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.