canadian marfan association

canadian marfan association

November 11, 2005 15:32 ET

Marfan Syndrome Community Benefits from Johnathon Larson Legacy

RENT, the Movie, Provides Forum to Address Marfan Syndrome and Nov. 13-19th is National Marfan Awarenss Week Attention: Arts/Entertainment Editor, Education Editor, Health/Medical Editor MISSISSAUGA, ONTARIO--(CCNMatthews - Nov. 11, 2005) - Toronto, Ontario, November 11th, 2005 - Jonathan Larson's 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, the Canadian Marfan Association 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 easily 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.

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. 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.

"Jonathan's death illustrates the major problem we all face today," said Reed Pyeritz, M.D., Chief of Medical Genetics, University of Pennsylvania School of Medicine and Chair of the Canadian Marfan Association's Medical Advisory Board. "Too many people with a high risk of aortic dissection are not diagnosed as having Marfan syndrome or one of the other genetic conditions that weaken major blood vessels. Educational efforts, such as those sponsored by the Canadian Marfan Association, are crucial to close this gap in awareness among health professionals and the public alike."

To help promote this awareness, the week of November 13 - 19, 2005 is National Marfan Awareness Week in Canada.

Potentially Fatal Genetic Disorder Holds National Awareness Week

The Marfan syndrome is a potentially fatal genetic disorder of connective tissue that
affects approximately 5000 Canadians.
Connective tissue is present throughout the entire body, as a result the disorder manifests in
many body systems, including the skeletal system, eyes, lungs, blood vessels and heart. Many
people with the Marfan syndrome experience an expansion of the aorta. Without proper
monitoring and medications to reduce the stress on the aorta, affected people are at high risk
for aortic dissection or rupture, which could result in sudden death.

National Marfan Awareness Week is November 13 - 19, 2005 and gives us an opportunity to educate Canadians about this potentially life threatening disorder. This week brings together Marfan patients, their families and friends to raise awareness within their communities. Volunteers are distributing information in hospitals and their workplaces, having mall displays, and talking to the media.

By recognizing the outward signs, you have the potential to save a life. Some of the signs include:
· Tall stature
· Disproportionately long arms, legs, fingers and toes
· Protruding or sunken chest bone
· Abnormal curvature of the spine (scoliosis)
· Nearsightedness, often with dislocation of the lens
· Flat feet
· Mitral valve prolapse

Life expectancy for people with the Marfan syndrome has increased in the past decade, but only
through increased awareness, earlier diagnosis and proper treatment can they expect to live a
normal lifespan.

The Canadian Marfan Association

The Canadian Marfan Association is a non-profit charity dedicated to assisting Marfan patients and their families through programs of research, education and support.

For more information about Marfan syndrome, please contact Eva Theofilopoulos, Executive Manager at the National Office toll free bilingual number 1-866-722-1722, or visit our website at


Contact Information

  • eva Theofilopoulos
    Primary Phone: 905-826-3223