One Day at a Time

Today Ava got a PICC line for better access. She is still on the bicarb and will now be fed. She will get a dose of bicarb to help get her lactate down. She had an Echocardiagram to see if there were any changes in her heart function. There were none. Tomorrow she will have an MRI to see if there are any metabolic changes in her brain. She will also have a nephrology consult to see how her kidneys are doing.

Through all this, Ava was sleeping peacefully. She was sedated for her PICC line placement so she was comfortable through out the rest of the time we were with her at the hospital. And of course she was sucking on her tube.

The geneticist at CHOP does not think that we would have any better outcome if Ava were transferred there. Which is a relief, since we didn't want to go that far from home. So we will stay at AI. Glenda the Good Doctor will go off service tomorrow, but she offered to remain our point of contact if we needed her to be. The next doctor coming on has been very compassionate with us. Hopefully that will go well. We seem to have a few old hurts from Eric with him. This is when I need to exercise some forgiveness.

We so appreciate how she is investigating every avenue she can for Ava, knowing it's what we don't want it to be. She is also trying to get as much information for us so we can make an informed decision. We of course thought we were doing all that before with all the testing and it all came up normal or it's not back yet. That's all pretty frustrating.

The most exciting news of the day is that because I threw a temper tantrum, and cried, I was able to hold Ava for a while. All I can comment on that is that it was and still is extremely emotional for me. I feel a little drained.

We don't know what the future holds for our beautiful baby girl. Nor do we know how much of a future she has. We continue to let God guide us, even when we feel like we are being led down a road we so don't want to go down.

Prayer

Ava was tucked in her hospital bed exuding peace and grace when we left tonight. That's not really what we walked into when we got home.

While Emily and Alexa were having such a hard time at the hospital and leaving it today, Elaina was having her hard time tonight. I can't imagine how her little 2 1/2 year old little mind is processing all of this. She is just catching up to the fact that she has a brother that is in Heaven and is so desperately trying to see about her sister in a hospital bed. She is actually getting it as best as she can, but it is just so heart breaking.

Emily and Alexa have a lot of questions about the hospital and the changes in the color of the tape they have on Ava's face (to hold the tubes). I have to be honest, it breaks my heart every time they tell me that they don't want Ava to be in the hospital. I just tell them that I don't want her to be there either.

We've been home in time for Bible and Devotional with the girls. Allan and I noticed that all of the Bible stories and Devotional pages are all about God's power and the miracles He performs. today we heard two stories from Mom's whose children were nearly dead and survived. We know that God can do it. We also know that what Ava is dealing with is not just some nasty life threatening infection. Until He tells us otherwise, we will continue to pray for a miracle and healing for our little girl. We will also pray for protection and peace for our bigger bugs.

Football and Cheesburgers

Things are a little more settled at the moment. Now, I use the word "settled", but bear in mind my daughter who lies 4 feet away is still critical. She visibly feels better, however. She seems to be responding to the antibiotics; the bicarb is working on the lactate; she is no longer acidotic; the ventilator is fully supporting her at this point, giving her a much needed break. And, in the midst of everything going on and all the extra "intervention" of the day, her eyes have been open for longer than we've seen in days.

Us? We continue to sit, to wait, to hope... we eat McDonald's (quarter pounders are very comforting). We watch the Eagles. We listen to Christmas music. We make bracelets (okay, she makes bracelets). Our nurse just commented on how "well" we are doing. I can tell you that there is a peace in this room that is not of this world. And that is a testament to many of you that are reading this- your prayers are carrying us, and our savior Jesus Christ intercedes on our behalf-and we are calm. And Ava rests.

Where is all this going? We still don't know. This is still frustrating. But we continue to walk by faith, because that is the path to which we are called, for now. We have great faith, but we are not naive. We know there are tough decisions on the horizon. Tomorrow is Monday, and the holiday weekend will be officially over. We hope to at least get answers on the status of results, if not results themselves. We pray for this, in order to have some assistance in making tough decisions. Today is Sunday and, for now, we rest. Dear Lord, let us emerge from this on wings of eagles that we may soar...

Not so Great

Ava is pretty sick right now. More IV's, more blood draws more support. Her lactate is up. So we have heavy hearts. But for some unexplainable reason, we are not ready to throw in the towel. Boy do we understand what is going on. My body is tired of the fight. But that little baby laying in that crib is fighting. She smiles, she cries, she opens her eyes. She's trying. It makes it so hard to know what to do.

There really is no human way that Ava can overcome this. There just isn't. We both have not gotten the "word" from God that it is time for her to go Home. So we sit here and wait, still.

We have no results, we have no answers.

We had the girls come in today. They needed to see their sister and she needed to see them. I can not begin to tell you the depth of my agony watching those girls love Ava, give her a snugly toy, and tell me that they want to bring her home. I get so upset that they have to deal with this in their young lives. It's when I cry out the most, "Why would you do this to them??" It's OK to question. It's OK to be angry. God can take it and He wants it from us. I will never ever know the answer as to why we are called to this. Why our daughters have to endure such heart ache and sorrow. What I would do to wipe that look from their eyes. I know this is part of God's plan for them as well and they are made to take it. But my heart is ripped out thinking about it. It's too raw, deep and yet right there, under the surface.

I keep hearing stories of children who almost died and who were healed. It happens. I wish it were for us.

Saturday

Ava is now settled in for the day. When we got here she was not herself at all. She was unsettled. Her heart rate was the highest we've seen it. She was all sweaty. She was uncomfortable and would cry. It seems her NG tube was not in the correct place. So once that was discovered and replaced she seemed to do some better. She was still uncomfortable though. oh, and she lost a little weight too. (Ever so slight but it's still not great. Understandable with being sick again).

Well, as it turns out, Ava definitely has pneumonia. Her temp is up and is now on antibiotics. She is actually a little more comfortable. But she looks like she doesn't feel good. The Attending thinks this is something she can help Ava get through. Tomorrow we will know the infection and see from there. For now she is on a 7 day antibiotic course.

I was talking with the doctor today about how I just don't get it. Eric was spilling over himself. He had no energy and he just gained weight. Ava, who seemingly has no energy, is burning calories faster than they are put in. It's the opposite. It makes no sense. The Attending and I are thinking a conversation with CHOP is in order on Monday. Our Attending also thinks that Ava may need to be transferred there. (Of course it did take 11 days to get a bed last time, so who knows how that will play out.) We don't know what we want to do or what is best for Ava. Or if we are going down a rabbit hole. For now, we are going to have to see what the rest of the weekend holds and how well so responds to her antibiotics.

I just wish there was something that I could put in her formula to help her get bigger and stronger. It's already rocket fuel formula so I guess that won't work.

Through all of this, we feel peaceful today. It's nice. We'll go with that for now.

Have I mentioned what a little fighter Miss Ava is?

Processing

Tonight we did get the chance to decorate our Christmas Tree. Every year I have a good cry when I put Eric's ornament on the tree. It's my moment to just be with my memories of him or maybe just to remember how much I just miss him I suppose. That is not what happened this year. Eric's ornament box came out and Alexa called out, "Who wants to hang Eric's Ornaments?" Seconds later Emily and Elaina were in front of her. They took his special ones out and appreciated each one. Alexa said, "We are hanging Eric's ornaments for him because our brother is in heaven with Jesus. So we will do it for him." How could I cry? It was just so special to see each one of those girls treasure the brother they never had the chance to meet.

I realized yesterday that Ava didn't have any ornaments yet. So Ava's Aunts and Uncles and Grandparents are getting her some. I just really wanted to have her ornaments on the tree while she is living. I don't know her outcome for sure, but just in case.

Then I realized that how can it be so profoundly sad to decorate the tree each year with two holes in my heart? It will be the one time of year that I will have something special for all 5 of my children in one place. After seeing how the girls handled Eric's ornaments I wonder if they can handle this way better than Allan and I can.

How can we be so filled with despair that we won't see the joy on their little faces and the lights of the tree twinkle in their eyes? There is always hope. There is always life. Jesus gives us the reason to have hope and to live life.

I'm not saying that this is an easy transition for me. Not at all. Our journey, individually and as a family is just beginning. God is revealing Himself to us in so many surprising ways. He really is in all the details. All things that I seem to worry about are just taken care of. Our needs are being provided for.

At Ava's bedside tonight I said that I will never know why God created Ava for this. I know it's just, I know it's sovereign, but I don't think it's kind. As I was saying it I was noticing the Grace with which Ava handles her medical struggles. I wonder if she hurts, if she feels trapped or frustrated. She does cry, but she smiles too. She opens her eyes more when we touch her or kiss her. She is not miserable. She looks peaceful and content for the majority of the time. So in the midst of me saying how unkind Ava's situation is, there is our daughter. She was created to carry this mighty burden and how we hate that she was. But there she is doing it, bearing it. Way better than we ever could. In what looks to be unkind, Ava is being imparted with God's grace so that she and press on as well. I never really thought that through until I started to write this. But there it is.

