Hello, I live in Virginia.
My childhood was very normal starting in New York and then growing up in Virginia. In High School I ran track, my best events being the High Jump and High Hurdles. After graduating in 1969 I went to college for 2 years. In 1971 I joined the Air Force which gave me the opportunity to travel to Texas, Mississippi, Florida and 2 years near Stuttgart, Germany.
After getting out of the USAF in 1975, I went to college in Florida for 3 years. Before starting a job I wanted to do some more sailing, which I had learned a lot about while in Florida. I found a 53 foot sailboat headed for Oakland, California and went along as First Mate to help deliver the boat by way of the Panama Canal and ended up being a 6 month trip. Next I was Skipper on a 55 foot sailboat to Cabo San Lucas, Mexico for another 6 months. After one year of sailing I decided to settle in San Francisco.
In California I learned to Snow Ski and would ski 25 days per season mostly in the Lake Tahoe area. Even joined a Ski Club and did some amateur downhill racing. The ski club is where I met my future bride, Sue. We were married in August of 1988.
In the fall of 1988 I was doing aerobic exercise to keep in shape and this is where I had the first symptoms. I was not able to hold my left arm straight out at shoulder level, could only raise arm to waist high (45 degree angle). I decided to see my Doctor and he referred me to a Neurologist. After some tests and finding some muscle atrophy in my shoulder, arm and hand he said I had ALS. My response was "no way!" After a period of about a year that diagnosis was changed to Motor Neuron Disease, a real catch-all label! My symptoms didn't change much and I was still skiing 25 days a season to the amazement of the Neurologist. In the Fall of 1998 (10 years later) my legs started getting weaker and my new Neurologist suggested I be tested for Kennedy's Disease. While waiting for the test date I got a call from my brother on the East Coast saying he had just been diagnosed with ALS. I told him about my upcoming test and suggested he ask his Doctor to test him for Kennedy's Disease, he did and actually got his results before I did. Positive for Kennedy's Disease for both myself and my brother. My androgen receptor gene has 50 repeats and my brother has 47 repeats.
Since 1998 my new sport is "extreme stair climbing" and we have added a chair lift to our home. My other symptoms include weakness in my arms, sometimes even pouring a gallon of milk is a challenge. Weakness in my grip making it almost impossible to open containers that require me to squeeze and turn the top. If not careful I almost choke on food or drink due to swallowing difficulties. I sleep on my back using a very flat pillow so as to keep my air passage as straight as possible. My butt hurts most of the time, even more after sleeping or sitting in one position awhile. I need a minimum of 8 hours of sleep per night, but many nights do not get enough sleep due to the pain. After about 8 hours the pain keeps me awake so I get up. I can only lay on my side for several minutes at a time due to pain in my hips when I lie on them, but it gets me off the butt for a few minutes. Also my left shoulder hurts when lying on it due to the atrophy muscles. When sitting for a long drive or flying I take my little pillow to sit on, it sure helps a lot. I have had some falls, mostly in the early days before I was aware of my limitations. Now I 'm a lot more careful. As my sister says "gravity is not your friend".
I've also been finding more about my Grandfather who was diagnosed with ALS and then lived 25 years after that diagnosis, passing away from cancer. He would be walking along and just fall to the ground. People that saw him would yell at him calling him a "drunk" or just walk around him and ignore him. This is according to his daughters (my mother and her sister). What he had was obviously Kennedy's Disease, however in the 1930's, 40's and 50's Kennedy's Disease was unknown. Dr. Kennedy did his research and made his observations in the late 1960's. We have found that all three of my Grandfather's daughters are carriers since several other cousins have tested positive or have definite symptoms of Kennedy's Disease.
Thanks Terry and Susanne for the Kennedy's Disease Association web site and the chance for those of us with Kennedy's Disease from around the world to share our experiences.
I’m finally back to update my story after about 5 years.
My love for sailing started in 1975. I stopped sailing and racing about 1992 as I was doing other things in my life and put sailing on the “back burner”. Then fast forward to 3 years ago when I found a disabled sailing club in San Francisco. The club name is Bay Area Association of Disabled Sailors, BAADS for short. The web site is BAADS.org. The first year I sailed a couple of time on the 30 foot sailboat. It wasn’t the same kind of fun I remembered as when I was able to fully participate in all of the aspects of sailing. Then about two years ago the club got 8 Access Dinghy’s designed and built in Australia for disabled people. That is when sailing really became FUN again and now I am there almost every Saturday. The club added 2 more dinghy’s about 6 months ago. Now I am Co-Dock Master for the dinghy program and my duties include supervising and rigging the dinghy’s on Saturday mornings for sailing, taking people sailing, giving lessons plus racing. I am able to get myself in the dinghy and sail without assistance. When it comes to getting out of the dinghy I wear a harness and get a hoist from one of the 2 lifts located on the edge of the dock. The end of May 2007 BAADS sponsored the National Regatta in San Francisco.
The club has 4 larger sailboats ranging from 20 to 36 feet with the recent donation of a 36 foot Islander. Since I already had my ASA certification I was checked out on the club boats and now I skipper for the Sunday big boat sailing. It is different and challenging to skipper a boat with different able body (AB) crew members. I have to give clear instructions as to what needs to be done and when so that we have a safe and fun sail. We usually have about 6 people on the boat including myself, 2 able body people with some sailing experience and then 3 other people including handicapped and caregivers. These Sunday sails usually last 3 to 4 hours then we meet in the yacht club to talk about our day of sailing.
I am still working full time for a construction company. The biggest difference is that I very seldom go to a job site. I am an estimator and project manager which I do from the office. The job is challenging and everyday is different which keeps the interesting. As for physical changes I have made in my life in the past 6 years. First I had a difficult time climbing stairs so I purchased and had installed an electric chair lift to get me up and down to the second floor of my house.
I started using a “loaner” electric scooter from the MDA loaner closet about 5 years ago. Two years later the MDA purchased a new electric scooter for me. I use the scooter for places like amusement parks, larger stores and malls, the marina and docks and any other place that is more than one city blocks of walking would be required. For shorter walks and around my house / yard I have a 4 wheel walker with a seat. It works well so that I can continue to do small chores around the house.
I also had a carpenter install a ramp outside the back door (2 steps) and inside the garage to the house door (3 steps). I even put a 3 foot folding aluminum ramp for the landing inside the front door. The step is only about 6 inches high but I have to use it many times a day and the ramp is much easier. Thanks to the ramp I am still able to use the hot tub in my backyard about three times each week. The heat feels very good on my muscles, just wish I could stay in it for hours!
Anything that makes life easier I have done and will continue to do. I look at that even able body people use things such as cars, bicycles, golf carts, riding mowers, etc to make their liver easier so why should it be any different for me. I just happen to choose things that help me because I have KD.