Latest Liver Clinic News

Unfortunetly last liver clinic wasn't exactly the best we have ever had. It was at first! We went early for ultra sounds which turned out pretty good. Meaning NO SURGERY!! For now at least. The timing has to be perfect. If they do it now they have a chance of tearing her portal vein again which could be fatal, and since her portal vein isn't that much worse then Decembers ultra sound, we still have some time. Of course, if we wait to long it could also be fatal, but it has definite warning signs such as bloated belly and enlarged spleen. In other words... 'the pre transplant look'. So that was good news. We go back this coming monday, but only so quick because of the happenings in April :). And Endocrinolgy clinic again in May.

The 'other' part of liver clinic wasn't so great as no surgery. Harmonee has some pretty bloody stools, meaning her tummy is bleeding from such a high dose of stress steroids. Funnnnn.... After a couple of days of absolutely NO sleep, the meds started to kick in and make her tummy feel better so she could sleep. Her new med schedule, 8 a.m. - Prograf, hydro and zantac, test blood sugar 10 a.m. carafate (which can NOT be given anyway closer then two hours of prograf) 4 p.m. zantac, hydro, test blood sugar 8 p.m. Prograf 12 p.m. hydro, dapsone, asprin, carafate, zantac, test blood sugar. Its not a very fun schedule, but it seems to be working with miss Harmonee.

Lots to Tell

I have been HORRIBLE at updating everyone. After the past few days of everyone asking me whats going on, I figure I'll update on the many things that have happened the past couple months.

Liver:
Liver labs, are still not good. Of course that has turned into the norm for Harmonee. There are many theorys as to why her labs are bad. The first is her clogged portal vein. This is the most voted for through out the doctors. They think that because the scar tissue it clotting her portal vein that her liver is not getting enough blood. Therefore, no rejection, but bad labs. The second theory is her low blood sugar.

Blood Sugar:
We have been checking her bloodsugar at least 3 times a day and if she is acting funny. So far, so good. We put cornstartch in her formula to help boost it up, along with a new set of stress steroids called hydrocortosone, instead of the usual prednisolone. She takes the Hydo every 8 hours so that her sugar doesn't have much of a chance to drop.

There are also a few theorys on why her blood sugar is funny. The least anxiety packed theory is that her adrenal glands have failed. During her ICU stay last year before transplant the doctors had to pick and choose organs, (this happens a lot with Harmonee). Luckily the doctors picked the heart and lungs and kidneys to focus on, unfortunetly in the decision Harmonee's glands were let go.  The second theory is a horrible theory. They have recently looked into her donors health history and found a disease that causes high blood sugar, and heart problems. So far her heart is good. But her blood sugars are struggling to stay up. One of the opinons is that Harmonee's body is fighting the donors disease and is over reacting making her blood sugars low. During her every other weeks labs there is a new test taken to show a certain hormone that goes a long wtih the donors disease. So far, the blood sugar is the only indicator that anything is going wrong with it.

Everything with blood sugar and liver are mostly just theory for now. She goes into surgery March 24 to unclott her portal vein. After the surgery endocrinology is going to start looking more into her odd sugars and liver clinic is hopefully going to confirm their liver theory and start working on her low blood sugars.

Physical:
HARMONEE IS WALKING!!! She NEVER stops now. She is still at the woddle stage that I love! Her and sister have races across the living room pushing their doll strollers, Layla, being the great big sister she is lets her win more times then not. Harmonee starts screaming when she wins "YAY!!!" She is talking so much. I attempted to email her daddy all the words she said and it came out to about 50! 0-3 has graduated Harmonee saying "she is up to date with a normal 17 month old baby!" Thats always good news. Of course, we still have speach therapy come visit. Her mouth trauma has gotten better but not by much. She still wont drink out of a sippy cup, and wont let anyone put food in her mouth except herself. She wont use a spoon or anything near her mouth, but she is getting better and has sipped out of a big girl cup!

I'll try not to take so long in updating next time!

Just Another Weekend at PC

Our weekend went something like this...

Friday: Woke up way late with Harmonee still asleep (not like her to sleep past 8 a.m.). I woke her up, and with in minutes she had fallen back to sleep on my lap, and this time... didn't wake up. I called GI clinic and Amy told me to take her blood sugar which turned out to be 38. After giving her her emergency stress steroids and some pancake syrup, Amy told us we had two options, I could take her to the ER myself, or have an ambulance come get her to take her to the ER. Either way, from the ER she would be transported to PC.

