Attention French-Canadian Descendants: Did You Inherit Oculopharyngeal Muscular Dystrophy?

The following article is from Eastman’s Online Genealogy Newsletter (25 Oct 2007) and is copyright 2007 by Richard W. Eastman. It is re-published here with the permission of the author. Information about the newsletter is available at http://www.eogn.com.

Not only do genealogists have the opportunity to learn about their ancestors, but they can also learn about various inherited diseases. Some of these medical conditions could be life-threatening while others are merely an inconvenience. By studying inherited diseases floating around in your family, you may save or prolong your own life or the lives of your loved ones. By identifying the risks early in a person’s life, medical treatment often can be much more effective than the limited choices available after the medical condition becomes obvious.

I find it interesting that one French-Canadian couple in the 1600s who are the ancestors of millions of living people have tentatively been identified as carriers of a common form of muscular dystrophy. It became more than “interesting,” however, when I recognized the names of this couple as my ninth great-grandparents. Suddenly it wasn’t just “interesting;” it was personal!

If you have French-Canadian ancestry, now is the time to check your pedigree charts.

Zacharie Cloutier and Saincte Dupont, who emigrated to Canada from France in 1634, may have harbored the genetic defect responsible for the majority of today’s French-Canadian cases of Oculopharyngeal Muscular Dystrophy (OPMD).

NOTE: Saincte Dupont’s first name has been spelled in many different ways. I have seen it listed as Saincte or Sainte or Xainte or Zainte Dupont. Zacharie’s name also is sometimes listed as Zacarie Cloustier. Succeeding generations have spelled the surname in many different ways and have sometimes Anglicized it as well. Zacharie Cloutier’s male descendants have spelled their last names as Cary, Cluchier, Clukey, Clurkey, Cluquet, Clouter, Lapensee, Nailer, and probably other ways as well.

Zacharie Cloutier and Saincte Dupont were married in St-Jean de Mortagne, ev. Sees, Perche, France, on 18 July 1616. They set sail from the port of Dieppe in mid-April 1634, accompanied by his family. The fleet consisted of four ships under the command of Duplessis-Bochard, arriving in Québec on June 4, 1634. Zacharie Cloutier was a master carpenter and is known to have helped build the manor house of Robert Giffard at Beauport, Québec. He also worked on the parish church and on Fort Saint-Louis in Québec. We can guess that he also helped build many of the houses of that settlement.

Oculopharyngeal Muscular Dystrophy is best known for a weakness of the muscles that control the eyelids, causing droopy eyelids, a condition also known as ptosis. However, that is not the only symptom. The disease often causes a weakness of the facial muscles and pharyngeal muscles (those in the throat used for swallowing), a condition known as dysphagia. Weakness of facial and limb muscles often occurs later. Symptoms of the disease usually don’t begin until the mid 40s or 50s, but can occur earlier.

A person with OPMD might have some of the symptoms, but not all. While the disease is best known for the droopy eyelids that severely narrow the field of vision, another person with the same disease might not display that symptom at all. However, a difficulty in swallowing is also quite common, even if it is not visible to others.

When muscle tissue from a person with OPMD is examined under a high-powered microscope, clumps of proteins called inclusions are seen in the muscle cell nuclei (the cellular compartments that contain the chromosomes), and bubble-like structures (vacuoles) appear in the muscle cells.

Oculopharyngeal Muscular Dystrophy is most common in French-Canadian families or families of French-Canadian descent. When the French-Canadian victims’ family trees are traced, Zacharie Cloutier and Saincte Dupont always appear someplace in the pedigree charts, often in more than one place. They appear to be the only couple that is always found amongst the ancestors of every French-Canadian sufferer of OPMD. However, there’s also a high incidence of OPMD among Hispanic residents of northern New Mexico. OPMD can also affect people who aren’t of French-Canadian or Hispanic background although that is far less common.

If you have difficulty swallowing or if you have droopy eyelids, check your pedigree chart. If you find Zacharie Cloutier and Saincte Dupont in the family tree, you might want to mention that fact to your doctor.

