THE ODDS (Debbie Does ALS)


Friday PM

I owe ten million emails, I should look at my bills, I should put away laundry, but I'm SO tired I'm going to just take my pills and go. to. bed.

If any dear readers are those to whom I owe a missive, have heart. My archaic and stilted diction will grace your mailbox soon, and then won't you be sorry.

(NB. Whilst composing this little entry, I fell asleep, sitting straight up, twice.)

Sweet repose and rest, and all that...


GSK1223249, via PLM

The drug GSK1223249 is being developed by GlaxoSmithKline to treat symptoms in patients with Amyotrophic Lateral Sclerosis (ALS).

The drug works by inhibiting the protein that prevents nerve growth (Nogo-A).

This is the first-time-in-human (FTIH) phase I/IIa study of GSK1223249, a humanised monoclonal antibody against Nogo-A, a neurite outgrowth inhibitor hypothesised to be involved in the pathophysiology of amyotrophic lateral sclerosis (ALS) and some other neurodegenerative disorders. This study will be a randomized, placebo-controlled, double-blind, sequential dose escalation, 2-part fusion protocol. Approximately 76 patients with ALS will be enrolled. In Part 1, single escalating intravenous (i.v.) doses of GSK1223249 are planned to be evaluated in 5 sequential patient cohorts (2 placebo and 6 active in each cohort) to determine single dose safety and pharmacokinetics (PK). Part 2 will also be of a sequential dose escalating design, but patients in each of the planned 3 cohorts (3 placebo, 9 active in each cohort) will receive 2 repeat i.v. doses approximately 4 weeks apart where, safety and PK will also be evaluated. In two cohorts in Part 1 and all cohorts in Part 2, blood samples and skeletal muscle biopsies will be taken from patients before and at the end of treatment to demonstrate whether or not GSK1223249 binds to its target and produces any measurable pharmacodynamic effect.

Refer link


And the next night...

Home. Late, but earlier than last night. Living in a state of being pooped, but tomorrow's visit with friends should end early.

Despite my fatigue, I wish tonight could have gone longer. Dinner with two of my very favorite people, and good wine, good conversation, good food. If I had the energy, I'd be more descriptive. Perhaps later.

Sweet dreams.

Finally home

Long day, I'm ready to drop. Would do it all over again.




I am so tired.

Up at 5:15, out the door at 6. Work by 7, hustled like a mad fool until my 10AM meeting. Left at 12 for home to pick up Tracy and Jordyn, then off to Mt. Vernon. Great visit, but hot and LOTS of walking. Worth every step, though, to see how much Tracy enjoyed it. Left there at 6PM for Wendy's, got there before 7. Left THERE at 7:15 and was home by 8:40. Grabbed C Claire and headed to the gas station, then the ever-nutritious McD's for C's dinner. In the door at 9.

My back is super-sore, my arms are tired, and I drove over 200 miles today.

Tomorrow I take C and a friend to a concert in DC and likely won't be home until late.

Wednesday and Thursday I have other after work commitments.

Don't I realize I have ALS?


A new chapter

Update: there have been no reoccurrences of the soft palate issue. The Claritin may have helped; I'm not sure, it doesn't matter. I think I panicked unnecessarily because these days everything is suspect. Let us now move on.

My half-sister, Tracy, has come for a visit. Though we have known about each other for some time, this is the first time we've ever met. Wendy, Tracy, and I have been enjoying a very comfortable "getting to know you" weekend. Yesterday we visited Washington DC (Tracy's first visit to Washington) and got to see the White House, the Lincoln Memorial, the house where Lincoln died, and took a trolley tour to other points of interest. We made a quick stop at the American history Museum. It was interesting for Wendy and me to play tourists; we are so accustomed to these sights as we have grown up with them and have worked around them most of our lives. It was fun to see the nation's capital through the eyes of someone who has never visited. The trolley tour was Wendy's idea, and really made a huge difference.

Through a pure stroke of luck we got to witness a presidential motorcade. Having worked near the White House for so many years, I find them irritating and inconvenient. Tracy and her daughter, however, were tickled to witness what Washingtonians consider a big pain in the butt and impediment to already congested traffic!

