Anne Mejia-Downs & Kathy Lee Bishop
INTRODUCTION
This chapter will address Practice Pattern 6C of the Guide to Physical Therapist Practice: Impaired Ventilation, Respiration/Gas Exchange and Aerobic Capacity/Endurance Associated with Airway Clearance Dysfunction. As you may recall from the oxygen transport pathway (see Chapter 5, Physiology of the Cardiovascular and Pulmonary Systems), the ability to exchange oxygen and carbon dioxide is dependent on an unobstructed path from the upper airways to the lungs. Normally, pulmonary secretions are easily removed from the airways by the mucociliary escalator, clearing the way for gas exchange to occur. When a dysfunction exists in the airway clearance mechanism, the inability to remove pulmonary secretions may result in decreased oxygen carried to the circulatory system, resulting in impaired muscle performance and aerobic endurance.
This chapter provides a review of the anatomy and physiology of the pulmonary system and describes the pathophysiology of cystic fibrosis (CF), an obstructive lung disease. The components of physical therapy (PT) management via Practice Pattern 6C will be illustrated, using a case study of a patient with CF. The management of airway clearance dysfunction is integral to this patient population and will demonstrate the effects of this dysfunction on impairments in physical function. Threshold levels or factors that affect ventilation, airway clearance, activity, and musculoskeletal function will be described and matched to appropriate interventions. The International Classification of Functioning, Disability and Health (ICF) will be used to demonstrate the body functions and structures evident in CF and their relation to activity and participation. Finally, though research efforts in this area are ongoing, there are limits to our knowledge about the disease of CF and the PT management that is currently used. Future directions for discovery in the realm of CF are discussed.
ANATOMY AND PHYSIOLOGY
Anatomy
The pulmonary system can be divided into two distinct categories: the musculoskeletal pump and a gas-exchanging organ.1–3 These components act in synchrony to achieve both ventilation and respiration. Whereas the pump is made up of the thorax and attaching muscles, gas exchange is carried out by the upper and lower divisions of the airways and associated components.
There are 23 generations or divisions of airways.2 The primary lobule or acinus consists of a transitional zone of bronchioles, alveolar ducts, and alveoli.2,4 Capillaries surround the alveoli for ease of gas exchange. The lungs are supplied with blood from the bronchial circulation (airways) and the pulmonary artery (alveoli).2,4 Gas exchange occurs by diffusion across the blood–gas barrier and follows the principles of Fick’s law. Basically, the rate of gas transferred depends on the area of exchange, thickness of the area, and the partial pressure between the two sides.2 If the lung tissue was spread out over the ground, the total size of the area would be similar to that of a tennis court.2The properties of the “gas” and tissue where the gas is being exchanged also affect the rate of diffusion.
Diseases such as CF may affect gas exchange in multiple ways: distance for gas exchange (clogged alveoli, chronically infected areas), size of gas exchange area (collapsed segments, shunted blood), and alteration in the partial pressure gradient (poor ventilation, poor distribution of air, elevation of arterial partial pressures). Chronic infected alveolar areas that are not cleared of secretions will result in shunting of the capillary blood away from the poorly oxygenated alveoli. This could result in a large portion of the pulmonary capillary blood being shunted that may lead to progression of right ventricular hypertrophy.5,6 This shunting is due to poorly oxygenated alveoli and results in vasoconstriction of the pulmonary vascular bed.7 In some cases, erosion into the capillary system can occur that causes bleeding.5,7
Surfactant is secreted in the alveoli to decrease surface tension and prevent alveolar collapse. Smoking, excessive secretion retention, exposure to general anesthesia, elimination of blood flow to an alveolar area (emboli), premature birth, and prolonged use of high concentrations of oxygen can lead to the elimination of the surfactant or a decrease in production.2,8 Without the surfactant to lower the surface tension in the alveoli, there could be alveolar collapse, atelectasis, and possible infection.
The pores of Kohn connect adjacent alveoli and are in theory vital for collateral ventilation of blocked alveolar units.2 They also participate in airway defense by allowing macrophages to move from one alveolus to another to engulf particles that are too small to be captured in the upper airway. This mechanism can also work against the lower airways, as in the case of exposure to tuberculosis: The bacilli are engulfed and cannot be digested but instead become part of the macrophage.9 In the case of various airway clearance techniques, utilization of the collateral channels via the Lambert canals (respiratory bronchioles and alveoli) and the pores of Kohn promote equalization from one blocked alveolus to another blocked with secretions.10 This allows movement of secretions from a blocked alveolus to the bronchiole and upward toward the carina (bifurcation of the right and left main stem bronchi) to be expectorated or swallowed.
Physiology
Mucociliary Escalator
Mucus is produced by the goblet cells, which line the tracheobronchial tree and acts as a medium to collect inhaled particles.3,11 The mucus is primarily made up of water, glycoproteins, carbohydrates, and lipids. Dead cells, electrolytes, and foreign particles can also be found in the mucus.1,11 More than 100 mL/d of mucus is produced by the respiratory tract.11
The lower airways act as a conduit for airflow and play a role in mucociliary transport.1,2 Mucociliary transport refers to the action of the sol–gel layer and cilia, which trap and then sweep up the particles to be expectorated or swallowed (see Fig. 17-1).1 This combination of a mucus layer and ciliary action is a major defense, protecting the lower airways from inhaled particles. Water, electrolytes, and several varieties of mucopolysaccharides account for the thickness of the mucus.1 The cilia beat upward in a rhythmic fashion, moving the sol layer and trapped particles upward and away from the lower airways. This layer is affected by smoking, anesthesia, dehydration, and various pathologies that lead to thick, tenacious secretions, and/or immotile cilia unable to sweep the mucus upward.1,12,13 (See Chapter 5 for a detailed presentation of physiology.)
FIGURE 17-1 Mucociliary escalator.
Cough
Exhalation is usually passive but can be forced with muscle contraction during a cough. Cough is an important protective reflex for the pulmonary system. An ineffective cough can lead to retained secretions, atelectasis, respiratory compromise, and in some cases respiratory failure.10 A cough can be elicited consciously or by reflex stimulation.2 A reflex cough occurs when irritants trigger impulses that are sent along the vagus nerve to the medulla. Mucus raised to the level of the carina stimulates a cough to expel the mucus.3 The phases of the cough include a large inspiration of air; closing of the glottis; contraction of the abdominal and thoracic muscles to build up intrathoracic and intra-abdominal pressure; and sudden opening of the glottis, with explosive expulsion of air.14 Forced exhalation or huffing may be used in place of or in addition to a cough to expectorate secretions. In order for forced exhalation to be effective, a large volume of air must be inhaled and the glottis must stay open.
Patients with CF or other obstructive lung diseases that are characterized by air trapping may have difficulty with effective forced exhalation or coughing because of premature closure of the smaller airways. A patient will often complain of the inability to catch their breath, but the problem is not on inhalation as might be assumed but rather on exhalation when the early collapse of the airways promotes air trapping. Over time, the respiratory muscles increase in length due to air trapping. This puts the respiratory muscles at a length–tension disadvantage. The poorly exhaled volumes combined with a dysfunctional musculoskeletal component and fatigue lead to poor timing of the cough sequence. The end result is a patient who is frustrated and fatigued and has been unable to effectively cough up the secretions that stimulated the cough.
The value of the cough as a defense mechanism is only effective down to the sixth and seventh generations of the bronchi.14 The mucociliary blanket must work in synchrony with the cough as a defense mechanism. Constant irritation from persistent mucus may lead to bronchospasm, narrowing of the airway, and poor airflow, thereby lessening the benefit of the cough.14 In chronic pulmonary diseases like CF, where coughing is a daily occurrence, supraclavicular retraction can be observed during paradoxical coughing, even in areas of the airway that have cartilage. Poor cough technique, excessive pressures generated during the coughs, and fatigue of the surrounding structures may lead to collapse in this area, adding to the ineffective and frustrating nonproductive cough.
PATHOPHYSIOLOGY OF CYSTIC FIBROSIS
Genetic Predisposition
CF is an inherited, autosomal recessive disease and the most common lethal genetic disease of the Caucasian population.15 The altered gene is located on the long arm of chromosome 7.15 The most common gene mutation is the delta F508.15,16 There is a deletion of phenylalanine at position 508. This deletion leads to an altered production of the protein cystic fibrosis transmembrane conductance regulator (CFTR). The resulting defect affects chloride ion transmission across epithelial cells. Excessive sodium reabsorption is a consequence of CF transmembrane conductance regulator, which results in dehydration of surface fluids, abnormally salty sweat, and thick mucus that clogs up tubes, tubules, and ducts.17
Both parents must carry the genetic defect. The odds ratio of having a child with the disease is approximately 1 in 4 (see Fig. 17-2). The lungs are reported to be normal at birth, but changes in the mucus lining the airways rapidly occur. For this reason, newborn screening has been instituted in many states to identify individuals with CF as soon as possible.18–20 In patients with CF, meconium ileus is a frequent occurrence at birth and has been considered one of the hallmarks for diagnosis. Essentially, meconium ileus is a bowel obstruction related to meconium (the substance found in newborn and fetal bowels), the change in the intestinal mucus, and pancreatic insufficiency.15 In most cases, the diagnosis of CF will be made when stimulation from pilocarpine iontophoresis produces a positive sweat test, defined as >60 mEq/L of sodium.15 Identification of the gene responsible for CF has made DNA testing to identify CF mutations possible as an additional diagnostic test.21 In individuals with a clinical picture of CF, identification of two known CF mutations by an accredited laboratory confirms the diagnosis.7,22 Although it is common for CF to be diagnosed at birth or in infancy, CF may not be suspected in patients until they are much older and symptoms have begun to interfere with their lifestyle. Presentation of exocrine pancreatic insufficiency, a family history, and chronic obstructive pulmonary changes confirm the CF diagnosis. In addition, problems with the reproductive system may lead to the diagnosis of CF; men are azoospermic and women have difficulty with fertilization as a result of thickening in the mucus lining of the uterus.5
FIGURE 17-2 The inheritance of CF. Each parent of a child with CF has one abnormal CF gene (blue circle). The abnormal CF gene causes no problems if it is paired with a normal CF gene (red circle). When two parents each of whom carries an abnormal CF gene have children, each parent passes on either the normal or the abnormal CF gene. The figure shows the possible combinations of genes which children of carriers can have (1) a normal CF gene from both father and mother; (2) a normal CF gene from the mother and an abnormal gene from the father; (3) an abnormal CF gene from the mother and a normal gene from the father; and (4) an abnormal CF gene from each parent. Each of these four combinations is just as likely to occur as the others, meaning that the chances of two carrier parents having a child with CF is one in four each time they have a child. (Used with permission from D. M. Orenstein, Cystic Fibrosis: A Guide for Patient and Family, 2nd ed., Lippincott Williams & Wilkins, 1997.)
