Breathing is not a problem if you opt for a vent and trach. Swallowing is not a nutritional
problem with a feeding tube. That pretty much takes care of the actual life threatening problems
of ALS!
What is left to consider are quality of life issues. Loss of speech is a hurdle that many
people find very difficult to deal with emotionally. I haven't reached that point but suspect
that the speech equipment available will work well for basic communication but can't give back
the pleasure of a simple conversation. Anyway, loss of speech won't happen because of the trach
itself (and the vent will give you the air volume for a louder voice), but because of the
progression of the ALS. With Bulbar onset, speech has to be considered very temporary, as does
swallowing.
As the ability to swallow worsens we face the "socially unacceptable" and therefore
embarrassing problem of drooling. The good news is that it can generally be reduced to an
annoyance rather than a major problem.
If all of that seems like something you could deal with, there is still the need to
recognize that the ALS will continue to progress. Losing the use of your legs is a walk in the
park (bad pun!) compared to losing the use of your arms. A wheelchair and lift system or Hoyer
lift pretty much solve the leg problem. Arm weakness will eventually require an eye tracking
system to allow you to do anything with a computer and that seems critical in giving any outlet
for an active mind.
Bottom line: All of these quality of life issues are going to happen whether or not you
choose a trach and vent. The difference is that they and you will go on longer if you do. Do you
and your family want the extended time under those conditions? Can you accept that your
progression might be so fast that the extended time is mostly after you have lost all function?
Not wasted efforts but probably not nearly the quality time you had hoped for. Can your family
accept the flip side of that, that you could live for years?
Quality of life is hard to anticipate or for anyone else to
determine. We have all heard that people can deal with far more than they thought they could,
but that ability only stretches so far. How far? Studies have shown that evaluations of a
persons quality of life by doctors and other professionals are consistently lower than the
person themselves rate it:
A 1992 life satisfaction study by John Bach compared responses by 80 Duchenne muscular
dystrophy ventilator users to responses by 273 MDA clinic directors. The clinic directors
significantly underestimated the users' reported life satisfaction. The study concluded
that patients who were perceived by physicians to have a poor quality of life were less likely
to be offered assisted ventilation.
In a 1992 study by Bach and Campagnolo of 395 ventilator-assisted people who'd had
polio, 86 percent reported their lives were characterized by hope, value, freedom, and
happiness. Forty-two percent of the ventilator users were employed and 39 percent were married.
Yet again, the health professionals used for controls underestimated the life satisfaction of
people using ventilators and overestimated the difficulties. It was concluded that physicians
should be aware of their inability to accurately gauge the life satisfaction of individuals and
should not use their perception of poor quality of life as a reason to deny ventilator
assistance.
In a 1994 life satisfaction study by Bach and Tilton, 42 ventilator-assisted quads and 45
autonomously breathing quads were compared to a control group of health care professionals.
Both groups with spinal cord injury reported higher life satisfaction ratings than were
predicted by the controls. Notably, the ratings for those who used ventilators were higher in
some areas than for those who didn't—the former were dissatisfied with sexual function
only, whereas the latter were dissatisfied with their jobs, health, and sexual functioning.
Vent users said life was friendlier, more interesting, more enjoyable, fuller and more hopeful
than non-vent users.
A 1992 study by Whiteneck, Charlifue and Frankel of people with spinal cord injury at least
20 years post- injury showed that those using ventilators rated their quality of life higher
than those who didn't, and had a lower rate of suicide ideation.
The above information
was taken from "Independent Living and Quality of Life Among Persons Who Use
Ventilators," an in-press book chapter by Margaret A. Nosek, Ph.D., and S. Ann Holmes, M.D.
Both authors are ventilator users themselves.
Each of us has a tipping point where quality of life goes from acceptable to unbearable.
Where that point lies is different for each of us. Unfortunately, we can't foresee or
accurately predict that point. Many people settle the issue by drawing a line in the sand early
on and saying “This far and no further”. And that is right for some people. But that
line is drawn in sand and sand can shift. Time can change technology, our circumstances, our
view of our disabilities. Drawing that line can give resolve and emotional peace. Drawing that
line can also lock us into a promise made that makes it hard to admit that we may be considering
otherwise.
The two subjects already discussed, cost and caregivers certainly play a huge part in the
quality of life, vent or no vent. So what does going on a ventilator add to the mix? First, what
can a ventilator do for your quality of life?
Relieve shortness of breath. It will not just ease breathing difficulty, it will completely
erase it. If respiratory problems are the worst part of ALS for you, worse than the frustrating
loss of strength and emotional stress, a trach and ventilator can be a good choice.
