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June 27, 2005
Learning More On Protein Clumps
In recent months scientists figured out that the protein clumps we see in cells due to HD are part of the cell's protective mechanism. A study funded by the Medical Research Council has learned more which will help in developing effective treatments. From the press release:
The new research...has concentrated on tiny molecular motors called dyneins, which are known to be important for moving proteins around inside nerve cells. The researchers found that dyneins play a crucial role in the delivery of toxic proteins to the waste disposal units of cells. When dyneins are defective or absent, this waste disposal system is stalled, clumps of proteins appear, and cell function is compromised.The study found that failure of the dynein system causes the degeneration in a form of motor neuron disease, and that it is also involved in other conditions such as Huntington's disease. ... Enhancing the degradation of these protein clumps has the potential to delay the onset of, or even reverse, this group of diseases. This might be exploited in new approaches to prevention or therapy.
The full press release:
New clues to the causes of neurodegenerative diseases, with implications for motor-neuron and Huntington's disease
Scientists funded by the Medical Research Council (MRC), the Wellcome Trust, BBSRC and the EU have made an important discovery about the mechanisms underlying degenerative brain diseases. The findings, published in this week's edition of the science journal Nature Genetics, may have implications for therapeutic strategies for some forms of motor neuron disease, Huntington's disease and Alzheimer's.
Late-onset neurodegenerative diseases are a major health burden on the population, yet little is known about the chemical changes that trigger the degeneration of nerve cells. However, many of these diseases are characterised by accumulation, inside nerve cells, of clumps of toxic proteins. The new research - carried out at the Cambridge Institute for Medical Research, the MRC Mammalian Genetics Unit at Harwell and the Department of Genetics, University of Cambridge - has concentrated on tiny molecular motors called dyneins, which are known to be important for moving proteins around inside nerve cells. The researchers found that dyneins play a crucial role in the delivery of toxic proteins to the waste disposal units of cells. When dyneins are defective or absent, this waste disposal system is stalled, clumps of proteins appear, and cell function is compromised.
The study found that failure of the dynein system causes the degeneration in a form of motor neuron disease, and that it is also involved in other conditions such as Huntington's disease. It also provided further evidence for the idea, pioneered by the same group of scientists, that a key factor in the severity of these diseases is the rate at which these toxic clumps of protein can be removed. Enhancing the degradation of these protein clumps has the potential to delay the onset of, or even reverse, this group of diseases. This might be exploited in new approaches to prevention or therapy.
The lead scientist, David Rubinsztein of the Cambridge Institute for Medical Research, said:
"These findings provide us with real insight into the molecular basis of certain motor neuron diseases and the mechanisms by which toxic proteins accumulate and are broken down. They also contribute greatly to our understanding of possible therapeutic strategies for these diseases."
Journalists should credit the MRC as a source of this story
For further information, or to arrange an interview, please contact the MRC press office on 020 7637 6011
Posted by Dave at 11:49 AM | Comments (0) | TrackBack
June 26, 2005
Boston Globe Article
Nice article from the Boston Globe on Huntington's Disease. The article is highlighting an annual HD fundraiser that has, so far, raised over $350,000 for medical research.
You know the article's writer, Kay Lazar, understands something about the disease when the article starts with:
Tina Boyer was scrambling after her 3-year-old son two weeks ago when she stumbled and fell. It's nothing, the Haverhill mom told herself.Still, she couldn't stop herself from wondering. Is this the first symptom?
An insidious disease is steadily robbing Boyer's mother, once a vivacious woman, of her ability to walk, talk, think, and even swallow. Boyer, 34, doesn't know when the disease will start gnawing at her, too.
But she knows it will.
Posted by Dave at 10:01 PM | Comments (0) | TrackBack
June 23, 2005
"Fiend" Is Accurate
Glasgow (Scotland) Evening Times gets the headline right: "Fiend jailed for savage attacks on sick girlfriend":
A MAN was jailed for three years today for two savage attacks on his girlfriend, who has an incurable disease.In a shocking case of domestic violence, Barry Adams, 24, left Michelle Bain so badly injured she had to have reconstructive surgery for fractured eye sockets.
A nurse described Miss Bain's facial injuries as the worst she had seen in five years in a hospital's accident and emergency unit.
Posted by Dave at 05:41 PM | Comments (0) | TrackBack
June 20, 2005
Marie Gets Some Press
Every year Marie Nemec bikes to the HDSA convention.
No, not just from the hotel...for several hundred miles. She does this to raise money and awareness for Huntington's Disease.
Visit her website here - http://www.bikeforthecure.org.
There is also a nice article on her efforts in the Augusta Chronicle (painful registration process in order to read):
If you see 62-year-old Marie Nemec and 69-year-old Charlotte Reicks riding their bikes toward Thomson today on their way to Washington, Ga., the two ask you to wave....
