Individuals with Chronic Secondary Cardiovascular and Pulmonary Dysfunction

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Individuals with Chronic Secondary Cardiovascular and Pulmonary Dysfunction

Elizabeth Dean and Donna Frownfelter

Key Terms

This chapter reviews the pathophysiology and medical management in relation to the comprehensive physical therapy management of individuals with chronic secondary cardiovascular and pulmonary pathology. Exercise testing and training are major components of the comprehensive physical therapy management of individuals with chronic secondary cardiovascular and pulmonary conditions, and this topic is presented separately in Chapter 25.

This chapter specifically addresses the comprehensive physical therapy management of chronic cardiovascular and pulmonary dysfunction secondary to neuromuscular, musculoskeletal, collagen vascular and connective tissue, and renal dysfunction. Considerations in the management of people who are overweight or obese are also addressed. The neuromuscular conditions that are presented include stroke, Parkinson syndrome, multiple sclerosis, cerebral palsy, spinal cord injury, chronic effects of poliomyelitis, and muscular dystrophy. The musculoskeletal conditions that are presented include thoracic deformity (kyphoscoliosis) and osteoporosis. The collagen vascular and connective tissue conditions that are presented include systemic lupus erythematosus (SLE), scleroderma, ankylosing spondylitis, and rheumatoid arthritis (RA). Finally, management of the patient with chronic renal insufficiency and management of the person who is obese are presented. The principles of physical therapy management are presented rather than treatment prescriptions, which cannot be given without consideration of a specific patient (see online Case Study Guide). In this context the goals of long-term management of each condition are presented, followed by the essential monitoring required and the primary interventions for maximizing cardiovascular and pulmonary function and oxygen transport. The selection of interventions for any given patient is based on the physiological hierarchy. The most physiological interventions are exploited, followed by less physiological interventions and those whose effectiveness is less well documented (see Chapter 17). With respect to physical therapy treatment prescription and monitoring, these principles must be considered when chronic secondary cardiopulmonary dysfunction is the diagnosis.

Principles of Physical Therapy Management in Chronic Secondary Dysfunction

Long-Term Physical Therapy Management Goals

Although short-term goals for patients with chronic cardiovascular and pulmonary secondary dysfunction are the immediate priority, long-term goals need to be considered from the outset. In addition to considering the patient’s activity and participation needs, identifying the long-term goals early may help identify short-term component goals.

Psychosocial Management Goals

Among the primary goals in managing the needs of people with chronic conditions are teaching self-management skills and promoting lifestyle self-efficacy. Without attention to these components of management, short- and particularly long-term outcomes will be suboptimal.

Patient Monitoring

Patients with chronic secondary cardiovascular and pulmonary dysfunction should be monitored in a number of ways to ensure success in a rehabilitation program. These data points are necessary with most conditions discussed in this chapter, but not all. Dyspnea, for example, is not monitored for as part of an examination for osteoporosis because dyspnea is generally not part of the disease process. Exceptions to the data points mentioned are discussed in the individual monitoring discussions.

In general, patients with chronic secondary cardiovascular and pulmonary dysfunction should be monitored for the following:

The following values should be monitored:

The Borg scale for measuring perceived exertion is well established as a reliable tool for assessing and evaluating patients at rest and during and after exercise. Reduced exertion for a given level of work after training is an important indicator of improved oxygen transport capacity and fitness.

Individuals with Neuromuscular Conditions

Stroke

Pathophysiology and Medical Management

Stroke or hemiplegia affects cardiovascular and pulmonary function of the survivors either directly or indirectly. A cerebral infarct involving the vital centers of the brain can affect cardiovascular and pulmonary function. Such infarctions, however, are likely to be lethal or leave a person in a refractory vegetative state. More commonly, after a stroke, chest wall movement on the affected side is reduced, and myoelectrical activity can be reduced (flaccidity) or increased (spasticity). Facial and pharyngeal weakness contributes to an inability to control oral secretions, swallow effectively, and protect the upper airway. Altered respiratory mechanics and efficiency reflect impaired chest wall movement, asymmetry, and the degree of muscle paresis and spasm.

Individuals with stroke have associated problems that contribute to cardiovascular and pulmonary dysfunction. These patients tend to be older and hypertensive and have a high incidence of cardiac dysfunction. Muscle disuse and restricted mobility secondary to stroke lead to reduced cardiovascular and pulmonary conditioning and inefficient oxygen transport. Spasticity increases metabolic and oxygen demand. Hemiparesis results in gait deviations, which reduce movement efficiency and movement economy. Reduced movement economy results in an increased energy cost associated with ambulation, which may reduce exercise tolerance because of fatigue.1 In addition, ambulating with a walking aid is associated with increased energy cost compared with normal walking. This increased energy cost reduces the patient’s exercise tolerance further and increases fatigue.

The notion of a motor recovery plateau in the management of individuals with stroke has been challenged.2,3 It has been argued that individuals adapt to the training stimulus and plateau when that stimulus no longer changes. Thus individuals are deprived of therapy unless they are responding. Capacity to improve can be augmented with changes in type of activity and the introduction of new exercises, as well as changes in the intensity, duration, and frequency of the exercises. Computer-assisted motivating rehabilitation employs the use of games and unconscious limb activation and movement, rather than engaging the patient in specific limb exercises.2

Individuals with stroke are at increased risk of having intercurrent ischemic heart disease, which compromises long-term survival and increases the risk of illness and inactivity.4 Furthermore, concurrent congestive heart failure will adversely affect outcomes after stroke rehabilitation. Clinical assessment, even if patients are asymptomatic, must include a cardiac work-up to establish the degree to which cardiac insufficiency limits mobility, endurance, recovery, balance, and fatigue. An integrated multisystem approach will ensure improved rehabilitation outcomes and prevent complications.

Principles of Physical Therapy Management

Goals of Long-Term Management

The management of people with stroke is shifting from a primary sensorimotor focus to an integrated management approach of which aerobic conditioning is an integral component. Long-term management goals particular to patients with stroke include the following, in addition to the long-term management goals of all patients with secondary dysfunction:

Structured, progressive rehabilitation programs for people with stroke augment therapeutic gains with respect to endurance, mobility, and balance, compared with spontaneous recovery.5

Body weight support has been examined to support an individual with stroke in the upright position to facilitate treadmill walking as a means of conditioning and gait reeducation.6

Treadmill training with support appears to have a somewhat greater effect in ambulatory individuals with respect to improved walking speed than other interventions.

Patient Monitoring

Stroke is often associated with sleep disturbances (e.g., obstructive sleep apnea). Thus activity and sleep patterns must be assessed to ensure sleep is maximally restorative and not contributing to the patient’s symptoms.

Traditional management approaches to stroke incorporate little exercise testing and monitoring; however, given stroke results from dysfunction of the cerebral vessels secondary to the same common pathway as ischemic heart disease and intermittent claudication, comparable monitoring and precautions must be instituted during treatment.

In addition to the monitoring necessary for all patients, patients with stroke and cardiac dysfunction require clearance from a cardiologist before participating in a rehabilitation program and may require electrocardiogram (ECG) monitoring, particularly during exercise.

