Dangerous Times

Living in the hospital is dangerous and that goes for any hospital, not just our beloved Vanderbilt. Hospitals are dirty nasty places that harbor the most deadly bacteria unseen right under your nose, and all around your fragile loved ones. The longer one is in the hospital the greater the risk of acquiring infection-that's just how it works-not just here, everywhere.

Avelyn has now been septic more than her fair share, from endocarditis in January (that nearly killed her and has set her down this hellacious path of complications including four months of intubation and kidney failure), to sepsis in late July (that severely damaged her left ventricle and nearly sent her into cardiac arrest), to sepsis (MRSA) and NEC last week that has again forced her to fight for her life. 

So the story goes....

Last weekend, while I was away working, Avelyn was really fussy. She was teething, had terrible diarrhea and a bleeding diaper rash. Her fussiness was chalked up to all of that, but again, maybe we should have been more weary. While driving to Nashville Monday, I got a call from one of her primary nurses saying that she wasn't acting like herself. Her work of breathing was increased and her color was worse usual. Some interventions were made, she pinked up and seemed to do better but was still really fussy. But again, her diaper rash was hideous and she was actively cutting a new tooth. 

By that evening Avelyn was in such pain that I knew something else was wrong and was scared. I requested to see the doc who had seen her through the week prior's anaphylactic episodes, and therefore knows her well. When she came in I told the doc I didn't know what was wrong, but that something was amiss and was I was concerned, very concerned. She was in pain, her color looked awful and she was crying like she does before a big crash. The doc looked her over, and at that given moment she looked ok. She said she would check in on her frequently, God bless this doctor for taking my vague concerns seriously. She saved Avelyn's life that night. Seriously. 

Not long afterward Avelyn had a bloody diaper that spilled onto her bedding. I changed her and asked the nurse for help changing her bed. I put her diaper on the scale (all of her in's and out's are calculated), we got her settled and then I crashed, believing the nurse would follow up appropriately. (I had been up nearly 36 hours at this point, with only a 1.5 hour nap before driving to Nashville after work, and despite my worry-I am human.)

An hour or two later, the doc came in to check on her and Avelyn woke writhing in pain again. I told her I was super concerned about the bloody diaper. I assumed the nurse had called and that's why the doc was there.

Nope. 

The doc didn't know about the diaper and was just there to check in because of me expressing my concerns earlier in the night (seriously, this doc is amazing). I don't know why the nurse hadn't told her, because a bloody diaper is a big deal in this sick kid world as it is often a precursor to sinister happenings (maybe she was just incredibly busy with the kiddo next door, maybe she wasn't, I don't know but I have tried not to dwell upon it). 

Anyway, hearing this the doc literally fished through the trash to find the diaper. After seeing it, she ordered a slew of labs and tests. These came back showing Avelyn had a terrible active infectious process and so she was immediately started on antibiotics. This was prior to the NEC or pneumatosis ever being found on her X-ray. 

I fully believe the quickness in which the doc ordered the antibiotics is what saved Avie's life. 

The rest of the week passed slowly given Avelyn was so critical and we were all so worried. Her blood cultures came back showing she had a MRSA infection in her blood stream on top of the NEC. Infectious disease doctors were in and out multiple times a day, monitoring her progress and asking us how we felt she was doing. Surgeons were in and out multiple times a day too, because in all reality her bowel could have perferated at any time. 

Sean and I again were forced to have talks no parents should. We questioned whether we could put Avelyn through a major abdominal surgery, even if it was considered life saving. Such a surgery for Avelyn would be devastating. Not only would removing part of her intestines be a major ordeal with likely life long consequences, but also it would mean disruption in her paratoneal dialysis. This would force her onto hemodialysis. None of this was discussed with us by the team, maybe because the NEC was caught so early but as usual my brain was miles ahead. After eight months of this hell, it's hard not to live that way.

But, by the grace of God and quick actions of our Amazing PCICU team, it didn't come to that. Our Avie fought through yet again with unbridled tenacity that continues to floor me. (I don't know where she gets it, but the kid is amazing.)

