The following article first appeared in The Echo of Cantley Volume 27 no 3, September 2015. This article is made available for the enjoyment of others with the express permission of the Echo of Cantley.
Oculopharyngeal Muscular Dystrophy (OPMD) is one of nine types of muscular dystrophy, affecting voluntary muscles: eyelids, throat, facial and limb muscles. If one parent has the disease (diagnosed through a blood test for the PABPN1 gene), each child has a 50% risk of inheriting OPMD. If you have the gene, you will get the symptoms. Men and women are equally susceptible. OPMD can be found on all continents but is more prevalent in some areas, especially Quebec. It is estimated that 1 in 1,000 French Canadians have OPMD.
Genealogical research can help us to find out who our ancestors were, where they lived, what they did for a living, but it is becoming more and more useful in terms of identifying medical conditions that can be inherited or at least that we are predisposed towards.
Dr. E. Willis Taylor first identified OPMD and its familial nature in a French Canadian family living in the Boston area in 1915. In 1988, a team of Quebec medical researchers discovered the PABPN1 gene, which is responsible for OPMD. It was during the 1960s that the most fascinating discoveries were made.
In 1962, André Barbeau, a French Canadian neurologist, embarked on a study to identify French Canadian families affected with OPMD in the province of Quebec. Over a period of seven years, he examined 249 affected individuals from more than 10 large families. His genealogical work found that all cases shared a common ancestral couple. Researchers traced 160 individuals with OPMD back 11 generationsusing Quebec Roman Catholic church records. They found one couple in Niort, France, which was common in all OPMD families - Jean Emard and his wife Marie Bineau. This couple never migrated to Canada. Of their eight children, only three of the daughters, Barbe, Madeleine, and Anne migrated to New France in 1648. Researchers also discovered OPMD in the Bineau ancestral line, not in the Emard line.
All three sisters, Barbe, Madeleine, and Anne, immigrated to Quebec on the same ship from La Rochelle, France in 1648. After her first husband, Gilles Michel, died, Barbe married Olivier Tardiff in 1648, in La Rochelle. They immigrated with Barbe's son and settled in the Château-Richer area of Quebec, where they had three more children. Madeleine married Zacharie Cloutier in 1648 in La Rochelle. They also settled in the Château-Richer area where they had eight children. Anne, the youngest of the three sisters, married Guillaume Couture in 1649, in Pointe-de-Lévy, Quebec, where they had nine children.
It is quite astonishing that the records are available to support this aspect of medical research going back over 450 years. These families are among the founding families of Quebec. They migrated here in its earliest days; they were contemporaries of Champlain! Every Tardif, every Cloutier, and every Couture, are descended from them. Among them they have hundreds of thousands of descendants, including, we are told: Celine Dion, Shania Twain, Madonna and the Dionne Quintuplets. These family names can also be found in the Cantley phone book. This research is invaluable for identifying and managing this genetic disease.
This article is based on notes taken at and the syllabus of a presentation by Johanne Gervais, Computer Specialist and Genealogist, at "Roots 2015", an international conference on Family History in Quebec, presented by The Quebec Family History Society (www. qfhs.ca) on June 19-21, 2015 at McGill University, Montreal. Ms Gervais has inherited OPMD through her father's side of the family, and has traced her lineage to Anne Emard.
She provided these useful links:
- Muscular Dystrophy Canada: www.muscle.ca
- Muscular Dystrophy U.S.: www.muscle.ca.org
- Facebook site called "OPMD Oculopharyngeal Muscular Dystrophy runs in my family": www.facebook.com/groups/362651267234189
Mary Holmes is a board member of Cantley 1889. If you would like to share your family's story,Cantley 1889 would appreciate hearing from you at firstname.lastname@example.org.