Cardiopulmonary Rehabilitation and Cancer Rehabilitation. 2. Pulmonary Rehabilitation Review

2.1 Educational Activity: To discuss the rehabilitation of a 37-year-old woman with limb-girdle muscular dystrophy and ventilatory failure caused by community-acquired pneumonia, now with a tracheostomy 

LIMB-GIRDLE MUSCULAR DYSTROPHY may present between childhood and early middle age. Progress of the disease is variable. Severe disability, with muscular weakness and contractures, scoliosis, cardiomyopathy, and respiratory insufficiency, can occur. Polyvalent pneumococcal and influenza vaccines should be administered as soon as an infection has been controlled.

The pulmonologist should assess the patient’s readiness for ventilator weaning. Barriers include severe anxiety or fear, need for strong pain or sedative medications, cardiovascular instability, severe chronic lung disease, electrolyte and fluid abnormalities, sepsis, and malnutrition. The volume ventilator can be used to measure readiness for weaning. Weaning parameters include: (1) ability to trigger the ventilator, (2) minute ventilation less than 10L/min, (3) rapid shallow breathing test or frequency/tidal volume of less than 105 breaths per min/L, (4) dynamic compliance greater than 25cmH2O, (5) static compliance greater than 35cmH2O, (6) tidal volume greater than 280mL, (7) respiratory rate less than 25 breaths per min, and (8) negative inspiratory pressure greater than 20cmH2O. A patient who cannot trigger the ventilator is not considered for weaning. The shallow breathing test, static compliance, and negative inspiratory pressure are the measurements shown to be most important in predicting which tracheostomized patients are likely to fail weaning.1 The patient can be weaned more readily if supportive noninvasive positive pressure ventilation (NPPV) is given during the weaning process.2 Techniques for mobilizing secretions and assisting cough should also be used. The physical or respiratory therapist or nurse can mobilize secretions by airway oscillation (Flutter valvea, b) or chest wall percussion, vibration, and oscillation (the Vestc).3 These professionals can provide manually assisted cough or use the CoughAssist machined for maximal insufflation-exsufflation (MI-E).4 Glossopharyngeal breathing is an air-stacking technique in which the tongue is used as a pump. It can be taught to the patient for deep breathing and for increasing tidal volume. Removing the tracheostomy tube can reduce airway secretions and facilitate assisted secretion clearance. A tracheostomy is required only if secretions cannot be managed in any other way or in severe dysphagia. Close monitoring of the patient continues throughout this process with involvement of the speech pathologist and otorhinolaryngologist in assessing the integrity of the upper airway.

The patient should be discharged to the community with a referral to a regional muscular dystrophy clinic. The patient and caregivers at home must be taught how to mobilize the chest wall, how to appropriately use NPPV, and how to manage secretions without a tracheostomy, especially during intercurrent respiratory infections.

Because artificial ventilation will be needed at some time in the future, NPPV must be introduced.5 Negative-pressure body ventilators and the rocking bed provide a substitute for inspiratory muscles. For convenience, patients usually prefer NPPV. Air is introduced cyclically through adaptive devices attached to the nostrils, nose, or mouth or to the mouth and nose held by headgear. No headgear may be necessary if the device is inserted in the mouth with a bite plate. When the patient is awake, NPPV can be delivered with a simple angulated mouthpiece. Mouthpiece ventilation allows variation of frequency and depth of individual breaths for speech and allows for air stacking, a necessary part of an assisted cough. If the patient is seated in a semireclining position in a wheelchair, intermittent abdominal pressure ventilation may be used, mimicking the expiratory action of the abdominal muscles.

2.2 Educational Activity: To evaluate a 48-year-old marginally ambulatory, morbidly obese patient with obstructive sleep apnea treated with nocturnal bilevel positive airway pressure who presents with back pain 

Obstructive sleep apnea (OSA) is characterized by snoring, arousals, sleep fragmentation, daytime sleepiness, and diminished quality of life (QOL). Most patients with OSA are male (70%), of middle age (range, 40–60y), and are moderately obese, with an average body mass index (BMI) of 32.5±9.0kg/m2. The BMI is calculated as weight in kilograms divided by the square of the person’s height in meters. In OSA, there is increased risk for cardiovascular disease and systemic hypertension in particular. Echocardiography can help identify ventricular hypertrophy, right ventricular failure, and pulmonary hypertension. The level of bilevel positive airway pressure (BiPAP) is set for the correction of upper-airway obstruction during sleep, usually at no more than 12cmH2O. The patient may have concomitant alveolar hypoventilation requiring higher settings of NPPV. Morbidly obese patients may have obesity-related hypoventilation. Low-flow supplemental oxygen allows maintenance of acceptable oxygenation, avoiding severe hypercapnia, without the addition of added inspiratory pressure.

