A fellow pALS once shared their frustration with ALS, saying that they felt as though they are melting away.

In a way they’re not wrong; most pALS experience a heightened metabolism due to muscle breakdown. So we are melting, sort of. However this applies metaphorically also.

Our families see us slowly, some quickly, disappear right before them. For those suffering dementia, well sadly they or who they were disappears long before their body does. This in and of itself is a true curse for the entire family.

ALS having no true treatment, let alone a cure, can’t be stopped from reaching it’s inevitable conclusion. Like trying to hold water in our hands, physical attributes simply slip through our grasp. Each molecule of water representing a function, as it slips through our fingers. Leaving a trail of “us”, followed by the tears of our loved ones.

Essentially our bodies are melting away. Visible to others by way of our gaunt physique, as we become a shadow of what we used to be. This disappearing act with our families as our audience, culminates as a one time performance, no encore.

With this in mind, give it your best, overact–go over the top, live loud! Before all that’s left is but a puddle…

TJ&O
(Dedicated to the Cranbrook Community Theater, Tuesdays With Morrie)

There are times when those near me believe I’m being rude or inconsiderate. Believe me when I say you will know if I am. What is actually happening is that I am most likely concentrating or focused.

Doing this takes on new meaning when living with ALS. How, you ask?

I’m so glad you asked!

Allow me to elucidate.

To a neurotypical person, that’s you, it takes but an instant to actuate your movements. For us pALS, even if completely immobile, because we will stubbornly try, it takes much longer, if at all. We are in fact attempting to accomplish the impossible, movement.

Every bit of focus and concentration is being exerted to move my feet and legs as I attempt to walk with my walker

I am screaming in my head trying to will my body to turn in bed. Or to pull the covers because I’m cold. Eventually breaking down and asking for help.

I am hyper focussed on my cell phone, texting with the only finger still under my command. Trying to finish my prophetic Facebook post before my finger relents and turns against me; typing indescernible text as it takes on a life of its own.

The majority of the times that it takes me a moment to engage it’s because I am grappling with my own body. Having a colorful, expletives filled exchange with my muscles.

You see, I’m not ignoring you, I’m not being rude. I am in fact heavily engaged in negotiations with me, myself and I. If you haven’t noticed “they” are stubborn!

TJ&O

Regardless of the rate of your ALS progression there is one aspect that all of us dread when it comes.

No longer driving.

Noooooo! You said that “D” word!

We all dread giving this one skill and privilege up. The thing we dreamt and fought for, begged our parents for. The liberating act of hitting the road, going wherever the blacktop takes you.

I will confess my family had to force me to stop driving. Even though I had a modified van. The last bastion of independence, so we believe. I do miss it a great deal though. Let’s be entirely honest with ourselves, there comes a moment when it’s simply not safe to wield a hurtling mass of steel.

But my legs are still strong.

The car is modified so that I can still drive.

My reflexes are not effected.

It matters not what we believe, it matters that it is documented in your medical records, Diagnosis: ALS.

That’s it! Nothing else is a valid argument on your (pALS) part.

Dems the facts folks.

Can you with certainty state that your ALS will not affect your ability to safely drive.

Answer: NO

ALS has no consideration, it takes without warning or care. Yes it requires the use of your arms, legs, spacial awareness and healthy reflexes. However ALS can compromise and betray us without warning. A yawn can cause a total body spasm, contorting our bodies.
Limbs can cease to function mid use. Our own saliva can throw us into an uncontrollable wracking coughing fit.

Can you really take the risk? Should you?

As difficult and painful as giving this up is, it has to happen. For your safety, your families safety and the public’s safety.

Don’t risk it, don’t give ALS the satisfaction.

Now you have to be driven to live with ALS.

As we have meandered through the ALS forest on this hike we haven’t shied away from the difficult trails, ALS won’t let us. We simply have to forge ahead and follow the path chosen for us. So what to do when we physically can’t take one more step? When our legs and feet no longer do our bidding.

Technology is the answer to this new challenge of ours, technology in the form of a wheelchair or powerchair. If you still have functioning hands you will appreciate the independence this provides. Let’s discuss some important considerations for a powerchair.

As one of the biggest investments for an ALS patient you must consider future needs for yourself and your care team. If you receive a donated chair, which many do, then you really don’t have the opportunity to ask for options.