Exercise

Aerobic exercise is an essential component of long-term management of the individual with stroke to optimize the efficiency of oxygen transport overall. Maximizing ventilation with mobilization is limited if the patient has severe generalized muscular weakness and increased fatigue. Although aggressive mobilization can be supported in these patients,8 appropriate selection of patients for such a regimen, judicious exercise prescription, and monitoring must be instituted to ensure the treatment is optimally therapeutic and poses no risk to a patient in this high-risk group. Chest wall exercises include movement in all planes combined with rotation. Body positioning to optimize lung volumes and airflow rates is a priority. Breathing control and coughing maneuvers are essential and should be coupled with body movement and positioning. Exercise is conducted with the patient in upright positions to minimize the work of the heart and of breathing during physical exertion. Recumbent positions reduce lung volumes and expiratory flow rates, impair respiratory mechanics, increase closing volumes, increase thoracic blood volume, and increase compressive forces on both the lungs and the heart.9 Thus, aerobic exercise for significant periods and intensities should be performed standing or sitting. Lower-extremity work is preferable to upper-extremity work in that the latter is associated with increased hemodynamic stress. Rhythmic exercise of large muscle groups is preferable to static exercise and exercise of small muscle groups, such as the arms, which produces smaller hemodynamic effects. Yoga-based exercise programs may be of some benefit for people with chronic stroke.10 Resistance muscle training for the limbs increases their muscle power in a dose-dependent relationship without increasing spasticity.11 Muscle training should be combined with aerobic training for optimal benefit and functional benefit.12

Ambulation or wheelchair locomotion should be as efficient as possible so that the metabolic demand of these functional activities is reduced. Performing these activities inefficiently on a frequent basis contributes to an excessive oxygen demand. The patient expends considerable energy in performing these activities uneconomically, which impairs the patient’s tolerance and contributes to excessive fatigue. Conserving energy by performing these activities more economically from an energetic perspective will provide more energy to perform more of these or other activities.

Parkinson Syndrome

Pathophysiology and Medical Management

Parkinson syndrome is associated with reduced dopamine in the basal ganglia, resulting in the loss of normal reciprocal inhibitory and facilitatory neuronal input in the execution of smooth coordinated movement. The clinical manifestations of the disease include stooped posture; stiffness and slowed motion; a fixed, mask-like expression; and tremor of the limbs. Patients with Parkinson syndrome are hypertonic, rigid, and inflexible. Movement initiation is impaired, and once initiated, movement is not fluid. The patient walks with a quick, shuffling gait. These factors contribute to an increased energy cost of movement. Physical activity is restricted and function is compromised, contributing to impaired aerobic capacity, reduced movement efficiency, and hence reduced movement economy.

Although chest wall rigidity and respiratory muscle weakness are associated with a restrictive pattern of lung disease in the patient with Parkinson syndrome, the obstructive type of respiratory dysfunction has been reported (e.g., reduced midtidal flow rates, increase airway resistance, impaired distribution of ventilation, and an increase in functional residual capacity).13 This obstructive defect may reflect parasympathetic hyperactivity, which has been associated with the disease. The degree to which these cardiovascular and pulmonary manifestations of the disease are offset with anticholinergic drugs (used to treat rest tremor and reverse dystonia) has not been reported.

The upper extremities are rigid and held slightly abducted from the chest wall during locomotion. The rigidity and dyskinesia associated with Parkinson syndrome lead to restricted movement and body positioning. The patient becomes deconditioned from disuse. Although the rigid, immobile chest coupled with reduced body position changes can contribute to restrictive cardiovascular and pulmonary pathology in this syndrome, chemoreceptor dysfunction has been documented.14

Principles of Physical Therapy Management

Multiple Sclerosis

Pathophysiology and Medical Management

Multiple sclerosis is a demyelinating disease of the central nervous system. The focal or patchy destruction of myelin sheaths is accompanied by an inflammatory response. The course of the disease consists of a variable number of exacerbations and remissions over the years from early adulthood. Exacerbations are also variable with respect to severity. The neurological deficits include visual disturbance; paresis of one or more limbs; spasticity; discoordination; ataxia; dysarthria; weak, ineffective cough; reduced perception of vibration and position sense; bowel and bladder dysfunction; and sexual dysfunction.15 Breathing disturbances, including diaphragmatic paresis, may occur. Autonomic disturbance in the form of impaired cardiovascular reflex function at rest and attenuated heart rate and blood pressure responses during exercise are relatively common in patients with multiple sclerosis.16

Principles of Physical Therapy Management

Exercise

Aerobic exercise is an essential component of the long-term management of the patient with multiple sclerosis to optimize the efficiency of oxygen transport overall. In mild-to-moderate cases the goals of aerobic exercise are to optimize cardiovascular and pulmonary conditioning and enhance movement economy. Optimizing cadence of walking or cycling is important to minimize discoordination, energy expenditure, and fatigue and to maximize safety. In more severe cases the goal is to maximize ventilation and gas exchange in the patient who has severe generalized muscular weakness, spasm, and excessive fatigue. Subjective parameters (i.e., fatigue and exertion) provide the basis for the intensity of the exercise program in conjunction with objective measures. Parameters such as intensity and duration may vary from session to session depending on the patient’s general status, which tends to be variable. Aquatic exercise may be an alternative for patients whose discoordination precludes ambulation and cycling or who are troubled by heat. The use of a fan may also enhance the patient’s work output.

Chest wall exercises include all planes of movement with a rotational component. Body positioning to optimize lung volumes and airflow rates is a priority. Breathing control and coughing maneuvers are coupled with body movement and positioning. If mucociliary clearance is impaired, leading to secretion retention, postural drainage may need to be instituted coupled with deep breathing and coughing maneuvers.

Ventilatory Strategies

Methods of facilitating effective coughing in patients with neuromuscular diseases are extremely important because they constitute life-preserving measures. Supported and unsupported coughing methods are described in detail in Chapters 22 and 23. Whenever possible, deep breathing and coughing are coordinated with chest wall movement to facilitate maximal inflation of the lungs before coughing and maximal exhalation of the lungs during coughing. Body positions are varied and changed frequently to simulate as much as possible the shifts in alveolar volume and ventilation and perfusion that occur with normal movement and body position changes.17 In addition, body positioning is used to maximize the patient’s coughing efforts.

Prophylactic Management

Patients with generalized neuromuscular weakness require prophylactic management, given their high risk of developing life-threatening respiratory infections and complications. Prophylaxis should include flu shots; avoiding polluted, smoky environments; smoking reduction and cessation; controlling the types of food eaten and chewing well to avoid choking; and regular deep breathing, frequent movement, and change in body positions (even just shifting and taking some deeper breaths while seated in a wheelchair) to promote mucociliary transport. An optimal time to take deep breaths and to cough is during transfers, which usually are physically exerting and stimulate hyperpnea.

Patients with multiple sclerosis often have remissions and exacerbations of their symptoms. Physical therapy focuses on all phases, with more intense treatment during stable periods. In this way it is hoped that periods between remissions are increased and exacerbations are less severe and shorter.

Cerebral Palsy

Pathophysiology and Medical Management

Cerebral palsy results from insult to the central nervous system that usually occurs before birth (e.g., from substance abuse and perinatal underoxygenation).15 The clinical presentation includes spasticity and residual deformity from severe muscle imbalance, hyperreflexia, and mental retardation. Although there are varying degrees of cerebral palsy severity, patients most frequently seen by the physical therapist have significant functional deficits and require long-term care. The loss of motor control and hypertonicity of peripheral muscles often restrict the mobility of patients such that they are wheelchair dependent. Loss of motor function limits physical activity and the exercise stimulus needed to maintain an aerobic stimulus and optimal aerobic capacity. Often coupled with motor deficits are cognitive deficits and mental retardation. These afflictions limit the degree to which the patient can follow instructions, perform treatments, and participate actively in a long-term rehabilitation program. Patients with cerebral palsy who are able to ambulate do so at exceptional energy expenditure both with and without walking aids.18 Central neurological deficits, generalized hypertonicity, and musculoskeletal deformity contribute to increased metabolic demand for oxygen and oxygen transport.