In patients with pain, narcotic analgesics are prescribed with caution because of depression of central respiratory drive. If BiPAP settings require adjusting to change oxygenation, adjust carbon dioxide levels, or correct worsening ventilation, an overnight sleep study is indicated.6 An oral mask for BiPAP that requires no headgear has been developed.7 Patients may prefer the oral route or use it when nasal BiPAP is not feasible. If the noninvasive approach is not effective, tracheostomy may become necessary.8

A program to lose weight requires readiness of the patient. If conservative measures fail, gastric bypass can be considered. A bariatric sports and fitness program is preferred, with an exercise physiologist providing supervision during aerobic or strength training. Treatment of heart failure and other conditions must be optimized. If the patient has residual daytime sleepiness, the stimulant modafinil can be used as an adjunct therapy. Wheelchairs should be used only in cases of compromised mobility and powered mobility used only when no other options exist. Motorized wheelchairs must be able to accommodate low-flow oxygen systems and NPPV and must support obese patients.

Obese patients are at high risk of becoming chronically ventilator dependent. The main cause for a moderately obese patient becoming dependent is a major central nervous system event. In morbid obesity, chronic dependency on a ventilator is often associated with multiorgan failure. Thirty-eight percent of patients with chronic ventilator dependency have a BMI of 30kg/m2 or more, and 12% have a BMI of 40kg/m2 or more—3 times the national prevalence.9 However, of all ventilator-dependent patients, those who are morbidly obese had the highest weaning success (76%) and lowest mortality (12%) and were more likely to go home or to an acute rehabilitation facility.

2.3 Educational Activity: To evaluate a 50-year-old cachectic woman who presents with bulbar palsy, aspiration, and respiratory insufficiency during an exacerbation of multiple sclerosis 

Multiple sclerosis (MS) has different patterns of progression that are categorized as benign, relapsing-remitting, secondary progressive, primary progressive, and progressive relapsing. It is more frequent in women. Pyramidal, cerebellar, and bulbar involvement is common. Acute respiratory failure (ARF) can occur, usually during an exacerbation in which severe weakness of all extremities is also present. Many other causes of ARF exist, including acute lung injuries (adult respiratory distress syndrome), respiratory failure associated with multisystem failure, previous lung disease, and complex medical conditions such as neuromuscular disease, heart disease, and debility after surgery. Bulbar involvement with ARF is most commonly seen in brain injury, advanced Parkinson’s disease with sepsis, amyotrophic lateral sclerosis, Guillain-Barré syndrome (GBS), myasthenia gravis, and the muscular dystrophies with pneumonia. Because of the autoimmune nature of MS, during an exacerbation, short-term medical treatment with high-dose steroids is used. The patient may already have been on long-term treatment with interferon-β. Other immune-modulating agents may be considered, including glatiramer acetate. Patients are managed in an intensive care setting, requiring intubation and mechanical ventilation. Feeding may be necessary through a nasogastric tube. With dramatic response to treatment, prompt weaning can occur. However, if weaning is delayed by more than 2 to 3 weeks, tracheostomy and percutaneous gastrostomy are indicated.10 Percutaneous dilatational tracheostomy (PDT) may be considered.11 Compared with surgical tracheostomy in the operating room, PDT is reported as safer, faster, and more cost effective. It can be performed by pulmonologists or surgeons at the bedside and has a lower rate of infection.

The rehabilitation team should be involved with the patient from the onset. Speech pathology continuously reassesses the ability to swallow and to speak, providing alternative methods of communication. The physical and occupational therapists treat the patient at bedside with the goal of rapid mobilization. Chest physical therapy is essential. Close attention should be given to passive range of motion, positioning, and splinting of the extremities. As soon as patients are weaned from ventilatory support during the day or are able to tolerate a portable ventilator, they should be transported to the therapy areas for treatment. If the patient has been completely weaned or only partial weaning can occur, the patient may be transferred to an inpatient rehabilitation service that is prepared to care for patients with ventilatory needs. Here, more comprehensive rehabilitation can be given with the goal of discharge to the community or a nursing facility.12

2.4 Educational Activity: To justify the use of oxygen support in a 68-year-old woman with severe kyphoscoliosis from osteoporosis and chronic obstructive lung disease from long-standing smoking 

