Grateful to Jerry Lewis
I have been a big fan of Jerry Lewis for many years. Not just for his
movies, but for the fantastic work he does with MDA.
I lost my mom, Esther Ruth Kooper, last year. She had been diagnosed
with ALS in May 2003. I did not fully understand the disease until my
sister and I paid her a surprise visit on Mother’s Day weekend.
That was the last time I saw her alive.
She was a very stubborn and determined woman. She was not going to
let this disease beat her. It did.
We need to stop this disease. My mom had a gift — her humor —
which I think keeps me going just like Jerry does. He keeps on plugging
to find answers to the questions. Thank you.
Ronald S. Kooper
Rochester, N.Y.
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ALS RESEARCH ROUNDUP
by Margaret Wahl
Cyclosporine Revisited
The pharmaceutical company Maas Biolab, of Albuquerque, N.M., and Lund,
Sweden, has received Orphan Drug designation from the U.S. Food and
Drug Administration to develop cyclosporine specifically for ALS.
Cyclosporine is an immunosuppressant drug that already has FDA approval
to prevent rejection of transplanted organs and to treat rheumatoid
arthritis and psoriasis.
In ALS, investigators have found, its main mechanism is likely to be
protection of the mitochondria, the energy-producing units of cells.
Unfortunately, cyclosporine doesn’t cross membranes that lie between
the bloodstream and nervous system (the blood-brain barrier), which
has until now limited its potential benefits.
In the 1980s, oral cyclosporine was tested in people with ALS, but
it showed only modest benefit for some of the participants. Researchers
have found since then that the drug extends survival in ALS-affected
mice bred with an especially permeable blood-brain barrier and in mice
given the drug directly into the brain.
If cyclosporine could be delivered by a more direct route to the nervous
system, it might be effective in humans without causing unwanted immunologic
effects, investigators have speculated.
The Maas product is designed to be put into the fluid surrounding the
spinal cord via a pump implanted under the abdominal skin.
Ceftriaxone Helps Mice
The antibiotic drug ceftriaxone, scheduled to be tested in a multicenter
clinical trial in ALS this year, has shown significant benefits in mice
with a genetic form of the disease.
Researchers at Johns Hopkins University in Baltimore and Columbia University
in New York reported their findings in the Jan. 6 issue of Nature. The
team included Jeffrey Rothstein, who directs the MDA/ALS Center at Hopkins.
Rothstein and others say they believe ceftriaxone increases production
of a glutamate transporter, a natural but potentially toxic substance
that removes glutamate from the vicinity of nerve cells after it has
transmitted a signal.
When mice genetically destined to develop ALS were given ceftriaxone
injections starting at 12 weeks, they showed significantly better strength
and higher body weight than did animals treated with a salt solution,
and the effect lasted for four to six weeks. They also lived an average
of 10 days longer than the mice treated with the salt solution.
When 10-week-old mice received ceftriaxone for two weeks, they had
significantly more muscle-controlling nerve cells than the non-ceftriaxone
group, and their spinal cords had higher transporter levels.
Ceftriaxone has to be given intravenously to penetrate the nervous
system.
Modafinil May Help With Staying Awake
The drug modafinil (Provigil), which has Food and Drug Administration
approval for the treatment of certain sleep disorders, may help with
the daytime sleepiness in ALS.
Physicians Greg Carter and Michael Weiss, co-directors of the MDA/ALS
Center at the University of Washington-Seattle, and colleagues, studied
15 people with ALS-related fatigue.
The participants were asked about their sleep habits and fatigue before
and after taking 200 or 400 milligrams a day of modafinil for two weeks.
The results, which are published in the January-February issue of the
American Journal of Hospice & Palliative Medicine, showed that modafinil
was well tolerated and that all the measured factors showed improvement.
The investigators suggest that a morning dose of 200 milligrams may
be the best regimen.
