Researchers Probe Protective Strategies

The idea that ALS may result from not just one, but a combination of factors, such as genetic variations and environmental exposures, is not new. But scientists now are beginning to home in on a number of specific protective factors, some of which have the potential to be exploited for therapeutic development.

Scientists study ‘protein stability’ factors

Several genetic mutations have been identified as causative for ALS, and several more have been implicated as risk factors for the disease (see ALSN Research Roundup, April 2009). In addition, some environmental exposures, such as the Gulf War of 1990-1991, are suspected of having combined with genetic predispositions to cause ALS.

And an increasing body of knowledge suggests that a network of biochemical interactions that maintain what scientists call “proteostasis,” or protein stability, may play a large role in protecting us from certain diseases, including ALS, and perhaps from the ravages of aging in general.

Proteostasis refers to controlling the concentration, shape and location of the body’s thousands of cellular proteins, as well as determining the proteins and other compounds with which a given protein interacts. This network kicks in when conditions inside or outside cells worsen, but its resources are limited.

Protein misfolding and clumping (aggregation), indicating a relative failure of proteostasis, have been seen in ALS-affected nerve cells, especially when the disease is caused by mutations in the SOD1 gene, as in one form of familial ALS.

Now, scientists at Northwestern University in Evanston, Ill., coordinated by Richard Morimoto, have conducted a series of experiments showing that seemingly minor variations in proteostasis can markedly affect the health of cells harboring a known ALS-causing mutation.

When these scientists introduced genes containing three distinct SOD1 mutations, all of which can cause ALS, into worms with normal proteostasis, they saw only mild cellular toxicity.

However, when genes with SOD1 mutations were expressed in worms in which proteostasis was mildly abnormal, their toxicity was markedly enhanced.

Heather Durham

Regulators of proteostasis, the researchers say, could be developed as therapeutic agents. To support this idea, they caused a proteostasis regulator called HSF1 to be overexpressed in cells with abnormally folded SOD1 protein (from an SOD1 gene mutation) and found the cells did not develop the hallmarks of ALS that would have been expected.

Heather Durham, who has MDA support for ALS research at the Montreal Neurological Institute of McGill University, is studying whether enhancing pathways involved in maintaining proteostasis can prevent various abnormalities in ALS-affected cells.

’Alternative splicing’ lowered risk of ALS in a Canadian family

Researchers in the United States, Canada and the United Kingdom have identified a multigenerational Canadian family of Filipino descent in which a known cause of familial ALS — a mutation in the SOD1 gene — does not always cause the disease.

MDA-supported Janice Robertson at the University of Toronto, and colleagues, published their findings in the March 31, 2009, issue of Neurology.

The investigators analyzed DNA samples from 12 members of this family of 20, three of whom had died of ALS. One of those three had inherited a unique mutation in the gene for SOD1 from both parents; his DNA was included in the samples that were studied. DNA was unavailable for the two others who had died of the disease.

In the additional 11 for whom DNA was available, there were no cases of ALS. However, eight showed a single copy of the SOD1 mutation, which normally is enough to cause the disease. (All 11 were older than 48 years, including four who were older than the expected age of onset of ALS.)

This particular SOD1 gene mutation, the authors determined, is in a section of the SOD1 gene called exon 2, and it prompts an effect called “alternative splicing,” which allows cells some leeway in interpreting genetic instructions. The result in this case is reduced expression of the mutant SOD1 protein, which in turn results in fewer cases of the disease.

Therapies based on the promotion of alternative splicing may provide yet another way to reduce levels of abnormal and toxic SOD1 protein and perhaps other toxic proteins, and protect ALS-affected cells.

Help Needed with Survey on Sexuality in ALS

Investigators at San Francisco State University have developed an online survey to learn more about sexuality in people with ALS and their partners. You do not have to currently be with a partner to participate in this anonymous, Web-based study.

