New ALS Gene Mapped to Chromosome 16

by Margaret Wahl

Robert Brown

MDA-supported investigators Robert Brown and Diane McKenna-Yasek at Massachusetts General Hospital in Boston, and Jonathan Haines at Vanderbilt University Medical Center in Nashville, Tenn., were part of a group that identified a small region of chromosome 16 that’s clearly associated with the development of ALS and a region of chromosome 20 that may also harbor an ALS-causing genetic change (mutation).

The group analyzed 16 families with inherited ALS in the Boston area and one in the Chicago area. Their findings appeared, along with similar findings by two non-U.S. groups, in the August issue of the American Journal of Human Genetics.

Second Genetic Clue

Until a decade ago, no genes for ALS had been identified, although it was well known that the paralyzing disease ran in some families.

Then, in 1993, MDA-supported researchers identified a gene on chromosome 21 that, when flawed, causes some 2 percent of all cases of ALS and 20 percent of clearly inherited cases. The gene flaw is inherited in a dominant pattern, meaning that a flawed gene from one parent is enough to cause the disorder in a son or daughter.

The identification of this chromosome 21 gene, known as SOD1, allowed scientists to develop theories of ALS causation and to study disease development and potential treatments in mice bred with SOD1 gene flaws.

Gene Discovery Expected

The new finding is expected to lead soon to identification of a specific gene on chromosome 16. That discovery should also allow new discoveries about how ALS occurs. The chromosome 16 form of ALS is also dominantly inherited.

Christopher Shaw of Guy’s, King’s and St. Thomas’ School of Medicine in London headed a second group, which found the same link between ALS and the chromosome 16 region in two large, unrelated European families.

Jacqueline de Belleroche of the Imperial College in London headed a third team, which linked the same region to ALS in a three-generation family in the United Kingdom.

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A NEW LIFE WITH ALS

Speeding Up the Scientists

by Steven and Jennifer Bishop

With the Jerry Lewis MDA Telethon successfully completed for 2003, we thought it would be a good time to review an event we attended this summer. It was a fantastic example of how the funds MDA raises during the Telethon and throughout the year are used to benefit people with ALS.

In June we attended MDA’s conference, ALS Clinical Trials: The Challenge of the Next Century, in Tarrytown, N.Y. We were thrilled to have this opportunity to speak to more than 150 experts in clinical studies and other avenues of ALS research. (See "Drugs, Stem Cells, Trial Designs," July 2003, for a complete report on the conference.)

When we told our MDA ALS support group that we were going to speak to researchers, one member, then in the late stages of ALS, typed out a message for us to share. This spirited and positive woman wrote:

"Speed ‘em up."

That was the theme of the conference and the message we shared — the urgency of getting research findings into clinical trials and treatments as quickly as possible — because friends and loved ones are losing the battle.

Intense Conversation

At the first evening’s dinner, we sat with Stanley Appel, director of the Ronny & Linda Finger MDA/ALS Center at Baylor College of Medicine in Houston, and his wife, Joan; Jeffrey Rothstein, director of the MDA/ALS Center at Johns Hopkins University in Baltimore; Rothstein’s research fellow, several other doctors and a bio-statistician. We were thrilled to hear the intense conversation buzzing around the table as these experts discussed theories and results.

These scientists’ dedication to ALS research was unquestionable, and the evening provided us with a renewed outlook on their ongoing efforts. We were both deeply moved, and Jennifer tearfully thanked them all for their support.

We attended the next morning’s session, chaired by conference co-director Hiroshi Mitsumoto, who had invited us to New York. He’s co-director of the Eleanor and Lou Gehrig MDA/ALS Center at Columbia University in New York.

We were very impressed with the detail in each presentation as well as the wide range of subjects covered. It was evident to us that the overall goal of the event would be achieved, i.e., to advance the methods and procedures of ALS clinical studies and to minimize or eliminate the need to reinvent the wheel.

The afternoon session on lessons from studies in other diseases and clinical trials was testimony to the progress MDA scientists want to make in ALS. From our conversations and observations, we came away believing that the conference had a profound impact on the participants. Several scientists commented on the need for collaborative efforts among institutions and suggested our time at the podium could encourage this theme. They felt that adding a human element to the scientific process would be of great value.

Personal Message

The evening dinner celebrated Lou Gehrig’s 100th birthday (June 19). After an overview of Gehrig’s life and presentations by several researchers, we were introduced by Victor Wright, a member of MDA’s Board of Directors.