This morning before everyone got up, I headed out to the battlefield for a 6.2 mile wog. It felt great -- despite the humidity -- and I expect this great feeling to last the entire day.

Today we will visit George Washington's boyhood home at Ferry Farm and perhaps a few other historic homes in Fredericksburg. I'm looking forward to it because I've only been to one in the 20 years I've lived here. I should be ashamed of myself!

If anyone thinks to bring a camera, perhaps we will have pictures. I'm not optimistic.


So, do I snore?

Well. I am informed by one who knows that I often sleep with my mouth wide open, lessening the significance of my earlier observation. Apparently I have been seen thusly by my dear sister. This is a relief as it reduces the drama queen pressure I am under.

Thank you, Wendy, for setting me straight and giving me a good laugh. The memory of Mom that you conjured up was sweet and amusing.

Next time I will wear my glasses. :-)


It's 11:37PM

This morning, during my stretches, the thing happened again. It freaks me out.

I recall waking up in the past weeks with a dry, open mouth. Maybe so I could breathe?

I've taken a Claritin D, on the chance my breathing issue is cold or sinus related.

I'm a little afraid to go to sleep. Maybe more than a little.


One thing and maybe another

Okay. Today was much better from an arm and hand perspective. I stretched quite a bit before falling asleep last night and as I woke up this morning. The drive to work has minimal turns and my arms and hands felt pretty much normal all day. The ride home was likewise uneventful and not fatiguing.

I want to record something today just in case it ends up being significant: the past three or so mornings I have noticed an unusual sensation when I exhale through my nose while lying flat on my back. It is as though a flap is closing, making it difficult to exhale. I can exhale through my mouth, I can exhale through my nose if I am on my side, sitting, standing, just so long as I am not flat on my back. From what I have read online, this seems to be something also experienced by other PALS. Apparently it is a weakening of the soft palate. There are devices available that can help, but finding one that is comfortable is a challenge.

Aren't these hideous looking? I googled "palatal lift" and this is what I found. The one on top looks fairly obscene, I can't imagine anyone even putting that in their mouth, no matter how lazy the palate! I think I might actually have seen something very similar to these in The Clink and the Kriminalmuseum.

It may be better just to sleep on my side.



My arms are really tired, and my hands seem very weak today. I'm finding it very difficult to hold a book in my hands, and to turn the pages. It's a little bit easier if the book is on the table.

I had several activity options open to me today but chose to stay home. Everything involved driving and I just wasn't in the mood to deal with my steering wheel.

I think about people who have lost their arms completely and I marvel at their ability to work around their loss. I have to confess I'm feeling a little frightened and overwhelmed today -- am I strong enough and resourceful enough to face what's coming? I think I am, but I'm not 100% sure.

Holy cow, this sounds like a potential pity party. There will be none of that! Goodbye!



In an attempt to be proactive and "avoid the near occasion of falling," I have added a shower chair to my bathroom. I'm still getting used to it; the getting in and out part needs to be refined, but the sitting part is working very nicely. AND an added bonus is the chair back, over which I put a towel, soap it up, and then wriggle against it with my back! No more having to bother Miss C Claire with a task I know she hates. Touching me is bad enough, washing my back takes an incredible amount of fortitude on her part. I would try to be sensitive and cover everything but the area needing the scrub, sort of like I was preparing the area for surgery. Hopefully it will be a long while before we have to go THERE again.

Washing my hair is a new, happy experience as I am able to support my elbows on my legs as I bend my head over--eliminating the need to raise my arms over my head. My new bath gloves are VERY effective (once I get them on, which, admittedly, takes some time). Even with the gloves, however, I am having a harder time washing my neck because I can't get my hands and arms up that high in that position. Where the gloves come in handy is when I soap up the back of my hand--it gives me a little better flexibility.

Another change I'm loving is the new lever-style shower faucet handles. We've had knobs up until now and they were becoming impossible to turn. No more--now turning the water on is easy peasy lemon squeezy.


