Pinals, Robert S. MD
Among American fiction writers, Flannery O'Connor has always been a marvelous anomaly.1-3 Since her death in 1964, her reputation has continued to grow, despite the limited volume of her work (2 short novels and 19 stories) and its quirky, provincial subject matter.4,5 She wrote about life in rural Georgia, where she spent most of her adult years, almost as an outsider. She was a devout Catholic in an evangelical Protestant world, an unmarried woman living in her mother's family farm, and a closet intellectual among her rustic neighbors. Yet, from this limiting experience, she imagined characters of enduring interest and complexity, in stories that combined themes of violence, comedy, and spirituality.
Flannery O'Connor developed systemic lupus erythematosus (SLE) in 1950, a time when most physicians were just becoming aware of this seemingly rare and generally fatal condition, because of the recent discoveries of a diagnostic test6 and an effective treatment.7 Most of the details of her illness, and her remarkable efforts to overcome its devastating consequences, are derived from her letters.5 A previous report of her case, dealing mainly with her personal struggle to cope with SLE, was published in 2003.8
ILLNESS AND DEATH
When she graduated from Georgia State College for Women in 1945, Flannery O'Connor had already decided that she would be a writer. She attended writers' workshops in Iowa and upstate New York, forming relationships that would sustain her for the next 2 decades.
Her first significant health problem occurred in December 1949 (age 24 years) when she returned to her home in Milledgeville, GA, for a holiday visit. She became seriously ill, but there was no description of her symptoms. Surgery was performed to correct a "floating kidney," and she was hospitalized for a month thereafter.5
In December 1950, while retyping the manuscript for her first novel, Wise Blood, she developed heaviness and aching in both arms. She was examined by a physician in Connecticut, where she was staying with friends, and told that she probably had rheumatoid arthritis. Further studies were recommended upon her return to Georgia for the holidays. Her symptoms, now including fever, worsened during the train trip, and she was hospitalized immediately upon arrival by her family physician, an internist. She was treated with a new "miracle drug," cortisone, with improvement in pain, but continued to have a high fever. Presumably there were laboratory results suggesting kidney involvement, because her physician called Dr Arthur Merrill, Georgia's first nephrologist. Merrill felt that the picture was compatible with SLE; he suggested that she should be transferred to Emory University Hospital, and this was accomplished in January 1951. Letters several years later suggest that she may have had a typical facial rash early in her illness. She was found to have a positive lupus erythematosus cell test and received 10 transfusions for anemia. She was placed on a strict low-salt diet, and cortisone was eventually replaced with adrenocorticotropic hormone (ACTH) injections 4 times a day. The frequency of injections was reduced, and she was discharged after a month on ACTH once daily.
In June 1951, she was well enough to return to her friends, the Fitzgeralds, in Connecticut. She learned from them that her diagnosis was SLE; her mother had asked her physicians to withhold this information for fear that the deadly prognosis would be too dispiriting. O'Connor was familiar with "lupus." Her father had died of this disease in 1941 at age 45 years, only 4 years after it first appeared as a discoid patch on his forehead.
In July 1951, she developed fever and other symptoms suggesting a viral illness. Dr Merrill gave her 2 transfusions and increased her ACTH dose. In January 1953, she wrote, "I am doing fairly well these days, though I am practically bald-headed on top and have a watermelon face," suggesting that she had significant alopecia and cushingoid features.
In early 1954, she developed a limp and was told that she had "rheumatism" in the hip. In 1955, she substituted the "newest wonder drug, Meticorten" (prednisone) for ACTH and was able to add salt to her diet. However, her hip pain had progressed, and she required crutches. X-rays later showed "softening of the top of the leg bones due to failure of the circulation to the hip." This condition, aseptic or avascular necrosis, resulted in progressive pain and disability. She was hospitalized late in 1960 at Piedmont Hospital in Atlanta for further studies. O'Connor wrote to a friend: "My last x-rays were very bad, and it appears the jaw is going the same way as the hip is. I had noticed a marked change in the position of my mouth." Two days later, she wrote to another friend, "What they found out at the hospital is that my bone disintegration is being caused by the steroid drugs which I have been taking for 10 years…. So they are going to try to withdraw the steroids…." Her SLE had been clinically inactive during this interval, and apparently, this was confirmed by laboratory studies. Dr Merrill probably referred to O'Connor's case in an editorial9 in 1961 on the use of steroids in renal disease; he described an SLE patient on prednisone 4 to 6 mg/d for 9 years, who developed bilateral aseptic necrosis of the femoral heads after 6 years and of the mandibular condyles after 8 years. Apparently, steroids were tapered during the first half of 1961. In May, she was taking chloroquine and mentioned that steroid injections in both hips a month previously had produced benefit for only 2 weeks. Hip surgery was under consideration, but 2 orthopedic consultants had conflicting opinions.
