Thursday, December 31, 2009

New Year

When life is so chaotic, I like to organize. Today my closet is the way I love it, weeded out and size and color coded. Refolded too. Allan is on his way to drop off the last minute donations to Good Will. Somehow this process helps, but makes the day hard too.

I find that I am suddenly remembering things about grieving that I've forgotten about. I wish I didn't have to remember it at all. I wish I didn't know what this type of grief is all about. I hate finding surprises that were tucked away. Clothes that still smell just right, a pacifier, a favorite outfit. It always happens. I feel like it's good to get it out of the way. The problem is that it's never really out of the way. There will always be little reminders that catch you off guard. A smell, a band aid, the way the day smells.

As sad and hard as it is, maybe it's a gift from God. A way for Him to remind us that our loved ones are still with us even if they are no longer tangible. You see the only guarantee that we are given in life is that we will all die. All the rest of life in between is up to us. We can choose to live on our own accord or we can choose to live according to God's will for our lives. It's hard to relinquish control and hand over the reins. You never know what you are going to wind up doing.

Honestly though, when you try to take over those reins, do you really wind up knowing what you are going to do? The main difference is, if you walk without God, there is no hope. Hope comes from the promise of eternal life. God sacrificed HIS own child for US. He did it so that we could have ever-lasting life. God knows how much it hurts to loose a child. He did not have to do that at all. But He did so that we could know what length He would go to for us.

See, even though we loose our loved ones and we are sad, there is SO much more to live for. If we confess that we are sinful (we do wrong things, as the bugs say) and believe that Jesus died for that sin of ours and we are truly reticent we, too, may have life ever lasting. A chance to spend eternity with God. There is no pain, sorrow or illness.

I'll tell you, walking through grief is so very difficult. Doing it without the One who loves you the most is next to impossible. I honestly don't care what the sin is. God forgives it, if you ask Him to. He just does. It seems simple, but you have to be willing to relinquish the control you think you have over your life. That's hard for us. It's hard to be humble and to submit. I will tell you first hand, it makes my life so much easier. When the weight of my sorrow crashes down around me and the buggie-boos and fighting and demanding my attention, He is there to pick me up and carry me through that wave. I'm riding with the tide, not getting caught in the undertow.

Believe me, there are times that it seems like I will get caught in that undertow. It seems so dark and there is no way out of the sharp pain and the dull long term ache that follows. But He is there to pull me out and show me what is important. He gives me the scent that reminds me of my loved one. I find a letter, a hat, a golf ball from my Grandpa. I find a pacifier. I look in Eric's memory box. I see a frog. I see someone who looks "familiar". I hear my Grandmother's voice in my head. I get sand on my towel. These are all cherished things from the people who have impacted my life and I love. People I know that I will see in Heaven. People who have already completed their journey through this world, impacted me, and many others, for Christ and are in the presence of the living God. This is why I have Hope. This is how I can face tomorrow. Whether it's a new year or not, your life can only be new if you give it over to God.

Wednesday, December 30, 2009


I've been thinking about all the babies in my life. We are in the season of life where we are all building our families. Eric was born in the "first round" of babies amongst our group of friends. After he died, I tried to be as involved as I could, but it hurt so much to have empty arms and watch everyone else with their babies. The problem is that I love those kids. They are a part of our extended family. I forced myself to be as involved as I could. But it was really hard. I missed out a lot.

Ava also has many friends born about the same time as she. These kids I also love. This time my house is full, but very empty at the same time. A big difference is that I am still a Mommy with a mission. After Eric I was a Mommy without one. I like having a mission still. It is just as hard to see those other babies, but in a different way. I want to be a part of their lives. Ava was made for the life she lived. These children are still unfolding into the wonders of God's promises and plans for them. I do want to hold them and love them. So I am trying to do that now so they don't become taboo. I'll tell you, it's not so bad.

One thing I pray for is protection over the children of our friends and family. I pray that no others have to walk this road that we are on. I know that is so not possible. Other children will die. But I ask God to keep families in tact. I don't see that there is a problem with that. I also know that we can support those who may have to walk that road someday. We are happy to do that too. It's not a fun club to be in, but it's our life.

I so wish that our babies had a problem that modern medicine could touch and help with. I wish there was at least some hope medicine could provide that the form of mitochondrial disease our children suffered from could be helped. That is not the case. I don't know if it ever would be the case, there is much more research that has to take place first.

I go to bed and not know what the next day will bring. Sometimes it's more tough than others, sometimes it's a hair easier. The sadness and loss we feel ripples through our family at different times. It's unpredictable and sometimes feels like the wind was knocked out of you. I am noticing that God is making sure one out of the 5 of us is a little more up to help those that are a little more down.

The ache will dull. The pain will not be as poignant. There will always be a hole though and that's OK. I wish I could fast forward this road. It took 5 years before I started to feel lots like me again after Eric. It's not fun knowing what you are in for. Then again, I don't really. Ava's life and death is and was way different than that of her brother. The aftermath of our grief will follow suit, I suppose. It already is. It doesn't make it hurt less, just different.

Monday, December 28, 2009

Family time

Today was a much needed good day after the tough one we had yesterday. The bugs flitted around the house in leotards and tutu's having "ballerina" lessons at 20 o'clock. Oh wait, they changed it to 5:30. There were couch cushions all over the place. Blanket piles and some strange chant that Allan and I couldn't figure out what was being said.

It was nice.

We had movie night and game night. It's amazing how one pizza served in the family room on a kid table with paper plates in front of the same mind numbing children's show (Strawberry Shortcake) can turn into a major event when you are 5, 5, and 2. If we ate it in the kitchen, where's the fun in that?

We spent the majority of the day trying to figure out the best way to approach Disney. They try to tell you that it's affordable. I'd like to know by whom. I heard there were lots of deals out there. Just not really now. We did find some good stuff, eventually. So I think we are going to try and go. There are still kinks to work out. (I am holding out for a better reduction in cost.)

