A Bridge Between Two Worlds
As the ALS TDI matures and gains stability, it fills a gap between academic and industrial research.

by Margaret Wahl

When Jerry DeZutter first came to the ALS Therapy Development Institute in 2005, meetings focused on “frank and open discussions about the life of the institute — whether it would survive,” he says.

DeZutter, now head of the in vitro (test tube) validation group at this MDA-supported institute in Cambridge, Mass., says it’s been gratifying to move from being “stressed out about the survival of the company” to being “stressed about the science” as the institute matures.

MDA, which has pledged $18 million to ALS TDI through its Augie’s Quest research initiative, has put the institution on solid footing. “We’ll be solvent at least for the next several years,” DeZutter says.

Tuning out noise

When DeZutter first came to the TDI, the focus was on high-throughput screening of drugs in mice. Now, though, “instead of going blindly into the mouse” with drugs, the TDI has moved to what DeZutter says is the industry standard: putting an experimental compound into cells in a culture dish first “to see if the compound is behaving as they say it does” before moving it into an animal (in vivo) model, such as a mouse.

Jerry DeZutter checks to see that compounds do what they’re expected to do in a simple system before they’re studied in mice.

“There’s too much noise” in an in vivo model, he notes, referring to the almost infinite number of biochemical events taking place in an animal, and the difficulty of determining which ones are related to the experimental substance.

Testing compounds in vitro first is more economical, he says. For instance, some of the short hairpin RNA molecules purported to dampen activity from genes with ALS-causing mutations have not worked well in vitro, he notes, and need to be perfected in these simpler systems first.

Some high-profile compounds do skip the in vitro step and go straight to animal trials, DeZutter concedes. One recent example is lithium, which showed great promise in slowing ALS progression in a small human study in Italy and moved directly into mouse testing at the TDI, as well as an MDA-supported human clinical trial. But for most compounds, the in vitro step is a crucial saver of resources.

Information management provides ‘brain extension’

Down the hall from DeZutter are hundreds of mouse cages, all overseen by Fernando Vieria, the director of animal studies.

Fernando Vieira says the ALS TDI’s laboratory information management system is like an extension of one’s brain.

TDI’s mouse studies are exquisitely controlled. Not even the technicians know which mice are receiving an experimental drug and which are receiving a placebo (fake drug), eliminating bias from assessments. TDI can test far more mice in any given experiment than academic laboratories, Vieria says, because of its industrial-scale mouse program.

Vieria is particularly proud of the institute’s laboratory information management system (LIMS), designed for the TDI by Sean Scott, the institute’s president, with software experts.

“It’s like an extension of your brain,” he says, pointing to the widescreen display on the wall. “You can search for a gene and see what it does in brain, muscle and spinal cord. It also has everyone’s tasks for the day laid out, and it prints out bar-coded labels for every sample.”

Noting that the TDI has tested almost every compound that other groups have found promising (and found little to get excited about), the institute now is developing its own targets and validating them with its strict experimental methods.

“Every step we take gets us a little closer,” Vieira says. “It’s a little more light in a dark room. I’m confident in our process. If we plug away, we will find something.”

Monitoring how cells change as ALS progresses

Annette Ferrari specializes in extracting pure cell samples using laser capture microdissection.

Annette Ferrari specializes in “laser capture microdissection” (LCM), using specialized equipment purchased with MDA funding.

This equipment allows a skilled technician to select a specific cell type — like motor neurons, which die in ALS — from a tiny sample projected on a large screen and dissect them out for further examination.

TDI now is looking at motor neurons in spinal cord tissue from day 60 and 90 of the disease in a mouse model of ALS. “We want to see what changes in the motor neurons over the course of the disease,” Ferrari explains. Next TDI will be examining the neuromuscular junction, the area where nerve and muscle fibers meet, which has become a new focus of attention in ALS research.

Bridging the academia-industry gap

John Lincecum, associate director of research biology, is philosophical about where scientific research is heading. He thinks the TDI has the right research model for challenges like ALS.

John Lincecum, shown here with a device for homogenizing tissue, says equipment for high-tech lab work is improving.