Chronic obstructive pulmonary disease (COPD) is an important risk factor for osteopenia and osteoporosis. The mechanisms responsible for osteoporosis in this patient population are not known. Several risk factors may predispose patients to accelerated bone loss and osteoporosis: reduced physical activity, poor nutrition, low BMI, smoking, decreased exposure to sunlight, nutritional deficiencies, hypercatabolic effects of ongoing inflammatory processes, and corticosteroid therapy. As airflow obstruction worsens, the risk of osteoporosis increases because patients become more debilitated, less mobile, and nutritionally depleted. Although osteoporosis is asymptomatic, reduced bone mass increases the risk of vertebral, rib, and hip fractures, which can add to the morbidity of obstructive airway disease.13

Restrictive lung disease may occur as a complication of scoliotic curves from osteoporotic vertebral fractures. People with severe kyphoscoliosis can no longer effectively ventilate their lungs or clear airway secretions. They can develop atelectasis, pneumonia, and respiratory failure. Scoliosis can lead to hypercapnia and lung hypoventilation, resulting in hypoxemia. When hypoxemia is treated with supplemental oxygen, ventilatory drive of the brain can be suppressed, and hypercapnia can worsen. On the other hand, COPD may increase ventilatory drive by causing hypoxia. Patients with either ventilatory or oxygenation impairment often complain of shortness of breath with activity. On the other hand, wheelchair users with ventilation or oxygenation impairment rarely complain of shortness of breath despite severe hypercapnia and impending respiratory failure. Patients with the combination of restrictive lung disease and COPD often will eventually develop dyspnea with increased activity. Supplemental oxygen therapy for hypoxia in this type of patient must be prescribed cautiously to avoid decreasing ventilatory drive and subsequent carbon dioxide (CO2) narcosis. Noninvasive ventilation, such as BiPAP at nighttime, will normalize CO2 levels. Once CO2 levels normalize, supplemental oxygen can cautiously be given for hypoxemia as needed. Monitoring vital capacity, end-tidal CO2, oxygen saturation via pulse oximeter, and peak cough flow in the sitting and lying positions are critical indicators for the management of these patients. Using an MI-E device (CoughAssist) can help to remove thick mucus, but its use must be carefully controlled to prevent barotrauma to the lung. Besides careful supplemental oxygen and noninvasive ventilatory care, bronchodilators, corticosteroids, and antibiotics as needed are considered effective therapeutic tools.14, 15

Many older adults have low bone mineral density (BMD) and a high prevalence of other risk factors for pathologic fracture, including advanced age, poor physical function, low muscle strength, decreased cognition, poor nutrition, and use of multiple medications. Calcium is essential for preventing and treating osteoporosis. Factors contributing to low calcium intake are restriction of dairy products, low fruit and vegetable consumption, and high consumption of low-calcium beverages. For older adults, calcium intake should be maintained at 1000 to 1500mg/d. Vitamin D is required for optimal calcium absorption and thus is also important for bone health. A recommended vitamin D intake of 400 to 600IU/d has been established for adults. High dietary protein, caffeine, phosphorus, and sodium can adversely affect calcium balance, but their effects appear not to be important in people with adequate dietary calcium. Regular exercise may reduce the risk of falls in older adults. Resistance and high-impact activities contribute to development of bone mass. In addition to dietary supplements and lifestyle interventions (including regular exercise, management of multiple medications, smoking cessation, psychologic counseling, dietary changes), pharmacologic intervention can be considered. Bisphosphonates increase BMD and consistently reduce the risk of vertebral fractures by 30% to 50%. Among bisphosphonates, alendronate and risedronate reduce the risk of subsequent nonvertebral fractures in women with osteoporosis and adults with glucocorticoid-induced osteoporosis. Raloxifene hydrochloride, a selective estrogen receptor modulator, prevents osteoporosis and reduces the risks of significant hip and vertebral fracture. Tamoxifen citrate, used in the treatment and prevention of breast cancer, can maintain bone mass in postmenopausal women. Salmon calcitonin showed a positive effect on BMD at the lumbar spine. Nasal calcitonin reduced vertebral fracture risk at the 200-IU dose but not at the 100- or 400-IU dose.16

2.5 Educational Activity: To assess an 8-year-old boy with congenital heart disease who is demonstrating mild cognitive dysfunction 

