CHAPTER 43

Rehabilitation in Chronic Obstructive Pulmonary Disease and Other Respiratory Disorders

Andrew L. Ries

Rehabilitation for patients with chronic lung diseases is well established as a means of enhancing standard pharmacologic and other therapies in controlling and alleviating symptoms and optimizing functional capacity.^1–4 The primary goal of any rehabilitation program is to restore the patient to the highest possible level of independent function. This goal is accomplished by helping patients and significant others learn more about the underlying disease, treatment options, and coping strategies. Patients are encouraged to participate actively in providing their own health care, become more independent in daily activities, and be less dependent on health professionals and expensive medical resources. Rather than addressing solely reversal of the disease process, rehabilitation focuses on improving disability from disease.

Historically, pulmonary rehabilitation strategies were developed and have been used primarily for patients with chronic obstructive pulmonary disease (COPD). However, pulmonary rehabilitation has also been applied successfully to patients with other chronic lung conditions, including interstitial diseases, cystic fibrosis, bronchiectasis, and thoracic cage abnormalities.^3,5–7 It has been used successfully in the evaluation and preparation of patients for surgery, such as lung transplantation and volume reduction lung surgery, and in maximizing recovery after surgery.^8–11 Pulmonary rehabilitation has been used to facilitate patient recovery from acute processes such as acute lung injury, or exacerbations of chronic lung disease requiring mechanical ventilation or acute hospital care. Pulmonary rehabilitation is appropriate for any patient with stable lung disease who is disabled by respiratory symptoms. Even patients with advanced disease may benefit if they are selected appropriately and realistic goals are set.

This chapter defines pulmonary rehabilitation and outlines issues related to patient selection and evaluation. Key components of a pulmonary rehabilitation program are described and results of rehabilitation programs reviewed. Finally, the role of rehabilitation prior to and following lung surgery is reviewed.

DEFINITION

In 2006, the American Thoracic Society and European Respiratory Society adopted the following definition:

     Pulmonary rehabilitation is an evidence-based, multidisciplinary, and comprehensive intervention for patients with chronic respiratory diseases who are symptomatic and often have decreased daily life activities. Integrated into the individualized treatment of the patient, pulmonary rehabilitation is designed to reduce symptoms, optimize functional status, increase participation, and reduce healthcare costs through stabilizing or reversing systemic manifestations of the disease.²

    This definition focuses on three important features of successful rehabilitation. First, the program is multidisciplinary. Pulmonary rehabilitation programs utilize expertise from various healthcare disciplines that is integrated into a comprehensive, cohesive program tailored to the needs of each patient. Second, the program is tailored to the individual. Patients with disabling lung disease require individual assessment of needs, individual attention, and a program designed to meet realistic individual goals. Third, the program addresses multidimensional outcomes that include physical, psychological, and social function as well as healthcare utilization.

The interdisciplinary team of healthcare professionals in pulmonary rehabilitation may include physicians, nurses, respiratory and physical therapists, psychologists, exercise specialists, and others with appropriate expertise. The specific team make up depends upon the resources and expertise available, but it usually includes at least one full-time staff member. Responsibilities of team members generally cross disciplines.¹²

Within this general framework, successful pulmonary rehabilitation programs have been established in both outpatient and inpatient settings and with different formats. A key to success is a dedicated, enthusiastic staff that is familiar with respiratory problems and can relate well to pulmonary patients and motivate them.

PATIENT SELECTION

Any patient with symptomatic chronic lung disease is a candidate for pulmonary rehabilitation (Table 43-1). Appropriate patients are aware of disability from their disease and are motivated to participate actively in their own care to improve their health status. Patients with mild chronic disease may not perceive their symptoms to be severe enough to warrant a comprehensive care program. On the other hand, patients with severe disease who are bed bound may be too limited to benefit greatly.

