But a month went by, and he wasn't getting better, so my aunt and I convinced my uncle that he needed to go to his doctor to rule out a stroke. After undergoing routine tests that all were negative, his doctor sent him to a neurologist. After another battery of tests, the neurologist called my Uncle and Aunt into his office. He said, "Herman, you have Bulbar ALS. I suggest, you get your life in order and spend some time with your family". That was that. My aunt couldn't get over the coldness of his tone as he told my uncle his death sentence. Having a neurological problem of my own, I knew that getting a proper diagnosis in neurology was very hit and miss, so I suggested going to Columbia Presbyterian Medical Center to get a second opinion.

After careful testing, with multiple teams of doctors coming and going from the examination room, the diagnosis came back. My uncle Herman had Bulbar ALS. This was the worst of all possible outcomes. Bulbar ALS starts in the throat and kills the quickest. The doctors at the hospital told us that their job was to his extend his life, and to make him as comfortable as possible. And so came the routine of taking my Uncle and Aunt to and from the hospital on Mondays and Thursdays, which were clinic days. With each visit my Uncle was a little bit worse. He became harder and harder to understand as his tongue and mouth became paralyzed. This affected his swallowing, so a feeding tube had to be put into his stomach so that he wouldn't choke to death. The muscles in his arms developed twitching, known as fasciculations, which is always present in ALS and then began the muscle wasting, first his arms, then his legs. But whenever I asked him how he felt, when he could speak, he would say fine and smile and when he lost his speech, he would give me thumbs up.

But as the Mondays and Thursdays passed, he quickly deteriorated. And I began to notice that the doctors seemed very laissez faire about my uncle's condition. They never volunteered any information without us prompting them to do so. We had to ask about the drug riluzole (Rilutek), to which they replied that they could put him on the drug, but it would only help prolong his life about 3 months. Then my aunt and I asked about any drug trials that my uncle qualified for. They told us about various drug studies that used existing drugs, so I started to do my own research into other trials. During my research, I learned much about ALS, more than most.

It has been 134 years since Dr. Charcot's discovery of ALS, yet we are no closer to understanding the disease. What we do know is that ALS is a motor neuron disease. For unknown reasons, ALS causes the death of the motor neurons, the nerves that make muscles move. A person with ALS will progressively lose the use of their limbs, throat and lungs. Most people will die of suffocation. According to the Baylor Institute of Medicine, approximately 150,000 people will get ALS every year. It is estimated that as many as 30,000 Americans have the disease at any given time. The rate of ALS is 2 out of 100,000 people, or about 15 new cases a day or 6,000 new case a year. Gulf war veterans have a rate double that of the civilian population. No one has identified the reason why. There are increasing numbers of ALS clusters in the United States. For instance, according to Christine Wallgrenin of the Boston Globe, the rate is 9.5 per 100,000 in Plymouth County and across the Southeastern part of the state of Massachusetts. The number of ALS clusters is growing. No one knows why. The average life span of someone with ALS is 2 to 5 years. Because of this short life span and the quick progression of the disease, there is little patient advocacy. The patients and their families are consumed by the day-to-day challenges of living with the disease. So there is little in the way of ALS awareness, walk-a-thons, or spokespeople. The disease just fades into the background. Also there have been no large endowments for ALS and minimal government funding for research. The two biggest advocacy groups are the Amyotrophic Lateral Sclerosis Association (ALSA) and Muscular Dystrophy Association (MDA), both of which fight over the scant funding they get.

According to Ken O'Donnell, in his article "Advocate for a Cure for Amyotrophic Lateral Sclerosis" and a member of Patients Like Me, which tracks ALS patients, there's an additional reason for the lack of progress in finding a cure. "The drug companies want more profits; their motivation is greed. Since it is estimated that there are 30,000 ALS sufferers alive at any one time, they consider ALS to be an orphan disease (defined as fewer than 200,000 sufferers). The drug companies focus their research primarily on the major killers of the US population, which are heart disease, cancer, and AIDS. As a result, much progress has been made with all of these diseases. The difference is that there is major profit potential in curing AIDS, heart disease, and cancer. Unfortunately for ALS sufferers, drug companies find it hard to calculate a return on their investment in finding an effective treatment for ALS. This is the main reason that we only have one drug approved for ALS (Rilutek) in the last ten years. The drug companies will not be investing in research for ALS unless they uncover something that occurs in other major neurological diseases that would have a common use for ALS. Thus, we cannot count on these drug companies to change their research programs to focus on ALS." This leaves the financial burden of ALS research on the federal government.

On the Federal level there has been some movement made by the ALSA. According to the Pittsburgh Post-Gazette, the National ALS Registry Act, which had already passed the House last year, would have appropriated $5 million to fund the collection of data on every person diagnosed with ALS each year and an additional $5 million to continue studying why armed service veterans contract ALS more often than the general population, which the ALSA hopes will help find environmental causes for the disease. So ALS will receive $10 million annually as opposed to AIDS, which gets 1.4 billion dollars annually, according to the Nationl Center for Political Analysis.

This past July 4 will mark the 75th anniversary of Lou Gehrig's famous farewell speech at Yankee Stadium, and since then more than 600,000 Americans have shared Gehrig's fate. There have been no significant developments toward finding a cure, yet the rate of ALS is slowly growing. The ALSA will be starting a PSA featuring Angela Lansbury, whose sister died of the disease and is working to increase government funding for research, for this soon may no longer be an "orphan disease". Though this is a beginning, it is not enough. ALS knows no gender, race, creed, or age. It robs people of their bodies, and their dignity, while leaving their brains fully intact to witness their own demise. It robs families of their loved ones, and often their finances, while giving them no hope and it's been going on for too long.

I loved my Uncle Herman. He was a kind and decent man, who loved mankind, loved animals, loved pretty girls and most of all, loved his family. He never called me his niece, but his nice. And on that early spring day that we buried my beloved Uncle, I walked with my Aunt Phyllis over to my cousin's grave. I heard her say in a whisper, "Paul, I brought your daddy to keep you company". I could barely contain the tears. It was then I promised myself that I would do my part to make my Uncle's death count. Please donate to this very important under-funded disease. The life you save may be someone you love.