Novartis Drug Fails ALS Test
Novartis Pharmaceuticals has announced that its experimental ALS drug,
TCH346, has failed to show any difference from a placebo (inert substance)
on measures of disease progression or survival.
In a phase 2, multinational trial that included 591 people with ALS,
the compound didn’t live up to expectations.
During the six-month study, participants took one of four different
doses of TCH346 or a placebo. No obvious adverse effects occurred in
the trial.
Novartis recommends that patients currently on TCH346 contact their
study centers immediately for advice on discontinuing the drug.
Researchers Associate Enzyme With ALS
Researchers from Massachusetts General Hospital in Boston, and University
College and King’s College in London, report an elevated activity
level of an enzyme known as reverse transcriptase in the blood serum
of ALS patients.
The investigators, who included Robert Brown, director of the MDA/
ALS Center at Massachusetts General, and Merit Cudkowicz, who has related
MDA support, analyzed the serum of 30 ALS patients without any family
history of the disease. They then compared the ALS serum to serum from
14 blood relatives of the patients, 16 of their spouses and 28 people
who were neither relatives nor spouses.
Activity from reverse transcriptase (RT), an enzyme that certain viruses
use to replicate themselves, was noted in 47 percent of those with ALS,
13 percent of their spouses, and 18 percent of non-spouse, unrelated
participants. Unexpectedly, 43 percent of the ALS patients’ blood
relatives showed RT activity.
The researchers, who published these findings in the Feb. 8 issue of
Neurology, say the RT activity in the patients and their relatives is
more likely caused by inherited retroviruses present in their shared
DNA, rather than by a new virus, which would likely be shared by spouses.
Inherited, dormant retroviruses are common, they say, and they’re
occasionally activated by a variety of factors.
The authors say there is some "biologic plausibility" to
the suggestion that activation of retroviruses in humans might have
a role in ALS, although that role is unclear.
Physical Activity Affects ALS Onset But Not Risk
A Dutch study of 219 people with ALS and 254 people without ALS has
revealed that physical activity, even if it’s extreme, doesn’t
increase the risk of developing the disease. However, those whose leisure-time
physical activity level was high showed ALS symptoms sooner than those
whose activity level was low.
Subjects who reported high leisure-time physical activities during
the 10 years prior to disease onset developed ALS an average of three
years sooner than the low-activity group.
Subjects with high scores on leisure-time physical activity before
the age of 25 developed ALS an average of seven years earlier.
The investigators, at University Medical Center in Utrecht, the Netherlands,
published their findings in the Jan. 25 issue of Neurology.
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Free Dasher Programs Easy As ABC
by Tara Wood
Karin Lewis probably never thought of herself as becoming a spokesperson
for a computer program, but lately, that’s exactly what she’s
been doing.
Lewis is "completely sold" on an innovative and free computer
program called Dasher.
Dasher is a text-entry interface (translation: a program that helps
you type or create text) for people who can’t use an ordinary
keyboard. It was created by a team of physicists led by David MacKay
at Cambridge University in Great Britain.
Lewis, 56, received a diagnosis of ALS about seven years ago, is just
barely able to move her hands, and uses a power wheelchair for mobility.
She worked as a secretary for General Atomics, a research and development
firm until about two years ago.
Lewis uses Dasher to enter text for e-mails and other lengthy writing.
"I like it because it is easy to use — it’s practically
effortless," said Lewis, who lives in the San Diego area, and read
about the program on an Internet chat transcript.
A "hands-on learner" and not a computer expert, Lewis said
the program was easy to download, and after some initial confusion,
she soon mastered its use by playing around with it.
Dasher is just one kind of assistive technology that fills the gap
for people who want to use a computer but aren’t able to input
data by typing the traditional way.
And although much of the latest technology can be expensive, Dasher
is a free "shareware" program that can be downloaded from
the Internet.
How It Works
You must be able see a screen, and in some way control the pointing
mechanism of your computer. That’s usually done with a mouse,
but also works with trackballs, touchpads, or eye-movement or head-tracking
systems.
