EDITORIALS
head extended, mouth widely open, a tongueblade gently applied to the anterior tongue. Strong light should be used. A normal epiglottis will usually appear. If obstruction is particularly severe, a lateral x-ray film of the neck should be made to confirm the anatomical process and, as the group from UC San Diego point out, a physician should be present. If one suspects the rare case of epiglottitis, the lateral x-ray film of the neck should be obtained first, with the team of clinician, otolaryngologist and anesthesiologist in attendance; if the roentgenogram indicates epiglottitis, the same team should transport the patient to the operating room, continue the diagnosis by direct, controlled visualization, then carry out a tracheostomy, obtain pharyngeal and blood cultures and initiate parenteral antimicrobial therapy. Although nasotracheal intubation has been used successfully by several centers throughout the country for the management of acute epiglottitis, the majority of clinicians and surgeons will wish to employ swift, facile tracheostomy. There is no place for anticipatory nonsurgical observation, inhalation of racemic epinephrine or use of systemic corticosteroids when a conclusive diagnosis of epiglottitis has been made. A perusal of the nuances located between the lines of the recent literature and personal observation suggest that lateral x-ray films of the neck do not always provide a conclusive diagnosis of epiglottitis. Tracheostomy is seldom required for a child with acute viral laryngotracheitis. Careful, sequential, clinical observation in the setting of modern intensive care units, supplemented by the judicious use of arterial blood gas analyses, will determine the candidates for tracheostomy. Nasotracheal intubation is inappropriate for this illness because of the damage incurred by inflamed, edematous mucosa. While mist tents represent a likely therapeutic myth for pneumonitis and bronchiolitis, in our experience topical aerosolization of distilled water can diminish dramatically the subglottic pathophysiology of laryngotracheitis. The humidification of concomitantly administered oxygen is adjunctive and reasonable since ventilation-perfusion dysequilibrium, highlighted by hypoxemia and eucapnia, occurs with laryngotracheitis. Indeed, these respirovirus infections probably represent diffuse nasopharyngolaryngotracheobronchopneumonitis. Intensive parenteral fluids may achieve the same therapeutic result, if restorative oxygen could be shown to be superfluous. Recent controlled studies of the use of
intermittent racemic epinephrine in the management of acute laryngotracheitis suggest that the beneficial effect is short-lived, the natural history of the disease is unaltered, and the most compelling benefit is the delivery of aerosol to the subglottic region. Corticosteroid therapy of laryngotracheitis remains an enigma. One would anticipate that antiphlogistic therapy should be beneficial, but, in fact, only one of numerous controlled studies indicates that corticosteroids hasten the clinical recovery of children with acute viral tracheobronchitis. Drama, controversy, sustained inquiry and new knowledge insure the continued viability of a biomedical topic; the vicissitudes of the diagnosis and treatment of acute viral laryngotracheitis and bacterial epiglottitis offer such a compelling agenda. JOSEPH W. ST. GEME, JR, MD Professor and Chairman Department of Pediatrics Harbor General Hospital, Torrance University of California, Los Angeles School of Medicine
The First Decade of Federal Medicine IT IS NOW almost exactly a decade since the federal government began to become seriously involved in the day-to-day delivery of medical care. Before 1965 there had been substantial and very productive federal support of medical research but direct intervention in medical education and patient care had been mostly talk with very little action. The national election of 1964 was interpreted by those elected to be a mandate for massive government action in the field of health care. During 1965 the Congress passed the first of what was to become a series of laws and programs attempting to deal with the widely proclaimed "health care crisis." While there were many who doubted the crisis was real in 1965 it is safe to say that whatever crisis there was has become a real mess in 1975, the result of attempts to administer poorly conceived laws and of various court actions which have also profoundly affected patient care. The clock cannot be turned back. That alternative is not open to us. Rather the clock ticks on. The only value of looking back is to see what can THE WESTERN JOURNAL OF MEDICINE
307
EDITORIALS
be learned that may be of use now or in the future. As one reviews the past decade, with its wave after wave of new health legislation, one is impressed with how much the costs have risen while there has been comparatively little improvement in health or health care directly attributable to the government programs, and with the fact that most of the federal programs simply have not worked. Rather federal programs have imposed a variety of regulations, requirements and restrictions which have had the effect of shackling the delivery system and increasing the cost of doing the same thing and very little more, when the goal was to bring many more services to many more people. Medicare might be viewed as an exception to this. Medicare seems to be running fairly smoothly and serving its purpose of helping the elderly with their substantially greater health care needs, although the program does not adequately meet the costs of the care rendered to its beneficiaries, particularly when they are in hospital, and forces the subsidization of Medicare patients by other hospital