Those with ALS who want to participate must be 18 to 80 years old and have a probable or definite ALS diagnosis. Partners also must be 18 to 80, have been in a relationship with someone with ALS since before symptoms began or diagnosis was received, and must be providing at least an hour a week of help or care for the person with ALS.

Go to the survey at www.ALSsurvey.org, or contact Carisa Bielecki at bielecki@sfsu.edu or Ryan Howell at rhowell@sfsu.edu for more information.

Latest Edition of ALS Guide Released

Amyotrophic Lateral Sclerosis: A Guide for Patients and Families, edited by Hiroshi Mitsumoto. 3rd ed., Demos Health (www.demoshealth.com or [800] 532-8663), 2009. $39.95.

This comprehensive volume, now in its third revision, contains everything you could possibly want to know about ALS. In its 455 pages, plus a user-friendly index, you’ll find new information on treating ALS symptoms and on noninvasive ventilation, American Academy of Neurology care guidelines, and the use of riluzole (Rilutek).

There are chapters on managing nutrition, communication, hospice care, financial realities and more. The book is edited by Hiroshi Mitsumoto, an MDA research grantee and director of the Eleanor and Lou Gehrig MDA/ALS Center at Columbia University in New York, with chapters written by experts in each area.

by Margaret Wahl and Amy Labbe

God grant me the serenity to accept the things I cannot change; courage to change the things I can; and wisdom to know the difference. — from a prayer by Reinhold Niebuhr

There are few things that are certain in ALS, including what to do to ensure (or avoid) longer survival time. There are several factors influencing longevity with this disease over which one has no control, but there are a few things one can do to survive longer.

Changing the things one can

There are generally three things you can do to prolong survival that have the support of experts in the field: maintain nutritional status, including getting a gastrostomy tube (which goes straight into the stomach, not down the throat) when and if necessary; use assisted ventilation as respiratory status deteriorates; and take riluzole unless side effects or other medical conditions preclude doing so. People with ALS generally agree on these interventions as well, often finding them less onerous than they had anticipated.

Get enough to eat and drink

Malnutrition, present in 16 percent to 50 percent of people with ALS, is a predictor for worsened survival, according to a study published in 2000. The main cause of malnutrition in ALS is swallowing dysfunction, resulting from paralysis of muscles involved in swallowing, but weakness of the muscles involved in chewing food also contributes, as does a higher than normal metabolic rate of uncertain origin in this disease. A vicious cycle can ensue, in which poor nutrition exacerbates weakness, and weakness in turn limits food and liquid intake.

Signs of insufficient intake of calories may be obvious, such as excessive and rapid weight loss. But for those who like an objective measurement, a body mass index (BMI) of less than 18.5 is considered a red flag. To calculate your BMI, go to www.nhlbisupport.com/bmi.

Most people find a gastrostomy tube is much more comfortable and easy to use than they thought it would be.

While you can still swallow and chew safely, take in foods and liquids that are dense in calories, such as high-calorie shakes and bars, and high-protein foods like meat, fish and eggs. Drink plenty of fluids to avoid dehydration.

When eating and drinking are no longer safe because of fear of choking, or when eating a meal takes so long you become exhausted, it’s time to consider a gastrostomy, or PEG, tube. PEG stands for percutaneous endoscopic gastrostomy tube, which relates to how the tube is inserted. The end result of PEG tube insertion is a small tube that goes directly into the stomach from outside, not something that goes through the nose or down the throat.

Most people find it’s much more comfortable and easy to use than they thought it would be.

A very active online forum at www.als.net/forum is operated by the ALS Therapy Development Institute (an ALS research institute in Cambridge, Mass., supported by MDA) and provides a variety of opinions and experiences with the PEG tube and other aspects of ALS therapy.

From a recent post: “Best thing I could have done! No more choking, no more dehydration, no more weight loss. ... When I got mineI was still walking and drinking normally but having trouble with food, and I’d lost 35 kilograms (77 pounds). It would be fair to say I was in denial, and it took a while for me to adjust to the idea of having limited food intake. Now I can’t believe I was so silly.”