“It’s an expensive endeavor to work on intractable diseases like ALS,” he says. “And when the marketplace doesn’t see dollars, they don’t do the work. Before the Bill Gates Foundation started working on malaria, no one was working on it. Gates made it possible by putting money into it.”

For the last 60 years, Lincecum notes, basic biomedical research has been funded mostly by the National Institutes of Health. “That worked well for a long time, until scale [size] began to overwhelm the system.” Academics don’t have the resources for challenges like ALS, he says; the pharmaceutical industry does, but generally isn’t interested.

“Big pharma can do it but won’t. They’re risk-averse,” he says, noting pharmaceutical companies are more comfortable tweaking a drug for erectile dysfunction than sinking resources into uncharted territory that might never provide a return on investment.

That’s where ALS TDI comes in. “The ALS TDI is a hybrid. It can lower the risk for pharma,” he says, describing the institute’s capabilities to provide large drug companies with the data they need to decide whether to invest $35 to $40 million into drug development.

Lincecum says the institute hires people who’ve worked in an industrial setting and applies industrial laboratory techniques, while keeping its “eyes on therapeutics” and expenses low. “We got these off the street, literally,” he says, pointing to conference room chairs covered in faded, brown fabric.

But the institute has spared no expense in purchasing equipment that speeds the research process. “What used to take three minutes now takes 10 seconds because of this machine,” he says, indicating a device that resembles a large food processor.

“We’re a bridge between two worlds.”

Research Roundup: Getting Past Barriers

Gene therapy experts discuss targeting ALS-affected cells
by Margaret Wahl

The 11th annual meeting of the American Society of Gene Therapy (ASGT) took place in Boston May 28-June 1. Among the dozens of sessions was an MDA-sponsored program on gene therapy for neurodegeneration.

Kenneth Fischbeck from the National Institute of Neurological Diseases and Stroke at the National Institutes of Health in Bethesda, Md., chaired the special session, which focused on identifying cellular targets and evaluating strategies for reaching them.

Like other neurodegenerative disorders, ALS primarily affects the central nervous system (CNS), the brain and spinal cord. Surrounding these tissues are protective membranes known as the blood-brain and blood-spinal cord barriers. These barriers prevent entry of many viruses and bacteria but pose a daunting challenge to gene therapists, because all ALS gene-therapy strategies require entry into the CNS, either directly or via muscle fibers.

Once these challenges are overcome, Fischbeck said, ALS gene therapy can attempt a variety of strategies, such as minimizing potential toxicity from glutamate; controlling damage to the mitochondria, which produce energy for cells; stopping abnormal clumping of proteins; blocking the effects of harmful genetic mutations; or protecting surviving motor neurons, the cells most affected in ALS.

Some researchers have used lentiviral vectors to deliver VEGF genes to nerve cells via muscle fibers.

Brian Kaspar, an MDA grantee at Nationwide Children’s Hospital in Columbus, Ohio, discussed how ALS is accompanied by a massive increase in the number of nervous-system support cells known as astrocytes. Early in the disease, astrocytes seem to help ailing motor neurons. But their proliferation also seems to activate another type of cell, the microglia, which can kill motor neurons.

The proteins VEGF and IGF1 delay the activation of microglia, and Kaspar’s group would like to deliver them to the ALS battlefront.

VEGF and IGF1 genes can be inserted into the shells of adeno-associated viruses (AAVs), a common method of transporting genes to muscle tissue. But getting these transporters into the CNS is challenging.

Some researchers have used transporters made from lentiviruses with success in mice. Lentiviral transporters (vectors) carrying genes for VEGF have entered muscle cells and traveled up nerve fibers to nerve-cell bodies, prolonging the lives of mice with an ALS-like disease caused by mutated SOD1 genes, Kaspar noted.

In addition, Kaspar said, cells deep in the cerebellar area at the back of the brain have been fitted with biological pumps that effectively deliver IGF1 to the nervous system, at least in mice.

Type 4 AAV vectors, Kaspar noted, can target special cells that line the ventricles (open spaces) in the brain, and they too could be used to deliver therapeutic proteins to the surrounding areas.

Jude Samulski says gene therapists are moving toward laboratory-engineered viral vectors.