After a really tough touch and go week, Avelyn seems to have stabilized. The infectious disease team came to the conclusion that her MRSA bacteremia was likely a central line associated infection. They encouraged the team to remove the affected PICC line and insert a new one. Once bacteria is set up in plastic, there's way to treat it. 

So yesterday she had a new PICC line placed. The hospital has a special team of nurses that come into the room and place PICCs. The team's only job is to place, monitor, and trouble shoot PICCs- they do it all day every day. 

I was woken by a member of the PICC team early yesterday morning to sign a consent form. While signing it, I specifically stated that Avelyn has been really sick with sepsis and that she is extremely fragile. I requested only an experienced nurse place the PICC and for no trainees to work on her. The nurse assured me that she would pass the message to her preceptor and they would take good care of her. The preceptor came in, literally five minutes later, and rushed me out. I figured she was rude because she had a busy schedule or maybe that's just her personality, now I question it. 

Did they take my concerns and requests the wrong way and treat my baby differently because of me? I hope not, but in these circumstances I can't help but question EVERYTHING.

A couple hours later I got the call that the PICC was placed, Avelyn did well, and I could come back. When I got to her room, Avelyn's nurse asked if I knew about the skin tear under her arm. I said no, and asked which arm. Of course l, and not coincidentally, it was the the same arm the new PICC was placed in. 

I asked to see the tear, because Avelyn's night time primary and I had just given her a thorough bath the night before. We checked all of her folds and powdered them because sometimes she'll get skin breakdown between them. We checked her diligently, her skin was fine. We then dressed her in a sleeveless shirt. There's no way I wouldn't have noticed a inch long >1.5 centimeter deep wound in her arm pit that was bleeding everywhere. Because that's what we found when the dressing was removed. 

The nurse seemed very surprised at how bad the wound was too, the PICC team had discribed as a "skin tear". 

At first I was in shock. 

How could this happen? 

Seriously, how in the hell could this happen!? 

How rough did they have to be with my baby to cause her skin to rip like this?! 

I was soon so angry and heart broken for poor Avelyn I could barely speak and was just crying for sometime. It took me a little while to compose myself and ask to see the nurse manager.  

To her credit, Avelyn's nurse handled the situation well. She wasn't in the room when it happened but told me exactly what she knew and was very upfront in answering my questions. From what I was able to gather, the nurse who placed the PICC was not the same one who prepped Avie's arm. From the look of the injury, her arm was ripped by being hyperextended and pulled too hard away from her body. The PICC team passed off the wound to Avie's nurse like it was already there, I figure they truly thought this. 

(I can't stomach the thought of someone knowingly causing such harm to a baby and then lying about it- I really don't believe Vanderbilt would employ someone like that.) 

Human error and/or negligence has significantly harmed my child, and I understand that happens sometimes. But that doesn't mean I am not pissed and heart broken for her. She is in such pain from this wound, every time she's awake and moves that arm she cries a high pitch cry of pain. 

I want whoever was too rough with her to know the pain she has caused and the risk she has placed Avelyn in. I want her to know that these babies are insanely fragile and you cannot treat them as you would a normal child. I want her to think how she would feel if someone had caused a gaping wound on her baby. 

I thank god for momma instincts I often don't understand but follow anyway. I was adamant about Avelyn recieving dilaudid, rather than the intended morphine, prior to leaving Avelyn's room for them to place the line. I pray she didn't feel the injury fully but I know it must've caused her serious pain either way. 

Despite this happening, it has reaffirmed my love for Vanderbilt. I know things like this can and do happen at every hospital. I work in a hospital and they are filled with imperfect people, most of whom do their very best but all of whom are fallible. 

I feel my anger and concerns are being heard and documented. The PCICU's manager is sending the manager of the PICC team to speak with me and, while at first I was indifferent about it, I am now looking forward to it. Avelyn cried much of the night and has been needing regular doses of morphine to treat the pain associated with the wound. 

I want the manager to know this and ensure no other baby experiences such an unnecessary and painful experience again. I want it documented that this happened and who was in the room when it happened. I want a paper trail. That way, in the case of another child sustains an injury maybe a pattern can be seen in behavior. 

