Copyright 2007 - Bruce Gaughran
His Seventh Son …
The definitions below will mean more after you finish reading the story.
- Man: (Noun) An adult male person who has a manly character (virile, courageous, competent)
- Manly: (Noun) Possessing qualities befitting a man
- Emasculated: (Verb) Deprived of strength or vigor
I have Kennedy’s Disease. It is an X-linked, adult onset, progressive muscle disorder. I often refer to it as the poor man’s version of ALS. Kennedy’s Disease does not show up until later in life and it gradually erodes your strength by killing off the muscles and motor neurons in your body. Doctors classify it as rare disorder and estimate that 1-in-40,000 men have it. There is no treatment or cure for the disease. Living with the disease reminds me of the paradigm, “Is it more difficult to face life being blind from birth or to lose your sight later in life?” I realize that neither are good options, but is one worse than the other? Is it more difficult to lose something that you had or never to have experienced it in the first place? Realistically, what does it matter? You are still blind ... or, in my case, physically handicapped.
Many people have asked why Kennedy’s Disease is called a “disease” when it is actually a defect in our DNA. When I looked up the word “disease” in the dictionary, it provided a clearer understanding. A disease is “an impairment of health or a condition of abnormal functioning.” Well, that explanation does fit pretty well. Kennedy’s Disease is a condition of abnormal functioning of the androgen receptors that fire the motor neurons. I still like the scientific name of Spinal Bulbar Muscular Atrophy. Now that is descriptive and it does not sound like something contagious. Does my being bothered by the word “disease” just show an increased level of frustration? Why should it matter what it is called? All I know is that I am still a “man,” but I no longer feel manly. I feel emasculated because something was stolen from me that I prize.
Kennedy’s Disease takes its toll on people mentally and emotionally as well as physically. Everyone tends to focus on the physical manifestations of the disease and not much is said about the psychological aspects of it. In some respects, the psychological aspects of the disease are far worse than the physical. As the muscle groups weaken, and basic movements become more difficult, thoughts and feelings also begin to deteriorate. Acceptance is often the most difficult aspect of Kennedy’s Disease. Acceptance is a hard pill to swallow. Even more difficult is admitting to others that you have the disease. It took me years just to tell my family and friends. It took even longer to admit it to my supervisor at work and co-workers. The last thing I wanted was to be considered physically handicapped … someone that could not do his job. I did not want people looking at me as different or considering me less than a “man.”
In my thirties, I occasionally tripped or stumbled when walking on uneven ground. I brushed these incidences off as me not watching were I was walking. More doubts began to surface as other symptoms began to show up. By the time I was in my mid-forties, I realized that I could no longer do certain things that I enjoyed. One such activity was hiking in the mountains with my wife. We loved being together surrounded by nature. We often hiked several times a month. Going uphill was difficult as my muscles weakened, but it was going downhill that became dangerous. My quads could no longer hold my weight. The longer I hiked, the weaker the leg muscles became. I fell many times in those years. Fortunately, my injuries were relatively minor and I always managed to get up and finish the hike.
Gradually, I had to give up more activities that I enjoyed. Racquetball and tennis were two sports that I was good at and I enjoyed playing. Eventually, I just could not safely play either of them. Then, in my mid-fifties and after several falls and a few broken bones, I realized that even stairs were out of the question. I now needed an elevator if a customer, warehouse, or mill had offices on the second floor. A few years later, just walking with co-workers or customers became a burden because I could not keep up with them. At first, I just gave some excuse and tried to bow out gracefully ... bad knee, recovering from an injury, etc. Eventually, I had to tell the truth and just say, "I sorry, I can't do it." Every time I said, "I can't," I thought of my father telling me that "can't never could do nothing."
Even more frustrating was the change required as a grandparent. How does one explain to your grandchildren that you cannot pick them up, play with them on the floor, or walk to the park to play with them on the swing set or slide? Children are extremely accepting, but they do not really grasp the reasons why. Fortunately, reading to them was one of the activities that I could still do and they both enjoyed the bedside stories.
The loss of independence is very difficult to accept. A couple of years ago I realized that I could no longer just hop in the car and go somewhere. Every trip became a logistical consideration. Are there steps or curbs that have to be navigated? Is the rest room handicap accessible? What is the weather forecast? Is the walkway paved, rock, or dirt? The list of questions continues to grow as the disease progresses and my negative experiences increase.
