Still Praying for Pee

The last week has been hard on us all. Avelyn was placed on a mechanical peritoneal dialysis machine last Thursday, which was the day I had to leave to return to work. Sean and I had our doubts from the get go as it seemed to not be pulling as much fluid off as the manual set up. As the days wore on the situation got worse. Every time I called the nurses or talked to Sean she was more and more fluid overloaded. By Monday she was really showing changes clinically and wasn't urinating at all. It was bad. So the cycler was abandoned for now. We may try again when she stabilizes more, or they might find a different cycler that will work better for her but in the mean time she'll stay on the manual set up. 

It's believed that she quit peeing because the cycler wasn't allowing her to fully drain her peritoneal cavity before refilling her with another round of dialysis fluid. This caused her to retain more and more fluid which eventually compressed and stressed the kidneys too much. 

The nephrologist ordered a renal ultrasound just to make sure Avelyn didn't have any acute renal changes. The results weren't good. Avie's kidneys are shrinking and have been since at least March. The shrinkage is significant. We were hoping to see signs or renal recovery by now, so the findings are devastating. The shrinkage could be due to her kidneys being very inflamed in March and that receding then scaring over. She could still recover some function of her kidneys, the left one looks a bit better off than the right. 

Either way these findings have hit us hard, especially because it coincided with Avelyn not producing urine for a couple days and with her renal labs looking worse. 

However, we'll hang onto hope because that's really all we can do. There's nothing to be done for her kidneys but to provide her time and pray they heal and regain some functionality-it could take a year or more to truly know how much of her function will come back-if any. However in a few months time, they'll get another renal ultrasound that will be able to show further shrinkage, stagnancy, or growth. We also have to pray that she remains cardiovascularly stable during this time as she cannot afford a heart cath with sick kidneys. It's the riskiest waiting game I've ever played. We're basically gambling her organs against one another. It's a terrible corner to be trapped in and Avelyn's life and quality of life are at stake. It's awful.

More has happened, but I'll try not to sulk about this weekend as much as I did last week and post another update soon. Best wishes to you all. Please keep the peepee prayers coming. She needs them more than ever. 

   

Tomorrow

Tomorrow is a day I have been dreading. I'll have to leave Avelyn and Sean here in Nashville and return to work, 5 hours away in Johnson City. While it's a day that has been lurking for a long time, I have so much to be thankful for.

First, I must acknowledge how blessed I am to work at such an amazing facility full of the most wonderful people. These people, many of whom I don't even know, have donated leave so that I could stay here with Avelyn during our darkest days. Not only did the leave prevent us from having to worry too much about our finances (or me losing my job), but it provided us TIME. I don't think you can ever truly appreciate the gift of time until you're told the life of a loved one is to be measured in days, rather than years.

I could write a novel about how much the gift of time means to me and my family and it would still not adequately discribe my gratitude or thankfulness. The leave my friends, work family, and complete strangers donated, allowed me to stay at my daughter's side when we thought all hope was lost during, what we thought, were her final days. I was able to tell her I loved her, over and over and over, when we were living hour to hour and day by day. 

I got to witness her fight her way out of the darkness when it seemed all consuming and overwhelming. I saw her come back from the brink of death with a furious tenacity that made me more proud and humble than I could have ever imagined. 

I was here for her first wet diaper in weeks (a beautiful 8 ml's), on the very day the nephrologist told me her kidneys would likely never show signs of recovery. 

I was here for her massive diuresis, when she lost more than kilo of fluid in a week's time and her prognosis shifted.

I was here for her extubation, when every one of the providers tried to prepare us for her failure but she prevailed.

I was here to be an extra set of eyes, to study her trends, progression, norms, and to advocate for her tirelessly (or exhaustingly may be more accurate).

I truly believe Avelyn has faired better than she would've otherwise because Sean and I have been here to constantly review, question and participate in her care. Don't get me wrong, I know we have missed a lot. But we have also caught few big things and many small things that could have set Avie back even further.

Anywho beyond the medical side of things, in the last couple weeks I have been able to be more of a mom. As sedatives are weaned, she grows more awake and alert. In the last week, she has seemed to truly know and respond to my voice again. When I talk to her from across the room, she looks for me, and when she focuses on my face my spirit soars. She settles best when Sean and I pick her up and truly enjoys our snuggles. 

