My daily 40

A local gastroenterologist taught me the proper way to swallow a pill.  

Said gastroenterologist was also the mom of a teenager in my care.

Teenagers famously swallow their acne medicine without so much as a sip of water.  Hence, the doxycycline sticks to their dry esophagus and creates an ulcer.  Said teenager then ends up in the pediatrician's office with chest pain.  Thus, I have discovered, teaching teenagers to swallow their pills with plenty of water is just practicing good, preventive medicine.

Here's what I learned from the gastroenterolgist-mom, and subsequently passed along to dozens of teenagers I treated for acne:

• begin with 8 ounces of water
• first take 2 swallows of water to moisten the esophagus
• then swallow each pill with the remaining water   

I take 40 pills a day, my daily 40.  That's roughly 1200 pills a month. 

If I followed my own advice, I'd be swallowing 320 ounces of water a day,  That's 2.5 gallons of water a day.  Peggy has suggested I try milk instead of water, to make the swallowing easier. Doing the caloric calculations for 2.5 gallons of whole milk, that would be an extra 5,760 calories a day.

In various mindfulness workshops, I've been instructed to eat one blueberry or one M&M at a time, savoring the individual experience of the moment.  And perhaps it would aid my healing to contemplate the action of each pill, individually, in the moment, as it fights Mr. ALS, but 2 1/2 gallons of water a day seems impractical, and potentially dangerous.

And besides, I eat blueberries with gusto by the handful.  Is it really a surprise that I'd take my pills by the handful too? 

When swallowing pills, I am careful to drink lots of water.  However, even well meaning techniques pose potential hazards. 

Depending on the size, shape, and quantity of pills in each handful, I might cough a bit, causing water to shoot up my nose.  This, by the way, feels exactly the same as getting water up your nose while jumping off a dock, into the lake, doing a cannonball to show off.   Such a show off.

Included in the daily 40 are 4 anti-retroviral medications (ARVs) that are part of a NIH clinical trial.  Why am I taking ARVs commonly used to treat HIV, you ask? Well, it turns out that I am among the ALS patients who have HERV-K floating around in their blood.  And, HERV-K, like HIV, is a retrovirus.  The NIH study aims to determine if ARVs can eliminate HERV-K from the blood.  And what might be the role of HERV-K in ALS, you ask?  Like most everything with Mr. ALS, the role of HERV-K remains illusive.  Sorry.

Ah, but here's the plot-twisting good news.

During the HIV epidemic, a small group of patients with HIV also developed ALS, or something that looked exactly like ALS.  When these patients received ARV treatment for the HIV, their ALS symptoms went away.  In other words, ARVs  completely reversed the ALS.  They were cured!

This current NIH trial lasts 24 weeks.  At the end of the study, the NIH will no longer provide ARVs, even if they seem to be helping clinically, because this is not a randomized clinical trial. The purpose of this trial is only to investigate the effect of ARVs on HERV-K in my blood.

Ok, just for kicks, let's imagine that I do experience a positive clinical effect, a reversal, from the ARVs, like those folks who had HIV.  If I want to keep taking the ARVs, the monthly cost would exceed $12,000, and insurance will not cover ARVs when used off-label for ALS.  A sad reality of our health care system.

To continue taking the ARVs after the NIH study ends, it appears I will need to contract HIV.  Now, no one wishes HIV on anyone, but this seems the only logical way to obtain insurance coverage for medication that might successfully defeat Mr. ALS. 

HIV to get ARVs to reverse ALS, why not.

NIH Day #3

Day #3 finds JimBob and PeggO weary at the outset. A decent breakfast is essential.  Peggo, as we all know, comes prepared to meet that task.  

This morning they begin with Peet's House Blend pour-over coffee, followed by maple flavored Brown Cow yogurt, the kind with cream on top, homemade granola, and hand-picked, ripe, local strawberries.

Vine-ripened strawberries are a wonder of nature, no?  

"Think about it," muses JimBob, "where else in the world do you see this color of red?"

