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. 

New Perspectives

Over the weekend Avelyn had a few new complications arise. We learned she has a peri-renal bleed, which was likely caused by the heart cath wires puncturing her inferior vena cava a few weeks back. When her heparin got to supra therapeutic levels this bleed got worse and made itself evident. Right now it hasn't damaged the kidney further but it'll continue to be monitored. Her heparin was stopped for a couple days and her GI bleed cleared and this peri-renal bleed seems to have slowed if not completely stopped. 

She also was detoxing off diluadid all weekend and underwent medication changes to help her right ventricle rest while also favoring kidney perfusion. Last night the detox caught up with her and threw her back into delirium, although not as bad as a few weeks ago. She restless and jittery all night. She couldn't get to sleep until around 6am. She's resting well now after getting risperadol, which she was able to have because her feeds have been restarted as of yesterday.

So far her kidneys haven't improved much, if at all. Her urine output has picked up a minuscule amount but her labs are a little worse than this time last week. The nephrologists still seem to be encouraged that she is producing urine at all. So we continue to hope for full renal recovery, but it'll take time.

Her left lung collapsed over the weekend again. Today it's back up but still atelectic. She is getting respiratory treatments to further improve the function. She came off the Nitric Oxide again last night. So far her Sats are in the low 90's most of the time, on 60% O2 but this will get better as she acclimates. 

Her labs that monitor infection have been jumping even though she's on a ton of antibiotics and an anti-fungal. They cultured everything yesterday and so far there hasn't been any growth but it's still really early. Hopefully it's due to something very treatable or just stress of detoxing. It's all such a Rollercoaster of ups and downs. 

Her echocardiogram showed a bit of improvement on her left side. She is now showing normal functioning of that side. Last week it was depressed, so that's good news. Her right side isn't yet showing any significant signs of improvement, nor is her tricuspid valve regurgitation. However the cardiologists believe with time her right sided function should improve. She'll never have the pulmonary or cardiac function of a normal healthy kiddo but she should improve to the point of being able to live a relatively uninhibited lifestyle, or that's the hope anyway. She will still need multiple open heart surgeries as she grows to replace her conduit and pulmonary valve. Since it is artificial tissue it won't grow with her. She'll also need a couple heart cath's a year to stretch her pulmonary arteries to optimal size. They likely will have trouble growing too due to all the surgical interventions they have needed. 

She is still pouring out her chest tube, some days it's still close to a liter a day. She can't really start to make significant improvement until that slows. Her kidneys likely can't recover fully until that slows. That may be a month or longer. She's been dealing with this for 6 weeks now. Everyday they have to replace all the intravascular contents she loses- protiens, electrolytes, blood products, ect. This complication is what is keeping her in such a fragile tenuous state. 

Despite all of this she is a little better than she was this time last week. I have decided we have to start considering the weekly outlook rather than the daily. She is doing her damnedest to get better and make it home to Appalachia. She has a lot to see and do. Hopefully by sometime late spring or early summer she'll get to see what outside these walls. For now we're taking it a day at a time and counting our blessings. Our wonder girl fights on and we couldn't be more proud or grateful. 

Love to you all. Thank you for each positive thought, prayer and every bit of support sent our way. We are forever grateful. 

New Perspectives

Over the weekend Avelyn had a few new complications arise. We learned she has a peri-renal bleed, which was likely caused by the heart cath wires puncturing her inferior vena cava a few weeks back. When her heparin got to supra therapeutic levels this bleed got worse and made itself evident. Right now it hasn't damaged the kidney further but it'll continue to be monitored. Her heparin was stopped for a couple days and her GI bleed cleared and this peri-renal bleed seems to have slowed if not completely stopped. 

She also was detoxing off diluadid all weekend and underwent medication changes to help her right ventricle rest while also favoring kidney perfusion. Last night the detox caught up with her and threw her back into delirium, although not as bad as a few weeks ago. She restless and jittery all night. She couldn't get to sleep until around 6am. She's resting well now after getting risperadol, which she was able to have because her feeds have been restarted as of yesterday.

So far her kidneys haven't improved much, if at all. Her urine output has picked up a minuscule amount but her labs are a little worse than this time last week. The nephrologists still seem to be encouraged that she is producing urine at all. So we continue to hope for full renal recovery, but it'll take time.

Her left lung collapsed over the weekend again. Today it's back up but still atelectic. She is getting respiratory treatments to further improve the function. She came off the Nitric Oxide again last night. So far her Sats are in the low 90's most of the time, on 60% O2 but this will get better as she acclimates. 