Clinical Manifestations of Cystic Fibrosis
The pathogenesis of CF is presented in Box 17-1. The pathologic changes in the lung occur at the bronchiole level. Inflammation and infection play active roles in destructive changes at this level. Staphylococcus aureus is one of the pathogens that can be seen early in the process, but Pseudomonas aeruginosa colonization is the primary culprit of infection. Initially, the organisms are nonmucoid, but a change occurs in which alginate, an exopolysaccharide, forms a gel-like substance incorporating the bacteria and protecting it from normal airway defenses.5 Over time the larger and more central airways become involved. The glands that secrete the mucus become hypertrophied, and thick tenacious mucus is produced. This combination of hypertrophy and enhanced secretion adds to narrowing of the airways. The normal mucociliary clearance mechanism cannot function appropriately to mobilize the secretions upward and out of the lungs. The structural integrity of the airways is changed, and bronchiectatic reconstruction ensues.
BOX 17-1
Other clinical changes occur in patients with CF. Clubbing, a widening and flattening of the terminal portion of the digits, is a sign of hypoxemia that occurs because of the interruption of normal gas exchange. Progressive hypoxemia can lead to complications of pulmonary hypertension and right-sided heart failure or cor pulmonale. Pneumothorax (air in the chest cavity outside the lung) and hemoptysis (blood in the sputum or mucus) are two complications that can occur as a result of the destruction of the integrity of the airways walls.23,24 Recovery from these conditions is dependent on the extent of the damage and the ability to heal and compensate for the decreased function of the gas-exchanging organ.
Clinically, in patients with CF, one may observe increased accessory muscle use at rest when the work of breathing becomes greater. Additionally, development of a barrel chest related to air trapping, a need for supplemental oxygen for exercise with progression to continuous oxygen use, and a decline in activity may occur. An increase in the amount and viscosity of secretion production, a darkening in the color of secretions produced, and an elevated heart rate (HR) related to a chronically infected and hypoxic state may also be noted. In addition, patients with CF often exhibit a short stature and experience a decrease in body weight. Children with CF typically have voracious appetites yet fail to gain weight. Often, this phenomenon is what brings the child to the physician’s office, where the diagnosis of CF is made. The inability to gain weight is caused by a high resting metabolic level and intestinal malabsorption. Malabsorption also plays a role in the development of osteopenia and premature osteoporosis. Decreased food intake is common in response to a sense of fullness from a barrel chest and flattened diaphragms compounded by a fear of vomiting with excessive, paradoxical coughs and traditional postural drainage with percussion for airway clearance.
In 2007, the mean survival according to the CF Foundation Registry was 37.4 years.16 In more than 90% of patients with CF, the reason for mortality is respiratory failure.25 Impending death is signaled by hypercarbia, hypoxemia, and acute exacerbations refractory to treatment.25
Table 17-1 provides a summary of the clinical presentation of a patient with CF.23,25–28
TABLE 17-1 Clinical Signs and Symptoms of Cystic Fibrosis
Case Study Patient/Client Diagnostic Classification
A key component of Pattern 6C of the cardiovascular and pulmonary preferred practice patterns in the Guide is airway clearance dysfunction.29 The inability to mobilize pulmonary secretions from the tracheobronchial tree and/or expectorate them may result in decreased oxygen transport. This may, in turn, impair muscle performance and aerobic endurance. Patients/clients in this practice pattern are characterized by symptoms of shortness of breath, decline in pulmonary function, and decreased ability to perform activity.
This practice pattern includes patients with acute as well as chronic pulmonary disorders, oxygen dependency, and those patients who have undergone bone marrow and solid organ transplants, tracheostomy, and other cardiothoracic surgeries.29 Patients who fall under these categories but have additional considerations may need to be managed through a different pattern or a combination of this pattern with another. Practice Pattern 6G covers patients aged 4 months or less, whereas older patients who may require mechanical ventilation are addressed in Practice Patterns 6E and 6F.29 Patients with severe impairments or multiple complicating factors are not necessarily excluded from Pattern 6C; however, the frequency of visits and duration of care may require modification.
A patient with CF has been chosen to illustrate cardiopulmonary preferred Practice Pattern 6C: Impaired Ventilation, Respiration/Gas Exchange, and Aerobic Capacity/Endurance Associated with Airway Clearance Dysfunction.29CF is included in the list of ICD-9-CM codes typically related to this pattern. It should be noted that CF falls under other cardiopulmonary practice patterns as well, namely, Patterns 6E and 6F.29
Description of the Case
Julia is a 35-year-old white female presently hospitalized with an acute exacerbation of CF. She was diagnosed with CF by a sweat test at 2 years of age when she failed to gain weight, even though she was eating a diet more than adequate for growth. This condition of “failure to thrive” is a clinical symptom of the gastrointestinal (GI) system, which should lead to further testing for CF.30 Her younger brother has also been diagnosed with CF.
History of Present Illness
The patient is admitted with an increased weight loss and decreased exercise tolerance. Julia weighs 110 lb and is 5 ft 6 in. tall; her body mass index (BMI) is calculated at 17.8 (below normal). The patient uses 2 L of supplemental O2 continuously and nasal intermittent positive-pressure ventilation (NIPPV) at night. Nocturnal NIPPV delivers positive-pressure ventilation through a tight-fitting mask (see Fig. 17-3). It is used to avoid endotracheal intubation and may prevent oxygen desaturation during sleep.31
FIGURE 17-3 Nasal intermittent positive pressure ventilation (NIPPV) mask. (Reprinted with permission from Hans Rudolph, Inc., Kansas City, MO; 2009.)
Past Medical History
Julia was treated solely as an outpatient in the hospital’s cystic fibrosis clinic; first in the pediatric clinic and after age 18 she was transitioned to the adult clinic. At the age of 24 years, when repeated lung infections from retained pulmonary secretions no longer responded to oral antibiotics, she was admitted to the hospital for intravenous (IV) antibiotics, which are able to be delivered in a more potent form. The following year, she had three hospital admissions and since that time has had an average of seven hospital admissions every year. Because of the severity of her disease, Julia was placed on the lung transplant list 1 year ago, at age 34. Her medical history includes sinusitis, otitis media, decreased bone mineral density (BMD), bronchospasm, pancreatic insufficiency, and hemoptysis.
Table 17-2 demonstrates the decline in Julia’s lung function over time; there are 3 years between each of the test results.
TABLE 17-2 Julia’s Pulmonary Function Testsa
Past Surgical History
At age 26, Julia had an indwelling venous catheter placed in the right anterior chest for ease of administering multiple courses of IV antibiotics. It is difficult to maintain patency in a peripheral IV line in patients with CF; peripherally inserted central catheters are also used for antibiotic treatments. A feeding gastric tube (G-tube) was placed, at age 30, to enhance weight gain with night-time feedings.
Social History
Julia earned a college degree and worked as a special education teacher until 2 years ago when she was placed on disability. She has no history of tobacco or alcohol abuse. Julia, who describes herself as very independent, lives alone and finds it increasingly difficult to carry out her treatment regime and activities of daily living (ADL) on her own.
Treatment History
Julia performs airway clearance (ie, techniques to mobilize and expectorate pulmonary secretions) three times a day, uses an exercise bicycle at home three times a week, inhales nebulized medication twice a day, uses a metered dose inhaler (MDI) for additional medications, takes pancreatic enzymes with food, and receives antibiotics by mouth and through her indwelling catheter.
Hospital Course
Julia demonstrates increased sputum production and decreased lung function upon admission. Her requirement for supplemental oxygen has also been increased—she currently requires 4 L of O2 to maintain her oxygen saturation at 92% or greater. Julia reports pain with coughing, as a result of GI and musculoskeletal symptoms, and often has discomfort associated with treatment (eg, chest percussion, drawing arterial blood gases [ABGs]). Sputum cultures were performed to tailor the administration of antibiotics to cover the microorganisms present.
PT Examination
Julia’s decline in pulmonary function tests (PFTs) on admission suggested the need for more aggressive airway clearance, which was addressed by increasing her airway clearance regimen from that performed at home. Julia was tachycardic at rest because of numerous factors including respiratory distress, deconditioning, and side effects of a prescribed bronchodilator. This increased HR affected her response to activity by limiting her ability for endurance exercise. Julia’s exercise regimen was modified to consist of intermittent activity, which was well-tolerated. Additionally, the initial PT examination demonstrated a desaturation in Julia’s oxygen levels with increased activity. This prompted a request for physician orders to allow titration of supplemental oxygen as needed during periods of activity.
Julia demonstrates impairments in pulmonary function, exercise tolerance, secretion clearance, posture, and muscle strength. The results of specific PT tests and measures will be described later. Julia’s case demonstrates several components of Practice Pattern 6C, specifically an obstructive pulmonary disease with recurring pulmonary infections and chronic oxygen dependency. Julia falls within the appropriate age for this pattern, does not have respiratory failure, and does not require continuous mechanical ventilation, any of which might require classification in a different practice pattern or combination with another pattern.
EXAMINATION
Patient/Client History
The patient examination begins with a review of the patient’s chart. This review is not meant to be a summary of the patient’s entire medical history, but rather a distillation of information pertinent to the PT treatment including a review of medications. Medications may affect the response of the patient to a PT intervention, and conversely, an intervention by a physical therapist may require an adjustment of the medication regimen. The physical therapist should also be aware of the results of laboratory and medical tests that have been performed. Medical studies illuminate a patient’s condition, giving clues to a patient’s course of illness and indicate how an intervention by the physical therapist may need to be modified. Tracking Julia’s PFT results informs the therapist of the possible need for a more aggressive regimen of airway clearance or a decrease in exercise intensity when the pulmonary flow rates show consistent decline.
Laboratory tests and medical studies document the progression of a patients’ disease and identify appropriate interventions. These investigations include sputum culture, imaging studies, ABGs, and PFTs. Culture sensitivities are performed on sputum samples of patients with CF to treat the microorganisms present. BMD is measured in adults with CF, as it has been demonstrated that this population is at increased risk of osteoporosis.23,32 Another routine medical test is chest radiography. A typical chest X-ray of a patient with CF demonstrates hyperinflation and flat diaphragms, usually accompanied by a kyphotic spine. X-ray changes initially occur in the upper lobes, especially on the right side for reasons unknown, and include increased interstitial markings in a cystic bronchiectatic pattern and atelectasis (see Fig. 17-4).33 Bronchoscopy is performed by introducing a lighted bronchoscope down the airway, usually starting at the nasal cavity and steering into first one bronchus and then the other to visually inspect the upper branches of the tracheobronchial tree. Secretions can be suctioned and bronchial washings may be performed and sent for culture.
FIGURE 17-4 Chest radiograph of (A) a patient with healthy normal lungs and (B) a patient with emphysema, similar in pathophysiology to cystic fibrosis. Note the hyperinflation, flattened diaphragm, and narrow mediastinum. (C) Chest radiograph of a young patient with cystic fibrosis. (A) and (B) (Used with permission from West JB. Pulmonary Pathophysiology: The Essentials. 5th ed. Lippincott Williams & Wilkins; 1998.) (C) (Reprinted with permission from Michelle S. Howenstine, MD; Indiana University School of Medicine.)
