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31 May 2012 @ 10:28 am
The Real LJ Idol: "Secrets"  
real lj idol | week 29 | 942 words


My sister is disappearing.

This is something I've never written about, and I rarely talk about it in real life. Some things are so heartbreaking and hopeless that all you can do is deny them until even that choice is gone.

My oldest sister has Huntington's disease. This genetic disorder is also known as Huntington's chorea, a label which comes from the spastic movements it causes and which increase over time. More is known about it now than used to be, but none of the new information is good. The Mayo Clinic website describes it as a disease

"…that causes the progressive breakdown (degeneration) of nerve cells in the brain. Huntington's disease has a broad impact on a person's functional abilities and usually results in movement, thinking (cognitive) and psychiatric disorders."

My father's first wife had Huntington's, so we knew there was chance that my three older siblings might get it. We'd hoped it wouldn't happen, even as my older sister became irritable and agoraphobic (and later, paranoid). When her body started to become jerky and unsteady, we knew that hope was gone.

When you're at risk for having a disease like this, you're faced with the question of whether to find out if you have it. With Huntington's, if you carry the gene, you'll develop the disease. But there's no cure for it. Would you want to live your life with hope, or with certain knowledge of just how bad it's going to get?

My oldest sister was tested once she developed strong symptoms, so that those could be better treated. I don't know if my brother was ever tested; he shows no signs of the disease, and should have symptoms by now if he were going to get it. My next-oldest sister chose not to be tested, and I understand her choice completely. Knowing won't help anything, but the results could overshadow the too-short life you might have left.

We didn't expect the disease to change my sister's personality so much. She got very unpredictable, very angry at her husband, and sometimes irrational. She cut off all relations with me about sixteen years ago, when she started to imagine that my husband didn't like her. I didn't know her well growing up (she's fifteen years older), and we've lived in different states for about thirty years now. So I didn't see most of the progression that led to her being put in a nursing home about four years ago, after falsely calling the police on her husband and driving off to another town when she probably should no longer have been allowed behind the wheel.

Nobody told us that one of the first things Huntington's would do was take away the person she used to be.

For unknown but fortunate reasons, she unexpectedly reconciled with me a couple of years before that incident. She met my children for the first and only time, then; I can't say why, but with all the pain her estrangement brought, the fact that she kept it up after my children were born and was never interested in them was the thing that hurt the most.

I believe she was fifty-eight when that crisis episode happened. I know her physical limitations had gotten much worse before that, and I can't guess how her husband managed. She was put in the hospital, and they stabilized her mood enough that she was able to see she needed to make the care transition to assisted living.

I can't imagine the bravery and loneliness involved in making that decision, knowing that it was a one-way trip to a place where no one ever wants to be.

When she first got there, she was able to go for walks inside the buildings and on the grounds. She is no longer steady enough to move without a walker. She lives in a care-facility in an isolated part of Idaho, and her world is still getting smaller all the time.

I have spoken to her on the phone a few times, usually on holidays. It's hard. We don't really know each other well, and never did. But more than that, her condition limits her to small talk. I think there is only so much her mind can carry anymore—a few memories, social pleasantries, and things in the present. It is a blessing that she has somehow arrived at a state of contentment. This would be an unrelenting hell for her if she had not.

I know I need to visit her. The logistics and distance are as challenging as wondering what we'd say to each other. It would be best to go on a weekend when her husband is also there. He knows how much she can manage, and more than anyone, he knows how to please her.

She's losing ground more quickly, I'm told, which is one of the things I try not to think about. Like other degenerative diseases, this represents the body's ultimate betrayal. Her ability to feed herself, to escape the prison of her room or bed, will also someday fade away.

Her husband remains steadfast and loving, and her remote facility was chosen not for convenience but for the quality of care. It's unbelievably sad to know that this is the best that's available to her, and that it still can't save her or make any of this easier to bear.

She had to give up riding horses more than a decade ago, and the little freedom left to her continues to slip away.

My sister is disappearing, from the world and from herself. Now you know exactly why I try to pretend it isn't true.

cindy: spn - dean looks away (by oxoniensis)tsuki_no_bara on May 31st, 2012 06:12 pm (UTC)
this is heartbreaking and horrifying. *hugs*
The Coalition For Disturbing Metaphors: Wanderinghalfshellvenus on May 31st, 2012 06:17 pm (UTC)
It really is. Now you know why I keep it so buried. It's just hard to think about it, to know that there's nothing that can be done. This was so hard to write, but at the same time... also cathartic, I think.

