Eur Reaplr J 1992, 5, 266-275 WORKING GROUP REPORT

Pulmonary rehabilitation In chronic obstructive pulmonary disease (COPD) with recommendations for Its use

Report of the European Respiratory Society Rehabilitation and Chronic Care Scientific Group (S.E.P.C.R. Rehabilitation Working Group)

C.F. Donner*, P. Howard**

Although smoking, the major risk factor for COPD, seems to be declining in the Western World, its consequences will last for decades.

The last 30 years have seen widespread efforts to define the components and role of pulmonary rehabili-tation in COPD patients; the task for the 1990's will be an extensive multidisciplinary application of scien-tific principles to pulmonary rehabilitation. In this perspective the empiric approach will be replaced by therapeutic principles aimed to achieve specific benefits for the individual patient, and to define the role of potential outcomes of pulmonary rehabilitation. Decreased mortality and morbidity may seem to be the most important outcome of a rehabilitation programme, but other issues such as physical and mental health, social and role functioning and perception of well-being can play a pivotal role to the individual response of a patient enrolled in a long-term rehabilitative pro-gramme. The effects of pulmonary rehabili tation on mortality will probably never be elucidated conclusively because the answer would require studies on large populations for many years in comparison to a control group.

Pulmonary rehabilitation aims to restore patients to an independent, productive and satisfying life and prevent further clinical deterioration to the maximum extent compatible with the stage of the disease. This goal may be accomplished, without materiaiJy improving lung function, by helping the patients to become more aware of their disease, more actively involved in their own health care and more independent in performing daily care activities, attempting to reverse the disability from disease.

Optimum drug therapy and smoking cessation are essential pre-requisites. An understanding of the

Members of the Working Party: N. A.tnbrosino (Montescano, I), P. Bardsley (Sheffield, UK), CJ. Oark (Glasgow, UK), R. Comudella (Barcelona, S), CP. Donner (Veruno, I), J.W. Fitting (Lausanne, CH), H. Folgering (Nijmegen, NL), P. Howard (Sheffield, UK), F. Ioli (Veruno, I), J.F. Muir (Rouen, F), A. Patessio (Veruno, I), C. Rampulla (Montescano, I), R. Sergysels (Bruxelles, B), F. Smeets (St. Ode, B), K. Str~m (Karlskrona, S), H. Worth (Duesseldorf, FRG), ]. Zielinski (Warszawa, PL).

factors limiting exercise, the effects and evaluation of retraining programmes will determine the selection of patients most likely to benefit from rehabilitation. Attention to psychosocial management, nutritional defects and physiotherapy form part of the treatment schedule. In respiratory failure, oxygen supplement-ation would appear essential and in advanced stages ventilatory support increases the therapeutic options. This statement crystalizes present views from a Work-ing Party of the European Respiratory Society Reha-bilitation and Chronic Care Scientific Group (formerly SEPCR Rehabilitation Working Group).

The rationale of pulmonary rehabilitation

The aims of pulmonary rehabilitation are : 1) a decrease of physical and psychological impairment due to the disease, 2) an increase in physical and mental fitness and performance and 3) maximal social re-integration of the patient to lower the handicap.

The methods to achieve these aims are incorporated into a programme of: a) an accurate diagnosis of the disease and the funct ional limitations, b) education about the disease to include its pathophysiology and the use of medication, c) physical training to improve phys-ical fitness and d) psychosocial support. Rehabilitation treatment pre-supposes maximal medical treatment with cessation of smoking before the start of any programme to be conducted by a specialist multi-disciplinary team. Exercise testing is the most important single test used to plan the rehabilitation programme.

In light to moderate obstructive disease (FEV1

usually ~60% of predicted normal values), exercise is limited by the cardiovascular system and/or peripheral muscle function. The rehabilitation programme is based on endurance training at high heart rate levels.

Head, Division of Pulmonary Disease and Director, Medical Center of Rehabilitation, Clinica del Lavoro Foundation, Institute of Care and Research, Veruno (NO), Italy. Head in Respiratory Medicine, Dept. of Medicine, University of Sheffield, Royal Hallamshire Hospital, Glossop Road, Sheffield SlO 2JF, UK.

REHABILITATION 267

Patients with ventilatory limitation (FEV1

40-60% predicted normal values or less) often show failure of the respiratory pump. Rehabilitation treatment will contain exercise training ergonomics, calisthenics and breathing exercises. Many patients need nutritional ad-vice. Respiratory failure requires special consideration.

Psychological support and restoration of self confi-dence is best conducted around the home environment with involvement of family members and care givers.

The benefits of pulmonary rehabilitation tend to decline after the initial treatment period. Follow-up programmes are essential.

Selection of patients

Any patient with symptomatic COPD should be considered. The Consultant Physician or Medical Di-rector of rehabilitation should assess patient enthusiasm and commitment, family circumstances, clinical severity and stability of disease and appraise financial resources. Exclusion criteria will include distracting disease such as severe cardiac failure, cancer and neurological dis-ability, poor patient commitment, adverse family cir-cumstance and limited financial resource.

Respiratory rehabilitation is conducted through in-patient or out-patient programmes and for the severely disabled, at home. In-patient programmes are required for individuals with very limited walking capability, when clinical diagnosis and assessment are uncertain, for unstable disease and for the instruction of patient and family in the methods. Out-patient programmes benefit the symptomatic but less severely disabled; they must be fitted around a job and are ideally suited for periodic monitoring of progress. Home based programmes will follow hospital in-patient or out-patient training. Special facilities will be required for the very severely disabled who infrequently leave the home such as for long term oxygen therapy (LTOT), ventilatory support, nursing and psychosocial management. End-stage terminal patients need specialist care which should be capable of application to the home.

Evaluation of rehabilitation therapy

Limitation of exercise capacity is not the same as disability which is defined as the "total effect of im-pairment on the patient's life". Tests of rehabilitation effect must recognise the multiplicative nature of affective factors and must be adjusted to suit il1dividual patient capabilities. While some degree of stratification in tenns of disease severity, age and sex is required, of most importance is the establishment of stable base lines, good control data and a realistic time scale. Very short periods of evaluation of days or a few weeks will afford limited responses. Quarterly or half yearly intervals are better.