ABGs identify levels of oxygen and carbon dioxide in the blood. This will identify hypoxemia, which results in a decrease in oxygen available to be delivered to the tissues or a problem with retention of carbon dioxide. A blood sample is taken from an arterial site, usually the radial artery, and sent to the laboratory for analysis. If the patient requires close monitoring while in the hospital, an arterial line is placed for increased ease in obtaining repeated samples. Physical therapists, not having access to real time ABG values during treatment, use a pulse oximeter to determine the arterial oxygen saturation (SpO2) level in the blood noninvasively. The ABG value for the partial pressure of arterial oxygen (PaO2) should be at least 60 mmHg (corresponding to 90% oxygen saturation per pulse oximetry) without a substantial increase in the partial pressure of carbon dioxide (PaCO2).34 On admission, Julia’s ABG results showed a PaO2 of 55 mmHg on 2 L O2 and a PaCO2 of 45 mmHg. Pulse oximetry revealed an SpO2 of 86% at rest. These results indicated the need to increase Julia’s level of supplemental oxygen.
PFTs provide information about the severity of pulmonary disease and treatment needed. PFTs assess flow rates and volume levels, diffusion, and airway compliance. These tests can assist with the diagnosis of restrictive or obstructive components of lung disease; patients with CF exhibit an obstructive pattern.23 PFTs are also used to document disease progression, since it has been shown that the forced expiratory volume in 1 second (FEV1) in patients with CF is correlated with survival.16,33 Normal test value predictions of lung function are based on a patient’s sex, height, race, and age.
A patient with CF is likely to be exposed to a variety of antibiotics throughout life to combat the persistent infections that are common to the disease.33 Although the choice of antibiotic is dominated by culture sensitivity, the growing resistance of microorganisms to antibiotics often causes the choice to be influenced by the experience of the clinician.35 In Julia’s case, she was treated with azithromycin as an outpatient for pulmonary infections.16 When her disease progressed and IV antibiotics were required, Julia was prescribed gentamicin or levofloxacin. The infusion was often initiated in the hospital and continued through her indwelling catheter at home after discharge. If the IV antibiotic was prescribed during a clinic visit, the necessary dosages were delivered to her home and a home health nurse initiated the therapy. Inhaled antibiotics, such as tobramycin (Tobi), are prescribed for a number of patients with CF,16,23 more often for pulmonary exacerbations than prophylactically.36
Other categories of pulmonary medications with which patients with CF become all too familiar include bronchodilators, corticosteroids, and nebulized hypertonic saline.13,16,36 There are different mechanisms and methods of delivering medication to dilate the airways; often more than one bronchodilator is prescribed. In Julia’s case, she uses salmeterol in a nebulizer and levalbuterol in a metered dose inhaler. Corticosteroids and cromolyn are often used in patients with CF who have a component of bronchospasm or asthma.36 Julia uses fluticasone, an inhaled corticosteroid (which has significantly fewer side effects than steroids delivered orally) to manage her bronchospasm. Hypertonic saline is used as a mucus therapy in CF.13 This agent is used on a regular basis by more than one-third of patients over 6 years of age.16 Dornase alfa is prescribed for patients with CF with the aim of thinning pulmonary secretions. Recombinant human deoxyribonuclease (DNase), marketed as Pulmozyme, actively digests DNA in the sputum to reduce the viscosity of pulmonary secretions.13,35 Julia’s use of DNase has resulted in a slight improvement in pulmonary function. See Chapter 8.
Oxygen should be included in the medication category as it requires a physician’s prescription for administration. In the early stages of lung disease, supplemental oxygen is usually not required. It often becomes necessary in more advanced stages of pulmonary dysfunction and precipitates significant lifestyle change.23,35 Supplemental oxygen is prescribed initially with sleep and increased activity and later may become necessary as a continuous therapy. NIPPV is used in patients with severe disease when increased levels of carbon dioxide become symptomatic.31 Julia’s case is representative of this pattern of supplemental oxygen use, starting with low levels of supplemental oxygen only for sleep and exercise sessions and progressing to continuous use of oxygen during the day and NIPPV for sleep.
Systems Review
The majority of the patient examination for this practice pattern focuses on the cardiovascular and pulmonary systems with attention to the musculoskeletal system, but a brief examination of the other systems should be included. The integumentary system gives clues to the patient’s state of hydration and nutrition. Julia’s skin is dry, suggesting dehydration, and she is pale in color upon admission. The integrity of her skin is interrupted by the indwelling catheter that is accessed in her right anterior chest. The neuromuscular assessment gives cues about locomotion and balance, especially with a change in position. No neuromuscular deficits were noted, as Julia presented no difficulty with any self-care tasks. Julia is well-coordinated in her efforts to move about her hospital room.
Another aspect of the systems review includes addressing the patient’s communication skills and learning preferences. Julia is oriented × 4 and has no difficulty communicating with the medical team overseeing her care or the other health professionals with whom she comes into contact. Her sense of humor and ease in conversation with medical personnel reflect her extensive experience with hospitalizations in the past. Julia prefers to hear all of the information available to health care professionals about her course of treatment and wishes to be intimately involved with decisions about her care. Because of the chronic nature of her disease, Julia is often more informed about treatment options and their consequences than is some of the less experienced medical staff.
Physical Therapy Tests and Measures
After the patient history has been taken, the next step is to gather additional information from tests and measures to further describe the patient’s status and to suggest appropriate interventions. Tests and measures that would be especially applicable to a patient with CF are described in the following section.
Aerobic Capacity and Endurance
Exercise capacity is reduced in this patient population so monitoring the response to activity is important.23,27,37 This is done by measuring vital signs and response of the patient at rest and repeating the same measures during activity and again during recovery. Some of the measurements to be recorded include heart rate (HR), blood pressure (BP), respiratory rate (RR), rating of perceived exertion (RPE) (be sure to document which scale is being used, 6–20 vs 1–10), dyspnea, and pulse oximetry. These measures should be recorded during an exercise test or the performance of functional activities.
A test commonly used for patients with CF is the 6-minute walk (6MW) test in which a patient is asked to cover as much ground as possible by walking in 6 minutes while the distance is recorded.23,27,37 The 6MW test is more accurate in patients with moderate to severe lung disease and is well-accepted at this stage because it is self-paced, unlike treadmill protocols. In patients with milder disease, a step test, shuttle test, or graded exercise test on a treadmill or bicycle would be appropriate.
CLINICAL CORRELATE
Evaluation of the exercise capacity of this patient population is vital because it is known that patients with CF who are physically fit survive longer than those who are less fit and that aerobic capacity is a marker for disease severity.38,39
Anthropometric Characteristics
The Guide to Physical Therapist Practice includes the measurement of body composition and edema in this practice pattern.29 Monitoring the anthropometric components of height and weight of patients with CF is an important part of the PT management in this population. Patients with CF have decreased digestion and absorption of nutrients in their GI tract; therefore, they often have difficulty maintaining or gaining weight.30 The energy expended in the performance of physical activity must be balanced with the intake of calories. A simple screening tool to document anthropometric status in patients with CF is the BMI, but skinfold thickness measurements and midarm circumference are also used.30 Referring the patient to a dietitian may be beneficial for recommendations for a higher calorie diet to offset the energy expended with a recommended program of physical activity.
Circulation
The patient’s circulation should be noted by observing the fingertips and lips for signs of cyanosis. Capillary refill can be assessed at the nail beds while observing the extent of finger clubbing present, a trait frequently possessed by patients with CF. Further discussion about cardiovascular symptoms and the response to activity is included under the section “Ventilation and Respiration.”
Muscle Performance
Strength, power, and endurance of the musculoskeletal system are crucial to the accomplishment of daily activities, work or school performance, and exercise or physical activity. Range of motion, muscle strength, and endurance should be measured and tracked over time. It is known that patients with CF exhibit a decrease in maximal muscle force even in the absence of diminished pulmonary status.40 Assessment and recommendation for a muscle strengthening program for patients with CF are therefore important. The primary and accessory muscles of respiration are forced to work overtime to compensate for the diseased state of the lungs.41 These muscles warrant special examination for strength and endurance.
Another group of muscles that require specific attention are the muscles of the pelvic floor. Many patients with CF and COPD experience urinary stress incontinence during periods of increased abdominal pressure such as during coughing, laughing, and exercise.27,28 A good rapport should be established with the patient before approaching this topic. It may be advisable to refer the patient to a physical therapist experienced in women’s health issues to fully address this aspect of muscle strength.
Pain
The chronic nature of CF and its effect on many bodily systems predisposes patients to many years of treatment. Unfortunately, pain is frequently associated with the systems affected and the treatment involved. CF-related joint pain is frequently reported.27 Chronic coughing and physical activity also contribute to musculoskeletal pain, and GI obstruction is often responsible for abdominal pain.23 Pain associated with interventions includes ABG testing, placement of a G-tube or indwelling catheter, and even percussion and shaking for airway clearance. Pain should be inquired about regularly and can be reported during rest, activity, and procedures using a simple 1 to 10 pain scale.
Posture
The optimal alignment or musculoskeletal balance of good posture is altered in many patients with CF (Fig. 17-5). This is in part due to the forward position of the head and shoulders adopted during repeated bouts of coughing and periods of respiratory distress. The length–tension relationship of the respiratory muscles is altered, necessitating attention to the examination of posture and chest mobility.27,41
FIGURE 17-5 Typical posture of a patient with cystic fibrosis. Note the barrel chest, hypertrophied neck (accessory) muscles, and the forward head.
Self-care and Home Management
The daily routine of a patient with CF can be complicated and time-consuming. Daily treatments may include self-administering airway clearance and medications several times a day, regular exercise, and getting adequate sustenance in the face of decreased absorption of nutrients. Managing equipment needed for treatments such as the delivery of supplemental oxygen requires attention as well. In addition, the frequency of airway clearance and antibiotic treatments increase with the severity of the disease so that the most compromised patients often find themselves saddled with the most exhausting treatments. Examination of the patient’s ability to carry out a prescribed routine is important in recommending assistance for the patient to accomplish necessary tasks of disease management and self-care.
Ventilation and Respiration/Gas Exchange
This area is the crux of this practice pattern and will consume the greatest amount of examination time. This portion of the examination should include observation, auscultation, and examination of the components of the patient as well as the ventilatory response to activity.
The initial assessment should include observation of the patient’s thoracoabdominal movements and breathing pattern; work of breathing; and use of the accessory muscles of ventilation, both at rest and in response to activity. The level and position of the diaphragms should also be assessed.
Auscultation of the lungs should be rendered before and after the patient performs airway clearance maneuvers to ascertain the effectiveness of these methods. In patients with moderate-to-severe CF, crackles are the norm, with the upper lobes being the most affected. Wheezing is indicative of bronchospasm, and its presence will necessitate the modification of an airway clearance regimen that prevents airway collapse. An additional component of the assessment involves listening to the patient’s phonation, specifically, the strength of the voice and the ability to finish a complete sentence without breathlessness.
Palpation of the chest wall and assessment of chest mobility should be performed to identify any limitations in air exchange afforded by the mechanics of the chest wall. A simple measurement of chest wall excursion with a tape measure can be performed before and after a maximal inhalation. An additional assessment involves the performance of the ventilatory muscles. The primary and accessory muscles of ventilation are forced to work overtime to compensate for the diseased state of the lungs and warrant an examination of strength, muscle length, and endurance.
