The Odds

Two years ago...

2009-11-13T23:38:00.001-05:00

I ran the Richmond Marathon, coming in at 4:19 (my second best time). As evidenced by the gloves it must've been a fabulously chilly morning, perfect for a race. I'd learned of my dx only a few weeks before.

Tomorrow is the 2009 race. Becky is running the half (along with bandit John), as is Ricardo. Lynne, Janice, Jenna and Jannette (and doubtless some whose names begin with other letters of the alphabet) are running the full. It's supposed to be good weather, thank heaven.

I suppose there will come a time when I won't feel pangs of sadness. Until then, I will plaster a smile on my face. Or blow kisses.

ISO: Calm

2009-11-13T04:54:00.002-05:00

Sitting on a beach, drinking a fruity drink with an umbrella in it, totally alone. That is where I wish I could be right now.

It was another difficult night with my sweet girl. We seem to be going through quite a rough patch these days. I won't burden you with all the details because, honestly, it's just more of the same. It is at the point, however, where I have neither the creativity nor the energy to find effective solutions. These sessions beat me down and leave me feeling frustrated, angry, exhausted. It is up to me to be a rock; something she can hold on to as she makes her way through the troubled waters of her episodes. I don't always do such a great job and in that regard I feel as though I'm failing her.

Hopefully the meetings with the therapist will prove beneficial despite C's seeming unwillingness. Maybe she can provide some guidance for me as well.

From Justin

2009-11-10T07:00:00.001-05:00

I hope I can get images from the 3T, too. In the meantime, do enjoy these lovely photos.

One more thing

2009-11-08T16:20:00.002-05:00

The last couple of days I have noticed the middle toe of my right foot seems frozen in place. It is irritating to feel it pressed against the second toe. Unlike my left foot, I am unable to spread my toes apart on the right.

Bit by bit. Damnation.

Same again

2009-11-08T10:11:00.004-05:00

The twitching, she is très apparent these last few days. Mostly in the bicep of the left arm, but everywhere else, too. And, more annoyingly, the left hand has begun increased cramping and finger spasming. The middle and ring fingers randomly jump and curl inward, often for several moments. At least that doesn't hurt, unlike when my pinky and the heel of my left hand decide to have a tantrum.

The right hand has been fairly quiet. The only interesting development is that the index finger no longer curls, rather, it points. All the time. When Mike noticed it during some ROM exercises on the right arm, he moved my arm a la John Travolta in Saturday Night Fever.

Yeah, Mike is a funny guy.

I am concerned that the heightened activity in the left hand is a portent. I mean, I KNOW I'm weaker--it's become a struggle to open the front office door--but does this signal a ramp up in the decline? Sigh. This is a ridiculous question and train of thought. The disease is progressive, after all. There is no point in worrying about it. Better to spend my energy and thoughts preparing for further adaptation.

I was up all night with a cough, the most recent iteration of this stupid cold. Naptime.

Bleah continued

2009-11-07T22:11:00.003-05:00

I was hoping to go for a walk tomorrow morning, but I'm not sure it's going to happen. Probably won't make it to support group, either.

Another day of enforced rest won't hurt.

How far did the apple fall from this tree?

2009-11-07T15:14:00.003-05:00

While sitting at a high school waiting for Cecilia to finish her SAT test, another mother in a PT cruiser came up next to me and rolled down her window.

PT: is the SAT test finished yet?
Me: no
PT: do you know when it is supposed to be over?
Me: no, I'm just waiting.
PT: have any kids come out yet?
Me: not yet.
PT: so it's not over yet?
Me: clearly not, we are all just waiting here and there are lots of cars still in the parking lot.
PT: my son said it would take about five hours (note: at this point it was four hours and 15 minutes into the test).
Me: I don't know, I'm just waiting.
PT: (stares at me, for what seems an eternity.)
Me: just sitting here waiting...
PT: (rolls up window, drives away)

I wonder what her son will get on his SAT. Perhaps he takes after his father.

Driving Miss Crazy

2009-11-07T10:35:00.002-05:00

Making a U-turn these days requires a little planning on my part. The left arm does most of the heavy lifting, the right arm (as I've said before) is more of a placeholder.

