Sepsis and Perseverance

Maybe one day I'll be able to start a post that doesn't begin along the lines of "it's been a hellacious roller coaster these last couple weeks". Today is not that day unfortunately.
Last Tuesday Avelyn got septic seemingly out of no where. She was re-trialed on the mechanical dialysis cycler on Monday and steadily grew more fluid overloaded as the hours wore on. She also became more agitated and Sean noticed her growing more mottled and fatigued. I had a terrible feeling all through Monday night and into Tuesday morning. Usually, I'll nap before driving back to Nashville but since I couldn't shake the sinking gut feeling, I left as soon as my 12 hour shift was over Tuesday morning.
When I got to the hospital Avelyn didn't seem too bad, but looking back I should have been more concerned. She was getting extremely upset and there were signs that she wasn't perfusing well. At the time, likely because I was in denial that something so terrible was at play, we were chalking it up to withdrawal.
By 2 o'clock, Avelyn went down hill fast. The following 4-6 hours were some of the most terrifying moments we've experienced, especially in more recent months. My perspective is skewed, but the last 7 and a half months have taught me that times of such panic and desperation often warp your memories into barely recognizable events.
Though I can't narrate many of the details, the facts are: her hart rate climbed into the 230's and eventually 240's and stayed there for hours.
Her temperature climbed from 99.1 to just above 104.0 in about half an hour.
Her color was awful, her eyes glassy, and she wasn't responding to outside stimuli.
Her labs came back and showed her white blood cell count and other markers for infection were through the roof.
She was given Tylenol and sedative to try and calm her and bring her heart rate down.
Sean and I were terrified that she was going to code or at the very least start having febrile seizures.
I was terrified her blood pressure was going to plummet and she'd go into septic shock.
She stayed in this state for what seemed like forever.
Eventually her temperature started to come down and her heart rate with it.
By the time her night shift primary got there she was stabilizing out.


The next day I begged for an echo to rule out endocarditis. While the echo was clear of any signs of vegetation, it showed that her heart had been severely damaged by the septic episode. Before her left ventricular function was normal but after it was "moderately to severely depressed", which explained why her color was so poor and why she wasn't perfusing well.
The news was crushing. Avelyn's life may likely be dependent on a kidney transplant. She may not be a candidate with a normally functioning left ventricle, let alone moderate to severely depressed left heart.
Given the left ventricular damage, she was started on a medication called Milronone, which helps her heart not have to work as hard. Through the week she slowly but gradually improved. By the next Monday, 6 days out from the acute sepsis event, the echo showed "mild depression" of the left ventricle. Meaning, by the grace of god and the PCICU staff's quick action in the face of crisis, our baby's heart is healing. It will likely take quite a bit of time for her to get back to normal left ventricular function, but we truly believe she'll get there.
The echo's also showed that Avelyn's pulmonary arteries are growing without intervention. This is HUGE. Many, or perhaps even most, children with MAPCA's and pulmonary atresia need serial ballooning and stenting of their PA's in the cardiac cath lab. Avelyn cannot go to the cath lab right now due to the state of her kidneys. The dye used during the cath procedures would likely completely knock out her kidneys. The longer we can put off a cath procedure the better for her kidneys- but that all depends on the growth of her PAs.
As of the last few days she's started to show signs of renal recovery. She is responding to diuretics for the first time in months. While it's still only about 1/6 of what she needs to make to sustain her self without dialysis, she is making urine and that's something to be celebrated. We are so stoked for her and are praying that her kidneys can and will do more as dialysis is scaled back over the next few weeks.


I know this is all very complicated and difficult to follow. I know I likely don't explain things in the easiest ways either. Thank you all for hanging in there with us and for checking in. Please continue to keep our girl in your thoughts as she fights battles no child should ever face. Specifically, please continue to pray for pee, growth of her PA's, and healing of her left ventricle.
      

Still Praying for Miracles

Our sweet baby has come so very far. She's overcome so many seemingly insurmountable odds but yet she continues to face such hardship. Hardship that may inevitably take her from us well before her time. 

In the last few weeks she has made great progress in all areas but renal recovery. She has been completely weaned off Ativan and has started her precedex wean. The precedex will take time to come off of- likely a couple more months. She is currently being weaned daily and handling that ok, but it's probable that the weans may need to be spaced further out as she gets deeper into the process. The constant state of withdrawal has kept her feeling not so great but she looks better than we've ever seen her. She's more interactive and more engaging than ever before. Her heart rate and temperature stay fairly elevated though and she's often pretty irritable. It sucks but that's just part of the weaning process. After the precedex comes off she'll need to be weaned from the methadone (which she needed to come off of her opiate drips) and the clonidine (which she is on to come off of the precedex and Ativan). Yet those weans are so far in the future it's not much of a concern right now. 

The biggest concerns center around Avelyn's kidneys. I am absolutely terrified when it comes to  her kidneys. The last renal ultrasound showed that they have shrunken and have scar tissue forming. Granted at that time we were reassured that there is still time, but it's been weeks and we haven't seen any improvement. We still have hope and are praying fervently for her kidneys to turn a corner and start working again but it doesn't look good. The nephrologist today said its looking less and less likely that her kidneys will make a come back. Plus chronic renal failure is usually a progressive disease. So, best case scenario- her kidneys come back and function at a quality of life sustaining rate, we'll still have worries of progressive worsening over the years. However, we've been told Avie's case is so unique that there's a very (very) small possibly that she will not have this progressive worsening. 

