Bigger Than The Sky

ALS & TECHNOLOGIES

Technologies

Communication

Dynavox On-screen keyboards: WiViK, SofType, Gus Manual spelling: Alpha-spell, Eyelink EZ-Keys Eyegaze ERICA

Respiration

BiPAP In-Exsufflator Vibrating Vest Tracheotomy/Laryngeal closure

Mobility

Canes Walkers Orthotics, braces, aids Transfer aids: Belt, lifts, boards Manual wheelchairs Power chairs: Jazzy, Moby I, Moby II

Nutrition

Utensils Food preparation: Thick-It, Puree Feeding tube

Hygiene

Shower/Toilet chair

Sleep

Manual turning Props Egg carton TURN-Q lateral turning mattress

Late 1993 - Early 1994

As I reflect back to when I first started noticing anamolies in my body that should have made the alarm go off I recall these in the late summer/early fall of 1993. I can't pinpoint the exact dates or order of observation.

Occasionally while jogging my left foot wouldn't rise on demand. It felt like I had an anvil attached to my leg instead of a foot. One time I had tripped on a curb and fell head over heels while jogging with my wife's cousin. I saw the curb, thought I lifted the foot far enough, but the lazy devil had the last laugh. Bet her cousin thought 'what a stooge'. However it happened so rarely I thought nothing of it.

I also noticed when playing tennis on some days I would miss hit shots I normally made or go whole matches playing even more terribly than my usual consistent terrible. If you play enough tennis, you can sense what “a bad day at the office" feels like. So you try different tactics or play yourself through it. This wasn't like that. I wasn't running in quicksand, hitting balls off the frame, didn't have lazy feet, wasn't peppering the parking lot with stray shots, etc. My strength and timing was just plain off. I felt my feet were moving, my position was good and my stroke was true. I was just hitting balls with less force than trying and in a direction I wasn't expecting. Again, rarely so I didn't think much of it. Must be that bio-rythym thing.

In addition, I had developed a mysterious intermittent cough while talking on the phone. My wife and I noticed it when I would call her from Ft. Huachuca in Sierra Vista, Arizona. Every time I called I'd start coughing now and then. The cough didn't manifest itself at any other time. How's that for bizarre? I thought, a phone allergy. I imagine my wife figured I'd taken up smoking while on the road.

By the Spring of 1994 additional anomalies started appearing. Those observed earlier had either maintained their malice or escalated in frequency or severity.

When I wasn't jogging or playing tennis, I was jumping rope for 30 to 40 minutes. Over the years I had gotten fairly adept at it, rarely missing. But I noticed a profound difference developing in performing this activity. I started missing more and more. Likewise, I started tripping more frequently during jogs. I also started noticing that muscles in my left arm and leg and back were twitching frequently. I reckoned that no matter how good a shape you thought you were in, age rules.

The phone cough became ever more persistent during phone conversations. And another bulbar anomaly appeared. I had developed an occasional swallowing glitch. It was like my throat was stuck in the upright and locked position with no way to get it reopened for a serving of air. Repeated, pressure swallowing finally brought it back. One particular instance comes to mind in May, while conducting a meeting with a few personnel at Ft. Huachuca I paused to take a sip of coffee. The glitch hit and I stood there speechless for what seemed an eternity gulping away to free my throat.

My wife met me in Tucson at the end of this month-long May contract and we went sight-seeing for two weeks in Arizona with no out-of-the-ordinary events. I thought maybe the symptoms had disappeared. No problems eating or drinking and no mishaps jogging. Even held my own hitting ground strokes with a club pro at a resort we splurged for. No phone conversations so couldn't assess that.

The reality quickly returned. After a short stay at home I was back at Ft. Huachuca the beginning of June and with the first call home the nagging phone cough reared its heels much to my dismay. On the second call home the cough was particularly bad. Wafter a lenghty discussion we decided I go see a local ENT doctor. Maybe he'd find a sinus problem, allergy, or something else treatable. After all, the symptoms seemed worse in Arizona. Of course he found nothing.

