Dignity

This is a term that for general purposes we use to describe appropriate interactions/actions with and to others. Modesty is frequently associated with dignity, when discussed. We are all familiar with it’s use under this premise. I would like to share another aspect of this term as it applies to ALS, end of life.

Although this dignity or dignified end of life concept has been present throughout all human history, it continues to be taboo. My question is, Why?

Death is very much an integral aspect of life itself. Yet as a society we shy away from this very real and unavoidable eventuality. Fear, narcissism, ego and many other factors feed and add life to the taboo public perception.

Recently the ALS community experienced the loss of two incredible human beings, who defined their dignity. I am not here to discuss anything other than the strength and courage needed to make such a truly personal and difficult decision.

Take a moment to reflect on the many hours of reflection, conversations with loved ones and the tears shed leading to this. Just imagine.

Given that ALS takes everything, dignity is totally dependent on others. I ask that you also take to heart the amount of love and respect for the individual by the family. That is true and selfless love, which is essential for dignity.

I won’t even venture to say “ I can imagine “ the discussions had. Ultimately each person defines their dignity and we who remain cherish their lives and memories, with DIGNITY.

J. Reyes

Family

sponsored#MTPApartner I’m proud to be partnering with Mitsubishi Tanabe Pharma America (MTPA) to help newly diagnosed pALS and cALS navigate their diagnosis.

#EmbraceYourNow

Imagine having your life turned upside down with the words, “you have ALS”. Imagine losing everything that gave you security, comfort, and support. This, in fact, happens to many people dealt a terminal diagnosis, abandonment. Losing your entire support network, all of it! It’s an insidious occurrence around the world, hidden in plain sight.

What then? Where to turn?

ALS has a tendency to do one of two things; rally your family and friends around you or bring the worst out in them. This is just a simple fact of life. Sadly, there may be many pALS who are abandoned and left to navigate this journey alone. How can these individuals embrace their new challenge as their body and family turn against them?

There simply is no solution. Each case requires a tailored approach for that pALS’ needs. Some resources are available through ALS clinics, various nonprofits and government agencies.

Considering the degree of care needed may be a challenging experience depending on what resources are available. I have found that in these challenging instances, it is the ALS community that must come together and embrace the pALS to show support.

Personally, I am blessed to have a very supportive family. I can’t fathom living with ALS and also being abandoned. My heart goes out to anyone dealing with this very situation.

Having experienced a medical emergency a year ago, I experienced firsthand the sense of helplessness. Unable to move and completely dependent on others. Given the current state of health staff shortages, it took some convincing to allow my family to help me. Without them I would have been miserable.

It’s not just a matter of who you embrace but who embraces you – “Embrace Your Now!”

MTPA offers the JourneyMateTM Support Program featuring a JourneyMateTM Resource Specialist who can help you and your loved ones learn about ALS and RADICAVA ORS® (edaravone) or RADICAVA® (edaravone) IV as a treatment option. Call toll-free 1-855-457-6968 or visit https://www.radicava.com/patient/journeymate/.

Interested in sharing your RADICAVA ORS® or RADICAVA® IV experience with others? MTPA offers the Share Your Story program to allow real people to share real stories. To learn more, and for a chance to share your story, call a JourneyMate Resource Specialist toll free at 1-855-457-6968 sign-up at https://www.ShareYourALSStory.com.

This information is intended for U.S. audiences only 18 years of age and older. RADICAVA ORS and RADICAVA are available by prescription only. Talk to your doctor.
IMPORTANT SAFETY INFORMATION
Do not receive RADICAVA (edaravone) or RADICAVA ORS (edaravone) if you are allergic to
edaravone or any of the ingredients in RADICAVA and RADICAVA ORS.

Before you take RADICAVA or RADICAVA ORS, tell your healthcare provider about all of your
medical conditions, including if you:
• have asthma
• are allergic to other medicines.
• are pregnant or plan to become pregnant. It is not known if RADICAVA or RADICAVA ORS will harm your unborn baby.
• are breastfeeding or plan to breastfeed. It is not known if RADICAVA or RADICAVA ORS passes into your breastmilk. You and your healthcare provider should decide if you will receive RADICAVA or RADICAVA ORS or breastfeed.

Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements.

What are the possible side effects of RADICAVA and RADICAVA ORS?
RADICAVA and RADICAVA ORS may cause serious side effects, including hypersensitivity (allergic) reactions and sulfite allergic reactions.
• Hypersensitivity reactions have happened in people receiving RADICAVA or taking RADICAVA ORS and can happen after your medicine has been given.
• RADICAVA and RADICAVA ORS contain sodium bisulfite, a sulfite that may cause a type of allergic reaction that can be serious and life-threatening. Sodium bisulfite can also cause less severe asthma episodes in certain people. Sulfite sensitivity can happen more often in people who have asthma than in people who do not have asthma.
• Tell your healthcare provider right away or go to the nearest emergency room if you have any of the following symptoms: hives; swelling of the lips, tongue, or face; fainting; breathing problems; wheezing; trouble swallowing; dizziness; itching; or an asthma attack (in people with asthma).

Your healthcare provider will monitor you during treatment to watch for signs and symptoms of all the serious side effects and allergic reactions.
The most common side effects include bruising (contusion), problems walking (gait
disturbance), and headache.

These are not all the possible side effects of RADICAVA or RADICAVA ORS. Call your doctor for
medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088. You
may also report side effects to http://www.fda.gov/medwatch or Mitsubishi Tanabe Pharma
America, Inc. at 1-888-292-0058.

INDICATION
RADICAVA and RADICAVA ORS are indicated for the treatment of amyotrophic lateral sclerosis
(ALS).

For more information, including full Prescribing Information, please visit http://www.RADICAVA.com.

Broken Hearts Keep BeatingBroken People Keep Fighting

We live surrounded, orbited by those that come into our lives. Most on the periphery, some fly close, even less shift our trajectory. But a few collide with us, forever changing us. Their impact leaving a lasting and enduring impression. When they leave us it hurts, yet we carry on, we must.

Recently we, our ALS family, lost two such persons. Each having left an incredible void in their families lives. Both fierce advocates, more importantly fierce mother’s . Both willing to fight not just for themselves, but for everyone afflicted by ALS.

Sandy, you altered the ALS research landscape by demanding and implementing accountability. Allowing those living with and affected by ALS to contribute directly to the impenetrable research machine. Your voice and presence never diminished by your failing body; when you spoke we hung on to every labored word of yours.

Taya, we met over zoom, and your energy and smile won us over. As we came to know you it soon became glaringly obvious, you were a force of nature. A fierce ALS advocate in your own right in Canada, and an avid fan of the Toronto Blue Jays. We were always in awe of your efforts and your candid perspectives.

I know my life is better for having met both of you. So with broken hearts and broken bodies we keep fighting. Our hearts continue beating with a broken cadence, having lost two resounding notes in our ALS song.

J Reyes

Dear FDA:

I am waiting for you to practice what you have promised… regulatory flexibility toward ALS treatment’s. I know and understand you have so many priorities, but I hope you understand that it’s very much a priority for me. You see it’s very personal to me, my family and every person afflicted.

I get it, although we are dying, you hold true to your practice of protecting us from potential deadly therapies. I snicker, well because as you know… we are already dying. I’m being polite, obviously.

In the turbulence of delay after delay, the wake takes it’s toll on our hope, bodies and minds. Not to mention our families hearts; so we wait.

However while you deliberate and decide our fate, we will continue to live in spite of ALS. We will continue to encourage, press and help you understand the human price of the science. We, the person with ALS, are the currency; what value do you assign to us?
As Lord Farquaad once said…
“Some of you may die, but it’s a sacrifice I am willing to make.”

I jest of course, I can because I’m on the inside.

I truly respect the very difficult task you have been charged with, but could you try being a bit more flexible, maybe just a skosh.