The only other requirement is that you must know the alphabet, Lewis
said, since users select letters to form words from lists in alphabetical
order.
Lewis sets a wireless mouse on her leg while tilted back in her wheelchair.
She’s learning to use a head mouse for the time when she no longer
can use her hands.
Dasher starts with a screen that’s divided into quadrants. On
the far right side you have a jumble of characters that are initially
in alphabetical order.
As you move the pointer to the right of the center vertical line, the
characters start moving from right to left. Each letter is in a different
color box, and the farther right you move, the faster the characters
move.
You point to the character you want, and the screen zooms or magnifies
the area around it. Once that character crosses the center line, it
is selected and becomes part of your word.
After you pick a character you’re offered the entire alphabet
for selecting the next letter.
Dasher has such built-in energy-saving features as word completion,
and also learns the words you use most often.
For example, Lewis often types "Karin" so when she selects
K, A is presented up front, and if she selects that, then it presents
RIN as a block.
And It’s Fun, Too
Lewis said the text she types is saved to a generic file, like a "Notepad"
program. Then she copies or cuts and pastes it into an e-mail or word
processing program.
She sometimes uses an onscreen keyboard to enter numbers or for Web
browsing, since Dasher doesn’t yet work for those functions.
| Download the Dasher program from
this Web site, home of the Dasher Project at Cambridge University:
www.inference.phy.cam.ac.uk/dasher
Tip: You can also find the site by just entering the word "dasher"
into Internet search engines like Google or Yahoo.
Dasher is available in many languages, and can be used on Macintosh,
Windows and Linux operating systems. |
A less tangible bonus of Dasher is the enjoyable challenge it provides.
Often compared to a video game, Dasher permits users to build up speed
quickly; the program’s creators say some users can type up to
25 words per minute.
"[The letters] come at you so fast, it becomes a challenge: How
fast can I think and point and get the right words without making spelling
mistakes," she said.
Lewis has enthusiastically spread the word about Dasher, but contacted
MacKay and his team of developers just to make sure she "wasn’t
stepping on any toes" by doing so.
In fact, because it’s a "shareware" program, the developers
are open to suggestions about how to enhance Dasher, and those who know
how can feel free to make changes to it.
Dasher can be loaded on to many handheld computers (pocket PCs) and
laptops. Lewis said she heard about one person who incorporated it with
a speech-generating program to create a low-cost communication device.
But especially since the price is right, Lewis hopes that others with
ALS will try Dasher.
"I feel very strongly that a lot of people would benefit from
this," she said. "It’s really amazing how it works.
In 15 to 20 minutes you will be writing fluid sentences."
Equipment Corner, a new regular feature of the MDA/ALS Newsmagazine, will highlight assistive equipment and new technology of relevance to people with ALS. If you have a topic, item or question about any type of equipment or device that you’d like to see addressed, please let us know at publications@mdausa.org.
Rules for the Care and Treatment of Caregivers
by Jeff Lester
As a person with ALS who has been fighting this disease for over
11 years now (seven of those on a vent), I realized early on that
if I were to have a somewhat normal life, I would have to depend on
my incredible wife, Lisa, to achieve it. This meant that I would have
to follow some rules about the way that I treated her, my primary
caregiver.
It’s essential and appropriate that caregivers and care receivers
have some ground rules about their behavior toward one another.
The only rules I would give to caregivers are: Keep the relationship
the same as it was before ALS entered the equation (don’t make
it an adult/child relationship). And don’t abuse the power you’ve
been given as the other person becomes dependent on you.
