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.