Tuesday, April 30, 2013

A Day in Parenting: Language Skills

Cora, today you referred to male genitals as a, "Tenis."

Louise, you started stringing words together today...to ask for more cookies.

Both events made me smile and laugh in equal measure

Monday, April 29, 2013

You Must be Sick

My post Reality, seems to have quadrupled the amount of Web traffic I typically see on a regular day.

I must conclude one of the following:
- My audience is senile, and must revisit the blog in order to commit a post to memory.
- People don't realize what is involved in preparing for the end of their life, and revisit the blog in order to check off the things they should do.
- People prefer my more morbid posts, instead of music videos

I looked at the possibilities, and realized -- any way you cut it -- you're sick!

No matter the reason, thanks for reading. There's nothing wrong with being sick.


I have completed an Advanced Directive; a legal document that outlines my wishes should I reach a certain, critical medical state.

I have completed my Will, documenting how all my various legal obligations should be handled.

Did I mention my expensive toilet seat?

I have had conversations about my life choices as ALS progresses. My favorite discussion sought to answer the question, "What is my ' Quality of life' threshold?"

I have modified my home to better cope with my functional needs as ALS progresses.

I fell over twice in 1 night last week.

I have ordered a comfy chair that also helps me to stand up. Yes, it's basically an ejector seat.

I'll need to find tennis balls to put on the legs of the walking frame I've ordered.

I ordered a wheelchair. A travel chair for when I feel tired on the road, and need to take a load off .

My wife has been forced to learn a lifetime's worth information on the subject of disability benefits, insurance, and the law regarding health care. In addition, she has been forced to learn every aspect of my daily routine...from how I like my food cut up, to how tight I like my socks pulled up.

Yet I feel full of love, laughter, and happiness for a ridiculous amount of the time.

I wish I could tell you that every day is free of tears and pain, but I can't...

I read a statistic that I'd never seen before: 50% of ALS patients die within 14 months of diagnosis.

My favorite nugget of information that I learned recently is, aside from 2 genetic tests, the only definitive test for ALS is conducted during an autopsy. I've heard rumors of new tests, but nothing definitive.

The toilet seat will clean my rear end for me. 

I believe I'll be happy as long as I'm able to watch my girls grow up, hear their laughter, and do something to help wipe away their tears.

Still, there are many more things that are many more times as important as planning the end of your life : Amanda must completely understand the offside rule, and comprehend aggregate scoring.

Saturday, April 20, 2013

Modifying Cellular Stress Response as a Way To Treat ALS

An MDA-supported research team has identified compounds that may prevent nerve-cell loss caused by dysregulation of a critical cell survival mechanism

Article Highlights:
  • An MDA-supported research team has identified a series of compounds that appear to work alone and in combination to protect the nerve cells that are lost in amyotrophic lateral sclerosis (ALS).
  • Worm and zebrafish models of ALS that were treated with the compounds showed less neurodegeneration and less paralysis than untreated animals.
  • The compounds all target various pathways involved in the endoplasmic reticulum (ER) stress response, a critical cell survival mechanism that works to decrease nerve cell stress

Wednesday, April 17, 2013


I'm sure there are many reasons why I shouldn't like this song...oh well https://www.youtube.com/watch?v=ekzHIouo8Q4&feature=youtube_gdata_player

Saturday, April 13, 2013

In the Eyes

Last week's visit to JHOC and their Assistive Technology Clinic was very good. There was a great selection of hardware on display. All the while, Amanda and I had to keep a simple mentality in mind; prepare for what we will need, not what we currently need.

While it was a smaller selection than I imagined, there were quite a few impressive pieces of technology for me to try at JHOC. The most simple technology was a full sized keyboard with attached small screen...type in the words you want to say, press a button, and the keyboard will say the words you entered. 

Most other technology was some sort of variant of that simple idea - a smartphone instead of a simple keyboard, a tablet instead of a smartphone.

With my hands and arms being the weakest part of my body, all of these products posed one major problem: How would I be able to interact with them when the functionality of my hands decreases further?

 The recurring mantra of making "future-proof" decisions.

 Our visit soon turned in the direction of a product that is definitely future proof; computers you control with your eyes. The product I got to test was the Eye-Gaze. The basic hardware was a 10" tablet with a camera facing the user attached to the bottom. The tablet was running on a version of Windows 7, with a complex piece of communication software layered on the top. The attached camera monitors where your eyes are looking, and a long blink serves as a click. There is a similar product on the market - Tobii - which I hope to try in the next few weeks.

 Why all the testing? Apart from the obvious urge to find the right technology for me, a retail price close to $20,000 makes it a fiscal decision - good or bad - that my family will have to live with.