He waxes on, "Strawberries are so fun to eat... plucking their little stem hats off as you pop them into your mouth." 

JimBob demonstrates, followed by a self-satisfied smirk.

"Delicious."

Gesturing with the strawberry top, he says, "No two berries are ever the same.  Isn't that amazing?"

"Yes, truly amazing.  You realize that we still need to take Delta for a walk."  PeggO is all business this morning.

Today's snacks:  assorted charcuterie, hard cheese, artisanal crackers, roasted pistachios, dates, and ripe strawberries.  Pamplemousse La Croix to drink.

PeggO knows what JimBob likes.

9 a.m. NIH 1st Floor radiology: Fluoroscopy Suite

The official schedule begins with a swallowing study, because people with ALS eventually lose the muscular control required to eat and swallow.  The first sign of a problem can be coughing while trying to swallow.  Have you ever choked a little bit on your own saliva?  Does it make you cough?  Maybe this happens when you are laughing.  Or, maybe, like JimBob, this happens when you try to talk and eat at the same time.   Mr. ALS has this way of transforming an innocent cough into a telltale sign of decline. 

As they approach the radiology check-in desk, Monique is waiting.  She introduces herself to JimBob and PeggO, and ushers them back to her fluoroscopy suite.

Monique is the energetic Speech Language Pathologist (SLP) who will do the swallow study, followed by 2 hours of speech and language evaluation.  JimBob connects with Monique immediately.  She is direct and in charge.  JimBob especially appreciates the ways in which Monique respects his dignity. 

The study will allow Monique to visualize JimBob's swallowing in real time.  She begins with a teaspoon of radio-opaque liquid, flavored with Hershey's syrup.

Monique stands in front of JimBob as he swallows the teaspoon of liquid.  In a formal tone, she queues the radiology technician to capture the dynamic image of swallowing which is visible to her, and to JimBob, on a small screen next to the x-ray camera pointed at JimBob's neck.

"Now," she calls out.  

JimBob and Monique watch the x-ray image appear.

A flash of white liquid glides across JimBob's tongue, down his pharynx, and into his esophagus.  No aspiration.  This is good. 

"Stop," Monique commands.  The screen goes blank.

Next they repeat the test with an ounce of the same Hershey's flavored liquid.  And after that, 2 ounces of the same liquid, followed by radio-opaque pudding.  Monique is visibly relaxing as all of the studies are normal.  JimBob wonders how often Monique is the one to break the bad news about unsafe swallowing due to the progression of ALS.

They finish the study with a radio-opaque capsule, to test if it's safe for JimBob to swallow pills.  Since he swallows 27 capsules a day, JimBob is relatively confident this will make it a clean round on the swallow study circuit.

And, it does.

They move on to Monique's office for tongue twisters, reading aloud, and oral-motor gymnastics-- things they all know will one day be impossible for JimBob.

For today, JimBob is happy to repeat them faster than Monique.  

Next are the tests for language processing which include verbally interpreting a drawing of people at the beach enjoying various waterfront activities.  JimBob expresses concern for the man looking at his phone while the smiling woman on the blanket in front of him is opening a bottle of wine.  After JimBob has completed his timed description, Monique comments that she shares his concern for the man, and has never before heard anyone call attention to their bare feet.  

Monique now administers the test that asks for lists of words beginning with a, s, and f.  Then a list of animals.  JimBob has just completed these tests with the neuropsychologist yesterday. Today he does not have concerns about cursing, and he sails through the test with flying colors, and a knowing wink from PeggO.   (See NIH Day #2)

Monique's final test involves ordering a fictitious executive's schedule based on the restrictions described in an accompanying narrative.  Naturally, JimBob suggests they pass this along to an able administrative assistant.

"I rather assumed you might say that," chuckles Monique, with a nod toward the test that means: "get to work."

Right.

JimBob buckles down and solves the tedious puzzle that includes when to order flowers for the wife and still see his most important customer, while making all of the day's deadlines.

