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.

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.

Smiles & Scars

Olivia on how to eat....

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

"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!

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.

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

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

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

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.