Chronic Pulmonary Failure by C

Postgrad Med J: first published as 10.1136/pgmj.34.390.184 on 1 April 1958. Downloaded from 184

CHRONIC PULMONARY FAILURE BY C. 11. Fitis, M.D., F.R.C.P. Honlorary Physician, Royal Melbour-ne Hospital; Honorary Physician, Thor01acic Departmient, Austin Hospital for Chr-onic Diseases

About 15 years ago I was asked to report on the cardiovascular state of a man of 48, almost crippled for want of breath, in whom no adequate reason for the disorder could be found. This man had passed unscathed through the First World Wrar, and since then had farmed successfully a sheep station of so,ooo acres. His land bordered what is called the 'back country.' It is a land of richness in good seasons, but subject to prolonged and terrible droughts. It is a dry and healthy climate. Until he reached the middle forties, he never spared himself and never recalled being ill. Grad- ually thereafter he became vaguely aware that all was not well and he was easily exhausted. So rapidly had this feeling increased over the last 12 copyright. months that as I watched him, he was painfully distressed at the mere effort of removing his shirt. Yet had I turned away, I would have been un- aware of this distress for the dyspnoea was almost noiseless. He stood there stripped, over 6 feet in height, and gaunt almost to the point of emaciation. His neck muscles tugged at his upper chest and his http://pmj.bmj.com/ abdomen caved in with the inspiratory effort. Not only did his chest seem fixed but the skin was so tautly stretched as scarcely to obscure the outlines Fi(I.i-H.A., aged 63, has a history of pro- of the pectoral and intercostal muscles. A light gressive dysnpnoea for twelve years. Note percussion was enough to set these muscles into the anxious facies and the tenseness of the action like the spread of ripples in a pond. Not accessory muscles of respiration. The limbs and body are 'wasted to the of only was one aware of the silent nature of the point on September 27, 2021 by guest. Protected enmaciation. He had heen confined to hed dyspnoea, but on auscultation there w,as silence or for manv months wvith the mistaken diag- a near approach to it. It was as though the fetch lnosis of cardiac failure. Note the intranasal of wind had ceased before it reached the alveoli. catheter. He had long been addicted to On fluoroscopy one saw the small heart within oxygen. large lung fields, the lowN, disphragm immobile except for slight further flattening when instructed of a great nlumllber of people witlh emiipliysemiia, and to blow his stomach out, so typical of advanced in particular oni a close and lonig-continued study emphysema. An X-ray film of the chest showed of 34 patients, all of wvhom presented with the nothing other than emphysema. Here then was a symptom of dyspnoea rather than xvith asthma and man whose illness began with shortness of breath; bronchitis. who had never been subject to bronchitis or asthma; who had no history of pneumonia; whose Sex days were passed in an outdoor occupation in ani Of these 34 patienits, 33 were imieni. It has exceptionally healthy climate. always been recognized that the disease is more This clinical study is based on the observation common in men. Postgrad Med J: first published as 10.1136/pgmj.34.390.184 on 1 April 1958. Downloaded from April 1958 1'ITTS: ('ICronic Pulnmonarpv Failure Bronchiectasis This was found only once in the series. This man had proven bronchiectasis with postural cough and purulent sputum and clubbed fingers. Nevertheless the whole course of this illness was that of emphysema with recurring spontaneous pneumo-thoraces on either side. Incidentally, this was the only patient with clubbed fingers. Bronchitis This was described in six patients and in only one did it appear that the bronchitis might have been a problem before the advent of dyspnoea...... Wheezing TI'welve of these patients had a wheeze at some time. It tended to be a late phenomenon long after the patient complained of dyspnoea. Unlike the chronic asthmatic, it was rare to find the chest noisy with rales and rhonci despite the consider- able dyspnoea, and often no wheeze was audible, though it might be brought out with exercise. FiC. 2.