Olivia's Big Adventure

Two years is too long

2011-08-16T22:19:00.000-04:00

It's been far too long missing your smile. Just once more... for even a second....

Miss you so much!

Love you always!

Happy 2nd Birthday Peanut

2011-02-19T14:21:00.000-05:00

Two years ago today you were brought into our world to change it forever. I wish today were truly a "Happy Birthday", but it's more of reminder of how much your absence still hurts. It's hard not to let the sorrow at your absence overwhelm the memories of joy we felt at your arrival.

Not a day goes by where I don't miss your smile, your throaty chuckle, or your full head of hair. I still feel your penetrating gaze looking upon my soul, giving me strength to draw upon when my sorrow goes too deep.

I know you're watching over our family, guiding us with your little sister. Thank you for that! I hope you're partying with all the other AGS angels eating cake to your heart's content.

I miss you & Love you my little sweet Olivia Anne.

A Bundle of Joy at the End of the Rainbow

2010-11-01T23:35:00.001-04:00

My Dearest Olivia,

I'd like to introduce you to your little sister Amelia Anne.

She was born 2 weeks ago today on 10/18/10. She tipped the scales at 7lbs 3oz & 20 3/4 inches long. She came into this world a very special child -- not just that's she's a Rainbow Baby, but she's got you as her older sister and personal Guardian Angel.

We know you've been watching over her since we first found out about being pregnant on your 1st Birthday. A day where we should have been giving you presents, you gave us the best present we could have ever dreamed of.

All throughout the next 9 months you have never been far from our thoughts and hearts; every Dr's visit, every test, every ultrasound, anxious days of waiting. You guided us and helped reassure us that all would be OK.

When we had the 3-D ultrasound, Amelia's face reminded us so much of you. The broad nose (yeah that's daddy's nose -- poor girl), your mother's lips, munching on her hand, even the little episode of Hiccups she had during the ultrasound reminded us of you.

Olivia's Little Sister

There were tears of joy and sadness when she was born; Joy because we finally got to meet our Rainbow Baby. Sadness because it reminded us of what we've lost. Your beautiful picture sat at our bedside, giving us strength all night long.

I'm sure you'd approve of her first out fit too!! Not only does she wear it proudly, it also has a nice Dragonfly on it :)

We miss you everyday peanut, but we know Amelia couldn't have hoped for a better Big Sister. Thanks for guiding her safely into this world. Thanks you for acting as her personal Guardian Angel now and in the future as Amelia grows into the healthy and wonderful person she's destined to be.

We'll love you always.

Love,
Dad, Mom, & Amelia

365 Days of Eternity

2010-08-16T00:38:00.002-04:00

One year ago today I put our sweet Olivia back to bed after her 2am feeding. I rocked her to sleep, kissed her forehead, rubbed her cheeks, and whispered "I Love You, Sleep Well." Little did I know, as I quietly pulled the door shut and crawled back into bed beside Sara, that I had just spent my last moments with our precious Peanut.

The stillness of that Sunday morning was completely misleading, soon to be broken by the frantic cries and screams of breaking hearts and shattered lives. When I woke up to sun streaming into our room rather than cries from the baby monitor, I knew something was wrong. I checked the clock, it said 9am, a bit past Olivia's usual wake-up time. As soon as I opened the door to her room, I knew my recurring nightmare had come true. Holding my precious Olivia, still and lifeless, no cooing, no crying, no giggling, no wiggling, just still; a quiet peaceful look on her face. She had departed this life after fitting more into her 6 short months than many people experience in a lifetime.

While the sun has risen and set 365 times since Olivia left us, there is not a day that doesn't go by where she's not at the forefront of my mind. The sharpness of the pain has receded to a degree, there are still daily reminders of what should have been. I suppose it's like any chronic ache, you figure out how to function with it or around it. Seeing children around Olivia's age is the most difficult because it's a direct reminder of what is missing. At times it also is difficult to believe that we were lucky enough to be with her for 6 months. As that time gets pushed into the distance and her absence becomes longer, the surreality of it strikes me more and more.

The most difficult times are the unique events in life we'll never get to share with her. We got up to watch some of the Perseid Meteor Shower the other night all the while thinking that Olivia would never get to experience such wonders of our world/universe. I did wish upon one of the 'shooting stars' for Olivia to find the peace and health she so well deserved in her life but wasn't blessed with.

I'll close with a Poem I wrote shortly after Olivia's death and a Song that's helped me a lot this last year.

Sleep Well
with your eyes of steel blue,
you pierced my heart.

with your red-golden locks,
you bound my heart to yours

with your toothless smile,
you stole my heart.

with your delicate fingers,
you held my heart.

with your laugh and giggle,
you tickled my heart.

with your cry,
you broke my heart.

with your silence,
you tore my soul asunder.

sleep well my angel,
you will be forever in our hearts.

OAH 2/19/09 - 8/16/09

Every December Sky by Beth Nielsen Chapman

Every December sky
Must lose its faith in leaves
And dream of the spring inside the trees.
How heavy the empty heart,
How light the heart that's full.
Sometimes I have to trust what I can't know
Sometimes I have to trust what I can't know
We walk into Paradise;
The angels lend us shoes.
'Cause all that we own,
We'll come to lose,
And Heaven is not so far
Outside this womb of words.
With every rose that blooms
My soul is assured
It's just like a song I've known
Yet still unheard.
And every leaf of fire lets go,
Melting in the arms of earth and snow.
And if I could hold you now,
You'd enter like a sigh.
You'd be the wind that blows
The answer to why?
You'd be the spring-filled trees
Of every December sky.

One Year Ago Today

2010-02-19T11:48:00.000-05:00

At 9:15pm on 2/19/09, Sara and I were introduced to our daughter Olivia Anne. We were well aware that our lives would change forever, yet totally unprepared for the direction of change. Today should be a day of celebration and laughter, yet instead it will be a day of sadness and tears.

The last 12 months have been a roller coaster of emotion and stress. Even though we were exhausted the first 36 hours of our life with Olivia were perfect. We did not yet know that she had a heart defect, we did not know her liver couldn't clear itself of bile, we didn't know only one kidney would function. How sweet those precious hours of peace were. I can remember waking up to her 'hungry' cry during that first night, the pride I felt in being a father, the overwhelming urge to protect and defend, the reality of being a family of three now.

Not a moment goes by where Olivia is not front and center in our minds. The loss of those bright shining eyes and dimpled smile changed who we are forever. The torn fabric of our family might slowly be reworked and mended, yet always will the scar remain. We have made many new friends along our journey, while some old ones have faded away. The support we've received from friends, acquaintances, bloggers has been critical to our survival and will continue to be so in the future. I ask you now to keep us in your thoughts, not just today, but next week, next year, five years from now. Light a candle, Say a prayer, send a card, an e-mail, a phone call, acknowledgement that Olivia touched your lives keeps us going.

Peace,

Ben, Sara, & Angel Olivia

Goodnight My Sweet Angel.... Happy 1st Birthday

Farewell 2009

2009-12-31T18:52:00.000-05:00

2009 started with such hope and promise for the future, only to come to a sad end, turning the page on a year that changed our lives forever. No matter how sad, angry, or lost we feel, I would not trade one moment of the life we had with Olivia to make the pain go away. I'm just glad that 2009 will soon be a fading memory, ushering in a new hope with 2010.

Sleep well my sweet. Happy New Year Olivia. We Love you.

Alagille Tribute

2009-12-14T14:41:00.000-05:00

Here's a video of our extended Alagille Family, put together so nicely by Julee Spalinger. At this time of year, please take a moment to reflect on what you have and what has been lost. If you are lucky enough to have fully healthy children count your blessings and don't take ANY time you have for granted.

Peace,
Ben

Make photo slide shows at www.OneTrueMedia.com

Happy Thanksgiving Peanut!

2009-11-26T17:30:00.000-05:00

We Love You & Miss You SO much....

Adele - Chasing Pavements

I've made up my mind,

No need to think it over,

If i'm wrong I aint right,

No need to look no further,

This ain't lust,

This is love but,

If i tell the world,

I'll never say enough,

Cause it was not said to you,

And thats exactly what i need to do,

If i'm in love with you,

Should i give up,

Or should i just keep chasing pavements?

Even if it leads nowhere,

Or would it be a waste?

Even If i knew my place should i leave it there?

Should i give up,

Or should i just keep chasing pavements?

Even if it leads nowhere

I'd build myself up,

And fly around in circles,

Wait then as my heart drops,

and my back begins to tingle

finally could this be it

Should i give up,

Or should i just keep chasing pavements?

Even if it leads nowhere,

Or would it be a waste?

Even If i knew my place should i leave it there?

Should i give up,

Or should i just keep chasing pavements?

Even if it leads nowhere

Should i give up,

Or should i just keep chasing pavements?

Even if it leads nowhere,

Or would it be a waste?

Even If i knew my place should i leave it there?

Should i give up,

Or should i just keep chasing pavements?

Even if it leads nowhere

It Means Nothing

2009-10-18T23:10:00.005-04:00

Sometime a song just hits home. I came across this song

when a friend posted it on Facebook shortly after Olivia died.

Somehow it just seems o capture a lot of what I can't seem to

express in my own words.

Did we lose ourselves again?
Do we take in what's been said?
Do we take the time to be
All the things we said we'd be
And we bury heads in sand
But my future's in my hands
It means nothing
It means nothing

You can find yourself a God
Believe in which one you want
'Cos they love you all the same
They just go by different names
When we fly our flag today
Are you proud or just ashamed?
It means nothing
It means nothing

It means nothing
It means nothing
It means nothing
If I haven't got you
If I haven't got you
If I haven't got you
If I haven't got you

And the sun sets in the sky
You're the apple of my eye
If the bomb goes off again
In my brain or on the train
I hope that I'm with you
'Cos I wouldn't know what to do
It means nothing
It means nothing

It means nothing
It means nothing
It means nothing
If I haven't got you
If I haven't got you
If I haven't got you
If I haven't got you

Olivia Memorial Compilation

2009-09-20T01:11:00.004-04:00

Here is a shortened compilation of pictures that were shown at Olivia's funeral.

The song is Somewhere Over The Rainbow/What a Wonderful World performed by Israel Kamakawiwo'ole

Thank you to everyone who has supported us and helped us through our ordeal. Everyone has been just wonderful in our time of grief. Sara and I are blessed to have such great friends. Thanks.

I'll keep posting every now and then so keep coming back.

Dragonflies

2009-09-08T08:52:00.005-04:00

I am still at a loss for words to describe our current journey. Three weeks have gone by, yet every moment has seemed to drag by excruciatingly slowly. When Olivia was born in February our lives changed forever. Our regular routines and freedoms were thrown into chaos. Slowly over the next weeks Sara and I established nice routines all centered around Olivia - Feedings, Playtimes, Baths, Dr Visits, Medicines, Etc. We were satellites orbiting the center of our universe -- Olivia- a very bright star. When she left our world 3 weeks ago, our Sun, our Center, Our Focus was taken away. Sara and I lost our center, we lost our focus, we lost our Star. Hopefully someday we will find pleasure again in the freedoms that have returned -- finishing a meal uniterupted, sleeping in on the weekends, getting in the car w/o needing to 'load up.' These are freedoms we glady gave up and don't want back, but we have no say....

Needless to say, we have searched everywhere to understand why. We look for the tiniest little sign that things will be ok. The other day we were fortunate enough to experience one such event. Sunday morning 2 weeks after Olivia passed away I noticed a large number of bugs flying around our back pasture. When Sara and I went out to investigate, we were overwhelmed by Dragonflies. There were probably 200 or more flying around in our couple acre pasture. We stood out there and marveled at a spectacle we had never seen before. In the 10+ years I have lived in this same house, never had I seen this many Dragonflies in one area.

As we stood and soaked in this display of gleaming wings and darting bodies, one large Dragonfly came up to us. It had an iridescent green body with a purple/blue sheen to it's tail. The wings were a transluscent silver, gleaming in the warm sun. It hovered in front of Sara's face for a few seconds, then it moved over to me and landed on my left shoulder. Soon it took off and dissapeared into the rest of the Dragonflies.