The women, who cycled to Augusta from Bamberg, S.C., on Sunday, tag-team the ride.
While one starts biking, the other moves their minivan to a point 10 miles down the road, Ms. Reicks said.
Each woman bikes about 10 miles at a time, and the team covers between 60 and 70 miles per day.
They stay with churches, Huntington's families and acquaintances along the way.
...
"My first concern with the first ride was safety," she said, adding they've never felt unsafe on any of their six previous rides. "People seemed like they wanted to help us get there. I came back from that first trip with a new concept of America."
Ms. Nemec learned of the disease through a friend who lost her husband to it. She had to do something to help others coping with the disease, she said, and with a mutual friend and Ms. Reicks, rode the first "Bike for the Cure" in 1999.
...
The women have raised more than $140,000 through their rides, and they hope to reach the $175,000 mark by the end of this trip.
Posted by Dave at 06:34 AM | Comments (0) | TrackBack
June 19, 2005
WHO Rankings
In researching the Canadian Supreme Court ruling the last couple of days I came across references to the World Health Organization's ranking of nations when it came to health care. Canada came in 30th and the United States came in 37th.
The U.S health care system may not be perfect (I don't know of one that is), the low score surprised me. So I did some digging...
The ranking was done in 2000, not too long ago. What I found was the ranking criteria was heavily ranked by who wrote the final check - Public or private health care spending. Medicare = good. Aetna = bad.
The report also ranked high their view of efficiency in spending. A criteria which will always work against the country that spends the most on health care (which is America). In this category Oman had the highest score even though it has a much lower quality of care(which includes an infant mortality rate twice that of the U.S.).
Another criteria was whether the cost of each individual's health care was based on 'risk' or on 'ability to pay'. This tends to skew against the more capitalist societies. Columbia came in first.
Another factor in the ranking was life spans. This isn't as useful of a measure as you would think. People in different countries make different lifestyle choices. Exercise & making healthy eating chioces (Japan/Utah) will tend to extend life spans regardless of the health system. However, due to differences in the health care systems a man in the U.S. is 24% less likely to die from prostate cancer than a man in Canada. Yet WHO ranks Canada seven spots higher in the world rankings?
So how heavily were those items weighted into the final scores? In these catagories the U.S. achieved the highest scores (best in the world):
Responsiveness to patients' needs for choice of provider
Dignity
Autonomy
Timely care
Confidentiality
So despite scoring 1st in these five (arguably important) categories, the U.S. ended up with a ranking of 37. So if you needed critical medical care which would you choose - America, Columbia, Oman?
Posted by Dave at 10:58 AM | Comments (0) | TrackBack
June 18, 2005
Oh Canada
Growing up just a few miles from the Canadian border I've always had an appreciation for the beauty of the country. If you ever have a chance to travel through British Columbia & Alberta, do it. Unfortunately, the country now has poor health system and there are those in this country (U.S) who wish to emulate it.
Recently the Canadian Supreme Court, by a one vote margin, acknowledged that fact. When Canadian politicians established the country's single-payer health system they were concerned that if private insurance was allowed, only the sick would participate in the government health plan. So they banned private insurance and forced the public to participate only (with some rare exceptions)in the public health care system. The arguments were the same as what we see in the U.S., by spreading the cost across all citizens and by providing equal access to health care the total cost would be better managed and the care would be better. Didn't happen.
As is par for the course in these matters, total costs started to rise. Patients started visiting their doctors for ailments that didn't warrant a visit in the past. The government didn't have an endless supply of money so big-ticket purchases such as MRI machines were postponed in order to pay for the increased clinic costs. Waiting lists started to develop for the use of the expensive diagnostic equipment. Lower medical salaries, the bureaucracy and the workload took their toll as fewer people entered the health care field and more moved out of the country. Waiting lists started to develop to see a doctor. There was equal access to health care, but it is equally poor and becoming poorer.
Last year, more than 800,000 Canadians, 2.5 percent of the population, were waiting for health procedures. The average wait to see a specialist, according to the Vancouver-based Fraser Institute, is nearly 10 weeks. The institute pegs the estimated cost to these patients at $2.2 billion, or roughly $2,700 a person.Canada ranks 20th of 25 industrialized countries in the number of MRI machines. It ranks 16th of 25 for CT scans, and 8th of 22 for access to radiation therapy machines. As for the relatively new Positron Emission Tomography (PET) Scans, at least one province flat-out refuses to invest in them.
Although touted by U.S. admirers as cost effective, the Canadian system is not inexpensive, it's merely cheap. Financed through heavy taxes, the average Canadian spends $7,350 a year to support the system. Yet for all this spending, Canadians are increasingly discovering they can't even get an appointment with a family physician. "It's like winning the lottery to get in and see the doctor," grouses Whitby, Ontario, Mayor Marcel Brunelle. Only 63 family doctors serve Whitby's 110,000 residents. According to a recent poll, 4.2 million Canadians are without a primary care physician.