Principles of Physical Therapy Management

Goals of Long-Term Management

In addition to all of the long-term management goals common to all other conditions, patients with cerebral palsy should have several additional goals:

Maximizing aerobic capacity and efficiency of oxygen transport and optimizing general muscle strength pertain to the patient with cerebral palsy that is mild in severity. Many patients seen by physical therapists have poorly controlled spasticity and marked intellectual limitations, which preclude full participation in aerobic and strengthening exercise programs. These patients are at risk for the sequelae of restricted mobility and recumbency.

Patient Education

In the case of patients with cerebral palsy, education should be directed at the patient whenever possible, but it more likely will be directed at the parents and/or care providers. Patients with cerebral palsy will require the same instruction common to all patients with chronic secondary dysfunction, and in addition will likely also need instruction on mobilization and coordinated activity.

Education on the importance of sleep and rest will also be important for patients in this population; individuals with cerebral palsy can be expected to have abnormal sleep patterns. Sleep patterns are often disrupted for the following reasons:

Mobilization

Mobilization is an essential component of the long-term management of the patient with cerebral palsy in order to stimulate aerobic metabolism and optimize the efficiency of oxygen transport overall, including maximizing alveolar ventilation and mobilizing and removing secretions.19 Maximizing ventilation with mobilization is limited if the patient has generalized spasticity. Furthermore, mobilization stimuli are selected specifically to minimize eliciting further muscle spasm. Prescriptive hydrotherapy and equinotherapy (horseback riding) can provide effective stimulation to the cardiovascular and pulmonary system in the individual with multiple handicaps and can minimize the effects of spasticity. With training, coordination of ambulatory patients can be improved and aerobic energy expenditure reduced. In addition, energy is conserved for performing more activity. Chest wall exercises include all planes of movement with rotation. Body positioning to optimize lung volumes and airflow rates is a priority. Breathing control and coughing maneuvers are essential and should be coupled with body movement and positioning. If mucociliary transport is impaired and this leads to secretion retention, postural drainage and manual techniques may need to be instituted with appropriate monitoring to ensure they do not have a detrimental effect.20

Ventilatory Strategies

In this patient population, clearing oral secretions and coughing maneuvers require special attention. Ventilatory strategies for facilitating effective coughing in patients with neuromuscular diseases are extremely important because they constitute life-preserving measures. Supported and unsupported coughing methods are described in detail in Chapters 22 and 23. Whenever possible, deep breathing and coughing are coupled with chest wall movement to facilitate maximal inflation of the lungs before coughing and maximal exhalation of the lungs during coughing. Body positions are varied and changed frequently to simulate as much as possible shifts in alveolar volume and ventilation and perfusion that occur with normal movement and body position changes. Microaspirations are likely a common occurrence in this patient population, particularly at night. Nighttime positioning must be prescribed for a given patient to minimize aspiration.

Spinal Cord Injury

Pathophysiology and Medical Management

The cardiovascular and pulmonary manifestations and complications of spinal cord injury are directly related to the level of the lesion.21 Cardiovascular and pulmonary impairment results from the loss of supraspinal control of the respiratory muscles and the heart below the spinal cord lesion. Loss of diaphragmatic innervation results in ventilator dependency. Loss of abdominal and intercostal innervation reduces the ability to cough, mucociliary transport, and the ability to clear the airways. Denervation of the heart and orthostatism are less problematic in that the heart’s autonomous function and increased responsiveness of the heart and blood vessels to circulating catecholamines adequately compensate. The cough mechanism of people with quadriplegia is ineffective in clearing the airways.22

Patients with quadriplegia are particularly prone to the effects of restricted mobility, given the extent of their functional motor loss and sensory deficits, particularly on cardiovascular and pulmonary function. Mobilization and physical activity are essential for the patient with a spinal cord injury to maintain optimal cardiovascular and pulmonary function and oxygen transport efficiency and the optimal strength and endurance of the respiratory muscles. Patients with partial cord lesions will have a greater probability of ambulation with or without aids. Walking with aids is enormously costly in terms of energy cost,23 however, and may not be practical in daily life.

Because of the complexity and multisystem involvement of spinal cord lesions, the team works closely together. Even though the nurse may be primarily responsible for teaching and monitoring bowel and bladder routines, the physical therapist keeps informed of these because genitourinary and gastrointestinal problems can jeopardize physical therapy goals.

Principles of Physical Therapy Management

Exercise

Aerobic exercise is an essential component of the long-term management of the patient with spinal cord injury in order to optimize the efficiency of oxygen transport overall, including maximizing alveolar ventilation and mobilizing and removing secretions. With higher lesions, exercise is usually confined to upper-extremity work in the form of wheelchair ambulation. Preservation of upper-extremity muscle function and minimization of overuse are primary goals from the outset. Patients can maintain adequate cardiovascular and pulmonary conditioning with wheelchair exercise; however, exercise prescription should be conservative to maximize the benefit-to-risk ratio of cardiovascular and pulmonary conditioning that relies on upper-extremity work. Patients who are able to walk with leg braces and crutches expend considerable energy doing so. A decision must be made regarding the benefits of walking at high energy demand versus conserving energy for other activities. Chest wall exercises can be used and should include all planes of movement with a rotational component. Body positioning to optimize lung volumes and airflow rates is a priority. Breathing control and coughing maneuvers are essential and should be coupled with body movement and positioning. Coordination of respiration with aerobic activity and wheeling is taught to maximize work output.

Ventilatory Strategies

Ventilatory muscle training has a role in the long-term rehabilitation of some patients with high spinal cord lesions. Such muscle training has long been known to increase the strength and endurance of the respiratory muscles24 and may improve the functional capacity of some patients. A stronger, endurance-trained diaphragm will not fatigue as readily as an untrained diaphragm. Standardizing the resistance of the training stimulus alone, however, is not sufficient to produce a training effect. It is essential that flow rate be controlled using a gauge.

Methods of facilitating effective coughing in patients with neuromuscular diseases are extremely important because they constitute life-preserving measures. Supported and unsupported coughing methods are described in detail in Chapters 22 and 23. Whenever possible, deep breathing and coughing are coupled with chest wall movement to facilitate maximal inflation of the lungs before coughing and maximal exhalation of the lungs during coughing. Body positions are varied and changed frequently to simulate as much as possible shifts in alveolar volume and ventilation and perfusion that occur with normal movement and body position changes.

A comprehensive program includes stretching of the chest wall and passive range-of-motion exercises of the shoulder girdle. Maximal insufflations are encouraged in optimal body positions. Glossopharyngeal breathing can enable high quadriplegic patients to be freed from mechanical ventilation for hours at a time. Assisted or unassisted coughing is coordinated with deep breathing and rhythmic rocking motion. Manual assisted coughing and mechanical coughing aids, including functional electrical stimulation and insufflation-exsufflation devices, can be useful.25 The pneumobelt is a device that can facilitate ventilation without a tracheostomy. This device counters loss of abdominal tone and helps preserve normal thoracoabdominal interaction during respiration, which is lost because of reduced rib cage compliance and increased abdominal compliance.

Chronic Effects of Poliomyelitis

Pathophysiology and Medical Management

The chronic effects of poliomyelitis affect a high proportion of survivors of poliomyelitis who contracted the disease during the epidemic of the 1950s. Three types of poliomyelitis were prevalent during the epidemic in the middle of the 20th century, namely, spinal (the majority of cases), bulbar, and encephalitic. Half of survivors report new symptoms consistent with postpolio syndrome (PPS) (see Chapter 6). New delayed symptoms include disproportionate fatigue, increased weakness, deformity, pain, reduced endurance, breathing and swallowing problems, and respiratory insufficiency. Although cardiovascular and pulmonary complications were not associated with the spinal form of poliomyelitis at onset, late-onset breathing and swallowing complications can appear as a late effect of the disease.26 In addition, these patients may be deconditioned and have poor movement economy (i.e., expend excessive energy because of postural deformities).1 Therefore, delayed-onset cardiovascular and pulmonary complications, coupled with the effects of overuse and general deconditioning, increase the risk of cardiovascular and pulmonary compromise, reduce the ability to recover from these, and increase surgical and anesthetic risk.