Impaired growth and physical development and delayed onset of adolescence are common features of many cyanotic and, to a lesser extent, acyanotic forms of congenital heart disease (CHD).17 Skeletal maturity (ie, bone age) is delayed in cyanotic children in relation to the severity of hypoxemia. The mechanisms of growth interference in such a patient are summarized in appendix 1.17

Besides physical impairment, there are associated inherent or acquired neurodevelopmental risks for patients with CHD. There is evidence of congenital abnormalities that involve the central nervous system in infants with life-threatening CHD. Preoperative abnormalities documented from cranial ultrasonography and magnetic resonance imaging may be present in 15% to 39% of infants and children with CHD.18

Several studies have investigated the developmental outcome after repair surgery for CHD. The developmental outcome of infants after the repair surgery of dextrotransposition of the great arteries showed that the children had increased learning disabilities and lower mean intelligence quotients when compared with siblings and normative populations. Postoperative encephalopathy with choreoathetosis is well known as the most devastating of neurologic complications after cardiac surgery in young children. Long-term outcome study19 of these children showed pervasive deficits in memory, attention, language, and motor functions. Persistent dyskinesia was reported in half of them. The pervasive and enduring cognitive and motor deficits in survivors of postoperative choreoathetosis indicate a guarded prediction of good outcome. Thus, careful neurologic and cognitive evaluations are recommended for all children with choreoathetosis after cardiac surgery. Overall, children with initial severe postpump choreoathetosis had worse cognitive function at follow-up than did children with initial mild postpump choreoathetosis.19

Cardiopulmonary bypass, with or without circulatory arrest, produces additional jeopardy for heart transplant patients. Tedious surgical arch reconstruction in infants with hypoplastic left heart syndrome adds minutes to the circulatory arrest time and the profound hypothermic period. Children having heart transplant who are most at risk for poor development are those with a circulatory arrest time longer than 50 minutes. Postoperative seizures and lower psychomotor development scores have been associated with longer circulatory arrest periods during surgery in children with CHD.20 Use of total circulatory arrest to support vital organs during heart surgery in infancy is generally associated with greater functional deficits than is use of low-flow cardiopulmonary bypass; however, both strategies are associated with increased risk of neurodevelopmental vulnerabilities in academic achievement, fine motor function, visuospatial skills, working memory, hypothesis generation, sustained attention, and higher-order language skills.21

2.6 Educational Activity: To design a multidisciplinary therapy plan for a 25-year-old person who has cystic fibrosis and who develops severe respiratory disorder 

Cystic fibrosis (CF) is an autosomal recessive disorder affecting approximately 25,000 people in the United States. Gene defects on the long arm of chromosome 7 lead to deficiency or abnormal function of the CF transmembrane conductance regulator, a chloride channel that influences the salt content and viscosity of cellular secretions. Abnormally high electrolyte concentrations in sweat are diagnostic. Thick, dehydrated secretions impair mucociliary clearance in the lungs, leading to progressive obstruction, inflammation, infection, and premature death. Abnormal secretions cause varying degrees of obstructive damage to multiple other organs, particularly in the gastrointestinal, hepatobiliary, reproductive, and exocrine pancreatic systems. Although CF can affect many organ systems, respiratory failure accounts for most of mortality seen in the disease.22

With advances in infection control, life expectancy has been dramatically extended from a median survival of 20 years in 1981 to 31.3 years as of 1998. A standard treatment regimen includes antibiotic therapy for pulmonary exacerbations and chronic suppressive therapy; airway clearance; exercise; therapy with mucolytic agents; bronchodilators; and anti-inflammatory agents, supplemental oxygen, and nutritional support. Antibiotic treatment of exacerbations improves pulmonary function, exercise tolerance, and QOL. Oral antibiotics, especially directed against Hemophilus influenzae, Staphylococcus aureus, Pseudomonas aeruginosa, or a combination of organisms, can be effective in patients with mild underlying disease. The choice of antibiotics should be based on sputum culture results and the patient’s previous clinical response. Aerobic fitness is the strongest correlate with 8-year survival, making an active lifestyle important. Several studies of CF exercise programs showed results dependent on compliance and intensity of training. Exercise has increased work capacity, improved cardiorespiratory fitness, improved ventilatory muscle endurance, and enhanced immune function. Although increased exercise may not improve pulmonary function, the slowing of progressive pulmonary decline from these other factors suggests an important benefit. No study of subjects with CF has shown harm from exercise. The occasional patient who experiences exercise-related oxygen desaturation can maintain adequate oxygenation through the use of supplemental oxygen during exercise. Exercise might acutely enhance pulmonary function because of its ability to mobilize airway mucus. Adding exercise to standard chest physiotherapy resulted in a clinically important and statistically significant improvement in forced expiratory volume in 1 second (FEV1).23, 24

