Silent Cries

Poor Avelyn has been struggling with so much pain since her hematoma evacuation surgery. In the initial 36 hours post op period it got progressively worse. By this morning I was at my breaking point of watching her suffer. 

I have been saying since immediately post op that she needs ketamine to adequately treat her pain. Before I begin my tirade, I have to acknowledge that the two nurses who've had her since sugery have tried. They too have been so frustrated. I told providers in each set of rounds that she needed ketamine. I heard both nurses tell various providers, "mom says Ketamine works.."

No one wanted to order it. 

I understand it's a more powerful drug. 

I understand it is associated with delirium.

I understand it's usually saved as a general anesthetic. 

This isn't our first rodeo around here. 

Avelyn's opiate receptors are saturated, throwing more diluadid and fentanyl at her does nothing but make her delirius. 

This morning I showed my butt, ironically it was to one of my favorite providers, an NP who knows Avelyn well.  She also knows me well enough to know I wouldn't campaign for over sedating my baby. I got upset telling her about how much Avelyn has been suffering and how I wasn't willing to let it continue. She suggested consulting the pain team and got Avie's surgeon to bring it up in surgical rounds. The pain team consult was great start and I knew they would help.....eventually. But Avelyn needed relief right then. I got upset when she needed to have her line resutured and PD dressings changed. I was rude and I hate being like that, but unfortunately it seems like sometimes that's the only way things will get done. 

I know my baby. I know her opiate doses look insane. I know for most babies it would be more than adequate, but most babies haven't had their chest open and closed four times-even here in our hellacious situation.

So anyway, she got ketamine and immediately found relief. That was around ten this morning, she didn't need another PRN until around four. She slept, and without a grimace, for the first time since before surgery.

Past the pain, Avie's doing alright but is a bit sicker as we expected post op. She's really puffy now, but not due to needing volume from a hemodynamics standpoint. Her blood pressure since returning from surgery had been stable, thank God. She didn't require any cardiac meds during surgery or after. She is puffy because her kidneys took a hit and urine output has dropped again. Her PD is also leaking again so her dialysis hasn't been as effective. She's really puffed up in the last 24 hours. It's so sad, especially because I know it will take her a long time to get the fluid off. However the good news is, she is still making urine. It may not be much, but it's something and that's encouraging. The bad news is she'll still need dialysis for a while. Maybe weeks, maybe months, maybe a year. There's no way to say. Right now she has a temporary PD port that was placed at her bedside, and it's her second. These temporary PDs are prone to dislodging and leaking. Once they start leaking they aren't really salvageable. 

So Avelyn is tentatively scheduled for general surgery on Monday to have a more permanent peritoneal dialysis catheter inserted into her belly. This one will work until she doesn't need it anymore then it will be surgically removed. That'll be a good day.

And finally I figured I should mention (as an extremely understated "after thought" as not to jinx it in any way), Avie's chest tube output is down. Way, way down-like less than 100ml/day down. For this we are so blessed and thankful. The end of chylothorax is upon the horizon, I just know it.

Please keep our precious girl in your thoughts and prayers. As I have explained before she will likely never be "out of the woods" but we might be finding our way out of a dark cave and making our way back onto the path of recovery. Only time will tell.

The photo below was taken of Avelyn's window before we got to go back to see her after surgery. You hold on to the strangest things in these situations, for instance the lack of rushing around in her room was very encouraging. The second is from when Avelyn was a day old. I know it may seem to strange to those who've never been in our situation, but I long to hear my baby cry again. I long to hear that sweet precious cry as evidence of her stretching her lungs and pulling air all on her own. It's been so long since we heard her sweet voice. Her silent cries grate at the soul and tear at the heart. Hopefully vent weans are in the new future and she tolerates them well. 

Thank you for checking in on our girl and for the continued support and encouragement. She's still putting up one hell of a fight. I couldn't be more proud to be her mom.