While we were in the throes of research Elaina bounces up to us and wants us to come "look I did". "Hold on Honey, just a minute." "Daddy, LOOK I did." "Hold on honey Mommy and I are doing something." "DADDY, LOOK I DID." "Hold on Honey, Mommy and I are doing something, that involves you being able to meet Mickey Mouse." "Oh." Off she went.

It was hysterical.

It feels good to laugh, even when it's outside of yourself.

Sunday, December 27, 2009

Onions and J-O-Y

Trying to get back in the swing of things is hard. I think about how much more difficult it will become as we absorb the responsibilities of our "usual" life. I am exhausted and have a low threshold for things that bother me. Poor Allan, it's like he is dealing with pregnant me. He was hoping to not have to do that I suppose. I want the broken or the breaking things in our house fixed NOW. If it's annoying me, I want it taken care of. I find I just don't have the extra energy to expend on that "fluff". (Drafty windows and a new roof isn't "fluff"', but kids stuff everywhere, a broken phone, a not functional storm door, and clothes that don't fit right could be considered as such).

The little reserve I have is to go into the ladybugs flitting around the house. They will usually stop for a hug and an 'I miss Ava'. Their regular conversation these days consists of: 'Look at the picture I'm coloring. I'm coloring in the lines. We used to have 5 kids and now we are back to 3. These are yummy mashed potatoes.' Sometimes I can deal with it OK. Today it's like little daggers in my heart.

I always wanted to have four kids. I've given birth to 5 wonderful little bugs, but I so wish that I had the opportunity to raise them all. When we found out we were expecting Ava to say we were surprised would be an understatement. We were thrown for a loop thinking that our family was complete. I went through a lot of processing to come to terms with having another one right now. When I was younger I wanted 4, when I came to terms with the surprise, I still wanted 4. I really think that we would have done a great job that the craziness of 4 would provide. I hate that I will never know now.

It's those thoughts and feelings that are piercing and raw. Every time I peel a layer of that onion my eyes sting and burn as I get closer to the heart of the matter. I can't look at the whole thing all at once. I think I'd blow a gasket. I keep saying that I can't do it anymore and how much more can I take. Yet here I sit, still doing what I feared the most and still taking it. Sometimes way better than others, none the less, taking it.

Allan and I were noticing that this time around we feel way more present in life than we did when Eric died. That is a blessing. We have to be. We have three active, wounded, and still happy ladies that we have to care for and nurture.

My soul is nurtured by God. I need someone to nurture my physical being. My body is sore, my mind is dull and my heart is so very broken. It is amazing to me how a simple (tongue in cheek) task, like taking 3 kids to church, and eating lunch can take all I have out of me.

I find that I am trying to rush each day to get to the next. The next holds the same loneliness and sorrow, but as the song says, "His joy comes in the morning". I keep looking for the glimmers of that joy so I can hold on and get to the next step. Sometimes it's so very intentional. We have a huge JOY sign in red on our front lawn. In the center of the "O" is Mary, Joseph and Jesus in white. Whenever I leave or come home it's there, in my face. J-O-Y and why we can have it. Sometimes it has to be blatant to be effective.

Friday, December 25, 2009

Tucking in

Tonight Elaina ca me bounding in to Emily and Alexa's room at bedtime. "Mommy, you tuck Emmy and Lexa in and you come and give me a kiss and I tell you a story, OK?." This is what she does every night. It's how I can see how she's processing what is going on in our home. It's her way of stalling before bedtime. Everybody wins.

During one of these "story times" Elaina was able to ask questions about Ava's burial service and why she was "in a box". I could hardly believe how much of the day her little 2 year old brain understood what happened that day.

Tonight our conversation/story was this:

E- "Mommy, why's Ava in Heaven?"
E- "She got a new body".
M- "What's she doing with it?"
E- "She holding God's Hand."
E- "Why Eric in Heaven?"
M- "Why is Eric in Heaven?"
E- "He Died."
E- "He got a new body."
M- "What's he doing?"
E- "He holding God."

To think about Ava holding God's hand brings me comfort. To think about God holding us brings me Joy.

Ho Ho Ho

Merry Christmas! It is a great day to reflect not on all we've lost, but what we've gained and have. The joy that I usually feel on Christmas is dulled, but I know it's there. It's reflected in the twinkle in the eyes of my three beautiful girls. They are full of hope and peace.

We gave them each a photo book of each bug with Ava. I took special pictures of them with her from the time she was born so they would always have them, in case she died. I am so very grateful that I made a point to do that instead of bury my head in denial of the very distinct possibility. This morning each of our bugs appreciated this book as much as all the other "kid" stuff they received. (Thank you to a whole bunch of elves disguised as Social Workers, Case Managers, Performance Improvement and some nurses from my work.)

As I type Allan is playing with one of his toys from those same elves: a remote control truck. It was so fun to hear him exclaim, "yeah!".

Our plans for the new year are lofty, but hopefully attainable. There will be a 5K for Ava in the spring. We are going to move full steam ahead on getting the Joy-Hope Foundation off the ground. I will continue to make prayer bracelets (with the help of Emmy and Lexa). They can't wait to help. We really need to potty train the headstrong two year old who is more than ready. We also need to regroup as a family and individuals and try to make sense of what just happened to us. We need to grieve and do so courageously. Allan and I may take a weekend away. We are also hoping to go to Disney so we can see the sparkle in those six gorgeous brown eyes. Who knows, maybe I will have a little glimmer in there too.

During the season of grief there are always abundant lessons and truths. They form in the darkness and shoot forth a ray of light for that moment. If you keep an eye out if can become a lifeline, so to speak. God throws us the proverbial rope so that we don't slip into a deep depression. A pit that is seemingly impossible to climb out of. Each situation that causes grief has it's own lessons to assist in the season of life that you are in.

My lesson that I have identified in these early minutes, before the reality of the loss sets in, is that I need to live by faith, but intentionally at the same time. It's crazy. I never thought those two things could go hand in hand, but they do. God has been providing for our every need, tenfold, during Ava's brief and bold life. Now, He is doing so even more in the aftermath of her death. I know He will take care of all the details. But I have to make a list of the things that I need to do so that I can get them done. He is directing the course we are on and we want to obey. The story of Eric and Ava is not over, God is telling us that. Their lives are complete on this earth, but there is more work to be done.