Assisted ventilation for respiratory failure has changed dramatically with the advent of noninvasive modes of ventilatory support and lung transplantation. Many patients will eventually become candidates for lung transplantation despite aggressive treatment. Currently, the best available predictor of survival is the FEV1. Patients with CF and an FEV1 of less than 30% of its predicted value have 50% 2-year mortality. Given that wait times for lung transplantation are approximately 2 years, according to Organ Procurement and Transplantation Network data as of July 12, 2002, an FEV1 of 30% of predicted is an often-quoted benchmark for lung transplantation referral.25

Because 85% of patients who have CF have exocrine pancreatic insufficiency, malabsorption of fat and fat-soluble vitamins (A, D, E, K) is common. They frequently have significant caloric deficits because of complications of their disease that limit both appetite and oral intake. Such complications include esophagitis, pancreatitis, extrahepatic biliary obstruction, cholangitis, constipation, unpalatable diets, and vomiting associated with paroxysms of cough. The effects of poor intake are compounded by the increased energy requirements associated with poor pulmonary function and chronic infection. To overcome the caloric deficiency, diets should provide 120% to 150% of the recommended daily allowance for calories. Vitamin supplementation, particularly of fat-soluble vitamins and occasionally vitamin B12, is necessary. However, the mainstay of nutritional treatment is replacement of pancreatic enzymes (eg, lipase, amylase, protease). In newer preparations, the enzymes are encased in pH-sensitive enteric-coated microspheres, which makes them more bioavailable. These exogenous digestive enzymes effectively correct the pancreatic insufficiency and partially reverse the malabsorptive process characteristic of CF.26

Early, aggressive, and CF-center–based care with a multidisciplinary team are all associated with better outcomes. As adults, CF patients generally have more severe pulmonary disease, a higher prevalence of diabetes mellitus and poor nutrition, and more complex financial and psychosocial issues. Therefore, a relatively higher intensity of pulmonary, respiratory therapy, and endocrine, nutritional, and psychosocial services may be needed. In addition, adult patients have unique needs, including vocational counseling, contraceptive and reproductive services, and obstetric care.25

2.7 Educational Activity: To assess a 45-year-old woman with pulmonary sarcoidosis who ambulates in a power wheelchair and who requires high flow oxygen 

Restrictive lung disease is diagnosed when the ratio of the FEV1 to the forced vital capacity (FEV1/FVC) is equal to or greater than .70 and the FVC is less than 80% of the predicted value. A prevalence of 6.6% was reported in 1 national survey.27 In the same survey it was found that restrictive lung disease is associated with an increased risk of being unable to walk a quarter of a mile, being unable to lift 4.5kg (10lb), needing help with activities of daily living, and self-reported poor or fair health.

Interstitial pneumonias and fibrosis cause intrapulmonary restrictive lung disease. Only since 2002 have the international organizations devoted to respiratory diseases been able to reach a consensus in classifying idiopathic interstitial pneumonias (IIPs) according to clinical, pathologic, and radiographic characteristics.28 There are 6 IIPs other than idiopathic pulmonary fibrosis (IPF). These are acute interstitial pneumonia, desquamative interstitial pneumonia, cryptogenic organizing pneumonia (previously termed idiopathic bronchiolitis obliterans with organizing pneumonia), nonspecific interstitial pneumonia, lymphoid interstitial pneumonia, and respiratory bronchiolitis-associated interstitial lung disease. All of these have a better prognosis than IPF.

IPF is described as a progressive disease characterized by usual interstitial pneumonia (UIP) histology. An environmental history is important because exposure to organic antigens may trigger UIP, nonspecific interstitial pneumonia, or classic hypersensitivity pneumonitis. Improvement or resolution can occur when exposure is eliminated. Familial pulmonary fibrosis exists in which a mutation in surfactant protein C has been found. Controlling fibroproliferation appears to be more important than controlling inflammation in IPF.

