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.

3 comments:

Sharron Johnson said...

Ashley,
Still praying. Looks like you're doing the right things, concentrating on the good and dealing with the bad as it comes. Will be praying for a relaxing and uneventful Thanksgiving and Christmas. Really glad to hear you'll be able to continue seeing her EP, what a blessing! Praying for you, C and the whole extended family....

Jennifer Wade said...

So glad to hear that things have settled down and that C is making progress in so many areas.

Did C's infectious disease doctor ever say anything about the fact that her missing thymus could be related to her T cell deficiency? The thymus is an immune organ that has to be removed during open heart surgery in newborns that is responsible for T cell maturation...they say our kids are supposed to have adequate T cells without it, but C obviously doesn't always follow the rules!

Jennifer Wade said...

A bit more digging revealed this new study: http://www.jci.org/articles/view/39269, showing that early thymectomy can affect T cell counts in young adults.