The treatment of the “untreatable” patient-revisited

Abstract

The limits of medicine have not yet been reached. Numerous human illnesses initially thought to be incurable are reversible under unique and unpredictable individual circumstances. This paper, and the preceding companion publication, describes instances of the successful treatment of patients previously labeled as untreatable, including instances of severe ulcerative colitis and Crohn’s disease.

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“We simply cannot predict what will happen to every given patient.”

— James S. Goodwin and Jean M. Goodwin - 1987

The recent (2002) social visit with a former, now healthy patient, first seen 54 years earlier, prompted the review of my 1960 paper entitled “The Treatment of the Untreatable Patient”[1]. In that publication, I described the improvement and recovery of a group of patients, some with organic disease (cancer, inflammatory bowel disease, and septicemia) and some with emotionally-related disorders who responded to sustained nutritional aid and emotionally supportive therapy after having been evaluated elsewhere as “medically untreatable.”

My former patient, R.K., had immigrated with his parents to the USA from Germany in 1938. Now age 66, and in good health, he was first seen by me in 1948 when he was 12 years old. He had been diagnosed at two major medical centers as having severe ulcerative colitis, “untreatable medically”, and “requiring a total colectomy and ileostomy”. His parents then had brought him to the University of Chicago for admission to my hospital service. Physical examination revealed a pale, undernourished boy, obviously sick. Diagnostic studies confirmed the presence of severe idiopathic ulcerative colitis involving the entire colon. Laboratory tests documented the presence of a severe iron-deficiency anemia and a lowered serum albumin of 2.8 grams (normal 4.0 grams).

In the 1940s and 1950s, it was possible at the University of Chicago Medical Center, and very helpful, to keep seriously ill patients in the hospital for extended periods of time, and R. K. remained for approximately three months. Therapy initially included blood transfusions, nutritional support, and sulfonamides. Supportive psychotherapy, emphasizing personal improvement and ultimate recovery, was provided for the patient. The patient’s parents were informed daily as to progress and provided daily encouragement. In an ambience of optimism, expectations of success, and the involvement of an enthusiastic and caring hospital staff, the patient began to improve. Steroids, immunosuppressive compounds, and biological agents, of course, were not yet available. After the anemia had been corrected with several blood transfusions and the serum albumin had been restored to normal, with infusions of human albumin, the diarrhea, rectal bleeding and the abdominal discomfort gradually diminished. His appetite improved and he began to gain weight. When he returned home after three months in the hospital, he also had increased in height. At home, the improvement continued without interruption. After six months, and in the absence of any symptoms, all medications and dietary limitations were discontinued. At age 21, he was 6’2” tall and weighed 190 lbs. He has remained well ever since. He married and raised a family of three children and 8 grandchildren, all healthy. Today, he continues in excellent health and is a successful businessman. In retrospect, R.K. and his colon were not “medically untreatable” initially, but rather difficult to treat. What was the therapeutic difference It was probably the sustained therapy, the optimistic approach, and the strong nutritional and psychosocial personal support. The patient himself includes the interest, skill and unwavering optimism of his physician.

While less common than hoped for, such instances of recovery from ulcerative colitis are not rare, as described in earlier papers[2,3]. In a second recent experience, A. T., first seen by me in 1952, with severe ulcerative colitis, had developed the disease in 1951; the treatment included nutritional and emotional support supplemented with injections of ACTH. The colitis recurred intermittently until 1959, when the patient “changed his life style” and “totally eliminated stress from his life”. The diagnosis of “multiple polypoid defects” on barium examination performed elsewhere, in 1991 had led to a recommendation of colectomy; but follow-up examinations found no evidence of colonic polyps. Expert colonoscopy was performed in April 2000 at the University of Chicago, and except for a mild “vascular distortion”, was unremarkable; colonic biopsies were negative for dysplasia. The patient has remained free of symptoms since 1960, now continuing for more than 40 years. Living in Hawaii, he organized and became president of a successful chemical company. In this patient also, the explanation for the apparent recovery from ulcerative colitis remains unclear: although “the avoidance of stress,” may be a contributing factor. Noteworthy in both patients with ulcerative colitis, therapy had emphasized nutritional and psychological support. In each instance, IBD improvement had coincided with personal achievement; issues rarely emphasized in today’s world of molecules and genes. Biologic therapy, cytokine inhibition and gene transfer are exciting treatment possibilities for the future; but until these complex measures are realized, and perhaps irregardless of their availability, treatment of inflammatory bowel disease should continue to emphasize detailed attention to the nutritional and personal needs of the patient.

The word “untreatable” does not appear in Webster’s dictionary; the Random House Dictionary of the English language, the American Heritage dictionary, nor in Roget’s Thesaurus. Webster’s Medical Desk dictionary blandly defines untreatable as “not susceptible to medical treatment”. Medicine has never fully accepted Hippocrates’ observation[4], “.... for it is impossible to make all the sick well....”, and has made remarkable progress in reducing the ranks of the “untreatable”. Indeed, as the Goodwins[5] have indicated: “Twentieth Century Medicine can be seen as a systematic denial of the impossible. ” Nevertheless, uncertainty in the course of human disease, and in its prognosis has characterized Medicine, throughout history as reviewed by J. Barondess[6] and by R. Fox[7].

