27. DYSPNEA

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CHAPTER 27. DYSPNEA
Kim K. Kuebler, Jerald M. Andry and Shawn Davis
The symptom of dyspnea, or breathlessness, has been defined as an uncomfortable awareness of breathing (Baines, 1978). The American Thoracic Society (ATS) defines dyspnea as “a subjective experience of breathing discomfort that consists of qualitatively distinct sensations that vary in intensity” (ATS, 1999). The experience is a combination of physiological, social, and environmental factors that potentiate physiological and behavioral response (ATS, 1999). Dyspnea not only is a common companion to pulmonary and cardiac diseases, but also often accompanies the dying process. The clinician providing management for the patient with dyspnea should always consider the underlying pathophysiology contributing to the patient’s breathlessness. Having knowledge related to the cause of dyspnea will direct the clinician on how best to strategize his or her diagnostic evaluation and consideration for specific interventions.
The literature suggests that there is a large variation in the reported prevalence of dyspnea, which ranges from 21% to 79% (Bruera, Sweeney, & Ripamonti, 2002). “These variations are a result of the different natures of patient populations reported by different authors and the lack of a general consensus regarding the assessment and evaluation of dyspnea” (Bruera et al., p. 359, 2002). A number of authors have reported the incidence of dyspnea in different patient populations that include patients in the last month of life, patients with advanced cancer, and patients without intrathoracic malignancies, chronic congestive heart failure, and chronic obstructive pulmonary disease (COPD) (Bruera et al., 2002). Most cancer patients develop progressive dyspnea over days or weeks, yet for those patients who experience a sudden onset of dyspnea, a medical emergency should be considered (Bruera et al., 2002).

ETIOLOGY AND PATHOPHYSIOLOGY

Dyspnea encompasses complex interactions between peripheral and central sensory receptors and cognition, while the underlying causation remains unknown. Physiological, psychological, behavioral, social, and environmental factors play a role in the pathogenesis and perception of dyspnea (Rao & Gray, 2003). Although the underlying medical condition may not be treatable in the palliative care patient, dyspnea almost always responds to intervention or treatment.

Normal Control of Breathing

As simplistic as inhaling and exhaling seems, it is actually a complex of physiological mechanisms that are constantly adapting to a multitude of psychological, social, and environmental factors. The main function associated with the respiratory system is to obtain oxygen from the atmosphere to supply functioning cells and remove carbon dioxide (CO) produced by cellular metabolism (Levitzky, 2003). All respiratory processes are controlled by the central nervous system, which responds to stimuli in order to maintain homeostasis. The primary example is how the respiratory center in the medulla oblongata responds to stimuli from four primary sources (ATS, 1999; Guyton & Hall, 1996):
▪ Chemoreceptors in the aorta, carotid arteries, and medulla sense changes in P o2, P co2, and pH and transmit signals back to the respiratory center to adjust breathing. The peripheral chemoreceptors (i.e., those in the aortic arch and carotid arteries) are most sensitive to changes in P o2. When P o2 decreases, ventilation increases. However, hypoxia must be fairly profound before this change in respiratory pattern is seen. The central chemoreceptors of the medulla are very sensitive to changes in pH. Changes in pH are closely related to P co2. Hypercapnia leads to a decrease in pH, which then stimulates ventilation.
▪ Mechanoreceptors in the diaphragm and chest wall sense changes in the work of breathing. When an increased workload is sensed, the respiratory center stimulates the diaphragm and respiratory muscles and attempts to expand the lungs.
▪ Vagal receptors in the airways and lungs also influence breathing. Afferent impulses are generated when (1) stretch receptors in the lungs are stimulated as the lungs expand, (2) irritant receptors in the bronchial walls are stimulated, or (3) C fibers in the interstitium of the lungs respond to increases in pulmonary interstitial or capillary pressure.
▪ Cortical areas of the brain affect breathing by allowing individuals to consciously increase or decrease their respiratory rate. It also appears that the chemoreceptors, mechanoreceptors, and respiratory center itself send messages to higher brain centers, leading to a cognitive awareness of the ventilatory demand.