Because of the nonspecific symptoms associated with PPS, it is a diagnosis of exclusion based on a detailed history.

Principles of Physical Therapy Management

Goals of Long-Term Management

In addition to all of the long-term management goals common to other neuromuscular conditions, there are additional goals for patients with chronic effects of poliomyelitis:

Although there are commonalities between the management of individuals with a history of poliomyelitis with and without PPS, injudicious management of the individual with PPS can lead to further deterioration. Special attention is therefore given to balancing rest with physical activity and developing modified exercise programs if they are indicated. Such programs are not indicated if an individual is exploiting maximal physiologic reserve capacity to meet the needs of each day. This individual has little reserve capacity and thus can lose functional capacity by increasing metabolic demands. With appropriate self-management and adherence to judicious recommendations, the prognosis for an individual with PPS can be favorable.

Exercise

Aerobic exercise is an essential component of the long-term management of the patient with the late sequelae of poliomyelitis to optimize the efficiency of oxygen transport overall. The two principal goals of exercise are to optimize cardiovascular and pulmonary conditioning and movement economy. Maximizing ventilation with exercise is limited if the patient has severe generalized muscular weakness and increased fatigue. Disproportionate fatigue and other symptoms experienced by patients with the late sequelae of poliomyelitis have been attributed to overwork of affected and unaffected muscles, terminal axon degeneration, and impaired impulse transmission. Exercise is therefore prescribed judiciously to provide an optimal aerobic training effect without contributing to further other use abuse (i.e., prescriptive parameters based on subjective responses using semiquantitative scales in conjunction with objective responses). Walking is the most functional type of aerobic exercise; however, aquatic exercise provides a useful medium for individuals with lower-extremity paresis who require crutches to walk or are confined to a wheelchair. Reducing physical activity and exercise is indicated in some patients to optimize aerobic and muscle power. The effect of resting affected and unaffected muscles enhances functional capacity.

Individuals with PPS are likely to experience greater functional decline over time compared with survivors of poliomyelitis without the syndrome. The need for aids, devices, manual wheelchairs, and electric wheelchairs and scooters should be anticipated for some people. These, however, can be introduced with their intermittent use to provide rest to overused muscles and joints, minimize local and general fatigue, and preserve remaining muscle function to the greatest degree. Excessive use as well as underuse of such aids can contribute to rapid deterioration and decline. Thus the rehabilitation program includes recommendations about the appropriate use of aids and devices to optimize function, and these recommendations require periodic review.

Ventilatory Strategies

Ventilatory strategies for facilitating effective coughing in patients with neuromuscular diseases are extremely important because they constitute a life-preserving measure. Supported and unsupported coughing methods are described in detail in Chapters 22 and 23. Whenever possible, deep breathing and coughing are coupled with chest wall movement to facilitate maximal inflation of the lungs before coughing and maximal exhalation of the lungs during coughing. Body positions are varied and changed frequently to simulate as much as possible shifts in alveolar volume and ventilation and perfusion that occur with normal movement and body position changes.

Progressive loss of pulmonary function in patients with ventilatory compromise at onset can lead to respiratory insufficiency. Comparable with other neuromuscular conditions, invasive mechanical ventilation is avoided. Alternatives include nasal and oral methods of noninvasive assisted mechanical ventilation. In addition, airway clearance can be further assisted with manual assisted coughing, glossopharyngeal breathing, mechanical exsufflation, and mechanical insufflation-exsufflation.27

Survivors of poliomyelitis with ventilatory compromise are comparable with other patients with generalized muscle weakness. Of particular concern in this population is the necessity to establish the role of mobilization and exercise as a first line of defense in the management and prevention of cardiovascular and pulmonary dysfunction. For patients with PPS and overuse abuse, however, additional exercise may be detrimental, though modified mobilization and exercise may be prescribed on an interval schedule (see Chapters 19 and 25).28 The patient exercises for a period of time and then rests or reduces to a lower intensity of exercise to allow the muscles to rest. In addition to the multitude of benefits of mobilization and exercise on oxygen transport overall, these interventions also optimize respiratory muscle strength and endurance. If the patient does not recover within a few hours, the mobilization or exercise stimuli are excessive and should be modified. Chest wall mobility exercises to facilitate breathing and coughing may have a role.

Muscular Dystrophy

Pathophysiology and Medical Management

Individuals with muscular dystrophy and other types of degenerative neurological and muscular diseases have increased life expectancy and thus can expect prolonged morbidity. Prevention of complications as an individual becomes weaker and more limited in terms of participation in life is a priority. In addition to peripheral weakness, these conditions can lead to respiratory muscle weakness and alveolar hypoventilation. Vital capacity, forced expiratory volume, airflow rates, and maximum inspiratory and expiratory pressures are reduced. These patients are at risk for the development of atelectasis, impaired mucociliary transport, and pneumonia. In addition, long-term generalized muscular weakness, particularly of the thoracic cavity and abdomen, as well as restricted mobility and confinement to a wheelchair predispose the patient to thoracic deformities (e.g., scoliosis and dropping of the ribs and further muscle disuse). Patients with Duchenne muscular dystrophy are susceptible to dysphagia and upper airway obstruction secondary to gag reflex depression and hypotonia of the pharyngeal structures.21 These factors further compromise or threaten cardiovascular and pulmonary function and oxygen transport.

Cardiac dysfunction has also long been reported in progressive muscular dystrophy.29 Although the majority of patients have no clinical evidence of cardiac dysfunction, a high proportion have abnormal ECGs at rest or during exercise and abnormal echocardiograms and radionuclide ventriculograms showing reduced left ventricular ejection fraction and abnormal ventricular wall motion. Fatty and fibrous tissue infiltrate the myocardium and conduction system, and electrical conduction is slowed. Thus subclinical cardiac involvement is prevalent in patients with muscular dystrophy and may explain sudden death in this patient population.

Chronic respiratory muscle weakness is characteristic of muscular dystrophy and other neuromuscular disorders. Because the cardiovascular and pulmonary systems are seldom stressed owing to musculoskeletal dysfunction in these patients, respiratory muscle weakness is seldom detected. Such weakness is significant, however, in that it contributes to several other serious problems, including thoracic mechanical abnormalities, diffuse microatelectasis, reduced lung compliance, a weak cough with impaired mucociliary transport and secretion accumulation, ventilation and perfusion imbalance, and nocturnal hypoxemia. Progressive respiratory muscle weakness has long been known to increase the risk of respiratory muscle fatigue and failure.30,31

The severity of disease is not consistently correlated with compromised pulmonary function; thus cardiovascular and pulmonary function must be assessed individually in each patient. Patients with mild-to-moderate involvement of peripheral muscles may exhibit disproportionate respiratory compromise. This may be explained by differential changes in the degree of involvement of the diaphragm and the abdominal and intercostal muscles. Over time, musculoskeletal changes of the chest wall lead to spinal deformity and stiffness with loss of its elastic recoil. Chronic alveolar hypoventilation leads to respiratory insufficiency and the need for ventilatory assistance. With progressive respiratory insufficiency, nocturnal hypoventilation with hypercapnia and hypoxemia develop. Nocturnal respiratory support should be considered early to postpone the need for intubation and mechanical ventilation, which is associated with a poor prognostic outcome in patients who have chronically reduced vital capacities and weak cough.