Now it's time for a real lunch in the lobby.

1 p.m. Electrophysiology Lab.   

There is a solid rationale for saving the EMG and nerve conduction studies until last.  They are generally presented to patients as "somewhat uncomfortable."

JimBob and PeggO asked about the need to repeat these studies at the NIH, since they were completed at UVA as part of the diagnostic work up for ALS.  The recommendation from everyone was to repeat the EMG, since it had been done at such an early point in the disease progression.  Repeating the test now should be helpful in a number of ways.  The nerve conduction study, on the other hand, was comprehensive the first time and would unlikely need to be redone. 

Entering the electrophysiology lab, JimBob and PeggO are introduced to a short, gray-haired woman in a long white lab coat.  She speaks sternly with a thick Eastern European accent.  Her face shows intense concentration.  She does not smile.  She gestures to two chairs by the wall and instructs JimBob and PeggO to have a seat.  Next, she hands JimBob a blue paper gown that she has produced from a gray, metal drawer.  As she leaves the room, presumably to allow JimBob some privacy, she chuckles to herself, saying the teenagers always want to take the blue paper gown home.  Huh?

PeggO and JimBob look at each other and need no words to express their hesitation for how this is likely to unfold.

The exam starts with an ultrasound exam of various muscles looking for fasciculations, or tiny muscles twitches.  Along with progressive weakness, fasciculations are the hallmark of ALS.  Clinicians debate whether fasciculations precede weakness.  If they do, then they may be able to predict the spread of the disease throughout the body.  

JimBob's fasciculations started in his left arm.  Currently, he feels fasciculations all day long in his arms, chest, back and abdomen.  The twitching is exacerbated by activity.  This means he has more fasciculations after many ordinary tasks like making the bed, working in the garden, typing, or standing for more than 10 minutes.  With a bit of rest, the twitching quiets down, until the next wave of activity.

Today's ultrasound and EMG will provide objective, scientific evidence of JimBob's everyday experience of fasciculations.

As she finishes with the ultrasound exam, the neuroelectrophysiologist (the white-haired woman in the long white coat) moves to the other side of the room and begins to pull the nerve conduction device closer to JimBob.  

JimBob starts to get nervous.  He remembers this experience all too well from the first time. 

A nerve conduction study measures the speed of electrical current running along a nerve.  To do this a receptor tab is placed near the end of the nerve, say at the wrist, and an electric probe is placed firmly against that same nerve, say in the elbow.  The neuroelectrophysiologist shocks the nerve repeatedly, with increasing voltage, sending a wave of electricity down the nerve.  The speed is recorded at different voltages, and the probes are placed along many nerves throughout the body to complete the test.

The first shock in each series feels like a little tingle and is easily tolerated.  As the voltage increases, the shock feels like an electric fence at the farm, and then like a shock from an electric outlet, and finally, the shock causes the limb to involuntarily jump off the table. The maximum shock is then repeated 3 times.

I'm not kidding.  This really happens.

The study begins and JimBob immediately remembers the "discomfort".  As they approach the maximal voltage, he instinctively clutches the edge of the exam table, bracing himself for the jolt of electric current.  

PeggO sits behind JimBob as the nerve conduction study progresses.  She is out of his sight line, but he senses her rise out of her chair with each maximal voltage. On the third sequence of  shocks, PeggO asks forcefully, "Is this really necessary?"

An awkward silence hovers in the room.

"This is the way I like to do these studies," responds White Coat, without turning away from her instruments.

"I knew we needed to repeat part of the EMG, but it was my understanding that the nerve conduction would not be required.  Has something changed?" asks JimBob.

No response.

With the next series of shocks, as JimBob's left foot and lower leg come jumping off the exam table, PeggO moves to get out of her chair, and White Coat calls it quits.

"He is so hyper-reflexic; I'm not sure this is worthwhile.  And it is obviously not his favorite, " says White Coat to her assistant.  She says this sitting next to JimBob...her hand still resting on his leg.