JR

ALS, Behind the Public Veil, P2


quad·ri·ple·gic
/ˌkwädrəˈplējik/
adjective

  1. affected by or relating to paralysis of all four limbs; tetraplegic.
    “quadriplegic patients”
    noun
  2. a person affected by paralysis of all four limbs.
    “a car accident left him a quadriplegic”

Paralyzed from the neck down. This is what a person living with ALS will experience; if they live long enough to reach this inevitable conclusion. So please forgive us if your well intended wishes are occasionally rebuffed. Or we may simply smile…graciously.

Many paraplegic persons are a result of injury to their spine. ALS on the other hand is a progressive degenerative disease, where by some unknown trigger, motor neurons die. Translation – the nerves that control movement die. The general public believes that arms and legs are the principle muscles that move. Once diagnosed with ALS you learn there are oh so many more.
• Diaphragm
• Neck
• Tongue
• Soft palate
• Swallowing
• Lips
• Eyelids

All of the above can be affected in too many variations and sequences. The typical prognosis for us is 2 – 5 years. So if it can take up to two years to be diagnosed, how much time does one actually have. Again you have better odds in Vegas than guessing anyone’s prognosis.

Okay let’s get into some of the odd things that ALS causes to our bodies, again some experience these some don’t. Sialorrhea! What the heck is that!? It’s the medical term for excessive saliva, drooling. The drooling isn’t the major issue, although uncomfortable socially, the real problem is choking. Many persons with ALS often aspirate drainage and end up with life threatening pneumonia. Paralyzed and choking on your own saliva, ALS is simply brutal.

PBA, Pseudobulbar Affect; emotional incontinence, uncontrollable crying or laughing. This is a little side hustle ALS has, although this condition can be triggered by a number of neurological conditions and head trauma. So what does PBA do to the person? It literally makes one think they’re loosing their mind. Total emotional breakdowns for no reason or caused by emotionally charged triggers. PBA also causes laughter or giggling for no reason, most often at inopportune moments. Luckily there are medications that can help suppress or control the intensity and frequency.

ALS, three letters that can be expressed in so many four letter words, expletives just don’t do it justice as to how devastating it is. So if you encounter a person living with ALS and their family, consider what they are enduring and realize how strong they are.

J. Reyes

They Just Can’t Help It

I have ALS, you do not, so why are you so awkward around me? I’m the one enduring the jail cell that is my body, as I lose every bit of my independence. I wouldn’t behave this way if it were you…or would I?

As a person living with ALS I am very aware that I am in a minority with slow progression. If one can ascribe “lucky” to ALS, I and my family are lucky. However I too experience the distancing of friends and family, I have siblings that simply can’t see me in my current state. And I’m still mobile and active.

Many families experience extreme isolation due to the immense focus required to care for us. No time to entertain guests, regardless of the good intentions of those guests. Yes it’s painful to see us bed ridden, emaciated, gaunt and sustained by technology; beep, hiss, click…

Think of it this way; if we can endure living with ALS, you can endure a fly-by. Better yet! Stay and learn to care for me. This way my spouse/child/parent/friend (AKA caregiver) can comfortably go to the bathroom or have a bite to eat.
“ But I’m squeamish, and I can’t stand to see you like this. It breaks my heart, I just can’t.”

Okay, honestly, if I may be blunt, that’s a “you problem”. I don’t have a problem with you seeing me in such a vulnerable state, I would love a visit. [Some with ALS do want to quietly and privately live their life, respect this.]
So if you as a person living with ALS receive company or run into acquaintances while out, don’t hold it against them if they say…

…how are you?
…you look good.
…hope you feel better.
…I’ll pray for you.

There’s absolutely nothing wrong with these statements. Yes we know full well that at this point in time nothing will help us. So don’t alienate others by chastising them for their awkward greetings or goodbyes. They just can’t help themselves.