After steroid withdrawal, O'Connor's correspondence mentioned no new health problems, until Christmas day in 1963, when she reported to a friend that she had fainted several days earlier and was restricted to bed. She was found to be anemic, apparently due to vaginal bleeding, thought to be caused by a fibroid tumor. On February 25, 1964, she was hospitalized in Milledgeville for a hysterectomy. Dr Merrill had been concerned that surgery might provoke a flare-up of SLE and expressed a preference for performing the operation in Atlanta. The procedure appeared to be successful, and she received intravenous steroids for 3 days postoperatively. She returned home on March 5, but developed cystitis 2 weeks later and wrote to a friend on March 28, "I suspect it has kicked up the lupus again. Anyway, I am full of infection and am back on the steroids."
She returned to the hospital for 10 days the third week in April because "…I woke up covered from head to foot with the lupus rash." In late May, she was admitted to Piedmont Hospital in Atlanta for anemia, weakness, and a 20-lb weight loss. Her blood pressure was "dangerously" high. She was placed on a low-protein diet, suggesting that she was azotemic, and received 4 transfusions because her hemoglobin was "down to 8." After almost a month, she was discharged home. She was growing weary of the hospital, and there were hints that her insurance was running out. "My dose of prednisone has been cut in half on Dr Merrill's orders because the nitrogen content of the blood has increased by a third." In late July, she received another transfusion and "a double dose of antibiotic for the kidney… and they are withdrawing the cortisone." However, soon afterward, she became critically ill and was rushed to the local hospital. She lapsed into coma and died on August 3, 1964. She was 39 years old. In her letters, she frequently expressed distress over her appearance, with moon face and alopecia. During the summer, she had finished rewriting her last story, Judgement Day. The last line was: "Now she rests well at night, and her good looks have mostly returned."
In late 1950, Flannery O'Connor had onset of arthralgia, myalgia, fever, and facial rash. She was hospitalized and found to have severe anemia, abnormalities on urinalysis, and a positive lupus erythematosus cell test. The latter6 had been described in 1948 and was available in most academic hospitals in 1950. The use of cortisone and ACTH to control symptoms7 of SLE had been described earlier in 1950, and she responded well to what was then considered to be a high dose of cortisone (150-250 mg/d, equivalent to 30-50 mg/d of prednisone). No details of her nephropathy are available, but it was recognized that this did not improve as dramatically as other SLE manifestations.7
One might speculate about the possibility that her "serious illness" requiring hospitalization a year earlier might have represented the onset of SLE. The diagnosis of "floating kidney" (nephroptosis) was frequently proposed as an explanation for pain in the back, abdomen, groin, or flank and treated surgically (nephropexy) in the early 20th century10 In the absence of details of the illness, this question cannot be resolved. However, lupus nephritis is usually not a source of pain.
Multiple blood transfusions were given, suggesting that she had significant anemia, which may have been hemolytic. Symptoms of SLE were controlled on ACTH, but she became aware of cushingoid features after the first year. During the third year of her illness, O'Connor developed progressive hip pain, which was later attributed to avascular necrosis (AVN). This would be the main source of disability for the remainder of her life.
Avascular necrosis or osteonecrosis was first reported in SLE in 1960,11 but O'Connor experienced hip pain several years before this report, with radiographic confirmation of the diagnosis in 1955. High-dose corticosteroid therapy has been recognized as the most important risk factor,12 and more recent magnetic resonance imaging studies have shown that AVN, which is often asymptomatic, may start within a few months after initiation of corticosteroids.13
After 8 years of treatment, she noticed painless misalignment of her mandible, which was found to be due to AVN of the condyles; this is a rare site for steroid-induced AVN.14 The progressive course of AVN is not altered by reduction or withdrawal of steroids, but this was not appreciated in 1961. O'Connor's low-dose prednisone was tapered and withdrawn, and treatment with chloroquine was initiated. This seemed reasonable because her SLE appeared to be inactive.