In hospitals, if it's not documented it didn't happen- so my focus right now is ensuring I take every step in the proper channels I can. 

I truly feel the staff and management are supporting me in this effort.The mark of a good hospital is acknowledging and apologizing for mistakes and wrong doing and then taking steps to ensure such does not happen again. 

Vanderbilt Children's is rising to that challenge and I am not at all surprised. This place has been such a blessing to us. I feel blessed to be here and on a unit, that for the most part, loves and cares for my girl as she deserves. There will always be bad apples and mistakes but overall our Avelyn is so very loved and well cared for. 

This unit's staff have saved her life time and time again, for that I am eternally greatful. In times like these I must keep this in the forefront of my mind, not just because it allows me to get through the day without losing my temper (that would be of no help to anyone), but because it is true and the hospital's PCICU team's greatness deserves to be acknowledged.

On another note, Avelyn's wound has already showed incredible healing overnight. It's not nearly as deep and I am so greatful to see its progress. I didn't take a photo yesterday, but it was much deeper-at least a centimeter. I am shocked that Avelyn is healing so well given she was off of feeds for a week. 

Besides that she is doing ok, not great but ok. She still has moments where her color is terrible and it's obvious that an infectious process continues to brew but those moments seem to be less frequent and intense. This week's attending thinks she is also suffering effects from going a week without nutrition and it'll just take time to see her feeling like herself again. She's still breathing heavy and the cause to that is difficult to pin point, hopefully it'll subside with as her over all condition improves. 

Of major note, after nearly three months of weans, she is finally off of precedex, which is the sedative she has been on since she was a month old. We don't know how this drug has effected her development or overall clinical picture and I am so glad she's off of it. Today's wean was twice the normal size (because the IV pump was already at the lowest setting), so she'll likely struggle with the wean more than usual. I hope not, but knowing Avelyn it'll be a rough day. With the precedex coming off, she is for the first time in 7 months completely off of IV drips. This is bigger news than Ican describe and I wish she were in a place I felt comfortable celebrating. Maybe next week she'll be feeling better and not in as much pain, that way we can find a way to celebrate this huge accomplishment!  

As usual thank you for checking in and following our sweet precious baby. She continues to give her all and fight through challenges no one should have to. We are beyond proud to be her parents and able to witness her remarkable journey. Sean and I are often overwhelmed by the love and support given by all of those who Avelyn's story has touched. You all are amazing and thank you for all your prayers and love.

Until next time, take care!

The photos below are in chronological order. As I have said before, I don't often take photos of her at her worst, it's just too sad. The photo of her in her diaper was the night before she sustained the wound under her arm. You would be able to see it bleeding if it were there. The photo of the wound was taken more than 24 hours afterwards, it's already started to heal amazingly (it was about a centimeter deeper, not that you can tell the depth well anyway due to the bleeding or poor photo in general), please pray it continues to do so. 

   P

Unfathomable Odds

I know I have started many blog posts with "where to begin..." But seriously we have a lot of crazy hellacious weeks. 

I guess I'll just try to update from where I last left off. A LOT has happened since then though, and for the sake of all of our time I'll try to be brief.

But given I do my best to be honest and straight forward on this blog, you should know up front...I'm crap at being brief. 

Anyway, last weekend that I worked I got an update saying none of Avelyn's labs were resulting out. We were told that it could be any number of things, from a clotted PICC line, to laboratory errors, to high triglyceride levels. We were told not to worry, it's all likely work out. The chances of something being drastically wrong were slim. I guess no one remembers what child they are speaking of when we get this kind of news. 

If something is exceedingly rare, or even better, only documented but once in the literature, it's likely to happen to Avelyn. Yea, because we have that kid. 

So turns out Avelyn's triglyceride levels were 23x normal. We had doctors tell us they had never seen such a thing before in 20+ years of practice. Her triglyceride levels being so high kept causing her labs to hemolyze and not result out properly. It also puts her at an extremely high risk pancreatitis, which could very likely be fatal. 

The thought still makes me sick, even though they have since come down to 10x normal (which is still insane). 

