Sacred questions

Recently I took part in a Tim Lowry ALS panel for occupational therapy (OT) students.  As panelists we answered many of the questions you might anticipate about how OT has improved our lives with ALS.  I was happy to tell the students about pencil grips, rocker knives, and splints.  The OT professor specialized in hand therapy and was eager to help with my current conundrum:  buttering toast. 

The question that most surprised me was directed to Tim Lowry who communicates via eye-gaze technologies.

"How do you prevent giving up when there is limited treatment and no cure for ALS?"

Fair enough. We tell the audience that we are an open book. Ask us anything. Still, it's not a question in the same league as how to butter toast.

Youthful students, given permission to be truly curious, will expose the elephant in the room.

"How do you face existential challenges, for real, in the day-to-day?"

Tim's poised response revealed the intentionality of a reflective life.  He has learned to nurture his mental and spiritual well-being to support the consequences of his on-going physical decline.

ALS poses its unique challenges to be sure.  Our motor neurons are dying, and it turns out that we really need healthy motor neurons to get dressed in the morning.

One option, I suppose, would have been to answer the youthful questions with a question: "How do you face the existential?  How do you keep from giving up?"  Life is chock-a-block full of existential opportunity:  our present climate crisis, raging gun deaths, a loved-one's eating disorder, a recurrent major depression, a father with ALS. How do any of us live with uncertainty and ambiguity in our lives?

I wonder, now that I have ALS, am I meant to understand more about managing existential crises?   I mean, I have stuff to do.  I have a garden to weed and water.  I have a Tim Lowry panel to prepare for.  I have asparagus to blanch for lunch with Zach and Isabelle.

Here's one thought.  What if we allowed our present reality to become sacred?  Fighting for climate justice and gun safety.  Being with a friend who is struggling.  Learning to put your socks on with one hand.  Blanching asparagus.  Living with ALS.  What if we approached all of it with reverence for the moment at hand?  

Fr. Gregory Boyle, S.J., the founder of Homeboy Industries, and author of The Whole Language, puts it this way:

"We remember the sacred by our reverence...This is the esteem we extend to the reality revealed to us. Jesus didn't abandon his reality, he lived it. He ran away from nothing and sought some wise path through everything. He engaged in it all with acceptance. He had an eye out always for cherishing reality. A homie, Leo, wrote me: 'I'm going to trust God's constancy of love to hover over my crazy ass. I'm fervent in my efforts to cultivate holy desires.' This is how we find this other kind of stride and joyful engagement in our cherished reality. The holy rests in every single thing. Yes, it hovers, over our crazy asses."

Holy tears

 

Tender

soothing rain

all night last night

lingers unto morning

like sweet tears

to begin this day anew

and whole.

I did not grow up in a culture of sweet tears, the kind that flow gently down the cheek, as plainly and innocently as a smirk might linger while contemplating someone's clever retort.  In truth, I learned to withhold smirks and tears at all cost, until I became a dad.

Dad tears are sweet tears. Dad tears are Holy Tears.

I rarely withhold tears anymore.

Last week I found myself in Howard Goodkin's office with Peggy and William.  Howard is the chair of neurology at the University of Virginia. He is also a child neurologist with whom I have shared many complicated patients. Howard invited us to join him and the leaders of the ALS Dart Center of Excellence to explore the possibility of a partnership with the Hummingbird Fund.

It is worth mentioning here that Howard is also the person I emailed when Peggy and I first seriously suspected a diagnosis of ALS.  We were lost, adrift, so I emailed Howard. He called my cell before I could get up from the computer.

A couple of months later, at the Hummingbird launch, Howard joined a dozen colleagues, friends and family as a docent, mingling with guests, wearing his docent's badge that read, "ASK ME ANYTHING."  He was terrific.

All of this history sat silently in the back of my mind as I calmly entered Howard's office and took a seat at the familiar, long oak table, much like I had done many times before while working at UVA.

We began with introductions and roles, going around the table, ending with me.

As I began to recount my diagnosis, and the journey which has led to the Hummingbird Fund, tears began to roll down my cheeks.  I was surprised by the tears, and I smiled at Howard who was sitting at the other end of the table.  I said, "Well Howard, this is the first time I've cried in your office."

Everyone chuckled quietly, and without missing a beat, Howard said, "Well Jim, it's not the first time someone has cried in this office.  Many people have cried in this office, including me."

More soft chuckling... followed by a brief, intimate silence... followed by me having a moment to regain my grounding as a man with ALS, in a room full of people who know Mr. ALS all too well.  The group moved on through our agenda, and we will have a meaningful partnership.

I am learning that tears and ALS are pretty much kissing cousins.  Fury, deep disappointment, grief, and heartbreak. This community also weeps for the fearlessness, dignity, and brazen honesty of our kin. The road forward is a road through tears.  Holy tears of love and courage.

ALSTLS

ALS is always a grim tale to tell, and to hear.  Sorry.  

What if we could tell a parallel ALS tale that was the opposite of grim?  I have an idea.  Read on.

ALS, Amyotrophic Lateral Sclerosis, is generally defined as a progressive, neurodegenerative illness with no cure.  Often, this straightforward definition is embellished with a clause or two: 

• eventually leading to the inability to walk, talk, swallow, or breath.  
• including complete paralysis with intact cognition.
• being 100% fatal with an average life expectancy of 2-5 years. 

ALS is commonly referred to as a brutal disease; the disease most feared by doctors.

As I mentioned, it's a grim tale.  Sorry, again. 

Did you know that the functional decline that accompanies ALS is scored as a way of tracking the progression of the illness?  There is a 12-item functional rating scale called the ALSFRS-R:  48 points = A+ = no disability.

