Archives for December 2010

I am ready for 2010 to be over with.  It was a really, really rough year.  If I listed the good and bad in a list, I will have to say that the bad definitely outweighed the good this year in terms of quantity.  Daddy getting laid off in February and being unemployed for 7 months, almost losing Hannah this summer and her being hospitalized for 6 weeks plus her regression and continuing recovery, moving from Texas to Nevada while she was hospitalized (never did get to say goodbye to our Texas house or our Texas friends), and just the realization of Hannah’s disease on our relationship, kids, extended family, and friends.  Oh yeah, can’t forget trying to sell our Texas house and having to pay double mortgage/rent for 6 months when we were already in a financial stress zone!

Quality-wise, the good realized at the end of 2010 will still outweigh the bad even if the listing on the bad side is longer.

• Hannah is still here, still fighting.  To say she is an amazing little gift is obvious, but it is so hard to put into words how true this is.
• Ethan and Abigail have proven to be such amazing older siblings to her — their patience, their enthusiasm, and their acceptance is better than anyone could have ever hoped for in a sibling relationship (if only they could be that good to each other! LOL).
• As tough as this year was, my relationship with my hubby is still as strong as ever.    We are talk about the tough things, we partner together on raising and disciplining our kids, and we still make each other laugh and smile.  Even though our relationship has been tested beyond anything I could imagine, we still are best friends.
• Reality checks.  There are people in your life, family and friends included, who show their true colors during difficult situations such as these.  People that you think would be Hannah’s number one fighters and supporters turn into those that disappear from your life and don’t want to be a part of Hannah’s life.  Then there are those you have not been close with in many months or years who become your biggest champions.   My heart has been very twisted these past couple of years, but I have realized that my energy needs to stay with those who nurture me not hurt me.
• I need to focus on me now.  I have disappeared.  I feel like for the past couple of years, these past 7 months especially, I am only a mom and wife.  I feel like I have forgotten how to be a woman and how to be ‘me.’  My health, my weight, my self-esteem, and my positive relationships all need to be worked on this year, and as hard as it may be to find time to do it, I do need to try.

I am definitely looking forward to putting 2010 behind me.  I am scared to see what 2011 brings.  I am nervous as to what my thoughts one year from now will be because of what had transpired the 12 months before.

I need to fight even harder for her in 2011.  Time is running out for Hannah.   I need to do more, ask for more, and give more…in so many areas.

The most important thing that Hannah is teaching me that I will bring into 2011 is that I need to be even stronger, more motivated, and truer to my self.  I owe it to her, Ethan, Abigail, my hubby, and most importantly, myself.

We finally got a followup for Hannah’s ‘slightly enlarged aorta’ that was first noticed on our initial trip to the NIH almost a year and a half ago.   It was my fault really.  It was just not high on my list of specialists to get settled, but I knew it had to be done once we were settled.  She last had it checked out back in February in Texas, and they said to follow up in a year.  So we did.

Hannah’s new cardiologist, Dr. Cass, seems like a good fit for us.   She asked the right questions regarding Hannah’s condition, and she was very up front with information.

Hannah had her EKG done first, and then Dr. Cass did the echocardiogram (the ultrasound) herself.   She did note that Hannah’s aortic root is still slightly enlarged, but it is no different really than it was when it was first found out.  So, it seems that in this case, Hannah ‘just happens to have’ an aortic root that is bigger than normal and not by much.

She also noticed some very slight mitral valve regurgitation, but she didn’t feel it was significant enough to show concern at this point.  Just something to keep an eye on.

I loved the fact the cardiologist does the actual echo herself.  She also told us that, within her practice, if one of her patients are in the hospital and needs an echo done, she will actually come to the hospital and do it herself.

Best part was — “Follow up in 1 year” — FINALLY!  An appointment that just seemed ‘normal!”

It has now been almost two weeks since we finished the Haldol taper, and she has been Haldol free.

We have noticed some definite changes in Hannah, mostly positive.

The good is that she hardly twitches when she sleeps now, so we are hoping that means we are getting her subclinical seizures under control with the removal of the Haldol and the upping of her Keppra.  Haldol is known to lessen seizure thresholds, so we are hoping that is the case and now that it is gone, whatever seizure activity she has been having is under control.

The bad is that her movement disorder has worsened to some degree.  Where once she used to hold on to toys and keep them in her grasp, she does not seem to be able to do that as well anymore.  I feel like we are having to put things in her hand and help her get the grasp going now.  She still loves to play with her toys, but it is more of a press-button cause-effect type of thing.  We got her Megablocks for the holidays, and she loves knocking them down.  But I think we are going to have to focus on her grasping and using her hands for fine motor toys again.

You can see this movement disorder most prominently in her face and her hand/arm movements.

