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The ALS Association

Life Does Not End with an ALS Diagnosis

Tom P. Lawrenceville, New Jersey

On January 11, 2013 after three months of tests, Dr. McCluskey at the University of Pennsylvania gave my wife Karolina and I the news as gently as he could, but there is no gentle way to tell someone they have ALS. I am 48, and we have three children, Sophia who is 10, Emma who is 7, and Zach who is 3. I have worked in high schools for over 20 years, half of that time teaching math and physics, and the last 10 years as the director of technology at the Peddie School, a private high school in Central New Jersey. My wife is also a teacher, and she teaches chemistry at Stuart Country Day School in Princeton New Jersey. The first sign that something might not be right was in late August 2012. For several weeks I had noticed some sporadic muscle twitching (fasciculations in doctor-speak), but in late August I noticed that the twitching was consistent in my right bicep. At this point I was not seriously concerned as a little Internet research showed that most muscle twitching is due to benign causes. I was working hard getting ready for the new school year, not sleeping enough, and drinking too much coffee all of which could easily account for my symptoms. Over the next several weeks I became more concerned as the twitching started to spread. After my primary doctor ran some standard blood tests which came back normal, I made an appointment to see a neurologist in early October. I will never forget that appointment. By this time I knew that ALS was a possibility, but I also knew that there were many other possibilities. However, after the doctor examined me the first words out of her mouth were "Could be ALS." When she saw all the color drain from my face she quickly backtracked and said that it could be other things, but I didn't hear another word she said. That appointment hit Karolina and I very hard. In many ways Karolina took the news harder than I did. From that moment I have always felt that the two of us were facing ALS, almost as if we were both ill. Jesus tells us to "seek first His kingdom and His righteousness, and all these things will be added to you. So do not worry about tomorrow; for tomorrow will care for itself. Each day has enough trouble of its own." Matt 6:33-34. We know what we are supposed to do, to seek His righteousness and not worry about tomorrow, but this is very difficult when tomorrow might bring a diagnosis of ALS. We prayed more than we ever prayed before and over the next couple of weeks God helped us to get much better at not worrying about tomorrow and we resolved, praying for God's help, not to let our life revolve around every test result. One thing many people do not know about ALS is that there is no definitive test for it, and so diagnosing it is a matter of eliminating all of the other things which could cause the symptoms until ALS is the only thing left. In October and November I began the tests: MRI's, cat scans, EMG's, and rare blood tests. In late October I was told I had either cancer or ALS, and so they began looking for cancer. I wonder how many people who are told they could have cancer hope it is true? After two more weeks of testing--a couple of weeks before Thanksgiving--and finding no cancer, we were told by our local neurologist that I had ALS, but he recommended I get a second opinion. After some research we chose Dr. McCluskey at the University of Pennsylvania and are very happy we did. Our first appointment was in the end of November. After spending a lot of time meticulously going over every test with us, he said that while it could be ALS, there were other possibilities and he was not going to give up looking until all other possibilities were exhausted. While we knew ALS was a real possibility, we were very happy to have a doctor who was not going to give up until all options had been explored. I had a few tests in December, but the major tests to come were a spinal tap on January 7 and then a follow up EMG done by Dr. McCluskey on January 11th. We knew that on January 11 we would probably know whether or not I had ALS. At the time we were a little upset that we would have to wait until January for answers. But we now thank God that the diagnosis was delayed. The delay allowed us to have our last pre-ALS Christmas which we thoroughly enjoyed. We were so nervous traveling to the January 11 appointment that Karolina almost fainted in the car, and I could not stop shivering even in the warmth of the waiting room. While we still had hope, we knew that this could be the day we were given the diagnosis of ALS. Dr. McCluskey performed the EMG test and then he prepared to give us his diagnosis. The look on his face told me all I needed to know. When he spoke he told us that the only thing which could explain my symptoms was ALS. You might think that three months of facing the possibility of ALS would have prepared us at least a little for this news, but it did not. I cannot describe how devastated we were. Last summer when we were on vacation Karolina and I talked about how great the next years would be. Zach would be 3 next May, diapers would be behind us, and we could start doing so many more things as a family: family trips, bike rides, lawn games in the back yard, ..., and perhaps Karolina and I could actually start going out on