Mother of the Year

My very sweet, very supportive and somewhat insane husband nominated me for a local mother of the year contest.  He was very complimentary in his entry but I thought he missed the deadline.  When I received the call that I was chosen by the staff as one of the finalists (top 10) I was quite stunned.  Yesterday we had to go to the news paper office and do a short on camera interview to be posted on the website for family, friends and general public to vote.  I was so nervous and flustered, I feel like I did such a horrible job during the interview.  All last night I just kept thinking how terrible (such a tool) I would seem to anyone watching these clips and felt so disgusted with myself for not saying all the things I really wanted to say.  To all my family and friends, here are the responses I really wanted to say.

The first question the interviewer asked was how I felt when I found out that not only was I nominated by Kevin but that I was chosen as a finalist.  Of course I did not think before opening by big mouth and just blurted out the first thing that I was thinking...that I was embarrassed.  Gee.  Great answer.  Instead of expounding on why I felt that way I clumsily stumbled through a weird explanation.  What I meant was that I was embarrassed because I do not feel that I do anything more extraordinary than any other mom.  I think every mom should be mother of the year and I feel that I personally know so many great moms that deserve this honor so much more than me.  All moms have issues, struggles and doubts. I do not feel mine are any more difficult than the single mom working multiple jobs and busting her butt to keep her kids on the straight and narrow or the mom diagnosed with cancer who wakes up every morning feeling horrific due to all the nasty chemo drugs but still smiles, loves her kids and makes their day good or the mother of a child with severe special needs like cerebral palsy or autism or the mother who has had to bury a child but pick herself up and keep living for the other children she still has or even the mother who has a child with colic or a baby who never sleeps.  As well meaning as I know these people are, I really do not like when other moms compare themselves to me and say, gee, my day is not so bad when I think of yours.  You know, honestly, I feel the exact same way as you.  There will always be someone, somewhere struggling more than me.

The second question was to describe my family.  I was not too sure what she meant as to me the question seemed pretty vague and broad.  I ended up mentioning the kids but then got sidetracked into taking about C and the upcoming Race for Baby C.  While I am all about free publicity, holy wow, what a complete tool I am to be talking about a fundraiser to benefit us.  Ugh.  Total face plant.  I would much prefer my answer to be how much I adore all three of my kids.  How grateful I am to be the mom to two such incredible boys who are funny, sweet, adaptable and love me no matter how much I fail them.  I wanted to say how Kevin and I both try so hard to make a normal family life for everyone as we never want the boys to feel like they are coming in second to C and her issues.  I wanted to express how we try and do one on one outings with the boys so they feel special and important and can have the day just by themselves with just one of us.  I wanted to shout how much I love them for also being upbeat and positive when the visit C in the hospital and how they never complain with how much I am gone or never think that I love C more because I have to be gone with her so much and how much they love and adore their sister.  I wanted to say I am and will be eternally humbled to know these are my wild and crazy boys and I am beyond blessed to be their mom.

And finally the third question that I totally flubbed...the one everyone asks and the one everyone wants the answer too, how do I do it all?  My totally unoriginal answer...we have a very strict, tight schedule that we follow.  Really, that is all I have to say.  How about I talk about how I cannot do this alone.  How about I say that I have a great husband who works his ass off at his job, does all he can to support me and still loves me no matter how much I yell, nitpick or blowup because he has not done something to my OCD way of liking.  How about my amazing, incredible support group of family, friends and coworkers who support, encourage and help me out without ever expecting anything in return.  Oh man.  Of all the questions I wanted to answer differently this is the one I feel the worst about.  I am not a supermom.  I do not and cannot do all that I do without help.  I get down and pray a huge thank you to God everyday for each of you that is in my life.  My coworkers who take over my office when I am gone and make sure everything is handled.  My boss who picks up the slack all on his own.  Everyone I work with who has embraced C and is okay with her still coming to work with me.  All the Judges who constantly ask how C is doing or bring her little gifts.  When someone tells me how lucky I am to have a boss like mine and coworkers like mine, I smile, nod and agree all the while thinking that the person has no idea just how much I understand how fortunate I am to work with all of these truly amazing people.  All of my friends who have supported us, encouraged us and done the little things that mean so much like letting my boys stay with them when we need to be out of town with C, make us dinner, attend our fundraisers and come and hang out with us (have a beer or two) and make sure we feel like we are a normal family.  All of my family who I would not have made it through the last 4 years without them.  I cannot even fully express my gratitude to all of my family (local and distant...mine and Kevin's) for all of things they have done for me.  And of course I cannot forget the wonderful ladies who formed Team Baby C.  Outside of the huge help of taking some of the financial burden off of me by having fundraisers, these women have welcomed me into their circle, inspired me to take up and continue running and have generally made me get off my ass and do something.  I will be forever grateful to all of these girls who make me want to be a better person.

One of the things that I did not get a direct question about but I feel is so important that I would have liked to address is who inspires me to be the mom I am.  Well, this is easy.  The list is long but please bear with me as each of these ladies have shaped me into the mother I am.  I will be forever grateful to my "second moms" Laura V., Carrie B., and Katie D. who have shown me how to be gracious, grateful, strong and to love with all I have.  All of my aunts, Ellen, Sherrie, Laura and yes, even you Janet :0...for giving me the best example of true family, faith and hope.  All of my cousins who I think are pretty great moms themselves.  I learn so much from my cousins who are all raising kids of various ages.  For all my super mom friends...how I try and be like you :)  I am grateful for my sister.  Even though I was a mom before her, she has shown me ways to relax, chill out and let my kids be kids.  She is the kick in the butt I needed to being healthier and making sure my family eats the foods we should be eating.  For my grandmother and great grandmother...I have never seen a more perfect example of a true lady and I only hope that I am half the lady you are.  And of course my own mother.  She is by far my best example of the type of mother I would like to be someday.

So, hopefully all the people I really care about will read this and better understand my answers to the questions.  I wish I could have done a print interview instead of an on camera one.  I am much better when I have an opportunity to think about what I am saying and can go back an correct my answers when I feel like I have totally made an ass of myself. 

Cath is Done

So Tuesday was the day.  The day that I have been dreading since January.  We took C to Tampa on Monday night so we could be at the hospital bright and early Tuesday morning for her cath.  Thank goodness she really was the first case and her cath started right at 8:30am as scheduled.  There is absolutely nothing worse than having the whole day drag on and on with a very thirsty, very hungry and very bored toddler. 

C of course was a champ. 

The nurse was unable to get an iv in prior to going into the cath lab so the anesthesiologist said he would place the iv once she was under (this happens at least 85% of the time with C).  Even though I do hate to have C stuck multiple times I am glad when they cannot get the iv because then I can go all the way into the cath lab with her and stay with her until she goes to sleep.  I know she feels better having me there with her and there is so much less drama.  Much better for mama too.

This cath was to measure all of C's pressures and test close her fenestration.  The prevailing thought on why C is still so blue from her Fontan last April is that she is shunting too much blood through the fenestration.  This hole that is made during surgery has shown to help kids recover faster after the Fontan and usually closes all on its own eventually.  Of course C has to be one of the few with a wide open fenestration and too much blue blood still mixing in her heart. The cath doctor explained all he would do, etc and she was off to the lab. 

We waited and waited.  C did fine during the cath but she did have some atrial tachycardia during her trans-esophageal echo.  This is a special echo the cardiologist does before all heart surgeries.  These are even more accurate then the normal surface echos.  Fortunately the tach was short lived and she converted back to sinus all on her own.  Still scary for me though because this is the first time I have heard that she went into tach during a cath.  Ugh.  But, the doctors did not need to intervene to get her out so that is good.  Her meds are still working.  I keep hoping and praying that as she gets bigger and her heart gets bigger that some of these issues with the tach will go away, which they can.

The good news from the cath is that with her fenestration closed, C sats at 92.  Holy wow!  92.  92 is a good 20 points higher than where she sats during the day now.  We were suitably impressed.  And grateful to know that with the fenestration closed C will be in the 90's with her sats.  And knowing that the cardiogist is correct in thinking that this is the reason she is blue, and not some other horrible reason why, is such a relief.

