In March’s MDA/ALS Newsmagazine, we met Sam Goldstein, 58, of St. Louis, who
agreed to report on his experiences with ALS since his diagnosis in August.
Here, Goldstein continues to explore the many changes in his life as a result
of this disease.
by Sam Goldstein
Since my last article, ALS has quietly taken over my left side so that it has
become more and more difficult to play my French horn, which is a left-handed
instrument and can’t be played right-handed. I can see an end to my music
career despite the Missouri Division of Vocational Rehabilitation doing
everything they can to prolong it by providing various tools and supports that
are available to musicians with disabilities.
A Painful Lesson
This Valentine’s Day, my wife, Jo-Ann, and I were looking forward to our monthly
MDA support group meeting and dinner with family members at a jazz club where
my oldest brother, Stanley, was playing the piano with his trio. But, at 3 a.m.
all those plans halted!
On my way to the bathroom, I slipped out of bed and fell to the floor. Jo-Ann
immediately wanted to call 911, but I felt that I wasn’t injured and, of
course, I could get up on my own.
After 30 minutes of trying, I got comfortable and went back to sleep on the
floor. At 6 a.m., we asked two neighbors to help — no luck — then called our
son, Ben, who came over and got me back on the bed. We cancelled all plans for
the day.
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The Goldsteins attend an MDA/ALS support group meeting, where they
visit with Reta Setzer, who recently received a diagnosis of ALS.
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Two days later, though, I had such pain in my back from the fall that I couldn’t
take it anymore. We went to the doctor, who couldn’t get me up onto his X-ray
table. He sent me straight to the hospital. With an MRI they discovered a
hairline fracture in a lumbar vertebra, painful but not dangerous. They did an
epidural in the hospital, a nerve-root block in the doctor’s office, and
switched all of my meds. That led to hospitalization number two, two weeks
later.
With the new meds came the side effects of severe constipation and consequent
impaction. The cramping was so severe we called EMS, and an ambulance
transported me to the hospital. The lesson I learned from all of this is that
when you can’t do everything for yourself anymore, you need to rely on others.
Call for help immediately!
I was only hospitalized for 42 hours, but in that time, I found I had to be my
own advocate at all times and had to take a stance with the doctors and nurses
about my medications, my food and my treatment, or they’d do as they pleased.
My problem was that my internist wasn’t on call that day, and a new doctor was
treating me.
I realized the need for all of us to put everything in writing in the form of a
medical directive so there’s never a question concerning our instructions about
our own care.
All of these experiences have given me the push to begin looking at my life from
a different angle. I now believe I can, in fact, live with ALS as
opposed to dying from ALS. I’m still scared of what’s to come, but I am
prepared to face it. I have a tremendous support group around me.
Learning to Adapt
Another huge change in our lives was the completion of our totally accessible
master bathroom. We have a roll-in shower, wheelchair-accessible sink, grab
bars, and handicapped toilet, all beautifully constructed with the latest
materials. You can even see it online at www.stlhomepro.com.
Having this bathroom now allows me to shower daily, which I couldn’t do before.
This gives me a whole new attitude and outlook.
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Sam and Jo-Ann celebrate Passover with their daughter, Alison Fox,
and her son, David.
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We attend a monthly MDA/ALS support group for newly diagnosed patients. Now that
I’m an “old hand,” I feel I can give advice to the new people that come.
Recently, a couple came in; the woman had recently received a diagnosis and was
complaining that her companion was urging her to climb steps when she said she
couldn’t. He thought that by urging her on, he was helping her, when, in fact,
we know that total exhaustion doesn’t help any of us. He didn’t understand that
until the meeting. The relief on her face when the rest of us explained the
situation to him was a joy to behold.
I can’t help people physically anymore, but I can get satisfaction from helping
others verbally and mentally.
Physically, I still use a walker inside my home, but now I must use a wheelchair
outside the house, which means that while I can still drive, I can no longer
travel alone. The next logical step is a power wheelchair, which we’ve begun
looking into.
I’ve reached the point mentally where I no longer look at these things as a
ratcheting down in my condition but rather as a tool to allow me the freedom to
continue my life as “normally” as I possibly can.
Thanks to all of you who e-mailed me (fhornsam@charter.net) after my first article. Sorry I couldn’t answer all of you, but I welcome any
comments and enjoy hearing from you.
Back to top
ALS RESEARCH ROUNDUP
by Margaret Wahl
ALS Community Exchanges Ideas at AAN Meeting
At the 58th meeting of the American Academy of Neurology, held in San Diego
April 1-8, the ALS research community learned of a number of intriguing
studies. Here are some highlights.