Which brings us back to the Canadian Supreme Court.
The Supreme Court found that waiting lists have become so long that they violate patients' right to "life and personal security, inviolability and freedom" under the Quebec charter of human rights and freedoms, which covers about one-quarter of Canada's population."The evidence in this case shows that delays in the public health care system are widespread, and that, in some serious cases, patients die as a result of waiting lists for public health care," the Supreme Court ruled. "In sum, the prohibition on obtaining private health insurance is not constitutional where the public system fails to deliver reasonable services."
Canada will now have to alter their health care system to adjust for this ruling. Done wisely, overall health care will improve. England modified their system to allow more private care and the quality of health care significantly improved.
So what does this have to do with those living outside of Canada with Huntington's Disease? There is no incentive in these single-payer health care systems to spend their limited treatment money on 'incurable' diseases such as HD. Communities like ours are hit hardest by these systems.
We need better solutions to health care, but a Canadian-style system is no solution.
Posted by Dave at 06:03 AM | Comments (0) | TrackBack
June 17, 2005
More On Canada's Health Care System
This isn't the first time I've posted on the subject of the Canadian health care system and it likely isn't the last either. As flawed as the US health care system is...it wouldn't be improved by following the Canadian model. Steve Chapman in the Chicago Tribune:
...Any debate on health care eventually arrives at the point at which one participant says, "We should have what Canadians have. Free care, universal access and low cost--who could ask for more?" ...The dirty secret of the system is that universal access is no guarantee of treatment. Sick Canadians spend months and even years on waiting lists for surgery and other procedures. In 1993, the average wait to see a specialist after getting a doctor's referral was nine weeks. Since then, according to the Fraser Institute of Vancouver, it has increased to 18 weeks.
The typical patient needing orthopedic surgery has time to get pregnant and deliver a baby before being called. The Supreme Court cited the testimony of one orthopedic surgeon that 95 percent of patients in Canada waited over a year for knee replacements--with many of them in limbo for two years.
In some cases, the delay lasts longer than the person enduring it. Or as the Supreme Court put it: "Patients die as a result of waiting lists for public health care." ...
After adjusting for the age of the population, the Fraser Institute compared 27 countries in the Organization for Economic Cooperation and Development that guarantee universal access to health care. By some mysterious alchemy, Canada has proportionately fewer physicians than most of these nations but spends more on health care than any except Iceland. ...
Admirers of our good neighbor to the north say the United States pours money into all sorts of fancy equipment but doesn't get better results by such measures as life expectancy. But life expectancy is affected by multiple factors, including education, crime rates and diet--with health care playing only a modest role. In those areas where modern medicine can make a big difference, the United States does very well.
Take breast cancer. In Britain, which is famous for its socialized system, close to half of all victims die of the disease, according to a recent Cato Institute study by John Goodman, head of the National Center for Policy Analysis. In Germany and France, almost one-third do. In Canada, the figure is 28 percent--and here, it's 25 percent. Our mortality rate for prostate cancer is 67 percent lower than Britain's and 24 percent lower than Canada's.
Posted by Dave at 01:19 PM | Comments (0) | TrackBack
June 16, 2005
Breakthrough In Growing Brain Cells
While politicians are busy arguing about embryonic stem cells we are seeing major advances in adult stem cell research.
cientists working in mice said they had found a way to identify master cells in the brain and grow them in large batches -- a potential way of helping patients grow their own brain tissue transplants.The scientists said they had found a process to make the cells multiply, which would be crucial in fighting degenerative brain diseases like Parkinson's and Huntington's.
...
These so-called adult stem cells could come from a patient himself so no donor and no immune system suppressing drugs would be needed.
...Writing in the Proceedings of the National Academy of Sciences, the researchers said they also found an efficient way to make the cells multiply.
"It's like an assembly line to manufacture and increase the number of brain cells," said Dr. Bjorn Scheffler, a neuroscientist at the University of Florida who led the study.
...
"As far as regenerating parts of the brain that have degenerated, such as in Parkinson's disease, Huntington's disease and others of that nature, the ability to regenerate the needed cell type and placing it in the correct spot would have major impact," said Dr. Eric Holland
Posted by Dave at 08:03 AM | Comments (0) | TrackBack
June 15, 2005
Thank You
After months of extremely irregular posting, I post two messages in a row and in response I get two quick (and very nice) comments welcoming me back!
Thank you. I'm surprised anybody is checking back after the irregular posting the last few months.
