Farewell 2009

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

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!

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

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

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

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

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

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)

... 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 Disco Crib Experience - 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

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

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

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.

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• 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!
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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

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.....

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.....

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

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.....

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

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....

Spending time with Grandma. . .

Just Pictures

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

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

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.