Criteria based on arbitrary lung function parameters or age alone should not be used in selecting patients.¹ Pulmonary function is not a good predictor of symptoms, function, or improvement after rehabilitation.¹³ Chronic lung disease is commonly associated with systemic features that contribute to functional limitations and may benefit from rehabilitation.¹⁴ In general, selection should be based upon a person’s disability from their disease, potential for improvement, and motivation to participate actively in a comprehensive self-care program. Also, pulmonary rehabilitation is not a primary mode of therapy. Patients should be stabilized on standard medical therapy and should not have other disabling or unstable conditions that might limit their ability to participate fully in the program and to concentrate on the necessary tasks.

The ideal patient for pulmonary rehabilitation, then, is one with functional limitation from moderate to severe lung disease who is stable on standard therapy, not distracted or limited by other serious or unstable medical conditions, willing and able to learn about his or her disease, and motivated to devote the time and effort necessary to benefit from a comprehensive care program.

PATIENT EVALUATION

The initial step is screening patients to ensure appropriate selection and to set realistic individual and program goals. The evaluation process includes the following components: interview, medical evaluation, psychosocial assessment, diagnostic testing, and goal setting (Table 43-2).

INTERVIEW

The screening interview is an important first step. It serves to introduce the patient to the program, review the medical history, and identify psychosocial problems and needs. Family members and significant others should be included. Communication with the primary care provider is important to establish the vital link for the rehabilitation staff to clarify medical questions prior to the program and facilitate subsequent recommendations. Care and attention in this initial evaluation helps in setting goals compatible with everyone’s expectations as well as appropriate programmatic objectives.

MEDICAL EVALUATION

Reviewing medical history helps to identify the patient’s lung disease and assess its severity. Other medical problems that might preclude or delay participation may be identified. Available laboratory data should be reviewed, including pulmonary function and exercise tests, rest and exercise arterial blood gas measurements, chest radiographs, electrocardiogram, and pertinent blood tests. Program staff can then determine the need for any additional information or action before the program begins.

PSYCHOSOCIAL ASSESSMENT

Successful rehabilitation requires attention not only to the patient’s physical problems but also to psychological, emotional, and social issues. Patients with chronic illnesses experience psychosocial difficulties as they struggle to deal with symptoms they may not fully understand.¹⁵

Neuropsychological impairment is common in patients with chronic lung diseases and cannot be accounted for solely on the basis of age, depression, or organic disease. Commonly, such patients become depressed, frightened, anxious, and more dependent on others to care for their needs. Progressive dyspnea is a frightening symptom and may lead to a vicious “fear–dyspnea” cycle: With progressive disease, less exertion results in more dyspnea, which produces more fear and anxiety, which, in turn, lead to more dyspnea. Ultimately, the patient avoids any physical activity associated with both of these unpleasant symptoms.

In addressing these problems, the initial evaluation should assess the patient’s psychological state and pay attention to “psychosocial clues” that may be apparent during the screening interview (e.g., level of family and social support, the patient’s living arrangement, activities of daily living, hobbies, and employment potential). Important clues in initial interviews to the patient’s emotional state may be evident in nonverbal communication, such as facial expression, physical appearance, handshake, and personal space (distance between individuals when conversing). Cognitive impairment that may limit the patient’s ability to participate fully in the rehabilitation program may be identified. Family members and significant others may provide valuable insight and should be included in the screening process and program whenever possible.

DIAGNOSTIC TESTING

Planning an appropriate rehabilitation program requires accurate, current information. The complexity of the testing procedures performed depends upon individual patient and program goals as well as the facilities and expertise available.

Pulmonary function testing is used to characterize lung disease and quantify impairment. Spirometry and lung volume measurements are most useful. Other tests (e.g., diffusing capacity, maximal respiratory pressures to assess muscle strength) can be added as needed.

Exercise testing helps to assess the patient’s exercise tolerance and to evaluate changes in arterial blood gases (e.g., hypoxemia or hypercapnia) with exercise.^16,17 This may also uncover coexisting diseases (e.g., heart disease). The exercise test is also used to establish a safe and appropriate prescription for subsequent training.

