Individuals with Chronic Secondary Cardiovascular and Pulmonary Dysfunction

Elizabeth Dean and Donna Frownfelter

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

Patient Education

Education is a principal focus of the long-term management of all patients (see general principles and guidelines in Chapter 28), but it is especially important for those with secondary dysfunction. Patients should receive instruction in and information on the following health promotion and preventative practices:

Infection control

Smoking reduction and cessation

Cold and flu prevention (including flu shots)

Aerobic exercise

Strengthening exercises

Information or instruction particular to each secondary condition is discussed in further detail in this chapter.

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:

Optimizing aids and devices to reduce unnecessary energy demands by optimizing postural alignment

Maximizing balance to also reduce unnecessary energy demands to maintain alignment

Optimizing chest wall excursion and ventilation

Optimizing secretion clearance as needed

Reducing the work of breathing

Reducing the work of the heart

Protecting the airways from aspiration

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.

Medication

Medication that is needed to maximize treatment response (e.g., antihypertensive and cardiac medications) is administered before treatment. Knowledge of the type of medication, its administration route, and time to and duration of peak efficacy is essential if treatment is to be maximally effective.

Primary Interventions

Primary interventions for patients with stroke include the combination of global interventions previously discussed, and in addition they require the following:

Body positioning instruction

Breathing control and coughing maneuvers

Spasticity control

Gait reeducation

An ergonomic assessment of the patient’s work and home, if applicable, may also help ensure the safety of the patient in those environments.

Patient Education

Patients with stroke will require the same instruction common to all patients with chronic secondary dysfunction.

Engagement of the social support network can have an important role in maximizing a patient’s outcomes. Social support is recognized clinically as an important component of the comprehensive management of people with chronic conditions; however the literature in this area is scant. Family participation has been reported to improve the strength and mobility of an individual with stroke.⁷

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.

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; control of 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.

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

Goals of Long-Term Management

The goals of long-term management for the patient with Parkinson syndrome include all of the elements common to all other conditions, but also include the following:

Optimize secretion clearance as needed

Reduce the work of breathing

Reduce the work of the heart

Protect the airways from aspiration

Patient Monitoring

Patients with Parkinson syndrome require the same monitoring parameters common to all of the secondary conditions discussed in this chapter. Patients with additional cardiac dysfunction require ECG monitoring, particularly during exercise.

Medication

Medication that is needed to maximize treatment response (e.g., L-dopa) is administered before treatment. Knowledge of the type of medication, its administration route, and time to and duration of peak efficacy is essential if treatment is to be maximally efficacious. In addition, patients with Parkinson syndrome may be taking a beta blocker to suppress action tremor. Because such medication reduces the heart rate and blood pressure, these patients are prone to orthostatic intolerance. In addition, heart rate and blood pressure responses to treatment and exercises will be less valid.

Primary Interventions

Primary interventions for patients with Parkinson syndrome include the combination of global interventions previously discussed, and in addition they require the following:

Body positioning instruction

Breathing control and coughing maneuvers

Gait reeducation

Flexibility exercise to facilitate participation in regular physical activity and exercise

For optimal functional outcomes, prescriptive parameters focus on cross training rather than a single aerobic type of exercise, and frequent, longer, less intense physical activity rather than less frequent, short, intense exercise.

Patient Education

Patients with Parkinson syndrome will require the same instruction common to all patients with chronic secondary dysfunction and in addition will likely also need instruction on airway protection.

Exercise

Aerobic exercise is an essential component of the long-term management of the patient with Parkinson syndrome to optimize the efficiency of oxygen transport overall, including maximizing alveolar ventilation and mobilizing secretions, as well as for its musculoskeletal benefits. Maximizing ventilation with mobilization is limited by the degree of hypertonicity and rigidity. Chest wall exercises 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.

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

Goals of Long-Term Management

In addition to all of the long-term management goals common to all other conditions, there are several more goals associated with this population:

Protect the airways from aspiration

Optimize secretion clearance as needed

Reduce the work of breathing

Reduce the work of the heart

Patients with multiple sclerosis may need prescriptive aids and devices to optimize function and activity.

Patient Monitoring

Patients with multiple sclerosis require the same monitoring parameters common to all of the secondary conditions discussed in this chapter. Patients with additional cardiac dysfunction require ECG monitoring, particularly during exercise.

Multiple sclerosis is associated with significant fatigue; thus activity and sleep patterns need to be assessed to ensure sleep is maximally restorative and not contributing to the patient’s symptoms. Fatigue can be assessed subjectively using a modified version of the Borg scale or assessed objectively using a log of activity and rest. The log will help the patient identify the optimal time to rest.

Medication

Medication that is needed to maximize treatment response (e.g., antispasticity medications) is administered before treatment. Knowledge of the type of medication, its administration route, and time to and duration of peak efficacy is essential if treatment is to be maximally effective. In addition, knowledge of the cardiovascular and pulmonary side effects of other medications is needed.

Primary Interventions

Primary interventions for patients with multiple sclerosis include the combination of global interventions previously discussed, and in addition these patients require the following:

Body positioning instruction

Breathing control and coughing maneuvers

Gait reeducation

Fatigue management

Prescriptive parameters are comparable to those for individuals with increased muscle tone—that is, frequent, less intense exercise, rather than the less frequent, intense exercise that might be appropriate for someone without such a condition.

Patient Education

Patients with multiple sclerosis require the same instruction common to all patients with chronic secondary dysfunction and in addition they will likely need instruction on fatigue management, as multiple sclerosis is associated with significant fatigue.

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.