February 12, 2010

Soldiers’ mysterious lung disease identified

Featured Image

U.S. Army veteran Jimmy Williams has experienced lung problems since serving in Iraq. (photo by Joe Howell)

Veteran talks about his lung disorder

Veteran, Jimmy Williams, came to VUMC on Jan. 29 to undergo advanced lung function testing. He talks about his lung disorder and why he has agreed to have surgical lung biopsy.

Soldiers’ mysterious lung disease identified

As the Chief Warrant Officer in charge of maintenance for the Blackhawk helicopters flown by 101st Airborne soldiers in Iraq, 52-year-old Jimmy Williams says he always felt protective of the young servicemen and women he worked with overseas.

Now, Williams has come to Vanderbilt University Medical Center seeking an invasive procedure — one that won't do anything at all for his health. But it’s one he hopes might help some of those younger soldiers.

He is preparing to undergo surgical removal of a small section of lung for biopsy to explain why he and many of his fellow soldiers can no longer breathe like they used to.

“I never used to get exhausted. Now when my wife and I go for walks I have to tell her to slow down. Simple work around the house or yard, or even playing with our grandchildren, makes me so short of breath I have to lie down and rest,” Williams said.

Williams is the typical example of what may be an emerging profile: a soldier who was fit, a lifelong non-smoker, and who returned from deployment in Iraq with permanent lung damage.

Since 2004, physicians serving the Fort Campbell Army base have been referring dozens of soldiers with exercise-induced shortness of breath to Vanderbilt, to see Robert Miller, M.D., associate professor of Allergy, Pulmonary and Critical Care Medicine.

Jimmy Williams and his wife, Ruth, talk with Robert Miller, M.D., who diagnosed Williams’ condition.

Jimmy Williams and his wife, Ruth, talk with Robert Miller, M.D., who diagnosed Williams’ condition.

The soldiers had already received all the conventional tests for shortness of breath: lung imaging, pulmonary function and exercise tests. Almost all of the tests were normal, yet the soldiers reported, inexplicably, that they could no longer catch their breath when they exerted themselves.

The soldiers also shared similar stories of exposure in Iraq to massive amounts of smoke from sulfur fires in 2003, or breathing air fouled by sand and smoke from burn pits all over the country. Miller began to wonder if conventional testing might not be enough. He made what he calls an “unconventional” move and recommended surgical biopsies.

“So far, all but a few of these soldiers we have biopsied have had constrictive bronchiolitis,” Miller said.

Constrictive bronchiolitis, also called Bronchiolitis Obliterans, is a narrowing of the tiniest and deepest airways of the lungs. It is rare, and can only be diagnosed through biopsy. Cases that have been documented in the medical literature show striking similarities to what is seen in the soldier's biopsies.

“These are inhalation injuries, suffered in the line of duty,” said Miller.

In 2008, Miller and pulmonary/critical care fellow, Matthew King, M.D., pulled together the first round of what they believe is solid evidence that soldiers are returning with serious and permanent lung injuries related to their service.

Most of the first patients biopsied were 101st Airborne soldiers who fought the Mishraq Sulfur Mine fires in 2003. Later, many soldiers reported exposure to burn pits, especially a massive, 10-acre burn pit in Balad, Iraq.

Curtis Boyd, left, here with his father, Rick Boyd, also experienced lung problems after serving in Iraq. (photo by Joe Howell)

Curtis Boyd, left, here with his father, Rick Boyd, also experienced lung problems after serving in Iraq. (photo by Joe Howell)

“We slept an eighth of a mile from the burn pits,” Williams said. “Those fires burned the whole year, just huge bonfires where they burned metal, tires, trucks, human waste, everything.”

Williams retired as the Division Aviation Maintenance Officer after his last tour in 2008, and he continues to work as a contractor for the Army. His papers list a few health conditions related to his 32 years of service.

He is already guaranteed some lifelong compensation for a bad back and knees, but there is no mention of a lung condition. He says it concerns him greatly, and he knows other soldiers in the same situation.

“It's an injury, but it's on the inside. Something they can't see. But it has changed me,” Williams said.

Making the connection

In 2007 The Army Public Health Command took notice of VUMC's work and requested information from Miller. At that time, the Army launched an investigation of its own, saying VUMC's evidence created a “plausible connection” between exposures during deployment and respiratory problems in some soldiers.

Miller and King presented their evidence at an American Thoracic Society (ATS) meeting in May 2008. Of the original group of 56 soldiers who came back from Iraq with unexplained exercise-induced shortness of breath, 26 of 31 lung biopsies performed at Vanderbilt showed constrictive bronchiolitis.

“In every war there is a unique health syndrome. It is possible that whatever is causing the shortness of breath will be the ‘agent orange’ of this war,” Miller said.

The evidence presented at the ATS meeting garnered precious little attention from the medical community. Meanwhile, a steady number of soldiers continued to find Vanderbilt's work online, including Sylvia Waters-Moujan, M.D., an Army Staff Anesthesiologist based in Texas.