Systemic lupus erythematosus did not recur for about 3 years after steroid withdrawal and was provoked by a hysterectomy. The exacerbation included anemia, rash, hypertension, and azotemia, with superimposed urinary tract infection. Reintroduction of steroids did not alter her fatal progression. Hemodialysis was available in 1964, but reports of its use in end-stage lupus nephritis were limited.15
Even without life-extending measures such as dialysis, O'Connor's survival exceeded expectations. She lived for 14 years after onset of SLE. A 1964 report of a large series of SLE patients, many of whom did not have renal involvement, found that only half were alive after 10 years.16 An earlier 1955 report, reflecting survival in the precorticosteroid era, found only half were alive after 4 years.17 O'Connor's excellent medical care may partially explain her longevity, but her personal qualities and lifestyle were probably even more important.
O'Connor coped with her illness by obsessive adherence to a daily routine, which gave the highest priority to her literary productivity. Her energy level was highest in the morning; she attributed this to steroid therapy. "Cortisone makes you think night and day until I suppose the mind dies of exhaustion"3 After the 7 a.m. morning mass, she wrote for 3 hours. Afternoon activities started with gardening (always wearing a broad-brimmed hat to shield her face from the sun). She tended to her chickens and other more exotic fowl, in which she had a lifelong interest. She had first come to brief national attention in the Pathe Movietone News as the 5-year-old Georgia girl who had taught a chicken to walk backward. Later in the day, she would read and correspond with friends and literary colleagues. From time to time, she received visitors. One of her biographers estimated that she made about 60 trips to give lectures or readings from her works in other cities, primarily at colleges and universities.3 During most of her years with SLE, O'Connor walked with crutches, but was functionally independent despite this handicap. Her bedroom was on the first floor, and she had few stairs to climb. She managed to travel alone by train or plane. She seldom complained or talked about her illness and regarded it as an obstacle to overcome and an opportunity for spiritual growth.
1. Gooch B. Flannery: A Life of Flannery O'Connor. New York, NY: Little Brown &Co.; 2009.
2. Cash JW. Flannery O'Connor: A Life. Knoxville, TN: University of Tennessee Press; 2002.
3. Simpson M. Flannery O'Connor: A Biography. Westport, CT; London, UK: Greenwood Press; 2005.
4. Fitzgerald S, ed. O'Connor: Collected Works. New York, NY: Library of America; 1988.
5. Fitzgerald S, ed. The Habit of Being: Letters of Flannery O'Connor. New York, NY: Farrar, Straus, and Giroux; 1979.
6. Hargraves MM, Richmond H, Morton R. Presentation of two bone marrow elements: the "tart" cell and the "L.E." cell. Proc Staff Meet Mayo Clin. 1948;23:25-28.
7. Soffer LJ, Levitt MF, Baehr G. Use of cortisone and adrenocorticotropic hormone in acute disseminated lupus erythematosus. Arch Intern Med. 1950;86:558-573.
8. Caldwell AL. Flannery O'Connor: life with lupus. J Med Assn Georgia. 2003;92:15-17.
9. Merrill AJ. Management of the nephrotic syndrome. Am Heart J. 1961;61:719-722.
10. Moss SW. Floating kidneys: a century of nephroptosis and nephropexy. J Urol. 1997;158:699-702.
11. Dubois EL, Cozen L. Avascular (aseptic) bone necrosis associated with systemic lupus erythematosus. JAMA. 1960;174:966-971.
12. Gladman DD, Urowitz MB, Chaudhry-Ahuwalia, et al. Predictive factors for symptomatic osteonecrosis in patients with systemic lupus erythematosus. J Rheum. 2001;28:761-765.
13. Oinoma K, Harada Y, Nawata Y, et al. Osteonecrosis in patients with systemic lupus erythematosus develops very early after starting high dose corticosteroid treatment. Ann Rheum Dis. 2001;60:1145-1148.
14. Cowan J, Moenning JE, Bussard DA. Glucocorticoid therapy for myasthenia gravis resulting in resorption of the mandibular condyles. J Oral Maxillofacial Surg. 1995;53:1091-1096.
15. Kimberly RP, Lockshin MD, Sherman RL, et al. "End-stage" lupus nephritis: clinical course to and outcome on dialysis. Experience with 39 patients. Medicine. 1981;60:277-287.
16. Kellum RE, Haserick JR. Systemic lupus erythematosus. A statistical evaluation of mortality based on a consecutive series of 299 patients Arch Intern Med. 1964;113:200-207.
17. Merrel M, Schulman LE. Determination of prognosis of chronic disease, illustrated by systemic lupus erythematosus. J Chronic Dis. 1955;1:12-32.
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