Hello Zeebah Naybuh

Before my trip I had gone to the Goodwill looking for a light rain jacket. I didn't have any luck, but I did see a FABULOUS zebra print jacket. Being uncharacteristically disciplined, I passed, albeit reluctantly. I later told a friend about my win over temptation and was consoled when I was told I'd get my zebra eventually, but in another iteration.

I do not want to be hopeful, but take a peek at this little number and tell me what you think:

Zebrafish offer hope for treatment for motor neurone disease

The tiny zebrafish, the tropical freshwater minnow so popular in fishtanks, could hold the key to a cure for motor neurone disease, scientists believe.

By Richard Alleyne, Science Correspondent
Last Updated: 5:17PM BST 29 Apr 2009

The discovery could help patients with MND, in which the motor neurone cells die and are not replaced.

Researchers at the University of Edinburgh have found that these fish are able to produce motor neurones, cells that control all muscle activity such as speaking, walking and breathing in humans, when they repair damage to their spinal cords.

By studying this process and harvesting these cells, they hope to treat motor neurone disease sufferers and reverse some of the devastating damage caused by the condition.

Dr Catherina Becker, from the University of Edinburgh's Centre for Neuroregeneration and Euan MacDonald Centre for Motor Neurone Disease Research, said: "Understanding how zebrafish can regenerate large numbers of motor neurones after damage to the spinal cord and how these motor neurones are produced by natural stem cells could help in finding treatments for Motor Neurone Disease.

"This could take the form of improving methods of generating motor neurones in the laboratory that could be transplanted or finding drugs which could help patients renew their motor neurone supply."

The discovery could help patients with MND, in which the motor neurone cells die and are not replaced. This disease can cause paralysis and severe breathing difficulties and is an ultimately fatal condition for which no cure exists.

The team are now screening small molecules with a view to finding drugs that could kick-start the process of motor neurone regeneration in zebrafish, with a view to translating their findings into treatments for humans.

The tropical zebrafish, which are transparent and just over an inch (3cm) long, produce the motor neurones from cells, similar to stem cells, found in the spinal cord that are able to turn into certain types of cells.

As well as looking at stimulating the production of motor neurones, scientists are working on ways to ensure that these cells are able to function by sending messages from the brain to the spine and then on to muscles. The research could also have implications for treating spinal cord injuries following accidents.

MND affects around 5,000 people in this country alone at any one time. In the UK at least five people a day die from MND. Life expectancy for most people with MND is just two to five years, and around half will die within 14 months of diagnosis.

I could wear that well, don't you think?

Life's little moments

I am a total fool but I had fun today. 10K race (no, I didn't run). Lynne ran and was approaching the finish and I jumped in next to her and ran almost to the end with her.
She had run 6 miles in the horrendous heat but still had to take her pace down so I could keep up that last 20 yards.

Reminded us both of the good old days.

Damme

Have noticed the last couple of days that my idiot STOMACH is twitching. It's an awful feeling --not painful, but heartbreaking. And it makes me think thoughts I don't want to think.

Today

I ran five+ miles. No raptures, it was just a slowwwwwwwwwwwwwww but solid run. When I stopped my legs felt funny, but walking helped.

I'm glad I got out this morning. I thought about my EMG and my "minimal" status and was pleased I am still able to put one leg in front of the other. Each step is a gift.

I heard some sad news today

My friend Kyle died April 7. I met him last year at a support group meeting not long after his dx -- his was bulbar onset. And his progression was rapid.

His breathing was so diminished that he was unable to survive an airline flight to Florida. He didn't die on the plane, however, he died not long after arrival.

On the one hand, he doesn't have ALS anymore. On the other hand, we don't have Kyle -- a good, decent man -- anymore.

It's not right. How I loathe this fucking disease.

Little things

I just came back to my desk and lowered myself into my chair. Midway through, I was aware of doing it, doing it naturally and as I have always done it. Evidence of progression notwithstanding, I am still lowering myself into my chair.

This realization made me very happy.

Oh those wonderful ALS patients

A few posts from today:

Fun things to do with your power wheelchair

I just discovered that running over bubble wrap with my power wheelchair is really fun. Even more entertaining when my cats jumped up in surprise.

Isn't that the greatest post ever? I liked this, too:

New trach!

I never thought I'd be so excited about a trach. I think I have the cadillac of all trachs now. It's a bivona tts, cuffed. I have a #9. It's actually comfortable. It's all latex or rubbery. I'm louder, not necessarily clearer. I don't gag or cough as much when it's moved or cleaned. It seems like I get more crap out when I'm suctioned. When I fill my cuff to eat, I don't cough and gag for five,minutes. I can breathe easier when off the vent than with the shiley.I wish I would have known this a year ago.

The Bivona is far superior to the Shiley.

I have a hard time reading that one but LOVE the enthusiasm of the poster.

