Three States to Begin Testing New Homebound Rules
Man with ALS Spearheaded Change
by Christina Medvescek
 |
|
MDA helped David Jayne travel to Washington
for the June 3 announcement of the three states that will test
the David Jayne Homebound Amendment. With him were, from left,
son Hunter, caregiver Gianna Wright, mother Georgia Jayne, and
daughter Hannah.
Photo by Kyle Kreutzberg
|
Attention people with ALS in Colorado, Massachusetts and Missouri who
receive Medicare home health care services:
Want to catch a movie?
In October, Medicare will begin a two-year, three-state test project
to see what will happen if up to 15,000 permanently disabled home health
care beneficiaries are allowed to go to the movies, their children’s
band concerts, barbecues with friends — basically anywhere they’re
able to go except to do paid work.
Medicare regulations now require beneficiaries to remain “homebound”
except for brief, infrequent absences of an approved nature. Violating
this restriction can result in loss of services.
The test project was created in an amendment to the Medicare prescription
drug bill, which Congress passed in November. It will gauge the effect
of dropping the homebound restriction on the home care program’s
cost, participation rate and quality of care.
After reviewing results, Congress will decide whether to suspend the
restriction for beneficiaries nationwide.
A Tiny Step
“It’s not a question of cost, it’s a matter of freedom,”
said Rep. Edward Markey (D-Mass.), expressing doubt that the test will
result in higher costs or greater participation rates.
“Lifting restrictions on the length, frequency and purpose of
departures from home would mean the world to these patients and their
families.”
The David Jayne Homebound Amendment was sponsored in Congress by Markey
and Sen. Susan Collins (R-Maine).
It’s named after the originator of the drive to reform the homebound
restriction, a Georgia home health care recipient and 16-year ALS survivor.
Jayne, who briefly lost his services for attending a football game,
organized a national coalition and traveled to Washington several times
to lobby for change.
The test project represents a tiny step in the movement away from
institutionalization and toward greater integration of people with severe
disabilities into their communities.
“This demonstration will give those with chronically disabling
conditions a chance to live full lives and contribute to their communities
while still receiving services in their homes,” said Tommy G.
Thompson, secretary of the U.S. Department of Health and Human Services
(HHS), in naming the three test states on June 3.
Who Benefits?
In-home services are available to Medicare recipients certified by
their doctors as needing daily skilled nursing or therapeutic care (not
simply custodial care).
Some beneficiaries only use the program for a short time. But the
test project is aimed at long-term users, such as people with ALS, who
have permanent, severe disabilities that aren’t expected to improve,
and who meet the following criteria:
• Need permanent help with three of five activities of daily
living (bathing, dressing, eating, toileting and transferring)
• Need permanent skilled nursing or therapeutic care
• Require assistance to leave home
• Aren’t working outside the home
People in the three test states who meet these criteria should contact
their home health agencies or local Medicare offices about participating
in the test project.
Enrollment Campaign Important
Jayne is concerned that lack of publicity and low interest from home
health agencies (HHAs) may make it difficult to enroll 15,000 participants,
which in turn could endanger the project’s success.
HHAs, which say they lose money serving high-need beneficiaries, “don’t
have any motivation to recruit participants,” Jayne said.
It remains to be seen how hard the Centers for Medicare and Medicaid
(CMS) will work to enroll beneficiaries, he said. CMS is the federal
agency that administers Medicare.
“If an adequate population sample isn’t achieved, CMS
most likely will not report favorably to Congress, regardless of the
data,” Jayne warned. “We need a vigorous information campaign
in the three states in particular, as well as nationally.”
For more information on changing the homebound restriction, visit www.amendhomeboundpolicy.homestead.com.
Back to top
MDA Opens ALS Centers in Little Rock, Rochester
MDA’s ALS Division has added two new MDA/ALS centers to its
roster, bringing the total of these ALS–focused centers at top
medical research institutions to 32.
The latest centers to be designated by MDA as focal points of ALS
service and research are at the University of Arkansas for Medical
Sciences College of Medicine in Little Rock and the University of
Rochester Medical Center in New York state.
MDA/ALS centers use a team approach to assist people with ALS to
better cope with the disease and to apply the latest therapies in
a positive and caring setting.
