She's a Keeper

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

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

Note the Absence of a Hospital Gown:

Rummor has it.....

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

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

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

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

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

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.....

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

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!!!

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

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

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

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!!!!

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Surgery still going well.

Off Bypass.

Will be closing shortly.

Hope to see her in a couple of hours.

1/2 Way There

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

Just got the first update. 

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

And the waiting begins

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

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?

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

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

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

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

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

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

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

Positive thought s please !

MRI Day

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

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

Looking cute with her feeding tube:

Sporting her new right handed IV Club

Loaded up for the ride to Ann Arbor

Sleeping comfortably

So it's been a good day

The results of the Echocardiogram confirm the findings of the cardiologist in Kalamazoo, but they are very 'happy' with the results. Olivia has excellent heart function/contractility. She has good blood supply going to her lungs -- though from the wrong blood vessels. Although her pumonary arteries are small, they are continuous -- meaning she doesn't have any missing segements which would mean more difficult future surgeries. 

The good blood supply to her lung come from what are called collaterals -- branches coming off her Aorta that have developed in response to her body's need for lung blood supply. The benefit of these collaterals is that it very possibly eliminates the need for an early surgery. They will confirm the extent and location of the collaterals, as long as blood is distributed evenly to the lungs she will not need a B-T Shunt.   YEAH!!!  

The MRI will give the Surgeons a good road map for any possible surgical intervention. *Fingers Crossed* for  another good result tomorrow.

Developing plan of attack

Olivia was just visited by a herd of MD's and Students during morning rounds. Echo will follow sometime today with an MRI tomorrow. There is the possiblity that as long as she does have good collateral bloodflow to the lungs, she might not need the initial shunt surgery. If that is the case and all else goes well, she would go home for observation and to grow to get ready for surgery at a later date.

Since a lot of the severe defects like this tend to group with other diseases they will test her for a number of different syndromes that are sometimes associated with her Tetralogy & Pulmonary Atresia defect. The two big syndromes being named are DiGeorge Syndrome  or Alagille Syndrome. Genetic tests have been sent out for DiGeorge Syndrome and I think they will sumbit samples for Alagille also.  

In quick consultaion with the Gastroenterolgist today, he does not believe that Olivia has any major Liver or Bilary disfunction. He did put in a request for some additional labs and a Liver Ultrasound to make sure. He believes that her liver function elevations are secondary to her cardiac issues.

The Echocardiagram has been done, but ahven't talked to the Dr's yet about results. Will post when available.

Settled in at U of M

The three of us have settled into U of M nicely today. Olivia slept the whole way over in the Ambulance with no problems. We've met with the first team of Cardiologists and they are happy with her situation/stability.  Olivia has actually been off oxygen since she got here and has done very well. She is set to have a second Echocardiogram tomorrow morning. She's also set up for an MRI for Tuesday to evaluate her lung blood vessels to allow planning for surgery to start.

UofM has a nice Hotel as a part of the Hospital - we were able to get a room there for at least tonight, and hopefully a few more. Now off to get some sleep in a bed ... first time in 4 days!!!!

We have a Bed

Jus trecieved word that a Bed has opened up in Ann Arbor. Collecting our things as we speak and will be transporting Olivia to her new room at UofM shortly.  More Later!

Happy Birthday Olivia 2/19/09

Olivia Anne Huelsbergen was born Thursday 2/19 tipping the scales at whopping 5lbs 14oz.

The first 36 hours in the hospital were very unremarkable, each member of a new family getting to know each other.  Feedings, diapers, crying, burping, little sleep, everything thst goes along with adjusting to a newborn.

A Trip to the Pediatrician 

Aside from a slight case of  

Jaundince on her discharge exam from the hospital on 2/21, the on-call 

Pediatrician did note a mild heart murmur (Grade 2/6). A chest X-Ray was ordered along with Blood Pressures, which both came back normal.  Up to 60% of newborns can have benign or functional murmurs at the time of birth, so no one was overly concerned due to the 'Lower Grade' of the murmur and the normal radiographs.

We took our little bundle of joy home to start a new chapter in our life as a family of 3 ( + Zoo of course). Olivia quickly helped us settle into our new roles as parents. She cried when she had a reason; hungry, dirty diaper, burping, comfort.  When she was clean, dry, fed,

 burped or held she was content.  She'd sleep 2-3 hours at a stretch, then let us know when she was ready for more. Af far as we could tell she was a healthy and happy Newborn; Eating, Sleeping, Drinking, Pooping, Peeing, and Crying.

On day Monday 2/24 we took Olivia for a follow-up visit with our Pediatrician to evaluate her Jaundice and overall well being after leaving the hospital. At that time Dr. Lee confirmed a Grade 2/6 systolic murmur and felt her Jaundice deserved some follow up bloodwork.  Dr. Lee did reccommend arranging a Cardiologist consult to make sure her Murmur was benign. 

Her labwork continued to show elevated Bilirubin levels, both Direct Bilirubin and Total Bilirubin. The contined elevation of these values, especially her Direct Bilirubin suggested close monitoring for any Liver/GI issues with a possible Gastroenterologist consultaion.  Olivia's (aka Little Peanut's) medical chart was starting to take on a life of its own at 10 days old.

A Trip to the Cardiologist

For Olivia's two-week birthday (3/5/09) instead of a big party, She wanted to see a Cardiologist, to make sure everything was ok. We took a sort drive to Kalamazoo to Bronson Hospital for an echocardiogram.

 To our shock and suprise the echo revealed that Olivia's heart mormur was associated with a quite severe Congenital Heart Defect (CHD) called Tetralogy of Fallot. Our Little Peanut wasn't done with surprises yet, because the ultrasound revealed she also has a malformation of her Pulmonary Ateries called Pulmonary Atresia.  This combination of Heart Defects occurs in about 1 in 10000 or less births. We knew our little Olivia was special, but we had no idea how special she really is.

Olivia was immediately admitted into the Pediatric ICU at Bronson Children's Hospital in Kalamazoo. She was started on IV's, medications and monitors to make sure she remained stable. As I am writing this (3/8/09) we are waiting on a transfer to University of  Michigan Hospital in Ann Arbor to further evaluate her heart and prepare her for the first of multiple surgeries to 

correct blood flow to her underdeveloped lung circulation. 

We will try to keep this blog update with any new developments as they happen to our sweet little girl. Initially things have been happening fast and furious, but we must soon settle into a rhythm, but will continue to use this site as our meeting space.

Thank you so much for all the support and love we have received over the last 3-4 days since Olivia's diagnosis.

Peace,

Ben, Sara & Olivia