Article below

Main takeaway for me:

The research is population based. Inherently this is flawed due to the fact that not all countries have the infrastructure to accurately account for ALS prevalence in their population. Leading to once again systemic under-reporting of this horrible disease. Even in the US ALS is captured predominantly on a voluntary basis. This is equivalent to asking the fox how many chickens are in the coupe, laughable.

I get that ALS is categorized as rare and not worthy of the attention other conditions, the science supports this stance. However… however! The financial toll this particular disease creates on families is without comparison. Until this burden is impactful to the government it will continue to remain the burden of families to navigate the cost and lack of viable treatments.

At this point in time it appears that treatments would lead to reducing the profit margin for services and goods for pALS. It appears this way, I prefer to think that there is not a Cabal scheming to keep us from treatments; that’s only in the movies.

I do believe that the science has not coalesced with the heart and soul of ALS to break down barriers standing in the way of viable treatments. What’s the solution? I wish I knew.

Make your own conclusion, read, digest, interpret.

TJ&O

https://www.karger.com/Article/FullText/493386

ALS and choices.

What choices do you mean Juan and Only?

Well I wish I could say, choices in treatments! But that is still a dream.

What I am referring to are choices in our daily living activities. I for one have failed from time to time and created unsafe scenarios. I guarantee you as a pALS have done the same. What is the cause of this? Control, stubbornness, fear? I venture to say all the above.

These choices, we must remember, affect not just us, but our care team. For example:

Wearing a catheter, which I resisted, needles to say, it is such a “relief” to everyone! Not having to rush or fear an accident.

Exerting yourself needlessly, i.e. insisting to transfer independently at risk of falling or exhausting yourself.

Using the walker/rolator even after you fall. The fall was the warning that your limbs are no longer able to support you or control the walker.

Three examples of situations that put us at risk, but also scare the crap out of our caregivers. Not to mention the scolding we are in for,😉. I’m sure there are more examples to add and please do, to help us identify them in advance.

There are also many big choices to make, very personal choices. However these daily ones are harrowing now. Ultimately we need to consider others in our daily activities and the choices we make. Don’t listen to that little voice when it’s whispering, “you can still do it”. When you come too, you may not be where you fell, but in the back of an ambulance.

Make good choices.
Make safe choices.

TJ&O

• 

I didn’t see myself here, at this point, in this way.

I saw Meg and I, now that she has retired, preparing our first and subsequent destination to hit with our RV. Or traveling the world with and without the kids. We had even toyed with the idea of working overseas, or fostering/adopting more children. Anything but this and what’s to come.

You can plan all you want, many plans, simple plans, complex detailed plans. What happens when those plans don’t pan out? Does your world collapse, universe implode or do you shrug it off? One can plan for many contingencies, in the course of our lives “recalculating” is inevitable. What is one to do?

For me, I must put my trust and faith in God, not an easy task, we are creatures hell bent on controlling everything. I have no control over my ALS, only how I respond to it. How I respond effects how others also respond; my family and friends. I can influence the pace of my progression with available treatments and the quality of my life, but little more.

So I choose to plan on living as best I can, one day at a time for as long as I am given. I see this now, this is where I am at this point, in this way.

TJ&O

Bruised Pride, modesty, surrender… terms that every ALS patient has to accept during the course of the disease.

Bruised Pride… because slowly your own body denies you the ability to do simple things for yourself. You have to set your pride aside and come to terms with needing assistance with everything. There are many conditions that require total and complete care many are due to unfortunate accidents some are due too poor choices in life. However ALS and many other MND conditions are simply one’s own body attacking itself. As if the body one day wakes up and says, f*** you I’m done! No more Independence for you. Time for you to become a lump…

Modesty… this is another word you have to come to terms with because you have to set it aside. It doesn’t matter how much of an introvert how modest you are or how much of an exhibitionist your modesty is out the window. It doesn’t matter how you feel about your personal appearance you have to accept the fact that your arms hands and legs won’t allow you to care for yourself. If you don’t want to stink to high heaven someone needs to wipe your ass when you use the toilet. Someone has to shower you and eventually they will have to brush your teeth for you feed you and move you from bed to wheelchair Etc… at this point it doesn’t matter what it’s exposed what matters is maintaining healthy hygiene to avoid medical complications.