TJO

ALS: Pulling Back The Public Veil

Recently I’ve come across those in the ALS community that feel the ALS public conversation is filled and represented by super-resilient personalities. A quick browse of the major SM Outlets (IG, FB, Twitter etc…), will prove this to be true, somewhat. For the most part these are individuals and families willing to put their life out there; primarily to generate awareness and catalyze treatments. I’m in this very category, my lane is more emotional support through creative writing… I’m ok with this.

Does this truly represent the destructive, unrelenting ALS toll on the body, family and the global ALS community. I have to agree it does NOT, if I must be honest.

So what does?

Let’s pull back the curtain for a moment.
Potential Triggers
The road to diagnosis is extremely frustrating, long and ultimately devastating. This is but the beginning of the assault. So insidious and heterogeneous (different case by case), that one has better luck in Vegas than guessing how progression will happen: fast, moderate, plateau or slow. This simply adds unbearable weight and stress on the person with ALS and the family.

Regardless of the onset, arms, hands, legs, speech, swallowing or breathing, the outcome is still the same, terminal. Let me be blunt, ALS kills everyone that gets it, every single person! The wake of destruction created by ALS is felt by the family for ever; some for generations.

This is what every person with ALS will experience, in no specific order:

Loss of limb function, total loss. You sneeze, you can’t lift a tissue to blow your nose. Walk today, with assistance, to being unable to bear any weight the next day. I believe the term is paralyzed, not a little, but completely.

Breathing, you know that thing we all do without thinking, yeah that disappears also. Some slow, some abruptly, oh and did you know this, the majority of us will die of respiratory failure. ALS literally takes our breath away, our last breath.

Until this happens, we and our families have to endure loss of privacy, modesty and dignity. As much as all those around try to protect our dignity, to the best of their ability…ALS is utterly undignified. Diapers, catheters, feeding tubes, personal accidents. Ok, full transparency… if we live long enough, we will piss and shit ourselves, did you expect a quadriplegic to not poop and pee? Well shit! Some will lose so much muscle tone that they are unable to bear down. Did you know that, probably not.

Do you enjoy food, so do we, ALS takes that also. Prior to loosing the ability to swallow, we experience episodes of choking, not just on food but our own phlegm. Did I mention our diaphragm stops working, essential for pulmonary toiletry. You know…coughing shit up. This in turn leads to weight loss, accentuated by atrophy. Making most of us gaunt, emaciated, skeletal, how’s that for a visual? Not pretty, well ALS, in my opinion, is one of the ugliest diseases.

I will leave you with this.

The person who is diagnosed with ALS, is not the person the family says goodbye to. Not in appearance, perhaps in mind and spirit but not the body. Long gone is every physical attribute that was the loved one. Except the eyes, our eyes will always convey our love for you, my (our) family(s), our world. We can’t live without your love and support, we simply can’t.

This is ALS, but I’ve only scratched the surface; there’s so much more, gross, ugly, uncomfortable, painful. To be honest you can’t handle the truth, you can’t handle ALS, neither can we, but we live in spite of it.

J. Reyes

Broken Cups

Sponsored #MTPApartner I’m proud to be partnering with Mitsubishi Tanabe Pharma America (MTPA) to help newly diagnosed pALS and cALS navigate their diagnosis.

#EmbraceYourNow

“Oh damn, I just dropped my coffee, there goes my favorite mug! It was a gift for father’s day…”

Photo Caption: My hands can no longer hold my favorite coffee mugs.

“Hmm, how did I just lose my footing on a perfectly smooth floor? That’s weird, what a klutz…”

“Why are my shoes all scuffed at the toes? I need to go shoe shopping…”

The above are reflections of internal dialogues with myself as I began to grow concerned as to the intensity and recurrence of these incidents. Ultimately it was this and noticeable atrophy of my hands that led to seeing my primary doctor.

Initially my doctor wanted to address existing health concerns to ensure my symptoms were not caused by them. Once he ruled these out, my two-year journey to diagnosis began. One of the greatest challenges with ALS is its diverse presentation, varying person to person. To be honest, when I received the diagnosis, it was a devastating blow, but also a relief. Having a major defining factor such as a name, returns a modicum of control. Allowing, at least our family, to create a plan and to frame our mindset.

