I hate that machine. I REALLY hate that machine. Late one August afternoon in 2005, my wife and I were in the waiting room of the Pediatric MRI department at Emory Hospitals in Atlanta. I was waiting my turn for a brain scan in my 3rd year of the PREDICT-HD observational study. We were a little out of place in Pediatrics, but my scan had to be done on a particular type of MRI machine. One poor little tyke was bawling his eyes out while being prepped for a scan. I felt like joining him.
My turn came and I told myself to stop acting like a baby and just get the thing done again. I got on the table and made sure the technician covered my eyes well to block out the sight of being rolled into a coffin. The scan began and with the magnets banging around my head, I sang songs to myself and thought about a large cold drink afterwards and a pleasant dinner in our favorite Atlanta restaurant. The 15 minutes passed fairly quickly and I got the heck out of there.
Later that evening my wife and I nursed glasses of wine after dinner at South of France, a remarkable little restaurant in a nondescript shopping center. A singer named Berne? softly sang La Vie En Rose as well as Edith Piaf did. I had forgotten the MRI machine and the stress of the day?s testing completely. All that remained was the quiet satisfaction of being a research subject.
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The day had begun back at the Center of Excellence at 9 that morning with paperwork. The study is looking for subtle, early indicators of disease onset in people without symptoms. In addition to the MRI every other year, it involves annual cognitive assessments, physical exams and neurological and psychiatric testing. Joan Harrison, the Emory study coordinator, keeps a large notebook for each of the Emory participants. There are over 500 of us around the world.
After answering some general questions about my health during the year, I updated my supplement list (many) for Joan and my medications (still none). She drew 3 vials of blood to be used for year-to-year comparisons and then turned me over to a neurologist, Dr. Claudia Testa. Joan went back to interview my wife about my behavior over the past year. Spouses are an integral part of the study and complete an extensive series of psychological and behavioral questionnaires about their gene positive better half.
The neurological exam is the standard Uniform Huntington?s Disease Rating Scale (UHDRS) series of tests, and it?s all captured on videotape for another doctor at the main research site at the University of Iowa to evaluate and score. The most public part is done out in the hallway of the clinic ? the heel-to-toe walk, or as I jokingly refer to it, the ?HD Sobriety Test.? I?m pretty good at that because I practice it for balance exercise. I resisted the impulse to show off for the cameras by doing it backwards after the required 10 steps forward.
One previous year, Dr. Testa was hobbling around in a cast after some knee surgery. After my sobriety test, she was heading back to her office and I yelled, ?Try it heel-to-toe! It?s not as easy as it looks!? She laughed good naturedly.
Then it was back to Joan for my own psychiatric evaluation. I filled out a number of questionnaires about my thoughts and behaviors. One that gave me a chuckle was the ?Disgust? test. One symptom of HD is the loss of the ability to be disgusted at things. You rate situations as ?not disgusting?, ?moderately disgusting? or ?extremely disgusting?. One of the tamer situations described was seeing someone in a restaurant eating with their hands. I was glad there was still some time before lunch after that test.
The psychiatric portion was followed by an extensive ?scratch and sniff? test to evaluate sense of smell, which might be an early indicator of disease onset. I felt kind of silly sniffing the daylights out of a little book to try to decide if that particular smell was cloves or turpentine. After that intensive olfactory workout, my wife and I broke for a quick lunch.
After lunch, the hard work began. I was honored to be tested by Dr. Randi Jones, a renowned HD researcher. She administered a battery of motor and cognitive tests that were more draining than running a marathon. Many of them were computerized, including several versions of the finger ?tap? tests similar to the one used by HDDW which the late Jerry Lampson was instrumental in developing.
Several tests that weren?t computerized were devilish. I found out that I am terrible at remembering a series of numbers and repeating them back. I was fine for about 5, but when it went to 7 and beyond my brain shut down on me. When Randi tried to get me to repeat them back alternating the sequence I had to admit total defeat. Another test that threw me was coming up with as many words as possible starting with a particular letter, in a limited period of time.
Perhaps the most devilish of all was a three part cognitive test. For the first, you were given a sheet that had rectangles of colors (red, blue, green etc.) in rows running across. You had to say the colors out loud as quickly as possible, moving left to right and then to the next row. The second part had the words for the colors, in the colors that they represented: Red Blue Green Blue Red Blue You had to repeat the colors that the words were written in. The third part was mind boggling. Again you had to say the colors, but they didn?t match the words: Red Blue Green Blue Red Blue
After my poor performance with numbers and words I was pleased to have done pretty well with that test, or so I thought. I?ll find out when the final study results are in after 4 years. PREDICT-HD participants aren?t told anything until the end of the study, although I?m sure you?d be told if a brain tumor showed up in an MRI.
There were two other computerized tests that I enjoyed, and got quite competitive while doing them. Randi chuckled several times at my intensity. One was using a cursor to move three different sized disks from one post on the left to one on the right. There was a post in the middle to use for intermediate moves. The rule was that you could only place a disk or disks on top of a larger one. After a bit of visualization I figured that one out fairly quickly.
A more difficult one involved a series of two spheres that appeared on the computer screen. Each had a different pattern ? lines on one half, cross hatches on the other half, for example. The rules of the test were simple. One was ?right? and the other was ?wrong?. If you clicked on the ?wrong? one, the computer emitted an obnoxiously loud buzz. If you clicked on the ?right? one, it gave you a pleasant ?ping?.
There was a rule for which one was right, and you had to figure it out by trial and error. For example, the right one might be the sphere with lines on the left side of the sphere, but only if it showed up on the left side of the screen. Once you figured it out, you got a pleasing chain of pings, until a new series with a new rule began.
After several hours of intensive work, I?m sure both Randi and I were ready for the end. It finally came, and I leaned back in my chair, drained. All I had left was my brain MRI, and I wondered if I might be tired enough to take a nap while in the machine. No such luck. Before we left for the hospital, Joan and Randi thanked both of us for participating. Research subjects at Emory are treated like royalty, which leaves a very warm feeling.