The techniques for airway clearance used by the patient should be thoroughly assessed. Many patients with CF are well-versed in a variety of methods and interchange them depending on the need for portability, the onset of an acute infection, or the stage of their disease.42,43 In addition to the mobilization of the secretions from the airways, it is important to observe the effectiveness of their expectoration. The strength of the huff or cough effort is integral to the efficacy of secretion clearance. Many patients with CF exhibit severe and frequent coughing fits, which are extremely energy consuming and require an inordinate amount of recovery time. These patients may benefit from recommendations to stem the coughing impulse by breathing through the nose or taking sips of water and to use the coughing effort more effectively. Lastly, the sputum should be examined and quantified. If the sputum has increased in amount or thickness, or hemoptysis has occurred, it may be necessary to alter the performance of airway clearance techniques. As was discussed in the test of aerobic capacity and endurance, the patient’s response at rest and with activity should be monitored. The HR, BP, RR, and SpO2 are all indicative of the extent that the patient’s ventilatory capacity affects the performance of activity. Subjective measures of RPE and dyspnea are important to consider as well, as these can be signals of limitations to the patient’s activity irrespective of the vital sign values. Ventilatory muscle strength and endurance can be assessed by PFT results or use of a peak flow meter or handheld spirometer in the clinic. Response to activity is also a suitable measure of ventilatory muscle function.
Work, Community, and Leisure Integration or Reintegration
Before recent advances in CF treatment became available, many patients did not live long enough to make plans for employment or a career and advanced education was not emphasized. However, now that patients with CF survive into adulthood, integrating medical treatment into the patient’s lifestyle to support continued employment and education is of greater importance. The tests of aerobic capacity will provide insight into the ability of the patient to carry out daily activities in these areas. Recommendations for modification of school or work schedules and daily activities are based on this information. In addition, modifications to the living environment or design of tasks related to community activities may be necessary.
APPLICATION OF THE GUIDE
Aerobic Capacity and Endurance
Julia’s consistent use of her exercise bicycle was spurred on by the realization that exercise training on a regular basis allowed her to perform her ADL, treatment regimen, and work tasks more effectively. This regular activity enabled her to readily identify a decrease in her exercise tolerance. The 6MW is an appropriate test of endurance in Julia’s disease state, and this test was performed on a regular basis to monitor this.
Julia’s following 6MW results for consecutive years demonstrate a decline in exercise tolerance (Table 17-3).
TABLE 17-3 Julia’s 6-Minute Walk Resultsa
Anthropometric Characteristics
In Julia’s case, one of her symptoms on admission was weight loss, with a BMI of 17.8, well below normal. The issue of shoring up Julia’s nutritional status needs to be addressed before her energy output is further increased with an exercise program. This is especially important at the time of an acute exacerbation.
Circulation
Julia was noted to have severe clubbing of the fingers and toes. Cyanosis was only present in the fingertips during occasional bouts of sustained coughing.
Posture
Julia’s posture is typical of a patient with moderate-to-severe lung disease: forward head, rounded shoulders, shortened pectoral muscles, overstretched posterior thoracic muscles, and kyphotic spinal position. A postural exercise program using resistive tubing was prescribed the extensors, and cues to correct posture were provided.
Self-Care and Home Management
Julia’s regimen at home consists of airway clearance three times during the day interspersed with nebulized, oral, and IV medications; pancreatic enzymes taken with high-calorie meals and snacks; maintaining oxygen equipment, regular exercise, and use of NIPPV and G-tube feedings each night. This routine would be taxing on even the most organized mind and well-tuned body. As Julia’s disease has progressed, the topic of home assistance is regularly discussed and consultations with a social worker have been included in hospital visits.
Ventilation and Respiration
Examination of Julia revealed that, although accessory ventilatory muscle use was not marked at rest, she did exhibit extensive use of these muscles during exercise, leaning her forearms on the stationary bicycle handlebars to make optimal use of them. On auscultation of Julia’s lungs, she was noted to have expiratory wheezes and crackles throughout both lung fields, most pronounced in the right upper lobe.
On admission, Julia had an increase in sputum production in recent days and that her sputum was streaked with blood, though without frank hemoptysis. Julia used autogenic drainage (AD) for airway clearance in the past, but this method became too energy consuming as her disease progressed, and she currently uses a combination of positive expiratory pressure (PEP) with inhaled albuterol and a manual percussor, which she uses in modified postural drainage positions. When admitted to the hospital, she prefers to continue her usual regimen, but this is increased to four times daily.
The following table is an excerpt of Julia’s response to exercise sessions over time as her disease progresses (Table 17-4).
TABLE 17-4 Samples of Julia’s Exercise Sessions as Her Disease Progresses
Work, Community, and Leisure Integration or Reintegration
As was mentioned earlier, Julia had completed a college degree and secured a professional position before her CF progressed to the point of requiring her to resign from her position as a special education teacher. Julia benefits from discussing her career accomplishments and how she enjoys using her precious free time to read. Discussion of energy conservation techniques is helpful to Julia as her disease progresses.
INTERVENTION
Multiple PT interventions may be used to improve ventilation, respiration, and aerobic capacity associated with airway clearance dysfunction. This section addresses patient and family education, therapeutic exercise, breathing strategies, and secretion clearance. Each of the subsections describes various techniques, which will promote achievement of anticipated goals and expected outcomes, and specific interventions to meet those goals will be proposed. Coordination of care and communication to the patient, family, and care team as well as documentation are also reviewed throughout the section. Goals for the patient include the following: to enhance airway clearance, decrease energy expenditure, enhance physical tasks related to community and work integration with decreased pulmonary symptoms, improve functional capacity, promote independence in ADL, reduce risks of secondary impairments, promote safety to oneself and caregiver, promote self-management of symptoms, and improve sense of well-being. Establishing goals, documenting progress, and achieving measurable outcomes are the foundations of intervention.
Coordination, Communication, and Documentation
All the interventions addressed in the following sections require interaction with the patient and should include the family or support system. Basic understanding of the underlying disease pathology and physiology of why and how the interventions may affect the impairments will assist the patient in learning and adhering to the interventions. Allowing the patient and support personnel to participate in goal- setting and development of the plan of care reinforces that this is an individualized plan and the input of the patient is vital to its success.
Patient-/Client-Related Instruction
Education of the patient should be done according to need. Any impairment that would inhibit the ability to learn should be assessed. Visual and hearing impairments need to be incorporated in the delivery of the education, whether verbal, written, or via demonstration. Assessment of understanding should also be included once the education has been completed by having the patient repeat the information, demonstrate the technique, list or say precautions, and practice the technique until no verbal or tactile cues are required. The availability of return visits may determine how much material should be taught during the initial session. Videotaping the interventions is helpful for reinforcement once the patient leaves the clinic or hospital setting. The language in all handouts should be simple and straightforward to ensure comprehension by patients with all levels of literacy, and a phone number should be included with any written material. Having figures and drawings in addition to the written information and using a large font for easier readability are also important as is limiting the information on any one page.
Use simple steps to provide a straightforward and thorough approach to the intervention. For example, if an airway clearance technique is being taught, assess the patient by auscultation and clinical presentation. For Julia, a 35-year-old woman living alone with end-stage CF, it is important to choose a technique that is effective for the patient, nonfatiguing, can be performed independently, and allows freedom of performance anytime during the day. Julia, like many patients with CF, would benefit from a home exercise program using a safe method of exercise to improve posture, promote increased endurance, and improve airway clearance. In the clinic, the therapist should assess desaturation with the same exercise intervention being used at home and give exact parameters for HR, rating of perceived exertion, and frequency, duration, and intensity of the exercise. Instruct in proper use of medication to enhance bronchodilation and improve oxygen delivery and make sure the patient is aware of the need to modify or stop exercise if symptoms or clinical signs worsen. Each detail of this plan must be reviewed with the patient to make sure that there is a clear understanding of the parameters for hemodynamic response and benefits of the exercise. These steps will promote adherence and safety. Finally, include when the goals and intervention will be reexamined and what discharge outcomes will be.
Procedural Interventions
Therapeutic Exercise
Aerobic capacity/endurance conditioning— Aerobic or endurance exercise has multiple benefits. It enhances physical function and health status, improves physiologic response to increased oxygen demand, and may shorten recovery from infection.3,15,17 Aerobic exercise is characterized by the use of large muscle groups, activated in a rhythmic fashion over time. Walking, bicycling, swimming, dancing, stair climbing, and jogging are examples of aerobic exercises.
Many systems of the body will be affected by aerobic training: cardiovascular, pulmonary, immune, metabolic, musculoskeletal, and neurologic.15,44,45 The heart and lungs become more efficient at gas exchange and oxygen delivery to the tissues, the muscles become stronger and more effective at utilizing oxygen delivered to the tissues, there is a reduction in bone loss during weight-bearing exercises, skill and coordination are enhanced, and there is a sense of well-being. Metabolically, the processing of free fatty acids and glucose is enhanced along with utilization of insulin, the immune system is positively affected, motility of the GI system is improved, and in some cases, recovery from infections is enhanced.45
Julia’s exercise prescription (Box 17-2) includes frequency of the aerobic exercise, intensity duration, and mode. In addition, strength training and flexibility exercises are included. The exercise prescription should also address precautions for implementing the exercise program. The prescription is developed once examination, systems review, and tests and measures are completed (see Chapter 3).
