Tag Archives: Tori Roloff

ethics, healthcare, Reality TV

Repost: Is it ethical to deliberately pass on genetic anomalies?

I am reposting this content from June 4, 2017, because it goes with yesterday’s reposted content about Huntington’s Disease. This post appears mostly as/is.

This morning, I read an interesting article about Zach and Tori Roloff of the TLC series Little People Big World.  I have seen the show before, but it’s been several years.  I didn’t know that Zach and Tori were expecting a baby, but the news popped up in my Facebook feed.  When I read it, I learned that their new son, Jackson, has achondroplasia, like his father, Zach.  That means that Jackson, like his father, has dwarfism.

Many different people shared this story.  I happened to read the version shared by George Takei.  Given the type of people who follow Mr. Takei’s Facebook page, the comments were a bit more controversial than they were on other places where this news was shared.  One guy– who was either really brave or stupid– posted this, along with an angry smilie.

The only species on earth that perpetuates its mistakes in genetic material on purpose.

Naturally, this comment prompted a number of outraged responses.  People were angry that this poster had the nerve to state that dwarfism is a “mistake”.  One lady posted this.

Excuse me? Being a little person is not a mistake.

Later, the lady who posted the above comment that identified her as a “little person” posted this…

No eugenics is also advocating that only people with “good genes” reproduce. Deciding whether or not to reproduce is a personal decision. My parents knew they had a probability to have a dwarf and still chose to have me. I am not genetically inferior. My birth and life are not a strike against us. Do you not understand what you are writing and how offensive it is to tbose of us who obviously do meet your criteria for being genetically correct.

I am not genetically inferior. I am shorter than average. Who decides what is a genetically superior height? I don’t need longer legs so I can hunt for food. My legs work well enough to walk through a grocery store, drive a car, and get me from point a to b. I don’t need strength to draw a bow. Science and technology keep me from needing to be a hunter and gatherer. I can type 130 wpm. I have a college degree and an IQ of 150. What is your idea of good genetics? Should I be 5’10”, white, blonde and blue eyed. I wear glasses. Should people with non 20/20 eye sight not reproduce.

I will grant that the poster who made the first comment was harsh and insensitive.  I can understand why people were offended.  At the same time, I wonder how many prospective parents are overjoyed to hear that their child has a genetic anomaly.  Do parents pray that their children will be born “different” somehow?  Do parents wish for their babies to be born with special physical, mental, or emotional challenges that might make their lives more complicated and difficult?  My guess is that the vast majority of them do not.  So, on one level, the first poster makes sense, even if the way he expressed himself is very objectionable.

This particular debate went on for a bit, so I’m not going to post the whole thing.  I will say that I noticed this subject brought out the emotions in a lot of people.  Many people seemed to be commenting from their hearts rather than their heads. 

It reminded me of an article I read in the Washington Post about fifteen years ago.  Bill and I were dating and, in those days, it was his habit to go out on Sunday mornings and pick up a paper for me.  I remember sitting on the floor in his shitty studio apartment and reading it over coffee and doughnuts.  I came across this featured piece about couples in the deaf community deliberately trying to have deaf babies.  I remember reading outraged comments about this movement.  Many people were offended that deaf parents would want to intentionally inflict deafness on a child.

From the article:

Several months before his birth, Sharon and Candy — both stylish and independent women in their mid-thirties, both college graduates, both holders of graduate degrees from Gallaudet University, both professionals in the mental health field — sat in their kitchen trying to envision life if their son turned out not to be deaf. It was something they had a hard time getting their minds around. When they were looking for a donor to inseminate Sharon, one thing they knew was that they wanted a deaf donor. So they contacted a local sperm bank and asked whether the bank would provide one. The sperm bank said no; congenital deafness is precisely the sort of condition that, in the world of commercial reproductive technology, gets a would-be donor eliminated.

So Sharon and Candy asked a deaf friend to be the donor, and he agreed.