Mechanisms Leading to Dyspnea

Patients may describe dyspnea by using several phrases such as “My breathing requires effort,” “I feel a hunger for more air,” or “I feel out of breath” (Harver, Mahler, Schwartzstein et al., 2000). In a palliative care patient, the perception of dyspnea includes several qualitatively distinct sensations that may be caused by one or more pathophysiological mechanisms (Manning & Schwartzstein, 1995). As described, receptors throughout the body will trigger the central nervous system when homeostasis is altered, which can ultimately lead to the feeling of dyspnea. However, specific data are not available to explain how dyspnea is processed by higher brain centers in humans (Guz, 1997). Research has shown that the known dyspnea pathways may be shared by painful stimuli such as heat, cold, and electrical stimulation (von Leupoldt & Dahme, 2005). Thus, there may be a common neural network explaining pain and dyspnea, which are common symptoms in the palliative care patient population. Before the brain can interpret changes and alter breathing patterns, peripheral stimuli must be gathered by various receptors.
Respiratory muscle abnormalities lead to a mismatch between the central respiratory motor output and the achieved ventilation. Respiratory muscle weakness, whether caused by generalized weakness or a neuromuscular disorder, is perceived as dyspnea. Many persons with advanced diseases experience anorexia, cachexia, and generalized weakness and are at risk for experiencing dyspnea. In diseases that cause hyperinflation of the lung and overexpansion of the thorax, the muscles of inspiration become weakened, leading to the sensation of dyspnea (ATS, 1999; Guyton & Hall, 1996). This dyspnea may be mediated via mechanoreceptor messages to the cortex.
Abnormal ventilatory impedance (e.g., narrow airways or increased airway resistance) leads to stimulation of increased central respiratory motor output. When the effort expended to breathe is not matched by the level of ventilation, dyspnea is perceived (ATS, 1999). This perception may result from vagal or mechanoreceptor stimulation of the cortex.
Cognitive factors also influence the perception of dyspnea (ATS, 1999; Guyton & Hall, 1996; Kuebler, Dahlin, Heidrich et al., 1996; Twycross, 1999). Although many physiological mechanisms lead to a perception of dyspnea, not all dyspnea is a direct result of pathological structural characteristics. Psychological states are interrelated to the dyspneic experience, which may be caused or exacerbated by anxiety, fear, hopelessness, and depression. Breathlessness precipitates anxiety, leading to increased breathlessness and even more anxiety; commonly called the “snowball effect.” The patient’s perception of dyspnea is decreased when he or she believes that the shortness of breath is treatable and that prompt access to treatment is available.
Dyspnea is predominantly experienced by persons with pulmonary, cardiac, and neuromuscular diseases (Ahmedzai, 1998; Carrieri-Kohlman & Janson-Bjerklie, 1993; Twycross, 1997). Table 27-1 summarizes many of the causes of dyspnea in the terminally ill. Note also that cognitive factors may contribute to all causes of dyspnea; thus, they are not included. Although respiratory muscle abnormality due to weakness may be present in almost all advanced diseases, Table 27-1 lists the diseases in which weakness is most profound.
TABLE 27-1 Causes of Dyspnea in Terminal Illness
Disease Process Blood Gas Abnormalities Increased Ventilatory Demand Respiratory Muscle Abnormality Ventilatory Impedence
Pulmonary Disease
Chronic obstructive pulmonary disease X X X X
Asthma X X
Cystic fibrosis X X X
Pneumonia X X
Pleural effusion X X X
Malignancy X X X X
Radiation pneumonitis X X
Pulmonary embolism X X
Cardiovascular Disease
Heart failure X X
Myopathies X X
Anemia X X
Superior vena cava syndrome X X
Neuromuscular Disease
Muscular dystrophy X X
Myasthenia gravis X X
Amyotrophic lateral sclerosis X X
Paralysis of diaphragm X X

