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.