“When I couldn't even run a mile because of the shortness of breath and my chest hurting, I was scared. I thought ‘My God, something is wrong,’” said Waters.

The former marathon runner and mother of 7-year-old twin daughters returned from deployment in Mosul in 2007 unable to pass her military physical fitness testing. Even her colleagues at the Army hospital couldn't help her pinpoint what was wrong. When Waters heard about Miller's work she came to Vanderbilt in 2008. Her biopsy confirmed constrictive bronchiolitis.

“As a medical officer, I am considered fit for duty because I can still work in the O.R., even if it is only one day per week,” Waters said. “But my future is uncertain. Once I leave the service it could be very difficult to get medical coverage because of my preexisting medical condition.”

Miller says he is concerned soldiers continue to be tested for shortness of breath across the country using only conventional methods. He says surgical biopsy and definitive diagnosis are required just to create the possibility of proper compensation, but even then, there is no guarantee.

“Even with positive biopsy, disability ratings have been highly variable,” Miller said.

Miller's Vanderbilt colleagues, who perform the operations and pathologic exams, say they are on board to continue, but they agree with Miller's own assessment that biopsy for these soldiers is “unconventional.”

“At first I wasn't sure that surgical biopsy was required, as the results were unlikely to change the course of treatment,” said Eric Lambright, M.D., assistant professor of Thoracic Surgery. “But it turns out (Miller) was right and the information is rather convincing. We have to ask ourselves, ‘what is our responsibility as a nation to these soldiers?’”

“It was gutsy,” said Joyce Johnson, M.D., professor of Pathology, of Miller's first orders for lung biopsy. “In that early series, with fewer than 10 soldiers, even the military didn't believe our results. Finally I got a colleague at the AFIP (Armed Forces Institute of Pathology) on the phone and talked with her about the slides, and sent them up for her review. They are starting to listen, but we need broad, national recognition that this is a complication of being in this theater.”

p>Lambright says he continues to be concerned about the risks of the surgical procedure, which generally requires a two-day hospital stay, because even with a diagnosis, there is no specific therapy for the disease.
“Hopefully, we will be able to diagnose this without a need for surgical biopsy,” Lambright said.


King, Miller, Johnson and Lambright are submitting their work to medical journals in the hopes it might galvanize medical and military support for widespread research and testing. In October 2009, Miller testified before Congress, hoping to garner federal support.

In a statement e-mailed to VUMC this week, Coleen Baird Weese, M.D., environmental medicine program manager, U.S. Army Public Health Command (Provisional), said the Army investigation has turned up no specific evidence that exposure to the sulfur fires in Mishraq increased pulmonary risk for soldiers, but there is evidence that deployment itself has increased respiratory complaints from soldiers.

On Feb. 19, Miller, King and other Vanderbilt physicians will join fellow pulmonary experts at National Jewish Health Hospital in Denver for a first-ever, collective discussion group entitled “Post-deployment Respiratory Health Issues.” Military health officials plan to attend as well.
King says the immediate hope is to better define the problem, or problems, and to devise a non-invasive way to diagnose permanent lung damage in soldiers.

But until that happens, Miller says he will continue to counsel soldiers who come to him requesting further testing. While there is no specific treatment for the disease, for now, biopsy can help to educate soldiers and their families as well as physicians who care for other soldiers with similar complaints.

Williams, who is scheduled for surgery later this month, says the discomfort and hospital stay were worth it, even if it changes nothing for his health.

“Soldiers have to be there for each other, whether in combat overseas, or right here at home. I want somebody to realize this is a problem, especially for the young guys. I am willing to sacrifice for them,” Williams said.

Diagnosis, treatment among lung disorder’s challenges

by Carole Bartoo

Constrictive bronchiolitis is an untreatable thickening of the tiniest airways deep in the lungs. It can be the temporary effect of a respiratory infection in children, the permanent result of rheumatoid arthritis, or a deadly side effect of lung transplant.

It is also well known to be the result of toxic inhalation. The condition made headlines a few years ago after workers at a microwave popcorn plant developed constrictive bronchiolitis (called “popcorn lung” in media reports) from inhaling butter-flavored chemicals.

The trouble with diagnosing the disease is twofold. The major symptom — shortness of breath — occurs only with exercise or exertion.

“The symptoms these people are reporting seem out of proportion with the degree of disease seen with the standard tests,” said Joyce Johnson, M.D., associate professor of Pathology.

“That's because these airways are normally closed at rest. They open when we need extra oxygen, like during exercise. In these patients, the tiniest, innermost airways are stiff, like a garden hose.”

The other problem is that current tests, like lung pulmonary function tests (PFTs) are designed to diagnose trouble in larger airways, like with asthma, and while CT scans and X-rays turn up tumors or large pockets of fluid, misplaced air or scar tissue of other diseases, they do not typically detect the microscopic scars characteristic of constrictive bronchiolitis.