This is a tricky thing for me. I don't ever want to be holding onto them because I can't let them go. If I did that I would get stuck in my grief. Time marches on and so must we. But there are things that we are feeling called to do, to assist those families who will have to walk a similar path that we are.

The list for the moment is as follows: Watch Allan play with his remote control monster truck and launch it over the ramp and pillow he set up. Cook the "roast beast" (standing rib roast). Delight in my children and my husband. Miss my babies. Play with my Mom and Dad. Find a reason to use my new griddle. Rest in Jesus.

Wednesday, December 23, 2009

Hustle and Bustle

Yesterday was Ava's burial and her Memorial service. It was an emotionally exhausting, yet beautiful, day. We are hard pressed to find the appropriate words to sufficiently thank all who have supported our family. The outpouring is humbling and overwhelming. The community around us is helping us keep it together, so to speak. We, of course, recognize that God is holding us together too. But He is working through all of you, and we recognize that too.

Today we try to live in the shadow of our sorrow and the sunshine of the Birth of Christ. (We are also trying to deal with the continual hacking cough and sleepless nights as a result said hacking coughs. Which is getting really old, I might add). So today it was off to the mall with the three bugs. Not the best judgement on our part, but sisters had to buy presents for each other. We tried to tell them that there are no more presents, but they weren't falling for it.

We plan on doing our best to have a good time on Christmas. There will be tears. There will lots of hugs and laughter too. We can do that. So we will.

Monday, December 21, 2009

Settling in

Alexa is sick...again. She was up off and on last night coughing and with an asthma attack. We are physically and emotionally drained and are struggling to find time to cry.

Sometimes we don't need to cry, we need to laugh and enjoy what is before us. Sometimes we just need to attack the task list. Which tends to be long, trying to "get ready for" Christmas and Ava's Memorial service all at the same time. There is hope in our despair. There truly is.

Sometimes I feel like I could just collapse from exhaustion. Then the doorbell rings, the mail comes, the phone rings. Whatever is at the other end/side or in the box is just what we needed to keep on moving.

I would say that my sorrow is so profound that I can't put words to it or experience it fully. It will come in time. At the same time we march along and try to regain "normalcy" a fraction at a time.

Saturday, December 19, 2009


Below is the text that will appear in the obituary of our local newspaper on Monday:

Ava Elisabeth Benton, age 4 months, went home to be with the Lord on December 18, 2009. This precious child is survived by her parents, Allan and Amy Benton, and her sisters: Emily, Alexa, and Elaina. Ava is also survived by her maternal grandparents, Pat and Bruce Willerup and her paternal grandparents, Duane and Carolyn Benton. She is predeceased by her brother, Eric William, who was afflicted with the same mitochondrial disease. Family and Friends are invited to attend a memorial service on Tuesday December 22, 2009 at 11:00am at Brandywine Valley Baptist Church, 7 Mount Lebanon Rd. Wilmington, DE 197803. Burial will be private. In lieu of flowers, donations may be made to the "Ava Benton Medical Fund" attn: Linda S Dilorio Wilmington Trust Bank 151 Lantana Dr. Hockessin, De 19707. To send an online condolence visit

Rebuilding one block at a time

I hardly know where to begin. I am so very proud of all my 4 ladybugs. Of Ava and her determination, courage and will power to fight that which she could not overcome. Of Elaina and the twinkle in her eye, her sense of humor and her concrete thought. Of Emily and the joy in her eyes as she reveals in the promises of heaven and anticipation of her life, everlasting. And of Alexa who just asks questions, tells us how she feels and how she does everything in her power to give the love we need and get the hugs she needs.

Today we woke up, got dressed, had breakfast, opened some presents early (we're sad so we decided we get to), played with aforementioned presents, played in the snow with good friends, had hot coco, enjoyed the company of lots more good friends, cleaned the biggest bugs room, cried, read the notes on facebook and the blog, got the ball rolling for burial and memorial service arrangements, laughed, squibbled, ate a bad dinner (apparently, I am a little out of practice to which Emmy said, "It's OK Mommy it's been a LONG time since you cooked."), laughed at that, answered tough questions, gave baths, did Christmas countdown, prayed, tucked in some pretty special bugs, answered more tough questions, made a list and checked it more than twice (especially since we can't seem to remember what we need to do from one minute to the next), caught up on email, facebook and the blog comments, cried some and now, write.

The girls got a little sadder as the day wore on and the reality set in. We talked about how we had a happy life before Ava was born and during her time with us here on earth. We talked about how Eric was here and gone before they were born and that they still have a happy life. We then talked about how I know that we can have a happy life again. We talked about how's it's OK to be sad, that it takes a long time to sort this type of sadness out and grownups don't even understand it, really. But we will do it together. We recounted the day over the under cooked chicken and crunchy rice casserole (I stirred it and it moved well, I really am a good cook). We remembered that today was a very sad day but we all had fun, we all laughed, and we all enjoyed parts of the day. So we can do it. We talked about how we need to feel our feelings and keep on going. (Poor kids have a social worker for a Mommy, they have no chance).

We honestly rejoice in the honor to know, love and have Ava in our family. We know that this was what God created her for and this is her life story. It was so short (she lived 5 days longer than Eric, so go sister), but it was mighty. It's not lost on me the words used to describe our littlest bug: fighter, mighty, strong, courageous. All of these things take energy, the very thing her body lacked the ability to produce to keep her here with us. Through that frail, broken (and long) body was a baby who was filled with character and drive. I would often joke in the PICU about what she would be like if her mitochondria were not broken and didn't have crystallization on it's tubulars (FYI that's not good even if it sounds pretty).

God does not do anything without intention. He does not make mistakes. His perfect plan will always be revealed. As I wrote on Facebook right after we found out that Ava was deaf: God is sovereign, even if we don't agree. Our sweet baby girl will leave a poignant hole in our hearts. It's one I will cherish, just as I do that of her brother's. Never as a child would I have dreamed that "this" is how my life would be. But it is. It's wild and unpredictable, but it's guided by the One who I put my trust in. God has never failed us or left our side. We can be angry and confused. He has been here the whole way through. He planned Ava's entire life right through her dying breath. He was in that room with us last night saying to our precious, precious fighter, "Well done my good and faithful servant."