It's not much, but I believe in miracles.

How could I not? I am currently cradling the most beautiful miracle I've ever witnessed in my arms as I type this out.

However, I am also a realist and know that miracles aren't often gifted.

A kiddo without CHD and with Avelyn's renal function (and no recovery) would need a kidney transplant. We don't know if Avelyn is a transplant canidate. While her heart function is currently not too bad considering all she has been through, she still has very real and serious cardiac issues. Her pulmonary arteries are quite small and will need serial ballooning and stenting as she grows. Each heart cath procedure will require contrast which is hard on the kidneys. She'll also require pulmonary conduit replacement procedures as she grows and likely tricuspid valve revisions. Heart lung bypass runs are hard on the kidneys too. 

What does this mean for Avelyn?

Honestly, we don't yet know. 

We don't know if she'll be a kidney transplant canidate. We just don't know. If she is, she'll have to grow another year or longer before she's big enough to receive the kidney (they don't transplant child sized kidneys, only adults sized). 

This isn't your average child growing for a year. Poor kidney function surpresses growth and development. And so does CHD. She'll be more prone to illness and at greater risk in general. 

Next week we'll have a care conference to discuss all of this in greater detail. I have a feeling the answers we will receive will be along the lines of "we'll have to wait and see" and "we don't yet know".

So much is still up in the air. Our daughter's very life is up in the air. Her future. Her health. Her happiness. Our little family of three. None of it is guaranteed. None of it. 

I think a lot about that. It's hard not to. 

Taking each day as it comes is easier said than done, but honestly it's all we can do right now.  

Please pray God sees fit to grant our precious one yet another miracle. Please pray for renal recovery, quality of life and healing. She's fought so hard. We just want to see her grow and be happy

As ever, she's our beautiful wonder girl and she continues to give her all.

    

 

  

Perspective

I don't think anyone ever considers that having a baby will bring about the most excruciating experiences in life. But for some of us, it does. For me, parenthood is marred with a deep seeded sorrow that will never go away. Some days that sorrow is overwhelmingly all consuming. Some days it's distant. Most days it's both.

Today is one of the harder days.

I thought that as time passed, leaving Avelyn and Sean to return to work would get easier.

It hasn’t. To walk out of her hospital room is agonizing. Getting to the car before I have a meltdown is a challenge I have yet to accomplish. Driving out of Nashville and 5 hours away from my extremely ill baby is physically torturous.

I know some people think I am crazy for leaving when she is still so sick. And while you would never say it to my face, I know many mothers are likely thinking they would figure out a way, whatever it took, to stay with their child.

I get it. It’s easy to judge when you don’t have a clue. It’s easy to say, you’d find a way when you’ve never had to. I know I likely sound dramatic and aggressive but I don’t mean to. I also do not mean for this to be some insinuated plea for help or attention.

I write this because other heart moms have asked me to share our story- the good, the bad, and the ugly. Me having to return to work is part of that story. So is feeling judged for not being at my baby's bedside. Then again, bearing the weight of judgment is but minuscule compared to the arduous load of my child's illness.

Being the parent to a critically ill child irrevocably changes your very being. How could it not? I must live knowing I’ve made decisions that have caused my child pain and suffering on a scale that I will never be able to fully comprehend. And though each day I thank God for giving my child the opportunity of life, my next breath carries prayers of forgiveness for all we've put her through.

Given most people tend to value duration of life above all else, I can see how this too may sound crazy.
But there comes a time when you may pray for the suffering and pain to subside, whether that means you get to keep your loved one or not. In that moment, you know in your heart that death isn't the worst possible outcome.

Reaching that reality is peaceful, numbing, and completely sickening. Every sense is heightened, yet so dulled that you can't process your environment. Each moment lasts forever, but is gone is the blink of an eye. Reality has never felt more absolute and yet so distant.

The memories of our worst times are etched into my brain too sharply. Sometimes I wonder if they really happened. Other times I wonder how I am ever able to think of anything else. Not infrequently, the most haunting moments visit me in my sleep or creep into my thoughts completely unprovoked.



The PTSD is real. I am not ashamed to admit it.



I am not some super human that people seem to think I am. I often don't know what I am doing or how I'll get through tomorrow. I don’t have some vast well of strength to draw from. I don’t have some incredible gift of insight or understanding. I worry constantly. I question everything.
I get angry at the lack of answers and the uncertainty surrounding my daughter's life, but that takes a lot effort and energy that I can’t afford to expend.

I guess what I am trying to say is, I am tired of people assuming there’s something special about me and my ability cope with Avelyn’s illness. I’ve started to feel that by allowing people to go on perceiving I have all of this figured out, I’m sending the wrong message. It belittles other parents’ journeys with critically ill children and that’s the last thing I want to do.

Every parent in situations similar to ours must cope differently. There’s no wrong way to cope, though I admit some coping mechanisms are more productive than others.

The only advice I have for families entering difficult situations like ours is to not let the darkness  keep you from loving and embracing those around you. Don’t waste too much energy being angry. Life is draining enough as is. Put your effort into trying your damnedest to be present in the moment, no matter how painful. And sometimes, it helps to just let the tears flow.



I feel the photos below help tell our story better than my words ever could. The first is of my and my girl the morning before I had to leave this week. 

The second is when I had to stop for gas. I was using my phone as a mirror to wipe my tears (a tear streaked face draws more attention than I wished to deal with). I accidently snapped this photo. I don't know why, but couldn't bring myself to delete it.  

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!