ALS From Both Sides
Care of an ALS Patient
By Diane Huberty, Retired RN, Certified Neuro Nurse
...and ALS Patient
Cramps and Spasms
Remodel or Move?
Advances "Around" ALS
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 first CPAP machine would acquire computer chips by the early 90s and become a Bi-level Ventilator . Bi-level ventilators didn't exist just 20 years ago and were not in common usage until about 10 years ago but today is 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 wheel chair, 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. Free standing overhead lifts don't require any construction or holes in the walls. And the slings for lifts are a
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 over size 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 paint brush 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 type 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 reserved 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.
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