The director of the new center in Little Rock is Stacy Rudnicki,
an associate professor of neurology and director of MDA’s outpatient
clinic at the university. The Rochester facility is directed by Charles
Thornton, an MDA research grantee who also co-directs the university’s
MDA clinic.
A complete list of MDA/ALS centers and contact
information can be found at www.als-mda.org/clinics/alsserv.html.
To make an appointment at an MDA/ALS center, please contact the
MDA office nearest that center. All of MDA’s 235 hospital-affiliated
clinics serve people with ALS.
Back to top
Support for VEGF Treatment Grows
by Margaret Wahl
 |
|
Peter Carmeliet |
The gene for vascular endothelial growth
factor (VEGF), when injected into mice with an ALS-causing genetic
mutation, showed significant benefits, say researchers at Oxford BioMedica in England, and the University of Leuven and the Flanders Interuniversity Institute for Biotechnology, both in Belgium.
The research team, which published its results in the May 27 issue
of the journal Nature, included Peter Carmeliet of the University of Leuven and the Flanders Interuniversity Institute, who receives MDA funding for work with VEGF that’s closely related to this study.
Flaws or deficiencies of VEGF, which plays a role in the development
of blood vessels and may have an additional role in supporting nerve
cells, have recently been implicated as possible causes of ALS and
spinal-bulbar muscular atrophy. (See “VEGF
Deficiency Increases ALS Risk,” August 2003, and “VEGF
Deficiency Implicated in Second Disease,” April 2004.)
ALS Onset Delayed
In one experiment, the researchers injected genes for VEGF into the
leg, diaphragm, intercostal (between the ribs), facial and tongue
muscles of 3-week-old mice.
These animals are ALS models — bred to develop the disease.
They injected another group of ALS mice with a gene that has no known
therapeutic value (a “marker” gene). Both injected genes
were packaged in an equine infectious anemia virus (EIAV) delivery
system.
The mice that received the VEGF genes began to show disease symptoms
at about 123 days of age, while the marker-treated mice showed symptoms
at an average of 95 days.
VEGF-treated mice also had a significantly longer life span than
marker-treated mice. They lived an average of 163 days compared to
125 days for the other group a 30 percent increase in survival
time.
Neurons Preserved
The researchers also tested mice with ALS symptoms to see whether
they could be helped by VEGF. This experiment has important implications
for treatment of people with ALS.
The investigators gave 90-day-old mice the same types of injections,
using either the marker gene or the VEGF gene. They found that the
marker-gene-treated mice survived to an average of 127 days, while
the VEGF-treated mice lived an average of 146 days — a 15 percent
difference in life span.
The VEGF treatment also slowed the loss of motor (movement) skills.
After the genes were injected into muscle, they were transported
up the nerve fibers (axons) into the nervous system. When the researchers
analyzed survival of motor neurons (the muscle-controlling nerve cells
affected in ALS), they found far more surviving facial motor neurons
and spinal motor neurons in the VEGF-treated animals than in those
receiving the marker.
In addition, the mice showed minimal unwanted immune responses to
the new genes or the delivery virus, and no unwanted changes in the
blood vessels were noted.
 |
|
After
inserting the gene for vascular endothelial growth factor (VEGF)
into a modified equine infectious anemia virus, researchers
injected the gene-carrying viral particles into the muscles
of mice. The particles were then transported from muscle cells
up into nerve cells, where they improved cell survival. |
Human Trials Planned
“We have shown that VEGF is a potent neuroprotective factor
with clear therapeutic effects in a model for motor neuron disease,”
the authors say in summarizing their paper. They noted that the compound
has potential as a safe and practical treatment for the motor symptoms
of human ALS.
“The therapeutic potential of this technology should now be
evaluated in patients,” Carmeliet said, adding that “plans
to move forward in this direction are under way.”
Carmeliet acknowledged there are some obstacles to virus-based delivery
of genes in people. His group is exploring delivery of the protein
made from the VEGF gene’s instructions directly into the brain,
he said.
Study Bolsters VEGF as ALS Cause
In another study, researchers at Roger Salengro Hospital, associated
with the University of Lille, France, added support to the theory
that VEGF deficiency could be involved in ALS development.