We got to the ER not even 20 minutes later and her blood sugar had dropped to 29. They gave her an injection of hydrocortozone and started a sugary IV drip. Oxygen was then their next priorty since she was only stating at a 70 and was working pretty hard to breath. Labs, coultures and xrays were taken. Her lungs seemed pretty foggy, and an ear infection was found.

By this time an ambulance was there getting ready to load Harmonee and take her to Primary Childrens. More tests were taken at PC, and Harmonee came back positive for RSV.

Saturday: We had a rough morning with lots of suctioning, coughing and throwing up. But by noon she wasn't doing any of this, and was up giving the nurses a run for their money before long. Endocrinology also came and decided to start taking test monday to see why her sugar keeps dropping.

Sunday: RSV hitting hard. Oxygen was turned up, suctioning has been every hour, and coughing almost non stop. Unfortunetly the doctors say she is going to get a lot worse before she gets better. She is still pretty dehydrated and its not helping her lungs break up the gunk they need to in order clear themselves.

Disaster Strikes in the OR

(Harmonee having fun in the rocking chair moving her bum and making herself rock throwing her hands in the air and squealing! Best part about a hosptial stay?: the rocking chair...)

On Wednesday night Harmonee's labs came back worse then before. Making everything move to a week earlier. Thursday morning Harmonee went into surgery.

It would be a quick procedure. They put a needle into her portal vein in radiology and push a catheter down to the narrowing in her portal vein. Then they will blow up a small balloon to help widen the narrowing and hopefully stretch out the scar tissue to keep it from closing. Its a fairly easy surgery that only takes about an hour and a half to two hours. With general anethesia and no risk except for accidentally tareing the portal vein, which is only about a 1% chance.

Surgery went to normal time when the surgean came to talk to me in the waiting room he asked me to go into the small room where they take you if they need to talk serious.

The surgeon said that Harmonee was okay but there was a complication so that they were not actually able to do the surgery. Disaster struck as soon as they put the needle into her liver. Because of the low blood flow her liver is getting chirosis and becoming hard. This made it twist funny when the needle was pushed into the vein, taring the portal vein.

They did an ultra sound and took labs to make sure that it wasn't leaking any blood and she was sent to the recovery room without a fixed portal vein.

Doctor Book and the surgeon decided that they would try this same procedure one more time. If this happens again Harmonee will have to cut open her scar for the 4th time and fix the portal vein this way.

As of now things are good. Her liver is hanging in there and even though she looks pretty tattered from the asprin dose, she is still happy go lucky!

Surprise! Its Surgery!

After getting out of the hospital last weekend and thinking everything was okay Harmonee's liver labs jumped again! Doctor Book called on Thursday to set up plans to go in to have a CT scan (cat scan) on Friday.

This scan was to figure out what is truly wrong with Harmonee's liver. Biopsy came back good, with out any rejection... then why high liver labs?

After Harmonee's cat scan recovery or in other words, 'sobering' her up from the sedation we went up to talk to Dr. Book about the results.

Apparently, Harmonee's portal vein looks like someone has tied it off with a rubber band. This causeing low blood flow, but not necisarily high liver labs. But first thing first, fix the problem that we DO no is wrong. On Tuesaday Harmonee is going in to dialate her portal vein. This is a very serious problem, but not a very serious surgery. This part of the surgery is done by a radiologist. Unfortunetly... and we are hoping this does NOT happen, if the radiologist cannot fix the problem, they will have to do a major surgery to get inside of her liver. Also, if her labs continue to rise... we will be starting from scratch to figure out what is making everything go off the records. But again... lets hope that doesn't happen either.

Biopsy Results

 Talking to Daddy through her IV. When the nurses came in she would hold up one hand to hold them away, put her IV to her ear and start talking to Daddy (Sad that this is what she knows is "daddy" I know.) Harmonee: "Hi Dad. Yeah. Uh huh. Uh uh. Yeah. Dad. Yeah. Yeah. Uh huh. No. Yeah. Bye Dad"... Daddy was there in spirit!

 

On Friday Doctor Book called wtih concerning news that Harmonee's liver labs had yet to go done, but up! We were admitted with a full schedule, that night she would have an ultra sound and in the morning a biopsy.

Ultra Sound:
The ultra sound came back not so good. Harmonee's portal vein has a spot of scar tissue that has made the vein narrow, not letting blood go through it easily. This happens with most liver transplants they said. It can make the liver act funny by making it swell. This is fixed by surgery.

