November, 2018

Thanksgiving, Hayden, Idaho

Sorry I have been so quiet for a while, but life has been interesting. After being approved for Keveyis, I had to work through what they call a titration schedule to determine the best dosage for me to take. I am very glad I did too. When I tried to change from 25mg twice a day to 37.5mg twice a day, just one dose of the 37.5mg made me feel as bad as I did before I started taking Keveyis. So I talked with the pharmacy, and my neurologist, and we decided that 25mg twice a day is the optimal dose for me. If there is one thing I wish Strongbridge Biopharma would do, and I have heard others on this medication say, is produce the medication in smaller amounts. Currently you can only get it in 50mg pills, but a lot of people have to start lower and/or never get to the 50mg dose because they can’t handle that much of the medication.

Since starting Keveyis my paralysis attacks have pretty much stopped. In the first 10 days of being on the medication I only had 2 paralysis attacks, and the only times I have had attacks since then have been when I have been injured or had severe triggers. It hasn’t cured me of my periodic paralysis, but has stopped the daily attacks and replaced it with muscle weakness. I can take walks around the block with my family now, and go to my daughters school without the wheelchair sometimes, and even drive again at times when I am having good days around town.

What I did realize recently is that I am not all better as I would have liked to have believed.  I came down with a cold a little over a week ago and it completely wiped me out.  It took me back to the fatigue I had before getting on the new medication.  I didn’t start having the daily paralysis again, but the fatigue was severe again and I had trouble doing daily tasks.  I had to use my wheelchair to go to my daughters school exhibition night because it would have been unsafe to go without it because of the severe fatigue.  It is really hard to feel more normal and then have a setback like that.  It has helped me realize that it isn’t going to go away and I will have good days and bad and that I just need to take advantage of them as I can.  The medicine has even worked well enough at times that I have been able to help with yard work before the weather started getting bad.

I have also started to attempt physical therapy again to see if we can make some gains and get some muscle strength back.  I only got two sessions in before I got sick and had to cancel a couple and even that was a lot.  I am finding out that there are still a lot of triggers that if combined can wipe me out for the day.  My physical therapist, Dawn, is amazing and has even talked with someone I met in a support group who has periodic paralysis and is trained as a physical therapist so she could learn about my rare disease to figure out the best way to help me.  I think she will be able to help me understand how to notice when my body is telling me what my limits are so I can make the best use of my energy without pushing to hard and causing more problems.  I am excited to see where we can get to.

The picture on this blog is of me and my parents on Thanksgiving at my in-laws this year.  Since I have been sick I was only able to head over for Thanksgiving dinner and then head back home after because I just didn’t have the energy to spend time there.  It was good to see my wife’s brother and family if only for a couple of hours.  I am hoping to be able to go over today and see everyone as my fatigue levels are a bit better and it is there last day here for this trip.  I am very thankful for the ability to find a medication that allows me to feel good enough to be able to spend more time with my family in less restrictive ways and have some hope for better days ahead.  Even though I am very sad that we found out my daughter also has this rare disease hypokalemic periodic paralysis, I am thankful that we know what it is and are able to find a doctor at Seattle Children’s Hospital that is familiar with it that we will be able to see next month.  Hopefully she will be able to help us find an appropriate treatment for her and allow her to have a more normal life instead of ending up as disabled as I am from living with this disease my whole life without knowing it and causing lasting damage.

You can also see a video of me below in my Action Trackchair attempting to plow snow for the first time.  This was the first reasonable snow of the season for us and a really wet one, so it seemed like a great one to do some practice with.  It is a bit long, so don’t feel like you need to watch it all, but it gives you an idea of how great it is to be able to have this amazing tool Chive Charities was willing to provide me to allow me to be a more productive member of my family and have a little fun while doing it.  I can’t wait to see how it does when we get a real good snow fall!

Until next time!

First Snow Plowing

This is my first attempt at plowing snow in my Action Trackchair.  It was less than an inch and very wet, so I thought it would be good to practice on.

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