Today was Hannah’s 5th swallow study.  The first three swallow studies she had passed.  The fourth one, which was done just 2 weeks after being trached, she failed.  Because the choreathetoid movements were so involved and intense, she had no suck or swallow capability.

We were hoping that since she has regained her suck, and she was tolerating stage 2 baby foods fine (where she wasn’t on that fourth study) that she would pass.  

As soon as Hannah got in the feeding chair, she had a meltdown.  We continued with the test, but she was so stressed out that she wouldn’t even try sucking or swallowing.  The ENT and speech pathologist were able to get some data though as gravity helped get the different barium textures into her throat.

Hannah aspirated on the nectar-like liquids (meaning it went into her lungs instead of her esophagus).  It was easily apparent watching the xray study next to me as I was trying to comfort Hannah in the chair.  We didn’t even bother trying thinner liquids because this result just shows she can’t swallow liquids at this point.

It seemed like Hannah aspirated on the pudding type foods (like stage 2), but the truth is that it was really inconclusive because Hannah was not even trying to swallow it.  She was doing her silent tantruming (she never had a vocal cry, hence the breath-holding spells), and she just let it sit in her mouth.  There was no aspiration, but there was attempt at penetration of the pudding into her lungs.  But her body redirected it.

The speech pathologist said that if it wasn’t for Hannah’s obvious non-participation in the study that all ‘by mouth’ foods and drink would be off limits.  But because we have had success with pudding-type foods like stage 2 without aspiration into her HME that we can continue with that on a very sparing basis.   I wouldn’t say she would be nervous about aspiration of these types of foods, but her comment was to “just do it for taste and oral pleasure not for a meal.”   Which in all honesty, that is what we do now.  During non-trachiitis days, she would only take a few spoonfuls to maybe one jar of stage 2 a day.

We will try again in 6 months.  Hopefully we will get the trach out and better manage her chorea movements with medication.  I doubt her participation will be any better because she is so over docs and procedures that even stepping into a medical-type room just upsets her.

Hannah had her first official playgroup today.  It was set up by Early Intervention, and it was Hannah with three little boys, just slightly younger than Hannah.  All with some sort of special needs.   I was so excited for her to go because this was the first opportunity we have had to put Hannah into any type of organized playgroup with other children.  Nothing like this existed in Texas.

Hannah would have absolutely none of it.  Less than 5 minutes in, she started getting very upset, putting her back to everyone, and throwing herself into my lap.  We tried the wonderful different sensory games they offered during playgroup, but she really just would tune everything out.  About 20 minutes before the hour-long playgroup ended, she shut herself down and put herself completely to sleep.

I have to admit that I was disappointed that it didn’t go better, but I think once Hannah gets used to it that this is going to be so good for her.  Especially since the people who run the playgroup are PTs, OTs, and other therapists.   It definitely stressed her out farther than I have let her go in any other therapy.   With all her thrashing around and having a tantrum throughout the whole experience, it was rough for both of us.

It is 3 times a month, and unfortunately the next one is not for two weeks.  But hopefully next time she will be through with her trachiitis and off the Cipro, both which has totally taken a toll on her little body .

I just know the more Hannah gets exposed to these type of situations (playgroups, classes, etc.), the better off she will be in the long run, how ever long that may be.  So many more experiences will be opened up to her if she will just let other people in to expose them to her.

It will just be a rough road and balancing act to get her there.

This trachiitis has really done a number on Hannah.   We are about 4 days into the Cipro, and it has definitely started knocking out the infection.  She is a lot less junky, and she is sleeping through the night much better now.  Last night, the earliest she woke up was 3:30 am (instead of every 30 minutes), and she went back down at around 4:30 am until 6:00 am.

Not only is it knocking her infection out, it is also knocking her out as well.  She is exhausted and fatigued almost all day long.  We get maybe 2 to 3 hours of “happy girl” time before she gets exhausted, fights it for an hour or two, and then she takes a nap in the afternoon.   Tonight, she crashed at 6:30 pm, which is really unusual for her, as she usually crashes around 8 to 9 pm.  Last couple of nights, she has barely made it until 7:30 pm.

Hopefully this time next week, once we are done with the Cipro for her trachiitis, that she will start to get back her energy again!

