One of the frustrations often expressed by those whose lives are affected by ALS is
that no progress has been made in the fight against it. I believe that they are aware
of the great progress made in understanding the disease process, the biochemical
processes at the cellular level, the genetic, genome, and chromosomal tracking of the
disease. I believe that what they are referring to is that all of this work has not led
to a treatment or cure, to anything that can make a real, practical, day to day
difference in our lives. Dedicated researchers have been kissing a lot of medicinal
frogs but haven't found a Prince. Rilutek rates as a "Sir" for its
effectiveness, or perhaps a "Lord" by virtue of being the first drug with
proven effectiveness, but sadly it is no Prince. Radicava is the latest drug. The
restriction of it being for newly diagnosed patients without respiratory involvement
limits its use and insurance coverage and the horrendous cost is beyond affordable for
nearly all patients. It requires repeated IV infusions. It is frequently not tolerated
by patients and some report increased weakness. Some have improvement but it remains to
be seen if improvement is sustained much less increased.
Do I sound skeptical? Always. Hopeful? Definitely.
However, if advances "in" ALS aren't yet affecting our lives,
advances "around" ALS certainly do. I am referring to technological advances
that have prolonged and improved the quality of our lives. This technology isn't
something that a person with a falling off a cliff kind of progression has time to
appreciate, but my progression has been a long slow tumble down a rocky slope. Over my
twenty-nine years of living with ALS, I have seen so many technological advances, each
of which I use every day and none of which I take for granted because I have seen life
with ALS without them.
Even before I was diagnosed with ALS, I had decided that I would never want to live
if living meant being paralyzed and on a ventilator. I was an Intensive Care nurse and
dealt daily with patients in that situation. Trying to read the lips of those who
couldn't speak, or worse, the eyes of those with facial paralysis, knowing they were
desperate to communicate basic needs was frustrating and heartbreaking. An itch they
couldn't scratch or an uncomfortable position they couldn't change would be bad enough,
but what if they were trying to tell me of a throbbing toothache, gall bladder attack,
chest pain? Making their lives physically comfortable was so difficult. Making them
emotionally comfortable seemed impossible. Their days were spent staring at the same
four walls and a television whose channel they couldn't change. I wondered how they
could bear the endless hours. No jaunts down the hall much less a wheelchair ride
outside. Just getting them into a chair was an effort because they were tethered to a
machine bigger than a dishwasher. Being at home would certainly be an improvement but
still, I hoped that their brains could somehow shut down imagination and desire,
restlessness and boredom.
When I learned I faced all that because I had ALS, I was horrified. I knew I could
refuse to be put on a vent but would I be brave enough for that? Would I even be able
to cope with the paralysis before the vent was needed? I couldn't see how.
But that was in 1985. Today I am a vent-dependent quadriplegic and quite a happy
person. The future I thought I faced in 1985 is far different than the life I lead
today! Even before I left ICU nursing in 1987, technology was making changes.
In the late '80s, there was talk of a new type of ventilator that delivered air
through a tightly fitted mask over the nose rather than a tube inserted through a hole
in the throat. Little did I imagine that CPAP machines would acquire computer chips by
the early '90s and become a Bi-level Ventilator . Bi-level ventilators didn't exist
until then but today are part of the Standard of Practice for ALS. That bit of
technology kept me alive and breathing comfortably for 6 years until an invasive
ventilator was needed.
Neither did I envision what that ventilator would be like. In 1985 ventilators were
the size of a dishwasher. By the late '80s, they were the size of a large microwave. It
was certainly not worthy of the designation "portable", but it was a start.
The vent of today is no longer the size of a kitchen appliance. It is the size of a
laptop computer or even a lunch box and hangs on the back of a wheelchair, making
it—and me—portable far beyond the four walls of 1985. I can take a jaunt
around the yard or neighborhood, a trek to the mall, or trip across the country.
Not all the technological advances have been in medical equipment. My day begins on
my air cell mattress that is far better than the "egg crate" foam pad of the
80's. Memory foam was an improvement but today's mattresses that alternate air pressure
to relieve pressure points are definitely a new step up. The newest mattresses turn you
gently side to side and you set how often, how fast, and how far. I move to my power
wheelchair where I sit on a glorified whoopee cushion. The technology behind pressure
relief has taken them to new levels of comfort and pressure sore prevention. I can
spend a 16 hour day sitting up, something impossible on a standard chair cushion. The
power wheelchair itself, although not a new invention, has benefited from technology
and is now moved, tilted, reclined, raised, and adjusted with computer technology that
gives a greater level of independent operation that is continually being improved
upon.
Equipment to move to and from the wheelchair is no longer limited to a Hoyer lift.
Ceiling mount lifts are far more convenient and can even move you into the bathroom and
the shower or tub. Freestanding overhead lifts don't require any construction or holes
in the walls. And the slings for lifts are a huge leap forward from the big
monsters that required major effort to get under the patient and might have a hole to
go to the bathroom through. Today's slings can be taken off and put back on in bed or
in the chair and U shaped design allows for easy use of a toilet, commode or
bedpan.