Friday, December 18, 2009


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

Holiday Cheer

I miss Ava today. We only spent a short time at the hospital so that we could spend more time with the bigger bugs today. I do have to admit that it felt nice to feel a little normal. We did some pre-Christmas toy sort. We also enjoyed a family Christmas dinner tradition with some relatives on Allan's side of the family.

For a few minutes I felt like darkness was not lurking around the corner and I enjoyed our time. When it was time for picture taking I was catapulted into reality and my arms ached at how empty they really are. I try so hard to not think of "how it could be" and focus on how it is. The way I try to do this is to remember that Ava was not born to be able to act like how a baby is "supposed" to be. Somehow that can numb some of the pain. This time it's easier in that I have three beautiful girls to hold in my arms. With Eric those same arms were suddenly empty.

Ava had a good day today. She really didn't seem to loose any of her skills from the prior days. I don't think she really built upon them either. I didn't get a chance to hold her, we weren't there long enough. I hope to have a chance to tomorrow.

I spoke with our genetic counselor at CHOP. It seems Baylor did receive the muscle biopsy sample on December 10th. The counselor was able to get the head of the lab to commit to expediting the testing. Hopefully there will be information by the end of the year. Seriously, I don't know how that's expediting anything as that will be about 3 weeks from the time the sample was received. Three weeks is the quote they give on how long these studies take. At this point it would be nice to know the information and if there are any ideas on ways to help Ava. We know Ava has the same disease Eric did the biopsy results on these tests will just provide more information.

We are tired and are wondering what we could tap into to find energy. I could fall asleep standing up, literally. Actually I fell asleep while trying to change sheets on the big bugs bed. Yet somehow God gives us the strength and energy to plod along on this crazy road we are on. We continue to trust He will lead the way.


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.

Wednesday, December 16, 2009

A Hard Day

I'm sitting by Ava's bedside for what seems the 10,000th hour. I am waiting and I don't know what for. But I do it.

I want to be home with my babies and not leave them. I don't want to hit and run parent them. I want to hold them in my arms and never let go. I wish I could always hold onto Eric and now Ava too.

I honestly don't know why Ava clings to life with such determination. I wonder if I need to learn to embrace the life I've been given in such a way. The life that God gives us is precious there is so much to learn from two little 4 month olds.

Tuesday, December 15, 2009

A good day

This morning Allan and I want to the biggest bugs pre-school Christmas Program. (I mistakenly called it a concert once to which Emmy corrected, "Mommy. It's not a concert. It's a PRO-GRAM). It totally warmed my heart seeing the girls sing songs about the birth of Jesus. They were so excited. It was awesome to see all their friends and all they worked so hard to do.

After the Program we were off to see littlest Bug. We really never know what we are going to walk into. We did our usual check of her "numbers" on all the monitors, scrub and kiss the little miss. We were surprised to see that Ava looked way more comfortable than she did last night. Her heart rate was well within normal today. ALL DAY. I then grabbed her hand. She squeezed mine and pulled on it. She had Physical Therapy and her therapist had tears in her eyes as Ava was responding to her touch, moving more and allowing her to do way more massage and range of motion than she's ever tolerated before. She finished by saying, "Ava's stronger today."

We are not reading much into this but for confusion. Yesterday we expected that Ava would be even weaker today than she was yesterday and her time with us would be coming to an end soon. Clearly today was not that day. Ava has more to accomplish. We truly just enjoyed her and enjoyed being amazed by her character, tenacity, and determination. She truly is a special little bug whom we are honored to love and know.

Of course we don't know what we will encounter tomorrow, but we will relish the gifts from today: Em and Lex signing, extra cuddle time with Elaina, and extra finger squeezes from Ava.


Emily is now the one who doesn't feel very well. It's just this awful cough. Not usually a big deal. It happens once or twice a year. We are not handling it well though. It's unfair to Emily who happens to be the last to fall in a long line of ill children. It's so overwhelming. Really it's just an asthma cough that needs extra attention. It's the extra and the attention that we are fresh out of.

Everyday Ava keeps trying. Everyday she is a fraction weaker. We are feeling like she is in more pain so we are wondering if we need to look at adding other medicines. The thing is that her pain is a result of her body entering into a metabolic crisis, so there doesn't seem to be much that is known to help her and lower her heart rate. I've noticed that the increased dose of bi citrate is easing her heart rate some as it was helping to even out her Ph level. This is way off, but the medicine helps some.

I am trying my best to get the hospital to listen to the need for a protocol for children with or suspected Mitochondrial Disease. This facility has a gap in care. There is no specialist on staff. The doctors are left to flounder about when a patient arrives in their PICU. Most just kind of throw their hands in the air, some try to get the patients transferred to CHOP. However, sometimes a transfer is not warranted. I often think about the decision to not have had Ava transferred. But I then realize she did not need to be put through that in her condition, AI really has all the same tools to care for Ava that CHOP does and we needed to be closer to our family and support system.

So, being true to my profession as a hospital social worker, I am looking for good to come out of bad in whatever tangible way I can. I've decided to lobby for change at AI. I have been talking with the Attendings in the PICU, the nurse Manager of the PICU (who I tried to connect with a Neurologist and a few nurses that I think have the right skill set), the Medical Director of the hospital all in an effort to establish a plan for these children that are unpredictable and as fragile as a flower.

The quandary is that these kids require intensive care medicine with a palliative care focus. This is a whole new model to incorporate into daily practice. While the Medical Director is looking into developing a program, which I would LOVE to see. It will take time and dollars. So I am also suggesting quick and easy ways to bridge the gap so that other children are not pushed over the metabolic edge is at all possible.