For more information about OPMD, look at https://www.mda.org/disease/oculopharyngeal-muscular-dystrophy, http://www.mda.org/disease/opmd.html, http://www.webmd.com/brain/muscular-dystrophy-oculopharyngeal, and on a Google search at http://tinyurl.com/2kgdwu.

By the way, most French-Canadians with ancestry in the greater Quebec City area can find Zacharie Cloutier and Saincte Dupont in multiple places in their pedigree charts. That is, most of these people have multiple lines of descent from this one couple.

A Mention in the Wall Street Journal

The following appeared in the Weekend Journal section of the October 12, 2007, edition of the Wall Street Journal:

The Photo Detective
Does the shoebox in the attic hold family secrets?
Alexandra Alter on how one expert unlocks the past.

MAUREEN TAYLOR has dated a photograph to 1913 by studying the size and shape of a Lion touring car’s headlamps. Armed with her collection of 19th-century fashion magazines, she can pinpoint the brief period when Victorian women wore their bangs in tight curls rather than swept back. Using a technique borrowed from the CIA, she identified a photo of Jesse James by examining the shape of his right ear.

With millions of Americans obsessively tracing their roots, Ms. Taylor has emerged as the nation’s foremost historical photo detective. During a recent meeting of the Maine Genealogical Society, attendees lined up a dozen deep as she handled their images with a cotton glove and peered at the details through a photographer’s loupe. One man offered a portrait photo and asked if it could be his great-grandmother who died in 1890. “It’s not,” Ms. Taylor said after about 15 seconds; she’d dated the hairstyle and billowy blouse to the early 20th century. When another attendee asked why her great-great-grandfather was wearing small hoops in his ears in a portrait, Ms. Taylor explained, “He was in the maritime trade.”

Read the rest of the article at a library near you….
or the article can be accessed from Maureen’s blog – visit http://photodetective.blogspot.com/

SOURCE: Alexandra Alter, “The Photo Detective,” Wall Street Journal, 12 Oct 2007, pp W1, W10

2007 Award of Excellence in Genealogical Service presented to Thelma Eye Brooks

Maine Genealogical Society at its conference on September 22, 2007, presented Thelma Eye Brooks with new Award of Excellence in Genealogical Service, in recognition of her years of service in the areas of society volunteerism, teaching and writing. Among her many accomplishments are the publication of the Hermon Roots newsletter from 1989-1999; authorship of a book on 3 generations of early Calais families (published by Heritage Books); and the self-publication of 13 books (5 family books and 8 funeral home records). She also wrote a column “Roots and Branches” which appeared regularly in the Kennebec Journal and Morning Sentinel for 15 years. Thelma has served as president, vice-president and secretary of Taconnett Falls Chapter of MGS, where she has also served on their library committee since 1990 and has served as its chair since 2000. Thelma has taught classes on genealogy, coordinated historical and genealogical research with the Waterville Public Library, and has been a frequent speaker for many genealogical and historical societies.

More than any of these specific accomplishments, the most wonderful thing about Thelma is that she is always willing to share her knowledge and experience with everyone. She is always a “can do” person and works tirelessly for Taconnett Genealogical Library, the chapter, and genealogy researchers everywhere.

Maine Genealogical Society honors her extraordinary contributions to the Society and for advancing the field of genealogy in the State of Maine.

2007 Award of Excellence in Genealogical Research presented to Marlene A. Groves

Maine Genealogical Society at its annual conference on September 22, 2007, presented Marlene A. Groves with its new Award of Excellence in Genealogical Research, in recognition of her outstanding scholarship in carefully transcribing and publishing volumes of Maine town vital records and for encouraging others to do the same.

Flemish DNA and Ancestry

An email was received from Guido Deboeck announcing that a book on the above subject has just been released by Dokus Publishing in Arlington, Virginia.

