Brenda Perrott Williamson has written a number of articles and recently her first mystery short story was published in Kings River Life Magazine. Brenda was first reader/editor on her husband’s books, including his first book, THE THIRD BATTALION MISSISSIPPI INFANTRY AND THE 45TH MISSISSIPPI REGIMENT: A CIVIL WAR HISTORY. That book answers the questions her father had about what his great-grandfather Pvt. William B. Johnston did in the war. Pvt. William B. Johnston is pictured on the back cover of the book, posing in his uniform with his “Arkansas toothpick.” Brenda lives in Mississippi with her husband, Civil War author David Williamson, and their cat, Wilde Oscar. Brenda will be tweeting occasionally on the subject of CJD and her father via @CJDandmyfather and @BrendaPerrott and @WildeOscarCat.
To any great book lover and reader, the worst thing that could happen would be to lose the ability to read and, in my father’s case, to lose the chance to watch the development of a book that answers all of his questions about an ancestor. That is what happened when he contracted Creutfeldt-Jakob Disease (CJD), an infectious disease sometimes called “sporadic CJD,” whose variant form many think of as the British “mad cow disease” or Bovine spongiform encephalopathy (BSE). “Sporadic” CJD has long been in the United States, more strikingly since the 1920s when farmers began to feed cows feed that included dead cow carcasses rather than just grass or corn. My dad’s neurologist at Ochsner, Geffrey Ellison, M.D., told me that as a diabetic his immune system made him more susceptible to CJD.
My father was Rev. Talmage Wayne Perrott, United Methodist minister and sociologist, and he died of biopsy-proven Creutzfeldt-Jakob Disease (CJD) on Tuesday, July 19, 1983, at Ochsner Hospital in New Orleans, Louisiana, at age 55, just under a month from his 56thbirthday. Born in Pike County, Mississippi to Rev. Talmage D. and Ruby Johnston Perrott, he attended Southwest Mississippi Junior College and graduated from Millsaps with a B.A. in Philosophy. He also earned S.T.B. and S.T.M. (M.Div.) degrees at Boston University School of Theology, as well as all coursework toward a doctorate in theology, and a Master’s degree in sociology from Louisiana State University where he had also completed all coursework toward his Ph.D. in sociology.
He was a man interested in ideas, always reading, always having a book in hand or nearby on a table or desk. The idea of him getting an infectious dementia that would take away his memory, speech and early on his ability to read was a cruel reality no one who knew him could have imagined. His first major symptoms began to emerge in March 1983 when he was the minister of Napoleon Ave. United Methodist Church in New Orleans, Louisiana. He had a good memory, was very intelligent, and good (it is obvious in retrospect) at covering up his difficulties, but on that March Palm Sunday he was unable to deliver his sermon and from his New Orleans pulpit told his congregation that if he had known he had a virus he would have gotten a substitute. He did not deliver more of a sermon than that. (He had always preached extemporaneously, but had begun to rely on notes and I noticed back in January 1983 that his sermons, usually cogent and easy to follow, had become difficult for me to follow, although I attributed my inability to follow his train of thought to my own tiredness and lesser intellectual capacity.) His speech had begun to slow and he had become somewhat hesitant months before, but his first glaring symptoms (full speech impairment and memory loss) appeared in March 1983, when he, for all practical purposes, lost his ability to speak and could not remember his own home phone number. I first heard the words, “Creutzfeldt-Jakob Disease” from neurosurgeon Carlos Pisarello, M.D. in an elevator in Baptist Hospital in March 1983, and that diagnosis later proved to be all too accurate.
In retrospect, a significant sign of his impairment occurred much earlier in 1982 when he stopped writing me letters. He previously had written me several times a week – he loved writing letters and exchanging ideas in writing – but by October 1982 he had completely stopped writing me. When I asked him about it, he said he just didn’t have time. I knew he was hiding something, but mistakenly thought that his heart was bothering him – he had had a heart attack in 1976 and his brother had died of a second heart attack so I thought it likely that or perhaps that his diabetes was getting worse and tiring him out.
Since my father loved reading and discussing things, losing his cognitive abilities in this slow, inexorable manner was the very worst thing that could have happened to him. It was terrible to watch him lose his ability to read. I had been baffled by his rapid departure from our favorite Magazine Street bookshop but realized only after he was first hospitalized in March that he had been unable to read that February day and had exited the bookshop in a near panic though he refused to say why and simply waited outside, something he had never ever done. Leaving a bookstore early was not something either of us would ever do.
I believe that my father’s exposure to CJD came from eating scrapie-infected barbecued goat brains during a church dinner at Pisgah United Methodist Church in Pike County, Mississippi in May 1978. (Scrapie is the disease as it appears in squirrels, goats and deer and it might be easier if we referred to them all as one of the transmissible spongiform encephalopathies or TSEs.)
He had a partially missing tooth at that time, a potential route of infection that Dr. Helen Grant and other CJD researchers have written about. Still, he had other potential sources of infection. He and his mother ate squirrel brains on many an occasion and in the mid-1970s he was sole caretaker of a pet baby goat that had every symptom of scrapie including the staggering gait whose burial he also handled. And, in the early 1970s a pet lamb, which may have had scrapie, had had its little throat slashed by a roaming pack of neighborhood dogs and my dad had been the one who, without gloves, had cleaned up and buried the poor animal.)
Many “sporadic” CJD patients have regularly eaten animal brains or have been in contact with a variety of potential contaminants as my father was on multiple occasions. For example, many times someone has first said to me that their loved one did not eat animal brains, but then rethought it and realized they had eaten tripe in potted meat or animal brains some years before after all.
It should also be remembered that “Mad Cow Disease” (BSE) was spread through European herds largely through the use of infected bone meal feed and that imported bone meal, as well as untested domestic bone meal, is routinely fed to our herds.
Unfortunately, this disease CANNOT be killed by routine autoclaving of hospital instruments. I believe, therefore, it is urgent and long overdue that hospitals should have a “rule out” list for those entering hospital emergency rooms with unknown neurological disturbances to avoid spreading the disease through contaminated instruments. Unbelievably, I have found that there are still neurologists who remain unaware that any form of CJD exists in this country so more education on this issue is clearly needed.
Daddy was always reading, always had a book in his hand. He had hoped to write one of his own someday. Many of his letters to me discuss what a novelist might do, how to make descriptions more vivid, and what different writers did and should have done. He dedicatedly wrote letters throughout his life – until his last year or so. Even as a poor and busy graduate student at Boston University getting little to eat every day, working, studying– sometimes living all day on a 5 cent breakfast discounted for students like him who worked in the cafeteria – he devotedly wrote my grandparents regular letters. I am glad that I lived far enough away for a bit of time in law school and afterwards to be the recipient of his wonderful letters. I hope that one day this awful disease will be taken seriously and stopped.
Additional Suggested Readings on the Subject of CJD and TSEs:
DEADLY FEASTS: TRACKING THE SECRETS OF A TERRIFYING NEW PLAGUE by Pulitzer-prizewinner author, Richard Rhodes.
MAD COWBOY: PLAIN TRUTH FROM THE RANCHER WHO WON’T EAT MEAT by Howard F. Lyman and Glen Merzer
LETHAL SOUVENIR by Patrick Singh, a sad account of the author’s daughter who contracted variant CJD while living in England and first evidenced symptoms while living in the United States, and is considered to be the first variant CJD patient in the U.S.
THE PATHOLOGICAL PROTEIN: MAD COW, CHRONIC WASTING AND OTHER PRION DISEASES by Philip Yam