My tennis game continued to deteriorate. Tripping, coughing, swallowing glitches, and twitching continued. Hmmm....

My wife and I were into recreational biking. We'd pick a 25-30 mile route from either a friend's recommendation or a guidebook and enjoy the better part of the day casually riding through the countryside or suburbia. I noticed some days it was hard to keep up pedaling. My energy level was drained. Come to think of it, I had the same feeling a time or two in the fall last year. This was the same low-energy, low-strength feeling I had playing tennis. Quite strange. I was in great shape and always full of energy. I continued to dismiss it as with everything else to something I ate or that I was coming down with a bug.

In late July, on a Sunday, as I was heading out on a business trip to Ft. Huachuca, we were on the phone with my wife's cousin. He and his wife are both physicians. Hearing the relentless cough and about the other peculiar traits I had picked up during the year, they strongly urged me to see a doctor. I believe they perceived the seriousness of the problem where I hadn't. Even offered to line up doctors in Orlando, Florida where they reside. I promised to check it out upon my return.

When I returned to town 2 weeks later I had an appointment with a cardiologist arranged by my wife while I was away. Why a cardiologist you ask? I have a family history of bad hearts (in the medical sense that is). After hours of testing, including an EKG and X-Rays I returned home to await the results. A couple days later I went back in and the only thing found was some scar tissue in a chamber of the heart most likely due to an adolescent illness, certainly nothing that is cause for concern or that would cause the symptoms I was experiencing.

Shortly after labor day my wife and I vacationed in Seattle and Vancouver Island. While hiking one day I was having a heck of a time keeping my legs going. They felt like lead weights. My energy tank was on empty as well. Extremely bizarre since we were hiking along a flat shore line at the time. My wheels finally started spinning in the area of self-diagnosis. For the first time I started to consider the sum of the parts and not each individually. Muscle weakness, coordination, and fatique. Jogging, jumping rope, biking, tennis, and now simply hiking on the shoreline. Muscle twitching. Coughing and swallowing peculiarities. Could it be multiple sclerosis? Afraid that it could be, I decided to wait until the symptoms were either unbearable or for a last straw to be piled on before seeking medical advice.

Fall 1994

It didn't take long for the camel's back to break.

In early October I headed out on another short business trip to Ft. Huachuca. While jogging I noticed a seemingly endless supply of saliva. I was literally spewing it out every couple hundred yards. This was a new phenomenon. I hadn't altered my routine or eating so I chalked it up to air quality. This wasn't the straw.

This was.

By now it was nearly the middle of October. A few days after returning home I noticed upon wakening one morning my left hand had a subtle numbness, almost as if it was asleep. Nothing unusual I figured, maybe I had slept on it. By nightfall it became unusual as it never went away. I decided to see my family physician; a pulmonologist/internist but shrewd and experienced in all walks of medicine. I had an appointment within a day or two. He listened to my tale of woes, asked me to raise my arms over my head as though he held me up at finger point, then examined my hands and asked how long I'd had that atrophy between the left thumb and pointer finger. Never noticed it. But how could I not? It looked like Crater Lake without water. No knoll when I squeezed my thumb and pointer together. So that's where the feeling I likened to numbness came from. He prescribed a barium swallowing test as well as an appointment with a neurologist at the local hospital.

Definitely neurological. But what? Was my MS thought correct? My wheels spun ever faster. The thing I knew now was that no pill could cure it.

The barium swallow test was scheduled for the following day at 9AM. I was to fast for 12 hours before the test. Not easy for a bloke who usually has downed at least 3 cups of coffee, a muffin or bagel and a couple Reeses Peanut Butter cups by 9AM. I solemnly drove to the lab where it was to be performed. After checking in and putting on one of those fashionable gowns, they had me drink this thick, chalky-tasting liquid. Bad, yet tolerable even to an empty stomach. Then I was asked to stand against a wall with X-Ray plates behind me while they zapped away. Sometimes I think the technicians take pleasure seeing patients grimace and pucker as they attempt to dispose the barium into the oral cavity as I was asked to force down more while they took additional X-Rays during and after swallowing. As expected, I had none of the problems I was experiencing from time to time. This is the typical behavior of automobiles. They never support your complaints when you take them in the shop. I figured they'd find nothing at the neurologist to substantiate my claims either. This conjecture turned out not to be the case.

