A New Year, A New Name

Those of you receiving the article in you email box will notice a new name: “French Settlement Disease” with the formal name being Hereditary Spastic Paraplegia (HSP).

We are there.

Not much is known about this disease, other than what is released in this article published by LSUHSC.

I spoke to my neuro and he said this was my diagnosis in the beginning and he is reaffirming it. I should do so with my family tree.

I am the genealogist in the family. However, I worked the tree with old tools. My neighbor, sharing much of my family tree, and with modern tools, has accomplished more conclusive research.

We did come from Livingston Parish. This is one of the requirements for HSD. HSD is thought to come from a single German couple that moved to French Settlement in Livingston Parish. Strangely enough, my wife had purchased a genetic test for me, prior to me scouring my medical records.

Within a couple of months, we will see if I have German Ancestry in my DNA. That would fairly well cinch the diagnosis. As I have the symptoms listed in the document and my treatment plan is exact as stated in the document, we will have little more to discuss.

I am a paraplegic.

I broke down yesterday. I was stricken to my bed most of the day. My wheels had to carry me most everywhere. The pain in my back and legs were immense. I would say even with my strong meds the pain level was above eight.

In part, I did it to myself. I enjoyed Christmas, a bit too much. I stressed my muscles. I went to a party and insisted on using my cane as long as I could. This contributed greatly to my physical downfall and current condition. I feel I will be in my chair for a while now, my legs and back still screaming at me.

My internal question is this: Should I forgo walking and just rely on the chair from now on? Or do I continue as I do, walking when I can and then recover for days afterward for exercising my temporary gift of verticality?

In Christ,

Jay C. “Jazzy_J” Theriot

2018 Dec 6th, Update

#KennedysDisease #SBMA #Success #ExtremeSpasticity, #AcceptanceWithoutSurrender

One month has passed since my last article and considerably longer since my last update.  Much of the reason for not publishing is due to the fact that I became disgusted with thinking about my condition and tried to focus my sights on getting things done.  I’ve had a few successes and my condition remains.

What we know:

The condition is a slow-progressive degenerative neuro-muscular disease causing many issues throughout my person.  A gross number of my muscles are implicated.  We thought they were just skeletal muscles, but now non-skeletal muscles are being affected as well.  The involuntary spasms occur globally and continuously.  Most are on a small scale resembling internal tremors, which I call micro-spasms.  Others are insanely painful and instantaneous, lightning spasms.  Some spasms efface as an undulation and finally some as cramps and twitches.  All are damaging. Continue reading “2018 Dec 6th, Update”

Internal Tremors Suck!

#KennedysDisease #SBMA #Success #ExtremeSpasticity, #AcceptanceWithoutSurrender

At the time of this article’s writing, I have been experiencing internal tremors for over 16 hours without a break.  I was able to sedate myself to get some sleep, but every second of every waking out they are there.

Through EEGs we were able to discern the cause is not a continuous seizure.  Like everything else about this confounded disease, they are enigmatic.  They just are.

We believe their origin is a continuous series of microspasms of all the muscles in my body.  There is no end to them.  My neck gets the brunt of the damage caused by them.  It must support my head.  With the internal twitching of my eyes, my neck constantly is trying to make corrections.  I can’t help but to put my head on a pillow after tens of minutes of it being upright.

It is during these periods of increased internal tremors that I am effected most emotionally.  I don’t want to be around people.  I don’t want to interact.  The tremors are minute enough to not be seen.  However, they are wreaking havoc on my body.  Thus, interactions with the outside universe is very divisive .  If you are unaware of what is going on in the inside, it is impossible for you to understand my reactions to a seemingly invisible assailant.

I get scared.  I have objects that comfort me by giving me external foci. Of course, I have my penguins.  I have music. I play my specially chosen video list.  I chose them subconsciously, but after much introspection, I realize the connective tissue which made them chosen.  The soundtrack is constant.  There are very few surprises in the audio track.  In some there are gunshots, but that is only for a very small portion of the movie.  All the dialog is soft-spoken.  There is no yelling.  For action movies, they are sedate.  These external points of focus allow me to escape myself and rest.

