The Journey Continues

So what’s new in my ALS journey?  Some things are the same and only slight changes are visible to me and to those people I see every day.  Stopping Tirasemtiv, my study drug, at 48 weeks appears to have had an effect on my muscle strength and/or fatigue.  You see, I believe that during the study, which lasted 48 weeks, I was receiving the real drug and not a placebo.  Stopping the drug for 4 weeks to enter the washout phase of the study was wonderful.  I was able to live every day out of the fog of the drug.  In truth, it was only a 1-2 hour period in the fog and toward the end of the 48 weeks there were times that I didn’t feel it at all.  But when I had to temporarily stop taking the drug, I did feel as if my muscles were weaker or more fatigued.  I needed a bit of help to do things I was able to do before like adjust myself in my wheelchair.  I still need that help but I’m hoping that it was fatigue and not a loss of strength.  This Friday my open label dose of Tirasemtiv will increase from 250 to 375 mg.  I’m anxious to see what effect it will have.

My speech is also worse now.  (I made a video recording you can listen to.)  The morning dose of meds combined with dragging myself out of that luscious sleep makes my talking almost incomprehensible.  A few people ask me to repeat myself and not all of them are hearing aid wearers or those that should be smiley-face-clip-art-emotions-400_F_9999900_9yijqIYv4KPY0vd4qCSNrLZx1W2gO0bO.  The feeling that I have not been understood continues to bother me.  It bothers me so much that I don’t like to talk on the phone anymore.  Repeating myself at the end of a long day is tiresome and a real pain in the a$$.  On my next visit to the ALS Clinic I will be asking about the latest text-to-speech technology.  I have an app on my phone (see Aug. 9, 2017 blog post) that works pretty well except for the small keyboard.

And walking is a thing of the past.  I gave that up in February of this year when The Pinkster arrived.  The Pinkster is my 400 lb. power wheelchair.  It gets me around quicker and safer than walking.  The only things not in love with it are the walls, door frames, gasket on the door of my van – you get the idea.  Your feet would also really dislike it; trust me.  Prior to walking I gave up driving.  I gave it up voluntarily upon diagnosis.  I figured the rest of the world would appreciate it since my right foot was the first thing that fell victim to ALS.

I do experience muscle fasciculations which are small involuntary muscle twitches.  If you ever felt an unborn baby moving inside his/her mother’s womb, you get the idea.  They are not painful to me at this point but can keep me from falling asleep when they are really active.  The only physically painful part of ALS to date is cramping.  Stretching can cause cramping.  I am also unable to reach behind my back without having my arm or hand cramp.  Using a lot of pressure on a knife to cut food also has the same effect.

Clothing is another new issue.  So far, when we are out and about my husband and I are able to manage with clothing and bathroom breaks.  I don’t know about how other female PALS feel, but the handicap stall in the ladies room is too small for a power wheelchair.  And can someone please explain what the grab bar behind the toilet is for?  We find that companion restrooms are best suited for my Pinkster.  It also eliminates the necessity of hanging a sign on the ladies room door indicating that a male is within.  Of course, since there is frequently only one companion restroom in an establishment (if I am lucky), I must hope that it is not occupied, therefore, waiting until the last minute to find it is not recommended.  If you are on the road, plan ahead and search for a Starbucks.  Besides good coffee, they have great bathrooms for power wheelchairs.  But please, can we put the towels and soap near the sink?  And how am I supposed to reach that faucet?  I don’t know how much detail I will divulge about the clothing issue, but going commando could be in my future!

Because of the always changing challenges of living with ALS I have been searching the internet and reading the blog posts of other PALS (person with ALS) for suggestions on how to adapt.  One site I found very useful for PALS and CALS (caregivers of PALS) is written by Diane Huberty, a former nurse specialist in neuroscience and a current PALS.  I think that you may find Diane’s information useful.  Her insight into living with ALS can be found by clicking here.

Wash-out at 60

Friday is the day my wash-out from the Tirasemtiv clinical trial is over.  I really did enjoy those fog-free days.  I felt less woozy and tired, but on the downside, my muscles felt more fatigued.  I am very interested to get back on the drug and see if my muscles have a bit of a comeback.  I missed them.  I don’t enjoy the muscle weakness.  The other day I tried to lift a half-gallon of milk to make my oatmeal moister, and I could not do it.

There are so many annoying things brought on by ALS.  My speech is pretty bad.  I can’t lift my legs onto the wheelchair footrests without some sort of assistance.  I can’t reach behind to scratch my back or pull up my pants without my arm cramping.  I can, however, still type.  So you will still be annoyed by my rants.  Lucky you!

IMG_6376I had a date today with my aide.  We went to brunch to celebrate my 60th birthday on Monday.  I never imagined celebrating 60 in a wheelchair, but it is what it is.  If there were sidewalks here, I’d be out more.  It will be interesting to see what happens in the winter.  When I took the trip to Boston, I started shivering the second I got off the plane.  Seems that 60 degrees is too cold.  Heat does not bother me though, or at least it doesn’t bother me now.

ALS constantly requires you to adapt.  You may enjoy reading this article about technology’s effect on healthcare.  The author had the same idea I did regarding her Amazon Alexa.  I even changed the name just as she did.

Remember to support ALS research!

Changes, Good and Bad

I am noticing some changes this week.  Monday I finished my clinical trial of Tirasemtiv, a drug designed to increase “skeletal muscle force and power while delaying the time to muscle fatigue” (Cytokinetix, 2017).  Read more about Tirasemtiv by clicking here.

Besides not being able to fall asleep the past two nights until after 3 a.m., I am not experiencing my usual woozy days since first starting the clinical trial.  This leads me to believe that I was actually receiving the real drug and not a placebo.

I am now in a four week “wash out” period before I receive the real drug.  After two weeks I can decide whether I want to continue on the drug free of charge for life.  (Life?  Who ever gets anything free for life?  Oh, people with fatal diseases.)  I will find out the results after the study closes sometime in October.

Last year at this time I had just left my job.  I was walking with a rollator (a walker with front wheels).  I had already stopped driving after receiving the diagnosis on April 29, 2016.  I still hear stories by people punching that time clock and wonder if they too are working to retire and enjoy life just as I was.  I enjoy it in a very different way now.  I have added some new friends to my repertoire who share the same disease.  It is easy to relate to people who are experiencing some of the same symptoms and heartache as I am.

So the keyboard is becoming my voice.  Some people have begun to ask me to repeat myself.  I already feel like the third wheel in conversations.  It takes too long to enter a conversation and get the words out.  A lot of people are impatient, myself included.  So often I want to add a comment or tell a joke but it’s too far into the conversation or too much effort.  It seems impolite to ask others to standstill while you’re trying to be conversational or clever.fullsizeoutput_c09

One magical technological advancement that is working to my advantage though is that I am able to use the text-to-speech app on my iPhone to talk directly into my husband’s hearing aids.

Alas!  I have so far successfully busted every husband’s dream of not hearing his wife.  Almost wish I had this app years ago.

@alsassociation #ALS @alsadvocacy #FindTheCure @alsncchapter


Cytokinetics.  (2017).  Putting our power behind a potential treatment for ALS:
Tirasemtiv.  Retrieved August 9, 2017, from