Back to the OR

Our sweet baby goes back under the scalpel today-within hours actually. Her surgery is listed as a hematoma evacuation. The tests found a large mass collapsing her right middle lung lobe and another smaller one collapsing the left lower. This mass is why she hasn't been able to come off the ventilator. She has had multiple echocardiograms, ultrasounds and an MRI to try and figure out just what the mass is comprised of. She was unable to go to FornamCT scan due to the damage the dye would inflict upon her kidneys. Without the CT our worst fears cannot be ruled out. The mass may be a very large aneurysm. The surgeon believes it's most likely a hematoma but there's no way to know for sure. The plan is to prep her for the aneurysm repair just in case. That means they will locate and prep the large vessels in her neck in case she needs to go on heart lung bypass. There are too many if's associated with an aneurysm that size. If it is an aneurysm she'll bleed. A lot. The surgeon may not be able to control the bleeding. She'll likely end up on ECMO again. She'll lose her kidneys, they are just still too sick to recover from another bypass run. She'll have to get so many blood products and fluids. She'll need more intensive dialysis than she's currently on to get that fluid off. Meaning I am sure hemodialysis will be on the table. So so many if's, each more terrifying and devastating than the last.

If it's a hematoma the surgery should be shorter and less complex than any she has had previously. She'll still puff up and be sicker through this week, but we hope with better pulmonary expansion and venous return she'll recovery more quickly. 

I am beside myself as any other mother would be. Waiting to hand off your helpless child to an awaiting surgeon never gets easier. It's feeling no parent should feel and yet we've grown accustomed to it. This will be the fourth time in the operating room. And that doesn't include the heart caths or the in room operations. 

Sean and I haven't been able to talk about her going back much. It's just too hard. It's too hard to think of her freshly healed chest mangled again. It's too hard to think of her struggling through withdrawals again. It's too hard to think of her too puffy to open her eyes. It's all just too hard. And yet it's what Avelyn must endure to live. I pray we've made the right decisions for her future and well being. I pray she has a future. I pray for independent breathing and pee pee. I pray for negative fluid balances and avoiding infections. I pray she improves and we can get to know her better. 

Until then I'll continue to etch each facial expression and detail of my itty bitty baby into my very being, because everything thing about her is precious.

Rooted in the Woods

Avelyn has been having another rough week. She has seen gains in some areas but serious set backs in others. 

Her chest tube output has plataued at a lower rate~ 200ml a day. This is great considering how much she was putting out just a week and a half ago. This means she's holding on to more nutrition and electrolytes. It also means she's not having to get as many replacements to help her maintain a baseline. However 200ml's is still a lot of output and she has a long way to go.

Her dialysis has been working pretty well. She has been able to get some of the extra fluid off that was making it difficult for her to expand her chest. She still has some edema left to resolve but she'll get there. The nephrologist came in Friday and said her kidneys are likely permanently and significantly damaged. She insinuated that they were no longer expecting her to make urine. The news broke my heart. Then later that same night Avelyn peed for the first time in weeks. It was only 8 ml but she peed. I was more proud than Inhave ever been in my life. I took the diaper around the unit and we all celebrated her 8 mls. Nurses were coming into her room left and right to congratulate her-it was a huge deal. 

Since then she has averaged about a small diaper a shift. I have been really encouraged by her urine output but then today we may have hit another snag. Her peritoneal drain was leaking into her diaper, which likely throws off her "urine" counts. She had the site redressed and secured today so hopefully this won't continue to be an issue. However it makes me wonder just how much of the fluid we have been celebrating was actually urine. Only time will tell. Keep the pee pee prayers and dances coming.

This week's bad news is Avie's has been having a lot of trouble with ventilation recently. She has been having a ton of gas that gets her heart rate up near 200min and her blood/right atrial pressures up. In these episodes she breathes really fast and shallow causing her to get acidotic, which just makes everything worse. 

