Friday, 15 September 2017

September 15, 2017

Hello everybody!  It's been a while since I lasted updated and a lot has happened.  So, May came and went and we are still going to CHOC every two weeks.  There are several reasons for that.  First, we had to get Health Canada's approval to bring the trial here to Canada.  Although, that only took 2 weeks to get approval for. 

Then we had to have the drug company and the doctor agree on everything, which has kept other sites from opening in the past.  However, this didn't seem like much of a hold up.  Both the drug company and our doctor are eager to start the trial in Calgary.

The biggest hold up has been the procedure itself.  Katha has had a hard time from the beginning getting an LP done (I have only learned this in the past couple of months.)  Because Katha isn't as flexible as most kids she isn't able to get in very good positions to get a lumbar puncture.

In California, they have started to do the procedure using an X-ray every time.  However, in order for the doctor in Calgary to do that, he would essentially have to go back to Health Canada and get all new approvals because he would be bringing another doctor into the procedure.

Also, the doctor in Canada wants to put a port into Katha's back to make the whole procedure WAY less evasive and Katha wouldn't need to be sedated at all anymore.  Which would be a huge improvement.  The doctor has already got the drug company to agree to this but the problem we are now facing is that the port that is approved in Canada for children won't work with Cyclodextrin.

So, the doctor is getting a port from Europe approved for use here so that it can be inserted and we can start doing the procedures that way.  The last time I spoke to the doctor, he didn't want to start doing the procedures here until the port is in.  I have convinced him to at least try the LP the same way we did it in California for the first year.  He has agreed to give it a try on October 18th and if he is unable to get it we will go to California, where they will do it by Xray.

We finally have a wheelchair accessible vehicle that we won't have to be worried about breaking down on all the trips we will hopefully be making to Calgary soon.  Amory and I bought a brand new 2016 Dodge Caravan and had it sent out to Winnipeg to get converted for a wheelchair.

We bought the van back in March and were told it would be done in April, then when all the financing was done for the van, they told us they wouldn't be able to pick it up until April, then May.  Then once they did pick it up they said it would take 4 weeks to complete.  When that four weeks was up, they needed another six weeks.  We finally got our converted van in July.  Once we figured out how to get the cans that hold the wheelchair in place out of the way, it works great! It's very loud in the back, but the kids are getting used to it.

Katha has finally received a test chair that fits her.  She was measured for an adult wheelchair back in January.  However, something happened with the company in Red Deer and all of our paperwork as lost.  So, finally in May the Occupational Therapist got involved and they re-measured Katha for a trial chair and told us it would be in in a couple weeks.  When that time was up they told me that all the parts for the wheelchair hadn't come in and they would need until August.

So, we waited until August and were told that the parts had still not come in.  Again, I placed a call to the Occupational Therapist who called the company in Red Deer.  Then, finally just this week, we received the test chair.  We will have it for 2 weeks and it will have to go back to the company in Red Deer so that they can get all the parts that are approved from AADL which should take another two months.

Also, we have recently seen a new doctor at the Children's about Katha's walking.  When Katha walks, she is always on her tiptoes.  After seeing the new doctor we have decided to put casts on Katha's feet to try to stretch the muscles.  Then, after 8 weeks of going every 10 days to put new casts on, Katha will have to wear a brace to keep it from happening again.  We were supposed start that by the end of August but have heard nothing back from the doctor yet as to when that will start.  I should add it on my to-do list!

As you can see, I'm kind of getting frustrated with dealing with companies at the moment.  It feels like I have to be constantly hounding someone to get all of Katha's needs met.

On the last trip we were pulled aside in customs because our passports expire within 6 months of travelling now.  I was expecting this, and it wasn't too big of a deal, but it did take a while longer to get through customs on the way in.  So, when I got home, I finished filling out the paperwork for passport renewals for Amory and I and a new passport for Katha. (16 and under can't just renew their passports for whatever reason). 

I had contacted the drug company and asked if they would be willing to pay to expedite the passports in order to have them for the next trip and they agreed.  After researching online, I discovered I had to take all the documents for our passports into a main office in Calgary or Edmonton to get them expedited.

