THE ODDS (Debbie Does ALS)



Sometimes I have all sorts of stupid ALS shit floating around in my head and I am reluctant to post, but then I remember the reason I started this thing to begin with. With any luck I'll purge all this stuff here and won't then burden the outside world with all. this. shit.

First. I was visiting the still-active blog site of a now-deceased ALS patient and found this link. It's short, very powerful. I listened using Windows Media but there is a Real Media option, too.

Second. My stupid left fingers are now all standing separate. This makes me madder and sadder than I can tell you. Just writing about it, looking at my hand, I feel the eruption beginning to form in my stomach.

Third. Lynne and I went to Villanova PA for a party at Michael and John's totally fabulous home. We met lots of interesting and wonderful people, and our hosts were gracious and kind. All was well until Sunday as we were getting ready to leave--my left arm cramped terribly as I was getting dressed and I had to call for help. The action I was trying to perform has been more troublesome of late and Sunday it just wasn't happening. I pushed--too hard--and was punished for my arrogance. Who the hell am I to think I can outwit this fucking disease. I tell myself I won't accept further deterioration but the goddamn progression continues anyway.

So Lynne assisted me. This little scene resulted in some conversation about what's to come, what I will and will not be able to do. I do not want Lynne to have to care for me (beyond shepherding me out of some bar) so I declared that, at some point, the road trips would end. I don't think she'd mind helping me, but I sure as hell mind it. She's my friend, I won't have her become my nurse.

Naturally this made me think about other things I will have to end. When is the right time? Earlier, so the memories are pleasant? Later, because I will hate to step away, say goodbye? I've been quite a mess thinking about this.

Lynne and I made it to Mass before we left PA. My hands and arms were twitching so violently it looked as if I had Parkinson's. And the twitching everywhere else--including my ass-that-is-as-wide-as-a-trailer--was the same. It worried me that, if the twitching in my arms was so apparent, what did the people behind me think about my big, jiggly butt? Oh well, I'll never see those people again...

And finally, a report on my right arm and shoulder. It becomes more and more difficult to bear weight on the elbow/shoulder, which has been my recourse since my wrist became useless. It's harder to raise my arm, and quite painful when I try to extend further than my shoulder will permit. I have been doing my PT homework but I am beginning to realize that will only do so much.

So today I acknowledge that I am worse. ALS is creeping, none too gently, sort of like an invading army on a weak and helpless country. Before too long, the language and the customs will change and the little country won't recognize itself.

Okay, purging done, for the moment. If, after reading this, you want to purge, please know that you are welcome.


The dog whisperer

A short, but funny, story to relate:

Our Christmas dinner was bountiful, to say the least. We had turkey, ham, salmon, green salad, fruit salad, bread, rolls, potatoes, stuffing, pies, and other goodies. We ate buffet style in the living room. Harley and Stella went around the room, sniffing and looking expectantly at everyone.

Both dogs stopped in front of Cecilia. They looked longingly at her plate with their big, soulful eyes. Cecilia looked right at them and said, "I have no meat." We all laughed when, together, both Harley and Stella, hearing those words, turned and walked away. They continued their circuit around the room but never stopped at Cecilia again.

It didn't work for anyone else.

Is it the same for you?

Unhappiness and disappointment live in the stomach, and, when provoked, cause the stomach to tighten, travel upward into your lungs forcing out all the air, through the throat then into the nose making it tingle, and come out the eyes. I've paid attention to this the last week or so.

Just an observation.


Merry Christmas

It's quiet.

C Claire and I made it to Mass at 7AM, the Mass Fr. Rooney calls "the best kept secret." That's the truth. It's quicker and there are fewer attendees compared to the later services. On the way home C and I chatted about one thing and another, and I reached my hand over like I do to get a squeeze from her. She commented on my weak grip (this is typical of her, my darling Aspie) and we talked about the progression, and how neither one of us thinks I'll ever need a wheelchair lift. I said it seemed God was being kind to me, she said God gave me the disease so I would think.

My profound little angel. Talk about a Christmas gift.

She's back in bed. I bet you can guess where I am.


Sometimes I really do forget

You'd think I'd know by now. It's very disconcerting when I am doing something, some regular, ordinary little task, and I start to use my right hand, only to fumble and be reminded.

I hate when that happens.

One little dream come true

I just woke up from a quick nap. I was snuggled under an afghan with my feet up, the lights were out, the little tree was blinking, the little stove was glowing, the music was soft in the background.

It's just exactly how I pictured it.

Christmas Eve

Music blaring from my Mac. Coffee brewing. Everyone asleep except me and the animals. Today I am going to try to get the house in some semblance of order. The clock, she is ticking.

Just gulped down a tart and tangy glass of tomato juice laced (heavily) with lemon. Oh baby!

Next up: brownies. And maybe friendship bread.

Just called Kendall. She's going to her parents' tonight and is going to stop on the way. Yay!

Hmmm. The brownies are in the oven. I had a happy moment while stirring: I positioned the rubber spatula in my right hand (between the swollen, puffy fingers and the swollen, puffy top part of my hand) and STIRRED. Not for long, but it was fun for a few seconds.

I'm doubtful about the brownie outcome, however. I poured all the batter into the pan and put it in the oven, then realized I used a rectangular pan, not square. Wonder what will happen.

Well, they don't look awful, and they smell wonderful. Keep your fingers crossed.

Also, switched from iTunes to Pandora. It's time for Christmas music.

