Stories of my Ancestors: OCULARPHARYNGEAL WHAT?

Saturday, May 30, 2009

OCULARPHARYNGEAL WHAT?

When you are looking for your ancestors sometimes you are not thrilled with some of the things you may find.
They could be crooks or criminals, crazed or coldhearted, dishonest or immoral. Or they could be genetically dangerous to your health.

As a French Canadian I was stunned to find out that I have a greater chance of having Oculopharyngeal Muscular Dystrophy. That is hard to say in French or English. It turns out that my 9th Great Grandparents, Jean Emard (Aymart) and Marie Bineau somehow were the carriers of this genetic disease. Jean and Marie were born in France but they came to New France (Quebec) and their daughters were born here.

Oculopharyngeal Muscular Dystrophy has many different syptoms. It can weaken the facial muscles and the pharyngeal muscles. This weakening of the throat muscles can cause a diffuculty in swallowing known as dysphagia. Another symptom of this disease is the weakness of the muscles that control the eyelids. Ptosis is the name for droopy eyelids that can cause vision problems by covering your eye. My 2 sisters have this problem and the doctors they saw thought it was just an droopy eyelid like lots of people get. It would be considered cosmetic to have it fixed. However it is not cosmetic when your eyelid blocks your field of vision and any persperation runs directly into your eyes. The doctors can look with a high powered microscope at the muscle tissue from sufferers and see inclusions in the cells along with bubbly structures.

Although not limited to French Canadians, those French people who do have it can trace their families back through time to one of the 3 daughters of Jean Emard and Marie Bineau. These women were Barbe Emard, Anne Emard and Marie Madeleine Emard.
My line is as follows:

Barbe Emard married Jacques Cochon in 1661¹.

Genevieve Cochon married Joseph Huot dit Laurent in 1708².
Joseph Huot dit Laurent married Marie Louis Cote in 1731³
Charlotte Huot married Jean Baptiste Marion in 1759⁴
Marie Amable Marion married Jean Baptiste Beaugrand dit Champagne in 1786⁵
Emmanuel Beaugrand dit Champagne married Madeleine Laderoute unknown
David Alexander Beaugrande dit Champagne married Melanie Carriere in 1873
Ziphirin Champagne married Marie Octavie Carriere in 1907

Next in line was my mother. I dont' ever recall her having any problems with her eyelids or with swallowing but I must say she had a whole host of other health problems she had to deal with. It leaves me wondering what else may have been passed down from our ancestors.