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Pulmonary Disorders II Zachary R. Smith, Pharm.D., FCCP, FCCM, BCPS, BCCCP Henry Ford Hospital Detroit, Michigan…

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Pulmonary Disorders II Pulmonary Disorders II Zachary R. Smith, Pharm.D., FCCP, FCCM, BCPS, BCCCP Henry Ford Hospital Detroit, Michigan…

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Learning Objectives 3 min

Pulmonary Disorders II Learning Objectives 1. Design a treatment plan for a cystic fibrosis (CF) exacerbation. 2. Develop a pharmacotherapy plan for pulmonary artery hypertension using a risk asse…

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Pulmonary Disorders II 2. Which adjunctive measure for a CF exacerbation has the highest quality of evidence for improving patient outcomes? A. Normal saline (0.9%) nebulization. B. Corticosteroi…

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Pulmonary Disorders II 7. A 66-year-old man presents to the ICU with acute respiratory failure from a chronic obstructive pul- monary disease exacerbation (ECOPD). He has had no exacerbations in th…

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Exam Content Outline 1 min

Pulmonary Disorders II BPS Critical Care Pharmacy Examination Content Outline This chapter covers the following sections of the Critical Care Pharmacy Examination Content Outline: 1. Domain 1: Critic…

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Pulmonary Disorders II I.  CYSTIC FIBROSIS A. Cystic Fibrosis (CF) – Chronic disease process that affects many organs, including the pancreas, liver, and intestine, but primarily the lung (Chest 200…

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Data Tables 3 min

Pulmonary Disorders II b. Notably, most pulmonary exacerbations are not due to the acquisition of a new microorganism, and patients with CF commonly have routine cultures collected throughout their…

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Pulmonary Disorders II 5. Exacerbation treatment outcomes a. Providers and practice will vary in defining when treatment is complete for pulmonary exacerbation. The STOP study identified the most c…

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Pulmonary Disorders II 4. Continuation of patient’s current chronic airway clearance therapy a. It is recommended to continue nebulized airway clearance therapies during an exacerbation (Table 2);…

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Pulmonary Disorders II II.  PULMONARY ARTERY HYPERTENSION A. Pathophysiology and Definitions 1. Pulmonary artery hypertension (PAH) is a chronic, life-threatening disease characterized by vascular…

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Pulmonary Disorders II 2. Clinical classification of pulmonary hypertension (PH) by group (Eur Respir J. 2024;64(4):2401324; Eur Heart J. 2022;43:3618-3731) a. Group 1 (PAH): Idiopathic, heritable,…

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Data Tables 3 min

Pulmonary Disorders II C. Risk Stratification of PAH (group 1 PH) (Eur Respir J. 2024;64(4):2401324; Eur Heart J. 2022;43:3618-3731) 1. PAH risk stratification is a multiparametric risk assessment t…

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Data Tables 3 min

Pulmonary Disorders II Multivariable Assessment of Mortality Risk to Guide Initial Therapy Assessment 3-Risk Strata BNP or NT-proBNP BNP < 50 ng/L NT-proBNP < 300 ng/L BNP 50–800 ng/L NT-proBNP 300–1…

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Pulmonary Disorders II c. Diuretics should be used for the symptomatic management of RV dysfunction and signs of fluid overload; choice of diuretic is variable. d. Digoxin increases cardiac output;…

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Data Tables 3 min

Pulmonary Disorders II 2. Treatment-naïve patients with PAH (nonresponders to vasoreactivity testing) with a PVR greater than 3 Wood units and an mPAP greater than 25 mm Hg. Although the definition…

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Pulmonary Disorders II c. Intermediate/High- strata i. Add a parenteral prostacyclin—or— ii. Add an activin-signaling inhibitor (N Engl J Med. 2023;388(16):1478-1490). d. High-risk strata i. Add…

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Data Tables 3 min

Pulmonary Disorders II 3. Soluble guanylate cyclase stimulators a. Riociguat exerts its effect through two mechanisms: Sensitizes endogenous soluble guanylate cyclase by stabilizing nitric oxide–so…

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Data Tables 3 min

Pulmonary Disorders II c. Oral treprostinil (Table 8): i. The addition of oral tresprostinil to ERA or PDE5i/riociguat monotherapy is recommended to reduce the risk of morbidity and mortality in p…

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Pulmonary Disorders II e. Approved dose is 200 mcg twice daily; increase at weekly intervals to the highest tolerated dose (maximum dose 1600 mcg twice daily); adjustment necessary for moderate hepa…

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Pulmonary Disorders II b. Direct effects on the pulmonary circulation from vasopressors may increase the PVR, potentially leading to further clinical decompensation. c. Few studies have been publis…

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Pulmonary Disorders II 7. Unloading the RV with pulmonary vasodilators is essential to controlling decompensated PH and RV failure. a. In select scenarios, temporary inhaled epoprostenol or inhaled…

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Data Tables 3 min

Pulmonary Disorders II III.  ASTHMA EXACERBATION A. Pathophysiology and Classification of Exacerbations (Global Initiative for Asthma 2024) 1. Asthma is a heterogeneous disease, usually categorized…