Powerchair considerations:
this is not an endorsement nor a comprehensive list

1. A chair should be fitted by the OT or vendor. This consult will (should) take into account current and future needs.
2. Brand- pertinent only if your OT has various vendors to offer you. Two principal brands: Permibile and Quantum. There are too many foldable travel chair brands to list here, Google is your friend in this case.
3. Drive- this is indicative of the position of the wheel that actually propelled the chair. The most maneuverability is offered by center drive, drive wheel in the middle of the chair.
4. Lights- only if you are active and venture out after hours, so you can see where you’re going.
5. Eye Level- ability to raise the chair to standing height.
6. Attendant controls- joystick on the back of chair allowing caregiver to drive.
7. The last thing I will mention is the seat cushion. There are many types, over long term a seat made with inflatable (adjustable) bladders have proven to be preferred by pALS.

That should suffice for now. One important aspect about using a powerchair, you will damage your home, furniture and your family/friends, or at least their shins and toes.
Now that we are on a roll…he, he, more to come…

The Juan and Only

So you have ALS
What can you expect…
(Part 6)
Last post we chatted about grief, let’s be honest with ourselves, ALS is a grief mill; it just churns out something to grieve about almost daily. Let me take your mind off grief for a moment.

But how, since it permeates my every thought?

Well, let me share the harsh reality of what you can expect as you progress. Don’t continue if you don’t want to know…

However your ALS started, limb, bulbar or both, slow or fast, you can expect the following:
Muscle atrophy, most noticeable in hands, shoulders and arms. Increased weakness to the point of failing, your limbs will cease to function. This will bring on bouts of anxiety, anger, frustration and lashing out. If left unaddressed this can lead to depression and beyond, which for some there is no return. This is just a fact, nothing about ALS is fair.

You will experience unimaginable fatigue; first with exertion then with the mundane, such as eating, talking, being dressed or of all things a BM, yes a BM. Every activity will take great effort and will eventually just not be possible. Oh yeah, I almost forgot, extreme temperatures will also effect you greatly.

Eating, breathing and speaking, all will be effected, if not already. Your options, should you wish to pursue them:
-Feeding tube
-NIV: None Invasive Ventilation aka BIPAP.
-Invasive Ventilation, tracheotomy
-Eyegaze: communication device that tracks eye movement and speaks your words for you.
–Letterboard: low tech communication board that uses a grid of the alphabet and eye movement

Now I know that I’ve painted a rather bleak outlook with the future of your progression, I’m simply not sugar coating it. However, as with any flip of a coin, there are two sides! Ultimately it’s both luck and choice, both require active participation. All of these effects of ALS can be mitigated with adaptations to your care. Many choose to implement all, some or none of these interventions. All options are personal and must be respected, thus the importance of honest and uncomfortable discussions regarding end of life wishes. Sorry, took a dark turn again, I’m trying to turn this post around and end on a positive note…but damned if ALS just won’t let me!!

Oh well, I will post a positive image to make up for it.

More to come…

The Juan and Only

As I perused social media when I was diagnosed I noticed images of patients under mountains of covers. I often wondered why? Now I know.

As our condition progresses our body looses muscle mass and as we move less and less we don’t generate body heat. Thus requiring more layers to stave off a cool environment. What may feel comfortable to you, may be very cold to us.

Muscles in general can generate body heat by simply flexing, shivering and by simply metabolizing fat and muscle tissue. You can see the obvious correlation here to weight and muscle mass. Just like more senior persons; a more sedentary lifestyle and decreased body mass brings on heightened sensitivity to changes in temperature.

A secondary effect of cold temps for pALS is it’s effect on spasticity. These highly”tense” moments can and often are preceded by a very exaggerated yawn.

What? No that’s ridiculous!

Ask a pALS if they yawn when cold, not to mention getting rigid, spazing out!

How can becoming sensitive to cold be troublesome? Well if you have a spouse that runs on the warmer side, arguments over the thermostat setting can ensue… “who changed the temp?” It wasn’t me! Or was it?

Damn it, now I’m hot!

Now that we sat for a spell, caught our breath and digested the mountain of information that is ALS. Let’s reflect on something that should be hitting right about now…Grief.