Assessment will be on an individual or group basis using several parameters. For individuals, pre-treatment

intervals provide the control data, for groups pre-treatment intervals or specifically selected control groups can be used. Tests must be applied in random order to avoid bias. Multivariate analysis rather than univariate analysis is more appropriate. It is also important to evaluate failure to attain physiological goals.

The evaluation of exercise programmes aims to answer the following two questions: 1. Does treatment improve exercise tolerance? 2. How is the improvement achieved? Three types of measurement are used: 1. Progressive incremental exercise tests 2. Timed walk tests 3. Endurance at submaximal exercise

Progressive incremental exercise (PIE) is performed on a treadmill or bicycle ergometer with increments adjusted to patie~t capability. End points are maximal oxygen uptake (Vo1max) or maximal power output of the equipment. Anaerobic threshold is measured where possible by analysis of the slope of the VcojVo

2 relationship. Breathlessness should be assessed using visual analogue scales or the categorical (modified Borg) scale. Wide intersubject variability will be a feature of the statistical analysis.

Sub-maximal PIE tests are less useful and should recognise the very wide subject and inter-subject variability, particularly in control data. Endurance at submaximal exercise is a better index but the conduct of the tests and selection of equipment will once again bestow peculiarities to the data.

Timed walk tests are indirect measurements of exer-cise capability but have the advantage of simplicity and minimal requirements of a corridor and trained super-visor. Six or 12 minute intervals are selected accord-ing to disease severity: distance and breathlessness are measured. Their main disadvantages are susceptibility to motivation of patient and supervisor, and inability to compare data between centres owing to the plethora of circumstances under which the tests are performed. Within individual centres stringent test/retest criteria are possible once learning effects on initial walks are taken into account. An analysis of failure of rehabilitation treatment will help dissect the components of individual disability. Factors such as programme adherence, motivation, psycho- neurologic disease, socio-economic factors, severity of disease and frequency of intercur-rent exacerbations will be revealed. An indication of the likely success of future rehabilitation attempts can be measured.

Mechanisms of exercise limitation

Decreased exercise capacity in COPD patients is measured by a reproducible decrease in maximum oxygen consumption (Vo

2max). The mechanism is

multifactorial: 1) alteration in pulmonary mechanics which increases the work of breathing and leads to decreased ventilatory capacity and respiratory muscle fatigue, 2) impaired efficiency of pulmonary gas

268 C.F. DONNER, P. HOWARD

exchange resulting in increased ventilatory requirement and 3) pulmonary vascular disease which inhibits cardiovascular accommodations to exercise. Maximum exercise ventilation (VEI11ax) is dictated by disturbed lung mechanics. In contrast to healthy persons, COPD patients increase ventilation during exercise, mainly by increasing respiratory rate. Expiratory flow during heavy exercise often impinges on the flow-volume loop recorded during a resting forced vital capacity manoeuvre. Expiratory flow limitation results from increased airway resistance and reduced elastic recoil of the lung. Functional residual capacity rises, forcing the respiratory muscle to operate on a less favourable length tension relationship. Premature fatigue sets in. The latter is enhanced by muscle wasting. Exercise hypoxaemia may further impair ventilatory muscle performance.

Mismatching of ventilation to perfusion leads to hypoxaemia and high physiological dead space (VoNr) which increase the ventilatory requirement (VE for a given level of exercise).

Pulmonary artery pressure rises sharply on exercise and cardiac output may be prevented from an adequate response. Such effects have variable consequence on right heart muscle performance. Insufficient informa-tion is at present available to determine the effect of pulmonary vascular pathology on exercise limitation. Physiological training references are obtained at levels of exercise above the anaerobic threshold. The latter is therefore an important determinant of exercise train-ing capability but cannot be reached by many patients with severe disability. Although their therapeutic responses will be less, benefits are never-theless realised even though they are as yet not fully explained. Rehabilitation programmes intended to increase the exercise tolerance of COPD patients are designed after determination of the functional impairment of the pulmonary and cardiovascular systems.

Exercise prescription

Exercise tolerance improves in COPD patients through programmes of supervised exercise. It has yet to be established, however, whether these improvements are based on physiologic changes in the ability to pet-form work or whether psychological factors play the major role.

In normal subjects, training increases the oxygen uptake at which lactic acid begins to accumulate in the blood (the anaerobic thr~shold) by 25-40% and the maximal oxygen uptake (Volmax) by 5-20%.

Exercise training does nothing to reverse the basic disease process in COPD patients: resting lung mechanics and gas exchange efficiency do not improve. Conflicting results in terms of improved exercise endurance have been obtained after training respiratory muscles. The ventilatory requirement (the ventilation for a given level of exercise) can be lowered by train-ing (through less col production from bicarbonate

buffering of lactic acid) permitting the same work load with less dyspnoea. Despite such benefits, no clear change in pulmonary hypertension or arterio-venous P0

2 difference have been observed.

If no major contraindications (hypercapnia, arrthythmias or systemic hypertension) emerge after a preliminary exercise test the training programme should be initiated under the supervision of an expert therapist. It must be realised however that the consequences of such prescription in COPD patients, despite the goal of achieving a physiologic training effect, are not yet fully understood. Training at a work rate associated with lactic acidosis is more effective in inducing a training effect than at a work rate not associated with lactic acidosis. Thus, a programme involving exercise of 30-45 minutes per day, 3-5 days per week for 5-8 weeks at a work rate inducing substantial lactic acido-sis seems a reasonable strategy. A maintenance programme of exercise follows which must be part of the rehabilitation process.

In patients with severe COPD, supplemental oxygen during training should be considered; oxygen is necessary when substantial desaturation occurs during exercise but it is still unclear whether the training effect is influenced by oxygen supplementation.

Inspiratory muscle training and rest

Respiratory muscle training aims to improve inspira-tory muscle strength and/or endurance in the belief that it may lead to less breathlessness, less fatigue, improved capability for physical exercise and better clearance of airway secretions. Respiratory muscle training is achieved to a limited extent through general body training or schedules of hyperpnoea. Targeted inspiratory training is the best available technique. Reasonable guidelines involve training sessions twice per day for five to seven days per week. Sug-gested treatment time is 15 minutes and the course of treatment should be at least 8 weeks. Breathing is performed through an inspiratory resistance at a level between 30% and 40% of maximal inspiratory pressure. Failure to maintain and visualise inspiratory pressure is responsible for the variable outcome of many studies.

