Marfan Syndrome Community Benefits from the Legacy of Jonathan Larson, RENT Playwright

Marfan Syndrome Community Benefits from the Legacy of Jonathan Larson, RENT Playwright

RENT, the Movie, Provides Forum to Address Marfan Syndrome; Larson Family Supports Education Campaign to Prevent Other Deaths

PORT WASHINGTON, N.Y., Nov. 14 /PRNewswire/ -- His hit Broadway show, RENT, featured characters struggling with, and dying from, AIDS but it was Marfan syndrome, a disorder of the body's connective tissue, that claimed the life of playwright Jonathan Larson before RENT opened on the Great White Way.

According to medical experts, tens of thousands of people who have the Marfan syndrome are not diagnosed. As RENT opens in movie theaters nationwide on November 23, Jonathan's family, in conjunction with the National Marfan Foundation, is hoping that more people will learn about the Marfan syndrome and get the diagnosis and treatment they need to avoid the fatal episode that struck Jonathan.

In January 1996, in the few days before the final rehearsal of RENT, Larson had visited the emergency departments at two different hospitals complaining of excruciating chest pain. One sent him home with a diagnosis of food poisoning; the second diagnosed a mild case of flu. In the middle of the night, without the emergency surgery that could have saved his life, Jonathan died alone in his New York City apartment. It was ten days before his 36th birthday.

The cause of the chest pain, and Jonathan's death, turned out to be an aortic dissection, a tear in the large artery near the heart. Only after his death did Jonathan's family and friends learn that his tall, lanky frame, indented chest bone, flat feet, long fingers and toes, and flexible joints should have led doctors to a possible Marfan syndrome diagnosis, which would have indicated a need for additional testing and immediate surgery. The survival rate for emergency surgery to repair a torn aorta is more than 90 percent.

"We cannot bring back Jonathan, but it's not too late for others," said Al Larson, father of the Tony Award and Pulitzer Prize winning playwright. "Many people have told us that RENT changed their lives. For those who now become aware of the Marfan syndrome and receive proper medical care as a result, the film can truly be life-saving."

Marfan Syndrome: Not A Death Sentence

While the skeletal problems associated with the Marfan syndrome are not life-threatening, the fragile connective tissue in the aorta makes it prone to enlarge and tear in affected people. As a protective measure, beta blocker medication can be used to slow the heart rate and reduce blood pressure, thus reducing the stress placed on the fragile aortic wall. Even when they are on this medication, people with the disorder have their heart and valves evaluated every year -- usually with echocardiography -- to monitor the size of the aorta. When it reaches a critical size -- 5.0-5.5 cm -- surgery is performed to replace the enlarged portion of the aorta. When the surgery is done before the aorta dissects, it is 98 percent effective; in emergency situations (after the aorta dissects), it is still successful in more than 90 percent of cases (N Engl J Med 1999;340:1307-13.).

Due to advances in diagnosis, medical treatments and surgery, the average life span for people with the Marfan syndrome is in the 70's (Am J Cardiol 1995;75:157-160). However, without a diagnosis and treatment, affected individuals are at risk of a sudden and early death as a result of a dissected or ruptured aorta. Jonathan Larson was only 35 when he died.

"Despite the advances in the diagnosis and treatment of Marfan syndrome, people who do not know they have the disorder are at high risk of aortic dissection and death without any prior warning," said Hal Dietz, M.D., Victor A. McKusick Professor of Medicine and Genetics and Investigator, Howard Hughes Medical Institute, Johns Hopkins University School of Medicine. "It is incumbent upon emergency physicians to consider aortic dissection in people who present to the emergency room with chest, back or abdominal pain in the absence of another obvious cause. Physicians must learn to recognize the outward features of Marfan syndrome. This can help raise the index of suspicion."

Larson Family Supports National Marfan Foundation's Emergency Medicine Campaign

Preventing deaths in the emergency department from undiagnosed Marfan syndrome and unrecognized and untreated aortic dissection is the objective of the NMF's Emergency Medicine Campaign, which was developed with the support of the family of Jonathan Larson. The goal is to educate emergency department personnel about the risk factors for aortic dissection and how to correctly recognize, diagnose and treat a dissection.

With the support of the Larson family, the NMF has developed and disseminated an array of medical education materials, including a CME-accredited video for physicians, Emergency Diagnosis and Treatment of Aortic Dissection; a booklet and PowerPoint presentation that is used in grand rounds presentations; a poster for non-patient areas of hospital emergency departments; and an education advisory for pre-hospital emergency providers about treating aortic dissection that was developed in conjunction with the State Emergency Medical Advisory Committee, New York State Department of Health.

The Larson family has also funded advocacy tools for people with the Marfan syndrome, including an Emergency Alert Wallet Card that was disseminated to more than 30,000 people with the Marfan syndrome.

The National Marfan Foundation

The NMF was founded in 1981 to provide accurate and timely information about the disorder to patients, family members and physicians; to serve as a resource for medical information and patient support; and to support and foster research.

For more information on the Marfan syndrome, contact the NMF at 800-8-MARFAN or http://www.marfan.org/.

Source: The National Marfan Foundation

CONTACT: Eileen Masciale, NMF Director of Communications,
+1-631-665-2163, publicity@marfan.org

Web site: http://www.marfan.org/

NOTE TO EDITORS: Local Marfan patients and doctors may be available for interview. If you are interested, please contact Eileen Masciale (631.665.2163) for more information.

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