Thursday, October 2, 2014

Marfan syndrome complexity confounds experts

Vincente Schiavelli had Marfan's syndrome
Carol Forsloff - Marfan syndrome is one of those genetic disorders that creates pain and problems for entire families, and even though it impacts many people, its complexity confounds medical experts.

Marfan syndrome, as defined by WebMD, " is an inherited disease that affects the connective tissue. Connective tissue is the most abundant tissue in the body and is a vital component to supporting the body's organs. Its primary purpose is to hold the body together and provide a framework for growth and development. It provides the strength and support to tendons, cartilage, heart valves, and many other parts of the body, as well as strength and elasticity to the blood vessels."

The description goes on to discuss how the connective tissue is abnormal with those suffering from Marfan Syndrome and that because of that threats can occur to the bones, eyes, heart and blood vessels, nervous system and lungs as well as to the aorta, the artery that carries blood from the heart to the rest of the body.

These people are often larger, unusually taller than others, with large feet, for example and hands, as "markers" of the disease.

While people enjoy Ripley's Believe It or Not, many of those people who are substantially taller than average suffer from Marfan syndrome.

And many of these people who have suffered from it are, or have been, famous.

Mary Queen of Scots, estimated as nearly six feet in height, is said to have suffered from Marfan syndrome.  She lived as a captive for 25 years during the reign of her sister, Queen Elizabeth, and was known for her beauty and her Catholic stance in a kingdom torn between Protestant and Catholic groups.

Abraham Lincoln is considered another famous person with Marfan syndrome features.  He was tall, thin, almost emaciated in appearance at times, and had some of the characteristics, although the possibility of his having Marfan syndrome is based on a distant relative having some of the features.

Vincent Shiavelli, a well-known actor, was active in the Marfan Foundation and died of lung cancer at the age of 57.


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