Episode 047-April 26, 2016
Our guest Muriel Normand was a registered nurse who directed the birth place at Southern NH Medical Center prior to retirement in 1998. Today she takes us through Genetics 101 so we can better understand the founder effect and the prevalence of certain genetic diseases in French-Canadians.
French-Canadian Genetic Diseases-Part 1
Muriel and I discussed the following:
Muriel’s introduction to OPMD (oculopharyngeal muscular dystrophy) was through a nursing classmate’s husband who contracted it through the Blais family. She then read an article by Dr. André Barbeau connecting OPMD to French-Canadians.
The Founder Effect
Founder population: 8500 settlers, 1600 of whom were women, provided a very narrow gene pool in Québec. Most settlers came from only a few areas of France, such as the port cities of Dieppe and La Rochelle. Eleven hundred settlers came from Normandy. Almost 1100 came from the Îles de Paris, and the next largest number came from the area of Poitou. Two hundred seventeen settlers originated from an area of Normandy called Perche and are responsible for about two-thirds of the gene pool. This led to the “founder effect.”
The top three families with descendants in Québec are Tremblay, Cloutier, and Martin/Langlois (through daughters). Tremblays show up in 46% of reconstructed genealogies of those with genetic diseases; Cloutiers show up in 77%. Both surnames originated in Perche. Abraham Martin and Marguerite Langlois show up in 86% of reconstructed genealogies of those with genetic diseases.
Founding couples had an average of thirty-six children and grandchildren before 1680.
The population of Québec was the largest and most rapidly reproducing in the 1800s. It doubled every thirty years from 1750 to 1875, partly due to the Revenge of the Cradle (Revanche de Berceaux). This was an effort by the Church to maintain its language and religion by producing large families.
After the British conquest, the French-Canadians were isolated and stayed among themselves. Immigration stopped and no “new blood” was introduced to the population.
Because they were running out of places to settle along the St. Lawrence River, many began to move and settle in other areas. They tended to move in groups or clans. One group moved from Québec to Charlevoix, immigrating up the Saguenay River to Chicoutimi. That group is responsible for at least 85% of the gene pool of Chicoutimi. This happened in other areas as well.
Sibship, children from one family marrying children from another family, also contributed to the loss of genetic variation.
There are twenty-eight Mendelian disorders (following Mendel’s laws of genetic inheritance) that occur in the Saguenay-Lac-Saint-Jean region. There is also a pocket in Trois-Rivières as well as five pockets of familial hypercholesterolemia elsewhere.
Common founder: one who was present in all of these disorders. In Lac-Saint-Jean/Chicoutimi, thirty-eight of the forty-four founders from Perche show up in almost all of the genealogies.
Allele: one copy of a gene. Everyone has two copies in every chromosome, one contributed by the mother and one from the father.
There are four types of genes:
- Dominant genes: these genes generally prevail over recessive genes
- Recessive genes: can only be expressed if both parents carry the gene. Diseases caused by recessive genes, when they are expressed, tend to be more significant.
- MtDNA: a substance found in the tissue surrounding the nucleus of the cell. It is passed on from mothers to both sons and daughters, but only the daughters can pass it on. MtDNA lives forever.
- X-linked gene: produces sex-linked diseases. Mothers can only pass on one of her two X-chromosomes. Men can pass on either an X or a Y. A child who inherits two X-chromosomes is a girl. A child who inherits a Y-chromosome from the father is a boy. If a woman carries a disease on one of her X-chromosomes, and she contributes that X-chromosome to a Y, then the boy child will inherit the disease because there is very little genetic material on the Y-chromosome to offset the diseased X. The mother is the carrier, but the male children can inherit the disease, unless they inherit the healthy X.
- Relatively new: Co-dominance. Genes are capable of blending. You can inherit a good gene and a bad gene, and you can exhibit less of a case or no case of the disease.
If you inherit a bad gene from both mother and father’s sides, then you tend to exhibit a worse case of the disease.
Emigration to US
What happens, genetically speaking, to families who emigrated to the US? The longer a couple stayed in Canada, the more likely the disease will occur. Couples who moved to the States earlier are more likely to have diluted the gene pool.