patients, providers or other sources. One cannot help wondering whether the congressmen and senators who passed the bills and the presidents who signed them into law really ever understood all the implications and ramifications of the health legislation of the past decade. Of course they did not-nor could they have all been expected to. And if they themselves did not understand it, to whom did they look for advice and guidance? The record of the past decade suggests that they have depended heavily on their own staffs and the power seeking bureaucracies in the Department of Health, Education, and Welfare. The input from the real world of medical practice and patient care, from those who must live with the legislation and try to make it work, has not been particularly welcome and has been relatively ineffectual in the places where these laws and programs have been decided upon and designed. Again, Medicare itself (Title XVIII of the Society Security Act) might be an exception. This was discussed over a period of years and the input from the real world of practice was loud and clear. Perhaps this has something to do with why Medicare seems on the whole to have worked better than most"of the other programs. If it is true that much of the difficulty with the health legislation of the past decade has been that it has been conceived, enacted and administered without "including in" the real world of health
308
OCTOBER 1 975 * 123 * 4
and those who must make it work if it is gowork, then who is to blame and what can be done to improve things in the future? As is usual in such situations there seems to be enough blame to go around for everyone, whether in the public or private sector, and part of the trouble may be a weakness in the democratic system itself, both in and out of government. In any case it is entirely clear that the health legislation of the past decade has been something less than a cooperative effort between government and those in the private sector who must make the laws work. In fact it has been quite the opposite. Government has sought to impose its will upon the private sector (which it should actually be serving in our system) and the private sector has so far failed to come up with workable solutions to problems identified by government and others as unmet health care needs of the people. And it must be admitted that the democratic system, under which both sectors operate, simply does not adapt easily to long-range planning, to the problems of human interdependence whether in health care or anything else or to the pursuit of any single course of action over any considerable length of time. What is to be done? First there must be a counterforce to oppose the evident determination of the federal government to impose its will unilaLerally upon the private sector in health care. This has just started. The private sector has begun to seek relief from the courts and reluctantly resorted to limited job actions to make their points. But these activities, necessary though they may be, will never clear up the mess which is only getting messier. Rather they will add to it. A second step is needed. The private sector should begin to recognize the health care problems for what they are and develop its own goals and objectives for what should be done in health care, based on firsthand experience with the problems. This will require a coordinated effort in the private sector, perhaps through some kind of coalition of agencies and associations. This second step should lead to a third. Since neither the private sector nor government can do it alone, a new and truly cooperative relationship should develop between the two. In this relationship, as in all others, it should be the role of government to be the servant and not the master of the people. As a practical matter, this can only occur when the people, in this case those responsible for the delivery of health care, have recognized the uncare
ing
to
EDITORIALS
solved problems for what they are, and devised workable solutions or approaches to solutions for them. If such a genuine partnership between government and the people, including the health professions, were ever to come about, it might also then be possible to address the as yet unsolved problem of long-range planning within a democratic system, and the steady pursuit of a single course of action over a considerable length of time-an accomplishment which is actually essential for the survival of any democratic society in today's interdependent world. The second decade of federal medicine, begun in 1975, is pregnant with opportunities to make our system work. It also carries the potential, perhaps even the likelihood, of compounding the mess which has been created. The first decade has been pretty well botched. Let us hope there will be a chance for us all to get together and do a better job of it in this next decade. -MSMW
Pancreatic Cholera PANCREATIC CHOLERA is a relatively rare clinical syndrome characterized by a non-beta islet cell pancreatic neoplasm, profuse watery diarrhea, hypokalemia and metabolic acidosis.1-3 Although in most of the published case reports patients with this syndrome have had an intraabdominal neoplasm or pancreatic non-beta islet cell hyperplasia,4 recently we and others5 6 have seen patients who have all of the clinical manifestations of pancreatic cholera but in whom no intraabdominal pathology is found at laparotomy or postmortem examination or both. Patients in whom no pathology is found constitute a particularly frustrating problem for clinicians, since such a patient's unremitting, fulminant diarrhea serves as a constant stimulus for physicians to carry out more extensive (and usually more invasive), hazardous diagnostic procedures to "find the tumor." Evidence that pancreatic cholera may be associated with extraabdominal neoplasms is found in the report by Said and Faloona5 of five patients in each of whom there was pancreatic cholera syndrome and bronchogenic neoplasm. This observation has obvious important implications for the diagnostic evaluation of patients with pancreatic cholera and one hopes that adequate