Monday morning, I had a destructive event.  The spasms were severe enough to tear muscles over a good portion of my body.  My head and neck are the only portions of my body that did not suffer damage.  I am still trying to recover.  The tremors are spasming on these damaged muscles. Consequently, my pain has not dropped below level 5 all week, even with narcotics.  I am fatigued, mentally and physically.  Nonetheless, I have been able to produce a few photos this week showing the beauty of the minuscule.  I look forward to the day when my body can absorb the demands of astro-photography again.  There is just too much equipment and positional demands to take photos of stellar objects at the current time.  Looking at the macro-environments are much less demanding.

I don’t have an ending for this post.  I’m sitting here, writing, shaking and drinking a cup of coffee.  My fingers are numb.  I use muscle memory honed over decades to effect typing.  My pointers search for the indices on the “F” and the “J” keys allowing my fingers to fall into place on the other keys.

Life goes on.

In Christ,

Jay C. “Jazzy_J” Theriot

Jay C. Theriot can usually be found as “Jazzy_J” on the IRC Channel #ExtremeSpasticity on Freenode.net –> see: irc://irc.freenode.net/ExtremeSpasticity. You will need an IRC Client such as HexChat to connect.


An Exploration of Luke 4:24 v. Kennedy’s Disease

#KennedysDisease #SBMA #Success #ExtremeSpasticity, #AcceptanceWithoutSurrender

Luke 4:24 New Revised Standard Version (NRSV)

24 And he said, “Truly I tell you, no prophet is accepted in the prophet’s hometown.

I can never doubt the truth of that statement.  The people that are closest to you are the ones that believe you the least.

There are many reasons for this.  I believe the major one is they know your history.  They know who you were, not who you are.

I remember the times that I was not disabled, and the idea of a slow-progressive neuro-muscular disorder (NMD) of genetic origin was not in my vocabulary, nor my dreams.

Now, the reverse is true.  NMD is in my vocabulary and those times are only in my dreams.  I wake to the horror that is me.

Kennedy’s Disease, as is the case of many NMDs, is a thief.  The disorder robs you of your health, your abilities, your finances, your confidence, your loved ones and your independence. I hate it, but it is who I have become, and I must accept the evil that is within me.

My closest relatives, do not have to do the same.  They do not see the evil inside.  I am very frank on this blog.  However, there are attributes of this disease that I won’t even write.  I save those for my wife, kids and closest of the closest of people.  Only those that have seen the horror of someone they love lose themselves to a disease can even begin to accept.  I originally wrote “understand”, but that is a lie.  I am the target of this NMD and neither my neurologist, nor I, understand this.  Thus, it is an impossibility that anyone can truly understand this.

I think of Jesus in His days.  What it must have been like when he realized he was different than everyone he knew, even the person he thought he was.  His parents tried to tell him he was just like all the other kids.  He always tried to fit it, but he never did.  Blaming himself for the inability of others to see and respect him for who, or what he was, he must have lived a life of solitude.

I must insist that, although I am comparing my mentality to Him, I do not consider myself even worthy to gaze at His feet, less clean them.  No, I am not He.  I just I an idea of what a minuscule portion of what it must have been like for Him.

One-by-one, my friends gave up trying to teach me how to play sports.  Football, baseball, softball, tennis, volleyball, and the list goes on.  I was always uncoordinated and spastic. I rarely play video games that demand fast reaction times.  I rarely survive the first round.  The only action-based game I ever partially excelled at was the Atari 2600 version of Pac-Man.  I memorized the first levels of the game.  There was no reaction time.  I just knew the pattern to travel in to avoid the ghosts.

I learned muscle memories and driving techniques.  I have been in a side-spin on black ice in a curve on the side of a mountain with an oncoming tractor-trailer and one behind me. I recovered the vehicle and was lauded praise by my passenger. I never wish to do that again.  The snapping back into the forward motion broke the differential of my BMW.  To say the lease, it was terrifying.

Fast forward twenty-five years, and a trip to the local grocery store has me upset if all the handicapped parking spots are taken.  I wonder if I can make it inside to the mobile carts before I have to sit on the cement because my legs have given out.  I leave, and go home, saving the shopping for more able-bodied members of my household…that is, my wife.  When my time is done, she should receive her angel’s wings, not me.