For days various specialists were in and out but none were able to provide any clear answers. The hepatic specialists told us the lipidplogists would have answers for us. The lipidplogists told us, it was likely due to hepatic dysfunction. So around in circles we went with no real answers. 

The team here on the unit thinks the cause is multifactorial and likely that she has been getting way too many carbohydrates, via fortified feeds and through her peritoneal dialysis and that she couldn't keep up with them. I can't help but agree with this theory. 

Sean and I worried for months that she was getting too much sugar/carbs in her fortified feeds (the main ingredient of the fortifier is corn syrup) and in her high glucose concentrated dialysate. We brought it up often. Actually a couple weeks ago I brought it up again with renewed vigor when a nurse practitioner joked about how chunky Avelyn looked, and a protein supplement was added after her growth (length) was noted to be stagnant. 

Anyway, to combat the insanely high triglyceride levels and elevated liver labs- she was started on fish oil and a medication intended to help with bile sludge (actigall). She was also put on a zero fat diet. She was given straight skimmed breastmilk with only the protein fortifier for three days.

Throughout all of this time she was also started on a new blood pressure medication due to hypertension. She got her first dose of the propranolol Thursday afternoon and it seemed to really be helping by the evening. 

Then at 3 am she woke upset and got mottled. I asked the nurse to turn on the light and we agreed her purfusion didn't look great but she pinked up very quickly-within a minute or two. 

I let it go. You'd think I would know better by now. I am still kicking myself for not making a stink of it. I know we live and learn as parents, but the stakes are so much higher with Avelyn. 

But anyway....

The next morning I left for work. When I was a ways past Cookeville, around two hours out, I got a call from Sean saying Avelyn had basically just gone into respiratory arrest. 

He said he walked in am from grabbing a coffee and she was  purple and graying by the second.The nurse was right behind him and luckily the doctors were next door. 

It all happened very quickly. 

One minute she was fine, the next her airway spasmed closed and she couldn't move any air in or out of her lungs. Her airway was completely spasmed closed. 

She got emergency rescue meds, including epi and albuterol to open her airway (while she was prepped for intubation) and luckily it worked, she was then placed on high flow respiratory support-and later continuous albuterol. 

Meanwhile, I am still on the road, driving in shock (yea, stupid, I know). I made it another 15 minutes down the interstate before I was in hysterics and had to turn around and head back. I don't know why I thought I still needed to go to work other than being in a state of shock and disbelief. It didn't take long to click. 

After all we've been through, Avelyn's track record isn't the best. I felt I needed to be there, even though Sean had done his best to reassure that she was stable- I knew in my heart I NEEDED to be there. Thank god for a mother's instinct (that shines even when the rest of your brain shuts down) because, she didn't stay stable for long. 

After the initial crash, where she was very very nearly intubated, she did alright through the rest of the day and night- not great but alright. The next morning she went down hard and fast again. After another bolus or epi and more continuous albuterol she stabilized but then at noon she again began to struggle for each breath. This continued well into the evening. It was awful. She was seriously in respiratory distress ALL DAY. 

The providers said that intubating patients with bronchial spasms often makes the situation worse, and that the plan was to try to support her through this acute reaction as best as they could. 

At first it was believed that this was all due to the propranolol but as time went on and she continued to get worse everyone was perplexed. Sean and I raised suspicions for other or additional causes. The only changes in her treatment were the propranolol, the biliary sludge med (actigall) and fish oil. Sean and all requested to speak with one of the doctors on over the weekend (who was/is amazing-as all of them here are) and we requested for all of the recent meds to be discontinued. Doubt was raised as to the likelihood of an anaphylactic response to fish oil or actigall (which we agreed with) but we requested them to be stopped anyway (Avelyn has been the one in a million baby too many times...). 

So she got her last dose of both medications at noon on Saturday, just before we talked with the doc. The unfortunate thing about these meds is both have an extraordinarily long half life (up to 89 hours), meaning they stay in your system a long time. 

Avelyn continues to have anaphylactic symptoms. She's four days out, is on a continuous epi infusion, is getting IV and nebulized steroids, IV benedryl and is still having bronchial constriction, swelling of her eyes, mouth, tongue and upper airway. She is still requiring continuous high flow respiratory support and sedation as she has bronchial constriction/spasms if she gets worked up. It's awful and there's no precedent for how to treat such a reaction in children, let alone one as fragile as Avelyn. 