Not worthwhile?  Not his favorite?

What is going on here?  Who is this for?

Next test: the EMG.

An EMG measures the electrical activity in muscles, especially as the muscles are stimulated by nerves.  To do this, small needles are placed in muscles all over the body, one at a time.  The needles are connected via a wire to the EMG unit which measures the electrical activity in that particular muscle.

White Coat begins by placing the EMG needle into JimBobs left shin.  She observes the readings on the EMG unit, and adjusts the needle by wiggling it around in the muscle or pushing it deeper into the muscle.  Once she has the reading she needs, she asks JimBob to flex the muscle as hard as he can with the needle in place.

White Coat repeats this in JimBob's left thumb, both biceps, and finally in JimBob's neck and back.  She decides against the tongue since that was tested the last time JimBob had an EMG.  

Wrapping up the consultation, White Coat sits facing JimBob and PeggO and delivers the news. The EMG confirms that JimBob has fasciculations in his arms, chest, back, and abdomen.

I'm not kidding, this is a true story.

3:30 Final Wrap-Up with Dr. Kwan

JimBob, PeggO and Dr. Kwan sit alone in a small, quiet exam room.  Pale yellow weariness hangs like a fog as Dr. Kwan begins to speak.  

"Without patients who are willing to participate in research, we can make no progress in understanding ALS," he says.   "I am so grateful for your participation in my research."

Together, the three doctors summarize the positive outcomes of the three days.

• Blood samples may detect HERV-K leading to an anti-retroviral treatment trial
• 85th percentile for rate of decline in people living with ALS
• Excellent results from neuropsychological testing
• Excellent forced vital capacity
• Excellent swallow study and speech
• Electrophysiological results that support a diagnosis of ALS
• No further need for EMG or nerve conduction studies
• No further risk for Frontal-Temporal Dementia
• A connection to the NIH and to Dr. Kwan for future consultations.

When it's time for closing questions, PeggO begins.  "Does the NIH have plans to expand this study to become longitudinal, tracking patients' progress over time?"

"Wouldn't it be more helpful to understand how and why patients progress over time, rather than just having data from one point in time?" she asks.

"Absolutely.  We would love to do that; however, at this time we do not have the bandwidth for that kind of study," responds Dr. Kwan gently.

JimBob clarifies, "Will every piece of data collected in the three days be used for research?"  

Dr. Kwan assures him it will.  

As they stand to say goodbye, Dr. Kwan thanks JimBob and Peggo once again.   It feels to JimBob like Dr. Kwan would welcome an embrace.   More than likely, ALS takes its toll on the doctor's heart too.

The three doctors linger, bowing slightly, tipping their heads, smiling and saying thanks and goodbye a few more times.

4 p.m.  

On the way back to the hotel, JimBob and Peggo begin to realize their cumulative fatigue from three full days of highs and lows at the NIH. 

Each evaluation held the power for good news or disappointment.  Each test brought the possibility for increased hope or further evidence of functional decline.  Each encounter carried its own risk, and required JimBob and PeggO to muster steady courage, stamina and good humor.  

5 p.m.on the road back to Charlottesville

"Well, are you glad we came," asks PeggO from the driver's seat.

"I am," responds JImBob, "I'm grateful for the opportunity." 

"I hope it helps someone down the line.," he continues.

"You never know,,, you might be HERV-K positive and then you could try ARVs" says Peggo with a hopeful tone.

"That would be so cool..." nods JimBob, "I would really like the chance to try ARVs."

'I want you to take ARVs too... it would be amazing if ALS could be the next HIV story," adds PeggO.

"Totally," says JimBob, looking out at the tree-lined streets of the passing Bethesda neighborhoods.

"It's kind of unbelievable that no one at NIH is running longitudinal studies for ALS," reflects PeggO.

"And there is no central coordination, like in the HIV days," adds JimBob. "There really doesn't seem to be anyone in charge, just a bunch of silo'd labs doing their own things."

