January 26, 2024

 Hello, folks!  It's only been 3 years since I last updated.  I am so sorry for those who have been wondering how Katha has been.

Katha has been doing spectacular!  She has deteriorated, she has almost no weight baring abilities at all anymore and she is almost completely non-verbal.  In fact, I have said in the past that she is the most communicative non-verbal person you'll ever meet.

We usually have to communicate with by giving her two options and holding out a hand when giving her options.  Then, she will pick a hand.  For example we will say "Katha, do you want to watch netflix (and put our left hand in front of her face) or Disney (and put our right hand in front of her face).  Then she will touch either our left or right hand.

She also will still say things but only when she's really upset and it usually is just a yes or a no.

Katha spends all of her time in bed or at the kitchen table watching shows (most of the time The Little Mermaid in every form it comes in).  When we leave the house she almost immediately falls asleep in the van and is very tired the next day.  So, we try to limit outings to essential appointments.

For the past 2 months Katha hasn't eaten more than a meal a day.  In fact, 3 days ago she ate the same plate of food for 13 hours.  She likes to graze the food we give her and would rather drink most of her nutrition in the form of chocolate milk.  The last time I updated the blog we had decided to not have a g-tube put in (which would be a tube directly to her stomach that we would put formula in).  We wanted her to be able to enjoy whatever time she has left.  

Because she has been doing so well but has seemingly lost interest in food, we spoke with her doctor about re-visiting the idea of putting in a g-tube.  He said that because she has definitely deteriorated (she has a hard time holding up her own head now) that no surgeon would be willing to do the procedure to put in the g-tube anymore.  It was honestly a relief to hear this because it took the decision out of our hands.

As I briefly mentioned above Katha is having some trouble holding up her head.  We are waiting for a new wheelchair that has the ability to tilt and with a head support.  This would not only shift her weight in her chair so that she does not get bed sores but would help with her having to hold her own head all the time.

We have also been through several home care workers now.  In fact, we haven't had any kind of in home support since August.  We have a new worker coming on Monday, so we are very excited about that.  Well, I am.  Katha isn't great with strangers, hopefully she'll like this one.

Other than that Katha is doing really well, she still chokes a lot so we have to look out for her aspirating which could cause pneumonia which the doctor is worried she wouldn't recover from but all of her organs are still in really good condition.  Also, she can still move her limbs fairly freely rather than them being stiff which for how progressed her disease is is amazing.

 She still eats by herself and mostly refuses to let anyone help.  She tries very hard to be as independent as possible.

 Since I last updated Katha turned 18 (almost 19 now!) so, technically, she did it.  She survived a fatal childhood disease.  We are so blessed to have her and all of her Kathatude.

 Onto the symptoms:

Enlarged Liver and Spleen: They are still large but are not to the point that they are troublesome.

Dysphagia (trouble swallowing):  It's bad, she chokes several times a day but is always able to recover herself.  She scares other people but we as a family have a gotten used to it.

Basal Ganglia (holding limbs in awkward positions): Katha has almost no small motor skills anymore.  She cannot walk and her feet turn in.  Her legs get tangled and her feet hurt to stand on.

Dementia (memory loss): Katha doesn't have much use for memory.  The other day she was having a really good day and said she wanted to go outside (it was -50 degrees out) I said she was crazy and it was too cold.  She replied that she was cold and I asked if she meant she was hungry and she said yes.  So, I think if she could communicate more clearly that the dementia would be quite advanced.

Seizures:  Katha did have one seizure last year.  It lasted 15 minutes which for a seizure is a really long time.  We went to see a neurologist who told us it was a focal seizure and not something we really had to worry about.  She has not had any other seizures since then so it is being viewed as a fluke.

Gelastic Cataplexy (falling down in response to a large emotional outburst): This happens every time Katha finds something funny.  In fact, she doesn't even usually have time to laugh before it happens anymore.  Now it looks like she faints for 10 to 30 seconds and then regains consciousness.  It is also not unusual for her to stop breathing for these episodes so we try not to trigger them to often but it can be difficult.  She is a very funny girl and she finds her mom quite hilarious!

Sleep related disorders: Katha still has weird sleep schedules.  Right now, she usually goes to bed between midnight and 3 AM then doesn't get up until 11 to 1 PM.  During the night she will wake several times, turn on her tv and then go back to sleep. We still encourage Katha to sleep as much as possible to avoid seizures.