documentation of this association will be forthcoming in future publications. From the clinical standpoint the pathogenesis of pancreatic cholera originates with a pronounced net secretion of isotonic fluid along the entire length of the small intestine. It has yet to be established whether this net secretion results from decreased absorption, increased secretion or a combination of the two processes. Whatever the mechanism, the result is that the colon is presented with excessive volumes (as high as 20 liters per 24 hours) of isotonic fluid having sodium, potassium and chloride concentrations similar to those in plasma. The mechanism through which the colon reabsorbs sodium and chloride can be conceptualized as involving two "exchange processes": sodium-for-potassium and chloride-for-bicarbonate.7 Thus, colonic absorption of sodium and chloride is accompanied by an increase in the intraluminal concentration of potassium and bicarbonate. In the pancreatic cholera syndrome colonic absorption of sodium and chloride is maximal and there is an associated maximal colonic "secretion" of potassium and bicarbonate. The fecal loss of the latter two
ions is primarily responsible for the hypokalemia and metabolic acidosis seen in this disorder. The hypokalemic acidosis can be significantly ameliorated in some patients by continuously aspirating ileal fluid using a tube with its tip positioned just above the ileocecal valve. This procedure, however, significantly increases the patient's total body loss of sodium chloride and parenteral administration of these ions must be increased correspondingly. The large fecal losses of water and electrolytes in this syndrome necessitate careful monitoring of the patient's state of salt and water balance and vigorous parenteral replacement therapy. When patients are first seen they are usually dehydrated and occasionally hypotensive. As they are given intravenous fluid their stool output increases and the greater the amount of fluid administered parenterally the greater the fecal losses. The difficult clinical decision to be made under these circumstances is what should be the patient's daily intake of salt and water. For example, in a patient with fecal losses of 12 to 16 liters daily the physician is continuously trying to determine to what extent the patient's fecal output is occurring solely in response to parenteral fluid administration and to what extent the fecal volumes represent the patient's "basal diarrheal state." Usually THE WESTERN JOURNAL OF MEDICINE
309
head extended, mouth widely open, a tongueblade gently applied to the anterior tongue. Strong light should be used. A normal epiglottis will usually appear. If obstruction is particularly severe, a lateral x-ray film of the neck should be made to confirm the anatomical process and, as the group from UC San Diego point out, a physician should be present. If one suspects the rare case of epiglottitis, the lateral x-ray film of the neck should be obtained first, with the team of clinician, otolaryngologist and anesthesiologist in attendance; if the roentgenogram indicates epiglottitis, the same team should transport the patient to the operating room, continue the diagnosis by direct, controlled visualization, then carry out a tracheostomy, obtain pharyngeal and blood cultures and initiate parenteral antimicrobial therapy. Although nasotracheal intubation has been used successfully by several centers throughout the country for the management of acute epiglottitis, the majority of clinicians and surgeons will wish to employ swift, facile tracheostomy. There is no place for anticipatory nonsurgical observation, inhalation of racemic epinephrine or use of systemic corticosteroids when a conclusive diagnosis of epiglottitis has been made. A perusal of the nuances located between the lines of the recent literature and personal observation suggest that lateral x-ray films of the neck do not always provide a conclusive diagnosis of epiglottitis. Tracheostomy is seldom required for a child with acute viral laryngotracheitis. Careful, sequential, clinical observation in the setting of modern intensive care units, supplemented by the judicious use of arterial blood gas analyses, will determine the candidates for tracheostomy. Nasotracheal intubation is inappropriate for this illness because of the damage incurred by inflamed, edematous mucosa. While mist tents represent a likely therapeutic myth for pneumonitis and bronchiolitis, in our experience topical aerosolization of distilled water can diminish dramatically the subglottic pathophysiology of laryngotracheitis. The humidification of concomitantly administered oxygen is adjunctive and reasonable since ventilation-perfusion dysequilibrium, highlighted by hypoxemia and eucapnia, occurs with laryngotracheitis. Indeed, these respirovirus infections probably represent diffuse nasopharyngolaryngotracheobronchopneumonitis. Intensive parenteral fluids may achieve the same therapeutic result, if restorative oxygen could be shown to be superfluous. Recent controlled studies of the use of
