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.