Now the not so good news.  Although C's pressures are still perfect and she tolerated the test closing of the fenestration very well, the doctor did not actually close her fenestration.  He is concerned about her pulmonary veins.  So not good.  All heterotaxy kids have issues with the pulmonary veins (these bring the blue blood back to the lungs to be oxygenated).  In fact, two of C's heart surgeries dealt with repairing these veins.  I say repairing very loosely as these veins are not able to be operated on and if these veins are shot, then there is currently nothing else that can be done to save them.  The only option is lung transplant.  We have talked extensively about C's pulmonary veins and the consensus has always been that her veins look good.  They are big and open.  Not narrowed or stenosed.  However, during C's TEE the cath doctor and other cardiologist noticed abnormalities with the veins.  The way it was explained to me is that the veins look to be thicker than they should be, more like the outside of an artery (more muscle) than a vein.  The blood that is supposed to flow through the veins back to the lungs is all passive flow (per the Fontan circulation).  However, because the veins appear to be thick, the blood flow is actually being restricted as the veins are pulsing (like an artery would).  So, the cath doctor and cardiologist doing the TEE, decided in the abundance of caution that they would not close her fenestration.  The thought being is that if the fenestration is closed then the lungs have no relief (or pop off) to reduce the pressure in the lungs.  If that happens and the veins are bad (and are getting worse) then C would go into heart failure.  With the fenestration open, should the veins be going bad, then the pressure can be released through the hole and C would just get more blue and instead of going into failure it would buy the doctors some more time.  The cath doctor is not positive this is an issue that is getting worse. He pulled up older echos from previous visits and he said he could see these abnormalities on those echos as well.  He said, in hindsight, it certainly explains why she limped along so badly and had so much fluid collection after the Fontan.  So now we just have to wait.  The doctor wants to monitor C for the next year to see if the veins are changing (getting worse) versus always being this way and now they are seeing this more clearly because she is so much bigger. 

So the news was pretty neutral.  She continues to need the oxygen at night when she is sleeping but since it really does not help during the day she can be off of it as much as she wants.  We will still keep all her stuff though because there are times now when she will be huffing and puffing after running or playing to hard or is too cold from swimming and needs the oxygen boost.  She also will not be starting sildenafil (viagra) as previously discussed because sildenafil will not help this issue at all.

Other than that not so great news, C was fine with the rest of the cath.  This time we had a practitioner with Healing Touch come in and do healing touch with C after the cath.  I really think this was helpful in keeping C nice and calm while she was coming out of anesthesia and getting her back to her normal self.

One of our very good family friends insisted that we needed to have this done with C.  I have been reading more and more about touch healing and I am all for trying anything that will help C.  In the picture above you can just see the ladies hand on C's chest.  I really appreciate Mrs. V telling us about this being available at the hospital.

Even though the cath doctor did not actually do anything interventionally, he wanted her to stay in the cardiac icu overnight because of the tach he saw during the cath.  We had already planned to be there overnight so was not a big deal.  Would have loved to come home but glad we got to see just about all of our very favorite nurses and PA's.  Everyone was so impressed and amazed with how well she is doing and how awesome she looks.  The last time they all saw her she was still very, very sick.  So, getting to see her doing so well, I am glad they could do so.

I am not sure where our journey will go from here.  Only time will tell us if C's pulmonary veins are shot and we are heading towards double transplant or if they veins are just the way they are and we can close her fenestration when she is older, bigger and even stronger than now.  In a way, we are getting a year's reprieve and right now, I will take it.

Cardiology Update

Tuesday we took C to Tampa to see the electrical cardiologist.  Nothing new or out of the ordinary as this was her 6 month check.  However, we had to see the other EP in the practice as we still cannot see the EP cardiologist she has been seeing.  Stupid non-compete clause and litigation.  Don't even want to get started on that. 

Good thing is the doctor was fully up to date on C.  I think because we pitched such a fit about not being able to still see the other doctor they are really trying to accommodate us.  C is not anywhere near a typical heart patient and just one tiny thing being missed could cause huge repercussions for her.  Found out her latest holter is in the acceptable range (does show small blips of tachycardia) but is actually improved from her previous holter.  The doctor is also very pleased that we are getting this much stability and control with her being on what he termed "a relatively small dose" of the medicine.  We were not too happy that he wants to leave her on this medicine until she starts showing signs that there is a problem (this medicine is highly toxic with lots and lots of nasty side effects) but I am ok with taking a more conservative approach right now.  The doctor we are seeing right now has been in the practice for over 30 years and has tons of knowledge so I trust that he knows what he is doing and that he is doing what he thinks will be the best course of treatment for C. 

He also does not want to do anything interventionally with her right now.  He does not feel that he would really accomplish much by doing and EP study right now and does not really want to put her under that much stress.  He is ok with her having the cath to close her fenestration.

So.  With all this, looks like we will be doing cath sometime soon.  Will be just to test close the fenestration to see if she tolerates the closing.  If she does tolerate the closing while in the cath lab then the doctor will permanently close the hole.  This is really not that big a deal as the hole was put there after surgery and will eventually close on its own anyway. Plus, it is standard procedure to go in and close up the fenestration after a year.  I am not looking forward to putting C through another cath but if this will help her sats and get her off the oxygen then this will all be for the good.

Not sure when we will be scheduled but probably in the nest two months.  Hoping it will be far enough out that we are not putting her in the hospital with all this flu still going around.  Her contracting the flu would be devastating. 

Forgot to mention the best part of the visit (other than that C is being well controlled with meds).  The doctor told us that on the grand scale that the tachycardia arrhythmia would be 3rd on his list of worries.  After, the big number one of infection and number tow, blood clots.  So, I feel a little better that he feels these arrhythmia's are not the most pressing of concerns and we need to approach the electrical issues the same as we approach the diagnostic (structural) defects.

Look for pictures from the night parade after this weekend.  The big parade is Saturday night. 

We Did A Parade

Every year, there are two parades that happen in my town.  One is for children only and for the last two years we have been saying that we wanted to make a float for C and promote awareness for CHD's.  The children's parade is always right in the middle of CHD awareness week and this year we actually did make the float and ride in the parade.  We made special shirts, had lots of decorations, lots of help and lots of walkers.  C was the star of the show and did her best to charm all the parade people.  We handed out heart beads and information.  My sister had two different families tell her that their own children were also CHD kiddos.  I think we accomplished our mission...raising awareness. 

On a side note, we took third place in our division (yay!) and even more impressive, my husband was personally asked by the parade chairman to participate in the big parade this Saturday.  Over 200,000 people to reach out to...lets keep spreading the news. 

C is just one of the 1 in 100.

What is Heterotaxy?

Carolena has Heterotaxy Syndrome, a rare, complex and complicated make-up of organ defects that mostly affect her heart.  She was born with only half of a functioning heart which puts her in the category of single ventricle anomolies.  Although she is similar to children with Hypoplastic Left Heart Syndrome, she has other issues she must face that children with HLHS do not.  Our biggest challenge, outside of her single ventricle and not having a spleen, is that she started having deadly arrhythmias at 9 months old.  Fortunately, she is stable with medication and has not needed too much intervention.  The electrical issues are the scariest thing for us as we never know when they will strike and if she will respond to treatment.  We were well versed and prepared for a child with HLHS, not so much for a child with Heterotaxy Syndrome.  For more information here are some great links:

http://www.heterotaxy.org/Pages/what_is_heterotaxy.aspx

http://www.chop.edu/service/cardiac-center/heart-conditions/heterotaxy-syndrome.html

A New Year

Yesterday we met with the cath cardiologist.  I really dread the appointments with this doctor.  Not because I dislike him, actually quite the opposite, I simply adore him but I dread these appointments because once we see him I know we are looking at doing some kind of procedure, most likely a heart cath.  Ugh. 

I knew this day was coming.  I knew that C would eventually need another heart cath.  I know that she will never, ever be to a point where she will no longer need any procedures.  Knowing all this, still this is not any easier.

However, yesterday was fine.  C did well except in the beginning.  I think she may still have some residual memory of the hospital/facility in Tampa as she was acting very scared during her work-up/eval.  She was unusually quite and still.  Very not like her.  She generally is in the nurses face, telling her what to do and how to do it.  C likes to help and will put on the ekg stickers, help with the blood pressure cuff, help with the pulse ox and loves to push the buttons to turn them all on.  Not yesterday, she laid completely flat on her back and would not move.  So, that was a little freaky but after that was done she was good and started to act more normally.  We generally do our cardio visits in Sarasota or even St. Pete so I think she must have thought that since we were in Tampa something big must be going on.

Fortunately for all of us, C's echo looked good and the only thing that remains to be a problem is her lower O2 sats.  She is still on oxygen, although it only really makes a difference at night.  So, the cath doctor wanted to talk about closing her fenestration.  He could tell from her echo that is large and wide open.  Since all the other issues that could cause her to be blue have been ruled out the cath doctor is very confident that Cs sats are still low due to too much blood shunting across her very large fenestration.  He talked to us about doing a cath, rechecking everything and then test closing her fenestration.  If she tolerates the closure while in the cath lab, then he would permanently close the fenestration.  With the fenestration closed then Cs sats would come up.  That is the plan at least.  The doctors all seem a whole lot more confident about this now then they did a few months ago.