This is so cool. I'm listening to classical Christmas music, it's light and airy and fabulous. I looked out the window just now and the wind was picking up leaves, gently, randomly, here and there--it looked like a ballet. It was so beautiful.

Holy cow. What a flurry of activity. Andrew and Will and Josh moved all the furniture that is allowed downstairs. The Serenity Room is a thing of almost perfect beauty. Damon is working on hooking the tv up to the cable. Jenny is cleaning the downstairs bathroom and helping me sort through junk. And there is a TON of junk.

Lynne and Kendall are going to be here later to share a glass of wine.

Hmmm. It's a puzzlement. We have audio but no video. Damon is poring over the instructions. I am confident he will conquer the tv.

So far the TV seems to be winning. Poor Damon had to leave, but the war isn't over yet.

Lynne, Kendall, and I christened the Serenity Room. We opened a bottle of wine, turned on the fake stove, turned on the little Christmas tree, and sat and talked for about an hour. I think it was a nice break for Kendall as she makes her way to her parents'.

C Claire and I moved some boxes and a few pieces of furniture upstairs. I can't do more without more help so I'm taking a break. Can you guess where?

In just a few, it'll be back to the kitchen. I cannot believe it's Christmas.

I wish you all a peaceful and happy and wonderful holiday. My love to you all.


A Day in my Head

So. Time to catch up.

Today is Christmas eve eve, Tuesday. On Sunday morning I spoke to the members of the Ni River Community Church; the topic was "live each day as if it was your last." I said to them what I say to everyone about how I want to do my best to live my best, focus on what I can do, not what I can't, and how I will focus on today instead of worrying about tomorrow. It was well received and, I think, successful.


My dear friend -- the one who was hurting -- is recovering. After thoughtful introspection she realized the value of the relationship, and told me that she felt she had to reconnect because the thought of losing her friend was too painful. She broke my heart when she told me how painful it would be when she loses me -- and she won't be able to get me back. I understand her motives, I just hope this reconciliation doesn't result in fresh pain.

This conversation took place after an exhilarating night time run. We met at 7 PM and ran through a residential neighborhood. The Christmas lights ran the gamut and we were not disappointed to see the one house on the corner made up as it always is with those awful blow up figurines -- they must have 25 of them! Anyway, I haven't run at night in a long time, and I have always loved dark runs. It requires heightened senses, so you are distracted from the fatigue. And the air--it was crisp and cold and delicious.


Earlier this week there was a PLM forum topic called "ALS and the fat guy." Lots of comments about "healthy" (translated "a little extra") weight possibly contributing to slower progression. One of the PALS posted this:

I went on a low glutamate, no red meat diet and quickly lost about 16 pounds to get to a "healthy" weight. My neuro told me to gain the weight back. He informed me that a study in France showed that PALS at least 10% over the recommended weight live longer. He explained that the body metabolises muscle after fat and that extra fat provides a safety buffer in ALS, because when we metabolise muscle, we can't grow it back.

Mouse studies have also shown ketogenic (high fat) diets result in longer life and slower progression in ALS and I have seen articles suggesting that high cholesterol is beneficial with ALS. (Whether the latter point is true or the high cholesterol is just a consequence of the ketogenic diet is unclear).

Now, I am no string bean and never have been. Since I've been diagnosed I've put on weight due to less exercise and possibly my meds. I'm generally okay about this but struggle: the benefits of the extra weight conflict with self image. We live in a society where thin is beautiful and not thin isn't. I know this sounds totally shallow but I hate that I am becoming--have become--this pudgy (and withered--there's irony) old cow. That said, I'm two years into this fucking disease and still have minimal progression. The PALS who wrote the comment above is also arm onset, a year into it, and is already much further along. Again I wonder, why is my progression slower? Is it at all related to my cowiness? And if there is even the slightest chance that it IS, can I be comfortable with my cowiness? Aaaarrrggghhhh!


Christmas snuck up on me. The house MIGHT be ready, but the rest I am just trusting to chance. I've not wrapped gift one, and don't plan to.

The Serenity Room is starting to take shape. The carpet is in, the baby faux wood stove is out of the box, and the iPod docking station is set up (and is totally fabulous!). It's a beginning.


Enough rambling. Go read something less ridiculous.

Lots of reading--and more hope

Posts from the PLM site:

Neuralstem, Inc. announced this morning that it has filed an Investigational New Drug (IND) application with the U.S. Food and Drug Administration (FDA) to begin a clinical trial to treat amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease).

The Company is planning to treat ALS patients through spinal injections of its stem cells via its patented Human Neural Stem Cell technology.

"Like all first human trials, this proposed trial is primarily designed to test the safety and feasibility of both our stem cells and our method of delivering the cells to the spinal cord in ALS patients," said Neuralstem CEO and President, Richard Garr. "We are also proposing secondary endpoints which we hope will be able to measure a slowing down of the degenerative process."

ALS is a progressive neurodegenerative disease that affects nerve cells in the brain, leading to the degeneration and death of the motor neurons in the spinal cord that control the muscles. Loss of the ability to initiate and control muscle movement ends in paralysis and, ultimately, death. ALS affects roughly 30,000 people in the U.S., with about 7,000 new diagnoses per year.

Neuralstem expects to conduct the trial at Emory University with Dr. Johnathan Glass, M.D., Director of the Emory Neuromuscular Laboratory and Director of the Emory ALS Center, as site Principal Investigator (PI). Dr. Eva Feldman, M.D., Ph.D., Head of the A. Alfred Taubman Medical Research Institute and the De Jong Professor of Neurology at the University of Michigan Medical School, will be the overall PI for the ALS trial program. Formal approvals from these institutions to conduct the trial can come only after FDA approval of the trial protocol.