To come home weary from the day, throw on some Christmas music and try to get jolly is a great way to unwind. To see the joy in those eyes, even if they are still parts of pale not feeling well faces, is a relief. Elaina just played with all the ornaments. They were on some kind of Princess convention. Alexa held up a star and told us it was the star Jesus died on (we just let that one slide). Then she found an angel and told us it was like the one He sent. Emily was lost in arranging the Christmas Village and astounding herself with all the memories of last Christmas that are flooding back. She is so excited to remember traditions, and where decorations go.

As we tucked the biggest bugs in they had more questions about Ava and "if she dies". Elaina prayed for "Ava in the hostival and Eric in heaven". Concepts that she's working on since our discussion last night. I feel tugs at my heart as I look at the stockings and wish I knew if I needed to make another one (which I can assure you would never have been done if Ava were healthy). I couldn't stop myself from wishing that next year there would four little ladybugs individually running under the mistletoe with Daddy for a kiss.

Tonight I am sad for what we are going through. But I am full of hope for what we can overcome. Whether or not Ava lives or dies we will have to muddle through together. We will have to have our family traditions. Some will be silly, some mixed with sad. But they are ours and they make us, us. If we keep ourselves fixed on the path that God leads us to, we will make it to our destination. The road is never easy. That is never promised to us. How I wish it were. This is way harder than loosing Eric. But God does promise He will be there. We have to let Him. We need to listen to Him, even when we are angry. He is there.

Tradition

We decided to take a little break yesterday. We spent most of the day at home and only a few hours at the hospital. It was an emotional day for all of our family. I was at home with the bigger bugs and my heart was aching. We came to see Ava and my heart was at home.


While we visited her, we observed her getting weighed. It was quite a production. I have to say that it was very telling for me, personally. She is so frail, so weak, so limp. My soul ached as I left last night. Reality came crashing down around me. I have not been able to hold our daughter for 16 days. I didn't know. I could only see her in the bed.


I'm fearful that I know now. That I don't need labs, test results and whatever else they throw Ava's way. I want to not fell this way. I want it to be wrong. I know that Ava can live if it's God's plan for her. He can heal her. I don't know if He will. I don't know if it's His plan.


I put Elaina to bed last night. She was having a hard time. I was worried that she was getting an ear infection as well. We chatted and I discovered that she is confused about what is happening in her world right now. She was able to tell me what she heard. That Ava is sick "lots" and that she may have to die, but we don't know yet. She then looked at me and said, "Mommy, I don't want Ava to die". I told her that I didn't either. She then asked me to sing "Little Rabbit FuFu" and some crazy Elaina version of "Itsy Bitsy Spider" that had a pig sliding down with it's feet out. Sure, I could do that, why not?


After the girls we all tucked in we had our traditional after Thanksgiving snack at 10pm (Turkey sandwiches). We then decided to enter the mayhem of Black Friday Shopping. We decided that no matter what happens with Ava we have to try and keep our family traditions the same. We of course never went out at midnight, or together, but we wanted a train for the base of the tree. We got to Toys R Us and the line was HOURS long. For the life of us we could not figure out what the hub-bub was about. We packed it in and went home. So much for our sense of adventure. I was pretty sure that Toys R Us wouldn't have enough toys to support the line.

My Dad and I got up and did some early AM shopping. I don't think either of us felt like it. But it's tradition. I found a train for the base of the tree. The smiles made it all worth while. We got ready and back to the hospital.

I didn't know what we were going to see when we got here as is true every day. Today Ava was not acting right. She was uncomfortable. She her sats weren't great. Not too bad, but not Ava. She was sweaty. She didn't do well with her C-PAP trial. She didn't gain much weight. Some, but not enough. She just didn't look good.

So it was time for x-rays, labs, and to go on full support of the vent. Ava's lactate is up, higher than it has been. Most likely due to working hard, burning all the extra calories she's given and now burning more muscle.

This points to what what we have been concerned about. Ava is most likely never going to be able to come off the vent. We are going to be faced with some decisions that we have been dreading. We feel inadequate to make. But we see how well is is now doing on the vent. She is more responsive, more comfortable. This is telling. Telling what we are not sure, but it is speaking to us.

We are headed out earlier tonight so that we can Fa La La La and decorate the Christmas Tree. (We don't feel like fa la la la laing, but the other bugs do and it's tradition.) We do need a reminder as to why we can live this life we are living, why we can have hope and why we can put one foot in front of the other. Even through our anger, our grief, our exhaustion. We can carry on not because of that baby in the manger. But because of what He did on the cross. We will never know why. We will never be able to understand why our family is called to this path. We are. We walk it. We accept it. We don't like it. We just will have to do it.

Sorting things out

Ava had another good day today by our assessment. She seemed to do better with her CPAP trials. She also has a lower setting when they out her back on the vent as well. Her tone is a little better: moving arms and legs. She moved her arm up near her mouth and now we are worried about her inadvertently pulling on her tubes. A little spunk never hurts I suppose.

I am still worried about her eyes and her ability to see well. I don't understand it. I wonder if it has to do with a medicine that she is on. I hope we can take her off of it soon. Her neurologist seems to go back and forth about it.

The new Attending is willing to entertain my hypotheses about Ava. She is trying to think about reasons why Ava would have bad lungs. She wants to see if someone can help her prove my late-onset GBS theory as well. Neither of us doesn't think that we can do that since she's been treated with antibiotics already. All the while we have to wonder about mitochondrial disease.

Ava's neurologist called on his way out of town. He is not really optimistic and wants to see what happens over the next couple days while he's out of town. When he returns he said we will talk about what we have to do. This is frustrating. We don't know what we have to do and we certainly don't want to be pressured into making any choices. We need clear direction before we do anything.

What is clear direction? I guess it could come in a number of ways. Eric's DNA could show a mutation on a gene and then we know Ava doesn't have what he had. Ava could get significantly better, her lactate could drop again. Ava could get significantly worse. Ava's CSF (cerebral spinal fluid) results from her LP (lumbar puncture) last week could come back. Ava's muscle biopsy results could come back. Or we could miraculously find out why our baby has bad lungs. (Like maybe it could be a prematurity thing). We continue to pray that it's the answer we all hope for . We ask God to reveal to us what it is that He wants us to pursue for Ava.

Tomorrow is Thanksgiving Day. We plan on staying home for the majority of the day to be with our still under the weather girls, my parents and my brother and his family. As much as we have weighing down our hearts we have lots to be thankful for. Because of all of you who read our blog, pray for us, donate to Ava's fund, support us in the myriad of ways that we can't even enumerate we can walk this walk. God has called all of you to support our family so we can do what we need to do for our children.

When Eric was sick and dying I felt the hands of God in my soul molding me, shaping me, helping me breathe. I don't feel that through Ava's illness. I've been frustrated. I KNOW that He is there. But I haven't been able to understand the difference. I couldn't feel Him. I can see the work He is doing through Ava, in the community, and for us. But I couldn't feel Him. Today I realized that I was looking in the wrong place. God is not supporting my internally, that was His gift to me during Eric's life. It's special because it is part of Eric's story. Ava is different. her story is different. My relationship with God is different. Today I realized that He is here and I feel Him. God is holding my together from the outside. I am not crumbling. I am standing. It is because of Him and His love for us.

Through my fits of anger, my temper tantrums of not wanting to do "this" again, my questions of where God is and why is this His plan for TWO of our kids He is there. He has never left our sides. He is using all of you to encourage us to carry on.

We have oh so much to be thankful for.

Confounding Variables

Someone used the term "confounding variables" the other day to describe Ava's medical situation. She might have an underlying mitochondrial disease, but clinically she looks like failure to thrive. She is having a hard time coming off the vent, but is this because of progression of disease, or is there a "confounding variable" that is causing this, such as weakness due to under nourishment? Her lactate has been elevated- again, is this disease progression or the result of her muscles breaking down because they are starved for food?

We walked in today to a baby that looked a little better, seemed to be little stronger, and did well on her morning CPAP trial. We then found out that her lactate from her overnight blood draw was 11.6 (the highest we've seen). How does one process that information? The attending came in to talk to us a short time later and stated that she was suspicious of the way the draw was done and wanted to re-stick her. I thought to myself "have a good time with that, but it's still not going to be any lower than 8 or 9". Imagine my surprise at the actual number: 5.8!! This is one of the lowest numbers we have seen in a while (although still elevated above normal). The attending is also concerned about her weight, which is down a little from the last time she was weighed on Monday. Is this because all of her energy is going toward learning to breathe again, or because of the fluid she got rid of when they gave her lasix? Or could it be related to being weighed on a different scale? Questions like this are going to drive us out of our minds! In any case they are going to increase the calorie content of her feeds. not sure if they will continue the CPAP trials or give her a break. All of it is very confounding, to say the least.