Thursday and Friday nights she had some pretty scary episodes. Thursday night she got a large mucus plug that completely blocked her right airway. She had no air movement in her right lung. Her sat's dropped for a few minutes; her heart rate and blood pressure also started to drop before they could bag it loose and suction her out. Her ventilation hasn't been the same since. She's needed to go back up on all of her vent settings. We were gearing up for extubation in the coming week but now that's completely off the table. Currently the team doesn't know what's going on. She was started back on nitric oxide to see if she was having issues with pulmonary hypertension. It hasn't seemed to help. Her pulmonary hypertension is due to small pulmonary arteries, which will only improve with growth (we hope) and cardiac caths, so I didn't figure it would anyway.

Also over the last week her cardiac margins have been expanding on her x-rays. We don't know the cause so far. She had an echo to look into cardiac function due to the increasing size but it didn't show any strongly significant findings. The expanding cardiac borders is a really terrifying new development. It could be something very serious or it could also be that her right lung is partially collapsed in the same area of her right atria-making her heart border look larger. The doc says this partial lung collapse would be best case scenario. She'll have a bronchoscopy tomorrow, thisnentails putting a camera into her lungs to investigate her airway. If they find any stubborn mucus they'll levage her. 

If they can't find the source of the problem she'll have an MRI later in the week. Normally in these circumstances a kid would have a CT scan but given her poor kidney function she wouldn't tolerate the dye. The bad thing about having the MRI is she'll have to have her pacing wires pulled before going down. This makes me nervous. Her pacing wires are inserted directly into her atria and ventricles and have saved her life or at least saved her from needing CPR in the past. No longer having them there as a fail safe worries me. 

I feel like just when we start to see significant progress and a possible light at the end of the tunnel our girl gets knocked back. I've been thinking a lot about how Avelyn will never be alright. Many CHD kids won't be. They will always struggle with their disease.

Even in the best cases, they may grow and thrive, but worry free vistas of health are not on the horizon. Instead we will always face forests of challenges. Avelyn will always need to be monitored closely by a cardiologist, and now likely a nephrologist as well. She'll always be more susceptible to illness. She'll have developmental delays and will lag behind her peers as they run and play. 

That's best case scenario. Worst case scenario, she'll never leave this hospital alive. That's a real possibility but I believe with all my heart that if any baby can over come these specific circumstances it's my Avelyn. She's tough and she's fighting with all her might. I believe she has a lot to offer this world and can grow to be someone remarkable. She is already such an amazing little person and she's never left her bed. 

So despite all her challenges and setbacks, I am not giving up on her. In the last few months we've pitched a tent in our proverbial woods but I think it may be time to build a cabin. Sean and I are in this for the long haul, because Avelyn is in it for the long haul. I'm alright with never getting out of the woods. There's a beauty to the Forrest that a wide open vista could never touch. When you live among the trees, you learn to see the beauty of the easily overlooked. The waterfalls and wet diapers. The liveliness of new life and the lasting impressions of those we've lost. The peaceful stillness and precious slow blinks. The rustle of leaves and the wiggle of toes. We might've missed or taken for granted a lot of these "little" but beautiful things if we were looking upon a vast vista.

We cherish our girl and will always be there for her, however and wherever she needs us.

. 

Progress, Setbacks, and Heartache

Sorry to have not updated this week. It's been busy and trying. One of the families that I've grown close to had to say goodbye to their precious baby yesterday. Sweet E fought so hard. Her battles are those of legends, and she overcame so very much in her short time. Her parents are some of the kindest most wonderful people you'll ever meet. Little E and Avelyn have a lot in common. Both are victims of circumstance. Both struggled against infections, blood clots and dialysis. They could've grown to be sweet friends, now Avelyn must carry the torch for them both. I hate it.

I hate that the world has lost such a precious soul. CHD steals lives before they've even begun. It's not fair; that precious baby deserved a beautiful life with her spectacular parents. It's not right. No baby should have to endure the horrors our kids have gone through. Yet here we are and here we'll stay, because that's reality. 

