The ALS Association

I am humbled by this disease

Stephen F. Columbia, South Carolina

Amyotrophic Lateral Sclerosis, or Lou Gehrig’s disease, is a progressive neurodegenerative disease that affects the nerves that control your muscles. It is a disease that was first identified in 1869 and was made famous when it killed Lou Gehrig 70 years ago. When you are diagnosed with it today, the treatments and the prognosis are largely the same as the one he received. On average, people live three to five years after the diagnosis, with the disease progressively robbing their ability to control their arms, to walk, talk, swallow and eventually breathe. Every 90 minutes someone new is diagnosed in the US. I was involuntarily volunteered to become an advocate for ALS when I was the one diagnosed in one of those 90 minute periods early this year. My hope is that more people become aware of ALS. More people being aware of ALS, means more people working to make sure effort and money are going where they are needed. More money and efforts from non-profits, pharmaceutical companies, universities, the federal government, corporations and individual donors will help conquer this disease that has ceded almost no ground in the past 144 years. If you feel like giving money, give to The ALS Association. If you give, I will be grateful, as will my family, and many other families affected by the disease. But I write this for awareness. I am a 36 year-old economics professor at the University of South Carolina. I have two kids under the age of three and a beautiful wife. For the past year or two, I have noticed my hands getting weaker. It started on my left and then moved to my right. Nothing major, but sort of annoying. Little things I did on a daily basis like buttoning little dresses, unlatching car seat buckles, opening champagne bottles, had become more difficult than they should have been. I was able to help Santa Claus put together a new swing set in the backyard, but only with frequent breaks to warm my hands (in South Carolina mind you). In January, I went to see an orthopedist. He did some tests and referred me to a neurologist, who did tests and referred me to a neuromuscular specialist. Duke has treated my family well in the past (Cara and I were married in the chapel, my parents met there, and both my brother and I had successful surgeries done there) so we went up there in February. We met with one of their neuromuscular specialists, who did more tests and concluded I had a Motor Neuron Disease, and referred me to Duke’s ALS Clinic. The doctor there confirmed that it was ALS. In the past few months, I have been humbled by the number of people who have reached out to me and my family. I signed up for a Walk to Defeat ALS the day before the event in Columbia, and in 24 hours, raised more money than anyone in the state. More than 10 times what I thought I would raise. Humbled. I am humbled by this disease. Humbled by the dedication of the doctors and specialists. Humbled by the devotion of my wife. Humbled by the support of from our family. Humbled by the generosity of our friends. The FDA classifies ALS as a rare disease. In my experience, it affects one out of every one person. In my wife’s experience, it affects one out of every one spouse. In my children’s experience, Mary Adair (age 2) and James (1), it affects one in every two parents. In my parents’ experience it affects one out of three children. The doctors tell me it occurs about as frequently as MS. It attacks roughly two people out of 100,000 a year, regardless of demographics, health status, etc. Roughly one in 300 men will get the disease, and roughly one in 800 women. The most prominent risk factor is military service. But the biggest issue is that there are very few survivors. Right now the only sure way to beat this disease is to go out and get hit by a bus. When you face a diagnosis like this, you want to prepare yourself for a fight. However, with this disease, it strikes me as more of a ride than a fight. There is one drug for ALS, and it extends life expectancy by a matter of months. There are no drugs that I can force myself to take, that will make me miserable, but will beat back the disease. My muscles do not regenerate normally, so if I push myself, I will atrophy more quickly. Hell, eating milkshakes on the couch may be good for me! Every morning I take a bunch of vitamins which may help treat the disease. So I have a disease that may kill me in three years, and I'm "fighting" it with some vitamins. This isn’t bringing a knife to a gun fight, I'm facing a gun fight with a butter knife or the little rectangular piece of plastic they put in a handi-snacks pack. If I could fight this, I would. If I could cut off my arms to stem the spread of the disease, I would do it 127 hours-style myself. Give me the handi-snacks blade! Right now this is a ride with the hope of a fight. Right now keeping a positive attitude and living day to day is the best I can do. Surprisingly this is not as hard as I thought it would be. I have a two year old and a one year old who only know how to live day to day. They don't have plans. Shoot, they don't know until after breakfast whether they are going to daycare or not. I am always thinking about the disease, but I am also living my life. It’s like a window on a computer screen clicked “fix to front.” It’s always there. Never minimized. Only sometimes maximized, but always there. We love to go to the Richland County Public Library. They have wonderful story times and just amazing concerts. However, I have found being there very emotional recently. Sitting on a rug having a great time with my kiddos, surrounded by other families doing the same. But, they don’t wonder for how long they will be able to sit on the rug and catch their kid before she runs off. They don’t wonder whether they will know their children as 6 year-olds or 8 year-olds. They don’t wonder whether they will be around to see their kid go off to their first day of kindergarten. This is the big occasion I hope to make it to. James’s first day of kindergarten. It may be overly ambitious; I hope it is way under ambitious (again, I pray my estimates are off by 10 times!), but this is the event I am targeting. Dropping him off at school or at the bus stop, name tag pinned on his shirt, lunch box in hand, wearing a new backpack, nervous and proud to go off to the big kids’ school. The first step in preparing to take on the world. I want to see that step. This is my normal. I’ll be laying on the ground with James crawling on me, drooling on me, laughing at me, and I will just want to scream in his face, “REMEMBER THIS!” “REMEMBER HOW MUCH YOUR FATHER LOVED PLAYING WITH YOU ON THE FLOOR.” “REMEMBER THE LOOK IN MY EYES.” If or when the day comes that I can’t roll around on the floor, or bounce you up in the air, I hope you at least see the same spirit in my eyes. Mary Adair now recognizes jokes. She will say little nonsense sayings and laugh. When I tell her a joke, she will laugh hysterically if I give her