We had an estate sale this week and a 50ish man showed up late to pick up my old Weber Genisis Silver NG grill. I helped him get it into his PU truck as it was dark. He already had a newer Weber on the truck so I asked him what gives? He said repairing Weber’s is his avocation. The gulf coast weather & salt destroy the thin Chinese metal quickly. He said he is disabled with Huntington’s Disease. He asked if I knew what that was and I replied that I didn’t. He stated that the disease was like Parkinson’s and Altizhmer’s on steroids. I listened to him for a while as we strapped the two grills down:

Huntington’s is hereditary. The Air Force had him tested and found that he had the disease. The test ratings go from 1 to 13, with 1 being the presence of the Huntington’s gene and 13 is the end. He tested 9 a few years ago and is now a 7. The disease progresses faster when you are younger. His mother, who is German, is in a nursing home and is in the last stages of Huntington’s. She is cognizant but cannot speak or move. He finally got to see her recently and they both wept. Several male relatives in Germany have committed suicide. He has tremors in his hands and feet and early dementia. He has a 34yo son and a teenage daughter. He stated he would go out on his own terms.

Huntingtons chorea killed Arlo Guthrie's father. He worried his whole life that he would inherit the disease. Not long ago, Huntingtons was diagnosed when it appeared, generally past 40. Genetic tests are now availalble and much work is being done on a possible cure.

The Medic who taught our EMT class in 2010 had Huntington's Disease. Onset for him as in his 40's. His Father had it.

As mentioned, it is like a combination of two very cruel diseases.

On days when I get caught up in stupid shit, like some asshole who cut me off on the highway, or some idiot customer service rep, I try to think about what my Instructor is going through, and it helps put things into perspective for me.

cne32507,

Good on you to lend a kind, compassionate and patient ear to this gentleman.

It probably felt cathartic for him to let some things out, especially the mention of his ‘terms’ pertaining his final release from the insidious disease (which I am guessing he would not burden his children with this information).

Rob

Huntington's runs in my Mom's side of the family. My great grandmother had it, my grandmother had it, and both of my Aunt's had/have it. Mom died of cancer, but as part of autopsy we had her tested and she did not have it. Current research is that I cannot have it or pass it along.

It's a cruel disease to watch someone you love lose control of both their body and their mind. I concur with the OP's Parkinson's statement, but IME the mental side is a combination of cognitive decline and a mental illness. It also makes any other ailment they have much worse and/or harder to diagnose. With my Grandmother, it masked dozens of small strokes before she had the big stroke.

My aunt died of cancer in a small town, and I don't think their hospice had ever seen someone with both cancer and Huntington's. Hospice is usually pretty accurate when they tell a relative that their loved one's suffering will end in a day or two. They were off by 3 weeks with my aunt.

quote:

Originally posted by ZSMICHAEL:
Genetic tests are now availalble and much work is being done on a possible cure.

It takes a special mental toughness to get the diagnosis when you have zero symptoms and the best doctors can do is the equivalent of a band-aid on a bullet wound. My living aunt lacked that special mental toughness and that is as much as I'll say about that.

quote:

Originally posted by Cookster:
cne32507,

Good on you to lend a kind, compassionate and patient ear to this gentleman.

It probably felt cathartic for him to let some things out...

+1

tatortodd: Is your mother's side European?

quote:

Originally posted by cne32507:
He said repairing Weber’s is his avocation. The gulf coast weather & salt destroy the thin Chinese metal quickly.

My focus went to Weber - in the market for a new grill.

I thought Webers were quality US-built - whats this Chineee steel stuff??

quote:

It takes a special mental toughness to get the diagnosis when you have zero symptoms and the best doctors can do is the equivalent of a band-aid on a bullet wound. My living aunt lacked that special mental toughness and that is as much as I'll say about that.

^^^^^^^^^^
Genetic counselors deal with these issues. BRCA is a far more common problem. In the past without genetic testing the person in question often had children. It is a sensitive and complex issue as we learn more about genetics.

quote:

Originally posted by cne32507:
tatortodd: Is your mother's side European?

Yes, English

quote:

Originally posted by ZSMICHAEL:

quote:

It takes a special mental toughness to get the diagnosis when you have zero symptoms and the best doctors can do is the equivalent of a band-aid on a bullet wound. My living aunt lacked that special mental toughness and that is as much as I'll say about that.

^^^^^^^^^^
Genetic counselors deal with these issues. BRCA is a far more common problem. In the past without genetic testing the person in question often had children. It is a sensitive and complex issue as we learn more about genetics.

I could understand the generation without children getting tested, but in my Aunt's case she already had teenage kids when she got tested. Only upside was my cousins knew to get tested before considering having children. Tremendous downside to knowing you're inevitably going to lose control of your body and mind, and the docs are going to give you a handful of barely helpful pills with dozens of side effects of their own.

quote:

Originally posted by sgalczyn:

quote:

Originally posted by cne32507:
He said repairing Weber’s is his avocation. The gulf coast weather & salt destroy the thin Chinese metal quickly.

My focus went to Weber - in the market for a new grill.

I thought Webers were quality US-built - whats this Chineee steel stuff??

That's what he said. He said he could feel the difference in heft on the newer ones; both Genisis and Spirit. I rebuilt my 2004 Weber after about 12 years. Rusted through everywhere except the aluminum tub & lid. But then, everything ferrous rusts if you live near the ocean.

My mother, her brother, two of my brothers, and a nephew have all died from Huntington’s. I have a niece that’s tested positive, but as of yet developed no symptoms. It is the most unimaginable disease. Because of this, I dedicated the last 30 years to make sure that if I ever developed symptoms, my wife and children would not ever have to deal with the disease by themselves, like my father did.

I am turning 52 this year, and I feel that I have beaten the odds.

quote:

Originally posted by tatortodd:
...

quote:

It takes a special mental toughness to get the diagnosis when you have zero symptoms and the best doctors can do is the equivalent of a band-aid on a bullet wound. My living aunt lacked that special mental toughness and that is as much as I'll say about that.

^^^^^^^^^^
Genetic counselors deal with these issues. BRCA is a far more common problem. In the past without genetic testing the person in question often had children. It is a sensitive and complex issue as we learn more about genetics.

I could understand the generation without children getting tested, but in my Aunt's case she already had teenage kids when she got tested. Only upside was my cousins knew to get tested before considering having children. Tremendous downside to knowing you're inevitably going to lose control of your body and mind, and the docs are going to give you a handful of barely helpful pills with dozens of side effects of their own.[/QUOTE]

The Wiki article I looked at stated that at the time before testing was possible, people with indicators were polled and overwhelmingly wanted to be tested. But after testing was possible, that proved to be false. Now if a person with familial indicators wants to be tested, they have to have 3 counseling sessions, IIRC.

I had a longtime client, for about 10-12 years, and his wife passed away from it about 10 years ago. She would have been in her 50's at the time. As soon as she died he sold his business and left Florida for the North Carolina mountains. He had been her primary caretaker.

I remember having an in depth discussion with him about the disease one day. She must have had the testing early, because they knew she had it and that the disease would develop as she got older. For that reason, they decided not have children, because there was a 50/50 chance the child would have the disease. I will never forget David saying, "We could easily wipe out this horrible disease if the people that have it would stop having kids."