Hearing Loss: It is still hard to believe that Katha has any hearing loss because quite often she will tell a silent room to be quiet.

Diabetes:  Katha has type one diabetes which can be quite difficult to control.  We have an endocrinologist who is very strict.  Katha has never showed any symptoms with high or low blood sugar and would often have lows during the night that didn't seem to affect her at all.  The new endocrinologist was quite worried about these lows so we have to make sure Katha goes to bed with high blood sugar to guarantee she won't have any lows before she's ready to get up.  

April 15, 2021

 So, today we had Katha's yearly check up.  As expected, we decided it was best to stop her medications.  The only medication she has now is her fast acting and long lasting insulin
plus an anti-nausea medication for her vertigo which she rarely takes.

The doctor wanted to know what we wanted to do if Katha starts having seizures.  He helped us decide that we only wanted to make her comfortable if that happens.  We pray that she will remain seizure free her whole life but she is deteriorating and it is a distinct possibility.  

He mentioned that he doesn't think that seizures will be a life ending incident but pneumonia from her choking. 

Speaking of choking, Katha now chokes at every meal, sometimes to the point of vomiting.  However, she is still able to eat unassisted and that includes assistance when choking.

Katha is no longer able to walk at all, she trips over her own feet.  If she has aid (either holding on to something or someone) she is able to stand by herself but can no longer move forward.

She also sleeps approximately 18 hours a day, waking only to eat, then go back to bed.

But, as the doctor said today, she's happy.  She sings, she laughs, she bosses her brothers around.  She's happy and that is our only goal anymore.  Although it's harder to understand her, she seems very content.

Onto the symptoms:

Enlarged liver and spleen: Apparently, they are quite large but the doctor doesn't seem concerned about this anymore.

Dysphagia (trouble swallowing): Getting worse and will continue that way.  The doctor figures she already aspirating into her lungs, it just a matter of time before she starts getting pneumonia

 Basal Ganglia (holding limbs at awkward positions):  Katha can no longer walk and has a hard time holding things because of the awkward position of her feet and hands.

Dementia (memory loss): Katha keep reverting.  She likes movies and shows she liked years ago.  She has trouble remembering much but there isn't much she needs to remember.

Seizures:  She still hasn't had a seizure.  We hope that this continues.

Gelastic Cataplexy (falling down in response to a large emotional outburst):  The doctor offered us medication to help control this.  We decided against it.  As long as we're careful to let her breathe when she has these episodes they don't seem to bother her.  Just like everything else, she has just learned to deal with it, because she's amazing like that.

February 13, 2021

 So, a lot has happened in the last few months.

We decided not to get a g-tube for Katha and just let her enjoy the time she has left.  Since the government doesn't know yet that Katha has deteriorated, they have not cut her off of her medication, Zavesca.  There is a good chance they will still cut us off in March when she has her yearly check-up and have to renew her prescription for it.

Then, a few weeks ago the drug company of Katha's other drug (VTS-270) announced that the risk to reward ratio is not good enough for this drug so are discontinuing the drug.  We have until October before they cut us off completely.  This is the drug that Katha gets put in her spine every other week.

So, after discussing it, Amory and I decided to take Katha off the VTS-270 immediately.  Katha was so happy she is doing better than she has in months.  As you can imagine, it is a very invasive procedure and she is very relieved to be done them.  When discussing this with the doctor he mentioned that because it's Katha's lungs that are the only organ essentially failing at this time, there is a good chance that this could stretch on for years.  Also, he doesn't expect that there will be a huge deterioration coming off of the medication but there will be a gradual deterioration the longer she is off of it. But when considering Katha's quality of life, we still felt this was the best option for us.

 She also was able to receive a hospital bed from AADL.  When the OT was coming to assess our needs she broke down saying she didn't want a new bed.  In usual Katha fashion, she wasn't taking well to change.  Well, the day after we told her she wasn't going to get anymore needles in her back, the bed was delivered.  

 I was expecting that we would have to fight her for a couple weeks about the bed.  In fact, before the bed was delivered, I refused to take apart her old bed frame just in case she refused to use the hospital bed. 

 Well, after it was set up I went to show it to her.  She immediately wanted to get into it and refused to get out until supper time!  The next day she woke up early and refused to get out until food was a must.  Then, after lunch, she decided she needed to lay down until supper time which is not part of her usual routine.  It's amazing and she just loves HER hospital bed.