One more tidbit, before bedtime

Do you remember this post?

My least favorite thing to do these days is put moisturizer on my face. NONE of my fingers on either hand cooperate; they exist separately and refuse to come together to perform this one little act. The lotion ends up between my fingers, not on my face. And forget the throat. Lotion is there applied using the back of my knuckles because not only will my fingers not assist, I can't easily get my hands near my neck.

It totally sucks. I really truly hate Louise.

On a delightful note, my daughter's bird sings while I brush my teeth. He likes the sounds of the toothbrush and accompanies it with what I call The Toothbrush Song. (Thanks to this book by Michael Chabon for the song idea.)

Sweet dreams.

To continue

So. The EMG today revealed what I already knew: there is evidence of progression in my legs and spine. Consequently, I am no longer labeled "possible," I am now "probable" and eligible for testing and trials.

I had a very good discussion with my doctor about ceftriaxone, lithium, slow progression, and my options. My doctor sensed my unease and concerns about making a switch, particularly if it resulted in a more rapid progression. We have decided the proper course of action will be this: I will continue with the lithium/rilutek cocktail for the time being. We will monitor my breathing and if (when?) my FVC starts getting closer to 60% (it's currently 90%), I will switch.

I was much relieved after this conversation and decision. I absolutely do want to participate in the ceftriaxone study, but not yet.

I won't deny I'm a little sad. Even though I knew my legs were going to show signs of progression, it's never easy to hear it confirmed.

Eeyemgee

No longer possible. Now probable, and eligible. BUT.....
More later.

Look at it this way

One perspective: I was slow, winded, needed help in some of the sketchier parts of the trail.

The preferred perspective: All the above notwithstanding, I still hiked for a couple of hours, saw some beautiful waterfalls, and didn't sit at home on this gorgeous day.

I need to work through the curve and accept help when offered, and ask for it when needed. Not be embarrassed. Proud. Stupid.

Here I am.

Site of the pit where victims of the Black Death were buried. Check.
Platform 9 3/4. Check.
Tower. Check.
Site near where Anne Boleyn was beheaded. Also check.
Crown Jewels. The Clink. The Thames. Buck House. Big Ben. Westminster. Trafalgar. St. Pauls. Piccadilly. Covent Garden. Carnaby Street. Check, check, check.

I am returned.

It was a lovely trip, complete with tea and pints and game pie and fish and chips. Oh. And meltdowns. They'd bubbled at the surface even before we left, by Tuesday the pump, she was primed. Long story short: shopping frustration at Hell (aka Abercrombie & fucking Fitch). I swear there should be an epic called Dante's A&F. My fingers are not up to the task of relating how awful were the lines, the heat, the oppressive stench of too many people (including ripe teenagers, bleah), the insufficient lighting, the music. All this was too much for me, and the absence of that which my dearest youngest craves but can never find tipped the scale. Out we went, she protesting and I gasping for fresh air; her protests became louder and louder, the passers-by stared, and then she stopped and declared she was going no further. None of my calm voiced attempts at reason did any good--they never do. Had we been home, I would have distanced myself (translated: escaped) to permit her the time to cool off away from me. Can't do that in a big city, but I did continue around the corner (from which vantage point I peeked to make sure she was still safe). After a bit I went back and she, clutching the iron fence and crying openly, came with me, the rumbling and grumbling persisting.

I walked ahead of my darling terror and looked defiantly in the faces of the curious...what could I do? Returning to the scene of Dante's next and worst was NOT an option, we had to move forward. I grabbed a delightful cab and directed him to the Lamb and Flag (known in an earlier day as the Bucket of Blood) and distracted/fed my sweet girl. It was a temporary calm, but incredibly welcome.

After we'd finished and walked some more, the rumbles began again and, while waiting for the bus, she growled and yelled. People moved away from us. I remained stoic and calm, but I will confess to having not-so-good-mother-type of thoughts.

What goes through her mind when she rages? I wish I knew, and knew how to untangle the knot of frustration that causes her distress. Conversation is useless; her responses are hurtful and irrational. It's wearing, exhausting.

Peace was ultimately restored back at the hotel: she had a book and I had a(nother) pint.

Life IS good, even with the meltdowns.

On Wednesday we left, taking the Underground to the airport. A young woman and her boyfriend were on the train, too, and I was struck with how abundantly she resembled the portraits I've seen of the 14th and 15th century English royalty...it's as though she was a perfect example of a classic English look.

(Anne B., in better, headier days. Hahaha, I just crack myself up!)

So we are home, and all is well. Work is busy. My EMG is Monday. There are so many collateral thoughts attached to that event and, again, my fingers aren't up to the task. I have failed in my attempts to smile-no-matter-what and even found myself near tears today. My idiot mouth persists in twitchiculatin' and I am conscious of "other things."