Maximal exercise of patients with chronic lung disease is limited largely by their breathing reserve, though chronic lung diseases are increasingly recognized as being associated with systemic effects that may also contribute to exertional symptoms (e.g., muscle fatigue). Simple pulmonary function tests such as spirometry can be used to estimate a patient’s capacity for sustained breathing (maximal ventilation) during exercise. The forced expiratory volume in 1 second (FEV₁) is most useful in this regard. However, lung function only provides an estimate of an individual patient’s maximum work capacity. Exercise tolerance depends also on the patient’s perception and tolerance of the subjective symptom of breathlessness. Therefore, it is important to exercise patients to assess their physical function and symptom tolerance.

Exercise evaluation for rehabilitation is most easily performed with the type of activity planned for training (e.g., treadmill for a walking training program). Laboratory exercise testing is most commonly performed using either (1) rapid, progressive, incremental levels to a symptom-limited maximum or (2) defined steady-state levels.^16,18 The former is most useful for determining exercise tolerance and the limitations to maximum performance. The latter may be preferred for assessing training prescriptions. Simpler exercise tests, such as the 6-minute walk test, have been used increasingly in recent years to measure exercise tolerance outside of a laboratory setting.¹⁹ These timed distance walk tests measure the maximum distance a person can walk within a defined period (e.g., 6 minutes). Such tests have the advantage of requiring less equipment and technical expertise; however, attention must be paid to the details of testing procedures because variations in factors such as the walking course, patient instructions, encouragement during tests, use of oxygen or monitoring devices, and number of tests performed will influence the results. Also, these tests do not provide the detailed physiologic data typically included in more formal laboratory exercise tests.

Measurement of arterial blood gases at rest and during exercise is important because of the frequent but unpredictable occurrence of exercise-induced hypoxemia.²⁰ Arterial blood gas sampling during exercise makes testing more complex. The noninvasive estimate of arterial oxygen saturation by cutaneous (e.g., pulse) oximetry is useful for continuous monitoring, but it has limited accuracy (95% confidence limits, ±4–5%).²¹

GOALS

After a patient’s medical, physiologic, and psychosocial state have been evaluated, specific goals should be set that are compatible with his or her disease, needs, and expectations. Goals should be realistic in light of the objectives of the program. Family members and significant others should be included in this process so that everyone understands what can and cannot be achieved. Programs should evaluate individual patients to document changes before and after pulmonary rehabilitation with standardized outcome measures of exercise tolerance (e.g., 6-minute walk distance) and symptoms (e.g., dyspnea) or health status (e.g., health-related quality of life).

PROGRAM CONTENT

Comprehensive pulmonary rehabilitation programs typically include several key components: education, instruction in respiratory and chest physiotherapy, psychosocial support, and exercise training (Table 43-2). Often, the various components are provided simultaneously; for example, during an exercise session, a patient may learn and practice breathing techniques for symptom control while being encouraged and supported by staff or other patients. Although there is no consensus regarding the optimal duration of a pulmonary rehabilitation intervention, typical programs last 6 to 12 weeks with 2 to 3 sessions per week, each session including several hours of supervised exercise training and individual or group education and psychosocial interventions.

EDUCATION

Successful pulmonary rehabilitation depends upon an understanding of lung disease and active involvement by patients and important others in providing social support. Education is an integral component; even patients with severe disease can gain a better understanding of their disease and learn specific means to deal with problems. Instruction can be provided individually or in small groups, but it should be adapted to different learning abilities. Topics discussed commonly include normal lung function, chronic lung disease, medications, nutrition, travel, stress reduction and relaxation, reasons to call the physician, and planning a daily schedule. Individual instruction and coaching may be provided on the use of respiratory therapy equipment and supplemental oxygen, breathing techniques, bronchial drainage, chest percussion, energy-saving techniques, and self-care tips. The general philosophy is to encourage patients to assume responsibility for their own care and become partners with their physician in providing the care.²²

Despite the importance of education, it is unlikely that increased patient knowledge alone will lead to improved health status. It is more difficult to change patient attitudes and behaviors. Patients require specific, individualized treatment strategies, instruction, and reinforcement. Thus, education is a necessary but not sufficient component of pulmonary rehabilitation.

