The life of a man suffering from Huntington's Disease, a genetic and fatal illness. Every child of a HD parent has a 50 % chance of suffering from this fatal degenerative neurological illness.
This is the story of one such person.
I woke up late today after almost ten hours of sleep. I don't know if I'm going to need that coffee but I'm making it anyway.
I want a T-Shirt that says "My emotions aren't working today. Thank you very much :)" I hate this but its happening more frequently. My emotions aren't in conjunction with my thoughts, it drives me crazy. I want to cry. I can type it, if I say this its in a monotone voice because 1+1=5 Why do I want to cry? Damned if I can tell you.
Before the days of Music Videos but love this audio.
Diana Ross-"Upside Down"
Chaka Khan-"I feel for you"
This woman was born and raised in the infamous Chicago Projects Cabrini-Green. She went on to win 10 Grammy Awards and is still going strong today.
Philip Oakey & Giorgio Moroder - "Be My Lover Now"
Very interesting remix of Irene Cara's "Flashdance Theme" by Giogio Moroder.
Personally I think this really captures his feel. If you don't like this he's probably not for you.
I just texted my gf, who is concerned I haven't been leaving the house enough, "Today is one of those days I feel autistic." I wonder if she'll understand what I mean.
Sting-"Children's Crusade"-Warning-violence
I just got a text back "You can always go to the Post Office." I hate when she 'get's me'. I'm supposed to be a stranger to her as well as to me. Instead, she finds errands that need to be done that require little emotional attachment!
Doing a little better, I think I'm going to try a late lunch before I leave for the Post Office. Feeling a little better on the emotional scale but generally feel crappy. I come back in from the bathroom mirror (my lip looks like I bit it in my sleep last night) and sit down. My leg starts jumping out of nowhere. It can stop now. Thank you.
Van Halen-"Jump"
Styx-"Mr. Roboto"
Did Styx know they were making crap when they made that last album "Kilroy Was Here"? I'm serious. The whole concept behind the story was bull. They wanted to sell to the emerging Electronic music market is what they were thinking with this album.
This gem fell through the floor when I was picking out Joel tunes as did the next one.
Billy Joel-"Allentown"
Billy Joel sings a very American "We Didn't Start the Fire"
The Kinks (TOTP)-"Lola"
Its not Monty Python
Jim Carey On Living Color does Vanilla Ice
he thought the most embarassing thing he would ever do was dress up like this and rap....
and then he starred in The Happening
Marky Mark and The Funky Bunch-"Good Vibrations"
Well, I've tried to find an emotional base today to feel something and at times I've succeeded for a little while, in terms of minutes. Its better than nothing however, and I'm glad I tried.
Its 10:30 p.m., at what time I normally begin to relax for the night...this evening my brain is starting to wind up. I feel it whirring like a hamster wheel. Shut the F*ck up!
NSFW (language)
Cobra Starship-"Snakes on the Plane (Bring It)"
I feel like that Enimem video with Rihanna and Megan Fox circling the burning house. I have that much burning confusion going on inside myself right now. I can literally feel it. The last two weeks have been filled with anything but calming thoughts and when I want to come back to normal, I feel that normal has moved since I last knew where it stood. Now all that's there is a charred circle to remind me where it once was. Does that make any sense?
Eminem - "Love The Way You Lie" ft. Rihanna
Just took my handful (now working towards filing up two hands) of night meds. I should take a picture sometime. J still doesn't believe it when I take them in one swallow. The nurse in the hospital didn't believe me either and I had to prove it to her. Maybe its some strange gift I was born with.
Another "oh-Shit Wish I was there" performance
Kim Carnes with Kenny Rogers - "Don't Fall in Love With a Dreamer"
This second half of our Kenny Rodger's Double Feature and beginning of Sheena Easton's double feature is what us men used to politely call make-out music. You put this on the turntable and she was as good as yours for the night.
Here she is opening up the James Bond Film For Your Eyes Only
Sheena Easton-"For Your Eyes Only"
This song captures how I feel most nights when I start to fall asleep.
Laura Branigan -"Will You Still Love Me Tomorrow"
One of my favorite Synth Pop groups of the early 80's wrote this: AlphaVille.
When I clicked on the link I didn't really think it would be this good. NOW I know why Debbie on Twitter so highly recommends her. Thanks Debbie for widening my horizons. Until recently, I had only known of her work on Moroder Soundtracks and "Gloria"
Also from Una Vez Mas in Chile
Laura Branigan-"Forever Young"
Another make-out Bond theme song...and why is it if you're not in the mood its great music to relax to?
Rita Coolidge-"All Time High"
Speaking of being in the mood..