Clinically, patients with muscular dystrophy demonstrate low functional capacity commensurate with the extent of muscle weakness and impaired cardiovascular and pulmonary function, including alveolar hypoventilation, orthopnea (shortness of breath on reclining), impaired mucociliary transport, difficulty clearing secretions, and increased work of breathing. Abdominal muscle strength provides an index of pulmonary function in that it is correlated with vital capacity and expiratory flow rates. The significant progressive functional loss associated with Duchenne muscular dystrophy increases the patient’s susceptibility to the sequelae of restricted mobility, including cardiovascular and pulmonary deconditioning and reduced efficiency of oxygen transport, circulatory stasis, muscular weakness, and bone loss.

Improved medical management of the complications of myopathies has significantly increased the life expectancy of patients such as those with Duchenne muscular dystrophy over the past 20 years. With advancing age, further complications will arise from age-related changes in cardiovascular and pulmonary function.32,33 Thus in the years ahead an increasing number of patients with myopathies will be requiring cardiovascular and pulmonary management and prophylaxis.

Principles of Physical Therapy Management

Goals of Long-Term Management

The goals of long-term management for the patient with muscular dystrophy are to reduce the impact of progressive debility and prolonged morbidity throughout their longer life spans. Respiratory complications may advance from mild respiratory distress to the need for nocturnal noninvasive mechanical ventilation. Respiratory failure is the primary cause of death.

In addition to all of the long-term management goals common to other neuromuscular conditions, the following are additional goals for patients with muscular dystrophy:

Patient Monitoring

Patients with muscular dystrophy require the same monitoring parameters common to all of the secondary conditions discussed in this chapter; however, those with additional cardiac dysfunction need to be cleared by a cardiologist before starting a rehabilitation program, particularly when it involves a mobilization or exercise program, to refine the prescriptive parameters of the program.

If supplemental oxygen is used, the fraction of inspired oxygen (FiO2) administered is recorded.

Assessment of nighttime and daytime cardiovascular and pulmonary function is needed because respiratory insufficiency often begins with nocturnal hypoxemia in patients with Duchenne muscular dystrophy.

Activity and sleep patterns must be assessed to ensure sleep is maximally restorative and not contributing to the patient’s symptoms; obstructive sleep apnea is related to hypotonia of the upper airway musculature and obesity.

Primary Interventions

Primary interventions for patients with muscular dystrophy include the combination of global interventions previously discussed; in addition, they require the following:

An assessment of the patient’s living and working environments should include a review of aids and devices (e.g., wheelchair type, weight, and size and noninvasive mechanical ventilation). Aids and devices are selected to minimize energy demand such that energy is conserved for other activities and undue fatigue is reduced.

The use of supplemental oxygen depends on the severity of the disease. Some patients have no need for supplemental oxygen, some need it only during exercise, and some patients require continuous oxygen with proportionately more delivered during activity and exercise compared with rest.

Mobilization

Mobilization is an essential component of long-term management of the patient with muscular dystrophy. It is prescribed to optimize the efficiency of oxygen transport overall and minimize the sequelae of restricted mobility. Maximizing ventilation with mobilization is limited if the patient has severe generalized muscular weakness and increased fatigue. Functional activities provide the basis for the mobilization prescription. Although heavy resistive strengthening exercise has been advocated for these patients,34,35 a conservative approach including an exercise program based on functional goals and energy conservation is more justifiable physiologically.36 Chest wall mobility exercises include all planes of movement combined with a rotational component. Body positioning to optimize lung volumes and airflow rates is a priority. Breathing control and coughing maneuvers are coupled with body movement and positioning. If mucociliary transport is impaired and leads to secretion accumulation, which is refractory to mobilization and body positioning, it may be necessary to institute postural drainage coupled with deep breathing and coughing maneuvers.

Ventilatory Strategies

Although the primary factor contributing to respiratory compromise is respiratory muscle weakness, ventilatory muscle training may have a role in selected patients (Chapter 26). Improved respiratory muscle endurance and strength may have a generalized effect on functional capacity. The effect of walking alone, however, may be superior to the effect of ventilatory muscle training on ventilatory muscle strength and endurance. Ventilatory muscle training should therefore be used selectively to elicit effects over and above those resulting from functional activities such as walking, given the multisystem and functional benefits of walking.

Patients with signs of ventilatory muscle fatigue, as opposed to weakness, benefit from ventilatory support at night. Rest of the respiratory muscles at night with continuous positive airway pressure or bi-level positive airway pressure optimizes their function during the daytime.

Exercise

Methods of facilitating effective coughing in individuals with neuromuscular diseases are extremely important because they constitute life-preserving measures. Supported and unsupported coughing methods are described in detail in Chapters 22 and 23. Patients on noninvasive ventilatory support who are unable to generate adequate peak cough expiratory flow rates can benefit from manual assisted coughing and mechanical insufflation-exsufflation, thereby minimizing the need for endotracheal suctioning.37 Tracheostomy is delayed as long as possible. Significantly reduced maximal insufflation capacity, however, is an indication for tracheostomy.

Whenever possible, deep breathing and coughing are coupled with chest wall movement. This facilitates maximal inflation of the lungs before coughing by increasing pulmonary compliance38 and maximal exhalation of the lungs during coughing. Body positions are varied and changed frequently to simulate shifts in alveolar volume and ventilation and perfusion that occur with normal movement and body position changes. Glossopharyngeal breathing is a nonmechanical method of assisting ventilation. The patient is taught to use the tongue and pharyngeal muscles to swallow boluses of air past the vocal cords and into the trachea. The efficiency of training is monitored with spirometry to ensure the patient is able to achieve acceptable vital capacities. Some patients are able to support their ventilation, ventilator-free, for several hours in a day.

One intervention that is prolonging the life of patients with muscular dystrophy, as well as of patients with other progressive neuromuscular diseases, is the use of mechanical ventilatory support.39 Home mechanical ventilation provides a noninvasive method of providing positive airway pressure through an oral or nasal mask. This provides considerable advantage over invasive, full body or tracheostomy ventilatory support. If used in conjunction with an insufflation-exsufflation device, pulmonary complications can be minimized and life expectancy increased. Other forms of noninvasive mechanical ventilation include intermittent abdominal pressure ventilation, rocking bed, negative pressure tank ventilator, and chest shell ventilator. The type of ventilation is determined individually based on the indications for ventilation and the patient’s status. The use of ventilatory aids as a component of a comprehensive rehabilitation program maintains pulmonary compliance and cough efficacy. Introduction of these devices early will facilitate increased use as the respiratory muscles progressively weaken. These aids are introduced to meet the individual’s needs and are changed over time. Excessive use or dependence can contribute to deterioration.

Prophylactic Management

Patients with generalized neuromuscular weakness require prophylactic management because of their high risk of developing life-threatening respiratory infections and complications. Prophylaxis should include flu shots; avoidance of polluted, smoky environments; smoking reduction and cessation; controlling the types of food eaten and chewing well to avoid choking; and regular deep breathing, frequent movement, and change in body positions (even just shifting and taking some deep breaths while seated in a wheelchair) to promote mucociliary transport. An optimal time to take deep breaths and to cough is during transfers, which usually are physically exerting and stimulate hyperpnea. Wheelchair design is important for comfort, biomechanical and metabolic efficiency, and reduction of unnecessary energy expenditure.