Paradoxically some patients with severe COPD suffer enhanced respiratory muscle fatigue after training. Respiratory muscle rest in these instances may be more appropriate than exercise training.

A conflict remains as to the recognition of patients who will respectively benefit from training or rest largely due to the difficulty of agreeing methods of measuring respiratory muscle fatigue. Muscle rest is variously achieved with negative and positive pressure non-invasive mechanical ventilation for defined periods. Tolerance of the equipment in COPD is inconsistent. Techniques of inspiratory muscle training and rest in COPD patients should remain topics for research at the present time and are not yet suitable for general clini-cal application.

REHABILITATION 269

Breathing retraining

Breathing retraining (BR) for stable chronic obstruc-tive lung disease takes several forms, low frequency breathing (LFB), pursed lips breathing (PLB), abdomino-diaphragmatic breathing (ADB) and "directed" breathing (ventilation dirigee). All these imposed types of breathing have a common aim to modulate and cre-ate a new type of breathing pattern which enhances tidal volume and lowers respiratory frequency without effect on the duty cycle 1VIToT. In addition, pursed lips breathing may modulate expiratory airflow avoiding dynamic airway collapse. All types of breathing slightly improve gas exchange but more by a "fre-quency effect" than by changes in the distribution of ventilation and perfusion.

The work of breathing increases during breathing retraining. Despite this a reduction in breathlessness and small improvements in lung function tests such as the maximum breathi~g capacity and respiratory muscle endurance have been claimed. Caution is required as increasing VT and/or 1i!I'roT induces in some COPD patients electromyographic signs of respiratory muscle fatigue. Breathing retraining techniques are not established. There is need for long term studies using slow breathing techniques which measure the effect on ventilatory control and lung mechanics to establish whether permanent adaptation of breathing pattern is really possible. Such studies must correlate changes of lung mechanics and the sensation of dyspnoea. Until more definitive evidence is available, breathing re-training cannot be recommended in COPD.

Chest physiotherapy and lung secretions

Chest physical therapy includes postural drainage, chest percussion and vibration administered by hand or mechanical means, shaking, cough and forced expiratory techniques. It is applied to many types of chest disor-der which include COPD with increased production of sputum (>30 mlday-1), bronchiectasis, cystic fibrosis and atelectasis when the problem is retention of secretions in the proximal airways. In patients suffering acute attacks of asthma, acute pneumonia, respiratory failure, or in need of ventilatory support and in those COPD patients with scant sputum expectoration, chest physi-otherapy is of little or no value or even harmful.

For hospitalised critically ill patients, four sessions per day would seem to offer the upper limit of effective-ness and tolerability. With more stable conditions twice daily sessions are more practical. At home physi-otherapy to clear secretions is only required during acute exacerbations of infection, except in cystic fibro-sis and bronchiectasis. The patient and family members should then receive an educational and technical programme of several sessions from a trained physi-otherapist. Prior use of bronchodilator drugs (through a metered dose inhaler or nebuliser) and/or mucolytic agents enhance mucociliary clearance and avoid the induction or worsening of bronchospasm. The order of

effectiveness of chest physiotherapy techniques in re-moving airway mucus is as follows: . 1. Forced expiratory therapy (FET) + postural drainage 2. FET alone 3. Directed coughing 4. Percussion/vibration + Postural drainage 5. Postural drainage and clapping/vibration alone (very low efficiency)

Smoking cessation

Cigarette smoking is the single most important preventable cause of respiratory disease. It acts synergistically with a host of occupational and envi-ronmental factors to produce lung damage. Not all individuals who smoke are affected and detection of the more susceptible in advance of disease is still impossi-ble. About 10% to 15% of smokers can be identified by accelerated decline of FEV

1 Motivation and

smoking behaviour are important. Addiction to nico-tine probably occurs to some degree in about two thirds of smokers. Smoking cessation must therefore have prime place in rehabilitation programmes and should precede definitive interventions such as exercise.

There are two broad therapeutic strategies: drug treatment principally involving nicotine replacement and behavioural intervention. The most widely used nico-tine substitute is nicotine polacrilex gum but other ve-hicles such as transdermal patches are under study. There is some evidence that clonidine in daily dose will augment nicotine substitution in alleviating withdrawal symptoms. The components of behavioural intervention include 1) self monitoring 2) goal setting or contract-ing, 3) stimulus control or alternative behaviours and 4) aversion. There is a strong community component to item (3) whereby environment associations of smoking can be progressively narrowed to reduce situ-ations where it is possible to smoke. Maintenance programmes are required for some time after quitting tobacco. Nicotine or its stable breakdown products cotinine and carbon monoxide (e.g. HbCO) are the most frequently used biologic markers. Self help and group programmes should be tailored for all ages but targeted at high risk groups such as the young and all those with COPD. Current studies suggest a 30% success rate of all programmes. There is little evidence to support hypnosis or acupuncture as effective.

Nutrition

Both obesity and wasting are features of COPD. Obesity and hypoxia are components of the "blue bloater" type of COPD. Obesity needs to be control-led by dietary regulation. Less attention has been given to the weight loss of the pink and breathless patients. Quantitative studies of fat and muscle often reveal protein caloric malnutrition of marasmic proportions. The cause is unclear but loss of appetite, food aversion and hypermetabolism through increased energy demand

270 C.F. DONNER, P. HOWARD

of COPD all seem involved. Correction of malnutri-tion by appropriate feeding seems feasible but has been difficult to achieve in the few patients studied so far. Nutritional status should be measured in all COPD patients who lose weight without clear cause. Nutri-tional intervention has not been fully evaluated and particularly as to whether it might improve body weight or affect long term prognosis.