Her labs that monitor infection have been jumping even though she's on a ton of antibiotics and an anti-fungal. They cultured everything yesterday and so far there hasn't been any growth but it's still really early. Hopefully it's due to something very treatable or just stress of detoxing. It's all such a Rollercoaster of ups and downs. 

Her echocardiogram showed a bit of improvement on her left side. She is now showing normal functioning of that side. Last week it was depressed, so that's good news. Her right side isn't yet showing any significant signs of improvement, nor is her tricuspid valve regurgitation. However the cardiologists believe with time her right sided function should improve. She'll never have the pulmonary or cardiac function of a normal healthy kiddo but she should improve to the point of being able to live a relatively uninhibited lifestyle, or that's the hope anyway. She will still need multiple open heart surgeries as she grows to replace her conduit and pulmonary valve. Since it is artificial tissue it won't grow with her. She'll also need a couple heart cath's a year to stretch her pulmonary arteries to optimal size. They likely will have trouble growing too due to all the surgical interventions they have needed. 

She is still pouring out her chest tube, some days it's still close to a liter a day. She can't really start to make significant improvement until that slows. Her kidneys likely can't recover fully until that slows. That may be a month or longer. She's been dealing with this for 6 weeks now. Everyday they have to replace all the intravascular contents she loses- protiens, electrolytes, blood products, ect. This complication is what is keeping her in such a fragile tenuous state. 

Despite all of this she is a little better than she was this time last week. I have decided we have to start considering the weekly outlook rather than the daily. She is doing her damnedest to get better and make it home to Appalachia. She has a lot to see and do. Hopefully by sometime late spring or early summer she'll get to see what outside these walls. For now we're taking it a day at a time and counting our blessings. Our wonder girl fights on and we couldn't be more proud or grateful. 

Love to you all. Thank you for each positive thought, prayer and every bit of support sent our way. We are forever grateful. 

Searching for Progress

I guess I have put off updating long enough. I kept delaying thinking that things would turn around and I would have something positive to report. That isn't the case. Avelyn continues to struggle. 

She is still in renal failure. Dialysis has been stopped but could be restarted at anytime. Her urine was picking up over the last couple days, although still only about a tenth of what we would hope for her- if that. Now it's all but halted. Her chest tube output was also improving for two days but today's looks like it'll be higher than ever. She is also

showing signs of heart failure again. She got an echo yesterday that showed decreased function of both ventricles and increased tricuspid regurgitation. The reversal of these findings is dependent on her kidneys, lungs and chest tube outputs improving. She isn't maintaining her oxygen saturations well either. The other morning she dipped into the 50%'s and had to be be bagged up with 100% fiO2. For a while her saturations didn't budge, even with the 100% oxygen and increased pressure. Her left lung is down and consolidated. She isn't diureasing the fluid that has third spaced into her chest wall interstitial spaces. This makes it harder for her to ventilate and harder for her right sided heart  to pump. She was put back on nitric oxide and epinephrine after her desaturation episode and echo showing decrease cardiac function. These seem to be helping her ventilate better.

She now has an active GI bleed in her stomach and tracheal infection to top things off. The echo and ultrasounds also show that the clot in her subclavian vein has broken up but that she has a new one in her inferior vena cava attached to her PICC line. We were hoping she could come off the heparin but that doesn't look possible with this new clot. The PICU doc's don't seem too concerned with the bleeding but I think they are just trying to be reassuring. They say it could be worse, and yea I know that. But she is a super fragile cardiac baby with a host of problems already. She can't handle allowing this bleed to go unchecked. I requested a GI consult. We'll see where that goes. For now the nurses are pulling about 10 ml of blood off her stomach every 1-2 hours. No amount of reassurance could convince me that this is inconsequential in a baby her size. 

I am so incredibly disappointed about her chest tube output shooting up today. We have been hoping and praying for a turn around and just aren't seeing that. If she wasn't pouring out of her chest tubes she would have the intravascular fluid volumes needed to produce urine and the protiens needed for healing properly. Right now she can't even go to the cath lab to balloon up her tiny left pulmonary artery (which would likely help decrease the chest tube output) because of her kidney failure. The dye needed to preform the procedure is quite hard on the kidneys. She wouldn't be able to handle it. So we wait and we watch our baby decline, or that's what it seems like is happening. Sure, she is showing improvement in some areas but not enough to quantify. 

We are surrounded by truly sick children, ours included.