Lisa’s and my close intimate relationship only partially prepared
us for the changes that occurred as I became dependent on her for
my most basic needs. Therefore, this transition could be even more
difficult for two people who don’t start out with an intimate
relationship. These changes are very difficult for both parties, especially
if the person who is becoming dependent starts neglecting his or her
role in the relationship. That is why the following rules are for
the people with ALS:
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From left, Lisa, Emily, Jeff and Kelsey Lester
|
1. Don't Take Advantage.
When I have to ask for something, I try to ask myself, "Is this
something I would have done myself or am I using my condition and
others’ empathy to get something extra?" For instance,
we shouldn’t demand a gourmet meal when Spaghetti-Os were OK
before, or insist on watching what we want on television (I have had
to develop an appreciation for Lisa’s soap operas since I’m
around all the time now). If we violate this rule, I feel it’s
entirely appropriate for our caregivers to call us on it.
2. End Pity Parties
Those of us with ALS are alone responsible for our happiness. I don’t
mean that we shouldn’t express our true feelings of frustration
or pain about our situation, but we shouldn’t wallow in it.
If we do, it’s right for our caregivers to point it out.
3. Be Useful.
No matter the limitations, there always are ways that people with
ALS can be useful and contributing members of our households and world.
It’s up to us to find out what those ways are, even if it’s
just listening to those around us and being supportive. Contributing
is vitally important because it allows us to see that our lives still
have meaning. This participation is much easier to achieve today because
of advances in computer technology.
4. Take Care of Yourself.
As much as possible, people with ALS need to stay involved in planning
for our needs, whether it’s the need for a lift, wheelchair,
adapted van, special diet, constipation aids, PEG tube — or
whatever. Also we must keep in mind easing our caregivers’ burdens
whenever possible. We should keep knowledgeable about the best way
our needs can be met, and when possible we should be the leader in
our care decisions. It is entirely unacceptable to take a passive,
or even worse, a resistant role in care decisions and then complain
when something is not done to our liking.
It’s inevitable that problems are going to arise between caregivers
and care receivers. It’s helpful for both to write down their
needs and talk together when they’re calm. I think the most
important thing that my wife and I do is to make sure my care doesn’t
interfere with our normal spousal relationship. We absolutely don’t
let disagreements over my care have any impact on our marriage. Care
and marriage are best dealt with as two totally separate relationships.
I feel strongly that those of us with ALS have responsibility for
our own care and actions, even though our lives may depend entirely
on the efforts of our caregivers.
May God bless our caregivers for all they do for us!
Jeff Lester ("ragingbear") founded and hosts the weekly
"Living with ALS" chat on the MDA Web site http://www.mda.org/chat/calendar.html every Monday from 4 p.m. to 6 p.m. Eastern time. In 2004, he became
a co-editor of the "Will Hubben ALS Research Digest" (www.als.net/research/hubben/signup.asp).
Besides working to help others cope with ALS, he keeps busy writing,
doing genealogy, helping in his family’s printing business,
and being a husband as well as dad for his two daughters (born since
his ALS diagnosis), Kelsey, 9, and Emily, 2, and expected third daughter,
Jordan, due April 24. Says Jeff, "I am a true believer that you
can LIVE with ALS."
WHEN ALS AFFECTS THE MIND
Organization, Medication Help, Says Psychologist Susan Woolley Levine
by Margaret Wahl
Susan Woolley Levine is a clinical psychologist associated with the
Forbes Norris MDA/ALS Center at California Pacific Medical Center
in San Francisco. She specializes in neuropsychology, a discipline
that explores the brain changes associated with various kinds of thinking
(cognition), feeling and behavior.
After earning a doctoral degree in psychology at the California School
of Professional Psychology in Alameda in 2000, Levine became a postdoctoral
psychological assistant and later a psychologist at California Pacific.
Recently, she contributed the chapter "Thinking and Behavior
in ALS" to Amyotrophic Lateral Sclerosis, by neurologists Robert
Miller and Deborah Gelinas and nurse case manager Patricia O’Connor,
released last year by Demos Medical Publishing. See the review in
January’s MDA/ALS Newsmagazine.
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Susan Woolley Levine Photo by Erin Lubin
|
Q: What kinds of cognitive changes occur in ALS?
