New Blog site

Due to ongoing problems with people unable to post comments, I have moved my blog to Wordpress.  Here's the site:

www.a-life-beyond-measure.com

The Good, The Bad, and The Ugly

I’ll start The Ugly

THE UGLY (what I don't like)

- Falling down (it hurts!)

- Ongoing grief (i.e.; I am constantly losing my abilities, so there's always something new to grieve the loss of)

- Struggling to turn over in bed (this is strangely hard and I can see the day when I won't be able to do this by myself)

- Choking on food or water which can cause me to have laryngospasm (When this happens to me, I know I’m fine but the people around me don’t. I just need to relax my vocal cords – which takes me perhaps 5-20 seconds before I’m back to normal - The only YouTube video I could find with an example is this one of an infant – listen to the first 20 seconds).

-  My left-hand “claw” (my hand has lost almost all ability to do any fine motor actions)

- Using forearm crutches to walk

- Walking really, really, really slowly

-  Using a walker in the mornings as I have so much trouble with balance in the early AM

-  Getting fitted for an electric wheelchair….and getting a loaner from the ALS Association Loaner Closet

- Dropping things

- Getting up out of a low chair (or even worse, a low toilet without handrails)

- Being so disabled that people hold doors open for me

-  Using a button hook to dress

- Having to wear an AFO on my left foot due to foot drop

- Listening to people telling me about their aches, pains, or minor health problems (do they really think that's the same thing I'm going through? I know they don't but it's weird when people do this)

THE BAD (what I miss)

- Running

- Walking

- Hiking

- Biking (on a normal 2 wheeled bike)
- My nightly beer (3 times I've fallen after having some beer - twice after one beer and another time after a half of a beer!  So I've sworn off beer until I'm in a wheelchair - NOT something I look forward to)

- Fixing things

- Working with tools in my shop

- Giving up my dream to fix up/rehab our 1966 27’ Airstream, and having to sell it instead

- Traveling in our Airstream trailer (we just finished a one month trip to northern California – which I fear will be our last)

- Talking normally

- Typing with six fingers (I'm down to four - three on my right hand and only the index finger of my left)

- Oh, and did I mention running?

THE GOOD (what I like – not many things but there are a few)

- Strangely Susannah, Lionel and I are closer than we have ever been and Susannah and I are in some ways, more in love then ever

- Family and friends

-  Riding my cool 3-wheeled recumbent HP Velotechnik Gekko fx bike with a Bionx electric torque-assist motor

- Having people hold open doors for me

-  Eating anything and everything I want (they want me to gain weight!)

- Great parking spaces

- Getting to ride those motorized disability carts at Safeway or Home Depot.  Unfortunately they have a sign on them that they can't be taken out of the store.  I figure I'll break that rule someday when I find another gimp who's game for a race in the parking lot

- Oh, and orgasms.  Because of the hyperreflexia that’s seen in ALS orgasms are kind of a full body experience.  Enough said.

ALS Clinic in March…. (Part 2)

So back in early March I went to my first ALS Clinic.  At that time the only thing going on with any impact on me was some left foot clumsiness.  This meant I walked more slowly and by this time I had stopped running.

When I first went to the clinic they had me fill out the ALS Functional Rating Scale-Revised which can be used to predict survival time.  Here’s a copy of my ALSFRSR at that time:

And here’s my score plotted on a survival curve:

The first clinician I met at the ALS Clinic in March with was the Respiratory Therapist.  He performed Pulmonary Function Tests (PFTs) on me.  Interestingly in all my years attending in the hospital I never had a PFT test, although I had seen a number of them done.  To make a long story short – the Forced Vital Capacity (FVC) is the most important test.  This test is done by breathing into a tube – taking the biggest breath you can, the exhaling as much and as long as you can.  It sounds easy but it’s remarkably hard.  It took me 4-5 tries before we got a good result. 

The reason why the FVC is important is that people with ALS die from respiratory failure because the muscle that help you breath stop working.  ALS has very few therapies which extend your life.  There are currently really only two – a drug called Riluzole (Rilutek) which extends life by an average of 2 months, and using BIPAP at night (it’s similar to CPAP).  BIPAP extends life by 18 months on average.  When your FVC gets to about 50% of predicted, they put you on BIPAP.

Well the good news was that my FVC was 110% of normal – possibly due to my running.

Other things that were covered in the clinic included the natural course of the disease, the need for possible Voice Banking, ordering a cane as well as an AFO, and started me on the medication Riluzole. 

At end of the visit they scheduled to return in three months to check how thing were progressing – that follow up clinic visit occurred earlier this week.  I’ll post an update soon about my most recent ALS Clinic visit. 

ALS Clinic in March…. (Part 1)

First – an apology.  I noticed that I wasn’t getting many comments on my blog, while on Facebook I’ve had lots.  I finally realized that I had not disable the CAPTCHA which makes you enter a ridiculous set of weird looking characters before you can post a comment.  I hate this.  So I disabled it.  It should be much easier to post comments now (although it won't post right away as I'll review them before posting - it cuts down spam).

Now about the ALS Clinic at Kaiser Permanente. 

In early March about 10 days after I got my diagnosis of ALS I attended the ALS Clinic at Kaiser.  Needless to say I was still in a bit of shock, not really knowing anything about ALS.  So I went to the clinic in a bit of a daze.  Wow.  What an amazing experience – in one three hour appointment I met with the Neurologist, Speech Therapist, Occupational Therapist, Physical Therapist, Respiratory Therapist and the Social Worker from the ALS Association.  Talk about efficiency!  That I could meet all of these folks in one place, in one afternoon sure made my life easier.