Robert Plant - "I'm In The Mood"
I thought I felt my Melatonin kick in but then discovered it isn't quite time. Its these relaxing tunes kicking in. Maybe I'll be able to sleep after all.
"Human, born to make mistakes...can you turn down the smoke machine please?"
Human League - "Human" on TOTP
Hotly debated in many circles, this is the only hit not written at least in part
by Oakey or another band member. Personally,
as much as I like this song it sounds overly produced.
I would love to hear this album (Crash) "naked" as The Beatles-
"Let it Be" was recently released stripped of Specter's Wall of Sound.
Godley & Creme - "Cry"
If I close my eyes now I'm passing out. Which is good as my mind is still turning wheels over these CAG numbers. Last night when I told J. about it, she agreed it might not have been useful for me now but may down the road, and she's right. For example I'm amping up some on my half of the mortgage so she won't be left with any of my half after five years. I'm working with a financial planner to get my creditors paid off as well. I'm so glad I had my advance health (and financial) directive in place when I was in hospital knowing if gosh-forbid I took a turn for the worst I'd have my bases covered. Also as my health agent no one can ban her from seeing me without cause. That's a big relief as marriage is not in my future plans.
The only thing I can think of, and this is pushing it is that my depression started back when I was 18 and dropped out after my O's which was not expected of me. I was expected to take 4 "A"'s and apply to Oxford but didn't have it in me and left. That's still way too late and too little symptomatically. I feel like EG Marshall in CreepShow when all the cockroaches come out of his body cavities. I feel that I'm on a path of clear destruction inside-out. You'd find it hard to sleep as well.
Still have Cat Stevens playing today. That man is a hell of a musician. Speaking of music getting stuck in my head, I dreamed about the video Blind Youth by THL. In this dream J and I were walking all around the building featured in the video. We took our time and toured the building as if we were considering to buy an apartment in it for our home. Really twisted dream.
I'm still sitting on this CAG count. It just doesn't make sense and I don't do well with illogical things. I'm generally one of a scientific mind, and this doesn't fit anywhere. I feel like its like a round peg I'm trying to fit into a square hole. I'm pounding and pounding it but it isn't going in.
How the hell did I get such a large (JHD range) CAG count when I didn't become symptomatic until my mid 20's? It just doesn't work. It has to fit.
My right arm is still jumping. It is driving me crazy. Not just when weight bearing, so I'll be walking around the house with a jumping right arm. That's also my slightly stronger arm by default. Although I'm ambidextrous most things in this world were invented with the right-hander in mind. Now I'm reaching for the left handed versions of tools simply to make it easier to perform the tasks. This is the same shoulder that I was bitching about a couple weeks ago. I have a business meeting to attend to this evening and I'm already thinking of ways to hide this movement.
Royksopp - "Sparks
Another Gem by Wall of Sound Recording
Sparks-"Something for the Girl Who has Everything"
Two cuts today from a BonaFide American Artist Billy Joel
Billy Joel-"Pressure"
Billy Joel-"Summer Highland Falls 1977 Live"
I want to curl up in a little ball and cry. I'm tired of living up to everyone else's expectations when I don't even know what my own are.
I remember an interview with Michael J. Fox discussing the early years after his diagnosis of Parkinson's Disease. He spoke about his public and private faces, and how exhausting it was hiding his symptoms. I get that today. It freaking drains you. Emotionally and physically.
Another American Classic: Roy Orbison-"Crying"
Mary Travers(Nov 9, 1936 - Sept 16, 2009) and the Kingston Trio -"Where Have All The Flowers Gone"
Bob Dylan-"Times They are A'Changing"
A few more songs from the country that has welcomed me into its borders. If I hear one more idiot say America doesn't have a culture I'm going to hit him over the head with Blue Bayou.
Let's just say my mood is low enough I don't want to be alone.
It is starting to look like the time to give up my driving licence. I don't use it very much anymore for starters. In all honesty, I'm fearful to get behind the wheel most of the time. What good is that?
I still have it, and will keep it around the house for emergencies but outside driving to the hospital I guess I simply shouldn't drive anymore. Even when I make plans most days I end up on a taxi or public transport. So off it will go into J's wallet tonight.
I don't know what is happening lately but I just want to cry and cry.
Maybe its the licence thing, I used to adore to drive. It was a relaxing activity, dating back to my years in Sheffield. Maybe its just my depression, goodness I was so depressed earlier I fell asleep in the middle of the night leaning on our living room table, not the most comfortable of places.
Holy Cow you can actually make out the lyrics in this version!
