ALS Life… Trilogy BIPAP pro-tip:

Equipment location considerations:

When placing the BIPAP near your bed avoid placing a dog bed near or next to it. Especially if you have an older dog that has a proclivity for highly fetid gastrointestinal expulsions.

The proximity of the pooch to the equipment will directly effect the expediency and concentration of said expulsions. The BIPAP being an efficient and effective device will capture the pervasive proppelant and deliver it. Deliver it to… YOU!

Being dependent on the BIPAP to deliver air to our bodies we are subject to receiving any and all aerosolized ambient bouquets. These putrid perfumes will propagate exponentially as the equipment concentrates them.

Last night I experienced such an occurrence. Which led to an expletives filled outburst by yours truly. Followed by a fit of gagging to near puking. Reminiscent of my experience in the tear gas chamber training while in the Air Force.

I think I now have dog fart PTSD.

No seriously!

TJ&O

What does ALS feel like?

Let me see if I can put it into words and help you understand or experience a taste of ALS.

Hands: have you ever carried a heavy item and when you put it down your fingers won’t move, they are stiff. After a moment they return to normal. Well for me they don’t, they remain stiff and unresponsive. They are also very weak, can’t grasp or lift a tissue.

Legs: when they aren’t cramping they are weak and while walking (very short distances) they don’t respond to my desired requests. They actually feel as though I have 100 pound ankle weights strapped to each leg. My knees and ankles give out from time to time risking a fall.

Eating: first off it is unavoidable that ALS takes the ability to swallow, however it begins with the tongue. You see the tongue becomes unresponsive and difficult to control. At this point imagine getting anesthesia in your mouth and your tongue is affected. In essence manipulation of food is challenging. This makes it difficult to move food away from the back of your throat causing frequent gagging. Now the epiglotis, the flap that separates your airway from your esophagus, well it starts to falter, allowing food to remain at the opening. This causes repeated swallowing and can lead to coughing and choking.

Laryngeal spasms: has your dog ever sounded like they are about to hack up a furr ball? Thats a spasm, in a person it sounds very scary. Imagine getting the wind knocked out of you, when you try and take a breath you cant… thats what it sounds like.

Neck: many ALS patients lose muscle strength and tone in the neck as it progresses, thus needing neck braces or head restraints. Imagin having a 50 pound weight on your head, eventually you can’t hold your head up. Many patients appear to be looking down constantly. Some become locked in this position.

Breathing, try this at home:

(Gather the following)
A large shake straw, regular straw and a coffee stirrer.
Now try this, take a normal healthy breath, now repeat breathing through each straw. You have just simulated progressive respiratory decline in an ALS patient.

Now don’t panic and catch your breath.

Last exercise: now find a a busy location in your home, sit in a chair and don’t move or speak. Think of your favorite drink or snack. Now without speaking or moving communicate to your family that you would like that drink or snack. You want to get the real experience, try using a patient’s eyegaze computer, let’s time how long it takes to get frustrated?

This is what ALS feels like. Let me know how it felt for you.

TJ&O

The Road to Diagnosis…

ALS is a condition that is considered a rule out diagnosis. What does this mean, you may ask? In essence it means that all other conditions must be ruled out before a specialist i.e. neurologist, will confidently make the diagnosis of ALS. This of course is if the symptoms are not glaringly obvious.

One of the biggest challenges, and the most common thread in ALS information, is the fact that every single patient manifests and progresses differently. Symptoms at onset can be any one of the following or any combination:
*Weakness in extremities

  • Loss of muscle mass or muscle tone in extremities
    *Difficulty walking
    *Tripping or falling
    *Uncontrollable emotional outbursts
    *Muscle twitches everywhere
    *Difficulty speaking
    *Changes in voice
    *Difficulty swallowing

These are just a small sample of how ALS can initially manifest. As you can see many of these symptoms can be associated with a slew of other conditions. Even in combination many of these signs and symptoms can be attributed to a number of conditions.

The following are some of the exams and tests that a patient might be put through in order to arrive to the diagnosis of
ALS:
*A thorough neurological exam
*Blood test for heavy metals
*Rule out if current meds causing symptoms
*Electro myelogram/ EMG
*MRI
*X-rays
*Family history
*Genetic testing

Once again this is a short list. Many patients go through multiple exams and tests and to multiple specialist before a diagnosis is rendered. There is no single definitive test for ALS. I’ve heard of patients taking up to two to three years to be diagnosed. I personally believe that the prevalence of ALS is much higher, many patients pass away before they are diagnosed, just my personal opinion.

My journey, in retrospect began summer of 13, thats the earliest I recall. I was kicking a soccer ball with the kids at a park an fell several times. I srugged it off of course.

Fast forward to late 14 when I noticed I was dropping things. My left hand was weak. A few months later my right hand began to lose strength and now both began to atrophe. By this time I was experiencing muscle twitches everywhere 24/7.

It was when I started having trouble recovering my balance that I became concerned. Early 15 my PCM referred me to a neurologist. This proved to be inconclusive, my PCM was not satisfied, neither was I. On to a second opinion…

Oct of 15, Meg Jankowski Reyes and I got the news. The news that altered our lives.

The Juan and Only

ALS… what is that?

ALS, what is that, isn’t it some old person disease?
Didn’t it get cured after the ice bucket challenge?
No it’s that disease that scientist had, right, Stephen something?
Nah it’s genetic, you only get it if it runs in the family, right?

