Tuesday, June 21, 2016

als - pseudobulbar affect and a Rx worth looking into

I want to raise awareness of Pseudobulbar affect, because until six years ago I had never heard of it.

First, here is some condensed information straight from Wikipedia
(Oh admit it, you’ve used this site too.)

“Pseudobulbar affect (PBA) is a type of affect characterized by involuntary crying or uncontrollable episodes of crying and/or laughing, or other emotional displays PBA occurs secondary to a neurologic disorder or brain injury. Patients may find themselves crying uncontrollably at something that is only moderately sad, being unable to stop themselves for several minutes. Episodes may also be mood-incongruent: a patient might laugh uncontrollably when angry or frustrated, for example.

“In some patients, the emotional response is exaggerated in intensity… For example, a sad stimulus provokes a pathologically exaggerated weeping response instead of a sigh, which the patient normally would have exhibited in that particular.

“Patients report that their episodes are at best only partially amenable to voluntary control… they often have insight into their problem and judge their emotional display as inappropriate and out of character.

“Such sudden, frequent, extreme, uncontrollable emotional outbursts may lead to social withdrawal and interfere with activities of daily living, social and professional pursuits, and reduce overall healthcare. This may lead to severe embarrassment and avoidance of social interactions for the patient, which in turn impairs their coping mechanisms.

“Pseudobulbar affect is a condition that occurs secondary to neurological disease or brain injury, and is thought to result from disruptions of neural networks that control the generation and regulation of motor output of emotions. PBA is most commonly observed in people with neurologic injuries such as:
traumatic brain injury (TBI)
multiple sclerosis (MS)
amyotrophic lateral sclerosis (ALS)
Parkinson's disease (PD)
Lyme disease  

“A study designed specifically to survey for prevalence found that 49% of patients with amyotrophic lateral sclerosis (ALS) also had PBA. "

I’m back.

I started experiencing this side effect.   For me, while it was not yet debilitating, it was increasingly frustrating to not be able to control my emotions as I had before.     I didn’t know why things were funnier or sadder than they ought to be.   There were times where I would be crying and in my head I was thinking, “What is going on?  This isn’t THAT sad, I have got to get a grip on things.”  I could reason out the situation as it was happening, but I was unable to control it.

In relaying this to my doctor he told me about pseudobulbar affect and recommended that I try Neudexta.

Within a week of going on Neudexta I began to feel more in control and it was such a relief.

Neudexta comes a pill form and is pricey and I have since switched to Dextromethorphan/quinidine which has the same ingredients but it is a fraction of the cost. It is mixed at a compounding pharmacy. 

Friday, June 17, 2016

als - just use the dang walker

I think I have had an overall healthy lifestyle for most of my life.   I figured while here on earth I get one body, so why not take care of it and then see what it’s capable of. 

Then slowly, bit by bit, the effects of als began to creep through and I began to lose my body.

The foot drop started in my left foot and that is when I began to trip. Sometimes I would catch my toe on the edge of a rug or an uneven sidewalk crack.  If I would put too much weight on the outside of a foot, pivot too quickly or walk backwards… Those careless movements would topple me over.  

I tripped and stumbled and fell with increasing frequency. 

For much of that time there was a walker in my bedroom sitting there gathering dust.

What’s that?  Why wasn’t I using the walker?  I would have to say it was because I was (am) stubborn.  I wanted to use up every independent step I had.    I knew once I started using the walker there was no going back to unassisted walking.

Losing the ability to walk normal was devastating.  Walking is just something you do.  You shouldn’t have to think about putting one foot in front of the other.  I have mentioned this before, but it blew my mind that for the first time in my life I couldn’t just exercise harder to get stronger.   I would still go to the gym and use the elliptical machine to try and maintain the muscle I had, although there was no hope of building any new muscle.

Eventually the process of going to the gym took too much energy.   I bought a stationary recumbent bike off of the classifieds and “worked out” at home.  I used to do a simplified yoga to stretch my always tight muscles.  

The day finally came when I was tired of always being worried about falling.  I sat on my bed looking at the walker and finally took hold.   And that was the end of unassisted walking.   But, more importantly, it was the start of increased safety.  The walker provided needed balance and when I was tired I could turn around and sit on the seat.  Once I finally started using the walker I felt a greater peace of mind.   

