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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