Surrender… this is one of the most important aspects should terminal condition. I’m not talking about surrendering to the condition and giving up on life. This has to do with surrendering yourself to others and accepting their love and support. If you don’t, and there are those that don’t, because they allow their pride to get in the way, you will live a miserable existence. Surrendering yourself to a higher power or your faith is a completely different post I will save that for another day.

If you are suffering with a terminal illness do not take the journey alone, do not push your loved ones away. Put your pride & modesty aside and surrender to the love and support that surrounds you.

Peace be with you,

The “Juan” and Only

• 

I have posted this before, sometimes I am reminded that although I use humor to cope there is a very dark side to ALS.

As persons and caregivers with ALS, you already understand this and don’t need reminding. However I write so others can get a glimpse behind the three letters.

While laying in bed last night waiting for my meds to kick in and put me into a chemically induced slumber I was reflecting on my condition. I came to the realization that ALS is just like another condition but only in reverse.

Allow me to illustrate.

Imagine The Following…

Many of you have seen this scene play out on many a motion picture and television shows. A loved one lays in a bed in a coma, surrounded by those closest to them and a robust medical team. When all of a sudden someone in the room notices a little twitch in the patients toe or finger. Everyone in the room comes to life realizing that this is the first sign of life they witness in their loved one. The scene continues, perhaps in multiple time sequences ultimately leading to the patient regaining consciousness followed by their speech and regaining movement. Followed by either a miraculous full recovery or arduous Physical Therapy, a recovery none the less.

This is where it gets dark, in my opinion. Imagine the reverse of a coma. You have all of your faculties and you slowly begin to notice the loss of motor function, it begins with a twitch somewhere in your body. Followed by loss of fine motor function leading to complete loss of the ability to move your extremities. Arduous physical therapy has no effect on your progression, in fact it can actually be detrimental. Range of motion exercises are essential though, in order to keep some flexibility in the joints.

As your condition progresses you lose your voice, your ability to swallow and ultimately your ability to breathe independently. All throughout this nightmare your faculties are still completely intact.

You are now completely immobile, surrounded by loved ones and a robust medical team. Dependent on them for your every need to include mechanical ventilation. If you are lucky enough to have the resources necessary to acquire an eye gazing computer you can still communicate with your surroundings.

So if you want to see a time-lapse of ALS all you need to do is play in reverse a program that follows a patient recovering from a coma…

In essence ALS is a reverse coma or a waking coma. Trapped in your own body, your body being both the perpetrator and the victim.

Sorry for taking a dark turn, I feel the need to share The Good The Bad and The Ugly.

Deep Thoughts From

The Juan and Only

They say ALS/MND is a painless disease. Who is they? Im guessing “they” are individuals who don’t have ALS, or know anyone with it.

I have heard from pALS and know myself this couldn’t be further from the truth. There is pain and discomfort with ALS.

Even though I take supplements and medications to prevent cramps I get them almost nightly. I get them in my big toes, ankles, shins and calves. They aren’t as bad as they used to be, I’m guessing because my body is less responsive to nerve conducion. Small blessings I suppose.

I experience more than fasciculations, I get spasms throughout my body. My neck locks up along with my jaw.
I get spasms in my six pack, don’t laugh its there, just well padded.

My hands, elbows and shoulders will also lock up and I have to power through it. All of these spasms are painful and take my breath away. Strictures due to muscle atrophy and frozen joints will surely follow, thus the importance of physical therapy.

Now recently I’ve heard from pALS having issues with Trismus (lock jaw). Due to no longer speaking, chewing and eating. One more thing to look forward to, joy.

These adove, of course are the physical pains. The emotional, mental and spiritual pains are all together a different discussion. These can and often are the most painful effect of ALS.

ALS is a painless disease… my ass!

TJ&O

It goes without saying that Healthcare professionals should at least have some awareness of ALS. This would be highly presumptive. It would be like saying every professional chef is aware of every recipe that exists. It simply is not possible, Healthcare professionals tend to focus in a specific area of interest to them. And although they do have a vast knowledge of diseases in general they are not required to have in-depth knowledge outside of their area of expertise.

Take for example a neurologist, I don’t know how true this is and I can’t recall who shared it with me. I have been told that a neurologist in the US may encounter 1 ALS patient in their career. I am not saying this to get called out, but to emphasize the overall lack of awareness of ALS as a disease.

There are all kinds of figures floating out there such as:

• there are approximately 30k ALS patients in the US
• the CDC reports 12 to 15k
• every 90 minutes someone is diagnosed or passes away from ALS
• military veterans are twice as likely to develop ALS
  *90-95% of ALS cases are sporadic, 5-10% are Famelial

As you can see it is daunting to keep up with all of the information on ALS. Add to this all the amazing research, clinical trials and endeavors to create awareness and one can get lost in the figures alone.

