Just when you are going along swimmingly, the phone rings. (OK, in all honesty we weren't too swimmingly today. The girls were bugging each other all day and Elaina was having a rough wake up from nap. Tears, needing to eat dinner practically on top of me, wanting milk, no I don't, yes I do. You get the chaos).
OK, now the phone rang. The caller ID said Nemours Foundation- an ID we would rather not have to deal with ever again, in all honesty. It was Ava's Neurologist. I extract myself from Elaina to take the call (cue screaming teething 2 1/2 year old in the background).
Ava's results from the muscle biopsy testing that was delayed is in. Our dear sweet baby girl not only had a Complex IV Deficiency, but a Complex I as well. Apparently Eric did as well, but we didn't know that until today. Her mitochondrial depletion in muscle studies came back. Ava only had 7% of her mitochondria. She was 93% depleted. Eric was 99% depleted.
I knew it was bad. DUH, do I have two of my five babies? I knew that Eric's results were so incredulous that the Geneticist at CHOP wondered the validity of his testing. They did not think that he should have even been alive at birth. So folks, here it is. We have two awesome kids in Heaven who seemed to defy some odds.
How do you live with only 1-7 percent of your energy makers? Why do you live that way? This news is so fresh it hasn't even sunk in yet. I'm sure it will take a long time to get through some of the questions that are forming a funnel cloud in my brain.
We are so glad to have the opportunity to meet these heaven bound bugs, but right now I wish I never ever had to care about mitochondria, DNA testing and all the other meetings we are going to have to have. I just want my miracle babies who never should have lived at all. (At the same time I am glad to had them while I did).
Showing posts with label Ava. Show all posts
Showing posts with label Ava. Show all posts
Friday, January 8, 2010
Friday, December 18, 2009
Victory
Today at approximately 6pm Ava Elisabeth Benton gave up her courageous fight on this earth and entered into the arms of her saviour. While we are incredibly sad, we rejoice in her complete healing and victory in Christ. We thank everyone for your faithfulness in prayer to our family. We request your continued prayer for our family for healing in our time of grief.
Thursday, December 17, 2009
Home
My wonderful big brother is going to sit with our beautiful baby this morning so we can get a little ready for Christmas. My heart is at the hospital, but I need to be here for a bit too.
Last night I didn't post what I started yesterday because the lap top's battery ran out. I was making photo books for the bigger bugs. Book of each of them with Ava so they can always see how well they love their sister and how much love she had for them.
To say that cycling through those pictures to find the right ones to use was hard is an understatement. It really showed me the progression of how unbearably ugly and degenerative this disease is. Ava is living her life right now on the brink of death. Yet she is doing it better than I think I live mine, fully functional.
I've wondered how Eric's life and death jumbled in with Ava's life and illness would shake out. How would I separate the grief the pain, the joy the hope. How would each child get their due in my humble mind. I can tell you all I know right now. It is different, because they are different people. I love each of our 5 unconditionally and I love them differently, where they are and for who they are. In sorrow and grief that individualistic love is expressed in a way unique to each child. This is a relief to me as I have been concerned how that would play out.
I KNOW beyond a shadow of a doubt that God can heal our daughter. But I ACCEPT without reservation that that kind of miracle would have to be in accordance with His plan. That type of miracle would not be for the Benton family, although we would benefit immensely, but it would be for His glory and purpose.
As much as I long for that to be in accordance with God's plan, I know that if and when Ava is called into His presence He will be glorified and she will experience the most amazing miracle of healing there is. She will become fully restore and will truly "fly on the wings of eagles." She will truly be able to "run and not grow weary and walk and be faint". I will be so earthly sad when and if that day comes. But I am eternally grateful.
Last night I didn't post what I started yesterday because the lap top's battery ran out. I was making photo books for the bigger bugs. Book of each of them with Ava so they can always see how well they love their sister and how much love she had for them.
To say that cycling through those pictures to find the right ones to use was hard is an understatement. It really showed me the progression of how unbearably ugly and degenerative this disease is. Ava is living her life right now on the brink of death. Yet she is doing it better than I think I live mine, fully functional.
I've wondered how Eric's life and death jumbled in with Ava's life and illness would shake out. How would I separate the grief the pain, the joy the hope. How would each child get their due in my humble mind. I can tell you all I know right now. It is different, because they are different people. I love each of our 5 unconditionally and I love them differently, where they are and for who they are. In sorrow and grief that individualistic love is expressed in a way unique to each child. This is a relief to me as I have been concerned how that would play out.
