Many diseases can, of course, cause death if not treated. High spinal cord injury, a serious infection, an appendicitis, etc., can kill when neglected. When treated, these are still serious conditions, but usually life can continue and death can be prevented.

It's true that the average length of life of people with ALS today is about four years. However, this reflects the fact that people with ALS often don't have optimal treatment and resources.

If ALS is to be fully treated, people with the disease should have:

* Adequate information about the treatments available
* Access to health professionals who are interested in and experienced with all the available options in the care of ALS
* The encouragement to go forward
* The needed resources to go forward
* The desire to use available care

When the patient lacks the desire to use available care, after having been fully informed and provided with a positive approach from professionals such as doctors, nurses, physical and occupational therapists, social workers and others, not proceeding is acceptable.

However, when any of the needs cited are insufficiently filled, not treating ALS to the fullest is regrettable. Ideally, this situation shouldn't exist.

What is "fully treated" ALS? The answer is my "wish list" for all people with ALS:

1. A positive and experienced team approach to assist patients and their caregivers in solving any of the problems that typically occur.

This means a patient should have access to a team of adept and enthusiastic health professionals who are available even when the patient isn't taking part in a research protocol. The team should include professionals who can make home visits and coordinate care as needed.

2. Very good nutrition to maintain appropriate weight

At some point this may include using a gastrostomy tube (feeding tube) if swallowing problems occur.

3. Regular social interaction with friends and the community

It's vital that the person with ALS get out of the house by using mobility assistance, stay engaged in living and maintain ways to communicate even if speech becomes difficult. Good alternative and augmentative communication devices are essential.

4. Personal assistance to cover each 24 hours as needed, without placing an undue burden on family members

(Editors note: The first rule of caregiving is that the caregiver must remember to take care of his or her own needs too.)

5. Vigorous treatment for infections, particularly respiratory infections

This can often be provided at home with appropriate antibiotics when needed. Medication can be given by mouth, via a feeding tube or even intravenously, depending on the person's condition.

6. Monitoring of breathing capacity so noninvasive assisted ventilation can be available when capacity is decreased and related symptoms are present

An ALS patient can receive mask-delivered or other noninvasive ventilation as long as this works, and then shift to tracheostomy (invasive ventilation, via a tube into the trachea) if that becomes necessary. Equally important are good attention to effective coughing and clearing of secretions, and prevention of aspiration (inhaling particles, such as food, into the lungs). The decision to stop using a ventilator at any time, or to use only noninvasive ventilation and not go on to tracheostomy ventilation, should be made by the patient.

7. Access to ALS research centers and protocols when desired, and access to excellent ALS care even when there is no desire to participate in research

8. Very good supportive care

9. Good symptom-relieving care when major aspects of treatment are no longer desired or appropriate

When most of these aspects of care are available to people with ALS, they can often continue living in a productive way and avoid life-threatening complications related to ALS most of the time. ALS motor neuron impairment may become severe, but life and spirit and social ties can continue.

Ideally, people with ALS should have choices. They should know that there are options that will allow living to continue even if the resources needed are considerable.

About the author:

Edward Oppenheimer of the Southern California Permanente Medical Group is an associate clinical professor of medicine at the University of California at Los Angeles and is a pulmonary consultant to the American Academy of Neurology's task force on clinical practice guidelines for ALS. He has also been a consultant to MDA.