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Wednesday, November 30, 2011

article I came across

This is from an article about Richard Rainwater a billionare who was diagnosed with PSP;
The nightmare of PSP
Even after retreating from dealmaking, Rainwater wasn't through making Texas-size wagers. In December 2005 a Fortune story, "The Rainwater Prophecy," reported that the billionaire, anticipating a catastrophic oil shortage, had gone long on energy stocks and oil futures, to the tune of several hundred million dollars.
To those who knew Rainwater, this play was somehow ... different. In Fortune, Rainwater vented his concern about "the survivability of mankind." He worried openly about sounding like a nut, but he had Moore install an emergency generator and 500-gallon tanks for diesel fuel and water on her South Carolina farm, just in case.
As it happens, like most of Rainwater's bets, this one paid off. Energy prices climbed, and Rainwater, deciding that a crisis wasn't imminent, unloaded his holdings for a handsome profit. But looking back, Moore wonders whether this fixation wasn't an early sign of her husband's disease, given that obsessiveness, loss of inhibition, and mood swings are among the psychological effects of PSP. (Rainwater wouldn't be diagnosed until three years later.)
Progressive supranuclear palsy is a riddle wrapped inside a nightmare. The disease is rare, striking about six people in 100,000, usually in their fifties or sixties. Frequently misdiagnosed as Parkinson's disease, it wasn't even identified in medical literature until 1963. Its cause remains unknown, and its effects are devastating.
PSP attacks nerve cells in the brain, causing something called tau -- a protein that is part of the normal neuron infrastructure -- to pile up in clumps. The resulting destruction impairs mental processing, balance, coordination, and vertical eye movement; two hallmarks of the disease are difficulty looking downward and terrible backward falls. Physical stiffness progresses, causing slurred speech, a blank facial stare, and trouble swallowing.
Worst of all for the patient, there's a painful lag between the deterioration of the brain and the body. Awareness, memory, and thoughts remain largely intact, often bottled up by an inability to wield them. "People are locked out of expressing," says Dr. Joel Kramer, a neuropsychologist and co-founder of the UCSF dementia center. Over time the dementia worsens. Patients become virtually helpless. Most die from choking, suffocation, or pneumonia.
In March 2009, when Rainwater was diagnosed in San Francisco, there were no treatments for PSP. Rainwater characteristically began scouring the Internet for hope; he found little comfort.
"I'm dying, Precious," he told Moore.
"We all are," she replied. "No one can tell you when you're going to die."
Accompanied by Dan Stern, Rainwater and Moore traveled to the University of Aberdeen in Scotland, where doctors were treating Alzheimer's and Parkinson's patients with methylene blue, an experimental drug. Rainwater began taking the medicine, which was thought to combat the buildup of tau, but it didn't help.
Around that time, Rainwater's family began organizing a new research program. Later named the Tau Consortium, it was a classic Rainwater-style deal: creative, ambitious, risky, and collaborative.
Moore and Stern asked Miller and Kenet to round up a medical dream team that Rainwater would bankroll. About 25 experts from around the world, ranging from bench scientists to clinicians, gathered in New York City in May 2009 for a three-day meeting to organize a research program. The group, which includes a Nobel Prize winner, has met four times since to share their findings. "I don't think there's ever been such a broad group of people focused this way in science," says Miller. "It has a Manhattan Project quality to it."
The scientists are attacking PSP on multiple fronts, studying elements of the disease in mice, flies, worms, and human cells -- Rainwater's cells, that is. Skin taken from Rainwater's leg -- presumably containing markers of his particular disease -- has been converted to stem cells for testing existing chemical compounds. If lab work shows promise for halting the accumulation of tau, an experimental treatment would be rushed into the clinic -- with Rainwater as the first patient.
As personal as all this seems -- with more than $20 million committed so far to try to save a single dying man -- it is, in actuality, far more likely to help others. Though they are widespread, brain diseases attract little private funding because progress is so difficult; they're regarded in the philanthropic world as a black hole. Rainwater's spending is "one of the most significant investments in dementia research by an individual ever," says Miller.
The research funded by the Tau Consortium assumes that tau is the cause of disease, not merely a byproduct, although that issue remains a subject of scientific debate. Abnormal tau clumps are also present in Alzheimer's patients. This means that Rainwater's initiative could contribute to treatments for them too. Says Miller: "We believe tau is the holy grail of dementia. This has broad implications. Treatment for Richard will be a treatment for the world." He acknowledges, though, that finding a cure in time for Rainwater will be "very difficult."

Thursday, November 10, 2011

todays blurb

11/10/11

Bernie’s eyes are closing more and more, we have about 2 weeks before we go back to the doctor for more Botox ejections. Funny how his blink rate has slowed down but his eyes shut on their own all the time, one would almost think that if the blink rate declined that the eyes would stay open! He tires easily; even walking around the mall seems too much for him now.  Stairs are difficult and getting off the couch is not easy either. I am starting to look into the power recliners; I think that is the next step.
It’s hard to try to guess what might be next, I wish someone who has been through this before would have a blog or posting outlining what they went through, even though “everybody” is different it could still be a guide. We are into our 6th year with this and the life expectancy for PSP is 7-10 years, and I would like to know if that is a reliable guide line. Not that I would do anything much differently except maybe try to travel more, while it not too hard to get around. We do live each day as it comes and try not to think about what tomorrow will bring, but I guess most people over fifty do that…you know the second phase of life! The first fifty we don’t think too much about, but after turning 50 we start to think it’s time to slow down the clock and try to enjoy life a little……

Monday, October 24, 2011

10/24/11

Noticing now that Bernie’s eyes are starting to close again on their own. Not as often as before the injections but it is getting there. I started to notice about 2 weeks ago that he was prying them open with his fingers again, and lately he has been doing it more and more. We are scheduled to go back to the doctor early December but just received notice from the insurance company that they have denied further Botox treatments as they deem it experiment. I am going to fight this and put in a grievance just hoping that they change their refusal to an approval.

