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,” Robert Epperson told Ivanhoe.
“He was very sick. He was very short of breathe,” Jihane Faress, MD, in the Pulmonary Medicine division at Cleveland Clinic and Epperson’s doctor, told Ivanhoe.
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,” Dr. 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.
“So you put it in and then you wash out. And you continue to wash because the stuff you get back looks very thick, almost like mayonnaise, and they get clearer and clearer as you wash out more lung,” Dr. Faress explained.
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.
BACKGROUND: 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: http://www.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: http://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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Jihane Faress, M.D., Pulmonary Critical Care Specialist and Assistant Professor of Medicine at Cleveland Clinic, talks about a new way to treat lung conditions.
Hoe was Robert Epperson when he came in to you?
Dr. Faress: He was very sick, he was very short of breath. He could barely speak and he was his face was very puffy because of all the Prednisone that he had been on and he was very emotional. He thought he was going to need a lung transplant because his disease was getting worse and worse over the last year.
But he was told he had one disease what was that?
Dr. Faress: Correct, he was told he had a disease called sarcoidosis.
What is that?
Dr. Faress: This is a type of inflammation that happens in the body. It affects the lung most of the time and we don’t know exactly what causes it but it’s mainly an inflammation in the lung.
Did you know immediately when he came to you that it wasn’t sarcoidosis?
Dr. Faress: I expected to see in his CAT scan, because he was not getting better, a lot of scarring. And when I started looking at the CT scan I didn't notice scarring and the shadows on the CAT scan didn’t look anything like sarcoidosis. And the fact he was not responding to very aggressive medication that we typically use for sarcoidosis made me think this has to be something else. Although the biopsy that he had done suggested sarcoidosis, I thought this had to be something else. And the more I looked at his CAT scan, i looked like alveolar proteinosis.
What is pulmonary alveolar proteinosis?
Dr. Faress: This is also a disease that affects only the lung, and it’s a problem with surfactant. Surfactant is a substance that people need to keep the alveolar sacs from closing off when we exhale. Too much of it is not a good thing. When too much of it fills our alveolar sacs, it makes it very hard on the oxygen to cross through these alveolar sacs to the blood and you’re going to lack a lot of oxygen.
So it stops your oxygen from going to the blood which would do what to your body?
Dr. Faress: It’s going to make you short of breath, your brain is going to lack oxygen, and your heart is going to lack oxygen. It’s going to put a lot of stress on all of your organs.
How would you normally treat it?
Dr. Faress: The treatment of Alveolar proteinosis depends on the severity. Some people who have a mild form of it may just be watched. Some people like Mr. Epperson had a very severe form. The main treatment for it is washing out all the material that’s stuck in there and filling those alveolar sacs.
What happens during the procedure?
Dr. Faress: It’s not simple. It’s a procedure that’s done under general anesthesia. Since they’re going to do both lungs they have first to make sure that one lung is getting the oxygen and breathing fine so they can work on the other lung. And they put a lot of water. For example, in Mr. Epperson’s case, they put around thirty liters of fluid. That’s a lot of fluid. So you put it in aliquots. The patient chest is fitted with a percussion vest to shake the lungs. The fluid is then drained by gravity. The fluid we get back is very thick at the beginning, like almost mayonnaise. It gets clearer and clearer as you wash out more of the lung. So you continue to do that until it’s completely clear.
It’s just saline water?
Dr. Faress: it’s warm sterile saline water. You put it in and you wash it out.
How do you put it in?
Dr. Faress: They put it through a long catheter placed inside the breathing tube.
Is it dangerous to put thirty liters of fluid in someone’s lungs?
Dr. Faress: Correct but you suction it almost all out. At the end of the procedure We actively suction what was not drained. There still could be a little bit left in there, so typically we’ll give people a water pill to get rid of what’s left. And sometimes people after the procedure might get worse because of that water. But this typically will go away. It will reabsorb.
Is there any risk?
Dr. Faress: There is a risk of general anesthesia and a risk of we could introduce an infection. There is a risk that the oxygen levels could drop. But if it does then we will stop the procedure. These are the main ones.
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