The trick is that these kids are so very tricky. There is so much research to be done and so much ground to cover and understand. This disease is known about in the broad sense in the medical world, but is widely lumped together with the wrong "strain" so to speak. It is also not really understood by the most brilliant minds out there. Some of the problem is that this disease is at the fundamentals of creation. God made us. No two of us are alike (Even identical twins, though strangely similar are VERY different). We have variations in genes so we can be different. We are all made in God's image and He likes to mix things up. Another problem is lack of awareness about a very confusing group of diseases.

Allan and I are wanting to start a foundation. It's name will be the Joy Hope foundation. We have some ideas of what it will encompass and we hope that we won't drop the ball in the middle of our grief so that we can do some good for others afflicted with this disease. We want to raise awareness about this disease. Sometimes it hits fast and sometimes slower, but it's ugly and aggressive. Another thing we want to advocate for is research into pathways that will ease the affects of disease. This may be short term, or even in education on how to teach medical professionals how to deal with and help with end of life issues for these patients.

It is awesome to have a focus and I hope I don't loose it in the middle of my grief. Allan and I feel like we are being called to this path right now.

Tomorrow (well later today actually) we look forward to the biggest bugs preschool Christmas program. It's going to be awesome and we can't wait to see the fruits of all the practicing. Then it's back to the hospital to see little Ava and hold her, kiss her and pray that God tell us clearly when it's her time. The girls know that in Heaven we will get a new body and that Ava's new body won't have and "broken parts". I would love to have her with me, but in her current sate, she would benefit most from having a new body and being totally healed. Are are waiting on the Lord.

Sunday, December 13, 2009

I think I'm finding the end of my rope

I am going to be completely honest. I've had it. My body and my soul are spent. We have not slept the night through in at least a week. The bigger bugs have been coughing and gagging for what seems like forever now. I feel shaky and like I'm getting the "cough". Every time we put them down to sleep someone is coughing to throw up the whole night through. It's heart breaking. The sad part is that Allan and I don't have the energy to handle it.

The questions the bugs are asking are mind blowing and gut wrenching. I fight to find patience and compassion as I try to manage their sadness and help them understand that which we cannot. My soul hurts for Alexa who asked why Ava had to go to the hospital on her birthday. My soul cringes every time Elaina coughs and wakes up crying. My souls weeps when I look into Emily's eyes, full of saddens and hope. My soul aches when I look at that all too familiar far away look on Allan's face. My soul screams when I see my helpless daughter laying in her bed, still trying with all she can muster. I sometimes feel like I, too, will crumble just like Ava and Eric's metabolism.

The thing I dread the most is the sadness that comes when your child dies. With Eric it was better in this regard as I didn't know what I was in for. With Ava we have experience. We know the depths of that pain. We survived it. With God we can again. But to muster up that amount of courage again. Courage to feel that pain and allow it to flow through us so that it doesn't get trapped. Courage to let go of holding onto someone who isn't here with us anymore. Courage to go to the place that is so dark and scary that you don't think you can come back up for air. The courage it takes can only be given from God. The thing is that if I have the courage to fully grieve Ava, then I won't get stuck in the process. I know that. I did that with Eric. I was dedicated to it. This time I just don't know if I will have the strength to muster up the necessary courage.

After Ave becomes completely healed we have to come home to a house full of treasures. We have to reign in the bugs and take control of our family again. We have to figure out how to grocery shop, get the kids to school, do laundry, cook, pay bills, work so we can pay bills. All the mundane that after Eric died we had the luxury of taking our time with and slowly adding responsibility in.

Now we have to manage the mundane with the emotions of 3 very hurt little girls. We have to manage that too. This is such a long road. It's windy and will be intense. I can already feel myself becoming annoyed with the dumbest of things. Right now I'm on the fence between feeling sad about what is going to happen and knowing how much more intense that sadness becomes.

If the truth be told I think I am the most upset with the fact that I have to be THAT sad again. I was commenting while I was newly pregnant with Ava that I finally, after 5 years, started to feel "normal" again. I took a deep breath to relish the feeling. I am not happy at all with the prospect of what is to come.

Ava is still with us on this earth and I got a small taste of the future. Before updating the Blog I was looking into how to approach planning a trip to Disney. I was registering for a planning DVD and had to fill out the years of birth of the children who would be travelling. There were 4 slots and I only got to fill out 3. It plain stunk.

On the flip side, when I talk with Emily about Heaven and how she will be reunited with her sister and her brother her eyes sparkle. She is looking toward the promises of God to get through her days. She can so beautifully express how she is feeling. While visiting her baby sister I told her that we don't know when God will decide to call Ava to Heaven, but I thought it would be soon. She told me, "I want it to be long". I told her that we all do and wish that could be true.
I then showed her how Ava can't really control her body. Emmy grabbed her sister's finger and Ava squeezed Emmy's. She then tried to open her eye for Emmy. (Isn't like kids to always do the opposite of what you are talking about?)

At bedtime Emmy told me that she wished Ava would be here long and that we all do, but we think it will be short. I asked what will happen to Ava when she 'goes to God'. Alexa said she'll get a new body. I told the girls that when this happens Ava will not be broken anymore. Emmy said, "But we thought it was just her ears". I told her that we found out that she has more broken in her body, like Eric. I reminded them that Ava's body can't make enough energy for her to control her body. ('Mommy we can control our bodies'). I asked them that what would be better for Ava. They both said that she would be better off in Heaven with God, with a new body, and playing with Eric. Emmy wanted to know if Ava would get her new body and be healed as soon as she met God. You should have seen the look of relief when she found out that Ava would be completely healed!

That sweet girl, she is so sad, but wants the best for her baby. The biggest bugs and I talked about how it's OK to be sad and that God KNOWS how very sad we are. I promised them that He will help us and we will help each other.

Saturday, December 12, 2009


Today has been more difficult than yesterday was. Ava can't seem to get comfortable. Her heart rate is just so high. We've been trying pain medication, repositioning, massage. She just can't settle in. I think it's her crumbling metabolic system. Unfortunately, there is no medication that can counter act that.

Ava and Eric have a disease that is little known by the general population. What's known in the medical world is minimal and broad. It's vague at best. There is a handful of brilliant minds that are thinking all the time on how to identify all the inner working of the mitochondria and how to ease the disease process. But to date there is nothing to speak of. It is just so complex and individualistic. It depends on so many factors known and unknown. Mitochondrial disease research is at the forefront of medicine.