Hindsight being 20/20, I should have started using the walker long before.

Some time has passed since then and now I only use the walker minimally during transfers and have moved on to…  The wheelchair.

Mentally this was a VERY difficult transition to go from walking to sitting.   The first time I took the wheelchair out I went on a walk with my sister and I was on the verge of tears the entire time.   I was grieving the loss of my ability to walk.

But, it was time.  The wheelchair has provided so much more freedom to go where I want.   I love being able to go to the park with my kids or to 7-11 and get slurpees.  I can go on trails, or to Red Butte Garden or the zoo and not be completely exhausted afterwards.  We have a lift on the back of our minivan to transport it around.  It works well enough since I can still walk a few steps with Jeff’s assistance to transfer from chair to minivan.   I am so grateful he has big muscles :)

Sometimes when Jeff will ask me, “What do you want to do today?”

I say, “I want to go for a run.”  

Don’t feel sorry for me (Ugh).   Pity and self-pity is something I try hard to avoid.

I just think that it would be so cool to break out of this body and RUN.  

Friday, June 10, 2016

als - the "no hands diet"

I currently refer to how I eat as “The No Hands Diet.” 

The idea behind this is that you cannot use your hands.  At all.    Every meal, every snack and every Costco sample you eat is given to you by someone else.  If you are checking out a bowl of peanut butter m&m’s you can’t have any until someone picks one up for you.   

Hypothetically let’s say you are at a party with a fantastic spread of food, and quite suddenly, you become aware of how people around you eat and eat and eat.   The entire time you are thinking, “All I want is an m&m.”   So you ask someone for help and they kindly say, “Of course.”  Then the person licks some guacamole off of their fingers before taking a handful of those m&m’s.  THAT kills your appetite pretty darn quick.  

Moving on.

I initially got on the No Hands Diet because my arm muscles are weak, but what if a person begins to have difficulties chewing and swallowing and find themselves choking on food.  Then there is the option of a Percutaneous endoscopic gastrostomy (PEG).  This is a flexible feeding tube that goes through a person’s abdominal wall into their stomach.  Nutritional supplements and fluids are poured in the tube bypassing the need to use your mouth.

Going back four years ago this fall, I found myself trying to decide whether or not to get a PEG tube.  At the time I didn’t feel like I needed it because I could eat well enough. But, I also didn’t want to put the procedure off until it was my last resort.   At the time I could still (very slowly and with increasing difficulty) feed myself.  I also didn’t want to get one because, for me, it was admitting the disease was progressing and I wasn’t strong enough to fight it.  (I realize the flawed logic now…)

In a very uncharacteristic Janae move I was proactive and I decided to get the tube.

While it is a relatively simple outpatient procedure, having a hole cut through your stomach wall does make for a painful recovery.   

I have had the tube replaced twice (in the same spot as the original, so no new hole)  and those times the follow-up recovery was easy. 

I resented the tube for quite a while.  I hated looking down and seeing the outline of the tube under my t-shirts and would try to wear clothing that disguised its presence.  It was rarely used that first year.

Now it is not a big deal at all.  It is just part of the way things are.  There are times where I am exhausted and it is nice that Jeff can just pour a can of “food” in and just be done.  I will add that even though those 350 calories per can are handy, it often leaves me with a slightly queasy feeling.

Since I have a BlendTec blender, we occasionally blend up real food to pour in.  We have to add a lot of liquid in order for the blend to go down smoothly. There are also various recipes online.  

Coming back to today, I average ½ my calories through the tube, ¼ via a smoothie or nutrition drink and ¼ my calories come from normal food.  Those calories, in their various forms, are all part of my No Hands Diet because now there is no way I could feed myself.  I’m grateful for the many people who help me.

Last week Jeff and I went out to dinner and we ordered the soup of the day. It was a Porcini Coconut Emulsion garnished with fresh chives and a coconut crouton.  I didn’t know what to expect but it turns out this soup is what dreams are made of.   There was no chance of that going in the tube.  I savored each spoonful. 

als 101

I think I will try a series of posts about als 101. 

Things I have done that have worked for me. 

And also things that I wish I had done differently.

Then again, I may get distracted by the warm summer sunshine and my garden and only do one or two posts.