Cystic Fibrosis

General Information
Case no.	7.A Cystic Fibrosis
Authors	Laura Friedman, PT, DPT, Board Certified Clinical Specialist in Cardiovascular & Pulmonary Physical Therapy Margot Miller, PT, DPT
Diagnosis	Cystic fibrosis (CF)
Setting	Outpatient pulmonary rehab
Learner expectations	☑ Initial evaluation ☐ Re-evaluation ☐ Treatment session
Learner objectives	Explain the pathophysiology of the patient’s diagnosis. Relate the pathophysiology and progression of pathology from a chronic pulmonary disorder to the clinical manifestations and activity/participation limitations seen in physical therapy practice. Select, implement, and interpret physical therapy interventions based on the medical examination findings. Develop an understanding of medical management and how it influences physical therapy plan of care.

Medical
Chief complaint	Shortness of breath, decreased activity tolerance, and lower extremity weakness
History of present illness	The patient is a 32-year-old man with CF diagnosed at birth referred to pulmonary rehabilitation (PR) to optimize conditioning as recently listed for lung transplant. He reports performing airway clearance with vest two times a day and utilizing positive expiratory pressure device. He was last hospitalized 2 weeks ago for 5 days due to infection and remains on intravenous (IV) antibiotics with peripherally inserted central catheter (PICC) line in place. He is also status post (s/p) gastric tube placement 6 months ago due to malnutrition.
Past medical history	CF, pancreatic insufficiency, osteoporosis, type II diabetes mellitus (DM), and malnutrition
Past surgical history	Gastric tube (6 months ago)
Allergies	None
Medications	Albuterol metered-dose inhaler (MDI), Hypertonic saline 7% (nebulized), Pulmozyme, Azithromycin, Tobramycin (nebulized), Symdeko, Advair, IV cefepime and Tobramycin since recent hospital discharge
Precautions/orders	Contact precautions Titrate oxygen to maintain ≥ 90% Activity as tolerated

Social history
Home setup	Lives in a two-story home with his wife. Three steps + one handrail to enter. One flight of steps + one handrail to the second floor, where bedroom and full bathroom are located. Half bath is on the first floor.
Occupation	Works as accountant, primarily sedentary.
Prior level of function	Independent with all mobility, activities of daily living (ADLs), instrumental ADLs (IADLs), and driving.
Recreational activities	Played soccer until about 3 years ago, but reports it became too hard on his breathing. Has a treadmill at home but hasn’t used it in over a month. Enjoys hiking with his wife.

Imaging/diagnostic test	Results
Pulmonary function tests (test performed at pulmonary clinic visit 1 week ago)	Forced vital capacity (FVC): 1.73 Forced expiratory volume in 1 second (FEV1): 0.55 FEV1/FVC: 32 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 computed tomography (CT; imaging performed at pulmonary clinic visit 1 week ago; Fig. 7.1; Fig. 7.2)	Impression: Bronchial wall thickening, cylindrical and early varicose bronchiectasis, lung hyperinflation, and multifocal areas of segmental and subsegmental atelectasis. Findings represent changes secondary to known CF. Multiple clustered nodules and focal nodular airspace opacities particularly in the right lower lobe could be secondary to airway obstruction or related to infection. No large consolidation seen. Severe atrophy of the pancreas (not unusual with CF)
Chest X-ray (imaging performed at pulmonary clinic visit 1 week ago; Fig. 7.3)	Findings: Cardiomediastinal silhouette: stable size and contour 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).

Pause points
Based on the above information, what are the priorities? Diagnostic tests and measures? Outcome measures? Treatment interventions?

Pause points

Based on the above information, what are the priorities?

Diagnostic tests and measures?
Outcome measures?
Treatment interventions?