"The filing of this IND is an important event for Neuralstem," said Garr, "but it marks only the beginning of a process which includes working together with the FDA to approve the first human ALS stem cell trial; refining our understanding of how to optimize delivery of our cells into patients; and ultimately delivering a new treatment for patients with this currently incurable disease."

Neuralstem's patented technology enables, for the first time, the ability to produce neural stem cells of the human brain and spinal cord in commercial quantities, and the ability to control the differentiation of these cells into mature, physiologically relevant human neurons and glia. Major Central Nervous System diseases targeted by the Company with research programs currently underway include: Ischemic Spastic Paraplegia, Traumatic Spinal Cord Injury, Huntington's disease and ALS. The Company filed an IND (Investigational New Drug) application with the FDA for ALS clinical trials in December, 2008, and has entered into a collaborative agreement with Albert-Ludwigs-University, in Freiburg, Germany, to develop clinical trials for Huntington's disease.

In pre-clinical work, the company's cells have extended the life of rats with ALS (Lou Gehrig's disease) as reported the journal TRANSPLANTATION, in collaboration with Johns Hopkins University researchers, and also reversed paralysis in rats with Ischemic Spastic Paraplegia, as reported in NEUROSCIENCE on June 29, 2007, in collaboration with researchers at University of California San Diego.


An Emory nurse friend tells that that they are in the process of writing the inclusion/exclusion criteria for the trial which could begin anywhere from a few to several months but guessed to be in 2009...


A sea change in stem cell industry investment is coming, says Richard Garr.

The CEO of Gaithersburg-based Neuralstem happened to be in New York last week, pitching his company to prospective investors, when a member of President-elect Barack Obama's transition team said one of Obama's first priorities as president will be to lift the federal ban on funding embryonic stem cell research imposed by President Bush nearly eight years ago.

When he returned to his biotech this week, Garr said, he had found that "the general feeling in the investment community is that this is going to be a stem cell-friendly administration and a stem cell-friendly Congress." Even in today's difficult investment climate, the stem cell industry is going to see definite benefits, he said.

Human stem cells can be grown into healthy sources of new cells, tissues and organs to help scientists find treatments for diseases such as Alzheimer's, Parkinson's, diabetes and cancer. Some oppose embryonic stem cell research because they believe extracting cells from a viable embryo destroys a human life.

Investors mostly look for signs of progress, politics aside. Garr said he felt "warm interest by investors" at the Acumen BioFin Rodman & Renshaw ninth annual Healthcare Conference in New York because Neuralstem is ready to file an investigative new drug application for the first time and will enter clinical trials with a stem cell drug for amyotrophic lateral sclerosis, or Lou Gehrig's disease, this winter.

"The timing [of the Obama statement] is very good for us," he said.

A change in the federal policy won't directly affect Neuralstem, he said, because his company doesn't receive federal or state money.

But, he said, "It will be a good thing if [Obama] lifts [the funding ban] because it will change the public perception of stem cell companies. ... It will remove the uncertainty of whether or not this is going to be a legal industry. That is huge."

Douglas Doerfler, president and CEO of MaxCyte in Gaithersburg, who testified at a U.S. Senate hearing on the issue in 2005, agreed.

"I believe it will be a huge boon for companies who have been involved in stem cells [and] several are located in Maryland," Doerfler said. "This is a phenomenal area of science, and watching this field closely adds a sense of marvel at the progress being made, especially in regenerative medicine."

Doerfler cautioned, however, that lifting the ban comes late for the U.S. to get "back in the game," as other nations have invested heavily in the promise of stem cells and took competitive advantage while the U.S. was not playing a major role.

Linda Powers, managing director and co-founder of Toucan Capital in Bethesda, Maryland's most active stem cell investment firm, said lifting the ban would be "just the first step" in making the industry more fluid. She hopes, for example, that treatments for heart disease, multiple sclerosis and other serious conditions now being developed in other countries using patients' own bone marrow cells would be developed here without the current requirements of years of clinical trials.

Other restrictions, she said, include regulatory "artificial efficacy" rules on stem cells.

"They are held to much more difficult standards than other medical products," said Powers, a member of the Maryland Stem Cell Research Commission.

Even if the stem cells used are a patient's own, eight to 10 years of clinical trials and hundreds of millions of dollars of expenses are required. "If a patient's own stem cells are used in a therapy, they are regulated as a drug just as stem cells that are not from the patient's own body," Powers said. "That is just plain crazy."

Commercial stem cell research operates in "a very restrictive patient environment as well," which could loosen if federal policies are changed, said Jonathan Auerbach, CEO of GlobalStem, Inc. of Rockville.

Funded partly by Toucan, GlobalStem sells bio-tools for stem cell researchers, such as growth media to feeder cells, characterization tests and assays. Lifting restrictions, Auerbach said, would help because some of the limited embryonic cell lines that are available, called the Bush lines, are old and are "not the best cell lines."

Bowen P. Weisheit Jr., a member of the state stem cell commission and of the state chapter of the Cystic Fibrosis Foundation, said that if Obama lifts the ban, he thinks the effect on current proposals would expand.

This report originally appeared in The Business Gazette.


Well, I did my part and bought 300 shares of Neuralstem (CUR) stock this morning.


Researchers Show that a Single Adult Stem Cell Can Self Renew

GEN News Highlights

Stanford University scientists report that they have demonstrated for the first time that a single adult stem cell can repair tissue damage in a live mammal.