But gone are the days of settling for what nature has provided in terms of AAV transporters, noted Kaspar and others during the meeting. Now it’s possible to manipulate surface proteins on AAV transporters in order to create new vehicles that can enter the central nervous system.

In trying some of these new transporters in mice, Kaspar has found therapeutic genes can enter 50 percent to 70 percent of spinal-cord motor neurons in mice after being injected into a facial vein, and that astrocytes can be targeted after an injection into a tail vein.

Jude Samulski, who has MDA funding at the University of North Carolina at Chapel Hill, said several gene-therapy clinical trials using AAV transporters already are being conducted in neurological diseases other than ALS. The limitations, he said, are “resources, not science.”

Samulski said each AAV type has different tissue targets and that gene therapists can’t assume they’ll all behave the same way in the body.

He noted there are nine basic types of natural AAVs, with new ones being designed in the laboratory. In his MDA-supported Duchenne muscular dystrophy gene-therapy trial, Samulski and colleagues are using a transporter known as AAV2.5. It’s a natural AAV2 viral shell with a laboratory-engineered change in five amino acids.

“The field is moving toward shuffled viruses,” Samulski said, referring to engineered viral shells like AAV2.5. He recommends doing high-through-put screening for AAV types that are good at getting past barriers and into nerve cells.

Echoing the views of several other presenters, Samulski said the immune response question has not been settled yet.

“It’s early in our understanding,” he said, noting that so far they haven’t seen any serious immunologic rejection of the viral shells in the muscular dystrophy gene-therapy trial. However, he noted, “You can’t infer too much from one study.”

In Search of Barrier-Free Living: Making the House an Accessible Home

by Bill Norman

After remodeling (above), a wheelchair ramp ascends to a home’s threshold level.

Remodeling living quarters to make them more accessible is one way to “stay ahead” of ALS.

Modifications can be simple, such as lowering light switches, replacing doorknobs with levers, elevating toilets and installing grab bars; moderate, such as building ramps and widening doorways; and large, such as adding a roll-in shower, ceiling-mounted lift or entire room.

Industry changes for the better

In recent years the construction industry has begun to devote more attention to universal design. Also known as barrier-free or inclusive design, it refers to home specifications that permit easy access and modification for people of all abilities.

The National Association of Home Builders (NAHB), motivated by the fact that most older people prefer to live at home, has developed its CAPS (Certified Aging in Place Specialist) program that accredits remodeling contractors who are proficient in universal design construction.

Finding a remodeling contractor

When looking for a remodeling contractor, ask friends and acquaintances for referrals, advises Greg Miedema, president of Dakota Builders, Inc., in Tucson, Ariz., and vice chairman for the NAHB Remodelers group. Another source of local referrals is www.angieslist.com, a free Internet site where local residents rate service providers on overall experience, price, quality, responsiveness and punctuality.

Other things to consider, says Miedema: Does the contractor belong to a trade organization (as opposed to being just a handyman)? Does he/she belong to a local builders’ group? How long has the contractor been in business? Longtime contractors have access to more resources, says Miedema, adding it’s always wise to check references with the Better Business Bureau.

The National Association of the Remodeling Industry (www.nari.org) offers a guide for hiring professional contractors that includes design tips.

Paying for the modifications

Extensive modifications (above) made this home’s shower fully wheelchair-accessible.

NAHB estimates that 80 percent of home modifications are paid for by the home’s primary occupants from personal savings.

(If financed from personal assets, many home modifications qualify as tax-deductible medical expenses on state and federal income tax returns. See IRS instructions for Form 1040, Schedule A.)

Other funding options, depending on the person’s age and other circumstances, can include reverse mortgages, second mortgages and home equity loans. (See Resources.)

Currently, 33 states offer alternative financing programs (AFPs) that provide low-interest loans for assistive technology such as home modifications. The program offers “flexibility toward funding anything that assists with your disability needs,” reported Robert Wolf of Ambler, Pa., who received an ALS diagnosis in June 2004 and used the AFP program to finance extensive home modifications. “It’s ideal for people who are worried about their day-to-day finances and just need a longer-term picture to be able to get through it.”

Another resource: the Rural Development Home Repair Loan and Grant programs (commonly called Section 504 programs), through the U.S. Department of Agriculture. People over age 62 who live in small towns and don’t exceed specific income levels may qualify for low-cost home modification loans.