We told our story and pleaded for all parties to work together and share their findings, failures and successes. We told them about the message from our friend in Denver, and emphasized that we have friends dying from this disease.

We were honored to be invited to the conference and to share our thoughts of appreciation, encouragement and urgency with this influential group. We commend Dr. Mitsumoto and his co-chairman, Serge Przedborski, as well as the other organizers, presenters and participants for the time and energy they expended on this event.

On June 17, two days after we returned home from New York, our friend who had urged researchers to speed up lost her battle with ALS. Her loss was another sad reminder of how important it is to get the word out about ALS and how many people it truly affects.

Thank you to MDA for sponsoring the conference and for trusting us to add meaningful insight. It’s events like this that continue to provide us with hope that there will be a treatment or cure for ALS — maybe in time for Steven, and certainly for others who’ll be affected by this revolting disease in the near future.

Steven and Jennifer Bishop are co-chairpersons of the ALS Division of MDA. Steven received an ALS diagnosis in March 2001. The Bishops live in Arvada, Colo., with their 5-year-old son, Christopher. The family was featured on the cover of Parade on Aug. 31 and on the Telethon broadcast.

Join the Bishops every other Wednesday for the PALS With Children chat at www.mda.org.

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Wings Over Wall Street Gets Ready to Soar

Wings Over Wall Street, an annual gala that’s raised over $2.6 million for MDA’s ALS research program in the past two years, will be held Oct. 2 at the New York Marriott Marquis at Times Square.

For the second year, actor William Baldwin will host the cocktail reception and auction, which benefits the research teams of Hiroshi Mitsumoto of the Eleanor and Lou Gehrig MDA/ ALS Center at Columbia University in New York, and Jeffrey Rothstein, director of the MDA/ALS Center at Johns Hopkins University in Baltimore.

Three awards will be given at the gala. The Diamond Award, acknowledging scientific contributions to ALS research, will be given to Thomas M. Jessell, professor of biochemistry and molecular biophysics at Columbia University, who is studying stem cells in treatment of ALS. Dr. and Mrs. Eliot Danziger of Brookdale, N.Y., will receive the Spirit Award for their commitment to funding a search for a cure. The event’s newest award, the Michael P. Beier Award, honors those who go above and beyond, and will be given to Wall Street trader Cathy Wilson Rosen of Zweig-DiMenna Associates.

Wings Over Wall Street was started in 2001 by Toni Diamond, a former United Airlines flight attendant with ALS, and her husband, Warren Schiffer. It got a big boost last year when it was chaired by Michael Beier, director of equity trading at Credit Suisse First Boston and an MDA vice president.

For information on making a donation, becoming a sponsor, buying tickets or participating in the auction, go to the MDA’s ALS Division Web site at http://www.als-mda.org and click on the Wings Over Wall Street icon, or call (212) 689-9040.

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ALS Research Roundup

Lab Mice Benefit From IGF-1 Gene Transfer

Jeffrey Rothstein, a physician-investigator who directs the MDA/ALS Center at Johns Hopkins University in Baltimore, was part of a research team that recently found that transferring the gene for IGF-1 into mice bred to develop ALS had considerable benefit.

IGF-1, or insulin-like growth factor 1, is a natural protein found in animals and humans that may have protective effects in the nervous system.

It’s been formulated as an experimental drug (Myotrophin) and has been tested twice in clinical trials. One large-scale trial found it had some benefit, while the other found it had no benefit. It’s now being tested again in people with ALS.

But the experiments conducted by Rothstein and colleagues have taken a new approach. Instead of delivering the IGF-1 protein, they’ve delivered the gene for IGF-1. And instead of delivering the compound in subcutaneous (under the skin) injections, they inserted it into an adeno-associated virus and then injected it directly into muscle cells in the back legs and the rib muscles of the mice.

The mice were expected to develop ALS at about 90 days of age because they carried an ALS-causing SOD1 mutation. But those given injections of IGF-1 genes at 60 days (prior to symptom onset) didn’t develop the disease until, on average, 31 days later than expected.

And when they did develop symptoms, they survived an average of 37 days longer.

Because few ALS patients ever see a doctor before developing symptoms, the scientists also had to find out what would happen if the mice were given the IGF-1 gene injections after symptoms developed.

Newly symptomatic mice that were 90 days old when they received IGF-1 gene injections lived, on average, 22 days longer than untreated mice with the same SOD1 gene mutations.

