We didn’t make it again to the nursing home, but this time it had nothing to do with Hannah.

Abigail seems to have caught a 24-hour stomach bug of some sort and had been vomiting every-hour-on-the-hour for almost 12 hours straight!    She seems a bit better today, but there just was no way that we were going to make a drive dealing with both her and Hannah.  Abigail was so upset that we had to postpone our “vacation” (even though we told her it wasn’t a fun vacation).

The nursing home coordinator is also not so pleased.  We rescheduled for next weekend, but I got the impression from her that “3 strikes and you are out.”

Great, no pressure there!

After a horrible attempt for a road trip last month to try and get our nursing home overnight stay requirement filled so that Hannah can be approved for the medically depedent children’s program (and medicaid), we are going to try again this weekend.

Last time, we only made it about 45 miles of a 200 mile drive before we had to return home.  She had 3 beginnings of a hypervenilation episode (as a result of a tantrum of not liking behind confined) and one actual hyperventilation episode, all requiring us to pull over for about 10 minutes, get her out of the car, give her some fresh air and cuddles, and then move on.  Also last time, she was teething horribly and completely congested, so in hindsight, it probably was not a good move to go that weekend.

We are going to try again this weekend.  We have to check in by 11 am on Saturday, so we are actually going to leave when the kids get home on Friday afternoon.  This way, we can drive as far as we can on Friday and spend the night in whatever city we can get to.  Hopefully we can make it the entire way Friday (it would normally be a 4-hour drive, but not with our situation).  But this gives us a cushion just in case we have to stop and give her a break.

I will admit that I am incredibly nervous about this trip.  The first 40 miles is in more of a rural area, so if for some reason we needed 911, it would not be that quick.  We will definitely be taking our oxygen with us, actually a couple of tanks “just in case.”   We have the DVDs ready to go, but even those only taking her mind off things for a period of time.

I don’t know what it is about her and driving.   She tends to do it when she is more on the tired side (not nap tired, but just up for a while), and when she is not feeling well.  We were able to make it to Katy last weekend (45-minute drive) without a problem, but on the way home, she had another episode, and we had to pull off the freeway and give her fresh air.

I hope, hope, hope this works.  I don’t know what we will do if it doesn’t.  We thought about flying closer to the nursing home, but there would still be an hour+ drive from the airport, but then we have to pay for airline tickets.  We thought about renting a small RV (which the kids would love), but even that is a few hundred dollars.

Just keeping my fingers crossed…

We met with Hannah’s liver doctor today, Dr. Carter at TCH.  We actually had not seen her since before Hannah was diagnosed.  I brought her up to date on everything that has been going on, including how Dr. V was concerned about some of her elevated liver function levels from as far back as Sept of 2008, which were still a bit high at the NIH.   What they mean, we don’t know yet, but Hannah had another set of blood work today so she can review it.

My main concern is that Hannah has been on the Cerezyme treatments for one year (next week), and her spleen and liver should have shrunk back to normal size by now.  They definitely aren’t.  Dr. V, her GI doc,  is actually concerned that it seems a bit bigger.  This along with the elevated numbers makes this something that needs to be looked into.

So we will be doing the liver function tests (blood work), and we are going to schedule a abdominal ultrasound downtown at TCH to get measurements to see where we are at.    Then, depending on the results of these tests along with the advise from Dr. Goker-Alpan at NIH, they may do a liver biopsy to see if there is any liver disease that may not be common with GD.  Apparently there are a few rare cases of “liver scarring” with GD type 1, so that is in the back of her mind.  The thing that sucks with the liver biopsy is that what would normally be a quick outpatient procedure would be an overnight stay downtown at the hospital to be monitored because 1] of her lower platelets and 2] because of the quality of platelets (which may not be as functionable as normal platelets).

We then talked about Hannah’s nutrition and how we have seen regressing in finger feeding.  I wanted to know “what to do” because we have been just winging it lately with milk, pediasure, and stage 2 baby foods in the bottle.   She brought in her dietician, and her dietician recommended a product called “Compleat Pediatric,” which is basically all the required food elements (proteins, fats, vitamins, etc) pureed together using natural foods.

She warned me that it is mostly used for g-tube feedings (feeding tubes), but she does have some patients that can drink it orally.  Apparently, it doesn’t taste that great, but since Hannah is used to stage 2 meats, veggies, and fruits mixed together, we would give it a shot.

We came home, and I put together her first bottle of 4 oz whole milk, 4 oz Compleat Pediatric, and one stage 2 fruit (pears and pineapple).  She downed the entire bottle!  Not only that, she drank most of her second attempt with it tonight (again 4 oz milk, 4 oz CP, and stage 2 fruit).  I’m going to email her in a couple of days and take her up on her office to get a rx for this.