While looking around on the internet about dragonflies, we came across some interesting facts. Native Americans believe that Dragonflies represent renewal and can be messengers from the spirit world. The story below was also found in our search, somehow it feels very appropriate.

The Dragonfly
Once, in a little pond, in the muddy water under the lily pads,
there lived a little water beetle in a community of water
beetles. They lived a simple and comfortable life in the pond
with few disturbances and interruptions.
Once in a while, sadness would come to the community when one of
their fellow beetles would climb the stem of a lily pad and
would never be seen again. They knew when this happened; their
friend was dead, gone forever.
Then, one day, one little water beetle felt an irresistible urge
to climb up that stem. However, he was determined that he would
not leave forever. He would come back and tell his friends what
he had found at the top.
When he reached the top and climbed out of the water onto the
surface of the lily pad, he was so tired, and the sun felt so
warm, that he decided he must take a nap. As he slept, his body
changed and when he woke up, he had turned into a beautiful
blue-tailed dragonfly with broad wings and a slender body
designed for flying.
So, fly he did! And, as he soared he saw the beauty of a whole
new world and a far superior way of life to what he had never
known existed.
Then he remembered his beetle friends and how they were thinking
by now he was dead. He wanted to go back to tell them, and
explain to them that he was now more alive than he had ever been
before. His life had been fulfilled rather than ended.
But, his new body would not go down into the water. He could
not get back to tell his friends the good news. Then he
understood that their time would come, when they, too, would
know what he now knew. So, he raised his wings and flew off
into his joyous new life!
~Author Unknown~

In the weeks to come I'll post a small memorial video of Olivia, keep checking occasionally, I'll keep the site active. Thanks again to everyone for their expressions of sympathy and support and of course to those who have donated in Olivia's name to The Alagille Syndrome Alliance. Take a moment to go there and donate $5 if you haven't. Thanks.

Thanks for listening.

Ben, Sara & Little Angel Olivia.

Time for Reflection

2009-08-23T12:59:00.002-04:00

Sara and I will be taking some time away in an attempt to recenter our lives. It won't happen in one day, one week, or even one year, yet we need to start the process.

We wish to thank everyone for their outpouring of prayers and support. It has helped sustain us through the darkest days we could ever imagine. Thank you we love you all.

If you are in any way able, making any size donation to the Alagille Syndrome Alliance in Olivia's name will go a long way toward helping other families affected by this insudious syndrome. Thank you so much to the generous outpouring we have already seen.

Continue to check in here every once in a while, I still have some things I need to say.

Until then,

Peace to you all,

Love --

Ben & Sara & Our Little Angel Olivia

Sleep Well

2009-08-16T20:38:00.003-04:00

with your eyes of steel blue,

you pierced my heart.

with your red-golden locks,

you bound my heart to yours

with your toothless smile,

you stole my heart.

with your delicate fingers,

you held my heart.

with your laugh and giggle,

you tickled my heart.

with your cry,

you broke my heart.

with your silence,

you tore my soul asunder.

sleep well my angel,

you will be forever in our hearts.

OAH 2/19/09 - 8/16/09

Stuck in a Rut.... (Part 1)

2009-08-04T09:49:00.021-04:00

... with babies we've found out that's not so bad of a thing.

Olivia has had a pretty hectic schedule for the last

2 weeks, leaving us all a little more or less frazzled :)

Week 1 itinerary:

Monday Evening:

The D i s c o C r i b E x p e r i e n ce - 10pm - ?

Tuesday:

8:20am U of M Liver Recheck
8:40am U of M Kidney Recheck
9:30am Vampire Blood Draw

12:30pm Kidney Ultrasound

1-3pm Eat Lunch, Nap, Travel Home

3:15pm Stop at Chelsea Teddy Bear Factory

3:18pm Acquire another stuffed animal

Wednesday & Thursday:

Normal Routine @ Home & Grandma's

Friday:

Late Afternoon: Kansas & Missouri Huelsbergen Clan Arrive

Olivia was not impressed with all the new arrivals: My Father, Brother, Sister-in-Law, 2 cousins, 2 dogs and 1 bird. She gave them all "The Look" -- trying to decide if they were going to poke and prod her like most all newcomers (Docs and Nurses) do. She showed her dissatisfaction with numerous grumpy faces and crying. Great now my family thinks she's a complete grouch....

Saturday:

AM: Hang out at home & watch her cousins freeze in the unseasonably cold pool. Rianna and Lianna had a blast splashing in the water even with chattering teeth. At least the afternoon swimming session had some bouts of sun. (Pic to the right: Sara, Olivia, Grandpa Huelsbergen, & Rianna) The true Olivia started to make some appearances in the morning with a few smiles here and there, but the hustle and bustle still had her on guard - shooting "The Look" more than a

few times.

PM: The weather warmed up in the afternoon for a leisurely stroll around the grounds at Leila Arboretum. (Pictured to the left -- Myself, Olivia, Rianna, and Lianna in the background with a walking stick as tall as she is.) Everyone seemed to have a great time soaking up some sun and fresh air. Olivia really enjoys riding in her papoose,

it gives her a great vantage point absorb her surroundings. Over the last few weeks she is becoming much more aware of her environment - movement, sounds, & new objects. This walk proved to be just the ticket to put our little diva into a nice long nap. She didn't even make it home before she fell asleep. Home for a Barbecue and more sleep.

Sunday - "We're Going to the Zoo!"

AM -PM: I feel we're very fortunate to have a very nice Zoo here in Battle Creek - Binder Park Zoo. For a non-metropolitan area, this zoo is quite progressive and attempts to place animals into a more 'natural environment.' The plan for today was to make it to "Wild Africa" and feed the giraffes. Olivia's cousins were very excited about this Safari. We packed our things early and were ready to go when Olivia woke up from her morning nap. We haven't gone on too many outings besides Doctor visits and little shopping

trips, so this was going to be interesting. Olivia did quite well, all the people, new sounds, and smells did make her look a little wide eyed at times. While riding the tram to "Wild Africa" it became a little breezy and Olivia had some pretty priceless expressions on her face when she got a blast of the air. "Wild Africa" exhibit is laid out in about a 2 mile loop taking you by

an assortment of African Wildlife -- Giraffes,

Zebus, Wild Dogs, Monkeys, and Birds. A little sprinkle of rain didn't dampen anyone's spirits. A quick lunch in the cafe, an Ice Cream Cone for Rianna & Lianna, and it was time to head home. Olivia had reached her limit of stimulation and was letting the world know she was ready for a break.

PM: On to dinner at Clara's on the River, a nicely restored railroad station from the later 1800's. A nice evening out with close family, good conversation, and good food... what more could

we ask for. Olivia was able to suck on her bottle in style with three Grand parents, her aunt & uncle, her cousins, and of course her parents.... not that she really cared who was at the table as long as someone supplied the grub. It was a late evening before Olivia finally got to her own bed/crib, but she 'slept like a baby' once she was down.

Olivia's Favorite New Song

2009-07-23T23:47:00.003-04:00

Olivia will stop anything she's doing when this video comes on -- even though she doesn't really use a pacifier at all.

Be warned, it's a catchy (annoying) little tune you'll have trouble wiping from your mind the rest of the day.

Olivia & Her Disco Crib

2009-07-21T19:54:00.008-04:00

We made our monthly pilgrimage to Mott Children's Hospital in Ann Arbor to check in with Olivia's Liver and Kidney specialists. We were able to schedule both clinics and her Kidney Ultrasound on the same day, making it much easier than having to make 3 separate trips of course. We have been spending the night before her appointments in a Hotel to avoid having to drag our butts out of bed before 5am and suffer the rest of the day.

Olivia actually travels very well. She likes her car seat, once we get in motion she tends to settle

right down . Consequently when we arrived to our hotel Olivia was well rested and ready to Parrrttaaayyyy!!! She was wound up and didn't want to settle down. We ordered room service, fed & changed Olivia, and got ready for bed. Last week Sara found a nice travel Crib/Bassinet (see picture) on E-Bay for Olivia so that Little Peanut would have a nice place to sleep, little did we know what we were in for. It plays lullabies and an electronically bastardized version of Pachelbel's Cannon - , it vibrates and has twinkling lights. After we placed Olivia in her new crib and turned out the lights ... finally bedtime or so we thought.... Sara and I look over to the crib and it looked like there was a disco going on. Flashing lights, swinging ornaments, and thumping feet -- all to Electronic Pachelbel's Cannon. Olivia has taken to lifting her feet and slamming them down onto whatever is in their way, last night it happened to be the end of the crib. This 'party' - punctuated with overtired baby wailing and foot thumping - lasted close to 30 minutes before she decided that sleeping was the best option. She was kind enough to let us sleep from 11pm until 5am without waking --- Yay!!

Our Tuesday started with a good weigh-in. Olivia easily topped 10lbs, tipping the scales at 10lbs 5oz! She managed to put on 1lb 3oz since her last Liver appointment. For her combination of Heart & Liver Disease everyone was very happy with this gain. Olivia got compliments from "Wow, she looks good" to "Hey you're getting some meat on those bones!" She actually fell within the normal desired growth rate! Obviously the new formula that she's been on for the last month has helped her a lot. She also seems to like it quite well, which, given all the crap we've had to medicate her with is a relief.

With her weight no longer an issue, we could turn to her other two issues on Tuesday's agenda: Itching and a Kidney Ultrasound. Over the last 2-3 weeks it has slowly become evident that Olivia's elevated Bilirubin values have are causing her to itch - she rubs her eyes with her

hands or rubs her whole face on our shoulders when she is being held. This is a very common development for anyone that has with severe cholestasis (bile build-up). We had been using Benadryl at night, but it seemed to be only helping minimally. Dr. Lopez wants us to try Hydroxizine (Atarax) to fight her itch, especially at night. During the day Olivia doesn't seem to itch too much, but when she starts to settle for the night, her itching starts, hopefully this new antihistamine will bring her some relief and make her drowsy enough to sleep through the night.

We capped off our visit with a quick detour to ultrasound land to settle a big question about her kidneys... Here's what I posted about our May visit with the Renal Team

The other semi-surprise today was that Olivia's Abdominal Ultrasounds (3 in total) taken over the last 2 months, don't all agree on the kidney findings. All the scans showed 'small' kidneys, but one -- the most recent one -- did not mention anything about a multi-cystic right kidney, while both the initial exams did make note of this finding. The possible problem here is that the Ultrasounds were not "Kidney Specific" but were more liver focused. A Kidney Specific Ultrasound has been set up for August to fully evaluate these discrepancies.

Our ultasonographer was able to get some very good pictures of Olivia's Kidneys. Her Right Kidney was obviously small

and not normal in consistency. We were able to see some of the previously described cysts on her right kidney. Her Left Kidney meanwhile "looked pretty much normal" according to the ultrasonographer. She did not feel that it was significantly smaller than it should be for Olivia's age. There were also no obvious cysts seen today. Mind you this was just the initial reading by the ultrasound tech, the study will still need to be read by a Radiologist, who will hopefully confirm these findings.

We've been graced with so many complications and negative developments over the last few months that today seemed to lift a great weight off our shoulders. This truly has been the best news we've received since, geeeze I don't know when. While Olivia has many more trials ahead, somehow the sun shone just a little brighter on the drive home today.

Save us a spot in your Disco Crib Olivia --- we want to join the party!

Odds & Ends

2009-07-14T09:48:00.008-04:00

It's been a very uneventful 2 weeks so here are a few miscellaneous notes/thoughts that wouldn't make a separate coherent post. No Dr Visits for another week :) So next week should have reports from both Liver and Renal.

Olivia had a cardiology appointment 2 weeks ago, everything went very well. Her Echo showed no changes. Her function continues to be good, her Oxygen saturations are steady in the 82-82 range. Dr. Dohmmer was very happy with Olivia's progress. While UofM would like Olivia gaining weight faster, Dr. Dohmmer feels that her weight gain is good/acceptable for her combo Heart/Liver issues. We don't have to go back until end of July

Still trying to get the UofM team on the same page.