dates again! Not only did this awful disease destroy all of our plans, it replaced them with a future which is terrifying to us, and doubly terrifying for what we fear our kids will have to endure. We knew that somehow we needed to learn all over again how to enjoy life, how to enjoy whatever is enjoyable about today and not to think about the future. How do we begin to do this? How do we begin to live again? We knew the answer had to start with God, for without God we saw no hope of an answer. We do believe in God, and we look to Him to show us the path forward. I myself did not always believe in God. In fact, when I was a freshman in college I was convinced that people who believed in God were either weak minded or were people who could not handle the reality of death. One book which was instrumental in shaking this conviction of mine was "Mere Christianity" by C.S. Lewis. I mention this because in "Mere Christianity" when C.S. Lewis talks about evil, he refers to our world as "enemy occupied territory." There are many wonderful things in this world beginning with life itself and with the love of others, but Lewis is right, we do live in enemy occupied territory, and so we can hate things that happen in this world without hating God. Jesus wept for Lazarus, but He did not then remove all suffering in our world, instead He suffered and died for us. It is with this in mind that we will look to move forward. We will fight this disease every way we can and we still have hope that I will be the ALS exception rather than the norm, but whatever the future brings we will look to God for strength and peace knowing that ultimately Christ's sufferings will bring us victory. At our church our pastor always ends each service quoting Hebrews 13:5, "I will never leave you; I will never forsake you." The minute we began to share our news, we knew that we were not forsaken. The outpouring of support we have received from our family, friends, church, and colleagues at both of our schools has brought us to happy tears many times. My wife and I spent many nights reading the emails and letters of sympathy and encouragement and they were a great source of strength for us. If you know anyone who is going through a similar thing (ALS, cancer, stroke, …) then I encourage you to write them a note, even if you “don’t know what to say.” If nothing else, just tell them that you are thinking of them, it means a lot. Since then the support we have received has been non-stop from dinners to babysitting to having our large lawn cut every week. When I was diagnosed, we scheduled a trip to Disney World even though we couldn't really afford it. We didn't ask for a dime, but spontaneous gifts made the trip almost free! We also have to mention the ALS clinic at The University of Pennsylvania and the ALS Association of Greater Philadelphia. There is no sugar coating a diagnosis of ALS as there is no such thing as an “ALS survivor.” However, our medical team and the amazing team at the ALS Association have helped us to see that life does not end with a diagnosis of ALS. How is it mentally/emotionally to live with ALS? We would be lying if we said we don't struggle at times. It is a difficult reality to wake up to each day. We also know that things will never be normal again; how could they? Nonetheless, there is life after ALS. Our appetites which completely left us after the diagnosis returned after a few weeks and we began looking forward to things again, just on a much shorter timescale. We are concerned about our children, but right now daddy is here so even though our daughters know that I am seriously ill with ALS, that blessed childhood proclivity to live in the present is serving them well. As a family we have all decided to join the fight against ALS and to keep fighting until a cure is found. While it is almost certainly too late for me, we can contribute to the effort to get closer to a cure so that someday ALS will lose its power to destroy healthy bodies and devastate families. I have been to our state capital in Trenton a couple of times, and we went as a family to the ALS Association's Advocacy CConference in Washington D.C. in early May and spoke with several congressmen. We also will be participating in a fundraising bike ride (http://www.als-express.org) in mid June and created a team called "Phelan's Phighters." I have not written about my symptoms until now because I know that some, especially those recently diagnosed and their families, just aren't ready to hear specifics. If this is you, please stop reading now. It has been nine months since I first noticed something wasn’t right and four months since definitively diagnosed. Let me say a few words about how I am doing. A month or so ago someone, very gently, asked me why I was still able to walk. It was a very good question. I would have wondered the same thing if the shoe were on the other foot. To answer it, I have to say a little about ALS symptoms. While most people know that ALS eventually paralyzes, it affects the body in so many ways that it takes a team of medical professionals to properly care for an ALS patient. When I go for my check-ups I am seen by eight to 12 people. ALS also affects each person differently. However, having talked with many PALS (people with ALS), it seems that most of us eventually suffer most of the symptoms. What does differ between us is the order in which