The researchers transplanted the skeletal adult muscle stem cells (MusSCs) into special immune-suppressed mice whose muscle satellite cells had been wiped out in a hind limb by irradiation. The stem cells restored lost function to mice with hind limb muscle tissue damage, according to the team.

The adult stem cells used in the study were isolated from a mixed population of satellite cells from the skeletal muscle of mice. Investigators genetically engineered the transplanted MusSC to express Pax7 and luciferase proteins. As a result, every transplanted cell glowed under ultraviolet light and was easy to trace.

The team used luminescent imaging as well as quantitative and kinetic analyses to track each transplanted stem cell as it rapidly proliferated and engrafted its progeny into the irradiated muscle tissue.

The scientists then injured the regenerated tissue, setting off muscle cell growth and repair, and subsequently showed that the MuSC and descendents rescued the second animal’s lost muscle healing function.

After isolating the luciferase-glowing muscle stem cells from the transplanted animal, the scientists cloned the cells in the lab. Like the original MuSC, the cloned copies were intact and capable of self renewal.

In further experiments, the researchers transplanted between 10 and 500 luciferase-tagged MuSC into the leg muscles of mice. These cells also proliferated and engrafted, forming new myofibers and fusing with injured fibers.

Unlike tumor cells, the transplanted stem cells achieved homeostasis, growing to a stable, constant level and ceasing replication. After demonstrating that the transplanted stem cells proliferated and fully restored the animal's lost function, the scientists recovered new stem cells from the transplant with full stem cell potency.

This research was reported at the 48th annual meeting of the American Society for Cell Biology (ASCB) on Sunday, December 14 in San Francisco.


No, I didn't buy the shares, one of the PLMers did. Maybe something to consider? Also, do any of you know the doctors/researchers mentioned? Just in case? You never know.



I learned today that another PLM-er "earned his wings." His name was Humberto, he lived in Brazil, he was a very active advocate for ALS awareness and worked hard on a lithium study. He was 42, had arm onset, first symptom in September 2006. He was diagnosed in March 2007. According to his profile, he was in a chair and used a feeding tube. His PLM avatar was multi-colored: orange head; red arms; green trunk; red legs.

I look at my weak hand and I am grateful.



Update. Not going to get the work done.

Thanks, wise friend, for your counsel.

What a weight has been lifted.



My friend, my dear friend, is working through a painful situation. I am so angry on her behalf and want so badly to lash out and remove that which causes the pain. I fear she will permit a recurrence, and if she does it's totally understandable. But tonight I want to take the pain from her.

Hang in there, cherished friend. It's going to be okay.


I am totally out of money and found out today my roof must be repaired. Sigh.

Where oh where oh where am I going to get the money? Santa?

It will all work out, somehow it always does, but right now I'm sunk pretty low.


A Day in the life

Morning Debacle
I got to work this morning at 640. The coffeemaker was broken and we had no Internet. I placed a troubleshooting call to the coffeemaker company and helped Steve identify who we needed to call about the connectivity issue. As well, the coffee cups I had set aside for the VIP visitors this morning had been "compromised," so I had to run a load of dishes. I gave Nick instructions regarding the coffeemaker, I gave David instructions regarding the coffee cups, and realized I had not eaten any breakfast. I rushed to the deli, ordered a breakfast sandwich, and ate it as I walked to the Metro. When I got to the Metro elevator I pulled my fare card out of my pocket with my ever dependable right hand; the card slipped right out of my fingers and into a puddle. Since my left hand was occupied holding the sandwich and my purse, I bent down and managed to pick up the card with my right. I wiped it off as best I could and hoped it would work in the machine. It did. So, off to clinic.

Waiting Room Observations
I found a nice surprise on the train in the form of Kendall. She was headed to Metro Center so we chatted a bit as we rode in.

Got to Foggy Bottom in really good time. Walked a block to the clinic and headed up to the seventh floor. There was a new face at the registration desk and I don't think she was very efficient. She must've sensed I didn't think much of her because she never told anybody I checked in. I was early, so I didn't expect to go in right away, but by 840 I knew there was a problem. I eventually told someone else I was there and was sent back to my room.

I have to note, however, that while I was sitting in the waiting room I noticed something -- I noticed people using their hands. I saw a man pick up his newspaper, another taking off his coat, a woman adjusting her top. I was struck with the feeling that I would give anything to be able to perform such insignificant tasks. These people weren't even aware they were using their hands -- we never pay attention to such things -- but I was and I do. When I arrive at work in the morning, I have to pick up the paper from the floor. To do so, I bend down and push the newspaper toward my foot, then I put my whole right hand under the paper and scoop it towards me. I should point out my left arm and hand are full of my coat, my keys, my purse, and the mail. Anyway, that was a pretty cool moment, noticing everyone using their hands. Don't take it for granted.

Good FVC
Today's FVC was 92%. And that in spite of this dumb sinus thing I have going on. I was leery of her at first but I really like the pulmonologist now. I don't think she knows quite what to do with me.

I also got my flu shot.

Wrong Room
I'm sure I've mentioned that I decline a visit from the psychiatrist when I am at clinic. When he appeared in my room this morning, I looked at him questioningly and said "wrong room," and away he went.

Searching Looks From Gwen
I had brief and enjoyable visits with the occupational therapist, the physical therapist, the speech therapist, and the dietitian. I dazzled them with my fancy footwork, my swallowing, my raised eyebrows, my puffed cheeks, my healthy (if plump) weight, my general good health. I've become quite fond of this team. They seem to like me, they seemed genuinely interested in my well-being, they are kind, and I think it pleases them that I don't progress quickly.

