Contact: Eileen Masciale
Consulting Director of Communications
631.665.2163
publicity@marfan.org

Legacy of Jonathan Larson, RENT Playwright, Continues

PORT WASHINGTON, NY, February 2, 2009 – The release of RENT: Filmed Live on Broadway by Sony Pictures Home Entertainment marks another chapter in the legacy of Jonathan Larson, the RENT playwright, as Marfan syndrome awareness gets another boost on the Blue-ray Disc™ and DVD.

Larson, who would have turned 49 on February 4, died from an aortic dissection – a tear in the artery that takes blood away from the heart – believed to be caused by Marfan syndrome the night before his revolutionary rock musical opened on Broadway. He had gone to two different New York City hospital emergency departments complaining of chest pain. One diagnosed him with food poisoning; the other blamed the 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.

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 could indicate Marfan syndrome, which would have indicated a need for additional testing and immediate surgery. People with Marfan syndrome are at up to 250 times greater risk of aortic dissection. The survival rate for emergency surgery to repair a torn aorta is greater than 90 percent.

A public service announcement about Marfan syndrome, featuring original Broadway cast member Anthony Rapp (“Mark”), is included in the extra features that accompany RENT: Filmed Live On Broadway. Signs of the disorder are shown and viewers are urged to visit the National Marfan Foundation website, www.marfan.org, to learn more. According to medical experts, about half of those with Marfan syndrome in the U.S. are not diagnosed.

“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 Marfan syndrome and receive proper medical care as a result, RENT continues to be life-saving.”


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 educational 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 Marfan syndrome, including an Emergency Alert Wallet Card that has been disseminated to more than 30,000 people with Marfan syndrome.


Marfan Syndrome: Not A Death Sentence

Marfan syndrome is a disorder of the connective tissue, which is found throughout the body. As a result, multiple body systems are affected. People with this condition are frequently taller than other non-affected members of their family, with disproportionately long limbs, fingers and toes. They often have an indented or protruding chest bone, a curved spine (scoliosis), flat feet, a high-arched palate in their mouth and loose joints. The weak connective tissue can also be found in the aorta, and the constant pounding of the heart can cause this fragile tissue to enlarge or even tear if precautionary measures aren’t taken. In about three-quarters of cases, Marfan syndrome is inherited from a parent, but in 25 percent of cases, it results from a spontaneous mutation.

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. 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, MD, 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.”

Research on Marfan syndrome is ongoing and a breakthrough study has identified a commonly prescribed blood pressure medicine, losartan (Cozaar), that prevents and may even reverse the potentially fatal weakening of arteries in mice with Marfan syndrome (Science, April 7, 2006). The National Institutes of Health has launched a clinical trial to study this medication in people with Marfan syndrome.

Losartan has also been shown to positively impact the muscle degeneration in mice with Marfan syndrome, providing further hope for the Marfan community and underscoring the need for extensive research on losartan and how it may impact the various body systems affected by Marfan syndrome.



The National Marfan Foundation, which was founded in 1981, is a non-profit voluntary health organization dedicated to saving lives and improving the quality of life of individuals and families affected by the Marfan syndrome and related disorders by:

• Educating affected individuals, family members and the health care community about the Marfan syndrome.

• Advocating for and funding clinical and molecular research into the early detection and treatment of Marfan syndrome.

• Providing a network of local and special-interest support groups to help affected people and their families share experiences.

For more information on the Marfan syndrome, contact the NMF at 800-8-MARFAN or visit the NMF’s web site at www.marfan.org.