Avelyn's eyes are sunken and often dulled by pain and or the medications needed to lessen pain. I wouldn't doubt we also exhibit the same hollow eyes. I've seen the look on so many other parents around us. If only our children could be healthy as all babies deserve to be. If only we could hold them, soothe them and see them smile. Hell at this point I'd be happy for a wet diaper. Seeing my child struggle and get no where is so disheartening. I read posts by parents complaining of feeling helpless. You don't know helpless. You may think you do, but you don't. Hug your child. Rock your child. Look into their eyes and know they recognize you and are aware of your presence. Please do these things and feel grateful for each. The day I can do any of them will be truly glorious. It's the day I pray for every day and am so often reminded that I am not guaranteed. 

While speaking to another heart mom, whose baby was not doing well after her heart transplant, she described looking at her baby's car seat the other day. I knew exactly where she was going before she said it. I've done it to. I've stared longingly at that stupid seat and cried. Why? Because we may never get to use it. That's helplessness. Looking at your baby on ecmo and not being able to find a place to kiss that isn't covered with a dressing, tube, wire, incision or bruising is helplessness. Not recognizing your own child due to the edema is helplessness. Sitting day in and day out at your child's bedside reading out loud because you can't stand where your thoughts wonder is helplessness. Carrying around and crying into a pair of booties during 16-18 hour long open heart surgeries because that's all you could grab before she was wheeled out of the room is helplessness. Waiting for a phone call to know if your child is alive or not, that's helplessness. So please, for the sake of those who know what being helpless feels like--rejoice in good health where it exists-- to some it is but a dream we chase but may never catch. 

The photos below show Avelyn in delirium, extremely dehydrated and the last is her on ecmo. I never shared it before because it is so painful to look at. It still sickens me to think of her like that. Today marks a month ago that she was on ecmo. This is what my child has endured. This is what she has endured and continues to fight against. I am helpless to save her from her pain and struggle. Truly helpless.

Praying for Pee

The last couple days have been yet another lesson in patience. Avelyn is in renal failure. We went forward with her extensive surgeries in hopes we could avoid this but alas, here we are. The day of and first day after Avie's surgery she didn't produce any urine at all. Now she is producing a scant bit here and there. Her labs still look awful but aren't getting worse at the rate they were the first two days. We're hoping she has plataued and we'll start to see some improvement soon. 

For now she has been stated on peritoneal dialysis to help her get fluid off and to take some work off her sick kidneys. It's too soon to know if it's really helping of not yet but she is tolerating it alright. Her blood pressure is still a bit labile but not as much as it was. Her chest tube output is picking back up. This is another thing we were praying the surgery would resolve. Again We are hoping to see some improvement with time, but are beginning to get discouraged. She may have to have another procedure to assess the competence of her thoracic duct. We'll just have to wait and see how it all goes.

She has seemed more comfortable since getting haldol the other night. She hasn't needed any additional doses so far and her PRN need has really declined. Today, if she remains comfortable they'll start to wean some of her sedative medications. Hopefully that goes well. It usually doesn't for our sweet girl, but I am hoping her new anatomy and physiology makes all this easier on her.

Sean also managed to find her a weighted blanket (the  blanket with owls below) that seems to be bringing her some comfort. It's a sensory comfort measure. It simulates being held. She really settles out with it on.

It breaks my heart that we have to resort to a weighted blanket to give our baby comfort. I wish more than anything she could be held like she so obviously needs and loves. This is all so frustrating. I miss cradling my baby but I am glad she is finding comfort, no matter the source.

Much love to each of you. Please keep her in your thoughts and prayers. If you feel so inclined, please pray for pee pee.

Pain and Delirium

I haven't a clue how to start this post. I am grateful but still so worried. 

I guess I should first discuss the surgery- or two surgeries, because nothing with my child ever seems to be simple. Avelyn was in the operating room for about 12 hours. Stating it was a long hard day for her is an understatement. As Sean loves to point out, she's had harder days but that only makes me feel worse. Our baby has been through too much. I really hope this is the last surgery for a while, preferably a decade but we're hoping for at least 4-5 years. Anyway the surgeries went well. It took the surgeon so long because he had to operate on two completely different areas. First he preformed the coarctation of the aorta repair via a thoracotomy, or incision in her back/side between her 4th and 5th ribs. Then he opened her chest back up and re-repaired her tricuspid valve and right pulmonary artery. 

The surgeries seem to have been successful. Her right sided pressures have already come down a bit and her tricuspid isn't leaking as much. She still has a lot of swelling and stiffness in the cardiac muscle but hopefully that will improve with time.

