Matt's hand holding a close-up bottle of Spinraza (nusinersen).. Matt's hand holding a close-up bottle of Spinraza (nusinersen)..

It’s been a long time waiting for a lot of red tape to clear up and a surgery to undergo (one year and four months, to be exact), but today my brother and I finally got our first dose of Spinraza (nusinersen), the first drug approved to treat spinal muscular atrophy.

I prayed for this day often as a kid but never seriously thought it would come. It is not a cure, but it is a treatment that has been shown to prevent much of the progression of SMA and to improve strength in many cases. It has mainly been tested on children, and by the chatter I hear, it has been slow going for adults trying to get access to the drug. This is largely because of insurance companies resisting to pay for it, as was our case. After three denials, we got funding through Biogen’s free drug program.

Another reason for the delay in some adults, including us, is that the spinal fusion we received as kids to prevent spinal curvature blocks all entrances for lumbar punctures, the method through which Spinraza is administered. Various strategies have been used to circumvent the spinal fusion and deliver the medicine. I can only comment intelligently about the strategy my doctor and surgeon used, and I’m led to believe Blake and I are the only ones who have had Spinraza delivered this way. So I offer an explanation here for the curious, as well as adults with SMA out there who also have a fully fused spine.

Delivery method

Matt holding a bottle of Spinraza.

About a month ago, a surgeon drilled a hole in our lower fused spine to make an entryway. But instead of injecting the drug there, he placed an Ommaya reservoir, which usually goes in one’s head, on our lower rib cage, right under the skin. A catheter, usually used to go from the reservoir into the brain, wraps, instead, around to the lower portion of our spine, where it enters and goes all the way up the spine until it reaches the top, close to the neck (thorax?, thoracic cavity?… something like that). This is supposed to make the treatment even more effective than if injected into the lower part of the spine, as happens with a regular lumbar puncture. The primary benefit, however, of the Ommaya reservoir is that is makes getting the multiple doses of Spinraza a simple, in-office procedure. No need to do a lumbar puncture for every dose. The doctor simply sticks the Ommaya reservoir on the rib cage with a butterfly needle, withdraws 5mL of spinal fluid, replaces it with 5mL of Spinraza, and puts back a couple of mL of your own spinal fluid in order to push all the medicine in the catheter out into the spine. Gotta be sure and get all of it!

There are four loading doses in the first two months, and then a maintenance dose every four months thereafter. So having the Ommaya reservoir right there under the skin is extremely convenient. I will say, though, that the surgery to initially place the reservoir and catheter wasn’t as easy as it was supposed to be, but with SMA, we expected there might be difficulties.

It’s been almost 12 hours since my first dose. Not feeling any different yet, but hopefully it won’t be long :-). I’ll be writing an update on this in a few months, I imagine.

Note: I wrote this several months ago but am just now posting, fwiw.

Cover Leaf

In this riveting biography of Dennis Rodman, Wolf Blitzer details the quest of the NBA star of ‘90s fame to save the future, a future where all Americans speak Korean and worship the God of Olympia (Rodman himself), second only to the Eternal Leader, Kim Jong-un.

Based on 90 hours of face-to-face interviews of the future Dennis Rodman (who has now come back to the present and married the only person worthy of himself — the current Dennis Rodman), Blitzer chronicles Rodmans’ journey, which begins on a crisp Saturday in October. A black van screeches to a halt not three feet away from Rodman, on an allegedly empty street in LA. When the van’s sliding door opens, Rodman is pleasantly surprised to see his second-best-only-to-Kim North Korean friend, an old-guard lieutenant of the DPRK and esteemed Korean intellectual, Richard.

When Richard debriefs Rodman on Kim’s plan to cryonize Korea’s Black Hope, a riveting tale of friendship and betrayal spirals out of control… eventually, when Rodman is decryonized 500 years later.

Nominated for the 2018 Nobel Prize in Literature, Dennis Rodman: A Biography will keep you on the edge of your seat and awestruck by how one Chicago Bull from Trenton will have saved the world in the would-otherwise-be-coming Kimpocalypse.

Review

Five stars

Blitzer’s masterful retelling of the not-so-distant almost future is bound to make readers stop to think about how far we’ve come in what some commentators have called a post-political world.

Although President Donald Trump is not mentioned on the cover leaf, his multiple colossal failures play a prominent role in the would-be coming global nuclear destabilization. We have already seen dozens of escalating speeches by Trump in regard to North Korea. Perhaps it should not surprise us that the Mentally Deranged U.S. Dotard, as Trump would have officially been referred to in the future North Korean world order, would have launched a nuclear bomb at North Korea in a fit of rage after Arnold Schwarzenegger’s The Apprentice finally would have surpassed its old ratings under Trump.

