a closer look at als

ALS WAYS TO HELP

If you know someone with ALS, youmay want to help but don’t knowwhere to begin. Here are some ideas.

ALS will affect the roles and respon-sibilities of family members.Spouses, parents, and children canfind themselves providing care andsupport to someone with ALS.Caregiving can make recreation,chores, and even employment diffi-cult or impossible to maintain for theprimary caregiver.

Many caregivers report high levels ofstress and stress-related illness.Caring for someone with ALS is chal-lenging. As time passes, the need forcommitment increases. Eventually,ALS requires round-the-clock care.

Friends and neighbours are importantsources of support for the family.Whether you live close by or far away,there is still plenty you can do.

KEEP IN TOUCHMaintain contact with family care-givers. A card, a call, or a visit meansa great deal. Caregivers as well as theperson with ALS will benefit fromyour visits or calls. Continue to sendcards or letters even if you don’treceive a reply. It’s a simple yetimportant way to show you care.

DO SMALL THINGS Little things mean a lot. When cook-ing, make extra portions and drop offa meal (in a freezable container). Ifyou’re on your way out to do anerrand, check with the caregiver tosee if there is anything needed.Surprise the caregiver with a specialtreat such as a rented movie, a librarybook, or a gift certificate for a mas-sage or dinner out.

GIVE THE CAREGIVER ABREAKEveryone needs a little time on theirown. Offer to visit with the personwith ALS so the caregiver can runerrands, attend a support group meet-ing, do a favourite activity, or attend areligious service. Even if the caregiv-er does not leave the house, this willprovide some personal time. Chancesare the person with ALS will alsoenjoy your company.

HELP WITH A SPECIFIC TASKMany caregivers find it hard to askfor something specific. Ask the fami-ly to make a “to do” list of hard-to-get-done chores such as laundry, yardwork, or shopping. Decide what youcan do, then spend some time on aregular basis to help out.

BECOME INFORMEDLearn about ALS and how it impactsthe person and their family.Information is available at www.als.caor from your local ALS Society.

PROVIDE A CHANGE OF SCENERY Plan an activity that gets family mem-bers out of the house. Invite thefamily to your house or to a nearbypark for a picnic or a walk.

LEARN TO LISTENSometimes caregivers just need to talkwith someone. Ask family membershow they are doing and encouragethem to share. Be available when thecaregiver is free to talk without inter-ruptions. Try not to question orjudge, but rather support and accept.You do not need to provide all theanswers – just be a compassionate lis-tener.

TAKE CARE OF THE CARE-GIVERCaregivers need to eat well, exercise,and get enough rest so that they canremain healthy. Encourage caregiversto take care of themselves. Passalong useful information. Offer toattend a support group meeting withthem. Information is available fromyour local ALS Society.

ALS. Three letters that change people’s lives. FOREVER.

AMYOTROPHIC LATERAL SCLEROSIS SOCIETY OF CANADASOCIÉTÉ CANADIENNE DE LA SCLÉROSE LATÉRALE AMYOTROPHIQUE

3000 Steeles Avenue East, Suite 200, Markham, Ontario L3R 4T9

Tel. 905-248-2052 • Fax: 905-248-2019 • Toll Free:1 800 267-4257 • www.als.ca

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Assisting families living with ALSAssisting families living with ALS

Over

REMEMBER ALL FAMILY MEMBERSThe person with ALS will appreciateyour visits, even if they are unable toshow it. Hold a hand, give a hug, talkwith the person the way you wouldwant to be talked to. Spouses, adultchildren, and even young children areall affected in different ways by ALS.Be attentive to their needs too.

GET INVOLVEDThere are many things you can do tohelp fight ALS. Consider making acontribution to the ALS Society tosupport research. Volunteer at yourlocal Society to raise funds for localservices for those affected by ALS.Get involved with the WALK forALS or Hike 4 ALS in your area. Bychoosing to do any of these, you areproviding help for today and hope fortomorrow.

The ALS Society has informationand support programs. Find the

society nearest you and give thema call. We are here to help.