Only one other example of a fish oil allergy exists in the literature and that person had anaphylactic symptoms for a week. That's crazy.

I feel terrible for Avelyn. She has been struggling so much through all of this. There's not a whole lot more that can be done to get her through. She's had labs sent off to see if she has antibodies against fish, those likely won't be back until next week-not that it would change her treatment course. 

So basically she just has to get through this week, maybe longer. All three medications are listed as allergies for now. Maybe eventually we'll be able to tease out the problem, maybe not. 

In the mean time, we've been snuggling a lot and just trying to keep her calm. There's not a lot else to be done for our air hungry baby girl. 

I'll update again soon. 

We love you all and thank you for checking in on our sweet baby girl. She continues to be quite the wonder.

The pictures below are in chronological order, you can see how swollen she is the last photo though at the time I took this photo to show how much her swelling had gone down. I don't usually take photos of her at her worst. It's just too sad. :(

     

If Only...

If only it were just the heart defect...If only. I find myself thinking that a lot these days. I know it's not a healthy train of thought but it's impossible to prevent it from seeping into my head when I least expect it.
There are a lot of "if only's" in the last 8 months, but none of them matter now. Because it's not just Avelyn;s heart that is sick, it's her kidneys too (and lungs to some degree). Recently we have been confronting the high probability that we will soon have to sacrifice Avelyn's kidneys in order to try to make her heart as healthy as possible and in turn, the best kidney transplant candidate as possible.
It's all very complex but basically, she needs heart cathertizations to balloon or stent her distal pulmonary arteries. Proximally her arteries look a lot better. Both have grown significantly (which was unlikely) without interventions but out closer to the lung fields, she is still believed to have narrowings. These narrowings cause the right side of her heart to need to pump harder to get blood through the small vessels into her lungs to be oxygenated.
It is believed that since growth is so rapid in the first couple years of life, that now is the best time to intervene with these narrowings. If they can be dilated now, the arteries will have a better chance of growing with her and not occluding over time.
So how does ballooning her pa's affect her kidneys?
Well to do the heart cath's the doctors will have to inject her with dye to be able to see her vasculature and find the places where intervention is needed. This dye is nephrotoxic, meaning it is damaging to the kidneys. Normal kidneys can filter out the dye, but Avelyn won't be able to with her decreased renal function. Her kidneys will worsen, possibly considerably, with each cardiac cath. She will likely need to be completely sustained by peritoneal dialysis, something we were hoping she could come off of.
While none of this is new information, it's being provided and insisted upon from a different perspective by Avelyn's providers. The providers seem to believe that if left alone her heart, while ok for now, will not be able to sustain optimal function needed to be a kidney transplant candidate as time passes. They also believe that if left alone, we'll miss the optimal window for cardiac intervention and she may not be able to achieve as good of results later, and will still nearly certainly need a kidney transplant at some point prior to high school. So the school of thought is to get her as healthy as possible from a cardiac perspective to be able to best handle the transplanted kidney when the time comes.
So why not go ahead with a transplant asap?
Well the above mentioned heart cath's for one. She'll need multiple of these in the first few years of life and the dye could jeopardize the transplanted kidney's function too. Also, she'll need another open heart surgery in a few years to upsize her artificial pulmonary conduit. The one she has won't get her into puberty. The conduit replacement will require a pump run (heart lung machine) which can also damage the kidneys (actually, this is how Avelyn's kidneys were damaged in the first place). So in order to give her the best shot, she'll likely need to have already undergone the conduit replacement prior to transplant.
So where does this leave us?
Taking all of this into account leaves Avelyn in a very precarious position. She'll need to be fully sustained on dialysis until the optimal time for transplant. That could be 2-3 years. That's more terrifying than the transplant process itself. While sustained by dialysis, her growth, blood pressure, bone development, hormones, electrolyte balances and a lot of other things will be affected.
Also finding a match for her transplant could prove impossible. Sean and I aren't matches, though there are organ swapping programs out there that may be an option. While we have loving and absolutely amazingly generous family members who have offered, a lot goes into being a kidney donor and a match, so even those willing to donate may not be able to.
Lastly the transplant road is not an easy one. She will suffer from a compromised immune system her entire life. She will be in constant danger of rejection. She will need very likely need multiple transplants over her life time and cadaver kidneys have the longest wait time, often exceeding 5 years. Realistically, Avelyn won't make it 5 years, so a living donor is likely her only option and it's not guaranteed.
I guess that's been Avelyn's life story up until this point. Nothing has ever been guaranteed and yet she fights on. As long as there's still hope, we'll continue to fight along side her. 