The other problem we have (and of course it is not a small problem) is that Cs EP (electrical) cardio left the practice and is now in Orlando.  This should not be a problem as we do not mind driving to see him and would still like him to be Cs doctor however when we tracked him down and spoke with his office we were informed that he could not see her due to legal issues.  Awesome.  The doctor did call and speak with Kevin and confirmed this so now we are very aggravated and frustrated to know that C has been reduced to nothing more important then some companies bottom line.  If we still want to see her EP then we should be allowed to, not be told that we cannot because then he would be breaking a non compete.  Really? 

So now we are kind of in a holding pattern again.  The doctors need to get all this stuff worked out since the cath doctor wants to get a good plan in place for her electrical issues before closing her fenestration.  He need to know what the EP wants to do about getting her off the Amiodarone and onto something safer and whether or not he wants to do another EP study.  Once the fenestration is closed then the EP doctor loses his good, easy access to the atria where all of Cs problems exist.  He could still get to it if he has to but the cath doctor wants to make sure all this other stuff is one prior to cutting off the access. 

The good in all of this is that C is still doing well.  So well in fact that she is back to her old, crazy, wild self.  She runs and jumps and plays hard with her brothers.  She adores the "school"  she gets to do with her Pre-K teacher.  She is really making improvements with her speech thanks to her speech therapist.  I can get her to consistently say "I love you" now and it is just so cute.  Although she is still not eating she is still interested in food and drinks and appropriately uses a fork, spoon and cup.  She loves mimicking all of us, especially me and will do girly stuff like brush her hair and put on make-up.  She is such a little love and brings so much fun and lightness to our house.  I am so very glad she is her normal again.  Although I am not at all looking forward to sorting out all this cath stuff, changing her anti-arrhythmia meds and putting her through two possible caths I would be super glad to get her off this oxygen so she can really enjoy all the things she loves, like going on the boat, swimming, jumping on the trampoline, the swings and slides at the park and generally just chasing her brothers without getting winded and without having to drag stupid oxygen tanks with us wherever we go.   

Stability

C is stable.  Good news and bad news.  I would like to focus just on the good but there is always bad lurking just behind the good. I think C being termed "stable" is a way to get around the fact that not one of C's doctors really have any answers to explain what is going on right now with C.  Of course the most basic answers are, she has heterotaxy and her body is still not really adapting to the new physiology of the Fontan.  However, C continues to confound everyone.

We know she has lower O2 sats but you sure would not know by looking at her.  Yes, she is more blue but if you are not looking for the blueness you just might not notice.  I see the blueness, her doctors see the blueness but most people do not notice as she is generally moving too fast for anyone to see anything but a blur.  She is climbing on top of everything and into everything.  She runs, jumps, turns in circles, stands on her head, does forward rolls and with nary a huff, puff or wheeze.  She keeps up with her brothers and is beginning to give up naps completely (again).  Her cardiologist is on board with us that the 24/7 oxygen does not really seem to work.  She needs the O2 at night but during the day her sats are low and the O2 does not really bring them up.  She should not be this active and tolerant with the lower sats if she truly is not tolerating the Fontan.

Cognitively C is leaping ahead as well.  She has always been on track and age appropriate but took a huge downturn and regressed post Fontan.  Those days are fast fading into the past.  C receives one hour a week of speech therapy and one hour a week with a medical homebound Pre K teacher.  In just one month C has relearned lost skills, like coloring, and is bounding ahead in her speaking.  She is using 2 and 3 word combinations now and is beginning to request.  C has always understood us and knows what is going on but she has never communicated back with us.  Her communication has always been reactionary instead of proactive.  She has rarely asked for items or asked to do things but in the last 2 weeks she is really finding her voice.  Her requesting is becoming more frequent and consistent. So much so, I am beginning to really consider potty training.  All the positive forward movement cognitively makes the neurologist happy.  He feels she is doing great and we may get to stop the anti-seizure meds if she keeps doing so well and has no seizure activity.  She will have an eeg at her next appointment and the neurologist will make a determination from the eeg.

C continues to need her anti-arrhythmia meds but her rhythm and rate have been controlled very well with the meds.  I had a brief moment of panic when I found out C's electrical cardiologist would be leaving the practice.  I seriously broke down and cried and questioned why.  Then I found out he is only going to Orlando and we can still see him.  I was much relieved as I really trust him.  He has brought C out of two deadly arrhythmia's and he just knows her so well.  Plus, he has plans to get her off the amiodarone, which long term, this medicine will do far more damage than good.  He is more aggressive than other EP's and will do whatever he can to get her safely transitioned to another med that has fewer negative side effects.

C's eating, while not improved to the point where she will eat and drink in substantive quantities is finally back and possibly improved pre-Fontan.  After having 8 glorious weeks post Fontan of thinking we were really making headway, like a switch, the eating and drinking stopped. We have had a very tough and discouraging road with her eating and drinking and also not sleeping through the night for the last 2 months due to waking up in the very early morning hours to gag, wretch and vomit.  However, the vomiting during the day has settled down, we added Zofran to combat nausea in the early morning hours and she is back to her normal.

While we still do not have an answer to why she is continually running fevers no one is super concerned.  We heard back from the infectious disease doctor that C does not have any recurring virus, illness or disease.  She did not contract HIV from all of the blood or blood parts she received and we will just continue to do what we do when she runs a fever.  The only thing that the infectious disease doctor found was a low T cell count.  But she reassured us that if C was showing a low T cell count and had HIV she would have had a positive HIV test.  Since C does not have any disease she referred us to an immunologist.  Not really sure we need to keep going with this as C has no spleen.  Of course she is going to have reduced immunity.  This is a new chapter we have yet to write.

The fluid collection seems to be resolving as well.  Even though she did have some fluid a month ago when we saw her cardiologist he did not feel he needed to do an echo when we saw him yesterday.  I assume this means he feels her body is finally stopping with all the inflammation.  She is still on the diuretics but hopefully for only a few more months.

So, stable can in fact be a good thing.  A great thing.

However, C cannot remain where she is forever.  This is the nature of her severe heart defects.  Which leads to why being stable is not so good. In fact, can be bad for C.

As she gets older, her body will demand far more oxygen and she will tire quickly.  Although she can continue to be tube fed forever, I would like to get her off the tube and eating.  Keeping her system in balance becomes much harder as she ages due to the increasing need of vitamins, minerals, electrolytes, calories, etc.  Even cognitively, C needs to be pushed to progress.  While she is doing great right now, as she ages her deficiencies become more apparent and dangerous.  She needs to have good O2 sats and good cardiac output to continue to grow and improve.  If she stays exactly where she is right now with her O2 sats and cardia output, all the good listed above will disappear to be replaced with a very sick little girl who will just exist and not actually live.

We will meet with the cath cardiologist in January.  I asked why we needed to do another cath. What I have figured out is that a cath is the only way for the doctors to try and find any answers to why C is this way.  A cath will tell them if her number and pressures are good.  Although we have done this before, I guess the feeling is maybe something could have changed or developed.  The point of the cath will also be to determine if C can tolerate having her fenestration closed.  I find all this pretty interesting because her fenestration will close over time without having any intervention and I do not see how something developing now would make a difference when C has been having these issues since surgery.  We will get our chance to ask more questions in January.  Not sure exactly how many answers we will get but at least we do not need to do anything for now and I know, deep down, her cardiologist will do whatever he can to make sure C is able to fully live, not just exist.  We are very much looking forward to a low key, relaxing holiday season with little medical intervention.

Over It

Yesterday C had a follow up cardiology appointment. She is still being seen pretty frequently as she continues to have lots of post Fontan issues. We had actually gone 4 weeks between visits but alas, that did not last as we will be going back to cardiology next Tuesday.