the right prompting. Will I be around long enough for her to figure out my jokes aren’t that funny? Will I be around long enough for her to realize how proud I am of every new trick she learns, of all of the things I know she will achieve? Walking my daughter down the aisle may be a pipe dream at this point, but there are so many things I want to do together in the meantime. Waiting in bed in the morning for her to yell, “Papa come get me” and being able to be the one who goes in and gets her (she will do this til she goes away to college, right?). Taking her to her first soccer game, first dance recital, first concert, first art exhibit im 5 years, in 10 years. Enough to know them and for them to know me. I think it’s only now after having kids, that I truly appreciate how much my parents love me. Two people who lead incredible lives but are most proud of their three children. It was incredibly hard to tell them my diagnosis, and it was only through having children of my own, that I could fully understand the depth of their sorrow in learning the news but also surprisingly cathartic. These are two people that love me down to their bones. Two people I am proud to be family to, proud to be loved by, proud to love. Two people who’s lives I want to be apart of for years to come. It is also crushingly sad to think about the affect this disease will have on my wife. She said for sicker or poorer. I heard her say it. A chapel full of people heard her say it. But I don’t think either of us were anticipating this. Maybe some of our memories we thought we’d have won’t be made. We have made so many amazing ones already, and I know she knows I love her beyond all doubt. But it is hard. Her life won’t be what she expected even a few months ago. She is an incredibly capable, organized person, and I know what ever comes, she will keep our family going. But it is hard to know I won’t be there all of the time to help. There to get between her and Mary Adair when their stubborn heads butt one another. There to calm her down when James responds to an admonishment with a grin and a hug. There on the couch when the kids are asleep, or out with friends, or off to college. I see how it already affects her as I have trouble getting James dressed and changed. She picks up the slack. She keeps the house going, while also doing her job at work the only way she knows how, better than anyone else. She is the one facing the looming burden. Through this she has told me, “I don’t need you to do anything, but I need you.” I don’t need you to do anything, but I need you. This is burned in my head. This is a part of the window fixed to front. She takes comfort in knowing that my sense of touch will likely not be compromised, so while I may lose the ability to squeeze her hand, I won’t lose the ability to appreciate her squeezing mine. My wife is willing to do anything for me as long as I keep breathing. Some day that may become a fight, but I have plenty to make that a fight worth fighting. Again, this is all crushingly sad, but I want to stress it is also surprisingly livable day to day. I have always loved my life, and I continue to love my life. I remember on my Princeton interview, the woman asked me what year of my life had been my favorite. I told her the current one, and I hoped throughout my life that would always be my response. Obviously, I would rather not have this disease, or face an uncertain future, but I love my life. I love my life. I don’t write that twice to convince myself. I right that twice because I mean it wholeheartedly, without any hesitation. I look back at the day I was diagnosed and I cherish crying in my wife’s arms in the Duke Gardens. Snailing snot all over her sweater just like James or Mary Adair do. I obviously wish this was not a part of my life, but make no mistake, I love my life. I am surrounded by people I love. People’s whose lives I am proud to be apart of. People whose lives I hope to be apart of for many years in the future. The hope is that I ride this long enough that doctors will make it a fight. There is reason to hope I will see that day. I am younger than the average person diagnosed with this disease. It has started away from my lungs and my throat and could stay that way for years. My legs are still in good shape. Stephen Hawking didn’t go on a ventilator until 35 years after his diagnosis. 10% of people with ALS live at least 10 years after being diagnosed. I am going to a multidisciplinary clinic staffed by specialists whose jobs, whose careers, are centered around keeping me going. Making sure my diet, my speech, my mobility, my ability to communicate, my ability to teach, my ability to chase rugrats around the house, my peace of mind, are all maximized given the state of my progression. Quality of life matters. It contributes to longevity. When you are facing a disease in which the most effective breakthrough to date extends lives a matter of months, these things matter. I am an extremely rational person. I have some sort of idea of what’s out there. But I know there are many reasons for hope. There is hope that the science will catch up. Right now I ride this disease out knowing that it could take over, and no doctors CURRENTLY could do anything about it. But that won’t always be the case. In 1990 there were 100 research papers published related to ALS. In 2000, there were about 400, and last year there were 1,400. Since the first discovery in 1993, researchers have uncovered more than 25 different genes that may cause the disease. There are increasing numbers of drug trials attempting to beat back the disease. ALS is one of the most promising fields for stem cell research. Two different teams are making huge progress. Phase I safety trials have already been completed, and Phase II trials will begin later this year. These are not hypotheticals. This is real science, with real implementations, with real cells, with real patients, real people, real families, giving real hope to me and my family. Ted Harada gives me hope. My hope is that I will see the day that if I'm willing to let them drill holes in my back and neck and let them inject cells into my spine that I can win. That this disease will switch from a ride to a fight. A fight that I and patients like me, families like mine, can win. I have been utterly impressed by the two ALS doctors I have seen. I know there are smart people working on a cure or a treatment, but I just wish there were more of them. Money helps. For instance, The ALS Association provides grants to post docs or investigators new to ALS working on promising lines of research. Hopefully this gets them addicted. Addicted to the idea of being the one who finds the AZT. The one who finds the chemo, the penicillin. The one who makes the discovery that means when the next guy who goes to the orthopedist, then the neurologist, then the neuromuscular specialist, the doctor will explain to him that he has ALS but as long as he is willing to fight, he can win.

 
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