Patient education and psychosocial support

Disability in COPD includes an important component of psychosocial damage which in many individuals can be just as important as physical limitation. Psychoso-cial support aims to regain the patient's damaged self esteem and impaired psychosocial function by restoring coping skills and learning stress management. It is essential that the patient understands his own essential role and responsibility and that his beliefs are tuned to positive outcomes. Mutual understanding and support of family, friends and personal physicians must be created. Psychosocial therapy is an essential part of a comprehensive rehabilitation programme in COPD. After assessment, intervention will involve education, support to achieve mental and physical awareness and will undertake activities commensurate with the sever-ity of disease. It is helpful to measure the improve-ments of the quality of life or alternatively failure of therapy by validated questionnaire. As pulmonary rehabilitation is a life-long activity, so will patient education be a never ending process.

Drug therapy

Optimum drug therapy should be established before definitive rehabilitation is commenced. This will in-clude relief of bronchoconstriction now recognised as a component of variable severity, intermittent antibiot-ics for infection and diuretics when peripheral oedema is manifest. Supplemental oxygen is given for respira-tory failure . A number of new products are under study, vasodilator agents for pulmonary hypertension, mucolytics and anti-oxidants, alpha

1-antitrypsin

replacement, anti-inflammatory drugs and respiratory agonists, particularly almitrine bismesylate.

For relief of bronchoconstriction beta2-adrenergic

drugs are administered through a variety of devices. The dose is adjusted for age, severity of disease and individual response. A willingness to use much higher doses than formerly, particularly through nebulisers, is apparent. Oral preparations are generally more helpful in children than adults. Newer long-acting ~2-agonists are under evaluation. Tolerance is consistently good, adverse reactions are principally muscle tremor and tachyarrhythmia. Ipratropium bromide, a parasym-pathetic blocking drug, has useful additional effect in many patients and is perhaps the first choice in severe emphysema. Theophyllines are effective but associated with a wider spectrum of side effects. Oral

corticosteroids benefit a subgroup of patients who must be defined by therapeutic trial. Long-term maintenance therapy should on current evidence be restricted to positive responders. Long-term inhaled corticosteroids remain under active evaluation. Individual exacer-bations of bronchial infection should be treated with short courses of antibiotics such as broad spectrum penicillins, oxytetracycline or cephalosporins. The policy of self administration of reserve antibiotics for exacerbations has generally worked well. Continuous cycles of antibiotics, particularly in the winter months, are seldom indicated except for bronchiectasis and cystic fibrosis.

Influenza infections have a devastating effect in COPD resulting in high mortality. Preventive vaccina-tion remains imperfect. Killed vaccines only should be used. Attenuated organisms regain their virulence in COPD. Killed vaccines are 50--60% effective against infections caused by drift of surface H and N antigens but much less so against substantial antigen changes (or shift) responsible for pandemics. Coryzal side effects are common. Pneumococcal, staphylococcal and haemophilus vaccines are of limited benefit and remain the subject of much research. Of the newer medicament pulmonary vasodilator drugs for pulmonary hypertension cannot yet be recommended, the indica-tions for mucolytics and anti-oxidants remain conten-tious and alpha,-antitrypsin replacement or enhancement is feasible through aerosol though expensive, but of unproven benefit in COPD.

Of the respiratory stimulants Almitrine bismesylate, a chemoreceptor agonist, is the most interesting. Taken orally it increases Pao

2 and reduces Paco

2 in hypoxic

COPD. Recommended dose schedules are now much lower than previously suggested. Effects of low dose Almitrine on long-term survival and clinical benefit remain to be established.

DomlciJlary oxygen therapy

Chronic respiratory failure, manifested by hypoxaemia and in some instances also hypercapnia, is associated with two broad categories of chronic pulmonary disease, obstructive airways disease (COPD) and restrictive disorders due largely to lung fibrosis or neuromuscular failure. Special techniques of low flow oxygen administration are necessary for severe hypoxaemic COPD. Oxygen supplementation for restrictive disor-ders generally demands higher flow rates; since hypo-ventilation is frequently a significant cause of oxygen lack, ventilatory support is also mandatory. The latter has generally worked in restrictive diseases but is not the subject of this report. Ventilatory support for severe respiratory failure in obstructive airways disease is more difficult to apply but is detailed below.

Oxygen supplementation for COPD patients in the home is supplied through cylinders, the oxygen concentrator or from liquid oxygen sources. Patient connection is by mask, nasal prongs and occasionally more invasively through catheters inserted into the

REHABILITATION 271

nasopharynx or trachea. Oxygen is given to relieve hypoxaemia and certain types of breathlessness. Before administration physiologic evidence of hypoxaemia should be obtained. Evidence of response should be obtained by measuring arterial blood gases or oxygen saturation. There are three forms of treatment, 1) long-term domiciliary oxygen (LTOT) 2) portable oxygen and 3) short-burst intermittent therapy.

Long-term domiciliary oxygen (LTOT)

Long-term domiciliary oxygen is the term applied to continuous oxygen supplementation in the home throughout the 24 hour day.

The minimum daily treatment period is 15 hours, it should include the night. A better survival response is achieved with near 24 hour daily periods. The absolute indications are a stable Pao

2 breathing air of 7.3

kPa (55 mmHg) or less with an FEV1

10% improvement on visual analogue scales) and/or increased walking distance (>10% baseline standardised 6 minute test levels).

Short burst intermittent therapy

In some cour;tries cylinders are provided in the home for short period use through a simple face mask for the relief of breathlessness, particularly after exercise. Use varies widely from several times per day of a few minutes duration, to once or twice per month. Such use is largely of placebo value, is expensive and cannot possibly relieve the consequences of long-term hypox-aemia. Recent double-blind studies have demonstrated some relief of dyspnoea in about a third of individu-als. Surprisingly not all were hypoxaemic. At the present time the following recommendation is made. Short burst oxygen can be given in COPD after dou-ble-blind tests against air breathing have demonstrated at least a 10% repeated reduction of breathlessness, measured on visual analogue scales. More research is clearly needed.

Expectation of benefit of long-term domiciliary oxygen (L TOT)

LTOT is administered in hypoxaemic COPD to improve survival, sleep quality, neuropsychiatric disor-der, quality of life and mobility and to reduce haemat-ocrit, pulmonary hypertension and oedema.