RESPIRATORY AND CHEST PHYSIOTHERAPY TECHNIQUES

Patients with chronic lung disease use, abuse, and are confused about respiratory and chest physiotherapy techniques. In pulmonary rehabilitation, each patient’s needs for respiratory care techniques should be assessed and instruction provided in proper use. These techniques may include chest physiotherapy to control secretions; breathing retraining techniques to relieve and control dyspnea and improve ventilatory function; and proper use and care of respiratory equipment, including nebulizers, metered dose inhalers, and supplemental oxygen.²³

BRONCHIAL HYGIENE

Patients with chronic lung diseases frequently have abnormal lung clearance mechanisms that increase problems with retained secretions and infection. Therefore, rehabilitation programs teach a variety of chest physiotherapy techniques for secretion control (e.g., coughing, postural drainage, chest vibration, and percussion). These are important for patients who experience excess mucus production during exacerbations as well as for those with chronic sputum production. The use of mucolytic agents to reduce viscosity of secretions is of questionable benefit.^24,25

BREATHING RETRAINING TECHNIQUES

Pulmonary rehabilitation typically includes instruction in breathing techniques, such as diaphragmatic and pursed lips breathing—techniques aimed at helping patients relieve and control breathlessness, improve their ventilatory pattern (i.e., slower respiratory rate and increased tidal volume), prevent dynamic airway compression, improve respiratory synchrony of the abdominal and thoracic musculature, and improve gas exchange.²⁶ Review of studies evaluating these techniques indicates that improvement in symptoms (e.g., dyspnea) is a more consistent finding than are measurable changes in physiological parameters. The diaphragmatic breathing technique is a maneuver in which the patient consciously coordinates abdominal wall expansion with inspiration and slows expiration through pursed lips. The primary effect is to slow respiratory rate and increase tidal volume. Pursed lips breathing is commonly taught to pulmonary patients, particularly those with COPD. This technique was observed by Laennec as early as 1830 and was advocated as a physical exercise for pulmonary patients in the early part of the twentieth century. As a maneuver assumed naturally by many patients with respiratory disease, pursed lips breathing is characterized by tensing the lips and narrowing the mouth opening during expiration. The aim is to slow expiration and maintain positive airway pressure to “stent the airways open” and prevent collapse.²⁷

OXYGEN

When chronic oxygen therapy is required, available delivery methods should be reviewed to help select the best system for the patient’s needs. Supplemental oxygen is beneficial for patients with severe resting hypoxemia. Long-term continuous oxygen therapy has been clearly shown to improve survival and reduce mortality and morbidity in hypoxemic patients with COPD.^28,29 The benefits of supplemental oxygen for nonhypoxemic patients or those with intermittent hypoxemia (e.g., during exercise or sleep) are less clearly defined. Although continuous oxygen therapy is feasible and safe, maintaining patients on supplemental oxygen presents several challenges. Handling equipment is particularly difficult for physically disabled and frail patients. Therefore, it is important to assess each person’s oxygen needs and provide appropriate instruction.³⁰

Several new developments have improved the efficiency of gas delivery systems and patient compliance with continuous oxygen therapy. Liquid oxygen provides more gas with less weight than tanks of compressed gas, particularly in portable systems. Oxygen conserving devices may increase the efficiency of delivery, reducing flow requirements and prolonging the life span of portable gas sources. Transtracheal oxygen delivery may help to improve compliance and avoid problems with nasal catheters; however, patients must be instructed carefully in caring for the catheter.³¹

EXERCISE

Exercise is important in pulmonary rehabilitation.^32,33 Considerable evidence supports favorable responses to exercise training in patients with chronic lung diseases.³ Benefits are both physiological and psychological. Patients may increase their maximum capacity and endurance for physical activity, even though objective measures of lung function do not usually change. Patients may also benefit from learning to perform physical tasks more efficiently. Exercise training provides an ideal opportunity for patients to learn their capacity for physical work and use and practice methods for controlling dyspnea (e.g., breathing and relaxation techniques). Of all the components in a comprehensive pulmonary rehabilitation program, exercise is probably the most costly and labor-intensive, considering the personnel, equipment, and expertise required. Principles of exercise for patients with lung disease differ from those based on normals or other patient populations because of differences in the limitations to exercise and the problems encountered in training.