But this biography is not about Trump or Schwarzenegger or Kim Jong-un. It is about one man and his will to save the future of Planet Earth as we know it. It will cause the sports world to reimagine Rodman’s career in light of the darkest historical timeline, which he singularly prevented through a mixture of basketball skills and worldwide fame.

This biography starts where it matters. Not in Trenton where Rodman was born nor during his back-to-back winning seasons for the Chicago Bulls — but rather in the year 2019, the year Rodman is frozen alive inside the Glorious Sanitarium of the Eternal Revolution.

The friendship between Rodman and Jong-un is notoriously well known, but Rodman’s biography puts to rest any speculation on the extent of the two world leaders’ friendship. In one of the most emotionally intense chapters of the book, a scene of love and rage is on full display as we are taken to Rodman and Jong-un’s reunion of 2019. Without giving away too many spoilers, Rodman did not want to be cryonized but eventually obeyed the Eternal Leader, all while scheming the dictator’s eventual fall.

We are taken into the future and back again, through picturesque Korean jungles in search of a mad scientist’s 5000-year-old time machine invention, to high-octane chases atop self-driving Uber cars. (Yes, Uber still would have existed in the darkest timeline.)

Dennis Rodman: A Biography is the most important book of our time. You will be held captive from page 1 to 843 by a narrative that’s not only wildly entertaining, but also sure to reshape our sociological wisdom for years to come.

One of the first things I had to do as a newly minted web developer was make elements on the page reactive beyond what I could do with my months of study of JavaScript and jQuery. I was introduced to Knockout and Vue at around that time, and I’ve tinkered a little with React.

Vue has been by far easier for me than React, mainly because the setup for Vue is much like other JS libraries (Slick, for instance) that I’m used to including on a project. That is, you download a file or folder and include it in a script tag on your web page, before your main script. Vue even looks a little bit like Slick with how it requires calling a function that just takes an object with properties to define your configuration. No ES6 classes, no npm or Webpack build processes to figure out, if you don’t want to mess with all that. Vue also can be easily used with the HTML you already have; no need to learn JSX.

The part about not having to compile code is the part I really like. I have been working on an app idea recently that requires a lot of compiling, and I’m just kind of sick and tired of it. Whenever I can, I try to go back to the good old days of my first web design training, when I could just write some files and be done, like magic.

For this reason, for the past week and a half, I’ve wondered away from my side project to do a funner side side project. That’s what reactive libraries were made for anyway, right?

The side side project is titled simply “Matt Watson’s Games,” and my idea was to push Vue components with string templates as far as I could without resorting to an npm build situation. I decided to give up .vue component files or any kind of URL routing or store state management, apart from whatever I might be able to write without using an npm package. (Not even any scss, so the new CSS variables ended up being super handy.) There’s just something I like about being able to code static files with plain old css, js and html, upload those few files to a server, and call it a day.

There is a live version running at mattwatson.org/games, and the repo is here. I’m in the middle of coding Mancala right now. All the games are purely frontend and meant to be played by two people on the same device. Data-persisting multiplayer games are completely another kind of project I won’t be doing any time soon, although I did just get a copy of the 447-page book HTML5 Games: Novice to Ninja by Earle Castledine. We’ll see…

I plan to come back to this project occasionally and keep adding games… until I don’t anymore. For every game, I make a new file containing the necessary Vue string template components. Not a very pretty system, but not bad considering there is no build system. And it’s a lot of fun to work on.

I haven’t been updating this blog much for the last two years, partly because it’s a blog and that’s what you do with blogs, and partly because I’ve spent the last two years doing a lot of things and making a lot of changes to my life, personal and professional.

As many of you know, I’m getting married in July to the love of my life, Dr. (!) Anna Wan.

Engagement photo.

We’ve been dating for I suppose long enough now and will be living our life together in the Hub City, of which, if you’ve landed on this web page from some random Google search, you might think to be New York or New Orleans or New England or some other very non-new city. In fact, the Hub City is right here in our neck of the woods — Hattiesburg, Mississippi!