Manfred Mann's Blinded by the Light
There is only one thing that sounds decent to me right now, but its not really an option. That's a drink. I would die for a good Absinthe but it would do the same for me.
Huntington's versus Alcoholism? That's a race I don't want to watch, thank you very much.
Bob Fosse's Cabaret has several excellent songs. Here are two of them as our Double Feature. First up is one of the most romantic scenes portrayed in a film. It simply consists of two ordinary lovers doing ordinary things. It doesn't try to be anything special, so it becomes gorgeous. That is Fosse's gift.
Here is one of the scariest scenes I've seen in a film. There is no blood or guts. There is no implied blood or guts. Yet Fosse has all of us know exactly what the people symbolize, and that is the death of eight million people.
Again, click through....Come on now...click through. It's Fosse so its worth it. Would I lie to you?
Okay, I wouldn't lie to you, but she might... The Eurthymics
There is so much to catch up on today. Even though I'm seriously depressed I'm finally recovering from the antibiotics that knocked all the energy from me.
First, I woke up to this interesting blog on being tested. I can't find the link now for the sake of me, but if my memory comes back I'll insert it for you. [Edited on Nov. 7, 2011, 08:27 P.M. Link is Here. I really recommend you take some time to read it] As you probably don't know (as only one person outside of my house has been informed) I finally went in, put the cash down and did the whole genetic counseling thing along with the blood draw. I asked J to go with me for moral support, which she was kind enough to do, in handfuls.
First, since this draw was done at a Center of Excellence they put A LOT of weight on the physical symptoms. It meant less than nothing to them that I had been on anti-depressants starting at age 25 after running across a freeway for example. This becomes important later.
After they spent time explaining to us (J came with me for the simple fact I was too scared to do this alone) where the affected Htt Gene is located on Chromosome 4 and so on. It was then that I realized most of this information she was hearing for the first time. I never deliberately tried to hide any information from her, but who wants to hear about the genetics of someone else's illness. I felt she would find it boring but just the opposite. She wanted to know about it. Everything about it. Each and every pamphlet that wasn't nailed down was in her folder by the end of the two hour session.
J looked as if she was researching for her new career
as a Paperback Writer!
So we go in for the blood draw and in a little over a month, and a second visit, I came back for the results. They won't tell you you're CAG repeats when they tell you the result. Since I have been diagnosed based on symptoms, I don't need to know if its in my genes. That's a bit obvious. I need to know what my CAG repeats are, as they CAN give an inclination to the speed my HD can move among other things. They finally admit that if I call when I get home and still wish to know they will share it at that time.
Six weeks later we drove back down. Sure enough, based on my CAG repeats I have Huntington's Disease.So here we are driving home and I've this piece of paper in my hands that states I do for a fact have HD. Its first real "proof" I've ever seen of my HD on paper. A third of the way back to Santa Barbara in Malibu it hits me like a ton of bricks and I start bawling. We pulled up and got down on the sand to watch some surfers. By then we were both cold and hungry. After struggling to get up and about, I managed to gather a menu and ordered a Malibu Priced burger, chocolate shake and really enjoyed it.
And no, they still won't tell me what the repeats are. Once we make the drive home I call as I enter our house. This is when I get shock number 2. My CAG repeats are over 150. That's JHD range! I asked the doctor on the telephone about this (while trying to get the speaker phone itself to work so J can hear) and he had several working theories. The first is that I got it from father and checked off the incorrect box. We both knew that was not true. Second, since my mother's family preferred to keep these things quiet.they may have encouraged her to hide some of her psychological symptoms from my father when they dated. If that is the case, she could have a higher CAG repeats as she died before genetic testing was available.
However, they didn't consider this during her pre-symptomatic period time (remember this was a Centre of Excellence and they really emphasis the physical symptoms). They have nearly completely ignored my anxiety and depression that left me bed ridden, caused me to drop out of school after my O levels for menial labor and caused me to run onto an busy motorway all by age twenty-five. It wasn't until I later left for the states and received a second chance at a new medication (Paxil) for depression & anxiety followed by obtaining a Bachelor's Degree in Library Science that I was able to begin to get my life on track.
Never seen so much talent in one video
and that includes all of the charity videos.
Johnny Cash - "God's Going to Cut Them Down"
Third and finally, with a CAG this high, I should most likely be dead, or in much worse condition than I am. The only thing I can guess, and this is truly a guess on my part, is that when I go from bad to worse, it always has been a very large jump. For example, I can tell you now that this next progression that has just started will most likely be primarily physical symptoms. When I drink I can already feel the liquid catch and try to go down the wrong way several times daily. Then the circular walking issue that started six months ago, and the issues where my typing words go on repeat. Plus of course the OCD that has been getting gradually worse and more controlling.