Fork required

Lunchtimes are becoming interesting, particularly when I eat a meal by hand. Wraps, sandwiches, tacos, burgers--all have become challenging and messy as hell. Not only is it uber difficult to hold anything securely in my hands at all; when I take a bite; the tiny bit of stability that DOES exist disappears and, more often than not, the sandwich threatens to fall apart. Burgers are the messiest of all, especially the good, juicy ones. Hot dogs, too--I have to readjust as I make my way down the bun.

Many napkins are used during the noon hour.


A follow up from Hopkins!

Hot diggity! Squeaky wheels, and all that...

---------- Forwarded message ----------
From: Elizabeth Mosmiller
Date: Thu, Jul 16, 2009 at 10:58 AM
Subject: RE: potential participant

Deb – just wanted to touch base before I go on vacation – we're working on getting you up here and enrolled in the study. Have to iron out a few billing/contract/budget issues, but at this point, it looks like we'll be able to enroll you. I'll get back to you as soon as it's sorted out. Have a great weekend.



Lou Gehrig's co-worker's grandson

I went for a nice walk tonight -- 3.4 miles. The weather this July is unusually temperate and walking is a joy.

When I left the YMCA I remembered I had to get gas. Cecilia was not with me, so I called Jenny. She was already at her house, too far for her to come help me. I decided to rely on the kindness of strangers at the gas station.

I pulled up next to a pump and opened the little gas cap door, then I began my search. After a short moment a man came out of the Wawa and headed toward his car. I pounced. He very kindly agreed to pump the gas for me. He told me that, earlier in the day, someone had lent him some assistance, and this was his opportunity to pay it forward. I explained that ordinarily I had help, but not this evening. I also told him I had Lou Gehrig's disease, which he knew about. As it turns out, his grandfather used to work with Lou Gehrig at the New York City parole office, where he went to work after leaving the Yankees. This man, Tom Brodkin, always thought it was cool his grandfather had had this connection.

There was a reason I went to the gas station by myself tonight.



I was guilty Sunday of only listening with half an ear, so it is only fair I should be paid back with my own coin. I noticed it several times today. I am less bothered by today's occurrences than I am by the realization that I was the half-eared one.

Tomorrow's goal: listen.

What I use to eat pudding

...which is packaged by our local deli in tall parfaitish cups. A small plastic spoon is not adequate, so I dug around in the breakroom and found this. My own personal pudding shovel.Yum.

I just noticed the image is reversed. Oh well, you get the idea.


Cái gì làm tình

Today I got a manicure and pedicure.

One of the technicians came over and was talking to the man who was doing my manicure. I don't understand Vietnamese at all but I could tell by the way she looked that she was very interested in my right hand. He responded to her inquiry (?) by turning my hand over. At this point I chimed in (ha ha, in English) and asked what was up. My technician said they were wondering why my hand looked the way it did. I told them the muscles were gone which satisfied them and the manicure proceeded.

Today's moment, brought to you by Louise the Disease.


The newbie

I was contacted today by a man newly diagnosed with ALS. He reached out to me because my profile indicates I am a runner; he, too, was an athlete.

I've seen his posts on the PLM website. He's searching for answers -- how, why, when -- just like we all do those first hours, days, weeks, and months after we are diagnosed.

He asked me about my running, whether I had given it up, was I worried about my muscles. He asked me about my diet, about my medications, about any studies I might have participated in. He asked me how I clipped my nails. He asked if I could swallow, or if my tongue quivered.

I answered him as best as I could, occasionally injecting as many positive aphorisms as I thought he could handle. During these early days, though, one doesn't necessarily want to hear Pollyanna-isms -- there's too much fear, anger, more fear, and sadness. It takes a while to understand that worry won't help; each day must be lived as best as can be with the hope you'll get another chance tomorrow.

Bless his heart.

Brother, can you spare a spinal?

Remember this?

