Huntington Disease Lighthouse Families

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Always Wanted to Ask

Posted by colleen 
Always Wanted to Ask
June 15, 2012 12:26AM
We all know that HD is a 50/50 disease so why does it seems that the numbers never add up?

Grama sibship 4/4
Dad 3/5
Mine 2/3

Does anyone come from a family of 50/50?

Better yet, anyone from a ..."nope.... no one got it in our family", where the 50% worked in their favor?
Re: Always Wanted to Ask
June 15, 2012 04:46AM
My wife was at risk as were her two sisters. My wife tested negative, and I thank God for that every day, her younger sister had HD and died from the complications a few years ago. She left behind a daughter that is 21 so we pray every day for her. My wife's older sister died of Diabetes at the age of 24 so we'll never know there. The girls father died of HD many years before that.
In a small town near us, an older man and his son have HD and the grandson has JHD. I'm not really sure if there are any more possibilities of people becoming affected by HD in that family.
Re: Always Wanted to Ask
June 15, 2012 07:40AM
Hi all,

I know when people hear 50/50 it's common to relate risk to the number of HD affected persons within a family unit, but 50/50 is the chance of inheritance each time life is created.

Meaning....each individual child has a 50/50 chance at the time of conception to either get the expanded gene or not.

We all have 2 copies the Huntington gene (one from each parent). Considering the typical situation in which one parent has HD and the other does not, the child will get either a normal gene or an expanded gene from the affected parent, in addition to the copy received from the unaffected parent. Thus the 50/50 chance.

The odds increase if two individuals, both with HD, have children together or one person has two expanded HD genes...not sure what the exact risk is in these situations?

Overall - 50/50 defines the risk for each individual person, not family. So you will find families where all, none, or any number in between get the HD gene.

Hope this make sense? Someone else may be able to explain it better than I did?

Take care,
Krista
Re: Always Wanted to Ask
June 15, 2012 08:47AM
I know that the risk for every person with a parent with HD is 50/50, but as well In our family its been 50/50 for the last two generations. My grandfather died from Huntingtons, not sure if there was another sibling with it or not, a few left home during the depression years and no family contact was made. My father was positive (don't know his count) and died at 49 yrs old. My aunt, his only sister was negative. I tested last year, after years of struggling with to test or not to test, and I'm negative. Very very lucky. My younger brother is not so lucky, in denial, untested, but displaying symptoms rather severely for the last few years. He is 47, and I fear will not live to see 50. I have two children, who are safe! He and his wife decided to not have children, so the gene in our family stops here. It has definately taken its toll and we all mourn the loss of our brother/son/uncle who was an amazing person, and still is but just in a different way.
Re: Always Wanted to Ask
June 15, 2012 09:45AM
It's like a Roulette Wheel. No matter how many times you spin it, the odds are still 50/50 for Red or Black. And, the Wheel has no memory - it doesn't know, for example, that the last 5 times were Black.

Will
Re: Always Wanted to Ask
June 15, 2012 04:03PM
Was just hopeful that there were families out there where HD was not so heavy-handed.
Re: Always Wanted to Ask
June 15, 2012 04:32PM
So far in my husband's family he is the only one to be diagnosed with HD. There are 3 others that I think had it, but were misdiagnosed. My husband has 2 siblings and so far they are both fine. Both are quite a bit older. I believe that my MIL had it along with her mom and uncle. Mil was an only child, but did have several uncles and an aunt.The uncle had 3 kids and they all seem to be fine. All of his grand kids are ok as far as I know too. So 3 people out of a fairly big family is quite low. Past my. Mother in laws mom and uncle I don't know the history going back further. I'm kind of thinking that in my husbands family everyone had late onset except for my husband who had a traumatic brain injury. So who knows, either there are a lot of lucky people in the family or they just aren't old enough yet to show symptoms. Only time will tell.
Re: Always Wanted to Ask
June 15, 2012 04:55PM
In our family, My paternal Grandmother and her sister had late-onset(probably came from her father). Her sister was diagnosed as Parkinsons. My ftaher and one brother had late onset HD (2 out of 4 boys). My father's brother's son drowned at the age of 17. He had a seizure disorder and that may have been Juvenile HD. We have no idea. My uncle's other child is 65 and asymtomatic as far as I know. Two of my father's cousins had mid-thirties onset. My brother had mid-thirties onset. (There werer 4 of us) I have 37 repeats and I don't know about my niece and nephew.(My deceased brother's kids) My niece looks OK when I see her pciture on facebook.She's in her early forties. I have no contact with my nephew. So we don't havwe a whole lot of people with HD.
Re: Always Wanted to Ask
June 16, 2012 01:04PM
In Ray's family there are eight children altogether -- 3 with HD and 5 without.
Re: Always Wanted to Ask
August 17, 2012 09:34PM
my husbands family his grandmother was diagnosed wrong was sent to a mental hospital we now kmow it was HD his mother and brother died from compicatiions of HD his sister has HD and in a NH with dont have any contact with her..there are 6 grandchildren all under age of 21 to 14 none have been tested yet. my husband was diagnosed in 2005 but i know symptoms started in 2001.
kjuni you post was worded perfect for everyone to understand thanks
Re: Always Wanted to Ask
September 09, 2012 08:21PM
My grandmother and one of her sisters (that we know of) died of HD complications. We think they inherited it from her father who died in a farming accident. Not sure about previous generations and other portions of the family including her deceased pHD sisters who refuse to acknowledge that it was HD.
Out of her 13 children:
1 uncle died in infancy of other causes
pHD: my mother, 2 aunts, and 2 uncles (1 died of HD)=5 so far
2 aunts tested neg
5 aunts/uncles are untested & asymptomatic
11 of the 13 all have lots of children and grandchildren
Of those next 2 generations:
my sister is pHD
no one else positive or tested yet....but many getting very close to the age where symptoms may start showing.
I pray often that it stops in our family...
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