Findings/impression: FVC and FEV1 severely reduced (< 35%); FEV1/FVC is reduced; flow volume curve demonstrates both an obstructive and a restrictive pattern; very severe obstructive defect is present.
Chest X-ray (imaging performed at pulmonary clinic visit 1 week ago; Fig. 7.3)
Lungs: hyperinflated with underlying emphysema and biapical pleural–parenchymal scarring. Coarse somewhat linear opacities in both mid lungs also likely underlying scarring from emphysema. No lobar consolidation, large pleural effusion, or pneumothorax.
Fig. 7.1 A 32-year-old man with wheezing, dyspnea, and productive cough. Unenhanced chest CT (lung window). Demonstrates extensive bronchiectasis in the right upper lobe. (Parker M, Rosado-de-Christenson M, Abbott G. Radiologic Findings. In: Abbott G, Parker M, Rosado-de-Christenson M, ed. Teaching Atlas of Chest Imaging. 1st Edition. New York: Thieme; 2005).
Fig. 7.2 A 32-year-old man with wheezing, dyspnea, and productive cough. Unenhanced chest CT (lung window) Note cystic bronchiectasis with internal mucous plug in right upper lobe. (Parker M, Rosado-de-Christenson M, Abbott G. Radiologic Findings. In: Abbott G, Parker M, Rosado-de-Christenson M, ed. Teaching Atlas of Chest Imaging. 1st Edition. New York: Thieme; 2005).
Fig. 7.3 A 32-year-old man with wheezing, dyspnea, and productive cough. PA chest radiograph demonstrates increased lung volumes and extensive bilateral bronchiectasis manifesting as ring, tram track, nodular, and reticular opacities most severe in the upper lungs. (Parker M, Rosado-de-Christenson M, Abbott G. Radiologic Findings. In: Abbott G, Parker M, Rosado-de-Christenson M, ed. Teaching Atlas of Chest Imaging. 1st Edition. New York: Thieme; 2005).
1. The patient is a 32-year-old man with CF recently listed for bilateral lung transplant s/p recent hospitalization for infection and now presents for outpatient pulmonary rehab evaluation. Upon initial evaluation, the patient presents with impaired aerobic capacity, bilateral proximal lower extremity muscular weakness, postural dysfunction, and impaired endurance, which contribute to inability to walk long distances or for long durations and to engage in usual exercise and sport activities. The patient is at risk for increased hospital readmissions and further deconditioning without physical therapy interventions. The patient is a good candidate for skilled physical therapy services as motivated to optimize strength and conditioning in preparation for future lung transplant.
4. Fortunately, since this patient is now active on the lung transplant list, he has routine visits with a lung transplant dietician to ensure he is gaining weight appropriately. The patient also has routine visits with a pulmonologist and endocrinologist to manage CF and diabetes, respectively. Any patient with CF would benefit from referral to a dietician as weight loss is very common, as well as to ensure on right regimen of vitamins due to associated pancreatic insufficiency with CF. Also, a referral to a psychiatrist or psychologist may be beneficial to help the individual cope with chronic illness, possible anxiety, depression, and potential life-changing events, such as lung transplantation.
5. The main reason malnutrition is common in patients with CF is a buildup of mucus in the digestive tract making it difficult to digest and absorb nutrients, malabsorption due to pancreatic insufficiency, and increased resting energy expenditure. Other attributing factors include CF-related diabetes, decreased appetite, and behavioral feeding problems.
6. An airway clearance routine is critical for patients with CF as it is a genetic disease that primarily affects the lungs and characterized by dehydration of airway surface liquid and impaired mucociliary clearance. Airway clearance is necessary to clear pathogens from the lungs to limit pulmonary infections and inflammation.
7. This patient appears to be below the mean for patients with CF as the largest study looking at CF patients had a mean of 1,725 feet (526 m). There is an additional study with CF patients with moderate airflow limitation that had a mean of 2,053 feet (626 m), but this study only had a sample size of 25 patients. One additional study in 2004 had 109 subjects but of varying airway restrictions and the mean distance was 1,049 feet (320 m). There is limited research looking at the 6-Minute Walk Test in the CF population. The patient is well below age-matched individuals as the predicted value for males of his height and weight is 2,468 feet (752.2 m). The minimally detectable change to show improved endurance that is most closely related to CF is that for chronic obstructive pulmonary disease (COPD), which is 177 feet (54 m).
8. The risk factors that can lead to postural dysfunction in individuals with CF include reduced FEV1, low lean body mass, CF-related diabetes, corticosteroid treatments, pancreatic insufficiency, physical inactivity, low vitamin D, chronic pulmonary conditions, delayed puberty, and Delta F508 genotype. There is ongoing research as to whether the CF transmembrane conductance regulator (CFTR) defect, which causes CF and which has been identified in bone and muscle cells, is a primary cause of bone complications, especially low bone mineral density, or whether postural problems are a secondary complication of the CF disease process. It may be the altered cough and breathing patterns plus inflammatory processes, as well as decreased activity levels with pulmonary infections, as the main cause of postural dysfunction. Also, the muscles of the trunk work to facilitate breathing while also supporting posture, and therefore the more demand placed on them for breathing, the less ability they have to support your posture.
