Denial … Shock … then Anger
"I’m sorry, what did you say?"
The doctor took off his reading glasses and paused for a moment before replying. "I said that the preliminary test results are in, and they indicate that you have Spinal Bulbar Muscular Atrophy. It is also known as Kennedy's Disease. It is a relatively rare ‘adult onset’ muscular dystrophy disorder that ..."
My brain just wasn’t digesting what the neurologist was telling me. "Doctor, I'm sorry to interrupt. Did you say muscular dystrophy? That can't be right … the tests have to be wrong. I just have a little weakness in my legs. Are you certain this isn't a pinched nerve or something? I certainly don't need a wheelchair or anything like that. Besides, I am 47-years-old, not some child ... like Jerry's Kids."
"Let me reemphasize, Mr. Johnson, these are preliminary results. Now that we have narrowed it down, we would like you to take one more test. It is a simple blood test; nothing like you went through with the EMG. We send the blood off to a special lab for a DNA analysis. It might take three weeks to get the results back, but the test will conclusively confirm whether it is Kennedy's Disease. Before we take you blood, I would like to take a few moments and explain what the disease is and how it will affect your life.
“As I mentioned earlier, Kennedy's Disease is a relatively rare muscular dystrophy disorder. It is often misdiagnosed because many of the symptoms are similar to other neuromuscular disorders. The disease is ‘X’ linked, meaning that a defective ‘X’ chromosome is passed from a female carrier on to 50% of her sons …”
His explanation was almost too much to bear. Somewhere in the middle of the neurologist’s explanation, I again zoned out. Occasionally, I heard words and statements; but more times than not, my mind just ran wild with other thoughts – What if … Wheelchair bound … Am I going to be a cripple or even worse, a vegetable … How will Carey react to the news. I shook my head and tried to refocus on the doctor’s explanation.
“Now, I am certain you have all kinds of questions, Mr. Johnson, but why don't we hold off on them until we have ruled out all other possibilities, okay?"
Still stuck on what the doctor said initially, I asked, "Spinal Bulbar what?" I wiped my damp, trembling palms on my pants. I was still a little dazed, perhaps in shock, and not really focusing on everything the doctor was saying. What is Carey going to say when I tell her? What am I going to tell our children? Thoughts continued to run through my head non-stop. I couldn't shut them off.
"Spinal Bulbar Muscular Atrophy or SBMA for short. If it is SBMA, there are some positive attributes to this disorder when compared to other similar neuromuscular diseases."
I didn’t want to hear about positive attributes. I had just one question: “Answer me this, Doctor, is there a cure?”
I knew his answer was going to be bad when he glanced down at his hands and then shook his head. “No, Mr. Johnson, currently there is nothing we can do for you. On the bright side, however, researchers have narrowed down the potential causes of the disease. There is a good chance that sometime within your lifetime there will be at least a treatment for the disease.”
“Sometime within my lifetime?” Now I was really in shock. My mouth was so dry I could barely get out the next question. "You mentioned something about the positive attributes - what are they?"
"Well, first and most importantly, the disease progresses relatively slowly. Second, your symptoms will ..."
I didn't like his choice of words so I interrupted again, "What do you mean by ‘progresses slowly’? How much worse will it get?"
The neurologist took my constant interruptions in stride, perhaps because he was used to giving out bad news. "Yes, it is a slowly progressing neurological disorder. What that means is that over time you will continue to lose strength in certain muscles groups – at first in the hands and feet and then slowly progressing up into the arms and legs. Now, Mr. Johnson, the key words you need to remember are: ‘over time’. You should have several good, productive years in front of you.
"Another positive attribute of this disease is that most men afflicted live into their late 60’s and early 70's. One major concern, however, is that your lungs will eventually weaken to a point where you become very susceptible to pneumonia. In fact, more deaths are associated with pneumonia or serious falls, than with the disease itself."
"Okay, Doc, I appreciate your patience, but I am still trying to catch up. You said I needed to have another test, a blood test, I believe, to confirm that I have this disease. Okay, let’s say I take the test. What if it isn’t Kennedy's Disease? What else could it be?" I knew that I was grabbing at straws – some last bit of hope that this was another misdiagnosis.
"Well, I hate to even mention the possibility, but until we see the results of the test, I am not ruling out ALS - Lou Gehrig's Disease, or even possibly Spinal Muscular Atrophy, it is another ‘adult onset’ disorder."
Just the words ‘ALS’ made me go numb. "You've got to be kidding! Is there really a chance I have ALS?" My God, what am I going to tell Carey?