Breathe well

As the muscles involved in respiration — the diaphragm and the intercostals, which are between the ribs — weaken, it becomes harder and harder to move enough air in and out of the lungs. This is especially true when lying on one’s back, as the weight of the abdominal organs creates even more resistance for the weakened diaphragm.

In recent years, a large percentage of ALS patients have opted for “noninvasive ventilation,” or NIV, to help them with their breathing. NIV delivers air under pressure (with a normal percentage of oxygen, not an oxygen supplement) to the lungs via a face mask or mouthpiece.

A 2007 study showed starting NIV earlier prolongs survival better than starting it later, when respiratory function is worse. This study used 65 percent of normal forced vital capacity (the amount of air one can exhale after taking in a deep breath) as the dividing line between early and late intervention. People who started using NIV when their forced vital capacity was at least 65 percent survived an average of 329 days longer than those who started it when their FVC was lower than 65 percent. NIV can be used for as many hours of the day or night as the person wishes and can easily be taken on and off.

Noninvasive ventilation can prolong life despite a decline in respiratory muscle function.

Although NIV may prolong life by months or even sometimes years, for indefinite survival with ALS, “invasive ventilation,” which means ventilation through a tracheostomy tube, is almost always required. A tracheostomy is a surgically created opening in the trachea. Not only the delivery route but the type of ventilation device used with it provides for more complete substitution for normal respiratory muscle function than does NIV.

Surprisingly few studies have followed the course of people with ALS who have opted for invasive ventilation. In fact, many studies use “death or tracheostomy” as an end point, implying that these are approximately equal. That, however, is certainly not the case, so it may be that following people with tracheostomy ventilation until death would take so long the study could not be wrapped up in a timely fashion.

A small study published in 2003 in the journal Neurology found that, out of 70 people with ALS in Tokyo who opted for tracheostomy ventilation, 33 survived more than five years on it. In that group were three people who lived for 10 years on tracheostomy ventilation and two who survived for 18 years. The most common cause of death was pneumonia, followed by cardiovascular disease.

The down sides of tracheostomy ventilation are that it requires respiratory care that poses a significant burden on family members or costs a great deal if professionals are hired to help, and that it may allow for prolonged survival even when all voluntary muscle movement has been entirely lost and even communication is difficult. Still, many people are happy with this option.

“My wife decided to follow the doctor’s suggestion and get her trach early,” noted an ALS TDI Forum visitor in March. “It’s been over two months now, and it has made a huge difference. She looks 10 years younger, has good color, and hasn’t taken a nap since the procedure. She was on BiPAP about 17 hours a day before the procedure. Her oxygen saturation has been excellent. ... Caring for her can be a bit overwhelming at times, but it sure is a lot better than the alternative. We are convinced we made the right decision!”

Consider riluzole

In the 1990s, a drug called riluzole (brand name Rilutek) was extensively tested in ALS and found reasonably safe and modestly beneficial. It was ultimately approved by the U.S. Food and Drug Administration (FDA) for use in treating ALS. So far, it’s the only drug to obtain FDA approval for this specific indication.

Riluzole is believed to interfere with the action of glutamate, a chemical transmitter of signals in the central nervous system that, if present in excess or for too long a time in the vicinity of nerve cells, can cause damage. This is generally considered to be the mechanism through which riluzole exerts its modest benefits in ALS.

A study published in 1996 of 959 people with ALS treated with either riluzole or a placebo (inactive substance) found that, after 18 months, 134 out of 236, or 56.8 percent, of those taking 100 milligrams a day of riluzole were alive and without a tracheostomy. In the placebo group, 122 out of 242, or 50.4 percent, had survived and didn’t have a tracheostomy. (There were two other dosage levels of riluzole, all of which showed some benefit for the drug.)