Stem Cell Transplants Benefit ALS-Affected Mice
Neural stem cells taken from mouse brain tissue and transplanted into the lumbar
spinal cords of mice with genetic ALS developed characteristics of motor
neurons (the muscle-controlling nerve cells affected in ALS), produced nerve
cell fibers (axons), and protected existing motor neurons, said researchers at
the University of Milan in Italy.
Stefania Corti at the University of Milan, and colleagues, found the treated
mice had a slower than expected disease course and lived longer than
ALS-affected mice usually do.
“The results [show] that the right stem cell may indeed reach a specific area of
the central nervous system and acquire morphological [structural] and
functional properties of mature, ‘normal’ neurons,” said Giacomo Comi, an
associate professor of neurology at the University of Milan, who was on the
study team.
Trophos Seeks to Stop Cell Death
Trophos (www.trophos.com),
a company based in Marseille, France, plans to test its experimental compound
TRO19622 in people with ALS later this year, after finding the compound safe in
healthy volunteers.
TRO19622 appears to work by stopping a cell death program known as apoptosis,
a goal of many investigational drugs for ALS. An early step in apoptosis is the
opening of pores in the membrane-enclosed “mini-organs” inside cells known as
mitochondria. When these pores open, fluid rushes in, and a membrane
surrounding the mitochondrion can rupture, allowing a chemical trigger for cell
death to leak out.
TRO19622, which was discovered by Trophos as part of a screening program to find
chemicals that keep nerve cells alive, has a cholesterol-like structure and
apparently interferes with the opening of mitochondrial pores.
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| The opening of pores in the inner mitochondrial membrane is an early step in the
cell death that TRO19622 may prevent. |
Detox Enzymes Vary in ALS Patients
Two independent groups, one of which included Denise Figlewicz, an MDA grantee
at the University of Michigan, and the other Teepu Siddique, an MDA clinic
co-director at Northwestern Memorial Hospital in Chicago, reported that
variations in paraoxonase enzymes may be a risk factor for ALS.
Paraoxonase enzymes help detoxify pesticides, insecticides and nerve gases. Both
groups found that some versions of the PON genes, which instruct for these
enzymes, were more common in people with ALS than in unaffected people.
The PON gene variations may help explain the increased risk of ALS in Gulf War
veterans. More data are expected this summer.
Blocking AChE Helps Mice Destined to Get ALS
EN101, a compound developed by Ester Biosciences (www.esterneuro.com) of Herzlia Pituach, Israel, apparently blocks the synthesis of the enzyme
acetylcholinesterase (AChE), thereby providing a modest life extension and
delay in symptom onset for mice destined to develop genetic ALS.
Marc Gotkine of Hadassah University Hospital in Jerusalem and colleagues
reported that their results support a role for AChE in exacerbating ALS and
suggest that blocking it may be worth pursuing.
ALS May Increase Blood Clot Risk
Researchers in Massachusetts studied 501 people with ALS between 1998 and 2004
and reported they had a higher than average incidence of deep vein thrombosis
(DVT), a type of blood clot known to form in people who are immobile (even
during a long plane flight) and have other risk factors.
DVTs are extremely dangerous, because they can break off and travel to the
lungs, causing a life-threatening event known as a pulmonary embolism.
In addition to prolonged immobility with pressure on blood vessels, dehydration
and a low-oxygen environment contribute to DVT development. (DVTs associated
with long-distance air travel in a cramped seat have been dubbed “economy class
syndrome.”)
In the general population, about 0.1 percent of people experience a DVT in a
year, while in hospitalized patients, the yearly incidence is between 0.8
percent and 1.3 percent. In the ALS group, the annual incidence was found to be
2.7 percent.
The research team, which included neurologist Merit Cudkowicz, who sees patients
at the MDA/ALS Center at Massachusetts General Hospital in Boston, recommended
that doctors and patients be alert to the increased DVT risk and that they
consider DVT preventive measures. (These usually include anticoagulant
medications and/or elastic stockings.)
Is ALS Becoming Less Aggressive?
Patients whose ALS was diagnosed at the MDA/ALS Center at Methodist Neurological
Institute (MNI) or at Baylor College of Medicine in Houston between 1999 and
2004 survived an average of four years, while those whose disease was diagnosed
between 1984 and 1998 lived an average of only three.
The increase in survival time and slower disease progression appear to be
independent of all known factors, reported Stanley H. Appel, director of the
MDA/ALS Center at MNI, and other investigators on this MDA-supported study.
