Clinical signs

• Progression of disease documented by any of these symptoms:
  • Dyspnea at rest
  • Dyspnea on exertion
  • Homebound/chairbound
  • Oxygen dependent
  • Copius/purulent sputum
  • Cyanosis: fingertips, lips
  • Barrel chested
  • Poor response to bronchodilators

Functional status

• Decline in Karnofsky score
• Increased hospitalizations for pulmonary infections
• Decrease in FEV1 on serial testing of greater than 40 ml/year
• Hypoxemia at rest on supplemental oxygen
• Unintentional weight loss in the past 6 months
• Resting tachycardia (more than 100 per minute)