OPMD (oculopharyngeal muscular dystrophy) tends to be found in the mill towns of Lowell and Lawrence, Massachusetts, and Nashua and Manchester, New Hampshire. It came along with people from L’Islet on the southern bank of the St. Lawrence. The incidence worldwide of OPMD is one in 200,000. The incidence in Québec is one in 1,000.
Symptoms of OPMD include drooping eyelids, and sometimes difficulty swallowing or weakness of muscles in the face or limbs.
Doctors in New England tend to be aware of the prevalence of these diseases in the Franco community. Doctors in other parts of the country…not so much.
In the 1960s, a Montreal physician, Dr. André Barbeau, noticed that most of his OPMD patients came from the same families. Working with the Drouin Institute, he reconstructed the genealogies of these families. They all went back to one couple, Zacharie Cloutier and Xainte Dupont, who emigrated from Perche in the 1600s. He went to Perche and could find no one with the droopy eyelids or other symptoms of OPMD. After much investigation, he discovered a pocket of OPMD in Niort and a gentleman by the name of Bineau who suffered from the disease. Genealogy showed that Zacharie Cloutier, Jr., had married one of three Émard sisters, whose mother’s maiden name was Bineau. The Émard sisters were the carriers of the disease.
By 1700, these three sisters had 1,031 descendants:
- Madeleine Émard married Zacharie Cloutier (many descendants; moved to L’Islet)
- Barbe Émard married Gilles Michel and Oliver LeTardif (few progeny; moved to Montreal)
- Anne Émard married Guillaume Couture (many descendants; moved to L’Islet)
These are the only founders known by name. After genetic studies began, institutes like BALSAC decided not to reveal founders’ names for privacy concerns.
BALSAC is a database begun by the University of Chicoutimi in conjunction with other universities doing genetic research. Their genealogical and genetic research now includes Acadians and Amerindians as well as French-Canadians.
If you have any questions about genetically inherited diseases in general or OPMD, send them in to maplestarsandstripes [at] gmail [dot] com, and Muriel will answer them in an upcoming episode.
- French-Canadian Ancestry and Relevant Health Conditions from 23andMeBlog
- Unexpected genomic change through 400 years of French-Canadian history
- Family Health History: Tracing Your Medical Family Tree
- From Ancestry magazine: Family Health Histories: Your Ancestors Could Save Your Life
- Mayo Clinic’s Medical History: Compiling Your Medical Family Tree
- My Family Health Portrait-a Tool from the Surgeon General
- Scientific papers from JSTOR: Hereditary Disorders in the French Canadian Population of Quebec. I. In Search of Founders
- French-Canadian disease prompts U.S. warning by CBC News
Bibliography Provided by Muriel Normand
- Bittingham, A, de la Cruz P. Ancestry Brief (US Census Bureau. 2004, June, Report no: C2KBR-35).
- Bouchard J-P, Andre Barbeau and the oculopharyngeal muscular dystrophy in French Canada and North America. Neuromusc Disord 1997; S5-S11.
- Brais, M.D., Ph.D, Rouleau, M. D. Ph.D, Bouchard, J-P, MD, Fardeau, M.,M.D., and Tome, Fernando M.S., M. D., Ph.D. Oculopharyngeal Muscular Dystrophy. Seminars in Neurology, 19, (1), 1999: 59-66.
- Codere F, Brais B, Rouleau G, Lafontaine E, Oculopharyngeal muscular dystrophy: What’s new?, Orbit-2001, Vol.20, No. 4, 259-266.
- De Braekeleer M, Dao TN. Hereditary disorders in the French Canadian population of Quebec 1. In Search of founders. Hum Biol 1994: 66 (2): 205-223.
- De Braekeleer, M, Dao TN. Hereditary disorders in the French Canadian population of Quebec. 11. Contribution of Perche. Hum Biol 1994: 66 (2): 225-249.
- Godbout, AG. (1944). Les trois soeurs Esmard. In Memoires de la Societe Genealogique Canadienne-Francaise, 1(3): 197-199.