C is now 4 months post op and still struggling with adapting to the Fontan. While we understood that the Fontan would be the most difficult and stressful surgery for C, we certainly did not think that she would still be limping along this long after the Fontan. The issues that she has now are ones that generally occur after the Glenn but before the Fontan with the Fontan being the great equalizer and fixer of issues from the Glenn. But C has not had any of these issues until she had the Fontan and is now stuck with all these issues with no real "fix" in sight. She has not ever had a problem with her lungs and things like fistulas and avm's have never been mentioned before until now.  She has been extubated twice right out of the OR meaning she has never spent that much time on a vent.  The craziest, most illogical part to all this, and also why the doctors are so stumped, is that C's heart function is awesome and all of her pressures are exactly where they should be.  Her numbers are excellent and her single ventricle squeeze is fantastic.  She is the perfect candidate for the Fontan.  With the way she is struggling, the general idea would be that her numbers are off or her heart is not functioning that well.  Well, not with Carolena.  She just likes to keep things very interesting for everyone.  Of course we keep hearing that the heterotaxy is why she has all this crazy stuff. We are so over that and we are so close to taking her to someone else who may have a better knowledge of heterotaxy.

On top of this, this is now the fourth month that C has run a super high fever. She seems to be on some weird cycle where she runs a fever and gets sick, we take her in and all the cultures are done. Nothing shows on any of the cultures or the viral panel, she takes strong antibiotics for a week and she seems better. Than a couple weeks later she starts the cycle all over again. When C broke with a fever last week we debated on taking her in or not. She has been back to the hospital with fever, etc. four times since her Fontan in April, meaning she has been admitted to the hospital at least once every month starting in April. When C started running a fever again last week, we just decided we have had enough. And selfishly we did not want her to be in the hospital for her third birthday. Believe me, Kevin and I completely understand how dangerous it is for C to run a fever but in all honesty, we are just so down right now I am not sure we have that much energy left to keep fighting. However, we will because C keeps fighting. The most odd thing about C running a fever the last three times is that her o2 sats skyrocket up. I am talking we were seeing her stay at 87-90 one night. We have not seen her sats like that in over two months, with the exception of when she is running a fever. Now that her fever is done and she seems to be better her sats are in the tank again with her no higher than mid 70's and desatting to the low 60's to high 50's at night.

Of course she was blue yesterday when she saw her cardiologist. She averaged 66 on her o2 sats. To say that her doctor was not pleased is an understatement. However, no one has been able to figure out why she continues to have these persistently low sats. We also learned that the sat swings during a 24 hour period are pretty rare. It is not uncommon for C to register 80 and then two hours later be in the 60's. Since no one can tell us exactly why this is happening, there are no solutions to fix the problem.

C remains on oxygen although we do not feel that it really helps all that much. She is really struggling with gi issues, particularly vomiting and not one of us has had a good night sleep in months due to her chronic low sats and vomiting all through the night. Of course GI offers us no solutions and keeps referring us back to cardiology (must be her heart function...um, right...see above) or to speech/feeding therapy (must be behavior/adversion related...yeah, cause she is waking up from a sound sleep to vomit everyone just to stick it to us...) or it must be that darn heterotaxy (yeah, except I know plenty of other kids with heterotaxy who do not vomit everyday and who actually eat).  So freaking frustrating and no help at all.  Even better I get to hear this all over again next week when we see GI...for what purpose, I am really not sure.

Due to all the fevers C is having plus the fact that she continues to develop fluid around her heart and lungs (we found out yesterday that her pericardial effusion and left lung effusion are back) her cardiologist now wants us to see an infectious disease doctor. He wants to rule out the possibility that she has some blood borne infection from all the blood and blood parts she received during and after her Fontan. I was quite stunned when he told us this. I mean really, with all the safe guards and checks that blood goes through now, there is still a possibility that my child picked up HIV. Are you freaking kidding me? Her cardiologist does not think she has AIDS however there is some other virus, CMV, that he wants to rule out. He did say she could get CMV just from exposure anywhere however, the infectious disease doctor will be able to determine if she has picked up anything from the blood she received. I am really not looking forward to that appointment. But, if they can rule out this possibility then they doctors will say that C is just getting a recurring virus, like a cold and it is just taking her awhile to fight it. On top of the danger to C of getting sick we also have to be super careful about the fevers as they could trigger her atrial tachycardia. I am so super on edge every time she is running a fever. I do not want to see her go into tach at home. It was so super scary in the hospital I just cannot imagine dealing with it at home. Ugh.

We are just worn out and so low right now. Carolena however is back to her normal, wonderful, crazy self. You would never know by looking at her that she is having so many issues. She certainly does not act like a child with super low sats and fluid collection. She runs and plays and chats all day. She is back to hit or miss with naps and is pretty damn happy all day. With all this I should be so grateful that at least she is her happy little self but I just cannot get past how hard it will be right now if we lose her. She just seems so normal (relatively speaking) and to know that she is just a ticking time bomb makes me violently ill.

There is not much else we can do at this point other than wait. I am not sure how this journey will continue. C may be getting another heart cath here shortly. I am not really for another cath at this point but I will think more about that as the time gets closer. I am not too sure but we may begin talking about Fontan takedown. If she just cannot adapt to the new physiology, even though she looks like a perfect candidate on paper,  I am not really sure what other options there would be. And then of course, if she just does not do well and takedown is not an option then we will begin the process of listing her for transplant. I am so overwhelmed with all this right now. I never had any grand illusions that surgery would fix her right up and we would not have to deal with issues. I never thought we would be looking at a longevity for her but I certainly held out hope that we would get through the Fontan and then we would be on cruise control until her heart showed signs of significant failure. I was so hoping for all those years where we could all just be a somewhat normal family of 5.

August 19, 2012

Happy birthday to my big 3 year old! Everyday I am reminded of how much you have been through (and still face) and how hard you have fought. I am blessed in so many ways to be the mom of such a special daughter. The day you were born, I remember thinking about what kind of future you would have and just how many birthdays we would get with you. I am truly grateful I was able to spend yesterday celebrating you and all that you have brought into my life. I love you more than you will ever know.