Last night, Cecilia and I were leaving a restaurant in Central Park (which is not a park and is central to nothing) and, since Central Park is so poorly designed, I found myself having to make a U-turn in order to go home. There was quite a bit of oncoming traffic so I began my preparation: left hand/arm pulled down on the steering wheel while the right hand kept it in place. The car ahead of me went through at the first opportunity and I inched a little further ahead, turning the wheel just a little bit more. Finally I spotted an opening and took my foot off the clutch only to feel the front left tire begin to go over the curb of the median -- I had misjudged its location and had overprepared for the turn. For a brief moment I considered just driving over the curb but, not knowing how this might impact the undercarriage and realizing this would set a bad example for the new driver in the car with me, I elected to stay put. I corrected my positioning and waited for the next break. Fortunately my second attempt was successful.

This episode is in direct contrast to the U-turn I negotiated on October 7, when Adam, Ricardo, and I went to see David Sedaris. I had arrived in DC too early so I decided to park near the theater and bide my time catching up on e-mail. The spot I had chosen was easy (too easy, as it turned out) to find and I congratulated myself on being so lucky. As you can guess, I was in a no-rush-hour parking zone and it was, in fact, rush hour. The meter maid did not give me a ticket but told me I had to move, and advised that the spaces on the opposite side of the street were not rush-hour spots. The trouble was there were no spaces. As I made my way up the next block a car vacated a space on the non-rush-hour side. I knew if I circled the block this space would be gone so I, crazy fool that I am, made a snap decision and started a U-turn in the middle of the street, cars parked on either side, GW students everywhere. Remember my limitations -- this was not a smooth maneuver, I call it my 18-point U-turn. It was ugly and my arms worked hard, but I got the space. I didn't take out any students and I probably pissed off only a dozen or so drivers. The ultimate reward came after the performance when I walked the half block to my car and was already pointed in the proper direction for home.

For what it's worth, I try to avoid U-turns whenever possible. Driving, anyway.

How weird

2009-11-06T23:33:00.004-05:00

Still sick. Just thought I would give you an update.

There were three voice messages on my phone tonight. One, I knew, had to do with the delivery of my new washing machine; the other two were mysteries. People rarely leave messages because I rarely listen -- I am more inclined to return a missed call. Anyway, since a message about the delivery of my washing machine is listen-worthy, I gave my voicemail a ring.

The first message was a total shock. I have not heard from this individual for more than a year -- the last time was when I was in New York City for the 2008 Marathon. At that time, he left me a message saying he had come to New York and wanted to see me, to support me at the race, I should call him at his hotel. I did not call, I deleted the message. This guy is one of those "negative energy" relationships I purged last year. Knowing that any conversation with him would be unsettling, I pressed "7" and deleted the message.

The other two messages were unremarkable; my washer will be delivered between 130 and 330 tomorrow afternoon.

It has also been more than a year since I received a communication from my brother. I suspect he is living comfortably with all his rationalizations.

I'm tired. Time for bed.

Better living through chemistry

2009-11-06T12:28:00.004-05:00

My favorite cold medicine. Life is improving.

I did decide to work from home today, a very wise move on my part. Rolled out of bed at 7--the time I usually get to work--and was immediately grateful for the extra sleep.

At 8:00 AM it was time for my baclofen, the first of three for the day.

At 10:00 AM it was time for rilutek and lithium.

2 PM called for another baclofen. I'll have the last of these at 8 PM.

4 PM will be time for another cold tablet.

10 PM is the time for the second rilutek and lithium cocktail, along with my multivitamin and COQ 10 pill. I will not try to explain what COQ 10 does, but Justin mentioned it and said it couldn't hurt, so I added it to my regimen.

Look what I've become. One of those people who live by their pills. Pretty soon I'll have to get one of those pill organizers. I'll dye my hair blue and will take to wearing muumuus.

Bleah

2009-11-05T21:26:00.002-05:00

I am angry at every person I may have been in contact with in the last several days who exhibited even the slightest hint of illness (most notably the idiot cougher on the plane who never even PRETENDED to cover his mouth) because I. AM. SICK.