On the home front, Alexa was up last night coughing at about 4:45. So into the bathroom with Daddy she went to turn on the steam. Emily seems to be doing a lot better, but complaining about her ear hurting. Our pediatrician, who has been calling daily to check on Ava, called a little bit ago- I told him about Emily's suspect ear infection and he is going to call in a prescription for us, knowing that we don't really have the time or capacity to take her in for an appointment right now. These are the little things that get us through right now.

So we continue to walk down a path with an uncertain destination, trying to make sense of every twist and turn. I trust that God will continue to give us enough light for our next step. But my humanity keeps taking over and I keep wondering why he won't just give us a 5000 watt search light. But if we knew where we were going would we continue to walk? To even think about it is terrifying...

That's all for now...
Allan

Ava is back on a one hour CPAP (breathing on her own) trial after taking the morning off. She showed signs of tiring on her last trial and her early morning chest x-ray showed more signs of some atelectasis. Why this keeps happening, we don't know. She also had a massage from her PT specialist which she seemed to enjoy and also had a good amount of awake time a little bit ago. She is sleeping peacefully as I type this and doing okay with her current CPAP trial.

Whether to be hopeful or not seems to be a matter of opinion at this point. Doctors have been dropping subtle and not so subtle hints about their skepticism concerning getting Ava off the ventilator. We already know this. Another doctor that we talked to yesterday evening (who will be on day shift service for a week starting tomorrow) is more hopeful. While she obviously has concerns, she feels that we should be able to extubate toward the end of her week. Of course no one really knows how this is going to go except for Ava and God, and so far neither one of them is telling us. And it's hard not to be discouraged. Amy has mentioned a couple times that it would be nice to have a fast forward button. I'm going to have to second that.

*********************************

So here's some up to the minute, breaking news ("This just is..." if you will): CHOP just called and the Mito-Meta Array was completed. Ava has no deletions or additions on any genes. So... there's that. This would seem to further support the theory that we are looking at a mutation on a gene that hasn't been discovered yet. Still waiting on 2 tests on Eric's DNA and the muscle biopsy for AVA.

That's all for now...
Allan

Roller Coaster

They finished up with Ava's PT and OT. She really seemed to enjoy it. Then came her one hour trial for the CPAP, during which I got in touch with our genetic counselor. Ava did great with her trial. When she was put on the vent for a 3 hour respite she continued to look comfortable and breath over it. She was also awake for an hour after the initial trial. During this time she moved more, sneezed, coughed better. Of course she sucked her pacifier. Her suck is getting louder and more coordinated. She had her eyes more open and was trying to focus. Her pupils are still dilated some and her irises are not all there. But the color has improved and her eyes seem a little more sparkly. (Just a bit, but I noticed). At one point Ava was trying to focus and made eye contact with me. She then moved her whole body when she could see me. It was wonderful!
She then took a nap, sucked on her tube while sleeping.

The conversation with the genetic counselor was pretty encouraging. I was reviewing Ava's labs with her. I was also asking her about respiratory failure and a Complex IV deficiency. I could not find anything about it. She said because respiratory failure is usually a secondary cause for death with Complex IV kids, not primary. Even in Eric's case, all other systems were failing first. She said that is not the case with Ava (I have to qualify with a for now, just in case). I told her all her labs are good, except for ones that point to muscle breakdown. So I asked her is it mitochondrial or is it nutritional? She actually told me that these values sound nutritional. Not a definitive diagnosis, but what I am cautiously taking away from this conversation is that it is not a slam-dunk mito disease. (Then again, what mito disease is slam dunk?) Anyway at least I know that I am not off my rocker thinking about such things.

Honestly, we have no new information. But Ava having a good day and being a little stronger are building blocks I'd like to continue. She has a great doctor on tonight and she will be back fro 7 day shifts in a row on Wed. She is open to my thoughts and ideas. She has a reasonable and conservative plan of care for Ava. She is also a Mom, which helps. She is an answer to prayer. I feel that she understands Ava better than anyone has tried. She is the only doctor in 12 days who came into our room and got the full story from us. From her birth on through. She, too, is concerned that Ava is not sedated. But then saw that she was not liking her chest PT. She made the comment that Ava is more awake. Then she thought that if she were to grow and get stronger she may have some fight about the vent. This doctor listened to me tell her how I observed Ava's personality as well.

She also thinks Ava looks better than she did last week. She also thinks her tone has improved some. She is going to get her into a different crib so we can hang toys for her to look at when she is awake. Her goal is that she is a feeder grower and to get the tube out. She thinks it's too fast to try and pull it because she is not strong enough. WHERE WAS SHE LAST WEEK!!!!!!!!!! At least we are on the same page.

So I like the feelings we have leaving the hospital tonight. I really like what Ava was doing too. I have never been so bold to think that we will outlive our children. I make a point to enjoy them while I have them and while I hope to have them for "longs" I know we may get them for "littles". It doesn't matter I'll take what I can take for as long as they are dishing it out. Tonight Ava looked like she was trying to wake up and show us what she is made of. I am laying my request before God: for us to take home a healthy Ava who needs no oxygen support, feeding tubes and will be on earth with us.

Trying to make sense out of the impossible

Ava is doing her own thing in the PICU today. She is sleepy. This is mostly because she is up at night. They plan on trying to start the CPAP trial again today. Every three hours, an hour on two off. She seems to have something going on in her left lower lung now. Maybe pneumonia, maybe not. She's still retaining some fluid. Not sure what that is all about. Could be the transfusion, could be something icky. They are going to get speech, OT and PT in. I was talking about that today and the Attending was thinking it too. That's all fine and good. In the same discussion the Attending informs us that it's very concerning that she does not require sedation while on the vent. We know this is a concern.

I am concerned with the notion that they are prepping us for some tough choices that we don't feel qualified to make. We don't have a definitive answer. We don't have much direction. I tried to page our contact at CHOP to see what is going on in their worlds. I would like to talk with our Neurologist so he can make sense out of this somehow. We know that there are more "experienced" places out there. But with a deficiency in complex IV I wonder why should we try? There are no answers and today it feels hopeless.

I can't even imagine a funeral for Ava or burying her. I am so not there. I so don't want to be there or have to try and . How do we decide what life is worth living? Eric made it easy for us to know what decision to make for him. I assume God will tell us too. Right now I feel like we are swimming upstream in a river of mud. And while we are trying to do that Ava's getting a little bedside OT/PT.

I do have to say on a little night sleep, we are not ready to deal with such an emotional blow. We will be fine. He is holding us. We just have to process and see.

Planning?

We enter today without much of a game plan. We have a loose idea of who goes where and how that will occur, but one the biggest bugs come fumbling down the stairs in all their sleepiness and chaos we won't know for sure. For someone who enjoys an action plan and a schedule this prospect is a little unsettling.

We have 3 sickies at home right now. Two are gloppy and in the midpoint. The other is a couple days in. She tends to be the worst sick of the bunch so it's not going well. Last night we were up about 3-4 times with fever, coughing, throwing up mucus, and a jammy change. We have often said that when Alexa gets sick, the whole house needs to be up. 4 out of 6 ain't bad. When she was three she would not go back to sleep. She would cry and cry and cry. She'd have to go potty. She was scared of a bug. We thought she was exaggerating. After about 2 hours we here this little voice crying out "I need a little love. I need a little love...." (Turns out that girlfriend had an ear infection. Oops). This is what we are faced with when Alexa gets sick.

Last night, Emily was keeping vigil for Alexa. She would rub her back and hug her. She would try and give her snugly toys. She cried and she prayed for her. We had a little chat at 3:30AM. Emmy was worried for Alexa. They always are for each other, but this time it was different. They were afraid that if they got sick then they would end up looking like Ava in the hospital. We touched on this topic before just in case their little minds spun out of control while I was unavailable. We talked about being big and strong and able to fight germs. We talked about how the tubes in Ava are helping her stay on earth with us. So even though they are icky, they are helping our baby. Necessary conversations that I don't mind having but really don't want to have to. Of course the poor kids have a social worker for a Mommy so, 'How do YOU feel???'

I was thinking that I was going to have to call the pediatrician and take some permutation of ladybugs in today. Questionable ear pain, various eyes tinted pink. However, it seems that some of the residual stuff is clearing for Elaina. Let's hope it's the same for the other 2.

So I have no idea which parent is headed to the PICU and which is headed to the pediatrician before the various childcare providers arrive. AHHHHH! I sure wish I could be split. Allan and I are going to have to play rock paper scissors to see which duty is the short end of the stick.