- Laberge A-M, Michaud J, Richter A, Lemyre E, Lambert M, Brais B. Mitchell GA, Population history and its impact on medical genetics in Quebec. Clin Genet 2005: 68: 287-301.
- Scriver CR. Human genetics: Lessons from Quebec populations. Annu Rev Genomics, Hum Genet 2001: 69-101.
Mass. Memories Road Show
The town where my French-Canadian ancestors have lived for more than 150 years participated in a Mass. Memories Road Show. Conducted by Carolyn Goldstein of UMass Boston, “The Mass. Memories Road Show is a state-wide digital history project that documents people, places and events in Massachusetts history through family photographs and stories. In partnership with teams of local volunteers, we organize public events to scan family and community photographs and videotape the stories behind the photos. The images and videos are indexed and incorporated into an online educational database at openarchives.umb.edu.”
Check out the towns of your ancestors here. Besides location, there are also themed road shows, like the one on Irish immigrants. Carolyn is interested in conducting a French-Canadian Immigrant Road Show. If any of our listeners from Massachusetts is interested in volunteering to help out, email me at maplestarsandstripes [at] gmail [dot] com.
- Sign up for the Maple Stars and Stripes newsletter before the deadline.
- If you are already receiving the newsletter, send an email to maplestarsandstripes [at] gmail [dot] com with the subject line “book contest.”
- The deadline is midnight on May 31, 2016. I will select a winner on June 1 and email that person for his or her mailing address.
Survey #46: Do you have any French-Canadian ancestors who lived in Vermont?
Results: Yes 67%; No 33%
Survey #47: How long have you been researching your French-Canadian lines?
- Less than a year
- 1-5 years
- 5-10 years
- 10-20 years
- More than 20 years
Send in your response to Survey #47 here.
The Franco-American Centre
- April 27, 7 PM, New Hampshire Institute of Politics at Saint Anselm’s College in Manchester: “New Identities of the French Language in Montréal” by Dr. Pierre Anctil, part of its Distinguished Lecture Series
- May 4, 7:30 PM, Hatbox Theatre in Concord, NH: magic show by Franco-American magician Andrew Pinard
- May 7, the Currier Museum, Manchester: another French Adventure. Use French vocabulary as you tour the museum.
- May 12, 7:30-8:30 PM, Dana Center Lecture Room in Manchester: David Vermette will give a talk in English on “The Cabot Mill and Brunswick, Maine’s Franco-Americans.” From the website: “David Vermette, Franco-American researcher, writer and blogger, details the development of the Franco-American community in Brunswick, Maine, and the history of the textile mill that began to bring French-Canadians to Brunswick in the mid-19th century.”
The French Canadian Heritage Society of Michigan
- May 14, 11 AM, at the Mount Clemens Public Library: “Breaking Down Brick Walls Using DNA,” with Bethany Waterbury of Next of Kin Research.
The Quebec Family History Society
- April 30, 1 to 3 PM: “How to Use Ancestry, Findmypast, FamilySearch and other Major Genealogical Websites”
- May 14, 1 to 3 PM: “Exploring the BAnQ Website” with Lorraine Gosselin
- All classes at the QFHS Heritage Centre and Library in Pointe-Claire, Quebec.
The Vermont French-Canadian Genealogical Society
- April 30: Lou Izzo and Don LaCross-“NosOrigines.qc.ca—a Database of French-Canadians”
- May 7: Mike Sevigny-“Genealogy Research in Acadia”
Classes run from 10:30 AM until noon and are held at the Vermont Genealogy Library in Colchester, Vermont.
Lynda.com: Check out their course offerings, then try out a 10-day free trial by clicking the banner below.
Get Maple Stars and Stripes
- Subscribe to the MSS Newsletter
- Subscribe on iTunes
- Subscribe on Stitcher
- Join us on Facebook
- Join us on Google+
How to Contact Maple Stars and Stripes
- Post comments on Show Notes page (MapleStarsandStripes.com/#-of-episode) or at end of blog post
- Go to MapleStarsandStripes.com/contact
- Email me at feedback [at] maplestarsandstripes [dot] com
- Send a voicemail by clicking on the ‘Send Voicemail’ link from any page at MapleStarsandStripes.com