Because I am Still Trying to Find the Time

I decided I would go ahead and post my facebook updates for the last nine days C was in the hospital from her original admission, post Fontan. I would really like to go back and try to write out those days but I just do not see it happening. Each day the memory fades and the sharp emotions become softer. I do remember being very elated after C's post Fontan cath with the expectation that she would truly recover in time and she would not need any further procedures done and then hitting rock bottom as the days post cath dragged on and on and on with no real improvements and no discharge day in sight. So, here are the updates..and of course Blogger hates me so they are going to post in one huge paragraph instead of being separated like they should...grrr. Day 15 Post Fontan - Looking like Friday for discharge. Our super awesome NP last night was rocking on the changes to get C out of here. C is no longer on any iv/pump meds. Everything is oral (g tube) now. Still need to get pacing wires out but C's surgeon wants to wait until Friday as she just switched from iv amiodarone to oral (arrhythmia). Also need to get all the oxygen stuff ready as C will be on continuous oxygen for awhile. Otherwise she is looking and feeling good and ready to break out of this joint. Day 16 Post Fontan - C is still on track for discharge tomorrow. She is super ready to get out of here. She did develop some fluid under her heart that we are watching. Unfortunately Dr. Doom is cardiology tomorrow again. Hope he gets out voted by other doctors because he is the type to keep us here on iv drips until the fluid is completely gone which could take a month like the last two times C did this. Will be praying extra hard that the fluid is decreasing so we can leave. Also waiting on oxygen stuff. Put a fire under them today about that so it does not hold us up either. Day 16 (again) Post Fontan - Well so much for leaving tomorrow. Echo today showed a little more fluid around heart today than yesterday. Increasing Motrin and Lasix. Even if fluid is less tomorrow there is no way Dr. Doom will let us leave. Here's to hoping for Saturday. Day 17 Post Fontan - Well the earliest we are getting out of here is Monday. Not safe to pull pace wires while there is fluid present around the heart. So now need the Lasix and Motrin to work to shrink that fluid. Has worked before so really praying it will work again. Day 18 Post Fontan - Today added steroids to try and knock out the fluid around the heart. If these start working then we are still looking at Monday discharge. C is so over the icu and so am I. Still trying to get better answer about why o2 sats are still so low. Being told to just wait it out does not sit well, especially since having C on o2 24 hours a day is nothing short of torture to an extremely active 2 year old and a full time job holding mama who has to get this all figured out. Day 19 Post Fontan - No Facebook post. From timeline and pictures I can see that the boys were visiting so C was pretty happy and we were all just enjoying our short time together. Day 20 Post Fontan - And we keep taking steps backwards again. Have no idea when we will be getting out of here and I am no longer even going to guess. In addition to fluid around heart, there is more reaccumulating around her left long. So now on iv steroids and going back to iv lasix. Awesome. Both require wean/stability period as well. And to make things even more awesome C is having rate issues again with super slow rate most of the morning. That will also require med adjustment. I am beyond weary. Day 21 Post Fontan - The bad news, C is still running low heart rate, especially when asleep. Continuing to wait and see what she will do once the one med discontinued is completely out of her system. My fear is that she still go in atrial tachycardia again. But must trust her EP doctor to know what is right. She continues to be on one med for the rhythm/rate issues. Will see if it is enough. The good news, C's heart ...continues to show impressive function with little to no valve leakage. The iv meds are working and the fluid around her heart is decreasing. Enough so cardio put her back on oral steroids which she will continue at home for a little while. The iv lasix is also knocking out fluid around lung. Still will get that by iv for now but could start wean to oral soon. The best news, we spent 2 hours in the playroom doing crafts, coloring and painting and got a visit from Gracie, a therapy golden retriever. C was beyond thrilled to see a puppy. Really hope we are headed in the right direction and can go home soon. Need meds to keep working and no tachycardia arrhythmia. Day 22 Post Fontan - Another day with no Facebook post. There where a couple days where Facebook was also giving me problems and not posting my stuff. Of course I do remember just being so discouraged at this point it is entirely possible I just did not feel like posting anything. Day 23 Post Fontan - Well, looks like the big day is tomorrow. C should be going home. I have not posted sooner in effort to not jinx the good news. Everyone around here is in serious discharge planning mode. Although C's heart rate at night remains lower, it is not going a low as 3 nights ago. EP cardio is happy with where she is and is cautiously optimistic that the one med will continue to hold her rate and rhythm ...steady. The fluid around her heart and left lung continue to decrease so as of today she is back to receiving all her meds by gtube. C will still be going home on oxygen, 24/7, but her oxygen sats are starting to look a little better. I am very grateful and seriously humbled by all the family and friends (new and old) who have said prayers for C. Thank you. Hoping my next post will be pic of us heading south on I75. :) Day 24 Post Fontan - This was actually C's discharge day. I was so super excited to be getting out of the hospital and taking C home. But the elation is tempered with the knowledge that my child was still very, very sick. There is just such a dicotomy of emotions because you know you are going home but what does going home mean? I admitted my child on April 10 for her Pre Fontan cath. C was a bubbly, super happy, super content, mischievious, getting into everything, independent, smiley, could not tell her apart from any other 2 year child. I had the utmost respect and trust for the surgeon and the doctors and nurses who would be working on my child and taking care of her but in the end what I took home was such a pale version of my child that I hardly recognized her. She was weak and tired all the time. And now, now she looked like a medically fragile, special needs child. She has the evidence all over her face with the nasal cannula and the oxygen tank. She is much more subdued and quiet. I miss her vitality and energy and smiles and laughs and her words. She was just really starting to talk. I hate that she feels bad all the time and is concerned and worried all the time. I hate that she has to go through any of this. Our whole plan of cath and surgery back to back was to prevent her from having multiple hospital admissions. Trying hard to keep her from being emotionally scared as well. Too bad that has not worked. And here we are, nine weeks later and C is still limping along and all the doctors have pretty much run out of answers. We get a lot of "well, we just have to wait" and "I don't knows." Kevin and I have been seriously considering taking her somewhere else in an effort to find an expert in heterotaxy who can give us a second opinion. We may be there sooner now.

One More Time for Good Measure

Yesterday marked the 42nd day C has been in the hospital since she was first admitted for her pre Fontan cath. Granted, she has not spent all 42 days in a row in the hospital. She has actually been home for 22 total days in the past 64 days. 64 days. 64 days we have been living with C post Fontan and all the complications she has had from the surgery. I am so over the Fontan it is not even funny. I know logically this is really the only real chance C has to extend her life for any reasonable amount of time. However, the Fontan has been horrible to her so far. Last night we got back from the hospital yet again. Her second re-admission since the Fontan. She continues to produce fluid and it continues to collect around her heart. Yeah, awesome. The best part is that the pericardial effusion post Fontan is not what "they" generally see. Pleural effusions, fluid in the lung space, are the common complication from the Fontan. The pleural space is where the fluid collects and this pleural effusions are what keep kids in the hospital for so long. Not pericardial like C's. Oh no. Not my daughter, the total 1%. IF the doctors tell us there is a 1% chance for a particular complication, you can pretty much guarantee that C will hit that 1%...and let me tell you, I am very, very tired of her being the 1%. If I had my choice her 1% would be in belonging to the 1% on wall street or the 1% who win the lottery. Anyway, after have massive amounts of Lasix pumped through her system with the result being an very high heart rate (which she hates and her sats hate and we hate because we are terrified it will trigger her atrial tachycardia) and the massive amount of Prednisone, the steroid, resulting in my precious 2 year old daughter looking like she is ready to suit up as an offensive lineman for the Gators the doctors have decided that they need to be more aggresive in fighting the ever persistent pericardial effusion. So, off to the hospital we go, again. And off to surgery C goes again. This time to have a thoracotomy with the placement of a pericardial window. Um, yeah. Whatever that means. In all actuality, this approach was much nicer and kinder to C. This procedure in normally done through the sternum or just below but both result in the cutting open of her sternum again. Thank goodness for C's rockstar surgeon who did not want to do that. So, he actually went in her left side, between her ribs to access the heart. So although she now has a brand new scar, yuck, she did not have to have her sternum reopened nor did she have the bleeding out issue which we would have to be prepared for by the surgeon going through all that scar tissue in the front. The surgery went well and C did awesome. The pericardial window is where the surgeon removes part of the pericardium allowing for the fluid to flow in the pleural space. Basically we took away the pericardial effusion by giving her a pleural effusion, which she did not have before. Acutally, she has had like no fluid collection around her lungs, aside from immediately post surgery when she was bleeing out into the left side. See, the 1 freaking percent. The hospital stay was not awesome. Not that any really are awesome but this one just really sucked. We were originally put on the main floor, um, really? And sat there for 4 hours doing nothing until the cardiologist used his pull to get us into the cardiac icu. Apparently the hospital was just slap full up. The cardia icu and the picu. We opted to go back to Tampa instead of St. Pete. Tampa is where she was for the month after her Fontan. Finally we get moved and then they are like, now we need an iv, etc. I think Kevin was about to blow a fuse. He actually came home early from a business trip to Atlanta since we were informed of how bad this was for C. So to sit on the floor for 4 hours with nothing and then have to go through all this crap around 7pm with shift change and C's eating and bedtime just made no sense at all. So, surgery was the next day and all was good. Then on Sunday, the day her 7th chest tube was pulled, and 3 days post op I am informed they are going to move her back to the floor. I was all like, oh hell no you are not moving my child to the floor. If she is stable enough to go to the floor, then we can go home. I know more about my child and taking care of her then the nurses on the general floor do. We lasted another day in the cardiac icu and then on Monday we were moved to the picu. I am ok with the picu, at least the nurses there are trained in critical care and they cross train in the cardiac side. But, I was still pushing to get C out of the hospital. Tuesday was the magic day. She is still on lots of Lasix, but thankfully less, so she is not as dry as she was and we are agressively weaning the Prednisone as it does not seem to be working anyway. Now we wait. Again. Awesome. We did not actually fix the problem. C is still producing too much fluid. Motrin and then Prednisone are what is used to try and stop the fluid production as both are anti-inflamatories with the Lasix acting the dumping mechanism. But being as though the Motrin and then the Prednisone have not seemed to work to help stop the fluid C now has the pericardial window. Which of course will only stay open for about 3 to 4 weeks. So in all actuality, we could still be looking at ways to address this problem in 3 to 4 weeks. Fontan take down has been whispered. But, C's cardiologist and surgeon are not on board with that yet. I say yet, because who knows what will happen if C continues to struggle and limp along like she is doing. Oh, and of course she is still on oxygen as nothing at all has helped bring her saturations up. I am bitter. There is no other way to say it but that I am bitter. C does not deserve this. I handed my energetic, full of life, happy, smiley daughter over to the doctors with the utmost trust and this C is what I have in return. Every day I work to not be bitter and try and adjust and be grateful for C still being here. C is not the child in the cardiac icu who likely died today after being put on ecmo two days ago. She is not the other child on ecmo in the icu either. She walked out of the hospital yesterday with a perma-grin on her face, truly happy to be going home and seeing her boys and for that, for that alone, I will be grateful.