The designation “untreatability” in medicine is both arbitrary and absolute. It may be a relative term, reflecting non-cooperation of the patient or an inadequate therapeutic approach, rather than the illness itself. Occasionally, “untreatability” is the inappropriate label for an initially erroneous diagnosis, as illustrated in the following patient, first described in 1960. A.J., a 62-year old photo-engraver, in 1940, had developed severe pain in the epigastrium and in the back with nausea, vomiting, and weight loss. Surgery at another hospital demonstrated an “inoperable carcinoma of the pancreas,” and the abdomen was closed. The patient then was referred to the University of Chicago for possible chordotomy to relieve the intractable pain. Here, the pain and vomiting continued. The alert hospital resident noted the vomitus as clear, watery-appearing, fluid; and having learned earlier that gastric acid secretion is a watery appearing fluid; he tested the vomitus with Töpfer’s reagent, and indeed identified the fluid as gastric secretion containing hydrochloric acid. The intragastric instillation of hydrochloric acid reproduced the pain and vomiting, and gastric aspiration, removing the acid secretion, relieved the pain. X-rays demonstrated a huge duodenal ulcer crater. Large amounts of calcium carbonate hourly, day and night, atropine sulphate, and frequent gastric aspirations were required to diminish the pain and heal the ulcer, events consistent with duodenal ulcer, penetrating into the pancreas, causing pancreatitis. The surgeon presumably had misinterpreted the inflammatory thickening of the pancreas as neoplasm. In August 1941, an appendectomy was performed for acute appendicitis; and confirmed the absence of carcinoma. Ulcer recurrences necessitated a supradiaphragmatic vagotomy; and gastroenterostomy in June 1944, with complete recovery. He later succumbed to a heart attack 35 years after the initial erroneous diagnosis of pancreatic carcinoma.

At times, the determined patient provides the vital spark, transforming an apparently hopeless situation into one of renewed hope and improved health. A striking example, cited in the 1960 paper, is that of a man, then 53 years old, with a history of alcoholism, cirrhosis of the liver and malnutrition, who was hospitalized with ascites and thrombophlebitis of superficial veins in the abdominal wall. X-rays revealed not only esophageal varices, but also a linitis plastica-type carcinoma of the stomach. When he became confused, the situation appeared hopeless indeed. It seemed only humane to grant the patient’s plea for simply orange juice and coffee. Astonishingly, instead of deteriorating, he improved. The thrombophlebitis subsided; the ascites gradually diminished, and in several weeks the patient returned home. Additional history revealed that he had swallowed lye during childhood, causing the gastric deformity simulating neoplasm. The patient survived for many years thereafter; and occasionally revisited us to demonstrate his continuing viability. The hepatic disease eventually became “untreatable”, and he died following a massive hemorrhage from esophageal varices; but had he been untreatable initially

Patients with emotional difficulties and associated gastrointestinal symptoms may be especially challenging, because of the difficulty in fully understanding emotionally generated symptoms[8,9] prematurely prompting the label of “untreatability”. This important problem has been reviewed recently[10]. Useful therapy includes the exclusion of structural disease and evaluation of the life situation of the patient, a reassuring explanation of the physiologic basis of the symptoms, avoiding criticism, and sustained supportive therapy[11]. Medication alone often does not suffice in these circumstances, and the art of medicine is as necessary as its science. Indeed, hope, faith and trust, together with sustained therapeutic effort salvage more sick people than many physicians realize[12,13]. H.G. Wolff[14] has pointed out that “hope, like faith and purpose in life, is medicinal”.

The significant beneficial impact of religious beliefs in some patients with chronic illness also has been documented[15]. The treatment of such patients, often is prolonged, and demanding; but the satisfaction following successful therapy is one of the rewarding personal experiences in the practice of medicine. An example of this type of “medical untreatability”, noted in the 1960 paper, is that of M.V., then a 50 year-old man, who repeatedly sought treatment for digestive symptoms, including a bitter taste, belching, flatulence, constipation and diarrhea, abdominal pain, and fatigue; diagnosed as an irritable bowel. Many laboratory and diagnostic studies were normal; and many consultations with physicians in other areas of medicine did not reveal additional problems. Psychiatric evaluation of the patient included the following: “... a passive, inhibited male... his hypochondriacal expressions are at once expressions of his primary concern with himself and defenses against object relationships (which are threatening because of their homosexual character...)... Psychotherapy could be of little value and is not indicated in the present circumstances”. However, the patient was regularly employed. In time he became involved in the affairs of his community and was recognized for this activity. The many medical visits undoubtedly provided him with a measure of support, though the continuing need for reassurance reflected limited acquired understanding of his situation. The patient was perhaps “untreatable” in that his basic emotional difficulty was not fully resolved. However, he was relatively treatable in that, with support, reassurance, and personal attention, he was able to function as a useful citizen. Since then, modern research has documented significant biological and physiologic relationships between mind and body[3,16,17] and has provided a scientific basis for the understanding and management of emotionally associated gastrointestinal symptomatology.

The limits of medicine, as these clinical experiences illustrate, are by no means rigid or irreversible. While much has been learned about human illness and its molecular nature, there is much more knowledge yet to be acquired about individual human illness[18], its neurological, immunological and genetic dimensions. Hopefully, as expressed by Barondess[6], “... through continued research and technologic innovation, fueled by curiosity and personal drive, by institutional agendas and by the insightful and compassionate physician in the service of human needs”, additional progress can be anticipated in the successful management of the “untreatable patient.”