Friday, May 27, 2016

Best Interests

The last week has been crazy. I guess it started last Friday with the pulling of Avelyn's chest tube. For those of you who don't know the significance of this, IT's A HUGE DEAL. Avelyn has battled chylothorax for months. There were many days that I thought we would lose her to it. 
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!

Thursday, May 19, 2016

Hope on the Horizon

The last week has been hard. The world lost yet another precious baby to CHD. Olivia Grace was Avelyn's neighbor who fought and overcame so many challenges during her 4.5 months on this earth. She could light up a room with a smile and did so frequently. She had the longest eyelashes and wavy ginger hair. She loved to kick and dance for her family and even did a few peepee dances for Avie.  
Her mother advocated for her with a tenacity like I've never seen before. Olivia was born into such unfair circumstances but she made the most of them. In her short life she touched thousands of hearts. After being next door to them for months Sean and I grew to love Olivia and her family. Her fight became our fight, and I know her momma and grand momma would do anything for Avelyn, just as I would've for Olivia. The loss of Olivia was and is felt acutely across the unit, just and those before her. In the five months we've been here, the unit has lost seven children. That's seven lives cut short and seven families torn apart. Each child lost is a tragedy keenly felt. It's awful. So awful.

Three weeks ago we were trying to prepare for our sweet Avelyn to be among that number. I use the word "prepare" so loosely. There's no way to prepare one's self for the loss of a child. How could you? While God has seen fit to continue to bless us with our little Avelyn, we have seen dark days. Days where Sean has literally had to pick me up out of the floor when my legs could no longer hold the weight of sorrow. Days when we discussed how we would memorialize our daughter when the time came. Days when hope was but a glimmer in the darkness. When we thought that glimmer was all but lost, it started to grow and she started to improve. I know Olivia has had something to do with it. As I told her momma, instead of resting behind the pearly gates, she went straight to work for her friends.
However, despite Avelyn's improvement over the last few weeks, we have no guarantee of ever seeing her grow into a strong young woman, go to kindergarten, see her first birthday or even ever hearing her voice again. Each night I pray for such things, but I am sure Olivia's momma did too. CHD is so very cruel.
I am not going into all of this for the heck of it. I just want to convey that our children are more than statistics. They are children first and foremost. Children- who've been stricken with the misfortune of being 1in 100. 
I can't remind the world enough that 1 in 100 babies are born with Congenital Heart Disease. And while most forms aren't as severe as Avelyn's or Olivia's, 1 in 4 born with CHD need some sort of surgical intervention to survive. 
If Avelyn was born 15 years ago, odds are she wouldn't still be here. The surgery to unify her MAPCAs was only first pioneered in the last 20 years. I owe my daughter's life to recent medical research, all of us heart parents do. I can't stress enough how valuable congenital heart research is. My daughter's survival, growth and development are all dependent upon ongoing scientific research. Please spread the awareness about the frequency and horrors of CHD. Please tell people of Avelyn's odyssey. Tell them of Olivia or Emma, or the countless other babies who have been lost to this disease. Spread awareness that with more research, more can be done to save these babies. Please look into the  http://www.childrensheartfoundation.org/ to learn more about CHD and donate if you can. Also look into joining "Team Avelyn" for the Nashville Congenital Heart Walk on Saturday June 4th. Every little bit donated helps and goes towards saving the lives of children who fighting legendary battles. http://events.congenitalheartwalk.org/site/TR?fr_id=1503&pg=entry