Even though we have had to “give up” on feeding her normal foods, there is a sense of comfort knowing that we are a step closer into getting her the nutrition she needs.    After talking to another of her doctors, it is time to stop her feeding therapy now and just move on and accept this is where we are at this time.   I guess there is a sense of comfort knowing that we aren’t needing a feeding tube at this point, but there is such a sense of loss/grief trying to accept this.

Hannah has quite a few favorite toys.  All of them are ones that are cause/effect and make noise/music when pushing buttons.  As I am looking at them, I realized they all have one thing in common.  They are all VTech toys!   After going through their website just now, I realize I could spend a few hundred dollars more on toys I know Hannah would love and get a lot of use out of.  THANK YOU, Vtech!

Vtech Learn and Drive Discovery

Sing & Discover Story Piano

Baby's Learning Laptop

Sit-to-Stand Activity Walker

There has been so much I have been trying to organize in the past few days that I’m just going to jot it all down now, and I will come back and elaborate later on.

• Visit with GI doctor.  Dr. V., Hannah’s gastro doc, is concerned about her old liver tests that showed a rise in two key areas (GGT and ALT or AST).  They were never followed up on.  He also felt that Hannah’s liver was actually bigger than last time he saw her (4 months ago), which shouldn’t be happening since she is on the Cerezyme.  So I am working on trying to get an appointment with Dr. Carter, Hannah’s hepatologist, but I am having a difficult time getting through to set up the appointment.
• Got the EEG scheduled for next Friday, but I have yet to get the BAER scheduled.  Dr. B. “reminded me” today to get that done.  Also, I’m waiting to hear back from the TCH “Blue Bird Clinic” (Neurology) to schedule a consultation with them, as Dr. B. thinks it is a good idea to get a local neurologist as well as Dr. Schiffmann.
• Found a SibShops workshop in our area for Ethan and Abigail.  Unfortunately, it is just once every other month for a couple of hours, but it is a good start.  I also have a couple of places I am trying to work with in our area to get them assessments for counseling.  Unfortunately I have not been able to find anyone who specifically works with siblings like I had hoped, but hopefully there will be someone who still will be able to help them.
• Working on getting the NIH scheduled again.  So far, we are tentatively scheduled for the week of 2/22.  But I am having trouble getting firm dates, which stinks, because my inlaws need to know because they offered to come stay with Ethan and Abigail here while we are gone.
• Research stuff:  Have quite a few conference calls and communications set up over the next 7 days.  Hopefully we can kick start nGD awareness and research again!  Need to find time to get my background work done before then.
• Hannah’s physical therapist is ordering Hannah a gait trainer!  This is great news, as I really think strength is Hannah’s biggest obstacle to being able to walk on her own.  So once we get this, she will be able to move somewhat freely around the house and when we go out.  I know she is just going to thrive on the independence of this, because she loves her little toy walker so much.
• Nursing home, take 2.  Unfortunately the place closer to our house would not work out for us to get Hannah on the MDCP/Medicaid program, so we have to try again to make the drive up north.  Even though it is normally a 4-hour drive, we are going to actually leave the day before (Friday after the kids get out of school) and spend the night along the way.  We just HAVE to get this done.  I just hope we can make it this time without too many hyperventilation episodes.
• Still working my two jobs, which total about 30 hours a week.  My med transcription job is scheduled from 6 am to 2 pm on Saturday and Sundays and 8 pm to 11 pm on Tues and Thurs nights.  My other job, which is working for an internet contractor, is only 10 hours a week, but I am finding it so hard to find the time to fit it in!
• Just got Hannah’s new SureStep SMOs (braces).  Hello Kitty pattern this time (Abigail picked them out).  Her foot has grown quite a bit in 7 months!!   Because her other ones were too small, she hasn’t worn them in a month or so, and it is going to take her a while to get used to them again.  But I KNOW it will definitely help with her walking and standing, as she has definite ankle rolling when she stands and tries to walk.
• The gagging/choking issue. Yesterday in OT, we let Hannah try goldfish again (something she has eaten for months without problem until recently), and for the third time in a row, she had a gagging/choking episode.  Fortunately, it didn’t last that long (maybe a minute), and she was able to get herself out of it on her own without oxygen or anything else.   Her OT feels that it may also have something to do with the buildup of saliva during that time, as golfish are very salty, and she says “from where the sound was coming from”, she feels that may play a part in things.  She definitely thinks it has to do with this progressive hypersensitivity defensive gag reflex. The two episodes that required 911 were both when she had cheerios (we know she was eating them on the first one, but we didn’t see her eat  it on the second one although she did have them in front of her and we were at the restaurant). Going by our OT’s logic, cheerios require a lot of saliva to break down because Hannah still doesn’t chew.  They started out exactly as the goldfish episodes, but they got progressively stronger, and she couldn’t get out of them without help.

I’m sure there is more, but this is all I can think of off the top of my head!