Riluzole was found capable of causing liver problems, so liver function has to be monitored, and serious abnormalities on tests of liver function may require discontinuation of the drug. In addition, some people in the study developed increased weakness, nausea, vomiting, dizziness and/or sleepiness that resolved with a reduction in their riluzole dosage.

Later studies led to the conclusion, which is often quoted, that riluzole prolongs survival with ALS by about two or three months. Unfortunately, many people have interpreted this to mean the two or three months would be spent in the final stages of the disease, with little function or life quality remaining.

This, however, is not borne out by research. In 1998, investigators reanalyzed the data from the 959-person riluzole study, dividing participantsinto mild, moderate, severe and terminal “health states” at various time points. (They were able to do this for all but five trial participants.)

They found the riluzole takers spent significantly more time in the “moderate” health state than did people taking a placebo but not in the mild, severe or terminal states of the disease. That study suggests thatmost people whose lives are extended by riluzole spend the extra time in an earlier, rather than later, stage of the disease.

A down side of riluzole is its high cost, estimated at about $600 to $900 a month. Some insurance plans offer full or partial coverage. Medicare prescription drug plans offer partial coverage.

From the Forum: “Each person has to make their own decision. ... I find it hard to believe my mom could have deteriorated faster without Rilutek, but maybe that might have happened.”

“How would you know if it was helping or not? I can guarantee if you don’t take it, it won’t help.”

Accepting the things that can’t be changed

The progression of ALS and length of survival after disease onset are both highly variable from one person to another.

That said, a number of studies have revealed some “unalterable” (outside the control of the individual) factors that appear to predict the course of ALS. (See “Factors in Survival.”)

One always should keep in mind that ALS ultimately is unpredictable. It’s generally not possible to know with a high degree of accuracy, using these or other factors, how one’s own disease might progress.

The medical reason why some with ALS survive for years, even decades, is unknown. But long-term survivors — and their caregivers — have a lot to teach about ALS nonetheless.

The articles below, from four long-term survivors and two long-term caregivers, offer insights and advice about dealing with ALS — whether for the long haul or the short run. Although each journey with ALS is unique, these “old pros” agree that the secret of survival has as much to do with ways to be alive as ways to stay alive.

30 Years … and Counting Wilfred Griebel received his ALS diagnosis in 1979. Wilfred Griebel of New Ulm, Minn., who was found to have ALS in 1979, says that keeping busy has helped him cope over the past three decades with his diagnosis. “Work hard, play hard, have fun, and keep a good sense of humor,” is his advice for others affected by the disease. “I have always decided that I will not let [ALS] stop me,” says Griebel, 82, who received MDA’s Robert Ross Personal Achievement Award for Minnesota in 2008. “I will keep busy and make the most of every day.” Griebel has a very slowly progressing form of ALS. He uses a manual wheelchair, with a scooter for long distances, and was able to walk short distances with a walker until he broke a hip in November 2008. He temporarily resides in a nursing home while his house is being remodeled. Having served as a military police officer in World War II, Griebel still is an active member of his local American Legion and VFW, attending meetings and activities. A retired farmer, Griebel and his wife of 59 years, Irene, have a farmhouse on the small farm they sold to a son, a daughter and her husband. They enjoy visiting the cows, horses, chickens, dogs and cats from their golf cart. The couple and their family (five children, 12 grandchildren and nine great-grandchildren) are major fans of car racing, watching the NASCAR races on television and attending the stock car races once a week during the summer. In the winter, the family stays involved with racing through a fantasy racing Web site, where they earn points based on how well their chosen teams do during the season. The family participates in MDA fundraisers and events, and answers phones at the local broadcast of the Jerry Lewis MDA Telethon on Labor Day weekends. Griebel also participates in ALS clinical trials. “I believe ALS has made my family very close and very strong,” he says. “Our family has learned to deal with the hard times and make the most of the good times.”

Larry Hill, with wife, Mary, and granddaughter, Melaina.

Larry Hill and his wife Mary Jane began attending their Missouri MDA ALS support group meetings 15 years ago, coincident with the year Larry learned he had ALS.