Hopefully when the bigger set emerge I can save a copay and hit the road.

As I type this Laina is feeding me homemade clay cookies before I've had my coffee. YUM. She's also been told that clay stays in the kitchen. So she's dancing around singing, "I in the kit..chen, I in the kit...chen."

Couple steps back, couple forward...

Well, Ava decided that breathing unassisted was not in her best interest quite yet. She was needing a bit of assistance to reduce her CO2 retention. So they tweaked her settings a bit. Nothing major. Her doctor didn't not want to be over zealous and tire her out. Then we could get her into a downward spiral. Hmmm, like when they tried too fast last weekend and then ignored her warning signs when mean doctor was on??? So honestly, we are grateful for this Intensivist's approach. Slow and gentle. Attentive and responsive.

I tried to review the day with Ava's nurse. I was trying to look at the positive. Something that I am trying hard to do. Ava is doing better with the weaning than she did last weekend. Today her CO2 was creeping up slowly and they jumped on it before she got tired. Last week it was CO2 up, O2 saturation down, quick desat when suctioned, body temp up, respirations up and Ava looked uncomfortable and like she was working hard. For tonight they will leave her on her new settings and see what happens from there.

She had some awake times today too. She's probably up now while I'm not there to play with her. Her eyes are still not "there" but her irises are clearing ever so slightly. She's making more Ava faces, sucking on her tube and not rejecting touch. I tried to massage one leg and one arm. She enjoyed it and moved against it.

Teeny Tiny Steps. Ava's motto. I see some over all improvement. She has now been fed for almost 72 hours straight. A record for her. I pray that this is what she needs to get back to her baseline. I am cautious about being too excited, usually a step forward means a bad day tomorrow. But I kept telling Ava that I'll take whatever she will give me when she gives it out. So for tonight I thank God for Ava's tiny steps and ask Him to safeguard her and make her strong. Eventually tiny steps turn into strides. I am asking Him to allow Ava to take strides and to eventually run. (I just think that would be cool).

Ebb and Flow

I sit her by Ava's bedside for the 11Th day in a row. It's encouraging to see that she was down on her vent settings and she looked significantly less swollen when we first got here. Since this morning they have been giving her a trial to see if they can pull the breathing tube. Depending on how she does the rumor is tomorrow that they would pull it.



Her protein level is normal and her urine cultures are too so it looks like her kidneys are doing OK and the transfusion was effective. That is an answer to prayer. All this information we asked the Fellow this morning and he said he'd get back to us. It wasn't until I finally got up the nerve to ask Ava's nurse about the labs did we find any of the information out. This frustrates me to no end. Doctor's and communication do not go hand in hand. I think it should be part of medical school training. That and how to give bad news.



Another testimony to how wonderful the medical system is popped up today. The attending came in and told us that CHOP just called. They have a bed for Ava if we want to transfer her there. When we were admitted 11 DAYS AGO they started a bed search at CHOP and we decided, with CHOP's geneticist that we didn't need to go. I have never heard of it taking 11 days for a transfer. Most length of stays are significantly UNDER 11 days anyway. We were like, 'way to go CHOP. Thanks for getting on that!'



I almost accepted the transfer just for a change of scenery. All the fish on the wallpaper boarder swim the same direction. I think they should embed a fish going the wrong way or other irregularities in the design to we can occupy ourselves. So far we've made up several games with her monitor. Now they are getting old. I was happily making prayer bracelets. Right now I am out of some supplies and I have to wait to get more. So for now, I blog. Allan naps. Two feet away is our daughter learning how to breath on her own. It's quiet which is good. She's not setting off any alarms.



Ava decided to keep her nurse company last night and was awake until 3AM. The little stinker!
I had a hunch that was going to happen since she started to open her eyes when we were leaving.


Ladybugs at home are all gross. I am praying that they don't need to go to the doctor and that they can kick the colds by themselves. So far we have Wheezy, Gloppy, and Stuffy. Sleepy is in the PICU, Happy is in Heaven with Jesus. Grumpy is typing and Bashful is napping. Yup now we are a Disney movie. At least they all live happily ever after at the end of those.

I am trying to sort out the feelings I've been having. It's complicated and all jumbled up. This time around is so different. I haven't figured out a good way to articulate it. Once I can I will. Right now I am just trying to plod through my emotional quagmire. I am feeling more angry, scared and sad. Feelings that I don't usually have for long are staying around. They feel uncomfortable. Like a pair of skinny jeans just out of the dryer after Thanksgiving dinner.

Ava did well with her four hour CPAP trial. She is now on minimal settings on her vent. Then they will do a couple 2 hour trials until they feel she is strong enough to keep it up.

This afternoon she was sucking on her tube and making loud sucking sounds when she did. She also smiled, with her eyes closed, at my friend who was visiting. It was sweet.

Waiting and more waiting

I am feeling very frustrated and a little angry today. It seems like all we do is wait. Wait for results. Wait for tests. Wait for Ava to get better. Wait for Ava to die. It's horrible to read. It's horrible to wait.


The implications of her probable mitochondrial disease are mind blowing. I find that I do some research and all I read are hypothesis and genetic mumbo-jumbo. There are think tank doctors and Neurologists. The result is a whole bunch of vitamins that don't really do a lot of good for a deficiency in Complex IV of the electron transport chain. Other complexes the vitamin cocktail works. Eric's deficiency was in Complex IV. It seems that people don't live too long with a deficiency there. This is exactly why we are so desperate for Ava to not have what Eric had. With that deficiency the road is different but the destination the same.


I wonder how long it will be before we don't have Ava anymore. I wonder what toll it will take on each of us and our family. I shudder at the prospect of feeling that level of pain and anguish again. I tire in fighting for Ava. I think to myself, "what am I fighting for?" but the answer is "For Ava".


I will never understand this road we are on and why we get to carry this heavy burden. I hold tight to the fact that God created Ava and therefore she is good. God can only create good. A broken person does not mean bad. In our humanness, we are all broken on some level. Whether it's mainly physical or mental brokenness we are broken. Only He is perfect. He loves all of us despite our brokenness.


Therefore, God created Ava. She is good and she is broken. But God loves Ava. She is on loan to us from Him. It is our job to love her too. He has a plan for her little life, no matter how long it should be. We know that. But we hurt so very much. And we just plain want a miracle that she is a keeper and does not have what Eric had.

We are drained today. There is an ebb and flow in a long hospitalization. I hope we are on an upswing tomorrow. Ava was admitted because she didn't have any reserve. We now don't have any left. We need to fill our tank to keep on going. Tonight my heart aches and is screaming for this nightmare to end. For it all not to be true. Then Ava tried to open her eyes a peak.

I look at her lying so helpless in that bed. I wish I could fight her fight for her. I can't. It's not mine to fight. I would do anything if I could be the one who had the tube in my throat and 2 in my nose. I would gladly boast 2 IV sights and a million blood draws and accuchecks. Instead I keep vigil at her bedside, longing to trade places.

I fantasize that there is a miracle cure in feeding her. That maybe they would uncover another reason that she is so compromised. I know we don't have a definitive diagnosis yet. Whatever it is, it will be a long way off. Today I just can't see past the tubes, the bed, and the edema.

Ava is on hold for vent weaning. She's developed some more atalectasis in her right upper lobe. She is very swollen tonight as well. Her protein level is low. Her Neurologist thinks that we need to see how she does with her nutrition as that should help. The Intensivitst agrees. (Tonight is our first night with him and so far we are impressed with him). They are going to give her some lasics, collect a urine sample and some more blood work. Ava may also need another transfusion as well. All this medical tweaking and balancing act is enough to drive anyone crazy. We are constantly waiting for the other shoe to drop. Each time something crops up we brace ourselves for what we feel is inevitable.

It happened to Eric, so we wait for it to happen to Ava. We know that Ava is not Eric. We know that God is in control. We know that His plan is just and good. We hope that Ava's story will be long and we will have her in our home with her sisters.

We came home and got to kiss the bigger ladybugs tonight. They were full of hugs, smiles, stories of their day. I look into their beautiful faces and am overwhelmed with how much I love them. How much I hurt for what they are going through. I look at our kids at home and our one in the hospital and wonder when and if we will all be together as a family. It seems so far away and so impossible. This morning I made pancakes, sausage, and eggs. We sat at the table and I thought I heard Ava. That was the first time through all this that happened. It gave me pause in my moments of feeling like a "normal" Mommy. I still am greatful for the breakfast and the snuggles. Even the fight over my lap (which usually bugs me to no end).


We are so tired of the intervention, the blood draws, the waiting for results we don't want to know about. We are tired of being separated from our children. We tired of waiting. We are suspended in time as the world goes on around us.