U.S. military veterans may get assistance from the Office of Veterans Affairs’ Specially Adapted Housing Grants program.

Many communities qualify for funding from HUD (U.S. Department of Housing and Urban Development) Community Block Development Grants that can be earmarked, at a community’s request, for home modifications. (Contact HUD for specific state information.)

Sometimes, small changes work just fine

Larry and Mary Hill of Hazelwood, Mo., have been able to get by with minimal modifications because Larry’s ALS progression has been relatively slow since his diagnosis in 1994. Larry, 70, uses both manual and power wheelchairs and a communication device.

“It was very scary in the beginning, because we didn’t know how long Larry would be with us. We just bought what we knew we had to have. Now it’s been almost 15 years, and we’re doing fine with just a few modifications,” Mary said.

The couple’s first investment was a front-door wheelchair ramp. Mary learned the hard way that the contractor didn’t know much about building codes. The incline is so steep that pushing Larry uphill in a manual chair is a major chore. Fortunately, he easily makes the ascent in his power chair.

The Hills have an assortment of grab bars throughout the house, which Larry uses for transferring. Besides those near the toilet, grab bars along a bedroom wall permit Larry to hold on and step sideways to the bed. Another bar on the wall above the bed lets him pull himself upright when he’s lying down.

Mary’s back-up plan, when Larry sometimes falls and she can’t lift him by herself, are the town’s fire fighters: “They’re great; I call them and they’re right there to help.”

Moderate modifications

A garage wall door lets Glen Houston (below) travel directly from a van to his home’s interior.

Alan and Theresa Page of Chester, Ill., began making modifications to the ground floor of their trilevel home after Alan, 52, learned he has ALS in November 2007. He’s currently using noninvasive ventilation, a walker and manual wheelchair, but anticipates getting a power wheelchair soon.

The Pages have widened their kitchen and bathroom doorways to 36 inches and installed grab bars in the shower and alongside the toilet. A special modification is a shower curtain rod that bows out, unlike the usual straight rod variety. “We got the idea from a hotel we stayed at,” said Theresa, who is Alan’s full-time caregiver. “It allows me to get in alongside and help Alan in his shower chair, and he doesn’t feel so tightly enclosed.”

The couple also converted most of their flooring surface to linoleum. Theresa said they checked out other more expensive options, but found linoleum ideal — it’s quiet and Alan finds it easier to navigate in both walker and wheelchair. To date, their total remodeling outlay is about $11,000.

The Pages are considering moving Alan’s bedroom into the dining area where he’d still have ready access to his vent, could visit with friends who drop by and wouldn’t “feel so off by himself.”

Other future plans include purchasing an adapted van and installing ramps from the house to the street.

“My advice to anyone who begins remodeling their home is ‘Don’t be afraid to ask,’” Theresa said. “There are no stupid questions in this process.”

Expanding the house itself

When Nancy Byler, 66, learned in 2004 she has the familial form of ALS, she and her husband recently had moved into a large single-level home in Tucson, Ariz.

They were fortunate that several doorways in the home already were wide enough to accommodate a wheelchair, although Nancy, who still is ambulatory, currently gets by with a combination walker/rolling chair.

Their biggest challenge was an undersized bathroom adjacent to the master bedroom. They solved it by having a new 400-square-foot bathroom and roll-in closet added to the bedroom (cost: about $40,000). It features a cut-out vanity, multiple grab bars for the toilet, roll-in shower and a Jacuzzi tub. A portable accordion-type lift chair in the tub lifts Nancy so she can step out.

At the front and back doors, the Bylers had flagstone laid on existing walkways to bring them up to the same height as the threshold.

Nancy said they solicited several bids for the remodeling project and were glad they did. “The first bid was twice as much as what we eventually paid,” she said.

A little bit of everything

Glen and Linda Houston of St. Charles, Mo., specified accessible design for their new home (their “villa”) from the very beginning. Glen, 67, received his ALS diagnosis in 2003, and currently uses a ventilator and power wheelchair.