Avelyn Update:
Avelyn is making progress! It's slow progress, intermingled with setbacks but progress none the less. Her ventilator setting have been weaned over the last week and she seems to be tolerating it well. While her lungs are still not as efficient in gas exchange as a normal kiddo's lungs, she has made some great strides.
She is currently being weaned off a couple sedative drips so she will better tolerate extubation. Getting her tube out with the drips in place may be dangerous as she could get too sleepy. The withdrawals have been bad and difficult to watch. But at the same time, narcotic weans mean she is in a much better place than she was just a few weeks ago. That's what I have to keep telling myself anyway. Thinking of how far she has come, makes it easier to see her struggle with the weans. 
Her kidneys are still in rough shape. She doesn't pee much but she is still peeing some. The biggest disappointment recently has been the return of the chylothorax when she resumed full fat breastmilk. As of now it's still low volume output but it seems to be trending up slowly. I don't know. I guess it's a wait and see kind of thing as to when the chest tube can come out, and what the plan for full fat verses skimmed. The body cannot reabsorb the chylous output like it can blood or serous fluid. So if she continues to have the fatty leakage and her chest tube is pulled it will build up and collapse her lungs. That would be bad and she'd need another chest tube put in. That's not something I want to chance. As for now I am advocating leaving the chest tube in place, it's already been in for months anyway. I don't see the point in rushing into pulling it out.
So that's where she is and where we are. We upon the cusp of uncertainty and gaze upon a future that terrifies and excites us. Big days are ahead. Avelyn could regress or not tolerate the coming interventions at anytime. Or she could do well. We pray for the latter, and hope you will too. 
Best wishes and love to each of you. Thank you for all the love, support , thoughts and prayers sent to us and our dear Avelyn. 
She may be small and her heart may be frail, but she has the spirit of a warrior.
    This last photo is of my precious friend Laura and her beautiful Olivia Grace, just days before Olivia gained her wings. I will always love and miss you sweet Little Livie.

Tuesday, May 10, 2016

The Agony of Limbo

The last few days have been yet another roller coaster ride. Avelyn made strides of improvement Thursday, Friday and Saturday. She got just over 700ml of fluid off in just three days. We were all quite astonished and despite our guarded hearts, our hope soared. 
On Sunday her dialysis was changed to a lower concentration of dextrose, due to the hospital running out of the higher concentration. (The higher dextrose concentration the more fluid is pulled off with the dialysis.) The team knew that there was a shortage of the dialysate on Friday, so it wasn't like running out was some massive surprise, but that's how everyone reacted. Also a different nephrologist came on Sunday. I don't believe the off going nephrologist communicated well to the on coming nephrologist. Avelyn's need for diuresis is dire, multiple organ systems and her life in general depend on her getting the extra fluid off. It's top priority right now.
However, the on coming nephrologist saw that the higher dextrose dialysate was low in magnesium and calcium-which are electrolytes that often build up in individuals with chronic renal failure. She thought using that would cause Avelyn's mag and ca+ levels to drop-which it very well could, but hasn't. She had been on this dialysate for days without issue, she had done extremely well on it. But alas, the nephrologist discontinued it and all of Nashville was searched for a dialysate bag with a high concentration of dextrose and normal concentrations of magnesium and calcium. None were found. So for two days she was on the low concentration of dextrose. Meaning for two days she did not pull any excess fluid off, and today she was positive 100ml. The significance being, she not only did not make progress but she regressed. She didn't just regress from a fluid over load standpoint, her entire clinical picture is worse. Her heart function, her respiratory function and ventilation,as well as her agitation level- all of it is worse. 
While it's not as bad as it was Thursday, it's a significant regression. A regression that Avelyn could not afford, especially due to lack of communication and logistical nonsense. 
Frustrated does not discribe how I feel about all that's happened. I am sickened, devastated, and angry but that still doesn't adequately describe how I feel. I have been watching Avelyn struggling to breathe today. She is working so hard that her temperature and chest tube output are both up. Her ventilator settings, that were just weaned a little yesterday, are back on nearly highest settings. It's awful and the fact that this could have been avoided and she could have made more progress only makes it worse. Avelyn can't afford these types of failures, because despite how the team has tried to convince me otherwise, that's what this situation is-a failure. It's a failure in doing all that is possible to help her. It's forcing her to struggle unnecessarily, something Sean and I have promised her we would prevent.
On Friday we decided to make Avie a DNR. Meaning of she were to go into cardiac arrest we have asked that the team not do CPR on her. As much as we love and adore our daughter, we think this is for the best. In our minds, passing away would not be the worst case scenario for our precious baby. Exposing her to further pain and suffering would be. She has had her chest opened four times now, with the most recent being only two weeks ago. Chest compressions on an unstable and unhealed chest would cause trauma and pain. So much pain. It's a struggle to treat her gas pain, let alone that of flail chest or broken ribs. Plus, the episode would only make her prognosis worse. She would likely receive more brain and organ damage than she has already sustained. It just wouldn't be fair to force her to endure such pain for a life also filled with pain and suffering. 
Deciding to make her a DNR was the hardest thing Sean and I have ever done. We did it because we love her, not because we are giving up. We want more than anyone can imagine to watch our baby grow, but are not willing for her to suffer for our selfish desires. We continue to stand by her side, cheering each accomplishment and advocating for her as best as we can. 
She continues to fight with unbridled tenacity. Her spirit continues to amaze me, and I am so proud to be her mother. If any baby can over come these circumstances it's my Avelyn. 
Thank you to all who have prayed for our dear Avelyn. The progress she has made is encouraging but please keep the prayers coming. Her survival and recovery are dependent upon a miracle. We are blessed to have seen her come this far but she still has many mountains to climb, with each being higher than the last.
 To all that have given aid to us, thank you. Your aid has allowed me to stay here with my baby. I will never be able to adequately describe my gratitude or thank each of you enough, but I will spend the rest of my life trying. Your generosity is awe inspiring and more appreciated than you will ever know. From the deepest most part of this momma's battered heart, thank you. 
 