I can't even describe how sad it is that there hasn't been much progress in the 6 years that have passed since Eric was with us on this earth. While I have always hoped that Ava was not afflicted with the same terminal illness, I feared it was true. I hoped that she could become stabilized. I hoped that because of Eric there would be more management, more time, more hope.

While we will never understand fully the "whys", I will tell you what I do understand. God created Ava and Eric. He made them imperfect in His perfect plan. They were created for this purpose. They were given to us so that we could have the honor of loving them, knowing them and holding them. Ava gets the extra special treat of being loved by three adoring sisters who couldn't love her any more if they tried.

We feel like we are moving into a new phase: Preparation. We are trying to prepare the girls for what will inevitably come. They are wise and sad. They ask tough questions which helps us know what we believe. How I would do anything to see those faces smile. The muted glimmer in their eyes does nothing short of breaking my already torn apart heart.

I love how they encourage each other. When one is down, another one will try and be strong and cheer up her sister. They love the idea that Ava was given to us because God knew that we could love her the best. I'll tell you we certainly do. (We even learned tonight that she love her bottom lip and chin rubbed. As big a smile she could muster she did. This goes on the list of things about Ava I cherish from today. Also added are watching her play with the suction catheter in her mouth, sucking on her tube, which we haven't seen for awhile, and holding her in a more natural fashion. She was also OK to wear an outfit for a few hours as she was not as hot and sweaty today as she has been.)

Now if only I can get Elaina to understand that when Ava goes to God Eric will not show up in her place. She's just so adamant about it.

The faith of a child is awe inspiring and a breath of fresh air.

Friday, December 11, 2009

Makes No Sense

So here we are. It's been exactly one month since Ava was admitted to this PICU. She is bigger and beautiful. A nurse who hasn't seen her in a while came to visit. She asked me if she was getting better. I told her no. But she's not dying either. I will tell you that she looks really good today.

We've had some really thought provoking conversations with the nursing staff today. Aptly timed and a blessing.

Today, I have hope for Ava and peace in my soul. It's been missing for the better part of the week, but today it's back.

The bigger bugs slept a little better last night, but they each had their own issues. Elaina is currently the sickest of the group. They are getting lots of love from grandparents and their special sitter, who really is more like family than anything else.

We don't know what the next step is. God does and He will direct us. Today I am going to relish the fact that Ava looks good and that she still is fighting. Can I tell you that girlfriend does it with grace and style too. I also relish the fact that she actually felt a little snugly when I held her today. You know I'm going to enjoy the fact that I'm going to get my hair cut soon too.

Thursday, December 10, 2009

Ava had a good day while we were with her. She seemed fairly comfortable and looked OK, however her heart rate was really high. That usually has to do with her positioning, or temperature. She got a massage and repositioned and seemed to settle down a bit. We were happy about that. We really wanted to hold her and didn't want to if her heart rate was so high. We were only able to stay for part of the day (9:30am-4:00pm) today. So Allan was the one who got a turn holding our sweetie. (OK, she is sweet, but is still a little salty).

It's been rough around our house for the last 24 hours. Alexa is having more issues with her asthma than normal. She was up last night from 1:30am-5:00am. This wouldn't have been too bad except I was up making "Ava Bracelets" and lost track of time. I just crawled into bed at 1am. At some point Elaina woke up and then Emily did too.

Despite exhausting all the tricks in our "Asthma Arsenal" Alexa did not respond like she usually does. Our pediatrician came through and called in a prescription for an oral steroid. This seemed to do the trick. We are just so grateful for that. Tonight was birthday party for a special friend at an inflatable party place. They so needed to do that. While I was up holding Alexa on the couch last night I just kept asking God to allow her to go.

I spent the night wondering if we were going to walk into a baby who has taken a turn for the worse. I was worried about Alexa and leaving her in that condition, knowing an ER visit was soon to be on deck if something didn't give. I was so concerned, as we have successfully managed all asthma flare ups and stayed out of the ER. I could sense that she was scared of the prospect of needing the care of a physician and Emily was so worried about her sister.

The steroid did the trick and off the the party they went under Daddy's watchful eye. They were given a strict warning that Daddy may make them take breaks and lay low for a bit. The report is that no one so much as coughed. I am using plural here as Emily was beginning to show signs of starting down the same path.

I stayed home with Elaina and had time with just her. She now has a fever and a runny nose, so I just held her and she watched her favorite shows in my lap. We even shared dinner. During her bath I made a duck noise (I'm multi-talented). She told me that her pediatrician makes that sound. I don't remember the last time she saw him, it's been a couple months, but he makes that sound when he looks in her ears. She then told me that he takes good care of her, "because I don't like those tubes in my nose." I asked her who has tubes, she said, "Ava". I asked her if they hurt her or if she is managing OK. She said, "She managing OK." I then asked her if they helped her or hurt her. She told me they helped her. Later I asked her if her ears were broken. She told me no. I then told her that she is big and strong.

So we tucked three borderline not feeling well little bugs into bed tonight. Two were coughing, but have stopped. I hope I get to sleep tonight, I am so emotionally drained and now physically tired as well. I am always wondering if today will be the second saddest day of my life and what choices we are faced with making. Today, I was fully prepared for it to be "the" day. Ava decided to peer open one eye (for the first time in 2 days) and respond to us with smiles and pleasant faces.

I struggle with how to bridge the ever growing gap between the medical team and us. Today not one physician came to chat with us. We are invited to participate in morning rounds, something that we have never wanted to do, but I am thinking that it is now necessary. I do not like the notion that they are thinking all that is happening to Ava is "progression of the disease" and that they are just waiting for us to give "the word". We know that this is progression of HER disease and we would like someone to think about ways that will help her be more comfortable.

We feel like God is asking us to "wait". So we are. We don't know why, but we do. I am trying to stay in each moment and not think down the road. When I look down the road I find I am focusing on my agenda and not relying on God's Holy agenda for us.