I KNOW beyond a shadow of a doubt that God can heal our daughter. But I ACCEPT without reservation that that kind of miracle would have to be in accordance with His plan. That type of miracle would not be for the Benton family, although we would benefit immensely, but it would be for His glory and purpose.
As much as I long for that to be in accordance with God's plan, I know that if and when Ava is called into His presence He will be glorified and she will experience the most amazing miracle of healing there is. She will become fully restore and will truly "fly on the wings of eagles." She will truly be able to "run and not grow weary and walk and be faint". I will be so earthly sad when and if that day comes. But I am eternally grateful.
Monday, December 7, 2009
On miracles, college philosophy, and eternal life
So what exactly is a miracle? According to Wikipedia, as if that were the expert on such things, a miracle is a "perceptible interruption of the laws of nature, such that can be attempted to be explained by divine intervention." This seems to fit with the discussion in my college philosophy class approximately 13 years ago: I recall a miracle being defined as God doing something outside of the laws of nature that he created. But if God created the "law", isn't that a miracle in and of itself?
Biblically speaking, miracles are easy to identify. God causes an enormous body of water to divide, allowing his people to walk across on dry land, only to have it collapse on their enemies. Jesus calls to his friend to come out of the tomb, and he comes back to life. Peter and John tell a crippled man to arise and walk, and he not only does that, but leaps and dances, praising God.
So it seems that we tend to have this picture of God "doing stuff", like his "regular routine", I guess, and then, at certain times he does "other stuff" which we deem as miraculous. But lets stop and think about that for a second. Is it "routine" just because He's God? He spoke, and the world came into being. That's miraculous! He breathed into Adam and Eve and gave them not only life, but purpose. That's miraculous! He "thought up" flesh and bone, organs and tissue, genes and cells and mitochondria. If that isn't miraculous, I don't know what is. So then, is it only miraculous when God takes away our sickness, or is it miraculous simply that an egg and a sperm come together and form exactly the person that God intends? Maybe a miracle is any act that God performs that reminds us that He is sovereign.
It's a miracle that Ava is here. Without getting too much into menstrual cycles, ovulation, and conception... it's a miracle, just trust me on that one. Of course we are all praying for a "bigger" miracle. But it is a miracle that she is still with us right now. She should have already died several times. Every day that we wake up and still have her is a miracle.
So, let me bring you up to speed, clinically speaking. Things are not looking good. Ava's lactate today is critically high (14). Her heart rate is up. She has been looking less comfortable to us, and is having almost no awake time lately. Our hearts are heavy. We are basically just waiting for a final "rubber stamp" on the muscle biopsy results, but the preliminary report has us knowing almost with certainty that Ava has a complex IV deficiency, which is exactly what Eric had and leaves no hope, medically speaking, of a positive outcome. We are still waiting for the final results of that test. The biopsy testing for mitochondrial depletion and Electron Transport Chain analysis has not begun as it has not yet been resent with the correct label for testing. We anticipate that it should be sent overnight today.
When Eric was here I prayed hard for a miracle with every fiber of my being, every minute of every day. After he died, and after some time gave perspective to the disease he had, I was left asking if a miracle was even ever possible. It is a genetic defect; it is the way that he was made. But does God still have power over that? Absolutely! After all, God "thought up" mitochondria, so he certainly has the power to fix broken genes and replicate mitochondria, if that is His will.
Of course this is what I want. I know that Ava is going to die- we all are. And God knit her together in the womb, knowing exactly her purpose and the number of days her earthly body would endure. I would sure like for this to be a miracle of "biblical" proportions, in which God demonstrates His power by completely restoring her, fixing her broken genes, and replicating her mitochondria., and so I still pray for that. But the hope that we have is rooted in the knowledge that God has already worked out our eternity ages ago with the most significant miracle in history:
That He, who was God himself, humbled Himself to take on human form, to be born, not as a king, but in a stable; As an adult was ridiculed, mocked, and beaten, though He was without fault; suffered, shed his blood and died a humiliating death on a cross as penalty for sin that was not his own; defeated that death and on the third day rose again, so that by belief in Him we might have eternal life...
...Miraculous.
Peace,
Allan
Biblically speaking, miracles are easy to identify. God causes an enormous body of water to divide, allowing his people to walk across on dry land, only to have it collapse on their enemies. Jesus calls to his friend to come out of the tomb, and he comes back to life. Peter and John tell a crippled man to arise and walk, and he not only does that, but leaps and dances, praising God.
