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.

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!

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.

Cardiologist Visit 5/28

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.

Ann Arbor: The Kidney Chapter

"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

Another Day of Testing

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.