They found that the level of VEGF is lower in the fluid surrounding
the brain and spinal cord (cerebrospinal fluid) in people during their
first year of ALS than it is in the CSF of people with other neurologic
diseases or in healthy people.
The finding is published in the June 8 issue of Neurology.
Survivors Tell Health Care
Professionals: Give Us Hope
 |
|
Cheryl Carter New |
Cheryl Carter New, an author in Inman, S.C., has had ALS for nine
years and sees herself as someone with a disability, not a fatal disease.
She interviewed several people who’ve lived with ALS for seven
to 15 years. In December,
she reported on “The Survivors: What Keeps Them Going.”
In this article, she conveys what these long-term ALS survivors like
their health care professionals to know about living with the disease.
by Cheryl Carter New
We asked the survivors what they would say to the health care professionals
they’ve encountered, including doctors, nurses and technicians.
Their comments are presented here as a series of tips or suggestions.
The common theme: Give us compassion, respect and hope.
Hope Is Not a Bad Four-Letter Word!
• First and foremost omit the words false hope from
your language. Always encourage patients to leave the doors of possibilities
open.
• Have compassion for a patient faced with receiving such a
disabling diagnosis and/or prognosis as ALS. Don’t lay such
an overwhelming load on a person’s heart unless they’re
accompanied by at least one loved one.
• We all have a choice within to consider ourselves as living
or dying. I am living!
• I know “normal” healthy people who are dying because
their spirits are broken. Not all dead people were “dying,”
and not all people alive are living.
• There’s more to life than our individual lives. Life
is also about raindrops splashing down to feed beautiful scenery within
the soil of the earth, and the sun beaming down to make the scenery
visible…. A crisp wind blowing to supply us with a scent of
this beautiful scenery…. The moon and twinkling stars to brighten
the night….
These events also feed my spirit and create beautiful scenery
within. The spirit within will never die.
Fatal or Manageable?
ALS was once just a fatal disease made famous by Lou Gehrig. Many
long-term ALS survivors now see themselves as having a disability,
not a fatal disease. But they remain interested in research to find
better treatments and cures.
Gehrig didn’t have choices, but we do — such as feeding
tubes (not gross at all) and improved respirators.
Christopher Reeve (who has a spinal cord injury) has a device like
a pacemaker controlling his diaphragm to permit breathing without
a respirator. Many research organizations are studying the technique
for other diseases involving paralysis.
• ALS has long been labeled a terminal disease and it’s
time to reconsider that label. Although no cure or particularly effective
treatment has been found, technology is making it increasingly possible
for people with ALS to outlive the grim statistics for many, many
years.
• Change the label from terminal to manageable.
• Decisions about feeding tubes and noninvasive or invasive
ventilation cannot be made far in advance.
You don’t know just how your disease will progress, how
you and your family will adapt, what your tolerance for dependency
will be, what your comfort level will be. You don’t know what
other medical problems might complicate things, what pastimes you’ll
find to keep you entertained and even productive, what financial assistance,
technology, help from friends, etc., will be available.
• Improved respiratory support can keep us alive, and computer
technology can make it possible to continue to communicate, a key
factor in maintaining an acceptable quality of life. There are still
many limitations to technology, and the price is high, both financially
and in terms of family commitment. But it’s time for health
care professionals to treat ALS as a severe and progressive disability,
not a death sentence.
• I would like more money and time to be spent in research.
As much as my electric chair means to me, I would give it up if that
money could be used to find a cure.
The Will to Live
Most long-term survivors said the factors that motivated them to
continue living with ALS had to do with family, faith and quality
of life. Health professionals seemed to play little or no part in
this aspect of their lives.
• With my wife, with our family, with my company, with our
church, the question of not living never came up. The question of
how we would manage life came up often, but there was always an answer
at hand.
• The trick to finding answers is simple: Ask. People who accept
the view that there are no answers will find no answers.
• Overcoming obstacles has simply been a practical matter
because of my slow progression. I’ve got no choice but to deal
with the hassles of increasing disability because I keep waking up
every morning!
• I’m not fighting to live, just finding ways to be physically
and emotionally comfortable. Using BiPAP wasn’t death-defying.
I knew that with my slow progression, death was still many, many months
away. I just wanted a good night’s sleep!