So it seems that we tend to have this picture of God "doing stuff", like his "regular routine", I guess, and then, at certain times he does "other stuff" which we deem as miraculous. But lets stop and think about that for a second. Is it "routine" just because He's God? He spoke, and the world came into being. That's miraculous! He breathed into Adam and Eve and gave them not only life, but purpose. That's miraculous! He "thought up" flesh and bone, organs and tissue, genes and cells and mitochondria. If that isn't miraculous, I don't know what is. So then, is it only miraculous when God takes away our sickness, or is it miraculous simply that an egg and a sperm come together and form exactly the person that God intends? Maybe a miracle is any act that God performs that reminds us that He is sovereign.
It's a miracle that Ava is here. Without getting too much into menstrual cycles, ovulation, and conception... it's a miracle, just trust me on that one. Of course we are all praying for a "bigger" miracle. But it is a miracle that she is still with us right now. She should have already died several times. Every day that we wake up and still have her is a miracle.
So, let me bring you up to speed, clinically speaking. Things are not looking good. Ava's lactate today is critically high (14). Her heart rate is up. She has been looking less comfortable to us, and is having almost no awake time lately. Our hearts are heavy. We are basically just waiting for a final "rubber stamp" on the muscle biopsy results, but the preliminary report has us knowing almost with certainty that Ava has a complex IV deficiency, which is exactly what Eric had and leaves no hope, medically speaking, of a positive outcome. We are still waiting for the final results of that test. The biopsy testing for mitochondrial depletion and Electron Transport Chain analysis has not begun as it has not yet been resent with the correct label for testing. We anticipate that it should be sent overnight today.
When Eric was here I prayed hard for a miracle with every fiber of my being, every minute of every day. After he died, and after some time gave perspective to the disease he had, I was left asking if a miracle was even ever possible. It is a genetic defect; it is the way that he was made. But does God still have power over that? Absolutely! After all, God "thought up" mitochondria, so he certainly has the power to fix broken genes and replicate mitochondria, if that is His will.
Of course this is what I want. I know that Ava is going to die- we all are. And God knit her together in the womb, knowing exactly her purpose and the number of days her earthly body would endure. I would sure like for this to be a miracle of "biblical" proportions, in which God demonstrates His power by completely restoring her, fixing her broken genes, and replicating her mitochondria., and so I still pray for that. But the hope that we have is rooted in the knowledge that God has already worked out our eternity ages ago with the most significant miracle in history:
That He, who was God himself, humbled Himself to take on human form, to be born, not as a king, but in a stable; As an adult was ridiculed, mocked, and beaten, though He was without fault; suffered, shed his blood and died a humiliating death on a cross as penalty for sin that was not his own; defeated that death and on the third day rose again, so that by belief in Him we might have eternal life...
...Miraculous.
Peace,
Allan
Thursday, December 3, 2009
Sounds
Nothing new is going on here at the hospital today. Ava is holding her own. At first she was VERY sleepy and not too responsive. We were feeling a little down. As the day wore on she did become more responsive to our touch and kisses. She is trying to open her eyes and can't quite do it. None the less she is responding.
Today she's been smiling, making pleasant faces. During her massage she actually started to coo. This made the PT and myself cry. I haven't heard her voice since Nov 11. It was such a special gift for us.
We are actually going to sneak out of here early so we can surprise our girls when they come home. Who knows, maybe throw up a few Christmas Decorations.
Today she's been smiling, making pleasant faces. During her massage she actually started to coo. This made the PT and myself cry. I haven't heard her voice since Nov 11. It was such a special gift for us.
We are actually going to sneak out of here early so we can surprise our girls when they come home. Who knows, maybe throw up a few Christmas Decorations.
Prayer Service for Ava
Some friends of ours have organized a prayer/praise/offering service for Ava. It is going to be this Sunday 12/6 at 6:00pm at Hockessin Baptist Church in Hockessin, DE. Put this address in your GPS:
505 Schoolhouse Rd
Hockessin, DE 19707
http://www.hockessinbaptist.org
We hope that many of you will be able to come out.
Thanks!
Allan and Amy
505 Schoolhouse Rd
Hockessin, DE 19707
http://www.hockessinbaptist.org
We hope that many of you will be able to come out.
Thanks!
Allan and Amy
Wednesday, December 2, 2009
How about this one
So, Ava's MRI/MRS results came back. They are normal. However, there is a warning that there were technical difficulties and due to Ava's size they were not able to view Ava's Basal Ganglia. They could only see her frontal lobe. However this was not an issue last week for her MRI. Nobody mentioned such issues to Ava's nurse who was with her the WHOLE time.