Biopsy:
The biopsy came back PERFECT! No rejection! YAY! But... there was some swelling found, but only caused by a small cold. The doctors said that her liver must have taken the hit, and then the cold finally hit Harmonee this morning.

Ending Results:
We were sent home today, with everything the same as it was. You cant be to careful with high liver labs. Fortunetly we now know about her portal vein, and will be able to watch it and quickly fix it before it does anything dangerous.

That was our weekend scare. I was a little nervous about it being like last december, but it wasn't and I hope this will be our last hospital stay this winter!

Lab Trade Up

If its not liver its kidneys, if its not kidneys its liver. UGH. HARMONEE LYNN!! Harmonees labs last week came back with a touch of CMV, but her kidney labs were looking better. Dr. Book ordered another set of labs on monday. This was not a good day. For starters, the weather was to bad to get to labs that morning. So... that night when we made it out, Harmonee ended up grabbing and taring out 4 separate pokes, blood going EVERYWHERE, burises and cuts and so much more, until the 5th time three people had her pinned down. Literally.

Tuesday morning came and we got a very worried phone call. Harmonee's liver labs are now showing VERY high. This could mean rejection. Fortunetly, Dr. Book is hoping that this increase is due to the medicine being used to treat her CMV. On Friday she has more labs. Depending on what they show, and hopefully they will look much better then Mondays, but if they dont, she will go in for a biopsy this weekend. Lets hope this doesn't happen. But I guess if it does, at least there are things that can reverse it right!?

Next up... Kidney Failure?

Over the past couple weeks Harmonee has been in and out of the hospital for dehydration. I figured this was just because she had been sick. The second time she went in for fluids I was a little sad since I thought the minimum amount of fluid intake might not have been enough to suffice the winter colds and illnesses. Along with a very low blood count, and finding this blood in her stool I was getting a little nervous as to what this could mean. Not to mention the constant flash back of before transplant the day she was admitted to the ICU.

Verdict:
Stool samples and urine culture's were taken both coming back with a positive for ecoli. Of all things. Like I said before... if you can get it, Harmonee will. How did she get this?... Believe me, I  am still trying to figure that one out myself. What are the side affects?... Possible Kidney failure! Awesome. How do you get rid of it?... By taking all antibiotics away.

Dont worry I was a little confused myself.

Time for Action:
For starters. Ecoli causes kidney failure. This is especially vital to Harmonee since her kidneys have already taken the mother of all beatings. But also vital to ALL transplantees because of our best friend... Prograf. Prograf is a tricky medicine. If it becomes to high, your kidneys fail, but too low too soon, your liver (in Harm's case) with reject. Therefore it is time to pick and choose organs... doctor book is decreasing the prograf to give Harmonee's kidneys a much needed break.

Unfortunetly we just have to wait this out. There are NO antibiotics that will take it away. Oddly enough, antibiotics make ecoli worse. Go figure. So all antibiotics were discontinued. Including the antibiotics she was on for the prograf in case if she got sick.

Where are we at now?:
The ecoli is already starting to affect her kidneys, so we are keeping close eye on them. I keep a log of urine and bowl output. Colors, frequency, and amount. We are doing labs every other day. Todays labs came back to show that ther kidneys have declined even more.

The optimistic approuch:
If her kidneys don't take to much of a hit, they will bounce back faster then if they are hit harder then hoped. I am also hoping that in the long run her kidneys will bounce back with out having a liver repeat. On a brighter note... Liver is doing AWESOME!

Bored of being in the ER.

Never lets it get her down!

Liver Clinics Milestone!

I thought I would NEVER see the day that Harmonee's NG tube would be taken out. It seemed most unlikely since she had yet to start eating. Gagging if anything even brazed her lips. Oddly enough, we gave her bacon... Whala! Harmonee is eating anything and everything that can fit in her mouth that is edible! Bacon and chicken, soup and salad, bread and cookies. You name it. But still no bottle. Until one day... being the the overly excited mom I am, filled her to full of real food until she got sick over tubing her formula. This made her exceptionally thirsty. Who knew that just making her a little more thirsty would actually make her drink her bottle! Since then... its been quick progress to saying "see you later NG."

Excited I went to show doctor B at liver clinic that my baby was actually a tubeless baby. She chugged her bottle right in the middle of everyone. Not until after she made a mess of things first though. (see pictures). Doctor book was impressed and headed out to make arrangements so that Harmonee wouldn't have to ever put that NG tube in again.