Sarcoidosis is another disease in which pulmonary fibrosis can be seen. At the present time, no single cause of sarcoidosis has been found. It has been suggested that the cause may be heterogeneous with different antigens producing different clinical presentations of the disease. In sarcoidosis, there is deposition of noninflammatory, nonnecrotizing epithelioid granulomas in a variety of body tissues. It is the pulmonary involvement that causes significant morbidity and mortality. Most patients do not need rehabilitation. A recent study showed that pulmonary function improved with less frequent need for rescue prednisone in stage 2 patients who were treated chronically with low-dose corticosteroids. Many patients either stabilized or improved with their use. However, some patients, as our patient, continue to progress. Methotrexate, thalidomide, and antitumor necrosis factor–alpha are other therapeutic options. With advanced disease and the development of pulmonary hypertension, there is significant fatigue, dyspnea, and exercise intolerance. Orthotopic lung transplantation can be considered when 2-year mortality without it is approximately 50%.29 Race, a mean pulmonary artery pressure of 40mmHg, and a mean flow of supplemental oxygen of 3L/min were the 3 variables found to predict mortality. African Americans had an increased risk of death compared with whites (odds ratio, 2.5). Survivors required continuous oxygen, as did patients who subsequently died.

Rehabilitation services should include psychosocial and behavioral interventions. Control of anxiety and depression can improve dyspnea. End-of-life discussions are necessary. Therapeutic recreation has many programs to enhance QOL. Today, whether living at home or in an institution, patients may have computers with Internet access for enhanced communication, education, and leisure activities. In the past 2 years motorized wheelchairs in which the motors and batteries have protective covers have become available for safe use with oxygen.

2.8 Educational Activity: To discuss the implications of compromised nutrition and limitations of respiratory reserve on both inpatient and outpatient rehabilitation programs for a 70-year-old woman with hip fracture, osteopenia, and COPD 

COPD is among the leading causes of morbidity and mortality. The mortality rate from COPD increased by 46.6% between 1979 and 1993, and COPD had an estimated direct and indirect cost in 1993 of $23.9 billion. Airflow obstruction is most common in smokers, affecting 15% to 20%; it can also be seen in 12% to 35% of women without a history of smoking.30 It is a common comorbidity in patients admitted to rehabilitation units for other reasons. Lack of recognition of the problem may lead to frustration as patients fail to achieve goals because of dyspnea. Failure to recognize the condition also may cause unnecessary morbidity, because patients do not receive appropriate management. Screening for COPD using the Global Initiative for Obstructive Lung Disease (GOLD) criteria (table 1) can help in timely recognition and initiation of oxygen therapy.31, 32, 33

Table 1. Global Initiative for Obstructive Lung Disease Criteria46

	Level of COPD	Criteria	Treatment
	Mild (stage 1)	FEV1/FVC <70% predicted FEV1 ≥80% predicted With or without symptoms	Short-acting bronchodilator
	Moderate (stage 2)	FEV1/FVC <70% predicted FEV1 between 50% and 80% predicted With or without symptoms	Regular treatment with 1 or more bronchodilators Rehabilitation Inhaled glucocorticosteroids if significant symptoms and improved FEV1
	Moderate (stage 3)	FEV1/FVC <70% predicted FEV1 between 30% and 50% predicted With or without symptoms	Regular treatment with 1 or more bronchodilators Rehabilitation Inhaled glucocorticosteroids if significant symptoms and improved FEV1 or if repeated exacerbations
	Severe (stage 4)	FEV1/FVC <70% predicted FEV1 <30% predicted Or presence of respiratory failure or right heart failure	Regular treatment with 1 or more bronchodilators Rehabilitation Inhaled glucocorticosteroids if significant symptoms and improved FEV1, or if repeated exacerbations Treatment of complications (osteoporosis, diabetes, hypertension, pulmonary hypertension) Long-term oxygen therapy Consider surgical options such as transplantation or lung-volume reduction surgery

Screening can be done at the bedside with a simple spirometry evaluating flow volume loops; adding pulse oximetry at rest and with exertion can complete the evaluation for patients at risk.34, 35 Treatment is dictated by the guidelines of the GOLD recommendations and is easy to institute.

As can be seen by the GOLD criteria, pulmonary rehabilitation is indicated for anyone with stage 2 and stage 3 disease, and screening for need for supplemental oxygen, as discussed above, would be indicated.

