BACKGROUND: About 140,000 Americans have been diagnosed with pulmonary fibrosis. It usually affects people between the ages of 50 and 75. Pulmonary fibrosis is a disease marked by scarring in the lungs. Tissue deep in the lungs becomes thick, scarred, and stiff. That scarring is called fibrosis. In certain cases, the cause of pulmonary fibrosis can be found, but most are unknown. It can develop quickly or slowly, and there is not a cure. Most people with the disease only live about three to five years after diagnosis. It can lead to other medical problems, including collapsed lung infections, blood clots in the lung, and lung cancer. As the disease worsens, it can lead to respiratory failure, heart failure, and pulmonary hypertension. (Source: www.lung.org)
CAUSES: In most cases, the cause is unknown. However, there are certain things that increase the risk of developing the disease. They include:
- Certain viral infections
- Cigarette smoking
- Exposure to environmental pollutants, including hard metal dusts and silica
- Gastroesophageal reflux disease (GERD). Some people who have GERD may breathe in tiny drops of acid from their stomach, which can injure the lungs. (Source: www.lung.org)
TREATMENT: Treatments aim at preventing more lung scarring and relieving symptoms. They include: medication, like prednisone to reduce inflammation; pulmonary rehabilitation programs that teach people about their disease and how to manage their disease; and lung transplants. (Source: www.lung.org)
NEW TECHNOLOGY: First, there was the "heart in a box," a revolutionary experimental technology that allows donor hearts to be delivered to transplant recipients warm and beating rather than coming form an ice cooler. That same technology is now being used to deliver "breathing lungs." The lung transplant team at Ronald Reagan UCLA Medical successfully performed the nation's first "breathing lung" transplant in November 2012. The transplant involved an experimental organ-preservation device known as the Organ Care System (OCS), which keeps donor lungs functioning and "breathing" in a near-physiologic state outside the body during transport. With the OCS, the lungs are removed from a donor's body and placed in the box, where they are immediately revived to a warm, breathing state and perfused with oxygen and a special solution supplemented with packed red-blood cells. "Organs were never meant to be frozen on ice. Lungs are very sensitive and can easily be damaged during the donation process. The cold storage method does not allow for reconditioning of the lungs before transplantation, but this promising 'breathing lung' technology enables us to potentially improve the function of the donor lungs before they are placed in the recipient," Dr. Abbas Ardehali was quoted as saying. UCLA is leading a multicenter pivotal clinical INSPIRE study of the OCS, developed by medical device company TransMedics. The trial is underway at centers in Europe, Canada, and Australia and will enroll 264 patients. "For patients with end-stage lung disease, lung transplantation can dramatically improve the patient's symptoms and offer relief from severe shortness of breath," Dr. David Ross, Professor of Medicine and Medical Director of UCLA's Lung and Heart-lung Transplantation Program and UCLA's Pulmonary Arterial Hypertension and Thromboendarterectomy Program, was quoted as saying, "The 'breathing lung' technology could potentially make the transplantation process even better and improve the outcomes for patients suffering from lung disease." (Source: http://newsroom.ucla.edu/portal/ucla/ucla-performs-first-breathing-241056.aspx)
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