End-Stage Pulmonary Disease:
Guidelines for End-Stage Pulmonary Disease & Related Disorders

LIFE-LIMITING CONDITION AS EVIDENCED BY:

• Dyspnea at rest and on exertion.
• Severe cough.
• Copious/purulent sputum.

SIGNS:

• Cyanosis: blue lips, fingertips.
• Retractions.
• Oxygen dependence.
• Depressed diaphragm.
• Accessory muscles of respiration.
• Pulmonary hyperinflation: barrel-chested.
• Increased expiratory phase: slowed forced expiration.
• Increased hospitalization for pulmonary infections/respiratory failure.
• Decrease in FEV1 on serial testing of greater than 40 mL per year*.
• Presence of cor pulmonale or right heart failure due to lung disease.
• Forced expiratory volume in one second (FEV1) after bronchodilator, less than 30% of predicted*.
• Pursed-lip breathing.
• Wheezing.
• Recurrent infections.
• Poor response to bronchodilators.
• Diminished breath sounds.
• Increased visits to Emergency Department.

EVIDENCED BY:

• Echocardiographic documentation.
• Chest x-ray.
• Hypoxemic at rest on supplemental oxygen.
• Unintentional weight loss > 10% of body weight in past six months.
• Resting tachycardia (heart rate > 100 per minute).
• EKG*
• Physical signs of RHF

* These tests are helpful evidence but should not be required if not readily available.

HOW WE HELP — END-STAGE PULMONARY DISEASE:

• Increased quality of life.
• Medication to relieve nausea, pain or shortness of breath.
• Coordination of comfort measures for patient including durable medical equipment and non-medical therapies.
• Education for the patient and family as disease progresses.
• Psychosocial support for patient and the family.
• Spiritual care and support for the patient and the family.