Renal & Liver aren't playing well together.... not that they are fighting, but they just aren't

talking as much as they need to for Olivia's sake. When one group makes a change in her medications or treatments it shouldn't be up to us have to inform the other group. This should be noted in her chart and be obvious to anyone reading them.... either things aren't getting put in the chart or they aren't reading the chart. Luckily we'll have our next meeting with BOTH Liver & Renal back to back next week. Maybe we can force the Dr.s to sit down meet each other, sing Kumbayah or something--- well let me dream at least.

Olivia's still gaining weight, she's now just over 10lbs., meaning she's put on 4.8lbs since her lowest weight w/o having her heart fixed and her liver malabsorption issues we think this is just dandy.. it doesn't matter what "Percentile she's at" what matters is that she's eating and growing w/o a feeding tube!

Olivia's getting excited about meeting her cousins from Missouri & her Grandpa from Kansas next week -- well in actuality O has no clue, but Sara and I are excited for her. If we get the pool warm enough, maybe she'll even get her first swimming lesson.

The Diva's finding her voice. She's been making cooing and gurgling noises for a while now, but over the last few days she's making a very distinct 'hiss/gurgle' noise when she's happy. We tried to capture it on the following video.

Just last night she started working on a second voice, a nice shrill squeal that I fear will develop in volume and intensity as she perfects it... ear plugs please. I managed to just catch a quick snippet of the sound here:

Do all Babies have a built in meal/food detector? Olivia can be in a deep sleep but as soon as Sara and I sit down to eat our meal, you can bet that Olivia will start screaming bloody murder. It doesn't matter if she's been sleeping for 5 minutes or 2 hours, she knows when we are about to eat. It doesn't matter if she's in her bouncy chair with us or across the house in her bedroom, that girl's got timing.

Many people have asked us "How are you doing and holding up?" Actually quite well. I like to describe what we're experiencing akin to a duck swimming on a pond: Nice and calm on the surface but paddling like mad below the water line. Of course that is a great simplification of my/our feelings, but for now that will have to do, there is much much much more to write on that subject, .... at a later date.

Once again Olivia, Sara and I would like to thank everyone for their continued inquiries, well wishes, gifts, meals and prayers; it's all greatly appreciated.

Alphabet Soup

2009-06-30T10:57:00.012-04:00

When we took Olivia to her new pediatrician last week , we came away greatly impressed. O's Cardiologist had recommended Trestlewood Pediatrics in Portage (Kalamazoo) for her routine care - Vaccines, Runny Nose, Etc - basically anything not Alagille related. When we walked in an talked to one of the receptionists, right away she asked if Olivia was still seeing Dr. Dahmer (Cardiologist). As it turns out, her daughter was born with a severe heart defect 6 years ago and has been under her care ever since. I just those few sentences she made us feel welcome and gave us a positive inspiration for the day.

We were shown to an exam room by a nurse who took Olivia's vital signs; 9lbs 8oz & 23 1/4 inches

long -- our little peanut is growing... slowly but steadily. The clinic was neat, clean, and fairly new. Good first impressions. When Dr. Van Es came in and introduced himself he said "Well I've read a lot about Olivia from all the specialists so it's nice to finally meet her in person." Dr. Van Es spent the better part of an 1 hour talking to us about Olivia's history and treatments so far. Asking about her milestones and telling us what to expect over the next months. When is the last time you got 1 hour face time with a physician? We were very happy.

He then did a very thorough exam -- which Olivia did take exception to -- finding nothing

remarkable outside of her known afflictions. Dr. Van Es made it a point to stress the importance of vigilance with 'The Diva', she walks a fine line health wise so any questions we have we should contact their office and they'll find an answer for us. He also made it an option to bring 'Her Majesty' in through the side door for her appointments, so she wouldn't have to wait in the main waiting room with any sick 'peasants.'.

Once the Dr. was gone it was time for the dreaded Vaccines.

Duh da daaaaaa ......

Olivia, having plenty of experience in the field of Pokeology, eyeballed the nurse as soon as she walked through the door. "The Look" is hard to describe, but it's very distinct. Olivia's facial expression doesn't really change, yet her eyes get slightly wider and steeled for the next event.A slight tensing of her body can be felt too. Her little Blue-Gray eyes observing every movement, rolling as far to the side of her head as possible as to not lose sight of her foe.

Olivia had only been given her HepB vaccine when she was born, all others had been postponed until now because of Olivia's Surgeries and hospitalizations. On this visit the Little Peanut (aka Little Turd), was subjected to an alphabet soup of vaccines: RV, DTaP, Hib, PCV, & IPV. This consisted of 1 oral and 3 jabs into her legs. I'm not exaggerating when I say jab, seriously it seemed like the nurse wound up to pitch a fastball with a needle & syringe. With

the little meat that is on O's thigh's, I was waiting for the needle to come out the other side and jab me in the leg.... Olivia's reaction I'm sure is identical to most infants/children's... there's a delay of 5 seconds or so before the stick registered in her brain, but the did she let loose a wail of pitiful proportions. Sara just commented again the other day that watching those vaccines was almost more difficult than holding her to get her blood drawn. Once the poking was over though, she quieted down quickly and slept most of the rest of the afternoon.

On our way home from this visit, it suddenly hit me that this was Olivia's FIRST wellness check-up. This Dr's visit had nothing to do Allagile, Tetralogy, or Kidneys!!!! Every visit up to this point had been to check something to do with her ailments. Only took us four months to get here, but hopefully before long I'll be writing more about these routine visits and normal baby milestones than her 'sick visits.'

Till the next time..... Olivia will be Chillin' in style.

Quest for the Golden Elixir.....

2009-06-23T12:27:00.008-04:00

Sounds like a setting for an adventure novel or movie, eh?

Two intrepid and dedicated adventurers set out to capture a few precious drops of this liquor of life. Little did they know when they set out early yesterday morning what trials they would experience along the way. Armed with multiple little collection baggies they brave the heat and humidity of a typical Michigan Summer day. Quietly and patiently they set their trap.
Tick, Tock
Tick, Tock
Finally their quarry was sighted, a slight dribble, then a stream, yet somehow it managed to fully evade their specimen trap! Aaaargh, sticky tape failure and the prey ran off to the safety of a diaper pad... A chuckle or two was had about their equipment failure, but they quickly reset their specimen trap, not knowing when the next dribble of Liquid Gold would appear.
With carefully applied sticky tape & apparatus the two decided not to hover, because we all know "A watched pot never boils." A short time later to their surprise the Specimen Trap had been filled. To the adventurers dismay it was filled with a much larger quarry they weren't looking for, contaminating the desired Elixir beyond use. For the third time in as many hours, another specimen trap was carfully applied. Not too low, not too high, hopefully just right along the quarry's path.
Tick, Tock
{Theme to Jepoardy plays in the background}
Tick, Tock
Yet another equipment malfunction, allowing the Golden Elixir to escape capture. The intrepid adventurers made note to contact the trap manufacturer when they returned form the wilds and give them an earful about their product....
Over the next 14 hours, make shift and modified traps managed to catch everything but the needed liquid. A modified zip-lock baggie was used to cover the quarry's whole living area, but managed to catch only spit-up, projectiled from the other end of the hunting area. With each daylight fading fast and frustrations levels reaching a fever pitch, one final bid was made to slay this formidable opponent. With a careful and deliberate manner the Golden Elixir's retreat was cleaned, dried, and prepared. The final trap was applied, carefully the sticky tape sealed completely, allowing no escape.
With much jubilation, a short time later their quarry had been trapped. The rare specimen was quickly transferred into a transport container and rushed to the waiting scientists for evaluation. Fourteen hours after starting their 'simple task' of collecting a small specimen of Liquid Gold, the two adventurers collapsed into their bed at home, wondering what tomorrow would bring.

So who would have though the simple task of collecting a urine sample on little O, could have turned into such a fiasco. Moral of the story though is, stick with it and you'll get what you need in the end, even if it's 14 hours later....

Please Don't Call Me a Mutant.....

2009-06-18T19:33:00.009-04:00

At our Tuesday Liver PowWow we also received confirmation of Peanut's Alagille status. Olivia is positive for 1 DNA Base Pair substitution at location 235 of the Jag1 Gene. Instead of a Cytosine nucleotide at that position, she has a Thymine nucleotide. Now think about this for 1 minute. There are 3 Billion total base pairs in the whole human genome, yet 1 lousy mistake/change has created ALL of Olivia's medical issues -- Heart, Liver & Kidneys.

What are the odds?:

Winning a State Lotto 1 in 18 million
Getting in a plane crash 1 in 11 million
Getting struck by lighting in your lifetime 1 in 7000

Kind of changes your perspective on things eh? .....

Ok let's back up a bit and talk Genetics and DNA to understand exactly what is happening here. Our DNA is basically only made up of 4 different molecules :

A -Adenine
T - Thymine
G - Guanine
C - Cytosine

These Bases are strung together into

23 long 'strings' which are know as Chromosomes. Along the length of each Chromosome different segments are what we commonly call Genes. As we all know, Genes are what controls almost everything about us, Hair color, Eye color, Height, Brain development, Organ development, etc.

Each Base matches up with it's

corresponding partner and only that partner, creating Base Pairs and what we know as the Classic Double Helix of DNA.

A with T
G with C

These base pairs that make up our

DNA/Genes are basically the instructions on how to build and run our bodies. Each group of 3 base pairs codes for an Amino Acid which are the building blocks of proteins. Each gene when activated will then create sequence of Amino Acids which fold into a protein that has a specific function -- building cell walls, assembling more complex proteins, assembling hormones, etc.

The trick is that there are thousands of Genes on each continuous Chromosome, so there have to be certain areas that label the start and end of a certain Gene. These areas are referred to as Start and Stop Codons -- a collection of 3 bases in a specific order. This is where the Little O has her mutation. A normal Jag1 Gene consists of just over 36,000 base pairs, creating a protein of around 12,000 amino acids. Olivia's Jag1 gene has a T (Thymine) molecule where she should have a C (Cytosine) Molecule. This single base change in the gene creates a STOP Codon (TGA) at the 235th amino acid position where it should create a Arginine Amino Acid (CGA) . Basically this creates a significantly shortened/malformed Jagged1 Protein. This protein is critical in the development/migration of vessels within the growing fetus. If we then think about Heart, Liver and Kidneys --- all structures that rely heavily on vessel formation/migration --- we can see how those 3 organs get his hard by Alagille Syndrome (Jag1 Mutations).

From my understanding this mutation is one of the more common 'de novo' - or new mutations, at this locations. 60 to 70% these mutations are not inherited from either parent. At some point both Sara and I will get tested to see if we are carriers of this mutation and passed it on to Olivia, or if she just got Unlucky.....

I hope I didn't bore anyone too much with my basic genetic review, but I find it amazing and fascinating what an intricate machine we all are lucky enough to inhabit. Thank your lucky stars (or the deity of your choice) daily that your body was assembled correctly, the tiniest change at conception (or before) can have immeasurable consequences, and it's all due to the magic of DNA & Genes.

Meet the Liver Team

2009-06-17T21:22:00.008-04:00

Tuesday's check-up in Ann Arbor went as smooth as can be expected. Sara and I have decided anytime Olivia doesn't end up hospitalized makes for a good visit. :) We met with pretty much the full complement of practitioners involved with Olivia's Liver care. This team consists of a Registered Nurse (Vicki), a Social Worker, a Clinical Psychologist, a Nutritionist, and of course Dr. Lopez. From what we gathered, Vicki seems to be the lynch pin to this team, coordinating and organizing everything.

Meeting with the Clinical Psychologist was interesting. He was interested in how we've been dealing emotionally with all of Olivia's problems. We had a good conversation about our hopes and fears concerning our Little Diva. Of course Sara and I have talked about these issues quite a bit, but it was good to

have a 'neutral'

party become part of the conversation and validate our feelings about our dearest Olivia. It sounds like we will meet with him every time we come for a Liver follow-up.

