Huntington's disease (progression question)

The Lonely One · Aug 15, 2009

Hello. I am asking a question about Huntington's disease, as I am in the final stages of editing and fact-checking a short story before submission.

WARNING: the elements of the disease in the story are both sad and uplifting as the MC couples with them, but if you're very close to this please know I have no intention of upsetting you. I know it's a serious disease and treat it as such in the story. Just giving a courtesy heads up if anyone would have trouble reading on.

My character's mother suffers from the disease, and ultimately succumbs to complications at the end of the story. Her progression with Huntington's is approximately over a 10 year period before ultimately she dies.

Is this time period realistic? (The best answer I can find online is that it "varies greatly" from person to person.)

Is there a minimum or maximum time period for which the disease progresses into the later stages where swallowing is difficult, infection and other problems more likely, etc.?

Thank you for your responses. I am attempting to be as sensitive but direct and realistic with the topic as possible. Always a hard mix.

Kitty Pryde · Aug 15, 2009

This is the best source of info on the subject that I know of. It's meant to explain the disease to the average non-medical-expert person: http://hopes.stanford.edu/index.html (And some of my old profs and classmates worked on creating it!

)

I believe severity of the disease depends on how many repeats of the disease-causing trinucleotide on the huntingtin gene the person has. The more repeats, the longer and tanglier the huntingtin protein is, and the more severe the disease is. General prognosis is 10-25 years between diagnosis and death, so I think you've done fine. And you're right that over time a person with Huntington's becomes more susceptible to various life-threatening problems. I hope that helps!

ChaosTitan · Aug 15, 2009

My grandmother is in the end stages of the disease, and she was diagnosed about twelve years ago. It is a very difficult thing to see a loved one go through.

Your timeline sounds realistic. The first few years after diagnosis were the most difficult, as both she and the family accepted and adapted. Medications and dosages were tried until a combination was found that worked for her - not only for the uncontrollable movements, but also for depression. Outbursts and fits of crying were not uncommon, and they still occur regularly.

Her condition is exacerbated by other things (including a stroke 18 months ago that left her wheelchair bound). She also suffers from acute dementia, although I'm uncertain if that's from Huntington's or age.

If you'd like me to take a peek at the story, feel free to PM me.

The Lonely One · Aug 15, 2009

Thank you both. I appreciate this very valuable advice.

ChaosTitan, and anyone for that matter, if you'd like to take a look at the story it's here in Literary SYW (See post 4 for most updated draft):

http://www.absolutewrite.com/forums/showthread.php?t=147642

It's called "My Mother Who Teaches Dance" in reference to the phrase Huntington's dance.

Feel free to PM me with responses if you would feel more comfortable doing so.

katiemac · Aug 15, 2009

I believe severity of the disease depends on how many repeats of the disease-causing trinucleotide on the huntingtin gene the person has. The more repeats, the longer and tanglier the huntingtin protein is, and the more severe the disease is.

Actually, I was under the impression the number of repeats of the trinucleotide will predict roughly at what age you will develop Huntington's but not the severity of it.

GeorgeK · Aug 15, 2009

My brother in law died of Huntingtons a few years ago. He and his sister were the first identified cases in his family because his parents and their parents died of unrelated problems at younger ages prior to the typical onset of HD. Women tend to get symptomatic at earlier ages than men but for men, the 10-25 year progression seems accurate for both what I've seen and what I've read in the medical literature. My BIL seemed to sundown a lot, (during the days seemed tired but affable, but according to my sister at night would become violent and almost demonic).

StephanieFox · Aug 17, 2009

A doctor I know told me she had an 89 year old woman patient who was in the beginning stages of the disease. I don't know if I believe this. I'm just reporting what I was told.

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