In fact, in April I made a film about it, which is on YouTube.  I’d like to set some context about the film – at the time of my diagnosis I was working with the Kaiser Permanente marketing department as the physician liaison with the employer groups that have Kaiser insurance (see About me).  This meant that I often worked with a number of Healthcare Insurance Brokers who advise the companies on their healthcare options.  I was scheduled to go to a broker conference in May – which clearly I was not going to attend.  So I made this video for two reasons; 1) to let people who I know well why I all of a sudden disappeared, and 2) I was truly amazed at the ALS clinic and the value it brought to Kaiser members like me. 

So please realize that the audience for my video was the brokers who potentially sell our health insurance.

Here’s the video: Broker Conference Video (word of warning, the video is about 5 minutes long).

In my next post I’ll outline what I learned at the ALS Clinic last March. 

Then after these two posts, I’ll post information about my 2nd visit, which was earlier this week.

More on Voice Banking

I mentioned Voice Banking in my last post – here’s a few more details.  Almost everyone with ALS eventually lose their voice – it can be one of the first signs in ALS called Bulbar Symptoms.  I find Bulbar Symptoms perhaps one of the most distressing aspects of the disease – but what is is.  And I don’t get to vote.

When I first heard about losing my voice I didn’t think it was such a big deal. I figured I’d just pick a voice out there; how about Steve Hawkins’ voice?  Or better yet – Darth Vader to scar the dogs!  However about a month ago Susannah mentioned that she wanted to hear my voice – not someone else’s.  So I started investigating Voice Banking. 

So I’ve started using an on-line program called ModelTalker– which is a free service for those losing their voice.  It’s funded through grants and donations – a truly remarkable organization.

There are a couple of things that you have to do before starting the process – first you have to get a very good microphone & headset.  I bought one they recommended from Amazon for about $45 – the Sennheiser PC 36 Call Control USB Binaural Headset. 

Next you have to find a silent location to record in.  When I started we were traveling in our Airstream trailer – which when all windows are closed is amazingly quiet.  So that was been my recording studio; now it’s my home office.

ModelTalker’s process is for you to record your voice using prescribed sentences.  They show you a sentence then the reader reads the sentence and you try to repeat the sentence as closely as possible.  They then rate how you repeated based upon amplitude, pacing and pronunciation.  The goal is to record 1600 sentences! 

Eventually ModelTalker will use the 1600 sentences to generate your own synthetic voice.  The sentences are from “The Wizard of Oz”, “The Call of the Wild” and other classics. 

Ironically long before my ALS I have frequently joked that the only foreign language I know is English, and I don’t even speak that very well (as an aside, I was born and raised in the U.S.).  At one point in the recording process I was having trouble pronouncing a word.  After a number of tries, I finally got it correct - I mentioned to Susannah that I’ve had trouble with pronunciation – she quipped “What’s new about that?”

It takes me about 45 minutes to record 100 sentences.  You can listen to your sentence recording and re-do it if needed.  You can also hear a sample of a synthetic voice based on the number of samples already recorded.  I’m currently about half way done with the process.  Here are 2 examples – the first is my current voice:

 While the second is a sample of the synthetic voice:

My voice has been changing – I am quieter then normal, my speech rate has slowed down, I’m having trouble with some pronunciation and it gets harder to speak as the day wears on.  This morning for the first time I noticed I’m getting tongue fasciculations. So there’s some urgency to my recordings now. 

Emotions, Voice Banking and Laughter

Part of ALS is that my body reacts without my being able to stop it.  If there’s a loud noise, I’ll startle/jump to the extreme.   Our bodies are very good at suppressing the startle reaction – mine isn’t any longer. 

One thing very strange about ALS is that emotions become magnified.  It’s almost like there’s a mind-body connection in that just as my physical reactions are magnified, so are my emotional responses.  I laugh a lot and at weird times and situations (I also get teary and cry more easily, but that’s another story).  My laughter doesn’t come out as just a giggle – this is a full-body-run-out-of-breath-gasp-for-air laugh!  It’s kind of infectious.  Things just strike me as funny and I can’t stop myself.  Word of warning – don’t say anything funny while I’m eating or I’ll either spit what ever I have in my mouth across the table, or aspirate it.  At which point you may have to do the heimlich maneuver on me ;-)

So a few days ago I was talking with Susannah about the “Voice Banking” I’m doing and she asked me “how does voice banking work – that is do you ‘bank’ specific phrases or what?”  

I told her “there are two types of voice banking – one is that you ‘bank’ certain phrases so that later when you’ve lost your voice you can ‘select’ phrases from a list on your computer/device.  The other way is that you record a series of sentences which are used to create an synthetic voice which sounds like your voice.  I’m working on creating a synthetic voice that sounds like me.  Then as I type in words or phrases, the synthetic voice will say whatever I’ve typed.”

I continued, “for example if was doing the first version of voice banking mentioned above, I could pre-record and store the phrase ‘WTF’ (I’ll let you fill in the initials) then when I want to say that I would select it off a list.  In my case I’m working on a synthetic voice.  So that means I’ll have to type in what I want to say; that is the synthetic voice depends on my typing things in – and since I’m a poor typist it would come out as ‘What’…..(I paused for 5 seconds)…. ‘the’ …….. (I again pause for 5 seconds) … “  At this point I never got to the last word because I was laughing so hard – which got Susannah going as well!