Still Waiting to Exhale

Two and a half years ago I took a huge, deep breath. I had just found out about my tiny, unborn daughter's majorly defected heart and told I should terminate the pregnancy. After getting a second opinion, my husband and I decided there was no other alternative. We were going to have a daughter, our third child, and we would do whatever it took to give her a good, quality life. I have sat by my daughter's side through countless doctors visits with multiple specialists, numerous gi tests, hours upon hours of therapy, multiple heart caths, three major heart surgeries, endless hospital stays and several bouts of life threatening tachycardia. I have seen more pain and suffering and know far more about the pediatric heart than any parent should ever see or know. And I have continued to hold my breath. Ever fearful to let down my vigilant guard or death would creep in and take my child and she would be nothing more than a statistic. Death hovers everywhere when you have a child like C. I do my absolute best to fill her world with light and sunshine just to not see the darkness gathering around her. Two and a half years of fighting for the absolute best for my daughter and I am proverbially as blue and she actually is blue. After everything C has been through, this last surgery was suppossed to be the top of the mountain. We climb that mountain, reach the top and we were suppossed to have a much smoother climb down. I was never given any guarantees or false hope, however, the thought process is that the first two to three years are the absolute most horrific and by getting through this, then we come out on the other side of cautious optimism. There are not supposed to be any more planned procedures. We are supposed to sit back, enjoy what time we have with C and wait for the next step, many years down the road. And yet, that is not what has happened with C. I am still holidng my breath, waiting to exhale as she continues to struggle and limp along post Fontan. She was supposed to rock this surgery. Her pre Fontan cath was the best you are going to get with a kid like C. She was the A on the bell curve of heart defects. And then the Fontan came and almost knocked her off the curve completely. C continues to have lower oxygen saturations and continues to be on oxygen 24 hours a day. All the doctors are perplexed and not offering much in the way of answers. C and I got to spend all of two weeks at home before she was back in the hospital for another nine day stay due to an ever persistent pericardial effusion that just would not go away. She had to have another pigtail chest tube placed, making six total tubes/drains placed in her two year old chest since surgery. She is back on massive amounts of steroids with a much slower wean down this time. She is swollen and puffy and just miserable. She is tired all the time, lacks her usual vitality and energy and is generally more hesitant and less curious about everything around her. She is running an elevated heart rate which makes me super concerned that the ridiculously poisionous anti-arrhythmia med she is on (you know, the one that will eventually do more harm by poisioning her than good) is not working well enough to control her atrial tachycardic arrhythmias. And this drug is the best one they have. She picked up another specialist and medication when she started having seizures in the hospital. Which is all very perplexing as well since she showed signs of clinical seizues and subclinical seiures on the eeg but those were not as concerning as the big episodes she was having that looked like she was not getting enough blood flow/oxygen to the brain. Again, the doctors are not sure why and are not offering any answers. You want to know the irony in all of this...C has not only stopped throwing up but she is actually eating, by mouth. Freaking unbelieveable. All along the doctors have told us she is very unique, even more so than the standard differences you see with cardiac kids. C has always done better and looked better than her numbers and statistics. Even now, she continues the trend. However, she is still very, very sick and I just do not know where to go and what to do from here. Our goal has always been to provide the best quality of life for her. We know, unequivically, that we do not have quantity with her. The best we hope for is twenty years before transplant becomes her only option. Maybe more if C is really, really lucky. But with the way things have been trending lately, I do not count on that. I pray every day for God to heal those areas in C which he can heal. I know logically that her heart will not be healed. She is not going to all of a sudden grow another ventricle. My hope is that we can go back to where we were before. No noticeable arrhythmias that threaten her life. No lung damage or heart issue causing the lower saturations and oxygen need. No more procedures neccessary to figure out what is going on. I just want to be able to release this breath that I have been holding for two and a half years and let some of this stress and tension and worry go free so we can enjoy our time together. Summer is here and I do not want C to miss out on doing all the fun things we ususally do because she is sick or on oxygen or whatever else may be wrong. I need to be able to exhale soon or I am going to pass out.

The Short Update

I know there are some people who are following C's progress only through the blog, so I do apologize for the lack of updates. We did finally make it home on Friday, May 4. C was in the hospital another 8 days after we were first told that we would be discharged. I have a lot of mixed emotions right now and I just have not had the energy or desire to write about C's progress. I will keep working as I do want to finish the posts on the rest of C's journey post Fontan as I really like to have a journal of what she went through. However, we are home. C is getting better and stronger each day. Her body is slowly adapting to her new physiology and starting to stop producing fluid. But, she is still on 1 liter of oxygen as her oxygen saturations remain severly low. I cannot even get into that right now but know that all the prayers and well wishes have and are working because C is home and doing much, much better.

Day 13 Post Op and Cath Day - Again

Day 13 - Tuesday - C did well overnight but was grumpy and uncomfortable. Started back on Tylenol and she would feel better, so figured she was just still feeling some lingering pain. Got back to her pretty early as wanted to spend as much time with her as possible before she had to go for her cath. Our awesome nurse for the day went and got a whole bunch of stuff C to play with including a little table and chair so she could sit somewhere other than the bed. I am so grateful for this because getting C out of bed is crucial. The nurse brought all kinds of fun stuff to play with including markers to color, play doh and C's favorite, bubbles. We had a grand time playing with all this stuff and C even played with the intensivist and cardiologist blowing bubbles and coloring. So good for her but sad to me thinking that in just a few short hours she would have to go under again and what if this time we really did lose her. Fortunately, one of my dear friends from childhood came by to visit and kept my mind off of what was coming up. She works for the hospital administration that oversees the hospital we are in and she wanted me to know that she had made contact with the higher ups about moving C to the picu like they did. Very grateful for at least we had our concerns heard. Cath was supposed to be at 11:30 but as usual things always run behind so they did not come to get C until 1pm. The cath nurses and anesthesiologists came up to C's room instead of making her go to the pre-op area and wait. They let me ride on the bed with her all the way into the cath lab and stay until she was asleep. I am so grateful they let me do that because C was so calm and not all at scared. Less trauma. Cath actually did not take that long, an hour maybe, surprised the hell out of me. So at first I stated to panic thinking he found something horrible that could not be fixed or something that could only be fixed with surgery. Again, to my surprise, he did not find anything wrong. No collateral, no narrowing and nothing additional opened up. Um, ok. Pressures are great in heart and lungs. Function looks great. There is absolutely no fluid in her left lung. All very good news. But still no explanation for the lower sats. The only things he found were oxygen desaturation coming back from left lung to heart, not great but not horrible either. She is shunting a bit more across the fenestration than they would like, but again, not horrible. And she has a pericardial effusion (fluid around her heart) but small and not horrible. He did place another chest tube, small, like the pigtail ones used for the lungs but the thing was not actively draining so he figured he pretty much got it all during the cath. So after all of that, no real answer to why such low sats even on oxygen. The cath doctor told me I was right, there was nothing there and the plan would be to send her home on oxygen and wait for her left lung to heal completely. Awesome. I so enjoy not getting any answers. But at least the news was all good about her heart. Even better, the anesthesiologist did not intubate her. He worked extra hard and just used her cannula. She did awesome and was awake pretty quickly. I cannot even tell you how relieved we were. So back to just waiting her out. The awesome NP that night was working overtime to get all of C's stuff done so we could move towards discharge. C was done with the heavy duty iv antibiotics so she went back to her normal amoxicillian. She got the EP cardio to change the iv amiodarone to oral, stopped the milrinone and increased the oral enalapril, and switched her lasix to oral to start her lasix wean. With ner chest xray clear, cath confirming no fluid in lung and cath draining of fluid on heart we were looking god to go. The NP got all her prescriptions written and discharge orders done. She also got the pacer disconnected, although wirex still remain. Now we are starting to see the light at the end of the tunnel. We are starting to see home. Neuro did visit Monday confirming there was nothing concerning on the ct or ct angiogram and that we would follow up with him in clinic. Plan is to see how C is doing and maybe get rid of seizure med at one year. Right now he wants to keep her on meds and let her brain relax. Fortunately the med she is on is the safest one they have with few side effects and no interaction with any of the meds she is currently on. So there is that. Now waiting on pace wires to be removed, chest tube removed and C to show she can tolerate all her meds orally. Plan is to have tube and wires removed Wednesday. Have oxygen people come by to give us the oxygen and supplies and then hopefully get out of here in time to be home for the weekend. For the first time in over 2 weeks, C can sleep unencumbered by wrapping tubes and pump alarms going off. She just needs to behave to keep her monitor from alarming.

