Tuesday, June 9, 2015

Guest Blog: Leber’s Optic Atrophy

By Sarah Semple

My dad came from good working class stock.  He was the fourth child of five and there was nearly a six year gap between him and his next older sibling. His mother decided to hold him back from going to school until his younger brother was old enough, so that the two siblings could keep each other company.  Dad had very little in common with his youngest sibling who was always getting into strife.  But being dad, he just went with the flow and didn’t question the decision.

Dad was a good sportsman, an average performer at school and had decided to become a teacher. He was 19 years of age, in his second year of teacher training when something extraordinary happened to him.  He lost his eye-sight.  He lost the sight in one eye in January 1954 and then the other in May 1954.  The medical system couldn’t explain it.  He could see nothing in front of him, and just shapes in his peripheral vision.  He spent 10 weeks in hospital and was told that his eyesight was bad but it wouldn’t get any worse.

Life was turned on its head.  He had to withdraw from teacher training and his beloved sport.  He lay in the hospital bed feeling very sorry for himself.  To cut a long story short, he heard a girl come in to his ward and tell people to breathe in and out.  He thought that this sounded like an easy job and enquired about it.  She was a physiotherapist, and he found out that there was a School for the Blind for Physiotherapy in London.  He got sponsorship from the New Zealand Foundation for the Blind and off he went -- alone on a six week boat trip, first time out of New Zealand with minimal eyesight. He had a fabulous three years training in London, establishing friendships with other blind physiotherapists that lasted a life time.

John "Jack" Alexander Semple in England, 1958; photograph courtesy of
Sarah Semple

So how does this relate to genealogy? Well, it all comes back to the rare eye disease called Leber’s Optic Atrophy.  When researching this disease, I found out that it is genetic, but that it is only passed on from the female.  So a male can inherit it (like my dad), but his children can never get it.  His sister however could both inherit the gene and develop the disease, as could her children.

So with this information, I approached a renowned Opthamologist who confirmed that as far as he knew there were only two families in New Zealand with this disease.  He had studied the other family to understand the heredity patterns of the disease.  

Jack Semple playing bowls; photograph courtesy of
Sarah Semple

I found that one of my dad’s aunts had also inherited the disease and had died after accidentally drinking a bottle of poison that she thought was a soft drink.  I also found women in dad’s family with poor eyesight that people had attributed to old age, however may well have been the same eye condition.

So when I come across or hear about people (especially young men) who lose their eyesight at an early age to Leber’s, I always ask the question… what was their mother and grandmother’s maiden names?  Chances are, we could be related.

Sunday, June 7, 2015

52 Ancestors #23: My Wedding

When my husband proposed, he thought I would want a wedding ceremony; he just hoped it would be small. His first surprise was learning I thought our parents would join us at the courthouse, witness our marriage, and we'd all go out and have a nice lunch! He couldn't believe it. I was an only daughter, surely we needed a wedding ceremony.

We compromised with a very small wedding. We mailed 16 invitations. There were 31 guests, including five children under the age of five. We were married in the historic district of New Bern, North Carolina, by my parents long-time minister. Our wedding lunch was at the historic Henderson House (which is no longer in business), and our overnight guests filled up the bed-and-breakfast across the street. My double-first cousin and best friend forever flew in from Luxembourg to be my maid of honor and Pete's Dad was his best man.

I'm on the left and my double first cousin, Joyce, is on the right

I carried a single white silk rose (Mom's idea) and wore that ivory silk suit on special occasions for several years.

Pete and his Dad, who was his best man

I did not walk down the aisle, something that terrified me. Instead, the wedding party sat on the first pew and at noon we stood up and were united in marriage. It took about 10 minutes!

Pete and I at our wedding lunch

We celebrated our 27th wedding anniversary last month. We've both changed a bit. Pete is better looking now and I, well, I went a different direction. :)

Pete's immediate family

My family

"Immediate" is a relative term for my family. My father and his brother married sisters! And we all grew up in the same town or next door. Then there was my grandmother and a beloved widowed aunt and a few other dearly loved family and friends.

This is my entry for Amy Johnson Crow's 52 ancestors in 52 weeks challenge optional theme Wedding.