I spent a longer time with Gwen. She is the nurse coordinator for the clinic, so she has a lot on her plate. She asks all the comprehensive questions, and tries to get a sense of where we patients are in our disease. We had a good conversation and I told her that I was going to be speaking at the church on Sunday. She cautioned me not to become overwhelmed, and I told her I knew how to set boundaries. I explained to her that I felt I was pretty well-adjusted to the situation, that I was not happy that I had ALS but I had mostly accepted it, was adapting, and was happy in spite of it. I asked her if other ALS patients had this attitude, she said no. During our conversation, Gwen looked at me very intently, searching my face to make sure I was sincere and not just putting up a brave front. I think she was satisfied.

Dr Bayat
Next came Dr Bayat. I think she, too, enjoys seeing me because I am so slow. More questions and the standard physical examination resulted in the same singsong response, "you are a slow progressor." She wanted to test my grip strength so went off to get the little gadget. While she was gone I pondered her words and thought about the big picture as well. I have ALS. Why? Why is it me? In the big roll of the dice, how did I end up here? I'm at the point now where I really don't ask this question very often. I think about it, but not much. The doctor's comment about my slow progression resonated in my mind, and I asked the same question: why is my progression slow? All those other patients who progressed so quickly -- why is my disease so different? Why am I so lucky -- and I AM lucky.

She came back with the gadget which indicated my grip had weakend in both hands. No surprise there.

As Dr. B. and I were finishing up, in came Dr. Justin Kwan from NIH. After some introductory pleasantries, it was revealed that Justin is involved in a research project and wants me to participate. It's not a drug trial, it's research to understand the effect of ALS on mood and memory. It will involve several visits to NIH and several very intense MRIs. I agreed immediately.

My final meeting was with Ellen Cochrane, one of my favorites. She is the patient services coordinator for the ALS Association, and is such a gem.

David Sedaris
On to other things. One of the books on CD that Jenny brought to me was David Sedaris live at Carnegie Hall. I've heard him on NPR, but only in short bursts. This CD had me laughing so hard I had tears running down my face! I highly recommend.

Grocery Stores
I left work and headed home. I called Cecilia and told her that I had to stop at the store and gas station on my way home. The first grocery store is a Bloom, formerly Food Lion. You know, a new name doesn't change a thing. It's still the yucky store it always was. Anyway, I was in search of a birthday cake with chocolate icing. I went to the bakery only to discover that all their sheet cakes have white icing. Grumbling, I left and headed to the Giant. My confidence in them was rewarded; when I walked in I could see the chocolate icing sheet cakes in the case. I walked over and was just about to pick up a cake when I realize what I thought was chocolate was in fact cardboard protecting the plastic case of a tray of cupcakes. I groaned. Fortunately there were two smaller cakes with chocolate, so I grabbed them.

In between grocery store one and grocery store two, I stopped at a gas station. The last time I purchased gas, a nice -- and very strong -- man put my gas cap back on. I can usually get the gas cap off, but not tonight. Luckily there were lots of people at the station so I was able to get help.

Cakes purchased, gas purchased, it was finally time to go home. Just as I was backing into the driveway, Kenny called. He is such a dear friend and his call was the perfect way to end the day.

Slow progression, funny CDs, and conversations with friends notwithstanding, I do occasionally get hit smack in the face with reality. I had one such smack today, but I checked it before it was too painful. Well, almost.

Keep smiling.


Clinic tomorrow

So. Just popped my meds and will go to bed after I post this entry. Need to be out of here by 5:30 so I can work a bit before I go to the clinic.

It should be a short visit, because I am still progressing slowly. I'll try and see the pulmonologist early -- that will help me get out early.

Yep, still slow. I felt slow on Sunday at the support group Christmas party. Let me think -- I believe there were five or six people in wheelchairs. Four of us were ambulatory, but only two -- Larry and I -- were minimally affected. Susan W. was there; her husband, Tony, died some months ago after a four year battle with ALS. So I'm thinking to myself, did his four years commence with his first symptom? Or his diagnosis? Because if it's from the first symptom, I'm two years into it.

Have I mentioned the thread on the PLM website that discusses whether or not the disease just stops? There's so much debate among the patients, but the importance of a positive attitude is paramount. I've said before that I latched onto Matt's suggestion that I will be one of the 5% that lives for a long time. I simply won't accept any other possibility. So there.

Well, this was just more of the same. I know it's repetitive, but I derive quite a bit of comfort when I shine a spotlight on the slowness. Thanks for putting up with me.

Sweet dreams.


Today's gift

Tree expert Mat arrived shortly after I published that last entry. He's a nice guy, gave me a fair price on the TWO TREES that need to come down. He also impressed me with his sense of charity. He does work for Habitat for Humanity and odd work for area churches. He asked if I wanted the wood from the trees, because if not, he'd give it to Habitat or a church. Said he could sell it but figured "I can make a few bucks or I can make a difference." I was moved by his kindness.

Nice guy.

I'm an idiot

I believe I've overdone it.

My arms hurt, my hands are twitching and spasming, my legs -- specifically, my quads -- are shaky and feel very weak. Very stupidly, I tried to take up tile from my basement floor. It comes off easily enough, however, it's not so easy for me. After only a few minutes, my quads felt like I'd run a marathon. My left arm, which was doing all the work, went into freak out mode and caused my hand to curl in a tight and painful spasm. My right hand and arm tried to contribute to the effort by picking up the tile that had been removed, but no good deed goes unpunished. I am breathing heavily and have an overall sense of fatigue. Sigh.