Their strength and function also lasted considerably longer, and they had about as many motor neurons remaining in their spinal cords at 110 days of age (20 days after their injections) as did healthy mice, though numbers declined sharply as their ALS progressed.

The researchers believe the IGF-1 genes were probably transported from the muscle cells, where they were injected, up nerve fibers into the nerve cell bodies in the spinal cord, where the cells followed the genetic instructions and made IGF-1 protein molecules.

The IGF-1 protein appears to have protected the motor neurons in a number of ways, the researchers say. Interfering with the cell death program (apoptosis), delaying the process of aggregate (clump) formation, and dampening an ALS-associated immune response in the nervous system all seem to have been involved.

According to Rothstein, scientists from Hopkins and from the Salk Institute in La Jolla, Calif., are now in discussions with regulatory agencies and pharmaceutical companies so that IGF-1 genetic material can be delivered as a drug and mandatory safety studies outlined by the Food and Drug Administration can be conducted.

"This process should take about a year," Rothstein said, "at which point, if all goes as planned, the first clinical trial of this treatment could begin."

Human Umbilical Cord Blood Cells Help Mice With ALS

A study in the June issue of the Journal of Hematotherapy & Stem Cell Research has found that mice with ALS that received transfusions of human cells isolated from umbilical cord blood showed delayed progression of their disease and extended survival time.

Investigators Paul Sanberg and Svitlana Garbuzova-Davis at the Center for Aging and Brain Repair at the University of South Florida in Tampa led a multidisciplinary team which found that mice given the human cells remained mobile until 19 weeks of age, in contrast to untreated mice, which typically lost mobility rapidly beginning at about 13 weeks.

In addition, the mice that received the cord blood cells survived an average of 17 weeks, while the untreated mice lived an average of 14 weeks.

Cells taken from umbilical cord blood are considered a type of stem cell, with the ability to mature into a variety of human cell types.

The investigators in this study aren’t sure of the mechanisms by which these cord blood cells helped the ALS-affected mice. Among the possibilities are that the stem cells became motor neurons, the muscle-controlling nerve cells that are lost in ALS; that the stem cells became supportive cells that helped prolong the life of the motor neurons; or that the transfused cells changed the behavior of the immune system in such a way that disease progression was slowed.

They suspect the second two mechanisms were larger contributors than the first, since relatively few cells appear to have developed into motor neurons.

MDA is working with Sanberg’s group and others to plan future studies of umbilical cord blood cell transfusion in ALS patients.

Minority of Long-Term ALS Survivors Lose Communication Ability

"The expectation that all patients with ALS using TPPV [tracheostomy positive-pressure ventilation] will ultimately develop the locked-in state [total inability to communicate] is not supported," say physicians Hideaki Hayashi of the Tokyo Metropolitan Neurological Hospital and Edward Oppenheimer of the University of California at Los Angeles School of Medicine in their recent study.

In a report published in the July 8 issue of Neurology, the doctors analyzed the communication abilities of 70 people with ALS receiving tracheostomy ventilation between 1980 and 1999 in Japan. They found that only eight of them (11 percent) lost all ability to communicate and developed a "totally locked-in" state, in which they can’t communicate either by speaking, by movement or with assistive devices. Six of those eight were among those who survived more than five years on ventilation.

The remaining 62 people (89 percent of the original 70) didn’t lose all ability to communicate, though many had impaired communication.

The authors recommend that people with ALS and their physicians consider long-term assisted ventilation without worrying that it will necessarily lead to a prolonged life without social contact. They also recommend that people considering TPPV decide in advance under what circumstances they want to discontinue ventilation (advance directives) and that they investigate assistive communication technology in advance.

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Bertrand Fights ALS by Being Himself

by Kathy Wechsler

William Bertrand Jr. sports a winning attitude in the face of his deteriorating limb function and loss of mobility caused by ALS.

THE BEST MEDICINE

"Laughter is the only drug that works on ALS, and it’s free. What a bargain," said Bertrand, a West Warwick, R.I. resident, who uses humor to overcome his many challenges.

Yiota and Bill Bertrand

Bertrand, 59, and his wife, Yiota, whom he met and married in Athens, Greece, where he was stationed with the U.S. Air Force, often get together with other people with ALS, where his bright outlook and sense of humor are greatly appreciated.

"I try to bring a little humor to anyone who might be a little depressed," he said. "Depression is a no-no with this disease."

Bertrand, who was found to have primary lateral sclerosis in January 1995 and was told in 2000 that his disease had progressed into ALS, has only partial use of his arms and hands and is unable to use his legs. He has trouble swallowing and can’t speak or chew food.