We have no idea why we are asked to do this again. But we do it. We feel it. We are living it. We are surrounded by the love of the Lord and an extensive support system. We are so grateful for that. Each prayer lifts a piece of our burden, our heartache, are praises, our hope, our sorrow, our request for a miracle. We would never be able to manage if it weren't for all of the prayer support, financial support, emotional support. The letters of encouragement. The meals. The snacks. The hugs. The socks, Yankees shirts, the bears and little somethings for our daughters. God is working we know it, we see it, we feel it.

The Air We Breathe

Not much to report today, which generally means it is a good day. Ava did well with her blood transfusion (amazing what a little blood will do for one's color!) They have begun slowly but surely implementing the "weening protocol" to get her off the ventilator. She is now on 16 respirations per minute, with a o2 level of 30% and a "peep" (positive expiatory end pressure) of 8. She is so far tolerating this very well, is taking several of her own breaths above the vent, and holding her own. In order to extubate, they will eventually get her down to 10 breaths and a peep of 5 (30% oxygen is already the minimum, as this is only slightly more than room air). Once that is accomplished, they will turn off the respiration rate, while still giving some assistance when she takes her own breath (since she is essentially breathing through a straw). If she can prove she can do this, the tube will come out.

Don't think that treading on this soil doesn't scare us to death. The fear with any baby this weak is that you won't be able to get them off the ventilator once a decision is made to intubate. Add to that an underlying mitochondrial issue that causes weakness, and it is downright terrifying. We pray that she is strong enough to do this. We pray that we return her to a baseline that involves holding her and starring into the beautiful eyes that have so captured our hearts.

Such a basic function of life that we all perform thousands of times a day and give zero thought to. Yet without it, we drown. How much is Christ like the air we breathe? Invisible, yet always present, surrounding us, "ventilating" our souls. Without him, we drown. Just like the song we sing in church:

"This is the air I breathe.
Your holy Presence
Living in me."

"This is our Daily Bread.
Your very word
Spoke to me."

"And I'm desperate for you.
I'm lost without you."

Butterflies and Eagles

This is a picture of the bandage the surgeon made for Ava. I really just thought that is was a sweet touch. He was just as kind when he came to talk to us. What I thought was really sweet was that he kept saying that he hopes we get good results.

Ava is now at a point where she can be fed and needs to get stronger. They are going to try and wean her off the vent soon too.

After the Ava I saw today I think she can do it. The Ava I saw yesterday has me concerned. It is all up to God and Ava. With His help she can. I really do hope that is in His plan. I would love it if it is. I would love her to get stronger and come home. I would also think it's cool to show up the know-it-all resident who is assigned to Ava.

Her blood transfusion is going well. I hope this helps to give her energy as well. The nice thing about the transfusion is that the Geneticist can't make her have any more testing. We were done anyway.

Please pray for Ava to get stronger and to have God's strength. The following is our prayer for her. Will you pray with us?

Isaiah 40:29-31

He gives strength to the weary and increases the power of the weak. Even youths grow tired and and weary, and young men stumble and fall; BUT those who hope in the LORD will renew their strength. They will soar on wings like eagles; they will run and not grow weary, they will walk and not be faint.

Ava just got back from the biopsy. Everything went well (as expected). Only minimal anesthesia was used, which is an answer to prayer.

Blood was drawn after the procedure. Her lactate is slightly elevated (7.8), but this could partially be due to the "trauma" of the biopsy. We hope this will level off again once full feeds are resumed. Of greater concern is a low hemoglobin, which means she will need a transfusion. We just got to cross of the last thing on our list that we so did not want to do to her, but we still couldn't avoid this, which stinks. She needs it; it will help her. But in a week and a half of haunting memories, this brings back possibly one of the most painful for me. Because of everything we had to do to Eric, who was at least 4 pounds bigger at addmission, he also needed a transfusion. He was actively dieing. His blood pressure had tanked. Our nurse that evening, who had not previously been assigned to Eric had so much hope: "Once we get this blood into him, you are going to see a world of difference..." she would say. But in my heart I knew otherwise. And I was right. There was no improvement, and he died shortly afterward.

This is different. She is different. She can take this. It will help her. And I still hold on to hope...

-Allan

Just as quickly as I was done typing, they came and took Ava down to the OR for the procedure. She will be down there about an hour. Will update later and let everyone know how it went...

Holding Patern

Knowing that Ava was not on the schedule for Muscle Biopsy attempt #3 until at least noon today, we elected to sleep in and spend some time with our other bugs. Elaina ended up in our bed sometime in the middle of the night. She is drippy and running a slight fever that is well controlled on children's Motrin. We had good snuggle time with all three in the morning--. Emily is also a little under the weather; Alexa should be soon to follow. Medication suppressed fevers aside, we had some good and much needed family time. Amy made scrambled eggs and when we got ready to sit down Emily said "Mommy- are you going to eat breakfast with us!?" If we could bottle that, it would get us through most any day.

Upon arrival at the hospital, Ava decided she might need to wake up and warm our hearts. She opened her eyes for a good period of time and was responsive, smiling, and almost playful! It's hard to describe how good this was for us! My poor little girl has been so tired and wiped out it's seems like forever since we've seen a glimmer of her personality. I think she knew we needed some cheering up.

As for the biopsy... as I said she was on the schedule for noon. It is now 1:20... I'm confident it will be any time now and that it will actually happen today (if it doesn't, it won't happen at all because we will be pretty much done with the whole thing at this point). We are used to waiting. Not necessarily good at it, but used to it. So, here we sit.

Pray for patience; pray that Emily and Elaina will feel better and that Alexa might be spared; pray that Amy and I will not get sick; and praise God for scrambled eggs, big hugs, and open eyes!

Peace,
Allan

Waiting, continued....

We are wiped out right now. Emotionally, physically, and mentally. We feel so torn. Be with Ava. Be with our other girls who we miss terribly. We got up early 2 days in a row so we could be at the hospital for this pending muscle biopsy. Because of this we missed the girls and haven't seen them since a brief visit on Sunday. Now they are all a little stuffy, drippy and gross. Which makes it especially hard to be away from them. (I must be exhausted I want to be around grumpy and drippy kids!)

It seems that the muscle biopsy will now happen tomorrow about lunch time. Today she kept getting bumped due to more critical needs. Totally understandable and expected. We are frustrated. Ava wasn't able to eat for 12 hours again today as we waited. I feel like feeding her is the only hope we have of getting her back to some level of functioning. I am so afraid that if we don't do something soon she will become too weak to recover.

Today Ava had to deal with moving all of her various tubes. All seemed to migrate. Her breathing tube was still in the wrong place. They moved it, taped it and it helped a little. The new Attending for the week thought it needed to be moved ever so slightly. Her lung was still "whited out". She did not enjoy this. She cried, silently with tears. It was so sad but encouraging at the same time. She was showing a little spunk. This new attending was really addressing the issue for Ava. She was given chest PT and Albuterol. These things really did help. By 4pm her lungs looked pretty good. I could hardly believe it. I never thought that she would be able to overcome that.

She just looks so tired. I keep waiting for her to bounce back. Instead she keeps having to overcome set backs. She's still persevering to what end remains to be seen. She is teaching me a lesson in tenacity.

She is a sweet baby. She is tougher than Allan or I could ever be. I am trying to hold on to the hope I have for her, but today it's waining. I'm just so sad. For so many reasons. The depth of its layers astound me. When I try to think about them or go down the "what if" road my breath is literally taken away. I am then pulled back in the moment and reminded to be still and Know. God is in the details and has the tomorrows taken care of. This is a hard lesson for someone who not only likes to plan ahead, but likes to have 3 scenarios planned out.

We are faced with some tough stuff. Some of the toughest out there. We are worried for Ava and having to face Eric head on. We wonder about the all too real possibility that Ava may not be a part of our lives for as long as we want her to be. We are concerned about the choices we are making and dread the potential of the ones we may have to make in the future.

When I am lost in these thoughts I am reminded that even though some doctors have written Ava off, God has not. He told me that it was time for Eric to go to Him. We have not heard that about Ava. I fear it. I realize that it's a possibility. But I don't KNOW it. I may someday, but not today.

I am hoping in the ebb and flow of our emotions we will find a peak and be able to recharge our batteries. I suspect playing with our bugs tomorrow before we go support Ava will do the trick.

Waiting, as per usual

Ava is still waiting for her biopsy. We have no time. She has had all kinds of tube placement issues today. Her breathing tube needed to be moved a touch and re-taped. Her feeding tube was not in the right place and it's needed to be readjusted twice.

She's only getting IV fluids now as we wait for her surgery. She's acting hungry. She's crying and trying to suck on her breathing tube. I hate to see her like that but am grateful for her appropriate responses.

