Thursday, 20 August 2015

August 20, 2015

Since I really should have updated sooner, I will try to be brief with all that has happened. 

First, the renovations.  It is overwhelming how much time, effort, love and support went into our home.  There is hardly a room in our house that went untouched by the amazing people at Neighbours. 

Katha loves her room so much that she hardly comes out of it. 

The boys have nicknamed their room the DJ cave.

 I love everything about our house.  The main floor laundry is awesome. 

We no longer have to worry about people parking in front of our house because they put in a driveway. 

I love baking on our new stove and the other day when Katha had a huge accident, it was so much easier to deal with it in our HUGE bathroom.

The kids all got Smart TV's with surround sound put in their rooms.  They sorted out the cables so you can't see them in the living room.  We have a new kitchen table and chairs.  Amory especially loves the new deck that's 2 to 3 times bigger than our old one.

And of course, the ramp and lift so that we can get Katha into the house is amazing!

The whole experience was just spectacular and the incredible people I have had the privilege to meet has been fantastic.

Katha has been pretty good these last few months.  I always worry about her not being as active during the summer because we have consistently been told that the more active she is the slower the progression of her disease will be.  Katha LOVES to swim and with the Abbey Centre right down the road, I figured, it would be a good way to keep her going this summer.  The very first visit there this summer I paid for a month membership.  After we got into the pool a man and a woman approached us and gave us another month membership.  It was amazing and I'm so appreciative to have this opportunity.

Katha's tremors are becoming worse and it makes it difficult to eat.  I often have to help her finish her meal because she just can't get smaller pieces into her mouth.  Her speech is becoming more and more difficult to understand.  On a good day I would say I catch about 50% of what she says which is usually enough to figure out what she is trying to say.  She still sings quite a bit but prefers to have a hair brush to sing into as a microphone. 

Her walk is shaky at best and I think it won't be long until she is in a wheelchair full time.  The smallest errands outside the house require a wheelchair and we now have a commode on wheels to get her to and from the bathroom in the morning because she has a hard time when she first wakes up.

Katha's cataplexy is consistent now.  If she laughs, she falling.  In fact, one of her favourite games is to try to race me back to her room.  I now have just enough time for her to turn around and start running to jump out of my chair and catch her before she falls going into the hallway towards her room.  Because she knows I'll catch her, this is still one of her favourite games.
So, in November we are planning a trip to Disney World with Grant and his girls, as well as Mare and Emma.  Katha is so excited about the opportunity to go on rides with her cousins.  Unfortunately, Dan and his family can't join us because they have a new adorable addition.  Because of this trip we were not going to go to Montana this summer in order to save money for the Disney trip.  Well, with all that was going on we forgot to cancel the rooms we booked in Montana last year.  So, mom, dad, Katha, Dom, Jay, Emma and I all decided to go down for a week.

Last year when we went to Montana we noticed a huge difference in Katha's behaviour.  To put it in the simplest terms, she lights up.  Her speech gets better, her walk get better, she laughs more, she talks more, she's just better.  When we noticed this last year we contributed it to a change in her medication.  This year, we noticed the same difference without a change to her medication.  I don't know if it's because we are all more relaxed and slow down, so she can just keep up or something else but it's an amazing transformation to witness. 

To give an example, she only used her wheelchair once while in Montana and it was at a mall where we were on our feet for an hour.  She had full conversations again and she hugged people voluntarily as well as giving me a kiss which she hasn't done in probably 2 months.

Here is an example of how well she was talking:

Me:  Katha, we have to go home the day after tomorrow.
Katha (starting to cry):  Why?!
Me:  Because this is someone else's house and they need it back.
Katha:  But, I'll take care of it.
Me: So, should I leave you here for the next family?
Katha:  No, they'll speak Ruff, Ruff.
Me:  You mean they'll speak a different language?
Katha:  Yeah, like Oreo.
Me:  Well, we are going to go to Church tomorrow.
Katha:  But tomorrow isn't Sunday.
Me:  Yes, but we'll be driving all day Sunday and we don't want to stop to go to Church.
Katha:  Which church are we going to, because there are like 4 around here.
Me:  The one in town, so it will only take us a couple minutes to get to it.
Katha (to a reflection of herself):  Save your breath, Katha, she doesn't know what she is talking about.

