The following letter was written by Lydia, daughter of George and Naomi Weber from Ontario, Canada. Since they are a Mennonite family, you may notice a quaint mixture of Pennsylvania Dutch and Canadian expressions in Lydia's letter. She candidly expresses her feelings about her disease and reveals some of the questions and issues faced by teenagers with a disorder like MSUD. The letter was written in early August 1997 when she was fourteen. She was the first to respond to my request for letters from teens and adults with MSUD. Lydia's mother wrote that Lydia left to work for an aunt after she had written this letter. Her formula was weighed out in advance for each day and she was responsible to mix and drink it.
Lydia's brother, Benjamin with MSUD, turned 17 in October. His mother wrote that he can handle hard physical labor even during warm weather. They drive horses and buggies and Benjamin is not afraid to drive the young, not too well-broken, horses. He enjoys farm work, but reading and writing are hard for him. He is a little self-conscious about eating the low protein cake his mother sends along for him, whereas his younger brother Noah, with MSUD, loves desserts better than vegetables. Naomi wonders if eating so many calories and getting barely enough leucine could cause Noah to scream in his sleep.
The third boy with MSUD in the Weber family, Ishmael, died rather suddenly at the age of four years. He developed a fever and began vomiting after becoming overheated and exhausted on a hot day. This was in August of '82 and an account of his death was related in the Weber family history printed in our April '84 issue of the Newsletter. That family history also told of Benjamin's puzzling diagnosis. His father took Benjamin to Toronto to be tested on the day of his birth. His test showed negative, so he was taken home again. However, Ishmael became sick when Benjamin was born, and Naomi was very busy caring for the sick boy. It wasn't until Benjamin was eight days old, and would no longer suck, that they realized something was seriously wrong with Benjamin. A test for ketones showed very positive. He was taken to Toronto and diagnosed with MSUD. So this family has had many varied and trying experiences with MSUD. Thankfully all three MSUD are now doing quite well.