I find it interesting that this sort of effort leaves me winded. I still can run, albeit slowly, but I don't get nearly as winded as I did just now.

I don't think I mentioned what happened Thursday night. Arriving home in the rain, I made a dash for the front door. I didn't fall but my right leg sort of "gave" and I wobbled a bit. I experienced a similar feeling just a little while ago.

At times like this -- feeling the way I do right now -- I wonder how long I'll be able to enjoy the improvements to this house. I miss my stamina, my endurance, my strength. It's so frustrating and it makes me so mad.

On another note, I'm waiting for a tree expert to come over. As it happens, there seems to be a large root under my basement slab, causing it to crack. It's not dire, but I don't want it to get worse so I've called an expert to give me an estimate on removing the offending tree.

It's always something.


My little grand-dog-ter

She's such a little doll!


Reading in the car

Note to self. If you're going to listen to a CD full of tender stories, bring extra makeup.


Again, thinking too much

Several things I observed this evening; naturally I have to share before I go to bed.

First, when I shower I love the way the water hits the crown of my head. For some reason it's like a "sweet spot" and I can't believe how good it feels. It's soothing -- totally delicious! I could stand there all night, feeling the water hit my head and run through my hair.

Second, I can no longer support my weight on my left wrist. It's been sketchy but tonight my wrist gave out twice. The default support will have to be my elbow, just like on the right side.

And third. The procedure was a huge success. I'm so grateful I can't even describe it. As I've said before, I can't imagine anyone helping me with "that." But it does put me in mind of other things, little things we take for granted but at some point I won't be able to do for myself. Like brushing my teeth. Or shaving my legs. Or washing my hair.

I went to the PLM website today, and noticed several of my co-pals have changed color again. I see orange arms and legs, red arms and legs -- I know what this means. I know they can't do for themselves, and I wonder who does for them. Do they have hired help? Family and friends?

I think about the help I've already received from those of you who love me. You've cut my meat, you've put butter on my rolls, you've helped me dress -- easy stuff, considering. I have to be honest, I shudder to think about the hard stuff. I wonder how we'll handle it. I give you fair warning, I'm very particular about my oral hygiene and my hair. You may want to escape now, while you have the chance!

Thank goodness none of this will be necessary anytime soon.

Testing 123

Well, it was a nine point lead and now it's down to four. Oh well, at least the Wizards are playing better than they have been.

I'm testing my voice recognition software tonight. MacSpeech Dictate is what I'm using, seems to work quite well. Still working out a few bugs, but for the most part it's quite useful.

House update: I should be able to move furniture around downstairs this weekend. I'm meeting with the carpet people Thursday night, and hope to have it installed the Monday before Christmas. (Okay that last sentence worked really well.) I've never been so broke, but I've never liked my house more. I can't wait to curl up in my "serenity room," reading a book, listening to music, basking in the little tiny glow of my fake wood stove. :)

Jenny and "the Pouffe" stopped over this evening. Little Miss Stella was wearing her adorable new Mrs. Santa outfit -- talk about beauteous fair! Jenny came over to deliver the audio books I've reserved. When my office moves, my commute is going to be longer. So I decided to make use of the time and catch up on my "reading."

Wizards update: Juan Dixon just shot and put the Wizards at the century mark! They may win this game -- but they've got a long way to go to get to 500.

Anyway, the selection of audio books Jenny brought over really runs the gamut. I'll start things off tomorrow with a collection of stories from NPR's StoryCorps.

Okay, it's late. I'm done playing with this new software so I will sign off. Maybe next time I'll have something worthwhile to say!


Just sitting here, convulsing...

Wow. The middle finger on my right hand is convulsing and jumps around like a needle on very expensive medical equipment. I have to be cautious when typing because it strikes rapidly and firmly and without notice. I've said before, except for it being a condition of ALS, I think it's pretty cool.

But I do have some questions. Does this convulsive activity mean it's getting worse? Okay, it's always getting worse, but does it mean it's getting worse THIS MINUTE and after the jumping and crazy behavior stops, will I have lost more function? Should I be concerned about these attacks?

It has stopped, for the moment. Like a bratty child looking for attention, it was showing off until I looked at it. And no evidence of immediate loss of function.

Ya gotta love it when I ask AND ANSWER questions in one short post. Well, answered one question. It'll do.

Dropping like flies

Not a good thing. Seems every week or so I hear of someone else I know getting laid off.

Georgie-boy, you sure did a hell of a job as president. Wish someone had let you go eons ago.


It snowed a little last night...

So this morning's run was very pretty! The course took us along a residential boulevard and then to a paved trail. The scenery was just wonderful, the way the first dusting always is. The snow sparkled and added brightness to an already sunny morning. The light layer of snow on the trail was troublesome for my companions, but it was perfect for me. My great big feet, the way I strike with a flat foot, my cautious pace--all combined to make the trail the best part of the run. Lynne, Tam and Nell had to walk quite a bit, but slow, steady, mountain goat ol' me just kept on and on. It was a great run. So I'm two for two!

I am told tomorrow evening there is a meeting for prospective student drivers and their parent(s). Can you believe C Claire is a prospective student driver? No, neither can I. But it's true and tomorrow we go learn what's going to inch its way into our lives during the ensuing months. Sigh. My little girl...