*hugs back*
never trust a big butt and a smileobeetaybee on May 31st, 2012 06:16 pm (UTC)
Oh, sweets. This is so heartbreaking and I just want to hug you up and never let you go.
The Coalition For Disturbing Metaphorshalfshellvenus on May 31st, 2012 06:19 pm (UTC)
*hugs* It's a hard truth to tell, after all this time, not just because I try to keep it at arm's length but also because it never gets better. *sigh*
A Sentient Being: Fairyalien_infinity on May 31st, 2012 07:59 pm (UTC)
This is so sad and truly horrifying.

The Coalition For Disturbing Metaphorshalfshellvenus on May 31st, 2012 08:43 pm (UTC)
It is such a terrible, cruel disease, and I hate that my sister is going through this.

Thanks so much for your kind thoughts.
Kizzyxo_kizzy_xo on May 31st, 2012 09:50 pm (UTC)

I don't know if I'd get tested if Huntington's or something similar ran in my family. From what you wrote, some of it eerily describes what my mother went through with her dementia. It is horrifying, no question, but witnessing it day by day has its own unspeakable terror.
The Coalition For Disturbing Metaphorshalfshellvenus on May 31st, 2012 11:48 pm (UTC)
I know I wouldn't, unless I was at risk and thinking of having children. Some things, it's just better not to know, if you can't do anything about them.

Huntington's does cause dementia. Back when my Dad's first wife was diagnosed, none of this was known. I think it was thought to be a degenerative neuro-muscular disease then, rather than something that attacks the brain. Seeing his daughter go through the same behaviors that caused him to divorce his first wife made my Dad feel incredibly guilty, because he hadn't realized it was part of the disease. But it wouldn't have changed the fact that he was miserable with her.

We didn't know any of that with my sister at first, either. We just thought she was becoming neurotic and frustrated, or suffering some other kind of mental illness.

I live in fear that my next-older sister will some day have this too. God, but I hope not.
(no subject) - n3m3sis42 on June 1st, 2012 02:24 pm (UTC) (Expand)
Vice Captain of the Universesweeny_todd on May 31st, 2012 10:36 pm (UTC)
*gentle gentle hugs*
The Coalition For Disturbing Metaphorshalfshellvenus on May 31st, 2012 11:49 pm (UTC)
Thank you. I think I need them.
Lose 10 Pounds of Ugly Fat...  Cut Off Your Head.n3m3sis42 on June 1st, 2012 02:22 pm (UTC)
I'm so sorry. I have a friend whose dad had Huntington's. She did get the test because she wanted children but didn't want them to go through any of what she did. It was a really hard decision for her. What a horrible illness. :(
The Coalition For Disturbing Metaphorshalfshellvenus on June 1st, 2012 05:06 pm (UTC)
It's definitely a choice each person has to make on their own.

The disease strikes earlier and earlier in successive generations, so if you were at the point where it might start affecting you at 30-35, the case for getting tested would be much stronger. It's different if you want children, and maybe also different now that people know how much of a behavioral effect it has. My sister's physical symptoms didn't come until after age 50, but the mental symptoms (that we didn't know were part of the disease) started maybe 10 years earlier.

I so wish someone would find a cure for this disease, but I know that it's especially hard with genetic conditions.
(Deleted comment)
The Coalition For Disturbing Metaphorshalfshellvenus on June 2nd, 2012 06:24 am (UTC)
It has to be just devastating to her husband, but he loves her and must still see glimpses of who she used to be.

My greatest worry is that it's hard for her, that she knows what should be and isn't, and lives with that sorrow and frustration. I hope not.
devon99 on June 1st, 2012 09:14 pm (UTC)
Oh sweetie. This is so heartbreaking. I found it very moving that you would share something so personal with us.

*hugs you*
The Coalition For Disturbing Metaphorshalfshellvenus on June 2nd, 2012 06:50 am (UTC)
I wasn't sure I would ever tell this story to anyone, because to tell it you have to live through it again, and just so much worse when there's no room for hope.

But this prompt finally was the thing that made me think that this story needed to be told, and that the terrible struggle of what my sister is going through should somehow be commemorated. It began earlier than we realized, and stole so much more from her than anyone should have to bear.