BOX 17-2
Julia’s Exercise Prescription
Aerobic Intensity
70%–85% heart rate range from peak 6MW
Resting heart rate plus 10 (low level)
RPE scale 11–15/20 or 3–5/10 (see Box 14-2)
0–1 ventilatory scale (number of breaths to count to 15 in an 8-second period; highest level 4)
Resting respiratory rate plus 4–10 (depending on severity of illness)
Aerobic mode
Stationary cycle (home program)
Walking in the house or outside on level surface (could be at a mall)
Treadmill (when admitted to hospital)
Aerobic duration
Goal of 20–30 continuous minutes of exercise in target intensity range
Interval training for initial program: build up to 2–4 intervals of 5–10 min/d; progress to continuous level when time >10 min/interval
Aerobic frequency
If continuous program, 4–5 times per week
If unable to do continuous program, 6–7 times per week
With severe disease, 2–3 times per week
Flexibility and posture
Warm up—5 minutes of low-level cycling without resistance
Cool down—5 minutes of low-level cycling without resistance
ROM exercises: pick three exercises and rotate each session (can also be part of warm-up and/or cool-down; exercises to be combined with controlled breathing to incorporate chest wall mobility and relaxed breathing pattern)
Cervical extension (turtle necks) 3 × 5 sets
Bilateral shoulder flexion with elbows extended 3 × 5 sets
Standing against a wall: heels about 1 ft from wall, buttocks, head, and shoulders against wall, feet shoulder-width apart: elbows at 90 degrees and shoulders at 90 degrees for scapular retraction 3 × 5 sets
Knees bent slightly (stand clear of any objects), feet are shoulder-width apart; hold a towel or a dowel stick in hands with elbows extended; raise arms to shoulder height; slowly rotate arms with towel or dowel horizontal; hold count at each end of the rotation for at least 5–10 seconds; 3 × 5 sets
Calf stretches: stand on a step holding the rail; balls of the feet on the edge of the step; slowly lower 1 heel down and hold for 5–10 seconds; 2 × 2 sets on each leg
Hamstring stretch: stand or sit for comfort; back as straight as possible; place a bath towel around the ball of the foot; other leg should be bent; leg with towel should be fully extended; lean forward until pull is felt behind knee; do not hyperextend knee; 2 × 2 sets on each leg
Quadriceps stretch: hold onto couch back, chair, railing; keep your thighs parallel and stand as straight as possible; cradle your leg with a towel; now stand on one leg while raising the cradled leg to at least 90 degrees or more of knee flexion; keep your trunk as straight as possible so the pull is in the upper part of the thigh lifted by the towel; 2 × 2 sets on each leg
Strength Training
General instructions: do every other day or rotate muscle group (pick at least one exercise from each area of the body and rotate that exercise each time you do strength training; exercises to be combined with controlled breathing to incorporate chest wall mobility and relaxed breathing pattern)
Bicep curls (seated or standing) 3 × 10 with 1–5 lb weight
Triceps extension (seated or standing) 3 × 10 with 1–5 lb weight
Abduction (seated or standing; singles or bilateral depending on breathing) 3 × 10 with 1–5 lb weight
Shoulder flexion (seated or standing; singles or bilateral depending on breathing) 3 × 10 with 1–5 lb weight
Shoulder extension (seated or standing; singles or bilateral depending on breathing) 3 × 10 with 1–5 lb weight
Abdominal curls (supine, knees bent, and feet flat on floor): (1) progress from pelvic tilts to single leg lift to 4 in. holding pelvic tilt and then adding 1–3 lb leg weight as tolerated); (2) practice pelvic tilt and progress to abdominal curl with maintaining pelvic tilt; (3) progress to obliques/rotation when above levels tolerated with maintaining pelvic tilt (3 × 12 sets with each level of progression)
Standing lunges: start without weights and progress up to 5 lb weights after 15 on each leg without balance loss and maintaining upright trunk extension and appropriate pelvic tilt
Hip abduction/adduction (standing/supine/or sidelying): begin with 3 × 10 on each leg and maintain pelvic tilt and trunk extension for alignment; progress to add 1–5 lb weights for each leg
Hip extension (standing or prone): avoid any rotation and keep knee extended; lift the leg up approximately 4 in. off the surface-hold for 5 seconds; progress from 0 to 5 lb; 3 × 10 sets
Hip extension (standing or prone): avoid any rotation and keep knee flexed to 90 degrees; lift the leg up approximately 4 in off the surface-hold for 5 seconds; progress from 0 to 5 lb; 3 × 10 sets
Precautions
Evaluation prior to starting an exercise program (oxygen saturation at rest and with activity)
Use bronchodilator prior to exercise to prevent bronchospasm
Avoid strenuous exercise with an exacerbation/fever/hemoptysis/untreated pneumothorax
Precautions for osteopenia/osteoporosis
Proper hydration and calorie intake
Avoid poorly controlled climate conditions (hot, humid, freezing, high levels of pollution, windy, etc)
Watch for signs of decompensation: color, breathing pattern, increase in dyspnea, etc.
Watch for sudden chest pain or any type of pain (musculoskeletal)
When indwelling venous catheter in place, avoid resistance exercise with affected upper extremity
Exercise with a buddy if possible; carry identification
Evaluate best time for airway clearance and timing of exercise
Be aware of any declines in activity level; keep a diary of exercise program/progression
Reevaluate exercise program in a timely manner (3–6 months depending on the level of illness and frequency of return visits to clinic)
Nixon and colleagues found that higher aerobic fitness levels in patients with CF were related to improved survival.38 In other words, the more fit and active a patient’s lifestyle, obtained through regular aerobic exercise, the better the chance of living longer. Julia should stay as active as possible while awaiting her lung transplant for two primary reasons: her capacity to tolerate the surgery will improve and her recovery following lung transplantation may be shortened.
Flexibility exercises—Flexibility is another important component of an exercise prescription. A commonly used phrase among physical therapists is “the position of comfort is a position of muscle shortening.” Flexibility is key in preventing injury, enhancing chest wall expansion, and promoting healthy postures. Most patients with CF similar in age to Julia present with distinct shortening of the anterior chest muscles, overstretched posterior upper thoracic muscles, and shortening of the hip and knee flexors. Weakness in the lower abdominal region and pelvic floor is not uncommon as is weakness and loss of range of motion in the cervical and thoracic spine. Usually the lumbar spine assumes a flexed position.
Stretching of target areas is vital for ease of chest wall movement. Proximal muscles of the upper and lower extremities as well as trunk musculature play a role in inhibition of chest wall movement if muscle shortening has occurred from poor posture, weak muscles, and muscle imbalances.46 It is important to stabilize the proximal segments to assess shortening of muscles, which cross two joints and influence movement of the chest wall. Instruction in corrective postures and integration of breathing strategies with flexibility exercises reinforce and encourage proper control of airflow while limiting discomfort and bronchospasm with exercise.
Strength, power, and endurance training for head and neck, limb, pelvic floor, trunk, and ventilatory muscles27,28,47,–53—Strength Training is also an important component of a therapeutic exercise intervention. Strength training will improve muscle tone and BMD, and in some cases, pulmonary function.15,46 Low weight and higher repetitions should be incorporated into a normal exercise routine. The exercises can be performed with dumbbells, soup cans, or resistance equipment. Breathing exercises should be utilized to decrease dyspnea and fear or anxiety that may occur with participation in this type of exercise. Exhalation on the “work” or “lift” part of the exercise will encourage good airflow, lessen the chance of a Valsalva maneuver, and improve strength and coordination of extremities and respiratory muscles. In addition, teaching patients to “brace” the pelvic floor by tightening the muscles before a lifting maneuver may prevent further stress on these muscles. Every other day, strength training in addition to rotation of targeted body segments is recommended to decrease the chance of musculoskeletal injury.
Relaxation—Breathing and movement strategies are important components of relaxation and energy conservation for patients with pulmonary disease. Although not intuitively included in the realm of relaxation, energy conservation techniques are probably best incorporated in this section, which includes breathing and movement strategies. Instruction in conservation techniques to promote a decrease in energy expenditure during daily activities will give the patient a sense of independence, decrease the fear of shortness of breath, and promote an increase in activity.
First pick simple activities like walking on level ground, getting out of a chair, or putting on a shirt before the patient/client is progressed to more energy-consuming and fearful activities like tying one’s shoes, climbing stairs, or carrying objects while ascending stairs. Planning out the day or even just an hour at a time will help lessen the fear and anxiety of rushing to do a task. If the patient knows he or she will be spending the majority of the day on the first floor of the house and the bronchodilators are kept on the second floor by the bed, a mental note or an actual list should be made of what the patient needs to bring before he or she moves down to the first floor. Planning ahead for bathroom needs is also important. Rushing up a flight of stairs to get to the bathroom is a task that makes many patients feel short of breath and anxious. If the patient is on a diuretic, steps should be taken to plan when the medication is taken, limit the distance to the bathroom after the medication is taken, and eliminate obstacles on the way to the bathroom, all of which may decrease the energy cost of getting to the bathroom. A final area for improving the ease of ADL should be the process of showering. One suggestion is to bring a chair into the shower or have a chair just outside the shower so the patient/client can be sitting during the shower or immediately after the shower. In addition, if a terry-cloth robe is donned after a shower, the energy-consuming task of drying the patient’s backside is eliminated. Pursed-lip breathing, planning ahead, and decreasing anxiety and fear are keys to conservation techniques and give the patient/client a sense of accomplishment. Relaxation techniques are very important for energy conservation, and are described elsewhere. They can be incorporated into daily activities or airway clearance techniques to decrease bronchospasm, control paroxysmal coughing, decrease metabolic demand or energy expenditure, enhance airflow, and promote a sense of well-being. The exercises or techniques can be performed in any position or location.
Functional training in self-care and home management—An evaluation of the home environment should include a review of daily activities. For example, on which level are bathrooms and bedrooms located, how many stairs are there in order to enter the house, are there handrails accompanying the stairs, where are the laundry facilities located, how accessible is the kitchen for demands on breathing, is there a gas stove versus electric (Julia uses oxygen), and how will she get her groceries? Julia presently functions independently at home, but she is finding it more difficult to carry out her day-to-day treatment regime and ADL. Education on ways to conserve energy (work of breathing), reduction of cough during ADL, and devices that may assist her to continue to live independently at home should be incorporated into the treatment (see sections on Breathing Strategies).
Functional training in work (job/school/play), community, and leisure integration or reintegration—Julia is no longer working and is on disability due to her illness. She does not have any hobbies besides reading and primarily focuses on her daily maintenance treatments. This topic should be examined in order to prepare Julia for discharge from the hospital. Julia may choose to visit with family and friends either at her home or away from her home. Evaluation of situations with which Julia may be presented during these times should be addressed by careful planning and assessment. A mockup of situations may be practiced in the hospital setting prior to discharge in order to evaluate hemodynamic responses and other challenges.
Once she receives the lung transplant, her functional training will have to be reassessed to see if she will be able to return to employment as a teacher. This evaluation should include examining the risk of infection, injury prevention and reduction, and safety awareness training during work, community, and leisure. Musculoskeletal components include assessment of osteoporotic changes, decreased activity, and falls from muscle weakness. Additional factors to consider are the ease of bruising, pain, and the physical side effects of the posttransplant medication including self-perception in the workplace as well as self-confidence related to these side effects. Finally, the evaluation should address the level of endurance related to work demands, hemodynamic challenges to the work area (stairs, inclines, uneven surfaces as on a playground), and other environmental considerations (smog, pollen, dust).
Manual therapy techniques—Techniques for mobilization of the rib cage, thorax, pelvic and shoulder girdle have been described to enhance ventilation and improve respiration. A working clinician can easily document the relationship between improvements in range of motion of the shoulder girdle, anterior chest wall, neck and upper thorax with improvements in relaxation, breathing pattern, perception of work of breathing (RPE), RR, level of anxiety, and in some cases, oxygen saturation. Applying the principles of proprioceptive neuromuscular techniques to the chest wall (musculoskeletal pump) can enhance relaxation, stimulate enhanced tidal volume or inspiratory capacity, and, as a result, may improve mucus mobilization and clearance.
Massage can be used to decrease muscle tension, anxiety, and work of breathing and enhance comfort. Massage of the upper posterior thorax and neck area may be beneficial following paradoxical coughing, vomiting initiated by coughing, or positioning, and to decrease musculoskeletal pain related to coughing and poor posture. Ventilation may be enhanced by utilizing massage in conjunction with manual techniques in order to provide relaxation of shortened accessory muscles.
Prescription application and, as appropriate, fabrication of devices and equipment—In the case of most individuals with CF, assistive or orthotic type devices are not indicated unless there is another superimposed pathology that would affect neuromuscular control. In the case of Julia, she had an indwelling venous catheter placed at age 26. If Julia and her care team decide that high-frequency chest wall oscillation (HFCWO) is the best form of airway clearance for her, fabrication of a device to offer relief around the catheter site may be necessary for comfort and safety reasons. The vest should be fit to Julia, and then the catheter area can be measured to have an appropriately sized padded device fabricated to prevent discomfort. This same type of device may also be utilized around gastric or jejunal tubes and chest tubes.54–56
Airway clearance techniques—When prescribing airway clearance techniques, many factors should be considered including the severity of disease, the patient’s lifestyle, and factors affecting adherence (Table 17-5). Goals for airway clearance should be measurable and time oriented. Measuring sputum production, monitoring changes in color or viscosity, and measuring hemodynamic changes (SpO2, HR, RR, and BP) allow appropriate goals to be set. Specific examples of goals included in this section are improvements in breath sounds, PFTs, chest radiography, subjective measurements, and treatment adherence. These goals may be obtained by performing the airway clearance interventions outlined in Box 17-3 and described in the following section.