They found no differences between the two groups with respect to use of
noninvasive ventilation, gastrostomy tubes or riluzole. Nor were there
differences in age, gender, the part of the body where symptoms began, the time
between symptoms and diagnosis, or baseline respiratory function.
The investigators concluded that the slower progression and longer survival are
due either to unmeasured aspects of care, or to the possibility that the
disease itself may have become less aggressive over time.
IV 'Naked' Gene Transfer Helps ALS-Affected Mice
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Gyula Ascadi at the cryostat, a device for slicing tissue
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A team that included MDA grantees Gyula Acsadi at Wayne State University in
Detroit and Jon Wolff at the University of Wisconsin-Madison reported they had
improved life span and sustained motor function in ALS-affected mice by
injecting growth factor genes without a viral transporter.
The researchers injected “naked” DNA for insulinlike growth factor 1 (IGF1) into
leg veins under pressure, and found that mice receiving these injections lived
an average of 28 days (25 percent) longer than untreated mice.
Acsadi later said he believes the IGF1 protein was either transported to nerve
cells from nerve endings or that IGF1’s presence caused the transmission of
neuroprotective signals.
The investigators concluded that IGF1 may be beneficial in ALS and that it may
be possible to deliver it without using viruses, a strategy that would improve
safety.
Two Meds Are Better Than One
A combination of two medications — sodium phenylbutyrate and AEOL 10150 —
appears to work better than either medication alone at extending life in
ALS-affected mice, say researchers at Weill Medical College of Cornell
University in New York.
Susanne Petri and colleagues, working in the laboratory of M. Flint Beal, an MDA
research grantee, found that a combination of the two drugs, given to mice with
genetic ALS after they developed symptoms, prolonged life span by 19 percent.
That compares favorably to treatment with sodium phenylbutyrate alone, which
allowed for a 13 percent life extension, or AEOL 10150 alone, which extended
life by 11 percent.
AEOL 10150 has been tested in ALS patients and found safe, and sodium
phenylbutyrate is undergoing safety testing, with results expected by fall.
The investigators, who published their results in the April issue of
Neurobiology of Disease, write, “This indicates that the two treatments
administered together may have an additive mode of action.”
Anti-Enzyme Compound May Help Preserve
Protection
The laboratory of M. Flint Beal at Cornell University (see “Two
Meds,” above), was also part of a group that recently published a study
of Ro26-2853 in mice with an ALS-causing genetic mutation, finding the compound
prolonged survival if administered early.
Ro26-2853 is an inhibitor of metallo-proteinases (MMPs), enzymes that digest
components of the gluelike matrix that surrounds and protects nerve and other
cells.
Previous studies have shown that there are fragmented, widely separated and
possibly “digested” bundles of this gluelike material in the spinal cords of
people with ALS.
In the current study, published online March 2 in Experimental Neurology, Stefan
Lorenzl, with colleagues in Germany, found that mice with genetic ALS have
higher than normal MMP activity in their spinal cords.
When the investigators gave Ro26-2853 to 1-month-old mice destined to develop
ALS but without symptoms, the animals lived an average of 13 days (11 percent)
longer than an untreated group.
However, when they gave the compound to 3-month-old mice, after ALS symptoms
were already apparent, it didn’t significantly improve survival time.
Nutrition
and Respiratory Support Investigated
A multicenter study directed by Edward Kasarskis of the University of Kentucky
in Lexington is under way to identify factors influencing how well people with
ALS tolerate noninvasive ventilatory support, and to develop measures of
respiratory and nutritional status for use in future trials.
Kasarskis, who is on the faculty of his university’s Graduate Center for
Nutritional Sciences, and colleagues at 11 institutions, are assigning 220
people with ALS to either a nutrition or a respiratory study group.
For information, contact Megan Thompson in Lexington, Ky., at (859) 323-8509 or megant@uky.edu. The
trial will probably remain open until late this year.
Combination
Drug Trial to Start Soon
A trial that will test two drug combinations, both of which have shown benefit
in mouse models of ALS, will open soon at about 20 centers throughout the
United States.
The study coordinators, who are based at the Eleanor and Lou Gehrig MDA/ALS
Center at Columbia University in New York, expect to enroll 120 people.
In the first part of the trial, the investigators will test two drug
combinations — minocycline with creatine, and celecoxib (Celebrex) with
creatine — to see which combination (if either) warrants further testing. In
the next phase, the winning combination will move to a large-scale trial.
For more information, contact Carolyn Doorish in New York at cd2141@columbia.edu or (212) 305-2027.
Back to top
EQUIPMENT CORNER —
Programs Make Vehicles Affordable
by Alyssa Quintero
Several helpful options for acquiring accessible vehicles offer flexibility and
peace of mind to people with ALS and their families. The plans acknowledge that
a client might not need the van for an extended period and help make the
purchase of a vehicle more affordable.