The Houstons were creative and exhaustive in their selection of accessibility options, including:

• Two ceiling-mount track lift systems, one in the bedroom, and one in the bathroom.
  They obtained one lift system free from a friend who no longer needed it (normally about $3,000). Another friend custom-fit the lift which works in conjunction with padded PVC-pipe arm rails.


• A two-way swinging bathroom door.
  Linda says if Glen falls in the bathroom with the door closed, the dual-swing action allows her to pull it toward her and enter to help him.


• Roll-in shower
  with grab bars and hand-held shower head.


• A garage-to-home entryway.
  The couple built an exterior concrete ramp from the driveway to their front door, but worried it could get slippery in bad weather. So they cut a doorway from the foyer to the garage. Now when Linda drives their adapted van into the garage, the side door opens, the van’s ramp extends out to the side and Glen rolls directly into the house.


• All passageway and closet doors widened to 36 inches.
  Some interior doors are pocket doors that slide inside an adjoining wall, taking up less space.


• Back-up power.
  The Houstons installed a large back-up electric generator that can power not only Glen’s medical equipment but all other electric devices in the house as well during power outages.

“All things considered, we felt that once the ceiling lift was in, we’d dealt with the hardest part of the accessibility issues,” Linda said.

New Adventures with ALS
Expect the unexpected when your regular caregiver isn’t there

by Jeff Lester

Author Jeff Lester’s daughter, Kelsey, is shown here helping out with his trach care. The family discovered communicating this and other vital procedures to the temporary nurses was not an easy task.

When you’ve been dealing with ALS for nearly 15 years, you assume you’ve dealt with everything new but, as with everything associated with this disease, you can be thrown a curve any time.

This particular curve was self inflicted, coming as a result of my nominating my wife, Lisa, for the “CVS Pharmacy/Good Morning America for All the Ways You Care” contest. Lisa was selected as a finalist in the contest, which meant she got to spend a weekend in New York City with our 12-year-old daughter, Kelsey.

National media/public relations firms don’t understand the planning involved in caring for someone in my condition (a quadriplegic on trach ventilation). We were notified that Lisa was a finalist less than a week before the trip, leaving us scrambling to cover the 24/7 nursing care that Lisa does everyday.

Further complicating matters was that the weekend of the trip was the same weekend we’d planned to move to a new town. Most of our stuff was moved or in boxes. And finally, this would be the first time, outside of hospitalizations, I would be alone with just a nurse for more than four hours — over 64 hours in all.

I learned some invaluable lessons. First and foremost, when you’re putting together a set of care instructions, you can’t be too detailed. The smallest oversight can make even the simplest task much more difficult than it normally would — or should — be.

For example, I need to take pills with sweet tea or juice, but definitely not water because of the swallowing issues caused by ALS and thin liquids. Even though this was stated specifically in the instructions, we didn’t define why this is important. Nurses still tried to give me pills with water, putting me in the position of having to purse my lips to avoid taking the pill. This in turn was misinterpreted by nurses who tried to force the pill in my mouth — which leads to the next critical point: communication.

I know some of you are thinking right about now: OK Jeff, there have been countless articles about communication methods and devices. Yes, there have been, so don’t become overconfident like I did and end up wishing you’d printed out a basic letter/yes&no chart (you’ve now been warned!!!).

But the communication I’m talking about here are physical cues. It should be fully explained that people with ALS are cognitively aware and have their mental faculties, so if they do something like purse their lips, they’re not being obstinate, but rather sending a cue that something is wrong. Another physical cue I use is to smack my lips a couple of times to get someone’s attention. But if I continuously smack my lips, then I have a serious breathing issue. It’s important for new caregivers to understand this communication distinction, which can be life threatening if misinterpreted.

Misinterpretation of written instructions is something that surprised me (you wouldn’t think after all these years I would be, but I was). In my case, the situation involved draining water from my ventilator tubing. I use a heated-water ventilator humidifier, which has the drawback of allowing water to build up and slosh around in the vent tubing. The biggest concern when emptying the tubing is keeping water from getting into the lungs, which can cause serious serious problems. This was the viewpoint from which Lisa wrote the vent tube instructions to the nurses.