Dr Lopez popped in for a couple minutes, took a quick look at Olivia and was gone again. He did seem happy with her overall progress, since she'd put on just over 1lb since her last visit 1 month ago and her blood values have been stable.

Kila, the clinical nutritionist, came in after Dr. Lopez and spent a good 15 to 20 minutes talking to us about Olivia and her weight progress. It was decided that Olivia was still at the bottom end of her growth curve - -not surprising due to the combination of Heart and Liver problems -- but that they would like to see her move up

some percentiles by her next visit. We will be starting Olivia on a higher energy formula -- 30KCal vs the 26KCal she's eating now. This will hopefully allow Olivia to put on weight faster taking in the same volume of food she has been eating.

Before we left the hospital, Olivia had to leave a blood sample for a full work-up. This blood work showed that her liver is doing 'well,' with all her liver values improving slightly across the board. The only concern at this point is that her Potassium (K) continues to be elevated. The increased BiCarbonate hasn't reduced the Potassium as much as we'd hoped. The new plan of attack now involves changing her formula to a lower mineral content formula, hopefully she will take to this food quickly w/o much difficulty.

As I mentioned earlier, overall the visit went well, at times though we felt as though not everyone was on the same page. The extent and rarity of Olivia's medical condition(s) are most likely the cause of some of the confusion/contradictions we ran into talking to the Liver team. For example the nutritionist was initially under the impression that Olivia's heart had been fully repaired, when we explained this was not the case, she had to change her assessment... Hopefully

as they all become more familiar with Olivia during her subsequent visits, everyone will be up to speed and we'll have less confusion about what's

been done or still needs to be done.

One final picture of Olivia with her new best friend: Charly -- picked up at the Chelsea Michigan Teddy Bear Factory on the way home from the Hospital.

On the Road Again... Again.... and Again.....

2009-06-15T10:45:00.003-04:00

This evening we leave for Ann Arbor for a follow-up Liver evaluation Tuesday. Luckily this is a scheduled exam. We're just heading out tonight and spending the night in Ann Arbor, to make tomorrow morning a little easier on everyone.

When we first set up this appointment, we were quite surprised to find out that it would be with the Pediatric Liver Transplant Clinic. Needless to say when we panicked a bit and called them right back. It turned out that this was just the best way to get an appointment with Dr. Lopez, it didn't mean that she was being evaluated as a transplant patient. **WHEW** The packet we received from the clinic showed that Olivia will have a full day tomorrow.

The Bigger Little Peanut will start off the morning with a fun visit to the Phlebotomist to donate more of her blood for testing. This should set the tone really well for the rest of the day.....hah. Then she'll get to meet with Dr. Lopez for her monthly liver check-up. Hopefully the news will be good again, maybe some adjustment to meds because she is growing, but otherwise we are planning for an uneventful visit. The pending Alagille genetic tests might be back by now also, so that could make the day even more interesting.

We are also scheduled to meet with a nutritionist tomorrow, which will be good and give us an idea about Olivia's feeding schedule -- i.e. how long does she stay on formula, when do we start adding solids, what solids & restriction will her condition place on her. From what we have read, heard, been told, nutrition will be critical to managing her Alagille Liver. Who knows this might even cause us to eat a healthier diet....

Wish us luck, the Little O deserves a string of good news.

A Nice Sunday Stroll

2009-06-14T21:02:00.011-04:00

Sara and I took Olivia to The Kellogg Forest for a nice stroll today. The weather was perfect, Low 80's sunny, slight breeze. We haven't taken Olivia many places just due to her condition, but mainly the fact that she has yet to have a full course of vaccines has made us reluctant to expose her to many things.

We've had a really good couple of weeks since our last Doctor's visit. Olivia has been eating well, feeling well, happy, smiling, only throwing a wobbly (tantrum) every once in a while :). She's had a couple of blood tests over the last 2 weeks, checking various kidney values. Overall the Kidney team is happy with her bloodwork, but would prefer to see her blood slightly less acidic. They have increased her Sodium Bicarbonate (again) to help fight this acidosis and to help keep her Potassium from creeping up too high, which is still sitting slightly higher than 'normal.'

Our walk was around the main loop, which is about 2.5mi lasted about 1 hour. Kellogg Forest is a Michigan State University Forestry Research facility which has many hiking/biking/walking trails. The gravel track is well traveled and goes through a number of different wooded sections, all with signs explaing their purpose. It is a very relaxing atmosphere, which quickly put little O to sleep. She didn't sleep the whole way, but was quite attentive to her surroundings most of the time.

We did run across a couple fun pieces of wildlife while out and about. Sara noticed a Box Turtle snacking on some grass and had of course had to stop for a quick Photo. The turtle became a little camera shy and quickly withdrew into its shell. While moving the turtle off the main path, a beautiful Indigo Bunting landed in a tree right above us. It didn't want to hang out with us and

flew off before we could snap a picture of it. For those that haven't been lucky enough to see one of these birds, they are spectacular.

Their feathers have and irridescent indigo sheen, that glimmers in the sun. Very unforgettable. Here's a picture I pulled off the internet showing the wonderful plumage of this bird.

A very nice Sunday with the family!

Happiness is....

2009-06-10T17:57:00.001-04:00

Spending time with Grandma. . .

Just Pictures

2009-06-09T22:03:00.008-04:00

Strapping in for a stroll around the yard

Trying on Grandma's Glasses

Finally Strong enough to sit upright in this bouncy chair

So many new things to see

Lounging with Maggie

Where's Ben's drool spot?

Top 10 Things Olivia has taught us... So Far

2009-05-31T23:02:00.006-04:00

10. Sour Milk/Formula smell becomes the colonge of choice.

9. Smile to Scream to Smile in 3.5 seconds... impressive!

8. Blonde hair hides grey very well.

7. No excuse needed to buy children's books and music

6. The cleaner the clothes, the more likely they are to get puked on

5. Never take a healthy baby for granted.

4. Everything is fascinating again.

3. A smile from a baby can instantly cure a bad day.

2. How overwhelming and instant our protective instinct is for your own flesh and blood.

1. Live in the now, enjoy every day, every moment.

Poetic Inspiration

2009-05-31T22:30:00.007-04:00

Olivia's Kansas Grandfather sent us this inspiring excerpt of a poem by the renown German poet Johann Wolfgang von Goethe. The poem is unamed, but comes from the play Singspiel (1777). I've included the original text and the English translation.

Allen Gewalten In face of all forces
Zum Trutz sich erhalten, maintaining defiance,
Nimmer sich beugen, never yielding,
Kraeftig sich zeigen, showing oneself strong-
Rufet die Arme summons the arms
Der Goetter herbei. of the God's to one's side.

Cardiologist Visit 5/28

2009-05-29T09:53:00.002-04:00

Olivia had a great Cardiology check up yesterday.

Her saturations are still up in the low/mid 80's. Her appetite continues to be good, she's up to 8# 14oz (with clothes), so close to 8# 11oz nekkid. She's almost put on 3# from birth her weight, and has actually gained over 3# from her lowest weight (5# 8oz).

Dr Dommer was very happy with her heart, so much so that Olivia didn't even get tortured with an Echocardiogram yesterday. We've been cleared for 4 weeks before we have to take her back to the Cardiologist.

Smiles & Scars

2009-05-24T11:46:00.002-04:00

Olivia on how to eat....

2009-05-23T21:28:00.005-04:00

She's really discovered her hands now and tries to stuff them in her mouth

Having trouble uploading the second video, I'll gets more up later today or tomorrow.

Ann Arbor: The Kidney Chapter

2009-05-21T17:17:00.006-04:00

"Whatcha Talkin' 'Bout Willis"

Today we took Olivia to Ann Arbor today for a follow up with the Urology/Renal Clinic. As always the appointments never turn out quite the way we expected beforehand. We saw a very nice Nurse Practitioner, who took a lot of time asking about Olivia's History and previous procedures. Overall she was happy with Olivia's bloodwork, except for her Bicarb (think Baking Soda) level, which was low -- again. It is very common for children with Alagille Syndrome to have slightly Acidic blood levels due reduced kidney function. It was decided to start suplementing BiCarbonate to aleviate this acidosis by raising her Bicarb levels.

The other semi-surprise today was that Olivia's Abdominal Ultrasounds (3 in total) taken over the last 2 months, don't all agree on the kidney findings. All the scans showed 'small' kidneys, but one -- the most recent one -- did not mention anything about a multi-cystic right kidney, while both the initial exams did make note of this finding. The possible problem here is that the Ultrasounds were not "Kidney Specific" but were more liver focused. A Kidney Specific Ultrasound has been set up for August to fully evaluate these discrepancies.

What does this mean?

Honestly we aren't sure, what we did learn from this visit :

Currently Olivia's Kidney function is good.
There have been no changes since her original diagnosis
"Small" kidneys aren't uncommon for babies Olivia's Size/Age
It will be important to closely follow her kidney's growth/development over the next years
It will be a while (2+ years) before a full determination of the full impact of her kidney dysplasia

Happy Birthday Little Peanut!

2009-05-19T15:34:00.002-04:00

Well 3 Month B-Day anyway!

We'll collect and treasure every milestone we can.

Potassium, Schmotassium. . . . . or how to get stomach ulcers in 3 hours.

2009-05-15T22:19:00.006-04:00

Olivia had a full blood work up performed Wednesday afternoon as part of her routine monitoring. Poor baby had 7 or 8 separate vials drawn, looking at Liver, Kidney, Vitamin D&E, Clotting times, Red & white cells. It took two separate draws to get all the needed blood. I thought we'd get a little prune back by the time they were done. All the while, Miss Olivia regaled all in her close proximity with her high pitched Operatic Solo meant to shatter glass.

At about 7:30 that evening we get a phone call from the GI doctor on call at U of M saying there was a problem with Olivia's blood and we need to get to the hospital ASAP to have the value rechecked/confirmed. He indicated that her Potassium level was extremely elevated (dangerously if it was true). Her Potassium was reading at greater than 7 was pushing into a range that could cause life threatening heart arrythmias. The Dr. was suspicious that the elevated value was due to lab error though and just wanted us to double check at the hospital.

We waited at the Emergency Room for well over an hour before they came to draw her blood and then had to wait for another hour or more to get her results. When the tech finally returned with her results, we were relieved to find out that Olivia's Potassium was 5.9 -- elevated, but not to any dangerous level. Most likely the way the blood was drawn earlier in the day caused some damage to the Red Blood cells. This damage can release significant amount of Potassium creating the falsely elevated value we saw. WHEW -- Dodged another bullet.

For those feline fans out there, here's a picture of some of our Cat Pride investigating a bottle that's been stolen off the table. They are really in love with the bottle nipples.

Happy Liver, Happy Parents, Happy Mum Day

2009-05-15T20:57:00.007-04:00

After Olivia's good Cardiology appointment last week, we also had a follow up appointment with her GI/Liver specialist last friday. Dr. Lopez was very happy with her progress. She has been putting on weight to the tune of 32 grams a day since her last weight at U of M. He said this rate of gain will allow her to catch up to the appropriate size/weight for her age. Standard weight gain at this point should be 23-30grams a day. He wants to continue to monitor her blood work every couple of weeks to make sure there one no negative changes to her Liver. He wants to see Olivia in Ann Arbor at the U of M transplant clinic in 4-5 weeks. As we found out earlier this week just because she's being seen at the transplant clinc doesn't mean she's due for a transplant. WHEW!!! It's just the easiest way to get to see Dr Lopez.

Last Sunday we had a nice dinner with Sara's Mum & Dad for a first of many Mother's days. As usual Sara's mom cooked a wonderful meal.

The company and conversation was also great.

(Jack we missed you we'll stop out and visit Soon.)