Day 12 Post Op

Day 12 - Monday Talk about being on pins and needles overnight and the next morning waiting to learn when C would have her cath. We had pretty much determined from everything we heard that this was no longer a question of if C would get a cath but when C would get a cath. The waiting is horrible but there is not much more to do when in the hospital. We go through rounds and everything is pretty much the same. C is still progressing but there will be no more changes until cath day and time is determined. So pacer is still connected even though she no longer needs pacer help. The iv amiodarone and milrinone stay. Obviously the pacer wires stay. The iv lasix is still on full force. However, they do decide to remove the trans thoracic line. Awesome news as that thing has been in for almost two weeks and with one line out of two clotted the thing is just sitting there acting as a possible breeding ground for infection and removing it b also means more moving around for C. One down side, they have to get very creative with C's pic line, the last remaining line, because her amiodarone cannot be mixed with lasix or one of the iv antibiotics that she is still on. So the nurses have to be very diligent about stopping the amio drip, flushing the line, running the lasix, flushing the line and then restarting the amio drip again. Gee, could you possibly give me anything else to worry about at this point? Now I an all freaked out the nurse will mess up and not keep them seperate and C will have a dangerous drug interaction. Freakin awesome. But our day nurse is fantastic and she has it all worked out and is able to give It to the night nurse. C tolerated the tube pull well and was a happy little thing when I saw her again. Even with the weird way her meds had to be run, I am always glad to see things disappear out of C and out of her room. Not too much later we got the call that C was scheduled for Tuesday at 11:30 to head to the cath lab. Well, guess we are doing this after all. Total terror. Poor C. Another procedure, more stopped feeds when we were just getting back into the swing of things and more anesthesia. Ugh. Her cath doctor did come by and talk with me in the afternoon. I could tell he was still hesitant but got out voted by the rest of the team. He is the one who has been dragging his feet trying to give her more time to adjust. However, he did say that he is just as concerned and perplexed as everyone else as to why her oxygen saturation is remaining so low even with oxygen on. He also said the risks are the same now as 2 months from now. I asked him about just letting her go home on oxygen and see how she does and he said if he could do something to avoid that then he would like to do so. The reason we had already waited so long on the cath compare to to what the other doctors wanted was because our cath doctor was on a mission trip to Jamaica the while last week. So, off to the cath lab Tuesday for C. I am continuing to get C up and walking 3 to 4 times a day. Now instead of wanting to go walk she wants to stay in bed and cries to get back in it. I am having nothing of that though and keep getting her up. I think she has figured out that she is not actually leaving and therefore has no desire to walk through the unit again and again. I would take her out of the unit except I fear she will never come back in. She is still tolerating her feeds and is pooping ljke a champ. Think she is making up for lost time. Kevin says again that the night is uneventful and she is getting more sleep at night. The monitor alarms and the pump alarms still freak her out though. Kevin has decided that he is going to invent some type of pump that will not alarm in the room but will alarm to the nurse directly. Considering the pumps generally are just letting the nurse know that a med or flush is done, sounds good to me. Maybe he will make us a bunch of money :-) Trying to get a decent night's sleep with a cath looming over our heads is near impossible.

Day 11 Post Op

Day 11 - Sunday Well, I spent the night with C and heard her monitor go off all night long sure to her oxygen saturations being too low. She mostly hung around 70-73. Really, really low for a kid post Fontan. So, I was super nervous when the doctors started rounds because I just knew C was out of time and the doctors were going to push for the cath. Sure enough, when C's EP cardiologist came by he said it was time. C would be discussed at cath conference Monday with cath possibly as early as Monday afternoon. Awesome. Of course I was scared as all get out to put C through more anesthesia and another procedure messing with her heart, you know, since the last one went so swimmingly well. Apparently I was nervous enough and terrified enough that C's EP cardiologist actually patted me on the shoulder to try and reassure me that it would be ok. I also asked what would happen if they did not find anything with the cath and was told that they would definitely find something. Ok. Still not completely reassured and wondering if it is really worth the risk if they are basically just going fishing. EP cardiologist did tell me that the cath risks now versus two months from now would be the same. And he figured if she had a problem would be easier to get hold of while she is still in the hospital. Nothing else changed as so many things were done Saturday. We continued to get up and walk and move around. C definitely needs to get out of bed. She has been laying there far too long and needs to get her strength back up. So, not super comfortable night as she was still hooked up to the pacer, still has pic line and still hs the trans thoracic line. Kevin was back and started with her Sunday and reported an uneventful night. I finally got her into some type of schedule and she was asleep when Kevin got back. She continues on her home feeds and juice and is tolerating it really well. So much better than I expected and I hope the trend continues.

Day 10 Post Op

Yeah, behind on updates again. But, again for good reason. C continues to make forward progress and with forward progress means spending more time with my angel and her spending more time on my tablet :-)

So Day 10 - Saturday  -

The doctors decided to push C to see how much she would tolerate and how well she would do. Did not see neurologist as he is not in the hospital on the weekends but already heard from cardiologist that her ct angiogram was clear. There was no sign of stroke, no clot, no annuerism and no injury to her brain. We were so relieved to get that news but still needed to hear from neurologist after he reviewed the scan and what his plan would be from there. Since got good news on scan, did not mind waiting until Monday to see neurologist.

C's EP cardiologist was on service for the weekend which was awesome. Hence why he was willing and able to push her when staff is reduced on the weekend. So he cut her iv meds way down again. Both her amiodarone and milrinone were cut way back. The milrinone could have been stopped and the amiodarone could be switche to oral but he was stil thinking impending cath and did not want to take her off the iv drips. The pacer also stayed connected. He turned the rate way down but left it on and connected. C has did well with that all day and has stayed in sinus rhythm. Very good.

They decided to see what C would do with no oxygen on at all, so she was switched to room air from 3 liter flow and 100% oxygen. I know, big switch right? But she did well all day and managed to keep her oxygen saturations in the 80's. Unfortunately both the flow and oxygen had to be turned back on as C desatted all night and hung in the very low 70's. I did night watch Saturday as Kevin went home to be with the boys. His mom was having a party for her 70th birthday and all of Kevin's family was in town. He was looking forward to going and so were the boys. Kyle also had a church retreat on Sunday for his sacremental prep and Kevin really wanted to do that with him. So, Kevin got to see the boys and sleep in our own bed. Somewhat jealous on both accounts but he totally deserve that night of sleep after all the nights he has spent in the hospital with C.

C also had her chest tubes pulled. Finally. Her xrays showed clear lungs and there was no further drainage so out they came. Oh what a happy girl C was once those came out. She was more comfortable than she had been since surgery. We were still giving her Tylenol as needed to make sure she had no pain.

The best part of Saturday was that they finally let her get out of bed and walk. She was so excited. Unfortunately she thought it meant we were leaving for good and threw an absolute fit when we told her she had to go back to her room. She was so worked up and screaming so hard she actually raised her oxygen saturation by opening up her lungs. But as much as we hoped the lower sats were a product of her lung she kind of proved us wrong when she could not keep them above 70 that night.

We also continued on with her feeds as we do at home. She did vomit once and of course all over herself so that required dressing changes. Awesome. But getting her up and walking really helps get her bowels moving.

Overall we had a great day and some hope we could avoid another cath before going home.

Day 8 and Day 9 Post Op

So I am a little behind with the updates. This time for a very good reason. Days 8 and 9 have been great for C. She is finally turning the proverbial corner and moving forward faster than moving backward.

Thursday - Day 8

Finally I get to hold C! Her bed needed to be changed and I got to her room just in time to hold her while tha was bein done. Of course I held on to her for quite awhile. I even refused to put her back on the bed when the eeg tech came to remove the eeg leads from her head. I told her we would move the bed a little so she could get to C's head and I did not care one whit if she got the remover solution on my shirt. Leads came off, C fell asleep (a real sleep...you know the ones I am talking about), and the neurologist came in. He said the second 24 hour eeg looked the same as the first one she had. He said he was still seeing the crazy patterns on the eeg when she was woken up, hyperventilated or was messed with. He still wanted the ct angiogram done so he could see her brain structure and where blood and oxygen are going. She was suppossed to have that this morning but ended up gettin postponed until Friday because after our very awesome nurse called and talked to ct several times beginning at 7am, in all the times she spoke wit them trying to get clear instruction, noone told her C would need to be heavily sedated. Um, yeah. So, our nurse was super pissed because she had everything ready, everybody ready and then at noon, they tell her about the sedation thing. She had already planned to have the NP here give a small hit of versed to keep C calm and then she and the NP would be with C the whole time, We are allowed to go with her and stay all the way to the actual machine running so th would stay through that. But, the kind of sedation ct was requiring meant the intensivist doctor would need to do the sedation and would need to stay with C. Two problems, the intensivist had told us just an hour earlier on rounds that we could go ahead and feed her. She wanted C feed and with fluids, getting her back to her normal. So, we fed her..therefore, she could not safely have sedation for at least 4 hours. That woud push the test time to around 5pm and noone wanted to do that. Second, the intensivist was not available. She had already moved on to the other patients she needed to see. So, our awesome nurse reset the ct angio for Friday morning, after really giving the ct tech what for, as she should. Everybody has C's back here. Rounds were actually fine. This day th whole group, including C's surgeon, was present. Does not happen like that every day. Some days you will see the doctors individually. Anyway, told us C would be on 7 day course of antibiotics because blood showing infection markers and with no spleen, noone wants to risk C getting sick. At this point, she still has several lines in including: arterial line in groin, two chest tubes, trans thoracic line, pacer wires and her pic line. Still, leaving iv drips on but cutting back on amount a little more...this is amiodarone (arrhythmia drug) and milrinone (heart function drug). Chest tubes still draining so those stay. Pacer turned down a little more just to catch C's heart if she goes too low, like during sleep. Oxygen wean is on hardcore since she has done well with no nitric. She was dropped from 8 liter flow to 6 liter flow. But of course the down side is still her lower oxygen saturations. She was put on he cath conference Monday so the whole team can talk and decide what to do. C had a relatively stable night, slept well and was pretty happy going into day 9.