There was no way either of us could have anticipated that the nurses would misinterpret this to mean they should empty the tubing whenever they saw the slightest amount of humidity. The result of this frequent emptying — at least hourly as opposed to the normal once-or-twice-a-day-as-needed schedule — was that I began drying out, which can cause a whole host of other problems such as thickened secretions, necessitating more suctioning and causing more coughing and discomfort, and in the worst case, causing mucus plugs which can cut off your airway.

The moral here is: Try to read your instructions with an uninitiated eye for items that can be misinterpreted. Better yet, if time permits, have someone not familiar with your routine read and interpret the set of instructions.

The final tidbit that I learned from my weekend was “categorize”! Lisa and I had come up with over four pages of written instructions for my care, but in our haste we failed to recognize a simple fact: It was over FOUR pages of instructions! For expediency’s sake, we should have inserted major headings, in order to eliminate problems like the nurses overlooking the essential information “only give pills with sweet tea or juice.”

I survived my weekend alone to tell this tale, but it would have been much better if we’d known these lessons beforehand. People with ALS and their caregiver(s) have developed a combination of personal shortcuts and language that can’t be easily translated by outsiders. For example, I prefer to sit in my recliner with one foot put about mid-thigh of my other leg. Occasionally I need to switch to the mirror position, which can be accomplished in seconds by either my wife or children when I say the word “switch.” This task at times took 15 to 30 minutes during my weekend with new nurses, and never achieved my normal level of comfort.

The simplest of things can make the difference between comfort and pain when you’re under the care of someone other than a loving and dedicated caregiver like my wife, Lisa, who’s been doing it for nearly 15 years. That’s why I nominated her for the Good Morning America caregiver honor, and how I ended up learning some new ALS life lessons. Hopefully my experience will help you avoid these potential pitfalls in the future. It’s funny how things happen when you’re living with ALS.

Jeff Lester, 41, of Lebanon, Mo., is a graduate student at the University of Michigan-Dearborn. He received an ALS diagnosis in 1993 and has used trach ventilation since 1997. He and Lisa have three daughters born after his ALS diagnosis. To learn more about Jeff visit www.ragingbearscloset.com. To learn more about the caregiver contest, visit www.forallthewaysyoucare.com/finalists/default.asp?id=7&name=lisa_lester

A Complete Communication Solution
It’s not just about text-to-speech devices anymore

by Alyssa Quintero

Tammy Brown uses her AAC device to record special messages for her children. Logan, 10, has helped his mom program messages like “Be nice to your sister,” and “I love you, Logan” directly onto the device.

Alternative augmentative communication (AAC) devices have come a long way since the early days. Now people with ALS can obtain speech-generating devices that provide more than basic text-to-speech output.

All-in-one, or “integrated” communication solutions offer seamless access to e-mail, Internet and other computer applications, as well as speech. They’re available as dynamic display (large touch screen) communication devices, which also serve as fully functioning Windows XP computers.

Users can record directly onto these devices to preserve their speech. The vast majority of integrated systems incorporate “smart” communication software that keeps track of the user’s speech patterns and commonly used words and phrases for greater rate enhancement and productivity. Word prediction and next-word prediction systems also work together to help the user get the message out faster.

Start early

It takes time to select, order, receive and learn how to use these devices.

Typically, it takes a few months to receive a device funded through Medicare and between one to six months with private insurance, says Amy Wright, an SLP at the MDA/ALS Center at Carolinas Medical Center in Charlotte, N.C. Many AAC manufacturers will expedite paperwork for people with ALS because they recognize the disease can change quickly.

Once you receive the device, it will take time to customize it and learn how to use it, especially if you have limited computer experience. Users also may require specialized training from an SLP and/or AAC vendor, advises Winston Cheng, an SLP at the Eleanor and Lou Gehrig MDA/ALS Research Center at Columbia University Medical Center in New York.

Think long term. While portable, hand-held devices are popular and less expensive, Cheng cautions, “They may not continue to be an efficient way to communicate as a person’s physical status changes, and this can be problematic if your insurance won’t provide funding for another AAC device.”

Adds Wright, “We try to anticipate people’s future needs, which is why we always recommend something that has multiple modes of access.”

What’s it cost?

AAC prices range from $2,500 to $8,500 depending upon software selections, other add-on options and choice of access methods (head mouse/tracker, switch scanning, etc.).

