Back in Agust of 2005 my wife was diagnosed with Huntington's Disease. At the time, her only symptoms were what I called "nervous habits". Constant hand movements, hand wringing, some facial grimacing, and the occaisonal head bob. These "ticks" started showing up just before our second child was born in 2003. We were told at the time of diagnosis that onset and progress speeds varied from patient to patient, but that 10-15 years was a typical timeframe.

I was not prepared for what the reality has become.

By May of 2006 I was forced to take away her driver's license because she was no longer safe to operate a vehicle. She could not focus on the task of driving and would often just drive off the road.

By July, 2006 it was clear she could no longer care for the children. They would wander off, and it would take 20 minutes before she even realized they were no longer in the room. That combined with an occaisonal violent outburst, and it was time for a nanny to watch the girls.

By the summer of 2007 we were having to remind her to take care of basic hygiene, and to chew her food. We had to cut everything into swallow size pieces or she would choke.

By January 2008 she needed 24/7 supervision. I was forced to put gates on the stairwells and locks on the bedroom doors to keep her from wandering the house at all hours waking everyone up, drinking all the milk, and falling. (Yes, she would drink an entire gallon and then get sick.) The nightly disruptions got bad enough that she started spending 1 week a month at my parents house so that I and the kids could get some sleep. By June that was up to 2 weeks a month, and by Christmas it was nearly full time. Mom's a registered caregiver, and get's help from the local nursing home via Adult day care. She's been a gift from God through all this.

Anyway, forward to today. She has been falling several times a day. (7 yesterday) We try to use a wheelchair and walker as much as possible, but she is insistent on walking around without them. She will push the walker away and then proceed to fall down almost immediately. With all the bruises, cuts and scars, she looks like a UFC fighter. The obsessions are through the roof...she would run over kittens to get a pepsi, and feels the need to visit the bathroom every 30 minutes or less. It is truly chaos and I just can't imagine things getting worse.

All through this we have been trying various cocktails to control the movements, fight depression and anxiety, etc. Even got a hold of some tetrabenazine through Canada. In most cases the side affects were far worse than the help. After all the trials, it ended up being 1mg of Clonazepame that did the trick. Her chorea is almost non existant. But the cognitive side of things has deteriorated so rapidly...It's not even been 4 years and it seems like we are near the end.

Her CAG is 51, so it's not extreme. Her mother had it, but was never diagnosed with it. She was diagnosed with MS, and my wife's grandfather was diagnosed with Parkinson's. Now that we know my wife's diagnosis, we can safely say that those were improper diagnosis. Anyway, I knew my wife's mother for 7 years before she passed away, and in that 7 years I saw very little deterioration. My wife is past the point where her mother died from choking. This rapid onset has caught me completely off guard.

Anybody else experience such a rapid progression of the disease?

First of all I want to say how much my heart goes out to you and your family.

My wife's progression has seemed to move pretty quickly, not as quickly as your wife, but from the time we met 4 years ago to now, she has gone from non-existant symptoms to not being able to hold a conversation and choking on everything and falling down all the time, running into everything in the room. We are at the point of not letting her drive anymore because she is definately endangering herself and everyone else on the road. Most people see her and assume she is drunk or on drugs.

The only thing I have been able to come up with , is that maybe she was symptomatic longer than we thought, and they were all just soft symptoms, and maybe when the physical symptoms appeared they were in a later stage than we realized.

Unfortunately every Phd is different and its hard to say with any certainty what is normal.

yahkin,
Just wanted to say I'm very sorry for what you are going through, how old is your wife? She's very blessed to have you.

Yes, the progression does seem rapid. I'm sorry to hear that things are so difficult but am glad you have her mother's support. My brother's symtpom's started in his mid thirties and he died at the age of 52 of an aspiaration pneumonia. According to his brain biopsy,after death, he was not yet in the final stages of the disease. I don't know what his CAG was since they didn't have the test then. Dad's symptoms were very mild and started in his sixties. He was still driving and died in a car crash at the age of 72. I thought a CAG of 51 was relatively high for an adult, but I don't know whether or not that leads to faster progression. There is so much that they are still learning about the disease.

She is 36 now. As I look back I realize the very first symptoms were when she was 29. She would do this odd thing with her hand all the time. When our second child was born the nurses were worried about how she was acting. I said that was normal for her...They said there was nothing "normal" about what she was doing. It's funny how things can change right under your nose without you even knowing it.

We didn't pursue any testing the first year of our daughters life...cuz, well...newborns are a lot of work. In 2004 we had her tested for MS since that is what her mother supposedly had. The neurologist we saw was a real jerk and wrote off what she was doing as an attempt to get attention. That turned us off to "those" guys for a while, but the movements kept getting worse. So, testing started in earnest for the cause early in 2005 with a new neurologist. She tested for everything in the book in hopes of finding anything but Huntington's.