Sweet E and little Avelyn are 2 of the 40,000 kids born annually, in this country alone, who must fight for their lives from the first breath. It's a cold and broken reality, but CHD can strike any family at any time. Please raise awareness and share the tragedy of this disease. We need more funding for the treatment and care of these little warriors. CHD research is vastly underfunded given the frequency and severity of disease.

Avelyn Update-

Avelyn started off the week in a bad way. 

Her PD got clogged and then drained all around the site. It's a long story but essentially this caused her to retain potassium and to become ulcerated around the PD site and her arterial line. She also become extremely delirious and ended up not sleeping for nearly 36 hours. During this time she was dealing with pain from her belly not draining, ulcerative wound sites and also extreme constipation.

 Her blood pressures stayed elevated and her chest tube output has been trending back up ever since. She cried much of that 36 hours. It was so heart wrenching. Her PRN dilaudid was changed to fentanyl and that seemed to help her pain and restlessness. As the week wore on she grew more edematous and her belly hardened. Wednesday she got a new PD site which allowed dialysis and feeds to be restarted. 

Since then she has lost a lot of the extra fluid and she's starting to look like herself again. She still has a long way to go but she is slowly getting some fluid off. 

She still hasn't peed but I have tried to not agonize over it. I believe she'll get there with time. I have to believe that, the alternative is too unbearable to consider.  

She is tolerating small weans on ventilator settings for now. Hopefully we can get all the extra fluid restricting chest expansion off in the next week to get her better optimized for extubation. Please pray the chest tube output starts the trend back down and of course, as usual, for pee pee.

Love to you all. 

Unapologetically Emotional

While Avelyn is by no means out of the woods, she has seen improvements in the last couple days. Her kidneys are still in terrible shape and have yet to improve at all, but her chest tube output is down by half. This is huge. She went from putting a liter a day out of her chest tube to putting half a liter the last couple days. And she seems to be maintaining this lower rate. However things could change at any time so we aren't counting chickens just yet. 

Her kidneys are doing a bit worse. She hasn't been producing hardly any urine at all and has been retaining more fluid. The extra fluid has made her trunk more rigid and is collecting in her lungs. Both of which has made ventilating her more difficult. She is on high vent settings again. Due to all the extra fluid and her not urinating, peritoneal dialysis was restarted. So far she is tolerating it alright but it's only pulling enough fluid to keep up with what is going into her. It hasn't been able to pull any extra fluid out of her interstitial spaces yet. Hopefully that will change before long but Avelyn is such a fragile baby. She can't handle big changes or aggressive fluid shifts. Slow and gradual is much better for her sensitive systems. She'll likely be on dialysis for a while longer.

So basically while she has taken a couple steps back with her lungs and kidneys, her chest tube output has improved. With the decreased chest tube output we hope she can start retaining her nutrients and protiens she needs for healing-though she still has a LONG way to go before the output resolves completely. Half a liter of drainage is still a lot for a tiny infant to put out each day. 

But we have faith. She can and will get through these challenges. We'll continue to pray from further improvements and do our pee pee dances. We can't give up, she's come too far. 

She is teaching us so much about patience and faith. I am not the same person I was four months ago. She has changed me in ways I never thought possible. For those of you who know me, you know I am not an emotional person. Before we leaned of Avelyn's diagnosis I could count the number of times I cried a year on one hand. I never allowed myself to become emotional in front of others. Not because I was afraid of showing weakness or anything of that sort, but rather because I have just always been one to keep my emotions to myself. 

Since having Avelyn and embarking upon our journey with her that seems to have gone out the window. I tear up all the time and am completely unapologetic about it. Some days, when Avelyn really isn't doing well or is in a lot of pain, I'm a mess all day. I don't try to hold back my emotions, not that I could anyway. Our lives are too stressful to worry about such frivolity. 