Olivia sat at the foot of the table smiling away. Each smile, coo, and cry reminding us how lucky we are to have our precious little girl in our lives. At times her problems attempt to overwhelm and distract us from the priceless gift we have been entrusted with to nurture and love. We just wish that everyone could be as blessed as we are to have such a beacon of hope and happiness warm their hearts. We love you Olivia.

Echo Again

2009-05-07T12:55:00.005-04:00

Olivia's Cardiologist visit of Tuesday went well. Dr. Dommer is happy with Olivia's heart function at this time. The echo shows that she does have some thinckening and enlargement of the right side of her heart, which is to be expected with her defects. The trick will be limiting this enlargement and thickening to retain adequate cardiac function. Olivia was pretty good for the ultrasound, but by minute 15 or 20 minute she was pretty much fed up with it and became quite inconsolable. This limited Dr. Dommer's ability to get a good look at the surgery site (it's on the back right side of the heart) and is difficult to visualize with ultrasound even with a cooperative patient. On our next visit (3 weeks) Olivia might get a little sedation so they can evaluate the state of her conduit.

The plan at this point is to let Olivia grow to the point where she basically 'outgrows' the current conduit -- meaning she will start having 'Blue Episodes' (Lower Oxygen Saturations 70% or so).Dr. Dommer expects this to happen somewhere between 4 and 6 months old. She is now anywhere in the range from 80-90%, depending on how stressed she is. Once her Oxygen consistently stays in the 70% range, she will be evaluated by the UofM cardiac team via a

cardiac catheterization.

If Olivia's Pulmonary vessels have grown the way we would like them to, hopefully the Cardiac Team can then attempt a full or partial repair on the internal heart defects. If the Pulmonary

Vessels are not as large as we'd like, she will just receive a larger conduit and we wait for the vessels to grow more.... Obviously we would prefer scenario 1.

Weight wise our little peanut is starting to outgrow the 'little' moniker, she is now officially over

8lbs. :)

Smile For the Camera

2009-05-04T17:20:00.004-04:00

Olivia had a good week again last week. She's been home for just over 2 weeks, a record for her :)

We did get her blood results last week (sorry, I didn't mean to keep people in suspense) it's just been busy with Heartworm season at work to get much computer time. Olivia's Liver (ALT & AST) values improved slightly, back into the 200's where they will most likely stay for the forseable future. Her Bilirubin/Jaundice levels are unchanged. Her latest weight last week was 7lbs 6.8oz, growing about 1/2oz a day now, right on schedule.

As you can tell from the picture and her previous video, Little Peanut likes to smile. She's about to start laughing in the next weeks, you can tell she's trying to put noises with the smile, but it's just not happening yet. Never fear, we'll torture you with more silly video once it happens.

She's got a big week this week though. Cardiologist tomorrow, Blood Work Thursday, and GI/Liver appointment Friday. Hopefully she'll meet all her benchmarks and get good progress reports. More as the results keep coming in this week.

Uneventful Week

2009-04-25T14:15:00.008-04:00

No changes this week at home. Olivia has taken right back to her old ways at home --- manipulating Mom & Dad to hold her as much as possible. Somehow she seems to have my number and always ends up sleeping half the night on my chest.... at least i get sleep that way.

No news yet on the blood work that was run Thursday, hopefully by early next week we'll have those results. When Sasquatch came by last week, Olivia weighed in at 7lbs 2oz !! The new formula, even though it tastes rancid, seems to be agreeing well with her.

Here are some pictures from this last week.

Chillin' in the bouncy chair

Shaking her new wrist rattle:

Striking a pose, showing off her band-aid:

Grumpy Girl gets swaddled and goes for a ride:

All that's needed for a good day; TIVO Remote & Cell Phone -- Bring on the day!!:

Happy Girl

2009-04-21T22:27:00.003-04:00

Olivia hamming it up for the camera with a little encouragement from dad...

Home Sweet Home

2009-04-19T15:27:00.002-04:00

We made it back home yesterday evening, after one final scare.

Olivia had increased her feedings nicely from 1oz Pedialyte to 50/50 pedialyte/formula to 2 oz formula over about 24 hours. She was acting great, eating well, but hadn't had a 'good' BM yet. When she finally did, Sara noticed some larger reddish/orange flecks & mucous in her stool. This was brought to the nurses attention, and was tested for blood. For the next 2 hours everyone was concerned that there was some GI bleeding that would require a longer hospital stay and more work-up. Finally the Occult Blood test came back as negative, and Olivia was free to go home. What had been seen in in the BM was color left by her multi-vitamin mix -- a fluorescent orange mix --- yum.

Olivia is still adjusting to home, it will probably take a couple days again to get into a nice wake/eat/sleep routine again.

Everytime she is dismissed from the hospital, her specialist visit list grows. She now has visits setup with her Cardiologist, Nephrologist, and the GI/Liver Specialists.

We'll keep posting as these visits come along and things change/improve.

Let's Try This Again

2009-04-18T09:07:00.002-04:00

Olivia has recovered very well since her Wed biopsy & procedures. She is back to eating full strength formula & on all her oral meds. The pediatric service wants to send her home alter this afternoon. She had some labs drawn earlier this AM, as long as they come back within her expected levels, she will be good to go :)

We got another chance to talk to Dr. Lopez -- The Liver Specialist -- yesterday afternoon. We all came away feeling quite positive about Olivia's situation. He stressed nutrition & vitamin suplementation as the keys to keeping her growing and keeping her liver as healthy as it can be. He also repeatedly stated that keeping her heart healthy and getting it repaired would be The Single Biggest Factor affecting her liver and providing a longterm positive outcome for her disease process.

Back to work, more later whe or after she gets home.

"I can call you Betty, and Betty when you call me, you can call me Al(agille)"

2009-04-16T15:20:00.005-04:00

Olivia has had a really good day. They stopped her Morphine infusion early this morning because she was having too much fun being gorked out of her mind and forgetting to breathe for herself. Once they stopped the Morphine drip, within 6 hours she was breathing on her own and was awake enough to be taken off the ventilator. So our little peanut sounds like a smoker again -- her cough is more like a 1/2 pack smoker this time :)

Dr. Lopez from the GI team did stop by and talk

to Sara and her mother while I was working today. He was "comfortable" with a diagnosis of Alagille Syndrome with our little Diva. The biopsies that were taken during surgery will most likely confirm the Doctor's suspicions. This Syndrome/Disease is mainly a management disease. We will need to find ways to support her Liver with food, supplements, & medications, there is no cure besides a Liver transplant. Dr. Lopez also stressed that the healthier we can keep her heart, the healthier her liver will be. Hopefully I will get a chance to talk to him sometime before discharge to get some more specific quesrtions answered.

I'll Post some more on Alagille this evening.

MMMmmmmm Morphine......

2009-04-15T23:26:00.004-04:00

I fear our little peanut will be a druggie before she's a year old. I might have to put double locks on the clinic controlled drugs to keep her safe :) She seems to like Morphine & Versed quite a bit.

She's still on the ventilator and sleeping/sedated after the procedure. Due to her Liver issues, she doesn't process the anesthetic drugs quite as fast as 'normal' , so she's still out of it. She should start slowly waking over the next few hours and be ready to be extubated & off the ventilator

sometime tomorrow.

She's also been reunited with her guardian angel Dr. Shah -- he brought her back after her cardiac arrest -- since she's actually recovering in the Cardiac ICU rather than the Ped ICU.

She's got a couple new scars to show off too, one through her belly button, another on the right side of her abdomen, and a small one on her right groin where the hernia was.

Now off to catch some sleep.

Out of Surgery

2009-04-15T19:18:00.002-04:00

1) Biopsy Done

2) Bile Duct Study Complete - Not consistent with Biliary Atresia - but not 'Classic' Alagille

3) Hernia Fixed

4) No Kasai Performed

5) Still No Official Diagnosis - Still most likely Alagille pending biopsy results.

Now headed back to Pediatric ICU (PICU).

I'll post more later

And We Wait

2009-04-15T15:55:00.002-04:00

Just heard from the nurse, that Olivia is doing well under anesthesia, still doing the Laproscopic procedure, haven't made a determination about the need for a Kasai procedure or not.

Just Starting Now

2009-04-15T14:05:00.002-04:00

Olivia just went to the Operating Room now. Estimated time of the procedure is about 2 hours as long as she doesn't need a Kasai Procedure. If she does need the Kasai time-wise, all bets are off.

Send good Thoughts/Prayers/Mojo her way.

Surgery/Biopsy Postponed Again

2009-04-15T09:33:00.002-04:00

Surgery has been put off until 12 this afternoon. Just a scheduling issue, not a problem w/ Diva. Will update as more info becomes available.

On Second Thought

2009-04-14T18:57:00.002-04:00

Well today was pretty much a wash. After not feeding Olivia for 7 hours and waiting for our Liver biopsy time, we find out that the biopsy was canceled. Ugh... kind of felt like a wasted day.

There are some Liver associated complications with Cystic Fibrosis, so today she was tested --- again --- for CF and for the first bit of good news in a while she did test NEGATIVE.

The biopsy has been rescheduled for tomorrow morning bright and early (7:30am). They will actually put her under general anesthesia and do a true surgical biopsy (open her abdomen). At the same time they will perform a contrast study on her bile ducts, giving the doctors a true view of the extent and connection of her biliary system. This dye study should give a difinitve answer to whether Olivia has Biliary Atresia or not.

If the test confirms Biliary Atresia they will then contiue on in surgery and perform a 'palliative' Kasai procedure to help move Bile from her liver to her small intestine.

If the dye study does not show Biliary Atresia, they will close her up and most likely the diagnosis will be Alagille Syndrome.

Tomorrow will be a big day. Keep us in your thoughts tomorrow.

Another Day of Testing

2009-04-14T08:35:00.003-04:00

Olivia gets to finish up her testing that started yesterday. She'll get one more series of pictures for her HIDA Scan. After her second series of pictures, very little to no dye had left her liver to enter the small intestine. Her final scan this morning will show if anything has moved out in the last 24 hours, hopefully there is some movement, which will show that there is movement of bile from the liver to the intestine, but just not as much as there should be.

After her final HIDA Scan she'll get to have a liver biopsy. Yipeee !!!

She'll also be visited by an opthomolagist sometime today to evaluate her for characteristics of Alagille syndrome. The majority of children with this syndrome have changes within the eye that should be evident at her age. Aliagille syndrome (while not a desired diagnosis) manages to explain a number of her issues -- Heart, Kidney, Liver, Metabolic Acidosis.

These three tests - the HIDA Scan, the Liver Biopsy, & the Opthalmic exam -- should help seperate out the two most likely candidates that are causing her liver problems.

It will be nice to just get a diagnosis so we can move on and figure out how to work with her issues.

Wait, Wait Some More, & Wait Even More

2009-04-13T14:16:00.002-04:00

Olivia had her first HIDA scan this morning and she's heading back shortly for some follow up pictures. Basically the GI team is still trying to rule out Biliary Atresia (shrinkage/disaperance of bile ducts - either in or outside the liver) with this scan. We had hoped that her original ultrasounds had eliminated this as a possiblity, but there is a version fo BA that can be progressive, developing over the first couple months of life rather than being present at birth.

She will also receive another ultrasound of her liver later this afternoon checking her gall bladder once again -- to make sure it's still present.

Poor girl has been fasted most of the day... needless to say she's NOT HAPPY and she's letting most of the hospital know, by screaming at the top of her lungs.

More later

Hangin' at the Mott

2009-04-12T22:20:00.003-04:00

So we made it to Mott Children's Hospital (I assume they're the Applesauce Mott's -- or related) this afternoon w/o any problems. Olivia travels well, the bumpier the road, the happier she is. Those of you that have been fortunate enough to travel on Michigan's wonderful post-winter roads will know that Olivia loved the ride :) Tire swallowing potholes were avoided, so both Olivia & her parents were happy.

Once we arrived at her room, the poking party picked up right where it left off two weeks ago. In the span of the afternoon/evening she's been visited by no less than 5 doctors, listening, poking, measuring, and drawing labs.