Before I travel down this road, I'm making a concerted effort, right now, to smile. See? Smiling. Easy as pie.

Takin' a bit of a break

I've seen things and eaten things and SIPPED things the past several days. Had a fabulous reunion with HRH Victoria, who has changed much in 17 years. She's a dear, plain and simple.

Have wwwwwwwwwwaaaaaaaaaaaalllllllllkkkkkkkkkkkeeeeeedddddddd so much my legs hurt but I know that will be okay once home. The jeans have been a huge success (thanks, Tiffany!).

Pictures to come.

Back to seizing, not the day just now, but night, and rest.

Sweet dreams.

Hiatus

Headed across the pond for spring break. Was invited to upgrade my economy status to first, at the low low (low?) cost of -- get this -- $24,000. $24,000!!!!!!!! WTF?!? Who spends that kind of money on airfare??

Pas moi. I can't afford this trip, but I don't care.

Because.

Despite my slowness, I know the clock ticks.

I reflected today on the rigidity I have in my right arm. PT is great and my range is above average, but it is still difficult and painful to raise my arm above a certain point. I know the same thing is going to happen to my left arm and, in some fashion, to my legs, and more. And I know it will get worse. Not yet, probably not for a long time, but I don't know when so I'm seizing the day.

When I take a break from seizing, I'll drop a note.

Hello.

Where to begin.

Okay, first. My most recent purchase of "pregger” pants -- a pair of jeans -- fit nicely once I was able to pull them up. Denim isn't as cooperative as thin cotton and I had quite a bit of difficulty. Undeterred as always, I came up with a solution. I asked my friends if they knew anyone handy with a needle. Tiffany responded and I laid out my grand plan: I wanted a handle/loop on each side so I could puts my arms through and hoist up my jeans using my arms instead of my hands. She worked wonders! She brought them by last night and I tried them on before I went to bed. It was so liberating, I felt so independent. I don't know how I will ever be able to thank her.

Next. My half-sister's half-sister -- who died of ALS -- succumbed 18 months post diagnosis. As you all know, I am 18 months post diagnosis. Lots to think about, more to be thankful for.

Next. A must see. This is another of the PALS I know from PLM. She's a slow progressor, has had ALS for five years. Arm onset.

Next. Had a SUPER visit with Mike today who was well pleased with my range of motion.

This is a good time to segue to something that has been on my mind, probably prematurely. Okay. I am 18 months post dx. I am progressing slowly. I am on lithium. As I've wondered before, am I slow because I'm slow or am I slow because of lithium? Now. I am presented with the possible opportunity to take part in the ceftriaxone study, if my EMG proves eligibility. It is not a cure, it is a POSSIBLE slower-downer IF I get the drug. There is a 2/3 chance I'll get the drug. Which will have to be administered into an implanted-in-my-neck IV catheter, twice daily. Both Dr Bayat and Dr Kwan are very keen on this study; it has much clinical data supporting it (unlike the lithium) and no one has died while taking it (like the lithium).

My questions:

1. What if my progression speeds up?
2. What if I discontinue the study but it's too late?
3. What if I shortchange myself by not participating?
4. What if my not participating shortchanges others?
   

I know. I am thinking way too much. There are lots of other questions, and I'm sure you have them too. What are your feelings? What would you do if you were me? What should I do?

Sorry. I started out quite positively, and then I turned the uncertainty corner. Disregard my dingdonginess, and take comfort and pleasure in the visual of me walking about with pull-up handles flapping from the hips of my pregger jeans. Heavens...after that, what's the big deal about a neck catheter?

By the way. Did you not simply ADORE those black crystally shoes? I almost swooned.

Sigh...

These, and more...

Sadly, my feet are too big--no 42s available. My checkbook is relieved.

It's sunny out, but...

I'm hiding. The storm will pass eventually, but it's best to take shelter away from it so it cannot lash out at me.

I'm missing my support group meeting today as a result.

On the other side of this stormy coin, it's a day at home and not unwelcome.

Ignore-amus

That's one of my new identities. If it hurts or makes me worry, it will be ignored. Eventually it'll go to a different place and won't bother me anymore.

Was going to write more but decided to start ignoring.

Meeting halfway?

I have a half-sister. We share the same father. Not a story for this forum, at least not yet. I mention it because I learned tonight that she had a sister (same mother, different father) who died of ALS.

I wasn't surprised to see her letter when I got home. I can't explain why, but I almost expected it. I called her, we chatted briefly for the first time in about 20 years, and that was that. Like I said, the back story isn't a subject to discuss now, but I decided tonight to reach back to her and see where it leads. All the original cast--our parents--are dead.

Who knows where this will go, if anywhere. But for some reason she has popped in--even if it's just for a moment--and it took a little bit of courage to do that.

I think it's a terrible coincidence about the ALS.