Where I am finding success

Success is how high you bounce when you hit bottom.
— Gen. George S. Patton

Sometimes I get accolades with the work I do putting my church on-line and creating accommodations for others so that they may attend services, virtually. It started as an ideal.  I seemed to have a lot of friends considerably older than I.  I would watch them drop from the ranks of service and then die.  I saw the Internet publication of the services as a way of giving them comfort.  That was my mission, that became my goal.  My phrase was “Computing Through Christ”, followed by “because I can do all things through him who strengthens me.” an alteration of Philippians 4:13. I thought it was doing it for them.  I enjoyed the integration of technology.  I had received state-level certification in teacher-training years ago and the concept always fascinated me.  The idea that technology should be seamlessly integrated into our mundane activities, always with a backup, so you can still get the job done, without electricity, was a killer theory.  I have since forgone the “without electricity” bit as I realized its existence was due to bureaucratic mandate and not true reality.

Little did I know, a few years after I would take up that path, my own journey would become.  I guess God saw unrefined talent and thought if I were a little more involved, my efforts would be better refined.  Indeed, I prayed that I would be able to be more involved in the church.  I couldn’t figure out how that would happen, but I knew I wanted to serve others.  Well, here I am.  That prayer was answered.  Largely, the church comprises a large part of what I have.  I was able to attend only a few services over the last two years.  Even now, I am dedicated to attending.  My attendance is about 50%, at best.  For me to attend, two days recovery time is required.  The exertion needed is incredible.

For that reason, I am designing and programing systems that will record and forward the service to my servers for me to produce, when locked in at home, and unable to attend the service. I have achieved success in the individual parts of the plan and now, I am assembling the pieces as fast as my body will allow.  However, what used to take hours, takes weeks.

But, with the power of Him, who strengthens me, I will get it done.  Although ever-present in my mind, the Nazarenes do not control who I am, or do.  They only control the memory of who I was, because I let them.

In Christ,

Jay C. “Jazzy_J” Theriot

Jay C. Theriot can usually be found as “Jazzy_J” on the IRC Channel #ExtremeSpasticity on Freenode.net –> see: irc://irc.freenode.net/ExtremeSpasticity. You will need an IRC Client such as HexChat to connect.

The Benefit of a Cryochamber

#KennedysDisease #SBMA #Success #ExtremeSpasticity, #AcceptanceWithoutSurrender

My condition has  steadily been degrading over the last 2-3 weeks.  I figured out why.

The window A/C unit used to cool my room into the 50s slowly died, going unnoticed until death.

The unit was replaced with a stronger one, with a major flaw:  The control would not let the unit cool below 64 degrees Fahrenheit.  I moved the thermocoupler into a zipper-seal bag and placed them into a cup of warm water.  Another modification to this design will transform it into an Alexa-controlled setup.  I envision a cup warmer heating a cup of Epson salt with the zipper-seal bag in it.  The electricity switched through a smart plug, timing controlled via programming.

The end result was that I feel better this morning than I have in probably two weeks.  My muscles are sore from all the elevated contortions I’ve had, but I slept like a rock! 

I remain in disbelief at the volatility that this disorder has put my body into, and the extreme conditions it must be put through to exist.

In Christ,

Jay C. “Jazzy_J” Theriot

Jay C. Theriot can usually be found as “Jazzy_J” on the IRC Channel #ExtremeSpasticity on Freenode.net –> see: irc://irc.freenode.net/ExtremeSpasticity. You will need an IRC Client such as HexChat to connect.

Two contradictory goals

#KennedysDisease #SBMA #Success #ExtremeSpasticity #AcceptanceWithoutSurrender

Two contradictory goals I am failing to remove from contradictory stances:

1) Lower, manageable electric bills.

2) Medical necessity to have a very cool environment. (65 degrees.)

Background:

1) Lower, manageable electric bills.

Currently, our electric bill hovers around $300. This is up from $125 before I installed a window unit to keep my room cool.

2) My condition, similar to multiple sclerosis, is exacerbated by higher temperatures. I have found that temperatures close to 65 degrees is my best operating temperature.

Additional information:

1) Lower, manageable electric bills.