You are not alone.


| ALS WAYS TO HELP

YOU MAY NOT KNOW THAT

ALS CAREGIVERS

• often feel alone and isolated from friends

• may need assistance, but are reluctant to ask

• are often unable to do errands or complete household tasks

• experience stresses that sometimes affect their health

• need regular breaks from caregiving

• may need someone to listen

PEOPLE WITH ALS

• need to feel valued

• face an uncertain future

• may worry about being a burden to their families

• need companionship

• strive to maintain independence and quality of life

ALS & Cognitive ChangesUntil recently, it was believed that ALSdid not affect thinking or behavior.Research in the last few years has shownthat ALS can cause cognitive andbehavioral changes in some people.Cognitive changes (differences in howpeople think, know, perceive andunderstand) usually result from brain celldegeneration in the frontal lobe. Somepeople with ALS experience behavioraldysfunction without cognitive change.However, many people with ALS willexperience no changes in either thinkingor behaviour. The following facts willhelp you better understand ALS andchanges in the brain that may occur. Ifchanges in behavior or thinking aresuspected, speak to your ALS medicalspecialist for further assistance.

o Research suggests that certainproteins, either tau or ubiquitin, maycontribute to cognitive impairment

o Chromosomes 17 or 9 may beimplicated in familial forms of ALS withcognitive impairment

o Approximately 30 to 50 per cent ofpeople with ALS experience cognitivedifficulties

o Mild problems, such as inattention andslowed thinking, are the most commonin people with ALS and are often onlyrecognized through neurological testing

o Approximately 20 to 25 per centacquire severe impairment, such asfrontal lobe dementia (FTLD), aprogressive condition involving selectivedegeneration of the frontal and anteriortemporal lobes of the brain

o A 2003 study showed approximatelyfive per cent of participants met the fullcriteria for FTLD, with 52 per centmeeting criteria for possible or probableFTLD

o FTLD appears as a change inpersonality and/or behavior

o Symptoms include apathy, restlessness,mood swings, loss of reasoning orproblem-solving ability, repetitivebehaviors

o Unlike Alzheimer's disease, behavioralsymptoms are usually the first to develop,rather than memory loss, which may notoccur at all

o The most common subtype of FTLDis frontotemporal dementia (FTD),which is marked by early decline in socialand personal conduct, lack of emotionand loss of insight

o The two other subtypes are semanticdementia (SD) and primary progressiveaphasia (PPA). SD is characterized byloss of vocabulary, poor wordcomprehension, word finding problemsand loss of insight; PA entails non-fluentspeech, poor grammar but goodcomprehension

o People who are older than 60, havebulbar onset ALS, poor breathing or afamily history of dementia may be moresusceptible to cognitive impairment

o Diagnosis occurs throughneuropsychological and neurobehavioralevaluations

o People with both ALS and cognitive impairment may have shorter lifespansthan those with ALS alone, possiblybecause they are less likely to chooselife-prolonging procedures due to loss ofinsight into their ever-changing needs

o Data suggest the pathologic process issomewhat different in people who haveboth diseases compared with those whohave one or the other

o Cognitive problems can't be cured butmay be relieved by psychotropicmedications

o To accommodate cognitiveimpairment, family, friends andcaregivers can speak slowly and simply,modify the environment and makedecisions regarding health-care andfinances early in the disease's progression

o Caregivers may need to be involved inthe decision-making process when theperson with ALS can no longer makeappropriate decisions

o Caregivers may require more supportwhen dealing with both ALS andcognitive impairment, as stress andcare-giving responsibilities are increased





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ALS QUICK FACTSALS is Amyotrophic lateral sclerosisa - absence myo - muscle trophic - nourishmentlateral - side (referring to the spine)sclerosis - hardening or scarring

The word "lateral" identifies the areawhere nerve cells in the spinal cord thatnourish the muscles are located. As nervecells die, the muscles receive no nourish-ment and waste away. Sclerosis occurs asthe area affected deteriorates.

