ALS Research Roundup

Stem cells entered nervous system but didn’t slow ALS

Although six men with ALS who received intravenous infusions (“transplantations”) of donated bone marrow stem cells failed to derive any apparent benefit from the procedure, the study showed that such cells can enter the central nervous system from the bloodstream, an important finding.

The ability of stem cells to enter the nervous system by penetrating the barriers surrounding the brain and spinal cord supports their potential use as carriers of therapeutic substances, such as neurotrophic factors (see “Supercharged stem cells.”)

MDA grantee Stanley Appel at Methodist Neurological Institute in Houston coordinated the study team, which published its results in the Oct. 14 issue of Neurology. (Appel, director of the MDA/ALS Center at Methodist Neurological Institute, is a member of the MDA Board of Directors and chairman of MDA’s Medical Advisory Committee.)

MDA grantee Stan Appel coordinated a study team that found that donated bone marrow stem cells can enter the nervous system in people with ALS, and that immune-system T-cells provide an unexpected benefit in ALS mice.

Appel said the results, while important, should serve as a warning to patients not to believe the “hype” surrounding currently advertised stem cell treatments for ALS.

In the recently published study, six men with sporadic (nonhereditary) ALS, ranging in age from 35 to 59, underwent radiation treatment to partially destroy their own bone marrow, followed by medications to suppress their immune systems and help them accept bone marrow stem cells donated by siblings.

All study participants accepted the donated cells, with four of the six eventually showing 100 percent donated cells in their bloodstreams.

Three patients had rapid progression of their ALS following transplantation, with no evidence of clinical benefit.

A fourth experienced slow loss of function and required invasive (tracheostomy-delivered) respiratory support about three years later.

Another experienced slow disease progression for the first 14 months after transplantation and then rapid disease progression.

Only one patient’s ALS progressed at an overall rate that was slower than average for this disease, living for 7.5 years after symptom onset. The investigators say his age (35 at study enrollment), rather than the bone marrow transplant, was the likely cause of his slow disease progression.

In three study participants, the brain and spinal cord were examined after death.

Two patients who had shown 100 percent donor cells in their blood showed 16 percent to 38 percent donor DNA at sites of ALS injury in the spinal cord. Both also showed donor DNA in the parts of the brain that their symptoms indicated were the most affected by the disease.

In the third patient, whose blood had carried only a small percentage of donor cells, there was no donor DNA in the spinal cord or brain.

The donated cells showed a marked preference for sites of injury, areas to which molecular “damage signals” may have attracted them.

“This first long-term study of bone marrow stem cell transplantation in ALS in humans is important,” Appel said, “in that it showed us that donated cells can get into the brain and spinal cord and home to the sites of injury specifically.

“Our study showed no real clinical benefit for ALS patients, which should send a warning not to believe the exaggerated claims for stem cell transplantation in ALS at the present time.

“However, although stem cells by themselves didn’t provide any benefit to the patients, further studies in which stem cells serve as delivery vehicles for therapeutic genes or proteins may prove of value, especially since donor cells appear to enter areas of the nervous system at sites of ALS injury.

“Risky injections of donor cells containing therapeutic compounds directly into an ALS patient’s spinal cord would probably not be required. Instead, delivering potentially therapeutic compounds via bone marrow stem cells could probably be accomplished with infusions into the bloodstream.”

Supercharged stem cells save nerve cells, lengthen life span in ALS rats

Stem cells (Photo: Emanuela Gussoni, Children’s Hospital, Boston)

Scientists at the University of Wisconsin-Madison and institutions in the United Kingdom and Switzerland have shown that stem cells derived from human bone marrow and engineered to produce a neuroprotective substance can significantly slow symptom progression in rats with a disease resembling human ALS.

Masatoshi Suzuki and Clive Svendsen, both at the University of Wisconsin’s Waisman Center, and colleagues, published their findings online Sept. 16 in Molecular Therapy. They say the stem cells acted as miniaturized long-term “pumps” that delivered GDNF (glial-derived neurotrophic factor) to the rats after being injected into their muscles. The neuroprotective compound apparently traveled to nerve cells from the muscle tissue. (A recent human study showed such cells can reach the nervous system via the bloodstream. See “Stem cells.”)