Medicare will cover up to 80 percent of the cost for an AAC device, and MDA offers a one-time $2,000 grant for devices prescribed through its clinics. MDA also will provide $500 annually for repairs and modifications.

Medicare won’t fund integrated communication systems, but virtually all such devices are available in cheaper “dedicated” versions that function only as speech-generating devices, not computers. Medicare usually covers dedicated devices, and the computer features can be “unlocked” after purchase, for an additional fee paid by the user.

All-in-one solution at work

Since February, Tammy Brown, a mother of two young children in Thida, Ark., has been using her communication device — the ECO-14 from Prentke Romich Company — on a part-time basis in order to customize it and learn about its features before she needs to rely on it for speech.

“I wanted to have my computer and communication device all in one,” says Brown, 35, via e-mail, noting that she chose the ECO-14 because it can access the Internet just like a laptop. “My husband set it up with a wireless card so I can be anywhere in the house and send e-mails or be on the Internet. It also plays DVDs, which my son thinks is cool, and you can add programs and games.”

Brown, who received an ALS diagnosis in December 2004, paid $150 to unlock her dedicated device, which cost $7,595. Paralyzed from the neck down, she relies on the device’s built-in head-pointing system, TrackerPro from Madentec ($895), which operates via a reflective dot on the corner of her eyeglasses.

“Everyone that sees me use the device wants to know how I control it, and after I show them they’re amazed,” she says.

Brown, who says her speech is getting worse everyday, spends the majority of her time voice banking onto the device, so that when she loses her voice completely, her children still will be able to hear “I love you” in her voice instead of a synthetic one.

“I want to be prepared for the future,” Brown explains. “I have a page set up for each child that’s unique to them. I have buttons set up asking my daughter about her date, which will be a while since she’s 12. My son helps me record a lot of the stuff, and we laugh the whole time because he likes to get a little crazy.”

While the ECO-14 is larger (with a 14.1-inch touch screen) than some devices on the market, Brown is sold on its ability to control appliances like the TV, its built-in DVD/CD drive, and the Internet access that allows her to keep in touch with family and friends. She also helps her children with homework, and helps her husband pay bills and stay on top of the family’s finances via the Internet.

“Since I’m unable to move anything but my head, being able to help out makes me feel like I’m part of the family again,” she says.

Programming the device has been challenging, but Brown’s had plenty of help from her husband and children.

“You have to just practice with it to understand how to get the most out of the device. We have shortcuts set up for me to get to the Internet, e-mail and Microsoft Word documents. Once it’s set up the way you want, you can expand on it from there.”

Try before you buy

What works well for someone else may not be the best for you, advises Cheng.

In addition to working with the SLP at the local MDA clinic, check with the local MDA loan closet to try loaner devices. Most manufacturers have rental or loan programs; in some cases, rental costs can be applied to the purchase price.

The upfront effort is worth it, says Brown. “The independence is the best thing of all.”

Note: ALSN will focus on eye-controlled AAC devices in a future issue.

Tips for Coping with Summer’s Heat

by Kathy Wechsler

Summer typically is the time when people head out and enjoy the great outdoors. But people with ALS have to take a few extra precautions during these hot months, warns Lora L. Clawson, certified registered nurse practitioner and director of ALS clinical services at the MDA/ALS Center at Johns Hopkins University in Baltimore.

• Watch for breathing problems.
  Clawson notes that as respiratory muscles begins to weaken, heat and humidity make it even more difficult to breathe because the air is very humid and dense, sometimes causing a feeling of not being able to breathe. She recommends heeding local weather advisories that warn to remain indoors when the air quality is poor.


• Shun the sun.
  Clawson also urges staying inside during the hottest portion of the day when the sun’s heat and ultra violet rays are most intense — usually between the hours of 10 a.m. to 2 p.m., but in summer this may go even later in the day. If you must be outside in the heat, wear a large floppy, ventilated cotton hat, ventilated long-sleeved cotton shirt and pants, or shorts with a light towel covering the leg skin to protect against sunburn.