A: Most people who have cognitive changes have mild problems,
such as difficulty paying attention in conversations, trouble concentrating,
and slowed thinking, such as trouble finding words. Shifting attention
from one thing to another can be particularly hard.
Q: How often do cognitive problems occur in ALS?
A: About 30 percent to 50 percent of the patients have some
cognitive changes, but most have changes that are so mild that they
wouldn’t be too noticeable unless testing were done. Progression to
true dementia — severe cognitive impairment — occurs in only a small
minority of patients. It’s not the same as Alzheimer’s disease
Q: What explains this?
A: There are some changes going on throughout the brain, not
just the motor parts. The frontal lobes, which are the "executive"
parts of the brain, are affected.
Q: Do these brain changes caused by ALS produce emotional as well
as cognitive effects?
A: Yes. The emotional effects can be things like apathy, not
being interested in things that one would normally be interested in,
and a decrease in motivation. Those things can sometimes be difficult
to tease out from depression, but they’re something very different.
Also, sometimes people can become less agreeable or more introverted
than they were. They might also have a little more difficulty detecting
or understanding the emotions that their family members might be experiencing,
and sometimes they don’t see the changes that their own personality
has gone through.
Brain changes can cause a person to become very frustrated or irritable
about a certain issue and get really stuck on it.
Q: Is it important to distinguish between cognitive and emotional problems
resulting from changes in the frontal lobes because of ALS itself and those
resulting from anxiety or depression associated with having the disease?
A: I would say it’s important to try to do that, because depression
or anxiety can be addressed with specific medications. (Editor’s
Note: Sorting out the subtle differences between cognitive dysfunction
that has its roots in depression or anxiety and that which may be
caused by frontal lobe changes is for a professional, not for family
members, to do.)
Q: What medications are commonly prescribed?
A: I’m not a physician, so I can’t speak directly to that. But
usually doctors try to address any mood problems that can be treated
with antidepressants or anti-anxiety drugs. Then, if those don’t seem
to help, they may try medications that are geared toward stabilizing
cognitive impairment.
There are no medications that are approved to treat cognitive changes
specifically in ALS. However, doctors may use those that are approved
for the management of Alzheimer’s disease, such as Aricept (donepezil),
Exelon (rivastigmine) or Reminyl (galantamine). These drugs affect
the acetylcholine (a chemical messenger) system in the brain.
We’re going to be conducting a clinical trial on the use of Aricept
in ALS. (Watch this publication and MDA's Clincial Trials page for information.)
Q: Other than medication, are there things family members and caregivers can do
to help the person with ALS-related cognitive impairment?
A: Organizing and simplifying are important. Have a specific
place for commonly used things, such as the television remote and
door keys.
Minimize distractions. If someone is trying to communicate via e-mail
or on the phone, make sure that the radio or television is off.
Encourage doing one thing at a time — not talking on the phone while
paying bills, for instance.
Help the person to write notes or use a tape recorder to keep track
of things. There’s no need for people to try to force themselves to
remember everything.
Minimize physical discomfort or pain. If the person is stuck on something
that’s upsetting him or her, that’s one time when a distraction is
a good thing. Try to divert attention from whatever is upsetting him
and bringing him to something enjoyable.
If the person is apathetic, try to think of things that have interested
him in the past, anything that the person takes to, like reading a
magazine or working on a puzzle, or playing with the dog. It doesn’t
have to be writing a life story.
Don’t try to convince the person that he or she has changed by pointing
out all the ways that he’s changed. That’s usually not very effective,
it wastes energy, and it can lead to a lot of frustration. It’s not
that the person with ALS doesn’t want to see. It’s usually
that they aren’t able to see these changes that they’ve gone
through.
Q: What about mental health counseling?
A: Family members and caregivers seek counseling more often than the patients themselves,
usually to cope with the diagnosis or with their own exhaustion. Looking at the pain associated with
their own disease can be quite overwhelming, but sometimes ALS patients seek help with relationships
or self-image.
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