Wednesday, October 19, 2011

Thoughts

10/19/11
Well we are done with 5 visits of PT and the insurance company won’t approve anymore, at this point I don’t think that matters. We have learned a few things to do at home and I believe as well as the therapist believes that PT is what you do every day at home. Bernie needs to slow down and think before he moves and he is finding that difficult. The problem with PSP is that your brain is telling your body what to do but your body doesn’t get the signal fast enough anymore or your body can’t do the task anymore. For instance when your brain is telling your feet that you are going to walk but your foot/feet won’t/can’t move but you don’t know your feet are frozen, your upper body leans forward as it would when you take a step but since your feet are not following you end up falling, and when you are falling you don’t have the coordination or reflex to put your hands out so you land hard. Standing up is also an issue, gets harder and harder to pull yourself up so you have to really try to throw your weight behind it and when you finally rise up your doing so at a faster pace and tend to fall forward with the momentum. Getting in and out of the car is difficult, moving your legs which are stiff and sore and having your balance compromised makes even that a difficult task. Everything is so much harder to do with PSP; it really steals your life in that sense. Talking is also difficult as your voice just won’t work anymore, words are slurred and mumbled and the voice volume is barely existent, so now it is just easier to not speak at all then to be frustrated when trying to speak. These are things as a caregiver you will see happening to your loved one, yet you need to stay strong and positive, for both of you.

Monday, October 3, 2011

PT

10/3/11

Bernie has started PT again; he has gone through several series of PT over the last 5 years. For the most part I don’t think either one of us felt it was doing much good. In the beginning he would drive himself back and forth but as his condition progressed and he was no longer able to drive I would take him and that is when I was able to see what was going on. The series of exercises they were having him do I felt were not beneficial to him, and although I am not an expert and have no degree I don’t think sitting on a large ball and lifting your leg and reaching for your toes is a particularly a safe position for people with balance issues. After speaking with the therapist and trying to explain what his needs were she told me she had experience with treating people with his condition (but I don’t believe her). Recently the PA at the movement disorder center recommended PT again telling us it would be helpful with the increased falling Bernie was experiencing. I told him my concerns with going again and he told me I would have to look for someone with specific knowledge on PSP or Parkinsonism. After three weeks of looking and phone calls I settled on Sunnyview Rehabilitation Center in Schenectady, and both Bernie and I are please. We met with a therapist for an evaluation and I had brought literature on PSP and what specifically was happening in people with PSP. After the evaluation we set up a series of appointments for PT, our first one being last Friday. On our first visit the first thing the therapist said to me was she read my pamphlets and what had occurred to her most was that PSP caused backwards falls…..and asked if anyone had ever tried lifts in Bernie’s shoes!! No one had, so she went and got the lifts and put them in his sneakers and Bernie said he noticed a difference as soon as he stood up…so if you have not tried them please do……He says he feels more stable now, not so wobbly like he’s constantly going to go over backwards. This is the difference between therapist who truly want to help and research conditions before starting. She then work mainly with his balance issues, footwork and walking, it was a eye opening appointment and I can’t wait to see what she has in store next.

Monday, September 19, 2011

Todays Thought

9/19/11

Still more falls, and this particular weekend was especially bad, Bernie’s balance was way off. He fell Friday night down 2 stairs and has quite the contusion on his thigh, and again hit an elbow. He did not have the pads on because it was the middle of the night and he does not sleep with them. Our bathroom (on the main floor) should be done in a week to 10 days, and then he won’t have to go down stairs to use the facilities. I will also be moving the TV from the basement upstairs also, so he will have no reason to go down to the family room in the basement, we are at that point where we have to make major changes to keep him safe. The TV in the basement has a home theater system hooked up to it so he likes to watch things downstairs but the stairs are unsafe so he will have to make some adjustments too. He is generally slowing down in all areas, there are noticeable progressions almost weekly, unless you are with him a lot you might not notice them but they are there. He will be starting another round of PT soon so I am hoping that it will strengthen his legs and arms so he can hopefully be better balanced, I just don’t know if that will help. Although we try to take it one day at a time I sometimes wonder if the progression picks up at this point. After having this condition for 4-5 years and neuron continue to die off it just seems like it will get worse at a quicker pace. I wish I knew someone to talk to who has been through this, just to see their story and timeline, it might help me to prepare for what’s ahead.

Monday, September 12, 2011

Still Eye Issues

9/12/11

The Botox injections are helping with the involuntary closing of Bernie’s eyes, however I have noticed that his blink rate is significantly less than before and much less than what is required. His eyes are now red and dry, and he has also developed an infection. The optometrists says it is because his eyes are not blinking which would lubricate the eyes normally. So is this because of the Botox injections or again a natural progression as it could be either. That’s what makes this condition so difficult, deciding what is progression and what is considered side effects. We go back to the neurologist in December so hoping he can shed so light on this.
Bernie has decided he wants to wear elbow pads to help with his falls, he seems to fall mostly on his left side and bang that elbow, and it has been bruised of months.  I am looking into a better elbow pad at this time because he bought a set at Wal-Mart for extreme sports and although they do help somewhat I would like to see if I can get something that is a better fit, these slide down on his arm. He has fallen on it so much that he has bone chips floating around it, I am surprised that he has not broken it yet. That is one of my biggest fears it that he will fall and break a bone whether is a hand, elbow, shoulder, hip, or God forbid his head, it could set him back to the point where he might not recover fully.
I am trying to find some time where I can sit and put more thought out there but for now all I have time for are these tidbit.

God Bless,
Kelly