"Mr. Johnson ... Mr. Johnson, please listen to me. The chances of it being ALS are very slim. A few years ago, many men with Kennedy’s Disease were misdiagnosed with ALS and were told they had only a few years to live. Thankfully, the discovery of the DNA test has significantly reduced the number of misdiagnoses. That is why we must perform the blood test. It will prove, or disprove, the initial findings. Besides, many of your symptoms are bulbar related. A person with ALS doesn’t normally show symptoms that are ‘associated with the face and throat’ this early.
“Now, before we discuss any other possibility, I am going to recommend that you have your blood drawn before you leave the office this morning. As I mentioned, it will take two to three weeks to get the results back. Once we know for sure, we can begin discussing what this disorder means to you and your family. If it is Kennedy's Disease, you might want to consider seeing a genetic counselor."
I sat in the chair not saying a word. Genetic counselor meant ‘genes’. Was there a chance I passed this on to my children? After what seemed like an eternity, the doctor's voice came through the fog. "Mr. Johnson, did you hear what I said?"
Wanting to ask, but afraid to know the answer, I nodded my head, but the rest of the body remained motionless. Eventually I said, "Yes, I think so.” It took a moment before I could comment further, “You said something about taking the DNA test today. Sure ... whatever ... let's get it over with."
The rest of the morning was just a blur. I don't remember much of anything. I am still amazed I was able to drive home without causing an accident because my mind was definitely not on driving. All I knew was that somehow I had to find a way to break the news to Carey and the children.
As I neared the house, my trepidation increased to a point that I knew I couldn't face her right now. I drove right by our house and continued down to the corner. At the stop sign, I sat there for some time wondering where I could go. I knew I had to tell Carey sometime, but I just wasn’t up to it now. Maybe later … perhaps after the results of the DNA test come back. When my hands slipped from the steering wheel, I realized how wet they were. I pulled out my handkerchief and wiped the wheel down. Before tucking it back in my pocket, I also wiped the perspiration from my forehead and hands.
An irate driver brought me back to reality with a long blast of his horn. Not knowing where I was going, I turned left on Beach Boulevard and hoped the driver behind me would turn right; thankfully, he did. I saw a sign for New Brighten Beach and that helped make up my mind.
Twenty minutes later, I parked the car on the shoulder and slipped off my shoes and socks. With rolled up pants legs, I started walking down the path to the beach. I glanced at my watch and saw it was nearly lunchtime. Taking out my cell phone, I speed-dialed the office. When Shelly answered, I was brief and somewhat evasive. I let her know that I would be unavailable for the remainder of the day. I also asked her to call my wife and let her know that I would be home in a couple of hours. When Shelly asked if I was okay, I told her I was fine. She took the hint and didn't ask any more questions. I told her I would see her in the morning and quickly hung up.
The smell of the sea air helped clear my head. I needed this walk. I picked up a stick and walked down the path swinging it back and forth, occasionally hitting a shell or stone. My thoughts screamed out, begging for an answer to the question of why this was happening to me. I suddenly stopped, looked toward the sky, and asked, "Why me, Lord? What have I done to deserve this?"
Not being particularly religious, I didn’t expect an answer, and my expectations were fulfilled. I tossed the stick off to the side and walked out on to the beach. It was a gorgeous day, and if it had been any other day, I would have had a smile on my face. The sun warmed my body and the slight ocean breeze mussed my hair. I walked down to the water's edge and stood in the wet sand, allowing the surf to run across my feet and ankles. The water was numbingly cold, but I didn't care. It made me feel alive.
I kicked the sand and stared out toward the horizon. A gull flew overhead, and I thought this would probably be the day it would crap on me. I shook my head knowing that I was just feeling sorry for myself. At this point in time, however, it was either that or anger - perhaps even rage. 'Why me' flashed into my mind again as I kicked the sand even harder, this time almost losing my balance.
I thought back, maybe sixteen or seventeen years ago, when I first began to experience some of the symptoms. At that time, I didn't realize that’s what they were. It was around then that Carey commented my legs were twitching during my sleep. Just a few weeks ago, the doctor called the twitching ‘nervous leg syndrome’. About the same time, I began having 'Charlie Horses’ - those painful leg cramps that have to be massaged until the knot in the muscle dissolves. Who would have ever thought these were the beginning signs of Kennedy's Disease. At that time, I was in the best shape of my entire life. I played racquetball several times a week, lifted weights every other day, played some tennis, and jogged regularly. I felt strong, healthy, and happy. Life couldn’t be better; or so I thought.
A couple of years later I began having a little difficulty with my balance. When playing tennis or when walking in the grass, I would often stumble and occasionally fall. A year or two after that, I began to notice a weakness in the legs when hiking or walking long distances. None of these signs by themselves drew much attention, but accumulatively they should have been a giant red flag.