Many approaches have been used to train the person with chronic lung disease. To be successful, the program should be tailored to the individual’s physical abilities, interests, resources, and environment. For general application, techniques should be simple and inexpensive. As in normals and other patients, benefits are largely specific to the muscles and tasks involved in training. Patients tend to do best with activities and exercises for which they are trained. Walking programs are particularly useful. They have the added benefit of encouraging patients to expand social horizons. In inclement weather, many can walk indoors (e.g., at shopping malls). Other types of exercise (e.g., cycling, swimming) are also effective. Patients should be encouraged to incorporate regular exercise into daily activities they enjoy (e.g., golf, gardening). Since many persons with chronic lung disease have limited exercise tolerance, emphasis during training should be placed on increasing endurance. Changes in endurance with rehabilitation are often greater than changes in maximal exercise tolerance and allow patients to become more functional within their physical limits. Increase in maximum exercise is also possible as patients gain experience and confidence. Resistive training is also used commonly in rehabilitation and can lead to significant increases in muscle strength that are important for many activities of daily living.³⁴

EXERCISE PRESCRIPTION

Selecting a training target based upon a predetermined percentage of predicted maximal heart rate or (_O2) is a well-established practice for normals or patients without underlying pulmonary disease. However, in patients with chronic lung diseases, the best method of choosing an appropriate training prescription is less clearly defined. Exercise tolerance in pulmonary patients is typically limited by maximal achievable ventilation and breathlessness. Such patients frequently do not reach their limits of cardiac or peripheral muscle performance.

Much controversy exists regarding the appropriate training intensity target for patients with chronic lung disease. Use of a target heart rate has been advocated by some, although it is recognized that such a target may not be reliable for patients with more severe disease. Many patients with lung disease can be trained at a high percentage of maximal exercise tolerance, with work levels approaching or even exceeding the maximal level reached on the initial exercise test. In a study of 52 patients with moderate to severe COPD, patients were able to perform endurance exercise testing at an average workload of 95% of their baseline maximum.³⁵ After 8 weeks of training, these patients were training at 86% of the baseline maximum. In fact, many patients with severe COPD were exercising at levels exceeding their baseline maximum. In another study that examined 59 patients with moderate to severe COPD who trained at levels near their ventilatory limits, a mean peak exercise ventilation of 100% of measured maximal voluntary ventilation was achieved after 12 days of training and at 3 months of follow-up.³⁶ These findings suggest that even patients with advanced disease can be trained successfully at or near maximal exercise levels.

Based on the findings noted previously, some pulmonary rehabilitation programs define exercise targets and progression during training more by symptom tolerance than heart rate, work level, or other physiological measurements. Ratings of perceived symptoms (e.g., breathlessness) help teach patients to exercise to “target” levels of breathing discomfort. A typical approach is to begin training at a level that the patient can sustain with reasonable comfort for several minutes and then to increase the time or exercise level according to symptom tolerance. Patients are encouraged to exercise daily and increase exercise duration up to 15 to 30 minutes of continuous activity. This graduated program helps patients to achieve a goal of improved tolerance for tasks of daily living, which often require a period of sustained activity.

BLOOD GAS CHANGES

A major problem in planning a safe exercise program for patients with lung disease is the potential for worsening of hypoxemia with exercise. Patients who are not hypoxemic at rest may develop changes in arterial oxygenation that cannot be predicted reliably from resting measurements of pulmonary function or gas exchange.²⁰ Normal individuals do not become hypoxemic with exercise. In patients with obstructive lung disease, Pa_O2 changes unpredictably during exercise. In patients with mild COPD, Pa_O2 typically does not change with exercise; in fact, it may even improve. However, in patients with moderate to severe COPD, Pa_O2 may increase, decrease, or remain the same. Patients with interstitial lung disease commonly develop worsening oxygenation with exercise.