I called on Wednesday, got voicemail, left a message. I did not hear back Wednesday or Thursday so, as you might expect, I called this morning. Betsy Mosmiller answered the phone on the second ring. She thanked me for calling and advised that Johns Hopkins was unable to enroll ALS patients because of cerebral spinal fluid (CSF) limitations. As it happens, the Biomarker Study people were going to pay $200 for each lumbar tap; $100 to Hopkins and $100 to the patient. But Hopkins charges $300 for this procedure, so there is a $200 deficit. I asked if Hopkins wouldn't be willing to waive the fee. I was told no. I said I would be willing to give up the $100 stipend. Still no. I understand that Betsy is not making these decisions, but I expressed my frustration and disappointment to her anyway. I explained that I have decent health insurance that might cover this procedure and asked if that would be acceptable. In my mind, it's crap like this nickel and dime mentality that are the real stumbling blocks to finding any sort of solution to ALS. Betsy said she would relay all of my comments and concerns to the organizers. Perhaps there is a way I can still contribute the plasma/DNA.

I wonder how much money was spent discovering a cure for erectile dysfunction.

Betsy did throw me a bit of a bone: they are doing skin cell studies at Hopkins. I offered to provide some of mine. She said the procedure is very simple -- very similar to having a mole removed -- but that there would be a little bit of scarring. I laughed and told her scarring was the least of my concerns.



Good Thursday morning

I had a great visit with Dr. Mike this morning. He was impressed with my shoulder's range of motion, and commented on the improvement in my left hip. Yay!

Mike has been my physical therapist long enough that I know something about his family. His grandmother, of whom he is very fond, had gone through a rough spot about six or eight months ago. Mike bought her a "life alert" system, which has been very helpful. He mentioned that in a recent conversation with her, though, she sounded lonely. He thinks the "life alert" system may be working too well. The system permits his parents -- who have the lion's share of responsibility for grandma -- to travel a bit more than they've been able to the last five or 10 years. Prior to having the system, Mike's parents stayed closer to home. The new system has been a bright spot for them as they have been able to escape a little, start living their lives again...

I remember all too vividly how it was when my mother-in-law lived with us.

Naturally, this made me think about my long-term future. As I think about my deterioration I realize that I have (in one respect, anyway) the easy part of this; those who care for me will have to do all the heavy lifting. While it will be done with love, a burden is a burden. I have much to think about on this score. I don't want anyone putting their lives on hold.


This evening I was able to get out and do 6.6 miles at Pratt Park. I walked all but the last bit; the slow run at the very end was all things wonderful. During the long walk I hit upon something. When people die, those who are left behind are the ones who feel the pain, who grieve for the loss. I think the hardest part for an ALS patient is that they are the ones left behind as each little bit of them dies. I know that every time I am faced with one more thing my hands, arms, and legs cannot do I grieve anew. It is very often difficult to stay bright and sunny under these circumstances, but somehow I manage! I remind myself over and over and over again that a gloomy Deb is a boring Deb. With the time I have left, I intend to adapt as I can and scare the hell out of Louise with my maniacal grin. Are you laughing, Wendy?


It's Thursday morning. I never got to post the above last night because I watched (translated: slept through) Harry Potter number five with Cecilia. We might give the movie another go tonight. Cecilia loves the parts that are filmed in London; she enjoys seeing on film places where she has been. I'm with her.


Just for the record, I am jonesing for an overseas trip. I have miles to use and (in Paris) a place to stay. I think it would be delightful to experience France in the fall, n'est pas?


A Collection Study to Find Biomarkers in Patients with ALS

Dear PatientsLikeMe Member,

Twenty-one NEALS ALS centers across the U.S. are seeking individuals to participate in a specimen collection study.

The purpose of the research study is to collect blood (plasma and DNA) samples from healthy subjects, and both blood (plasma and DNA) and cerebrospinal fluid (CSF) samples from people with amyotrophic lateral sclerosis (ALS), suspected ALS, as well as other neurological disorders, such as Multiple Sclerosis (MS), hereditary spastic paraparesis (HSP), or hereditary neuropathies such as Charcot Marie Tooth disease.

Through comparison of these samples, the researchers hope to learn more about the underlying cause of ALS, as well as find unique biological markers, which could be used to develop new ALS therapies.

Participants must be at least 30 years of age and be able to answer brief questions about their medical and family history, as well as be willing to have blood and/or CSF drawn for the study.

If interested, please visit our website to contact one of our NEALS ALS centers.

- The Northeast ALS Consortium (NEALS)

I'm going to call.