Avie isn't a fan of bright over head lighting ;) She seems to actually like these glasses her Grammy got for her. Plus she looks pretty darn cute if I must say so myself!

Thursday, May 5, 2016

Bent Knees and Broken Hearts

As you all know, our sweet Avelyn has been plagued with complications. Her prognosis going into her first open heart surgery was quite positive, but things have drastically declined since those early days. She has survived seemingly insurmountable odds in her short life, and yet we are only faced with more. 
Our precious Avelyn has struggled since her tenckhoff catheter placement and most recent heart surgery. She has packed on so much fluid that at times she was barely recognizable. She has struggled with pain and mechanical issues like a clogged and/or leaking PD catheter. She went days without dialysis due to these issues. Now she struggles with ventilation. She is on such high ventilator support it is causing damage to her lungs. Yesterday she developed a pnuemothorax and today her left lung collapsed due to trying to keep her right side elevated to heal the pneumo. We have also learned her kidneys are scared, meaning permanent damage-though we don't know the degree. 
When you add all this up it doesn't look good for our precious girl. We had a care conference today, where we met with those involved in her care. We were told that the chances of her surviving at all aren't good, and the chances of her surviving with a quality of life Sean and I would hope for her are quite slim. 
She was given a week to try and the extra fluid off. Getting her back to normal size will be a challenge that she may not accomplish. Even if she does manage to get back to her dry weight, she'll still have so many other mountains to climb. First and foremost, she'll have to come off the ventilator. We don't know if she'll be able to accomplish that, the doctors aren't too hopeful that she will. She's been ventilated for months.
While we have not given up hope, or given up on her, we have to be realistic. And that means the next couple weeks are likely all we have left with our precious baby. She has been put through so much pain, and while she continues to fight on, we do not wish to put her through any further pain unnecessarily. 
As one can imagine, Sean and I are beside ourselves. I am supposed to return to work tomorrow but can't bring myself to leave her room, let alone drive 5 hours away. We have been trying to savor every moment we can with our beautiful daughter.
She is such a precious soul. 
She deserves comfort and peace, please pray God sees fit to grant her such and that we may bear witness to it.
 

Wednesday, May 4, 2016

Rest Now My Love

Rest now my love 
For the scalpel's leavings are fresh
Rest now my love
For it is time to heal

Rest now my love
For I know you are weary
Rest now my love
For I see exhaustion in your eyes

Rest now my love
For I know you are hurting 
Rest now my love
For I see your pain and tears

Rest now my love 
For tomorrow is a new day
Rest now my love 
For the scars shall surely fade

While you rest my love 
Know you are strong
Know you are brave 
Know you are beautiful 

Rest now
For you are loved