Brisan and Parker Stults | Living with Niemann-Pick Type C

Posts Tagged ‘Gelastic Cataplexy’

Parker lately has been having gelastic cataplexy attacks very frequently. We've had both Brisan & Parker on medicine to help this but the last 3-4 weeks Parker seems to be reacting differently to the changes Niemann-Pick Type C is placing on his body. This is all to be expected.

Parker laughing is a joy to watch because he is so innocent with his sweet soul. The bad part is when he does start this laughing “fit”, it creates a loss of muscle control & tone. He essentially becomes a noodle and falls. Saturday (1/29) he was out of his chair getting a diaper change and he stood up for a few moments and then accidentally fell into the corner of the wall gashing his head open. We've been trying to get video of him trying to walk and when he has these cataplexy episodes. Hard to plan for those things…

Sadly we have to keep him and Brisan more in their chair although we hate doing this. The other challenge is we can't be a 100% of the time hovering over both of them to keep them safe. It just isn't possible and especially with a little squirrelly three year old running around.

Starting on the next school day (depending on when that will be with all the snow expected tonight), we will be sending Parker to school with his wheelchair just like Brisan. Now that doesn't mean he can't get out and roam around but it will provide a safe haven for when he is unstable. This has worked well for Brisan at school. It just takes one good fall to do some serious damage. You and I don't think much about this because we typically have enough control to protect ourselves on a fall. It would be like free falling out of the sky and hitting the ground. There is nothing you can do to soften or protect yourself from the impact.

Last week Parker fractured his nose (1/22) because he fell. That all happened within a matter of minutes.

Here is a video we shot on Sunday (1/30) showing Parker laughing. He was going really good earlier before I grabbed the video camera to record. You'll notice he has a couple good head drops along with the vertical gaze palsy (issues with moving the eyes up/down/side to side. the “awkward” eye movements.) Plus the poor kid needs a hair cut????!!!!

On Saturday June 26th we had a fundraiser for Brisan and Parker. Northland Auto Body and Mrs. Darla Sartain organized a great event for the boys! It went super well and saying thank you will not even begin to describe how we truly feel! If you'd like a t-shirt we still have some available. Please send Jenn an email @ jennifer @ bripardun .com .

Brisan has had a pretty hard time this week. In the last two days he has had a couple gelastic  cataplexy attacks that has sent him once into the tile on the fireplace and the other one tonight into the ottoman. He lost both of the battles! I wanted to get a picture up here to show everyone but didn't get that accomplished this evening. His poor nose, lips, head, and whole face is just bashed up. He has scratches on his nose and his upper lip/ nose area is all swollen! Now some of you are asking “doesn't he have a helmet?” Why yes, yes we do but it doesn't protect the front of your face.

The next step is to see the neurologists and see what medications they can give him to help this sudden lose of muscle tone. Some of you might not know but this is apart of Niemann-Pick Type C and its progression. We really don't want to keep him in his chair the whole time while he is inside our home. He needs to be able to move around and keep working his muscles out!

Parker and Brisan both have had bleeding this past week (we all know Brisan has with all his facial injuries). They've upped their infusions of DDVAP to twice a week now. So we almost have a nurse in our home everyday during the week now!

Parker isn't doing well eating his food. We know the kid loves to eat but he just doesn't chew his food and can put Joey Chestnut (hot dog eating champ) to shame! The only problem is he starts to get bug eyed and starts choking / aspirating his food. At this point we can't just take his food away. He'll have that cruel situation to deal with later when his brain forgets how to do that. This is tough on us because we don't want either to happen.

Tomorrow (June 30) is the last day of summer school for the boys! Please keep them in your prayers. They aren't doing well except they aren't in the hospital which is a great bonus! NPC is progressing and although it is variable for every patient, it isn't fun to watch them lose the things you and I take for granted.