Another big change has been in my career. In the summer of 2016, I was getting along with pursuing a PhD degree in Spanish literature in the great state of Alabama… where there are lots of BBQ…

Dreamland in Tuscaloosa
Dreamland in Tuscaloosa

While I learned a lot there and became a better Spanish teacher and reader, I decided to leave the program early and pursue a job I was offered at Mad Genius in Ridgeland, Miss., as a web developer. It was something quite new for me, but I have fallen in love with coding and am thrilled to have been given this opportunity. My brother also works there and has been teaching me his Jedi ways.

So, from here on out I’ll be posting more informal chatter than I have before (trying to get away from Facebook as much as possible anyway). The switch to coding/programming has been a two-year transition, and there have been a lot of tidbits I’ve been thinking would be fun to blog about. I’m sure I won’t update as often as I want to in this moment, as I sit here writing at 11:10pm on a Thursday, all psyched up about blogzz. But hopefully, I can keep it in a consistently barely revived state. I’ll try tagging or categorizing my boring coding posts so that you’re forewarned if that’s not your thing.

The late French philosopher Rene Girard was known for his theory of mimetic desire — that we desire something after seeing someone else desire it, then fight over it, then find a scapegoat to blame all the aftermath on so that we can go back to being at peace with one another.

Earth’s resources are limited, inhibiting the fulfillment of all of our desires. Without a fitting scapegoat to blame, we turn to fighting each other and power grabbing.

A lot of bad things happen in Mississippi, as I suppose they do anywhere in the world.

Change occurs rarely here, but there is one change agent that always seems to stir up things from time to time. That change agent, and all-around scapegoat, is Yazoo clay.

You see, Mississippi is home to the Yazoo Formation of the Jackson Group, a “fairly homogeneous unit consisting of blue-green to blue-gray, calcareous, fossiliferous clay, cropping out in a northwest-southeast trending belt across nearly three fourths the width of central Mississippi.”1

In fact, it’s so fossiliferous that great big prehistoric whales have been found here, such as the Basilosaurus, our state fossil. Or Cynthiacetus maxwelli, discovered in Cynthia, Miss., by Britt Maxwell, an engineer who blogs about things like Yazoo clay.

I grew up in the clay region. I lived the life of a city slicker in the metropolitan that is Jackson, so I have hardly ever seen or touched this clay personally, but I heard about it so often it feels like I’ve been digging in this stuff my whole life.

Is there a crack in the wall? Is the doorknob jammed? Do the closet sliding doors not meet in the middle exactly right?

It’s the Yazoo clay.

This special dirt, according to Maxwell’s blog, once thrived on other lands. The polygon cracked surface formations of deserts in parts of Texas, called hogwallows, are the result of prehistoric Yazoo clay-like conditions. Maxwell’s extensive blog posts on this subject show that even Mars may have had Yazoo clay, because it has hogwallows too. It’s everywhere!

Or at least it was everywhere. Texas and Mars are better off nowadays. I’ve been to Texas, and they have massive, high bridges the likes of which I’d never dreamed living here in Mississippi. The Martians are said to have better technology too. I choose to blame that on our wet soil and clay, which is very much still alive.

Sometimes I even wonder if the federal government had a hand in the shifting mess we’re in. That’s right, the feds. I was having dinner with someone in Hattiesburg the other day who told me half-jokingly that the foundation problems of a new residential development down there could be related to the atomic bomb they detonated 30 miles southward.

What?

Yes. “Just google it: ‘hattiesburg atomic bomb,’” he said and went back to eating his pasta. It’s true. It’s all true.

Home foundations are one thing, but the roads — they are the real doozies. The roads in central Mississippi are terrible.2 Everyone else in the nation has immaculate roads compared to ours. Many a concerned citizen has brought the issue up before city councils and boards of aldermen. Investigations have been initiated to find the councilmen and -women responsible for all the potholes and bumps. News reports abound, calling attention to the matter. There is much finger-pointing to go around.

You can blame potholes on city governance, a faulty foundation on the construction company, and a door jam on the guy from Lowes.

But I like to think that on most days we just carry on with our lives and blame it on the Yazoo clay.

Because you can’t stay mad forever, and Yazoo clay, lying entombed under the ground and out of sight, is as good a scapegoat as any.

Matt
March 26, 2017, Byram, Miss.

  1. Stover, Curtis W., Ross D. Williams and Charles O. M. Peel. “Yazoo Clay: Engineering Aspects and Environmental Geology of an Expansive Clay.” Circular 1. Mississippi Department of Natural Resources, Bureau of Geology. 1988. 

  2. As well as the rest of the roads in the state, but the clay is in central Mississippi, and that’s what I’m talking about right now, so don’t change the subject.