9. The patient’s posture includes rounded shoulders, mild thoracic kyphosis, and a barrel chest. The kyphosis is more prominent during ambulation. In sitting, a slumped posture is noted. Unfortunately, the chest wall muscles have likely shortened due to the chronic manifestations of CF.
10. To improve the patient’s aerobic capacity, the patient could coordinate deep breathing with upper extremity D2 proprioceptive neuromuscular facilitation (PNF) pattern. The patient will inhale through his nose while raising arms and exhale through pursed lips as they lower the arms. This will aid in chest expansion and will be a great exercise to carry over to the post lung transplant phase. A second intervention would be to utilize an inspiratory muscle trainer.
The pectoralis minors can be gently self-stretched in supine, aided by gravity. The patient flexes shoulders and elbows to 90 degrees (“goal post” position) and then slides arms toward the head of the bed until a gentle stretch is felt. The patient can hold this stretch for 10 to 20 seconds at a time, increasing duration to tolerance, up to 10 minutes total per day.
Shoulder retraction exercises can be initiated in supine, with attention given to proper form; progress the exercises by performing in upright, as well as introducing a resistance band for increased resistance and strengthening. As the shoulder retractors are postural stabilizers, emphasize strength training for endurance, that is, lower load, higher repetitions.
12. To improve endurance, the patient could utilize the treadmill to increase duration and intensity of walking. For example, the patient could start with 10 to 15 minutes for three trials at a light to moderate intensity (30 to < 60% of peak work rates). The goal would be to first increase duration to get to 30 to 45 minutes of continuous activity. Once this is achieved, intensity can be increased to vigorous (60–80% of peak work rates). This could be initiated in pulmonary rehab and then continued as part of home program. The upper body ergometer or NuStep machine could be utilized in addition to the treadmill to incorporate upper extremity work. Again, duration would be increased first, followed by intensity.
13. The Aerobika is an oscillatory positive expiratory pressure (OPEP) device that is utilized to improve mucus clearance by providing resistance as exhale into the device. The acapella is another commonly used OPEP device. Both of these devices have the ability to be combined with a nebulizer treatment, with the medication being administered prior to use of the device.
Two other interventions that could be performed are percussion and postural drainage and active cycle of breathing therapy (ACBT). The percussion and postural drainage would especially be indicated if specific area(s) of lungs sound more congested during auscultation. The ACBT is an ideal intervention as it can be done independently by the patient and requires no equipment. ACBT includes three phases: breathing control, chest expansion exercises, and forced expiratory technique or huff coughing. The patient starts by taking slow, relaxed breaths ideally through pursed lip breathing technique and once breath control is achieved, the patient moves to taking deep breaths in which the patient tries to hold inhalation for 2 to 3 seconds for three to five deep breaths. The final phase is to take two to three huff coughs to try and remove secretions as the huff cough is more effective than regular coughing in removing mucus from the smaller airways to the larger airways. The cycle can be repeated as many times as necessary to clear secretions (Fig. 7.5a,b).
15. It is important to measure an individual with diabetes blood glucose before, during, and after exercise to ensure safe tolerance of exercise/activity. It is important to note how the patient’s body responds to exercise in terms of potential fluctuations in blood glucose. Exercise can cause the patient’s blood glucose to decrease and therefore it is ideal for the blood glucose to be higher than 100 mg/dL prior to starting exercise. It is also dangerous if blood glucose is elevated above 250 mg/dL as it puts the patient at risk for ketoacidosis. It is recommended to check the patient’s blood sugar every 30 minutes during exercise, especially if starting a new exercise program. Symptoms of hyperglycemia include increased thirst, dry mouth, increased urination, blurry vision, fatigue, difficulty concentrating, and headaches. Symptoms of hypoglycemia include feeling shaky/weakness, sweating, chills, irritability, confusion, anxiety, dizziness, tachycardia, and fatigue.
16. Individuals with CF have decreased ability to clear bacteria/pathogens from their lungs and many of these pathogens are drug resistant and some strains may always be present. Contact precautions are in place to prevent the spread of infection. It is important to keep CF patients separated from one another in all clinical settings and individuals with CF should always be wearing face masks to cover their mouth and nose when in health care settings. Clinicians treating individuals with CF should wear the following PPE at all times: gown and gloves. They should also practice good hand hygiene regardless of respiratory tract culture results of their patient.
Postoperative day (POD) 1 bilateral lung transplant (clamshell incision; Fig. 7.6).
The patient is a 32-year-old man with CF diagnosed at birth. He completed a comprehensive workup for lung transplant listing about 10 months ago. Shortly thereafter, his listing was made temporarily inactive, as he was determined to be “too well” for active listing. His listing was reactivated 8 weeks ago due to worsening health status documented during a routine pulmonary clinic visit. He had been participating in outpatient pulmonary rehab 3 times a week up until approximately 6 weeks ago when his supplemental oxygen demands increased, and it became too cumbersome to transport additional tanks to rehab. Additionally, he endorses decreased tolerance for activity. He was admitted to the hospital yesterday for possible lung transplant.
CF, pancreatic insufficiency, osteoporosis, type II diabetes mellitus, malnutrition status post (s/p) gastric tube placement, multiple respiratory infections—most recently 3 months ago, necessitating hospitalization, peripherally inserted central catheter (PICC) line placement, and intravenous (IV) antibiotics.