This new book is about the history of three Flemish families over five centuries. The research for this book was based on conventional genealogy complimented with an innovative part on genetic genealogy. The conventional genealogy part shows how to find and document family history given the vast resources made available on the web and in Family History Centers. After an introduction to genetic genealogy this book includes concrete DNA results of several of the three family histories described in this book. It also discusses the deep ancestry of Flemish people, hence anyone with Belgian or Flemish roots can find out where their oldest ancestors came from. The scope of this book also includes elements of war, business and immigration history, which Professor van der Wee, a well known historian called “very informative, even fascinating”.

You can preview or take a tour of this book at https://www.amazon.com/Flemish-Dna-Ancestry-Centuries-Conventional/dp/0972552677. Copies of this book can be ordered via the order form on the above website or the form enclosed.

For Users of The Master Genealogist

The TMG Southern Maine group generally meets on the third Thursday of every month at 7-9 pm at 321 Brackett Street in Portland. The meetings are posted on the website http://www.tmgsome.com/.

In October we are not meeting on 10/18 but are having a longer session on Saturday 10/20 from 10 am to 3 pm at the same location – again the details are on the website.

Anyone interested in TMG software is welcome to attend. Most of the meetings are very informal and often go off on tangents depending on what people are interested in.

2008 Genealogy Seminar at Sea Announced

Fly Away Travel is now taking reservations for the 2008 Genealogy Seminar at Sea, October 25th – November 1st. The seminar will be hosted on board Royal Caribbean’s brand new, unparalleled, Liberty of the Seas, which will be sailing to the warm Eastern Caribbean islands of Puerto Rico, St. Maarten, and Royal Caribbean’s private paradise of Labadee.

This Seminar at Sea stands to be the best of the best in terms of its stunning array of some of the most sought after speakers in the genealogy community today. These include John Phillip Colletta and George G. Morgan (featured speakers at our own 2006 and 2007 conferences), along with Paul Milner, Laura G. Prescott, Donna M. Moughty, Paula Stuart-Warren, Michael J. Leclerc and Stephen J. Danko.

Passengers will have to opportunity to learn the latest and greatest techniques in areas such as: methodology, writing & publishing, internet research, problem solving and geographic areas around the world. Guest’s will have the opportunity to choose up to 15 lectures that will take place in Liberty of the Seas four state of the art conference centers over a period of three days during the seven day cruise. The authored speakers will be featured for book sales and autographs during a special group activity.

Check out the exciting all-star line-up of topics for this event at http://www.genealogycruises.com/.

The cost of experiencing this week long seminar/vacation begins at just $829 per person, including all port taxes and seminar fees. Traveling companions not attending the Seminar at Sea qualify for a rate reduction, as well as the 3rd & 4th passengers sharing one cabin. (Ask for details).

Fly Away Travel is a full service Travel Agency with nearly 60yrs of combined experience. Fly Away Travel specializes in Group Travel, Honeymoons, Destination Weddings & Family vacations. Contact Cindy Lorenz at Fly Away Travel 800-837-0295 or by e-mail: FlyAwayInc@aol.com.

Timothy Crocker, lived in Standish and Machias

Searching for information on Timothy Crocker who moved from Cape Cod to Standish, Maine, and then to the Machias area. He died at Bristol. He married Hannah Meserve, but have no death date or place for her.

Brother John Crocker moved from Cape Cod to Nova Scotia where some of his children were born; he then also moved to the Machias area, but his son remained in Nova Scotia. There I am having trouble separating out the various John Crockers.

Dave Eldridge
E-Mail: deldrid1@bellsouth.net

Welcome!

As another means of communicating information timely and efficiently to the members of Maine Genealogical Society, we have established this blog presence. With our newsletter published quarterly, sometimes announcements come in that just don’t allow us to get the information distributed prior to an event or deadline, or due to space constraints with a print newsletter, can’t be fit.

All postings on this blog will be related to genealogy – announcements and press releases related to genealogical events, publications, products, etc. We’ll also be able to post queries and questions from members here as well as we move forward.

We hope you enjoy this new channel of contact!