|Theresa Pageau, the woman to whom this site is dedicated, passed away due to complications from a disorder known as Marfan Syndrome.
According to the National Institute of Arthritis and Musculoskeletal and Skin Diseases(NIAMS): "Marfan syndrome is a heritable condition that affects the connective tissue. The primary purpose of connective tissue is to hold the body together and provide a framework for growth and development. In Marfan syndrome, the connective tissue is defective and does not act as it should. Because connective tissue is found throughout the body, Marfan syndrome can affect many body systems, including the skeleton, eyes, heart and blood vessels, nervous system, skin, and lungs.
Marfan syndrome affects men, women, and children, and has been found among people of all races and ethnic backgrounds. It is estimated that at least 1 in 5,000 people in the United States have the disorder."
(Excerpt courtesy of the NIAMS website)
In the case of Marfan, the connective tissue is defective and does not operate properly, leading to one or more of the following problems:
=Circulatory system problems including: faulty heart valves; dilation and weakening of the aorta.
=Skeletal abnormalities such as: elongated limbs and digits; a protruding or indented sternum; height
=Issues with the eyes such as: dislocation of the lenses; detached retinas
=Other issues involving the skin, nervous system, and (occasionally) the lungs.
Marfan Syndrome is passed genetically, through a problem with the gene involved with determining the structure of fibrillin, an important protein that is part of the body's connective tissue. A child born of a parent with Marfan Syndrome has a 50 percent chance of inheriting this disease.
I am, by no means, an expert on this disease. But I have seen first-hand what it can do in a person. Of the signs listed above, Theresa exhibited some of the skeletal and heart issues. She was about 6 feet 2 inches tall, as well as the elongated fingers and the protruding sternum. Most crucial, however, were the heart problems. A heart valve in a person with Marfan can leak. Hers did, and was replaced with an artificial valve (in fact, she had a valve put in on two separate occasions). It was this valve on which a blood clot formed in June of 2000, and, as a result, Theresa was rushed to the hospital in the throes of a heart attack. That heart attack, and the resultant heart damage, put Theresa into a condition called Congestive Heart Failure. Her heart could not keep up with the demand placed on it by the rest of her body. Her only hope was a heart transplant. That hope never came to fruition, and we lost Theresa on a cold morning in late January, 2001.
The stakes can run pretty high when Marfan Syndrome comes into play. Yet, in my previous experience, I did not know it to be a commonly-known condition. To be honest, I had never heard of it until I met Theresa. In fact, other than the people I have talked to regarding Teri and this site, the only time I have seen Marfan mentioned publicly was on an episode of the television series "ER", on an episode entitled "The Longer You Stay". In this episode, two ER doctors are attending to a patient brought in from a rock concert and whose EKG is indicating a massive heart attack. One headstrong doctor, thinks it is drug-related and pushes a course of action before all the facts are in. When another doctor walks in and deduces from the x-ray that the patient has Marfan, the other doctors realize they took the wrong course of action and the patient dies.
Why the TV reference? Well, other than the fact that my jaw dropped when I heard the reference, it made me think about how much needs to be known in the public about Marfan. The episode aired nine months after Theresa's passing, and, if not for her, the reference probably would have went over my head. I thought about people watching this who exhibited signs of Marfan who had probably never heard the name. It made me think about how many people get screened and how many people probably need to be. After all, it is not just a personal health issue, but an issue that can be easily compounded upon entering into parenthood.
If you are on this page, no doubt you have already heard of Marfan. But if you need more information, please feel free to check out the following links to sites run by people who know a lot more about Marfan by way of either profession, or just plain flat out living with the condition.
NIAMS - This is the site of the National Institute for Arthritis and Musculoskeletal and Skin Diseases, the division of the government's National Institutes of Health that deals with Marfan. Provides excellent background information on Marfan Syndrome.
www.marfanworld.org - A very good site for an overview of not only the specifics of Marfan, but living with it (and its effects on those you love) as well. Peter, the MW webmaster, lost a father, a brother, and a son to Marfan. Offers worldwide Marfan resources.
www.marfan.org - The home site for the National Marfan Foundation, an organization looking to further knowledge, communication, and research related to Marfan Syndrome and its effects.
Stanford University - The site of the Stanford University Center for Marfan Syndrome and Related Connective Tissue Disorders. A medical center, located in Stanford, CA., dedicated to Marfan treatment and research.
www.marfanlife.org - Good page with links to the Marfan community, and a listing of personal sites of others dealing with Marfan.
www.marfanssyndrome.net - Michael, this site's webmaster, is a college student with Marfan Syndrome.
www.jrmarfan58.com - The Living With Marfan Syndrome website. Jon, the site's owner, is a gentleman in his mid-40s. He has Marfan Syndrome, and he tells you his story as his life with Marfan Syndrome progresses. A very good read for those wanting to learn more of Marfan's effects over time.