 Onto the symptoms:

Enlarged liver and spleen: The same as far as I know. We will have an ultrasound in March that will let us know for sure.

Dysphagia (trouble swallowing):Getting a lot worse.  She now chokes at every meal but is able to get it up without assistance.  She also has begun choking at night, which means she having trouble swallowing her own saliva.  We are hoping the inclined hospital bed will help with this as well as helping her sit up.

Basal Ganglia (holding limbs at awkward positions):Katha is having a very hard time walking.  Walking more than 20 feet is impossible for Katha now.  She also trips over her own feet a lot.  We only walk her from bed to the bathroom each morning because usually she's so wet that she would immediately soak her wheelchair.  She also stands up to transfer to and from the wheelchair.

Dementia (memory loss):This is about the same, she knows her family but not really anyone else.  

Seizures:  She still hasn't had a seizure.  We hope that this continues.

Gelastic Cataplexy (falling down in response to a large emotional outburst): We have to be very careful when we make Katha laugh now because she will quit breathing almost every time she does this. Often, Amory and I will just be interacting with each other and she will find something funny and "pass out" coming to with a gasping breath.  

Sleep related disorders:  Katha only stays up for about 6 hours at a time and can sleep up to 30 hours.  But generally she sleeps for 12 hours and is awake for 6to 8 hours.

Vertical Supranuclear Gaze Palsy (Trouble moving the eyes up and down): Honestly, she has this but it rarely impacts her anymore.

Hearing Loss: She will probably have a hearing test in March which will tell us if hers has gotten any worse.

 

October 6, 2020

 So, in June, Amory finally got laid off.  He lasted longer than most and we were very thankful for that.  He tried to do dry-walling for a while but it was ultimately not a good fit for him.  So, at the end of June I got a job as a COVID screener/greeter at a Seniors Lodge in Lacombe.  I love it and it's been an amazing experience.  The people and the job itself is exactly up my alley and I have really been enjoying being able to get out everyday.

However, we did notice around this time Katha had started to decline.  We noticed in July that she started choking on solid foods.  I mentioned to her doctor at one of her procedures that she had started choking on solids.  He was immediately concerned and told us that if she is choking on solid foods, that was a significant sign of progression in her disease.  We needed a swallow test immediately which, if showed she was choking significantly, she would need a G-tube.  Even with a G-tube her life expectancy would be months rather than the years we had discussed back in March.

A G-tube is a tube that would be surgically implanted into her stomach and we would feed her formula through a bag into her port.  We feel that this would really be a bad thing to happen to Katha because she loves to eat and it is a huge motivation for her to keep fighting on this path she been on for her entire life.

So, we had the swallow assessment done and when I was in the room with them, the first time the technicians saw her swallow on the floroscopy, they all gasped.  So, I figured it wasn't good news.  As we were leaving the test (which took 15 minutes instead of the hour it usually does) they told me that the doctor should be able to give us the results later that afternoon.

The test results weren't in until the next day and they were worse than expected.  Katha can no longer simply swallow any consistency of food.  Everything gets stuck in her throat and the only reason she hasn't already asphyxiated is because of her voice box and they don't know how long it will be able to keep her from asphyxiating.  

Basically, they told us it was a miracle that she hasn't asphyxiated already, that she probably will soon and that they need to put in a G-tube as soon as possible.  

Even with the G-tube, Katha still has to swallow her saliva and she gets sick a lot at night.  We have been told that she is now medically terminal (which generally means less than 6 months).

As you can imagine, this has been a huge blow for the family.  We are just enjoying our time that we have left with her and being thankful for having so many more years than the doctors had originally thought. We will be putting in a G-tube so that we have to worry less about choking but know our time is short.

We will try to keep the blog updated as much as possible but it may get difficult in the coming months. 

Please keep Katha in your prayers.  

Now the symptoms:

Enlarged liver and spleen: The same as far as I know. We had an ultrasound but heard nothing from it and usually no news is good news.

Dysphagia (trouble swallowing): Obviously, bad.  Katha has been choking at least once a meal, we are just waiting for her to start asphyxiating.

Basal Ganglia (holding limbs at awkward positions): Katha has a hard time with washing her hands or anything where she has to hold her hands straight.  She also can't straighten her legs or her ankles anymore.