Improvement of survival is clear and ideally doubles survival period or adds five years of life. Oxygen therapy corrects hypoxaemia, not hypercapnia but fails to arrest the progression of airway disease. Much less benefit has been recorded for neuropsychologic impair-ment, quality of life and mobility. Patient compliance with this demanding therapy has not been fully stud-ied in relation to the outcome variables. The benefits of LTOT in terms of survival are demonstrated only in those with stable daytime hypoxaemia. Therapy should not be commenced too late as benefits are small in pre terminal disease.

Home ventilatory support

Long-term domiciliary oxygen (LTOT) fails to arrest the decline of Pao2 In advanced hypercapnic respira-tory failure mechanical ventilatory assistance, either

272 C.F. DONNER, P. HOWARD

mandatory or elective, has been studied. The goals are to 1) extend life, 2) enhance quality of life 3) reduce morbidity 4) improve physical and physiological func-tion and 5) be cost beneficial, in particular to decrease hospital admissions. It should be considered for those patients with frequent decompensation& of respiratory failure to prevent a fatal episode, in those who fail to wean after an acute exacerbation and where Pao

2

cannot be maintained over 7.0 kPa (52 mmHg) with-out excessive hypercapnia (10.0 kPa or 75 mmHg).

There are a number of methods, of ventilatory support; positive pressure ventilation through a trache-ostomy or by nasal or facial mask and negative pressure ventilation by cuirasse, shell or poncho around the chest and abdomen. Treatment may be intermittent, nocturnal or continuous. Long-term results are unclear and hampered by lack of control studies. The use of tra-cheostomy is associated with poor outcome, 8% survival at 10 years in one study. Non invasive nasal intermit-tent positive pressure ventilation is a new technique under evaluation in hypoxaemic COPD. Devices may be volume or pressure controlled. BIPAP (expiratory and inspiratory positive airway pressure) nasal ventila-tion machines, a derivative of CPAP (continuous positive airway pressure) devices provide a novel type of pressure supported ventilation. The machines are connected to the patient through a specially fitted nasal mask. The aim is to use ventilatory support only at night leaving the patient undisturbed in the day. The most suitable patients will be those developing persist-ent elevation of Paco2 (>8 kPa, 60 mmHg) an LTOT. Although some successes are recorded many patients find it difficult to accomodate the machines for more than a few hours. The respective indications for vol-ume dependent and pressure controlled ventilators in COPD remain to be defined.

The potential for ventilatory support in acute exacerbations of respiratory failure in COPD is consid-erable. Preliminary results of external negative pressure ventilatory techniques are disparate. In COPD tolerance for more than 2 hours is difficult but im-provement of blood gases, dyspnoea and respiratory muscle strength have been recorded. Nasal intermittent positive pressure ventilation (NIPV) using methods de-scribed above offers an alternative approach. It may well find a useful place but is still under evaluation.

The use of home ventilatory support in COPD remains a research procedure. Successful transfer of a patient from hospital to home requires all the resources of a well organised and trained home care team.

Home respiratory care

Home care is the provision of health services in the patient's home rather than in hospital or other institutional setting. The aim is to provide the best possible lifestyle for the individual at considerable saving in cost.

Successful home care will depend upon a clear as-sessment of disease severity, firm understanding of

therapeutic benefits, careful patient selection for differ-ing aspects of the rehabilitation programme, properly organised services using trained personnel and equip-ment of defined specification, maintenance, alarm and breakdown schedules. Recognition and therapy for personal and family psychosocial impairment is man-datory. Home care in COPD has advanced principally in the provision of LTOT. Irregular interpretation of admission guidelines to LTOT has increased the health care burden in some countries without clear patient benefit. The home care team is best supervised by a physician with the help of a General practitioner. Nursing staff and other members of the home care team are variously provided by state, domiciliary, charitable, commercial and agency organisations with equally variable quality of training. Only in the United States have respiratory home care therapists a defined train-ing programme but even so, still compete with a mul-titude of other home care providers.

In Europe, job descriptions should be agreed for respiratory home care therapists and appropriate training programmes commenced. Such personnel will combine duties of nurse, physiotherapists and social worker in this special area and have a working knowledge of the equipmen employed.

Acknowledgement: The working group are grateful for the support of the Clinica del Lavoro Foundation, Pavia, Italy for making the prepa-ration of this document possible.

References

Rationale of pulmonary rehabilitation

1. American Thoracic Society. - Pulmonary rehabilitation. Am Rev Respir Dis, 1981; 124: 663-666. 2. Levine S, Weiser P, Gillen J. - Evaluation of a ventilatory muscle endurance training programme in the rehabilitation of patients with chronic obstructive pulmonary disease. Am Rev Respir Dis, 1986; 133: 400-406. 3. Sandler HS. - Psychosocial issues in chronic obstruc-tive pulmonary disease. Clin Chest Med, 1986; 7: 629-M2.

Selection of patients

1. American Thoracic Society. - Standards for the diag-nosis and care of patients with obstructive pulmonary disease (COPD) and asthma. Am Rev Respir Dis, 1987; 136: 225-244. 2. Hodgkin JE, Zom EG, Conners GL eds. - Pulmonary rehabilitation. Guidelines to success. Butterworth publishers, Boston, 1984. 3. Woolf CR. - Respiratory rehabilitation: a practical guide. Tower Liths Company Ltd, Toronto, 1986. 4. SEP Task Group LTO. - Recommendations for long-term oxygen. Eur Respir J, 1989; 2: 160-164.

Evaluation of rehabilitation therapy

1. Make BJ. - Pulmonary rehabilitation: myth or reality? Clin Chest Med, 1986; 74: 519-540. 2. McSweeny AJ. - Quality of life in COPD. In: Chronic obstructive pulmonary disease; a behavioural

REHABIUTATION 273

perspective. Lung biology in health and disease. A.J. McSweeny, I. Grant eds., vol. 36. Marcel Dekker Inc., New York, 1988. 3. Wasserman K, Hansen JE, Sue DY, Whipp BJ. -Principles of exercise testing and interpretation. Lea & Febiger, Philadelphia, 1987; 3(i): pp. 27-46; 3(ii), p. 62. 4. Cochrane LM, Clark CJ. - Benefits and problems of a physical training programme designed for asthmatic patients. Thorax, 1990; 45: 345-351. 5. Jones NL. - Qinical exercise training. 3rd edition, W.B. Saunders Co., Philadelphia, 1988.