The recovery/post-op period has been really difficult for our sweet little baby. She has been intubated so long that she is extremely tolerant to opiate and benzos, plus she's experiencing delirium again. You must also consider how much her tiny body was put through yesterday. She had three major surgeries in one day. Surgeries that usually take fully healthy adults weeks to recover from. She had all of these within hours. She has two major incisions, and thoracotomies are known to be especially painful. Her intercostal muscle was sliced and her ribs separated enough to allow an operation to occur. Then her chest was re-split and spread wide. Her tolerance to pain medications makes everything so much more complex.

The providers have been chasing her blood pressures up and down. Treating her pain drops her blood pressure so she gets fluid which has plumped her up. She'll have to pee all that fluid off but right now her blood pressures are still too low due to her ever increasing need for pain medication and sedation. 

Her kidneys took a major hit during the surgeries, that were done partially to save their function. The decending aorta had to be clamped for 20 minutes to repair her aorta. That's 20 minutes her kidneys and gut went unpurfused. 20 minutes of more kidney damage. Today she is barely peeing at all. Diuretics can't be started yet because her blood pressure is still too low. We'll just have to wait and see how her kidneys do over the next couple days.

Good news is her chest tube output has decreased. Hopefully this is a signal of the chylous effusion clearing. Her effusion couldn't be attributed to any one thing, hence the extensive operation yesterday. We'll have to wait and see how she continues to do in that department too. It's possible she won't have to stay on the skimmed breastmilk any longer if she tolerates her feeds better when they are restarted in a few days. That'll be nice.

Her oxygen saturations have been low since she came out of the OR. For most of the day she has been on 100%fiO2 in addition to nitric oxide to lower her pulmonary hypertension. Her surgeon expected these findings though. I don't think the high O2 levels have done anything for her, nor has the NO. The oxygen has been weaned to 80% in the last couple hours with no change on her saturations. I think she's just going to hang in the 80% saturation range for the next couple days until the internal swelling lessens. 

Her major issues right now are delirium, pain and sedation . She's been intubated and sedated so long that her tolerance is through the roof. Plus there's a threshold where no matter the dose it just doesn't do much anymore, the receptors are just saturated. Ketamine was added back today. Then she got chlorhydrate. Neither did much. She was so restless and her peak respiratory pressures have been high. As the day went on it became evident that she was delirious. She wasn't settling either. So she was started in haldol. Yes, my infant is on haldol. I hate it. I know I have said that about a lot of things, but each has been warranted. 

If you don't know what haldol is, it's a first generation antipsychotic with horrifying potential side effects. Granted most have to be on it for a while before these become evident. Avelyn shouldn't need it very long but still. It hasn't been researched much at all in children-let alone infants and now it's being injected into mine. Believe it or not, I am grateful. My baby is now resting. She isn't in pain, she isn't kicking and waving her arms purposelessly. She's resting,  and seemingly comfortable. 

It's strange to hate something but be grateful for its existence. I have felt this contradiction so often in the last few months. I will be glad when there's less hate in my life. I can't wait for her to rest without the need of antipsychotics. I can't wait for her to not need pain meds or diuretics. There are many things we are not so patiently waiting for. But for today we feel blessed for peaceful rest, even though it's somewhat fleeting. There's still the pain and struggle of recovery but we hope it's the time she'll be put through this for a while. 

Much love to you all. Thank you for your thoughts, prayers and support during her surgeries. We hope to see major progress in the coming days.

Surgery and Selfishness

We learned this afternoon that Avelyn's surgery was bumped up to tomorrow morning. Then we learned, just now, that it will be more than we were initially thinking. We've known about the possibility of her valve being damaged to the point of needing surgical repair since before the heart cath. Yet we have been hoping for the best but best case scenarios don't usually find their way to Avelyn. So once again she will endure another full on open heart surgery that requires the reopening of her chest and a bypass pump run. Her surgeon also feels it's best to surgically reoptimize her right pulmonary artery. This should relieve the right sided pressures that are now immenently threatening to damage her kidneys and liver pernamently.

I have known this would be a possibilty since we received the heart cath results. I have been fighting with myself whether or not we should allow more surgical interventions to be made to her pulmonary arteries when cardiac caths are an option, albeit much slower for the same results. One ballooning would likely not be able to get the results that surgical optimization could get. Meaning our girl would likely be living months and months without the optimized right sided blood flow, in addition to not good left sided flow. Her left sided PAs will not be touched tomorrow. These are too fragile to be messed with at all. They will need to grow and to be slowly ballooned up over time, likely every six months for a few years. 