Oh yeah, on a side note, the ENT’s nurse sent over the wrong order for the swallow study on Friday.  So obviously, we weren’t able to do it.   I hope that gets rescheduled really soon because I would really love to reintroduce liquids if she is able to tolerate them!

Late tonight, Samuel, a little boy from back where we used to live lost his battle against an incurable mitochondrial disease.   He was only 4 years old.  His mom was a wealth of information for me as I was trying to navigate the Texas medicaid system, and she has been such a source of strength and understanding since then.    When I heard the news from a mutual friend, I just lost it.

Before Hannah, I never knew anyone who had lost a child personally.  I knew of people who knew someone, but that was the extent of it.

This week, two other young children lost their lives against rare, incurable diseases.

• Mallorie, a beautiful little girl whom I met when Hannah was in the PCU this summer, passed away earlier this week.    Her mom and I shared quite a few conversations during those two weeks on the PCU floor before we were transferred.
• Mario, a handsome young teenager, lost his battle against Gaucher’s disease type 3.  Yes, type 3.  His death also hit me hard as it was a slap-in-the-face reminder that type 3 is just as deadly as type 2.  It may not happen as quickly as type 2, but in many cases, type 3 also robs children of their lives.   His mom was one of the first people to reach out to me when Hannah was diagnosed.

Before learning about Samuel, I had a wonderful phone conversation with an old friend who had lost her son almost a couple of years ago when he was just 11 months old.  I love talking with her because we both can be very frank about our kids situations and how we are feeling.  No beating around the bush, no candy coating, no avoiding.  Just flat-out, this is how I feel.

It was actually just about 20 minutes after I got off the phone with her that I learned about Samuel.

I feel really devastated right now.   Scared.  Heartbroken.  Angry.  Anxious.  I’m glad Daddy was sleeping because I even found myself crying tonight.

This new world I have been thrust into, a world where children die because of lack of research, treatment, and cures.   A world where there are no telethons, no celebrity public service announcements, or no major fundraisers to help save our children.  A world where conversations between moms don’t include gardening or gossiping but talk about medical situations and fear of our children dying way too soon.    A  world where moms and dads talk about funeral arrangements instead of elementary school.

Three children that I knew about personally passed away this week.   Three.   There are quite a few others whom I have grown close to over the past two years that I worry constantly about because I know that their lives may likely be taken away way too soon.

I know saying “It is not fair” is just not the politically correct thing to say.  But screw it.  IT IS JUST NOT FAIR!!

We had a fantastic visit with the developmental pediatrician.  I was incredibly impressed that she had thoroughly researched Gaucher’s disease beforehand and that she asked tons of questions, some that no one else has asked of us before!    We were with her for almost an hour as we discussed almost every aspect of Hannah’s health and condition.

Hannah was exhausted (it was naptime), and she was thrashing around in my arms as the doctor was listening to her chest.   At that point, Hannah pulled out her trach … again.  Right in front of the doctor.  The only reason we noticed was because we heard actual crying noises, and Daddy and I looked at each other and both knew right away.  It was actually good that the doctor saw it because she noted that Hannah’s color was good, she was not having any breathing difficulties, and that she was fine during the whole episode.

Towards the end of the meeting, the doctor said something that stuck with me.   Basically, “we need to work to get her to her 4th or 5th birthday.”  Straight up, no beating around the bush, etc.   That was her ‘guesstimate’ prognosis based on what she learned of Hannah’s disease progression symptoms.

What struck me was that this was the same prognosis I have had in my brain for the past few months.  The age of 5 years.  I have no factual basis for it, but just from what I know of this disease and how she is progressing in terms of it.   I hope, hope, hope it is longer.   But I also have to be realistic too.

But to hear another doctor say the same thing was just an eye opener.

Reality is though, our goal every day is to just get Hannah to that next birthday.  Whether it be her third birthday, her fifth birthday, or her tenth birthday.    We live our life knowing that Hannah is a gift that we were chosen to get to experience the joy of loving and being her parents for however long she is with us.   For every day that Hannah goes to sleep after a happy and painfree day, we know that we are doing the best we can for her. 

Her third birthday is coming up in …. 304 days!