Editor's note: This is part of our continuing series of stories about Bruce Kramer, the former dean of the College of Education, Leadership and Counseling at the University of St. Thomas, as he copes with life after being diagnosed with ALS, also known as Lou Gehrig's Disease. You can read all the stories in the series by clicking here.
Nobody likes taking tests, whether in school or a doctor's office. But not everyone has experienced tests the way Bruce Kramer has.
"I didn't like being measured like this," Kramer said the other day, "but you have to get over that."
Kramer, of Minneapolis, has amyotrophic lateral sclerosis, or ALS. The condition takes a slow but inexorable toll as the disease robs limbs and organs of the ability to function.
Since his diagnosis in December 2010, Kramer's doctors have been monitoring his progression using a variety of tests and something called the ALS Functional Rating Scale, or ALS FRS. It's a 48-point system that gives doctors a snapshot of the patient's functional status: how he's moving, breathing, swallowing. The patient receives a numerical score for each function.
"The rating scale is helpful, I think, for them to decide where they need to put their resources," Kramer said. "You do get tired of the measurement and I've gotten to the point now with the rating scale where I can talk very openly...'Well, maybe I'm a 2 here, but I'm doing this, so that kind of qualifies me as a 3; but I'm not doing this, so maybe I'm a 1.'"
Although the score does not measure the approach of death, Kramer knows that he is dying, and his declining score on the FRS is a reminder of the direction he's heading.
"I watched myself go from a 43 to...in the teens," Kramer said. "I once asked Dr. Jones, 'Well, what happens if I hit zero? Do I die?' And he kind of said, 'Well, not necessarily.'"
Dr. Lyell Jones is Kramer's Mayo physician. He explained that a patient's score is "a useful tool, but it's not a perfect predictor of how long a patient will live with ALS. And to be honest, we really don't have a really ideal tool to measure or predict how long a patient will live with ALS."
"Usually, when the patient nears the end of life with ALS, the ALS FRS score is lower," Jones said. "It depends on a number of factors. Patients who begin with weakness of the breathing and swallowing muscles may not have an overall score as low as some other patients who have preserved function in those areas."
Kramer initially saw the tests as a challenge. "When I first realized there was a scale and where I was on the scale, you know," he said, "I treated it for two years as a competition and I didn't want to lose. Well, you know, we're all going to lose."
Eventually, he came to understand the scale as a useful tool, and he lost the fear that his medical team would focus on his disease instead of on him. "I just wanted them to see me as the human I was, and not a collection of symptoms or a set of measurements, and to their credit they're very good at that," he said.
Dr. Jones said that was no accident. "It's an important principle to recognize that we treat patients, we don't treat numbers that derive from measuring them," he said. "The numbers are a useful snapshot, if you will, but nothing really replaces how the patient is doing. So if I have a patient who has a low number but they're looking good and feeling good, I feel good, too."
Going on four years since his diagnosis, Kramer doesn't need a numerical system to tell him what he already knows: He's tired.
His weak arms and legs feel leaden, spent and sore, the way they did after the hundred-mile bike rides he took before ALS rendered his limbs useless. The energy it takes to eat and talk can be exhausting. The physical fatigue practically drapes itself over Kramer.
"I actually experience it visually," Kramer said. "It comes down over my eyes. The world gets a little grayer. My breath comes a little more difficult...The fatigue has been with me since the very beginning."
The effort it takes to get out of the house can be too tiring, so Kramer recently canceled his quarterly Mayo Clinic visit. At this point, he figures there's not much the clinic can do for him anyway. He has felt for months, he said, that he is "winding down" as more and more of his body's functions diminish.
Jones said that as a patient with ALS nears the end of life, there are usually problems with breathing and getting enough nutrition.
"So regardless of where a patient's ALS began in their body — if it began in a hand or a foot — eventually, when it comes to affect those muscles, that's usually when it determines the timing of the end of life," Jones said.
"It's hard to see patients get worse over time," he said. "But the patients themselves are remarkable people. You really learn what strength means, seeing a person deal with this disease over time...We hope that in the future we'll have better treatments for this disease, and so that helps keep us going."