Once she mentioned Huntington's as a possibility, I read up on it and had myself a good cry. I knew from my experiences up to that point that the test was just a formality. I also knew right away that her mother had been misdiagnosed. Hindsight's 20/20.

Today was a good day. Only one fall in the living room...and onto her butt. One day at a time.

Philip Wrote:
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> The only thing I have been able to come up with ,
> is that maybe she was symptomatic longer than we
> thought, and they were all just soft symptoms, and
> maybe when the physical symptoms appeared they
> were in a later stage than we realized.
>

I've wondered this myself. After our first child was born, she seemed to be a lot more clumsy. We of course wrote it off to hormones from being pregnant and breastfeeding. How many symptoms did we just overlook because of the pregnancies and lack of sleep that comes along with newborns?



Edited 1 time(s). Last edit at 07/27/2009 08:41PM by Yahkin.

Yahkin,

I'm sorry to hear about your wife. It sounds like you have gone way beyond the call to take care of her.

My wife suffered a similar decline and in about the same timeframe. Her CAG was 46. 51 is pretty high really.

How is the rest of the family taking it?

Gee, i'm so sorry to hear this. It does seem fast to me, but ive heard of others here on the forum that their spouse has been fast too. It makes me feel very sad, and fortunate too. I was diagnosed in 04 as early stages, but here it is 09, and ive actually had some improvements. My cag is very low, and so that helps. Nice to meet you and welcome.

Yahkin,

My son Tim, also with a CAG repeat of 51, was diagnosed in 2007 at the age of 28. He was married, working and socially active. He has progressed very rapidly in the last 2 years. His wife and he are now divorcing and he lives with me and my husband. He no longer is able to drive or work and has almost no social interaction because his friends "can't bear to watch him decline".
Our only respite from care is our other son (at risk) who takes him for a weekend or a day when he can.
We have been through/will probably continue to go through, the behavior disturbances, obsessions and refrigerator raids (on milk) . He falls and says he didn't, barely speaks and blasts the stereo so loud I can hear it from two houses away...the list goes on and on.
We finally got some medication for him after he threatened to kick my husbands butt and take his truck because he "can drive!"
The last couple of months have been relatively quiet as far as outbursts. I think he had symptoms much earlier than we had thought, possibly from very early on. He was treated as a child for ADHD and his teens were tumultuous to say the least.
Keep coming here, it has saved my sanity on more than one occasion!!
Take care of you and the kiddos...
Carla
PS There are also some very wonderful, warm and nice days, when he thinks clearly and engages in his surroundings sprinkled in. Those are what I hold on to when Huntington's rears its ugly head.

Fred Wrote:
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> How is the rest of the family taking it?

Kids are resilient thankfully. Our youngest loves to help out where she can. I really make sure that she doesn't feel obligated though. I don't want her feeling any responsibility. They still know it's mom, and love to cuddle up in her lap and watch some TV with her.

Our oldest is special needs as well. She has Angelman's syndrome, so she cannot talk to us about what is going on. It is clear she understands things are not as they should be though. She get's frustrated with us having to give mom so much attention. (Yes, two completely unrelated genetic disorders in the same family. Perhaps I should be playing the lottery.)

Both began to really struggle in school when she was keeping us up at night, but with her staying at my mom's now, they have recovered well. What doesn't kill us makes us stronger.

carlaj Wrote:
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> Our only respite from care is our other son (at
> risk) who takes him for a weekend or a day when he
> can.

Not sure what state you are in, but Minnesota has been a lot of help as of late. We put her in daycare 5 days a week, and they even offer in home help so we can take vacations, etc. Getting a break is vital...I was going literally insane before we got help.

> We have been through/will probably continue to go
> through, the behavior disturbances, obsessions and
> refrigerator raids (on milk) . He falls and says
> he didn't, barely speaks and blasts the stereo so
> loud I can hear it from two houses away...the list
> goes on and on.

lol...I totally forgot about the TV blaring. We have to take the batteries out of the remotes or it's at max volume after about 10 minutes. What's up with that? heh.

And one of those locking boxes on the thermostat was needed as well. I was working on my computer one day and realized I was sweating my head off. Went over and the thermostat was set to 93! Apparently she was cold.

> We finally got some medication for him after he
> threatened to kick my husbands butt and take his
> truck because he "can drive!"

Fortunately, the "I can drive" stage did not last very long for us. We got a lockbox for the keys because she kept threatening to drive to town when i wasn't looking.

> Keep coming here, it has saved my sanity on more
> than one occasion!!
> Take care of you and the kiddos...
> Carla
> PS There are also some very wonderful, warm and
> nice days, when he thinks clearly and engages in
> his surroundings sprinkled in. Those are what I
> hold on to when Huntington's rears its ugly head.

Yeah, I get surprised from time to time as well. She'll have a great day, be understandable to talk to, and often times brings up fun things we did in the past.