Mechanisms of exercise limitation

1. Loke J, Mahler DA, Paul Man SF, Wiedemann HP, Matthay RA. - Exercise impairment in chronic obstructive pulmonary disease. Clin Chest Med, 1984; 1: 121-143. 2. Lockhart A. - Factors limiting exercise in chronic obstructive lung disease. Bull Eur Physiol Res, 1979; 15: 305-317. 3. Bye PTP, Farkas GA, Roussos C. - Respiratory fac-tors limiting exercise. Ann Rev Physiol, 1983; 45: 439-451. 4. Wassermann K, Whipp BJ, Davis JA. - Respiratory physiology of exercise: metabolism, gas exchange and ventilatory control. Respir Physiol, 1981; 23: 149-211. 5. Matthay RA, Berger HJ, Davies RA et al. - Right and left ventricular exercise performance in chronic obstructive pulmonary disease: radionuclide assessment. Ann Intern Med, 1979; 93: 234-239.

Exercise prescription

1. Sinclair DJM, Ingram CG. - Controlled trial of supervised exercise training in chronic bronchitis. Br Med J, 1980; 1: 519-521. 2. Belman MJ. - Exercise in chronic obstructive pulmo-nary disease. Clin Chest Med, 1986; 7: 585-597. 3. Weg JG. - Therapeutic exercise in patients with chronic pulmonary disease. In: Cardiovascular clinics. N.L. Wenger ed., Exercise and the Heart, 2nd edition. Davis, Philadelphia, 1986; pp. 261-275. 4. Casaburi R, Wasserman K, Patessio A, Ioli F, Zanaboni S, Donner CF. - A new perspective in pulmonary rehabili-tation: anaerobic threshold as a discriminant in training. Eur Respir J, 1989; 2: Suppl. 7, 618s--623s. 5. Alpert JS, Bass H, Szucs MM, Banas JS, Dalen JA, Dexter L. - Effects of physical training on hemodynamics and pulmonary function at rest and during exercise in patients with chronic obstructive pulmonary disease. Chest, 1974; 66: 647~51. 6. Degre S, Sergysels R, Mesin R, Vandermoten P, Salhadin P, Denolin SH, De Caster A. - Hemodynamic response to physical training in patients with chronic lung disease. Am Rev Respir Dis, 1974; 110: 395-402. 7. Casaburi R, Patessio A, Ioli F, Zanaboni S, Donner CF, Wasserman K. - Reductions in exercise lactic acidosis and ventilation as a result of exercise training in patients with obstructive lung disease. Am Rev Respir Dis, 1991; 143: 9-18.

Inspiratory muscle training

1. Leith DE, Bradley ME. Ventilatory muscle strength and endurance training. J Appl Physiol, 1976; 41: 508-516. 2. Bellemare F, Grassino A. - Force reserve of the diaphragm in patients with COPD. J Appl Physiol, 1983; 55: 8-15.

3. Grassino A. - Inspiratory muscle training in COPD patients. Eur Respir J, 1989; 2 (Suppl. 2): 581s-586s. 4. Belman MJ, Shadmeher R. - Targeted resistive ventilatory muscle training in chronic obstructive pulmonary disease. J Appl Physiol, 1988; 65: 1726-1735. 5. Rochester DF, Martin LM. - Respiratory muscle rest. In: The Thorax. C. Roussos, P.T. Macklem eds., M. Dekker Inc, NY. Part B. 1985; pp. 1303-1328. 6. Rodenstein DO, Stanescu DC, Cuttitta G, Liistro G, Veriter C. - Ventilatory and diaphragmatic EMG responses to negative pressure ventilation in airflow obstruction. J Appl Physiol, 1988; 65: 1621-1626. 7. Nava S, Ambrosino N, Zocchi L, Rampulla C. -Assessment of diaphragmatic rest during negative pressure ventilation by pneumowrap in normal subjects and COPD patients. Chest, 1990; 98(4): 857-865. 8. Ambrosino N, Cobelli F, Torbicki A, Opasich C, Pozzoli M, Fracchia C, Rampulla C. - Haemodynamic effects of negative pressure ventilation in COPD patients. Chest, 1990; 97: 850-856. 9. Rochester DF. - Does respiratory muscle rest relieve fatigue or incipient fatigue? Am Rev Respir Dis, 1988; 138: 516-517.

Breathing retraining

1. Erpicum B, Willeput R, Sergysels R. - Does abdomi-nal breathing below FRC give a mechanical support for in-spiration? Clin Res Physiol, 1984; 20: 117. 2. Gimenez M, Martin R, Peslin R. Incidences mecaniques de la reeducation respiratoire des bronchiteux chroniques. Bull Eur Physiopathol Respir, 1971; 7: 587~01. 3. Grassino A, Bellemare F, Laporta D. - Diaphragm fatigue and the strategy of breathing in COPD. Chest, 1984; 85: Suppl. 6, 51s-54s. 4. Ingram RH Jr, Schilder DP. - Effect of pursed lips expiration on the pulmonary pressure flow relationship in obstructive lung disease. Am Rev Respir Dis, 1967; 96: 381-388. 5. Miller WF. - A physiological evaluation of the effects of diaphragmatic breathing training in patients with chronic pulmonary emphysema. Am J Med, 1954; 17: 471-477. 6. Sergysels R. - Bases physiologiques de la kinesitberapie respiratoire. Acta Tub Pneumol Belg, 1978; 69: 161-177. 7. Sergysels R, Willeput R, Lenders D, Vachaudez JP, Schandevyl W, Hennebert A. - Low frequency breathing at rest and during exercise in severe chronic obstructive bronchitis. Thorax, 1979; 34: 536-539. 8. Willeput R, Vachaudez JP, Lenders D, Knoops Th, Sergysels R. - Thoracoabdominal motion during chest physiotherapy in patients affected by chronic obstructive lung disease. Respiration, 1983; 44: 204-214.