Everyone seems to be quite happy with her condition-- aside from her jaundice. Even the ICU doctor who resuscitated her dropped by because he saw she'd been admitted. He was happy to see it was not heart related :)

So far her labs have come back pretty much the same as last week's, this in itself makes us feel somewhat better because it indicates her condition hasn't progressed/deteriorated much if at all in the last 5 days.

The plan is for her procedure (HIDA Scan) to start around 10am tomorrow morning. Hopefully by afternoon, we'll have some answers, or at least the next step in the diagnostic plan. In the mean time, here's the little peanut sporting her Easter IV line.

On the Road Again

2009-04-11T22:29:00.003-04:00

We're getting things together tonight to head back to Mott Children's Hospital for Olivia's Liver work-up. It's hard to say what they will find wrong with our little peanut, but as much as we'd like to put our head in the sand, and continue as if nothing is wrong, we need to stand and face anything that is to come.

Olivia has met every challenge placed before her with a grace and vigor that Sara and I can only marvel at. The strength contained within our young daugther makes us so proud. It will help bring her through any and all these trials.

Here's another cute shot of our Diva posing with her seahorse toy (a gift from her Auntie Melinda)

Deja Vu All Over Again or Olivia! My little Yellow Friend....

2009-04-09T22:29:00.009-04:00

Olivia's Jaundice continues to get worse. The blood that was drawn earlier this week came back yesterday (Wed). It showed that not only have her Bilirubin values continued to increase, but a couple of her Liver enzymes are also on the rise. I forwarded these results to Olivia's cardiologist to keep her informed. Dr. Dommer contacted us later that afternoon with her concerns about the abnormal liver values. She had already contacted the UofM GI team about Olivia's newest Blood Values. Needless to say the GI team was not happy with the increases seen.

For all her hard work at putting on weight nicely this week, Olivia has been rewarded with another visit to Ann Arbor. She will be admitted to the

Hospital Sunday night in preparation for a fun day of poking and prodding at her Liver

on Monday. Hopefully this visit won't last much longer than a day or two until they figure out what her liver is doing ... or more correctly not doing.

Here we go again..... It's becoming quite the routine that just when we feel good about her progress, something comes along and pulls the rug out from under us again. At some point his ride better stop or we're going to go crazy. The things keeping us going are our Little Yellow Diva and the support of all our family, friends, and clients.

Please keep Olivia in your thoughts and prayers over the next days/weeks. It sure seems like we're in for many more twists and turns.

(My thanks & apologies to Blake Edwards & Peter Sellers for the title's inspiration: Cato!! My Little Yellow Friend.)

Movin' Up a Weight Class

2009-04-07T14:59:00.002-04:00

The visiting nurse (aka Sasquatch ... she has really big hands....) was by today and gave Olivia a once over. She now weighs in at a whopping 6lbs 4oz !!! Yeah!!!

More blood was drawn to check her Bilirubin & Liver values. Last weeks values had crept up again (Total Bili was up, Direct was down, Indirect was up).. go figure. Her liver values also had crept upwards from the time of discharge. Hopefully today will bring some better news on that front. Visibly Olivia looks a lot less Jaundiced than she did last week, so hope her bloodwork will follow suit.

She is eating really well, somewhere between 2.5 -4 oz per feeding -- about every 3 hours.

She was dissapointed in the basketball game last night, she was hoping for a closer affair than the blowout provided. She also can't wait to grow into her Jayhawk Bib to show her true colors.

Another Day Another Doctor's Office

2009-04-02T18:46:00.003-04:00

We took Olivia back to the Cardiogist for her 1-week post Hospital discharge visit. Everything went very well. Olivia's Oxygenation was 86% -- even while screaming -- that was a new record for her. Her Heart Ultrasound also showed no new problems. Dr. Dommer was happy with Olivia's heart function (ejection fraction -- the degree of herat contractility) , which had decreased slightly after surgery, but now is back to presurgery levels. There are no signs of fluid build up around the heart which we have been told is one of the more common complications 3 to 6 weeks post surgery. Olivia will need to see Dr. Dommer again in 2 weeks for another echo -- sounds like this will be at least a monthly occurence for the forseeable future.

We have not yet heard about her latest Bilirubin values, hopefully tomorrow when the Visiting Nurse comes we'll get a copy of the bloodwork. Dr. Dommer was also right on top of this, contacting UofM while we were there to get results w/o any success.

We have been extremely impressed and comfortable with our cardiologist (Dr. Fountain - Dommer), she has been very pro-active and involved. Sara and I truely believe that she was and will continue to be a vital part to Olivia's continued success. In that vein we will also be changing pediatrician's offices to someone that is more familiar with the special health needs of our One-Kidney, Jaundiced, Heart Defective Diva :) Dr. Dommer gave us some names of pediatricians in Kalamazoo that are more used to dealing with special needs kids.

Some New & Old Pics

2009-04-01T15:27:00.002-04:00

Happy in Dads arms

Sleeping on Mom's Lap

Not Happy about a Doctor Visit

Poke me one more time and I'll pop ya

Thumbs: The Pacifier You Never Drop

She's a Keeper

2009-03-30T16:23:00.002-04:00

Olivia is making a lot of new friends. She's had 2 different visiting nurses come and visit since she's been home, and she always seems to make quite an impression. During the first visit, Olivia decided screaming was not the only way to express her displeasure at the nurse's poking and prodding. She decided that projectile pooping across the floor during the exam might give Heidi a clue about her state of mind.

Her vitals are doing very well during these visits. Her Oxygenation is staying in the upper 70's -- which is acceptable for her condition but 'lousy' for the average human -- for a healthy human we'd like to see oxygenation at or above 95%.

She's also gaining weight, which as we've been told can be a struggle for cardic kids. She is now officially 6 lbs 1 oz -- which means we don't have to toss her back. :) Her appetite is excellent, there are no signs she's slowing down at all.

She also has had more blood drawn to keep an eye on her Liver & Bilirubin values. Hopefully those results should be back in a day or two.

I'll get some more pictures up in a day or two of her chillin' at home.

Have Bottle Will Travel

2009-03-26T10:16:00.005-04:00

Olivia's first night at home went as smooth as we could have hoped for. She has quickly fallen back into her Eat/ Sleep/ Waking pattern she had established before this little heart thing interrupted our Family Time. We're so glad that she has given up 1am - 4am as her prime Cranky Time(tm). Her visiting nurse is supposed to come by today to give her a first checkover, as long as it's not every 2 hours we're cool with that.

Now it's a mad scramble to clean a house that has been run by pets for almost 3 weeks before the nurse gets here..... no rest for the wicked eh?

Most likely posts will not come as frequently for a while. As lab results trickle in I'll post about them. I'll also try to get some good video/audio of the peanut's scream, it is something to behold & fear. Our next milestone will be 1 month check-up in Ann Arbor with the GI team.

Now to get back to some semblance of normal life.

2 weeks, 6 days, 2.5 hours

2009-03-25T15:58:00.002-04:00

Note the Absence of a Hospital Gown:

Rummor has it.....

2009-03-25T08:48:00.002-04:00

Rumor has it that our little Diva gets to fly to coop today. I'll believe it when I se it.

None too soon either, I think one more day of nurses poking her repeatedly through the night might have resulted in someone needing a real get out of jail free card. Why was it every time we'd get her settled down and sleeping that it is all of a sudden time to "Check her Vitals" again......

Who Needs Two Kidneys Anyway

2009-03-24T12:18:00.003-04:00

The Kidney study to check for urine reflux into Olivia's good kidney was done this Morning. From the sounds of it, the Nephrologists seem to be into some strange form of S&M. After placing a urinary catheter, they strapped her to a contraption akin to a Medieval Rack. I feel terrible I wasn't able to provide moral support to Sara and Olivia during this torture escapade. Her head, torso, arms and legs were strapped down with Velcro ties, while they forced a dye up into her bladder to see if it would reflux back to the kidneys (a no no). All the while Olivia is flexing her vocal range and squeezing Sara's fingers :(

Good news is that on first blush there is no reflux from her bladder into her good kidney. There was reflux into the bad kidney one time, which most likely explains what happened in Utero to shut the kidney down. The Nephrologist wanted to review the films more closely, but felt good about the results.

Olivia also had another abdominal ultrasound performed this morning (before being strapped to the rack), no news yet on the results, but the GI team doesn't expect anything different from the initial results.

Don't Call Me Yellar

2009-03-23T09:43:00.004-04:00

The last couple of days have been nice and uneventful. Sara and I have been trying catch up on some sleep. 2+ weeks of bedside vigils are taking their toll. My cold is finnaly gone only to be passed on to Sara and her mother. Now if my gut hadn't managed to pick up a nice hospital bug I'd be wonderful.... hahaha. Sleep has become a very precious and elusive commodity never again will we take a nice soft bed for granted.

As I mentioned in the previous post, Olivia's Heart Doctors are very happy with her progress and want to get her home ASAP. Only now her Bilirubin values have been creeping up again. The Dr from the weekend wants to get the GI service involved again to make sure nothing was missed. Olivia looks great, feels great, eats great so hopefully this is just a remnant of all the medications she's had pumped through her little body in the last 2 weeks.

I'll post when we hear from the GI people (hopefully later today).

Make Hay While the Sun Shines

2009-03-21T10:18:00.002-04:00

Everything's good. Just tryhing to catch up on some much needed sleep while she's content. I'll post more later today.

One Less Tube is A Good Thing

2009-03-20T10:09:00.002-04:00

Olivia is slowly shedding her extra appendages. Her chest tube and pacing wires were pulled this morning. She was not very excited about the 6 hour fast before the sedation/procedure -- and let us know it throughout the night. She was quite the Fussy Pants, Pedialyte just doesn't seem to cut it when she's hungry.

Later this morning she is supposed to get one a final Echo to make sure everything still looks good. If she doesn't hold still they'll have to wait until monday to give her more sedation. Hopefully she cooperates today :)

I C U Later

2009-03-19T10:45:00.004-04:00

Morning rounds went well. The ICU Cardiac Team is comfortable enough with The Peanut's progress to ship her off to another ward today. Just waiting for a bed to open up - probably sometime this afternoon.

Here's the latest in baby Haute Couture - Oxygen

Nasal Canula, Feeding Tube, ECG Electrodes, Butterfly Tape Sutures, all tied together nicely by a light cotton twill wrap with hospital striping. Watchout Fashonistas there's a new kid in town:

I C U Later Aligator

The Sun WIll Come Up Tomorrow, You Can Bet Your Bottom Dollar.....

2009-03-19T08:34:00.002-04:00

Now that I have placed that wonderful little ditty in everybody's head for the day -- you can thank me later -- Olivia had a pretty uneventful night. When we saw her last night, she ate a little for us, but mostly was resting after a stressful day.

I guess she was not a very cooperative patient during the Echo, had to be restrained by Mom & Grandma for about 45 minutes to get all the pictures they needed. Her subsequent blood gasses showed that she had exerted herself quite a bit. Ever since then her values have improved with each lab. Her Kidney values continue to improve, but her Bilirubin values have crept back up. Hopefully with regular feedings those will come back down, as they had before surgery.

She slept most of the night, had a couple hour spell where she was not very consolable, but finnaly settled down after they angled her upright in the bed, possible gastric reflux making her cranky. She was sleeping peacefully when I said "See Ya Later" to her this morning. Hopefully today can be another day of rest and recooperation and possibly moving to a general ward.

Luckily I'm only working 1/2 day today, so I get to see her this afternoon.

Hungry Hippo

2009-03-18T15:52:00.003-04:00

Needless to say the Echo was good. I guess that's the unoffiocial word at this time, but there don't seem to be any issues withe surgery site. Las t I heard she was eating like a little piggy again too.

Rumble Strips!! Wake UP!!!

2009-03-18T12:54:00.003-04:00

As I mentioned earlier, Olivia had quite a fussy night. She just didn't want to settle down, didn't want to eat, Blood Gas values off somewhat, and running borderline fever (100.7). During rounds this morning they mentioned being concerned about infection -- UTI? -- Intestine? -- They had changed her from broad spectrum Vancomycin to Cefazolin antibiotics yesterday, so maybe something isn't responding to the med changes?