I'm permanently installing some voice recognition software, hopefully tomorrow. I'm still doing okay typing, but occasionally feel tired in the right hand, particularly if I'm cold. I'll practice with it here, so there may be some LONG WINDED posts coming up. I'm sure I'll find it very useful when I write a bestseller. Yeah, right. That was a big fat joke!

Almost medbed time. Sweet dreams!



As I got dressed this morning, I was nervous and eager. Isn't that ridiculous? But I was.

When I got to the meeting place I was excited.

There were all the "team" members: Lynne, Tam, Nell, Cathie, Tiffany. And me.

We took off, the five of them running a good, strong pace while I stayed very conservative. No talking, no pushing. Just running the way I run. Now.

As I ran I thought of good things:
  • Muscles Lynne and Tam
  • Cold air
  • Legs that still run
  • Lungs that still breathe
  • Muscles that still propel
  • Cold air
  • Mittens
Ran the full course, about an hour long. Didn't feel tired at all, suffered no ill effects in the "procedure region." Decided that, in spite of it all, I am still a runner. Thank heaven.


DQ does not mean Dairy Queen

I observe the last several posts have been gloomy, and yesterday's was downright DramaQueeny. My apologies.

I listened to NPR's Story Corps this morning coming in. They told a story of a mother and daughter, and the daughter has a rare aging disease. Their story was simple, tender, beautiful. My eyes filled with tears as I listened and realized there is SO MUCH I have to be grateful for, I have no right at all to be unhappy.

So today I'm going to be happy.


I'm going to run

I was told after the procedure to wait two weeks before running. I can't stand it anymore. I told Lynne I was going to run Saturday morning. She suggested I call Dr Walker and let him know and see what he says. I will call. If he's not there or if I do not hear back before Saturday morning, I will still run. If I tire I will walk. But I am going crazy, I'm crying, I'm sad, I want to die, and I know it's the running. Oh, and ALS, but mostly the running.



*Pity party alert. I know I'm tired and that probably contributes to the tone of this post. Just letting you know.

My legs continue to feel heavy. Yeah, I know, I'm tired. But it's DIFFERENT. Just like my right arm is different--more different. My shoulder is more limited. We work on it at PT, I do my homework. End of the day, it hurts too go too far, which didn't used to be too far. My right hand can barely make a fist anymore. My left hand is feeling a lot like the right hand when I thought the right hand felt funny.

I'm getting a glimpse of what's ahead and I don't like it.

I was told today I'm handling this well. I'm not, not always, not really. Today is one of those days. Today I'm sad about things, a life I won't have. A life I want desperately and won't get to live. Things I will have to give up, likely sooner rather than later. Sometimes I want to give them up now because it hurts so much knowing that I'm really just drawing things out. But the thought of losing these very precious parts of my life fills me with more sadness.

Tomorrow I'll be asked how I am. I'll say I'm fine, maybe even peachy. And on one level that will be true and has to be true because what else is there?

The what else is where I am tonight.


Just making a note: my legs feel like lead today. My first few steps after sitting are awkward. Everything is suspect.

There's a silver lining somewhere.

Today is one of those days. Midnight to morning meltdown, lack of sleep, job issues...these things and more contributing to a funk.

What I need to do is face all these negatives and push past them. And drink gallons of coffee.


First trial in patients with a potential treatment of the incurable ALS muscle disease

2008-12-01 09:00

Leuven (Belgium), Stockholm (Sweden) - Permission has been granted to start the first safety and tolerability trial on patients for a remedy for ALS. ALS is an incurable, paralyzing neurodegenerative disorder that strikes 5 persons in every 100,000. The disease commonly affects healthy people in the most active period of their lives - without warning. Researchers from VIB at the K.U.Leuven have previously shown the possibilities for the use of VEGF in the treatment of ALS through work in animal models. The Swedish Biopharmaceutical company NeuroNova has already built upon this research. Together with UZ Leuven they'll start the first evaluation of safety and tolerability of the drug in patients by the end of this year. This is an important step in the development of a new treatment. It will take several years before the protein can be made available as a medicine.

An incurable disease of the muscles

Amyotrophic Lateral Sclerosis (ALS) can strike anyone. The Chinese leader Mao Tse Tung, Russian composer Dimitri Shostakovich, the legendary New York Yankee baseball player Lou Gehrig, and astro-physicist Stephen Hawking have all been afflicted with ALS. About half of the patients dies within three years - some even in the first year - usually as a consequence of suffocation.

In ALS, the patient's nerve bundles that extend to the muscles deteriorate. As a result the patient loses control of the muscles, and progressively becomes paralyzed. The originating mechanism of this deadly disease of deterioration - which has an enormous medical and social impact - remains obscure. At present, the disease is totally untreatable.

VEGF: a promising candidate drug

VEGF is a substance that controls the growth of blood vessels. Unexpectedly, VEGF also helps neurons survive under stressful conditions. In 2001 Peter Carmeliet's team showed that too little VEGF causes ALS-like symptoms in mice. Later the group of Diether Lambrechts, Wim Robberecht and Peter Carmeliet showed that persons who produce too little VEGF - due to certain variations in the gene that codes for VEGF - have a higher risk of developing ALS. This was the starting point of a search for a possible treatment with the VEGF protein.

Testing the treatment on rats with a severe form of ALS and on rats with a milder form, the researchers found that, in both groups, the VEGF-treated rats manifested the disease later than the untreated animals, and they lived considerably longer.