For over thirty years, I just took my body for granted. I just assumed that when I needed to walk, run, lift, climb, or whatever, the appropriate muscles would just respond accordingly. It did not take any thought; the muscles just knew what to do. There was always synergy between my central nervous system, motor neurons, and muscles. As the disease progressed, however, I found that I needed to tell, or perhaps even will, certain muscles to function correctly. Unfortunately, even that does not always work today.
Several of the muscle groups are no longer strong enough to perform the necessary task. I find myself having to compensate, or perhaps even cheat, in order to perform basic movements like standing up from a chair. Additionally, because some of the signals no longer reach the appropriate muscles at the correct time, certain muscles will fire at the incorrect time. This causes muscle spasms or twitching that are often noticeable to others. Even stranger is when a motor neuron sends a signal to a muscle to perform a task that is already completed. One example that has happened several times is when I hold a glass of water. Occasionally, the wrist will just turn over as if I am performing the drinking motion function. This happened several times at business dinners while I walked around visiting with people. I could be just talking to someone when the wrist just turns inward and dumps the contents of the glass on the floor or on my shoes. It is embarrassing and difficult to explain.
Observing the gradual loss of the use of my hands and legs is almost unbearable at times. I often try to do too much and occasionally end up hurting myself. Yet, I cling to that hope that through some divine intervention, I will be able to do something today that I could not do yesterday. Call it stubbornness or denial; it exists because I still have hope. I repeatedly need to prove to myself that I can no longer do something as simple as walking or bathing. Loved ones cannot seem to understand why I still want to try something one more time even though I have failed several times before. I am just not willing to accept the inability to perform these simple tasks. Will I ever just give up? No way, because “Can’t never could do nothing.”
Over the last ten years, the disease has also taken its toll on my face and throat muscles ... those muscles controlled by the bulbar region of the brain. Initially, I started choking while I was sleeping. In the middle of the night, I woke up gasping for air and could not clear my air passageway. Talk about something frightening for my wife. A few years later I found it more difficult to swallow certain foods. If I am not focused on chewing and swallowing, food will lodge in my throat. In recent years, my jaw muscles have weakened to a point where they cannot always hold the lower jaw in place. If I eat a bagel, for example, the pressure caused by chewing on something hard forces the lower jaw to pop out of joint. The initial pain is not too bad, but for the next several days I have to eat with my hand pushing on the lower jaw to keep it in the socket. Occasionally during the night the lower jaw will just fall out of its socket. Meanwhile, the facial muscles continue to weaken and collapse.
Over the years, I have fell dozens of times. Almost all of my falls in the earlier years were forward as if I stumbled. Fortunately, only a few of those falls were serious. About six years ago, however, I was stepping into the house from the garage when my knees collapsed. I fell backwards, cracked my head on the concrete floor, and went out like a light. I suffered a concussion and had some bleeding on the brain. Fortunately, the only long-term damage was to lose a portion of my sense of smell. Then two years ago, my left knee buckled twice in three months. Both times I went straight down with all my weight collapsing on the left knee joint and lower leg. The first fall fractured the left fibula and ankle. Within a few weeks of receiving a clean bill of health, my left knee buckled again. This time the left fibula and tibia (the weight-bearing bone) fractured. The doctor told me I could not place any weight on my left leg for three months. For a person with normal leg strength, the healing process would be difficult. For someone with Kennedy’s Disease, whose leg strength is not half of what it should be, the three months of inactivity was an eternity. I have never had a worse experience.
Not only was it difficult for me, it was even more difficult for my wife. She had to be there for me every day. The only time I could leave the house during that time was in a para-transit vehicle specifically designed for a wheelchair. During one of my doctor visits, he showed me the x-ray of my left leg and foot. My bones looked like Swiss cheese. He said I had severe osteoporosis and another fall could crush the bones. I made the decision that day to give up walking and begin using a wheelchair. It was a difficult decision, but I could not put my wife through another six months of hell like this again.
Pain ... what can anyone really say about pain? What is pain to one person is only an ache to another. I had to learn early on to live with the aches and pains associated with the disease. When I was in my twenties and thirties, I experienced severe muscle cramps in both legs. Many nights I woke up with a painful cramp and had to massage the muscle until the pain subsided. In the forties, the cramping miraculously disappeared. However, it was replaced with deep muscle aches. This usually happened when I exceeded my limitations for the day. At the same time, I begin to experience extreme fatigue. It often took a day or two to recover. In recent years, I am learning to live with muscle aches almost every day. In addition, several days a month I wake up with deep aching pain in specific muscles. It is almost as if I can feel the muscles dying. Fortunately, I must have inherited my father’s tolerance for pain because I can normally live with the nagging aches and pains. Occasionally, when the pain is too intense and I cannot sleep, I have to take an Ibuprofen.