-This youth suffered from polio- myelitis in childhood. He has gross Dyspnoea bilateral hronchiectasis but not emphx- sema. Despite cough and sputum he is Though the difficulty in breathing is generally not distressed. Contrast the facies an(d insidious in onset and in its early progress, at times the accessory respiratory muscles with patients will say that they became aware of it com- copyright. Fig. I. paratively suddenly. In the early stages they may use the terms exhaustion or fatigue rather than Age shortness of breath, but it is in fact the latter that The only woman was first seen at the age of 34. forces them to slow down or to stop during work. The average age of all patients when first seen was The silent nature of the dyspnoea is evident as the 5I. The duration of symptoms when first seen patient moves or undresses and on auscultation the greatly diminished breath sounds are in marked was not less than twelve months and up to 12 http://pmj.bmj.com/ The disease is, therefore, not one of elderly contrast to those heard in compensatory emphy- years. sema, in chronic bronchitis, and in asthma. With people. the relentless progress of the disease, certain characteristics of the dyspnoea may become ap- Occupation parent. It is a distinctive feature of this type of Most of these people had led active, athletic emphysema that the dyspnoea reaches its peak lives and their occupation shed no light on the after rising in the morning. Thus R.B., a Union actiology of the disease. There were four men secretary, aged 46, who sleeps well fully recum- on September 27, 2021 by guest. Protected who had been regular soldiers, two of whom had bent, describes his shortness of breath as being spent their service lives in the instructional corps. worse when he gets out of bed in the morning. He Trhey all had the parade ground posture, which gets some relief by standing up and leaning for- possibly aggravated their difficulty in coping witl ward, and says that he always eats his breakfast emphysema. standing up. He improves as the day goes on and is at his best between io a.m. and 4 p.m. Cold air Infection upsets him and he is worse after leaving the fire- Ten patienits gave a history of pneumoniia. In side. This story of the waxing and waning of the five of these the pneumonia occurred when em- dyspnoea is told over and over again. It must be physema had already been diagnosed, or long after admitted that once a certain stage has been the dyspnoea had become apparent. Only one reached, it is rare for the dyspnoea to be arrested patient related his illness to the pneumonia, but or appreciably retarded. Such people may be so he had been away from work for only two weeks, distressed that life narrows down to the problem and an X-ray film of his lungs showed no abnor- not of food to eat but of air to breathe. In their mality after his recovery. extremity, patients may sit rigid and immobile Postgrad Med J: first published as 10.1136/pgmj.34.390.184 on 1 April 1958. Downloaded from 186 POSTGRADUATE MEDICAL JOURNAL April I 938

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ge ?> ,...... i?:. .:? .. S Wl s: H2 ||?" . -.jpe at 22wi . EiE.,. >, |x, .xe - P;: - ::. i:}t: ,i..: .... axs . .:'] .:: .: .':: 'i.:: .. :. -. *::..*! ' FIG. 4.-X-ray of the chest of patient W.B. (Fig. 3). Note the paucity of the lung markings and extrene narrowness of the heart FiG;. 3.-W.B., aged 43. He first noticed silhouette. shortness of breath seven years ago wshen chopping wood. He had been an athlete be assumed that the presence of bronchitis with and a champion axeman. He had lost mucopurulent sputum provides the copyright. four stone in weight in seven years and is explanation totally disabled by dyspnoea. Note that for emphysema. Insufficient care may be given this man has not the conventional barrel to the sequence of events. Patients present so chest, and note also the development of often after years of illness with cough and sputum, the accessorv respiratory muscles. shortness of breath and a wheeze that the diagnosis of asthma and bronchitis with emphysema is made sipping air as though it were hot soup. Generally wrhen the right diagnosis is emphysema followed the descent is gradual but relentless, although it by bronchitis and bronchospasm. may be interrupted by episodes of more acute http://pmj.bmj.com/ dyspnoea. Season and climate, unwonted exer- Loss of Weight tion, or the onset of bronchial spasm may bring Nearly all of these patients were of light build about a moderate aggravation. Bronchitis or but there are few chronic diseases in wvhich loss of pneumonia