ASSESSMENT AND MEASUREMENT

Patients who complain of being breathless will often seek some relief through position changes, pursed lip breathing, use of accessory muscles, and/or use of environmental aids such as a fan or sitting near an open window or refraining from being exposed to humidity. Breathlessness can be easily observed (objective evaluation) in the patient who is struggling to breathe—but is often considered a subjective experience. For the clinician, a simple approach used to assess and evaluate the patient’s dyspnea would be to ask the patient to quantify his or her difficulty in breathing from a numerical scale that is rated from 0 (no breathlessness) to 10 (severe breathlessness). A numerical value provides the clinician with an understanding of the patient’s expression of his or her dyspnea intensity. The Edmonton Symptom Assessment System (ESAS) is an example of a numerical rating scale to evaluate the patient’s intensity associated with multiple symptoms including dyspnea (Bruera, Kuehn, Miller et al.,1991).
There are several psychometrically valid and reliable dyspnea assessment tools that are used in clinical and research settings to assess and evaluate the symptom of dyspnea. The traditional approach to providing care for patients with chronic respiratory disease has primarily relied on pulmonary function tests (PFTs) to quantify the severity of the patient’s disease and/or their response to therapy (Mahler, 2000). However, patients often present for medical attention as a result of their symptoms, particularly dyspnea and an impaired ability to perform activities of daily living, with both contributing to the interference of the patient’s perceived quality of life (Mahler, 2000). The patient’s perception of breathlessness that interferes with quality of life may not always be accurately reflected in the most recent spirometry evaluation (GOLD, 2003). As previously mentioned, simply asking the patient to rate his or her dyspnea or difficulty breathing from a numerical rating scale may serve as a useful indicator of the patient’s self-description of his or her discomfort. Other examples of dyspnea evaluation tools will be briefly described below. The patient’s physical status should be considered in instrument selection, since lengthy instruments are not generally appropriate in palliative care populations.

The St. George’s Respiratory Questionnaire

The St. George’s Respiratory Questionnaire (SGRQ) contains 76 items that are divided into three sections and include:
▪ Symptoms—affecting normal respirations to include the frequency and severity
▪ Activity—interference with the ability to engage in activities as a result of breathlessness
▪ Impact—influence that breathlessness has on a wide array of social and psychological difficulties:
Each of these sections is scored and then a final score is calculated to discern the severity of patient breathlessness (Jones, Quirk, & Baveystock, 1991). The SGRQ was developed to measure quality of life in patients with airway disease with the intent to exert greater sensitivity and be useful in patients with mild as well as severe disease (Jones et al., 1991).

Baseline and Transition Dyspnea Index

The Transition Dyspnea Index (TDI) was developed to provide a discriminative and evaluative assessment of dyspnea in pharmacotherapy trials for COPD (Witek & Mahler, 2003). This tool is divided into the Baseline Dyspnea Index (BDI) as a discriminative instrument to measure dyspnea at a single point in time (Mahler et al., 2004) and the TDI as an evaluative tool to assess changes in dyspnea from baseline. These tools consist of dyspnea indices that assess breathlessness in domains related to functional impairment, magnitude of task, and magnitude of effort (Mahler, Weinberg, Wells et al., 1998; Mahler, Ward, Fierro-Carrion et al., 2004).
The BDI/TDI evaluation tool has been proved useful in multiple clinical trials seeking to evaluate medication use to relieve dyspnea in patients with chronic lung disease. Successful demonstration of the relief of dyspnea with drug therapy depends on achieving consistent results using valid instruments (Witek & Mahler, 2003). The BDI/TDI, however, has received criticism from the interview process maintaining that the interpretation by the interviewer may introduce bias. Recently, the BDI/TDI was modified to allow patients to perform self-administration through a computerized venue. This has been further validated (Mahler et al., 2004).

