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.