may bring more serious results. These wveight proceeds so relentlessly over the years. patients are prone to episodes of high fever, which Three patients were seen in whom a provisional come with the suddenness of a malarial attack, diagnosis of carcinoma of the stomach had been often with intense pleuritic pain. A spontaneous made because of anorexia and loss of weight. on September 27, 2021 by guest. Protected pneumothorax may bring about a dramatic change, and so may the increase in tension in a bulla. Car- Physical Signs diac failure, commonly though not necessarily of The physical signs of emphysema are wNell knowNn the right side of the heart, may occur. Finally, hut uindue significance has been given to the there is the imponderable factor of the play of the barrel chest. I'his has not beeni an obvious emotions in the production of dyspnoea in a characteristic in the patients unlder discuissioni. person already oppressed by an awiareness of a bodily function he has always taken for granted Radiological Signs and by the effects of a long illness unresponsive to On fluoroscopic examination it may be found treatment. that the diaphragm is virtually immobile, the lung fields are easy to see, and the heart silhouette is Cough small. The pulmonary artery is unusually clearly The mere presence of a cough is not sufficient seen in the left oblique view and often becomes evidence upon which to base a diagnosis of bron- more prominent with the passage of time even in chitis in a patient with emphysema; nor should it the absence of right heart failure. Cardiac cathe- Postgrad Med J: first published as 10.1136/pgmj.34.390.184 on 1 April 1958. Downloaded from Ipril iI95 'I"IS: Chroni,c( J11/1/uouIry IFailu,rXe 1 87 I

F5I. v. A.F., aged 63. Ile first becanme aware of FIG. 6.-A.H., aged 63. 'I'he air has now been absorbed serious shortness of breath four years ago. This from the left pleural cavity. There is re-expansion X-ray at first sight suggests a complete left spon- of the upper lobe. The space in the left lower taneous pneumothorax. There is a pneumothorax, thorax is a large tension bulla compressing a certain but there is also a large tension bulla at the left amount of useful lung tissue. There is also bullous base (vide Fig. 6). emphysema in the right lung. copyright. terization, however, did not showr any significaint difficult and in these patients was nearly always rise in pulmonary artery pressure, yet there is a made by fluoroscopic examination or by an X-ray clarity about the lung fields and a paucity of vas- film of the chest. An increase of dyspnoea noticed cular markings in the periphery that is matched by the patient over one or more days and without only by pulmonary hypertension. an aggravation of bronchitis or a pulmonary infection should call to mind the possibility of a spon- Complications taneous pneumothorax. There are two complications which deserve The second complication, which is equally diffi- http://pmj.bmj.com/ special mention. The first is spontaneous pneu- cult to diagnose, is due to tension in an emphy- mothorax. This occurred seven times in 34 sematous bulla. The symptoms and signs are patients. Two of these patients had recurring commonly those of the less acute forms of spon- pneumothoraces, in one confined to the left lung, taneous pneumothorax. The bulla may reach an in the other affecting both lungs at different times enormous size and appear to occupy the whole of and also associated with mediastinal emphysema. the hemithorax. It will obscure and compress

The difficulties in diagnosis can be very great. The healthy lung tissue as can readily be demonstrated on September 27, 2021 by guest. Protected presence of a pneumothorax often cannot be by the re-inflation that follows thoracotomy and diagnosed by physical signs. On percussion there removal of the cyst. is no demonstrable increase in the resonance Recognition of these two complications may be already present because of emphysema, and on vital for the patient's survival and when treated auscultation the breath sounds are already they provide some of the few rewarding experiences diminished or absent. The symptoms may be in the management of emphysema. dramatic and the patient appear to be in acute circulatory failure. There is extreme shortness of Prognosis breath with shallow breathing, pallor changing to By coincidence the group of patients upon whom a