Wednesday, December 9, 2009

Joy in Sorrow Hope in Despair

Ava is stable for Ava today. She, of course, loved her massage and was full of Ava smiles. She looks comfortable for the most part. Sometimes she squitches around, but i think she she just wants to get comfortable, go potty and the like.

Allan and I were "checking in" with each other today. We need to be unified so we can whether this hurricane together. We need to know how we are emotionally and spiritually so that we can connect and support each other when it is necessary.

I am amazed at how God is pulling us together and toward Him. There is some advantage to having gone through this once already. (Yes I really did write that). We KNOW that we can do it and that we can survive it. We KNOW that we can thrive through it as well. It's a lot of hard work and it HAS to be intentional. But it will happen.

I will never stop asking God to restore Ava's fragile and beautiful little body while she is with me. I believe that God is capable of such a feat. But if He chooses not to, my faith and confidence in Him will not be shattered. It's a sticking point for many. God does not give us what we want. He gives us what is best for us and His kingdom. He gives us the grace and peace to handle what lies before us. He gives us the ability to have hope when we feel despair and our soul groans with sorrow, frustration and anger. We can feel Joy in the midst of sorrow.

God called Allan and myself to walk this walk. To feel all the emotions for which I have yet to identify or name. He blessed us with our 5 precious children whom we love as best we can with all we have. He gave us the ability to be see that though life is not scripted in a fashion that we could have anticipated or wanted to experience He knows what is best. If we persevere and press on we will be refined and will continue to have Joy and Hope through our sorrow and despair.

I am not looking forward to experiencing the depth of that pain again. I'm not thrilled that I most likely will have to. I will continue to put one foot in front of the other, get dressed, teach myself to care about baseball, love my babies and hopefully affect some change in the world of mitochondria.

Tuesday, December 8, 2009

Family Fun

The bigger bugs descended upon the PICU today. They were so loving toward their sweet and salty sister. Ava clearly loved having them here. Her countenance changed. Her heart rate went down and she made pleasant faces. It was something they all noticed and I will forever cherish. Elaina held her sister's hand and said, "She love me." She sure does!

We also had a little family craft project time. We made four hand prints on a piece of paper which we will frame.

Our time together as a family of 6 was happy and enjoyable. No one was sad. It was beautiful.

We sure do love our bugs!

Monday, December 7, 2009


Our dear little baby is still fighting. Tonight, though her eye lids are so very heavy to her, she tried to open her eyes while in her Daddy's arms. With a lactate of 14 she grasped my finger and pulled against gravity.

After some temper tantrums and some choice phrases on my part, the Attending came in to speak with us about Ava's plan of care. (For example treating her unbalanced PH with bicarb. Not responding to critical results quickly, not knowing how high the lactate actually was. Not informing us of Ava's acidic state. Talking about why they stopped the bicarb. We weren't sure when they did and neither are they.) I did make certain that I informed the doctor that I am tired of thinking for them and I would like to be Ava's Mommy now. I am just fed up with the whole flawed hospital system. No one is responsible. There are too many cooks in the soup, per se. There is a lack of communication and a willingness to take responsibility for actions. "That wasn't on my shift." "That's not anything I can control." These are notions that frustrate me in my professional life as well. They are not something that only AI has issues with either. It makes me sad that you have to kick and scream to get heard. Your care is only as good as those who are willing to advocate for you. I simply want for them to communicate with us, advocate for Ava, keep us apprised of changes and write in the chart that we can hold our baby and have her sisters visit. But no, with every shift change comes a new approach and red tape.

I spoke with CHOP today. There is no argument amongst anyone that Ava has mitochondrial disease. There is discussion that Ava is putting this world into a whole new realm. She is the kid who has not yet been reported. (I made certain that the Attending at AI knew that so that I could remind him that Ava is here so they can learn from her. I also reminded him that Eric was another kid they could have learned from but he doesn't remember that he took care of him). I am hoping that we gently care for Ava and see what she needs. It is not my goal to let her camp out with a high heart rate, ignore copious sweat and salty taste. They did "make note of it", because of course that will help. They did listen to my suggestion of a sweat test. ( I'm not that smart, the idea stemmed from one of her nurses). I think the information is important to have.

There is less than a 1 percent chance for the general population to have the 2 mito babies we had. This makes them so very special. God called Allan and I to marriage. This is part of His plan for our family. We are blessed to actually have the living children we do. Ava is still living. She is still fighting and we are blessed to have her too.

Last night Emmy was sad and was missing Ava. I told her that she did not go to God yet. Emmy asked me if I thought she would. I told her that I thought so. Alexa asked why. I told her it seems that Ava has a lot more things broken in her body like Eric did. Alexa asked if Ava was born like that and if she was made that way. I told her yes. I told her that God loves Ava even if she's broken and He loves all of us too. She said, "Then why did we get her?" I told her I didn't know why God made her this way but we have her so we can love her. (love her we most certainly do!)

I have often said that although I would never want to relive Eric's life and death again I would to have him again. And to have him was to live through that experience. Eric never would have been who he was if he weren't "broken". As much as my heart is broken, I know that Ava is the special, sweet (well actually salty), tough little girl she is because God made her that way; broken and perfect. I am so glad that I've had the chance to love her, and know her and allow for all of you to know her too. It is my honor to be the mother to two very special and broken babies and to three very healthy and vibrant little girls.

I am sometimes ready to give up on Ava. I wonder if today is that day. I was actually convinced that it was. I've been convinced about that before as well. But today was not the day. Ava is refusing to let me give up on her. God is not done with our little baby girl, our tiniest ladybug. We, of course, will not give up the hope that God could restore her and keep her on this earth. As long as there is breath there is hope. I do know that there is no human or medical way that this child should live. I still don't know what God's plan is for Ava but I do know that I hope that she is restored and with us. God wants us to express our hopes and tell Him what we want He understands that and our need to do it. We need to understand that our life is not our own, no matter how hard we try to hold onto that "thing" or control it.

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...



