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.
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