ALS may also be called …• Lou Gehrig's disease• MND (Motor Neuron Disease)• SLA (Sclérose latérale amyotrophique)• Maladie de Charcot

ALS is a rapidly progressive neuro-muscular disease• Motor nerve cells die• Voluntary muscles degenerate• The senses are unimpaired• The intellect may remain unaffected• ALS is not contagious

Some cases are hereditary• Less than 10 per cent are familial ALS

ALS is a fatal disease with no effec-tive treatment and no cure• Eighty per cent of people with ALS die

within two to five years of diagnosis• Some people die within a few months• Ten per cent of those affected may live

10 years or longer

ALS is the most common cause ofneurological death in Canada• The mortality rate for ALS is

approximately 2/100,000 per year. • Approximately 2,500 - 3,000

Canadians over 18 currently live withALS

ALS can strike anyone• The incidence rate (number of new

diagnoses) of ALS is estimated to be2/100,000 people per year

• Less than 10 per cent of cases of ALSare hereditary

• ALS can strike both men and women• ALS affects all ethnic and

socio-economic groups• ALS can strike young or very elderly

adults but is most commonly diagnosedin middle and late adulthood

ALS affects the whole family• People with ALS require costly equip-

ment and care• Eighty per cent of the burden of care is

shouldered by family members• ALS is a drain on the physical,

emotional, and financial resources of caregivers

The ALS Society of Canada• Funds research for a cure• Supports the Provincial Societies • Provides information to build

awareness

Ten Provincial ALS Societies• Provide care and assistance• Provide information and referrals• Provide support for caregivers• Provide equipment for people with ALS• Advocate on behalf of people with ALS

June is ALS Awareness MonthALS Canada and the provincial ALSSocieties work together to raise aware-ness of ALS and to raise funds forresearch and support services. Please buya cornflower in June to support thosewho suffer from this devastating disease.

TEN PROVINCIAL SOCIETIES

ALS Society of Alberta (and NWT)

250 - 4723 - 1st Street SW

Calgary, AB T2G 4Y8

403-228-3857

www.alsab.ca

ALS Society of British Columbia (and Yukon)

1233 - 13351 Commerce Parkway

Richmond, BC V6V 2X7

1 800 708-3228

www.alsbc.ca

ALS Society of Manitoba

493 Madison Street

Winnipeg, MB R3J 1J2

1 866 718-1642

www.alsmb.ca

ALS Society of New Brunswick

P.O. Box 295

Moncton, NB E1C 8K9

506-532-5786

www.alsnb.ca

ALS Society of Newfoundland & Labrador

P.O. Box 844

Corner Brook, NL A2H 6H6

1 888 364-9499

www.envision.ca/webs/alsnl

ALS Society of Nova Scotia

900 Windmill Road, Suite 113

Darmouth, NS B3B 1P7

902-454-3636

www.alsns.ca

ALS Society of Ontario and Nunavut

3100 Steeles Avenue East, Suite 402

Markham, ON L3R 8T3

1 866 611-8545

www.alsont.ca

ALS Society of Prince Edward Island

P.O. Box 1643

Summerside, PEI C1N 2V5

902-439-1600

[email protected]

ALS Society of Québec

Société de la SLA du Québec

5415 rue Paré, bureau 200

Montreal, Québec H4P 1P7

514-725-2653

www.sla-quebec.ca

ALS Society of Saskatchewan

90 C Cavendish Street

Regina, SK S4N 5G7

(306) 949-4100

www.alssask.ca




Toll Free:1 800 267-4257 • www.als.ca

| Fact Sheet

July 2011

ALS, MS AND MD

AMYOTROPHIC LATERALSCLEROSIS (ALS)ALS is a form of motor neuron disease.It involves the loss of the actual motornerve cells. The nerves affected are inthe spinal cord and those that travel tothe voluntary muscles, so there aresymptoms of both central and peripher-al involvement, with weakness and wast-ing in arms, legs, and mouth/throat(bulbar).

The loss of nerve cells results in second-ary atrophy – or wasting (amyotrophy) –of the muscles served by those cells. Thehardening in ALS involves the lateralcolumns of the spine as a secondaryphenomenon, providing the ‘lateral scle-rosis’ part of the name.

Although people with ALS usually haveinitial symptoms in one side more thanthe other, both sides are involved and theeffects usually become more symmetricalas the disorder progresses. Sensation isnot affected.

Approximately 3,000 Canadians live withALS. Two to three Canadians a day dieof ALS. Less than 10 per cent of allcases of ALS are genetic.

Treatments for ALS are being developedand while the cause of ALS is unknown,research provides hope for a cure.

MULTIPLE SCLEROSIS (MS)Multiple sclerosis (MS) is an unpre-dictable – at times disabling – disease ofthe central nervous system: which con-sists of the brain and the spinal cord.