They have done another Cardiac Echo this morning, making sure the repair looks good, won't have results until later today. They have changed her antibotics back to Vancomycin. She has been recatheterized to get a urine culture. A blood culture has also been taken.

Since they've done this work-up, Olivia's been sleeping in Sara's arms. Temp is 97.0, Bloodgas values have returned to normal. All she needed was mom's touch :)

Hopefully this little hiccup today was just that, a little warning from our Diva to not get over confident.

As long as the light we're seeing at the end of the tunnel isn't a train... we're happy.

As soon as I hear about the Echo I'll pass the news on.

Eewwwww That's Not Breastmilk

2009-03-18T08:27:00.002-04:00

I quickly checked on Olivia before I left Ann Arbor this morning. I guess she had a pretty fussy night. She never really settled down well and cried a lot. She finished all the pumped Breatsmilk last night and wanted no part of the Formula they were offering her. They did switch the formula type so it very well could be a flavor issue.

Her Oxygen values weren't as good overnight as they had been during the day, and her Lactic acid in her blood had built up again also. Both of these can be explained by her crying and fussing all night, hopefully Sara can manage to settle her down this morning and return her to yesterday's more comfortable level. Hopefully the chest tube can come out today which could be causing a lot of her discomfort.

It was tough again to leave this AM to go to work, especially since she's going through another rough patch. I didn't get a chance to talk to the Drs, but hopefully Sara will find out more of the plan for the day during Morning Rounds.

Keep the good thoughts coming, Olivia still needs them.

No My Name is Not DiGeorge

2009-03-17T14:55:00.002-04:00

I just contacted Olivia's original cardiologist in regards the Genetic testing that was performed in Kalamazoo. She does not have a deletion in her Chromosome 22 @ 22q11.2 also known as DiGeorge Syndrome.

U of M has submitted a more sensitive test to detect smore specific gene mutations rather than the large Chromosome22 deletion. Now we wait for the more specific results, but overall this is another great development.

Long Distance Update

2009-03-17T11:44:00.003-04:00

I miss my babies :)

I talked to Sara this morning and everything is going very well. Olivia is now on ad lib feeding. As long as she is meeting her minimum amount set by the Dr. they will not have to tube feed her. Right now the plan is to keep in in the PCTU one more night and then on to the general ward. The Drs feel comfortable enough to send her to the general floor today, if they need bedspace tonight.

Here's one more picture: Back in Mother's Arms

Feed Me... Feed Me NOW!!!!

2009-03-16T22:34:00.005-04:00

It's been a very boring day in the grand scheme of things today, but as we've quickly learned; In Cardiology "Boring = Good"

As you know 'Little Diva Olivia' had her breathing tube removed this morning and has been respiring on her own since then. She surprised many of the Nurses and Cardiologist by her speedy recovery so far. Hopefully all our steps will continue to be forward.

She has been started on Breastmilk again (through her fedding tube), but has been taking Pedialyte via a bottle. She still has quite a good suckling reflex going. We've been told that frequently after having a breathing tube in, they 'forget' how to suckle or are too uncomfortable to try to suck, not our little piggie!!! She has been complaining all day that we're not feeding her enough.

If she tolerates her small feedings via NG tube, we can hopefully start feeding her milk via a bottle in the next 24 hours. This puts us one step closer to leaving the PCTU (Pediatric Cardio Thoracic Unit -- Carciad ICU) and heading to Moderate care. Yeah!!!

On a seperate note, you might have noticed that

we haven't posted any recent pictures of Oliva. Sara and I decided that the pictures of her on a respirator and with her chest open were too

private & personal to be placed on a Public Blog. There is no need for our teenage Oliva to be haunted by such pictures in the Public Domian. So to prove our little Peanut is doing well, here's a shot from this morning w/o breathing tube...

I'm writing this from home, because I'm resuming work tomorrow, its going to suck not being at her bedside to watch her continued improvment. I also feel bad for leaving Sara alone to deal with any issues that come up. Thank God for Sara's mom, she's been invaluable to us in our time of need!! We love you!

I'll have to encourage Sara to post any new developments to keep feeding our Blog Junkies thier Olivia Fix. I will probably also do a post about Congential Heart Disease and what we can do to help fight this common problem. For now Good Night & Sleep Tight

Our Little Smoker

2009-03-16T10:24:00.002-04:00

They just pulled her tube about an hour ago and she's doing great. Oxygen levels staying around 80. Lungs sound good. Blood Pressures good. Very positive development.

Now that the tube is out she's got a nice little hoarse voice. A cough that sounds like she smokes a pack of unfiltered cigs a day. Poor baby.

As long as she continues this progress, she will start feeds this afternoon. As long as she tolerates feeds, they will try to wean her off her Calcium. Once she's off the Calcium drip she can be moved to Moderate care. Hopefully this will happen in the next 24-48 hours.

Training for the Marathon

2009-03-16T07:21:00.002-04:00

Overnight Olivia did very well. They replaced one of her IV lines in her groin - they went from a double lumen catheter to a triple lumen catheter so they could start her on IV nutrition. She was slightly sedated for the procedure, no adverse effects when it was done, bonus that she was then getting IV food.

She also did one more Sprint training off the respirator for an hour and did very well. While she is still on the ventilator, most breaths she is initiating on her own. The vent is set to 16 BPM (Breaths per Minute), but she's breathing at 28 on her own. She gets one or two more sprint training episodes later today before they pull her tube and let her run her own marathon.

She is now off all meds to keep her BP up, she is still on the vasodilator for her peripheral circulation. Not sure if this is something she'll be on long term or not. She has also been off all her diuretics since yesterday evening and is about even for fluid in & fluid out, which is right where she should be.

Hopefully the steady progress continues and we don't get any steps back.

Here's to another good day!!!

Peeing like a Racehorse

2009-03-15T19:44:00.003-04:00

Olivia is doing wonderful. She's now only on one BP medicine to keep her pressures up and one to dilate her peripheral circulation. She's down lowest dose of Morphine before weaning. Her pressures have stayed right in the mid to upper 50's, right where they want them .

She has also been taken off all Diuretics for the time being because she is urinating like crazy. (which is good, but again too much can be bad). The Cardiologist suspects that her one 'bad' kidney actually produces urine, but is indiscriminate about what is being peed out. As infants with these cystic kidneys age, the kidney continues to shrink and eventually disappears, leaving all the work to the one healthy kidney.

As I write this they are preparing to start her first 'sprint' on the respirator. She'll be almost turned off the respirator trying train her to breath on her own again. They'll do this three or four times over the next 12-24 hours. As long as she responds well, she'll come off the respirator tomorrow.

Probably won't post until tomorrow morning unless things change dramatically.

Thanks again for reading and keeping us in your thoughts.

Olivia, Sara , & Ben

She's a Champ

2009-03-15T13:38:00.003-04:00

We just got back to see Olivia. She made it through without any problems. Her BP have actually come up nicely so they will probably wean her off her Epi & Dopamine over the next 12 hours. Her Blood Gas Labs look good.

When Sara and I came back and said 'Hi Olivia' she slightly raised her eyebrows and we could see a slit of eye under those tiny little lids. It's amazing how she responds to our voice even while still sedated. I just can't imagine not having someone she knows by her side while she is in the Hospital. There seem to be too many kids here that don't have family around them all the time. It can't be healthy for them to be 'abandoned' like that. I understand that life must go on (work, other kids, etc) but they know if you are there or not, even at this age.

We were also quickly reminded how fragile these little ones are. The bed next to Olivia is now empty...... if our worst thoughts are confirmed our thoughts go out to her family.... so sad.

Doing well but still waiting

2009-03-15T12:38:00.003-04:00

I just talked to Olivia's nurse, things are going great. Her pressures dipped slightly (expected) when her chest was closed, but they have since come back into the 50's w/o adding any medications. They have also turned her ventilator down quite a bit, hoping to wean her in the next 24 hours.

They haven't opened the ward back up yet, making us worry for the baby next to Olivia, her procedure should have finished 1 hour ago.....

The nurse was nice enough to let us know the delay didn't have anything to do with Olivia :)

We'll update when we're back with the baby.

Closing Her Chest

2009-03-15T10:04:00.002-04:00

Moring rounds went well. The whole team was happy with her progress, so they decided they will try to close her chest this morning.

This procedure is done right on the ward, it's set up to be a sterile surgery room if necessary. The closing shouldn't take more than 1 hour. We'll probably have to wait 2+ hrs to see her again, because they were going to close another chest right after Olivia's closure.

Olivia's Kidney values are also pretty good, her Creat has crept up to 1.0 from 0.9 yesterday (normal = 0.5). The Renal team suspects she will creep up a little more before she comes down, because of her arrest episodoe the other night. Overall they seemed comfortable with her kidney function and think things should recover to baseline as long as no new insults occur.

Ahhh Sleep

2009-03-15T07:34:00.002-04:00

Sara and I finally got a good 6 straight hours of sleep (in a bed) last night. That's the first time in over 10 days. Feels like I could sleep another 12 easily.

Olivia did very well overnight. No changes. Her pressures remain unchanged from last night. She's down to the lowest doses of BP meds they use before stopping. They probably won't change anything more until they close her chest.

She's been urinaring very well overnight. So well in fact, that she's back to her 'dry weight' - weight before surgery & fluids. This means she could probably have her chest closed earlier than they first thought. Whether they decide to do so today, we'll see what the surgeons think during rounds this morning. Either way they are going to have to back off her diuretics (water pills) or they will make her too dry and have blood pressure issues that way. ...what a fine line ...

Here's to a Quiet Night

2009-03-14T23:11:00.002-04:00

Well so far so good. Continuing to wean drugs down. She's now been off the Vasopressin for 30 minutes and has recovered nicely. Every change in meds has been met with her little body picking up the slack.

No other changes planned for the night.

Just letting her body sleep and heal.

Good night my sweet, see you tomorrow.

"Status quo, you know, that is Latin for the mess we're in." - Ronald Reagan

2009-03-14T18:16:00.003-04:00

It's been a good day. mostly uneventful. Slow weaning and tweaking of medications has left her with pressuresa of 89/38 with MAP 56. Down some from earlier, but she's been reduced on two blood pressure meds and increased on a Vasodilator to improve her peripheral circulation.

Rythms are good, Urine output is increasing, Chest drainage is normal. Heart no need for pacing machine. Hopefully this contibues through the night. As long as things continue to look stable we'll try to get a longer night's sleep than yesterday.

The plan is to try and close her chest Monday, as long as she continues to urinate well and get rid of her excess fluid.

Earlier Olivia woke up a little while Sara was with her, opened her eyes and was looking around. Our little Peanut Diva is still there, underneath/behind all the tubes and wires.

Keep up the good work Olivia, slow and steady wins the prize.

It's All About Blood Pressure

2009-03-14T11:25:00.003-04:00

Well Olivia didn't have any more complete crashes after her episode last night. Watching the nurse fill out a Cardiac Arrest Record for her was just bizzare. The good thing was that she never fully arrested, it was just that her BP and HR kept dropping to the point chest compressions were necessary to keep things circulating well.

Through the night she had a couple more dips in BP resulting in two more IV medication drips and a Rythm Pacing Machine. Since they started the Vasopressin at 4AM she's been extremely stable around 95/40 with a MAP of 60. The goal for Olivia is to keep her MAP (Mean Arterial Pressure above 45).

The last 6 hours have been very good. They have turned down the Heart Pacemaker so she is actually keeping her own rythm. They have also started to wean her off the Vasopressin drip. So far she's keeping things up on her own.

While she's still on the ventilator, she's now taking a breath on her own every minute or two.

The next big task of the day for the little Peanut is to get her kidney function back to where it was before surgery. Her Creatinine was 0.5 before surgery, now it has jumped to 0.9. They've given her a couple of diuretics to try and get some better kidney output. Most likely the Creat will continue to go up for another 24 hours before it levels out, and then hopefully goes down.