In the bathroom, I have another little gizmo that, although available for decades,
has improved greatly in 20 years. The electric toothbrush is now battery operated,
rechargeable, and as of 1987, has a much more effective rotary brushing direction. Not
an earth-shattering breakthrough but definitely one that kept me dentally independent
much longer and now makes my care easier. Although I don't have one, many people
with ALS use heated and padded toilet seats with spray bottom washers and warm air
dryers!
Throughout the day I am surrounded by other little gadgets that make my life
easier. In the 1990s, when semiconductors for emitting and receiving infrared radiation
were developed, remote controls gradually switched to that technology and the
proliferation of remotes began. Remote controls are not just for TVs anymore! Stereo
systems, VCRs, garage doors and car doors have all gone remote—and the remote
craze hasn't ended there. Heaters, fans, gas fireplace logs, lights, window shades,
doors—nearly everything can be put on a remote. Can't keep track of all the
remotes? Get a single programmable remote and teach it to do everything. Can't
handle those tiny little remote buttons? Get a remote with oversize buttons. Can't
handle buttons at all? Get a light pressure switch and scanning device.
Safety has improved as well. Cell phones make family members reachable wherever
they go. Emergency call buttons summon help if we have fallen and can't get up.
Chairs with lift seats reduce the risk of falls.
I end my day with a favorite invention that hit the market in the mid-80's. The
simple invention of CDs has been a daily joy for me. I love to read and when I could no
longer turn book pages I began listening to books on tape—and dealing with
hissing, snarling tapes and the headaches of rewind and fast forward and buttons I
couldn't push. CDs are a major improvement. Remote controlled multi-disc players make
books and music accessible far longer than the tape deck of the '80's. Libraries
have books on CD's for listening or downloadable books for reading on a computer or
iPad.
Gadgets like these simplify many things for the paralyzed, but for improvement in
the quality of life, nothing tops the computer. I use it as a remote to control the TV,
lights and anything else I can plug into an X-10 outlet. I can use it to answer the
phone, to speak words for me when my trach is cuffed and I can't talk, to play my
favorite music or movies. And all of this is done with glorious, delicious
independence!
Beyond being an environmental control unit, my computer saves me from my worst
fear, the mind- numbing isolation and boredom, the need to shut down my brain to
preserve my sanity. I am occupied, distracted, entertained, informed, and even
productive thanks to my computer. Aside from the time-passing simple entertainment of
computer games, I use my computer to maintain records for our neighborhood association,
retouch, restore, and print photos, design and print flyers and cards, t-shirts, family
photo CDs. I can't wield a paintbrush or hammer but I have planned projects from
simply rearranging rooms to kitchen remodeling, deck design, and landscaping. Being
able to see how something will look before beginning has really helped overcome the
hammer wielders resistance to doing things my way. Power in decision making!
Not only have computers come a long way from the basic functions of my Apple IIe of
the early 80s, handicapped access to computers has evolved as well. Today I use
Click'n'Type, and Dasher, two different types of onscreen keyboards, to type
without using my keyboard. Point'n'Click pushes the mouse buttons for
me—all for free. As my ability to move the mouse to move the cursor deteriorates,
I can go to a device that moves the cursor by following slight movements of my head. A
step up from that are devices that track eye movements to move the mouse. The very
latest technology is a page right out of science fiction. Computers can now be operated
by thought! This technology is in very early development but is already being used and
holds promise for the future.
I had to stop working in 1995, coincidentally the same year that AOL gave access to
the new and developing World Wide Web. I had dabbled with the Internet previously but
it wasn't until the Web arrived that I found being online to be useful. In just a
few years the Web exploded from a few websites to a massive source of information,
entertainment, communication, and commerce. Today I read newspaper and magazine
articles (replacing the printed versions I can't manage), use the Internet as a
giant encyclopedia to research any topic, comparison shop and sometimes buy, select
books on CD to be downloaded or renewed for me at my local library, keep in touch with
family and friends via email, and keep informed about ALS and communicate daily with
others with ALS.
The greatest reward I get from my computer and the Internet is that it isn't a
one-way street. I can give as well as receive. My involvement with ALS groups online
lets me share my hard won knowledge of ALS with others. I have been able to put up a
personal website to relay my nursing information about ALS. I get great personal
satisfaction from this technological ability to help others and have even made my first
venture into designing a commercial website. (The heck with personal
satisfaction—I could even make money designing websites!)
Speaking of money, obviously, all these tech gadgets aren't free. The medically
necessary ones are covered by insurance and Medicare, but the convenience ones are not.
Just as when grocery shopping, I compare prices, wait for sales, look for used
equipment locally and on eBay. Having had 20 years to accumulate my
”toys"—and upgrade them!—has certainly been a perk not many with
ALS have, but I have learned that the expense is far outweighed by the quality of life
these things give me and the help they give my caregivers.
There isn’t a day that goes by that I don't use several things that were
not available when I was diagnosed with ALS in 1985 and not a day that I don't
think how lucky I am to have ALS today instead of before technology contributed to
these advances "around" ALS.