I have also been here to help her along in her motor and psychological development. She's doing a lot better with head control, face recognition and, as of today, has started practicing tummy time. 

I was here for all of this because of you all. 

And while I'll likely miss many firsts while away working, I know that's all they'll be. Firsts. Not one and only's. Not lasts. Not one of the few. But firsts. 

I'll see her grow. I'll see her explore. I'll see her love. I'll hear her voice. 

I'll bring her home.

I am trying to keep this frame of thought in the forefront of my mind, but I know it won't be easy. I will be an emotional train wreck every day I am away. The frequency of my calls will likely drive Sean and the nurses crazy. I am not usually an overly emotional person, but in the coming months I highly anticipate breaking down without warning (I'll just go ahead and apologize to my coworkers in advance). 

Yes, the coming days will be hard, but they won't be the hardest. I feel blessed that my returning to work coinsides with Avelyn's recent progression and recovery. I know this is an answered prayer. I also know that while she is still surrounded by uncertainty, and surely has very difficult days ahead, Avelyn is and will continue to be in good hands. Hopefully she won't notice my absence too much, and it's just me and Sean that struggle with this transition. 

In the mean time, if you see me out and about in Johnson City, please be kind. Please don't ask me how I am fairing. I can assure you, it won't be well. I very well may be barely holding it together. Neither of us want to see me ugly cry. Because that's sure to be awkward. As surely as I know it will be difficult, I know I'll be ok and we'll make it through these next couple months just like we have the last six. We won't be unscathed, but we'll survive.

As always, thanks for the support and prayers. Avelyn continues down the road of slow daily progress with a pit stop here and there due to complications. She continues to struggle with medication weans but is toughing it out like only the wonder girl could. Please continue to pray for pee and do your peepee dances. We still believe her little beans will respond and she won't always need the dialysis (though she is tolerating it well). 

We moved to the less acute side of the PCICU!!

Gaining head control is such hard work for our itty bitty.

She now bounces between .05and .025 liters of O2, we're hoping she can be weaned completely off in the next couple weeks.
 

Tummy time wore her out, and also made her mad :/ She didn't seem to care about how well she did with it, she gave me this glare for a good while afterward....

Moving Forward

The last week was full of milestones and more are on the horizon. Avelyn's oxygen requirement continues to decrease, although at a much slower rate than last week. Today she went a couple hours without it all together before falling into a deep sleep and dropping her sat's into the low eighties. While it's possible she may continue to need the 10th of a liter that she's currently on for months or  even years, it's also possible that she won't, or may just need it when she's sick. It's still up in the air....(see what I did there). 

The main progress made this week concerns her benzodiazepine, Ativan. She's tolerated it being weaned substantially. By the end of this week she should be off of it, which is huge considering she was on such a hefty dose after four months of intubation and 6 + surgeries. 

For so long she really needed the drugs but each comes at a price. The weans are really hard on her and it's likely to get worse. She's been tachycardic and uncomfortable quite a bit as the Ativan is chipped away. Withdrawals increase her the work of her heart, which increases her oxygen demand and work of breathing-and that's really over simplifying it. 

It's hard to watch her suffer so much from something so seemingly trivial. I can't wait for her to be past withdrawals and off all of these substances. It'll be so wonderful to see her little personality without it tainted by detox, sedatives, and delirium.

Anyway, after the Ativan is weaned away, the plan is to begin to chip away at her Precedex, which is another sedative she needed while intubated. She's on a super high dose of the precedex and it'll take a long time for it to be weaned safely, it could very well take a couple months. Since it's an IV drip- Avelyn can't leave the PCICU until she is weaned off of it. Meaning she'll be here for quite some time. 

She has also made some progress with peritoneal dialysis. She is nearly at her goal fill volume which will allow her to start using a cycler, which is machine that performs the dialysis rather than it being done manually by the nurses. She'll have to be on this machine and tolerating it well to go home. She'll also need to be tolerating not being on continual dialysis like she is now. Getting to that point will likely be another lengthy process with innumerable variables. 