The mystery about the muscle sample is that someone at AI in the lab mislabelled the sample with our deceased son's name. So a new sample or that same sample relabelled is going to be resent to Baylor. They did not send the entire specimen so there should be enough to do what they need to do. It doesn't matter, we would not have Ava endure another biopsy.
No results on the CSF (Cerebral Spinal Fluid) studies. Tick tock tick tock.
Ava is content today. Moving a bit more. Not opening her eyes a ton. Soon Allan will be able to hold his baby for the first time since Nov. 10.
Anyone getting a clear message that we are not supposed to know anything right now? Yes we are upset and frustrated. But we have peace. We are (The women who can to follow up is the same one who sits at the front desk and smiles at us every morning. Today I found out that she's been praying for Ava too.) God is cool.
The mystery about the muscle sample is that someone at AI in the lab mislabelled the sample with our deceased son's name. So a new sample or that same sample relabelled is going to be resent to Baylor. They did not send the entire specimen so there should be enough to do what they need to do. It doesn't matter, we would not have Ava endure another biopsy.
No results on the CSF (Cerebral Spinal Fluid) studies. Tick tock tick tock.
Ava is content today. Moving a bit more. Not opening her eyes a ton. Soon Allan will be able to hold his baby for the first time since Nov. 10.
Anyone getting a clear message that we are not supposed to know anything right now? Yes we are upset and frustrated. But we have peace. We are (The women who can to follow up is the same one who sits at the front desk and smiles at us every morning. Today I found out that she's been praying for Ava too.) God is cool.
Tuesday, December 1, 2009
Frustration, doesn't even explain it
Today was a fairly relaxed day. Ava was moving a little tiny bit more than she has been. She was not really that awake, but would still try and respond to us when we kissed her. She was a little uncomfortable and would cry. We think that she had some gas. She needed her tubes to be re taped as well. She was all done with the futzing around. Then she had a little PT/a massage. Ava really enjoys her hands and fingers to be massaged the most. She really just speaks with her eyebrows even with her eyes closed.
She was still on IV fluids and getting fed as well. The feeds were stopped for her MRI, so they were keeping her on IV fluid for that. She should just be on feeds by tomorrow. Ava's lactate is down and she will stay on the BiCarb for now. Ava's chest x-ray yesterday showed significant improvement in regard to her pneumonia. This mornings x-ray showed some more improvement. There is still some concern in her right upper lobe.
Allan and I enjoyed a nice, quiet day at Ava's bedside, waiting or her MRI. This was scheduled for 4pm. They actually took her early. We won't know the results until at least tomorrow. While Ava was at her MRI the Attending came to speak with us. We are sad that Glenda the Good doctor is going off service, but hope that the new Attending will continue to advocate for Ava.
One of the things that we learned tonight was in reference to Ava's muscle biopsy. This is a complicated story but for all intents and purposes the bottom line is that AI's lab mislabeled Ava's sample with Eric's name and requested a gene test for the sample. It was not run and the sample is at the Baylor lab. There is some craziness having to do with needing to send the sample back to AI. Then the sample would have to be resent to Baylor. We don't know why that would have to happen. We don't know if the sample would still be intact. We certainly don't know who screwed up and why. What we do know is that the studies that should have been completed by next week, haven't even been started yet.
To sum up, what still is pending for Ava? Urine Organic Acid studies, CSF studies, and three studies for her muscle biopsy. For Eric's DNA we are waiting on his POLG1 sequencing to see if there is a mutation there. (We don't expect to see that, but we privately paid for it and they should complete the study. We've been told for WEEKS that it would be done by Friday). I am beginning to feel like waiting for these results is like waiting for Godot. (He just never comes).
The hope is that all of the pending information would become useful in deciding what would be in Ava's best interest. One thing we know for sure is that Ava is responding to the treatment that she is being given and she is responding as well as can be expected. She is also responding faster than the doctor could have anticipated. This is a testimony to all of the prayer that has been said on Ava's behalf. PLEASE DON'T STOP. Let's ask God for a miracle of healing, for the atalectisis in Ava's lungs to clear up and STAY AWAY, for Ava to become strong and improve in her tone.
We also need prayer. We need to make a decision for our daughter's life with seemingly little to no useful information back. We are at the cusp of life and death and we are asking for wisdom in making choices for Ava. We are asking that God make it abundantly clear as to what HIS plan is for our daughter. We don't want to make a choice for us, but for Ava. This must be in line with the will of God.