ALS, grieving and mourning our future.

Grieving when you experience a loss is a natural process of life. Especially when you lose a loved one. But what do you do when you lose the life you had planned on, the life you dreamt of spending with your loved ones? Your still very much alive it’s just that you have to alter all of your future plans.

What do you do in this situation…? You mourn, you experience anger, denial, bargaining and everything else that comes along with it. Having experienced this personally with a diagnosis of ALS I can imagine that many if not all patients go through the same process. The moment Meg and I left the clinic after receiving the news our minds were abuzz with a whirlwind of questions. Meg being a planner, I’m sure was already wondering what plans we have to put in place to deal with this. These thoughts we’re only superseded by the thought of having to break the news to our children and family.

After the news settled in we went about our lives starting to plan and think of changes we would have to consider. At the same time that I was diagnosed we were invited to attend a support group and the annual walk to defeat ALS. Our emotions were to raw to consider attending either. Eventually we worked up the courage to attend the support group. It turned out to be one of the best decisions since being diagnosed.

Meg and I reflected on the advice we received from other families and decided to talk and discuss the importance of a unified approach. So what has changed for us? For starters I had to retire permanently in order to arrange future benefits for Meg and our children. Meg has to consider early retirement to care for me. Barring any incredible discoveries in the treatment of ALS in the immediate future I also realized I may not see my children grow up into adults.

We are in the process of making memories such as destination vacations. So far we have been on two cruises and 9 years ago before my diagnosis we had an Incredible 2 weeks RV trip up to Mount Rushmore, Yellowstone, Colorado and New Mexico, it was amazing. I even went skydiving six months after diagnosis. Yes our life has changed inextricably and forever. But has it ended? Hell no!!

Let’s take a moment to focus on just the caregiver, shall we.

ALS is a very labor-intensive medical condition, what do I mean by this? Well simply put, it requires a lot of time and attention to the patient. This is wholly dependent on the severity of the pALS condition and how advanced it is. The more advanced the more minute by minute care that is needed. Of course the whole point of this is to sustain a decent quality of life for the patient.

Because of the intensity of the Care needed by the patient it is very common for the caregivers to become overwhelmed. Although grieving begins at diagnosis the focus on care by the caregiver can interrupt the natural course of this process. Coupled with the desire to not leave their loved one for any amount of time it can become untenable for this to continue unchecked. Most of the time it’s because there is no one else to take their place.

Grieving in this instance becomes incremental; mourning the loss of function, speech, touch, independence and even friendships. Mourning the life you had planned for your self or each other.

Im tired, let’s take a break.

More to come…

The Juan and Only

Alright, so far we have touched on diagnosis, treatment paths and the language of ALS. To be honest we’ve simply dipped our toes into these dark waters. Dark waters that can mimic a warm embrace, not because we can feel comfort in its embrace, but because it numbs us with it’s chokehold.

Oh my you’ve taken a dark turn Me. Juan and Only.

Yes I have, why you may ask? Because ALS is dark, scary, suffocating and ultimately liberating, I will get to that later.

The dark and scary…

“I’m sorry you have ALS”
“There are no treatments or a cure”
This leaves us without hope in the medical establishment. So why engage? Because it, the medical establishment, can and does help us and our families cope with the daily trauma of ALS. Every day we experience anticipatory loss, what next, when? Until what we anticipate loosing in fact is lost.

So what can you expect?
Is ALS painless? Yes, but not its effect on the body and our minds. As it shapes our bodies against our will, it is painful. Strictures, frozen joints, out of control emotions and the pain in our families eyes…but ALS is painless (my ass).

But how will our lives change? That’s what we really want to know?
Change? No it won’t, “change” is not apropos! I will let you choose the word, I have a slew of expletives myself, lol.

ALS will change you though, some changes good, some not. The obvious changes will require you to depend on others for EVERYTHING. These changes will be painful not because your body is at odds with itself, but because you are at odds with yourself. You will however, if you allow it, appreciate time and moments much more. You will also meet amazing souls, many of which are battling ALS themselves. Once again I encourage you to surrender, not to ALS, but to the love and support offered and given. Don’t get me wrong, ALS is a shitty condition. There’s plenty of reminders every day, most are unpleasant, some embarrassing and many simply necessary.