"It's enough to be a patient, and now we're supposed to figure out how to get the NIH organized," grumbles PeggO.

 As they move into rush hour traffic, the mood in the car goes blue-gray. 

"I thought you were going to jump across the room when she kept zapping me for the nerve conduction," interjects JimBob, knowingly changing the subject.

"I was ready to,,,"  says PeggO without missing a beat. 

"I could tell.  I could totally feel your energy in the room.  It was great," smiles JimBob.

"We're a team," responds PeggO.

"Yep," sighs JimBob quietly looking away, "We're a team."

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PeggO & JimBob visit NIH: Day #2

 9 a.m. 

The day begins with Peak Flow Testing in the Respiratory Therapy Department on the 5th floor.  JimBob is an old pro at PFTs.  PFTs are a hallmark data point for every patient with ALS, and the test is repeated at every ALS appointment.  

ALS causes neuromuscular degeneration, which sadly means ALS causes the diaphragm to weaken over time.  Patients with ALS lose their speaking voice because they do not have enough strength in the diaphragm to force air across the vocal cords.  In the end, most ALS patients die of respiratory failure. 

PFTs are a high stakes test in the land of ALS.  The results are quoted like marathon times, with splits at 10K and the Half.    JimBob knows his PFT numbers.  JimBob always goes for a PR.  Always.

The testing involves wearing a blue plastic clothespin that is produced from a clean cellophane wrapper.  A disposable mouthpiece is attached to a long hose that is perched in front of JimBob who is ready to race.  The hose drapes across a short void, linking it to a large, foreboding, stainless steel machine where the respiratory therapist (RT) stands to watch the results pour in.

JimBob's challenge is to blow as much air as possible through the hose, with as much force as possible, for as long as possible. JimBob watches the face of the therapist. His secret goal is to make her eyes pop out when she sees his numbers. 

Actually, this test is less a marathon and more like a sprint.  JimBob gets three attempts to best his PR of 120% for FEV1 and FVC.  Of course, most people are happy with 100% predicted for height and age.  Not JimBob, no way.

And today is his day!

"Quite remarkable," says the RT nodding. "124% predicted."

"It was all your coaching," JimBob says smiling.

Lefty and Righty are doing a happy dance while JimBob glides out of the lab to find PeggO. 

"Excellent," says PeggO.  "So far it's a very good day."

9:35 a.m.

En route to Starbucks, before the next appointment, JimBob and PeggO find themselves in one of many long hallways, this one lined with black & white photos of mostly white men.  Anthony Fauci is among them.  The photos honor the NIH scientists who have won the prestigious Lasker Award.  The Lasker is known in academic circles as the American Noble.

As JimBob reads their names and accomplishments, he is surprised to see Bernard Beryl Brodie, the namesake of PeggO's chair in medicine. Bernard B. Brodie (August 7, 1907 - February 28, 1989) was the first scientist to determine how neurohormones, like serotonin, effect the functioning of the brain.  PeggO is a wisdom scholar at the University of Virginia, School of Medicine and this was their first meeting.

10 a.m.  Neuropsychological Evaluation

A small percentage of people living with ALS develop Frontotemporal Dementia.  The neurologists refer to it as FTD.  People with FTD have personality and behavior changes, like crying and cursing inappropriately.  They also have problems with decision making, and language.  

Tell a person with a rare disease, like ALS, that the chances are slim they will develop dementia, and they might just look at you, thinking OK, but I already have a rare disease and both my parents died with dementia.

JimBob is ready for high stakes test #2. 

The psychologist is all business.  They will have 2 hours to get through at least a dozen tests of reasoning, memory, language, logic, and depression.

Fortunately for JimBob, most of the tests are like puzzles.  And JimBob likes puzzles.  

"No one is meant to get 100% on these tests," instructs the psychologist.  She smiles behind her mask, seated across from JimBob as though they are about to begin a match of chess.

This does not relax JimBob.  He is ready to roll.