Instead, I ignored them and kept on telling myself that this was just part of the aging process. I suppose you could call it a form of denial, but I used these moments of weakness as an excuse to exercise more often and with more intensity.
If I just would have gone to the doctor then, it might have made a difference, or so the neurologist explained this morning. It seems the more intense the exercise, the more damage I did to my muscles. Who would have ever thought exercise was bad?
By the time I reached my early 40's, I pushed myself harder than ever. I also noticed more dramatic signs of weakness. When my G.P. noticed something unusual in my blood work-up, I ignored his advice to see a specialist. About the same time, Carey and I moved to Seattle and started hiking in the mountains. As usual, I pushed myself too hard and suffered some pretty nasty falls. I also noticed that if I over-did one day, the next day I would be too weak to do much of anything except lie around the house. This was also about the time that I began to lose some feeling in my hands and feet. Still, I chose to ignore all of these signs figuring that what you don't know can't hurt you. Boy was I wrong.
I picked up a shell, brushed off the sand, and studied it for a moment. The constant pounding of the surf had severely pitted the shell, killed the creature living within, and removed all its color – all of its beauty. Something like what is happening to my muscles, I suppose.
Feeling empty, alone, and vulnerable, I clenched my fists, raised my arms into the air, and screamed, "WHY ME!"
Bargaining … Guilt … then Depression
"We just have to make the most of it, Honey." Carey smiled, but I could tell it wasn’t very sincere. She was trying to put on a brave front for me, but her eyes gave her away. She was as concerned as I was with my health and our family’s financial future.
"And, if your doctor is right, a treatment could be discovered within the next few years.” Carey grabbed my arm and gave me a peck on the cheek.
“The doctor said sometime in my lifetime,” I corrected. And, how bad off will I be by then, I wondered.
"You're right, Love, I shouldn't worry about it. Worrying won't help anything." I forced my own smile and reached out to her, looking for a hug. Both of us didn’t want to worry the other any more than we already were.
It was tough trying to be brave following the confirmation meeting with the neurologist. Yes, the DNA test confirmed it. I had Kennedy's Disease.
Josh and Emily, our two teenagers, seemed to take the news in stride. Neither asked about what this meant to them or my future. Oh, to be young again with no cares in the world. Since then, however, both had privately come to me and asked several questions about Kennedy's Disease. Josh also asked if he could have Kennedy's Disease. Emily wanted to know if she could be a carrier and, if she was, what that meant if she wanted to have children some day. These were excellent questions and reinforced the need for family counseling.
For weeks after the confirmation, no - actually months, my thoughts wouldn't slow down. Every day and almost every night I continued to ask 'why' and, even more importantly, 'why me'.
I found myself lashing out at the ones I loved - especially Carey - as I tried to deal with my anger. I knew that I was making her life miserable, and that if I didn't get help soon, our marriage would be over. Over the past several months, my outbursts had driven a wedge between the two of us. Both Carey and I were fed up with my anger. I had to do something before I permanently damaged our marriage.
It was time I called upon a higher power. I began to pray twice a day. In my prayers, I initially said that I would change my ways and lead a better life if God healed me of this disease. A couple of months later, I upped the ante and offered to dedicate my life to God in any way He, or She, desired.
Unfortunately, God didn’t answer my prayers and the disease continued to gradually eat away at my muscles, strength, and spirit. I began to feel even more sorry for myself - for what was happening to me - and for what I was doing to my wife and children. I knew that I was letting them down. Not only was the disease reducing my strength and stamina, it was also threatening to reduce my earning power. Being that I was the sole breadwinner in the family, what were we going to do? How were we going to live?
Since God didn’t come through, I next decided to ask a professional for help. My company had psychologists available for consultation to help with personal emergencies. I had never used these services, but I was running out of options.
Unfortunately, talking to someone about your problems when you already knew the answers didn’t help much. Besides, I already had myself to talk to, so I didn’t need another ‘listener’ in my life. I needed answers and these answers had to come from within.
Carey and I continued to go over our balance sheet, net assets, investments, and retirement accounts. Fortunately, we had always been 'savers', so we had some money put aside for an emergency. Nevertheless, depending upon the number of working years left, we would have to be a little more frugal. A big worry for both of us was whether we could afford to send Josh and Emily to college.
Carey, God bless her, hung in there. She was always a positive influence telling me we'll do just fine, but it didn't help. I was letting her and the children down. They counted on me to take care of them and now they would end up having to take care of me.