You have two options ultimately; focus on what you lose, or focus on what you still have. To be honest the balance is between both. But it’s perfectly alright to be off balance, trust me your family will pull you back to center, whether you want it or not. You’d be surprised what a slap on the head from your wife will do, ha!

Don’t fight ALS, fight to live, fight for moments, fight for each other!

Let’s sit for a moment before we continue on this journey, catch our breath.
More to come…

The Juan and Only

Our journey continues…

Now that we have addressed the initial assault and the paths before us, let’s meander through the language of ALS. Let me preface this by saying that some terms are established and some are unique to our community, the ALS community.

ALS: Ameotrophic Lateral Sclerosis
MND: Motor Neuron Disease (how ALS is referred to outside the US)
ALS/MND is a syndrome within what are known as Neurodegenerative Conditions.
Within ALS there are two main categories: Bulbar Onset and Limb Onset. In essence the brain and the body. Neither are mutually exclusive, either one can occur independently or simultaneously. If onset is one or the other eventually it will evolve to both.

Slow Progression -v- Rapid Progression.
Here is the rub…you can have both, no really you can. Slow progression can include plateaus and it can also include episodes of rapid loss. I don’t assign time frames, the definition of fast or slow is subjective. Fast progression is well, fast, you must take into account how long diagnosis took. Rapid can be from days, weeks and months, I’m referencing to loss of abilities and function and ultimately the inevitable. Sadly there is no rhyme or reason as to who or why one or the other.

pALS & cALS: abbreviations for Person or Caregiver of someone living with ALS. Not accepted by all, but it prevents writing the longer terms.

fALS: Famelial ALS, the persons afflicted with the generational genetic form of ALS, hereditary.
vALS: Veteran with ALS

Clinical Trials: A structured research program developing targeted treatments for a disease (s); all require patient participants for human safety.

ALS-FRS-R: An ALS questionnaire-based scale that measures and tracks changes in a person’s physical function over time.

PEG/Mikey/Button: A feeding tube. The name refers to the style of tube; normal profile-long tube or low profile (button).

BIPAP: Bilevel Positive Airway Pressure, may improve respiratory function. In ALS it is intended to prolong diaphragm function and aid in CO2 exchange.

Tobii Dynavox/Augmentative Communication Device/Eyegaze Computer: A computer/tablet augmented with technology that allows navigation on the computer by tracking eye movement. These devices have advanced to allow a person not just to communicate but to also control their environment.

Ok, I think I’m done for now. These are some of the terms you will most likely immediately hear as you begin your journey. There will be many more to learn, before you know it you’ll be fluent in ALS-speak. It’s not a language anyone ever wants to learn, it is a must to navigate ALS though.

More to come…

The Juan and Only

Let’s continue, shall we.
Now that you’ve had a moment to catch your breath and clear your search history on everything ALS, what’s next?

Several paths will present themselves: 1. Accessing an ALS Clinic (Center of Excellence), 2. If a veteran, accessing a VA ALS clinic and your benefits, 3. Pursuing alternative health care, independently or combined with traditional medicine, lastly 4. No treatments or therapies.

All of the above are choices that are deeply personal and must be respected. However, and I mean this sincerely, your choice requires consensus by your support network, a.k.a. family. Why, in case you are unaware, you will require total care and support, no one escapes this, regardless of rate of progression. The blunt reality of ALS is that none of us know how it will progress for each of us. There will be some who offer their insights as to onset, progression and a slew of other aspects regarding ALS. Tread carefully is the only thing I will say.

Once you choose though, depending on that choice, you can and will alter your approach as needed. Remain hopeful that therapies are imminent, remain open minded and keep your options open.

This brings me to the topic of clinical trials. In my humble opinion, trials are not designed well for us but they are evolving with pressure from our community. The reason to participate in trials, hope! Hope that the therapy meets our needs. I could give you a bunch of figures and science, let’s be honest none of that matters, all we want is a chance.

I will end part 2 with…
ALS has introduced itself to you and is becoming an important aspect in your life. But for a very small percentage, you are and will continue to be the same person. One major difference, your physical limitations require you to surrender to the love and support you’re surrounded by. If you are blessed to have such support. For there are many who either don’t or loose it entirely.

More to come,

The Juan and Only