This Changes Everything.....

"Don't worry, Momma. I got this!"

When I was a teenager, my mom used to say, "You just wait! One day, you're going to have a daughter who will be ten times more rebellious than yourself!". Suffice it to say, I have a strong instinctual impression that she's looking down on me, and right about now, laughing her ass off. In Little Bird's world, so much can happen in such a short amount of time. March 12th is proof positive of that. For as quickly as she has been known to crash, she's also had periods of insane recovery. When she managed to rebound from the bowel perforation and septic shock, we were pretty impressed. But what I'm about to explain to you is almost ridiculous.

As of two weeks ago, the plan was to transfer her to a respite care facility and give her the opportunity to reach her TPN/GT feed goal ratio while MFH and I regained our emotional bearings. Little Bird never plays by the rules. She never has. She's made her own more times than I can count, and broken every one of them, too. Once again, she's made up her own mind. And once again, she's completely defied all laws of western medicine.

By all calculations, she was expected to meet her specialist's expectations sometime around the end of July at the very earliest. Realistically, we were suspecting that it might even be longer. You can't exactly rush a kid who has less than a quarter of her intestinal tract to work with. A milliliter at a time, every few days. Watch and wait for signs of rejection. Signs of liver or kidney failure. Pray to God that she could handle it. Handle it, she did. After a few minor adjustments and tweaks to the University of Pittsburgh's recommended protocol, Little Bird took the ball and ran with it. 

"I grewed a tooth!"

Throughout the past two weeks, she decided to be a show-off, and prove every scientific study wrong. It's taken her literally fourteen days to nearly accomplish something that could have very easily taken that many months. In six days from today, she will meet this goal like a boss, and her TPN is scheduled to be not weened down, but discontinued. Spectacular progress in terms of her growth and weight gain, and beyond that of what any of us could have ever hoped for. And as if that weren't enough, she put the icing on the cake by what she's accomplished on the vent.

When I wrote the last update, I actually forgot to mention something. Something that was certainly not overlooked by us. When Little Bird was transferred to the PICU last December, her PEEP setting was pretty high. Single digits weren't something we thought we'd see for quite some time. But her team felt otherwise, and immediately began making every effort to get her to do the same. Remember when you were small, and couldn't yet blow up a balloon by yourself? Remember how difficult getting one started was? That is exactly how breathing is for Little Bird. Once that initial inspiration is made, she can do it. The vent just gets her started, and keeps her lungs from deflating completely. We all breathe at a PEEP of five to six. This is the measure of the amount of pressure needed to keep our lungs from collapsing in between each breath. In January, she succeeded in being lowered from eighteen to seven. A few weeks ago, she was lowered, once again, to six. And four days ago, she astounded us all by smashing her own record.

"I can has my puppy now?"

Her team began to notice some nuances in regards to her efforts. Things like her not panicking when her trach was being changed, or her sats not plummeting during circuit changes. These, combined with the massive growth spurt she's most recently experienced. On March 12th, we finally understood why she was meant to endure a tracheostomy months before that. It all began to make sense. Because she certainly wouldn't have been able to withstand another emergency procedure that night, and the ventilator being so readily available to her turned out to be a blessing in disguise. For the last three months, we questioned the universe once again, trying to understand why Little Bird had to crash that night. We were so close to coming home. It didn't make any sense, and it angered me beyond extent to have to look at the gaping scar across her abdomen. The constant reminder of how close we came to having it all, and almost losing everything. Why her? Why the damn TPN pump, on top of everything else? What more could she take? How much longer until her labs would start to come back indicative of liver failure?

Not A Scar, But Proof Of Life

Our fears about intravenous nutrition are not unwarranted. It may seem simple enough, but I can honestly tell you that her dependence upon a machine in order to breathe is a lot less complicated. If the vent should ever fail, for any reason, MFH and I can still assist her via an ambu-bag. If we misdose a single TPN feed, her liver will shut down faster than we could catch the symptoms. What a lot of people may not realize is that even though TPN does save lives by providing a means of sustenance, the odds of a short-gut patient requiring a liver transplant because of this are frighteningly high. Especially for a child. But when there is no other way to feed your baby, what other choice does a parent have than to take that leap of faith, and pray to God that things work out in the end?

And when does it all become too much? She was already trach'd and vent dependent. She already had a G-Tube. And both of these, we were used to. We had several months to prepare. The added technology of intravenous feedings was for us, the straw that broke our backs. We started to come to terms with that truth. That we couldn't handle the responsibility and the weight of one more medical intervention. And the events that occurred on March 12th were suddenly infuriating. If it weren't for that damned pump, she'd have been home by now. If it weren't for that damned pump, we'd be able to do this. As we came to this honest conclusion with ourselves, we admitted as much to our team. And no sooner did we say the words did Little Bird work her magic, once again. 

