Tetralogy of Fallot - An Overview

I thought it might be helpful to give some insight in to Finley's heart condition and what she is having surgically repaired on Tuesday.  Finley was born with a congenital heart defect called Tetralogy of Fallot.  It is actually one of the most common congenital heart defects and the prognosis after repair is usually quite good.

Well what is it?
Tetralogy of Fallot, a condition present at birth, is a combination of four ("tetra") heart defects.  I still remember the doctors sitting us down on the evening of August 9th after doing what seemed like an all day echocardiogram and telling us what the name of this condition was and that it was characterized by FOUR defects.  FOUR?  Isn't one enough?  Anyway, I digress.  The four defects cause several problems and usually cause the skin to turn blue due to poorly oxygenated blood.  This is actually why they called the transport team to pick up Finley and bring her to Childrens - she had a bluish-gray tone to her.  I will also never forget, when Mike and I walked into the NICU that day, I asked the cardiology fellow that was doing the echo what she was looking for - and she told me - very matter of factly - she was looking for a "blue baby disease" or in other words a defect that causes cyanosis - the condition of having that bluish pallor.

The four defects associated with Tetralogy of Fallot include:

1) A hole in the heart wall between the lower heart chambers (ventricles - called a ventricular septal defect or a VSD) that causes oxygen -rich blood and oxygen-poor blood to mix.  Finley has a large VSD that will need to be closed.  And, as a bonus, she also has an atrial septal defect - ASD - which is a hole between the upper chambers (atriums) of the heart).

2) A narrowed or blocked passageway between the lower-right heart chamber (right ventricle) and the main blood vessel leading to the lungs (pulmonary artery- infundibular stenosis) that causes reduced blood flow to the lungs and requires the heart to work harder.

3) A shift of the main blood vessel carrying blood to the body (aorta) to the right that causes oxygen-poor blood to mix with the oxygen-rich blood (overarching aorta)

4) Abnormal thickening of the muscle of the wall of the lower right heart chamber (right ventricular hypertrophy)

So how will they correct this?
The surgery is an 6-8 hour procedure that involves the closure of the ASD and the VSD with a patch (which is actually Gortex) so that the blood can flow normall from the left ventricle to the aorta.  The narrowing of the pulmonary valce and right ventricular outflow is then enlarged by a combination of cutting away obstructive tissue in the right ventricle and enlarging the outflow path with a patch.  The hope is that her pulmonary valve will be salvagable - meaning not too tight so it can be widened enough to allow proper blood flow but won't leak.  If the valve is too tight, they will attempt to cut the valve open and put a patch in to make it wider (as previously explained).  The third option would be to put in an artificial valve if its too tight to salvage.  Either of the last two options will mean that Finley will eventually need to have that valve replaced - fortunately this can be done through a catheterization process vs. another open heart surgery.

The interesting thing about the repair and Finley's condition is that they only actually repair two of the four defects.  The act of closing the VSD will correct the overarching aorta and the procedures in total will allow the parts of the heart to do their jobs equally and will, over time, correct the hypertrophy of the one side of her heart.

During the procedure, Finley will be put on the heart/lung bypass machine, her heart will be stopped and the machine will do the work of the heart.  The process of getting her up on the bypass machine and then back off is actually what takes a considerable amount of the time she is in surgery.

Finley's surgeons Dr. Backer and Dr. Russell are the same doctors who performed Finley's first open heart surgery and are two of the best doctors in the country.  We feel very fortunate to have access to such wonderful physicians and know that she will be in the best hands possible.  Finley should be the first case on Tuesday morning so I hope to have some updates by the afternoon or early evening. 

Please keep Finley in your prayers.

Happy 9 Month Birthday Princess Finley!

Wow.  It was 9 months ago today that Princess Finley came in to our lives.  Its been the longest, yet the shortest 9 months of our lives.  Its amazing to play back those tapes in my head and think about all that she has been through and how far she's come.  And now, as we celebrate her 9 months and prepare for her surgery on Tuesday - it amazing to think she made it THIS far before she needed her heart repair surgery.  When she received her shunt, the doctors told us she would need her repair at 6-8 months.  And here it is 9 months - and we're not doing the surgery now because she's outgrown her shunt - we are doing it because now is a good time.  She is big and she is healthy and she is ready!!!