The standout aspect of their attendance at the monthly meetings is Hill’s selection of hat, which he wears to jibe with the spirit of the season. This April, for example, his headgear consisted of large rabbit ears.

He says that humorous approach, along with his odds-defying tenure with ALS, inspires others in his support group to embrace a sense of fun while continuing to confront the realities of the disease. He also wonders if his take-it-as-it-comes outlook could be a reason he’s avoided a final showdown with ALS as long as he has.

“What’s in my life is there. I’m not going to surrender to it. I’ll worry about tomorrow, tomorrow, if I choose to worry at all,” he says. “That would be my advice to others with ALS, too.”

Hill was a truck driver before his diagnosis, active in bowling, fishing and playing softball in his church league.

One of his biggest joys was being with family and raising his daughter.

Today he’s immensely proud, and humbled, that his daughter became a physical therapist after watching how doctors and therapists worked with him. He uses a DynaVox communication device, has a feeding tube (although he prefers to eat normally) and can transfer with the help of a walker.

Now largely ensconced in an easy chair at home (Mary Jane works full time, but can come home on short notice if needed), his greatest joy is his granddaughter. Watching her discover new things, from new foods to playing house, is one of the most meaningful activities in his life, he says, as well as one of the ways in which he continues to grow.

Hill’s outlook for the future is optimistic.

“When I was diagnosed with ALS, the doctors told me there was very little they could do. Today, with mice and stem cell research, I feel a cure is very close,” he says. In the interim, he continues to stay abreast of new research findings through his support group, and buoys his faith through his church — and his hat collection.

’It’s What I Do’ It’s all about family and faith, says Linda Gibson of Arlington, Texas, a full-time caregiver to her daughter Marcie, who has lived with ALS for more than 15 years. Linda Gibson has been her daughter’s full-time caregiver for 15 years. A preschool teacher for 25 years prior to Marcie’s illness, Gibson sometimes misses her career, but says, “taking care of Marcie is what I’m meant to do now, and I’m so glad I get to do it.” To this end, she has become an expert in Marcie’s high-tech care and also has learned to take time to care for herself. A grandmother of two, Gibson says her mother-friend-caregiver relationship with Marcie, now 38, has evolved since Marcie received her diagnosis at age 23, just as she prepared to graduate from college. “I had to learn how to stop treating her as a child and start treating and listening to her as an adult,” admits Gibson, who prides herself on being able to read Marcie’s eyes. “I always try very hard to respect her wishes, and Marcie always makes her own decisions.” Gibson finds ways to take some time for herself. While Marcie’s using her eye-tracking communication device, Gibson surfs the Internet and communicates with other parents of children with ALS. She also enjoys gardening, crocheting and scrapbooking in free moments here and there. In the evenings, while Marcie’s father is at home, Gibson goes out for a 45-minute to an hour walk, or runs errands. She advises other parents/caregivers to try to live as normal a life as possible. “Your child is always your child no matter what their age. I’m very proud of my daughter and the way she’s fighting ALS. I just do anything she needs. It’s just what I do.”

Treatment Decisions Keep Him in Control

“My quality of life is good because of my wife and kids and my mother and sister, and the VA benefits mean I don’t have the financial crises that most people with ALS face,” says Frank Healy, 55, of Bloomington, Ind., who received an ALS diagnosis in 1996.

Frank Healy gets a hug from daughter, Claire.

Healy says his wife, Maria, is always there “if I need her,” and they hire a caregiver Monday through Friday so she can work, in part, “to stay sane.

A Gulf War veteran, Healy has used a power wheelchair since 1999 and a trach and feeding tube since 2007. Three years after his ALS diagnosis, he was found to have Lyme disease and was treated for 15 months with antibiotics. One of the antibiotics, ceftriaxone, is now being tested in ALS in clinical trials. “I’m guessing that the antibiotics may have had some effect on my longevity,” says Healy, adding that Robert M. Pascuzzi, director of the MDA Clinic at Indiana University Hospital in Indianapolis, has a similar opinion.