Rats with mutated SOD1 genes and a disease resembling human ALS that were treated with the supercharged stem cells lived as much as 28 days longer than their untreated counterparts. Even in rats with a very severe, rapidly progressive disease, caused by high numbers of mutated SOD1 genes, the average survival time was prolonged by 18 days.

The stem cells the researchers used, known as “mesenchymal” stem cells, normally give rise to bone, muscle, cartilage, tendons or fat cells. The investigators note that these stem cells, even without being supercharged to produce GDNF, secrete other proteins that may be neuroprotective, reduce inflammation and enhance blood-vessel formation.

In fact, the stem cells preserved significant numbers of motor neurons, the nerve cells that control muscle activity that are lost in ALS, even when they didn’t produce GDNF. However, the benefit was greater when the cells were equipped with GDNF genes.

The researchers note that muscle is an accessible tissue compared to the nervous system and that delivering GDNF or other protective genes via stem cells has advantages over other gene therapy approaches.

For instance, they say, delivering therapeutic genes directly to the recipient’s existing cells, which are already undergoing ALS-related degeneration, might not be as beneficial as delivering these genes via new cells that are healthy and bring additional advantages to the targeted areas.

“This was a combined therapy approach,” Svendsen said. “Both the stem cells, and the GDNF they released, seemed to work together to provide these effects.”

T-cells provide protection in ALS mice

T-cells, the foot soldiers of the immune system, are involved in protecting motor neurons (nerve cells that activate muscle) in ALS, says a group of scientists led by MDA grantee Stanley Appel at Methodist Neurological Institute (NI) in Houston.

Methodist Hospital in Houston, home to the MDA/ALS Center in the Methodist Neurological Institute (Photo: The Methodist Hospital System)

Lead authors David R. Beers and Jenny S. Henkel, working with Appel at NI, published their findings online Sept. 22 in Proceedings of the National Academy of Sciences.

First, the researchers developed mice with a disease resembling human ALS but without functional T-cells and another group of mice with the same ALS-like disease but with functional T-cells. They found that the mice without the T-cells had an accelerated disease course and died earlier than the mice with T-cells.

When the ALS mice lacking functional T-cells were given an infusion of bone marrow stem cells, T-cells appeared in their spinal cords, apparently from the infused stem cells. Their disease course slowed, and their survival was extended compared to the ALS mice without T-cells. (These experiments were designed to test whether ALS mice did better with or without T-cells and didn’t test whether extra T-cells or bone marrow stem cells would be beneficial.)

The findings came as somewhat of a surprise, Appel says, since his team had expected that T-cells would be toxic and not protective in the nervous system in ALS. Previous studies, some of which were conducted in his laboratory, had shown that other immune-system cells, called microglia, can cause dangerous inflammation in the central nervous system, the site of injury in this disease.

However, he notes, “It turns out that activated T-cells can be a good thing in ALS. Instead of animals living longer with no T-cells, they died in a shorter period of time.”

The investigators zeroed in on “CD4-positive” T-cells as the T-cell type that’s likely conferring the benefit. “At least in this animal model, CD4 T-cells are critical,” Appel said. “T-cells in general modulate the complex activities of the immune system, and our study demonstrates that they can be neuroprotective.” Appel added that T-cell therapy could be of value in human ALS, although further studies are needed.

“It’s been known for some time that T-cells are present at sites of injury in ALS patients as well as in mouse models of ALS,” said investigator David Beers. “But until now, the role of these cells was unknown. This study demonstrates that T-cells, through their interaction with other cells in the immune system, are protecting the cells in the spinal cord that cause muscle movement. It’s now critically important to understand how T-cells provide this neuroprotection.”

Investigator Jenny Henkel noted, “These [CD4 T] cells may eventually provide a readily accessible target for therapeutic intervention, not only for ALS but other neurodegenerative diseases.

FDA finds no connection between statins and increased ALS risk

Taking cholesterol-lowering medications known as statins does not appear to increase the risk of developing ALS, said the U.S. Food and Drug Administration in a statement released Sept. 29. The agency recommended no change in the prescribing guidelines for these drugs.

In June 2007, the World Health Organization reported in the journal Drug Safety that there might be a connection between statin use and an ALS-like syndrome. (See “ALS-Statin Interactions Probed,” ALS Newsmagazine, September 2007.)

After its own adverse event reporting system signaled further investigation of the subject was warranted, the FDA undertook an extensive review of a possible connection between ALS development and statins such as atorvastatin (Lipitor), lovastatin (Mevacor), pravastatin (Pravachol), simvastatin (Zocor) and others.