dusky hue, sweating, a rapid weak pulse and a these observations are based include five patients fall in blood pressure. In a very few minutes the known intimately to the writer for more than 30 patient may be unconscious. Presumably these years. Two played first-class tennis, one was a are the symptoms and signs of anoxia, for recovery rowing blue, and two led the healthy outdoor life is equally dramatic when air is taken off and oxygen of the regular soldier between the two wars. Two given. In less acute forms, the diagnosis is equally of these men have died in great distress from what Postgrad Med J: first published as 10.1136/pgmj.34.390.184 on 1 April 1958. Downloaded from I POS'ITGRAD)UATE'I' \IED)ICAL JOURNAL A1Ypril 1958 might be termeicd pulmnoniary failure, in their early sixties. Two survive, but in their fifties are in chronic pulmonary failure. The fifth, whose history is known since early childhood, is now aged 55. He had pneumonia two years ago, but no previous history of chest infection. He makes little physical demands oo himself and is not often aware of his limitationis. There have been i dleaths ainid only one of these has been clearly due to cardiac failure. Most of these patients have died comparatively suddenly and one at least from an unsuspected pneumothorax. One patient quietly expired with breathing so shallow that he appeared merely to sip air. Two patients died with a terminal bronchopneumonia. F. .. T'wo patients have been seen with episodes of :i.. right heart failure, one being the only woman in [.,C:.. E.. the series. Both have been redeemed from their Ir ...d] u .l failure rather more easily than is the case with r:..:l -a. systemic hypertension and left heart failure. w-Z: Though one sees these patients over many years *2L anid the prospect is for years of life, the disease is a one. Indeed if we Fic. 7. Recurrent spontaneous pneurnothorax. distressing recognize malignant Note the partial collapse of the right lung hypertension, we should recognize that there is a and fluid levels in several eniphvseniatous malignant form of emphysema. hullace It the right hase. Treatment of recurring pneumothorax, and particularly when I know of no form of treatment mentioned in the collapsed lung fails to re-expand. Thoraco-copyright. the literature that has not been tried for these tomy, removal of the walls of bullae, and the re- patients. These include breathing exercises, the expansion of the remaining lung tissue with relief of infection, the control of bronchial spasm, obliteration of the pleural space can be a most the use of oxygen by mask, catheter and tent, the satisfying operation. The third indication which empirical use of cortisone, pneumoperitoneum, has been followed has been thoracotomy and abdominal binders and psychotherapy. Some of removal of bullae for the reason that they are dead these may be palliative; none has had any appre- spaces and a hindrance to satisfactory respiration. ciable effect on the progress of the disease. This has been the least successful operation. http://pmj.bmj.com/ On one occasion, when it seemed justifiable to try any method that might give the patient a rest Discussion from his misery, controlled respiration was used 'The 34 patients upon whose records this paper after anaesthesia with pentothal, preceded by om- is based all suffered from chronic pulmonary nopon and scopolamine and with the addition of failure. This term embraces the two functions of flaxedil. This was the most distressing case in the ventilation and respiration. Ventilation is a writer's experience, and at least it was shown that mechanical process concerned with the movement on September 27, 2021 by guest. Protected the anaesthesia did not endanger the patient's life. of air between the atmosphere and the alveolo- Every patient with emphysema should be taught capillary membrane. Failure of function is res- breathing exercises. It is uncommon for these to ponsible for dyspnoea. bring about much obvious improvement, but on Respiration is a physico-chemical process con- rare occasions they do so and it is not easy to cerned with the diffusion of oxygen from alveolar predict those who will have nothing to gain from spaces into the blood and the diffusion of carbon them. Emphysematous patients readily become dioxide from blood to alveolar air. Failure of addicted to oxygen and its use is not without function is responsible