A 78-year old woman, tall, thin, Caucasian, and reserved in manner goes to her doctor for an annual checkup. Her physician notices that she is frequently suppressing a cough. It turns out she’s been struggling with it for more than a year. Subsequent diagnosis showed she had Lady Windermere syndrome, a lung infection related to bronchiectasis (BE), a chronic airway disease. Suppressing her cough may have made this woman more susceptible to a particular bacterial infection.
“I have seen some women who have bronchiectasis develop an infection from Mycobacterial avium complex,” says pulmonologist Gerard Frank, MD, UCLA Medical Center. “The bacterium is in the same family as tuberculosis, but unlike TB, is not contagious.”
In 1992, two researchers coined the term Lady Windermere syndrome, which comes from a character in an Oscar Wilde Victorian-era play. They used the term to imply that some women may feel that coughing in public is socially unacceptable. The researchers’ paper focused on six older women who had no significant history of smoking or underlying pulmonary disease, yet they developed a pulmonary infection from the Mycobacterium avium complex (MAC) pathogen. The researchers theorized that the habit of suppressing the cough (which helps clear the lungs of mucus) predisposed these women to MAC infections. MAC bacteria are common in the environment, yet most people don’t get sick from them. Neither Lady Windermere syndrome nor BE is contagious.
Causes of BE
It is not entirely known what causes BE. But, as Dr. Frank explains, the mucus glands in the lungs become more active, which is partly why in severe cases people are coughing up mucus all the time. “They also can’t effectively clear the mucus,” says Dr. Frank. “The inability to clear it creates a breeding ground for infection, leading to a vicious cycle of frequent infections, chronic inflammation and ongoing structural damage.”
BE may result from poorly treated or untreated lung infections from such pathogens as bacteria, viruses, or fungi. The pathogens damage airway walls, making them wider, flabby and scarred. Thick mucus piles up and sticks to the walls. The condition is also caused by cystic fibrosis, an inherited disease caused by an abnormal gene. BE is slow-growing, progressive and mostly found in older white women. Because it’s not typically diagnosed until it has advanced, it’s possible the disease is more prevalent than previously thought.
No Longer Rare
In the United States, a rare disease is defined as affecting 200,000 or fewer people. A study published in Chest Journal, a publication of the American College of Chest Physicians, reports that this disease has surpassed the threshold of what can be considered rare. Researchers came to that conclusion by analyzing Medicare prescription drug plans. Newly diagnosed patients were a mean age of 76 years, predominately women (65 percent), and predominately white, non-Hispanic (84 percent). The study identified more than 250,000 Medicare patients with a BE diagnosis over a nine-year period. Thus, study authors concluded that BE is likely no longer a rare disease in the U.S. and the upswing of disease found in the aging population deserves further attention.
Diagnosis and Treatment
Because the symptoms of BE (daily coughing with mucus production) are similar to other lung diseases, such as asthma, COPD, and pneumonia, physicians will look for conditions associated with BE, such as recurring or untreated respiratory infections or cystic fibrosis. Mucus is typically examined for infection and cultured to determine the type of pathogen (usually bacterial) causing the illness. A computed tomography (CT) scan is considered the gold standard for confirming a diagnosis and assessing the extent of lung damage.
There is no cure, but BE can be successfully managed. Treatments are based on underlying causes. As bacterial infections are common, antibiotics are frequently prescribed. Drinking plenty of fluids can help thin airway mucus, enabling easier clearance. Expectorants and mucus-thinning medications, often with a nebulizer, may be prescribed. Another mucus-clearing strategies include chest physical therapy, which involves tapping on the chest and back (usually done by a respiratory therapist).
People with BE are more prone to anxiety and depression and may be embarrassed to attend social outings due to frequent coughing. Support groups can help. Find them at inspire.com, or at 1-800-LUNGUSA. Both of these are resources of the American Lung Association.
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