In May 2006, I was distressed to learn that my stepmother, Jerene Johnson, had begun experiencing an unexplained loss of cognitive function, which at the time was minor but seemed to be progressing. Extensive testing revealed several significant health problems, but none that could account adequately for her symptoms. The doctors kept saying “come back in a month and we’ll see how you are then”, but Jerene’s symptoms continue to worsen rapidly. Finally, a friend at the UCLA Medical Center pulled some strings, and Jerene was admitted to the hospital in late July, where she soon came under the care of the neurology team.

The UCLA neurologists quickly ruled out all of the most common causes for Jerene’s symptoms, and began to suspect an uncommon one. On July 28, we received the devastating diagnosis: Creutzfeldt-Jakob Disease (CJD), a degenerative and invariably fatal neurological disorder caused by protein pathogens called prions, the same infectious agent found in mad-cow disease. Jerene’s form of the disease was the so-called “sporadic” variant, which basically means that no one knows exactly how it happens; it’s probably caused by a random genetic mutation. Though sporadic Creutzfeldt-Jakob Disease is the most common variant, it is still exceedingly rare, with only about 300 cases per year in the United States—literally one in a million.

Since there is no treatment and no cure for CJD, there was no point in keeping Jerene in the hospital once the diagnosis was confirmed, so she was discharged to home care on July 31. During the subsequent month, her descent was extraordinarily rapid, even by the standards of CJD, which typically results in death between four and eight months after the onset of symptoms. Since I was involved in many aspects of her care, I could see how her cognitive function and motor skills declined on virtually a daily basis, resulting in an effectively comatose state within a couple of weeks. This meant that she was unaware of her condition and thus experienced no pain—one of the few mercies afforded by this terrible disease. She died on Sunday, August 27, 2006.

Since most readers of this post did not know Jerene, I would like to tell you a little bit about her, and especially about her influence on me. She was born on August 22, 1949, in Inglewood, California, and grew up in the city of Garden Grove in Orange County. As a child, Jerene was a precocious musician, and showed great talent for the piano in particular. By the time she was 13, she already served as an instructor for the younger students, in addition to pursuing her own more advanced piano studies. She was also an accomplished choral singer. Over the years, her piano skills were often on display, especially during the holiday season, when she would accompany our traditional family Christmas caroling (always in parts, of course). Jerene did much to foster my own love of music, especially classical music and musicals, and provided much encouragement as my interest in singing grew over the years. She was a particularly enthusiastic audience member at many Caltech Glee Club concerts.

Jerene and I shared another close affiliation. Starting when she was around 20, Jerene worked for several years as a secretary for Professor Harrison Brown in Caltech’s Division of Geology and Planetary Sciences. She loved Caltech, and even went so far as to marry two Caltech graduates (though not at the same time). Those marriages didn’t last, but her affection for Caltech and its denizens did. One story she particularly liked to tell concerned the Ricketts House annual Apache party and its infamous garter contest. Though Apache (as always) featured plenty of scantily clad women, in the late ’60s most girls would still stop their dates from pushing a garter all the way up. Jerene, never one to be prudish about such things, won the contest easily. She was especially pleased that part of her prize for winning was the honor of sitting on the lap of a certain well-known professor who frequented the party. (The Caltech people out there will no doubt already have guessed that the professor in question was every Techer’s favorite physicist, Richard Feynman.)

When my father married Jerene, now more than twenty years ago, he married into a large and loving family, one that embraced not only him but also me and my sister as well. Obviously, this has been an incredibly difficult time for us all, but, as hard as it has been, it would have been so much worse without the incredible support of the family and friends surrounding Jerene and my dad. It has certainly driven home for me the importance of my own network of colleagues, mentors, and friends. If you are part of that group, I’d like to thank you.