Dementia (memory loss): Katha doesn't know the days of the week, the month or even the year.  She knows her family, but doesn't really pay attention to anyone else to remember them.

Seizures:  Still none

Gelastic Cataplexy (falling down in response to a large emotional outburst):  Katha still "passes out" when laughing.  If she does this too many times in a row she will stop breathing as well as seem very confused when she regains consciousness.

Sleep related disorders: Katha will sleep for 30 hours then be awake for 30 hours.  There is no way to regulate her sleeping.  We just try not to have to wake her up very often.

Vertical Supranuclear Gaze Palsy (Trouble moving the eyes up and down):  This comes and goes.  She can sometimes look up a little but not very much.  Sometimes she can't look up at all.

Hearing Loss: Katha still refuses to wear her hearing aids but doesn't seem to have trouble hearing us when we are talking about procedures.  She will immediately start crying, even if she isn't anywhere near us.

 

 

March 30, 2020

In the last year a lot has happened.  We had to fill out Katha's green sleeve.  For those of you unaware, that is instructions if we want Katha resuscitated or if we would want her to have life saving operations, things like that.  We were warned that this would be a very difficult conversation.  We found that since we have been thinking about this separately for the last eight years.  It was kind of nice to have an open and honest conversation together about it.  It was like Amory and I could finally talk about our deepest fears of what Katha passing will look like.

We tried casting her feet again and it didn't work.  The doctor informed us the only thing that would help Katha be able to stand straight again would be surgery.  We have finally gotten to the point with her procedures that it's a fairly streamlined process.  The last thing poor Katha needs is another surgery.  So, we decided to let her feel just stay pointed.  It makes walking for her harder but she manages to get around the house when she needs to.

Her knees are permanently bent as well.  They are close to the point where walking wouldn't be possible.  The thing is, Katha isn't about to go for long walks anytime soon.  So, we have decided to let nature takes it course as far as her legs go.  However, her feet have started to turn blue.  We have been told this is because of lack of circulation because she is always sitting and have been advised to talk about her endocrinologist (her diabetes doctor) about this because they have more experience with this.  We have an appointment later today on the phone with them.

Katha is back on insulin and has been for quite a while.  She started at a few units of insulin a day.  She is now at 30 units long lasting insulin and almost 1 unit for 1  gram of carbs.  From what I understand, that is a lot and could be a symptom of her organs shutting down but all it really tells us is that her pancreas isn't working like it should.  We won't know how her liver and spleen are doing until we get her blood work back and an ultrasound done.

With the schools help we came up with a new schedule for Katha this year.  Instead of her going to school, we have an EA that comes over every Tuesday for an hour.  This has resulted in Katha being excited to learn again.  Every Tuesday when I tell her the teacher is coming she says "OOoo!"  Which is fantastic!  We are so pleased that Katha likes this EA so much.  She is a very kind woman and we really like her a lot.  She is very understanding of Katha's schedule and has no problem just watching shows with Katha if that's all Katha feels up to that day.

So, as you all know, this pandemic has shut down the world for the last 2 weeks.  It's stressful for everyone.  It has affected us in ways that are the same as everyone else.  Dom and Jay are home with us all day.  I have been finding work for them to do such as reading, writing and some math.  We have all been doing some exercise every morning and Katha has been enjoying her brothers being at home with her.  The boys are starting online schooling today, so we will see how this plays out.

Amory is constantly worried that his job will be shut down by the government.  Right now he is working 6 AM to 2:30 PM.  Everyone has to stay 6 feet from each other.  They have staggered lunch times so that no more than 8 people are in the lunchroom at the same time.  They also have to wipe down everything they touch once an hour as well as wear face shields constantly.

As far as Katha goes, COVID-19 has affected her life in ways that are different from most.  Katha just had her yearly check up in March.  Which, almost got cancelled because we were afraid to take her to Calgary because of all the cases there.  She needs blood work done and was able to do it at the Lacombe hospital a couple days after her appointment in Calgary.  She also needs an ultrasound done but that has been cancelled indefinitely as well as any doctors appointments that are not deemed necessary.  This includes: hearing tests, vision tests, gynecologist and neurology.  In fact, we were lucky to see her metabolic doctor but because he's the one that does her procedures, he was able to do a quick assessment while we were there.

Her procedures are still a go for now.  That could change at any moment.  We were determined to do her last one on March 19 in case we are unable to go back for a long time.

