Patients with rare lung disease get a good wash

UCHealth University of Colorado Hospital is a top center for a procedure that cleanses the lungs of a dangerous protein build-up
November 15th, 2017

 

When Ashley McAfee moved from Atlanta to Denver in 2011, she began to feel the effects familiar to most who suddenly climb several thousand feet in altitude: shortness of breath and fatigue after what she considered normal exertion.

The symptoms were unpleasant but typical and McAfee, now 31, expected them to diminish. She was physically fit and active. She grew up playing softball and running track and practiced yoga. But in her case, the discomfort worsened over time.

“I was having a really hard time acclimating,” said McAfee, 31. “Simple things like going from the couch to the bathroom, I’d get exhausted.” She tried to stick with her normal activities, but couldn’t keep up. She also lost about 10 pounds.

About a year after the move, McAfee’s body delivered an even more powerful message. In the midst of a yoga class, the instructor asked her to step out of the room.

Ashley McAfee
With the help of lung lavage procedures at UCHealth University of Colorado Hospital, McAfee stays active, as shown here on a jaunt at the Garden of the Gods in Colorado Springs.

“My lips were blue,” McAfee said. Shocked, she quickly visited a physician, who measured her blood oxygen saturation and found it was in the 60s. It should be at least 90. She went immediately to an emergency room and landed in the hospital for three or four days of tests. Clinicians initially suspected a blood clot in the lungs, but had to rule that out, along with several other possibilities.

A pulmonary specialist looked at an X-ray of McAfee’s lungs and saw a leopard print of black and white patches. He sent her to an intensive care unit in the Denver area and referred her to James Ellis, MD, a pulmonary specialist who now practices at UCHealth University of Colorado Hospital After a lung biopsy, Ellis diagnosed McAfee with a rare condition known as pulmonary alveolar proteinosis, or PAP. It affects only about 7 of every 1 million peoplebetween 2,000 and 3,000 in the United States.

Battling the build-up

McAfee was among those unlucky few, but she had also found the right person to help her. Ellis, who delivers care at UCH through its Comprehensive Lung and Breathing Program, has been treating PAP for nearly 50 years and is uniquely qualified to fight a mysterious foe that as yet has no known cure.

There are three types of PAP – McAfee’s is the autoimmune form, by far the most common – but they all produce the same problem. In normal lungs, small air sacs called alveoli stay open with the help of an oily protein called surfactant. As oxygen passes through the sacs during breathing, cells signal the body to remove the surfactant. In people with PAP, the signaling goes awry, and the surfactant builds up, in effect gumming up the works in the lungs and making it harder for oxygen to pass through the alveoli to the blood. Breathing gets more and more difficult as oxygen levels sink. Left untreated, PAP can be fatal.

The solution is a technique known as lung lavage, or lung washing, to flush the surfactant build-up from the lungs. Clinicians insert twin endotracheal tubes into the lungs while the patient is under anesthesia. They stream saline into one while the other receives oxygen to keep the patient breathing. The saline breaks up the accumulated protein, while a vest wrapped around the patient’s chest shakes the lungs, helping the two to mix. Suction then removes the liquid from the lungs. The team repeats the procedure until the suctioned liquid runs clear, showing the excess protein has been removed completely then the process is repeated on the other lung.

McAfee had never heard of PAP before she met Ellis. She was scared and nervous about the procedure, which she described as “kind of rough” because of the prolonged intubation and the shaking. But it hasn’t discouraged her from repeating it every four to nine months because it allows her to get back to normal life for extended periods.

“It has helped me tremendously,” McAfee said. Last January, for example, she spent a weekend skiing with husband Charles (CJ) a little more than a week after her lung washing.

“It’s crazy how fast I see the results and how much better I feel,” she said.

A wash-day veteran

The difficulty other providers had in diagnosing McAfee’s PAP isn’t surprising, given its rarity. The Cincinnati-based PAP Foundation designates only about a dozen clinical centers for treatment – UCH is the sole provider in the Rocky Mountain region – based on their experience in treating the disease. No specialist has more experience than Ellis, who said he performed his first lung lavage in 1970 at the University of California San Francisco and estimates he’s done about 1,500 since – more than double anyone else in the world.

Ashley McAfee and husband, CJ,
Ashley McAfee and husband, CJ, keep a careful eye on her symptoms of PAP, a rare lung disease.

The disease was unknown until the early 1960s, Ellis said. It’s not known what triggered it. “It occurred spontaneously and worldwide,” he said.

Jose Ramirez-Rivera, MD, performed the first lung lavage to treat PAP at the Veterans Administration Hospital in Baltimore in 1966. He co-authored an article on the procedure and the disease the same year – it was one of just two that Ellis had read when he faced the challenge of doing one himself.

“It was a trial by fire,” Ellis recalled. He had no equipment to measure blood oxygen or fiber-optic imaging to guide him. Nor was there a percussive vest to shake the patient. The team did that by hand.

Ellis has all those tools at his disposal today, but uses the same basic technique to perform 30 to 40 lung lavage cases a year at UCH. The procedure consistently provides relief to PAP sufferers, he said. He provided 133 lavages over a 20-year period in one case, but the treatment is not necessarily a lifetime commitment for patients, including McAfee, he added.

“Patients I have taken care of, I see their need for lavage typically for four to six years, and it does remit spontaneously,” he said. Medications also now help to limit the illness, he noted. McAfee, for example, inhales nebulized leukine, a drug originally used for cancer patients, once a day to help clear the surfactant from her lungs.

While there are no answers as to the cause of McAfee’s PAP, the move to Denver probably played a role in its emergence, Ellis said.

“The altitude does make a difference from the standpoint of symptoms of shortness of breath,” he said. “It’s like you or I going up to 14,000 feet and trying to walk. It really does affect it.”

Perhaps the biggest stumbling block to treating PAP is the failure to recognize it, as McAfee’s case demonstrates, Ellis noted. Providers examining a patient with shortness of breath might make a diagnosis of pneumonia and prescribe antibiotics, for example, leaving the true problem untreated. The delay is typically six to nine months before a patient with PAP sees a pulmonologist for a bronchoscopy or lung biopsy to make the correct diagnosis, Ellis said.

Maintaining a normal life

For her part, McAfee has learned to manage her life with PAP. She has Fridays off from her job as manager of a dental office and schedules her lung lavages – she had one in early November – so she has the weekend to recover and avoid missing work.

“I know I can go in, rest for a few days and then be ready to go back,” she said.

Nor does she fight the disease alone. CJ also keeps a watchful eye on the signs of PAP push-back.

“He can usually tell that I’m having symptoms before I’m willing to admit it,” McAfee said. “He notices when I start clearing my throat and coughing, when I’m out of breath going up the stairs.”

She is grateful to get care from a specialist she trusts completely.

“I think the world of Dr. Ellis,” McAfee said. “He is very compassionate and understanding and I have 100 percent confidence in his ability to take care of me.”

The symptoms still build and “hit like a ton of bricks,” she conceded, and they affect her life. “But now I know what to do,” she said. Ellis’s care and lung lavage have helped McAfee get back to the ballfield, the hiking trail and the yoga mat.

“When I’m feeling good, I can still do all those things,” she said.

The help she received from Ellis illustrates the broad-reaching care offered by the Comprehensive Lung and Breathing Program, said its director, Tim Wimbish.

“It doesn’t matter if a patient has seasonal allergies or a rare and complex condition like PAP,” Wimbish said. “We can address those needs and determine the right treatment for each patient.”

About the author

Tyler Smith is a freelance writer based in metro Denver.