Once the gravity of ALS settles in, allowing you to catch your breath, you plan. This plan that encompasses daily living, long-term arrangements and treatment options, is at best fluid. After my diagnosis, we talked about treatment and trials. And we talked about these with my doctor.

When it came to RADICAVA® (edaravone), I extensively researched any information available and had lots of questions and in-depth discussions with my neurologist. If you think a treatment including RADICAVA might be for you, visit http://www.radicava.com to learn more. As with any course of treatment, each and every pALS should thoroughly discuss all available options with your doctor.

RADICAVA ORS® (edaravone) and RADICAVA® IV are indicated for the treatment of ALS. RADICAVA ORS ® and RADICAVA® IV may cause serious side effects, including hypersensitivity (allergic) reactions and sulfite allergic reactions. The most common side effects include bruising (contusion), problems walking (gait disturbance), and headache. See Important Safety Information below.

Having a good understanding of the effects to any regimen empowers pALS and cALS to have peace of mind. Having peace of mind may allow us to embrace moments that fill our hearts and souls with joy, love and happiness—to “Embrace Your Now.”

Ultimately, what I desire is another cheesy Father’s Day cup, no more stumbles and time to build my shoe collection. I can’t forget silly socks also, LOL.

Photo Caption: My shoes no longer get scuffed since I don’t walk due to ALS.

J. Reyes

If you are interested in learning how RADICAVA may help, visit: https://www.radicava.com/patient/understanding-radicava/

Interested in sharing your RADICAVA ORS® or RADICAVA® IV experience with others? MTPA offers the Share Your Story program to allow real people to share real stories. To learn more, and for a chance to share your story, call a JourneyMate Resource Specialist toll free at 1-855-457-6968 or sign-up at https://www.radicava.com/share-your-als-story/.

This information is intended for U.S. audiences only 18 years of age and older. RADICAVA ORS® and RADICAVA® IV are available by prescription only. Talk to your doctor.

IMPORTANT SAFETY INFORMATION

Do not receive RADICAVA (edaravone) or RADICAVA ORS (edaravone) if you are allergic to
edaravone or any of the ingredients in RADICAVA and RADICAVA ORS.

Before you take RADICAVA or RADICAVA ORS, tell your healthcare provider about all of your
medical conditions, including if you:
• have asthma
• are allergic to other medicines.
• are pregnant or plan to become pregnant. It is not known if RADICAVA or RADICAVA ORS will harm your unborn baby.
• are breastfeeding or plan to breastfeed. It is not known if RADICAVA or RADICAVA ORS passes into your breastmilk. You and your healthcare provider should decide if you will receive RADICAVA or RADICAVA ORS or breastfeed.

Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements.

What are the possible side effects of RADICAVA and RADICAVA ORS?
RADICAVA and RADICAVA ORS may cause serious side effects, including hypersensitivity (allergic) reactions and sulfite allergic reactions.
• Hypersensitivity reactions have happened in people receiving RADICAVA or taking RADICAVA ORS and can happen after your medicine has been given.
• RADICAVA and RADICAVA ORS contain sodium bisulfite, a sulfite that may cause a type of allergic reaction that can be serious and life-threatening. Sodium bisulfite can also cause less severe asthma episodes in certain people. Sulfite sensitivity can happen more often in people who have asthma than in people who do not have asthma.
• Tell your healthcare provider right away or go to the nearest emergency room if you have any of the following symptoms: hives; swelling of the lips, tongue, or face; fainting; breathing problems; wheezing; trouble swallowing; dizziness; itching; or an asthma attack (in people with asthma).

Your healthcare provider will monitor you during treatment to watch for signs and symptoms of
all the serious side effects and allergic reactions.

The most common side effects include bruising (contusion), problems walking (gait
disturbance), and headache.

