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The patient is a 25-year-old third-year graduate student working on her PhD in biochemistry. She was diagnosed with cystic fibrosis (CF; ΔF508/ΔF508) during newborn screening. Relevant medical history includes two hospitalizations for acute CF exacerbations (at age 17 and 19 years), and a diagnosis of CF-related diabetes 1 year ago. She has “moderate” CF lung disease (forced expiratory volume in one second [FEV1] 62% on last clinic visit).1 Her last sputum cultures were positive for Pseudomonas aeruginosa and methicillin-resistant Staphylococcus aureus, but not for Burkholderia cepacia. At baseline, she produces approximately 30 mL/day of greenish yellow sputum using a combination of high-frequency chest wall oscillation (The Vest) and vibratory positive expiratory pressure (PEP; acapella) 1 to 2 times per day each. She walks to school each day, including six blocks uphill on the way home, takes the stairs at school (her lab is on the fifth floor), and plays intramural soccer. However, for the past several weeks, her daily mucous production has increased (to ~100 mL), the color changed to greenish brown, and it has been thicker and more difficult to expectorate. She initiated an inhaled antibiotic (Tobi) and increased her airway clearance frequency. She also reports general malaise, decreased appetite, and increased dyspnea and leg fatigue after climbing the stairs/walking uphill. As a result of these symptoms, she has been driving to school, taking the elevator, and ceased any form of exercise. Yesterday, she came to the emergency department after several episodes of frank hemoptysis (~250 mL in < 24 hours), increased shortness of breath at rest, and new onset right-sided chest pain. She was diagnosed with a CF pulmonary exacerbation and a spontaneous right upper lobe pneumothorax and admitted to the pulmonary step-down unit. The physical therapist is asked to evaluate and treat her on the second day of hospital admission.

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Based on her health condition, what do you anticipate will be the contributors to activity limitations?

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What are the examination priorities?

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What are the most appropriate tests and measures?

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What are the most appropriate physical therapy interventions?

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What precautions should be taken during physical therapy interventions?

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  • HEMOPTYSIS: Coughing up blood or bloody mucous; can range from scant (< 5 mL) blood-streaked mucous to massive (> 240 mL) amounts of pure bright red blood; treatment can range from monitoring at home to hospital admission for bronchial artery embolization
  • PNEUMOTHORAX: Collection of air in the pleural space, leading to a collapsed lung; occurs “spontaneously” in CF; can be treated with observation, needle aspiration, or insertion of a chest tube, depending on the severity
  • PULMONARY EXACERBATION: Acute worsening of respiratory symptoms, including increased cough and sputum production, shortness of breath, chest pain, loss of appetite, weight loss, decreased exercise tolerance and decline in lung function; typically requires treatment with intravenous antibiotics in a hospital setting

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  1. Describe the multisystem involvement of CF.

  2. Describe and recognize common pulmonary complications associated with a CF pulmonary exacerbation.

  3. List common medications used for the acute and chronic management of CF and describe potential adverse drug reactions (ADRs) of these medications and how they may impact physical therapy management.

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