2.9 Clinical Activity: To plan the rehabilitation for a 20-year-old man with CF who will be having a lung transplant 

Pulmonary transplantation is the most difficult of the solid organ transplantations. Currently, most patients undergoing lung transplantation have CF, COPD, interstitial lung disease, and, less commonly, pulmonary hypertension. The survival for patients with transplants has improved, but the chronic debility from prolonged illness before transplantation must be appreciated. Rehabilitation interventions in lung transplantation must address both the pretransplantation and posttransplantation period.36, 37 Before transplantation, patients with end-stage lung disease often have had years of steroid use, with associated osteopenia and muscle loss. Additionally, nutritional status is often quite compromised, with pulmonary cachexia or pancreatic insufficiency. Chronic hypoxemia often leads to cor pulmonale.38

Pretransplantation patient management should include educational programs about what to expect in the peritransplantation and posttransplantation periods and a conditioning program. By coordinating exercise with supplemental oxygen and prostacycline, mobilization of even the sickest patients is made possible. The rehabilitation mirrors the program in COPD, with a focus on strengthening, conditioning, education, pulmonary toilet, and medication and oxygen use. Patients often report that they have significant isolation, depression, and anxiety before transplantation, and the rehabilitation and maintenance program can help improve psychologic status.

Posttransplantation rehabilitation should start in the intensive care unit on the initial postsurgical day and should progress to ambulation as soon as possible. Pulmonary toilet and chest physical therapy are essential. Some patients can benefit from either acute or subacute inpatient rehabilitation, especially patients with more severe deconditioning from poor pretransplant mobilization or with a very complex postoperative course. The rehabilitation should be done at a facility where the team is familiar with the needs of patients with transplants. The education program should include the new medication regimen and education about immunosuppression and rejection.39

After the patient returns home, a comprehensive outpatient pulmonary rehabilitation program can consolidate the patient’s education and help him achieve maximum levels of function. A maintenance program is useful to ensure compliance with exercise prescriptions.

2.10 Educational Activity: To manage a 44-year-old severely tetraparetic woman with partially paralyzed vocal cords who sleeps with an open tracheostomy at night and who is being readied for discharge from the hospital after rehabilitation for GBS 

Thirty percent of patients with acute inflammatory demyelinating polyneuropathy or GBS will require mechanical ventilation during the acute phase of the illness.40 In 30%, treatment with intravenous infusion of immunoglobulin or plasmapheresis does not stop the progression of the paralysis. Axonal involvement is common. The 16% left with some degree of permanent disability usually have had axonal involvement and have required a ventilator to survive. This patient was capable of being weaned from the ventilator. However, because her paretic vocal cords cause partial obstruction of her airway in sleep, she continues to need an open tracheostomy tube at night and will require humidification at the tube. It is best to deliver humidity by aerosol to a tracheostomy mask with a heated or cooled humidifier, but a heat and moisture exchanger can be used if there are minimal secretions. A tracheostomy bib that covers the opening of the tube can also be used.

A speech pathologist should have followed up the patient throughout her hospital course. Initially the patient may have used a specially designed tracheostomy tube that permits speech with an inflated cuff, then a fenestrated tube or a phonation valve with a cuffless tube. Capping the cuffless tracheostomy tube during the day decreases tracheal dryness, limits secretions, and makes vocalization less difficult. The tracheostomy tube is downsized for optimal speech and respiration.41 This patient did not have significant residual difficulty with swallowing and is allowed to eat regular solid food and liquids. However, during intercurrent infection or with aging, swallowing function may worsen. The diet may need to be downgraded temporarily or permanently. Thus, ongoing swallowing assessments are necessary.

When the patient is discharged home, adaptations to the home must be made to accommodate a motorized wheelchair. A mechanical lifter for transfers is necessary. Caregivers must be taught care for all systems of the body, particularly the tracheostomy site and tube.11 It is necessary for the state in which the patient resides to recognize through legislation that caregivers other than licensed nurses are capable of being taught the proper techniques for daily tracheostomy and ventilator care. Home care services that include a primary care physician and a home care nurse who makes regular visits are preferred.

Comprehensive care must be provided at a center with multiple specialties, including otolaryngology, pulmonology, and rehabilitation medicine. With loss of 1% to 2% vital capacity per year, the patient’s vital capacity may fall to a range of 25% to 50% of that predicted, at which time she may require mechanical ventilation again. This is more likely to occur during an intercurrent respiratory infection. The patient and caregivers must learn assisted coughing techniques with air stacking and manual thrust on the lower chest wall or epigastrium.4 They must also learn MI-E with a cough-assist machine that can be used at the tracheostomy. Her family must be trained to use an oximeter to monitor for secretion buildup during respiratory infections and to use it as a way to gauge whether she must go to the hospital to rule out pneumonia, receive intravenous antibiotics, or have other more intensive care.4

2.11 Educational Activity: To discuss the consequences of exercise-induced asthma in a 16-year-old basketball player with a knee injury 

