The house is eerily quiet this morning.  My mom flew in from California on Friday afternoon and flew back with Ethan and Abigail yesterday.  I’m not used to so much quiet in this house! 

I will admit, I am getting very anxious about this trip to the NIH to meet with Dr. Sidransky and her team.  Our flight leaves in about 24 hours or so.

There are 14 different scheduled procedures for Hannah for the 4 days we will be there.  There are only two that really, really worry me — the brain MRI and the BAER study.  Both of these require Hannah to be sedated.  I know they know what they are doing, but with her vocal cord paralysis and breathing issues, this just makes it more dangerous. 

Argh….

Pam, Hannah’s physical therapist, put together a PT evaluation for Dr. Sidransky and her team at the NIH.  

Hannah Ostrea was seen for the initial Physical Therapy evaluation on May 06, 2009 here at the Texas Children’s Hospital HealthCenter Initially, Hannah was able to roll supine –> prone with SBA (stand by assist).  She was able to roll prone –> supine with min. assistance x 1. She was dependent on her caregiver to move in and out of the sitting position.  She was dependent on her caregiver to move in and out of the stance position.  She demonstrated Poor static sitting balance with a wide base of support.   She was reluctant to weight bear through the upper extremities while in the sitting position.  She required maximum support in the stance position.

     Hannah has made significant progress since starting PT.  Presently, she demonstrates Good dynamic sitting balance.  She demonstrates lateral balance reactions when in the sitting position.  She tolerates the quadruped position with min. assistance x 1. She is able to move sit to stand with CGA (contact guard assist) after correction of her foot placement.  Hannah wears Surestep SMO’s due to bilateral pronation of the forefoot.  She is now able to stand using upper extremity support without resting her upper body on a nearby stationary object.   She will take steps with HHA of 2. 

The most recent short-term goals for Hannah is for her to crawl reciprocally, pull to stand and walk with a weighted push toy.

I have been soul searching and trying to figure out why this probably tracheostomy need is eating at me.  After talking to a couple of friends and reading thoughts from other moms who have gone through this, I know what it is.

Denial is a beautiful thing.  I remember at the Texas Parent 2 Parent conference’s grief session, the woman running the session commented how being able to be in denial at times just makes everything else survivable. 

Right now, Hannah is a medical-equipment-free special needs child.   With the exception of her braces, which honestly I consider such a benign piece of equipment, no one looking at her would know that she is affected by this horrible fatal disease.    She just looks like any other beautiful baby girl you would see on the street.   No one really sees a special needs child.

Even more so, *I* get to feel like she is any other beautiful baby girl on the street.  The way she acts, smiles, rolls over to find me, etc., she is just like any other child.   I get to live in denial for many hours a day that anything is wrong with her or very minor things that are wrong.   For those many hours, I don’t feel like I have a child who may only be with me a few more months to a few years.

However, having to put in a tracheostomy will kill my freedom of being able to be in denial about what is happening to her.  It will be a constant visual reminder every moment of every day that she is losing this battle with Gaucher’s disease, that this disease is affecting her more and more.  

As long as she is without the trach, I feel that there is still the possibility that something may come up to save her life, that I will find something or  someone to help her, something to stop or even reverse the damange this disease has caused.  

It is almost as if this trach is the “fine line” between hope and loss.   It is also as if this trach is the “reality check” that we have to face with sharing with Abigail and Ethan about how serious Hannah’s condition is because they don’t know of any other child with a trach. 

I know that I will get used to the physical act of taking care of her with the trach, I know that.  I know that the trach will improve her quality of life since she is still learning new skills and still is an active and coherent child.   

I need to be able to continue to have my many hours a day of denial right now.  I’m not stupid, I know what the future will hold, which is why I need as much “denial time” now to really allow the freedom of having a somewhat normal family for Ethan and Abby and even ourselves.  

Because once we are no longer able to live in denial about this, I am afraid of falling apart and losing it.    I mean, seriously losing it.  I just can’t afford to do that.

So, that is what it is.   I so don’t want to be faced with having to do this.   I’m so scared about the emotional fallout from myself, my husband, my kids, and our families and friends once we have to cross that line.

There is so much to talk about, but I just don’t have the real desire to do so right now.  I will most likely elaborate at a later date, but I want to make sure that I put it here so that I remember to do just that.

Audiology appointment yesterday was fine.  Hannah’s peripheral hearing was fine, and her middle and inner ears are clear.  The audiologist offered to do a non-sedated ABR on Hannah to see how hearing is since Gaucher’s disease affects the hearing in the respect that what she hears and what is actually being heard become two separate things.  Basically, it would be like me saying “Hello, how is your day” and you hearing “Heshkok, felso difm wsh.”   We were originally going to do one back in February, the normal sedative kind, but we just had too many concerns about sedation.  I have a feeling we may be doing  one at the NIH next week, so we may get answers on that then.

The Parkinson’s doctor appointment went well.  It wasn’t exactly as I had hoped, but they were very open to my concerns, and he was open to looking at possible treatments for some of Hannah’s neuro issues from a different perspective, his perspective.  He was aware of the recent link, but he admitted that there is so much that has to be looked at.   He asked if he could take a video of Hannah, and he is going to present her and her case to his Parkinson’s group at their weekly meeting next week to see if anyone has any suggestions or ideas on how to get more involved.  They agreed to be part of Hannah’s “team,” which is a great thing.

NIH Visit.  Getting ready for that next week.  We leave Monday morning.  Had a horrible time getting the medical records from TCH.  Requested them 5 weeks ago, confirmed they had it 3 weeks ago, apparently “never had it” 2 weeks ago.  Went to the office after Hannah’s last Cerezyme treatment on 6/29 and paid for a RUSH service and filled out a new form.  Called yesterday, and they couldn’t find it, and the manager had “gone home already.”  Called first thing this morning, and they couldn’t find it again.  I faxed over the receipt, and the manager said she would have it done immediately and that we could come pick it up at noon.  Daddy went over there at noon, picked up the book of Hannah’s records, and sent them FedEx to the NIH this afternoon.

Getting the kids ready for California.  Mom comes in Friday night.  I’m looking forward to seeing “my mommy.”  She, Ethan, and Abigail leave Saturday afternoon. 

Hannah’s first birthday party plans.  Since we will be in California for her actual birthday on 7/25, we are having her party the weekend after here at home.  I’m so looking forward to this.  More than anyone can possibly imagine.  After yesterday, I just keep hoping to keep her trach-free for her birthday.

As for the trach issue, I still don’t really want to talk about it.  I know, me not want to talk about something.  But I decided to wait to see what the evaluation at the NIH says about the issue before really getting obsessed with depression over this.