Tuesday, August 23, 2016

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. :(
     

Saturday, August 13, 2016

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. 

       

Monday, August 1, 2016

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.