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Startled easily by sudden noises or light?

Published July 12. 2011 05:01PM

Q. A friend of mine was diagnosed with Jumping Frenchmen of Maine. Have you ever heard of this?

Not until now. Jumping Frenchmen of Maine is a rare, unexplained disorder that produces an extreme startle reaction to a sudden noise or sight.

Jumping Frenchmen of Maine was first identified during the late 1800s in Maine and the Canadian province of Quebec.

It was discovered among an isolated population of French-Canadian lumberjacks. Since the discovery, the extreme startle reaction has been found in other societies in many parts of the world.

Jumping Frenchmen of Maine is one of almost 7,000 rare diseases.

In the United States, a disease is classified as rare if fewer than 200,000 people have it. About 25 million people in the U.S. have a rare or orphan disease.

Some familiar orphan diseases are cystic fibrosis, Lou Gehrig's disease and Tourette's syndrome. Orphan diseases don't attract as much research funding as major diseases because they aren't as profitable to the healthcare industry.

In 1983, Congress passed the Orphan Drug Act (ODA). The ODA created financial incentives for drug and biologics manufacturers, including tax credits for costs of clinical research, government grant funding, assistance for clinical research, and seven-year periods of exclusive marketing. At the same time, federal programs began encouraging product development, as well as clinical research for products targeting rare diseases.

Since 1983, the ODA has stimulated the development of more than 250 orphan drugs, which now are available to treat a potential patient population of more than 13 million Americans.

In contrast, the decade before 1983 saw fewer than 10 such products developed without government assistance.

An excellent source of assistance for these diseases is the National Organization for Rare Disorders

(NORD), a federation of more than 130 nonprofit voluntary health organizations.The NORD web site includes information on medication assistance programs and networking programs, a resource guide, and links to other online resources. Here's the contact information: National Organization for Rare Disorders, 55 Kenosia Ave., PO Box 1968, Danbury, CT 06813-1968; (800) 999-6673;

New rare diseases are discovered every year. Most are inherited and caused by alterations or defects in genes. Others can be caused by environmental conditions.

To browse through a list of this rare diseases, go to the Genetics Home Reference website at:

If you would like to read more columns, you can order a copy of "How to be a Healthy Geezer" at

The Times News, Inc., and affiliates (TIMES NEWS) do not endorse or recommend any medical products, processes, or services or provide medical advice. The views of the author do not necessarily state or reflect those of the TIMES NEWS. The article content is not intended as a substitute for professional medical advice, diagnosis or treatment. Always seek the advice of your physician, or other qualified health provider with any questions you may have regarding a medical condition.

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