I wish this were more black and white but there are risks to every option. If we wait and choose to only intervene on her right PA with cardiac cath, she may lose function of her kidneys and/or liver. What kind of life would she have with a fragile heart and multiple other dysfunctional vital organs? This is what we must take into consideration. 

Since we learned of Avelyn's diagnosis we have had the painful discussions of quality of life vs. quantity of life. For us, quality of life is much more important than the number of days, even though our selfish hearts often lose sight of that. As things currently are, Avelyn's prognosis is still good. She can still live a long relatively healthy life, if we make the right decisions at the right time and always ensure we take steps to optimize her growth and development. Granted she will still need multiple subsequent open heart surgeries to replace her pulmonary valve/conduit as she grows, and she will need cardiac cath interventions to optimize her PAs. But she can recover. She has the potential to do and be whatever she chooses. That's what keeps us going. 

Leaving her in her current state jeopardizes her functional independence. I don't want her to be worrying whether she'll need a kidney transplant or dialysis when she should be worrying who will ask her to prom. I don't want her to not be able to grow because her liver can't handle metabolizing proteins. 

Our decisions carry serious weight and it's terrifying. Last time we sent her into the OR we nearly lost her. I am physically sick at the thought of sending her back. It would be so much easier not to. However she deserves us to be strong enough to make decisions in her best interests.

I hope and pray we're choosing what's best. 

Please keep our sweet baby in your thoughts and prayers. She deserves the best case scenario operation results tomorrow. She so truly does. She is loved dearly and unconditionally. 

Thank you for your support, we look forward writing about much happier times and decisions soon.

Daddy Daughter Days

First off I'll say Avelyn is still hanging in there, although she is starting to show new symptoms from her increased venous pressures. Her liver is now enlarging. Her liver function is still alright but the actual size of her liver is growing due to her increased veinous return pressures and congestion. Her chest tubes and PD drain outputs are about the same, maybe a little higher. Yesterday she put out just over a liter. Her kidney function isn't improving but it doesn't seem to be getting worse either. Right now her CoA surgery is scheduled for early Thursday morning. It may be bumped up a day or so though due to the PCICU being full and having to cancel scheduled surgeries for those not already on the unit. 

As bad as it sounds, I don't think this surgery can come soon enough. Her surgeon doesn't think it'll help her current situation a whole lot but the cardiologist (who does her cardiac caths) seems to think it will. I don't know. It has to help at least a little with high pressure in her left ventricle and back flow through her VSD- which is likely contributing some to right sided high pressures. The cardiologist doesn't want to do her cardiac cath until she is six weeks out from her unifocalization surgery. Right now she is only 3 weeks out. He believes she is too small for stents unless it's an emergency. If he puts them in now they'll just have to be surgically removed when she grows, so he'd rather ballon her vessels up. We'd prefer this too but she needs to be 6 weeks out before there isn't a high risk of rupturing her suture lines in those delicate Pulmonary vessels.

So we sit tight and we wait. It's so frustrating.

On another note I am still sick and haven't been able to stay with Avelyn since Thursday. In that time Sean has barely left her side. She and I are so lucky to have him. He's amazing.

Throughout my pregnancy he questioned whether he would be able to be the father he wanted Avelyn to have. I always knew he would be great, but he had serious fears. Seeing him with her is so wonderful. His love for her could never be questioned from the minute she was born. He escorted her to the NICU while I was still in the OR. He stayed with her until she was stabilized. He sent me photos and described her to me as best he could. Those hours are a blur to me because of the sedation and pain meds from my c-section but I remember being so relieved and so much less worried because her daddy was with her.

I know I get more credit than he does. It makes sense, I publish my thoughts and perspective and am generally more outspoken. After the last few days of having to stay away I just wanted to highlight our unsung hero a bit because he deserves it.

Sean is my constant and now little Avelyn's as well. He has been there for me for nearly a decade. He has cared for me through years of Lyme disease, which mostly affected me neurologically. He would often sit with me to ensure I ate before succumbing to fatigue. He would wake me during my 20+ hour slumbers  to make me drink water, eat, or take my antibiotics. Even when I was awake, he would bring me my antibiotics-I didn't know the day of the week, let alone the time of day. He would patiently answer the same questions over and over, because he knew it would upset me to know I didn't remember already asking. He has to live with the memories of me at my worst. Memories I don't have. He lives with the worries of my relasping and yet he is still here and still fighting for both of us. 

