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2025 PACUPrep BCCCP Preparatory Course Cystic Fibrosis Critical Care Management of Cystic Fibrosis

Lesson 4, Topic 4

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Critical Care Management of Cystic Fibrosis

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Life-Threatening ICU Complications in Cystic Fibrosis

Life-Threatening ICU Complications in Cystic Fibrosis: Hemoptysis, Pneumothorax, Respiratory Failure, and Sepsis

Learning Objective

Identify and manage massive hemoptysis, pneumothorax, acute respiratory failure, and sepsis in critically ill CF patients.

I. Overview of Life-Threatening ICU Complications in CF

Advanced Cystic Fibrosis (CF) lung disease—characterized by bronchiectasis, bullae, and chronic infection—predisposes patients to massive hemoptysis, pneumothorax, respiratory failure, and sepsis, each driving high morbidity and mortality.

Pathophysiology of ICU Complications in CF

Chronic Airway Inflammation

→

Friable Vessels

→

Massive Hemoptysis

Subpleural Blebs/Bullae

→

Pneumothorax

Impaired Gas Exchange &
Secretion Clearance

→

Acute Respiratory Failure

Colonization by
Resistant Pathogens

→

Severe Sepsis

Figure 1: Pathophysiological Pathways to Critical Complications in Cystic Fibrosis. This diagram illustrates the primary mechanisms by which advanced CF lung disease leads to life-threatening events requiring ICU admission.

Key Pearl: Overlapping Events

Overlapping events are common (e.g., hemoptysis precipitating hypoxemic failure); anticipate sequential complications.

II. Management of Massive Hemoptysis

Hemoptysis >200–600 mL/24 h is life-threatening. Goals: protect airway, hemostasis, minimize iatrogenic injury.

A. Airway Protection Strategies

Position bleeding lung dependent to prevent contralateral soiling.
Avoid elective intubation—preserve cough and secretion clearance.
Indications for intubation: refractory hypoxemia, airway obstruction, altered mental status.
Use double-lumen tube or bronchial blocker if intubated.
Aggressive endobronchial suctioning and minimal positive pressure.

Clinical Pearl: Intubation Caution

Unnecessary intubation may worsen bleeding and impede clearance—reserve for true airway emergencies.

B. Bronchial Artery Embolization (BAE)

Indication: persistent or recurrent massive hemoptysis despite conservative measures.
Technique: selective catheterization of hypertrophied bronchial arteries; embolize with particles or coils.
Post-procedure: monitor for rebleeding, chest pain, spinal cord ischemia.
Rebleeding risk: up to 20–40%—plan for repeat BAE or surgical consultation.

Clinical Pearl: Early IR Involvement

Early Interventional Radiology (IR) involvement expedites control; do not delay if >200 mL bleed persists.

C. Airway Clearance Modulation

Withhold chest physiotherapy and aerosolized agents during active bleeding.
Resume airway clearance 24–48 h after hemostasis under close observation.
Balance risk of mucus plugging against rebleeding potential.

Clinical Pearl: Restarting Airway Clearance

Restart gentle airway clearance post-embolization to prevent infection and atelectasis.

III. Pneumothorax Management

Rupture of subpleural blebs leads to spontaneous pneumothorax; CF patients exhibit high recurrence rates.

A. Chest Tube Placement

Tube size: 24–28 Fr for significant air leaks; smaller tubes may suffice in stable patients.
Technique: lateral insertion in mid-axillary line; secure to prevent dislodgement in bullous lung.
Management: water-seal system preferred; low suction (−5 to −10 cmH₂O).
Monitor for persistent air leak and infection at insertion site.

Clinical Pearl: Suction and Persistent Leaks

Minimize suction to reduce barotrauma; persistent leaks >5 days warrant surgical consult.

B. Recurrence Prevention

Indications for pleurodesis or VATS: recurrent pneumothorax, persistent air leak, or failure of conservative therapy.
Ventilation adjustments: tidal volume 4–6 mL/kg PBW, plateau pressure <30 cmH₂O, PEEP ≤8 cmH₂O.

Clinical Pearl: Surgical Referral

Early surgical referral after second pneumothorax reduces hospital days and recurrence.

IV. Acute Respiratory Failure Management

Begin with noninvasive ventilation (NIV); escalate to lung-protective invasive ventilation when necessary.

A. Noninvasive Ventilation (NIV)

Indications: hypercapnic acidosis (pH <7.35), tachypnea >30 breaths/min, increased work of breathing, intact airway reflexes.
Initial settings: BiPAP IPAP 10–20 cmH₂O, EPAP 4–8 cmH₂O; titrate for comfort and gas exchange.
Monitoring: mask seal, patient tolerance, arterial blood gases every 1–2 h.
Failure predictors: rising PaCO₂, persistent tachypnea, inability to clear secretions.