Individuals with Musculoskeletal Conditions

Thoracic Deformities

Pathophysiology and Medical Management

Respiratory insufficiency can result from abnormalities of the chest wall secondary to congenital deformity, acquired neuromuscular disease, and trauma.21 Congenital deformity of the chest wall reduces the mobility of the bony thorax, thereby increasing the work of breathing. Shallow, rapid breathing often results. Minute ventilation is increased at the expense of alveolar ventilation. Severe deformity leads to compression of the mediastinal structures. The heart can be displaced and rotated, impeding its mechanical function. Examples of chronic deformities that impinge on pulmonary function are kyphoscoliosis secondary to poliomyelitis, tuberculous osteomyelitis, and other causes and ankylosing spondylitis. Other examples of deformity include traumatic injury of the vertebral column, ribs, and sternum. Routine cardiovascular and pulmonary assessment should include a musculoskeletal examination of the spinal column and thoracic cavity.

Normal pulmonary function and gas exchange depend on symmetry of cardiovascular and pulmonary anatomy and physiological function. Asymmetry of the chest wall interferes with normal lung mechanics, regional gradients of ventilation and perfusion in the lungs, and the distribution of inspired gas. Significant decrease in lung compliance and increase in work performed against the elastic resistance of the lung are characteristic of kyphoscoliosis. Altered pressure gradients and uneven lung movement during the respiratory cycle may contribute to altered lung water balance and impaired lymphatic drainage. The effects of physiological dead space and shunt may be magnified, producing hypoxemia and hypercapnia. With severe chest deformity, a cycle of respiratory acidosis, pulmonary hypertension, and right-sided heart failure can result in a life-threatening situation.

Principles of Physical Therapy Management

Exercise

Aerobic exercise is an essential component of the long-term management of the patient with thoracic deformity in order to optimize the efficiency of oxygen transport overall. Abnormal postural alignment may contribute to excessive energy cost and reduced movement economy. Improving alignment will help offset this effect. In severe cases, surgical correction is indicated.

Maximizing ventilation with exercise in patients with severe deformity may be limited. Optimizing alignment to minimize the cardiovascular and pulmonary limitations of the deformity during physical activity, exercise, and rest is a priority. Chest wall exercises include all planes of movement with a rotational component. Body positioning to optimize lung volumes and airflow rates is a priority. Breathing control and coughing maneuvers are essential and should be coupled with body movement and positioning. If mucociliary transport is impaired leading to secretion retention, postural drainage may need to be instituted, coupled with deep breathing and coughing maneuvers.

Ventilatory muscle training may have a role in the management of patients with reduced inspiratory pressures and associated decreases in total lung capacity and hypoxemia.

Methods of facilitating effective coughing in patients with musculoskeletal deformity are extremely important because they constitute life-preserving measures. Supported and unsupported coughing methods are described in detail in Chapters 22 and 23. Whenever possible, deep breathing and coughing are coupled with chest wall movement to facilitate maximal inflation of the lungs before and maximal exhalation during coughing. Body positions are varied and changed frequently to simulate as much as possible shifts in alveolar volume and ventilation and perfusion that occur with normal movement and body position changes.

Osteoporosis

Pathophysiology and Medical Management

Osteoporosis is a condition associated with reduced bone mass per unit volume and appears to be on the increase (see Chapter 1). Age-related bone loss begins earlier and accelerates faster in women, particularly after menopause, than in men. Lifestyle factors, such as diet, exercise, and smoking, have a significant role in reducing bone mass. Caffeine has also been implicated as a contributing factor to bone loss secondary to increasing urinary calcium loss.

Osteoporosis is classified as idiopathic osteoporosis unassociated with other conditions, osteoporosis associated with other conditions (e.g., malabsorption, calcium deficiency, immobilization, or metabolic bone disease), osteoporosis as a feature of an inherited condition (e.g., osteogenesis imperfecta and Marfan syndrome), osteoporosis associated with paralytic conditions prohibiting weight bearing and activity, and osteoporosis associated with other conditions but with a pathogenesis that is not understood (e.g., RA, alcoholism, diabetes mellitus, or chronic airflow limitation).15

The most common clinical features are vertebral pain and spinal deformity resulting from vertebral compression and collapse. Vertebral bodies tend to collapse anteriorly, contributing to cervical lordosis, thoracic kyphosis, postural slumping, and loss of height. Acute episodes may be relieved by restricted mobility. Straining and sudden changes in position can exacerbate an acute episode. Cardiovascular and pulmonary complications of osteoporosis are secondary to spinal deformity, chest wall rigidity, and cardiovascular and pulmonary deconditioning resulting from restricted mobility. Vertebral compression fractures are also a feature of advanced osteoporosis. Collapse of the anterior surfaces of the vertebrae leads to kyphosis.

Osteoporosis is a condition associated with aging and older age groups. The pain of acute episodes leads to periods of restricted mobility and significant cardiovascular and pulmonary dysfunction in older persons.32,40 Exercise that is weight bearing and loads the muscles around bone maintains bone density and decelerates bone loss and thus has a central role in preserving bone health. Generally, the growth and remodeling of bone depends highly on the exercise prescription parameters (e.g., type of exercise, intensity, duration, and frequency). Bone mineral content is more closely related to cardiovascular and pulmonary conditioning than physical activity level. Furthermore, any detrimental effect of exercise on osteoporosis appears to relate more to malalignment and injury rather than activity itself.

The primary physiological mechanism underlying osteopenia and osteoporosis is negative calcium balance. Loss of calcium is associated with lack of exercise, smoking, alcohol consumption, consumption of caffeine, and meat consumption. Calcium supplementation is only part of the remedy, as calcium loss may be the greater concern.

Principles of Physical Therapy Management

Exercise

Aerobic and strength training are essential components of the long-term management of the individual with osteoporosis to optimize the efficiency of oxygen transport overall. Upright, weight-bearing aerobic exercise is essential to maintain bone density or reduce the rate of bone loss. Maximizing ventilation with exercise is limited if the patient has severe generalized muscular weakness and increased fatigue. Balance problems have also been identified in patients with osteoporosis, and these need to be addressed to minimize the risk of falling and its severe complications.

Chest wall exercises may have a role and include all planes of movement with a rotational component. Breathing control and coughing maneuvers are essential and should be coupled with body movement and positioning. Straining, Valsalva maneuvers, and jarring activity and exercise are contraindicated.

Methods of facilitating effective coughing in patients with osteoporosis are extremely important because they constitute life-preserving measures. Some patients fracture ribs and vertebrae during coughing. Patients at risk should rely on huffing maneuvers that do not require closing the glottis and do not generate high intrathoracic pressures.

Individuals with Collagen Vascular, Connective Tissue, and Rheumatoid Conditions

Systemic Lupus Erythematosus

Pathophysiology and Medical Management

SLE is a condition characterized by the presence of multiple antibodies that contribute to immunologically mediated tissue inflammation and damage.41 The condition affects the major organ systems, including the central nervous, musculoskeletal, pulmonary, vascular, and renal systems. Symptoms include arthralgic and myalgic stiffness, pain, and fatigue.

The cardiovascular and pulmonary manifestations of SLE include atelectasis, which results from inflammation of the alveolar walls and perivascular and peribronchial connective tissue, effusions secondary to lung infarction, reduced surface tension, and splinting secondary to pleuritic pain. Other manifestations include pleuritis with or without effusion, pneumonitis, interstitial fibrosis, pulmonary hypertension, diaphragmatic dysfunction, pulmonary hemorrhage, systemic hypertension, myocarditis, constrictive pericarditis, dysrhythmias, tamponade, pericardial pain, arteritis, and defects of the mitral and aortic valves.42 Other manifestations that affect cardiovascular and pulmonary function include anemia, leukopenia, thrombocytopenia, thrombosis, splenomegaly, ascites, gastrointestinal bleeding, nephritis, and renal insufficiency.15

Principles of Physical Therapy Management

Exercise

Aerobic exercise is an essential component of the long-term management of the patient with SLE in order to optimize the efficiency of oxygen transport overall, including maximizing alveolar ventilation and mobilizing and removing secretions. Parameters of the exercise prescription are based on subjective responses (e.g., discomfort, pain, breathlessness, and perceived exertion in conjunction with objective responses). Optimal types of aerobic exercise include walking and cycling. Aquatic exercise may be preferable for patients with musculoskeletal involvement that precludes walking and cycling. Chest wall exercises can be used and should include all planes of movement with a rotational component. Body positioning to optimize lung volumes and airflow rates is a priority. Breathing control and coughing maneuvers are essential and should be coupled with body movement and positioning. If mucociliary transport is impaired, leading to secretion retention, it may be necessary to institute postural drainage coupled with deep breathing and coughing maneuvers.