Along with the aches and pain, my body now reacts negatively to cold temperatures. Anytime the temperature is below 50 degrees, I lose the use of my hands. Sometimes even gloves do not help. My legs and feet are also constantly cold and do not always function properly. I wear insulated underwear, thermal socks, and gloves from November through March. Occasionally, I have to wear gloves in the house just so my hands can function better. I have also lost most of the feeling in my feet and some of the feeling in my hands. I do not always notice hot and cold or cuts and scrapes. I have burned my fingers and not known it until I saw the blister.
As the inability to perform basic and often simple tasks increase, frustration and anger often become side effects of my fight to remain independent, mobile, helpful, and a “man” in my own eyes. Resignation, especially at first, was something unheard of. Meanwhile, since there is no treatment available, the wasting away continues.
As my muscles weaken further, so does my self-esteem. I begin to wonder if I am more of a burden then a blessing to my wife. My perceived manhood ... what is important to my belief system ... is now being challenged every day. Instead of being the protector of the family, my wife is now trying to protect me. A few years ago, another challenge showed up and this one is even more difficult to handle. It is not bad enough that the disease has robbed me of my strength; it now has taken away my libido. And, just like everything else with this disease, it slowly takes away those special moments of intimacy that I once shared with my wife. It is impossible to walk hand-in-hand with my wife on the beach or to sit on the couch together. It is difficult to give a good hug or hold and comfort my wife from a wheelchair. It is even more difficult to kiss, snuggle, and caress my wife in bed when I cannot hold my head up for more than a few moments and it is difficult just to roll over. Rolling over in bed is something similar to watching a beached whale trying to free itself ... plenty of action, but very little movement. Talk about romantic.
Everything that was important to me is slowly disappearing from my life. Every emotion and belief is being challenged. I find myself asking, “Can I bear my soul and my pain and still be considered a man?” As I feel more isolated from the world around me, I begin to wonder what my wife really thinks of me. I know she still loves me, but can she continue to love me as I become even more of a burden. Or, will she just end up taking care of me out of some sense of responsibility … the “in sickness and in health” portion of the vows? At other times, I become frustrated because I feel that I am no longer needed. If I am not the breadwinner, the lover, or “the man of the house,” then what am I?
As a parent, I never wanted to do anything that might harm my children. Today, and every day, I live with the guilt that I passed the defective “X” chromosome on to my daughter. In addition, there is a chance that the defective gene was also passed to my grandchildren. Hoping for a treatment or cure, whether medical or miracle, is what I live for today. In desperation, I look for alternatives with a willingness to try almost anything that might help bring about a slowing or remission of the disease. I am looking for anything that might help my daughter and her children.
Living with the disease is often just as difficult for caregivers, family, and friends. Yes, I need support through times like these, but unfortunately, I often look upon it as “mothering” or even “smothering.” The primary care-giver is occasionally the unintentional beneficiary of my anger and frustrations. I do not mean to lash out, but sometimes I just need to vent and she is often just the person who happens to be in the wrong place at the wrong time. I realize there is no excuse for my anger, but I just lose control. Afterwards, I feel guilty for hurting her. I know she never signed up for this when she married me, but for some reason she continues to put up with it. As the outbursts continue, the guilt that follows leads to even greater frustrations because I am hurting the one that I love so much. At times like this, it seems I am caught up in a vicious never-ending cycle of self-destruction.
Someone once said that everyone is afraid to die. In my case, I am not so certain that is true. I have been exposed to many dangers in my life including several life-threatening moments in
. Death never seems to concern or consume my thoughts. I believe I can deal with the prospect of death, but find that the future unknown of living with this disease is much more frightening and much more difficult to face. Vietnam
Recently I was watching “Saturday Night Lights.” The high school football team was down ‘26’ to ‘0’ at the end of the first half. The coach ended his halftime pep talk to his players with the following. “Every man at some point in his life is going to lose a battle. He is going to fight and he is going to lose. What makes him a man is that in the midst of the battle he does not lose himself.” For me, this battle is not over, but I need to find my ‘Self’ once again.