A funny story. Her father gives her $20 a week just to spend on whatever. (This started because she was stealing from the nanny's purse for money...even though she didn't need it.) Well, now she is fully supported and really doesn't have an opportunity to spend the money, she likes to keep track of how much she has. The other day I was talking with her and she brought up the amount of cash she had in her purse. I reacted with surprise and asked her if she had been turning tricks again. Totally cracked her up. She laughed for a good 5 minutes which got all of us laughing as well.

I think 51 CAG is very high - over 60 and it is counted as Juvenile HD. There has been some suggestion that trauma, including pregnancy and childbirth but also car accidents etc, can bring on HD, or accellerate progression. We all know stress is bad for PHD, I'm guessing a traumatic event is stress times 100. I would really like this concept investigated further.

Also the hearing - a lot of PHD's have been misdiagnosed with hearing loss. They do have an increased wax buildup, but most do not have hearing loss - just slower auditory processing. From a young child, Dad would be very sensitive to noise - turn the TV up a fraction, down a fraction, no too much! Hated any noise - from kids, musical instruments, anything. Then later he was diagnosed with industrial deafness. They have had to flush his ear canal out several times. He also suffers vertigo more frequently than the rest of us and it really affects him. Definately needs more research on HD's affects on the ear and hearing.

It sounds like you have done a brilliant job on caring for your young children and protecting their mother - you should be nominated as Father of the Year! It is certainly no easy job! Take care, Shy

OMG, your child has special needs and your wife has full blown HD?

Wow. You are one helluva man to hold all that together.

I just wanted to say welcome to the forum . You have a lot of challenges . I hope some of the thoughts of peoples here will give you some insight . It has certainly helped me over the last few years . My best. db

Yahkin,
I live in Missouri on Huntington Drive if you can believe that! I am going to be looking into some form of care giving aid after Tim is divorced. Right now we have so many irons in the fire, the thought of sitting in a state office for hours just wears me out.
Loved your funny story, yep it's those kind of moments that help us get through!
Carla

carlaj,

What kind of "care giving aid" are you talking about?

Well Fred,
It would be nice if someone could come and get Tim, take him to a movie or the mall. I feel like I never have any time at home by myself. He is mobile, loves to play video games, mini golf and stuff like that but I just don't have time or energy to go do all those things. so I think if I could find someone who would be more like a companion, near his age just to get him out more and give me some space and time...but his friends have dropped out of the picture for the most part. also I would want someone who understood Huntington's and wouldn't be embarrassed by the symptoms. Like right now, he's downstairs practicing up on his Playstation because he is going to his brother's house Friday night. He's bound and determined to beat him! It's so funny.
That's all I was talking about. He's not a burden, I was just used to living alone and my husband works nights, so weekends are the only time we have together. Also I think Tim really enjoys getting out of here too!

I never did find such a program. I don't think one exists. Does anybody reading this know of such a program?

They have medicade programs for home nursing, but none for social interaction like a "big brother" for anyone. Think of the liablity issues. What if this person took him somewhere, and he stumbled and fell or walked in front of a car? (These things have happened in my wife's family on more than one occasion).

I took the disablity payments my wife received and hired a person to come in and care for her a few hours a day. That freed me up to do other chores and keep the place clean and organized. That was a big relief. I was lucky enough to find a concerned neighbor.

I have heard of churches doing what you describe, but I don't believe there are any such programs available at all. If you find one, please post it and describe how to get it!

Our church family has helped where they can...but each person helping eventually burns out. I warn people when they are about to offer help. I tell them that if they offer, I am going to take them up on it, and I will use them until they say no. It's saved a lot of grief because there are those who will say, "If you need any help..." but don't really mean it.

Most respite care programs I have found are run by the county and usually funded by the state...if they have them at all. My Mother-in-law had a person that would spend time with her, etc. It was partially funded by the county she lived in and it gave my father in law a nice break. My county did not have any such program, and I had to pay for Adult Day care out of pocket. That plus the cost of a caregiver for the girls and the finances were getting ugly.

When my wife hit more than 50% of the time at my parents house, she suddenly became eligible for lots of low income based programs and other programs in the county where they live. We were not aware this would happen when we made the move, but it was a nice surprise. My income was no longer deemed with hers, and she can now get SSI and medicaid. (She was not eligble for standard disability because she took time off to raise the kids....that was a kick in the rear when we found that out.)

Adult daycare has been great. She loves going because they have all kinds of activities...bowling, Wii, bingo, etc. and the people running it are all CNA's or RN's, so they can handle the less than desirable stuff too. The county and state programs pay for all of it now that she no longer is full time at my house.

I would contact your county's social services office and have them send out a social worker. Here they come right to your home and will sit down with you and lay out all the options available. She even filled out the majority of the paperwork for us.

Yahkin,
Thanks for the info. I will check into it, I know they have alot of services for the elderly in our county so maybe they have someone/somthing to help with getting Tim out a little more.
Take care
Carla