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Title: WHO Calls For Screening Of Emphysema, Chronic Bronchitis And Asthma
URL: http://www.pslgroup.com/dg/2888E.htm
Doctor's Guide
May 20, 1997


WEST HAVEN, Conn., May 19, 1997 -- A genetic disorder that can lead to a severe and potentially lethal form of emphysema is surprisingly common, according to a recent report of the World Health Organization (WHO). Based on its estimated gene frequency, the disorder -- known as alpha1-antitrypsin deficiency (A1AD) -- is one of the most prevalent hereditary disorders among people of Northern European descent. A1AD may affect as many as 100,000 Americans, but only a small number have been diagnosed.

As Common as Cystic Fibrosis

WHO estimates that as many as 100,000 Americans, or one in 2,500, have severe A1AD caused by an inherited inability to produce sufficient quantities of an important protein that protects the lungs. Of this number, 40,000- 60,000 people are estimated to have chronic bronchitis, asthma and/or emphysema caused by A1AD. To date, however, just 3,500 individuals have been identified.

"A1AD is approximately as common as cystic fibrosis, but it is less frequently recognized because it has long been considered a rare disorder," said Robert A. Stockley, M.D., of Queen Elizabeth Hospital in Birmingham, England, and a principal investigator of the ADAPT study. "Prolonged delays in diagnosis often mean that, for many patients, serious lung disease progresses unchecked."

Early Symptoms Often Misdiagnosed

Emphysema is the most prevalent clinical disorder associated with A1AD and the most frequent cause of disability and death. Because A1AD is thought to be rare, its early signs and symptoms are often misdiagnosed as allergies, asthma, or chronic bronchitis. According to studies cited in the WHO report, patients experience an average delay of more than seven years between the first onset of symptoms and the initial diagnosis of severe A1AD.

"By the time of diagnosis, it is often too late to prevent disabling lung disease in patients who have this problem," said Edward Campbell, M.D., of Salt Lake City, Utah, who is the other principal investigator for the ADAPT study. "Earlier detection is important because there is much that individuals with A1AD can do to help prevent or delay lung disease progression."

Disease Prevention and Treatment

The WHO report recommends that all patients with chronic bronchitis and/or emphysema and adults and adolescents with asthma be screened for A1AD, which can be done using a simple blood test. Individuals with A1AD should not smoke, which greatly accelerates disease progression, and should also have adequate immunization against influenza and pneumococcal pneumonia. Respiratory infections should be treated promptly. Patients should take care to exercise and maintain a healthy, nutritious diet, and avoid environmental and occupational air pollutants.

Although there is no cure for A1AD-related emphysema, replacement therapy with human AAT (also called augmentation therapy) raises the level of AAT in the blood and in the lung. Based on studies of biochemical efficacy, augmentation therapy has been widely recommended for A1AD individuals with established emphysema. Therapy must be administered intravenously on a regular basis and taken throughout a patient's life for its biochemical effect to be sustained.

Unique Research Initiative

Much about A1AD is poorly understood, and there is an urgent need for further research to continue advancing researchers' understanding of the disease and optimal treatment, according to the WHO Report. To meet this need, Bayer has funded the establishment of a new Lung Resource Center at Queen Elizabeth Hospital in Birmingham, England, and a major new clinical research initiative to be conducted there. Thousands of people will be screened for A1AD as part of the study, known as ADAPT (Anti-trypsin Deficiency Assessment and Programme for Treatment), and investigators will seek to determine optimal treatment regimens for A1AD individuals.

Bayer Corporation is supporting a major new clinical research initiative and a global awareness campaign to address two priorities identified by WHO in its report: the urgent need for further research, and screening to detect A1AD in patients who may be misdiagnosed with asthma or chronic obstructive pulmonary disease (COPD), including chronic bronchitis and emphysema.

"Bayer has been committed to understanding the complexities of A1AD and providing patients with treatment options for more than a decade," said Jan Turek, Senior Vice President and General Manager for Bayer's Biological Products unit. "By demonstrating how widespread A1AD actually is and its impact on patients, the WHO Report makes clear that we must continue our efforts to both learn more and educate others about this serious disease."

In the U.S., Bayer Corporation has also supported a multicenter registry sponsored by the National Heart, Lung, and Blood Institute of the National Institutes of Health. The study is assessing the natural history of A1AD, including its effect on lung function and mortality, in individuals receiving and not receiving intravenous augmentation therapy with alpha1-proteinase inhibitor (human) (Prolastin(R)). A report from this observational study is anticipated in late 1997.

Bayer's worldwide biologicals business includes plasma-derived as well as biotechnological (recombinant) products. The business is a part of Bayer Pharmaceuticals, a division of Bayer Corporation. Bayer Corporation is a research-based company with major businesses in life sciences, chemicals and imaging technologies.

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