Eric Colman, a physician in the Division of Metabolism and Endocrinology Products at the FDA’s Center for Drug Evaluation and Research, coordinated a group of FDA-based specialists in cardiovascular disease, neurology, epidemiology and statistics that analyzed data from 41 clinical trials of statins.

The Food and Drug Administration found that cholesterol-lowering drugs (statins) didn’t appear to increase the risk of developing ALS, but the case isn’t completely closed.

The investigators, who published their findings online Sept. 29 in Pharmacoepidemiology and Drug Safety, only included clinical trials that lasted at least six months and in which statins were compared to a placebo (inert, look-alike substance).

Among participants in the statin trials, they found nine people who were on a statin who developed ALS and 10 who were taking a placebo and developed ALS. “Although we observed a ... signal for ALS with statins in FDA’s AERS [adverse event reporting system], retrospective analyses of statin clinical trials did not reveal an increased incidence of ALS in subjects treated with a statin compared with placebo,” the researchers concluded.

They also noted that the antiinflammatory properties of statins and the relatively stable incidence of ALS in the United States over the past two decades, despite a 30-fold increase in statin use, argue against the possibility that statins cause ALS.

However, they say, because these drugs are widely prescribed and ALS has such dire consequences, “any degree of uncertainty justifies further investigation.” They’re expecting results from a study now under way through MDA grantee Lorene Nelson at Stanford (Calif.) University to shed further light on this subject. Nelson, an epidemiologist, has MDA funding to conduct a “case-control” study of ALS and cholesterol-lowering medications, including statins, in which she’s comparing the medication histories of people with and without ALS.

ALS Registry Act passed

On Sept. 23, the U.S. Senate passed the ALS Registry Act, authorizing creation of a national registry to gather and store data on ALS. (The U.S. House of Representatives passed similar legislation in 2007.) The federal Centers for Disease Control (CDC) will be responsible for creating the registry and coordinating research.

“Registries are very helpful in identifying trends that should be followed up in research studies,” said Valerie Cwik, MDA medical director and senior vice president of research. “We’re hopeful that a national ALS database will result in enhanced efforts to define treatments.” MDA is currently funding the ALS Connection registry at www.alsconnection.com.

ALS disability benefits for vets confirmed

The Veterans Administration (VA) confirmed in September that, “the development of ALS at any time after discharge or release from active military, naval, or air service is sufficient to establish service connection for that disease.”

Prior to this time, only veterans with ALS who had served in the Gulf War (August 1990-February 1991) were accorded “service-connected disability” status by the VA. It’s the agency’s highest disability rating, and it makes the greatest degree of no- or low-cost VA medical care available.

“For seven years, the ALS advocacy community has pushed to get all ALS-affected service people included in that package of care,” said MDA Vice President of Advocacy Annie Kennedy. “This is wonderful news for the ALS community.”

'Tis the Season for New Holiday Traditions

by Kathy Wechsler

Almost everyone loves holiday traditions. Besides being fun and giving us another reason to look forward to the holiday season, traditions promote family togetherness and camaraderie among friends, and provide an excellent way to make and share memories.

When ALS interferes with favorite holiday traditions, then it’s time to start new ones or modify the old ones to better meet your needs.

Before Michael’s diagnosis, the deKruifs would always bundle up a week before Christmas and head off to a tree farm, in search of the perfect spruce. An artificial tree is now the centerpiece of their Christmas, but other family traditions continue.

May these festive stories provide inspiration for ways to keep the holiday season as memorable and meaningful as ever.

A needleless Christmas

The week before Christmas, Michael deKruif and his wife, Donna, of Waterford, Mich., would bundle up the kids and drive to one of three self-cut tree farms to harvest their Christmas tree. The family tradition came to an end a few years ago when ALS made it difficult for Michael to hold a saw.

Even more than the smell of a freshly-cut Christmas tree, deKruif, 56, loved the tradition of spending quality time with his family, both at the tree farm and warming up at home afterward.

So the deKruif family started a new tradition of family togetherness. Now, instead of chopping down a 6-foot spruce, they spend time together assembling a 6-foot artificial spruce. Pine-scented reed defusers give the house a holiday smell. One part of the old tradition remains the same: listening to Christmas music and decorating the tree as a family.