He is an amazing man. His patience is inspiring and his love for Avelyn and I unquestionable. I don't know what we ever did to deserve him, and I don't know where we would be without him. He is the best partner and husband I could ever imagine. While I have seen some pretty amazing fathers, I can't say I've seen any more wonderful or kind. His love for Avelyn is so fierce yet so gentle. Despite his likely doubts, I know he will never let her down. 

We love you Sean Aldin :)

Readers

I'll begin with saying that Avelyn is still maintaining. Her kidney function hasn't improved but her lungs have a little bit. She is struggling with being weaned off opiates and benzos. For now they have halted the weans because she has been so uncomfortable and her temperature has been too elevated. She needed a cooling blanket to bring her core temperature down today. We don't want withdrawal symptoms to keep her out of the OR next week. They might be able to wean a bit more tomorrow but only if she seems comfortable, and honestly we'll probably request that they leave her be.

Sean has been staying with her all the time since I have been sick, not that he wasn't there most of the time before. He just rarely leaves now since I can't be there. I call a lot to check on her during the night and he gets up to go visit her from the sleep rooms. He's such a good dad and I am so thankful for him. Still though, I can't wait to be back with her. 

On another note, this blog reached 30k readers yesterday. That's mind boggling to me. I initially started it to update close friends and family- I never thought of reaching more than that. Why would I? I never conceived so much support and concern for our baby. We have been so blessed and humbled. People we love and complete strangers alike have done and said wonderful things. I can't begin to thank you all enough. Without your love and support I don't know where we would be. I don't know if I would have been able to keep up with this blog either. Sometimes it's a welcomed distraction, while in the thick of things it can be laborious. However after a recent conversation with Sean I am so glad I have a real time record of our experience.

He very causally asked me the other day how I was going to save the posts to ensure Avelyn would one day have access to them. I was dumbfounded. I have seriously never considered that our baby girl would one day read these posts. It seems so logical that she would. I mean this blog would be more informative than anything but her actual medical records. The thought of her reading all that I have written hit me like a ton of bricks though. What will she think? Will she comprehend how much she has always been loved and how sorry we are for all she's been put through. I hope so.

I hope she reads these posts with an open yet reflective mindset. Maybe she'll understand us a bit better after reading  it. I hope she finds strength within herself. I hope she reads how she triumphed through these circumstances and feels motivated to live fully and freely.

I was talking to another heart mom tonight about how we owe it to our kids to not limit them and to live completely in the moment. If our babies deserve anything after trekking through hell its to be allowed to live fully. I look forward to the Avelyn's robust rowdy days when I will worry about her little heart but never let on. She will be allowed to live and grow as she will. She deserves to. She will play, she will climb, and we will live fully. We will not sit at home in fear. Avelyn may have CHD but CHD will not have Avelyn. She will not be defined by this disease and we will not allow her to live in fear. She will see and experience the world. 

I hope she feels we have kept this promise . I hope she realizes how strong and magnificent she is. 

Until then I guess I'll just keep reminding her everyday. Not that I mind to at all ;) 

I love and adore you more than anything my WONDEROUS Avelyn Marie. I always will.

Back to the Waiting Game

Over the last few days Avelyn has maintained and that's been a feat in and of itself. She will not be able to improve until she can have her coarctation of the aorta repair and an interventional cardiac cath. The CoA repair should be next week, but we have yet to secure a date. The cath will likely be the week after or the next.

She has held her own with the weaning of her opiates and benzodiazepines, these are the medications meant to sedate her. Yesterday she seemed to have rested comfortably and appeared more alert when she was awake. The risperadol is really keeping her delirium in check. Her eyes still wander a bit but she doesn't have the dead pan gaze she did before and her hyperstartle reflex and gagging seem to have improved. It's nice to see that spunky spirit in behind those pretty blues again. 

They have also been weaning her ventilator settings. She has been tolerating it well considering how long she has been ventilated and all she is currently dealing with. Yesterday her left lung was completely collapsed but today it looked better. Not great but better. We're hoping for even more improvement tomorrow. I am not sure if they plan to extubate before her next surgery. I guess it just depends on the date and how she does over the weekend.  

Yesterday I fell quickly with flu-like symptoms. I didn't feel comfortable staying in the hospital and risking spreading it to Avelyn or other kiddo's. It's been so hard to be away, probably harder than anything I have done so far for my sweet girl. I am feeling better today and haven't had a fever since the wee hours of the morning. I could not handle the thought or action of going a day with laying eyes on her. I know I shouldn't and I have been fighting myself all day. Finally I gave in and masked up. I gazed upon her from outside her room. She truly is the wonderful. She is so strong. So courageous. So beautiful. Oh how I miss her.