Physical Therapy Examination
Subjective
“I want to be able to run around again without shortness of breath.”
Objective
Vital signs	Resting		Maximum with exercise	Postexercise
Blood pressure (mmHg)	122/64		140/66	124/64
Heart rate (beats/min)	102		136	112
Respiratory rate (breaths/min)	28		44	28
Oxygen saturation (SpO₂) on 4L/min O₂ via nasal cannula (NC)	97%		91%	96%
Modified Borg Dyspnea Scale	2/10		6/10	2/10
Pain	0/10		0/10	0/10
General	Walked into the clinic independently. Lines notable for NC, right upper extremity (RUE) PICC line, gastric tube. Observations: thin male, rounded shoulders, mild thoracic kyphosis, barrel chest, moderate clubbing noted to fingers (Fig. 7.4) and toes, no cyanosis.
Head, ears, eyes, nose, and throat (HEENT)	Patient wears contacts/glasses. Otherwise intact.
Cardiovascular and pulmonary	Auscultation: mild to coarse crackles throughout, no adventitious heart sounds. No edema noted in upper or lower extremities. Cough: strong, currently nonproductive. Patient reports performed airway clearance prior to arrival. Breathing pattern: symmetrical, minimal accessory muscle use noted at rest and increased with activity.
Gastrointestinal	Gastric tube intact, no signs of infection. Patient reports nightly feeding through tube.
Genitourinary	Patient reports no current issues.
Musculoskeletal	Range of motion	Bilateral upper extremity (BUE): within functional limit (WFL) Bilateral lower extremity (BLE): WFL
	Strength	Bilateral upper extremities (BUE): 5/5 Bilateral hip flexion: 4/5 Bilateral hip abduction: 3 + /5 Bilateral knee flexion: 5/5 Bilateral knee extension: 5/5 Bilateral Ankle Dorsiflexion: 5/5 Five Times Sit-to-Stand: 13.2 seconds
	Aerobic	6-Minute Walk Test: 1,140 feet (347 m) on 6 L/min via NC
	Flexibility	Mild hamstring tightness bilaterally
Neurological	Balance	Static sitting, unsupported: independent Dynamic sitting, unsupported: independent Static standing: independent Dynamic standing: independent
	Cognition	Alert and oriented (A&O) × 4
	Coordination	Finger to nose: intact bilaterally Heel to shin: intact bilaterally
	Cranial nerves	II– XII: intact
	Reflexes	Patellar: 2 + bilaterally Achilles: 2 + bilaterally
	Sensation	BUE/BLE: intact to light touch and proprioception
	Tone	BUE and BLE: normal tone throughout
Functional status
Bed mobility	Supine to/from sit: independent
Transfers	Sit to/from stand: independent
Ambulation	Ambulated × 1,140 feet independently Gait deviations: unremarkable
Stairs	Ascend/descend 12 steps + 1 rail independently, demonstrated reciprocal stepping pattern

Fig. 7.4 Digital clubbing.

Assessment
☑ Physical therapist’s	Assessment left blank for learner to develop
Goals
Patient’s	“I want to get stronger, get a lung transplant, and return to normal.”
Short term	1. Goals left blank for learner to develop. 2.
Long term	1. Goals left blank for learner to develop. 2.

Plan
☐ Physician’s ☑ Physical therapist’s ☐ Other’s	Will continue to see patient 3 times per week for 6 weeks. Treatment will include therapeutic exercises (proximal lower extremity and upper extremity strengthening, stretching, postural exercises, aerobic conditioning, breathing exercises), airway clearance as indicated, and energy conservation strategies.

Plan

☐ Physician’s

☑ Physical therapist’s

☐ Other’s

Will continue to see patient 3 times per week for 6 weeks. Treatment will include therapeutic exercises (proximal lower extremity and upper extremity strengthening, stretching, postural exercises, aerobic conditioning, breathing exercises), airway clearance as indicated, and energy conservation strategies.

Bloom’s Taxonomy Level	Case 7.A Questions
Create	1. Synthesizing the medical data and physical examination findings, develop an appropriate physical therapy assessment of the patient. 2. Develop two short-term physical therapy goals, including an appropriate timeframe. 3. Develop two long-term physical therapy goals, including an appropriate timeframe.
Evaluate	4. Determine potential referral sources to other health care providers. 5. Explain why malnutrition is seen commonly in patients with CF. 6. Explain why an airway clearance routine is critical for patients with CF.
Analyze	7. Based on the 6-Minute Walk Test, is the patient above, at, or below the mean for individuals with CF? Is the patient above or below age-matched individuals? What is the minimally detectable change that needs to be achieved to show improvement in endurance? 8. Analyze the relationship between the primary diagnosis of CF and postural dysfunction. 9. Analyze the patient’s posture during sitting and gait.
Apply	10. Design and implement two interventions to improve the patient’s aerobic capacity. 11. Design and implement two interventions—one stretching and one strengthening—to improve cervical and shoulder posture. 12. Design and implement two interventions to improve endurance/activity tolerance. 13. Describe the purpose of the patient’s Aerobika. Describe two other interventions that could be utilized to improve airway clearance during pulmonary rehab.
Understand	14. Describe the purpose of the medications this patient is currently taking. Will these medications have any physical therapy implications?
Remember	15. Based on the patient’s medical diagnosis of type II DM, is it important to measure the patient’s blood glucose before, during, or after pulmonary rehab sessions? What would be signs the blood glucose level may be too low or too high? 16. Why are contact precautions in place for CF patients across all settings? What personal protective equipment (PPE) should be donned by the clinicians treating this population?