Friday, April 12, 2013

Banking First Words

"Voice Banking" is a term that most people don't know. These days, you'd be forgiven for thinking it was some sort of app for a smartphone that allowed you to conduct transactions using your voice. Come to think of it, that technology probably exists.

In the realm of ALS, it refers to the act of recording yourself saying specific words. The main reason for voice banking is to give you a synthetic voice when ALS robs you of your real voice.

Here's my question: What would be the first 10 words you would record?

Tuesday, April 9, 2013


Hearing you say my name is the most wonderful sound

Monday, April 8, 2013

Assistive Technology

Amanda and I are heading up to Baltimore this evening. In the morning I will attend my first ever Assistive Technology Clinic at JHOC.

As you might suspect from the name, the AT clinic will look for ways to implement technology in my battle with ALS.

I already use various pieces of technology in my daily life; a tablet computer, a smart phone, the Internet, and a camera...at a glance.

If I recall correctly, tomorrow's appointment is a chance to take various pieces of technology for a test drive. The goal of the appointment, as with many aspects of ALS, is to determine what my needs will be in the future, and to prepare for those needs as best we can.

I'll do what I can to gather information on the devices I test, and share them on here at a later date.

Saturday, April 6, 2013

huge, Huge, HUGE

I am in a strange mood. A small army of kind people descended on our house yesterday, and undertook a large number of spring projects.

It really was awe - inspiring to see so many people show up to just help. I think that is where this strange mood comes from; without ALS, these would all be projects I would be taking care of. So it's a little bittersweet.

Then again, it's totally uplifting to live in the kind of town where a throng of people will show up on a week's notice to help out.

huge, Huge, HUGE thanks to everyone for their support!

Monday, April 1, 2013

88 and Frosty

Technically, Thursday's visit to Hopkins is a clear success.

Along with my weight, my lung capacity is the biggest indicator of my general wellbeing. Someone out there with the right credentials (or Google) could give you a detailed explanation of how they reach a numeric value for my lung capacity. All I know is that I'm given a percentage value to indicate how well - or badly - my lungs are doing.

At the beginning of the drug trial my lung capacity was 74%. A value of 50% represents the threshold at which a typical patient decides if they would like to use medical hardware to support their breathing... or not.

I believe that I've commented on the professionalism and beside manner of the staff at Johns Hopkins before. If I haven't then it's long overdue. They are amazing.

I am usually in a jovial mood when I visit the Johns Hopkins Outpatient Center (JHOC) . I can't explain why. Maybe I goof off because I'm nervous. Maybe it's because I feel the need to break the tension of what would otherwise be a potentially morbid exchange. Maybe it's just part of my nature.

I think the folks at Hopkins have become accustomed to my sense of humor. I halfheartedly guess at my blood pressure and heart rate before they are measured; usually 120 over 80, and about 80bpm.
I usually throw in one or two self deprecating jokes... add in a few jokes making fun of a simple clerical error - - or something similar - - and that just about sums up a typical visit. Even a new member of staff will oblige me with a giggle or two.

Combine the fact that Jake has become my shadow on my trips to JHOC, and together... well, Jake, always attract one or two visitors. Lora came visiting this time , and as the appointment went on it became clearer and clearer she was having a bad day. I think she used that days faculty meeting as an excuse for having an off day.

This particular visit had me performing a grip strength test before checking my lung capacity. Since my arms are easily the most affected part of my body, I have an acute sense of hatred for this particular test.

It's not that I don't try, but I certainly tune out. I tuned out so much that I almost didn't hear the score of my breathing test: 88%. I'm delighted with the score. Maybe it's because of the trial drug, or the fact that I'm trying to be better about taking my asthma medication (advair).

Either way, I am happy. But not ecstatic.
 That subtle difference has given me able food for thought, since the visit. Test scores, blood work, getting used to a new pair of shoes, the lack of strength in my arms, the sensation of my strength decreasing in my legs, my stride turning in to a shuffle... really just a long period of sensory and emotional excess. Then I suddenly had a thought about Lora; I was the reason she was having a bad day. It's certainly possible that I'm totally misreading the situation... surely the sight of me shuffling around can't be that distressing. Then again, recent changes have been particularly difficult for me to deal with.,, and

ALS & MND News

Researchers discover new clues about how amyotrophic lateral sclerosis develops
Medical Xpress
Johns Hopkins scientists say they have evidence from animal studies that a type of central nervous system cell other than motor neurons plays a fundamental role in the development of amyotrophic lateral sclerosis (ALS), a fatal degenerative disease.
See all stories on this topic »