book reviews, family, healthcare

Repost: A review of Watching Their Dance: Three Sisters, A Genetic Disease and Marrying into a Family At Risk for Huntington’s

I originally reviewed this book on September 2, 2017. It appears here “as/is”.

I have mentioned several times on this blog that I once had neighbors whose family was profoundly affected by Huntington’s Disease, a fatal genetic condition that robs the afflicted of their minds and bodies.  Last month, I purchased Therese Crutcher-Marin’s 2017 book, Watching Their Dance: Three Sisters, A Genetic Disease and Marrying into a Family At Risk for Huntington’s.  I just finished reading the book today.

In the 1970s, author Therese Crutcher-Marin met her husband, John.  She fell in love with him and grew to love his three older sisters, Lora, Marcia, and Cindy.  John and his sisters had grown up without their mother; she was committed to a psychiatric hospital when they were young.  She later died, but it wasn’t until they were adults that they found out she’d had Huntington’s Disease.  Compounding the issue was the adult children’s father, Big John, who had never been much of a parent to his kids.  Big John had a second wife who was not particularly friendly to them, either. 

Every child born to a parent who has Huntington’s Disease has a fifty/fifty chance of inheriting the gene that causes the disease.  Every person who has the gene for Huntington’s Disease will eventually get the disease if he or she doesn’t die of something else.  It’s not possible to have the gene and simply be a carrier.

Therese loved John, but knowing that he may one day develop a very demanding disease that would eventually kill him at a young age was very difficult for her.  Eventually, the author decided that life is a crap shoot anyway.  She married John and they launched their careers and started a family, eventually having two children.  Meanwhile, each of John’s sisters developed Huntington’s Disease.

This book is mainly about Therese Crutcher-Marin’s experiences watching her beloved sisters-in-law getting sick and eventually dying.  The author’s husband opted not to be tested for the gene until after he was already past the age at which symptoms usually appear.  Fortunately, he did not have the gene, since he and the author had two children together.

For the most part, I found Watching Their Dance very informative and interesting.  It’s well-written and I admired how dedicated Therese Crutcher-Marin was to her husband’s family.  However, there were some parts of the book that I felt were a bit extraneous.  Sometimes the book read like a very newsy letter home; it included some information that didn’t necessarily seem relevant.  Therese does explain that she has problems with obsessive compulsive disorder.  She is a meticulous planner.  Perhaps that’s why this book seemed a little more detailed than it needed to be about things that weren’t pertinent.

I also feel that although Crutcher-Marin’s writing is mostly very functional and correct, her style isn’t particularly eloquent.  Some authors write effortlessly and colorfully.  Crutcher-Marin’s writing is serviceable, but not very artistic.  I got the sense that writing the story was hard work, although she did the work to high standards.

I did appreciate Crutcher-Marin’s candor about what it’s like to watch loved ones with Huntington’s Disease.  It’s a rare disease and a lot of people have never heard of it.  I happened to have seen it in person, so much of what she wrote about made sense to me.  Sadly, Huntington’s Disease has no treatment or cure.  The only thing that can be done is controlling the symptoms.  Moreover, it’s very difficult to find adequate care for people with Huntington’s Disease.  Those who have the disease do not die of it; instead, they die of complications arising from the disease process.  Many sufferers waste away because they can’t eat adequately or they develop an infection, like pneumonia.   

I can’t imagine how difficult it must have been for Therese, John, and their children to watch as Lora, Marcia, and Cindy each developed symptoms and eventually died.  Each of the sisters suffered in her own way.  One sister died quickly after developing a brain bleed after a blow to the head.  She had been taking Coumadin.  Another sister languished for years with Huntington’s Disease before she finally died.  The third sister developed the disease in her 40s, a late onset by most Huntington’s standards.  She managed to be independent and travel for some time before she, too, got very sick and died in her fifties.

I think Watching Their Dance is well worth reading, especially for those who know or love someone with Huntington’s Disease.  I am not aware of other books about what it’s like to be a caregiver to someone with HD, so this is a valuable book. 