The disease attacks the protective myelincovering of the central nervous system,causing inflammation and often destroy-ing the myelin in patches. The severity ofMS, rate of progression, and specificsymptoms cannot be predicted at thetime of diagnosis.

While symptoms may be similar to thoseof ALS (weakness and wasting of thearms and legs) sensation is often affectedin MS because of demyelination in partsof the nervous system where sensation istransmitted.

The actual word ‘sclerosis’ comes from aGreek word meaning ‘scar’. Scarring inMS distorts or blocks nerve signals.

An estimated 50,000 Canadians havemultiple sclerosis. Canada is a high-riskarea for MS, which occurs more often incountries which are further from theequator.

MS is not a fatal disease. The vastmajority of people can be expected tolive a normal or near-normal lifespan,thanks to improvements in the treatmentof symptoms and in other therapies.The cause of MS is unknown butresearchers are closer to finding theanswer.

MUSCULAR DYSTROPHY (MD)Muscular Dystrophy (MD) is the nameof a group of about 40 different muscledisorders that are characterized by pro-gressive weakness and wasting of thevoluntary muscles that control bodymovement. As muscle tissue weakens and

wastes away, it is replaced by fatty andconnective tissue. MDs are not diseasesof the central or peripheral nervous sys-tem, but exclusively of the muscle.

Two-thirds of MD strikes children,although dystrophies such as opthalmo-plegic (affects muscles of the eyes andpharynx) and late-onset MD strike inadulthood.

MD is inherited or caused throughgenetic mutation.

Although there is no cure for musculardystrophy as yet, physiotherapy andoccupational therapy can help peoplewith muscular dystrophy achieve theirmaximum level of independence in dailyliving. In some cases, certain surgicalprocedures can also improve the qualityof life for many individuals with muscu-lar dystrophy.





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ALS, MS, and MD: How do they differ?ALS, MS, and MD: How do they differ?Often, the public confuses Multiple Sclerosis, Muscular Dystrophy, and Amyotrophic Lateral Sclerosis.

• ALS is a rapidly progressive and fatal neuromuscular disease.

• MS is a scarring and hardening of thesheath around the nerves in the brain,spinal cord, and optic nerve.

• MD is a muscular disorder withspecific kinds of MD involvingdifferent muscles in the body.

• MD is almost exclusively hereditary.MS is sporadic with some familialtendency. ALS is sporadic in all but10 per cent of cases.

ALS vs MSHow to explain the

differences

A diagnosis of ALS is devastating toall concerned, as it is a rapidly pro-gressive disease with no known cureyet and no effective treatment.Rilutek®, generically known as rilu-zole is the only FDA-approved drugfor use in treatment of the disease. Acostly drug that may have significantside effects in some people, it hasbeen shown to prolong the life of aperson with ALS up to severalmonths. No other drug or treatmentbesides Rilutek® appears to alter thecourse of ALS.

Faced with the limitations of avail-able treatment, people with ALS andtheir caregivers may be inclined toseek alternative treatments. It isimportant to be aware of the poten-tial dangers in this pursuit, as thereare some people who will take finan-cial advantage of those who are des-perate to find a treatment or cure.

The global community ofALS/MND health professionals is asmall but dedicated group that keepsabreast of current developments inALS research and communicates fre-quently through scientific meetings,the internet, and newsletters. Anyonewho maintains membership or con-tact with a recognized ALS/MNDassociation or society may be surethat they are receiving up-to-date

information on the latest researchand therapies.

While the internet provides an invalu-able tool for communication amongALS professionals, it is also a sourceof a great deal of misinformationgenerated by unscrupulous or ill-informed sources of therapies andeven "cures" for this as yet incurabledisease. Even respectable newssources, including radio, television,and newspapers, contribute to themisinformation, as health newsbecomes a more and more mar-ketable commodity among an agingpopulation, and competition amongnews agencies becomes fiercer. Thegains in understanding ALS beingmade by the research community arenot generally the headline-grabbingsort; and, there is a tendency amongnewsmakers to overstate such glim-mers of hope as "miraculous break-throughs," creating false hope inthose who have ALS and placingpressure on the medical communityto grant access to new therapies.

The following information is intend-ed to provide an overview of thera-pies either in development or on themarket, and to assist in makinginformed choices in the treatment ofALS.