intermittent racemic epinephrine in the management of acute laryngotracheitis suggest that the beneficial effect is short-lived, the natural history of the disease is unaltered, and the most compelling benefit is the delivery of aerosol to the subglottic region. Corticosteroid therapy of laryngotracheitis remains an enigma. One would anticipate that antiphlogistic therapy should be beneficial, but, in fact, only one of numerous controlled studies indicates that corticosteroids hasten the clinical recovery of children with acute viral tracheobronchitis. Drama, controversy, sustained inquiry and new knowledge insure the continued viability of a biomedical topic; the vicissitudes of the diagnosis and treatment of acute viral laryngotracheitis and bacterial epiglottitis offer such a compelling agenda. JOSEPH W. ST. GEME, JR, MD Professor and Chairman Department of Pediatrics Harbor General Hospital, Torrance University of California, Los Angeles School of Medicine
The First Decade of Federal Medicine IT IS NOW almost exactly a decade since the federal government began to become seriously involved in the day-to-day delivery of medical care. Before 1965 there had been substantial and very productive federal support of medical research but direct intervention in medical education and patient care had been mostly talk with very little action. The national election of 1964 was interpreted by those elected to be a mandate for massive government action in the field of health care. During 1965 the Congress passed the first of what was to become a series of laws and programs attempting to deal with the widely proclaimed "health care crisis." While there were many who doubted the crisis was real in 1965 it is safe to say that whatever crisis there was has become a real mess in 1975, the result of attempts to administer poorly conceived laws and of various court actions which have also profoundly affected patient care. The clock cannot be turned back. That alternative is not open to us. Rather the clock ticks on. The only value of looking back is to see what can THE WESTERN JOURNAL OF MEDICINE
307
EDITORIALS
be learned that may be of use now or in the future. As one reviews the past decade, with its wave after wave of new health legislation, one is impressed with how much the costs have risen while there has been comparatively little improvement in health or health care directly attributable to the government programs, and with the fact that most of the federal programs simply have not worked. Rather federal programs have imposed a variety of regulations, requirements and restrictions which have had the effect of shackling the delivery system and increasing the cost of doing the same thing and very little more, when the goal was to bring many more services to many more people. Medicare might be viewed as an exception to this. Medicare seems to be running fairly smoothly and serving its purpose of helping the elderly with their substantially greater health care needs, although the program does not adequately meet the costs of the care rendered to its beneficiaries, particularly when they are in hospital, and forces the subsidization of Medicare patients by other hospital patients, providers or other sources. One cannot help wondering whether the congressmen and senators who passed the bills and the presidents who signed them into law really ever understood all the implications and ramifications of the health legislation of the past decade. Of course they did not-nor could they have all been expected to. And if they themselves did not understand it, to whom did they look for advice and guidance? The record of the past decade suggests that they have depended heavily on their own staffs and the power seeking bureaucracies in the Department of Health, Education, and Welfare. The input from the real world of medical practice and patient care, from those who must live with the legislation and try to make it work, has not been particularly welcome and has been relatively ineffectual in the places where these laws and programs have been decided upon and designed. Again, Medicare itself (Title XVIII of the Society Security Act) might be an exception. This was discussed over a period of years and the input from the real world of practice was loud and clear. Perhaps this has something to do with why Medicare seems on the whole to have worked better than most"of the other programs. If it is true that much of the difficulty with the health legislation of the past decade has been that it has been conceived, enacted and administered without "including in" the real world of health
308
OCTOBER 1 975 * 123 * 4
and those who must make it work if it is gowork, then who is to blame and what can be done to improve things in the future? As is usual in such situations there seems to be enough blame to go around for everyone, whether in the public or private sector, and part of the trouble may be a weakness in the democratic system itself, both in and out of government. In any case it is entirely clear that the health legislation of the past decade has been something less than a cooperative effort between government and those in the private sector who must make the laws work. In fact it has been quite the opposite. Government has sought to impose its will upon the private sector (which it should actually be serving in our system) and the private sector has so far failed to come up with workable solutions to problems identified by government and others as unmet health care needs of the people. And it must be admitted that the democratic system, under which both sectors operate, simply does not adapt easily to long-range planning, to the problems of human interdependence whether in health care or anything else or to the pursuit of any single course of action over any considerable length of time. What is to be done? First there must be a counterforce to oppose the evident determination of the federal government to impose its will unilaLerally upon the private sector in health care. This has just started. The private sector has begun to seek relief from