Scleroderma

Pathophysiology and Medical Management

Scleroderma is characterized by the overproduction of collagen and progressive fibrosis of cutaneous and subcutaneous tissues.43 The cardiovascular and pulmonary manifestations of this condition result in interstitial pulmonary fibrosis with significantly reduced vital capacity, diffusing capacity, and arterial oxygen tension.15 Reduced static compliance is the primary mechanical deficit. Pulmonary hypertension may be a complicating factor. Bronchoalveolar lavage is consistent with an acute inflammatory process. Cardiomyopathy is associated with ischemia, areas of infarction, and myocardial fibrosis.43 Fibrosis of the conduction system predisposes the patient to conduction defects and dysrhythmias. Other cardiovascular and pulmonary manifestations include pericarditis with or without effusion and pulmonary and systemic hypertension from renal involvement. Half of patients with scleroderma have renal involvement including intimal hyperplasia, fibrinous necrosis of the afferent arterioles, and thickening of the glomerular basement membrane. Fibrotic changes and stenoses occur in the small arteries and arterioles systemically. Similar changes in the lymphatic vessels may obliterate lymph flow.

Esophageal involvement contributes to regurgitation of gastric contents, which is exacerbated when the patient is recumbent or bends over. Bloating and abdominal discomfort may reflect paralytic ileus and intestinal obstruction. Ascites and fluid accumulation in the gut increase abdominal pressure and encroach on diaphragmatic motion.

Principles of Physical Therapy Management

Exercise

Aerobic exercise is an essential component of the long-term management of the patient with scleroderma to optimize the efficiency of oxygen transport overall. The exercise program is modified according to the signs and symptoms (see Chapter 25). Chest wall exercises can be used and should include all planes of movement with a rotational component. Body positioning to optimize lung volumes and airflow rates is a priority. Breathing control and coughing maneuvers are essential and should be coupled with body movement and positioning. If mucociliary transport is impaired and this leads to secretion retention, it may be necessary to institute postural drainage coupled with deep breathing and coughing maneuvers.

Ankylosing Spondylitis

Pathophysiology and Medical Management

Ankylosing spondylitis results in reduced total lung capacity, vital capacity, and inspiratory muscle function. Ventilatory capacity is preserved, given that the respiratory muscles are not involved. The condition can result in spinal and chest wall rigidity; thus there is greater reliance on diaphragmatic contribution to ventilation (84%) compared with healthy persons (68%). The patient with ankylosing spondylitis has an increased dependence on diaphragmatic function and therefore is at risk if administered respiratory depressant medications or if he or she undergoes thoracic or upper abdominal surgery. These changes can lead to an increased respiratory rate during exercise to meet ventilatory demands. In combination with impaired respiratory mechanics, the work of breathing can increase.

During exercise, patients with ankylosing spondylitis show minimal chest wall expansion compared with otherwise healthy people. Although peak workload is reduced, diaphragmatic fatigue is more likely to be the limiting factor than ventilatory capacity, ventilation-perfusion mismatch, or abnormal blood gases.

Principles of Physical Therapy Management

Exercise

Aerobic exercise is an essential component of the long-term management of the patient with ankylosing spondylitis in order to optimize the efficiency of oxygen transport overall. Maximizing ventilation with mobilization is limited if the patient has extreme spinal rigidity. Chest wall exercises can be used and should include all planes of movement with a rotational component. Body positioning to optimize lung volumes and airflow rates is a priority. Breathing control and coughing maneuvers are essential and should be coupled with body movement and positioning. Ventilatory muscle training in conjunction with exercise may have some additional benefit in maximizing aerobic capacity.

Consistent with the physical activity and exercise pyramid recommendations of daily exercise, individuals with ankylosing spondylitis can benefit from frequent, prolonged, low-intensity cross training to offset the potential for loss of flexibility and range.

Rheumatoid Arthritis

Pathophysiology and Medical Management

RA is a multisystemic condition that is associated with well-documented cardiovascular-pulmonary effects, including pleuritis with or without effusions, interstitial fibrosis, pulmonary vasculitis, an increased incidence of bronchitis and pneumonia myocarditis, epicarditis, endocarditis, dysrhythmias, neuritis, and vasculitis.44

Individuals with RA have significant cardiovascular risk factors. Compared with individuals without RA, diastolic blood pressure and levels of thrombotic variables are elevated.45 Risk factor modification has an important role in the comprehensive management of individuals with RA.

Functional capacity is limited by pain and stiffness in the affected muscles and joints, weakness, the number of joints affected, fatigue, and whether the patient is having an acute episode. Self-limited physical activity and exercise contributes to cardiovascular and pulmonary deconditioning. Movement such as walking is often inefficient because of limping. Peak exercise tests are limited by musculoskeletal complaints; thus submaximal tests are more functional in this population. Tests of cardiovascular status must be non–weight bearing to enable the patient to reach an acceptable stress level without confounding joint pain.

Prolonged use of steroids contributes to bone fragility. Thus physical activity and exercise prescriptions are modified accordingly.

RA is hallmarked by exacerbations and remissions. A lifelong health program helps to reduce the frequency and severity of exacerbations and enhance recovery from these. Furthermore, such a program may help reduce the need for potent medication.

Principles of Physical Therapy Management

Exercise

Aerobic exercise is an essential component of the long-term management of the patient with RA in order to optimize the efficiency of oxygen transport overall with respect to both cardiovascular and pulmonary conditioning and improving movement economy. Mild-to-moderate exercise during subacute periods can be beneficial. Cycling efficiency of patients with RA is comparable to that of healthy persons. The efficiency of walking, however, is less in patients with RA because of limping and associated deformity and pain. Non–weight-bearing exercise is beneficial in patients with severe deformity and pain (e.g., aquatic exercise or water walking). Given the fluctuations in the patient’s condition from day to day, exercise prescription is modified frequently in consideration of the patient’s changing condition. Chest wall exercises include all planes of movement with a rotational component. Body positioning to optimize lung volumes and airflow rates is a priority. Breathing control and coughing maneuvers are essential and should be coupled with body movement and positioning.

For patients with RA, 15 to 35 minutes of graded, low-intensity aerobic exercise performed three times a week can be sufficient to enhance aerobic capacity. In addition to improving aerobic capacity, such an exercise prescription results in increased exercise time, reduced affected joint count, improved activities of daily living, and reduced joint pain and general fatigue. Should a joint flare-up occur while the patient is participating in an exercise program, a few days or weeks of restricted mobility and abstinence from exercise frequently ameliorate the symptoms. Gentle mobilization (preferably weight bearing) coupled with range-of-motion exercises during this period will minimize the negative effects of reduced activity.

Individuals with Chronic Renal Insufficiency

Pathophysiology and Medical Management

Patients with chronic renal disease have significant systemic complications. Cardiovascular and pulmonary manifestations include left ventricular hypertrophy and congestive heart failure secondary to chronic volume and pressure overload. Patients have a high incidence of atherosclerosis, coronary artery disease, glucose intolerance, and diabetes. Also, generalized muscle weakness and fatigue compromise functional work capacity.

