Robert Epperson, a 44-year-old engineer from Virginia, could barely get off the couch or speak more than a few words because he'd get so winded. But today, he is breathing easy again after having his lungs washed.
Doctors told Epperson he had sarcoidosis, a disease that causes inflammation in multiple systems of the body, but mainly in the lungs and lymphatic system. He had seen several specialists and was taking medication that had no effect.
After his second bout with pneumonia in May 2013, he was admitted to the local emergency room, treated and sent home and put on oxygen.
“I went completely downhill from there. I couldn’t put a sentence together without gasping for breath,” said Epperson, a father of two boys, 21 and 14. “I had absolutely no energy. ... During that time, I would go from my bed to my desk at work to bed.”
Just before Thanksgiving of 2013, he sought help at the Cleveland Clinic and was diagnosed with pulmonary alveolar proteinosis, or PAP, a disease in which protein and lipid material called surfactant builds up in the air sacs. Typically, it strikes 20- to 40-year olds, and is often mistaken for other diseases and not treated correctly.
Epperson underwent a procedure called lung lavage, which while not new is used exclusively for PAP, which can be tricky to diagnose, according to Epperson's surgeon, Dr. Basem Abdelmalak, director of anesthesia for bronchoscopic surgery at the Cleveland Clinic.
He was fitted with a chest percussor, a vest that shook his chest, while doctors intubated him, allowing one lung to breathe for two, and literally washed the other lung with a saline solution, cleaning out the excess surfactant from his air spaces. Then they switched lungs and washed the second one, all in the same procedure.
At the start, the drained liquid is a dirty brown color, but hours later, it’s clear. For Epperson, doctors cleaned about 30 liters.
There are risks, which is why lung lavage must be done in a specialty clinic with a team that includes not only a surgeon but a radiologist, anesthesiologist and pathologist.
Epperson has the primary form of PAP, which is caused by an autoimmune response; a secondary form can be triggered by environmental exposure.
Patients with PAP have too much surfactant coating the alveoli in their lungs. Premature babies with respiratory distress syndrome have the opposite problem, having too little surfactant.
“Normal surfactant keeps the airways open,” said Abdelmalak. “The blood that comes through the thin walls exchange with the air and that’s how we get oxygen and get rid of carbon dioxide. The altered surfactant protein makes this wall very thick, so the air doesn’t exchange well. There is a lack of oxygenation, and the patient becomes short of breath and fatigues quickly, and is barely able to leave the house.”
Epperson credits his pulmonologist at the Cleveland Clinic, Dr. Jihane Faress, with making the definitive diagnosis of PAP.
“We see a lot of misdiagnoses of patients who are labeled sarcoidosis, but have something else,” she said. “That’s why it’s very important not to ignore how a patient is doing clinically and what an X-ray looks like. A diagnosis is not just made by a biopsy, we put it all together in these complex cases.”