Test after test, puzzle after puzzle, they zoom along.  The squiggly image of a double-decker bus is a tip-off for a test designed in Britain.  This is useful to JimBob when a teapot, that does not look much like a teapot, pops up later.  Cha Ching.

JimBob's favorite test required listing as many words as possible starting with the letter F.  It is a timed test: 60 seconds.  After F, they repeat the process with words starting with S, and again with words beginning with A.

The psychologist must write down all of the words as they are spoken.  

Stop watch in hand, the psychologist actually says, "Ready, Set, Go!"

JimBob cruises along until he slams hard against the wall of expletives.  He must not say too many expletives for fear of exposing the personality changes associated with FTD.

JimBob's mind races temporarily as he tries to get past "Fuck", and "Fucker" in the F's; "Shit" and "Shitfaced" in the S's; and "Asshole" in the A's.  Frustrated and humored at the same time, JimBob tosses off Aardvark as a final A.

Little did JimBob know at this low point in testing that his peak effort was about to transpire gloriously: name as many animals as possible in 60 seconds.

Again, aardvark made the list.

JimBob's facility for animal names drew completely from the travel journals he has kept with his family over the years, collecting a lengthy log of animals sighted on vacations.  JimBob began by continent, then zoos, then regions of the USA and finally to the common household and barnyard animals.  When the psychologist ran out of room on her paper, she asked him to stop, well before the 60 seconds had elapsed.

JimBob had been determined not to be demented, and it turns out he is not.

After lunch, the much discussed spinal tap.

1 p.m.  PeggO and JimBob enter an outpatient procedure room for a lumbar puncture, commonly known as the spinal tap.  This is a purely elective procedure to collect samples of JimBob's spinal fluid for research purposes. 

As a pediatrician, JimBob has done many LPs on babies as part of the newborn sepsis workup. "In the right hands," an LP is generally easier than drawing blood on a baby.  JimBob is not worried, although he detects a bit of angst in the room.  PeggO decides to wait outside due to the perceived angst.  

"You'll be fine.  I'll be right outside."  PeggO does not really want to leave, but she is an expert at reading the room.

JimBob sits on the edge of the bed, hunched over the bedside table.  Taryn, their NIH nurse practitioner chats about what she is doing to prepare.  Dr. Kwan offers a pillow for the bedside table.  They have reviewed the risks in two separate occasions as part of consent.  There are no real benefits to JimBob, since this is for research. 

They all chat collegially.

The first stick is a no go.  Too low.  No worries it happens.

The second stick produces a sharp pain in the spine and left flank.  

"Yeouch!" JimBob yelps, prompting Dr. Kwan to come around to the other side of the bed.

"The spinal fluid is flowing.  She got it," he says softly.

Dr. Kwan gives Righty a few gentle pats, and just before it might seem like a simple "there, there" pat, Dr. Kwan allows his hand to rest gently with Righty.

"She is almost done.  The fluid is clear.  Everything looks good."

And the LP is done.  Taryn has 4 tubes of spinal fluid on ice, and is on her way to the 2 labs who need ALS spinal fluid for their research.

PeggO returns and reads JimBob's face.  She lets him be his cheerful patient self. She knows they will talk later.

Dr. Kwan wants JimBob to lie flat for an hour.   To make use of the time, they deliver a large IPad which connects to the genetic counselor for neurology.  She uses the hour to collect a genealogical history, since 20% of ALS is genetically familial.  Gratefully, JimBob's genetics have already been tested and he is in the clear on this one.   

4 p.m.

On the way home, PeggO asks about the pain in JimBob's back.  JimBob asks about what the pain might be.  PeggO speaks in a reassuring doctor's voice.  The pain is not unusual; an auxiliary nerve likely got tweaked as the needle went in.

"You can take a couple of Advil and lie down while Delta and I go for a run.  Then, we'll find some Mexican food for dinner."

PeggO's voice reveals the weariness they are both feeling from a day of scientific inquiry.  JimBob is fairly glad that NIH Day #2 is now in the books.