Chronically increased fluid volume, although regulated with dialysis, contributes to increased stroke work of the heart and cardiomegaly and hypertension. With respect to pulmonary function, increased fluid volume increases peribronchial fluid and airway closure. After dialysis, the reduction in body weight is related to a reduction in closing volume, increased vital capacity, and forced expiratory flow rates.

The pulmonary-renal syndromes reflect the close relationship between the lungs and kidneys. These syndromes are characterized by altered immunological status, alveolar hemorrhage, interstitial and alveolar inflammation, and pulmonary vascular involvement.

The kidneys have a primary role in the production and regulation of certain humoral regulators of metabolism, hemodynamics, fluid balance, and oxygen transport. Thus pathology of the kidneys affects those life-sustaining processes. Patients may be taking potent medications that are associated with severe side effects that can further compromise function.

Principles of Physical Therapy Management

Goals of Long-Term Management

In addition to all of the long-term management goals common to all other collagen vascular and connective tissue diseases, there are five additional goals for patients with chronic renal insufficiency:

Exercise

Aerobic exercise is an essential component of the long-term management of the patient with chronic renal insufficiency in order to optimize the efficiency of oxygen transport overall. Maximizing ventilation with exercise is limited if the patient has severe generalized muscular weakness and increased fatigue. Maximal oxygen uptake increases in hemodialysis patients along with improvement in other indices of cardiovascular and pulmonary conditioning. Patients may decrease or eliminate the need for antihypertension medications. Exercise carried out during hemodialysis treatment sessions is feasible and safe for appropriate patients. Because hemodialysis treatments require sessions of several hours multiple times weekly, aerobic training (e.g., cycle ergometry) can be effectively incorporated into treatment time. The exercise prescription of patients with blood glucose abnormalities and coronary artery disease is modified accordingly.

Chest wall exercises can be used and should include all planes of movement with a rotational component. Body positioning to optimize lung volumes and airflow rates is a priority. Breathing control and coughing maneuvers are essential and should be coupled with body movement and positioning.

Individuals Who Are Obese

Pathophysiology and Medical Management

Obesity and its multisystem sequelae are epidemic in industrialized countries; thus obesity must be managed by the physical therapist as a primary condition as well as secondary to other diagnoses. Any other pathology or problem is accentuated when compounded with obesity, and morbidity and mortality are increased for all health concerns.46,47

A detailed assessment is conducted to identify organ systems that are affected and may limit exercise. Central abdominal obesity (apple obesity) constitutes a greater cardiovascular risk than hip obesity (pear obesity).48,49 In younger people, low aerobic fitness in people who are obese appears to reflect deconditioning rather than a primary cardiac problem.50 Individuals who are morbidly obese, however, may develop heart failure. Noninvasive approaches to weight control are superior to invasive approaches in terms of promoting lifelong change and multisystem benefit, health and well-being, and cost-effectiveness.

Pharmacological approaches and surgery are extreme measures whose use is generally not restricted to those with morbid obesity. Vertical banded gastroplasty is increasingly being performed on patients with morbid obesity. One study that compared exercise responses before and 6 months after weight loss with surgery showed that cardiac diastolic function is improved, along with VO2 and anaerobic threshold.51 The extent to which these and additional benefits would have occurred with conservative weight loss, however, and the long-term changes effected by both, were not studied.

Health risks associated with obesity are multisystemic (see Chapters 1 and 6). The cardiovascular and pulmonary risks are life-threatening. In particular, the work of breathing, owing to alveolar hypoventilation, and the work of the heart are increased with increasing body mass. The heart may enlarge to accommodate increased workload, even at rest. Body mass over the chest wall can lead to alveolar hypoventilation and airway closure and reduced oxygenation. Individuals who are obese have problems when recumbent, particularly during sleep (e.g., obstructive sleep apnea, dysrhythmias, and shortness of breath). These problems are identified in the comprehensive assessment.

Principles of Physical Therapy Management

Goals of Long-Term Management

Obesity can be a primary referred problem or a secondary condition. Physical therapists are uniquely suited to counsel and coach patients with weight problems and promote physically active lifestyles, recommend exercise programs, and provide basic knowledge on nutrition and diet commensurate with supporting these activities. Lessons learnt from tobacco control and smoking cessation have been extrapolated for use in the management of the obesity epidemic.52

Physical activity through the course of the day must be increased, and patients who are obese need to participate in a regular program of exercise. The goals of long-term management for the obese patient include all of the elements common to all other conditions.

Patient Monitoring

Patients with obesity require all of the monitoring parameters common to all of the secondary conditions discussed in this chapter.

Maintaining a healthy weight is a function of optimal nutrition and exercise (see recommendations in Chapter 1). The individual’s readiness to change dietary and activity patterns must be assessed to determine the optimal time to introduce a structured program to maximize and sustain long-term outcomes (see Chapters 1 and 28).

A weight loss program is introduced concurrently with an increasing volume of regular physical activity and a structured exercise program. The parameters of the prescription, however, may require modification because of biomechanical stress and discomfort. As weight is lost, the prescriptive parameters are modified to increase the intensity and volume of exercise that is performed. Also, the individual’s movement economy and efficiency will improve as the body’s biomechanics normalize. Initially the hemodynamic responses to even low levels of exercise are likely to be excessive because of the weight that the individual carries during the performance of the activity. Before significant loss of weight, cycling may initially be preferable to walking if the person’s biomechanics limit endurance. Physical activity must be comfortable both for sustainability and injury avoidance.

The role of the physical therapist as health coach is critically important in providing both support to the individual who is obese and long-term follow-up. The probability of success is increased with the involvement of a health care provider and follow-up. Facilitating weight loss and adopting long-term healthy lifestyle behaviors can be lifesaving for a patient. Given the current epidemic of obesity, assisting an individual to attain a healthy lifestyle through optimal nutrition, exercise, and weight loss is a singularly important health outcome.

Summary

This chapter reviews pathophysiology and medical management as they relate to the comprehensive physical therapy management of individuals with chronic secondary cardiovascular and pulmonary pathology. Exercise testing and training are major components of the comprehensive management of these conditions and are presented separately in Chapter 25.

This chapter specifically presents the comprehensive physical therapy management of chronic cardiovascular and pulmonary dysfunction secondary to neuromuscular, musculoskeletal, collagen vascular and connective tissue, and renal dysfunction. The neuromuscular conditions that are presented include stroke, Parkinson syndrome, multiple sclerosis, cerebral palsy, spinal cord injury, chronic effects of poliomyelitis, and muscular dystrophy. The musculoskeletal conditions that are presented include thoracic deformity (kyphoscoliosis) and osteoporosis. The collagen vascular and connective tissue conditions that are presented include SLE, scleroderma, ankylosing spondylitis, and RA. Finally, management of the patient with chronic renal insufficiency and the person who is obese is presented.

The principles of patient management are presented rather than treatment prescriptions, which cannot be given without specific consideration of an individual patient. In this context, the goals of long-term management of patients with each condition are presented, followed by the essential monitoring required, and the primary interventions for maximizing cardiovascular and pulmonary function and oxygen transport. The selection of interventions for any given patient is based on the physiological hierarchy. The most physiological interventions are exploited, followed by less physiological interventions and those whose effectiveness is less well documented.

These principles must be considered if a patient has a chronic secondary cardiovascular and pulmonary condition as a secondary diagnosis for which the patient is being treated by the physical therapist. These principles have implications for treatment prescription and monitoring in the management of the condition for which the patient is being primarily treated.

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