Medical Research Council

The Medical Research Council Scale was developed in 1959 to provide patients an opportunity to grade their breathlessness based on a single dimension (i.e., daily tasks). This tool has been tested in patients with dyspnea from a variety of respiratory and cardiovascular origins (Fletcher, Elmes, & Wood, 1959).

HISTORY AND PHYSICAL EXAMINATION

Patients experiencing the sensation of dyspnea or breathlessness will often seek medical attention. The clinician encountering the dyspneic patient should consider asking the patient specifically about his or her shortness of breath. Because dyspnea is considered a subjective complaint, it is important to note that patients may often present with tachycardia and look in distress yet may not describe being dyspneic or distressed versus the patient who is not tachypneic or in apparent distress yet may describe having severe breathlessness (Pereira & Bruera, 2001).
The etiology of dyspnea may be easy to discern in most patients by taking a complete history and performing a physical examination. A detailed examination with a focus on the cardiac and respiratory systems is essential (Bruera et al., 2002). Simple measures such as a chest radiograph, digital oximetry, and a complete blood count and comprehensive metabolic profile tests can help in differentiating the cause of dyspnea (Bruera et al., 2002). Pulmonary function tests (PFTs) are useful for the patient with obstructive and restrictive pulmonary disease. These tests are easy to perform at the bedside and can also provide the clinician with valuable information on how the patient responds to specific interventions. If done appropriately, the patient will use a bronchodilator after the first evaluation and measurements are considered before and after bronchodilator use.
Differential diagnostics are important as dyspnea is a complex symptom that may arise from multiple insults. Common contributions to dyspnea can include the following (Bruera et al., 2002; Pereira & Bruera, 2001):
▪ Primary lung malignancy
▪ Pleural or pericardial effusion
▪ Carcinomatous lymphangitis
▪ Pulmonary embolism
▪ Chemotherapy-induced fibrosis
▪ Superior vena cava syndrome
▪ Depression and anxiety
▪ Pneumonia
▪ Muscle deconditioning (cachexia)
▪ COPD, neuromuscular disease
▪ Anemia
▪ Congestive heart failure (cor pulmonale)

DIAGNOSTICS

Diagnostic procedures can provide assistance in identifying the causes of dyspnea and monitoring the course of the illness, but the practitioner must evaluate the appropriateness of such diagnostic tests in palliative care. A diagnosis can sometimes be made based solely on the clinical presentation alone; thus, the burden and cost incurred by the patient must be considered. When a diagnosis is more complicated, a clinician may have a clinical history, physical examination, laboratory tests (complete blood cell count, metabolic panel), and possibly a chest radiograph as an initial work-up. Pulmonary diagnostics, such as spirometry and PFTs (outlined later), provide supporting information to help the clinician make the diagnosis (West, 2003). Additionally, spirometry and PFTs are valuable in following the progress of a patient’s dyspnea over time. Other diagnostic tests that may not be used in palliative care include the following (Karnani, Reisfield, & Wilson, 2005; West, 2003):
▪ Pulse oximetry
▪ Echocardiography
▪ Brain natriuretic peptide
▪ Arterial blood gas
▪ Ventilation-perfusion ( B978141603079950035X/u27-01-9781416030799.jpg is missing/ B978141603079950035X/u27-02-9781416030799.jpg is missing) scan
▪ Bronchoscopy
▪ Lung biopsy

Spirometry

Spirometry is a highly effort-dependent diagnostic test that measures the volume of air (liters) exhaled or inhaled by a patient as a function of time (Evans & Scanlon, 2003; Karnani et al., 2005). Spirometry allows clinicians to distinguish between obstructive (i.e., asthma and COPD) and restrictive (i.e., fibrosis, chest wall limitation, pleural diseases, neuromuscular disorders) diseases, in which patients experience dyspnea (Karnani et al., 2005; West, 2003). The typical end points measured via spirometry are the forced expiratory volume in 1 second (FEV 1), the forced vital capacity (FVC), and/or the forced expiratory volume in 6 seconds (FEV 6). FVC can be extremely challenging in older or impaired patients, so FEV 6 has been shown to be an acceptable surrogate alternative that is available in most newer spirometers. Spirometry has limitations because it does not allow clinicians to measure lung volumes; other PFTs are used to potentially identify the underlying cause of the patient’s dyspnea (Karnani et al., 2005).