Alleviate the underlying feelings of anxiety and nervousness or even panic attacks that
being short of breath causes. Those feelings are not mental or emotional indications that you
aren't coping well with ALS. They are normal physiological reactions that poor oxygenation
triggers in everyone. If you are drowning or suffocating your body wants you to rev up and
fight and not just relax and say "What, me worry?" That reflex remains strong in
ALS.
Stop headaches caused by low oxygen.
Allow you to eat or have tube feedings without your body rebelling against having to deal
with shifting blood flow and already scarce oxygen to digestion.
Improve your energy level so that you can enjoy your family, have a social life again, go
places and enjoy things without total exhaustion that lingers for days.
Make you look better with pink cheeks instead of gray and without the dorky looking head
gear and mask of NIV.
Allow weight gain because your calories can be stored rather than sent immediately to keep
your struggling lung muscles going.
Let you think more clearly—and worry about something other than breathing!
Spare you (and your family) from those terrifying sessions of congestion when you cough and
cough but all your weak efforts only succeed in bringing the mucus to your upper airway and
threatening to block it entirely. With a trach and vent, those awful and life threatening
minutes are reduced to a quick, painless and routine to the point of monotonous
suctioning.
Next, what are the things that a ventilator cannot do for you?
Slow progression of ALS. With fast progression, a ventilator can relieve the breathing
problems, but the ALS will continue to progress. If speech and swallowing haven't already
been taken away, they will be, along with any remaining muscle function. The question becomes
whether or not venting will give you quality of life if every thing else disintegrates rapidly.
It must be said, however, that there certainly are people who find life worth living without
the ability to speak or eat.
Relieve pain and overall discomfort. People with fast progression have the most pain,
probably because the ligaments and tendons have little time to adapt to the changes in muscle
tone. In slow progression that is less a problem and as an extra bonus the horrible muscle
cramps and irritating fasciculations taper off as more and more muscles fade away.
Pneumonia is a common cause of death among ALS patients whether they are on a ventilator or
not. If you have had more than one bout with pneumonia you will probably remain prone to it. If
it was due to aspirating food or saliva into the lungs, a feeding tube and a cuffed trach will
be more helpful in preventing pneumonia than the vent itself.
Improve other health problems. Poor health and chronic diseases may be helped by better
oxygenation but they will still be there. Age is certainly a factor when considering a vent but
age is more than a matter of numbers. If you were physically fit and in good health before the
ALS and have a family history of long life, then a ventilator isn't a bad choice even if
some people some people consider your numerical age a problem.
Life on a vent requires adaptability. Can you find things to occupy the hours? You will be
able to be out of the house much better, but there will still be most days spent at home. A
computer is a great source of entertainment, news, interaction with others if you are willing to
work with one. By the time you need to decide about going on a vent, you will probably be very
reliant on others for care and have a good idea of their ability, willingness, and tolerance for
continuing to care for you. There is nothing more demoralizing than a main caregiver who is
resentful, angry, or acting the martyr. Family and friends may have already deserted you. Good
riddance to the lot of them. Your spouse, partner, child, or parent will most often be your
primary caregiver and their health, availability, and suitability must be considered. Regardless
of their willingness, they must have help. Someone must be shown how to suction you and commit
to at least a few hours a week at a scheduled time. Ideally, you will have more than one person
for this so that your primary caregiver can get out to get groceries, keep appointments, etc.
People uncomfortable with taking care of you can commit to things like lawn mowing, emergency
shopping,
A trach, vent, and feeding tube can keep you alive indefinitely. At some point (assuming
pneumonia, heart attack, stroke, flu, or killer bees don't get you first) you may decide it is
time to explore the great beyond. Whether or not you have a trach and vent, dying will be
similar, just at a different pace. (With a non-invasive or invasive ventilator you can
choose when, without them you have to wait until the process of dying begins naturally.) You
will be given sedation to minimize anxiety and morphine to reduce the feeling of "air hunger"
(polite medical term for suffocation) by dampening respiratory drive. If you aren't using any
type of breathing machine, you will continue these comfort measures for days, weeks, possibly
months. Doses will be increased as needed to keep you comfortable. Eventually, you will slip
into unconsciousness and die in a few days.
If you have ordered that the breathing machine be discontinued, the process will be faster
and the unconsciousness hurried by heavier sedation and morphine. Only when it is clear that you
are too out of it to experience any struggle to breathe will the breathing machine be turned
off. I have always said I am not afraid of dying—I just don't want to be there when it
happens! This seems like the way to accomplish that.
