Saturday, 18 February 2017

February 18, 2017

Well, our year in Part B of the V-tesse trial is coming to an end quickly.  In fact, we only have 2 more trips in our year.  We have found out that V-tesse recommends that for the beginning of Part C that we continue to go to CHOC for 4 trips.  This is in case she was not getting the medication since we are now guaranteed to get VT270.  If she has an adverse reaction, we will be with doctors who know how she usually reacts to the procedure.

So, that means that our first trip to (hopefully) Calgary will be on May 19th.  We haven't heard much from V-tesse in regards to that other than they are working diligently on getting a trial site open in Alberta.

Katha is still having a lot of trouble walking.  She is almost exclusively in the wheelchair now.  In fact, she has just been measured and we are waiting for an adult wheelchair.  Her current wheelchair is way to small for her.

The procedure is getting more difficult because Katha absolutely dreads it.  The staff have a very hard time finding veins to start Katha's IV.  Which, of course, is the part of the procedure Katha hates the most.  However, after having to poke her 5 times at one procedure they brought in the PIK team.  They use an ultrasound to start an IV and it went much easier.  The last procedure I asked them to just immediately bring in the PIK team and it went much smoother.  If we could just figure out how to make getting the IV in as painless as possible, I think we could get in and out of the procedure just fine.

On our last trip Katha's cousin Emma was able to come with us.  It was amazing!  Katha was excited for a trip for the first time in months.  She laughed so much that I almost think she spent more time unconscious than conscious.  We came back after a day after a day of Disney and I flopped face first down on one bed, and Emma did the same on the other.  We looked over and Katha was face down on the table giggling away.  Partly because she was copying us and partly because she was laughing so hard that her cataplexy caused her to pass out.

When Katha's cataplexy affects her she looks like she's been hypnotised and the hypnotist just said SLEEP.

The long trips have now been extended as well.  They went from being from Tuesday to Saturday to Sunday to Saturday.  Our last long trip will be in the beginning of March.  It will actually fall on Katha's 12th birthday.  Which is pretty spectacular because when Katha was diagnosed that was the birthday the doctors didn't think she would make it to.  So, that, I believe is what VT 270 is doing for us.  It's keeping her stable, it's keeping her here.  Which is amazing.

Katha doesn't have a lot of energy when we are home.  She just wants to be in bed, not necessarily sleeping, just not wasting energy on anything else.  She finally went to school for the first time in 3 weeks on Thursday and then went on Friday as well.  It was a big deal and I was really proud of her because I know it's not easy on her. 

She is also eating us out of house and home!  Katha has spent a lot of time just eating lately but all healthy food.  Yet, we know because they weigh and measure her at every procedure, she hasn't gained any weight.  She has actually lost a little weight.  I believe she is actually taller than me now!

On one trip we decided to go down a little early and go to Universal Studios in Hollywood.  On the Sunday it rained hard.  Harder than they've seen in 4 years and there we were at Universal.  There is one place in Universal called Ollivanders where a wand chooses a person.  The wait to see this little show is usually at least an hour but because of the rain we walked right in.

After you get into the little room they choose one person out of your group of approximately 25 to get a wand.  Of course, they picked Katha.  The wand master picked out one wand and handed it to Katha, told her to say wingardium leviosa and point it at some boxes.  I said the incantation for her and she waved the wand.  The boxes she had pointed at kind of jumped (almost like they were exploding) and I immediately said "Katha, what did you do?!" and of course she giggled.  They handed her another wand and told her to point at some drawers and say alohmora.  This time, she said the incantation herself and waved the wand and the drawers opened and closed by themselves.  The wand master handed her a third wand.  The lights brightened, music started playing and it was amazing.  Katha was so excited to be a wizard.

We left Universal to go to our new hotel that day.  We had to stop at our hotel and pick up our suitcases then drive from Hollywood to Anaheim.  When we got out at our usual hotel it was chaos.  Katha was slowly getting out, Jay ended up falling down and twisting his ankle, my poor friend Jo was trying to get our luggage out while the cab driver yelled at the hotel employees for telling him he was pointed the wrong way.  In all the chaos, we forgot the wand.  We realised later that night and immediately called the cab company who told us that the driver had signed out for the night right after he dropped us off.  So, they would call him as soon as he signed on in the morning and get him to look for the wand.

They called us the next morning to say that the cab driver couldn't find the wand.  I personally think he probably thought he could get a pretty penny for it and decided to keep it.  Katha was heartbroken.

These wands are not cheap but after the whole little show we had to get it for her.  However, spending another $50 on a wand was not something I thought Amory would go for.  So, I decided to email Universal Studios to see if we could get some kind of discount to be able to replace it.

After we got home from that trip, a person from Universal emailed back and they are sending Katha another wand for free.  I haven't told Katha yet but I plan to record her opening it and putting it on their facebook with a big thank you. 

I think that's pretty much it for now.  Onto the symptoms:

Enlarged liver and spleen:  Still the same as far as we know.

Dysarthria (slurred speech):  People tell me that they actually understand Katha better than they used to.  On the plane ride home last time I realised that even when no one is listening I repeat everything Katha is saying so I stopped and we had a really good conversation just the two of us.  Mostly about how much she couldn't wait to get home to hug her brothers but still we talked for probably a half hour straight.

Dysphagia (trouble swallowing):  Katha has begun choking on liquids again but only occasionally.  In fact, during the last trip she was eating beef jerky, strawberries and apple juice and began choking quite badly.  Even while she was gasping for air she was kept eating strawberries until I had to tell her to stop and finish choking before she ate anymore.  (There's a reason I've nicknamed her Crazypants.)

Basal Ganglia (holding limbs at awkward positions): When Katha does walk short distances she needs support.  She holds her arms in such a way that it makes it difficult to hold her up.  She usually has her elbow tucked in with her arm straight out with her wrist bent at an awkward angle.  Plus, when getting measured for her wheelchair the noticed that her calves and ankles are unusually tight and recommended we have someone come in and help teach us how to stretch her out.

Dementia (memory loss):  Katha has no concept of time.  So, it's really hard to tell how bad the dementia is.  She will tell you something that happened 2 weeks ago happened yesterday and she will tell you something that happened today happened years ago.  Plus, she has a hard time keeping things straight.  I just discovered that when she talks about Legoland, she actually means Disneyland.

Seizures:  Still none.

Gelastic Cataplexy (falling down in response to a large emotional outburst): As I mentioned above, she no longer just falls down but completely passes out.  If she is standing or sitting I need to immediately grab her.  The thing I love about her is that it doesn't stop her from laughing.  It scares her and she will often fall but it never even occurs to her not to laugh and when I tell her to stop laughing, it just makes her laugh more.

Sleep related disorders:  Katha still has a very hard time going to sleep and it's not unusual for her to sleep until noon because of it or take small naps during the day.

Vertical Supranuclear Gaze Palsy (Trouble moving the eyes up and down):  She is still doing really well at this.  Although, we still have yet to go to the Tiki room because she has a hard time looking up.  However, she seems to be able to lay on her back much better without vertigo.

Hearing Loss:  Katha still hates her hearing aids but keeps them in for school.  But, since she hardly goes we don't really have to worry about it.  We tried taking them on a trip once and they bugged her so much we were still in the plane getting to LA when she took them out and refused to put them back in.