Unlike my teenage years I can now enjoy both
Heaven 17 and Human League.
In Sheffield you had to take a side between the two bands.
Somebody wants to curl up here at midnight. I started the Melatonin (thank you Melatonin Mice for your service) tonight and feel it starting to work so I'm going to head off with those two pleasant thoughts.
I came home from the hospital after spending a couple days in there for pneumonia.
The entire stay was complicated. I went into the Emergency Room at two in the morning, only to check out at noon. I wasn't feeling well, and thought I was coming down with my annual bronchitis. Only I wasn't. I had come down with walking pneumonia. Not so good. They give me a bag of IV antibiotics and then I'm welcome to leave. The doctor's directions are to return if my fever tops 102 degrees. My memory at this point is fuzzy, but I do know the time before my fever goes to 103 is about an hour and that it goes up a whole degree on the drive p. J was so upset at the hospital releasing me that she has my GP meet her in the ER before she will drop me by again. Her valid argument is that he knows enough basic medicine than to release me again, and she's right. He checks me in under his name. I breathe knowing I'll getting better medical care for the night.
Suddenly I realize that I'm expecting something many people in the United States don't have: reasonable health care. This should to be a basic human right. B A S I C. Okay, I'll rant about that on another day
Chose this song because I'm feeling blue but as soon as I started watching this song I realized how much I have to be grateful for. Beautiful piece by Elton, as usual.
Once I get over this pneumonia (and the sooner the better) I'm looking forward to being joining the Occupy Santa Barbara movement and actually protest with them, moving from simple support to full support. After spending some time with a friend who had been one step from homelessness twenty years ago due to mass job layoffs in her line of business, it has made me feel that this is very important.
I've been doing a lot of thinking lately, with all this time on my hands. I've been thinking about spiritual matters. I'm back to the basic "is there a god" mental puzzle.
So tired of being in bed, and the medicine has knocked the wind out of me. Its hard to read as the letters are swimming in front of me, although I do have the movies to go along with Easy Riders, Raging Bulls, and Robert Evans' The Kid Stays in the Picture.
I could watch those. If I get off my butt enough to do it. I have no get up and go. It could be from being sick or HD related depression. My money is on the latter. I would really like to see David O. Russell's Three Kings (Yeah the guy's an a$$hole but its a brilliant film).
One thing that really s*cks, at least for me, about HD is that like many chronic illnesses, once you get something else you it aggravates the chronic condition. So in my case not only do I have pneumonia but I've had trouble making sure all my liquids go down the right pipe and my words are spoken clearly. My depression, memory and anxiety have all gone through the roof. The only good part of this is that I know what the new symptoms of HD will be ahead of time. For example, a couple of years ago I took ill with a nasty flu. This brought out a new HD symptom, missing keys on the keyboard. For example instead of "T" I will hit the letter key to the left of it, the "R" key. When the flu went away I stopped hitting the left key.
Then a few months later, sure enough, suddenly I started hitting the letter key to the left next to one I want to type on a regular basis.
The woman in this video looks like she walked right off the Pan-Am set!
This time when I started to get pneumonia, I began to repeat words when I typed them. Sometimes I would proofread them several days in a row before I noticed.
Before I log off (its midnight and several naps later I never did see that movie) I need to get some sleep for the night after my two hour nap, I wanted to share a wonderful experience I had with another HD FaceBooker. This person sent me a message in chat about 8pm and we ended up chatting for a couple hours. For those couple of hours, I didn't feel alone in this HD universe. There was someone else out there who understood where I was coming from.
It was this person's birthday but I was the one who received the gift. Thank you.
As I sign off here is an example how tired I've been feeling. It takes me three days to get this entry written, and I still don't have the energy to add more than four songs. I want too, I just don't have the energy. However, I'm taking the advice of a fellow author. In basic English. if I think I'm pushing myself, there is s good chance I am.
Now I've got all the new music on my Droid, haven't listened to it, either. It takes a lot less energy to listen to it, so I'm making a date with some good albums Sunday when I wake up. If I strayed away from Facebook Chat, then I forgot its even open (You have to love those HD memory issues).
My right shoulder started shaking and it actually woke up J, which is a first. I'm going to let her try to relax it so she can go back to sleep.
Still here, still sick as crap. I feel on fire, my chest hurts from coughing so much and I don't even care that I'm not at home right now
J went home for a few hours this afternoon. She looks exhausted and she's not going to sleep very well here. So here I am sitting in the hospital with a bunch of good matured nurses in brightly colored costumes. My roommate and I watched the film The Nightmare Before Christmas, the Tim Burton Halloween themed claymation musical.