“Donna says the artificial tree is great, because there are no needles to clean up,” says deKruif. “There’s less expense all around, and anything that cuts expenses when struggling with ALS is a help. Lastly, there’s no dead tree to dispose of, and one less tree cut down.”

Donna also loves that they can carry on the season longer without worrying that the dry tree is a fire hazard.

Have them come to you

For many years, Dorothy “Dee” Drago had traveled to California, Arizona, Massachusetts or New Hampshire to visit her five grown children and their families for Thanksgiving and Christmas. After her ALS diagnosis in 2007, it was difficult for Drago to travel. Now her family spends the holidays at her house in North Las Vegas, Nev.

There are downsides to this new tradition: “I won’t be able to see their houses — and they have the decorations and all that,” says Drago, 80.

But in addition to getting help with her own holiday decorations, the new tradition allows Drago to visit from the comfort of her own home.

Drago also used to go Christmas caroling around the neighborhood with friends, but now she saves energy by having the carolers come to her. She’s still able to enjoy singing along.

“It was wonderful for me to go out caroling with them,” she says. “But I like them to come to my house.”

Snack in the New Year

When Scott could no longer go out to celebrate New Year’s Eve, the Stafnes revived an earlier tradition of cooking up a batch of late-night pancakes. Scott, Kirsten and Eva, above, display their “2008” flapjacks before Scott and Eva chow down.

Like any young, childless couple, Scott Stafne and his wife, Kirsten, of Woodbury, Minn., spent the first few New Year’s Eves of their marriage going out with friends. After returning, the friends enjoyed a late-night snack of pancakes shaped like the numbers of the new year.

Stafne, 36, who was diagnosed with ALS in 2004, started having weakness in his left arm and trouble walking right around the time Kirsten, gave birth to their first daughter, Eva.

“Having a child affected what we could do on New Year’s Eve before the disease did,” says Stafne. With the birth in March of their second daughter, Miranda, going out on New Year’s Eve with two young children and “a guy in a wheelchair” would be a lot of work for Kirsten. “In practical terms, I am like a third child for my wife — she might even say that I act like a child sometimes, but either way, it makes getting all of us out the door a real challenge, especially in the winter.”

Since they needed to modify their old New Year’s Eve tradition into a family- and ALS-friendly affair, last year the Stafnes decided to hold a family New Year’s Eve dinner featuring pancakes shaped like the numbers of the coming year. They hope that this becomes an annual tradition.

“We struggle to maintain a sense of a more traditional family, with the two of us as parents, instead of a patient, caregiver, and two young mouths to feed,” he says. “So creating and maintaining traditions for any time of year seems more important to us now.

“Spending time with my girls is the most important thing to me — there’s no comparison.”

Giving the gift of memories

One holiday tradition Tim White and his wife, Angie, of Cedar Hill, Texas, are hoping to implement this Christmas is a letter exchange, where instead of exchanging gifts, each member of the extended family will write meaningful letters to other family members.

White, 50, who was found to have ALS in 2006, and Angie say the purpose of the exchange is to share funny stories, special memories and thoughtful gestures that are significant to each family member.

“It may seem a bit emotional, and some might consider it a bit morbid, because it’s almost what you would say in a eulogy, but it’s meant to be shared while our loved ones are still with us,” says Angie. “And it blesses everyone — not just the person with ALS.”

Reasons Why I’m Grateful to ALS

by Mike Bougher

I have been living with ALS for over 10 years. In that time we have become close friends. I no longer consider this disease to be a life-robbing, horrible thing, but a life-enhancing experience. I am grateful for the new perspectives I have been given. This is not just knowledge that I understand and accept intellectually. These are truths that have become part of my being. I now understand and enjoy my existence more than I thought possible, ALS or not.

Author Mike Bougher, who also created the digital painting above, “Soma Vineyards.”

One day, many years ago, it occurred to me that an ALS diagnosis was not the end of my life. I realized that if I got a feeding tube and a ventilator, I could live a lot longer, but quality of life would be an issue. I must surrender to that over which I had no control (my body’s deterioration) and begin to build on that which still had great growth potential (my mind and spirituality). I could spiral downward into misery, or use my circumstances as a catalyst for mental and spiritual expansion. I had a choice! The elation that paradigm shift brought became the spark that inspired a profound transformation.