TABLE 17-5 Decision Making for Airway Clearance Techniques
BOX 17-3
Airway Clearance Techniques
Breathing strategies
Active cycle of breathing
Assisted cough/huff
Autogenic drainage
Paced breathing
Pursed-lips breathing
Techniques to maximize ventilation
Manual/mechanical techniques
Assistive Devices (positive expiratory pressure (PEP), including oscillatory PEP, high-frequency chest wall oscillation, intrapulmonary percussive ventilation)
Chest percussion, vibration, shaking
Chest wall manipulation
Suctioning
Ventilatory aids
Positioning
To alter the work of breathing
To maximize ventilation and perfusion
Pulmonary postural drainage
Exercise
Aerobic or endurance exercise
Breathing strategies: active cycle of breathing or forced expiratory technique10,43,57–59—The forced expiratory technique is based on optimal airflow and avoidance of a cough to prevent premature airway collapse to improve secretion mobilization and airway clearance. The technique can be done in any position. Quiet, tidal volume breathing is performed by the patient prior to a mid-to-large inhalation initiated from the lower rib cage. Then the glottis remains open and the air is then “huffed” out. The sound should be very breathy and the mouth should be in a shape of an “O.” There should not be a high-pitched wheezing sound, as this would indicate too forceful of a maneuver, which would promote airway narrowing. The technique is easy to learn, can be performed independently, and can be taught to youngsters by using games that employ bubbles, cotton balls, handheld mirrors, and ping pong balls. The individual is taught to use huffs to loosen and then clear audible secretions until the huff sounds dry.
The active cycle of breathing technique (ACBT) combines the forced expiratory technique, bronchial drainage, and manual techniques. This technique is easy to learn, easy to teach, and can also be performed by the patient independently. The individual assumes a bronchial drainage position and focuses on a quiet breathing pattern using the lower rib cage area without upper chest movement. This is followed by a large inspiration again initiated in the region of the lower rib cage, a breath-hold for 3 to 4 seconds, and finally a sigh out through an open mouth. The theory of the inspiratory hold allows for air to equalize from an “open alveoli” to a “clogged one” to assist with secretion clearance from the blocked alveoli, thereby increasing the efficiency and effectiveness of the technique. This cycle can be repeated as dictated by the patient and then followed by one to two huffs to clear the secretions. The full cycle can then be repeated (see Fig. 17-6B). A caregiver or the patient may assist with manual techniques during expiration (such as vibration or shaking), but this is not necessary; rather, it is indicated if the patient feels it is beneficial.
FIGURE 17-6 Autogenic drainage (AD) (A) versus active cycle of breathing (ACB) (B), both from spirograms of normal individuals. AD: phase 1 = peripheral loosening of mucus; phase 2 = collection of mucus in large airways; phase 3 = transport of mucus to the mouth. ACB: BC = breathing control, FET = forced expiration technique. (Republished with permission of Lippincott Williams & Wilkins, from Savci S, Ince DI, Arikan H. A comparison of autogenic drainage and the active cycle of breathing in patients with chronic obstructive pulmonary disorders. J Cardiopulm Rehabil. 2000;20(1); permission conveyed through Copyright Clearance Center, Inc.)
Breathing strategies: assisted cough/huff techniques5,10,14,43,57,59—The huff or forced expiratory technique was explained previously. The assisted cough can be employed independently or with the help of an assistant. The technique can be as simple as placing a pillow over an incision to help splint the area or as vigorous as using a manual technique at the time of the cough. Massery describes four types of manual assistance: costophrenic (hand placement), abdominal thrust, anterior chest compression, and a counter-rotation assist.14 Refer to Chapter 20 and the CD-ROM for explanations and demonstrations of these maneuvers. Pain, fullness of gastric contents, mental status, innervation, and expertise of the instructor or caregiver are a few factors to consider when determining whether an assisted cough is appropriate. After a surgical procedure, the simple act of coughing may be limited because of pain, which will inhibit a large inhalation and a forceful exhalation. Assisting a patient with splinting of the incision, along with assuring that adequate pain medication is provided, may improve the pain tolerance.
In Julia’s case, she has pain with coughing from GI and musculoskeletal symptoms. During forced expiratory techniques, huffing, or controlled coughing, she may use hand placement to brace her lower rib cage, a towel wrapped around her lower rib cage, or a pillow to brace against the abdominal area and lower rib cage to lessen the complaint of pain and assist with a more effective cough. If the pain subsides with the bracing, Julia could be instructed to press inward on the lower rib cage and upward on the abdominal area to improve her cough.
The cough is best performed using a flexed posture. Another way of performing an assisted cough is to instruct the patient to assume certain postures to encourage flexion, such as sitting forward while in bed. Julia could be instructed that sidelying with hips and knees flexed may be an advantageous position to enhance her cough. Also while in bed, her head could be elevated on pillows (or by raising the head of the hospital bed) to increase flexion; Julia may already have her trunk and hips flexed. The disadvantage of elevating the head of the bed to place the patient in the flexed position is that the volume of air inhaled during the initial phase of the cough may be limited. If in a standing position, the patient could be taught to bend forcefully at the waist during the cough to assist with the movement of air. One disadvantage of this position is safety; if the patient is unstable or has near syncopal episodes with coughs, there is an opportunity for injury by bumping or falling against objects.14
Breathing strategies: autogenic drainage10,43,57,59–61—AD is an airway clearance technique that can be used independent of assistance. This is a challenging technique to learn, requires a great amount of concentration, should only be instructed by experienced clinicians, and initially may be time consuming to use. However, these disadvantages are offset by the great freedom the technique offers to patients with pulmonary disease.
The technique can be done in sitting position and, once learned, can be performed nearly anywhere. Coughing is suppressed initially, and only lower chest wall movement is encouraged. Because the technique utilizes some of the same theories of active cycle of breathing (equalization of air across alveoli for mobilization of secretions), the bronchioles and alveoli should be fully developed to get the full benefit. This physiological consideration, plus the great level of concentration and patience required, makes this technique less suitable for patients younger than 12 years.
The patient is instructed to breathe out through an “o”-shaped mouth (or the nose) while learning the technique. The patient should be taught to listen during inhalation and exhalation for noises indicative of secretions such as high-pitched wheezes, gurgling, or popping sounds. The timing and pitch of these sounds give cues to where the secretions may be located. If the sounds are heard initially on inhalation and are lower in pitch, most likely the secretions are in the larger, upper airways. These airways must be cleared with huffs or coughs prior to continuation of the technique. If these larger airways are not cleared, the patient will experience frustration from trying to continuously suppress the urge to cough. The patient should practice quiet breaths, using only the lower rib cage. A mirror is a good teaching tool to make sure the upper chest remains still during the technique.
Once the patient is comfortable with using only the lower rib cage, he or she is instructed to exhale down into expiratory reserve volume. This should be “sighing” out rather than a forceful exhalation. Once expiratory reserve volume is reached, the individual should inhale a “tidal volume” breath at this level. If the patient feels light-headed or dizzy at any time, he or she can resume a regular breathing pattern until the feeling subsides. The sounds described here should occur following multiple cycles at this low lung volume (a tidal volume breath just into expiratory reserve volume). Once the sounds are heard close to mid-exhalation, the patient then inhales to a slightly larger volume to move closer to a volume of breath where normal tidal volume would be performed. Again, if the patient feels light-headed or dizzy, he or she should resume normal tidal volume breaths or a couple of larger breaths until these symptoms pass. The patient is instructed to resume a “midlevel” of breathing and not to move to a higher level until the popping, wheezing, and gurgle sounds are heard midway through the exhalation phase. Once the sounds occur at this point in the breathing cycle, the patient can take a much deeper breath to reach the highest part of the pattern. Again, once the highest level of breathing is reached, only the amount of air in a tidal volume is used. If symptoms are experienced anytime during this phase of the cycle, instruct the patient to take a regular or larger breath until the symptoms pass and then resume the cycle where he or she left off. Once mobilized, the secretions are cleared through huffing or coughing.
The keys to this technique are airflow and volume control, suppression of cough until secretions are mobilized, inspiratory hold at the end of inhalation to equalize air across alveoli, and most importantly, patience. Because of the immense amount of concentration and the requirement of using audible and tactile cues, this technique is not appropriate for all patients with excessive production of sputum. See Figure 17-6 for a schematic of AD compared to active cycle of breathing.
Breathing strategies: techniques to maximize ventilation, pursed-lip breathing, paced breathing4,15,62,63—Although this category of breathing strategies is placed under the heading of Airway Clearance in the Guide, these techniques are also useful in other situations when secretion removal is not the primary goal. Many of these techniques may be incorporated into daily activities or exercise routines. This section also includes techniques useful for promotion of energy conservation or relaxation.
Techniques to maximize ventilation: The terms diaphragmatic breathing or lower rib cage breathing are both used to describe strategies to expand the lower chest in place of upper chest expansion. In order to teach lower rib cage breathing, the client should be in a comfortable position. The preferred position is one that enhances the movement of the diaphragm against gravity (side-lying or semifowlers). A tactile cue of a hand or a tissue box over the lower rib cage will help visualize how the lower rib cage should move on inhalation and exhalation. On inhalation, the hand on the lower rib cage or tissue box should rise, indicating air filling the lungs. When done correctly, the upper chest will have little movement because there should not be large volumes of air moved during a relaxation technique.
Stacking breaths is a useful technique to maximize ventilation when the volume of air a patient/client can inhale is limited. This may be due to a neuromuscular insult, postsurgical pain, trapped air, weak muscles, or large inspiratory airflow leading to bronchospasm. Breath stacking is accomplished by taking a small-to-moderate size breath and adding it to two or three additional breaths to increase inspiratory volume, thereby decreasing atelectasis, moving air behind the secretions, and increasing inspiratory volume to enhance a huff or cough. The patient is instructed to take in siplike volumes of air on top of one another without exhaling. After three to four breaths, an inspiratory hold should be done for 1 to 2 seconds followed by a huff or a controlled cough. It may be helpful for the patient/client to see a demonstration and use a mirror for visual cues. Any symptoms of dizziness or light-headedness are indications to stop the technique. The inhalation and inspiratory hold phases of breath stacking can be incorporated with many other airway clearance techniques such as the forced expiratory technique, AD, active cycle of breathing, PEP, oscillating positive pressure, and during bronchial drainage and manual techniques.