My appointment with the neurologist came two days later. I showed up at the hospital and was placed in a rather bland lab room. The room seemed over sized in respect to it's content. A stretcher type bed along with a couple pieces of equipment that I had never seen before. One looked like an electrocardiogram (EKG) machine with different tentacles. I would soon learn that this was the infamous Electromyography or EMG. It's not a pleasant test by any stretch of the imagination, but not as bad as you might expect if you witnessed one. In this test they insert a very small needle electrode into a muscle and measure the ability of the muscle to respond when the nerves are stimulated while it is at rest, contracted, and expanded. An oscilloscope displays the wave forms which yield the results to an educated observer. What makes the test unpleasant is that nearly every group of muscles is covered, even the tongue. And some muscles are more sensitive to the intruding needles than others. I also had a few Nerve Conduction Velocity or NCV tests. In this technique surface electrodes are placed at various locations along a motor nerve. One of the electrodes gives a “mild” electrical impulse. The NCV is calculated by measuring the time it takes for the impulse to go from electrode to electrode.

After an hour or so of combined testing, the good doctor called in his resident in to instruct on his findings. She was a pretty gal and a might pregnant. I could tell by her ballooning mid-section. She smiled cordially at me, said “Hello” and then observed as the good doctor ran a couple tests. Subsequent to each the doctor pointed to the screen where I suspect an ominous result was indicated. He'd explain a few things to her in a low voice so as to be indecipherable to me. She didn't have her best doctor's poker face that day. Although she tried her best not to show emotion, the brilliant smile turned topsy-turvy for a brief moment after the first dog and pony show. During the additional tests and conversation she managed to regain her “doctor poker face”. She smiled as she exited the room much like the smile upon entry. Let it be known that this didn't tip me off to suspecting a bad diagnosis, it only reinforced my personal diagnosis. Upon exiting the lab the neurologist said he'd have the results to my doctor in the morning.

Well, my mind went into analysis mode. Which neuromuscular disorder exhibited all these symptoms? Wham, like a brick hitting my head. But of course. I had done a paper on heroes my junior year in high school and one of the heroes was, you guessed it, Lou Gehrig. I started remembering doing some research on the disease for my paper. Symptoms were matching up text book. I hoped I was dead wrong. My doctor called the next day himself and we set-up a time to come the following day. As if I needed reinforcement that it was a bad diagnosis, the fact that he himself called was augmented by asking that I bring my wife with me. It looked as though I was dead right.

We showed up the next day and the overly compassionate nurse in his office said to my wife as we waited in the waiting room, “And you're such a pretty thing”. She meant no harm. We were escorted into the doctor's personal office, as opposed to an examination room, where he gravely pushed across a piece of paper inscribed with those profane three words in combination, Amyotrophic Lateral Sclerosis (ALS). Recall the brick that hit my head while thinking about what it could be, well it bounced off mine and tagged my wife. Subsequent conversation centered on where to go from here. He suggested looking into an ALS center. His parting words, “I hope you have a faith, cause you're going to need it”. That part we had covered. Like two explorers off into a new wilderness we ventured.

We stopped on the way home at Helene's parent's house to tell the tale of woe to her mom. Hard to say how much she understood about the disease and how serious it could be. In any event, the visit was short and to the point.

