2008
National
Conference
June 6-7, 2008
Valley Forge, PA
Contributed by Beth Anne D. Shultz
The 2008 SPF Annual Conference was an exciting,
and wonderfully executed event. The weekend was a time to renew old
acquaintances, meet new people, and learn about the discoveries occurring in
the medical and scientific fields concerning Hereditary Spastic Paraplegia
and Primary Lateral Sclerosis. The news was one of exciting new
possibilities and great advances, but, as seems to be the trend with our
diseases, each new turn leads to more questions and neurological mysteries.
 Jim
Sheorn, President, gave a warm welcome to those of us who attended. On
Friday evening, his remarks centered on the number of attendees -
approximately 100 people attended the Welcome Dinner and an additional 50
were present for Saturday's schedule of events. There were also around 20
individuals who took advantage of the Sunday Trolley Tour to the historical
areas of Valley Forge. According to Jim, this was the largest group of
attendees to date. We even had a representative from Canada! What a great
start to the 2008 SPF Annual Conference.
Jim's introduction Saturday morning began with stressing his appreciation of all
the members of the Spastic Paraplegia Foundation and was delighted that so many
of us attended. He highlighted, in particular, four special groups in our
organization. It is the people in these four groups, whose talent, commitment
and determination are crucial to the existence of our organization. In brief,
they are: The Conference Champions, The Board Members of Past & Present (to
include our talented medical advisor, John Fink, MD - University of Michigan),
State Ambassadors and Major Donors.
 The
Conference Champions included Anna Bonanni, Chairwoman of the SPF Annual
Conference & Event Coordinator. Her willingness to take on this enormous
task is greatly appreciated. Also, through Anna's contacts, the leather
journals, speaker gifts, signs and lanyards were donated to SPF for the
meeting. Medtronic and Athena Diagnostics were corporate sponsors. The two
companies' financial gifts have been invaluable in continuing our annual
meetings. Medtronic and WalkAide were our Booth Sponsors and gave
presentations and/or demonstrations at the patient breakout session.
The founding members of our
organization, our Board Members (past and present) and our medical advisor,
John Fink, MD are the real heart of the Spastic Paraplegia Foundation.
Without their creativity and dedication, the idea of a national foundation
for such rare diseases would never even have occurred. The reality of our
existence truly belongs to them and all of the volunteers who have supported
SPF over the past six years. One group of volunteers that was recognized at
the meeting were the State Ambassadors.
Last but most certainly not least, are the major donors who give us
financial support. As Jim emphasized at the end of his introduction, the
sharing of information and experiences is an important aspect of our
foundation. However, it is the financial gifts that keep us a viable
non-profit organization dedicated to the continuation of research,
knowledge, and eventually a cure. Every penny counts, but in this world of
high tech research and medical techniques, it is the major donors who allow
us to more easily hold fund-raisers, meetings and support groups. However,
as our 2007 financial bottom line indicates ($503,728.00), it takes ALL OF
US to help keep our organization in the public eye; especially one that
needs to continue to grow. If you would like to know more about how you can
help, please visit our website at:
www.sp-foundation.org.
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SPF Board of Directors with Ben Franklin
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Dr. John Fink, SPF Medical Advisor, and Annette Lockwood, Board Member
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Past Board Members, Marlene Doolen and Kathi Geisler along with Carrol Doolen.
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Thanks to all of the volunteers!
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Our NH State Ambassador, Judy Johnson with her husband Carl.
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 We had five interesting and
informative speakers at this year's conference. Separate papers have been
written on four of them, and are available by clicking here. Patricia Leisner Clements, Attorney at Law, gave a presentation about Social Security
Disability Benefits. Specifically, she concentrated on guiding us through
the application process in a step-by-step format. She also addressed the
procedure of appearing before a Social Security judge, and then concluded
her speech by discussing some of the aspects of Medicare. I found it quite
easy to follow and she answered several questions that I have had about the
SSDI process. Additional information can be found at
http://www.socialsecurity.gov/pgm/links_disability.htm.
Peter Baas, PhD, is a researcher at Drexel University and a recipient of a
SPF research grant. He gave a fascinating overview of a
theory he is researching regarding HSP. The specific title was: Microtubule
Severing in HSP: Enough is Enough. This sounds complicated but he did a fine
job of making it easy to understand. His research revolves around the gene
SPG4, which is believed to account for up to forty to sixty percent of all
cases of HSP. His theory is that within this gene, there are two different
proteins that have mutated and that one of them – M1 is toxic and
responsible for the degeneration of the axons. In addition, this toxic
protein does not weaken over time, but continues to multiply. This causes
more and more difficulty for the axons, and thus, he believes, for those of
us with HSP. Speech
Summary
 Mary Kay Floeter MD, PhD, of the National Institute of Health gave an
insightful presentation about her ongoing research with PLS. One of the more
interesting aspects of her speech was the mention of a new classification
scheme for PLS. This is not universally agreed upon, but, PLS is now being
classified into three different categories: PLS; PLS-Plus; and Secondary
Lateral Sclerosis. The differences are minimal but the theories behind them
have huge ramifications. The actual topic of Dr. Floeters’ speech was about
the challenges in current PLS research. The first difficulty is that PLS is
such a rare disease. This leads to a simple problem of economics – lack of
funds. The second culprit is defining a universal definition of PLS and then
choosing appropriately accepted strategies to find the underlying cause.