"Other than that, I feel great," joked Bertrand, who communicates with a LightWRITER device and through e-mail.

FAMILY AND FRIENDS

"I think my disease has affected my wife more than it has affected me," Bertrand said. "It is her that has to care for all my needs and all the work that I used to do." Bertrand and Yiota have two grown daughters, both graduates of Rhode Island College, who travel from other states to assist their father on the weekends.

Bertrand has many fond memories of the Providence, R.I., Post Office, where he worked in accounting for 12 years. Before retiring in 1999 because of the effects of his disease, he joked around with his co-workers to keep up his spirits.

"They would take off one of my wheelchair wheels if I said something bad about their beloved Boston Red Sox, and leave me immobile," Bertrand said. "They made me laugh so much, and I was sorry to have to leave them."

Before ALS, Bertrand enjoyed bowling, tennis, running and photography. Though devastated that he isn’t able to hold a camera anymore, he’s found other activities in which he can take part.

Besides using his keen sense of humor to make people laugh, Bertrand loves to read and watch sports on television. Because he fatigues easily, his "outdoor activities are limited to an occasional movie and a trip to the bookstore."

After retiring, Bertrand began writing humorous poetry as a way to occupy his time. See his "ALS Is for the Birds" below.

MDA MAKES LIFE A LITTLE EASIER

"MDA has helped us greatly with expensive equipment," Bertrand said. MDA helped to pay for his wheelchair and LightWRITER. MDA also pays for wheelchair repairs.

Bertrand uses a lift to transfer from chair to bed. "It was donated to us by a very dear friend of ours whose husband passed away from ALS last year," he said. "My wife will pass it on also."

Excited to be interviewed for a piece on the local broadcast of this year’s MDA Telethon, Bertrand exclaimed, "My wife and I have always loved Jerry Lewis, and I probably owe my positive attitude and sense of humor to Jerry Lewis."

Bertrand has handed out ALS awareness information for MDA and wants to do anything to help the Association because of all it has done for his family.

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ALS IS FOR THE BIRDS

by William Bertrand Jr.

I remember the days when I was able to pump my arms and my legs, as I cut through the wind, running road races. I felt like a (Beep! Beep!) "ROADRUNNER." But that was before ALS began "ROBIN" me of the use of my limbs and now has made it difficult for me to chew and "SWALLOW" my food.

When I found out that I had ALS, it would have been easy for me to say, Well, my "GOOSE" is cooked. I could have walked around with my head down, as if I had an "ALBATROSS" tied around my neck. I could have gone stark "RAVEN" mad.

However, that is not my style. I do not walk away from a fight and have no plans to "DUCK" this one either. After all, I am still happy as a "LARK," as mischievous as Heckel and Jeckel, those forever-in-trouble "MAGPIES," and as feisty as a "ROOSTER" in a "HEN" house.

A wise old "OWL" once said, It’s not about the amount of time you spend living, it’s about what you do and accomplish with that time.

I can still enjoy nature in all its splendor. Watch the "CARDINALS," "GOLDFINCHES" and "CHICKADEES" as they come to my backyard feeder. Yiota and I will remain "BLUEBIRDS" of happiness till the day that we die.

Who knows, in the afterlife, maybe I will regain the use of my arms and legs, feel the wind in my face, and, once again, feel like a (BEEP! BEEP!) "ROADRUNNER."

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Regaining the Simple Pleasure of Regularity

by Christina Medvescek

It’s a subject that lends itself easily to jokes, but when it happens to you, it’s no laughing matter. Constipation is a real pain in the you-know-what.

Constipation is defined not by how frequently you move your bowels, but by whether the stools are hard, dry and difficult to pass. It’s not necessary to have a daily bowel movement. If you’re only going every couple of days, but can complete the task without straining, then you’re not constipated.

Does ALS Cause Constipation?

Diane Huberty

Although not a symptom of the disease, constipation is a common side effect of ALS for several reasons, says Diane Huberty of Fort Wayne, Ind., a retired neuroscience nurse who has ALS.

• Decreased movement: Inactivity slows down peristalsis — the smooth muscle contractions that move waste through the intestines. "Anything that changes the speed with which foods move through the large intestine causes problems," Huberty says. The longer waste sits, the more water it loses to body absorption and the harder it becomes. This problem is further aggravated if you don’t go when you feel the urge, perhaps because you’re waiting for assistance or to use your home toilet.