These are not all the possible side effects of RADICAVA or RADICAVA ORS. Call your doctor for
medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088. You
may also report side effects to http://www.fda.gov/medwatch or Mitsubishi Tanabe Pharma
America, Inc. at 1-888-292-0058.

INDICATION
RADICAVA and RADICAVA ORS are indicated for the treatment of amyotrophic lateral sclerosis
(ALS).

For more information, including full Prescribing Information, please visit http://www.RADICAVA.com.

The Most Difficult Gift

Recent events have brought our family, more so Meg and I, to solidify an unsavory but necessary aspect of ALS. Final wishes, honestly this applies to everyone, however living with a terminal condition makes it more pressing.

Why do I call it a gift though? Because it is in fact a gift to your loved ones, to ease the stress, pain and inevitable interjection of extended family. Yes I said it, extended family can in fact, unintentionally, create additional stress. As difficult as it is, “we” as the person with ALS, should consider this. I know, on top of all the sh** we have to endure with this relentless condition… final wishes.

I’m not in a position to offer specific details, but here are a few things to consider:
• Type of service, interment, cremation, donate to science. Of course this entails the specific details for each.
• Significant personal belongings (heirlooms).
• Ensuring all legal needs are met to avoid hiccups with assets and property. (Will, Trust, Life Insurance, VA Benefits, Gifts etc.)

Whether of modest means or not, I believe probate can and does apply in most cases. This can cause an undue financial and emotional burden on surviving family. I recommend seeking professional legal counsel to address this, at the least to know what best suites you.

When we prepare for the inevitable, which we must all face, we give our loved ones peace of mind. Allowing them to focus on the love and memories of our life. Mourning will be inevitable, but your celebration will be by your design, a reflection of you.

As a society we have moved far away from what used to be considered a natural fact, our own demise. As such we have become uncomfortable with these essential conversations. I don’t want to think about this, but I must, it’s important to our family.

ALS and Blind Trust

Recent discussions regarding genetic testing have brought me to realize a couple of things about myself. Which in turn led me to contemplate how others may deal with the same eventual realization. This in fact has to do with self advocacy and not allowing the healthcare community dictate your path.

Allow me to elaborate. Having been in patient care my entire military career, I have a very healthy respect for those in the field. This includes the nurses, allied health professionals and of course the physicians. So when I was first diagnosed, the trauma of such a gut punch led me to relinquish my condition to the professionals. They know what’s best, right? Let me clarify that this is not meant to disparage the dedicated healthcare professionals; this is a personal realization.

Initially I digested all the ALS information thrown at me, it was a reflection of everything I was being told, so why question it. Through interactions with others living with ALS, I have come to learn there are many gaps in ALS care. This is to be expected, primarily due to the conservative number of persons diagnosed and treated. But this is changing as persons living with ALS and their families become more vocal and are empowered by organizations such as I Am ALS.

But what does this have to do with genetics, you ask?

I, for example, didn’t believe I needed to do this, I have no family history of ALS. Again, my interactions with advocates, researchers and those living with ALS educated me on the importance of genetic testing. I know this is a very sensitive subject for those afflicted with Familial ALS, positive gene mutations and newly diagnosed persons. The first thing out of my son’s mouth, when we told him of my ALS, was “is it hereditary?” I understand the apprehension of both, knowing or not knowing.

I am as random or sporadic as they come, given current known mutations. In a recent webinar I also learned the value of repeat testing, new gene mutations are being identified; so six months from now you may test positive for the newest discovered mutation. I know it is an extremely personal and difficult choice to be tested, especially if you have a family history of ALS. So I will simply appeal to you as such.
 Genetics hold the power to unlock not only ALS, but many other related conditions. You hold the key within you. I encourage you to consider genetic testing if diagnosed, with or without a history. As a family member with Familial ALS, you have an unenviable and extremely difficult path on either side of this conversation.
The take away is this…

Be your own advocate, don’t assume the professionals are current on all matters ALS and lastly, do not underestimate the value you bring to research, the conversation and the ALS community. I for one continue learning and evolving as a person living with ALS.

TJO