As you can imagine, I was killing myself laughing by the end of this conversation.  Usually, I'm lucky to get a yes or a no out of her.

She has been pretty confused this summer.  She is convinced that she has been missing a boat-load of school.  Everyday she asks if she's going to school today and if it's her birthday.  So, I've started a countdown to the beginning of school.  However, she ALWAYS knows the day of the week.

Amory was laid off for 3 months before getting back to Evraz (except for the month he spent at Ledcor), who in the meantime switched Health Insurance providers.  Since the switch, the new insurer has decided to cover Katha's medication again.  So, for the past few days, I've been trying to figure that out.  Katha is apparently almost 100 pounds and just under 5 feet tall.  This is going to increase her dosage.  So now, her medication is going to cost $15,000 a month instead of $10,000.  According to the provider there is no limit on the coverage of this medication, but that's what the last one said so we'll see how long they will cover it.

Because the medication is so expensive the pharmacy here doesn't want to order it in case the insurance provider won't cover the medication before it's ordered which is often the case.  I can't say I blame them, they are a small business in a small town.  It sounds like we are going to try to keep getting the pharmacy that is sending it to us now to keep sending it.  If that doesn't work for whatever reason, we'll have to get it through the Alberta Children's Hospital again.

We have also recently noticed that Katha is going through an early puberty.  After consulting with doctors, pharmacists, talking with other NPC parents and reading some studies on line we have found out that this  is fairly common in NPC patients.  Although, they are not sure if it's caused by the medication or the disease itself.  This has presented us a with few problems, mostly with the fact that Katha has been becoming increasingly frustrated more easily and will throw small temper tantrums.  She is easily diverted and we are learning how to avoid these situations as best we can.  Another concern was Katha taking Midol or children's Tylenol with Zavesca.  The pharmacist is looking into which medications will have the least side effects on Katha.

Sorry this has been so long.  I think that's the end of our update.  I'm impressed if you have gotten through this massive wall of text.  Onto the symptoms:

Enlarged Liver and Spleen: Still the same

Dysarthria (slurred speech): I am honestly impressed when someone understands her anymore.  I only get about 50% of what she says which causes her to become frustrated but she will usually just point to what she wants or start getting it herself until I come to help her.

Dysphagia (trouble swallowing):  She chokes easily but recovers quickly.  Still downing that steak whenever she can get her hands on it though.  Seriously, if you ever want to win Katha over, just bring her a big, juice steak and she'll love you forever.  (Or at least until she finishes the steak, lol)

Basal Ganglia (holding limbs at awkward positions):   Katha's arms are becoming very stiff with her hands curled towards her body.  It makes washing her hands difficult because she is unable to straighten them most of the time.

Dementia (memory loss):  Katha can't tell you what month or day or even year it is.  But, boy can she tell you what day of the week it is and when her and Emma's birthday is.  Katha can no longer count to 10.

Seizures:  No seizures that we are aware of.

Gelastic Cataplexy (falling down in response to a large emotional outburst): She took a big spill on the concrete at the pool because she got scared.  Poor Emma tried to catch her but she's nearing 100 pounds now.  She got pretty scraped up but luckily that's when the dementia kicks in and she forgets it even happened.

Sleep related disorders:  Katha stays up pretty late and gets tired really easily.

Vertical Supranuclear Gaze Palsy (Trouble moving the eyes up and down):  Katha doesn't really look up anymore.  Every once in a while she has a good day and will look me directly in the eye.  Other than that her eyes will roll a lot and stay on the ground.

Medication:  Amory's work is going to cover it again for a while.  We have in writing that it's until next June and they say that's without limits.  We'll see how long this lasts.  We are also upping the dose from 400 mL a day to 600 mL a day.

1 comment:

  1. The house looks wonderful! The Montana change could be because of the slower pace but it could also be atmospheric (temp, humidity, elevation). There are so many unknowns with the disease. So happy to hear you have the drug covered again. Check into the new possible clinical trials for eligibility. Love and hugs.