for anoxia and cyanosis. danger. It should be withheld as long as possible. These two divisions of function are not clear-cut The surgical treatment of emphysema is of because ventilatory insufficiency, if severe enough, limited value but there are special indications. will necessarily induce respiratory insufficiency, These are, firstly, the relief of tension in emphy- and on the other hand, respiratory insufficiency sematous bullae, more particularly when it is will stimulate the respiratory centre and lead to probable that useful lung tissue is being com- increased ventilation. pressed. The second indication is in the treatment Sir James Kingston Fowler (1898) wrote: 'All Postgrad Med J: first published as 10.1136/pgmj.34.390.184 on 1 April 1958. Downloaded from FTpril 1958 l'l"lX ('Cronic Jdlmoeiry Failuire 189 LLOYD-LUKE Books that enshrine profound thought LCGYlAOLOGE GENERAL PATHOLOGY (2nd edition) edited by SIR HOWARD FLOREY, M.D., F.R.C.P., F.R.S. Professor of Pathology, University of Oxford The call for a second edition of this book, which appeared first under the title Lectures on General Pathology, has enabled the authors to revise its contents and add new chapters on throm- bosis, metabolic changes following injury, atherosclerosis and tumours, thus enhancing its value by covering a wider field. xvi 932 pp. 410 illustrations 3 colour plates (2nd edition 1958) Just published 84s. net RECENT TRENDS IN CHRONIC BRONCHITIS edited by NEVILLE C. OSWALD, M.D.(Cantab.), F.R.C.P.(Lond.) Physician, St. Bartholomew's and Brompton Hospitals, London; Honorary Physician to H.M. The Queen The main purpose of this book is to bring together under one cover for the first time recent views upon the various facets of chronic bronchitis. Much of the text consists of the original work of the several contributors. Culled from their experiences with chronic bronchitis at the Brompton Hospital since 1950, the authors present their views on the diagnosis, prevention and treatment of this distressing and often killing disease. viii t- 200 pp. 76 illustrations, 2 colour plates (1958) 30s. net Just published LLOYD-LUKE (Medical Books) LTD., 49 Newman Street, W.1 copyright.

wrho have studied the subject of emphysema from males is unexplained, but the same predominance a clinical standpoint must have met with cases in exists in spontaneous pneumothorax. http://pmj.bmj.com/ which the ordinary exciting causes of the disease have apparently been absent.' Kountz and Summary Alexainder (I934) and Christie (I944) have made Observations are made on the clinical study of the same comment. It appears to the writer that 34 patients who presented with the complaint of the clinical association of chronic bronchitis with shortness of breath without preceding bronchitis emphysema has brought little but confusion to the or asthma. study and understanding of emphysema. It is not Trhirty-three of these patients were males. on September 27, 2021 by guest. Protected what happens in the bronchial tubes that is im- This form of emphysema begins commonly in portant, and neither bronchitis nor bronchiectasis the fourth and fifth decades and proceeds relent- will lead to emphysema unless there is bronchiolitis lessly to chronic pulmonary failure rather than to as well. Bronchitis and bronchiectasis are both cardiac failure. associated with a cough, but the cough itself will The features of the dyspnoea are described as not lead to emphysema unless there is bronchiolitis. well as the constitutional disturbance. This group of 34 patients developed emphysema The relationship of emphysema to bronchitis is without bronchitis. There can be no doubt that discussed. the seat of their disease was in the bronchioles and BIBLIOGRAPHY alveoli. In this region the cough reflex is virtually (CIIRISTIE, R. V. (1i44), Brit. mied. 7., i, 143. non-existent so that the single presenting symptom FOWLER, J. K., and GODLEE, R. J. (1898), I'Tlhe Diseases of the was dyspnoea. It seems logical to the l,ungs,' Longmans, Green & Co., I,ondon. infer that KOUNTZ, X. B., and ALEXANDER, H. L. (1934), EmZtphysemta one essential feature of emphysema is bronchiolitis, Medicine (Baltimore), 13, 251. Nlcl,EA.N, K. H. (1956), ''the Pathogenesis of Pulmonary and there is much to support this in the excellent Emphysema.' studies of McLean (i956). The predominance in "rhesis for Doctorate of Philosophy,' University of Melbourne,