Back to my original concern, awareness by Healthcare professionals. I will stick with one area of healthcare, emergency departments. Having worked in Emergency and Trauma myself I can attest to this. I knew very little about ALS.

Emergency departments can be very dangerous to ALS patients in advanced stages. This is due to the fact that if the patient is not able to communicate and the staff are not aware of the patient’s condition trouble will ensue. There are protocols ER’s apply to every patient depending on the immediate needs. For trauma it is spine boards, C- collars and oxygen; all three can be deadly to an ALS patient. Sedatives and painkillers can also compromise an ALS patients respirations.

Covid turned everything upsidedown and made impossible to remain with pALS when admitted last year. I suspect a number of pALS passed not from covid, but from the effects of limitations of access to personal Caregivers while in hospital.

So what can you do to lessen complications from ER visits and EMS services.

• As your condition progresses make contact with your nearest First Responders and offer to educate them on ALS.
• Have either a written statement that includes medical history and meds.
• Never throw away pill bottles if you use a pill organizer! If admitted to hospital they will require original container to administer “YOUR” own meds.
• Take all meds to hospital, Hospitals do not stock every medicine, they may not have access to highly specialized/unique meds.
• Plan for communication challenges. *Always have a low-tech method of communication available if your pALS can’t communicate.
• Prepare a packet of medical/legal documents. Better yet compile everything on a tablet or app, accessible to anyone needing it.

!!Most of all remember that EMS and ER staff are extremely well trained and will always focus on life threatening needs first!!

For many years there has been a push to establish a national hotline for healthcare professionals when treating the ALS patient. Last I heard is that the logistics for this are too daunting.

I could go on but you get the message. Awareness and education is important at every level.

I went for a run today, after waking, through the neighborhood. I stretched then opened my front door and was greeted by a cool breeze and the overcast morning.

I started out the door down the driveway and onto the asphalt, it had a slight sheen to it’s surface from a light drizzle. My feet struck the ground rhythmically, a slight ache starting in my quads, warming up with every footfall and contraction of the muscles. Up the street, intersection… turn left, deeper into the community. Homes on either side, grass glistening as the suns rays strike the green blades. Yards well manicured, with an occasional obstinate weed breaking the surface; as if to declare “I’m still here!”

I continue on my quest for a good stride as I coast through the quiet streets. Crossing the road from time to time to give way to a neighbor walking their dog; politely nodding, “good morning” expressed in the gesture. They respond in kind, as they subconsciously tug on the leash.

I navigate through the neighborhood, left, right, cul-de-sacs and so on. Having reached my point of return, I retrace my course. Again encountering friendly faces, wagging tails and lolling tongues. It’s a beautiful overcast morning, a beautiful day.

I approach my house, come to a walk and begin a brief cool down. Relishing the ache in my legs and the burning in my lungs as my body normalizes. I take one more look at the morning sky before opening my front door, as I do, I am enveloped by the aroma of fresh brewed coffee.

I ran through my mind this morning.

It’s my only way due to ALS. It feels real every time though, no not the running, but the ache in my legs and the burn in my lungs… because of my ALS.

TJ&O

ALS: What does it demand?

Yes we know it demands physical functions and abilities, but what beyond this does it demand?

Do you have a moment?
Ok, thanks…

ALS demands of the person afflicted to abandon selfishness. It demands pALS to abandon Ego. It also demands us to abandon hopelessness; for all we are left with is hope.

Why selfishness? At the onset once we resign ourselves to this disease we in a sense want this disease to afflict not one more person. We want to not burden our loved ones with a protracted condition. On the other hand we want to please our loved ones by also being here and present with them. Don’t even get started on the selflessness of our caregivers/family.

Our Ego, well ALS is a big gut punch to our Ego. It brings us to our knees and humbles all in its presence. There isn’t much more to say about this, ALS strips you bare of any and all that you thought was important.

Hopelessness, at some point after feeling hopeless for some time, a shift happens. Hope begins to break to the surface. Why? We are left with nothing else. Currently no viable treatment, yet we fight by raising our voices, by just being obstinate enough to just be present daily. What if not hope and hope alone creates this drive.

So what is left? One choice in my eyes…

Live Life with Abandon!

TJ&O