Clinical Pearl: NIV Failure Recognition

Early recognition of NIV failure and prompt intubation avoid crash scenarios.

B. Invasive Mechanical Ventilation

Indications: refractory hypoxemia, NIV failure, hemodynamic instability, airway protection.
Lung-protective strategy: tidal volume 4–6 mL/kg PBW, plateau pressure <30 cmH₂O.
PEEP: 5–8 cmH₂O to maintain alveolar recruitment; avoid overdistension.
Permissive hypercapnia acceptable if pH >7.20; adjust sedation and neuromuscular blockade as needed.
VAP prevention: elevate head-of-bed, daily sedation interruption, oral care protocols.

Clinical Pearl: Limiting Ventilator Pressures

Limit ventilator pressures; CF lungs are stiff and prone to barotrauma.

Table 1: Key Ventilatory Support Settings in CF Respiratory Failure
Ventilation Mode	Parameter	Target/Range	Notes
Noninvasive Ventilation (NIV) – BiPAP	IPAP (Inspiratory Positive Airway Pressure)	10–20 cmH₂O	Titrate for comfort, tidal volume, and gas exchange.
Noninvasive Ventilation (NIV) – BiPAP	EPAP (Expiratory Positive Airway Pressure)	4–8 cmH₂O	Maintain upper airway patency, improve oxygenation.
Invasive Mechanical Ventilation (Lung-Protective Strategy)	Tidal Volume (V_T)	4–6 mL/kg PBW	PBW = Predicted Body Weight. Minimize volutrauma.
	Plateau Pressure (P_plat)	<30 cmH₂O	Minimize barotrauma.
	PEEP (Positive End-Expiratory Pressure)	5–8 cmH₂O	Maintain alveolar recruitment, avoid overdistension. Higher PEEP may be needed in severe ARDS but use with caution in CF.

V. Sepsis and Multi-Organ Dysfunction Management

Apply Surviving Sepsis principles, tailored to CF hemodynamics and pathogen profile.

A. Hemodynamic Resuscitation

1. Fluid Resuscitation

Balanced crystalloids (e.g., lactated Ringer’s) 30 mL/kg initial bolus.
CF-specific caution: right ventricular dysfunction, hypoalbuminemia → fluid tolerance limited.
Use dynamic indices (stroke volume variation, passive leg raise) to guide further fluids.

2. Vasopressor Therapy

Norepinephrine first-line: start 0.05 µg/kg/min, titrate to MAP ≥65 mmHg.
Vasopressin adjunct: fixed 0.03 units/min for refractory shock.
Epinephrine reserve: risk of tachyarrhythmias limits routine use.

Clinical Pearl: Early Vasopressors in CF Sepsis

Consider earlier vasopressors in CF to avoid fluid overload in compromised right hearts.

B. Early Source Control

Identify sites: lung abscess, empyema, infected devices.
Interventions: chest tube or surgical drainage, debridement of necrotic tissue.

Clinical Pearl: Prompt Source Control

Delay in source control increases mortality; coordinate IR and surgical teams promptly.

C. Adjunctive Pharmacotherapy

Corticosteroids (hydrocortisone 200 mg/day) only in refractory shock; routine benefit unproven.
Renal replacement therapy for AKI with fluid overload or severe acidosis.
Cytokine adsorption and novel immunotherapies remain investigational.

Clinical Pearl: Steroid Use Considerations

Weigh steroid risks (hyperglycemia, infection) against marginal hemodynamic gains.

References

Flume PA et al. Cystic Fibrosis Pulmonary Guidelines: Treatment of Pulmonary Exacerbations. Am J Respir Crit Care Med. 2009;180(8):802–808.
Dentice RL et al. A randomised trial of hypertonic saline during hospitalization for exacerbation of cystic fibrosis. Thorax. 2016;71(2):141–147.
American Thoracic Society; Infectious Diseases Society of America. Guidelines for the management of adults with hospital-acquired, ventilator-associated, and healthcare-associated pneumonia. Am J Respir Crit Care Med. 2005;171(4):388–416.
Saiman L et al. Antibiotic susceptibility of multiply resistant Pseudomonas aeruginosa isolated from patients with cystic fibrosis, including candidates for transplantation. Clin Infect Dis. 1996;23(3):532–537.
Aaron SD et al. Combination antibiotic susceptibility testing to treat exacerbations of cystic fibrosis associated with multiresistant bacteria. Lancet. 2005;366(9484):463–471.
Smyth A et al. Once vs three-times daily tobramycin regimens for CF exacerbations—the TOPIC study. Lancet. 2005;365(9461):573–578.
Dovey M et al. Oral corticosteroid therapy in CF patients hospitalized for pulmonary exacerbation. Chest. 2007;132(4):1212–1218.

2025 PACUPrep BCCCP Preparatory Course

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