Healy makes a point of being involved in decisions about his treatment and care. For several years, he tried various alternative therapies, including oriental medicine, acupuncture and herbs, spiritual healers and eating strictly organic foods to detoxify his body.

“In spite of all this, I still have ALS, but I am in my 13th year,” says Healy, who still has some ability to speak but tires easily. “I will never claim that I have slowed my progression, but I also won’t die wishing I had tried harder.”

Husband Caregiver Stays Flexible Curry Worsham is the primary caregiver for his wife Erin, who received an ALS diagnosis in 1994. But he has help and is grateful for it. Caregiver Curry Worsham, with wife, Erin, and son, Daniel. “The state of Tennessee provides private duty nursing for certain chronic cases. We are lucky to live in a state that provides this care. Most do not. On most days we have an eight-hour day nurse and an eight-hour night nurse. For eight hours a day, I am her sole caregiver. If a nurse is sick or can’t do her shift or the office doesn’t have anyone — happens often — I do the shift,” he says. Worsham, 54, has a vending machine business in Nashville where they live. He works up to seven hours a day, up to six days a week. He tries to work while a nurse is at home with Erin, but his work hours are flexible if he needs to rush home. He says his most important responsibility “is just being there ready to respond to an emergency.” Emergencies often take the form of ventilation problems, in which Erin’s air intake becomes blocked and immediate action must be taken or she could die. Worsham notes, “We think we’ve dealt with every possible scenario, but there is always a new one.” Erin is very determined and creative, and that’s important, Worsham says: “Long-term survivors with ALS should definitely have something creative they love doing, as opposed to simply auditing and observing.” (Quest has published several of Erin’s digital paintings, which she creates by using a sensor between her eyebrows to operate a computer.) For Worsham, the most challenging aspect of caregiving is dealing with Erin’s anxiety. “She’s always been prone to anxiousness and panic attacks,” he says. “Now, with ALS, when things get stressful, it’s particularly difficult for her.” Their son Daniel (born nine months after Erin’s diagnosis) has been a tremendous asset in that regard: “The job of watching him grow has, literally, taken our minds off this stuff (ALS). “I don’t think I get too stressed out [by the demands of caregiving] because I pull back and give myself a break,” he says, adding wryly, “Besides, I get so stressed out over the financial side of things that it makes everything else seem like a piece of cake.”

He Treats ALS as Both Obstacle and Gift

Jeff Lester says he has managed to cope so well for so long with ALS (he learned he had the disease 15 years ago when he was 27) mainly because of his parents and his wife.

“My dad instilled in me the values, drive, attitudes and most of all tenacity to overcome any obstacle in my life to find success, fulfillment and happiness. The most important lesson he and my mom taught me was to live your life to the fullest and to love completely.

“That allowed me to find true love with my wife Lisa. Without Lisa, I would not be here today.”

Jeff Lester, surrounded by wife, Lisa, and their three daughters.

Lester married Lisa a year after receiving his diagnosis, and went on to father three daughters. “The much deeper appreciation I have for them because of my ALS gives me a pretty great quality of life — better than most,” he says.

Despite complete immobility, Lester stays busy. On trach ventilation since 1997, he’s halfway to earning a masters degree in business administration and a masters of science in finance. His ultimate goal is a Ph.D. Eventually he hopes to become an online professor.

Lester’s advice for others with ALS is “to truly live and not merely exist, because that’s not living. Save the regrets for after you’ve left this life because then you’ll have plenty of time.”

He also feels he’s still growing. “I truly believe, regardless if you are well or sick, if you allow yourself to stagnate, you will wither and die,” he says. “I hope I am having the same positive influence in my daughters’ lives as my parents had in mine.”

Lester says he even still feels like he’ll walk again, even though it’s been 11-plus years since he last did. “In my dreams,” he says, “I still envision myself as physically whole.

