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.”
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.
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.