I asked my doctor to fill out and submit the forms for my state’s Blind and Disabled Library, and soon I received my free four-track tape player. Their large catalog of books is available for browsing online, and selections can be mailed to you free, with the click of a mouse.

I gorge on books that satisfy my intellectual curiosities, and others that point me toward spiritual growth. One book seems to dovetail with the next. Recently I have been exploring astrophysics, quantum mechanics, world history, classical music from the Baroque period, the nature of human consciousness, physical anthropology of the Americas, the cultures, music, plights and spirituality of various Native American nations, and Asian music, religions and philosophy.

I also meditate and pray, leading me to the abundant, joy-filled life I enjoy today.

Insights for which I am grateful

We live within a universe that is all one. Everything and everyone is interdependent, made by and from one single source. I feel connected to and kinship with all other people on this planet. I am grateful for the ability to truly understand and be a part of the interdependent nature of our universe.

Because of the kinship I share with all other people, I cultivate compassion and not blame others for their circumstances. I am grateful for the empathy I experience toward all daughters and sons of God.

I have found a way to help ease the suffering of others through the sale of my art. I am grateful to have been shown a purpose for my life — a purpose that feels much more meaningful than my previous self-serving goals.

I have ample time for prayer and meditation. I have time to focus on the meaning of this life, the suffering of others, the true nature of reality, and time to commune with God. I am grateful for the timeless inner peace I experience.

I have a physical form that from the outside appears distorted and out of proportion. I can’t speak audibly or smile, and I drool. I am grateful that I have been placed in circumstances to counteract my large ego. (My biggest spiritual stumbling block.)

I am nearly always present in the moment. This makes me a better listener and keeps me from mourning the past or dreading the future. To truly live life we must experience the present moment without coloring it through thoughts of the past or future. I am grateful for being shown the joy of living in the NOW.

Despite complete paralysis from the neck down, and requiring others to do everything for me (except use the computer), I still retain my dignity. No one can take that away. I am grateful that I have been made to understand that I am not my body or my thoughts. I am the silent space from which all is created.

I am most grateful for my loving wife Jen who has enthusiastically agreed to take this journey with me, and has never faltered in her love or companionship as we travel this path together.

This is only my experience, and I am in no way trying to invalidate or belittle others who are suffering through more negative circumstances. My hope is that in some small way, even one person will begin to realize that suffering is caused by the content of our thoughts, not the situations in which we find ourselves.

A former project control systems designer and project manager for Telstar, Mike Bougher, 44, of Benicia, Calif., was a self-proclaimed workaholic before his ALS diagnosis. In recent years, assistive technology has enabled him to pursue a new passion, digital painting. He created the above image from scratch using his computer and “paint” software. (See ALSN June 2007, “Equipment Corner: A Chat with Digital Painter Michael Bougher” for information about the technology he uses.)

One Man’s Search for Family in Two Worlds

by Bill Norman

Peter Cassidy and Johanna De Luigi in the United States with their 5-year-old son Cameron.

Infant Ernesto Sibrian’s mother cradled him in her arms as she fled from soldiers through the El Salvador jungle. The bullet that killed her tore through her body and lodged in Ernesto’s arm. His 6-year-old sister pleaded with the soldiers not to take him, but they pushed her to the ground.

A few months later, Ernesto was adopted by Kathleen Cassidy, a social worker from Princeton, N.J., who found him in a South American hospital. She was told he had no family. She named him Peter Cassidy.

Today Peter, 25, is about to graduate from Delaware University. He has a 5-year-old son. He learned five years ago he has ALS.

In November, he’s going to walk 26.2 miles in a New York City marathon. He’s also putting finishing touches on a movie he made about his life.

“I’d like to write a book, too, if I can find a ghostwriter. I think my experiences would be motivational to other people with ALS,” he said.

Kids hurt by war

Cassidy was one of more than 2,000 children, victims of the 1980-1992 Salvadoran civil war, who were uprooted from their families. His sister and grandparents agonized over his unknown fate. Children often were given or sold to international adoption mills by government troops fighting guerrillas, who often hid in peasant villages in the jungle. “They gave us out like chickens,” said a boy quoted in a New York Times article about the situation.

Unlike most, Cassidy discovered his birth family when he turned 15. The group Asociación Pro-Búsqueda de Niñas y Niños Desaparecidos (Association for Missing Children), aided by DNA testing, confirmed that his sister and grandparents lived in a tiny mountain village not far from where his mother died. Their home had no running water or electricity. Pigs, goats and turkeys shared the house. His sister had never used a telephone.

