FW: ALS; How families can stay ahead of dread disease

[Guest guest]

SOURCE : Toronto Star

DATE : June 6, 2002

HEADLINE: How families can stay ahead of dread disease

BYLINE : Ellie Tesher, Toronto Star

HOW DOES anyone handle receiving a death sentence from a doctor?

No one can imagine, yet individuals who get diagnosed with ALS

(amyotrophic lateral sclerosis or Lou Gehrig's disease) face a brick

wall with nowhere to turn for hope.

But, according to ALS expert Dr. Neil Cashman, researchers believe

that grim prospect is on the verge of changing, though it may take

several more years.

I was struck by the devastation of this incurable neuro-degenerative

disease when a long-time acquaintance, businessman Harry " Butch "

Freedhoff, a superb athlete all his life, known for his high energy and

spirits, died last month at 63, after ALS robbed him of his speech, his

motor skills, and finally his breathing, all within nine months.

" Fewer than 1 per cent of patients decide on full mechanical support to

keep them alive, " says Cashman, alluding to permanent attachment to a

feeding tube and ventilator. Physicist Hawking is the best-known

practitioner, having survived this way for 38 years, but Cashman says

many people consider that option " a living hell. "

Some ALS patients eventually ask to be taken off life supports or seek

assisted suicide, as in the much-publicized Sue case. Freedhoff's

wife, Sandy, is both a cancer care nurse and grief counsellor. He

benefited from all the emotional and palliative help possible during his

illness yet couldn't bear to go on in that state.

" He willed himself to die, " his wife says.

Now she wants to alert other patients and their families to what's

available to cope with the progressive ravages of the disease.

Coincidentally, June is the month of ALS awareness programs. There's a

conference on quality care at Sunnybrook and Women's College Health

Sciences Centre for patients, their families, caregivers and

professionals on June 14, and a fundraising walk on Father's Day for

funds for patient care and research.

" ALS was more about huge emotional pain and loss than physical pain, "

Freedhoff says.

Her husband experienced many panic attacks, yet, because his disease

started in the head region with slurred speech (it more commonly begins

in the limbs) and severely restricted communication, he couldn't express

his anguish. In his last family photo with his children and grandchild,

there's haunting fear in his eyes.

In the research community, however, there is growing hope.

" There's never been a time when more people are working on ALS. We all

feel something is going to happen soon, " says Cashman, director of

Sunnybrook's neuromuscular clinic.

Cashman says major changes for ALS began with the drug Riluzole, approved

in Canada last year, which slows down the disease by 10 to 15 per cent.

" It's a psychological breakthrough, " which finally shows victims of the

illness that they can fight back, he says.

ALS attacks motor neurons that control the voluntary muscles, causing

muscles to become weaker and wasted until there is complete paralysis,

while the mind remains active and alert. Early symptoms are weakness

and atrophy in a hand, a dropped foot, slurred speech, difficulty

breathing.

It hits men more than women, often the most physically fit and most

commonly in their mid-50s. Only 10 per cent of cases have a genetic

link. The incidence rate is relatively small at 1 to 2 people per

100,000, but its impact is overwhelming. Most victims die within four

years.

Diagnosis is difficult and only becomes clearer when persistent muscle

deterioration requires referral to a neurologist. Delays are compounded

by months-long waiting lists in this city. Ontario's largest ALS clinic

is at Sunnybrook, which treats 130 new patients a year. London has a

multidisciplinary clinic; two smaller clinics are in Hamilton and Ottawa.

Freedhoff's message is: Families must stay one step ahead of the disease,

so that they get the adaptive aids needed as the condition takes hold.

These include equipment ranging from suctioning apparatus to aid

breathing, to communication boards, available through the clinics or the

ALS organization.

" Communication was the most difficult loss, " says Sandy Freedhoff. Butch

used a computer, then a writing board, then a thumb-activated voice box,

but eventually lost all hand movement so that pre-arranged signals for

yes, no and other needs, became vital.

But there are still appalling gaps in the healthcare system, notably the

difficulty in getting adequate home care and more hospital funding. For

example, Sunnybrook's clinic has 300 patients on its roster, sees 30

people in a day, yet only provides one clinic nurse co-ordinator, Myrna

, who also makes home visits and runs support groups to help

patients, " live while dying, not die while alive. "

For more information on the conference, free for patients, or the walk,

call the ALS Society of Toronto & Area at , ext. 217-218,

or see www.alstoronto.ca.

Ellie Tesher's column appears in The Star Tuesdays and Thursdays. She can

be reached at etesher@....