This is important because how it is defined will determine the strategy used
to find a cure. Speech
Summary
Rosette Biester, PhD, of the University of Pennsylvania spoke about the
challenges of living with a chronic disorder. Her views and methodologies
revolve around Cognitive Behavior Therapy and Theory and can be applied to
both spouses – the one living with disease and the one designated as
‘caregiver’. Biester’s presentation gave those of us living with a chronic
disorder six challenges: Don’t run from problems (Identify, Define &
Generate Solutions); Ask for help – REALLY; Build in personal activities
each day; Change routines; Challenge yourself with new educational pursuits
or hobbies; and SOCIALIZE.
 The
fifth speaker was, of course, Dr. John Fink, of the University of Michigan. He
was quite excited to tell us about significant advances made in research
concerning HSP and PLS.
The first advance in HSP and PLS research that Dr. Fink discussed was that
his research facility is now doing experiments on rats instead of worms,
fruit flies and mice. This may not sound like a huge step, but it is in the
scientific world. The spinal cord of a rat is longer and larger than those
of mice, and this makes it easier when dealing with microscopic entities.
The current experiments are comparing the ability of rats (healthy and
‘sick’) to: walk across a narrow ledge; run on the inside and outside of a
wheel; and chart the reflexes of their hind feet. The short film showing a
disabled rat held up by his tail (gently), left no doubt that its hind feet
were having trouble moving. It was evident that the movement was awkward,
jerky, and stiff. Amazing!
The second advance in Dr. Fink’s research is the most exciting. His facility
will be ready to hold clinical research trials for all of those with HSP and
PLS within 12 to 24 months. This is an incredible step and is the first such
opportunity for anyone with these diseases. The trials will be divided into
high tech and low tech sessions. The high tech research tests need to be
performed at his facility in Michigan, but the low tech tests can be
performed in our homes with materials and instructions sent via the mail.
ANYONE with HSP or PLS can sign up for this and he made it very clear that
he needs as many of us as possible. Please consider participating in this
important step. Notification of the start of the trials will be posted on
the SPF website.
Both PLS and HSP are disorders of “motor nerves” (“motor neurons”). Dr. Fink
also discussed advances in our understanding of motor neuron diseases in
general. He said that researchers are learning more and more that motor neuron
diseases often involve other parts of the nervous system (not just motor
nerves). He also said that there is significant overlap in the symptoms caused
by each disorder.
 During
the question and answer period following Dr. Fink’s presentation, two areas were
discussed for an extended period of time. One area of concern is whether or not
daily exercise can slow the progression of HSP and PLS. Many of the attendees
avidly support the concept of a daily exercise routine.
Dr. Fink also recommends
exercise. He stated that this recommendation was not based on scientific
studies but rather on the reports of dozens of individuals with HSP and PLS who
have found exercise to be beneficial. Dr. Fink referenced an ALS (Lou Gehrig's
disease) experiment performed on rats concerning this issue. The results were
fascinating. The animals that were exposed to mild exercise on a daily basis
lived longer than the animals that were exposed to moderate and severe
exercise. Exercise if you wish, but be cautious as to the level of intensity
and include your doctor(s) or physical therapist in the process.
The second topic discussed during the question and answer period concerns
the financial expense of research. Please note that this WAS NOT part of Dr.
Fink's speech. However, the two questions asked were this: Why isn't more
research being done to find a cure? Why is the research process so slow? The
answer to these questions has to do with the availability of funds. Dr. Fink
feels that methods are available to discover or develop treatments but that
research is limited by insufficient funding. Simply stated, research is very
expensive. Dr. Fink currently has at least seven new medications that are ready
to be tested in animal models of HSP. Dr. Fink stated that he would like to test
each of these medicines (and more) simultaneously instead of one at a time
(which would take many years). The price tag of testing one medicine for a
period of six months is $40,000. Wow! This illustrates the need for aggressive
fundraising by each and every one of us. Please join us in the 2008 TeamWalk
Campaign and begin to solicit sponsors for yourself. You can attend one of the TeamWalks scheduled,
host your ownTeamWalk, or participate as a
Walker by
Proxy. For more information on TeamWalks click
here.
The two breakout sessions divided the group into patients and caregivers.