I started the blog almost two years ago. I am not at risk for getting Huntington's Disease but it has deeply affected my life. Starting the HD Blog was something I wanted to do to help the community at large. In response to my postings I received so many kind comments and emails from people who said that the website had made a difference in their lives. I can't tell you how gratifying that is to hear that!
The last few months have been a challenge for me. Suffice it to say, I have not participated in local HD activities out of respect to the wishes of another in the community. Between that, a hectic work life, and merging a family (I'm getting married next month)...the blog (and the readers) has suffered. My apologies. You deserve better.
There really has been a tremendous amount of wonderful news for the Huntington's Disease community that I have not posted here. I'm hoping to change that in the months ahead.
Do not give up hope! We are entering an amazing age in medicine and scientific research. The HD community is one of the early beneficiaries of these new advances.
Posted by Dave at 08:47 PM | Comments (0) | TrackBack
TREND-HD Study Now Enrolling
If you or someone you know has mild to moderate symptoms of Huntington's Disease and is over the age of 35, call the Huntington's Study Group at 1-800-487-7671.
They are enrolling for a new Phase III trial for Miraxion/LAX-101 called TREND-HD. In the last Phase III trial, those with a CAG count under 45 showed a 23% improvement after taking the drug for a year!
This study is being conducted at 43 locations in North America so there may be one near you. Those participating will either get Miraxion or a placebo the first six months. After that, all participants will get Miraxion the following six months. For more information visit the Huntington's Study Group website. You can also read Amarin's press release below:
Amarin Announces Commencement Of Recruitment By Huntington Study Group For Miraxion Phase III Clinical Trial
LONDON, United Kingdom, June 14th, 2005 – Amarin Corporation plc (NASDAQSC: AMRN) today announced that the Huntington Study Group ("HSG") has commenced recruitment for the U.S. Phase III clinical trial of MiraxionTM in Huntington's disease.
The HSG will be conducting a clinical study (TREND-HD) of ultra-pure ethyl-EPA in persons 35 years of age or older who have mild to moderate Huntington's disease. The HSG is a worldwide, not-for-profit group of physicians and other clinical researchers who are experienced in the care of Huntington's disease patients and dedicated to clinical research in Huntington's disease.
Huntington's disease is an inherited disease of the brain that usually begins between the ages of 30 to 50, and includes motor, cognitive and behavioral signs and symptoms. While there are medications to help relieve some of the disease symptoms, there is no known treatment to slow the progression of Huntington's disease, which affects about 30,000 people in North America.
Miraxion is thought to stabilize nerve cell membranes and improve neurological functioning, although the exact mechanism of action is not currently known. An earlier trial in subjects with Huntington's disease indicated that Miraxion at 2 grams daily was well tolerated over 12 months. This current trial is designed to determine the effect of this daily dose of Miraxion on motor (movement) signs and symptoms of Huntington's disease.
The current 12 month study includes a 6 month placebo controlled phase with research subjects being randomly assigned to receive either Miraxion (2 grams total daily dose) or a matching placebo. This will be followed by another 6 month observation phase in which all participants will receive Miraxion. Researchers at 43 sites in the United States and Canada will each enroll approximately 7-8 research subjects with early signs of Huntington's disease who are independently ambulatory (walking) and fully self sufficient in activities of daily living, such as eating, dressing and bathing.
There is no cost to participate in the study. Participants will be followed as "out-patients" at regular intervals for 12 months. Individuals with mild to moderate Huntington's disease who are interested in participating in this study should visit the Huntington's Study Group website at: www.Huntington-Study-Group.org or call the toll free number +1 (800) 487 7671.
Rick Stewart, chief executive officer of Amarin, commented; "commencement of recruitment to the Phase III clinical trial for Miraxion is a major milestone for Amarin. An immense amount of energy and dedication has been expended by all involved to achieve this significant objective."
Posted by Dave at 07:23 AM | Comments (2) | TrackBack
June 14, 2005
True Love
It's not all too often that we hear a happy story involving Huntington's Disease but I believe this one counts. From the Greeley Tribune:
Bob Rumsby held both of his wife's hands tightly, looked into her eyes and professed his love to her.He did that 46 years ago and he did it again Monday afternoon at the Windsor Health Care Center.
Rumsby, 69, knows what it means to stay with his wife, Sue, through sickness and in health.
"I believe in that," he said.
The couple celebrated their 46th wedding anniversary by renewing their wedding vows in a far different place than the church they were married at in 1959...
Rumsby said his wife suffers from Huntington's Disease, an inherited degenerative neuropsychiatric disorder that affects the mind and body. A sixth-grade teacher for 20 years in Fort Lupton and Berthoud, Rumsby said his wife quit teaching when diagnosed with the disease in 1989...
As health care resident Sue Thornton released one of the balloons that lifted into the blue skies following the ceremony, she said: "That was marvelous. It's like getting married again. True love stands the test of time."
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