Now to tell our daughter, my mother and my siblings. I selected the phone as the medium of choice for my siblings. A mom and daughter are too delicate to hear it in such an informal manner. I felt they needed to see my positive face. Timing couldn't have been much better as both their birthdays are toward the end of October so requesting that they visit shouldn't arouse too much suspicion. Asking them to fly down for a weekend from upstate New York to Virginia is bound to raise some though, especially on short notice. The actual disclosure is best described in the book. Since our daughter had a couple semesters of nursing school behind her, she was well aware of the significance of the diagnosis. My mom, on the other hand, could only sense it from the discussion. The rest of the weekend was somewhat somber, but given the circumstances, quite mirthful.

A week later I headed out of town on my first official post ALS business trip. I dreaded having to tell the people at Ft. Huachuca that I had this problem, more so than co-workers and clients at home. I always wanted to be thought of there as the “hired gun” who never lost a gunfight. I guess it wasn't meant to be. Nonetheless, the trip went without incident.

Now to find an ALS clinic or center.

A friend suggested we try the University of Maryland hospital in Baltimore since they had a large neurological center. We called, explained the situation, and set up an appointment in mid November. This proved to be a bad, bad move.

We were told to have a seat in the hall and were given some paperwork to fill out. After a lengthy wait in a hall, I was led into an examination room. Lengthy in my world is more than 15 minutes. In this case over an hour. The examination room was more like an interrogation room, small, dark and lifeless. After another long wait a doctor came in, introduced himself, reviewed the paperwork I had been asked to fill out, asked a few questions, then examined the paperwork I I had brought me from the previous EMG, NCV, etc. A couple additional questions were addressed before he announced that someone will be right with you.

That someone turned out to be two residents that showed up a short time later wheeling a cart with an EMG/NCV machine. Great. They subjected me to nearly two hours of testing, mainly NCV with a couple EMG tests. Remember I said the electrical impulse was presumably “mild” in NCV testing, well these guys must not have read the manual. Every impulse felt like a 110V shock. Several had me nearly jolt off the table. I was basically acting as as a lightening rod. Had I been led to the wrong room? Could they have gone to the wrong room? I feared that maybe they were meteorology rather than neurology residents.

After all that waiting and testing the good doctor came back in after yet another wait and said they could confirm the ALS diagnosis. We anxiously awaited the and part. It wasn't pleasing. He said since they didn't have an ALS clinic there's nothing more they could do. Hello, you couldn't told us this before so I wouldn't have wasted my time. We had explained the situation, diagnosis and mission, when we made the appointment. Next time I'll demand payment for being a training subject. The saving grace was we treated ourselves to a delightful dinner in the Little Italy section of Baltimore. The walk from the hospital through the inner harbor area to the restaurant on that brisk fall evening provided time to vent our frustrations and allowed us joyful dinner conversation. The walk back was enjoyable as well.

What now was the topic of discussion on the drive home. We had heard so many good things about Johns Hopkins that we decided to try it. Plus, we found out, they did have an ALS clinic.

Well, it got off to an even worse start. The neurology center is at an end of the building. It's setup was much different than the one at Maryland. There is an extremely large waiting area, I estimate seating for 50 people or more, fashioned around a receptionist area. The area is bordered by windows looking out into the next building on the backside and walls on the inside. Two open hallways on each side of the reception area allow access to the interior. After registering we were asked to take a seat and wait for the doctor to call my name. We found seats along the outside window on one of the extremities and sat down to fill out the paperwork. Sure enough, doctors would come out of one of the open doorways on the side of the reception area, call a name, the party would join the doctor and they'd disappear down the respective hallway. After a half hour I started to froth at the month. At the hour mark I was ready to leave. If it weren't for Helene's determination to see it through, I would have hit the road. Finally, after nearly two hours, a Dr. Ralph Kuncl came into the waiting area and called out “Steven Nichols”. We were quick to learn that the waiting area is usually quite full by 9AM so your wait could be lengthy if your doctor gets backed up. We would schedule subsequent appointments as first in the day.

After brief introductions he led us down the corridor, hooked a right into a second corridor, and another right into a semi-circle of small examination rooms. We entered the first one on the left. Very neat and well lit, quite unlike Maryland's. This was more like a typical doctor's examination room, at least the ones I'm familiar with. He immediately apologized for the lengthy wait. His first appointment got delayed in traffic and that backed everyone up. My ire was dampened.