the courts and reluctantly resorted to limited job actions to make their points. But these activities, necessary though they may be, will never clear up the mess which is only getting messier. Rather they will add to it. A second step is needed. The private sector should begin to recognize the health care problems for what they are and develop its own goals and objectives for what should be done in health care, based on firsthand experience with the problems. This will require a coordinated effort in the private sector, perhaps through some kind of coalition of agencies and associations. This second step should lead to a third. Since neither the private sector nor government can do it alone, a new and truly cooperative relationship should develop between the two. In this relationship, as in all others, it should be the role of government to be the servant and not the master of the people. As a practical matter, this can only occur when the people, in this case those responsible for the delivery of health care, have recognized the uncare
ing
to
EDITORIALS
solved problems for what they are, and devised workable solutions or approaches to solutions for them. If such a genuine partnership between government and the people, including the health professions, were ever to come about, it might also then be possible to address the as yet unsolved problem of long-range planning within a democratic system, and the steady pursuit of a single course of action over a considerable length of time-an accomplishment which is actually essential for the survival of any democratic society in today's interdependent world. The second decade of federal medicine, begun in 1975, is pregnant with opportunities to make our system work. It also carries the potential, perhaps even the likelihood, of compounding the mess which has been created. The first decade has been pretty well botched. Let us hope there will be a chance for us all to get together and do a better job of it in this next decade. -MSMW
Pancreatic Cholera PANCREATIC CHOLERA is a relatively rare clinical syndrome characterized by a non-beta islet cell pancreatic neoplasm, profuse watery diarrhea, hypokalemia and metabolic acidosis.1-3 Although in most of the published case reports patients with this syndrome have had an intraabdominal neoplasm or pancreatic non-beta islet cell hyperplasia,4 recently we and others5 6 have seen patients who have all of the clinical manifestations of pancreatic cholera but in whom no intraabdominal pathology is found at laparotomy or postmortem examination or both. Patients in whom no pathology is found constitute a particularly frustrating problem for clinicians, since such a patient's unremitting, fulminant diarrhea serves as a constant stimulus for physicians to carry out more extensive (and usually more invasive), hazardous diagnostic procedures to "find the tumor." Evidence that pancreatic cholera may be associated with extraabdominal neoplasms is found in the report by Said and Faloona5 of five patients in each of whom there was pancreatic cholera syndrome and bronchogenic neoplasm. This observation has obvious important implications for the diagnostic evaluation of patients with pancreatic cholera and one hopes that adequate
documentation of this association will be forthcoming in future publications. From the clinical standpoint the pathogenesis of pancreatic cholera originates with a pronounced net secretion of isotonic fluid along the entire length of the small intestine. It has yet to be established whether this net secretion results from decreased absorption, increased secretion or a combination of the two processes. Whatever the mechanism, the result is that the colon is presented with excessive volumes (as high as 20 liters per 24 hours) of isotonic fluid having sodium, potassium and chloride concentrations similar to those in plasma. The mechanism through which the colon reabsorbs sodium and chloride can be conceptualized as involving two "exchange processes": sodium-for-potassium and chloride-for-bicarbonate.7 Thus, colonic absorption of sodium and chloride is accompanied by an increase in the intraluminal concentration of potassium and bicarbonate. In the pancreatic cholera syndrome colonic absorption of sodium and chloride is maximal and there is an associated maximal colonic "secretion" of potassium and bicarbonate. The fecal loss of the latter two
ions is primarily responsible for the hypokalemia and metabolic acidosis seen in this disorder. The hypokalemic acidosis can be significantly ameliorated in some patients by continuously aspirating ileal fluid using a tube with its tip positioned just above the ileocecal valve. This procedure, however, significantly increases the patient's total body loss of sodium chloride and parenteral administration of these ions must be increased correspondingly. The large fecal losses of water and electrolytes in this syndrome necessitate careful monitoring of the patient's state of salt and water balance and vigorous parenteral replacement therapy. When patients are first seen they are usually dehydrated and occasionally hypotensive. As they are given intravenous fluid their stool output increases and the greater the amount of fluid administered parenterally the greater the fecal losses. The difficult clinical decision to be made under these circumstances is what should be the patient's daily intake of salt and water. For example, in a patient with fecal losses of 12 to 16 liters daily the physician is continuously trying to determine to what extent the patient's fecal output is occurring solely in response to parenteral fluid administration and to what extent the fecal volumes represent the patient's "basal diarrheal state." Usually THE WESTERN JOURNAL OF MEDICINE
309