Scratchy throat, which alternates between swollen and irritated to swollen, very irritated, and painful. Runny nose. My eyes feel like someone poured dust into them. I don't even have a word to describe how my ears feel, but they are not happy. Nope, not happy at all.

An edict was issued at the office directing anyone who was sick to stay home. I do believe I will work from home tomorrow. I'll load up on echinacea and vitamin C, I'll sleep if (haha, when) the fatigue hits, and I'll not spread this vile crud any further.

I really feel like shit.

Notes to self

2009-11-05T07:39:00.001-05:00

Stop fighting it. Just buy new pants.
Gett off my fatt butt and arrange to gett the stairliftt.

Debrief: Louise

2009-11-04T23:07:00.000-05:00

This trip was great but verrry taxing. Methinks--no, meknows--there won't be any more to places that require so much walking or have stairs instead of escalators or elevators. I'm done.

I tripped going up the stairs at one of the tube stations. My right foot caught the step and I fell forward, landing on my left knee, elbow and pinky. It took several minutes to readjust, shift myself into a seated position, and (with Lynne's help) get back on my feet. Props to the kind Londoners who stopped to offer assistance.

Was this a true ALS fall? I think so. The fatigue I experienced after so much exertion (walking, sightseeing) surely impacted my ability to lift my foot sufficiently.

If I wanted to attempt another such excursion I would not be able to go without bringing a companion as the simple, everyday tasks we take for granted are difficult, even impossible: putting on and taking off a coat and other garments, using keys (including key cards, which are très difficile), not to mention shouldering a backpack.

So long independence.

Lynne was a champ and was always there to assist. She helped with everything, and I truly put our friendship to the test. As a gift to her I am going to keep out of her way for a while.

Anyway, we returned home Monday and today -- Wednesday -- I am still feeling pretty beat up. When we got off the plane my arms and legs felt so sketchy I was worried I would not be able to drive. Fortunately this was not the case, but the fatigue has stayed with me. I did not sleep well Monday or Tuesday night and tonight I am again up later than I had hoped. The only saving grace is the two hour nap I took earlier this evening.

Saturday I do not have anything at all planned. Well, yes I do: I am going to spend the day in my serenity room with my book and my iPod, and will give in to any drowsiness I feel at any time. With any luck Sunday will find me restored and rested, back on my game.

Home again.

2009-11-03T05:04:00.001-05:00

Update is a work in progress. Short version: lovely trip, despite a few glitches. Stay tuned.

Dear Louise

2009-10-26T05:25:00.001-04:00

Happy fucking anniversary.

Random thoughts for a Sunday afternoon

2009-10-25T21:25:00.000-04:00

Yesterday was the ALS walk in Richmond. The morning was unusually warm and humid and the threat of rain loomed. I arranged to meet Kendall and Lynne early for a pre-walk walk, followed by some pre-walk refreshments. Yes, ALWAYS the refreshments!

While we were enjoying our beverages, one of our local running club friends arrived on the scene with his son and his son's family. The friend, Art, was one of the contributors to my team, and his son's family (including in-laws) were there to show support as well. Coincidentally, the father-in-law had lost his mother (also a Deborah) to ALS some years before and has a sister-in-law who has it. Way too many coincidences.

The walk started at 11 AM. All my muscles were there: Jenny, Becky, Wendy and Mike, John Wallace, Jeff and Patti, and, of course, Lynne and Kendall. Other of my muscles were there in spirit, having contributed very generously to the walk. Another muscle, my half sister Tracy, had mailed hoodies embroidered with "McGee's Muscles" for us to wear. I certainly do abound in support and love, don't I?

You will notice that John and Cecilia are not listed. Unfortunately, the stress that has been plaguing my youngest daughter continues. It was best that she stayed home.

The rain that had threatened all morning decided to make intermittent appearances just before and during the walk. Everyone else pulled up their hoods, huddled in shelters, or pulled out their umbrellas, but I held my face up and let the rain fall. It was refreshing, even cleansing. It didn't last very long and before we knew it the sun was trying to peek out. When we returned there were hot dogs and cookies -- unfortunately, no more beer! That didn't matter (too much) as we were all having a very good time laughing and talking.

It was a good event. Kudos to the ALS Association for all they do for us.