After reviewing my history (paperwork) and examining the results of my two previous ventures, we engaged in both technical and friendly conversation. During this discussion he explained that they had to rule out other diseases which mimic ALS symptoms before concluding it was ALS. The EMG/NCV was only a part of the diagnosis. Blood work and a spinal tap were the other components.

I was quick to learn that all neurology centers and neurologists indeed do not accept the testing standards of other centers as he informed me I'd be subjected to another battery of EMG/NCV testing. Thus, I was escorted to another examination room farther down the hall. It was also one in a semi-circle, but void of some of the amenities found in the initial room. Therefore it was considered lab room. After the testing was completed, we met briefly again with the doctor. He informed us he had scheduled the spinal tap a couple days later. As for the blood work, I was to stop by the drainage department to have several vials of blood siphoned from my arm on my way out.

We showed up for the the spinal tap on the scheduled day. Same registration process, same questions. This time he led me straight to a “lab” room which was next-door to the one I had the EMG/NCV testing. The extraction was nearly painless. We didn't see Dr. Kuncl afterwards. Thankfully I had none of the bothersome side-effects that can manifest subsequently. Matter of fact, I went back to work a couple hours later although I did play it cautiously and laid down on the back seat most of the hour plus ride home. We were holding on to the ever minute hope that it was Lyme or another copycat disease.

Well, as they say in the old ball game, three strikes and you're out. Make that times two for me. Three locations, all concluding an ALS diagnosis. Three testing components at Hopkins, in combination, concluding ALS. So in early December I was officially crowned a Person with ALS (PALS). I had a neurologist, a clinical management center, a will and a faith. I was readily equipped for the war.

My neurologist would be Dr. Kuncl. The clinic, Hopkins. The action plan was to meet once a quarter for examination, testing, counsel and needs. The rest was up to me. Make that us.

At the close of 1994 I was getting a little more cognizant of my new life-long partner. I was still able to do just about everything - household chores, work, travel, tennis, jogging, jumping rope, eating, speaking, etc. although the left hand and arm were becoming noticeably flawed in manueverability and overall balance and coordination were waning.

The First Year

Don't remember a particular effort that caused it, but I developed a hernia early in '95. Slightly embarrassed by it, I tried to hide it by having the operation done in Arizona in February during one of my stints. My brother Peter came out to visit me in Sierra Vista a couple days post-op. He was a big help.

In early April Helene and I traveled to Amelia Island, Florida for our first women's tennis tournament. We had a wonderful time.

The progression of ALS for the most part is linear and my measurements at my quarterly Hopkins visits indicated around an 8-10% loss in Forced Vital Capacity (FVC) which verified this hopothesis.. Physically, I was getting a bit more unsteady on my feet. Weakness in my legs and arms, especially left arm and hand, was becoming more noticeable. My speech was becoming a tad slured. But I wasn't giving up my routines. I still jogged (occasionally my face would introduce itself to the pavement, ground or track), jumped rope (what a tangled web I could weave) and hit the tennis ball but playing competitive singles had evaporated totally by mid-summer. Every activity I used to take for granted was showing significant signs of decline as the year progressed. By October I would need a steadying hand beside me to walk and by years end my balance was so bad that I had to resort to a can for walking.

I was still able to drive which meant I could go into the office so work was only mildy affected. I made 4 solo trips to Ft Huachuca between February and July.

Late that summer I learned I had been selected in the lottery to receive the drug Rilutek. The FDA approved that a thousand or so patients could receive the drug prior to it's official approval under the fast access program. Very lucky indeed. At the time the drug was to be shipped, I was called back to Huachuca. My wife took time off from her work to escort me out on what I knew would be my last business journey to Arizona. I had the first shipment of the drug shipped there. All in all, not a bad year.

The Sixth Year

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