From what I’ve read so far about Zach and Tori Roloff, which is admittedly not much, they didn’t necessarily plan to have a baby with dwarfism.  I’m sure they knew the risks and were okay with them.  And really, thanks to TLC shows like Little People, Big World and The Little Couple, more and more people are becoming acquainted with dwarfism and the challenges it presents, as well as the fact that little people can lead normal lives, perhaps with a few alterations of their environments.  I remember watching Jen Arnold on The Little Couple go about her business as a doctor.  I watched Jen and her husband building a house that was custom made for people who aren’t a normal height.  

Hell, the other day, I even started watching a German TV show (filmed in Stuttgart, no less) called Dr. Klein.  Klein is the German world for small and the star of the show, actress Christine Urspruch, is herself a dwarf who plays a doctor with dwarfism.  She wears a sexy red dress and red high heeled pumps.  She drives a little red car.  She has a family– including kids, although if memory serves, the kids on Dr. Klein are all normal height.

So yeah, a lot of people are being exposed to people who are different and realizing that they can have normal lives.  But does the fact that people who have congenital “defects” (for lack of a better word) mean that we should, as people, be actively trying to promote them?  Is it fair to deliberately pass on genes to a child who may have a hard time adapting to that condition?  Is it fair to handicap a child on purpose?

I have seen videos of hearing impaired people who hear something for the first time in their lives.  The looks on their faces are unforgettable…

This deaf lady hears music for the first time…
Grayson hears his father speak for the first time.

I’m sure it’s different for little people, although there are so many different types of dwarfism and they seem to affect people differently.  A person with a normal sized trunk and short arms and legs might have different challenges than a person whose body is proportioned, but very short or small.

In any case, regardless of how a person feels about this particular issue, I will go on record as saying that I agree that people must make their own reproductive choices, although I do think those choices should be made with much thought and consideration.  In the past, I have written about my neighbors whose family was heavily affected by Huntington’s Disease, a congenital and ultimately fatal disease that causes people to lose control of their bodies and eventually their minds. 

I remember my neighbor, who died at age 39.  She’d been a mother of three.  When we were children, she told me that she had a fifty percent chance of developing Huntington’s Disease.  I remember her father and her brother, both long dead now, who were very sick and disabled.  My neighbor’s father was forced to move into the local psychiatric hospital when he was in his 30s.  At one time, he’d been a perfectly normal man who was able to father two children.  When I met him, he was 32 years old and wheelchair bound.  He couldn’t speak normally or walk.  His son, then about 13, was also very sick.  He drooled constantly, required home care, and could not ride the shiny red bike parked in the garage.  My parents later bought that bike and gave it to me after the boy died at age 14.  I don’t think he’d ever been able to ride it, so it was like brand new.

Many years later, the boy’s sister, my neighbor and a friend, was afflicted with the same devastating disease.  Two of her three children are now dead, though neither died of Huntington’s Disease.  Both died in car accidents.  My friend’s daughter, aged 2, was tragically killed when her mother accidentally ran over her.  Her oldest son died just a couple of years ago in a more normal car accident.  They left behind a brother who has a fifty percent chance of getting Huntington’s Disease, and now he has a son of his own.  I wonder what it must be like for him, knowing that he might die at a young age of a cruel disease that his mother knew full well she was at risk of passing on to him.

Maybe dwarfism isn’t the same thing as a disease like cystic fibrosis or Huntington’s Disease.  Maybe being a little person is more like being born with one blue eye and one brown eye (I knew someone with this genetic “anomaly”, too).  Maybe it’s like being born with perfect pitch, which I have– although I’m not sure that is necessarily a genetic thing, since I don’t know of anyone else in my family who has it.  Maybe being a little person presents few challenges other than being really short and perhaps disproportionate.  Or maybe there will be challenges.  I don’t know.  But I must admit that article and the comments really got me thinking today.   

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ethics, healthcare, memories, Neighbors

Repost: Perpetuating the genetic nightmare…

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).

One of Woody Guthrie’s most famous songs…
Arlo Guthrie performs “Alice’s Restaurant”…
Cathy Guthrie and Amy Nelson performing as Folk Uke.

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. 

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