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healthcare, history, nostalgia

Repost: Remembering David Vetter, the boy in the plastic bubble…

This post originally appeared on January 31, 2016. I am reposting it as/is today because it accompanies a book review I will repost after this.

The 70s and 80s were an interesting time to grow up.  They don’t seem like they were that long ago to me, but now that I’ve reached middle age, I can say with honesty that they were.  One story that intrigued millions of people when I was growing up was the story of David Vetter. 

David Phillip Vetter was born on September 21, 1971, about the time my parents conceived me.  He was from Conroe, Texas, a suburb of Houston.  He had an older brother, David Joseph Vetter III, who died the year prior to his birth.  Both David and his brother had a genetic disease called SCID (severe combined immunodeficiency).  It meant that they were born without immune systems that could effectively fight off infections.  The slightest illness could be fatal to someone with SCID.

A program about David Vetter.

Because David had this disease at a time before there was a treatment for it, he was forced to spend all but two weeks of his life in a plastic bubble.  I distinctly remember hearing and reading about David.  I also remember the 1976 film John Travolta, Robert Reed, and Diana Hyland famously starred in called The Boy in The Plastic Bubble.  I saw the movie many times when I was growing up.  It was based on the real life story of Ted DeVita, a teenager who had severe aplastic anemia.  Ted DeVita lived in a sterile hospital room for over eight years.  DeVita’s and Vetter’s experiences were fascinating to people all over the world.  DeVita died in 1980 of iron overload, caused by too many blood transfusions.

A preview of John Travolta’s movie loosely based on David Vetter’s case. At this writing, you can see the whole thing on YouTube.

I’m reminded of David’s story this morning.  There was a link to a New York Times retro report on my Facebook feed.  Though I hadn’t thought about David Vetter in a very long time, I quickly found myself recalling him as I watched the 12 minute video and read the accompanying article.   

Had he lived, David would be my age.  He had a bone marrow transplant that initially worked.  Unfortunately, the marrow had a dormant strain of the Epstein Barr virus in it.  The virus activated and David ended up with a virulent cancer that overwhelmed his body.  He died on February 22, 1984.

On David’s grave, the epitaph reads “He never touched the world… but the world was touched by him.”  Even now, knowing that children with SCID are no longer kept in plastic bubbles, I can’t help but wonder what life would have been like for David had he managed to become an adult.  Though he was able to accomplish a lot in his twelve years and provided science and medicine with new knowledge about a rare disease, there were so many things he couldn’t do.

A New York Times video about David Vetter.

NASA made David a space suit, which he wore a handful of times.  The suit allowed him to emerge from the bubble, though he remained tethered to it by an eight foot long cloth tube.  He never felt his mother’s kiss until he emerged from the bubble for the bone marrow transplant.  He never would have been able to have sex.  I even wonder if he ever saw a dentist… though, I guess if you live in a sterile environment, bacteria is not an issue.  Doctors worried what life would be like for David if he made it to teenhood or even adulthood.  Would he be able to tolerate life in the plastic bubble for en entire normal lifespan?

I am amazed by what David Vetter’s twelve years on earth did for the advancement of science, ethics, and medicine.  I am also amazed at how old I am now.  It seems like yesterday, I was just a youngster.  I look at those photos and videos of David Vetter in his germ free environment, knowing that was state of the art medicine for the 1970s and 80s.  What happened to him then would never work today. Nowadays, kids who are identified with SCID before they get sick are given bone marrow transplants.  In fact, in the video posted with the New York Times story, there is even a story about a woman whose son had SCID identified in utero.  He had a bone marrow transplant before he was even born.

When I was in high school, one of the most popular guys school developed aplastic anemia.  He ended up going to the National Institutes of Health in Bethesda, Maryland, where he died just weeks later.  The day Mike Haury died was coincidentally also the day 55,000 Armenians died in a massive earthquake… December 7, 1988.  I have read that aplastic anemia is now much more successfully treated than it was in the 80s.  Again, it just doesn’t seem like it was that long ago.  I guess it was.

Edited to add: Although I know that COVID-19 life is nothing like it must have been for David Vetter, in a weird way, the constant focus on contagion and masking kind of reminds me of him. I’m looking forward to having the next vaccine.

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