Man with motor neurone disease suffocated by wife, court told

A woman tried to suffocate her husband because she could no longer cope with him suffering from motor neurone disease, a court heard.

By Murray Wardrop
Published: 9:05PM BST 07 Jul 2009

Gary Roffey, 37, pleaded for his life as his wife, Tracey, tried to murder him by placing a pillow over his face at their home, jurors were told.
Initially, the father of three thought his attacker was a burglar but, realising it was his wife he allegedly begged her to stop, saying: "I love you, I love the kids – what are you doing?"
Chelmsford Crown Court was told that although Mr Roffey's health had severely deteriorated due to the disease, the couple had never discussed him ending his life.
It is alleged that when Mrs Roffey, 37, realised the pillow was not working, she put a plastic bag over his head crying "I love you, I don't want to do this" and "I cannot live like this any more".

The court heard that the alleged attack took place as the couple's three-year-old son sat waiting in their car outside the family home in home in Church Langley, near Harlow, Essex.
Prosecutor Richard Kelly said the incident in November last year happened shortly after the couple's sex life had broken down.
He added: "He paid for her to go on holiday to Spain believing she had recently fallen out of love with him.
"When she returned she announced they would no longer be having a sexual relationship and she would be coming off the pill.
"Also, lurking in the background was a potential financial background.
"They believed she would receive £350,000 insurance if he died but, it would, in fact have been just short of £200,000."

Jurors heard that Mr Roffey was employed as an IT worker in the City before being diagnosed with MND in May 2006.
By November 2008, his condition had worsened and his wife of 15 years required the help of three carers to look after him.
It is alleged that on November 4, Mrs Roffey dropped their daughters, aged 10 and 14, off at school before returning and attacking her husband as he slept.
Describing his ordeal, Mr Roffey said: "I was half asleep. I thought it was a burglar. A pillow was forced over my face and she was crying 'I cannot live like this any more'.
"I was saying 'I love you, I love the kids – what are you doing? You'll go to prison'.
"She tried to stop me breathing then dropped the pillow and put a Next bag over my head to kill me. I felt myself drifting away."
He added that as he lost consciousness, he thought to himself: "What a way to go."

Mr Kelly said that Mr Roffey was found by his carers shortly afterwards and was rushed to hospital, but discharged later that day.
He added: "Nobody would try to minimise the effect of the disease on a partner. There had been no discussion about euthanasia."
Mrs Roffey denies a charge of attempted murder.

The trial continues.



Becky brought this back from her recent vacation.

I love this Hawaiian guy...he's so cute! I had to share.


Why am I still up?

Perhaps this will do the trick. I would say ALS is a premature decay, or a brain-fag, or is a disorder consequent upon a reduced state of the nervous system, would you not?

I'm going to bed now. Sweet dreams.

My sweet girl

You come when called, whether it's 6am or 11pm. Unhook this, pull here, adjust this, please pour me some water, open the jarbottlebagcontainermicrowave, please scrub near my neck, please get the laundry, feed the cat, unlock the door, lock the door, turn on my lamp--it never ends and you never complain. You just do it.

I am grateful. And I am so very, very sorry.

Every day gives us a gift, a new "great joy" to recognize and carry with us as we go forward. Today, for me, it was when I felt your soft, cool, little hand in my weak, swollen hand. I told you I loved you and you told me you loved me. The beautiful crazy hazy moon hovered above. It was a moment in time I hope to remember forever.

Monday Musings

I came home tonight and did my usual site scan, which includes PLM. Two more PALS are gone. I am not inured to this, but I am weary of feeling upset about it. I hope I do not sound unfeeling.

Conversations at work today were often recaps of our weekend. I shared my experience at the game, including my disappointment at missing the Lou Gehrig speech. Several of the kids were unaware it had been 70 years since that speech was originally delivered. A couple times I was asked about strides that had been made, and I said that no progress had been made toward a cannot cure something if you don't know how it starts. I explained how even the one drug the FDA has approved isn't good for more than giving someone an extra couple of months.

All the death and inertness notwithstanding, somehow I have not fallen prey to a pity party. I know my fate--remember, we all die.