Segmental breathing combines manual cues and breathing control to improve ventilation to specific areas of the chest wall. If during evaluation of chest wall movement asymmetry is identified, this could coincide with the underlying pathology of pneumonia, an area with pleuritic chest wall pain or an area with poor air movement from retained secretions. Placing a hand on that area and coordinating chest wall movement with downward hand movement will enhance expansion in this area. Facilitation or inhibition of a segment can be controlled with proper timing, hand placement, and verbal cues for breathing coordination. Utilization of the principles of proprioceptive neuromuscular techniques will allow the therapist to increase chest wall movement, stimulate a productive cough in some cases, and improve overall ventilation and chest wall symmetry.
Combining pursed-lip breathing during exhalation with diaphragmatic breathing should enhance relaxation and promote a better overall breathing pattern with less accessory muscle use. Pursed-lip breathing is accomplished by breathing in through the nose to a count of “1, 2” and out via pursed lips to a count of “1, 2, 3, 4.” This will prolong the expiratory phase, slow the RR, and delay small airway closure. It will also decrease dyspnea, improve airflow, and calm anxiety. Instruct the patient to sit in front of a mirror or use a handheld mirror for feedback. Repeat the previous sequence of taking a breath in through the nose and exhaling via the lips in a whistle-ready position. If the patient or client is very anxious, it is not as important how the breath is taken in, as how the air is exhaled through the pursed lips. If the patient or client has end-stage lung disease and the diaphragms are flattened from air trapping, diaphragmatic breathing may not be as beneficial as pursed-lip breathing.
Pursed-lip breathing and diaphragmatic breathing should be incorporated into functional activities like walking. This strategy is referred to as paced breathing. The patient is instructed to take a breath in through the nose and walk two steps to a count of “1, 2.” The patient then exhales to a count of “1, 2, 3, 4” as they walk the next 4 steps. The inspiration-to-expiration ratio is 1:2, thus prolonging the expiratory phase and delaying small airway closure. Once the patient is able to use these strategies on level surfaces, they can advance to stair climbing. Instruct the patient to use a “step-to” strategy (ie, one foot meets the other on the same step), and avoid “step-over-step” (ie, one foot moves past the other to the next step above). Also make sure that his or her foot is placed fully on the step and not on the edge before going up to the next step. A handrail may allow the patient to use accessory muscles as needed. A handrail may also lessen the fear of falling, thereby reducing the anxiety that accompanies fear. Fear of falling promotes anxiety, which leads to shortness of breath and poor airflow.
Expiratory exercises that prolong the expiratory phase can be used as measurable outcomes as well as interventions. Instructing the patient/client to read a phrase, sentence, or paragraph aloud promotes expiratory control. The number of words stated during exhalation can be measured by the patient/client for feedback and demonstration of progress. This same technique can incorporate singing for expiratory airflow control. The patient can place a hand on the abdominal area to palpate abdominal muscle activation during the technique. This exercise will promote endurance training of the expiratory muscles and can be used during ADL in combination with conservation techniques.
Manual/mechanical techniques: assistive devices5,8,10,31,42,44,57,60,61,64–74—PEP, oscillatory PEP (Flutter or Acapella), HFCWO, intrapulmonary percussive ventilation (IPPV), and the Frequencer are all mechanical devices used for airway clearance.
PEP can be produced using pursed lips, a mouthpiece, a mask, or various devices. For the purpose of airway clearance, only the devices used for PEP will be discussed in this section. The technique(s) are easy to learn, can be performed in the sitting position, done independently, taught to children, are very portable, and have been shown to be an effective method of airway clearance. There are many devices available in the market at a wide variety of prices. The devices used should include an exhalation port, a release-type valve, and be used for only a single individual. Ideally, they should also have a port for either oxygen or nebulized medication to be delivered during the technique. Pressures between 10 and 20 cmH2O are optimal to build up back pressure and promote equalization of pressure across alveoli for mobilization of secretions. The back pressure is also theorized to promote airway stabilization to increase time for secretion mobilization.
The individual is instructed to sit upright with his or her elbows on a table, to exhale just greater than a normal tidal breath, and inhale into the mask or mouthpiece of the device. A manometer is placed in-line to measure pressure levels and give visual cues. A resistor is placed in the exhalation port to promote positive pressure in the range of 10 to 20 cmH2O. When initially teaching an individual how to use the device, a larger resistor is used with progressively smaller ones put in place until the desired pressure range is reached. The manometer acts as biofeedback until the individual has learned the sequence. If a mouthpiece is used instead of a mask, a nose clip should be used to prevent air leakage. As with other devices used for airway clearance, secretions are expectorated by huffing or coughing.
Oscillating positive pressure is an alternative to a stable level of pressure in PEP devices. These pocket-sized devices (Flutter or Acapella) provide the benefits of a positive-pressure device plus the “interruptions” from the oscillations that promote changes in the viscosity of the secretions and enhance expiratory airflow. This category of PEP devices has been shown to be an effective mode of airway clearance. Pneumothoraces, claustrophobia, recent facial or nasal surgery, or injury are precautions that should be considered when weighing the benefits against the risks of using positive-pressure devices for airway clearance.
HFCWO (the Vest Airway Clearance System or the SmartVest System) is an individually sized chest wall jacket powered by a generator, which promotes secretion clearance of the entire lung fields while performed in the seated position (Fig. 17-7). HFCWO offers the advantage of independence and has been shown to be an effective airway clearance method. The device has been used in the home, acute and long-term care settings, and intensive care settings. The principal theory of the vest is that at various pressures and oscillations, airflow is enhanced and viscosity of secretions is altered, which promotes ease of secretion mobilization and clearance. The device requires an electrical source, comes with a prefitted vest, a compressor unit, a hand or foot pedal, and tubes to connect the vest to the compressor. Three frequency ranges are used to enhance secretion movement: 5 to 10 cycles/s, 10 to 15 cycles/s, and 15 to 20 cycles/s.
FIGURE 17-7 The Vest airway clearance system. (Courtesy of Advanced Respiratory, Inc, St Paul, MN.)
The individual is instructed to start at the lower settings to allow loosening of secretions from the periphery and then progress to the higher settings to move the secretions toward the upper airways for expectoration. More than 10 minutes should be spent at each level to promote the most efficient and effective airway clearance. A nebulizer can be used along with the vest. A properly fitted vest is imperative for comfort and to limit side effects such as nausea, abdominal discomfort, chest wall discomfort, and complaints of urinary urgency. If an indwelling venous catheter is in place, padding can be placed around the site to limit discomfort. These devices are expensive but are covered by most insurance companies.
The IPPV device works in a similar way to HFCWO. The device is based on theories of airflow and oscillation but is delivered internally via a mouthpiece compared to HFCWO, which is external. Cost and comfort are two key areas of patient concern, as a feeling of claustrophobia and chest fullness may be experienced during the application of the device. The changes in frequency and pressure delivered internally assist with airway stabilization, thereby decreasing secretion viscosity and enhancing secretion mobilization.
The Frequencer is a newly developed device for airway clearance. The electroacoustical device, which has recently received FDA approval in the United States, applies vibrations at a rate of 20 to 120 Hz to the chest wall to assist with the mobilization of mucus. A preliminary study demonstrated that the Frequencer was equal to conventional postural drainage and percussion in 22 patients with CF when used between 25 and 40 Hz. Although safety and efficacy of the Frequencer have been demonstrated, its use is not yet widespread enough to determine acceptance by the CF population.
Manual/mechanical techniques: chest percussion, vibration, and shaking8–10,12,42,43,57,60,72,75—Manual techniques have traditionally been used to enhance bronchial drainage. These techniques include percussion, vibration, and shaking, which are often collectively referred to as traditional chest PT. Treatments with these techniques can be performed with an assistant, independently via self-percussion/vibration, or with mechanical devices. The mechanical devices may be difficult to hold in the correct position to get the similar effect of manually delivered percussion or vibration. The mechanical devices can be expensive if not covered by insurance and could be unreliable at producing the correct rate and pressure required for optimal airway clearance. On the other hand, performing the technique manually can be fatiguing for the person performing the technique as well as for the person receiving the percussion, vibration, or shaking. Correct hand posture and position are needed to prevent injury to the performer and receiver of the percussion. The caregiver may be at risk for a repetitive-type injury at the wrist, elbow, or shoulder, and the individual receiving the technique may be at risk for bruising, soreness, and fractures of the ribs if too much pressure is applied and the patient does not communicate to the caregiver their tolerance to treatment.
The cupped-hand position is used for percussion to transmit energy through the chest wall to loosen thick secretions. The technique may be done concurrently with a drainage position to enhance secretion mobilization. Once the secretions are loosened with percussion, the techniques of vibration and shaking help to mobilize the secretions from the periphery and move them toward the trachea for expectoration and evaluation of the secretions. Vibration and shaking are done in a rhythmic pattern. The caregiver’s shoulders should be positioned directly over the hands. As the patient exhales, a downward motion is made by the caregiver in a vibrating motion while maintaining full contact of the hands on the chest wall. Shaking results in an exaggeration of vibration and appears more like a plunging motion.
For each of the manual techniques, hand placement should avoid bony prominences such as the scapula, spinous processes, and clavicles. Ribs and breast tissue may be very sensitive and special care should be given in those areas. The patient receiving the manual techniques should be allowed to rest after three to four cycles. The caregiver should watch for fatigue and signs of decompensation: increased RR, reports of shortness of breath, a change in coloration or mental status, and a change from baseline breathing pattern. An individual may use self-percussion and vibration independent of a caregiver for certain drainage positions, but these exclude any of the posterior regions. Box 17-4 lists precautions for percussion and vibration.
Positioning for airway clearance5,8,10,42,43,57,60–62,75—Bronchial drainage or postural drainage has been utilized for treating pulmonary congestion for decades. The primary principle of the technique is to utilize the shape and direction of the lung segments and to place the individual in gravity-enhancing postures or positions that drain the uppermost segment of the lung once in that position. Ten positions are used to drain all the segments of the lung (see Fig. 17-8). It may not be necessary to use all 10 positions. The treatment should be based on the PT examination to focus on the areas most in need in addition to being tolerated by the patient. Elevated BP, anxiety, esophageal reflux, and decompensation of the cardiopulmonary system are precautions, which should always be observed if this technique is utilized. Bronchial drainage can be done independently and modified to reduce the aforementioned precautions; however, performing all 10 positions can be very time consuming. Precautions for postural drainage should be observed (Box 17-4).
FIGURE 17-8 Postural drainage positions. Position 1 = patient leans back 30 degrees; position 2 = patient leans forward 30 degrees; position 3 = patient flatlying; positions 4 and 5 = patient with head down 15 degrees, rotated one-quarter turn backward; position 6 = patient with head down 30 degrees, sidelying; position 7 = patient with head down 30 degrees, prone; position 8 and 9 = patient with head down 30 degrees, rotated one-quarter turn forward; position 10 = patient prone with bed flat. (Reprinted with permission from the Cystic Fibrosis Foundation. An Introduction to Chest Physical Therapy. Bethesda, MD; 1997.)