****************************

The leaves are turning. On Monday last week they were just hinting at color, by Friday they were a beautiful sight to see. I had a thought as I was driving down one particularly lovely road: these trees and leaves are the most beautiful just before the leaves die. Is that irony?

****************************

Tuesday. Day after tomorrow. Air travel. Large pond. Pints. Aaaahhhhh.

****************************

I walked this morning. It was cold and bright and breezy and fresh. And for the first time in seeming AGES, I ran. Just a little. Breathing wasn't too bad, but I don't have the wind I once had. Tonight my legs are a little tired, but I wouldn't change a thing.

On the negative side, I had a very unpleasant experience in the loo.

For some time I have had increasing difficulty in "pulling down" which is even more problematic when the situation becomes "urgent." I no longer wear certain pants for fear of having an accident. When walking today, I wore some of my loosest cropped pants.

About halfway through my third lap I felt it start. I knew if I was walking I'd be okay until I could get to the restroom. The closer I got, the worse it got. I made it to the stall but was having no luck getting my hands into the waistband. The more I struggled, the worse it became. I finally managed to start "pulling down" but was too late. I couldn't help it.

Don't misunderstand--it wasn't a complete drenching, but it was enough. I made my way back to my car, found something to sit on, and came home. Embarrassed, angry, and sad.

Thank goodness I'd had a little bit of good runnin' or it would have been a miserable morning.

****************************

Today was also the Marine Corps Marathon. Enough said about THAT topic, but it's still hovering around in my messed up little brain.

****************************

Two and a half hours until Monday. The 26th.

Goodnight.

Another article

2009-10-23T08:04:00.003-04:00

Approved Enzyme Slows ALS in Mice

October 22, 2009

A chemical cousin of a drug used to treat sepsis dramatically slows the progression of amyotrophic lateral sclerosis, or Lou Gehrig's disease, in mice.
The results offer a bit of good news in efforts to develop a therapy to stop or slow the progression of a disease that generally kills its victims within just a few years.

In a paper published in the Journal of Clinical Investigation, scientists studied the use of an enzyme known as activated protein C (APC) to slow the cell death that occurs in ALS.

"We're able to significantly extend the lifespan of mice who have an aggressive form of ALS," says co-principal investigator Don Cleveland. "The compound also extended the length of time that the mice were able to function well despite showing some symptoms of the disease, and it reduced the pace of muscle wasting that is a hallmark of ALS."

While the investigators say that more research must be done before the enzyme is tested in people with the disease, they're encouraged that the work involves a compound that's already been proven to be safe and currently given to patients via a common injection for another condition. The team hopes to test a treatment in patients within five years.

The work was done by researchers from the Univ. of Rochester Medical Center, The Scripps Research Institute, the Univ. of Notre Dame, and a Rochester-based start-up biotech company, Socratech. First authors of the paper were Zhihui Zhong at Rochester and post-doc associate Hristelina Ilieva at UC San Diego.

The researchers studied mice with a mutation in a gene called superoxide dismutase 1 (SOD1), which plays an important role in keeping cells safe from damaging molecules known as free radicals. While the cause of most cases of ALS is unknown, scientists know that SOD1 plays a role in approximately 3% to 4% of cases-–providing an opportunity to study the disease's initial steps, which occur long before key nerve cells appear sick or die. In addition, recent studies have suggested that the accumulation of mutant forms of SOD1 is linked to most cases of sporadic ALS.

Cell death is central to the symptoms of ALS, a chronic disorder of motor neurons in the brain, brainstem and spinal cord, which results in a progressive paralysis that generally kills individuals within five years of onset. Currently there is no cure or even a treatment that can effectively slow disease progression.

In a surprising finding last year, a team led by Cleveland found that SOD1 mutations weaken the crucial natural barrier between blood and the spinal cord. In effect, blood vessels in the spinal cord become leaky, allowing toxic substances to flood into the spinal cord. Because of the defect, motor neurons are exposed directly to biochemical byproducts of hemoglobin such as iron, which forms reactive oxygen molecules that injure or kill neurons.