During the Patient Forum, representatives from Medtronic and WalkAide spoke
about their products. WalkAide was also able to provide a demonstration of their
walking device. Medtronic is heavily involved with ITB Therapy (Intrathecal
Baclofen Therapy) or more specifically, the Baclofen Pump. The implanted pump
and catheter are surgically placed beneath the skin directly into the space
where fluid flows around the spinal cord. The pump is programmable and due to
the precise placement of the pump and catheter, the drug is delivered directly
in the spinal fluid and does not circulate throughout the blood. This helps
minimize side effects while significantly reducing spasticity. For more
information, please visit the web site at:
www.medtronic.com.
 WalkAide
is a medical device that was developed after years of research. It is now FDA
approved. WalkAide addresses the ‘foot drop’ problem that many of us develop
with diseases that damage upper motor neurons and the pathways to the spinal
cord. This device is a small, self-contained unit that consists of a
battery-operated electrical stimulator, attachment cuff, two electrodes, and
electrode leads. It is applied directly to the leg below the knee and can be
worn discreetly. A cuff holds the system in place. The stimulation from the
electrodes activates the muscles that cause foot drop to flex the foot at the
appropriate time during the gate cycle. For some patients, this produces a more
natural pattern of walking and can help minimize falls. For more information,
please visit the web site at: www.walkaide.com.
Athena Diagnostics (one of the corporate sponsors) wasn’t able to participate in
the event. Athena Diagnostics is a laboratory dedicated to the development of
diagnostic testing for neurological disorders. The genetic tests are quite
expensive, but available. For more information, please visit the web site at:
www.athenadiagnostics.com.
The Caregiver Forum was facilitated by Craig Gentner. Approximately 30 people
attended. This was an open forum intended to share concerns, issues, feelings,
and helpful ideas. Craig used a few poems from Thurza Campbell’s book, Carpe
Diem, to stimulate conversation. Craig summed up the meeting by saying the
following: “The more we talked, the more we all realized that regardless of the
disorder of our spouse or friend, we all have similar issues, concerns, and
feelings. Some look at it from the negative side – why me? While others look at
it from the positive side – how can I help? The open discussion by the group of
both positive and negative comments help in the process of understanding how to
improve one’s role as a caregiver.”
Summaries
and Presentations
Peter Baas, PhD -Microtubule
Severing in HSP: Enough is Enough
Presentation
Mary Kay Floeter MD, PhD -
Challenges in PLS Research
Presentation
Rosette Biester, PhD -
Living with a Chronic Disorder
Presentation
Patricia Leisner Clements, Attorney at Law -
Social Security Disability Benefits
Jim Sheorn -
Presentation
Author's Note: I wrote this synopsis of the 2008 SPF National
Conference with the help of many people. I wish to thank Annette Lockwood for
giving me the opportunity to participate in this way. There aren’t many people
who would give an "unknown" the chance to write about such an important topic. I
appreciate the time Peter Baas, PhD, Mary Kay Floeter, MD, PhD, and Rosette
Biester, PhD, gave to me in the form of phone calls, email, and copies of slides
so that I could write individual papers about their presentations. But most of
all, I wish to thank all of those involved with this annual meeting. It took a
tremendous amount of time and energy to put it all together and to pull it off
in such a successful manner.
As someone who has Hereditary Spastic Paraplegia, I wish to conclude this paper
on a personal note. I attended my first National Conference in 2007. I can still
remember the relief and joy of finally meeting others with the same disorder. As
we all know, just getting diagnosed correctly is a long ordeal. By the time my
Mom convinced my husband and me to attend the Nashville conference, I was
feeling isolated and in complete desperation for any information about this
disease. Those two days, in 2007, did wonders for me. Though the research news
was not encouraging as far as a “quick fix” goes, it did give me hope that some
day my children’s children would not be afflicted with this mutant gene I carry.
So I end with this quote from Lewis Carroll and hope that everyone who suffers
with HSP or PLS can eventually attend at least one National conference.
Alice laughed. "There's no use trying," she said. "One can't
believe impossible things. I daresay you haven't had much practice," said the
Queen. "When I was your age, I always did it for half an hour a day. Why,
sometimes, I’ve believed as many as six impossible things before breakfast."
Lewis Carroll
Through the Looking Glass
References
Two articles were used to help with my synopsis of Dr. Fink's presentation. They
are:
*NeuroCAST.com – Athena Diagnostics
*A Look at Upper Motor Neuron Diseases – Hereditary Spastic Paraparesis and
Primary Lateral Sclerosis
*Reviewed by John Fink, MD
*Authors: Carmel Armon, M.D.
Nam-Jong Paik, MD, PhD
John Fink, M.D.
Ashok Verma M.D. D.M.
Walter G. Bradley D.M. F.R. C.P.
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