“My thoughts are that if you want to follow my path, quit thinking that ALS is a terminal disease, but rather an obstacle that you need to overcome. For anyone, I would say view your ALS as a gift. It has freed you of the everyday worry and stress that makes most miss the important things in life.”

Alex Grausnick

Bob Stimson

Claudia Burrows

A former lawyer who wrote his life story with his eyes. A retired Air Force general setting a new example of courage for his grandkids. A mom with two young children who still finds innovative ways to stay involved in their extracurricular activities.

Mark Rosen

Phyllis Swenson

Each day during the month of May, MDA is featuring the life story of one person living with ALS, in a special online series called “ALS: Anyone’s Life Story.” Now in its third year, the series provides a way to inspire others with ALS, and to encourage local media to highlight people with ALS in their communities, to bring more attention to this little-known disease.

The 31 individuals highlighted in MDA’s series live across the U.S., are of all ages and backgrounds, and are at various stages of progression with the disease. They are, in order of appearance:

Brig. General Thomas Mikolajcik, Charleston, S.C.; Loren Dils, Albuquerque, N.M.; Robert Paulson, New York City; Phil Kelleher, Cherry Valley, Calif.; Laura Woodall, Davidson, N.C.; Alex Grausnick, Shoreview, Minn.; Scott Lew, Van Nuys, Calif.; Patrick Guide, Channelview, Texas; Susan Fox, Kansas City, Mo.; Bob Murray, Bethel, Conn.; Charlie Lipari, Wilmington, N.C.; Bob Donato, Los Angeles; Teresa Kiser, Pana, Ill.; David Deitch, Las Vegas; Oscar Sanchez, Denver, Colo.; Mary Forehand, Hemphill, Texas; Tony Soper, Brunswick, Maine; Claudia Burrows, Muncie, Ind.; Glenn Bolkema, Jasper, Minn.; Jeremy Megroz, Laguna Beach, Calif.; Layne Kidney, Hubertus, Wis.; Sheila Bayer, Brookings, S.D.; Larry Hill, Hazelwood, Mo.; David Fick, Tacoma, Wash.; Mark Rosen, Sudbury, Mass.; Tonya Edwards, Lowgap, N.C.; Doug Posorske, Oshkosh, Wis.; Bob Stimson, Bloomington, Minn.; Cynthia Murphy, Lancaster, Calif.; Muiris Foley, Tucson, Ariz.; and Phyllis Swenson, Sarasota, Fla.

To read the ‘Anyone’s Life Story’ series, go to the MDA ALS Division Web site, www.als-mda.org.

On April 21, MDA launched its new social networking site, myMDA. With the goal of creating “strength in community,” the site provides an easy way to connect with other individuals and families coping with ALS in order to discuss health care, research, caregiving strategies, parenting questions and life in general.

ALS is just one of the disease-specific groups on the site, which is designed for use by anyone affected by one of the 40-plus diseases covered by MDA. Other groups exist for those interested in advocacy and for Spanish speakers.

Participants also can create groups around a variety of other special interests, such as parenting, caregiving, athletics, hobbies and more, as well as upload photos and videos, blog, chat and create personal home pages.

To join the myMDA community, go to www.mda.org/myMDA. Registration is free.

Nancy O’Dell, longtime host of the entertainment-news show “Access Hollywood,” has accepted a new role as MDA National ALS Ambassador. O’Dell will spread awareness of MDA’s fight against ALS through media interviews, public appearances and public service announcements.

For a second year, O’Dell will co-host the Jerry Lewis MDA Telethon this Labor Day weekend.

In June 2008, ALS claimed the life of O’Dell’s mother, Betty Humphries, who was 74.

by Kathy Wechsler

Partners: Kathy Hult with Joy, her Labrador retriever service dog.

When Kathy Hult of Eden Prairie, Minn., was matched with a Labrador retriever named Joy six years ago, it was love at first sight.