Olivia earned a new nickname from the staff last night : DIVA. She didn't want to share any of the attention with any of the other patients, it was all about her last night. Oh Boy....

Hopefully it will be an uneventful day, slowly weaning IV drips (12 different ones at last count) and getting her to pee. The Dr's are thinking of closing her chest Sunday or Monday depending on how much fluid they get out of her system.

More later as things progress.

In lieu of a picture of her all wired up, here's a pic from 1 week ago. Man... I just want to be able to cuddle her again.

Sleepless Nights

2009-03-13T23:54:00.004-04:00

I have a feeling it's going to be along couple of days. Overall she's been doing pretty well, she seems quite sensitive to any changes right now. Small changes in meds or fluids send her blood pressures moving from one extreme to the other.

She just had a pretty bad pressure crash after stopping one drip. They ended up asking us to leave while they worked on her. Not a fun 30 minutes. They had to bolus significant fluids and start an epinephrine drip. They also hooked her up to a pacing machine to make sure her Heart Rate doesn't drop below 100. The doctor did say they had to do some chest compressions to keep her pressures up while the meds kicked in. She stablized fairly well after that.

We're back in with her now and gave her a stern talking to about pulling anymore crap like that. She's stable again, but not giving us any rest.

Keep the positive thoughts coming our way. While the surgery was a HUGE step, the next 24-48 hours are even more critical for her boby to start to adjust to the new blood flow and all the trauma the surgery induced.

How Surreal Life Can Be

2009-03-13T17:12:00.002-04:00

We've been by Olivia's side for the last couple hours and she's a real trooper. Her pressures are stable (as much as they can be at this point), her Oxygen Saturations are good, ECG is stable. She's actually on quite a low dose of heart meds to help keep her blood pressure up, all the Dr's and Nurses seem happy with her condition given what she just went through. Whenever an alarm sounds on her monitors, there someone there right away to analyze and react if necessary, very impressive.

Here's this 5lb infant surrounded by 100+lbs of equipment. IV pumps, Breathing Machine, Chest Tubes, & Drains. Her chest covered with a clear yellow plastic drape, her little heart beating just below the surface....

Never in a million years would Sara and I imagined ourselves in this position, it was just last Thursday afternoon when we were heading in to a 'routine' cardiac ultrasound to evaluate a mild childhood murmur. Huh.... that seems ages ago. Everything has stopped for us while the world around us continues undaunted. We hadn't even stepped outside for close to a week until we went home for a couple of hours yesterday evening.

We'd again like to thank everyone for beimg so supportive and helping out with everything. We'd also like to thank any of our clients following this Blog for their understanding, hopefully whithin the next week the clinic schedule should return to some semblance of normal.

Thank You.

She's Done

2009-03-13T13:20:00.003-04:00

Olivia did well.

Recovering in ICU.

Will hopefully get to see her within the hour.

So it sounds like the last hour of surgery was just watching for bleeding, placing new stitches and watching. They weren't able to close her chest due to the swelling, but it sounds like that is not unusual. They plan on closing the chest in a couple of days.

Just waiting

2009-03-13T12:17:00.002-04:00

Still in Surgery, watching to make sure there is no bleeding at site of the shunt.

One spot was oozing, but they seem to think they have it controlled.

Still doing well.

Off Bypass

2009-03-13T11:40:00.001-04:00

Surgery still going well.

Off Bypass.

Will be closing shortly.

Hope to see her in a couple of hours.

1/2 Way There

2009-03-13T10:50:00.000-04:00

1/2 way done.

Shunt is in R side. Now attaching to Pulmonary Vessels.

Another 1/2 hour or so of stitching, then she comes off bypass.

hopefully done by 12 12:15

Then the real work of recovery begins

Everything going well

2009-03-13T09:42:00.002-04:00

Just got the first update.

Anesthesia induction went well, just placed on bypass, everything going well.

And the waiting begins

2009-03-13T07:35:00.003-04:00

7:30am we handed over our precious little bundle to the care of the best team we could hope for. Please keep Olivia, The Surgery Team, & Us in your thought this morning.

Our little fighter, puttin' up her dukes:

1st Surgery of the Day

2009-03-12T22:07:00.003-04:00

Olivia is scheduled to have surgery first thing in the morning. Anesthesia will come get her around 7:30am EST. If all goes well she should be back in ICU by early afternoon.

I'll post whenever I get the chance, might not be until after the surgery.

Thank you so much for all your comments, I haven't had much time to answer many of the messages, but be assured we do read them and they have brought comfort to our hearts and tears to our eyes.

We love you all. Pray for little Olivia tonight!

What's one more conern?

2009-03-12T09:19:00.002-04:00

So we had a visit from the Pediatric Kidney team yesterday. They were called in because it was noticed on ultrasound that one of Olivia's Kidneys is Cystic and Hypoplastic (small). They are not sure what the implications of this are finding because overall her kidney function seems good/normal. The Doctors did state that functioning on one kidney is more common than forst thought, often it is an incdental fnding while working up other problems.

They of course want to monitor this, especially since one of the complications of being on a bypass machine is to have an acute kidney reaction shortly after surgery. The best newsconcerning the acute kidney problem is most often a short term treatable/recoverable issue. The Cardiologists & Kidney specialists will monitor her closely in the Cardiac ICU after surgery for any kidney complications.

More later after we meet with the Cardiac Surgeon.

Olivia's Specific Anatomy

2009-03-11T22:19:00.008-04:00

Thanks to Dr. Thorsson for the freehand drawing of Olivia's Anatomy.

The left picture shows Olivia's Collection of defects. Specifically the Atretic/Non-Existent Main Pulmonary Artery. This defect was caused by a failure of the Aorta to migrate to the correct position over the Left Vebtricle. The 'middle' migration of the Aorta basically squeezed out the atachment site for the Pulmonary Artery. Amazing how a relatively small change in developmental movement can have huge consequences for life

The right picture shows the the planned shunt going from her right ventricle to the junction of her Pulmonary Arteries. This should increase the blood flow through both sides of the Pulmonary Vasculature, hopefuuly stimulating her undersized vessels to grow.

Surgery Date Set

2009-03-11T16:32:00.002-04:00

Olivia does have a Surgery Slot set for Friday 3/13/09.

We do not have a time yet, we'll post as soon as we know.

We'll also have some other tidbits to pass on & talk about Tetralogy of Fallot / Pulmonary Atresia Anatomy a bit.

So Let's Talk Anatomy

2009-03-11T14:17:00.006-04:00

So what exactly does Olivia have?

I know I've linked to the general pages with some diagrams, but even in our mind its still dificult to envision exactly what's going on.

So let's start with Tetralogy of Fallot. As the name suggests, there are 4 components to this group of problems.

1) VSD - Ventricular Septal Defect - A hole in the wall between the Right and Left Ventricles allowing mixing of Oxygen rich Red blood and Oxygen poor Blue blood

2) Thickened right Ventricular Wall -- due to increased pressures and increased work by right side of heart

3) Overriding Aorta -- Aorta has moved from the left side of the heart to the sight side sitting right over the VSD.

4) Pulmonis stenosis - Outflow obstruction of the Pulmonary artery coming from the right side of the heart.

In general terms this cardiac defect allows mixing of Unoxygenated Venous Blood (Blue Blood) to mix with Oxygenated Arterial Blood (Red Blood). This decreases the amount oxygen that is delivered to the rest of the body resulting in the classic "Blue Baby" symptoms. Olivia never showed these Blue or Cyanotic signs because of the collateral circulation going to her lungs. As She aged though, she would have started showing more and more symptoms.

In Olivia's case she not only has the Pulmonic Stenosis, but she has Pulmonary Atresia where her Pulmonary Artery has actually alomost completely failed to develop. She is getting no forward outflow out of her right Ventricle. All her bloodflow is coming from her Left Ventricle.

The Pulmonary Artery plits into right and left branches to go to the right and left lung sides. In Olivia's case, she has under developed pulmonary arteries to both the right and left side. The good news is that these vessels are continuous, ... small but continuous.

The general plan for surgery is to place a shunt (gortex) from the top of the Right Ventricle to the junction of her R and L Pulmonary Arteries. (I'll post a drawing the Cardiologist did a little later.

Two Steps Forward One Step Back

2009-03-10T21:14:00.004-04:00

Sara and I met with the Cardiologist earlier this evening and didn't quite get the news we had hoped for. Olivia will need a B-T Shunt (or version thereof) to provide adequate blood supply to her lungs.

The colaterals that seemed adequate on the echo, after being visualized in 3-D on the MRI were a dissapointment. One was "kind of satisfactory" and the other was "puny." This turns out exactly why they wanted an MRI done - to be able to visualize in 3-D vs just the 2-D Ultrasound pictures. The cardiology team was not comfortable that the existing collaterals would allow her to grow to an acceptable size for surgery.

Since Olivia's main Pulmonary Artery exiting the right side of her heart running to her lungs, is pretty much non-existent, the branches off this main vessel that feed the right and left side of her lungs are also extremely underdeveloped. The Cardiology team feels that the sooner we ca

n restore 'adequate' blood flow to her minimal pulmonary areteries, the better our chances will be to stimulate them to grow the way they should. Hopefully the re-establishment of normal pressure gradients will also cause stretch in these vessels , leaqding them to respond the way they would have, if her main Pulmonary Artery would have developed. The window to get these vessels to respond starts to close soon after birth, so in Olivia's case, doing surgery soon, is better than waiting 3-4 months.

The Cardiology Team, Cardiac Surgeon, & Radiologist are planning to meet sometime tomorrow morning to discuss treatment options and will devise a surgical plan for our little Angel.

This could happen as early as Thursday, but more likely Friday or Monday.

MRI Complete

2009-03-10T18:24:00.002-04:00

Olivia is recovering from her anesthesia today. She was out for almost 4 hours while they did the MRI study. The anesthesiologist said Olivia did excellent, no problems at all while she was under. She woke up crying and swinging her arms :) That's our girl.

We should have some info about the MRI later tonight.

This little 5lb girl is jumping through hoop after hoop without difficulty so far!

I'll post once we get some MRI results.

MRI Time

2009-03-10T12:24:00.001-04:00

Well she's out and the MRI people are doing their thing.

Positive thought s please !

MRI Day

2009-03-10T08:56:00.003-04:00

So today our little one has to go under general anesthesia for the MRI later this morning. She's pretty darn cranky right now, she hasn't eaten in 5 hours because of the upcoming anesthesia -- sugar water on a pacifier just doesn't cut it.

On the genetic testing front, even though the official tests haven't come back yet but there were a couple of good developments. The ultrasound showed that she has a well developed Thymus which is frequently absent in DiGeorge Syndrome -- this doesn't rule it out yet completele, but with her lack of classic facial features, normal Calcium levels and presence of a Thymus are three good signs that DiGeorge is unlikely or only a milder form if present.

The Gastroenterologist also believes that Alagille Syndrome is unlikey due to severity of the heart defect. Usually the heart defect in this syndrome is minor while the Liver abnormalities are significant. Another positive :)

... but we'll have to wait for all the testing to be definitive, right now though our little Peanut is passing tests right and left :)

Thank You

2009-03-09T21:12:00.003-04:00

Sara, Olivia & Iwould like to thank everyone for all your support. The last 5 days have been quite an emotional rollercoaster. All the messages, e-mails, prayers, visits, and calls have helped smooth out those rough bottoms helping us to the next peak.

Not to get too far ahead of ourselves, but over the next days, weeks, months, years there will be many more trials for us and we will continue to need you help. Please bear with us if some messages go unreturned for extended periods, we still love you all, it's just that we might be preoccupied :)

Thanks SO Much!!!!

Love,

Olivia, Sara, & Ben

Some Photos from the last 24 hours

2009-03-09T21:00:00.003-04:00

Looking cute with her feeding tube:

Sporting her new right handed IV Club

Loaded up for the ride to Ann Arbor

Sleeping comfortably