Advanced Lung Disease and Transplantation

By advanced lung disease (ALD) we mean any lung disease that has advanced, or worsened, to the point where the patient is severely disabled and/or faces death from the disease in a short period of time.

This state can occur in all three basic types of lung diseases.

  • Obstructive Lung Diseases are characterized mainly by blocked airways. Examples that can lead to ALD include chronic obstructive lung disease (COPD), and bronchiectasis (from, for example, cystic fibrosis).
  •  Interstitial Lung Diseases (ILD) include many primary diseases of the lung, like Idiopathic Pulmonary Fibrosis (IPF). But ILD can also result from diseases like Systemic Lupus Erythematosus (SLE) that can engulf the lung tissue in it’s systemic destruction.
  • Pulmonary vascular diseases affect the blood vessels that go through the lung to pick up oxygen. Primary Pulmonary Hypertension, for example, is a disease that causes progressive narrowing of these blood vessels, and progressively low oxygen in the blood.

Some of these diseases progress to a point of ALD, in spite of best standard medical practice. Generally ALD is present when the lung function has dropped below about a third of normal function. At that point most patients require oxygen and are quite limited.

Options for ALD are limited, but include ongoing best treatment of the causative disease, and support with oxygen, as well as pulmonary rehabilitation. We have hope that some novel treatments such as stem cells will eventually help us to rebuild damaged lungs. However, those therapies are still in the research phase, and are least 10 – 15 years from practical use. Meanwhile, however, lung transplantation is proving ever more effective for ALD. Lung transplantation is still reserved for a select few, but can be life saving and restore quality of life.

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