Chest physiotherapy

1. Conference on the scientific basis of in-hospital respiratory therapy. Am Rev Respir Dis, 1980; 122 (part 2 of 2 parts): 1-161. 2. Sutton PP, Pavia D, Bateman JRM, Clarke SN. - Chest physiotherapy: a review. Eur J Respir Dis, 1982; 62: 188-201. 3. American Thoracic Society. - Standards for the diagnosis and care of patients with chronic obstructive pulmonary disease and asthma. Am Rev Respir Dis, 1987; 136: 255-244. 4. Selsby DS. - Chest physiotherapy. Br Med J, 1989; 298: 541-542.

274 C.F. DONNER, P. HOWARD

Smoking cessation

1. Weiss ST, Tager IB, Schenker M, Speizer F. - The health effects of involuntary smoking. Am Rev Respir Dis, 1983; 128: 933-942. 2. Lam W, Sze PL, Sacks HS, Chalmers TC. Meta-analysis of randomized controlled trials of nicotine chewing gum. Lancet, July 4, 1987; 27-29. 3. Glassman AH, Stetner F, Walsh T, Raizman PS, Fleiss JL, Cooper TB, Covey LS. - Heavy smokers, smoking cessation, and clonidine. lAMA, 1988; 259: 2863-2866. 4. Lichtenstein E, Harris DE, Birchler GR, Wahl JM, Schmahl DP. - Comparison of rapid smoking, warm smoky air, and attention placebo in the modification of smoking behaviour. 1 Con Clin Psycho/, 1973; 40: 92-98. 5. Health and Public Policy Committee, American College of Physicians. - Methods for stopping smoking. Ann Intern Med, 1986; 105: 281-291. 6. Anda RF, Remington PL, Sienko DG, Davis RM. -Are physicians advising smokers to quit? lAMA, 1987; 257: 1916-1919.

Nutrition

1. Goldstein SA, Thomashow BM, Kvetan V, Askanazi J, Kinney JM. - Nitrogen and energy relationships in mal-nourished patients with emphysema. Am Rev Respir Dis, 1988; 138: 636-644. 2. Donohue M, Rodgers RM, Wilson DO, Pennock BE. -Oxygen consumption of the respiratory muscles in normal and in malnourished patients with obstructive pulmonary disease. Am Rev Respir Dis, 1989; 140: 385-391. 3. Fitting JW, Frascarolo Ph, Jequier E, Leuenberger Ph. -Energy expediture and rib cage abdominal motion in chronic obstructive pulmonary disease. Eur Respir 1, 1989; 2: 84~ 845. 4. Wilson DO, Rodgers RM, Wright EC, Anthonisen NR. - Body weight in chronic obstructive pulmonary disease. The National Institutes of Health intermittent positive pressure breathing trial. Am Rev Respir Dis, 1989; 139: 1435-1438. 5. Efthimiou J, Fleming J, Gomes C, Spiro SG. - The effects of supplementary oral nutrition in poorly nourished patients with chronic obstructive pulmonary disease. Am Rev Respir Dis, 1988; 137: 1075-1082.

Patient education and psychosocial support

1. Hi Taik Kim, Knecht PA, Hiscox DE, Glaser EM. Psychosocial factors and pulmonary patients. In: "Pulmo-nary rehabilitation guidelines to success", J.E. Hodgkin et al. ed., Wobum, Massachusetts Butterworth Publishers, 1984; pp. 207-238. 2. Levin LS. - Forces and issues in the revival of interest in self-care: impetus for redirection in health. Health Edu Monog, 1987; 5: 115-120. 3. Satir V. - Conjoint family therapy. Paolo Alto, C.A. Sciences and Behaviour Books, 1968. 4. Hopp J, Maddox SE. - Education of patients and their families. In: Pulmonary rehabilitation guidelines to success, J.E. Hodgkin et al. ed., Wobum, Massachusetts, Butterworth Publishers, 1984. 5. Criner GJ, Make BJ. - Ambulatory and home care for individuals with COPD. In: Chronic obstructive pulmonary disease current concepts, J.E. Hodgkin, T.L. Petty eds, W.B. Saunders Co, Philadelphia, 1987. 6. Kirscht JP, Becker MH, Evelan JKP. - Psychological

and social factors as predictors of med.ical behaviour. Med Care, 1976; 14: 422-431. 7. Guyatt G. - Measuring health status in chronic airflow limitation. Eur Respir 1, 1988; 1: 560-564. 8. Pearlman RA, Jansen A. - The use of quality of life, considerations in medical decision making. 1 Am Geriatrics Soc, 1985; 33: 344-352.