"What's This?"
The Soundtrack from The Nightmare Before Christmas
J came sometime after the film and I woke up surprised to see her back here. She said something about not being able to sleep, and I understand that, remembering all too well when she was on a cruise once. That was a cruise, with nothing to worry about. Well, except those pesky ships known as the Titanic and Lusitania but they were flukes, right?
Titanic-The Sequel
Can I only imagine what life would be like if our roles were reversed the last few days? I'd be freaking out. I don't do deal well with things in my life being out of control.
The wink wink nod nod J is getting from the nurses when she asked when she must leave isn't helping my mind quiet down. She asks once and I feel it in the air like one feels the heat off a burning stove. I never thought I'd say this but I hate being alone. The only thing I hate more is not making the decision myself. Like I said, I'm a control freak in some ways. I hate having to change my routine. I hate being scared. I also hate feeling confused with a million pairs of eyes on me.
[insert here]I talked to my Neuro prior to dinner time and asked him if they were aware of this tendency of mine to be OCD. He said he had notified them of that portion of my medical condition. That's likely where I got that impression they are giving me a bit of leeway as long as I don't abuse the visiting privileges. It would make sense, as my RN's job would be easier as well. [end insert]
Oh, back to watching the film. Suddenly I started smiling broadly ear to ear, and my twenty something roommate asked what I was smiling about. I told him I was thinking of a friend of mine, Jan. Jan's daughter Keely had loved that movie. I met Jan online through Facebook's HD Community.
Last year, Keely spent her last Halloween dressed as her favorite movie character, Jack Skellington. You see, she was dying of Juvenile Huntington's Disease.
To put it in basic terms JHD is HD on steroids. I have several people who suffer from JHD on my Facebook and they are my true heroes.
Off to sleep here, I'm feeling wretched.
Please take a few minutes and educate yourself on this rare version of HD. Highlights are mine. You can learn more at the reference link at the bottom.
What is JHD?*
Juvenile Huntington’s disease is a Neurological degenerative terminal brain disease…JHD refers to Huntington’s disease that has an age of symptom onset ranging from infancy to 20years of age.
JHD takes away all of a child’s abilities over time, so for a child it makes it hard for them to do simple things like go outside to play with their friends on the playground or even blow out their birthday candles. There are many milestones that these kids might never experience. There is NO CURE.
Please help us find one.
Children who develop symptoms at a young age have a variation of differences in the disease progression than that of adult onset. Often the disease progresses more aggressively and their lifetime is anticipated to be anywhere between 3 to 10 years once diagnosis has been made ,However this does not always hold truth for all cases. Symptoms may come and go and the age of development and presentation of symptoms, combined with the lack of professional experience with these kids might be factors in untimely or miss diagnosis and lack of understanding the child’s full needs. This can contribute too many difficulties for family and professionals caring for these children.
Only an estimated ten percent of people at risk for Huntington’s Disease develop the juvenile form, but it’s hard to get accurate numbers. There’s so many more then that!! The younger the onset, the faster the progression. Which makes it harder for family, friends and even doctors to cope with.
A huge question might be when should we be concerned that my child might have JHD? Due to the variability and the truth that children are developing and growing, and many other factors, the best answer would likely be “when there is an obvious change”, particularly one that is impacting the child’s quality of life. There is approximately a 40% chance that someone who develops JHD will develop epilepsy. Some ways to tell if someone may have JHD are if they have a decline in school, behavior issues, personality changes ,impulsiveness, memory loss, difficulty with comprehension, balance or movement difficulties. The list could be quite long.
A significant question might be when should we test the child? This has been a controversial question. The response will vary dependent upon who you speak with. NO one wants to give a child a terminal diagnosis. A diagnosis is a quite serious step, keep in mind, diagnosed or undiagnosed if someone is symptomatic their genetics will not change. Taking the time to be sure everyone in the immediate family is as prepared as they can be is likely invaluable, while weighing the child’s care and needs for treatment in this process also. An experienced genetics and or pediatric neurology team is important in assisting the family along with involving a social worker with the HDSA.
It’s always important to not place expectations on a child with JHD, adapting their world to them, vs. adapting them to the world. Keeping in mind that all changes are not necessarily permanent, a child may be unable to walk one day and then the following day walk again as if there had never been an issue. MOST IMPORTANTLY to keep in mind that just because the child is terminally ill with a degenerative condition DOES NOT mean there is no HOPE or that nothing can help. Diagnosis is a new beginning NOT an end.
Children with JHD are quite special young people with invaluable lessons to teach anyone who’s life has been touched by these kids.