• Strong family support, especially from my wife, makes all
the difference. Having other resources provides necessities, such
as wheelchair, vans and communication devices, so I’m not a
tremendous financial burden on my family. Finding spiritual strength
to accept the disease and learn what I can from the experience is
important.
Words to Eliminate
Strike the word terminal from their vocabulary!
• Health care professionals shouldn’t cause ALS patients
to commit suicide.
•Don’t be so negative. You have no business in our decisions
except to give us options.
Last But Not Least
We can’t yet cure ALS or prevent it or stop it, but modern
management techniques and assistive technology now give us the possibility
of surviving it with an active and satisfying life. That possibility
is already in our grasp. We know how to do that.
Health care professionals: If you think life with ALS is unbearable
and that your clients with ALS are going to die, send them to someone
else before your “health care” kills their will to live.
Ex-Missionary Faces ALS With Ingenuity
and Faith
by Tara Wood
As a dentist, Charles Deevers made a lasting impact on the dental
health of his community.
But unlike most in his profession, Deevers’ community extends
from his home in Clinton, Miss., all the way to Western Africa.
Deevers spent 14 years as a dental missionary in Cote d’Ivoire
— the Ivory Coast — a country roughly the size of New
Mexico that borders the Atlantic Ocean.
Deevers ran a dental clinic there, and his wife, Dianne, oversaw
a hospital ministry. He also taught dentistry skills to several young
men “straight from the bush” (the sparsely inhabited backlands),
Dianne Deevers said.
“What is so precious to him, now three of those boys are still
doing it,” she said, including one young man who opened a clinic
in the neighboring country of Burkina Faso when government officials
saw that his skills were more than adequate.
Deevers also ministered to prisoners in Cote d’Ivoire when
dozens were starving to death each month because food funding had
been cut off. Deevers saved many prisoners’ lives with day–old
bread he got free from local bakeries, and a protein-rich soup made
from fish heads he bought cheaply with relief money from the Southern
Baptist Convention, which sponsored his ministry.
From Africa to ALS
Deevers, 65, started having symptoms of ALS about 11 years ago, a
few years after he and his wife returned to the United States.
ALS finally forced him to sell his Mississippi dental practice and
retire, a move he fought until the last minute, Dianne said.
 |
|
Charles and Dianne Deevers |
Although his days don’t include dentistry anymore, they’re
by no means dull. The couple stays busy keeping up with four adult
daughters and two grandchildren. Their youngest, Christy, 20, lives
at home and attends college.
“He goes to church and Sunday school and we go out and eat
on Friday nights. We have a standing engagement with some friends
we just love,” Dianne said.
Exploring the Internet, bird watching, and enjoying the natural beauty
of the outdoors are favorite activities.
Deevers can no longer speak or walk, but he can move his right arm,
and has some head and neck control.
“We pray for that right hand all the time because that’s
the key to keeping him mobile in his wheelchair,” Dianne said.
He uses a LightWRITER to communicate and supplemental oxygen when
needed; an accessible van has also enhanced Deevers’ ability
to stay active.
Innovation and Some Good Tea
While necessity may be the usual “mother of invention,”
Deevers learned that a complaining caregiver can be just as inspiring.
After Dianne complained about how heavy her husband was for her to
lift, “he realized that was not going to last that much longer
and he better come up with something else, and he did.”
He invented an innovative pulley system for several areas around
his house that lets Dianne lift and transfer him to his bed, the toilet
and shower with little effort.
Deevers engineered another simple support: a small piece of copper
tubing that he slips over the tip of his index finger. The device
helps him use his LightWRITER and personal computer for longer periods
of time.
The Deeverses have also found help from herbal tea and scopolamine
patches.
Intended for treating motion sickness, scopolamine has a side
effect of drying up oral secretions, Dianne said. That helps the couple
deal with a common side effect of ALS.
Nightly cups of Smooth Move, an herbal laxative tea made by Traditional
Medicinals, keeps constipation at bay.
Sharing the Wisdom
The couple sometimes share what they’ve learned in more than
a decade of life with ALS with a local MDA support group. Charles’s
words are particularly meaningful to people with recent diagnoses.
“He’s kind of the one that pumps them up. When they see
that, ‘Hey, you can last that long and have a pretty decent
life,’ that gives them hope,” Dianne said, not to mention
the jokes and funny poems he’s programmed into his LightWRITER.