Her carbon dioxide retention levels are better, but her lung still looks a little funky. So now she is getting a nebulizer treatment and chest PT to help her get the gunk out. We are also going to increase the PEEP, which was dropped this morning.

I really just want to hold her.

Slide Show of Ava...

http://www.slide.com/r/lHoGUqTmxT-4MbnE6rF40sqi7LrkBsMS

Click the link for a slide show of Ava. Thanks to our good friend Libby Foester of Libby Portraits for generously and lovingly doing this for us. Please visit her web site at http://libbyportraits.com/ or search for Libby Portraits on Facebook.

Juggling

We are juggling so much right now. It's a shame that Allan is actually the only one of us who can actually do it.

Ava is an "add-on" for her muscle and skin biopsy tomorrow. She will stop her feeding at 4am, this way she won't be without food for a long period of time. It is hard to know what to do. What is more important, feeding or samples. We continue to pray for guidance. We want Ava to have every chance to get stronger so she may be able to learn to breathe on her own again. Nutrition is a big part of that. We don't want to wait for her to get on a schedule because we don't want her to get too reliant on the vent. Plus I don't really want to understand all of this. There are too many mind blowing implications.

So we juggle her feeds, her biopsy and eventually her settings. We hope they are the right choices as we are shooting blind here. Of course God is directing the trajectory. The current working plan is that we will hope she gets an early time and have the procedure. We also hope that her lung is inflated. If it takes too long, then we will restart feeds and probably scrap the biopsy. So we will wait and see. The biopsy is important to see what is going on in Ava's body. It won't help to "cure" her. We will just know. The geneticists want it for research and to get an answer. This is not our top priority. The annoying thing is that it will take about a month before we know what is going on in Ava's body. I hope we have her that long.

I am worried about her ability to come off the vent. I hope she is strong enough. I wish she could get food, uninterrupted for 48 hours. Baby steps I know.

I want to find Ava's baseline. I want to raise her to her full potential as an adult. I want to teach her about Jesus and His love for us. I want to raise a hearing impaired child. I want to hold her and never let go. I so very dearly want all of this to be false. And I really want to be with all my girls. I want Alexa to keep her "A" buddy. I want Elaina to smother her baby with love. I want Emmy to hold Ava and sign to her. I want her to sit, stand, walk, talk, run and feel the wind. I want to say to her, "Ava, I remember when we almost lost you". I want to be annoyed by her, laugh with her and be able to put her in time out. I want to raise the four children I have. These are the desires of my heart. I know that God is ABLE. He does all things according to HIS purpose.

A song that is in my head: I am pressed, but not crushed. Persecuted, not abandoned. Struck down, but not destroyed. I am blessed beyond the curse. His promise will endure. His Joy will be my Strength. Tho the Sorrow Lasts through the night, His joy comes in the morning. I'm trading my sorrows. I'm trading my pain. I'm trading them ALL for the joy of the Lord. I'm trading my weakness. I'm trading my shame. I'm trading them all, for the Joy of the Lord.

Trade them with us. It just makes life livable.

Peace Like a River

Ava had a repeat chest x-ray at 9:30 and her right lung is still not properly inflating (this is called "atelectasis") To correct this they have re-positioned her tube twice now and have increased her "peep", which will assist in better inflating her lungs (It's amazing how doctors can throw out words like "atelectasis" in one breathe and "pop her peep" in the next). The atelectasis is not a major concern, but it is a reason for anesthesia to not be comfortable in sedating her for the surgery. So the muscle/skin biopsy has been called off for today and we have started her feeds back up. So again, we wait...

Ava seems to be much happier with her breathing tube in a better position, and is having a much better day today. She is resting comfortably and peacefully and expelling the CO2 from her body much more efficiently (go figure!). Peacefulness for Ava goes a long way for peacefulness for mommy and daddy as well. So, for now, we rest in that...

Procedure update

How many times have I said that when you walk into the hospital you never know what you are going to find? This morning we found her Neurologist at her side and a pretty drugged and gross looking Ava. (I almost typed Elaina's name. Possibly because she is always covered in some sticky substance). However, this icky looking Ava had her eyes open too. She is becoming more awake, but not alert and oriented yet.

The reason for her appearance is due to the fact that Ava had a chest x-ray in the night. Apparently the tweaking of the vent was not working well. As it turns out, the vent was not positioned well and her right lung was not inflating. So they gave her some sedation and a paralytic, repositioned the tube and retaped it too. They got a repeat chest x-ray. Lung still not inflated, tube needed to be moved a little and adhered properly. So her biopsy is on hold until about 11 or 12. Assuming her lung will inflate. (This is where I would like to insert that 18-24 hours ago I was wondering if she needed a chest x-ray.) I am praying that the fact that she will be at least 12 hours without food will not set her back even further.

Last night I thought Ava was going into respiratory failure as a result of her presumed mitochondrial condition. Today I find out it's tube placement. Maybe next time we try to wean her off the vent we can make sure her tube is in a good place and we can listen to Mommy when she says something is up.

I am not bold enough to think that these crazy ideas are coming from me. I know that God is putting them in my mind. This stuff is way too complex to know it without training.

Tues

So no phone calls through the night means Ava must have done OK. We are going to go sit by her bedside and continue to wait. The girls are all tucked in their beds. I got to kiss each of them last night. No one woke up either so that was a bonus. When I kissed Emily and Alexa I saw that they were all intertwined, just how they slept as babies. Little Lain-Lain fluttered her eyes, rolled over and sucked her thumb. When I kissed Ava goodnight last night she too fluttered her eyes and peeked them open.

I long for a chance to tuck them all in myself, kiss them in their own beds and wake up to face the day with my four wonderful daughters under my care.

Ava has her muscle and skin biopsy in a couple of hours. We will not have any results for about 4 or more weeks. We hope we have her when her results come in.

Sometimes, often really, we find ourselves wondering How this became our life. We hold tight to the fact that this is God's Plan for us. Since it's His plan, it is perfect. Hard to say, hard to swallow, but acceptable because as strange as it sounds, we KNOW that He loves us. Even and especially through this.

Monday

Today has not really been a great day. Ava's had some set backs and that is never fun. Her labs are essentially the same, which is always good. She needs to have her vent settings tweaked still. I have been bugging our nurse all day about it and he has been bugging the doctor. New shift = New approach. I hope we didn't set her back too far. Right now it looks like all this hulabaloo is because of what they are doing to her. I really hope that 's it. I guess I just want it to be that.



Ava had a busy day and she is wiped out. She had a lumbar puncture and an EEG. She's been uncomfortable and crying off and on today. She really just seems that she's had it with all the poking, prodding, Accuchecks, lactate draws. There was a point today where the Geneticist at CHOP ordered blood tests as a check and balance for her Lumbar Puncture labs. It was going to be a WHOLE LOT of blood (for you medical folks 10 cc's). I got on the phone and made them tell me what the top priority was and WHY they thought that it was necessary. I also made sure that they knew that we would have never allowed the LP if we knew that all those extra labs had to be drawn. Well, as it turned out, we suddenly only needed 2 cc's of blood. One value for 4 cc's was never even elevated in Ava and the other was drawn within the week (another 4 cc's). That one had to do with long term storage so why would it matter what the value is 5 days later?

When we got to the hospital I made it clear that we will not do things to Ava just because we have pens and can write an order. Ava is a unique patient and suddenly we want to find things out just to "know". I told the Geneticist that Ava will not have so many labs drawn that she requires a blood transfusion. I told them that we are now starting to do all the things to her that we did to Eric and I will not let her be tipped over the edge like he was pushed.

It is just so infuriating.

As a result of all today's events we decided that we wouldn't proceed with the muscle and skin biopsy tomorrow. Our Neurologist came to talk with us about it. He was very kind and completely understood our concerns and desires not to do it. As we were talking he was assessing Ava. He had already seen her in the AM. He noted that Ava was doing much better than she was this morning. He was actually impressed. He also thought that she could handle the biopsy, if we choose to proceed.

We prayed over Ava and feel that we should proceed. So we will.

The Attending in the PICU feels that we haven't found Ava's baseline yet and that she has lots of room to improve. She also feels like we will be there for a long time. She reported to us that the neurologist was actually surprised and impressed by how well Ava did tonight form his standpoint.

That seems to be how Ava rolls.

Ava has no seizure activity on her EEG so we are reducing her meds there. She has no metabolic changes on her MRI in her brain (Eric really didn't either). She has no lactic peaks on the MRS and no other findings at this time. And I just learned that glucose production is tied into lactate production if it's not working properly. I really need a diagram about that.


I do not know what to make of all these things. I feel torn. Ava needs us at her bedside to advocate for her. Emily, Alexa, and Elaina just plain need us. I have these 4 kids and I don't feel like I am Mommying them at all. We are needing to come up with a plan that will keep every one's best interests at heart.