Therefore, we came home from the hosptial with a glucometer since her blood sugar isn't staying up very well, and a scoop of cornstarch in her formula to add more sugar to her blood.

Here we are. Harmonee is tubeless, and straight on her way to walking! Early intervention is even planning Harmonee's graduation party since she is progressing so quickly on her own with having no Speach therapy, PT or OT since July.

Reading Dr. Suess in liver clinic!

I don't abuse her! I swear it! Its just a VERY large dose of apsrin and a baby who is learning to walk I promise.

Some how she got wrapped up in the paper, she drooled so much it tore right off while she was rolling around.

Birthday Picture

 

Happy Birthday Harmonee

There were times this past year that I never thought we would make it this far! I was almost certain that Harmonee's 7 month birthday could, and would be the last one we celebrate with her in person. This past year has been one for the making. Its been crazy and hectic, chaotic, organized, messy, EMOTIONAL, eventful, scary and at times a little much to bear. In ways I am excited that we have hit the one year mark, it gives me the freedom to say "This year WILL be different." But in other ways I am sad to see this year end. It is sad to think that Harm is already 1 yr old, it seems as though I have just brought a 'new born' baby home from the hospital (for a second time). This time to stay home, and for me to actually be the mommy (not the nurses) and raise her! Brandon asked Harmonee one day while she was sitting on her rocking horse rocking as hard as she could and laughing at the feeling, "who gave you the permission to grow up?" Unfortunetly we came to the conclusion that she missed the memo while stuck in the hospital.

This year has definitly taught us some things that we would have NEVER learned without going through this ourselves. Things that I was oblivious to and selfish enough to not think about them. We are a better, stronger family because of it, and a better person in and of itself. It has been hard, but then again... it brings whole new meaning to the phrase, "NOTHING IS IMPOSSIBLE"

OCT 8, 2009 - OCT 8, 2010

Oct 09- Harmonee was born, 7 lbs 9 oz, healthy and perfect, but full of trouble!

Nov 09- First signs of white stool and Jaundice

Dec 09- Diagnosis of BA, and first surgery "Kasai procedure"

Jan 10- Determination of failed Kasai

Feb 10- Work up for Transplant list

Mar 10- NG placement, Official placement on Liver Transplant Waiting List

Apr 10- ICU, heart, lung, kidney, and liver failure. DNR, on-off transplant list, observations from 'the giving side' of organ donation.

(Monday April 26)

(Tuesday April 27)

May 10- Liver Transplant

(May 18, after transplant)

June 10- Release from 2 month stay at PCMC, POST TRANSPLANT

July 10- Bye-bye oxygen, NJ and Daddy for training

Aug 10- Bye-bye Broviac, Hello rolling, and sitting up (ALL BY MYSELF)

Sept 10- Hello first tastes of baby food, CRAWLING, working on pulling up, early intervention, and DADDY from training

Oct 10- Hello 1 year Birthday, BACON and other meats! Standing, and preparation for walking. Bye-bye Daddy again. 

 

So you see... this past year has been INSANE beyond belief! This next year I hope wont have so many medical milestones! 

Apples and Bananas!

Harmonee is EATING by MOUTH! The FIRST time since MARCH!!

NO force, NO syringes, NO gagging, NO mess! All by herself!

Miss Paige... and More Great News!

Not all news is great news. And as we all know from personal experience, or by our liver babes stories, Liver babies come with either extremely wonderful news or absolutely horrible news!

For Harmonee, her news became absolutely horrible, before becoming extremely wonderful! My biggest fear is to have Harmonee's absolutely horrible news become the reality of other liver parents. Even though, I cannot to anything about it but to be there for the parents with an understanding.

I have recently gotten to know another very amazing liver mom! Along with all of the rest of the amazing liver parents. Unfortunetly I met Amy during Harmonee's PICU stay before transplant. If that isn't bad enough, her daughter was diagnosed and in the PICU just after her Kasai when we met, scaring poor Amy to death. But today Paiges news came. There is a HEALTHY second chance liver waiting for Paige right now. Paige will soon be a healthy post transplanted pink baby! YAY!

In other news, I am a bit slow. But the story is still there. Harmonee was in the Deseret News on Sunday with a really great story! Check it out!

http://www.deseretnews.com/article/700061126/Utah-baby-gets-a-new-lease-on-life-with-a-liver-transplant.html