Bloom’s Taxonomy Level	Case 7.A Answers
Create	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. 2. Short-term goals: Patient will increase hip flexor strength to >/=4+/5 manual muscle testing (MMT) grade within 3 weeks to allow for independent reciprocal stair negotiation without rail for 12 steps. Patient will increase 6-Minute Walk Test by 150 feet within 3 weeks to demonstrate improved endurance. 3. Long-term goals: Patient will decrease Five Times Sit-to-Stand time to < 10 seconds within 6 weeks to show improved BLE strength. Patient will increase 6-Minute Walk Test by 300 feet within 6 weeks to demonstrate improved endurance and ability to return to leisure/sports activities.
Evaluate	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.
Analyze	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.
Apply	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. 11. To improve cervical and shoulder posture, address shortened anterior muscles, such as pectoralis minor, via stretches, as well as strengthen the scapular retractors (middle trapezius, rhomboids). 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. Overall flexibility exercises can also improve posture, and strength and yoga and Tai Chi can have a positive impact on posture, strength, and flexibility. 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).
Understand	14. The purpose of the medications are as follows: Albuterol: a bronchodilator and utilized to relax muscles in the airways and increase airflow to the lungs. Tachycardia is a potential side effect that could impact physical therapy. Hypertonic saline: helps lung function and decreases risk for infection by thinning mucus in the airways. Pulmozyme: a synthetic protein that breaks down excess deoxyribonucleic acid (DNA) in the mucus and therefore improves lung function and reduces risk of infection. Azithromycin, tobramycin, and cefepime: antibiotics used to fight infection. Patients with CF can be on oral and/or IV antibiotics based on severity and strain of infection. Common side effects that could impact physical therapy are diarrhea and nausea. Symdeko: a CFTR modulator that is taken to correct the malfunctioning protein made by the CFTR gene. Side effects that could impact physical therapy include dizziness, nausea, headaches, and liver problems. Advair: a combination medicine that has a steroid and a bronchodilator and therefore prevents inflammation as well as relaxes the muscles in the airways to improve breathing. The side effect that would most likely impact physical therapy is tachycardia, which occurs in 1 to 10% of patients taking Advair.
Remember	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.

Fig. 7.5 (a) Aerobika. (b) Acapella.

Key points
1. It is important for the physical therapist to understand what interventions will best prepare the patient for lung transplant surgery.
2. It is important to understand all the systems, especially the pulmonary, gastrointestinal, and musculoskeletal systems, can be impacted by CF.
3. Ensure, that early on, a physical therapist provides a home exercise program that highlights primary impairments, such as quad strength and endurance in this case, and educate on appropriate use of home treadmill for endurance training that compliments versus hinders pulmonary rehab.

General Information
Case no.	7.B
Authors	Margot Miller, PT, DPT Laura Friedman, PT, DPT, Board Certified Clinical Specialist in Cardiovascular & Pulmonary Physical Therapy
Diagnosis	Cystic fibrosis (CF)
Setting	Acute care hospital—cardiothoracic intensive care unit (CICU)
Learner expectations	☑ Initial evaluation ☐ Re-evaluation ☐ Treatment session
Learner objectives	Identify the benefits of early mobilization in the ICU setting following lung transplant. Understand common side effects of immunosuppressive/posttransplant drugs. Select and implement physical therapy interventions based on the physical therapy examination findings in the context of the acute care setting and patient goals. Develop an understanding of medical management and how it influences physical therapy plan of care.

Medical
Chief complaint	Postoperative day (POD) 1 bilateral lung transplant (clamshell incision; Fig. 7.6).
History of present illness	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.
Past medical history	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.
Past surgical history	Gastric tube (placed 9 months ago)
Allergies	None
Medications	Albuterol metered-dose inhaler (MDI), Hypertonic saline 7% (nebulized), Pulmozyme, Azithromycin, Tobramycin (nebulized), Symdeko, and Advair
Precautions/Orders	Activity as tolerated Weight bearing as tolerated (WBAT) bilateral lower extremities (BLE) Contact precautions—immunocompromised Fall precautions

Social history
Home setup	Lives in a two-story home with his wife. Three steps + one handrail to enter. One flight of steps + one handrail to the second floor, where bedroom and full bathroom are located. Half bath is on the first floor. Most recently, the patient has been sleeping on the ground floor living room to avoid stair climbing due to increased shortness of breath.
Occupation	Works as an accountant, primarily sedentary.
Prior level of function	Previously, independent with all mobility, activities of daily living (ADLs), instrumental ADLs (IADLs), driving. More recently, he is ambulating household distances and independent/modified independent with basic ADLs (BADLs), utilizing a shower chair and increased time due to shortness of breath He has an active license but has not been driving for the last 6 weeks.
Recreational activities	Played soccer until about 3 years ago, but reports it became too hard on his breathing. Has a treadmill at home, which he tried to begin using after discontinuing participation in pulmonary rehab. He reports tolerating 3 to 4 minutes of continuous walking before needing a seated rest break to catch his breath.