Exercise-induced asthma (EIA) is a temporary narrowing of the airways associated with strenuous exercise or activity. EIA may begin during exercise (typically within 15min) or after exercise has ended. It usually resolves within 20 to 30 minutes of onset. Poorly controlled asthma is commonly misdiagnosed as EIA. Developing symptoms shortly after beginning exercise is more characteristic of poorly controlled asthma. Poorly controlled asthma, then, makes it impossible to complete any significant amount of physical activity. The symptom of nighttime cough also is indicative of chronic asthma. Occasionally, a person who has EIA will experience symptoms at night, usually 4 to 6 hours after completing exercise, which is a late-phase response. This is a response to an influx of inflammatory cells in the airway. Similar events occur in these patients when exposed to an allergen. Symptoms of EIA may include coughing, chest tightness, wheezing, and difficulty breathing. Some patients describe feeling winded, tired, or dizzy after exercise, and some may complain of stomach pain. EIA occurs in 80% to 90% of people who have asthma and in almost 50% of people who have allergic rhinitis (ie, hay fever). Studies have shown that about 5% to 10% of collegiate athletes have EIA, which is similar to the incidence in the general population. Children are more likely to have EIA than adults. They may notice that they have difficulty keeping up with friends when running and playing. However, EIA in children frequently is overlooked by parents, health care professionals, teachers, and even the children. Unfortunately, many children and adolescents who have experienced respiratory difficulties during activity learn to avoid physical exertion that produces these symptoms. These children should have a trial of interventions that effectively prevent or treat EIA. Avoidance of exercise is problematic for young people because it can have a major negative impact on their psychosocial development.42

The administration of an inhaled corticosteroid before exercise is useful to inhibit the late-phase inflammatory response. Inhaled or oral corticosteroids are not effective as a pretreatment for EIA. Cromolyn sodium is the preferred treatment modality to prevent symptomatic episodes from exercise. Beta-adrenergic agonists (eg, albuterol) are used for rescue medications for EIA during exercise. The antileukotrienes are oral anti-inflammatory asthma medications designed to inhibit the effects of leukotrienes on the airways. Antileukotrienes appear to be more potent than cromolyn sodium, but they are not as effective as the inhaled corticosteroids. Anticholinergic agents have been used as bronchodilators. They improve pulmonary functions in the moderate to severe pediatric asthmatic when used in conjunction with albuterol nebulization. Anticholinergics do not replace β-adrenergic agents (table 2).43, 44, 45

Table 2. Pharmacotherapy of Asthma

	Medications	Mechanism	Types	Indications
	Corticosteroid	Anti-inflammatory or anticholinergic	Inhaled: fluticasone, beclomethasone, budesonide IV: methylprednisone	Mainstay of chronic asthma therapy More effective than oral corticosteroids Acute exacerbations of asthma
	Short-acting β2-adrenergic agonists	Bronchodilation	Inhaled: albuterol Nebulized: albuterol IV: terbutaline IV: salbutamol (albuterol)	Mainstay of acute asthma therapy To correct hypoxemia caused by acute asthma Severe asthmatic children in impending respiratory failure: to avoid mechanical ventilation
	Long-acting β2-adrenergic agonists	Bronchodilation	Inhaled: salmeterol (Serevent), formoterol (Feradil)	For severe asthmatic children: more effective when used with inhaled corticosteroid
	Inhaled anticholinergic agents (synthetic atropine)	Bronchodilation Anti-inflammatory	Inhaled, nebulized: ipratropium bromide (Atrovent)	Used as an adjuvant to β-agonist therapy
	Cromolyn sodium	Anti-inflammatory action	Inhaled: Intal Inhaler Oral: Gastrocrom	An effective inhibitor of both the early- and late-phase response to exercise, allergens, and some pollutants; give 30min before exercise; prevents symptomatic EIA during exercise
	Antileukotrienes	Anti-inflammatory action	Oral: zafirlukast (Accolate), montelukast (Singulair)	Chronic asthma

Abbreviation: IV, intravenous.

Use of a spacer device with a metered-dose inhaler increases the chances of delivering the correct dose to the respiratory tract. A true spacer has a 1-way valve that allows the patient to receive the medication on inhalation but prevents spraying the medication into the oral pharynx when the inhaler is actuated. In spacer use, large particles of the medication (>10μm) are deposited into the spacer and are not inhaled, which decreases the total dose and potentially decreases the incidence of dose-related adverse effects.45