I can’t believe that it has been 8 months since we last visited the NIH!  Time definitely flies by.

I’m not expecting any real surprises, but honestly, that is what I am anxious about.  Hannah is known to surprise us and throw things into the mix, so I don’t want any surprises.  I know that she is doing another EEG, BAER study, meeting with PT/OT, having another abdominal ultrasound and/or CT scan, and a couple other things.  Nothing as drastic as having to be sedated for an MRI or having to deal with an IV this time, thank goodness!

I’m looking forward to seeing Dr. Sidransky, Dr. Goker-Alpan, and Catherine, their coordinator.   They are such wonderful, down-to-earth, and caring specialists.  We really felt like they were taking a personal interest in Hannah, spent quality time with our family, that she was just not another number.

What do I hope to leave this visit with?  Good news.   But honestly, I just want to know exactly how Hannah is doing in relation to this disease.  They are the ones who can tell us this more than anyone (even continuing our “no prognosis at this point” is good news!)

We finally got our Cobra information last Friday in the mail.  Our insurance lapsed on March 1st since my husband was let go from his job.   As soon as we got the info, we read it, found out how much our payment was going to be, and wrote a check to cover 4 months worth of insurance.  Then my hubby high-tailed it to the post office to send it overnight so we can get coverage reinstated as soon as possible.

I got a call from Texas Children’s Hospital’s billing office saying that Hannah’s infusion on Monday may be disrupted because our insurance is showing ‘inactive.”  I explained to them that we elected the Cobra and that we were waiting on them to reinstate the insurance, which we read is retroactive all the way back to the first day it lapsed.   She kept saying, “Well, I will need to talk to someone about this, but I may have to call you back to cancel it.”

I talked to the Infusion Center director, and she said that she can have Hannah’s doctor write a letter of medical necessity that Hannah get her infusion without problem so that they will go ahead with the infusion.  Worst case scenario, she said, was that we would be charged for the services until Cobra is activated and just not to pay the bill when it comes to us.

Of course that makes me nervous, but Hannah NEEDS to stay on schedule with her infusions.  She has not missed one yet, and I don’t want to start now!

I’m keeping my fingers crossed that they don’t cancel on us for this Monday’s infusion!

Hannah has really started improving on her crawling.   Until about 4 or 5 weeks ago, the longest distance she would crawl would be around 10 feet.  She really never would initiate crawling to go any distance (like to the other side of the room), she always stayed put in about a 10-foot area.

In her play area in the living room, we have a stone walkway separating the living room and my office (where she can easily see me).  She would see me at my desk, and she would just cry out when she wanted me.  Or she would crawl up to the stone walkway, but she would never go across.  It was almost like an imaginary barrier for her.

Over the past 2 weeks, Hannah has started branching out in terms of getting some distance in her crawling.   Last weekend, during our family movie night (DVD in our bedroom with the kids on the floor), Hannah ended up crawling from her upstairs little play area all the way across our room towards the balcony!   As soon as we realized where she was, we all were shocked and laughing!  Thank goodness we had the upstairs gate closed!

Since then, she has started crawling from our bedroom into our bathroom when it is her bathtime.   Even though this is only about 10 to 12 feet, she is going from carpet to floor.  And she has done it every bathtime since she first figured it out!  She will even follow Daddy or I into our closet, which is about 20 feet away from her playarea in our room!

Fast forward to tonight…

Abigail was taking a shower in our bathroom tonight.  She left her pajamas on the floor in front of the shower.  Hannah, on her own decision (since we were in our room), crawled to the middle of the bathroom (past the shower), turned around, and started to crawl back.  She stopped at Abigail’s pajamas, played with them for a bit, and then decided to take them back with her as she crawled into our room!

It was so dang cute watching her crawl with these long pieces of clothes on her hands!   Then, if that wasn’t cute enough, she ended up plopping back down on the carpet in our room, and just started staring at these pieces of clothes on her hands.

It was hysterical!   And best of all, it was a reminder that Hannah is still learning new tricks….just at her own pace.

(And yes, next time I’m bringing my video camera up here!)

We have SO much going on around here lately, it is just crazy!

• Our next National Institute of Health visit is already set and ready to go!  We are going to be going during the kids’ Spring Break so they can go share this experience with us.  I know they are going to have a good time at the Children’s Inn at the NIH with all the activities and themed dinner parties every day.
• Liver function tests.  We got the results of Hannah’s liver tests back today, and they all came back in the normal range with the exception of her elevated GGT, which means there is something going on with her liver.  Waiting to hear back from the NIH docs to see if they want to have a liver biopsy done or not.  It is not extremely high, but the question is it high enough to warrant intense scrutiny or continue to watch it.
• EEG came back normal!  I knew in my heart she was not having seizures.
• Liver size on abdominal ultrasound – back to normal!
• Spleen size on abdominal ultrasound – still “moderately enlarged,” 8.6 cm.  The NIH is going to do another ultrasound and compare it to last July’s when we were there.
• Mild Right pelvo-calyceal dilation – new finding on ultrasound — we need another test to make sure there is no reflux of urine going back up towards the kidneys from the bladder.  Waiting to see if we do that at the NIH too or here at TCH.
• Auditory Brain Response, Normal?!  The reason that this is a questionable result is because the day after we took this test, we saw Dr. Roy, Hannah’s ENT.  He was really puzzled with the fact that it could be normal because Hannah’s ears are filled with fluid still.  So this is another test we will be redoing at the NIH.
• Gait Trainer.  Hannah gets her get trainer next Wednesday.  I am so so so excited for her!  (here is what it looks like).  She has been able to go about 40 feet on a walker during PT, so I KNOW she is going to be all over the place in this!
• Vegas move.  Finding quality medical and therapy services is a full-time job on its own!  Add to that trying to find decent schools for the kids plus a place to live plus getting this house ready to sell plus figuring out how to move…. exhausting, but exciting.
  

