Saturday, July 30, 2016

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.
      

Wednesday, July 13, 2016

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.
    
 
  

Saturday, July 2, 2016

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.