Now, the team has shown that APC dramatically lessens the activity of the SOD1 mutation. This protects neurons that are under assault by blocking the synthesis of aberrant forms of the molecule in motor neurons and other cells in the spinal cord. These include microglia cells, which the Cleveland laboratory has shown play a key role in the inflammatory response and progression of ALS. In addition to reduced SOD1 activity, the flow of dangerous byproducts of hemoglobin into the spinal cord was eliminated by APC, saving neurons.

Currently, the group is studying alternate forms of APC, in an effort to create the form that best quells the symptoms of ALS while causing fewer unwanted side effects, such as bleeding. The researchers say the form of APC currently used to treat sepsis carries an increased risk of bleeding and likely will not be appropriate for treating ALS in humans.

While other researchers are exploring the possibility of silencing SOD1 to treat ALS, Cleveland noted that most approaches would require invasive surgery and delivery by direct infusion into the spinal cord. APC, in contrast, is already approved as an injection.

Source: Univ. of Rochester Medical Center

In the news...

2009-10-21T11:57:00.001-04:00

Sepsis treatment 'may slow Lou Gehrig's disease'

(AFP) – 1 day ago

WASHINGTON — An enzyme similar to that used to treat serious sepsis could be used to slow the progress of the motor neuron disorder known as Lou Gehrig's disease, according to new medical research.

A study, released late Monday, found that mice suffering from the disease experienced slower cell death when treated with the enzyme, called activated Protein C or APC.

The protein also extended by about 25 percent the life of mice suffering from a particularly aggressive form of the disease, which is formally known as amyotrophic lateral sclerosis, according to the study.

The report, which was published in the online edition of the Journal of Clinical Investigation, was produced by researchers from a group of US universities and a biotech startup company in New York state.

Among other promising developments, the study found the compound extended the amount of time that mice suffering from the disease were able to function normally, despite showing some symptoms of the disorder, which is incurable and almost always fatal.

The protein also slowed muscle wasting associated with the illness, which is named after the star US baseball player Lou Gehrig, who died in 1941 after suffering from the disease.

Though additional research will be needed before the treatment can be used in humans, the researchers said they were encouraged that the breakthrough involves a compound that is already used to treat patients with sepsis and has been proven safe.

They hope clinical trials on patients could begin within five years.

"The success of this research project has been very gratifying, and we are hopeful that a form of APC will ultimately be useful as a treatment for this disease," said Berislav Zlokovic, a professor of Neurosurgery and Neurology at the University of Rochester.

The researchers found that APC may also be useful in mitigating some of the secondary effects of the illness, including a mutation that weakens barriers between blood and the spinal cord, allowing toxic substances to enter the spinal cord.

Cheers

2009-10-20T20:38:00.003-04:00

Thank goodness I have a three mile walk on Saturday, one that I've been training for EVER so long, or I might just fall back into those awful doldrums that recently had hold of me.

And after the walk will be the family version of Oktoberfest -- where we celebrate all the birthdays that occur in October. That will be a nice diversion.

And, one week from today, I will be on an airplane headed to a distant land where I will consume many pints with my buddy, Lynne.

So it seems I am sufficiently occupied, yes? Yes. For the most part. There is one more thing I plan to do, sort of a ritualistic cleansing of both my spirit and my closet. I have a bin in my closet that contains a lot of running gear, running gear I no longer wear. The odd pair of shorts -- once considered much too large -- may be kept, as well as a few nice, loose, long sleeved technicals. Beyond that it's off to Goodwill. There is no point in keeping these things around.

The same holds true for some other random items. If I am unable to manage them, it is time to go. One might think this is a trifle depressing but I view it in a very positive light: I am transitioning.

When I'm finished I will raise a glass full of the pint-y stuff, thanking all that is being discarded for their years of faithful service. It will be a fine tribute.

Shoes

2009-10-18T21:17:00.002-04:00

Yay! The shoe video has been located. It's nothing special, just further evidence that a zipper pull has many uses.

Dim Sum

2009-10-18T17:41:00.003-04:00

This morning I met friends for brunch at a great dim sum place in Rosslyn. The idea was put forward by my neurologist at NIH (Justin, next to me on the right) several months ago and we finally got around to making it a reality. Joining us were Adam (blurry, on the left), Kay (my OT/PT from GW), and Ricardo (in the center, not that I had to tell you that). The food was fabulous, the company was even better.