In the last month and a half I have only held her once and Sean hasn't held her at all. It's not only hard on us, it's hard on her. She loved being snuggled, rocked, and bounced. At a month old she would call out just to be rocked. I miss that so much. I miss her. I can't wait to have her back. To be able to scoop up and comfort my baby is my life dream. I can't describe the agony of seeing her cry and not being able to soothe her without drugs. I know I should be grateful with our situation. She's still here and she can and will recover. That's what matters. That's all that truly matters, but sometimes it's easy to lose sight of that. Babies aren't meant to go months without being in their parents arms. Babies aren't meant to not remember the feeling of a full belly. Babies aren't meant to experience pain or withdrawals. 

Ugh... 

It's hard, so very hard. We can't wait to have her home and begin teaching her what being a baby is all about. It'll come, I know that. We just need to have more patience and to keep fighting. We'll get there. And as an FYI, you'll likely continue to read sappy posts until we do. 

This momma is tired of seeing her baby in pain and being completely helpless.

Unexpected Findings

So, where to begin...

I guess to start out Avelyn's pulmonary arteries look alright, not great but alright. Her left PA is still quite small, but we already knew that. She'll need a cath in about a month to expand the artery branches on the left to lessen the pressure her right ventricle has to pump against. Actually, she'll likely need this procedure every six months for a few years and intermittently as she grows. 

The major finding is, Avie now has what's called coarctation of the aorta. Her aorta is narrowed due to scar tissue where her surgeon took the MAPCAs off to be grafted onto her left PA. The narrowing causes resistance that her left ventricle has to pump against to empty. The added pressure in the left ventricle back flows through her ventricular septal defect into her right ventricle. This added pressure, coupled with that from the small PAs, increases the pressure of her veinous system and may have again ruined her newly repaired tricuspid valve. It is hoped that fixing the coarctation and allowing her heart more healing time will decrease vascular resistance. With decreased vascular resistance the chylous effusion should clear. It's a possibility that we won't know if the tricuspid valve is alright or not until after her right sided pressure is under control. They likely will want to fix the coarctation soon and then send her in for another cath to expand her left PA in a month when the suture lines are stronger. We'll probably be here until both of these procedures are done. The coarctation repair will likely be within the next week. It's not technically an open heart procedure but nearly. They'll preform what's called a thoracotomy, meaning instead of going through her sternum, the incision will be on her side/back. The surgeon will then put a patch on the narrow portion of the aorta that will not prohibit normal growth and development. 

We are not pleased to hear she will need another surgery so soon or so many heart cathertizations. However today's findings could have been so much worse. We were expecting collapsed pulmonary arteries, which would have been devastating for our girl. While CoA is not a good diagnosis to receive, it is repairable and Avelyn should still be able to recover. 

We don't know when the CoA repair surgery will be. Knowing her surgeon, he'll throw her into the first open slot he has. It could be anytime in the next week, we may not know until the day of. 

We'll keep you all posted. Thank you for the support and prayers. Your outpouring of love has given us strength that allows us to continue to fighting alongside our sweet baby, even through some very dark hours. We'll never be able to repay you all for such a gift. Much love to each of you.

Not Again.

Throughout the day yesterday Avelyn continued to put out large volumes from her chest tubes, much of it chylous. Her surgeon became concerned and ordered an echocardiogram. Sean and I received the potentially devastating news later last night. Her pulmonary arteries could not be seen at all, this coupled with right atrial and ventricular pressures at three times normal. Her tricuspid valve is also leaking terribly again. Her surgeon believes her right pulmonary artery has narrowed to near occlusion and likely has some left sided narrowing too. She goes for a heart cathertization in just a matter of hours. They may be able to do some interventions in the cath lab but it's not likely. She is not far enough out from surgery to have much done via heart cath. Interventions could pop her delicate suture lines. 

So what does all this mean? 

While we don't know for sure yet, it likely means Avelyn will need another open heart surgery to re-correct the tricuspid valve that the increased pressures have likely torn, and to re-patch and open her pulmonary arteries. I don't know how going back in rehashing everything and allowing more scar tissue to form on top of that she already has will affect her, because it has to. I don't know how we'll be able to trust sending her under the scalpel again. We almost didn't get her back last time. 