Cassidy had a cell phone and a beeper, hung out with a hip ninthgrade crowd and couldn’t speak Spanish. But he had always wondered about his birth family. His adoptive mom and he agreed that they needed to go to El Salvador to meet them.

Beans, tortillas and cornflakes

Peter, center, visiting his grandparents and cousin in El Salvador.

Peter/Ernesto had a tearful reunion with his birth family, but also encountered major culture shock. He didn’t like beans and tortillas, which was about all the clan had to eat. Instead he’d walk into town and load up on good old American fare like corn flakes and soft drinks. He desperately missed television. The outdoor toilet and general lack of sanitation appalled him.

But after that first visit, and then others lasting a month or more that occurred each year thereafter, he came to know and love the people of his heritage, and to speak their language. “There’s not a whole lot to do in the village, so I’d visit for a few days, then backpack or take a bus to neighboring countries like Guatemala, Costa Rica and Honduras. Then I’d head back to the village for a few more days. Eventually, when I got back to the States, I realized I had a split personality, but in a very good way.”

Embracing two worlds

Cassidy said many adopted children, especially if they’ve reached adulthood, don’t stick around too long after being reunited with their birth families — if indeed that ever happens.

“They just don’t have a whole lot of interest or time to get involved,” he said. “But that wasn’t the case with me. I have good friends in the U.S., and when I visit El Salvador, I have friends who’ll pick me up at the airport, feed me, give me a place to stay.”

He still spends a lot of time with his adoptive mom, but he also carries a treasured photo of his birth mom, taken when she was 20. When ALS struck when he, too, was 20, he urgently felt the need to track down his father, in part to determine whether the disease affected any members of the family on that side.

He found his dad in Honduras, and learned he had a half-sister and three brothers, all very successful in life. There was no history of ALS on his father’s side.

Recorded for posterity

The intertwining of his life with the lives of his relatives in two distinctly different worlds forms the essence of his film, which originally comprised 45 hours of tape painstakingly shot in both countries. His footage is good enough that a Canadian news broadcasting company used some in a documentary about children displaced by the Salvadoran war.

Cassidy says he needs to finish the film, just as he needs to walk the 26.2-mile marathon, while he has time. Since his diagnosis, he’s lost most muscle control in his hands, although he can still type and hold a job. His feet and legs are telling him they’re next. He’s trying lithium under the guidance of Lewis Rowland, founder of the MDA/ALS Center at Columbia University in New York.

On the upside, he started a brief but successful business buying, racing and selling horses; has a responsible management position with ING, a large banking and investment firm; and has job offers from others when he graduates from college.

Best of all, he has families in two hemispheres who love him.

by Amy Labbe

In Sandstone, Minn., Alea, 16, and Alexandra, 13, have added “caregiver” to the long list of roles they assume in life, including daughter, sister, friend, teenager and student. The girls’ father, William Anderson, learned he had ALS on March 26, one day after his 49th birthday.

Emily Tuttle helps her mom, Tamara, apply makeup. The Tuttles were featured recently in the new MDA ALS Caregiver’s Guide.

To date, Anderson’s symptoms include weakness and lack of coordination in his hands, arms, shoulders, chest and back. He retired from his job as a corrections officer with the state of Minnesota due to the progression of his symptoms, but still serves as a volunteer with, and president of, the Pine County Sheriff’s Department, planning meetings and events. He uses braces to preserve strength in his wrists, but even so has trouble managing forks and spoons, getting dressed, handling tools such as hammers and wrenches, cooking and folding clothes.

Anderson knows eventually he’ll require the help of a professional caregiver and perhaps even the services of an assisted living facility, but for now caregiving duties have fallen to his daughters (Anderson was divorced from Alea’s mother in 1993 and separated from Alex’s mom in February). It’s a responsibility the two teenagers take on gladly.

’I’m learning so much’

Anderson’s daughters help out by doing the cooking, cleaning, laundry, shopping and whatever else needs doing. Alea, who recently received her learner’s permit, drives Anderson to appointments.

William Anderson makes sure his daughters and caregivers, Alex and Alea, also make time for themselves: “It’s always on my mind that it’s not all about me.”