Ava decided to let us know that she is not too keen on weaning her vent settings down yet. She is still on minimal support, but needed to go up on the number of breaths she required a minute. She was holding her own, but as the day wore on she was showing signs of tiring. When we finally left tonight, she seemed much more comfortable. She was also on 5 breaths a minute less then when she got to the PICU.

We are trying not to let that get us down or read too much into it.

Tomorrow we will know Ava's repeat lactate level and the results of the MRS. Ava will also have an EEG. As expected, we are concerned at the prospect of hearing things that we don't want to hear or know.

I pray that she has a restful night and can work on getting stronger throughout her whole body. She is a sweet baby.

The girls will spend tonight with Allan's parents and then my parents will take over for a few days. I so wish they were home so I could kiss them good night and help them get ready for school in the morning.

It was nice to see all our ladybugs together today. I noticed that the bigger set were having a harder time today. They are worried for Ava and miss her terribly. They miss us too. We certainly miss them. They are sweet girls. Observant too. Alexa immediately wanted to know why Ava had another tube. I didn't know what she meant at first. Then I realized that she was talking about her feeding tube.

Tomorrow is a new day. I hope it's not too draining.

Sunday Morning

Ava had another good night last night. Let's keep stringing them together. Actually, she needed to be sedated today because her plethora of tubes we aggravating her. So far she is beautifully tolerating her feeds and the vent weaning process. I got to change 2 messy diapers this morning.
Her gross movement was better and she was trying to open her eyes some. She is off the IV fluid and is getting an ounce of 24 calorie formula an hour now. As a result she is less puffy and looks a little more like our baby girl. She was getting all hot and bothered when they repositioned her. It's nice to see some level of spunk.

Never in a million years would I have thought that Ava would be fighting back the way she is. I have great hope for her today. I also am experiencing a floodgate of love for her. I've been holding back since we found out that she failed her newborn hearing screen. I've been so fearful of loosing her. But today it's all there and I can't hold back any longer.

I was remembering how I was so over the moon when little Elaina was born. I went back to work just so in love with her. I've been sad that all that joy I felt for Elaina has not been there with Ava. I've been filled with worry, and concern about her health. Today I look at that beautiful girl who was on the cusp of death and I admire her. I adore her. No matter what may be "wrong" with her I want her. There is so much more on the line now that I am feeling my true feelings. But If I didn't allow it and then she did die how would that be fair to her? She deserves the unabashed love that we can give. When she was born we talked about how she was the gift we never even knew we wanted. But we got her and we so want her.

There are two recurring themes since Ava was given to us. The first is that Ava just needs to eat. I said this after her critical blood sugar after birth. Actually I said "it's not Trisomy 18, Downs Syndrome or mitochondrial disease. Ava was hungry". It was amazing how she perked up after that early feed. The other is that we've often spoken about through Ava God maybe getting us where we need to go in Eric's aftermath. In a grand scheme I really don't know what all that means. I just know that they are things I can't get out of my mind.

Right now I'm going to go an kiss my baby so she can hit my head with her IV laden arm. Then I plan on locating some not gross coffee. After that I'm going to get ready for the Ladybug invasion as they are coming to kiss their baby today.

Hope for more than a half an hour

Ava is making some strides today. She is now off of IV fluids and is getting an ounce of formula an hour through her NJ tube. She is still on the ventilator, but is beginning to be weaned off of it. She will not come off until at least Tues as she will have her muscle and skin biopsy's. She is moving around some too.

Still no final read on the MRS. That won't be done until Mon. The MRI portion has nothing different to report than from before.

I miss our girls. We feel so torn, knowing that it's OK. We just want to be with them too. Elaina has been crying out for us at night in her sleep. This breaks my heart. They all are handling this well though.

We cannot express how grateful we are for the outpouring of prayers, love and support we are receiving. While Eric was sick and after he died, we dug deep to pray for our son and through our grief. The reward was profound as we felt the Hands of God guiding us. The emotional toll was pricey. To this day we are unable to expend that energy like that. We pray, we cry, we even laugh. The intensity with which we approach the day is not nearly the same. What I am seeing is that God has called thousands to pray for Ava, for our family with ferver, intent, passion and desire. He knows that we are drained and He is calling hosts of people to support us in the area where we have the greatest deficit. Thank you for honoring that call and helping our family walk this long and frightening road.

Our little ladybugs are precious to us and are His gift to our family. For that we are so grateful.

Saturday

The doctors have not made their rounds on Ava yet so we don't know any official results. What struck us this morning is that the Neurologist covering for Ava's doctor stopped by her room today just to introduce himself. He wanted to let us know that he is aware of the plan of care. He is aware of our story and family history. He will not be actively involved but if we need him he is available. That is the way medicine should be practiced.

Allan and I both woke up this morning feeling down. This of course made us nervous. You never know what you are going to find in the morning. This is so true for a critical patient. Also it was true at our house (ours house as Elaina used to call it). This morning we found 2 extra people there. My brother in law and niece were flying home from Spain and did not make their connecting flight in Philly. Off to hotel Benton they came. (We don't charge much). Mark has not had a chance to meet Ava, so we feel truly blessed that he will have that opportunity.

Coming to the hospital we were hoping for good Ava surprises too. I was expecting that there would be bad news. There wasn't, per se. Her lactate level increased in the last 24 hours, but not by much at all (.9). She was fed all night and is going up on her feeds. She is stable as she can be. The kind Neurologist looked into her MRI results yet. The final report is not in however the preliminary report shows that there is no obvious stroke. Also there are no peaks of lactic acid build up in her brain. (I did not know that that could happen either.) We are waiting for the final official results but are encouraged.

A friend of mine messaged me and told me that she has hope for Ava today. I like that. I think we will too.

Addendum

The Resident just came in and informed me that the Geneticist and the Neurologist are just as confused by Ava's elevating lactate and her normal labs. We are now going to be doing and EEG and an MRI as well. This is to check if she was having seizures or had a stroke.

There are so many things swirling around in my mind. Nothing tied together, just thoughts rattling around.

Ava's lactate level is going up. This is not really good news. It's indicative of her malfunctions in her mitochondria. Now, Ava hasn't eaten since our admission here. Her mitochondria are hungry. So we are trying to feed her. This is with a ND tube. Hopefully she will get some nutrition and this will help to stabilize her fragile metabolic health. They started to feed her last night. Her lactate increased with 2 separate draws. They decided to stop feeding her. We came in this morning heard the news and got the kibosh put on that. Girlfriend needs some food. They called the neurologist and he agreed. So Ava is fed.

We hope that her underlying metabolic disease is not too insult that she can't recover from it. Again, only God can do this. We just support her and try to get her whatever shot is available.

She is moving around a lot more today. It's fun to see.

Because we are at a point in DNA research that for the most part reveals that Ava does not have any mutations on any of the KNOWN genes that cause mito depletion and COX deficiency AND Ava is now symptomatic we can assume she has what Eric had. Therefore we are needing to have the testing done on Ava that we so wanted to avoid. On Monday or Tuesday Ava will have a lumbar puncture, a muscle biopsy and a skin biopsy. These things will tell us more information about her disease. They will also take months to come back.

We are forced to live in the moment. We don't know how to balance that with the ladybugs at home. We need to figure out how to incorporate school, work, three healthy kids at home and one very sick on in the hospital. We can't be everywhere all at once, but we wish we could. We are spread so thin. We need to work so we can continue to provide for our family. But the added stress that comes with that is overwhelming, at best.

I feel like we need to be here to make sure that Ava is getting the slow and patient care that she requires. That we are gentle. Not quick as medicine tends to be. I did suggest to Ava's resident that she should get out her pen and take notes because we have an interesting case study here. Also that AI needs to establish a mitochondria protocol. It is my feeling that as the research base increases and more genes are identified there will be more reported cases of this type of disease.

We are so happy that the physicians and staff here are really listening to us and following what we, as a treatment team, feel is in Ava's best interest.

If I look down the road I become overwhelmed. As one that likes to have an action plan that is frustrating. I miss my bigger bugs. I want to hold my littlest bug. I can not fix it, I so wish I can. I wish I could bear these tests and this illness for her. But that is not God's plan for Ava.

It's overwhelming to know that we have a disease in our family that is on the cutting edge of genetic research. We are paving a way to what we don't know.

God brought Allan and I together. I know that this is His plan for our family. I also know that Ava's sisters were made by God and are given the "stuff" to handle Ava's life and her illness. I have to hang onto that when I worry about how this will affect them.

Gearing up for today

This morning we woke up to a house full of our children. It's been so quite here without them. It's so nice to have a little normalcy. Elaina woke up about 5:50 crying for us. I scoop her up and and snuggled her in bed. It was the best hour of sleep I've had in a long time.