  • The central HVAC would need to be set into the 60s to get the house at a more livable state for me. This would considerably raise the monthly electric bill. Even with payment averaging, the dramatic rise would continue to be so. In South Louisiana, our winters are like other’s summers.
  • Visitors to the house would need to be provided blankets.
  • Other occupants of the home would need to use electric blankets at night so they could sleep comfortable.

2) Medical necessity to have a very cool environment. (65 degrees.)

  • My muscles contract in temperatures above 68. They contract all the time, but above 68, considerable pain is noted.
  • My ability to breath is impacted. My chest tightens and I feel like I can’t fill my lungs. It is more than a feeling. My vital capacity is lowered, however O2 levels remain between 86% to 97%. It follows the traditional bell-curve, centered on 94% VO2.
  • Functioning outside of my room is problematic. However, if I don’t want to be in a 14’ x 14’ prison, I spend as much time out of my room. Retreating to it only when I can no longer perform at any level.
  • My room is a mess. Largely because when I am in it, my ability is marginalized. I spend most of my time in my room, as my ability is marginalize about 95% of the day.

Contradiction:

Experiments have shown decreasing the overall temperature of the house provides me with a greater amount of freedom of mobility and overall quality of life. However, the impact on the electric bill is dramatic. I tried it one time and the bill spiked to almost $400.

How can these two opposing goals be rectified? I’m at a loss.  I ask for comments and suggestions.

In Christ,

Jay C. “Jazzy_J” Theriot

Jay C. Theriot can usually be found as “Jazzy_J” on the IRC Channel #ExtremeSpasticity on Freenode.net –> see: irc://irc.freenode.net/ExtremeSpasticity. You will need an IRC Client such as HexChat to connect.

The Surrealilty of it all

#KennedysDisease #SBMA #Success #ExtremeSpasticity, #AcceptanceWithoutSurrender

Something I don’t generally write about.

My 9 pm medicine alarm woke me from deep sleep. I had a lot of issues, so I had lain down at 7 pm.

I was in the middle of a dream. In the dream, I was normal and had all my facilities. I woke and was confused.

I could not figure out why I could barely move or why I had a wheelchair at my bedside.

All my mind could figure was that I was in an automobile accident. I hoped that I hadn’t killed anyone. I panicked and began asking my wife what happened. She didn’t understand what was going on and so told me that my alarm was going off and I needed to take my medication.

In a minute or so, I realized where I was and I wasn’t in an accident.

This is some of the things I have been reticent to write in my blog.

You relive the horror of what is you over, nearly every time you awake from a dream.

In Christ,

Jay C. “Jazzy_J” Theriot

Jay C. Theriot can usually be found as “Jazzy_J” on the IRC Channel #ExtremeSpasticity on Freenode.net –> see: irc://irc.freenode.net/ExtremeSpasticity. You will need an IRC Client such as HexChat to connect.

I can type again! Yeah! and fallout from yesterday.

#KennedysDisease #SBMA #Success #ExtremeSpasticity, #AcceptanceWithoutSurrender

Finger Resetting

Evidently in yesterday’s episode, my body decided to dislocate the ring finger on my right hand and pretty much all the fingers on my left hand.

I woke up during the night and before I was fully awake, manipulated a finger or two on my left hand and they reset properly.  Fully awake, I continued to reset the rest of my fingers.

This is not as horrible a process as you would think.  What I do is bend my fingers a little.  Then twist them back and forth until they reset.  You may have to do each finger a few times until it gets into the perfect position.  Relief will be immediate.

Fallout, remnants from yesterday’s episode.

Strained or Pulled muscles cover about 60% of my body.  With the help of my meds, I’m walking with a walker this morning.  I expect to be in the chair later.  I’m shocked that I’m doing as well as I am.

I do want to shoot photos of the moon.  It is a clear night and the moon is full.  However, I’m not that good at the moment.  I do have to say photography is a heck of an incentive to work myself to get better.  I just wish I could enlarge the world in which I can shoot photos.  I’m hoping with the work the Myetin is doing, I’ll regain function enough to actively shoot in locations away from home.  One target I want to shoot, in activism, is the Apple Snails that are invading our environment.  I would like to do a spread, with researched articles, identifying why this invasive species must be stopped.

