Breath of fresh air
New treatments and UVA's expertise is good news for those living with a lung disease
By Kathleen Phalen Tomaselli
For millions of Americans, breathing doesn't come easy. A wide range of conditions from emphysema to pulmonary fibrosis can alter the regular in and out rhythm most of us rarely think about. If you, or someone you love, has trouble breathing, here's what you need to know about the latest treatments and expertise offered at the University of Virginia Health System-ranked among the nation's top centers for treating respiratory diseases according to U.S. News & World Report.
Emphysema and Chronic Bronchitis
Shallow, uneven breaths make life with emphysema and chronic bronchitis (both known as COPD or chronic obstructive pulmonary disease) very difficult. It's estimated that more than 12 million people live with COPD, the fourth leading cause of death in the U.S., and women are more likely to die from the disease than men. In fact, women are more than twice as likely to be diagnosed with chronic bronchitis as males.
While emphysema is considered the most serious form of COPD, it often co-exists with chronic bronchitis. Both conditions progress over time and frequently are not diagnosed and treated until the disease is in an advanced stage. Chronic bronchitis comes from a long-term inflammation of the breathing passages, resulting in cough, mucous and other breathing troubles.
With emphysema, the elegant patterns of the lungs are destroyed either by smoking, pollutants or genetic factors. And as lungs lose elasticity and fragile air sacs are permanently damaged, air gets trapped inside; "Try breathing after you have filled your chest with air while only letting a little bit back out-that's what it feels like," says Jonathon Truwit, M.D., head of the Division of Pulmonary and Critical Care Medicine at the University of Virginia Health System.
Truwit and other UVA pulmonary experts are searching for new ways to help these patients get the air out (see Emphysema Clinical Trials below). One trial uses the Intra-Bronchial Valve (IBV) System, which are one-way, umbrella-shaped devices placed inside the lung's upper lobe, redirecting airflow to healthier portions of the lung. "Air gets out but can't get in," explains Truwit. "We'll see if patients feel better; see if shortness of breath improves." Another clinical trial at UVA (the Broncus EASE trial) uses stents to try to create new pathways in the lung for trapped air to escape.
Lung volume reduction surgery (LVRS), originally used in the 1950s, has reemerged for patients with upper lobe damage. In the procedure, surgeons remove the most damaged portion, hoping to restore lung elasticity. LVRS for this particular group of patients is proving successful, according to a national study. Patients with upper lobe damage and low exercise tolerance prior to surgery showed marked improvement. When the most damage is in the top part of the lung, says Truwit, "they do well."
Pulmonary Fibrosis and other Interstitial Lung Diseases
Interstitial lung diseases (ILD) are a group of nearly 200 illnesses. They have in common various degrees of lung injury, inflammation and scarring. Some ILDs occur in response to inhaled dusts or allergens, workplace exposures (such as farmers and miners), medications and connective tissue disease such as lupus or rheumatoid arthritis. The cause of other ILDs is not known.
Diagnosing ILDs is tricky; these illnesses are often mistaken for bronchitis and pneumonia. "The average delay in diagnosis is several years," says UVA pulmonologist Borna Mehrad, M.D. "And nearly 90 percent of my patients referred to our ILD clinic are labeled as pulmonary fibrosis. When we look into it further, we often find another diagnosis and are able to treat it. "Because UVA's ILD clinic treats hundreds of patients each year, diagnosis is more accurate and treatment more specific. "This is all we see," notes Mehrad.
Idiopathic pulmonary fibrosis, the most common ILD of unknown cause, is a serious condition that has increased significantly in recent years. Between 1992 and 2003, the death rate increased by more than 28 percent in men and more than 41 percent in women. There is no real treatment, but ongoing clinical trials offer some hope. "If you catch IPF early, patients are more likely to be good candidates for clinical trials, which may prevent progression," Mehrad says.
Bosentan, a pill already in use for pulmonary hypertension, is now being studied for IPF. "Perhaps it will change the course of the disease," he says.
Because IPF patients cycle in and out of stable and acute episodes, each worsening the disease, UVA physicians and scientists are studying ways to measure and predict these events before they happen. "We have found that, compared to healthy volunteers, patients with ILD have much higher numbers of a particular cell type in their blood," says Mehrad. "We now want to study whether the number of these cells in the blood can predict worsening of lung function in these patients. We hope this research will lead us to identify patients who will benefit from therapy that can help prevent irreversible damage."
Acute Respiratory Distress Syndrome
Within 24 hours of some kinds of trauma-a near drowning, a fire rescue, or a drug overdose-a cascade of deadly inflammation can happen, filling lung air sacs with liquid and causing them to collapse. You won't be able to breathe on your own.
This life-threatening phenomenon is known as acute respiratory distress syndrome (ARDS). It affects 150,000 people every year. Symptoms usually develop within 24 to 48 hours of the original injury or illness, and often occur along with other organ system failure.
The only treatment is mechanical ventilation, but UVA researchers have identified a molecular target, or receptor (CXCR2), that holds promise for the development of new drugs to treat the condition.
New mechanical ventilation techniques are also significantly lowering the number of deaths and number of days dependent on a ventilator, says Truwit. Called low tidal volume, this ventilator technique allows for less stretch of the lung sacs (stretching appears to slow lung tissue recovery and cause damage). Strategies that reduce the fluid volume in the body help lungs dry out faster and patients are on the ventilator two days less, Truwit explains.
Vitamin supplements and fish oil might also help; scientists are studying the effects of feeding ARDS patients foods rich in omega-3 such as salmon, and mega doses of vitamins, says Truwit. "Studies suggest less inflammation and, when there's less inflammation, there's more healing."
Emphysema clinical trials
Because of UVA Health System's extensive experience treating patients with emphysema, it is among only a handful of sites in North American selected to conduct pilot studies of the Intra-Bronchial Valve (IBV) System and the Broncus stent placement.
To learn more about these clinical trials now enrolling adults, call the trial coordinator, Peggie Donowitz, at 434-982-1801
Find UVA doctors who specialize in emphysema
This article appeared in the spring 2008 issue of Vim & Vigor, a family health magazine sponsored by UVA Health System. If you don't already receive it at home, sign up today. It's free.