we get each symptom, the speed at which each new symptom appears, and the speed at which symptoms get worse. About 75% of the time muscle weakness starts in the arms and legs. This is called “limb onset” ALS. Limb onset PALS lose the ability to walk early in the battle. The answer to the question regarding my ability to walk is that I do not have limb onset ALS. My ALS started in my brain stem and is called “bulbar onset.” Most of my symptoms are not apparent to the casual observer, so I would appear quite normal if passed on the street. Looks would be deceiving. The muscle twitching that started in August, which was my first indication that something was wrong, continues unabated in my arms and upper body and sporadically in the rest of my body. This used to bother me, especially at night, but now it is a non-issue. At this point, it would feel weird if my muscles stopped twitching. In December my hands began feeling stiff. Starting around Christmas my hands and arms went downhill quickly with my right side worse than my left. My fingers are extremely weak. Things that are now completely beyond the realm of possibilities include: putting on socks, shaving with a razor, buttoning my jeans, touch typing, connecting an outside hose, and taking the top off of a Sharpie marker. Fortunately, my thumbs, while not normal, are significantly better. I am writing this on a Nexus tablet using my thumbs. My arms are more puzzling. Some muscles, such as my biceps, still aren't too bad, but others including my triceps and shoulder muscles are pretty bad, especially in my right arm. My right shoulder is becoming quite stiff and can be very painful if moved the wrong way. Things that I can no longer do include: putting on a jacket, drying my back or head after a shower with a towel, reaching an item on a shelf over my head, and walking across the room with a glass of water without spilling it. This last thing is a problem more to do with having reflexes that would make a superball envious than muscle weakness. I think these "brisk" reflexes are also why my arms bounce when I walk. My legs, while not normal, are still quite good. I can definitely feel a degree of unsteadiness and can easily be knocked off balance. I have already fallen a few times and am therefore very careful going down stairs. Catfish Hunter, who had ALS, died from a fall down the stairs. Nonetheless, I am very thankful that I can still walk as well as I can. The symptom that currently bothers me the most is the gradual loss of my voice. I first noticed subtle changes in my voice back in September on a family trip to Duke Farm, but at the time I dismissed it as due to being a little tired. My voice started getting significantly worse in the late winter and has continued to steadily get worse. Last week I had to call Verizon to activate a new DVR, the technician was having trouble understanding me, so I apologized and explained that I had trouble speaking due to a medical condition. Her tone instantly softened and in a very kind voice she promised to speak more slowly and told me that I should feel free to ask her to repeat instructions. Sigh. By this time next year, if not much sooner, I will almost certainly have lost the ability to talk. This, combined with the loss of the use of my hands, will make communication very difficult. Even now, before talking I often find myself thinking, "Is what I have to say worth the effort?" Only communicating the bare essentials: hunger, thirst, pain, would make things pretty boring, and lonely. I confess that this scares me. A symptom close behind the loss of my voice is the weakening of my throat. The throat is a muscle, and mine is dying. Eating is difficult. It takes time and concentration to eat in a manner that keeps food from going down the wrong way. Food going down the wrong way is not only extremely unpleasant, it can be dangerous. Eventually, I will lose the ability to take anything, solid or liquid, by mouth and will need a feeding tube. Unquestionably, the most bizarre symptom I have is called pseudobulbar effect. This affects a minority of ALS patients and can affect patients with other neurological diseases such as Parkinson's Disease and Alzheimer's Disease. In simple terms, I smile/laugh and cry easily, sometimes uncontrollably. What is strange is that I don't think things are funnier or sadder, but I do physically smile/laugh and cry much more easily, almost like an involuntary reflex. There are drugs for this but my symptoms aren't severe so I don't take anything. Along with all the major symptoms above, ALS comes with a host of what I call nuisance symptoms. Some of these that I have been graced with include burning eyes, biting my cheeks, swollen hands, thick phlegm, jaw quivering, and (sorry about this one) constipation. The symptoms described above can have a profound affect on quality of life, but they rarely kill. What usually kills ALS patients is respiratory failure or pneumonia made deadly by a weakened respiratory system. The typical life expectancy for ALS patients is two to five years from onset of symptoms, with bulbar onset patients on the low side of that range because the respiratory system is often affected early in bulbar onset patients. I have already lost respiratory capacity, and every indication is that I am a typical bulbar onset ALS patient.

 
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