Based on these observations, it is important to evaluate a patient’s oxygenation status both at rest and during exercise. Such testing is also used to prescribe oxygen therapy at rest and with physical activity. With the availability of convenient, portable systems for ambulatory oxygen delivery, hypoxemia is not a contraindication to safe exercise training.

OTHER TYPES OF EXERCISE

Exercise programs for pulmonary patients typically emphasize lower extremity training (e.g., walking or cycling). Since exercise conditioning is largely specific to the muscles and tasks involved in training other forms of exercise may be particularly valuable for persons with chronic lung diseases.

Upper Extremity Training

Many patients with chronic lung disease report disabling dyspnea with daily activities involving the upper extremities (e.g., lifting, grooming) at much lower work levels than with the lower extremities. Upper extremity exercise is accompanied by a higher ventilatory demand for a given level of work than is lower extremity exercise. Given the aforementioned muscle specificity of training, upper extremity exercises may be important in helping pulmonary patients cope better with common daily activities.³⁷

Ventilatory Muscle Training

The potential role of ventilatory muscle fatigue as a cause of respiratory failure and ventilatory limitation in patients with chronic lung disease has stimulated attempts to train the ventilatory muscles. Techniques of isocapnic hyperventilation, inspiratory resistive loading, and inspiratory threshold loading have been shown to improve function of the respiratory muscles in both normals and patients. In normals, respiratory muscle function does not limit exercise tolerance; therefore, specific respiratory muscle training is unlikely to be of clinical benefit. In patients with COPD, the patient group most extensively studied, improvement in general exercise performance from ventilatory muscle training alone has not been demonstrated consistently. Thus, the role of respiratory muscle training as a routine component of pulmonary rehabilitation has not been clearly established.

PSYCHOSOCIAL SUPPORT

An essential component of pulmonary rehabilitation is psychosocial support, the goal of which is to help patients combat progressive feelings of hopelessness and an inability to cope with chronic, progressive disease.¹⁵ Depression is common in patients with chronic pulmonary disorders, as are anxiety (especially anxiety over dyspnea), denial, anger, and isolation. Patients become sedentary and dependent upon family members, friends, and medical services to provide for their needs. Excessive concern over other physical problems and psychosomatic complaints arise. Sexual dysfunction and fear are common and represent often unspoken consequences of chronic lung disease. Patients may also demonstrate cognitive and neuropsychological dysfunction, possibly related to or exacerbated by the effects of hypoxemia on the brain.

Psychosocial support is provided best by a warm and enthusiastic staff who can communicate effectively with patients and devote the time and effort necessary to understand and motivate them. Family members and significant others should be included in activities so that they can understand the disease and help the patient cope. Support groups are also effective. Patients with severe psychological disorders may benefit from individual counseling and therapy. Psychotropic drugs should generally be reserved for patients with more severe psychological dysfunction.

BENEFITS OF PULMONARY REHABILITATION

A growing body of evidence supports the expected results and benefits of pulmonary rehabilitation in the management of patients with chronic lung disease (Table 43-3). Evidence-based guidelines were published by a joint effort of the American College of Chest Physicians (ACCP) and the American Association of Cardiovascular and Pulmonary Rehabilitation (AACVPR) in 1997 and updated in 2007.^3,38 The 2007 ACCP/AACVPR Panel made and rated 26 recommendations and statements regarding pulmonary rehabilitation. Of these, seven were given the highest rating representing strong evidence and documented benefits. These included improvements in lower extremity exercise training, dyspnea, and health-related quality of life and the decline in benefits over 12 to 18 months from a 6- to 12-week intervention. They also felt that there was high-quality evidence supporting exercise in pulmonary rehabilitation regarding both high and low intensity training, increase in muscle strength and muscle mass from strength training, and upper extremity training. Other benefits with moderate level evidence included greater physiologic benefits from higher exercise training intensity, incorporation of education in pulmonary rehabilitation, and benefits for some patients with chronic respiratory diseases other than COPD. It should be noted that once a patient is stabilized on a standard drug treatment regimen, lung function (e.g., spirometric flow rates, lung volumes) does not change after pulmonary rehabilitation.

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