A Debt of thanks to a neighbor

During the onset of the event, I could feel it coming on and coming on strong.  I had no spasms!!! But, I could feel the muscles pulling, and contorting and becoming steal.

I quickly sent an email to the managers of a fair that my game is going to be played at today. I told them I was likely not going to be there this morning.  However, I think I may be able to.  My daughter is getting service hours, so I have to drop her off.  I’m going to load up my chair and a laptop and see if everything is going well. Then return home and monitor the servers.

I then messaged my neighbor, asking her to come over for coffee. I was having an event and it was scaring me.  She came and stayed with me until things settled down.

I don’t always get scared during episodes.  However, this one was different.  It lacked spasms, which was a great thing.  Even though there were no spasms, the muscles were tightening up quick and hard.  One of the earmarks is tears flow from my face due to my facial muscles tightening around the tear ducts.  There is no controlling it.  They just flow.

I had a few cramps and twists. My abdominal wall contorted under my pump which, not usually painful, completely freaks me out.  The muscle wall contorting against an embedded metal object is really freaky.

After the episode completed, my wonderful neighbor went back to her home.  I cannot express how thankful I am that she was here.

I knew things were going to hurt later.  She left, I ate and then finally slept.

When I woke up.  It was payback time. My body realized it’s potential to inflict pain.  I superloaded my body with protein, muscle relaxers and pain killers, causing my muscles to relax and heal.

This morning, I am thankful to God, my neighbor and modern medical professionals.  They are the true heroes in this event.

In Christ,

Jay C. “Jazzy_J” Theriot

Jay C. Theriot can usually be found as “Jazzy_J” on the IRC Channel #ExtremeSpasticity on Freenode.net –> see: irc://irc.freenode.net/ExtremeSpasticity. You will need an IRC Client such as HexChat to connect.

Hubris will get you, every time

#KennedysDisease #SBMA #Success #ExtremeSpasticity #AcceptanceWithoutSurrender

Never brag.  Accept what you have and move on. 

I actually made plans today.  I felt great when I woke.

Now, I can’t count the pulled muscles.  There are at the least, two on every appendage and more on my left leg.

The disease is a thief.  It hurts to push the keys.  The muscles from my elbow on down on my right arm got angry.

Ciao. for now.

Is the Myetin working on my efferent nervous system?

#KennedysDisease #SBMA #Success #ExtremeSpasticity, #AcceptanceWithoutSurrender

About two months after I started taking Myetin, I regained my afferent (sensory) nerves.  I had lost feeling over about 70% of my body, my sense of smell, taste and I was having hearing issues.  All those have returned, although, there is no sense of “listening.”  My lovely wife will vouch for that.

My problem lies with the efferent (motor) nerves.  How will I know if the Myetin is repairing those nerves?  That is the whole point.  Regaining function.

With the afferent nerves, I became acutely aware when the specific nerves regained viability.  The area would become hypersensitive. It was a funny time.  I sometimes felt like I could literally feel people looking at me. Then, over a period of 18 – 36 hours, my brain would calibrate the response and sensory input would normalize.

But, what metric will slap me in the face to let me know the motor neurons are coming back?  What should I expect?

I’m not certain, but I think I may have an answer.  The last couple of nights, this one included, I wake from a dead sleep burning up.  Not drenched in sweat, but just uncomfortably hot.  I feel warm all over.

Heat causes my muscles to violently cramp and spasm. I sleep in temperatures in the 60s to help prevent them.  The 60s have not been low enough for the last three days. 

I am recovering from considerably damaging my muscles last week.  I’ve been in a chair most of the last two weeks.  I can walk short distances, but then I pay.  I can’t say definitively that I’ve regained function in my muscles because of the path I’ve traversed in the last couple of weeks.

I’m not sure what is happening, but it is happening. I dream of a day when I can travel a distance to visit friends.

In Christ,

Jay C. “Jazzy_J” Theriot

Jay C. Theriot can usually be found as “Jazzy_J” on the IRC Channel #ExtremeSpasticity on Freenode.net –> see: irc://irc.freenode.net/ExtremeSpasticity. You will need an IRC Client such as HexChat to connect.