Thanks so much for reading and commenting. I know this one wasn't easy.
basric: Basric dragon black gifbasric on June 1st, 2012 11:50 pm (UTC)
Another horrifying disease they don't spend enough money on research for. Well done.
The Coalition For Disturbing Metaphorshalfshellvenus on June 2nd, 2012 06:58 am (UTC)
Reading up on it for this story was hard-- it is a much more terrible disease than I realized, because all I knew was the "old knowledge" of it affecting the body's muscle control. It is so much worse than that, and like so many other genetic conditions, where do you start when the problem is encoded into the most basic parts of who we are?
Kristenpixiebelle on June 2nd, 2012 03:57 am (UTC)
This is heartbreaking. Degenerative diseases are scary. I've heard of Huntington's disease briefly, but you brought it to life here.

The Coalition For Disturbing Metaphorshalfshellvenus on June 2nd, 2012 07:01 am (UTC)
It's just so, so sad. "House, M.D." used to touch on it (and I mean "touch," because they never gave its impact its full due), and I was so glad when that storyline went away. It's too personal a condition, with three siblings at risk for it.

Thanks for the kind words. They do help.
Jemima Paulerjem0000000 on June 2nd, 2012 05:30 am (UTC)
The Coalition For Disturbing Metaphorshalfshellvenus on June 2nd, 2012 07:02 am (UTC)
I need those now more than ever, after having written this. I wasn't kidding when I said this story would be the Angst of Doom, because I knew that going in. It's such a painful topic and truth.
whipchickwhipchick on June 2nd, 2012 06:22 am (UTC)
I am more frightened of having a degenerative disease than anything else - my friend with MS said the hardest part was losing (the use of) her hands.

Sympathy, yes - but this is also a well-written piece, and you've done a really skillful job juxtaposing your personal fears and worries with the actual medical issues your sister is facing.
The Coalition For Disturbing Metaphorshalfshellvenus on June 2nd, 2012 07:09 am (UTC)
Knowing that every bit you lose is not coming back makes degenerative diseases so much harder, I think-- and that it's often also a slow decline. These conditions are so awful and so very unfair.

I hope that, in the end, this story is more about my sister than it is about me. No amount of worry or sorrow can ever be as hard as having to live through this happening to you.

I can't tell you how grateful I am that her husband has stuck by her and visits her every other week. The only true piece of luck she ever had was in finding him.
jacq22jacq22 on June 2nd, 2012 07:20 am (UTC)
I am so sad for you and the family. It really is a horrible disease, and I worked in a nursing home a few years back and was involved with one man (in his 50's) who had it, and the day to day battles were tragic. I know his wife, and we talked a lot. He is no longer around. I feel for you because its the hardest thing to deal with. He had three children and one did not get the test.

A brave entry.
The Coalition For Disturbing Metaphorshalfshellvenus on June 2nd, 2012 07:45 am (UTC)
I see you've had experience with this up close, and think again about how lucky your nursing home patients were to have you. My sister's facility seems to have good people who like and care for her, and that matters enormously when your journey ends at a place like that.

When I look back at that long period where she cut off all contact with me, it's even more frustrating to realize that we lost that time to a stubborn delusion. But they ARE all stubborn in a case like this, because the person doesn't realize their mind is lying to them. Everything else must seem relatively normal enough for them to believe their perceptions are correct.

I'm just glad she got past that particular paranoia, even if there was very little time left before she lost a lot of other things as well. At least, for the moment, her personality is in a pleasant state. It's a saving grace, considering the day-to-day she still must cope with.

Thanks so much for reading and offering your thoughts on this. It's such a hard thing to discuss, and I know it must be terribly hard to read as well.
nodressrehersalnodressrehersal on June 2nd, 2012 02:29 pm (UTC)
Heart-wrenching and well told. By sharing your sister's story, you've allowed others to share their experiences as well; and although nothing changes, I believe there's comfort to be found in community.

The Coalition For Disturbing Metaphorshalfshellvenus on June 4th, 2012 05:10 pm (UTC)
That is a wonderful thought, and I think you're right. I felt so incredibly sad after writing this, but reading other people's thoughts and experiences in the comments has really helped. I don't understand why, but it really does.

Thanks so much for reading this one.
(no subject) - nodressrehersal on June 4th, 2012 06:46 pm (UTC) (Expand)
imafarmgirlimafarmgirl on June 2nd, 2012 07:35 pm (UTC)
This is so sad. It is a disease that robs people and families of so much.
The Coalition For Disturbing Metaphorshalfshellvenus on June 4th, 2012 06:35 pm (UTC)
It really does. We knew it was bad, years ago when it was still just a possible threat, but the mental/emotional component makes it far worse than we imagined.