I have spent so much time dreaming of her future. I have so many wishes for her. Simple things like nursing her, watching her grow, seeing her experience all her "firsts". My wishes for her aren't huge, but they are everything to me. She is everything to Sean and I. We want so eagerly for our baby to survive and thrive. I have prayed so earnestly and continuously. I don't know why these things keep happening and threatening her survival. I wish I did. I don't know why she is in this state to begin with.

My logical mind tells me it's not my fault. 1 in 100 babies are born with CHD. Some with more serious defects than others, mine with MAPCAs. I did all I could to create a healthy prenatal environment for her. I know that. I know that it's not any one person's fault that she got endocarditis. I know that we have gotten more time with her than many parents of children born with similar defects do. 

Yet still, I am angry. I find myself searching for a source of blame. I know it's not productive but I can't help it. We question everyday whether she'll see the outside of this hospital. She may not. Thinking about it makes me want to bundle her up and take her away. Take her somewhere I can protect her. But that's the thing, Sean and I can't protect her. There's nothing we can do for her. We have made decisions that have put her through hell. If she doesn't survive, I don't know how I'll live with myself. We have tortured our baby in hopes it will provide her with life. We have put her through painful procedure after painful procedure. I sincerely believe she will make it. That I will one day be reading her bedtime stories while smelling her freshly washed hair. I believe that someday I'll tuck her into bed and watch her sweet face while she sleeps and dreams comfortably. 

I have to believe that. But why? Is it for my own selfish reasons or for her benefit? I really don't know. I don't know much anymore. 

This morning Sean looked up from her bedside with more love in his eyes than I have ever seen and said, "She looked at me, like really looked at me. That's the first time in weeks." Her antipsychotic medication seems to be bringing her back to us through her withdrawal induced delirium. 

We are cherishing these moments. I have found myself trying to memorize her warmth, the length of her eye lashes, the roundness of her nose, the rich blue of her eyes. She's so beautiful. More beautiful than I could have ever imagined. She is so loved. She is such a wonder. 

Please pray we will be bless d enough to keep her.

Stagnancy

Little Avelyn continues to endure. She isn't making much improvement at this point. Her renal function labs came down slightly over the weekend and now seem to have plateaued. She isn't making much urine so I am not surprised. She continues to dump fluid out of her right chest tube and her Peritoneal drain. The fluid coming out of her chest tube and PD is chylous. This means it contains chyle or fats which have not been absorbed properly from her small intestine. The fat within her breast milk is emulsified into her lymphatic system and then leeching out. Her capillary beds also continue to leak high amounts of fluid. 

It's discouraging but also dangerous for Avelyn. She is chronically intravascularly dehydrated and at higher risk for infection. We were informed this sometimes happens with the type and severity of her repairs and her subclavian clot only complicates the issue. This is not something that is likely to resolve quickly. For some kids it takes weeks and/or surgical intervention. I hope Avelyn isn't one those, but knowing her luck it's likely. 

She is also being weaned from her sedative and pain medications. Ketamine was completely discontinued yesterday after a hard weaning period. She has spent the last 24 hours in a serious state of delirium. She hasn't rested much, despite liberal use of PRNs. She is just too tolerant. Psychiatry has been called in. She'll be placed in low dose antipsychotics to help get her through this tough period. She also has been started on Methadone and Ativan. These will inevitably help with her detox from versed, diluadid, and precedex but they won't keep her from having withdrawals or other detoxing symptoms. It'll just make the symptoms less intense. She'll still likely be uncomfortable and restless though. Right now she is so sad. Her left eye is wandering, making her look like she has strabismus. She can't track faces or objects in front of her face. She jumps in the little bit of sleep she does have and wakes herself up. Her eyes grow more blood shot by the hour and are encapsulated with dark circles. It's sad. I hate it and there's nothing I can do.

We play her music or white noise and take turns stroking her head and talking to her. That's literally all we can do. We're helpless, absolutely helpless. It's not something I would wish upon anyone. We have to keep reminding ourselves that compared to where she was a couple weeks ago she is doing so much better. But then again her rate of improvement seems to have stagnated. 

Her vent settings will be turned down bit by bit today. They hope to extubate in the coming weeks but right now it's not on the radar. Her left lung is still partially collapsed. The area of concern is the upper lobe. An ultrasound was done yesterday that showed an enochulated pocket of fluid in too risky of an area to drain. Right now it's just being watched. Hopefully it will start to diffuse into one of her many drains. I don't know if I could bear yet another chest tube sticking out of my baby. Let alone one centimeters from her so very fragile heart and vasculature. 

The good news is she is still here and putting up one hell of a fight. She's our wonder girl. Much love to you all.