CLEVELAND, Ohio - Imagine not being able to breathe without struggling: every breath you take is work; every breath you take could be your last. That was the case for one man who became dependent on an oxygen tank to stay alive.
"It's nice to actually sit down and have a conversation without having to gasp for air," said Robert Epperson.
"He was very sick. He was very short of breathe," explained Epperson's doctor, Jihane Faress, MD, in the pulmonary medicine division at Cleveland Clinic.
After suffering years from a misdiagnosis, doctors at the Cleveland Clinic discovered Epperson was actually suffering from a rare condition called pulmonary alveolar proteinosis, or PAP. The disease causes a protein called surfactant to build up in the air sacs of the lungs.
"Surfactant is a substance that we all need to keep our aveolar sac from closing off when we exhale, when we breathe out. So we need it, but too much of it is not a good thing," Faress said.
In a procedure, doctors fitted Epperson with a vest that shook his lungs, then pumped 30 liters of salt water through each lung.
Epperson is breathing, and talking, like a new man.
"Being able to just get up and do little things was tremendous for me," he said.
There are two types of adult pap. Epperson had the primary form, which is caused by an autoimmune response. A secondary form can be caused by exposure to toxins from the environment or associated with blood malignancy.
Pulmonary alveolar proteinosis, or PAP, is a disease that causes a protein called surfactant to build up in the air sacs of the lungs, making it difficult to breath. Surfacant is needed to keep the lungs from closing off when we exhale but, too much can be harmful to your lungs. The rare disorder affects people ages 30 to 50, and is more common in men. There are two kinds of the condition that affect adults, known as secondary PAP and acquired PAP. Acquired is the most common type of the disease, and accounts for about 90 percent of all cases. A third type, called congenital PAP is diagnosed in children, and is generally linked to a genetic mutation that affects the gene encoding process of surfactant. (Source: papfoundation.org/forthemedia.html)
CAUSES: The cause of pulmonary alveolar proteinosis is unknown. It can occur with lung infection or an auto immune problem. It can also occur with certain cancers of the blood system or after exposure to high levels of silica or aluminum dust. There may be no symptoms, but sometimes there can be cough, fatigue, fever, shortness of breath, or weight loss. (Source: thorax.bmj.com/content/55/1/67.long)
NEW TECHNOLOGY: A procedure called a lung lavage can now help treat the effects of PAP. A whole lung lavage is done with general anesthesia and intubation with a specific tube allowing ventilation of one lung, while the other lung is repeatedly filled with 30 liters of saline. The liquid is then drained to clean out the material from the air sacs. The lavage is repeated in the other lung, either during the same procedure or at another time, depending on the experience and approach by the doctors. The procedure takes two to five hours to complete. Because of all of the fluid that is pumped in and out, the patient is given a water pill after the procedure to get rid of any water that is left .Risks include a drop in oxygen levels and infection, although 40 percent of patients only need one lavage. The other 60 percent may need to repeat the lavage until the surfacant is gone, as it can come back. (Source: Dr. Jihane Faress)
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