Friday - Day 9

So I get to C right as they are getting ready to go to ct angio. We have another of our favorite nurses and I am so happy. Plus, the decision was made to have the pediatric cardiac anesthesiologist come and sedate C and stay with her the whole time. The intensivist was being super cautious because of the seizures and meds she is on for that and th arrhythmias ad the meds she is on for that. So awesome she is so complicated but I was so very grateful that the anesthesiologist agred to do this for such a small thing. C sailed right through the whole thing and popped right back from the sedation. My husband and were so grateful. The cardiologist on this week came in and said the last 24 hours have been the best for C since surgery. Finally, 9 days after this nightmare began, C is leveling out. I felt kind of bad for this cardiologist becaus I think he thought he was always giving us bad news, so when the ct from Wednesday came back clean, he was practically giddy to give us the news. Then when the echo came back with great function he practically ran into the room with the news. I am pretty sure he was so happy to end the week on an upswing. Of course we are super happy C is finally coming around. Today she was awake most of the day. I got to hold her again and we ate yogurt. She is back on full feeds of the food we make and she is gettin her apple juice/Gatorade mix. We watched movies, played games and did more of our normal stuff. C is able to move around more because the intensivist won out and she had C's arterial line removed. Plan still same, wean, wean, wean and feed. CT angio came back with radiologist report of unremarkable...meaning C's brain is just fine. Blood and oxygen going where they need to, structur is good and there is no evidence of clot, annuerism or stroke. Awesome news. C is down to 4 liter flow and staying steady. Only negativity is the possibl cath next week hanging over our heads. We obviously would prefer to wait it out a little longer and see what she does. She is she can apapt at home and have her sats come up. Everything else is pretty much the same. She still has iv meds and still has pacer attached even though rate is super low, just to catch if she goes too low. The very best thing of all, the last two nights I have been able to lay with C and get her to sleep. She is going to be so spoiled but honestly, I don't care if she wants to sleep in my bed every night at home, she can.

Day 7 Post Op

Well, here we are. One week out from surgery and C is still struggling to get back to herself. 7 full days after surgery.

This Fontan surgery was not suppossed to be this difficult. Coming into this surgery, we already knew about the things that would be a challenge. We knew she could develop pleural effusions, fluid around the lungs, keeping us here while the fluid drained. We knew her body would have a harder time adapting to her new psyiology, new circulation. We knew all the same risks and complications from the previous two surgeries. Even knowing what we did, I was not prepared to see C, 7 days later, still struggling. She was such a super, rockstar the first two surgeries. She sailed right through those with minimal, to no complications. Our roughest patch to date was when she went into arrhythmia 8 days post op after her second surgery. Even with that, she sailed through that actual surgery and was her sweet, smiley self by day 3.

Now here we are, after surgery, and C is the sickest she has ever been. The most ironic thing, she was super sick before her previous two surgeries and she rocked them, coming out better than before. This time she was super strong and the healthiest she has ever been. She was in the perfect place before going in. And, now I am living my very worst nightmare. As I was finally coming to terms with the fact I would indeed have a child with an unfixable broken heart, I realized there really were many things I felt I could handle. The one thing I never, ever wanted is for C to suffer or die in the hospital. I have always wanted a much more comfortable ending for her, surrounded by love and softness during such a harsh time. These are things you will never get in a hospital. Instead you will get medical personnel, people with pity faces and curious parents in the icu for other kids who can't help but look but are secretly thanking God that your child and not their child just became a statistic.

Have I mentioned how much I really hate being in the hospital. I am eternally grateful for such a fabulous institution and the incredibly selfless people who work to help save kids like mine, but I hate the hospital.

So 7 days later, C is still in the hospital. Tonight makes the 10th night I have been away from my home and my family has been seperated. 10 nights of extreme stress and worry. 10 nights of not sleeping in my own bed. 10 nights of being away from boys and C seperated from her brothers. And as of now, I have no idea when the end will be. All Inpray for is that we do inded get to leave with C and the C we had before this awful nightmare began.

Now for the positive news of the day. The EP cardiologist came by again this morning and he told us C is finally back in sinus rhythm (normal rhythm). She no longer needs to be paced to break the super fast a tach she was in. He even was able to discontinue one of her three meds sne is on for the arrhythmia. Yesterday he had made that med a half dose and today, gone. He is still monitoring to figure out which medication and how much of it C wil need to keep her in sinus rhythm. Regular cardiologist told us her left lung continues to clear and look better each day. Her heart function is looking good and he will consider weaning her off the iv med she is getting right now to help function. He was a little concerned with her bloodwork today and becaus she is heterotaxy and aspleenic he had cultures run again and put her back on the high powered antibiotics. At this point, I am not arguing. The very last thing she needs is to get sick. Heartvwise, C is heading in the right direction.

We saw the neurologist again this morning. Still trying to get a feel forvthis whole brain thing and how the doctor is. I was not completely sure I understood what he was saying other than 1) yelling at us for blocking the camera too much, 2) yelling at us when we asked why she seemed so unresponsive to us and he got in there and really woke her up and 3) that he does not think she is having tecnical seizures. He told us what he is seeing loks more like vascular restriction when she is upset thus preventing oxygen from getting to all areas of her brain. At first he was going to add a seative on top of the seizure medication but after we asked him to come back in and have him chastise us some more, he did monitor her a bit while on the eeg and decided the sedation was not necessary. However, C was reconnected to the eeg machine for another 24 hours and he ordered a ct angiogram for her to check brain vasculature tomorrow. Hoping we have more answers tomorrow because everyone seems confused including the neuro doctor.

We really only had one big, bad set back today and that was involving C's line. Poor kid just cannot get a break. She is so super hard to get a line into and she is still on iv meds that absolutely have to go through a stable line like a central line. And wouldn't you know that not only did she lose her very last peripheral iv in her hand, she also lost one of her trans thoraic lines. Of course she had to lose one of the stable central lines and of course one of the meds she is on for the arryhthmia cannot be mixed with other drugs and of course she still is showing depeltions in areas that need to be replenished like potassium and of course she is still on heavy diurectics....grrrr. So she had to have a pic line placed. Seriously. Right now when she totally freaks out and her body shuts down and she has some weird seizure like episode when anyone touches her. Ugh. But, she did well and they got the pic line placed. Much better alternative than having the intensivist place another central line in her neck. So all is good. Then they come back to mess with her because the xray showed the pic line was flipped the wrong way. After doing their stuff, xray had to come back. By then C was just done again and she prety much went to sleep after that.

However, after the pic line was placed and before the pic team came back, I got too see my C again. She was wide awake when we came back after the pic was placed and she said mama, twice. Joy. Then we played games on the tablet for quite awhile and we played with the minnie mouse balloon I got her. And the best thing of all....she laughed. She was laughing with me. I just so overjoyed to see me C is still in there. After seeing her all day yesterday in a catatonic state, I cannot even describe how incredible it was to see my sweet angel laugh with me while doing silly stuff.

I am really hoping C is finally turning the corner to recovery. Although she is not back on food yet, stopped yesterday after the first seizure/episode, we were able to give her juice through her tube. Taking it very slow right now as everything seems to upset her. Getting more meds through gtube instead of through iv, always a step in the right direction. She was dialed down on her other iv arryhthmia med and they may turn tne pacer off tomorrow which means she is closer to getting her pace wires out. We are getting there, I may be starting to see a light at the end of this tunnel. Hoping tomorrow brings more improvement and no further set back