I got a couple seconds to explain to the Emily and Alexa that Ava is getting a little bit better, that we don't know if she will come home with us, but we hope so. I also told them that the doctors do think that Ava has other things broken in her body, not just her ears, just like Eric. I told them that that doesn't mean she's going to go to God right away. So we are to pray that Ava gets stronger. I told them that only God can make Ava strong. So simple, but it actually makes sense to me.

We can give her vitamins, feeding tubes. But God is the one who created her. He is the only one who can sustain her. I will never ever ever understand the whys. But I do know that if he chooses, He can and will do it.

Yesterday I had a hole in my sock. It was driving me crazy. I thought, 'I really need new socks'. Ava received 2 stuffed bears during her hospitalization. I gave each one to Emily and Alexa so that they can hug them for Ava and love them for Ava until she can. I thought, 'I need another bear'.

One of our visitors bought me a pair of socks. Another provided the 3rd bear. I thought, 'Lord clearly you can do anything. So how about you now fix my baby.'

I know He provides, even if it's as seemingly as insignificant as socks and a bear.

Peace

We walked in today to find that Ava had a restful night and was fairly stable. We were concerned to learn that her urine output was not what they would have liked it to be. We, of course, were concerned that her kidneys were symptomatic and that she was beginning to suffer further from her mitochondrial disease.

We spent most of the day waiting to see if we were going to transfer Ava to CHOP. After talking to her geneticist we decided that she would best be served at this hospital. There is nothing different that they would do there for her than they would do here. We are glad to know that we don't have to transfer her now. She needs to rest and try to get better.

Due to some of Ava's labs and her tone it can now be presumed that she does have a mitochondrial depletion disease. We can also pretty safely assume that it is the same that Eric suffered from and that we are not going to know the route cause of it at this time. It seems we may just harbor a gene that has not yet been discovered. We did not expect this to go this way, but here we are.

Our doctors do not think that given Ava's current picture that she is suffering from a terminal illness at this time. We will try and continue to get her off the ventilator, get her bigger and stronger. We are going to try and give her a vitamin concoction to support her weakened mitochondria. Ava is very fragile now. She is still very sick. There is no cure for metabolic disease. What we can hope for is that Ava will not have a horrendous a course that Eric had. That she will somehow surprise us and be remarkably strong. We fee like if anyone can do that, it will be Ava.

We are having to make further decisions for testing for Ava. These are things we never really wanted to have to do. We know that God will lead us. We don't know what we are doing and are so concerned that we will cause her harm.

Ava is starting to take breaths over the vent, is trying to open her eyes and is moving some. This is all so nice to see. Her urine output is also increasing.

We got to see our other ladybugs today. It was the best medicine for these tired parents. They were able to visit with Ava too. They all handled it beautifully. Each one kissed Ava and she opened her eyes ever so slightly with each kiss. She loves her sisters and they sure do love her. I am so thankful that God gave each of our daughters that to hold onto. No matter what Ava's outcome is.

We do so hope to keep this little girl. We boldly ask that she is not very harmed by her condition. We pray she gets healthier, bigger and stronger. We are praying that her little body with her injured mitochondria stay strong and keep on producing energy.

AI DuPont

Emily Signing "I Love You" to Ava.

We are sitting by Ava's bedside with some incredibly heavy hearts. Ava has a serious case of pneumonia. It also looks, based on her labs that a mitochondrial disease is rearing an ugly and unwanted situation.

We are angry and heart broken.

We have some very tough decisions to make for Ava and for the rest of our family.

Please pray for discernment and peace as walk this haunting walk through the valley once again.

Urgent Update

Dear Friends,

I am taking the liberty to update the Benton's blog. I just received a call asking for urgent prayer for Ava Benton.

She is being transferred to A.I. DuPont PICU. She has been having seizures and has been intubated.

Please keep the entire family in prayer. I will update the blog as I get more information.

Joy Cronauer

Ava held on to and played with her monkey forever yesterday. She kept pulling it on top of her. It was really precious. Allan had to take it away so she would sleep.

Stuff in my Head

I went to bed last night a little unsettled. Not too bad, but not what you would have expected after the nice day we had yesterday. Then I realized that Eric went to the hospital with what we hoped was an infection. It was the disease fast, furious, ferocious. We would get intermittent good news not knowing if it was good at all. Eric would have good moments. He had a monkey too. He discovered it and was comforted by it in the hospital. After each good moment, that I now view as a "breather" and a gift from God to help us get to the next step, something terrible would happen and the day would stretch into what seemed like a year. We would hold onto any thread of hope that we could grasp, knowing deep down that it was futile but all we had. We watched him slip away from us every day, slowly but surely our prayer requests changed from just let it be this to OK if he's only like that we can do it. To finally, Lord please heal our baby and take him Home.

We know Ava is sick right now. We know she's got no reserve because she has had a rough time getting bigger. I know we got some really encouraging news yesterday. But I feel like I am still waiting for the other shoe to drop, so to speak. I can't possibly believe that she will get well from this, that pumping her full of calories will improve her neurological status, that this nightmare will end.

Do I HOPE for all those things? Absolutely. I feel that they have a better shot of coming true than they did for Eric. But I can't let myself get carried away with it because we've been so crushed before.

I've always told people in the aftermath of Eric's death that there is never any guarantee that it wouldn't happen again to our family. God does not promise us that. He promises that if we believe in Him and that His son is our Lord and Savior we will have eternal life. We will be completely healed from our sin and be ushered into His presence. There is no sin, there is no disease, there is no ugliness of this world with Jesus.

I've also tried to make it very clear to God and everyone around me that while we are not "good enough" to be spared from that type of suffering again we certainly would prefer to never have to do that again. Seriously, we aren't kidding. This time around we have so much more on the line having our three daughters to usher through this. We know He is carrying and protecting them.

So I Hope for my deaf baby who had a rough go. I hope that I get to keep her, hold her, touch her. I cling to what I can find to get me through the next minute, hour, day. I know that He has the only answer. I know that He will help me continue to breath. I hope that Ava continues to recover and annoys me just as much as her three wonderful sisters do someday.

A Good Day: We'll Take it When it Comes

Emily, Alexa, Elaina, and Ava are all tucked in for the night. The older three are on "vacation" at Grandma and Grandpa's house. ( It's a vacation for us and for them. Not so sure about for Grandma and Grandpa however....) Ava is snuggled in for the night at the hospital.

This marks the end of a good day. Who would have thought that a failure to thrive preemie with pneumonia would ever be an answer to prayer? But it is and she is. Ava is tolerating her new feeding regimen well. She is also slowly but surely beginning to perk up some. She had lots of awake time today. She played with her monkey and a new Beanie Bear that one of the hospital pediatricians gave her. Tracked them, batted at them. Held them in her hand. Allan put her hand around the ring of the monkey and she held it for over an hour. She'd stare at him, move around a bit, pull on it. It was really cute.

The Cardiologist read her Echo Cardiogram. Everything looks great. She had a small VSD and a questionable Ductus a month ago. On this exam, both are not detectable. He is not concerned with Ava's heart at this time. YEAH another specialist we get to put o the back burner! I even get to cancel her upcoming appointment with him!

I spoke with our Neurologist by phone today. He called the floor to check on our sweet baby. He was pleased that she has pneumonia and that her lactic acid level was what it was. He was also really happy to hear about how she was playing with her toys.

We will be testing Eric's DNA for mutations on 2 genes now: POLG1 and DGOUK. POLG1 is actually in process and should be ready in 2 weeks. This is the same time that the Mito-Meta array will be ready for Ava's DNA. We actually thought that POLG1 wasn't started yet due to the trouble in the lab. So this is good news. Once paid for we will start DGOUK- Hopefully by the end of the week.

We know that Ava may have underlying disease, but it nice to think about how to treat an infection and turn her around nutritionally. It seems too simple and I have my doubts, but I see it starting to work. So I'm going with it.

Please pray for strength for Ava. She really needs to grow and move more. Develop muscle tone and head control. She is tough and a fighter. We see that.

Please pray that the girls are protected and not too scared by all that is going on.

Please pray that Allan and I are sustained and can discern what God is telling us.

Please pray for Ava's doctor's and nurses that they can be wise in her treatment.

(I would still love some supernatural timing on results. I am a little tired of waiting and have never really been that patient. I also pray that Eric does have a mutation on one of these two selected genes. Then our search is over. Plus from a cost standpoint, we would really love to get it right with one or two tries. If we can't find a mutation on Eric's genes or Ava's then we have to face the fact that they have not discovered the gene that is mutated yet. We don't want that. It is what it is, however.)

My heart and soul long to be done with all this mess and know. We so want to know that Ava is OK. We know that God knows and it's all in His timing.