Drug therapy

1. McFadden ER. - Beta1 receptor agonist: metabolism and pharmacology. 1 Allergy Clin lmmunol, 1981; 68: 91-97. 2. Larsson S, Svedmyr N. - Bronchodilatiog effects of beta,-adrenoceptor stimulants by different modes of adminis-tration (tablets, metered dose aerosol and combination thereof). Am Rev Respir Dis, 1977; 166: 861-870. 3. Paterson NAM. - The addition of an aerosol anti-cholinergic to an oral beta agonist plus theophylline in asthma and bronchitis. Chest, 1982; 82: 300-305. 4. Rail RW. - The xanthines. In: The pharmacological basis of therapeutics. A.G. Gilman, L.S. Goodman, R.W. Rail, F. Murad eds, 6th ed. New York, MacMillan, 19'80; 592-607. 5. Marlin GE, Hartnett BJS, Berend N. - Assessment of combined oral theophylline and inhaled beta-adrenoceptor agonist bronchodilator therapy. Br 1 Clin Pharmacol, 1978; 5: 45-50. 6. Mendella LA, Manfreda J, Warren PW, Anthonisen NR. - Steroid response in stable chronic obstructive pulmonary disease. Ann Intern Med, 1982; 96: 17-21. 7. Nisa M, Walshaw M, Earis JE, Pearson MG, Calverly PMA. - Assessment of reversibility of airway obstruction in patients with chronic obstructive airways disease. Thorax, 1990; 45: 190-194. 8. Harding SM, Freeman S. - A comparison of oral and inhaled steroids in patients with chronic airways obstruction. Thorax, 1978; 29: 214-218. 9. Postroa DS, Steenhuis EJ, Vanderweele, Sluiter HJ. -Severe chronic airflow obstruction: can long term corticos-teroids slow down progression? Eur 1 Respir Dis, 1985; 67: 56-64. 10. Howard P. - Vasodilator drugs in chronic bronchitis and emphysema. Eur Respir J, 1989; 2: Suppl. 7, 678s-681s. 11. British Thoracic and antituberculous Association. - A controlled trial of the effects of bromhexine on the symptoms of out-patients with chronic bronchitis. Br 1 Dis Chest, 1973; 67: 49-59. 12. Boman G, Blicker U, Larsson S, Melander B, Wlihlnder L. - Oral N-acetylcysteine reduces exacerbation rate in chronic bronchitis. Eur 1 Respir Dis, 1983; 64: 405-415. 13. Stark RD. - Dyspnoea: assessment and pharmacologi-cal: manipulation. Eur Respir 1, 1988; 1: 280-287. 14. Woodcock AA, Gross ER, Geddes DM. - Drug treatment of breathlessness: contrasting effects of diazepam and promethazine in "pink puffers". Br Med 1, 1981; 2: 343-346. 15. Voisin C, Howard P, Ansquer JC. - Almitrine bismesylaste: a long-term, placebo controlled, double-blind study in chronic obstructive pulmonary disease. Bull Eur Physiopathol Respir, 1987; 23: (Suppl. 11), 167-182. 16. Laforce FM, Eickhoff TC. - Pneumococcal vaccine: an emerging consensus. Ann Intern Med, 1988; 108: 757-799. 17. Assessment of inactivated influenza. - A vaccine after three outbreaks of influenza A at Christ's hospital. Lancet, 1979; i: 33-35.

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18. Gadek GE, Fells GA, Crystal RG. - Cigarette smok-ing induces functional anti-protease deficiency of the lower respiratory tract in humans. Science, 1979; 206: 1315-1316. 19. Hubbard RC, Crystal RG. - Augmentation therapy of a,-antitrypsin deficiency. Eur Respir J, 1990; 3: Suppl. 9, 44s-52s.

Oxygen therapy

1. Medical Research Council Working Party. - Long-term domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema. Lancet, 1981; 1: 681-686. 2. Nocturnal Oxygen Therapy Trial Group. - Continuous or nocturnal oxygen therapy in hypoxemic chronic obstruc-tive lung disease. Ann Intern Med, 1980; 93: 391-398. 3. Cooper CB, Waterhouse J, Howard P. - Twelve year clinical study of patients with hypoxic cor pulmonale given long-term domiciliary oxygen therapy. Thorax, 1987; 42: 105-110. 4. Davidson AC, Leach R, George RDJ, Geddes DM. -Supplemental oxygen and exercise ability in chronic obstruc-tive airways disease. Thorax, 1988; 43: 965-971. 5. SEP Task Group Long-Term Oxygen therapy (LTO). -Recommendations for long-term oxygen. Eur Respir J, 1989; 2: 160-164. 6. Levi-Valensi P, Weizenblum E, Pedinielli JL, Racineux JL, Douwos H. - Three months follow-up of arterial blood gas determination& in candidates for long-term oxygen therapy. Am Rev Respir Dis, 1986; 133: 547-551.

Home mechanical ventilation

1. O'Donohue WJ, Giovannoni RM, Goldberg AI et al. -Long-term mechanical ventilation. Guidelines for management in the home and at alternate community sites. Report of the ad hoc committee, respiratory care section, ACCP. Chest, 1986; 90 (Suppl. 1-37). 2. Plumrner AL, O'Donohue WR Jr, Petty TL. Consensus conference on problems in home mechanical ventilation. Am Rev Respir Dis, 1989; 140: 555-560.

3. Muir JF, Levi-Valensi P. - Should COPD patients be ventilated? Eur J Respir Dis, 1987; 70: 135-139. 4. Co-operative group and Antadir. - Multicenter study of 259 severe COPD patients with tracheostomy and home ventilation. In: Proc. World congress on oxygen therapy and pulmonary rehabilitation, Denver, March 1987. 5. Robert D, Gerard M, Leger P, Buffat J, Jennequin J, Holzapfel L, Mercatello A, Salamand J, Bertoye A. - Ven-tilation m&:anique A domicile defmitive par tracheotomie de l'insuffisant respiratoire chronique. Rev Fr Mal Resp, 1983; 11: 923-936. 6. Carroll N, Branthwaite MA. - Non-invasive positive pressure ventilation. In: Proc. International conference on pulmonary rehabilitation and home mechanical ventilation, Denver, March 2-5, 1988; p. 62 (abst.). 7. Bach JR, Alba A, Mosher R, Delaubier A. - IPPV via nasal access in the management of respiratory insufficiency. Chest, 1987; 92: 168-170. 8. Leger P, Gerard M, Lamzevin B, Robert D. - Home IPPV nasal mask in COPD patients. Eur Respir J, 1989; 2 (Suppl. 8): 683s (abstr.). 9. Braun NMT. - Effect of daily intermittent rest on respiratory muscles in patients with CAO. Chest, 1985; 86: 595-605. 10. Lucas J. - Home ventilator care. In: Pulmonary rehabilitation: From hospital to home. J.A. Oryan D.G. Bums eds, Year Book Med. Publishers, Vol. 1, 1984, p. 260.

Home care

1. Pierson DJ. - Home respiratory care in different countries. Eur Respir J, 1989: 2 (Suppl. 7): 630s-636s. 2. Howard P. - Cost effectiveness of oxygen therapy. Eur Respir J, 1989; 2 (Suppl. 7): 637s-639s. 3. Alpers JH. - Domiciliary oxygen. The cost benefit dilemma. Med J Austr, 1987; 146: 62-63. 4. American Medical Association. - Home care in the 1990s. lAMA, 1990; 263: 1241-1244. 5. Meany-Handy J, Lareau SC. - The role of home care. In: Pulmonary rehabilitation. Guidelines to success. J.E. Hodgkin, E.G. Zorn, G.L. Connors eds, Butterworth Pub-lishers, Boston, 1984; pp. 311-322.