• Swallowing problems: To prevent choking, many people don’t eat nutritiously or drink enough liquids, both of which make stools harder. People with ALS sometimes drink less to minimize their bathroom visits as well.

• Muscle weakness: As respiratory, chest and stomach muscles weaken, it becomes difficult to "take a deep breath and bear down, something we don’t even realize is important in having a bowel movement until we cannot do it," Huberty says.

Diet, routine, position and laxatives are the best ways to keep things moving
smoothly.

Diet Is the First Defense

Fiber and fluids are the key ingredients in an anti-constipation diet, but they must be taken together.

Fiber is simply indigestible bulk that absorbs water in the colon (helping waste product stay softer) and fattens up the stool (helping stimulate involuntary muscles to move it along). Obviously, fiber can’t do its job if there isn’t enough fluid present; in fact, it can be counterproductive.

Gentle dietary fiber can be found in raw fruits and vegetable like apples, strawberries, lettuce and celery. Because this type of fiber is at least partially digestible, it has a mild effect on peristalsis. The fiber found in bran, seeds or high-fiber cereal or bars is completely indigestible, and so acts more vigorously. Without adequate fluid, this type of fiber can become cementlike and cause additional constipation. When muscle weakness is severe, this type also can make the stools too bulky to pass.

If swallowing problems make it hard to drink enough fluid, consider thickening your drinks into slushes or milkshakes, or using a no-taste thickener such as Thick-It. Many natural sources of fiber, such as fresh fruits, already contain fluid, so you get both at once.

The same dietary principles — fiber and fluids — also apply in tube feeding. Check with your doctor about using a high-fiber formula.

A Good Routine, A Good Position

Greg Carter

The body is a creature of habit, so if you can get used to going at a certain time, you increase your chances of regular success.

Greg Carter, co-director of the MDA/ALS Center at the University of Washington Medical Center in Seattle, suggests these steps for creating a reliable bowel routine:

1. Pick a time of day and stick with it. Peristalsis is naturally stimulated about 20 to 45 minutes after a light meal, and is usually strongest after breakfast (although lunch and dinner work as well). Choose a time when you’ll have at least 15 uninterrupted minutes on the commode.

2. Drink a hot beverage, preferably with caffeine, to stimulate your bowels.

3. Get on the toilet and insert a Dulcolax suppository, then lightly circle the skin around the anus (on the outside) to trigger the colon to move.

In chronic constipation, the colon may have become distended and lost muscle tone. It can take up to six weeks to establish a normal bowel routine in this case, so be patient.

Position on the toilet is also important. Sitting upright, especially with your feet off the floor, does little to help with elimination. Squatting is better. This position opens up the muscles of the pelvic area, angles the bottom for the best mechanical advantage, braces abdominal muscles and pushes the thighs into the abdomen to provide extra force.

A squatting position, with the body leaning forward and feet resting off the ground, is best for moving the bowels.

To achieve a reasonable and safe squat on the toilet, rest your feet on a footrest that’s about a foot off the ground, such as a stool or walker rung, then lean forward. For additional force, hug a pillow to your abdomen. If you use a hygiene or toileting sling that holds you over the toilet, ensure that your buttocks are the lowest part of your body. This position usually results in a bowel movement in about one-tenth the time required when lying down and using a bedpan.

Lowdown on Laxatives

Basically, there are three kinds of laxatives:

• Bulk or fiber laxatives, such as Metamucil or Citrucel, are essentially a concentrated form of dietary fiber.

• Stool softeners, like Colace, keep water content high or lubricate the stools.

• Stimulants, such as Senokot, increase involuntary muscle contractions, moving the stool along quickly.

People with ALS should exercise caution with bulk or fiber laxatives, especially if swallowing is difficult. Without adequate fluid intake, fiber laxatives can swell up in the throat, esophagus or intestine, creating a dangerous blockage. They also can gum up a feeding tube unless adequately flushed with water. Follow package directions carefully.

Carter recommends a daily capful of MiraLax (available by prescription) in eight ounces of water. This strong "osmotic" laxative pulls water into the intestines and helps soften stool.

Tahseen Mozaffar, co-director of the MDA/ALS Center at the University of California at Irvine, says that when adding fiber doesn’t do the trick, he may prescribe Mestinon, a drug used to relieve muscle fatigue in ALS patients. A common side effect of Mestinon is increased bowel movements, "which works fine with people who are constipated," he says.

