I still vividly remember calling my pawpaw in hysterics in early April. I had just been told there was simply nothing that could be done for her but to sit, wait and hope it got better. I was beside myself. Who wouldn't be after months of seeing their child slowly waste away due to pouring vital nutrients from a chest tube? My sweet pawpaw has always been my go to Mr. Fix It, and while I don't think he alone fixed Avelyn's chylothorax, I know his prayers and encouragement gave me strength to continue.
So anyway, her chest tube was finally pulled. And surprisingly after months of hoping and waiting, when the day came, I begged them not to pull it. She had been having chylous output again and I was (and still am) extremely nervous she'll get a chylous effusion. See the body cannot reabsorb the fatty chylous output like it can blood or serous drainage, if she gets an effusion she'll need a new chest tube implanted. So far she is doing extremely well with it and for that I am so thankful.
Then Saturday morning rolls around. We're not even 24 hours out from the pulling of her chest tube, and I am told she's to be extubated. I was floored. Terrified. Ecstatic. Ok quite honestly, I didn't know how to feel. While I knew she was on minimal ventilator settings and that extubation was on the horizon, we weren't expecting it until this week. When the time came Sean and I were in a surreal state of anxiety and excitement. Extubation?!
We've had been waiting for this day since she was first intubated on January 29th for endocarditis. We prayed for this day. Hoped for it. Longed for it. We were told on multiple occasions it may never come. We were told to expect failure but we hoped for the best.
We spent the morning telling her how much we believed in her; how proud we were of her; and how strong she is. She looked so good, she was playing and interacting with us all morning. Then, seemingly so suddenly after four long months, the time came.
I didn't know what to do with myself. Sean and I didn't call or tell anyone she was being extubated, partly because it was so surreal and partly because we had been told so often to expect failure. But we hoped. We prayed. We paced. We fidgeted. I was a bundle of raw nervous energy. She has been intubated for four months. She doesn't remember life without a ET tube down her throat. Sometimes I too wondered if I remembered her without the ET tube. It's just been so long. To complicate matters, in all that time her little muscles have grown deconditioned.
She was extubated to a BiPAP, which is basically like a CPAP that provides extra support when she initiates a breath. When the doctor came in to tell us she succeeded in extubation, I can't begin to describe the joy, relief, and fear that consumed us. Mind you, we were still being told to expect failure in the first 24 hours. The providers were expecting her to get tuckered out and need reintubation. As the hours passed and her blood gases looked good, we saw providers dancing in the halls. Everyone was (and still is) shocked. Nurses are still stoping in "just to see her" without the tube. That Saturday the whole unit seemed to be in celebration.
For the first two days she did great. Initially, she did much better than anyone was expecting and then things started to turn. Her secretions started to thicken and turn darker. Sean and I knew she had an infection brewing but her lab work looked alright, so the team held off on antibiotics despite our requests. Then BiPAP function began to cut in and out. Meaning there were times that Avie wasn't getting the extra help when she took a breath and other times it was providing rapid bursts of air that she didn't need.
Sean and I brought it to the team's attention right after it happened the first time. When the respiratory therapist came into investigate it was working properly (figures). Then as time went by it got worse. It would cut out more frequently and for longer periods of time. Avelyn progressively started to look worse and worse.
By Tuesday night, I felt a clear pattern had established itself. I asked for her to be switched to a high air flow nasal cannula. While this sort of intervention is technically less support than a properly working BiPAP, I figured it was worth trying. The attending that night didn't think it was a good idea.
By Wednesday morning Avelyn was struggling, like really struggling. At this point her BiPAP function was cutting in and out frequently no matter what the respiratory therapists and nurses tried. Again, Sean and I asked the morning attending to try vapotherm. We were told it wouldn't provide her enough support and that the BiPAP would be better for her. I argued that a working BiPAP may be better, but that this one wasn't working. He agreed and to this, and to his and the respiratory team's credit, they tried. They worked for hours trying to get the BiPAP function to work. They tried different ventilators, different masks, and in the end a nasal pharyngeal tube.
During all these attempts Avelyn struggled to breathe. She spent hours struggling with each breath. She was suffering and it was awful. Finally, with the NP tube in just the right place and at just the right angle, it worked but I knew in my heart that it would be short lived. As I figured, two hours later we were in the same boat. Avelyn was stuggling and suffering yet again and Sean and I weren't having it anymore. I was to the point that I was ready for her to be reintubated. At least then she would be able to breathe. Allowing her to suffer like that any longer would have been cruel. When the night attending came in, I pleaded with him to try her on the high flow delivery system, called vapotherm. He thought about it and with an air of "well she couldn't do much worse" agreed.
Once she was on vapotherm her breathing got better nearly immediately. She wasn't working nearly as hard for each breath. She looked comfortable, completely exhausted but comfortable. After and hour the nurse got a blood gas, and to everyone's surprise it looked really good (considering). It looked better than any of her BiPAP gases, even when the damn thing was working. After one of the top ten worst days in Avelyn's life (which is really saying something, given all she has been through), all three of us could finally breathe easy.
It was a terrible, awful day and I believe a lot of it could have been avoided. While that makes me unbelievably angry and sad, as I am sure it does you all as well, I've had to take a step back. Sometimes, especially in situations such as this, it's easier to be angry. It's easier if you can place blame on someone or something. To look at the situation from another's point of view is hard, and that's without the added complication of watching your child suffer for hours. But I ask you all to not blame the doctors who didn't switch her to vapotherm when I initially asked. To give them credit, the request likely seemed absurd. Here I was asking for my baby, who was in respiratory distress, to be given "less" support. If you think about in those terms it's crazy. She could've done worse on vapotherm. It could've led to reintubation. The doctors who said 'no' to my requests for vapotherm did so because they thought it was in Avelyn's best interests.
Do I wish they would have considered my plea and reasoning before saying 'no'? Heck yes!
Yet, I'm sure parents ask them to do crazy things.l all the time.
Am I more informed than many other parents? Probably.
Do I wish this was taken into account before they just tell me 'no'? Sure I do.
But do I have a medical degree and years of ICU experience? Nope, not even close.
Also, do I think they were being neglectful? Not at all. I think they were being cautious. In many aspects, I respect that. Don't get me wrong, I am still upset that my baby struggled so long but I understand the doctors' reservations.
Being an advocate for Avelyn isn't easy. Sometimes it's a battle. It is exhausting to continue to advocate for what we believe is right even after being told it's a bad idea.
However, it's episodes like yesterday's that break our hearts and renew our vigor. Avelyn is fighting for her life and Sean and I can't afford to merely be observers. Our girl deserves our best and that's what we try to provide her.
So in short, this week was hard on our girl but as usual, she persevered. She continues to amaze and surprise everyone with her tenacity and wonderous spirit. She's still on vapotherm and tolerating it well. She's also still fighting a respiratory infection that we hope will start to clear soon. Please keep her in your thoughts as she continues her war against CHD. She still has such a long road ahead with so much uncertainty. Yet I continue to believe if any baby can overcome these challenges-it's our wonder girl.
Much love to you all. Thanks for checking in on us and for supporting us through Avelyn's oddessey. We surely would not have made it this far (and remained remotely sane) without your love.I've included photos of Avelyn's siblings because they've been troopers too and I miss them terribly. We look forward to the day we're all together again!