I am reposting this piece from August 30, 2017, because it goes with the book review I reposted today. It appears as/is. Special thanks to Leevan Jackson who made the featured photo available through Creative Commons.
I have written a few times about my childhood neighbors, people who lived across the dirt road from us in Virginia. In 1980, when we moved to Gloucester, they were a family of four. There was a mother, father, brother and sister. The father did not live at home. He was in his early 30s and lived at the local psychiatric hospital in Williamsburg because he was suffering from Huntington’s Disease. The mother was raising her children, twelve year old Michael and nine year old Leslie, by herself.
In 1980, Michael was also suffering from Huntington’s Disease, having inherited the defective gene and developed the disease much earlier than most people with Huntington’s Disease do. He died in 1982. His and Leslie’s father died a couple of years after that.
I was eight in 1980. I met all of these people when I was a child. Leslie’s mother worked for my dad for several years until Leslie’s paternal grandmother died and Leslie’s mom inherited some money. Leslie’s mom bought her own picture framing business and competed against my dad. The business eventually failed.
Leslie and I weren’t close friends, but we did grow up together. My parents included Leslie and her mom on a couple of family trips. In 1985, I remember we all went to Kitty Hawk, North Carolina together. I seem to remember Leslie and her mom coming with us to Natural Bridge, Virginia once, too. I grew up waiting for the bus with Leslie and used to dog sit for her mom when they’d take trips to the Smoky Mountains. Leslie was bright and talented and probably could have done some great things had she not been doomed to get Huntington’s Disease while still fairly young.
Leslie died in 2010, having battled the disease for several years. She was 39 years old. She’d given birth to three kids. One of them died in a freak accident in 1995. Leslie’s little daughter, just two years old, was accidentally run over by Leslie at the local Walmart. For some reason, Leslie had allowed the little girl to stand up behind the driver’s seat. If I recall correctly, she was just coasting forward to the drink machines and, for whatever reason, decided not to put the kid in a car seat. Leslie had inexplicably left the car door open and the girl fell out and ended up under the car’s tires. Her older child, then just three years old, was also there. In 2012, when he was 21 years old, that child would also die in a car accident. I have to wonder if the accident was really an accident or if the young man had started getting symptoms of Huntington’s Disease and decided to commit suicide.
In 1996, Leslie had her third child, another son. He is the only one of her children still living. I have never met Leslie’s youngest child, but I know his family well. I also know his dad, since he was in my class in school. In the 80s, Gloucester was the kind of place where everyone knew each other. I also knew of Leslie’s older son’s family, since his grandmother used to clean my parents’ house.
Last night, I decided to look up Leslie’s sole surviving son. I see that he recently became a father. I have to wonder how much exposure he got to his mother when she was sick. I didn’t see Leslie during those years because I left our hometown, but I do remember meeting her father and seeing her brother on a daily basis. I remember what Huntington’s Disease looked like at an advanced stage. It’s absolutely devastating.
I just started reading a book about a woman who married into a family with the Huntington’s Disease gene. The woman fell in love with her husband before he knew his mother had Huntington’s Disease. She was dating him when he and his three older sisters found out why their mother wasn’t around when they were growing up. She’d been in a psychiatric hospital. The family patriarch wasn’t much of a father figure, so it was left up to the eldest daughter to take care of everyone. Somehow, the four kids grew up not knowing that their mother had a genetic disorder. I’m probably halfway through the book so far… The author decided to play the odds and have a son with her husband.
I learned in the book I’m reading that famed songwriter, Woody Guthrie, had Huntington’s Disease. He had eight children, five of whom died young. His second of three wives, Marjorie Guthrie, started what would eventually become the Huntington’s Disease Society of America. Marjorie had four children with Woody, including famous singer-songwriter Arlo Guthrie. Woody was married to his third wife for just a year before they split; he died in 1967 at age 55. Woody was never really treated for his disease. People thought he had schizophrenia or was an alcoholic, due to the extreme mood swings the disease caused. Because he was famous, his death brought awareness to Huntington’s Disease. It looks like Arlo and two siblings have escaped their father’s fate. Today, I very much enjoy listening to music by Arlo’s daughter, Cathy, who plays ukulele in the duo, Folk Uke, with Amy Nelson (Willie Nelson’s daughter).
Every child who has a parent with Huntington’s Disease has a fifty percent chance of developing the disease. Huntington’s Disease, although genetically perpetuated, is not like cystic fibrosis. With CF, both parents must have the genetic defect. Even then, a child born to parents carrying the CF gene has a one in four chance of getting cystic fibrosis, a one in four chance of being clear of the gene, and a two in four chance of being a carrier. With Huntington’s Disease, it’s a one in two chance. And if you have the gene, you will get the disease and likely die from it. There is no treatment or cure for Huntington’s Disease.
A few months ago, I wrote a post about the ethics of knowingly passing along defective genes. That post was inspired by Zach and Tori Roloff, stars of the TLC show Little People Big World. They’d just had a baby and some people were saying that they shouldn’t have, since Zach has achondoplasia. Their son, Jackson, also has achondoplasia, which is a type of dwarfism. For the record, I will say that dwarfism is not quite the same thing as something like CF or Huntington’s Disease. A person who has achondoplasia can be basically healthy, though abnormally short. CF and Huntington’s Disease are very serious and debilitating.
I think Huntington’s Disease, in some ways, is crueler than CF is. Many people with CF are sick from babyhood. They grow up sick, although some sufferers are much sicker than others. They often know from a young age whether or not they will be affected by CF.
A person with a family history of Huntington’s Disease can start life completely normal and not get sick until they’re approaching middle age. They can develop lives, start families, have careers, and ultimately be stricken by a disease that makes them lose control of their bodies and their minds. People with Huntington’s Disease grow up wondering if and when it will strike and whether or not they should get tested for the gene. If they get tested, the news could be good. They might not have the gene. Or it can be bad; they have the gene and will eventually get very sick and probably die young.
Leslie’s family was devastated by Huntington’s Disease. It seemed the gene in her family was worse than some others. I remember hearing that her grandfather, whom I never met, had the disease. He’d been adopted and never knew he had a genetic anomaly, so he and his wife, Vashti (whom I did meet), had a family. I know that besides Leslie’s dad, at least one other sibling got the disease and died young.
I remember my mom telling me, quite emotionally, that Leslie’s mother should have had her daughter’s tubes tied when she was a baby. I explained to my very practical mom that it would have been unethical to tie Leslie’s tubes. What if she had been born clear of the gene? There was a fifty percent chance that she had the defect, but there was also a fifty percent chance she didn’t. She could have lived a completely normal life. In 1971, when Leslie was born, I doubt the technology was there to know. By the time genetic testing was available, I’m sure Leslie didn’t want to know.
I wish Leslie’s grandson much luck. I truly hope he isn’t going to be afflicted by this terrible disease. Life is a crap shoot. He has an aunt and uncle who may or may not have had the genes for Huntington’s Disease and still died very young. Not having the misfortune of being burdened by a genetically passed disease myself, I can’t even know what it’s like to live with the knowledge that I’m doomed. Hopefully, Leslie’s son and grandson have escaped Huntington’s Disease. That disease is a fate I would not wish on my worst enemy. On the other hand, if Woody Guthrie hadn’t had children, we would be missing out on some great music.
I’ll write a review of the book when I’m finished with it. It’s amazing what provides food for thought… And it’s also crazy that I know so much about someone I’ve never met. It’s not the first time this has happened, either.