CONVENTIONALLYAPPROVED DRUGS

Rilutek® is currently the onlyapproved drug for the treatment ofALS/MND.

THERAPIES THAT SHOWPROMISE IN ANIMAL MODELS

Coenzyme Q10, COX-2 inhibitors,minocycline, and creatine are a fewexamples of drugs that have shownpromise in the superoxide (SOD1)mutant mouse model that was creat-ed to mimic a pattern of familial ALSpresent in 20 per cent of inheritedcases. None of these, or any otherexperimental drugs have translatedinto the same degree of efficacy inthe human form of the disease.

OFF-LABEL DRUGS

"Off-label" describes the legal use ofa prescription drug to treat a diseaseor condition for which the drug hasnot been approved. Such use of pre-scription drugs may present a prob-lem, because they are often pre-scribed by a physician who is notfamiliar with ALS. Without rigoroustesting, there is no knowledge of thecorrect dose or of interactions withother medications used in the treat-ment of ALS patients. While the useof drugs in combination, or poly-therapy, may be an approach to the


ALTERNATIVE TREATMENTS




| Fact Sheet

treatment of ALS, without testing onspecific drug combinations, optimalcombinations and doses remainunknown. Most insurance companieswill not reimburse people with ALSfor the cost of a drug that is notspecifically directed for use in thetreatment of that disease.

NON-CONVENTIONAL(ALTERNATIVE)MEDICATIONS

Non-conventional, alternative, and"natural" therapies form the broadestspectrum of available treatments, andare the most likely to come fromquestionable sources. An onlinesearch for ALS treatments will turnup substances to "target and reversethe brain and neurological imbal-ances," ways to "beat the system"through nutrition, special supple-ments, and detoxification, and"cures" that will help your body"regain its natural state of health andvibrancy." At best, some of thesetreatments may or may not be harm-less, at worst is they hold out cruelhope for a cure that does not yetexist.

On the less extreme end, manypeople with ALS use readily availableover-the-counter treatments, nutri-ents, herbal supplements, and naturaltherapies that they feel are helpfulwith managing symptoms. For themost part, these treatments do noharm. Since there are few formaltrials involving these products, thereis no evidence showing their benefits.In addition, many natural medicines

contain the same active molecules as

chemical drugs, but produced from aplant source rather than synthetically.Therefore, it is necessary to discusstaking these with your medicaldoctor.

Canada has recently passed legislationto govern the production, labeling,and sale of natural health products(NHPs) under which manufacturers,importers, packagers, and labelers ofNHPs in this country will be requiredto comply with stringent regulationsregarding medicinal and non-medicinal ingredients, source,potency, and recommended use.Health claims for NHPs sold inCanada will require standards ofevidence such as traditional refer-ences, observational studies, expertcommittee reports and clinical or trialdata. These regulations should makeit easier for people to make informedchoices when using alternativemedicines.

STEM CELL THERAPY

Stem cell research presents hope forthe treatment of many diseases,including ALS. While this researchholds promise, it is in its early stages,and since ALS is a complex disease, itis unlikely that stem cell therapy willoffer a simple cure in the near future.Uncontrolled, non-approved stemcell therapy is available in certainparts of the world, and people havepaid great sums of money for treat-ments that may be dangerous andhave no guarantee of success, orworse, show evidence of failure.

COMPLEMENTARYTHERAPIES

Complementary therapies are notused as a replacement but in conjunc-tion with conventional medicine.They are not meant as a cure, butmay provide relief from symptomsand make day-to-day life more com-fortable for people with ALS.Complementary therapies includemeditation, hypnosis, acupuncture,massage, and other physical andmental therapies used to relieveanxiety, stress, stiffness, pain, anddiscomfort. To learn more aboutthese options, please refer to ALSCanada's fact sheet, ComplementaryTherapies.

SOME USEFUL RESOURCES:

International Alliance ofALS/MND Associationshttp://www.alsmndalliance.org/

The Motor Neurone DiseaseAssociation (U.K.)http://www.mndassociation.org/index.html

The ALS Association (U.S.)http://www.alsa.org/

Rilutek® ALS Info Web Sitehttp://www.alsinfo.com/index.jsp

By: Elaine MacNeill


| Fact Sheet

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