We have been online a lot worried about Katha getting this virus, as it could kill her.  However, there have been 3 different studies by 3 different groups of doctors which are finding that NPC patients are mostly likely immune to COVID-19.  Something about how their metabolics don't allow the symptoms to appear in people with NPC1 mutations, which is what Katha has.  However, she would still be able to carry it and give it to other people.  So, we are happy to stay isolating ourselves.  Especially since a common cold could have the same devastating effects as COVID on Katha.

Today, as I mentioned above, we are having a diabetes appointment over the phone.  We have never done this before, so it should be quite interesting.

At our yearly appointment, the doctor kind of gave me trouble.  He feels the amount of Migulstat I've been giving Katha isn't having an effect.  She is currently getting 200mg a day and she should be getting 600mg a day.  Once we are able to get an ultrasound done, he would like to take her off this medication completely to see if there is any difference in her cognitive abilities and her organs.  This would mean Katha would no longer be lactose intolerant and would be able to start eating many of the foods she has had to give up for 8 years.  I know she'll be extremely excited.

Onto the symptoms:

Enlarged liver and spleen: The same as far as I know.

Dysphagia (trouble swallowing):Since Katha has diabetes, the only drinks she can have regularly are chocolate milk and water.  Because the chocolate milk is thicker, she has stopped choking almost completely.

Basal Ganglia (holding limbs at awkward positions):Katha has a very hard time holding her hands straight and her feet are constantly pointed making walking difficult.

Dementia (memory loss):  Katha's sense of time is completely gone, which has been kind of sad because she was always the one that knew what day of the week it was and that would have been really handy right now as all of the days kind of run together.

Seizures:  Still none

Gelastic Cataplexy (falling down in response to a large emotional outburst): Katha had an episode of Cataplexy the other day that lasted approximately 45 seconds.  This wouldn't be a big deal except that she quits breathing during these.  It was truly terrifying.  I mentioned it to her doctor who told me that this happens because her tongue is blocking her airway.  He recommended that she gets an oral airway which is a plastic tube that we would put in the mouth/throat that would keep her airway open during these episodes.

Sleep related disorders:  Katha sleeps when she wants to sleep and stays up when she can.  Generally she's only awake about 6 hours a day.

Vertical Supranuclear Gaze Palsy (Trouble moving the eyes up and down):  This comes and goes.  She can sometimes look up a little but not very much.  Sometimes she can't look up at all.

Hearing Loss:Until we can get a hearing test done, we won't know if this has gotten better or worse.  If I had to guess, I would say it has stayed the same.

February 13, 2019

So, it's been about a year since I updated.  So, it's time!

We have been going to Calgary for Katha's procedures for over a year now.  It has been so much easier but we definitely miss California, especially the people.  Also, the drug company cut out any reimbursements for travel expenses which has been difficult but we are making it work.  Luckily, the doctor let us book a later time so that we don't have to stay overnight anymore.

Katha started school at the local school after running into a lot of difficulties trying to register her for home schooling.  The boys school said they would help with Katha and that we should register her there.  So, that's what we decided to do.

It started out great!  She got to school a few times in the first couple weeks of school.  Then, on September 11th, she had an operation.  It didn't go quite as expected and there was no way that she would be able to attend school.  We ended up going back and forth to Calgary a lot.

Then, in October, we had to rush to the local hospital because of what we thought were complications from the surgery.  Turned out that Katha is also diabetic.  It took a while to find out if it was type one or type two because, of course, Katha showed signs of both. 

At first, that was a tough blow, thinking of all the pokes she was going to have to endure when she's already had to go through so much.  Not to mention, she has no drug coverage because of the $10,000 biweekly that her Zavesca was costing out insurance.  But, we were told that she would have drug coverage again come December 1st.  She now has drug coverage again which is going to all of the medications and supplies needed for diabetes.

The diabetes nurse gave her a machine that monitored her blood sugar with a sensor that sits on her arm and only had to be changed every two weeks.  Also, she was only getting a little bit of insulin so there was only one needle a day at the beginning.

We had to change her diet slightly (thank God for Fairlife chocolate milk that's lactose free and only half of the sugar!) but we caught the diabetes early.  She is right now going through what is known as a honeymoon phase and isn't on insulin at all and hasn't been since November.

However, she is type one diabetic and will have to go back on insulin eventually.  Hopefully, it won't be too much.