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Pulmonary Disorders II 5. Seasonal weather changes 6. Poor adherence to inhaled corticosteroids C. Mortality Risk Factors (Multidiscip Respir Med. 2016;11:37; Global Initiative for Asthma 2024) 1.…

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Pulmonary Disorders II H. Oxygen 1. Oxygen therapy is important in managing acute severe asthma. 2. Oxygen by nasal cannula or mask should be administered to patients with severe exacerbations with…

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Pulmonary Disorders II L. Anticholinergic Agents 1. Inhaled anticholinergic agents selectively bind to the muscarinic receptors on smooth muscle cells in the airways and thereby reduce bronchoconst…

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Pulmonary Disorders II c. Data for magnesium sulfate administration in severe asthma exacerbations are mixed. In addition, data exist using nebulized magnesium rather than intravenous, but evidence…

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Data Tables 2 min

Pulmonary Disorders II IV.  ACUTE CHRONIC OBSTRUCTIVE PULMONARY DISEASE EXACERBATION A. Chronic Obstructive Pulmonary Disease (COPD) (GOLD 2024) 1. COPD is defined as a heterogeneous lung condition…

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Data Tables 3 min

Pulmonary Disorders II 3. Diagnosis of ECOPD a. Symptoms, severity of dyspnea using a visual analog scale, and documentation of the presence of cough should be assessed. b. Signs (tachypnea, tachy…

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Data Tables 3 min

Pulmonary Disorders II 5. Definitions of ECOPD respiratory failure (GOLD 2024) Table 13. Definitions of ECOPD Respiratory Failure No respiratory failure • RR ≤ 24 breaths/min • HR < 95 beats/min •…

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Pulmonary Disorders II D. High-Flow Oxygen Therapy by Nasal Cannula 1. Alternative to standard oxygen therapy or noninvasive positive pressure ventilation (GOLD 2024) 2. Improves oxygenation and ve…

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Pulmonary Disorders II 2. Literature review a. Reduction in the Use of Corticosteroids in Exacerbated COPD (REDUCE) was a randomized, noninferiority trial. Patients (n=314) were randomized to predn…

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Pulmonary Disorders II ii. Data remain inconclusive regarding treatment failure, mortality, and repeated exacerbations for patients who are hospitalized outside the ICU with severe COPD and for outp…

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Pulmonary Disorders II Patient Cases 7. A 79-year-old woman (weight 70 kg) is admitted to the ICU for the management of hypercapnic respiratory failure related to an ECOPD. She has had several admis…

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Pulmonary Disorders II REFERENCES Cystic Fibrosis Akkerman-Nijland AM, Akkerman OW, Grasmeijer F, et al. The pharmacokinetics of antibiotics in cystic fibro- sis. Expert Opin Drug Metab Toxicol. 2021…

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Pulmonary Disorders II Smyth A, Tan KH, Hyman-Taylor P, et al. Once versus three-times daily regimens of tobramycin treatment for pulmonary exacerbations of cystic fibrosis—the TOPIC study: a rando…

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Pulmonary Disorders II Hoeper MM, Benza RL, Corris P, et al. Intensive care, right ventricular support and lung transplan- tation in patients with pulmonary hypertension. Eur Respir J. 2019;53(1)…

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Pulmonary Disorders II controlled trial. Am J Med. 1983;74(5):845-851. https:// doi.org/10.1016/0002-9343(83)91076-8 Garner O, Ramey JS, Hanania NA. Management of life-threatening asthma: severe asth…

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Pulmonary Disorders II Acute Chronic Obstructive Pulmonary Disease Exacerbation Abroug F, Ouanes-Besbes L, Fkih-Hassen M, et al. Prednisone in COPD exacerbation requiring ventilatory support: an op…

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Pulmonary Disorders II exacerbations of chronic obstructive pulmonary disease. Cochrane Database Syst Rev. 2006;(2):CD004403. https://doi.org/10.1002/14651858.cd004403.pub2 Sayiner A, Aytemur ZA, Ci…

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Answers & Explanations 4 min

Pulmonary Disorders II ANSWERS AND EXPLANATIONS TO PATIENT CASES 1. Answer: C The patient presents with a CF exacerbation, probably caused by an infection. The most likely causative organ- ism of he…

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Pulmonary Disorders II (fever, purulent sputum) and a severe ECOPD with respiratory failure. This patient has a history of anaphy- lactic reaction to penicillin and repeated exacerbations that may p…

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Self-Assessment 4 min

Pulmonary Disorders II ANSWERS AND EXPLANATIONS TO SELF-ASSESSMENT QUESTIONS 1. Answer: D It is imperative to recognize that this patient has acute respiratory distress syndrome (ARDS) caused by a…

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Pulmonary Disorders II incorrect; Answer B is correct). Antimicrobial treatment should be initiated if (1) all three cardinal symptoms of an ECOPD (increased dyspnea, increased sputum pro- duction,…

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