• Drink up.
  People with ALS should drink a liter to a liter-and-a-half of fluids on a daily basis, says Clawson, and even more when out in the heat. Water is preferred, but if thin liquids pose difficulty, substitute drinks without much caffeine, sugar and alcohol, which are dehydrating. Fruit nector juices, Jello, custards, puddings, or fruits like oranges and watermelon can provide extra fluids. Additives, such as Thick-It, thicken liquids so they can be more easily managed, without changing taste.


• Take small sips.
  Dumping in a liter of fluids in an hour is a sure way to fill the stomach and the bladder before cells have had time to absorb the fluid. The key is to take small sips of slightly cooled fluids throughout the day to allow the body to absorb the fluids without excreting them.


• What goes in must come out.
  Even with slow sipping, urination needs likely will increase with increased fluid intake. If toileting is difficult or time-consuming due to mobility challenges, drinking the recommended amount of liquid may sound like a nightmare. But it’s not healthy, especially for people with ALS, to decrease fluid intake in order to cut down on trips to the bathroom. To make toileting easier, try a male or female urinal, or check with your doctor about using an external or internal catheter. Another option is easy-to-remove adapted clothing such as Velcro-modified clothing, side zip pants, “backless” pants (available online at Silverts, www.silverts.com), or for women, cotton skirts and dresses worn without underwear.


• Watch for danger signs.
  Darker-colored urine means the body needs more fluids. Dehydration is characterized by increased thirst, dry mouth and a swollen tongue. Other signs include weakness, dizziness, mental confusion, inability to sweat, fainting, chest palpitations and decreased urine output.

The bottom line: stay cool, cover up, drink up, and enjoy the summer!

She Walks Miles, But in One City Block Realizes a Dream

by Amy Labbe

Despite ALS, Lorri Coppola continues to train, walking a mile a day, three days a week, usually at the College of Marin close to her Greenbrae apartment. Photo by Shirley Pierce.

Lorri Coppola learned in 2005 she has bulbar-onset ALS. Although the disease has stripped away her ability to speak and eat, she continues to walk one mile, three days a week, to keep her legs strong.

Coppola, 68, from Greenbrae, Calif., taught physical education and coached middle school track for 33 years. A champion racewalker, she earned gold medals in 10-km and 5-km events in two World Masters racewalking competitions (1995 and 1998). She also has officiated at college track meets for the last 12 years, and, since 1976, has attended three Summer Olympics as a spectator.

This April, however, Coppola realized her own Olympic dream when she carried the Beijing Olympic Torch in the relay run through the protester-filled streets of San Francisco, the only North American stop for the widely recognized Olympic symbol. (The protests were against China’s human rights record, especially in Tibet.)

To be selected as a participant in the run, Coppola wrote an essay describing her love of the Olympics and her history as a World Masters athlete, PE instructor and coach. When she learned she would be a torchbearer, she was thrilled to do it “for the athletes who work so hard to attain that [Olympic] goal, and also to bring attention to ALS.”

Assigned to run in pairs, Coppola found herself teamed up with Olympian Chris Duplanty, who competed for the United States in several Olympics water polo events. After a last-minute route change aimed at avoiding confrontations with protesters, the two carried the torch down a block of Van Ness Avenue.

“I was crying,” Coppola said. “Some of the police thought I was scared — [but] no, I was just overwhelmed.”

Coppola’s three-miles-a-week training served her well, although she said she wanted to jog, “but the media truck in front of us was so slow we ended up walking.”

As someone with a love of athleticism and competition, Coppola found it exciting to be a torchbearer and to meet the many Olympians who participated in the relay with her. As someone with ALS, she recognizes how fortunate she was to achieve her dream.

“I’m proud to have been a part of the relay and to bring attention to ALS,” she said, noting the numerous newspaper, television and radio stories produced about her.

“Life is good — now back to reality.”

TV Personality Honors Late Mother with ALS Popular television host Nancy O’Dell is partnering with MDA’s ALS Division to raise awareness and funds for ALS research, in response to her mother’s battle with the disease. O’Dell’s mother, Betty Humphries, 74, learned she had ALS in November and died in early June. O’Dell is currently in her 12th season with “Access Hollywood” and has been the show’s co-host since 1999. In addition to appearances she will be making on behalf of the MDA ALS Division, she and MDA have established an online donation portal in honor of her mother at https://secure.mdausa.org/nancy/.