Here is the princess's 9 month birthday picture:


Its been nice to have Mike home - Nate is of course thrilled.  The night before he got home, he gave Finley a kiss in her crib and told her to "dream about daddy".  And then every airplane that flew over our house Thursday night, Nate would say, "do you hear that?  Daddy is on that plane!".  Needless to say there was a lot of excitement - Nate was so happy he picked these for mommy, daddy, Finley and all of "daddy's friends":

And here is a picture of the little man with his daddy - reunited and it feels SO good!  LOL! (note the Iron Man in the background)

And so we are having a relaxing, family weekend at home - one of the last ones we will have for a couple of weeks.  And daddy got to do one of his favorite weekend activities - napping with the kiddos...

How crazy is that hair right?  I keep (half) joking that they are going to need to put one of those paper shower caps on Finley when she goes in for her surgery!

I hope that everyone had a wonderful Mother's Day tomorrow!  The last 9 months have given new meaning to me to what it is to be a mom and so this Mother's Day is extra special and I feel so lucky to have my two little beautiful children in my life!

Welcome Home Daddy!

Mike gets home in the morning.  Man his flight can't land soon enough.  I am tired.  I look like hell.  I had three hours of sleep last night.  Did I mention I look like hell? I've had exactly two showers since Mike has been gone.  He left on Saturday!  I know, I shouldn't be admitting that.

Mike is on the way home as we speak and we (collectively) couldn't be happier.  I am pretty sure Nate is sick of me - if I have to hear DDDDDAAAAAAADDDDDDDEEEEEEEEEEEEEEEEEEEEEEEEEEEE one more time, my ears are going to explode.  And Nate, he's been in that "That that doesn't kill you will only make you stronger" category for me this week.  I love the kid to bits but Dear God he's been trying my patience.  He's had tantrums that have put every other tantrum to shame.  At times I have been pretty sure his eyes were going to bug out of his head and his head was going to spin.  Seriously, its been that bad.  I can't wait to get out of this house for some alone time tomorrow - I have a hair appt and eyebrow wax tomorrow afternoon - I need to take action on that third sentence in the opening paragraph!

And Finley?  She's been happy as a clam. I said this in my last post, but it bears repeating - she has been such a peach.  I don't think I've seen her so happy and so healthy in a long time - maybe ever.  I have really enjoyed the week with her at home with Gigi here - the two of them in the den and me working in the dining room - I can just hear her chatting away - making all sorts of crazy sounds and noises - giant smile on her face.  I wish I could bottle her up right now and open it up next week after her surgery when I am dying to see that smile.

Nanny 911

With Mike in Argentina this week, Nate has been missing his daddy a lot.  And needless to say, he's been a bit of a terror.  If I had a dollar for how many times Nate has told me since Saturday that I "wasn't his best friend", I'd be able to go on a nice little shopping trip.  As awesome an age as three has been, its been equally frustrating and Mike and I often find ourselves really hoping that its just the age and that he doesn't really have issues.  We continue to be hypersensitive toward Finley's hospitalizations and the impact they could possibly have on Nate.  Although we try to keep things as normal as possible for him during those times, he's definitely started to be more emotional around those times.  I tried to begin the conversation with him last night about how Finley would be going back to the hospital last week and he started to cry - so I thought we'd save that for another time.

Anyway, Nate and I started off with a bang yesterday morning.  He was adamantly refusing to get ready to go to school, was running when I tried to get him dressed (and laughing at me) and subsequent time outs didn't work - he just doesn't care about being punished (and he laughed at me some more).  So I took his DVD player away.  And he took notice.  After he finally got dressed, I agreed to give him a second chance with his "DDD" player (as he calls it) but after school was more of the same - so not got "grounded" from his DDD player and the TV.  All night long, he kept asking me, "Do you want to let me watch my DDD player?  I can tell you sorry." or "Let's watch Madagascar mom".  And then I kept telling him - he made that decision he said, "I can have it in the morning time?".  Needless to say, Nate and I had a nice dinner together and a nice quiet, TV less evening.  And since he had nothing else to do, he wanted to cuddle with his mommy a lot - and we both fell asleep at 8:30 pm!  I think I can get used to being the bad guy (LOL!).