It's not uncommon for me to tear up just looking at Avelyn. She shouldn't still be here, it's by the grace of God she is. We've nearly lost her half a dozen times. 10 years ago she likely wouldn't have made it to this point. She's our little miracle baby. 

And what's more amazing, she isn't the only little miracle baby here at Vanderbilt. The unit is full of them. We've grown close to the staff on the unit as well as other families. I truly love many of the people we've met on our heart journey. The victories and set backs of our friends' children are keenly felt. We root for one another's babies like we root for our own. The staff not only save our babies' lives but they grow attached while caring for them day in and day out. You can see love in some the staff's eyes as they talk and interact with sweet Avelyn. Some of these people truly love my baby. These people were strangers four months ago but have cared for my child as though she were their own. They have cried with me on the bad days and offered encouragement when we've grown weary. These people have become like family. Our PCICU family. I love this unit and the people we've encountered, though I wish we'd never needed them. I will never take the fact that my baby wouldn't be here without them for granted. I will be forever grateful and will never forget those we've met here-families or staff. 

 

Praying for Miracles

What do you do when doctors tell you there nothing more they can do for your child? I don't know. I have been trying to figure it out since Monday when Avelyn's surgeon spoke to me at length. It seems my fears are anything but unfounded. 

Avelyn is in such a dangerous place. Her chylothorax and renal function have not improved at all.  Her infectious markers have been trending up for three days. She was tolerating being weaned off the ventilator well over the weekend but now isn't. They are having to go back up on settings. With all the new complications it is feared something else may be inhibiting her progress. Blood, trach and urine cultures have been obtained and she is scheduled to have an abdominal ultrasound and echocadiogram tomorrow. 

Her chest tube and PD are still putting out about a liter a day. It hasn't showed any signs of slowing or stopping. Her urine output is scant. The nephrologist came by today and admitted that while she could regain renal function if things turned around very soon, each day makes the possibility less likely.

We have yet to hear back from CHOP, but I fear they won't be able to do anymore than what is already being done. We should hear back in a week or so.

The unit's centrifuge is still broken so Avelyn still isn't getting skimmed breastmilk. She's getting a special formula instead and I don't know if she is tolerating it. I was at first told that I couldn't manually skim my breastmilk for her, and then days later told I could. So I went through the whole process twice of skimming it (the milk has to sit for 12-24 hours before each skim) but it still had some fat in it. The nurses saw it and tossed it all. I couldn't help crying. Right afterward, the doctor came in asked me where it was and said it could be sent down for testing to see if it was alright to give her. I didn't trust myself to tell her it had just been thrown out, I was already on the verge of a breakdown.

 That milk was to be my only contribution to my baby's health. Skimming it was literally all I could do for my baby. And then it's thrown in the trash. It'll take two days to skim more. Two days of Avelyn going without the potential benefits. The centrifuge is supposed to be working again by Friday. I pray it is. I know breastmilk won't cure her, I am not stupid. But I also know it's better nutrition than she is currently getting. She is so malnourished due to her chest tube losses. TPN and lipids have fattened her checks but her limbs are emaciated. Her belly is so distended from fluid and liver enlargement. 

Oh, she's just so very terribly ill and yet,

she's fighting so hard. 

So here we are again, facing the potential loss of our sweet baby girl. We are so scared and are at a complete loss. I know I should feel blessed to have spent the last three months by her side, and I do. But I want so much more time. We need so much more time. Sean and I want to see her smile again. We want to hear her voice. We want to see her grow and laugh. The thought of not getting to is unbearable. 

Thank you to all who have given to us, supported us, prayed for us and encouraged us through this terribly difficult time. I hate to continue to ask but please keep Avelyn in your thoughts and prayers. Please pray the drainage slows and then stops. Please pray no further complications arise and her little kidneys recover. Pray God blesses us beyond measure and heals our baby girl. At this point we're waiting on a miracle. 