See details here.

• Speech
• Salivation
• Swallowing
• Handwriting
• Using utensils
• Dressing and hygiene
• Turning in Bed
• Walking
• Climbing stairs
• Dyspnea (breathing hard with activity)
• Orthopnea (difficulty breathing while lying down)
• Respiratory insufficiency

I am currently a 43 or 44.  I was a 41 or 42, until I taught Righty to use a pen.  Honestly, I could probably be a solid 44, if I used a rocker-knife.  After all, if we can enhance or adapt function to increase inclusion and participation, we diminish the disability.

It is worth noting here that Mr. ALS's march toward disability does not necessarily include mental, emotional, or spiritual decline.   ALS causes motor nerves and muscles to die. The thinking-mind remains completely intact.  Spirit, drive, passion, motivation, personality, the ability to give and receive love, all remain in full force.

Let us remember that intact abilities often compensate for innate or acquired disability.  People who are blind develop an exquisite sense of touch, smell, and hearing.  Kids with spina bifida who have never had use of their legs learn to scoot around as fast as their playmates and siblings.

What Lefty is losing in function, Righty happily takes on with aplomb.  

Joining the ALS community through organizations like I Am ALS, I am inspired by a fierceness of spirit, a courageous drive for change, a passionate desire to leave the world a better place.  These ALS champions and their loved ones are my new team.  Everyone is welcome.  Everyone gets into the game.  Kindness is not a zero-some proposition.  Love abounds.  Courage and hope prevail.

I understand the need for the ALSFRS-R.  But, wouldn't it be great to have a partnered scale to measure positive progress?  We could call it the ALSTLS.  The ALS Turbo-charged Living Scale, measuring:  

• adaptability
• humor
• kindness
• compassion
• hopefulness
• resilience
• altruism
• passion for creating change
• fierceness and drive
• advocacy for self and others
• ingenuity
• vision and meaning

What do you think?  Great idea, right?

Expanding the Frontiers of Hope

With the advancements of science and technology, it is now possible to reliably diagnose rare conditions early in pregnancy.  Likewise with the explosion in genetic analysis, we can now identify the cause of previously uncharacterized syndromes.  Whereas these diagnostic capabilities offer the certainty of a diagnosis, they also have the potential to launch families into new, and often uncharted waters filled with an array of questions about the future. 

www.trisomy18.org

Many rare conditions carry risks of future cancers, intellectual disability, autism, psychiatric co-morbidities, or seizures.  The risks of future diagnoses are based on small numbers of patients with conditions that have variable presentations and outcomes.  Parents are faced with statistics that we might try to represent as a slim possibility, but we must remember that they are seeing this slim possibility through the lens of a parent whose child has a rare condition.  

Receiving a diagnosis of a rare condition is a random and unimaginable event for any parent.  Making the leap to seeing your child with a whole series of future random and unimaginable events is actually quite natural.   If we then add to this the anachronistic label of “lethal diagnosis”, our offers of the potential for ever-expanding technological boundaries may not seem so comforting. 

My experience has been that most parents will do anything within their means to help their children.  Parents of children with disabilities or medical complexity are no different and are frequently quite innovative in their attempts to create opportunities for participation and inclusion.  When the status quo is not enough, they advocate for something different or they figure out a way to make it work. 

Statistics also provide a window into survivability.  We know with certainty that many conditions carry the prognosis of a limited lifespan.  Since it is nearly impossible to predict the length of a lifespan, especially as the boundaries of science and technology continue to expand, we land, once again, in the arena of uncertainty.  Parents and clinicians alike must learn to live with uncertainty.

So, where does hope fit into this equation?  We all need hope to continue in the day-to-day care of our children and patients.  We certainly need hope to support one another.  We also need hope to innovate, to advocate, to make meaning and to risk love.

And what about the notion of false hope?  If we know that an outcome is unlikely, is it wrong or misleading to encourage abundant hope?  Is it truly in the best interest of the patient, family or clinician to protect each other from the disappointment of a sad outcome, even if it is anticipated?  Is it any less sad if it is unexpected?   Is it false hope, to imagine with a parent that their child will walk, or talk, or make it to Disneyworld with a ventilator in tow?  I don’t think so.

Encouraging hope in a parent or caregiver is an act of kindness.

Recently I was invited to take part in an annual round table dinner-discussion hosted by the Anne L. Brodie Fund for medical education.  The fund also supports students as they learn the "skill and heart to care for patients" with the aim of becoming The Brodie Renaissance Physician.

The Brodie Renaissance Physician: Is called to artful, compassionate, 

patient centered care. Takes responsibility for the whole patient / person. 

Is adept with the newest technology but also possesses 

finely honed listening and physical exam skills 

to limit dependence on technology. 

Strives to improve on each individual patient encounter, 

and also on systems of practice. 

Is a scholar, teacher and role model.

In preparation for our dinner discussion, each guest was asked to reflect on the intersection of technology and caring, and to give an example of a situation where technology could never replace the physician.  Offering hope, and expanding the frontier of hope seemed an apt example from my work with families of children with medical complexity.

As I have gotten to know families whose children carry a “lethal diagnoses” or live with significant, life-limiting disability and medical complexity, I have seen magnificent hope blossom amidst slim possibility for success.   I have experienced the frontier of hope expand as parents and clinicians join together with proximity to the challenges and the risk of disappointment. 

 

Extending hope across the chasm of feared disappointment is a great kindness.  Learning to expand the frontier of hope is as significant as extending the boundaries of science and technology.   We need to challenge ourselves with equal energy and determination on both fronts.