Finley is doing awesome.  Her smiles abound and she seems to be feeling the best she has been in a long time.  Prior to her hospitalization she had this pesky cough and runny nose for weeks and now its completely clear.  When she sleeps, she is so quiet, I have to put my face down really close to her to make sure she is breathing! She has been so actively chatting, kicking her feet and doing her Miss America wave (she does this thing where she holds her paci in one hand and twirls her wrist and when she does it, the other hand simultaneously moves and does the same thing!).  Her progress though as made us think - that certainly the antibiotics and steroids have helped to get her better so maybe that wasn't just a cold she had?  Its something I plan to talk with the ENT about next week.  Perhaps the hardest thing for us right now is wrapping our heads around the fact that in less than a week she will be back in the hospital and while planned, it will likely be the most serious surgical procedure of her life (hopefully).  And to anticipate that we will be missing those smiles, the chatting, those waves, for a couple of weeks while Finley recovers is heartbreaking to say the least.  So for now, we'll just soak them up...


And this about sums up Nate's mood for the past few days...

Look what we did this weekend!!!


Finley got her first real tastes of baby food this weekend.  Sweet potatoes.  She loved it!  It was really interesting though to watch a child who hasn't taken anything orally in her first almost 9 months.  I could tell she was really sort of puzzled with the texture the way she was moving it around in her mouth and on her tongue.  Then it occurred to me.  How bizarre that we seemingly just went from 0 to 60 on feeding.  She skipped right over bottles (she hasn't been cleared for thin liquids yet) and went right to food.  Maybe its just strange for me to think through.  In any event - we're excited and hope this continues to go well!

Nate had an interesting weekend of eating himself.  Nate is a big fan of Iron Man - and with the new movie just out and everything - Burger King's kids meals have Iron Man toys in them right now - much to my chagrin.  Needless to say, Nate had Burger King (and I am embarrassed to even admit this) four times this weekend.  That's right.  I guess fortunately for me, he was less interested in the food and more interested in the toys.  Mommy can't wait until this promotion is over.  Here is a picture of Nate playing with his action figures in our bedroom.  He likes to take his step stool and put it by the window by Mike's nightstand and line his guys up on the window sill and play there.  This one is particularly cute because you will see - he has his Lightening McQueen slippers on with his underwear - and that's it!  Oh yeah - and he's also watching his Iron Man DVD while he plays.  You are probably reading this thinking - wow - Tracy is the mom of the year, right?


One more important thing to note that sort of just got lumped into the pile of "all that is less significant" while Finley was in the hospital is the fact that we FINALLY passed our final inspection on our addition last week.  Officially - the permit came down out of the window and everything. Now, this is not to say we don't have some straggler punch list things out there - but that madness has finally ended.  Here is a before picture:


And an after picture (don't mind the garbage and recycling bin!):
I realize its likely not all that exciting for most but going from a one car garage in which we could never park our cars because Nate's toys took up too much space, to having a place where we can actually park our cars is pretty cool.  I will take some pictures of the inside and post those soon.


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Finley's Medical History

Born on 8/8/2009 - Finley is taken to Children's Memorial Hospital via the transport team 12 hours after she is born.  She is having trouble breathing and is turning blue.  She is immediately admitted to the NICU.

8/9/2009 - Finley is diagnosed with Tetralogy of Fallot, a congenital heart defect and laryngomalacia, a condition that causes her to aspirate whatever she swallows in to her lungs.

8/21/2009 - Finley has open heart surgery to place a Blalock Taussig shunt to help facilitate proper blood flow.  When she is older, she will require a full repair of her heart.

9/10/2009 - Finley is released from the NICU.  Because of her laryngomalacia and reflux, she is fed 100% through a nasal gastric tube.

9/14/2009 - Finley is re-admitted to the hospital for an infection in her surgical wound.  Finley's genetic test comes back - she is diagnosed with CHARGE Syndrome.

9/15/2009 - During a CT scan to assess the infection, the doctors notice something strange on her CT.  It appears Finley has a malrotated bowel and will require surgery.

9/23/2009 - Finley is examined by the opthalmologist and she is found to have colobomas, a condition common with CHARGE Syndrome, in both eyes.  The degree of her visual impairment is unknown.

