Our primary concern then will be two fold (if all else goes well): will Aurelia's smaller left ventricle be capable of pumping blood for her entire body without assistance and will her heart's electrical tissue be damaged, requiring a pacemaker? Unfortunately, one of her VSDs is located in and around a place where her heart's electrical tissue exists. It is possible that Dr. Spray will have to stitch in that area, and if so, it may disrupt her heart's normal rhythm.

So for now, all we do is sit and wait.