Alea says it’s hard helping someone she thought would never need help, but she’s happy to do it. It takes “a lot of strength,” she adds, but she’s proud that she and Alex are able to handle it.

Alex says it may sound like a cliché, but helping her dad is “rewarding.”

“It gives me a sense of security to know he’s OK and to be able to see him,” she says. “I know he gets better care with me and Alea, the people who know him best, than he would in any nursing home, or with another relative. I’m learning so much about life and myself in the process of helping my dad.”

Both say it’s difficult watching their father decline, but cite a long list of positives to the caregiving experience.

Alex notes caring for her dad has taught her “not to be selfish,” and to “value time, value life and care about others.” She says it’s taught her to be strong and to always look for the bright side of things, and that “relying on other people sometimes is OK when you can’t get through something by yourself.”

Alea explains that she’s learned “to take advantage” of what she has, because she knows she might lose it. She says she’s grateful for the people in her life, more forgiving than she used to be, and has learned to think “more about the good things than the bad.”

Both see a therapist when they need someone to talk with outside of the family, and both rely on family and friends for support as well, but they admit they find the most strength in each other.

The two also have outlets for downtime. Alea plays softball, listens to music or seeks the companionship of friends, while Alex sings in a choir, helps out with theatrical productions at school and seeks out “quiet places,” such as a quarry nearby, where she goes to think.

Both girls have plans for the future that include attending college.

The hardest thing, they both say, will be telling their dad when caregiving becomes too much to handle.

“I never want to tell him that,” Alex says. “But I know it will happen.”

Alea echoes her sister’s thoughts.

“I know it has to happen at some time, but I really don’t want it to,” she says. “It will be very hard, but I know if we tell him he will love us just the same. He just wants us to be happy, no matter where we are. If we leave or stay, his love for us will never change.”

’They know they have a choice’

Anderson recognizes the difficulties and challenges the added responsibilities of caregiving present his daughters.

“I do everything in my power,” he says, to help the girls be children and “not force them to grow up too fast.”

That includes making sure they do things together as a family, like taking walks or drives, or going to the movies or video arcade. Anderson encourages Alea and Alex to make time for themselves as well, to visit friends or host sleepovers and movie nights for their friends at home.

He also stresses open communication and makes sure the girls know they can come to him with problems, to tell him they need a break, or to tell him when the time comes that they can’t handle caregiving any longer.

“I still watch them for signs of being tired, sadness, hopelessness, wanting to be alone, school grades falling, or just being sad,” Anderson says. “It’s always on my mind that it’s not all about me — they come first.”

Anderson has made the faculty at the girls’ school aware of their situation so that teachers and counselors can help watch for any signs of trouble. Although no formal arrangements have been made, Anderson, the girls and their extended family members agree that if/when their father’s care becomes more than the girls can handle, they will move back with their mothers “with the complete understanding that they did a great job helping their dad.”

Most importantly, Anderson says, the girls know “they have a choice in this matter.” They choose to do it “because they love me and care about every minute we share together. This is a learning tool for all of us in life — it will help them grow and teach them of all that surrounds them, good and bad.”

’Caregiver’ shouldn’t replace ‘carefree’

Christopher Boys, a pediatric neuropsychologist and assistant professor of pediatrics at University of Minnesota Medical School, says it’s helpful for families affected by ALS to approach the situation as a “family change,” where everyone has to work together as a team, “change and chip in.”

Boys notes that age-appropriate tasks (typically focused more on housework than on personal care) help children and teens become involved and feel a part of what’s going on, but cautions that kids shouldn’t be pressured into doing more than they’re able to handle or allowed to “slip into acting like an adult.”

He urges parents to hire home health aides or arrange for professional care before the situation gets to the point where children become “too involved.”

“You’re looking for a balance,” agrees psychologist Barry Jacobs, director of Behavioral Sciences for the Crozer-Keystone Family Medicine Residency Program in Springfield, Pa., who wrote “The Emotional Survival Guide for Caregivers” (2006, Guilford Press).

“The challenge is to give kids a meaningful role, but not to give them one that overwhelms them or takes them away from age-appropriate activities,” he notes.

Jacobs has seen children gain a real sense of appreciation for family members by being able to contribute in some way, but notes that “striking the right balance is a difficult thing.” He warns that parents must ensure children don’t become overwhelmed by caregiving duties, as it can lead to a feeling of having “lost their childhood.”