9/30/2009 - Finley has the Ladd's Procedure to correct the malrotation and also has a g-tube placed.  

10/7/2009 - Finley has a sedated ABR to check her hearing.  She has a mild hearing loss in her right ear and a moderate to severe loss in her left.  Her loss can be at least partially corrected with hearing aids.

10/8/2009 - Finley is supposed to be released from the hospital today but she has been having low blood sugars.  The endocrinology is called in to evaluate her.

10/29/2009 - Finley is finally released from the hospital with a unconfirmed diagnosis of hyperinsulinism.  Actual diagnosis requires fasting blood tests - because of her heart condition, it is not safe for her to fast.  She needs to have her blood sugar tested every three hours and we are taught how to administer an emergency glucagon injection in case her blood sugar gets to low.

11/1/2009 - Finley requires emergency injection because she cannot sustain her blood sugar.  She is taken to the local ER and transported via the transport team to Childrens Memorial.

11/10/2009 - Finley is discharged from the hospital on a 24 hour continuous feed.  We add polycose to her milk to help her keep her blood sugar at an acceptable level.

11/22/2009 - Finley is taken to the local emergency room with a bad cold - she is having trouble breathing and her oxygen saturations are low.   She is transported via the transport team up to Children's Memorial.

11/25/2009 - Finley is released from the hospital - just in time for Thanksgiving.

12/8/2009 - Finley is taken to the local emergency room for observation - she has a bad cold.

12/21/2009 - Nate is chasing the dog and the dog runs in to Finley's IV pole, knocks it down and pulls out her g-tube.  She is taken to the ER at Childrens to have it put back in.

2/10/2010 - Finley is admitted through the ER at Childrens - she has a bad cold again.  Oxygen saturations are low and breathing is labored.

2/13/2010 - Finley is released from the hospital.

4/17/2010 - Finley is taken to the ER at Childrens - she has pus draining from her belly button.  They suspect a urachal cyst - they send us home with orders to return on  Tuesday (during our scheduled visit) for an abdominal ultrasound.

4/20/2010 - Finley is admitted to Childrens for an overnight stay.  She will have a swallow study and abdominal ultrasound on Tuesday and a CT and sedated echocardiogram on Wednesday which will require anesthesia.

4/21/2010 - The anesthesiologists have trouble intubating Finley, so they call in her ENT to have a look at her.  He observes that her airway is 75% closed off - partially due to scar tissue from previous intubation and partially due to an infection.  The diagnosis is subglottic stenosis and may require her to have surgery on her airway.  She is immediately admitted to the PICU and put on a 24/7 breathing treatment and antibiotics and steroids to help reduce the inflammation.

4/26/2010 - Finley is well enough to be taken off of all oxygen.  Immunology and Infectious Disease are called in to examine Finley because the culture of her throat are growing staph and strep.  Infectious Disease is trying to figure out what could have caused her infection.  Immunology labs will be drawn to determine if Finley has an immune problem which sometime occurs with children with CHARGE.  This is the second significant infection she has had since she was born.

4/27/2010 - The lab calls to say that they mis-read Finley's airway culture.  It's not growing staph - its only growing strep.

4/28/2010 - Immunology labs are back and don't show anything significant.  Finley's lymphocyte counts are low but that isn't unusual for a child who is fighting an infection.  The doctor wants to re-examine her before her first birthday.  In the meantime she cannot have any live vaccines.

4/29/2010 - Finley is discharged from the hospital.  The ENT will do a bronchoscopy before her heart repair surgery to determine next steps on her airway issue.

5/11/2010 - Finley has open heart surgery to repair her heart.

5/17/2010 - Finley begins to show signs of infection which is later identified as a urinary tract infection.

5/25/2010 - Finley is discharged from the hospital.

5/30/2010 - Finley goes to the ER at Childrens because of fussiness, sweating, heavy breathing.  And echo later identifies that the pressure gradient around Finley's pulmonary valve is an astounding 90%.

6/1/2010 - Finley is admitted to Childrens to try a medication called Propranolol to help ease the pressure in her heart.

6/2/2010 - The Propranolol brings the pressure in Finley's heart down from 90% to 50%.  Finley has a positive test for C. difficile which will require antibiotic treatment.

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