In other news, her little kidneys have yet to declare themselves. The nephrologist we spoke with today suspects we should know if her kidneys will have any recovery in about six months, but the likelihood of that happening lessens with the passing of each month. Please join me in praying for renal recovery and pee. She is peeing some with the help of very strong diuretics but currently the her kidneys are likely working at less than 10% of the capacity that she needs.  

Lastly, as the sedatives are weaned Avie spends more time awake. She's started to try doing actual baby stuff like chew on her fingers and hold her head up. She's got a long way to go and is giving it her all in physical therapy and with the speech therapist (working up to relearning to suck after her long intubation). Hopefully as time goes on our worries will continue to be in terms of her catching up to her peers in development, rather than on the life threatening issues. There are still a lot of dangers she has to surpass and overcome but we know she can do it. As we've heard from multiple sources, she currently the shining light of Vandy's PCICU. 

Much love to you all. I'll update again soon! There's big things on the horizon! 

 

 

Strides

In the last week Avelyn has made stunning progress from a respiratory standpoint. She has gone from 20 liters of flow at 75% O2 via vapotherm to just 1 liter via nasal cannula. She has maintained stable blood gases despite the intensive weans meaning her gas exchange is steadily improving. She isn't showing signs of labored breathing and looks fairly comfortable in her ventilation. It's still up in the air if she'll need oxygen for home use. No one really knows how or at what rate her lungs will recover after four months of intubation, but for now she's doing well. 

Her kidneys still aren't showing much signs of improvement but they aren't getting worse either. She is responding well to peritoneal dialysis and for that we are blessed. She has a long way to go before she reaches the point that she is ready for the cycler, which is a machine that performs the dialysis. It requires her to be on double her current volume of dialysate, so she'll have to work up to that. It'll take weeks, if not a month for her to get to that point. In the mean time she'll need to remain in the PCICU on manual dialysis. 

Another aspect keeping her here in the PCICU is her need to detox off of a sedative called precedex. She's been on a very high dose of this medication ever since she was intubated at the end of January for endocarditis. When the dose is weaned her body reacts horribly. One of the attendings here told us that the drug has never been studied in children for chronic use. She's likely not the only one who's reacted this way, but given the rarity of circumstance, it's not something the providers have much experience with. Saying that, she'll need to stay in the PCICU until she can be weaned from this drug and that will take a couple months at best. And while I hate that she'll be in the hospital all summer, this drug  affects her that drastically. It's safer to wean it slow, and after nearly 6 months here, a couple more seems like a drop in the bucket.

Also on the horizon- Avelyn still needs a heart cath soon to balloon up her pulmonary arteries. The PAs are small and pumping blood into such small arteries puts a lot of strain on the right side of the heart. A recent echo showed positive signs that her heart is currently tolerating this extra strain alright, but it's not something that should brew. However she cannot go to the cath lab until she is solidly stable and her kidneys have "declared" themselves (meaning she'll regain function or not). 

So in short, we are blessed beyond measure but in this for the long haul. Avelyn is recovering and we are getting to snuggle her close. The feeling of holding and rocking my baby after longing so desperately to do so for so long is indescribable. I was afraid she would have an adverse reaction to human contact after lying in bed with mainly negative touch associations from getting the care she needed. However she seems to have missed it. I have cried with joy too many times to count in the last week due to knowing she accepts and likes being loved on. I was truly terrified she would be revolted.

She is only just rediscovering her voice, but she is learning that even the faintest whimpers will result in snuggle time. Those faint whimpers, while so sad and heart breaking, are music to my ears. We went months without hearing her voice. It was a possibility that such a long intubation may have paralyzed her vocal cords. But it didn't. Each day she regains more function of her vocal cords and rediscovers her ability to make sound. It's so special to watch her figure it out. 

In the last couple weeks, we have been more than happy to oblige her every desire after four months of hell. She is rocked and bounced most of the day and it's doing wonders for all of our hearts. 

Thank you all who have prayed and sent us love and support. Our Avelyn is recovering and we could not be more ecstatic or thankful. She still has such a long and uncertain road ahead of her. As I have said before, she'll never be out of the woods but it looks like we'll get her home to the mountains. With that in the forefront of our minds, a couple more months here seems more than doable. 

As usual, much love and best wishes to you guys!