Another good is that she is no longer taking two 2-1/2 to 3 hour naps each day.  Even better, she seems to be trending towards sleeping through the night again!  She still takes 1 long nap each day and maybe a second  power nap, but she definitely seems much less sleepy during the day (even though she still seems exhausted much of the time).

But the plan stays the same for now.  We wait until late January to start working on fine tuning her meds for her movement disorder.  We need to get the keppra therapeutic as well as make sure the Haldol is completely out of her system.

I know they say that this movement disorder is not a Gaucher thing.  But I can’t help but think that the oversedation and polypharmacy during her hospitalization this summer created a firestorm neurologically that may not be able to be reversed.

We had a fantastic Christmas, but I want to wait until my pics are done uploading before I share all about it.

Tonight Daddy changed out Hannah’s trach like we do every Sunday night.  Out with the old, in with the new.  Trying to keep things as clean and sanitary as possible.

I was with Ethan in my office when I heard Daddy yelling for me to get over there quick.  Ethan panicked (as he usually does at first) and I ran to the family room.

Hannah was visibly upset and panicked, and she was trachless.   For whatever reason, this time she got very upset when Daddy changed out her trach to the point where she clenched up, started breath holding, and completely freaking out.  When this happens, it is much difficult to get the trach in because it is tighter and she is freaking out.  Abby even started noticing that “Hannah’s face is changing colors.”

She started turning blue around the mouth.  She was trachless for about a minute at this point, and I just sat there holding her hand trying to talk calmly to her to get her to release the tension in her throat.  Daddy got the smaller size trach, and with that combination (her calming down a bit, the smaller trach, her becoming less tight), we were able to get her trach back in.

The color around her mouth started to come back from the bluish-grey.   Daddy was able to change the trach back out from the smaller one to her normal size without any problem.  She was really junky afterwards with all the manipulation, so we had to suction quite a bit of blood-tinged secretion to get her clear.

She is fine right now.

But the one thing that really freaks me out, after the fact, is that Hannah did not try to breath through her mouth and was having a breath-holding spell during this.   She can breathe fine through her mouth when she is capped, but when she panics, she breath-holds.  It is a neurologic response, not something we can tell her not to do.   This is what sent us to the hospital back in May — the tantrum, the breath-holding, the hypoxia-induced seizure, and the possible anoxia-induced stroke.

Daddy and I talked about it tonight, and we both think that maybe getting rid of the trach in the spring may not be in her best interest.  Not because of swallowing difficulties, not because of laryngospasms (the typical Gaucher reasons to get trached)… but because of these damn breath-holding spells.  It is disheartening to come to this conclusion, and we aren’t making any decisions about it right now, but NOW we are not expecting to get rid of the trach in the spring like we have been.   It is now something we will have to think more seriously about.

So many things go through your mind when your little baby is turning blue and your two other children are right there watching it…sucks.

It is 2 am. I just finished Hannah’s overnight feeding and put on her humidified trach collar. Then I went into the hallway and looked over our ledge upstairs to our family room below. It is overflowing with Santa presents. We really overdid it this year with the gifts, especially for Ethan and Abigail. Let me rephrase that…SANTA overdid it this year. (Yes, Ethan and Abby still both believe in Santa still!)

A lot of these gifts are ‘guilt’ gifts.

I had a very fun and  enjoyable Christmas Eve with Daddy and all three kids tonight. We baked brownies, had a delicious Christmas dinner, and spent time all together in the family room.

Even so, I couldn’t help snuggling closer to Hannah as much as I could. I kept having these horrible thoughts in my head about wondering what next Christmas may look like for us? How much I wish I could change Hannah’s prognosis.

I don’t want Hannah taken away from me. I don’t want to spend next Christmas without her or the Christmas after that or the ones after those. I don’t want Hannah’s condition to worsen any more than it already has.

I know Christmas is a tell of celebration and happiness. But as happy as I should be because she is still here this Christmas, I am scared to death about what next Christmas may hold for us. I know how horrible it would be for me and Daddy, but I don’t even want to think about how it would scar Ethan and Abigail.

So I the ‘guilt’ presents are just that. I want Ethan and Abigail to remember this Christmas with lots of love, baking, playtime as a family of 5. I want to have them spoiled rotten today because Hannah is as good as she will ever be (unless the miracle we pray for comes through), and they love playing and interacting with her, and she loves smiling and playing with them.

In just a few hours, Ethan and Abigail will be running to the ledge and see the presents and be horribly anxious to get downstairs. We will all wake up, and then the present unwrapping will begin. What will make it even more special is that, like last year, they will be even more excited opening up presents for Hannah and sharing them with her, because they love her just so much.

Dear God, please give us another Christmas together. I beg of you…