When I went into the office the lady who check's to make sure you have all your documents told me that we didn't qualify for an "urgent" renewal because we weren't travelling that day or the next.  They told us we do qualify for the "express" renewal and that our passports would be ready for us on Friday.

However, when I got to the window to actually renew my passports the officer told me that they couldn't guarantee that the passports would be ready by Monday and because we weren't travelling until Monday afternoon that I couldn't receive them until Monday morning. After trying to explain to him that it was a medical trip and we need our passports he told me that I should have thought of that before and not made plans before renewing my passport.

I don't know why, but I cracked.  I cried in the middle of the passport office!  The officer's whole demeanour changed.  He told me he would make the passport come in on Thursday, that way if we ran into issues we would  have a couple days to deal with it.  He also told me he has 2 sons with special needs and he knows how stressful travelling with kids with special needs is.

I went on Thursday and got our passports without any problems.  I guess it just goes to show that God has your back even when you feel like all you can do is crack under the pressure.

Katha has been amazing.  Ever since we started getting the procedure done by Xray, they have moved the procedure to the hospital where they sedate her with propofol rather than ketomine like in the clinic.  She is usually good to go the next day, while with ketomine she took until Tuesday or Wednesday to start feeling like herself.  Her speech has been better (which isn't always a good thing with her little Kathatude).

When we went to Montana this year, I honestly thought it would probably be her last year going to my parents time share.  Every other year this has been a big highlight for her and she's excited for weeks in advance.  This year every time I asked her about it, she said she didn't want to go.  Then, not long before the trip, we found out that the condos we were in this year both had stairs to get in. 

However, after a bit of finagling, we found out that one of the condos on the first week had a ramp to the building.  We would still have to figure out the stairs the second week, but we had to go to California for the last half of the week, so surely that wouldn't be too bad.

Well, a few days before leaving Katha finally decided she actually wanted to go.  In fact, she even promised me she would try swimming again.  Swimming used to be one of Katha's favourite things to do but she is afraid of any pool that is anything but completely white on the bottom, which is hard to find, but the indoor pool at the resort is completely white.

So, not only did Katha end up going swimming twice while we were in Montana but she also got up and down the stairs with very little help.  We were amazed!

Since starting school (she went back to Grade 5 at St. Teresa just like we planned) she has been consistently going at least once a week.  Considering by the end of last year she was making it approximately once a month we are ecstatic!

Also, because of the rewards points we have been saving through West Jet and Marriott, I was finally able to book a trip for my mom, my sister and I to New York for January as a thank you for all of their help.  My mom has been crucial for us for the past year and a half.  I'm really excited for a girls trip with two of the most amazing women in my life.

Everyone always comments on how hard it must be for me and Katha doing all the travelling.  I'm telling you right now, I couldn't do anything without my mother.  She has kept the boys for almost every single trip.  The ones she hasn't, she is usually on the trip with Katha and I while Dad takes care of the boys.

I think that's pretty much the end of the update, so onto the symptoms:

Enlarged liver and spleen:  We just had an ultrasound done and have not heard any results, so in true medical form, I'm taking no news as good news.

Dysphagia (trouble swallowing):  Katha will choke periodically on liquids, but it's not really troubling as it doesn't last long.

Basal Ganglia (holding limbs at awkward positions):  This is what caused Katha to walk on her tiptoes.  She also holds her arms with her wrists at odd angles but she can straighten out her arms where she can't with her ankles.

Dementia (memory loss):  There are times where Katha is convinced that Jay is a baby and cannot talk.  She will also think things that happened long ago happened recently.

Seizures:  Still none.

Gelastic Cataplexy (falling down in response to a large emotional outburst):  Katha still loses consciousness for a second when she finds something really funny.

Sleep related disorders:  Katha still has a hard time getting to sleep and is very tired in the morning as a result.  She will often get her days and nights switched.

Vertical Supranuclear Gaze Palsy (Trouble moving the eyes up and down):  Katha has a hard time looking up.  Down is still no problem.

Hearing Loss:  Katha has completely given up on her hearing aids.  She doesn't like them and prefers not to wear them at all.  She will take them out and hide them given the opportunity.

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.