Katha isn't really walking by herself anymore, but she's still standing on her stander everyday.

After all the casting last year, her feet are back to being pointed, even with wearing the braces everyday.  That doctor figures we will probably have to put Katha in casts for 2 months every year.  It's kind of a pain, but she is so much more stable when her muscles are stretched.

We are restarting casting in March.  I hope it doesn't hurt her too much.

The complications from Katha's surgery have subsided and we are able to get back into her regular routine.  However, she is so over school.  It's just not worth taking her.  I have to drag her into school where she is miserable the entire time she's there.  I'm thinking it might be time to have a chat with the school about getting her registered for home school next year.  The problem is that the Alberta Government wants parents to agree that the home schooled children will have certain abilities by a certain age and they will check that they are learning these things.   Things that would make absolute sense with most kids but Katha is never going to be able to read or things like that.  So, it's all very frustrating. 

We did finally get her adult wheelchair.  Remember the expression "be careful what you wish for because you just might get it", this wheelchair is the physical embodiment of that for me.  Well, it was at first.  It scraped everything, couldn't get over the smallest bump and was almost too big for the doorways.  However, we have gotten used to it since March.  Katha doesn't really push herself around anymore because it is big and awkward.  We are very thankful to have a wheelchair that Katha fits in and doesn't fall apart all the time.

So, it passes through my mind a lot that back in 2012 we were told we would be lucky to have 5 years and here was are, going onto 7.  We are so grateful everyday to be able to play, talk and interact with Katharina, or as I have recently nicknamed her, Fargle Schnoogen.  (I called her Katha and she corrected me to Katharina so I told her since I had named her I could call her Fargle Schnoogen if I wanted).  We love her laugh, her singing and most of all, her sass.  We are so thankful to have this amazing experience of just knowing her incredible strength and personality.

So, onto the symptoms:

Enlarged liver and spleen: The same as far as I know.

Dysphagia (trouble swallowing): We had this retested, she is having slight trouble swallowing and they recommended that we thicken her liquids.

Basal Ganglia (holding limbs at awkward positions): Katha has a hard time holding her hands out to be washed.  They are basically always a little curled and she cannot walk or stand flat on her feet, she is on her tiptoes whenever she stands.

Dementia (memory loss): Katha has no sense of time and unless she really knows you, she probably won't remember you.  There are very few people Katha bothers to remember.

Seizures:  Still none

Gelastic Cataplexy (falling down in response to a large emotional outburst):  So, I have recently discovered that this is actually a type of seizure.  So, I guess Katha does have a type of seizure but they don't seem to effect her cognitive ability at all.  Plus, it's fun to make her laugh so hard she "passes out".  In fact, she will sometimes do it to herself.

Sleep related disorders:  Katha has a very hard time going to sleep a lot.  She will often get her days and nights mixed up.  There have been times where she wakes up at 10 PM and will be going to sleep at 7AM.  This is another big reason it's hard to get Katha to school

Vertical Supranuclear Gaze Palsy (Trouble moving the eyes up and down): This has been pretty good anytime I've tested it.  She has trouble looking up but can look further up than when she was first diagnosed.

Hearing Loss: Honestly, I couldn't even tell you where her hearing aids are.  She hasn't worn them in probably a year.

January 17, 2018

Hello Everyone!  It hasn't been too long since I last updated but a lot has happened!

We still have not received Katha's adult wheelchair.  After getting measured again and having the test wheelchair for two weeks we got a phone call about a month later that the Occupational Therapist had to confirm that the chair fit.  So, we booked a meeting with her and everything went well.  

A couple of weeks later, we got another call that AADL had decided that we needed to "grow" Katha's current wheelchair.  Which, essentially means to adjust it to it's maximum size.  This wheelchair is still a child's wheelchair, and Katha is now taller than me, not to mention she's 170 pounds!

So, we went back to the store in Red Deer and they confirmed that the wheelchair would not be able to grow to Katha's size.  So, AADL had to approve that.  Then the kind of wheelchair we tested was no longer covered by AADL so they had to find one that was as similar as possible that would be covered.  The good news is, the wheelchair has FINALLY been ordered.  It could still take a few months, but at least it's coming in.

We have Katha's stander and it's amazing!  It keeps her able to stand for exersize to help keep her calves stretched.  With Katha, that is a big deal!  It also has massive wheels so she's able to do things like try to catch her brothers.