100 Days

Avelyn hasn't had a good week. She hasn't gotten any better. If anything she's gotten worse. Her chest tube output is still around a liter a day. Her urine output and function has also gotten a little worse. By Wednesday I couldn't handle to the sit and wait approach any longer; I told the team I wanted a second opinion. I had researched chylothorax and thoracic effusions quite a bit and found that Children's Hopsital of Philadelphia is the leading treatment and research facility in the world. That's what I gathered anyway. We sent Avelyn's treatment records on Thursday and hope to hear back soon. 

In the mean time I guess we are still waiting but that doesn't mean things have slowed. Avelyn is still being weaned from her sedative medications and is having a hard time of it. Her heart rate and blood pressure have been high and she's restless. The team started her feeds back on top of the withdrawals. The centrifuge that was skimming the fat out of her breastmilk is broken so she got straight formula. It's a special low fat formula due to her chylothorax. She had it before her most recent surgeries as a fortifier with skimmed breastmilk, and tolerated the combination well. However yesterday when the hourly rate of formula was increased, she started to get uncomfortable and having extremely watery stools.  The diarrhea could be withdrawal related or a signal of her not tolerating the formula for whatever reason. I asked them to test her stool for blood last night just in case. Of course, it was positive. She's on heparin again and I am hoping she just isn't tolerating the formula in general. I pray it's not a purfusion issue or NEC. Her X-ray showed a possible pneumotosis so we're headed down the NEC rule out road again.

Her chest tube site looks awful. It's been in for two months now, and has been draining an average of 30-50ml an hour. Sometimes it leaks around the insertion site and dampens the dressing, which has caused the site become ulcerated. It looks so painful but there nothing to really do about it.They can't pull it because she'll suffocate if the fluid isn't drained from her chest. They just have to change the dressing a couple times a day and try to keep the site dry. It's so frustrating. It just something else paining her that supposedly nothing can be done about.

Her breathing has seemed more labored over the last couple days too. Again, I hope its withdrawal related. My gut tells me it's not but we'll just have to see. Either way she won't be coming off the ventilator any time soon. 

We had great experienced nurse yesterday. She thought Avelyn was stable enough to let us hold her. It's been two months since she was last held. It took four nurses to get her into my arms. I cried for the first couple hours and Sean was emotional too. It was a nice change. She has changed so much since she was last in my arms. She is so much heavier and longer. Her eyes hold more expression.

Living in this environment, it's difficult to feel like parents. We never get to hold our baby. We don't get to change her diapers. We've never loaded her up in the car and taken her anywhere. She's never seen outside of the hospital. She's never felt the wind on her face or seen the brightness of the sun. She knows nothing but her Hopsital crib and room. 

And it's been so long..... 

Yesterday I saw that her length of stay was 99, and given we've been here since she was born that means she was 99 days old. 

Today marks 100. 

100 days.

100 days of our sweet baby fighting for life. 100 days of looking at her through or over rails. 100 days of tubes, lines, beeps, blinking lights, doctors, surgeries, scars, drips, pain, and struggle. 100 days of uncertainty. 100 days of longing. 100 days of tears and questioning 'why'. 100 days of avoiding other young families because we just can't handle it. 100 days of staring into those deep blue eyes and apologizing for the pain and discomfort.

100 days with our sweet precious Avelyn. I so wish her life was full of kisses, smiles, bottles and baby chatter. I miss the few precious weeks I got to hear her sweet little voice. It's been so long. We've been here so long. I don't know how long she can keep going in this state. She is so fragile. It's terrifying. Everyday I worry about what new complication will find its way to her. There's always something to hold her back and keep is here longer. 

I am still so hopeful that the day will come when she will be free of her cords, tubes and monitors. When she will breathe easy not not require pain medicine. When people will remark on her rosiness of her cheeks and not the pale blue of her lips. We'll get there one day. I hope it doesn't take another 100 but we'll get there.