One notion that has been present in my mind is that we don't need anything except God. We are living a life that is not ours. We have no control of the life we have. We aren't primarily caring for our home, our children, our jobs are on hold. Yet every need we have seen or unforeseen is being met. The "answers" we are looking for are lost, out, delayed. No doctor actually knows what the whole story is. Ava's Geneticist and her assistant are confused. They are talking to top metabolic doctors across the country and they have not much to weigh in with. When Ava did not do well on her hearing test I told Allan that there is no one who will be able to help us. Right now it sure feels that way. The resounding thought is that God is telling me that all we need is Him. We have to trust Him. So we do. So we will. We will wait upon the Lord. We can not make a decision until He reveals to us His plan for our beautiful baby's life. She, like all of our children, are on loan to us. We are here on this earth to do the work of God's will for us. No matter how scary or inconvenient a path we walk. If we walk it with God as our guide it is right. We are learning, daily, hourly to walk by faith and not by sight.
She was still on IV fluids and getting fed as well. The feeds were stopped for her MRI, so they were keeping her on IV fluid for that. She should just be on feeds by tomorrow. Ava's lactate is down and she will stay on the BiCarb for now. Ava's chest x-ray yesterday showed significant improvement in regard to her pneumonia. This mornings x-ray showed some more improvement. There is still some concern in her right upper lobe.
Allan and I enjoyed a nice, quiet day at Ava's bedside, waiting or her MRI. This was scheduled for 4pm. They actually took her early. We won't know the results until at least tomorrow. While Ava was at her MRI the Attending came to speak with us. We are sad that Glenda the Good doctor is going off service, but hope that the new Attending will continue to advocate for Ava.
One of the things that we learned tonight was in reference to Ava's muscle biopsy. This is a complicated story but for all intents and purposes the bottom line is that AI's lab mislabeled Ava's sample with Eric's name and requested a gene test for the sample. It was not run and the sample is at the Baylor lab. There is some craziness having to do with needing to send the sample back to AI. Then the sample would have to be resent to Baylor. We don't know why that would have to happen. We don't know if the sample would still be intact. We certainly don't know who screwed up and why. What we do know is that the studies that should have been completed by next week, haven't even been started yet.
To sum up, what still is pending for Ava? Urine Organic Acid studies, CSF studies, and three studies for her muscle biopsy. For Eric's DNA we are waiting on his POLG1 sequencing to see if there is a mutation there. (We don't expect to see that, but we privately paid for it and they should complete the study. We've been told for WEEKS that it would be done by Friday). I am beginning to feel like waiting for these results is like waiting for Godot. (He just never comes).
The hope is that all of the pending information would become useful in deciding what would be in Ava's best interest. One thing we know for sure is that Ava is responding to the treatment that she is being given and she is responding as well as can be expected. She is also responding faster than the doctor could have anticipated. This is a testimony to all of the prayer that has been said on Ava's behalf. PLEASE DON'T STOP. Let's ask God for a miracle of healing, for the atalectisis in Ava's lungs to clear up and STAY AWAY, for Ava to become strong and improve in her tone.
We also need prayer. We need to make a decision for our daughter's life with seemingly little to no useful information back. We are at the cusp of life and death and we are asking for wisdom in making choices for Ava. We are asking that God make it abundantly clear as to what HIS plan is for our daughter. We don't want to make a choice for us, but for Ava. This must be in line with the will of God.
One notion that has been present in my mind is that we don't need anything except God. We are living a life that is not ours. We have no control of the life we have. We aren't primarily caring for our home, our children, our jobs are on hold. Yet every need we have seen or unforeseen is being met. The "answers" we are looking for are lost, out, delayed. No doctor actually knows what the whole story is. Ava's Geneticist and her assistant are confused. They are talking to top metabolic doctors across the country and they have not much to weigh in with. When Ava did not do well on her hearing test I told Allan that there is no one who will be able to help us. Right now it sure feels that way. The resounding thought is that God is telling me that all we need is Him. We have to trust Him. So we do. So we will. We will wait upon the Lord. We can not make a decision until He reveals to us His plan for our beautiful baby's life. She, like all of our children, are on loan to us. We are here on this earth to do the work of God's will for us. No matter how scary or inconvenient a path we walk. If we walk it with God as our guide it is right. We are learning, daily, hourly to walk by faith and not by sight.
Monday, November 30, 2009
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.
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.
Sunday, November 29, 2009
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...
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.
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, November 28, 2009
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?
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?
Friday, November 27, 2009
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.
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.
Tuesday, November 24, 2009
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
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
Monday, November 23, 2009
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.
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.
Sunday, November 22, 2009
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).
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.
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.
Saturday, November 21, 2009
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 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.
Friday, November 20, 2009
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."
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."
Thursday, November 19, 2009
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.
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