I do find myself wondering about my breathing. Most PALS die of respiratory insufficiency (is that the same as suffocation?). I get out three to four times a week now for a brisk walk or wog. Will it help? That's anybody's guess. But this old lady is gonna go out with guns blazing.

You play the hand you're dealt. I think the game's worthwhile.

— CS Lewis


Where I wog

The Fredericksburg Battlefield at Lee Drive.


...Root, root, root for the home team...

Four-run rally lifts Nationals past Braves

Washington's offense comes to life in eighth vs. Atlanta 'pen

Great game. The Braves were up 3-1 until the 8th, and then the Nats woke up. Final score was 5-3, Nats. I'm glad they won--it made up for missing the speech which was delivered BEFORE the game, not during the 7th inning as we'd been lead to believe.

Added bonus: traffic was great both ways.

July 4th. Be safe, be happy, be thankful.

"Fans, for the past two weeks you have been reading about the bad break I got. Yet today I consider myself the luckiest man on the face of this earth. I have been in ballparks for seventeen years and have never received anything but kindness and encouragement from you fans.

"Look at these grand men. Which of you wouldn't consider it the highlight of his career just to associate with them for even one day? Sure, I'm lucky. Who wouldn't consider it an honor to have known Jacob Ruppert? Also, the builder of baseball's greatest empire, Ed Barrow? To have spent six years with that wonderful little fellow, Miller Huggins? Then to have spent the next nine years with that outstanding leader, that smart student of psychology, the best manager in baseball today, Joe McCarthy? Sure, I'm lucky.

"When the New York Giants, a team you would give your right arm to beat, and vice versa, sends you a gift - that's something. When everybody down to the groundskeepers and those boys in white coats remember you with trophies - that's something. When you have a wonderful mother-in-law who takes sides with you in squabbles with her own daughter - that's something. When you have a father and a mother who work all their lives so you can have an education and build your body - it's a blessing. When you have a wife who has been a tower of strength and shown more courage than you dreamed existed - that's the finest I know.

"So I close in saying that I may have had a tough break, but I have an awful lot to live for."

-- Lou Gehrig, July 4, 1939



I've been keeping a record of the distances I've covered since I got back on track. Since June 22 I have covered 31.1 miles--equivalent to a 50k--walking and wogging. I don't know why, but keeping a tally provides me a little bit of comfort.

During my high intensity running days, I used to keep a spreadsheet called "Freakout Mileage," so called because my friend Adam and I were worried we weren't training enough for our multiple marathons. It wasn't unusual then to do upwards of 35-40 miles in a week; add to that the biking and rowing and occasional "twofer" runs. I was obsessive about recording the mileage per day, per week, per month, per year, and per pair of shoes. Yeah. Way, WAY over the top.

My new spreadsheet is less comprehensive. Date, distance, and whether it was a walk or a wog is all that I record. Milestones--like achieving 31 miles--bring me a gentle pleasure and sense of accomplishment, but there is no goal to do better, go further in less time, etc. It's enough to have done it at all.



The glamour shot.

Fat, puffy fingers, same old thumb atrophy. Note the ring finger. This is its constant position, making it the finger of choice (on the right) when manually typing.

The knuckles of the first two fingers have been swollen for several weeks. I think it's the new normal--gah. I hate it. I hate, too, that my thumb is permanently curved inward now.

Look at that stupid, gross protuberance. It's like a camel's hump on my hand.

Grip and Grin

The new steering wheel is a huge success! My arms are still tired, but at least I have better grip. Thank you, Andrew, for putting the cover on last night.

So, when Andrew put the cover on he had to lock the steering wheel for stability. I know you know what's coming. Right. When I went out to the car this morning, I could not move the steering wheel. I had to go in and wake up John. He succeeded in unlocking the steering wheel and went back inside. However, he had put up the emergency brake to a point where I was unable to release it. I had to go back in and ask him if he could release the brake for me. I was pretty frustrated after all that mess.

Fortunately, being able to grip the steering wheel made it easier to bear the frustration.

My next goal is to find a cupholder that fits in the driver's side window. I can't lift my coffee cup from the center console. It's always something.

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