Stimulant laxatives, while working quickly, can result in dependence if overused — in other words, you can’t go without them. But if nothing else will keep you regular, this may be your best option, so consult your doctor.

Carter says he’s heard positive reports about an herbal stimulant laxative tea called Smooth Move (made by Traditional Medicinals and available in health food stores or online). Patients report using it on a weekly or monthly basis with good results.

Mineral oil and enemas should be used only occasionally. Mineral oil can interfere with the absorption of some vitamins, and enemas distend the bowel, which can eventually cause loss of bowel tone.

One final tip: "Comfort is important in order to concentrate on the job at hand," says Huberty, noting that shrunken buttocks muscles can get sore on a hard seat. Her padded toilet seat has been "a real lifesaver!"

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Photographer’s Work Featured in Quest

Angelo Sciulli and physical therapist Trish Koplas of the MDA/ALS Center at Carolinas Medical Center in Charlotte, N.C.

Be sure to check out the September-October issue of MDA’s magazine, Quest. The cover and a feature story present photographs taken by Angelo Sciulli of Lancaster, S.C., a nature and wildlife photographer who’s affected by ALS.

Sciulli’s article, "Seeing Is Believing," recounts his trip last year to the Galapagos Islands and contains several photographs of animals on the islands.

Sciulli’s photographs can be seen at the MDA/ALS Center at Carolinas Medical Center in Charlotte, N.C., and at www.scnature.com.

His photos from Kenya, British Columbia, Alaska and other locations have been widely published in prestigious magazines. Sciulli now publishes and exhibits his photographs only to raise awareness of ALS and disabilities.

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Social Security Changes Rules to Benefit Applicants With ALS

by Christina Medvescek

John Hunter, speaking at an MDA meeting (top), helped pass a Social Security rule change that grants automatic disability benefits to unemployed people with ALS. Hunter is shown below with former Cleveland Indians pitcher and Hall of Famer Bob Feller, at an event to raise ALS awareness.

It’s now much easier for a person with ALS to get Social Security Disability Insurance (SSDI) benefits, thanks in part to a push by an Ohio man with ALS, John Hunter.

Social Security Administration (SSA) Commissioner Jo Anne Barnhart recently changed SSDI eligibility regulations in order to automatically grant disability status to anyone who isn’t working and has received an ALS diagnosis. Although applicants still must wait five months before receiving their first checks, this new "presumptive eligibility" ruling guarantees they won’t be turned down for benefits.

The Office of Management and Budget must approve the rule before it takes effect; approval is expected by late October, said Mark Hinkle, SSA spokesperson.

"Before we made this rule change, we were approving more than 90 percent of applicants with ALS at the initial level, but this will close the gap for the rest," Hinkle said.

Claimants with ALS sometimes were initially denied because they weren’t "disabled enough yet" to qualify. That’s what happened to John Hunter, 40, of Litchfield, Ohio.

Although his rapidly progressing ALS had disabled his arms and hands, he was turned down for SSDI because he was still walking at the time of his application in 2002. A scathing local TV news story about Hunter’s situation resulted in an abrupt reversal and approval by the SSA office.

Angered that SSA had been playing with time he didn’t have to waste, Hunter teamed up with local TV reporter Duane Pohlman and traveled to Washington to advocate for a change in the eligibility process for all people with ALS. Hunter and his wife, Jonna, spoke to members of the Ohio congressional delegation as well as SSA Deputy Commissioner Martin Gerry.

"We’re very excited to hear that this change has happened," said Hunter, who is recovering from a broken shoulder caused by a fall. "It’s an overwhelming feeling."

Hinkle said the rule change follows up on legislation passed in 2001 eliminating the Medicare waiting period for SSDI recipients with ALS. Although the new rule is unofficially being called the "John Hunter Rule," Hinkle noted that SSA doesn’t "name our regulations.

"But his situation certainly illustrated that we could do more to help people, and that’s why we did it."

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Medicare Covers Cough Device

In July, the J.H. Emerson Co., makers of CoughAssist, announced Medicare’s adoption of a national coverage policy for this device, which helps people with failing respiratory muscles to clear secretions. This new policy applies to people with an ineffective cough due to ALS and other neuromuscular disorders involving weakness of the respiratory muscles.

Using HCPCS code E0482, "cough-simulating device, alternate positive and negative pressure," will make it easier to obtain Medicare reimbursement for the CoughAssist. For further details, contact J.H. Emerson at (800) 252-1414, or visit www.jhemerson.com.

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