In October, we were finally able to start Katha's casting.  We did it for 2 months and Katha hated it.  They were big, and clunky but she got through it.  She was amazing as usual and we now have what are called AFOs.  Which, is essentially a fancy way of saying that Katha has braces on her legs.

She wears them 12 hours every day and it helps to keeps her calves stretched along with the stander.  She was able to pick a patter for them and although she's not a huge fan of them she wears them every day like the warrior princess she is.

Some of the big news is that we are finally going to Calgary for the procedures.  Her doctor did the first lumbar puncture in October and things went very smoothly.  We have returned to Calgary every 2 weeks since then and things have been going well.

The best part is that we come home right after the appointment and Katha is able to sleep in her own bed.  You have never met anyone who loves their own bed as much as Katharina.

Things had been going well in Calgary up to the last appointment.  Unfortunately, that was when our luck run out.  Her doctor poked her 6 times and was still not able to get spinal fluid out.  So, he has decided to bring in radiology to help guide the needle to be able to get into the spot he needs.

This, of course, brought us back to discussions of the port.  He has put in a port for review by the drug company but had heard nothing back.  To make matters harder, he thought he had finally made some headway and the drug company was sold.  So, now we have to start all over at the beginning again.  Hopefully the person who was in charge of making that decision keeps their job with the new company and it will get approved quickly. 

There are other kids with ports for this medication, however, our doctor told us that the ports are meant for veins and not spinal fluid and he has found one that will withstand the medication and is meant for spinal infusions.

So, the reason I decided to update the blog today is because we had kind of a big day.  Due to unforeseen circumstances, Katha's amazing EA has had to quit.  Today was her last day.  So, I made an appointment with the principal and we have decided to take Katha out of school.  I'm sure there will be lots of paperwork to sort out and we'll have a day when Katha will be able to say goodbye to her class but essentially, she's done.  She is thrilled about this decision, it's been a little harder for me.

This wasn't a decision we made lightly.  It was really difficult because it feels like we're giving up.  I know this was a battle we were going to lose eventually but it's still hard to admit defeat.  However, Katha went to school for the first time yesterday in six weeks.  I dropped her off at 11 AM and by the time I got there at 2 PM, she was meeting me at the door and telling me she was too tired to stay any longer.  Apparently, she had been saying that since noon.

Also, the kids she goes to school with this year are not the kids she grew up with.  They are from the year  below her, so she doesn't remember them.  I know she has felt all year like she is going to school with strangers.  Not that the kids haven't been amazing, they have!  They make her feel welcome every time she goes.  It's just that, with the dementia, she just can't find it in herself to get excited about school anymore. 

So, the fact that I have to fight her constantly to go to school when she is there simply to be social was a huge factor in deciding that it's just not worth it anymore.

Well, I think that's it for now, onto the symptoms:

Enlarged liver and spleen:  She had an ultrasound done and I heard nothing so I guess it's the same?

Dysphagia (trouble swallowing):  Katha will choke occasionally but is able to eat by herself still.

Basal Ganglia (holding limbs at awkward positions):  This is what we've been fighting with the casting.  So, obviously, it's bad in her legs.  Her arms are still held at awkward positions frequently.

Dementia (memory loss):  Her dementia seems to be improving.  She now remembers that Jay is a boy and not a baby.  But, that probably has a lot to do with the fact that Jay has found a new love of annoying his older sister whenever possible.  It's amazing to see them interacting because they never really have before.  In fact, when her casts came off, the doctor told me that we would have to tickle her legs to get them desensitized and I knew just the boy for the job!  Jay thoroughly enjoyed his task.

Seizures:  Still none.


Gelastic Cataplexy (falling down in response to a large emotional outburst):  This seems to be improving as well.  In fact, at our Simon Christmas we usually have a little program every year.  This year Katha's cousin Emma sang for us and Katha KILLED herself laughing the entire time but not once did she lose consciousness!

Sleep related disorders:  Katha usually gets her days and nights mixed up at least once a week.  She will be unable to sleep until 3 AM then will sleep in until noon.  It usually takes her a couple days to get back on track.

Vertical Supranuclear Gaze Palsy (Trouble moving the eyes up and down):  Katha has trouble looking up but is able to do it some days.

Hearing Loss:  Katha still hates her hearing aids and doesn't seem to need them.