Victory! (she caught the camera strap!)

A phone call on Monday morning, from one of our nurse practitioners. They decided to give her a shot at breathing. On her own. For the last three months, she'd been receiving double the amounts of nutrition that she would have been, had she not needed TPN. Double her caloric needs meant double the weight gain. She managed to pack on more pounds during this time than any other. Something that had been a goal since October when she became what was said to be very possibly permanently, mechanically dependent. If she could overgrow the vast scarring in her lungs, there was a chance that she'd be able to one day breathe without assistance. In all honesty, this wasn't something that was discussed in terms of months, but of years. Perhaps by the time she'd be entering Kindergarten, at best. It was the most realistic time frame that we were ever given. But her recent and sudden influx of growth made her respiratory team wonder. What were the chances that she might be able to now handle ten or fifteen minutes without the vent?

And so, they gave it a shot. They disconnected her circuit, and stood watch next to her, waiting for any signal of distress. And ten minutes turned into six hours. Not willing to push her beyond that, they decided to wait until the next morning to try it again. MFH and I were cautiously optimistic. But even if it was a fluke of nature and she couldn't accomplish this again, it was hope. On Tuesday morning, they again disconnected the machine. This time, she achieved eight hours with nothing more than the assistance of a tiny bit of oxygen, a heated moisture exchange piece, and a Passy-Muir speaking valve. The latter, providing her with a little bit of resistance, but enough to mimic that of the pressure of the vent and keep her from panicking. Clearly, this wasn't a fluke. Little Bird is able to breathe. On. Her. Own. 

"This way to da finish line!!"

On Thursday afternoon, MFH and I arrived at the PICU in the midst of her ninth hour of freedom. Her respiratory technician was on the floor and watching her the entire time. She asked us how we thought she looked. Little Bird was sitting up in her crib, playing with some toys, and patiently waiting as her nurses prepped her trach change. She looked fantastic. So we all agreed to keep the trial going. We changed her trach and her outfit. We watched her show off her ability to also now stand upright, supporting her own weight with just a little bit of help balancing. We watched her nap. And we dodged her splashes during bath time. All without her being attached to the machine. This is a very big deal. This changes everything. 

The vent, collecting dust in the background!

Even though she can't vocalize it, there is no denying that Little Bird is making her voice heard, loud and clear. She knows what she wants. She wants to come home. For whatever reason, she feels that the timing is perfect. And let's face it, this kid is nothing if not always ahead of her time. I can so totally envision her sleeping on some random sidewalk, twenty years from now, waiting days in advance for concert tickets. It's been almost a year since we were instructed to follow her lead, and that advice still rings true. When she is ready, she will let us know.

Her team isn't willing to allow her to go beyond twelve hours unassisted yet. But from here on out, as long as she isn't sick or showing signs of distress, she will be disconnected from the ventilator from eight o'clock in the morning, until eight o'clock each evening. Half of each day. I can't even begin to describe how liberating it feels to be able to pick her up without the weight of the circuit-the constant reminder that eight feet of tubing is a lot shorter than it seems. It's going to take us some time to get used to the silence in her room, as the white noise of oxygen and humidity hissing in the background is no longer present. It's ironic, isn't it? The addition of the TPN pump, and the havoc that that wreaked on our family turned out to be an unforeseen blessing. Had it not been for the extra nutrition this provided her, she wouldn't be breathing on her own. 

"Wait'll they get a load of my next trick..."

MFH and I walked out of that hospital last night in utter silence. Shock. Dismay. It's almost as if the entire last twelve months had been a bad dream. As if someone woke us up, and here she is. Little Bird. No ventilator. And in a few short days, no PICC line. How is it that you get pushed to the brink of your breaking point, fall to your knees and cry 'uncle', and then suddenly? Suddenly, an invisible hand reaches out and grabs you by the back of your collar. Pulls you back up on your feet and pushes you in the direction you were meant to go. How does that happen? Little Bird is coming home. On Tuesday afternoon, our team called off the scheduled meet and greet with the representatives of the respite care facility that we'd chosen for her. We will no longer be requiring your services. I don't know how she managed to pull all of this off. And I don't know why she chose us to be her parents. All I know is what I've witnessed. That she knows what she's doing, even if we don't. And that sometimes, everything happens for a reason.

 
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