BOX 17-4
Precautions for Postural Drainage and Manual Techniques
Precautions for Bronchial Drainage and Manual Techniques5,42,44
Esophageal reflux
Hemoptysis
Dyspnea
Orthopnea
Bruising/rib fractures/flail chest
Coagulopathy
Cardiac arrhythmias
Desaturation/hemodynamic decompensation
Large pleural effusion
Bronchospasm
Spinal instability
Recent burn graphs
Osteopenia/osteoporosis
Requires assistance
Pain
Level of alertness
Risk for injury to caregiver and/or recipient
Nausea and vomiting
Untreated pneumothorax
Increased intracranial pressure
Recent surgery
Appropriate light, loose clothing
Indwelling venous catheter
Feeding tubes (jejunal, gastric)
Timing of tube feeds prior to treatment
Mechanical percussor/vibrators need electrical source, rate may be inconsistent
Exercise for airway clearance15,17,43,60,75—Although not listed in the Guide under Airway Clearance Techniques, exercise may be used to enhance clearance of secretions. Exercise promotes improvement in ventilation, airflow, air volume, chest wall movement, secretion movement, and functional capacity. Exercise has not been advocated as an independent method of airway clearance for patients with chronic pulmonary conditions and should be performed in conjunction with other techniques. Exercise to enhance airway clearance should involve large muscle groups, thereby promoting an increase in tidal volume and airflow and be done regularly for a training effect. Exercise should be age appropriate and enjoyable for the patient. Oxygen desaturation and hemodynamic decompensation should be a key concern when initiating any type of an exercise program with a patient with pulmonary disease and excessive secretion production. Monitoring pulse oximetry is a must for patient safety. Precaution for musculoskeletal injury should also be included in the plan. Substituting one of the multiday airway clearance treatments with exercise will encourage independence and enjoyment and promote another component of health.
THRESHOLD BEHAVIORS
Threshold behaviors have been defined as measurable behaviors at the pathology, impairment, functional, disability, or quality of life level that triggers an intervention.76 Identifying threshold behaviors in patients with CF may provide insight into which of these patients would benefit from the specific intervention techniques described. Many of the tests previously described measure levels of impairment and function in patients with CF. The following tables will identify numerous threshold behaviors for patients with CF and match them with appropriate interventions based on the level of impairment or function that has been identified (Tables 17-6 to 17-9).
TABLE 17-6 Threshold Behaviors: Impaired Ventilation and Respiration/Gas Exchange
TABLE 17-7 Threshold Behaviors: Airway Clearance Dysfunction
TABLE 17-8 Threshold Behaviors: Aerobic Capacity/Endurance
TABLE 17-9 Threshold Behaviors: Posture, Muscle Performance
INTERNATIONAL CLASSIFICATION OF FUNCTIONING, DISABILITY, AND HEALTH MODEL (ICF MODEL)
In the first edition of this chapter, the Nagi Disablement Model was used to describe the relationships among disease pathology, impairments, functional limitations, and disability. Included in the model were the risk factors for a particular pathology, factors that are specific to an individual patient’s health status, and extraneous factors that impact a patient’s treatment and lifestyle.77 The components of the more recent ICF, a common language for function, disability, and health model78 have been applied to the pathology of CF79 using Julia’s case to demonstrate a potential path to identify impairments, limitation, and disability (Table 17-10).
TABLE 17-10 The International Classification of Function, Disability and Health (ICF)78–82
The ICF model “attempts to provide a coherent biopsychosocial view of health states from a biological, personal, and social perspective.”79 Three key levels of human function are identified to better describe the patient’s limitations: body functions and structures and activity and participation. These levels are interconnected to the patient’s health condition and contextual factors. In this model, health condition replaces the terms for diseases, injuries, or disorders.79,80 Some examples of contextual factors would be environmental or personal factors that influence the patient’s perception of how disablement is experienced.79 Body functions and structures refer to physiological functions and anatomical parts of the body, and impairments are identified problems in those areas. Activity and activity limitations relate to tasks or actions by an individual and any difficulties encountered executing the activity.79Participation is the involvement of the individual in a specific life situation, while participation restrictions refer to problems experienced in a life situation.79,80 Some examples of subdomains used in both activity and participation, which further identify patient limitations or restrictions, are communication, self-care, domestic life, and learning and applying knowledge.79
This newer classification emphasizes the components of health versus the consequences of disease.81 From the clinician’s perspective this shift in how disability and health are examined or classified focuses the patients’ functioning and health away from merely the consequences of a disease or condition.82 The ICF model takes into consideration how the patients’ health and functioning are associated with their disease as well as the influence of personal and environmental factors. A full description and discussion of the qualifiers and scoring of the ICF is beyond the scope of this chapter. For Julia, assessing factors such as her home setting, her inability to work, her worsening CF, and her continued need for psychosocial support from the hospital staff will all play a role in her present admission for exacerbation and future recovery from lung transplantation.
Many factors affect the medical management of CF. The earlier the diagnosis is made, the more promptly the treatment can be initiated, delaying damage to the lungs from infections. Infants diagnosed through newborn screening programs appear to have improved status compared to those patients in whom treatment was initiated because of symptoms.33 Additional factors may complicate the treatment regimen. A history of pancreatic insufficiency, sinusitis, and low BMD is frequently seen in patients with CF, as are bronchospasm and gastroesophogeal reflux.32,34,35 Hemoptysis may complicate the course of CF and requires treatment with antibiotics, modification of airway clearance, or embolization if severe.34 Medical procedures such as the insertion of a G-tube for supplemental feedings to promote weight gain and the placement of an indwelling venous catheter for IV access are commonplace for patients with severe CF. In addition, the chronicity of CF predisposes patients to multiple diagnostic and surveillance procedures, including ABG testing, pulmonary function evaluation, and chest radiography.34
As previously discussed, the daily routine of a patient with CF can be taxing, especially in the case of severe disease. Multiple medications taken throughout the day, airway clearance techniques, and therapeutic exercise consume a large portion of patients’ time.26 Maintaining supplies for the use of supplemental oxygen and nocturnal breathing support further add to a patient’s responsibilities. It is not surprising that rates of adherence to treatments involved in CF care are low.35 The amount of support a patient receives from his or her social network and the interaction with the physical and social environment play important roles in disease management. Support from family, friends, and health care providers impacts significantly on the ability of the patient to successfully incorporate the recommended treatment into the daily lifestyle. Educational and work experiences normalize the patient and enable them to enjoy their lives in a role separate from the sick role that chronic disease often dictates.
Additional factors that impact a patient’s status include age, family history, attitude toward the disease, and lifestyle. Patients who have lived past the current median survival age of 37 years16 may feel they are living on “borrowed time” and adhere more strictly to treatment recommendations. Patients with genetic diseases may have grown up with parents who experienced guilt as a result of passing a disease to their children, and this may affect psychosocial interactions of family members in many different ways. A patient’s attitude toward CF and the lifestyle they lead are important factors in the management of the disease. Regular use of tobacco would have negative consequences on disease progression, as would disregarding treatment recommendations or failing to take medications as directed. On the other hand, a positive attitude paired with the ability to accomplish airway clearance, exercise, and the taking of medications regularly would have a positive impact.
The main pathway of the ICF model allows linkages to be made between the body functions and structures of CF, to activity levels and the participation. The pathology of CF begins at birth, with many infants experiencing lung infections at an earlier age than previously thought and frequently without symptoms.33 With the increasing age of survival, patients experience a high number of infections by the time they reach adulthood. Another result of CF pathology in later stages is a dependence on supplemental oxygen. The resulting impairments in activity, musculoskeletal function, and pulmonary status necessitate multiple interventions in an attempt to reduce the effect of the health condition. Although exercise has not been shown to improve pulmonary function specifically, many training effects can occur in patients with CF.34,83 These include improvements in exercise endurance and cardiorespiratory fitness and inspiratory muscle strength.83 In addition, strength training has been shown to improve skeletal muscle strength in patients with CF.83 Nutritional therapy is important to consider in conjunction with exercise, as poor nutritional status impacts on muscular ability.83 Oxygen supplementation should be used to achieve an oxygen saturation level of at least 90% in those patients who exhibit desaturation, as this will decrease the ventilatory demand and may improve exercise tolerance.34,83 Additional effects of exercise seen in patients with CF include increased sputum production, improved posture, decreased breathlessness, and positive psychological benefits.26,34,83 Adults with CF are able to maintain maximal exercise capacity even in the face of declining lung function.83 The impairments noted in Table 17-10 lead to limitations in the activities of the patient with CF. The ability to care for oneself and perform the tasks necessary for disease management is related to environmental and personal factors. Physical inability to perform routine tasks has been cited as a determinant of quality of life.83 In addition, the restrictions of the patient’s social connections through leisure and community involvement frequently lead to a patient’s withdrawal from the world outside home and health care settings.
The resulting restrictions on the patient with advanced CF are characterized by unemployment and reliance on outside sources for income and assistance with living needs. This lack of ability to participate is illustrated in the decreased independence that patients experience as their disease progresses. Decreased exercise capacity has been shown to be associated with a lower quality of life reported by patients.83 This association is demonstrated by the pathway from the body structures and functions of CF to its resulting limitations in participation.
THE LIMITS OF OUR KNOWLEDGE
Manifestations of Cystic Fibrosis
There continue to be tremendous advances in the diagnosis and treatment of CF.84 As genetic testing of CF is employed, the number of identified mutations has grown. Research continues into the correlation between genotype and clinical manifestations of lung disease.21 As more states adopt newborn screening of CF, the identification of infants with the diagnosis allows earlier treatment.19,20 Since the identification of the CF gene in 1989, research continues to play an important role in the development of new treatments available to patients with CF.21 The trials and disappointments of gene therapy demonstrate that there are still many challenges to overcome.
With longer survival also comes an increased incidence of pulmonary complications of CF such as respiratory failure and hemoptysis and an increase in lung transplantation to treat end-stage disease.23,24 Further research continues to search for optimal methods of managing this chronic disease to decrease the impact on disability and improve quality of life. Although the connection between the aforementioned structural changes and resulting functional limitations is apparent, the extent of the impact cannot be easily predicted.
Physical Therapy Interventions
The growing numbers of patients with CF surviving into adulthood depend on the expertise of the health care team to direct and support their treatment. The physical therapist plays an integral role on this team, with the ability to intervene in musculoskeletal, cardiovascular, and ventilatory impairments to improve the function of the patient. Although in the United States, we are historically more experienced in exercise interventions, we now have an increased variety of airway clearance techniques at our disposal. It has been demonstrated that many of the techniques are as effective as traditional postural drainage and percussion.10,42 What is not proven, however, are the optimal strategies for patients at different stages of lifespan and disease. In addition, it is not known if there are certain patients who may in fact do well without any formal intervention, especially in light of the fact that there are now patients with the genetic diagnosis of CF, but without clinical signs and symptoms. We have yet to demonstrate that airway clearance prolongs life if done prophylactically, starting with infants.
Likewise with exercise interventions, we are challenged to discover which exercise training regimen is the most effective or appropriate for individual patients at different stages of their disease. For example, how long should we wait (if at all), before initiating exercise in a patient admitted to the hospital with an acute exacerbation, and what is the optimal balance between endurance and strength training in a patient with limited exercise tolerance, and finally, are there patients who will decompensate with exercise to the extent that it will prove harmful in the long term?
Finally, as the number of patients with CF increases because of augmented survival and new advances come to light in the treatment of CF, we are challenged to modify our PT interventions in order to optimize the benefits of these new therapies for our patients.
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