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.
Friday, February 15, 2013
Monday, February 11, 2013
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.
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.
Tuesday, February 5, 2013
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
http://www.heterotaxy.org/Pages/what_is_heterotaxy.aspx
http://www.chop.edu/service/cardiac-center/heart-conditions/heterotaxy-syndrome.html
Friday, January 11, 2013
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.
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
Friday, September 28, 2012
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.
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.
Wednesday, August 22, 2012
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.
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.
Monday, August 20, 2012
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.
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