What do you think? Which statement above is true and which is not?

  1. It has not been cured, in fact there are two medicines that slow it down (not by much), but no cure.
  2. Stephen Hawking, Lou Gehrig are synonymous with ALS.
  3. 90% of cases are random, RANDOM, the remaining are or can be genetic (familial ALS).
  4. It can strike anyone, at any age, any race. Fact: Juvenile ALS has been recently created as a category, why? Children are developing ALS.

ALS does not discriminate, it strikes every race, every socioeconomic category; however it effects Veterans twice as much as the civilian population.

Some notable individuals who have passed of ALS:
Lou Gehrig (MLB)
Stephen Hawking (Scientist)
Sam Sheppard (Actor/playwright)
Stephen Hillenburg (Spongebob creator)

There are quite a few athletes afflicted by ALS, Veterans, First Responders and so on. When someone passes of ALS, you don’t normally hear of it unless it’s a celebrity. Typical of a nation consumed with celebrity idolatry, we mainline any and all news regarding celebs. So as hundreds pass of ALS in a given week, you won’t hear of it, unless the person was of notoriety. I would like to share a few whom I’ve encountered that are notable to me and their families.

J.t. Inocencio
Andre Williams Sr.
Dr. Walter Root
Rex Roberts
Magie Caballero
Greg Kenoyer

The individuals above personally touched my life as examples of persons Living with ALS. Inspiring me and compelling me to honor them by living life fully. Their memories live on in their families hearts and in mine.

Remember ALS doesn’t give a single fu*# about status, wealth, power or influence. ALS takes who it wants, when it wants.

TJ&O

ALS An Insidious Thief

ALS is a thief, it is also a cruel Warden and betrays you without warning. ALS is a four-letter word… Trust me it is. ALS ruins relationships, families and finances and never apologizes for doing so. ALS is divisive, devious, devastating and most of all it is demanding, relentlessly demanding!

How is ALS a thief you may ask?

ALS is a thief because it steals mothers, fathers, brothers, sister and children from their loved ones. As a thief it begins by stealing one’s physical abilities, such as a person’s balance, their ability to walk the capacity to grasp with one’s hands, but it doesn’t stop there. ALS also steals one’s voice and eventually their breath and in rare cases their mind. The sad part about ALS being a thief is that your body is the perpetrator and the victim, a cruel twist of fate.

For the most part ALS is a random Thief however there are some families where it dwells and wreaks Havoc family member to family member, this is known as familial ALS. Can you imagine living your life waiting for the thief to strike.

ALS is a cruel and heartless Warden, what do I mean by this? As ALS ravishes your body and your physical abilities it leaves your mind in tact. Eventually when you lose your ability to speak to move and to take your own breath you are truly a prisoner in your own body. ALS betrays you without warning due to the fact that from one day to the next you won’t know if an ability or capacity to use your body will be present that day. Some patients experience drastic changes While others progress at a much slower pace. This is one of the cruelties of this condition, it varies drastically from Patient to Patient.

ALS is fatal and has no cure at this time. As a terminal condition it ruins lives, not just the patients but everyone connected to pALS (Person/Patient with ALS). Sadly there are life partners that are overwhelmed by the condition and the commitment required to care for a patient, that are broken and are not able to stay in the relationship. ALS ruins families financially due to the fact that it requires on average $200,000 to care for an ALS patient. This is compounded by the extremely complicated and ineffective Healthcare System our country has.

Lastly ALS is relentlessly demanding, never giving an inch never stopping. It demands your pride, your modesty and ultimately your life. It demands every ounce of energy you have, it challenges your State of Mind daily sometimes from moment to moment. It demands and exacts a toll on your loved ones as they attempt to keep their spirits and energy up as they meet your every need. Caregivers of terminally ill patients suffer a double insult due to the exhausting nature of caring and ultimately losing their loved one.

Yes ALS is a slew of medical terms, signs and symptoms, but to those affected by it it is more then scientific words and terms… It is a THIEF!

TJ&O

Time In A Wheelchair

It’s time to transfer to my powerchair, I wake, I’m helped up and in I go. From the moment my tush hits the seat I feel empowered, ready to go wherever my wrist dictates.

First stop, the kitchen, for my morning dose of coffee!

Sitting on the kitchen island is a tall cup of the miracle nectar, piping hot, bent straw in place. Next, raise my chair to drinking height, sip, sip… ahh.

S²D², Ground Hog Day, Rinse and Repeat.

The days blend together after a while, this includes the feeling that I’m witnessing life just whizz by at warp speed. I sit in the center of my home and I see my kids and wife going a mile a minute, in and out, buzzing past me.

Hi dad, by dad, as I sit.

I move about, never catching up, just when I do, they bounce in another direction. Yet as the blur of their movement leaves a visible trail in the light spectrum, like specter’s moving through time. I can’t catch them so I sit patiently, until they orbit me and notice… me.

It’s not them that has sped up, it’s me that has slowed. ALS has forced me to now experience life from a new vantage point, a much slower vantage point.

Patience is the mechanism by which we find the right pace to meet each other in this dichotomous universe we find ourselves in.

Time in a wheelchair, it’s a paradox.

TJ&O

ALS Life… I’m melting

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)

ALS Life… Concentration

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

ALS Life… Driven too live

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.

So you have ALSWhat can you expect…(Part 7)

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