Pulmonary Function Tests

PFTs are used in addition to spirometry to determine the volume of air in the lungs at any given time. Such volumes include the total amount of air in the lungs at full inspiration (total lung capacity [TLC]), the amount of air left in the lungs at the end of normal expiration (functional residual capacity [FRC]), and the amount of air remaining after maximal expiration (residual volume [RV]) (Evans & Scanlon, 2003; Karnani et al., 2005). A clinician will may also order a diffusing capacity (D Lco) to estimate the patient’s ability to absorb alveolar gases in the lung. The DL co substitutes CO as a surrogate for oxygen in order to measure the amount of oxygen that is absorbed into the bloodstream (Evans & Scanlon, 2003). By knowing lung volumes and the amount of oxygen absorbed, clinicians are able to narrow the pathophysiology associated with dyspnea.

Radiography: Computed Tomography and Magnetic Resonance Imaging

Clinicians do not typically order radiographs, high-resolution computed tomography (CT) scans, and/or MRI for each patient who presents with dyspnea. However, such diagnostics are ordered to rule in and rule out underlying pathologies that are seen in palliative patients. High-resolution CT could be used to diagnose bronchiectasis and identify pulmonary embolism or idiopathic pulmonary fibrosis (Evans & Scanlon, 2003).

INTERVENTIONS AND TREATMENT

The treatment of dyspnea begins with determining and treating the underlying cause. This includes the appropriate selection of disease-specific and palliative therapies, taking into account prognosis, adverse events, costs, and potential outcomes to the patient. The most common reversible causes of dyspnea are bronchospasm, hypoxia, and anemia (Dudgeon & Lertzman, 1998). Appropriate management of dyspnea requires pharmacological and nonpharmacological treatments. Two types of drugs that have proved useful in alleviating dyspnea in palliative care are opioids and drugs that decrease anxiety. Table 27-2 summarizes pharmacological options for the management of dyspnea based on cause. Many of the other medications described in this chapter concerning dyspnea relief have primarily been studied in other disease states such as asthma, COPD, and cancer.
TABLE 27-2 Correctable Causes of Dyspnea/Breathlessness
From Twycross, R. (1999). Correctable causes of breathlessness. In R. Twycross. Introducing palliative care (p.123). Oxon, UK: Radcliffe Medical Press. © 1999
Causes Management
Respiratory infection Antibiotics
Expectorants
Physiotherapy
Chronic obstructive pulmonary disease and asthma Bronchodilators
Corticosteroids
Theophylline
Physiotherapy
Hypoxia Trial of oxygen
Bronchial obstruction and lung collapse Corticosteroids
Mediastinal obstruction Radiotherapy
LASER therapy
Stent
Lymphangitis carcinomatosa Corticosteroids
Diuretics
Bronchodilators
Pleural effusion Paracentesis
Pleurodesis
Ascites Diuretics
Paracentesis
Pericardial effusion Paracentesis
Corticosteroids
Anemia Blood transfusion
Erythropoietin
Cardiac failure Diuretics
Digoxin
Angiotensin-converting enzyme inhibitors
Pulmonary embolism Anticoagulants (if appropriate)

Opioids

Opioids are commonly used medications to treat dyspnea in palliative care. The mechanisms of action by which opioids alleviate dyspnea are unclear but may include decreasing the central perception of dyspnea (similar to pain), decreasing anxiety, decreasing sensitivity to hypercapnia, reduction in oxygen consumption, and improved cardiovascular effects (Ahmedzai, 1998; Twycross, 1999

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