Using a pump

The researchers also investigated what the optimal technique would be for administering VEGF. An ordinary injection proved to be ineffective. But continuous administration of the VEGF protein directly into the cerebrospinal fluid (the fluid that circulates around the brain and the spinal cord) was quite effective. This was possible by means of a small pump that continuously pumps the VEGF protein in the brain. Furthermore, this technique permits a patient-oriented approach by enabling the administered dose of the VEGF protein to be easily controlled.

A story with several players

These encouraging and promising results were only the first steps on the way to a new remedy. Anders Haegerstrand and his team of the Swedish company NeuroNova have taken the development of the treatment further. After additional studies this research has reached the stage of starting the first trial in patients. Wim Robberecht (UZ Leuven) and Markus Jerling (NeuroNova) will co-ordinate this first trial which is intended to evaluate the safety and tolerability of the drug and the infusion system. It is planned to start at the end of this year, and the investigator Dr Robberecht is currently looking for patients who are eligible for participation. These regulated studies on ALS patients will have to demonstrate the safety of the VEGF administration, and in a later stage the efficacy of VEGF as ALS therapy, before the protein can be made available as a medicine. Such procedures can easily last several years.


Given that this research can raise a lot of questions for patients, we ask you to please refer questions in your report or article to the email address that VIB makes available for this purpose: . Everyone can submit questions concerning this and other medically-oriented research directly to VIB via this address.

For practical information concerning the clinical trials you can contact Petra Tilkin (

More information

For more information on this project, please contact

the VIB Communication Service: +32 9244 66 11

Peter Carmeliet: +32 16 34 61 42 of +32 475 87 13 79

Wim Robberecht: +32 16 33 07 70 (0486 09 85 69)

Anders Haegerstrand, CSO, NeuroNova: +46 8786 0900,

Markus Jerling, NeuroNova: + 46 8786 0900,

For more information on NeuroNova, please contact

Ulf Ljungberg, NeuroNova: +46 8786 0900,

More info on

Relevant scientific publications

  • Lambrechts et al., Meta-analysis of VEGF variations in ALS: increased susceptibility in male carriers of the -2578AA genotype (J. Med. Genet., epub, July 2008)
  • Zacchigna et al., Neurovascular signalling defects in neurodegeneration (Nature reviews Neuroscience, Vol 9, March 2008, 169-181)
  • Lambrechts and Carmeliet, VEGF at the neurovascular interface: Therapeutic implications for motor neuron disease (Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Vol 1762, Issues 11-12, November-December 2006, 1109-1121)
  • Storkebaum et al., Treatment of motoneuron degeneration by intracerebroventricular delivery of VEGF in a rat model of ALS (Nature Neuroscience, Vol 8(1), January 2005, 85-92)
  • Azzouz et al., Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration (Nature, vol 429,27 May 2004, 413-417)
  • Lambrechts et al., VEGF is a modifiere of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death (Nature Genetics, Vol 34(4), August 2003, 383-94)
  • Skene & Cleveland, Hypoxia and Lou Gehrig (Nature Genetics, vol 28, June 2001, 107-108)
  • Oosthuyse et al., Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration (Nature Genetics, Vol 28, June 2001, 131-138)

Note for the editor

The fundamental preclinical VEGF research was done by Diether Lambrechts, Wim Robberecht and Peter Carmeliet of the VIB Vesalius Research Center, K.U.Leuven, under direction of Peter Carmeliet (, )

The further development of the research is done by NeuroNova ( The clinical trial is done in collaboration with Wim Robberecht, linked to UZ Leuven ( and the research group ‘Neurobiology' of the VIB Vesalius Research Center, K.U.Leuven (More info:


VIB, the Flanders Institute for Biotechnology, is a non-profit research institute in the life sciences. Some 1100 scientists and technicians conduct strategic basic research on the molecular mechanisms that control the functioning of the human body, plants, and micro-organisms. Through a close partnership with four Flemish universities - Ghent University, the Katholieke Universiteit Leuven, the University of Antwerp, and the Vrije Universiteit Brussel - and a solid investment program, VIB unites the forces of 65 research groups in a single institute. Their research aims at fundamentally extending the frontiers of our knowledge. Through its technology transfer activities, VIB strives to convert the research results into products for the benefit of consumers and patients. VIB also develops and distributes a broad range of scientifically substantiated information about all aspects of biotechnology. More info at:


The University of Leuven is Belgium's largest university and one of the oldest universities in Europe, founded in 1425. It is a comprehensive university with 14 faculties, with a long tradition of high-quality interdisciplinary research and teaching. The University of Leuven has over 33,000 students (12 percent international) and over 17,000 staff members (8,600 in the various university departments and 8,700 at UZ Leuven, the university hospital). More info at:


NeuroNova ( is a Swedish bio-pharmaceutical company based in Stockholm, Sweden. NeuroNova has two drug candidates nearing clinical development for Parkinson's disease and ALS. NeuroNova works with neurogenesis and neuroprotection for the treatment of several currently incurable neurodegenerative diseases, including Parkinson's disease, Alzheimer's disease, amyotrophic lateral sclerosis (ALS) and Huntington's disease.

UZ Leuven

UZ Leuven is a university hospital with a reputation throughout Europe as a centre of medical excellence. High-quality, customised patient care, multidisciplinary cooperation, innovation and continuous training all go hand in hand at UZ Leuven. Thanks to the dedication and drive of our motivated staff, we are able to achieve our mission day in, day out.

Mention both VIB and the university

When reporting this research, please always mention VIB as well as the university concerned.


Acknowledging a gift.

Do I say it too often? Do I say it enough?

I am one of the luckiest people in the world.

Lynne. My friend. Thank you.

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