Boys and Jacobs say warning signs that may indicate a child or teen is having a hard time handling caregiving duties include:

• irritability and a decreased tolerance for frustration in younger kids;
• a tendency to withdraw or display depression or anxiety in older children; and
• moodiness, sleep problems, nightmares and a general resistance to performing caregiving tasks.

The amount of responsibility a child can handle, says Jacobs, "is dependent on the particular child's personality."

Parents can help their children enjoy childhood by making sure they have the opportunity to indulge in their hobbies and interests, and that they have a place they can go to find support when they need it. They also should make sure there’s time for all the things that go along with being a kid, such as extracurricular sports and activities, friends and TV. Parents might consider asking adult friends and extended family members to do things with their children, such as attending sports practices or games, when they can't be there.

Although the possibility exists that children will become overwhelmed, Boys says many kids have indicated they appreciate the responsibilities they learn through caregiving, as well as the opportunity to contribute to their parents and families.

As the Anderson family has learned, when the right balance is attained, caregiving can be a positive experience for all involved.

For booklets created for teens and children coping with a parent’s ALS, visit the ALS Society of Canada at www.als.ca/als411/.

Online Support for ALS Family Caregivers

Want help but don’t know where to turn? This list provides a starting point for locating resources and online support for family caregivers.

In addition, check out MDA’s Chat calendar at www.mda.org/chat/calendar.html to learn more about the “Living with ALS” chat. The new MDA ALS Caregiver’s Guide also includes extensive resource lists; go to www.als-mda.org/publications/alscare, or call your local MDA office at (800) 572-1717.

ALS Chat Groups

ALSforums
www.alsforums.com

ALS TDI Forum
www.als.net/forum

Living with ALS
http://health.groups.yahoo.com/group/living-with-als

NeuroTalk Communities — ALS
http://neurotalk.psychcentral.com

Information, support, volunteer organizations and more

AARP Caregiving Online Community
(888) 687-2277
www.aarp.org/community/groups/Caregiving

ALS Care
(336) 340-6400
www.alscare.com
Free online educational resources, plus information about fee-based services such as telephone consultations with a registered nurse specializing in ALS care.

CarePages
(888) 852-5521
www.carepages.com
Create an easy, no-cost Web site to update family and friends about your situation and needs.

CaringBridge
(651) 452-7940
www.caringbridge.org
Similar to CarePages.

ElderCare Online
www.ec-online.net
Resources, tips, information, support and a directory of online elder care support groups.

Empowering Caregivers
(212) 807-1204
www.care-givers.com
Links to message boards and online forums.

Extra Hands for ALS
(732) 223-1602
www.extrahands.org
Find high school and college students who want to volunteer services for people with ALS and their families.

Family Caregiving 101
www.familycaregiving101.org
Site includes a message board for family caregivers.

Family Caregiver Alliance
(800) 445-8106
www.caregiver.org
Online caregiver discussion group and directory of programs at national, state and local levels.

Family Caregiver Support Network
(800) 449-4481
www.caregiversupportnetwork.org
A host of resources, plus a caregiver discussion board and a section for requesting respite help.

Full Circle of Care
www.fullcirclecare.org
Support for family caregivers, including respite services.

Lotsa Helping Hands
www.lotsahelpinghands.com
A service of the National Alliance for Caregiving, this easy-to-use site helps caregivers organize volunteer helpers.

National Family Caregivers Association
(800) 896-3650
www.nfcacares.org
E-communities, caregiver message boards and a Family Caregiver Pen Pal program.

National Respite Locator Service
(919) 490-5577
www.archrespite.org
Find state and local respite services.

Parents with Disabilities Online
www.disabledparents.net
Resources and stories from parents with disabilities.

RespiteMatch
(678) 884-0281
www.respitematch.com
Created by a long-term ALS survivor, this “classified ad” site connects individuals with home health care professionals; also provides info on hiring outside help.

ShareTheCaregiving
(646) 467-8097
www.sharethecare.org
Share the Care is the original model for organizing and maintaining a volunteer caregiving group.

Strength for Caring
(866) 466-3458
www.strengthforcaring.com
Resources and an online caregiver community.

Today’s Caregiver
(800) 829-2734
www.caregiver.com
A digital magazine, resources and caregiver discussion forum.

Well Spouse Association
(800) 838-0879
www.wellspouse.org
Support groups and online discussion forums.