So, on January 10th, we went for an EEG. For those of you who don't know what that is (which I certainly was unsure) they take a bunch of little wires and stick them to your head using what looks like a mix of Vaseline and wax and try to find out if you are susceptible to seizures.
They want you to sleep through this test. So, they had us keep Katha up until midnight and get her up at 4 am and keep her up until the appointment at 8 am. Which, actually, was a lot easier than I even thought it would be. I rented a hotel room for the night because my thoughts were, the shorter the drive, the easier it would be to keep her awake. I think it was a good idea because the roads were TERRIBLE because we were in the middle of a blizzard. In fact, the technician was late because of the roads.
Katha got to the test exhausted, and the technician was having a bad day, I think. Katha was absolutely sure this guy was going to hurt her in some way. So, when he went to measure her head to figure out were to put the wires, she FLIPPED. She broke down in a way I haven't seen her break down in a while. He also only tried for about 2 minutes before he threw his pen and went for help.
The woman he brought back to help him was a godsend because she almost immediately got Katha settled down and got her head measured out and the wires on. However, the guy was still the one to run the test. Katha, still convinced the guy was out to get her, refused to go to sleep. She kept saying that he was just waiting for her to fall asleep to put needles in her head. I felt so bad, but she was not going to listen when I told her she was fine.
Exhaustion finally won out and she fell into a light sleep for about 10 minutes. Then they put on a strobe light and flashed it in her face for about 5 minutes.
After the test we went to see her neurologist. Katha was excited about that part because she loves her neurologist. The test showed that Katha did not have a single sign of having a seizure. Dr. Smyth also told us that she has been doing a lot of research about NPC and found out there's a 50% chance that she will never have a seizure and that's apparently on the pessimistic side.
That news was like a great weight taken off of our shoulders because we were informed that it was just a matter of time until her first seizure. To find out she may never have one?! It was hope where there was none before and that's a wonderful feeling.
Other than that, the doctor noticed she hadn't regressed anymore since our last appointment. Which is also a big deal because she was. I really think the Zavesca is helping. She walks a lot better, and hasn't really fallen in a couple weeks. Although, the day of her test she was so tired that she wasn't doing anything. It was like she was in a trance. You would call her and she would just look through you. It was scary.
Also, the day we went for the test my mom showed me an article in the Stettler paper that my cousin wrote about our experience with the Bashaw Community Theatre. It was amazing. If you have access to a Stettler or Bashaw paper, I highly recommend it. It's all about the benefits of living in a small town and the example of the generosity of the theatre group shown to Katha. It really was an experience I will never forget.
I got an e-mail from the cyclodextrin trial. It was on hold because the FDA was concerned the medicine and the tube that goes into their head is not compatible, but apparently they have resolved that. Hopefully we will know whether or not Katha is in the trial in the next month or so (fingers crossed and lots of prayers).
So, onto her symptoms:
Enlarged liver: Still enlarged
Enlarged spleen: Still enlarged
Ataxia (unsteady gait): That walks on her toes all of the time. However, she is no longer stomping when she walks and hasn't fallen down in a while.
Dysarthria (slurred speech): She still talks really slowly (slower I think) and is still quite hard to understand. However, she seems to be more "with it" and able to communicate what she's thinking more. If your willing to let her take her time to say what she needs to say, she can carry on quite a conversation. But, you have to be very patient.
Dysphagia (trouble swallowing): She still drools, and it happens quite a bit. When she coughs or chokes, it's like she can't cough very hard. I don't know whether she's trying to do that or whether it is a symptom.
Basal Ganglia (holding limbs at awkward positions): I still notice her doing this once in a while, but it's not constant anymore. She also has gained some more small motor skills. She still prefers her drinks in something with a lid because she still has some trouble holding things.
Dementia (memory loss): I thought this was becoming a problem, but recently she has started talking about things that have happened in the past. Which, she wasn't for a while. I think her memory is improving slightly as well.
Seizures: No seizures, hopefully it stay that way!
Sleep related disorders: Katha hasn't been as tired after school when she does go. She has been more alert since starting the Zavesca, but has continued to stay up later than usual.
Gelastic Cataplexy (falling down in response to a large emotional outburst): This has definitely improved. She is able to laugh again! At her appointment the doctor wanted to see it and since she was tired, I thought she might do it, but I couldn't get her to fall. Of course, the next morning while mom and I were giving her a bath, she did it. That was mom's first time seeing it.
Sensitive Touch: Katha has started letting other people touch her again, even if it's just holding her hand or a quick hug. She still doesn't really liked to be hugged by people other than Amory, the boys and I, but she will do it.
Bladder control: This remains to be a problem. It's better when she goes to school, and she doesn't have nearly as many accidents at home anymore but it still happens. Of course, the day of her test she had the worst reaction to the Zavesca I seen her have yet. In fact, I had to stop at home from Calgary to mom's to get more pants because something she ate must have had milk in it. She had 3 accidents because of loose bowels in the hospital but after that she was fine.