When it’s time to purchase a van, Dana Roeling, executive director of the
National Mobility Equipment Dealers Association, suggests that you contact
NMEDA (www.nmeda.org) to learn about its
member-dealers in your area and those that are certified under its nationally
accredited Quality Assurance Program (QAP).
Buy-Back Options
The Ride-Away Handicap Equipment Corp., a mobility dealer based in Londonderry,
N.H., designed a buy-back option for people with diseases with predicted
survival rates of less than five years. In 2005 alone, Ride-Away bought back 30
vehicles.
“I started the buy-back program 15 years ago when I met a client with ALS,” said
Ride-Away’s CEO and presi-dent, Mark Lore. “He was trying to make sure that his
family wasn’t saddled with a lot of debt when he passed away.”
Lore explained that when Ride-Away sells a van, it considers the typical market
depreciation for the vehicle. Ride-Away then puts into writing the buy-back
value guaranteed to the consumer during each month of the financing commitment.
Lore usually encourages a shopper with ALS to purchase a slightly used vehicle
because “the loss that a person is going to take on a used vehicle is going to
be less than the loss on a new vehicle.”
Rollx Vans, a conversion company that only sells directly to the customer,
offers the Preferred Option Buy Out Program, which allows the client to “opt
out” of the finance agreement without penalty and turn in the vehicle.
The terms of the agreement include: a five-year finance term; a 20 percent down
payment; a minimum of 24 monthly payments; a vehicle in good condition; an
annual mileage limit of 12,000 miles.
If the van isn’t turned in, the client owns it after five years, or 60 monthly
payments.
Rollx President Mike Harris explained that the company designed the program with
ALS clients in mind. In addition, Harris said, the program’s also beneficial
because “the money they put into the vehicle is an investment and not a loss if
they end up keeping it for the five years.”
Lease Cancellation Program
Caraleasing, based in White Plains, N.Y., is a nationwide leasing company for
wheelchair-accessible vans, co-owned by Caral and Hal Masback. The company
offers a special five-year lease that can be canceled if the lessee no longer
needs the van.
Through the early cancellation program, a person must lease a new or used
vehicle for five years. To qualify, the lessee must have good credit and
provide a $2,000 down payment. The lessee can cancel at any time after making
15 monthly payments.
If the vehicle is returned prior to 60 months, the lessee must pay a one-time
cancellation charge equal to five months’ payments. For example, if the payment
is $500 a month, one must pay $2,500 to cancel the lease.
“The whole concept of the early cancellation program is when your future is
unclear,” Masback explained. “This just gives you so many choices, and you
don’t have to commit to owning a vehicle. You only get it for as long as you
need it, and it’s less expensive [than financing].”
Caraleasing’s terms cover the cost of the vehicle, including the modifications.
Some leasing companies don’t include the cost of the adaptive equipment in the
lease value, Masback added, so be sure to do your research.
Caraleasing won’t lease a vehicle that’s older than five years through the early
cancellation program.
Masback said that the company takes an individual’s credit rating into
consideration, especially when it’s affected by mounting medical costs related
to a disability.
“We really work hard to get people into a vehicle that’s going to be within
their means,” she said.
Furthermore, Masback suggests that consumers contact Caraleasing, which will
locate a qualified NMEDA or QAP mobility dealer in the client’s area.
Selling on Consignment
Consignment programs vary from dealer to dealer, but the general idea is the
same — for a fee, a mobility dealer will sell the vehicle before the finance
term ends and send you a check once the vehicle’s been sold.
For example, under a consignment agreement with Access Vans of Louisiana, owned
by Marcus Smith, the client keeps his or her insurance on the vehicle and signs
over the title to the dealer. The client and dealer agree on the selling price
of the vehicle, and once the dealer sells it, he mails the client a check.
Stephen Estes, sales manager for NorCal Mobility in Chico, Calif., explained
that when he takes a vehicle on consignment, NorCal Mobility charges an initial
fee of $250 for a detailed inspection.
From there, NorCal Mobility determines the retail value for both the chassis and
the conversion. The consignment fee is based on the overall value.
If a problem is found during the inspection, Estes said that repairs must be
made before his dealership will agree to sell the vehicle. The repair,
inspection and consignment fees are deducted from the sale price, and the
remainder is sent to the client.
NorCal Mobility also offers a buy-back option, but Estes added, “A person
benefits the most from a consignment agreement . . . if you used a buy-back
plan, . . . you only get trade-in value.”