Kay, who is Pakistani, is going to host the next get-together at her home where we will eat authentic Pakistani fare. She promises it will be spicy, my idea of perfection.

It was a wonderful way to spend a Sunday morning, with wonderful friends. I am very much aware how fortunate I am.

Prepare to be amazed

2009-10-18T17:40:00.002-04:00

So here it is, what you have all been waiting for: a demonstration of how I use the zipper pull to put on my socks. I apologize for the poor sound quality and for the amateurish feet, but I think you get a good sense of how this works. On the second foot the pull got stuck so it was not as fluid a demonstration as I would have liked, but it did give you an opportunity to see how I then pulled up the sock by my heel.

There was also a video demonstrating how I put on my shoes -- it seems to have gone missing in cyberspace. I will try to re-create at some point in the future. Until then, please enjoy the sock demonstration.

Such a lovely morning

2009-10-17T08:36:00.003-04:00

A little more than two years ago I was recovering from the hell that was the Chicago Marathon. Lynne, Adam, Jeff, and I had trained through the heat of summer and were eagerly anticipating the blustery cold of October in Chicago. I don't remember exactly when I started keeping track of the weather but, knowing how obsessive I am about these things, I wouldn't be surprised if it was four weeks in advance of the race. Things weren't looking good; long-range forecasts predicted warm temperatures. There may have been one day which gave a glimmer of hope -- I believe I read it was going to be in the 50s on race day. I must have been on drugs that day, because I never read another forecast like it again.

It was warm on the Friday we flew from Washington into O'Hare, it was warm on the Saturday we busied ourselves around the city of Chicago. We were all fairly resigned to our fate and decided to make the best of it the next day. Sunday morning we awoke to temperatures in the 70s, ungodly weather in which to start a 26.2 mile race. In defiance of my own rule never to run a marathon if the temperature was more than 50°, I took my place among the thousands of other stupid people.

I don't remember all the specifics but I do remember it was painful. At mile 10 I thought I was going to die; at the half I got sick and made the decision to quit. Having been instructed by Adam to carry my cell phone, I called him and discovered that both he and Jeff had called it quits as well. We made our way back to the hotel and waited for Lynne and Adam's sister, Anne. Those two fools had decided to stay in the race, despite the now 92° temperature AND despite the fact that the race had been canceled due to the intense heat. I am still amazed and impressed that these two pulled it off.

That was my last race pre-diagnosis. I ran in Richmond in mid-November and did a great time. For me.

What got me thinking about this? Today is cold and rainy, almost perfect marathon weather. As I poured my coffee I started to think about what sort of running gear I would wear on a day like this: shorts (naturally), a long-sleeved technical shirt (over a tank, as the long sleeves would come off around mile 3), a ball cap, mittens, and my leopard print ear bags. Instead, I am wearing my jammies, drinking coffee, writing this, waiting for Cecilia (who is taking her PSAT). Being out in the rain isn't much fun if you aren't running, especially in these temps. Anyway, as I was thinking about what I would be wearing today, I remembered what I was doing this time last year, and I started thinking about what I was doing two years ago. Chicago was a race I had hoped to run a second time in cooler weather. Oh well. Maybe I will go back to Chicago one day just for the hell of it.

Today I start taking baclofen. Any of my fellow ALS-ers have any personal insights into this drug?

I pick up C Claire in an hour. I may just have time for a quick nap.

I wish I could...

2009-10-16T11:02:00.004-04:00

clean my glasses.
open my lipstick.
butter bread.
pick up coins.
hold a q-tip.

More to come.

All I want to do

2009-10-15T07:45:00.001-04:00

Is go back to bed. I love my job, but it's cold and gray and rainy and my pillow felt SO good this morning...

I didn't make things any better when I ordered this:

Hot chocolate is a sleepy time drink.

When I was in line at Starbucks I had every intention of ordering an Americano but when my eyes saw the words "hot" and "chocolate" I was powerless to resist. Those two words leapt out of my mouth before I had any idea what was happening. Do I regret it? Not one iota.

I don't regret the whipped cream, either.

Okay, enough of this. Time for coffee. Unless anyone has a nice pillow they'd like to give up...