I think that is it for now!  Phew…

Since Daddy was let go from his job, we have been doing a lot of thinking about what to do next.  Should he find a job here in Texas or do we take this opportunity to move closer to family?  More specifically, do we remove Hannah out of the care of local wonderful doctors, especially her amazing pediatrician and wonderful infusion nurses, in order to give our family the support that we don’t have here, a chance for the kids to grow up right by the grandparents, aunts, uncles, and cousins?

That is really what it comes down to.

At one point, we were just an easy flight away.  But now, with Hannah’s illness, nothing is easy anymore.   We have a few friends here, but I think at this point, especially since we know that Hannah’s disease course is progressive, that we all need to have our family close by.  Especially the kids.  If something were to happen, just being able to drop them off at the grandparents house or aunt and uncle’s house would just be so reassuring.   Having the kids grow up with their cousins, especially being able to see them all the time, would be wonderful.  Even for Hannah, who will have two younger girl cousins to grow up with and socialize with.

But it definitely comes at a cost.  We are leaving such a top notch medical community.  As silly as it may sound, losing our pediatrician, Dr. Bhakta, scares me so much more than I realized.  He is truly a gift to our family, someone who I know is there for our kids without question and always with honesty and an open mind.  We also will be losing the TCH infusion staff nurses, the ones who have saved Hannah from getting a port surgically implanted because of their skill and caring.   We also would be losing Dr. Roy, Hannah’s ENT, who is one of the best in the country and who truly seems to care about Hannah’s progress and care.

Las Vegas, where my inlaws live, is going to be a medically difficult situation for us.   They have no lysosomal storage disease program in any of the hospitals (of which Gaucher is one).   They just don’t have medical team with the expertise and experience that we have at Texas Children’s Hospital.  Lastly, will we even be able to find a pediatrician who is half as good as Dr. B?

We also have to start all over with Medicaid and other special needs services.  Just trying to figure out the Texas maze of how to get Hannah’s services was a chore.  Now we have to start all over again in a new state, new city.

The bright spot is that the doctors at the National Institute of Health and Dr. Schiffmann will still be our backbone and have agreed to help out however they can.  We plan to go to the NIH every 6 months, and we will be seeing Dr. Schiffmann in Dallas (just a plane ride) every 6 months.   This way, Hannah will be seen by a Gaucher expert every 3 months.   They also recommend us getting in touch with a expert team in Los Angeles that they are going to help us find.

You know, it is just such a hard decision to have made.  I know that moving closer to family is going to be such a huge and wonderful thing for our family, especially all the kids.  But I just hope I’m making the right decision and can find the right new medical team in Las Vegas to give Hannah the attention and support she needs.

As I have mentioned before, we removed basically all finger foods and solids from Hannah’s diet.  For the past 6 weeks, she has had nothing but bottled pureed food, milk, juice, and formula (yes, all together sometimes!).  We have allowed her ice cream dots, syrup with whipped cream, and any other liquid or melting kinds of foods.

It proved what Dr. Schiffmann, the Gaucher specialist, had told us when we saw him last.  Hannah’s hypersensitive gag defensive gag reflex was likely causing her gagging episodes, many of which required oxygen to help her get through them, and two that landed us in the ER after having to call 911 for hypoxia (lack of oxygen).

To understand what this reflex does, just image how you feel when you feel something caught in the back of your throat.  You cough it up.  But just imagine that you can’t seem to get it dislodged or moved.   You start gagging on it.  You start to panic as you are still gagging.  That is what was happening to Hannah — the catch?  Because her gag reflex has become so hypersensitive, the food could have already been moved and gone, but her throat kept telling her it was stuck.

So after 6 weeks of a completely oxygen-free, gag-free period (which has been quite nice!), we decided to take baby steps and try to reintroduce some finger foods back into her diet, those that had not yet caused any gagging problems earlier.  We know that goldfish, cheerios, and vienna sausages are no longer options. 

But we did try the big Cheetos many times for the past few days, and this morning we gave her some pancake.  She LOVED having her foods again, and neither of these brought up any gagging episodes.  My guess is that because they literally melt in her mouth without having to gnaw much or chew on them too long. 

She is such an oral motor child.  She ALWAYS loves having things in her mouth.  At least 80% of her playtime will show her with either a toy in her mouth or her pacifier.  At least now we can let her satisfy this oral feeding sensation with a couple of her favorites for now!  Hopefully she will not lose this ability, but for now, at least we can give her a couple of snacks to help satisfy her oral needs.

99% of her calorie intake will still be by bottle.  But at least we have that little bit of her day where she can munch on snacks and enjoy that sensation.  THAT is a great thing!