Saturday, December 5, 2009

Our World According to a couple 5 year olds

I think Allan and I are running out of steam. We need to get the results of the muscle biopsy, but it is still not in the correct location. The lab at Baylor will not use the mislabelled sample, even though they can verify that it is Ava's at DuPont. (Red tape, licensing etc). This test apparently has information we need to know. The quote is three weeks from the time sample is received. I have not been overtly angry about this until about now. I am sick of the whole entire thing.

We came home tonight beaten down and weary. Waiting is hard. It's hard on us, it's hard on Ava, it's hard on the girls, it's hard on the grandparents who rotate caring for our girls. I told my sister tonight that I am tired of my life being on-hold and that I just want to get back to the regularly scheduled programing. To do that, I may not be able to at least see and kiss my daughter every day. So in a way I am content to wait too. We know that this is what God is asking of us, so we will obey.

Tonight the bigger bugs were very affectionate. We did our Christmas count-down (a gratis a friend/pre-school Mommy). The story was about how Sarah wanted to have a baby and waited her whole life for one. She prayed faithfully for her dream. When she gave up on the notion of having her own child, God blessed her with baby Issac. She was stunned. She was really really old. We then talked to the girls about how God answers our prayers. Emily mentioned that God answers our prayer about healing Ava. We talked about healing for only a minute (you know there were pictures to color). I asked Alexa how God heals. She said, "Heaven".

God can heal us imperfectly in that we are still "broken" and we continue to live this life on earth. He can also heal perfectly in that we are totally healed and restored as we are ushered into His presence. This is accomplished through our death and our belief that Jesus died for our sins and was resurrected. Healing in this way is the ultimate goal. In heaven there is no brokenness. Alexa knows about this because she knows that her brother was completely healed.

I took some time to individually tell the girls how special to me they are. To tell them that I miss them like crazy when we are not together. To see if they understand and accept that we need to do this for Ava right now. They are so gracious and understanding. They also want us to come home. They keep asking us when we can come back. They ask if Ava can come home. I told them that I didn't know. Alexa then told Emily that Ava may have to go to heaven. They so miss their sister.

Emily then told me that if the doctors say it's OK for us to come home then they will stay and take care of Ava for us. Did I know that some doctors stay up ALL night and sleep in the day so that they can take care of Ava? They are kind of like bats. You know, they sleep all day and just fly around at night.
This morning we were very blessed to have some much needed work done in our yard. DiSabatino Landscape Company generously donated their services and braved the cold and rain to help us clean out some flower beds and put down some mulch. This has been a source of stress for us, and the work they did looks fantastic. Thanks to Dave, Cara, Barkley, and Jose. Please visit their Web site:

Things are pretty much the same here at the hospital today- status quo, as much as that term can apply in an ICU. Ava is still stable and currently enjoying some chest PT. Outside the snow is falling, making me wish I was home to see the girls excitedly running from window to window. But for now I'm enjoying the peacefulness of the snowfall in a much quieter setting.

That's all for now...

Friday, December 4, 2009

Still waiting

We really don't have tons to report today. I guess that's a good thing. Results are looming, nothing is actually in our hot little hands. We are content to wait. We enjoy Ava and her sweet little faces and smiles. Today I held her for about 45 minutes. She was a little concomfortable, so we got her cozy in her bed. Her pupils were less dialated and a little more reactive then I've seen them in a while. She also tried to open her eyes more when it was darker in the room. That was fun.

Now we have the lights up so we are not sitting in the dark and so I can continue to string Ava's prayer bracelets. Ava doesn't seem to mind so much right now.

Ava's Chest X-Ray looks great, even the atalectasis in the right upper lobe is mostly gone. So now we need to pray for her to replicate her mitochondria. This can only be done by God. He can do it, so I'm praying for that. Will you too? Living is looking like it can be impossible for Ava, yet she still is. We are getting a clear message from God that we are to wait and not give up on her yet. I will tell you I have no other insight than that. There is nothing more to read into it. We will humbly obey.

Also we would request prayer for our bigger bugs. This is wearing on them. The need to feel the love of God in a tangible way.

Allan and I feel an amazing peace. Thank you for praying for that for us too.

Thursday, December 3, 2009


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.

Baseball Confessions

Allan and I met at church. We were going to go to see the Yankees play the Phillies for inter league play in NY. Circumstances were such that no one else who signed up to go went and we only got half way there. (My job required our return). After I was done with what I was called back to to, we watched the baseball game together. We have been together ever since.

One deciding factor for our relationship to continue was that the Yankees and the Phillies were in different leagues so it wouldn't get in our way. (We each cheer our team on with zeal). We also never really expected our marriage would have to face the test of a Yankees/Phillies World Series. (I am happy to report it was dicey but our marriage survived).

After Eric died, I had a hard time enjoying the game I've come to love over my lifetime. This was especially difficult because it was the initial reason that Allan and I "bonded". It was our thing. I just couldn't see the value in it. High salaries, misguided egos, steroids. I was just sad that Eric couldn't see any games with us. Allan escaped into the games, I escaped from them. I couldn't figure out how to enjoy something that seemed to have no heavenly purpose.

One day, while I was pregnant with Ava, we talked about this again. I was watching games and trying to get into them. Then this year I noticed that I was starting to care about it too. I was beginning to feel like I could enjoy baseball. Allan told me that God gave the pro ball players the gifts that they have for us to enjoy the game. No matter what direction the player is going in that's the basic idea.

We are here on this earth, not only exercise God's will for our lives, but to enjoy the gifts that HE gives us. Drink in the talents that He bestows on us. Everyday someone, somewhere is faced with tragedy, a miracle, peace or a life changing event. It does not mean we stop our lives and the enjoyment therein. Even though we are suffering. We are still enjoying our lives. (Sometimes more than others). In this hospital room there is a gamut of emotion: Hope, Peace, Sorrow, Joy, Agony. Because of Him we can feel them all. Even though we don't know what is before us and the struggles therein we know that God WANTS us to enjoy what we are given. It can be the smile of our baby, decking the halls or a simple crack of a bat.

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

We hope that many of you will be able to come out.

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.

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.

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.

Sunday, November 29, 2009


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, November 28, 2009


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?

Friday, November 27, 2009


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.


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.