A Multidisciplinary Effort to Save a Woman with a Rare Lung Condition

Donna-PAPDonna M. Hall arrived at Jefferson University Hospitals at the edge of life.

Her body was producing excess amounts of a natural surfactant – a fluid necessary in small amounts to transfer oxygen and carbon dioxide in the lungs. The fluid was clogging both of Donna’s lungs – she was literally drowning.

Donna had an advanced case of a very rare lung disease known as pulmonary alveolar proteinosis (PAP), which affects 1 in 100,000 people in this country. The cause is largely unknown.

The condition had reached a critical state for the 45-year-old mother of four from Delaware County. Both her lungs were clogged with the fluid.

She was transferred to Jefferson because of the expertise of Mani Kavuru, MD, director of the Division of Pulmonary and Critical Medicine and one of the world’s top experts on PAP, and his colleague pulmonologist Boyd Hehn, MD.

“Donna’s disease was extremely advanced,” said Dr. Hehn, who was waiting in the intensive care unit (ICU) when Donna arrived at Jefferson. “She came in on a ventilator working at about 90 percent of capacity. We had to act quickly to clear the surfactant.”

The treatment for PAP involves washing of the lungs repeatedly with a saline solution to remove the excess surfactant – called a whole-lung lavage. But to perform a lavage, the lungs must be shut down one at a time.

“That was not an option,” said Dr. Hehn. “Donna did not have one lung that could work independently as we cleaned the other. We had to find another way.”

The pulmonologists quickly turned to Nicholas C. Cavarocchi, MD, FACS, FCCP, director of Surgical Critical Care at the Hospitals and leader of our extracorporeal membrane oxygenation (ECMO) team. ECMO provides respiratory and cardiac support to patients whose lungs and/or hearts cannot function on their own.

“It was the only way to keep oxygenated blood circulating for long enough to drain both lungs,” said Dr. Cavarocchi, who along with another cardiothoracic surgeon Hitoshi Hirose, MD, PhD, FACS, joined Donna’s team of physicians and other providers.

“Our team has been trained to deliver a level of complex critical care that is sophisticated and consistent,” said Dr. Cavarocchi.

ECMO had only ever been used a few times in the country for a lavage, with none of the patients surviving the procedure.

“It was the only option to save her,” said Dr. Hehn.

Dr. Cavarocchi and his team deliver the best survival rates in Pennsylvania — 70 to 80 percent — for patients with serious lung and/or heart dysfunction.

The ventilator provided Donna with oxygen while ECMO kept oxygenated blood circulating throughout her body for the 12-hour procedure. Each lung was isolated, flushed with saline solutions and drained multiple times until the surfactant cleared.

The procedure would need to be repeated again over the next two weeks to completely clear the lungs. Eight weeks later, Donna was weak but off the ventilator and breathing on her own.

It was a great success – for Donna and for her multidisciplinary team at Jefferson that included physicians from pulmonary and critical care, cardiothoracic surgery, anesthesia, nursing and more who mobilized quickly to save a critically ill patient.

After more than two months in the hospital, Donna was able to return to her home in Clifton Heights, Pa. She needs to be closely monitored as her recovery continues.

“We don’t know what caused Donna’s PAP or if and when it will return,” said Dr. Hehn.

Despite those concerns, her Jefferson team is pleased to have gotten her to this point.

“It is almost unheard of in someone with such advanced disease,” said Dr. Kavuru.

“The team at Jefferson saved my life,” she says. “I am forever grateful.”

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