“Joy just has been such a huge part of, not only my life, but my quality of life,” says Hult, 56, who received a diagnosis of limb-onset ALS in 1999.

ALS trail blazer

Hult learned about the service dog agency Helping Paws in 2001, from a woman with one of their dogs. She contacted the local organization and went through a lengthy qualification process, interview and home evaluation.

Within a year, Hult had been matched with Joy and the pair had endured three physically and mentally draining weeks of team training at the Helping Paws facility in Hopkins, Minn., making Hult the first individual with ALS to receive a service dog from Helping Paws.

“I was thrilled that they would consider me to be placed with a service dog,” she says. “I just hoped at the time that my slow progression of the disease would continue, so it would be a positive experience for the organization to place a dog with an individual with ALS, which would lead to more dogs being placed with people affected by the disease.”

Hult, who lives alone in an assisted living apartment, uses a power wheelchair and has difficulty raising her arms. She says Joy has dramatically increased her level of independence by retrieving dropped items, turning on and off lights, and opening and closing doors. Joy also haslearned to remove Hult’s jacket and socks by tugging them with her teeth. The 9-year-old service dog knows the “speak” command and will bark until help arrives, or go and get help when needed.

Even more than physical assistance and security, says Hult, Joy offers emotional support, filling her life with humor.

“I’m so much happier with her,” she says. “Her unconditional love and loyalty allow me to feel good about who I am through the progression of ALS.”

From running errands and dining at local restaurants to visiting Disney World and taking a cruise to Alaska, the duo go everywhere together. Joy’s presence often brings people over to Hult and is a great conversation-starter.

Finding the right organization

Not all service dog organizations will place their dogs with people affected by ALS, says Hult, who spent three years as Helping Paws’ treasurer. She has heard many people with ALS complain about the difficulty of finding organizations willing to serve them.

Because ALS often progresses so quickly and life span is shortened, and because so much time, money and effort go into training a service dog, some organizations feel it’s not practical to place a dog with a person with ALS, Hult explains.

It may take time to find a service dog organization that looks at the individual, not just the disease, but don’t give up, she urges.

“You have to go in and say ‘There are ALS success stories.’ Get your physician behind you,” she says. “If you think your progression is slow, you should be able to present that argument to them.”

Hult calls getting Joy a “phenomenal” experience. “I know quite a few other people with ALS who have gotten service dogs, and it’s been fabulous for them, as well.”

Think twice

Despite her enthusiasm, Hult warns against making any hasty decisions when considering a service dog.

Consider carefully, with input from your ALS physician as to how quickly the disease is progressing, she advises. Your support team also should be involved, because the dog has needs that may be difficult for a person with ALS to meet.

“If you have a rapid progression of the disease, it’s hard enough to deal with the constant changes that are occurring,” she warns. “To add getting a service dog may not be the best idea. I personally would question that decision.”

Even those with slower progression should think twice. “Getting a service dog to work for you takes a lot of work and commitment,” she says. “People think that when a dog is trained, it will automatically do everything for you, but it’s not a robot and has to want to do things for you.”

It takes time and energy to bond with a service dog and establish dominance by becoming the “alpha dog.” Then after the dog is fully trained, “it’s a matter of working with her so that she responds to you giving the commands,” says Hult, who runs Joy through her list of commands every day.

Adapting to the future

Kathy Hult was the first person with ALS to receive a service dog from Helping Paws.

Even a slowly progressing ALS, such as Hult’s, requires some adapting for both members of the service dog team.

When she first received Joy, Hult used a walker, and Joy learned to walk beside the walker. Then Hult began using a scooter, and Joy had to adapt to that. And then they both had to adapt to a power wheelchair.

“I was able to go from living independently in a house to an assisted living apartment with her, and she adapted beautifully,” she says.

Hult, who still has the ability to speak, plans to record commands into a communication device before she loses her voice, so Joy will respond to orders from the machine.

Like everything else in ALS, says Hult, even with a service dog, “you have to be proactive.”