       

The Irrelevance of Normalcy

Normalcy. What does it mean to you?

For me, normalcy is loving a child I could lose. Normalcy is living out of an ICU for nearly 8 months.

Though the challenges Sean and I have faced are not comparable to those Avelyn has overcome, our road has not been easy. Our baby has been hospitalized for 228 days.

228 days. 

Think about that for a moment.

She was born at 275 days gestation. By the time she's discharged from the hospital, she will have spent more time in the ICU than she did in utero. That's a long time. And though we are blessed to still have our baby, each and every day has taken its toll on us as parents, people, and a couple.

Having a hospitalized child is exhausting--physically, spiritually, emotionally, and intellectually. To have a critically ill and chronically hospitalized child goes beyond that.

In the beginning you’re naive and don’t understand what’s going on around you or how the ICU operates. Things are a blur and everyone you know calls, texts, or tries to visit constantly.

At first you reach out, you get to know the other families and children on the unit. Then you learn what the overhead alarms mean and your stomach drops with each and every one. You know what it is like to be the family who’s baby is coding. You know there’s nothing you can do or say to make it better but pray and try anyway.

Time continues to pass. The calls and texts become fewer and the visits shorter and/or less frequent. The months add up, the exhaustion builds, and you learn the interworking’s of the unit.

You quit reaching out so much because it’s just too damn hard. You’re still grieving for the lost little loves and their families from the early days. But that doesn’t mean you don't notice when a room empties too soon. Each child lost, whether you know their name or not, takes its toll on you as a parent and a person. On those unbearable days, it’s all just too real. You hold your precious little one close, not knowing why she’s still here and so many just as strong, just as deserving aren’t. You continue to pray earnestly and fervently for her to be spared and to see her grow-- because you know, beyond a shadow of a doubt, that life is precious and nothing is guaranteed.

More days go by. The world around you goes on, but yours doesn’t. Yours is still behind locked doors and within the eyes of a baby giving her all. You spend your days trying to parent around medical interventions, assessments, alarms, illness, lines, chords, pain, withdrawals, strangers, and countless other distractions and interferences—because she deserves as much of a babyhood as you can provide.

On the good days you rock and soothe your baby with hopes of easing the discomfort and pain. On others you can barely find an open bit of skin free from chords, lines, or dressings to stroke or pat. On the worst days you can only hope your child doesn't feel excruciating pain—though in desperation you pray that they feel love you're emitting from your very being, because god knows you can’t do nothing.

Still, time goes on. Calls, texts, and visits are rare. Confusion and anger start to seep in as people you love go about their lives. You see more rooms empty too soon and each one hits home.

Eventually you get to a point where you're too exhausted to care much about social convention or other people's feelings. Because in their world, children are smiling, healthy and pain free--whereas in yours, children fight for their lives and all too often die.

You just want them to know that your world exists and to care about those who’ve been lost but you’re so exhausted it all comes out wrong.

Time wears on. You feel your world closing in. But then she studies your face and she smiles. It's such a simple gesture for any other baby, but you know it’s so much more given all she’s been through. 

She is still here. Still fighting. She carries the torch for all who’ve been lost. Your heart overflows and you’re reinvigorated, ready to take on tomorrow.

With each passing day it becomes more clear that normalcy itself is overrated, if not completely irrelevant. 

So you  rise, ready to meet each day because in your heart, you know she, and all the beautiful children like her, are worthy of the world and our fight to provide them life.

Bring on the next 228 days.
    

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.