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.