The couple advises others with ALS to seek out new places and people
to stave off depression.
“Keep your mind busy. Best thing is just keep going as much
as you can. Keep mobile, keep moving, get out and don’t stay
in all the time,” she said.
Charles Deevers has also borrowed an attitude from a friend in Africa.
While riding in the back of a truck on a bumpy road one day, Deevers
asked his friend how he tolerated the frequent discomfort.
“I just think about it like it’s a sport,” the
friend said. Now that’s how Deevers chooses to face ALS: with
the heart and strength of an athlete.
A “vibrant faith in the Lord” is also crucial, his wife
added.
“It’s our faith that sustains us. I mean, Charles knows
that whether he lives or whether he dies, he’s still going to
win because God’s with him.”
Back to top
ALS RESEARCH ROUNDUP
by Margaret Wahl
Minocycline Shown Relatively Safe in Early
Trials
 |
|
Robert
Miller, director of the Forbes Norris MDA/ALS Research Center
at California Pacific Medical Center in San Francisco, conducted
one of the pilot studies of minocycline. |
Published results of two trials of minocycline in ALS show that use
of the drug in the disease is safe, but effectiveness hasn’t
yet been measured.
An MDA-funded, large-scale, phase 3 trial of minocycline to test
the drug’s effectiveness in ALS is now under way and still accepting
candidates. (See “Research
Roundup,” November 2002, or www.mda.org/research/view_ctrial.aspx?id=84.)
Minocycline, an antibiotic used to treat infections, is thought to
have cell-preserving and anti-inflammatory effects that could be beneficial
in ALS.
Results of the two trials completed in 2002 and 2003 were published
by Paul Gordon, co-director of the Eleanor and Lou Gehrig MDA/ALS
Research Center at Columbia University in New York, and colleagues,
in the May 25 issue of Neurology.
One trial, in which 19 participants each received 200 milligrams
a day of minocycline, showed no adverse effects.
 |
|
Paul
Gordon , who was then director of the MDA/ALS Center at the
University of New Mexico Health Sciences Center in Albuquerquue,
conducted the other trial. |
The other trial, in which 23 subjects received up to 400 milligrams
a day, found a suggestion of more gastrointestinal side effects in
those who received minocycline than in those taking a placebo. The
minocycline-treated group also showed more elevated enzymes from the
liver (suggesting possible liver damage) and a higher blood urea nitrogen
level (suggesting possible stress on the kidneys).
The second trial found that the average tolerated dose of minocycline
was 387 milligrams per day.
Researchers concluded that the drug’s safety level was acceptable
and that a larger trial in ALS was justified. However, they say it’s
premature to draw conclusions about the drug’s effectiveness.
They also caution doctors against prescribing minocycline for treatment
of ALS until the kidney and liver abnormalities can be further studied.
ALS Patients and Caregivers Overrate One Another’s
Stress
People with ALS and their caregivers each overestimate the psychological
and social impact of the disease on each other, says a study published
in the May 25 issue of Neurology.
A team led by E.E. Adelman at Northwestern University in Chicago
studied 60 patient-caregiver pairs at a time when the persons with
ALS were eligible for hospice programs and had significantly diminished
respiratory capacity (50 percent of normal or less).
They found the two groups agreed in their ratings of the patient’s
pain, control over ALS, optimism and will to live, and that this level
of agreement remained high over time.
However, caregivers rated patients as having less energy, more suffering
and greater weariness than the patients indicated for themselves.
And patients estimated caregivers as more burdened than the caregivers
reported for themselves.
The researchers said the findings should be reassuring for both those
with ALS and their caregivers.
ALS Online
Clinical-research chats in July and August will run from 5:30 to
6:30 p.m. Eastern time. Check at www.mda.org/chat/cli-res-host.html for an updated schedule.
John Bach, co-director of the New Jersey Medical School’s Jerry
Lewis MDA Neuromuscular Clinic and medical director of the Center
for Ventilator Management Alternatives at University Hospital in Newark,
will host a chat about Respiration July 28.
The NMD Chat en Español will be held Aug. 11, led by Carlos Garcia, MDA clinic director and
professor of clinical neurology at Tulane University in New Orleans.
Return to Issues
Index |