As the months wore on and with the slow, but steadily weakening of my body, it appeared my life, as I knew it, was over. I began to withdraw from my work and social relationships, fearing that I was a hindrance or that others would view me as a cripple.
Carey encouraged me to talk with my manager at work, and I eventually found the nerve to sit down with her.
Actually, our conversation went pretty well. She was supportive and said that I could work as long as I was capable of performing my duties. She also called in our Human Resources Manager and explained the situation. She asked him to research all of the company benefits and alternatives for someone with a disability.
It took about a week, but the H.R. Manager did a good deal of research and provided an impressive report of my options and benefits. The news gave me a slight boost. That evening I went over the report with Carey. I can still remember her words: "Do you see, Hon, I told you we could make it. Now, will you relax a little and quit being such a 'worry-wart'.” She was right ... as usual.
About six months later, however, things changed. My manager approached me and asked if I would consider taking a different position within the company. This newly created position had substantially less responsibilities, less stress, and only had one person reporting to me compared to the twenty currently under my management. I thanked her for the consideration, but asked what if I didn't accept the offer? I remember her shaking her head and explaining that the business was doing away with my current position. She encouraged me to take the new position and advised that I would continue to receive my current compensation. She also said that if I did have to travel, I could fly 'First Class'. I know the company felt they were doing me a favor, but, in fact, this was another 'ego-buster' for me. They were actually telling me I could no longer perform my duties. Another BIG DOWNER!
It was less than a year later that my manager asked how long I thought I would continue to work. If the job change damaged my ego, this question destroyed my self-confidence. I understand the company has to protect itself, but I also feel that the senior managers really need some sensitivity training.
Meanwhile, my neurologist recommended that I stop exercising. It appeared that I was doing more damage than good. This was crushing news. Exercising helped release some of my anger and stress. It also helped my ego by giving me the impression I was still capable of ‘pumping iron’. Without the daily routine, I became even more depressed. Along with the changes at work and my inability to perform the simplest tasks at home, I felt pretty worthless.
Something else happened that caused the family additional hardship. I was falling more frequently and many of the falls resulted in minor and occasionally serious injuries. In the first fifty years of my life, I never had one serious injury. In the last four years, I broke several bones, tore a few ligaments, tore a meniscus, and experienced severe head trauma resulting in bleeding around the brain.
Carey and the kids constantly worried that almost any activity could result in a serious injury. Carey took on all responsibilities around the house and became upset if I tried to do any work myself. I had become the vegetable – the object that needed watching at all times to make certain that I didn’t fall or hurt myself. I knew she was just trying to be helpful and was concerned for my wellbeing, but along with my loss of capabilities came a dramatic loss in my self-esteem. As a result, I often slid into this deep void within my mind – this place where no one could penetrate - and began to hide out there for hours at a time. I know Carey thought that I was just being moody, but in reality, the hiding place was my only safe haven from the crumbling world around me.
The more I sat around, the more time I had to think about my loss of freedom and what the disease was doing to me as well as my family. Carey often tried to help me out of this quagmire, but I just pushed her away thinking I needed to work it out myself. I was the one who was wasting away and would eventually end up in a wheelchair. I was also the one who eventually wouldn't be able to feed or even clean myself. Even worse, Carey would have to be my caregiver, relinquishing her life and freedom to take care of me.
Even though she accepts that responsibility now, how would she feel in ten or twenty years? Would she end up hating me? Furthermore, what happens when she can no longer care for me? She would have no choice but to lock me up in some 'home' - costing the family thousands of dollars a month. My God, I never want to go to a nursing home. I would rather die first.
I also began to believe that the family would be better off if I were dead; at least they would have my life insurance. At that moment, I made a decision: I needed a 'living will'. I needed to make certain that if I had a serious accident or illness, no one would work overtime to keep me alive. Carey didn't need any more grief in her life.
I also made another decision that morning. Quality of life was more important than how long I lived. If I couldn’t take care of myself, I didn’t want to alive.
Loneliness … Acceptance … then Hope
It started slowly, but, over time, I withdrew from most of our social activities. Occasionally we would still get together with friends, but we always had to consider whether I could perform the activity safely. Logistics became the name of the game in everything planned. Were there any steps - if so, how many? Were there handrails on both sides of the steps? Would I have to walk very far and how long would I have to stand? The list of questions grew with every new experience and every accident. More times than not, it was just easier to not participate.
Carey started going alone to many of the activities. I encouraged her, because I didn’t want her to feel shut-in - that would just lead to even more resentment. Yet, while she was away, I missed her. A part of me actually wanted to be alone, because I could wallow in my self-pity. Another part of me hated being alone, because I missed being involved, and, more importantly, missed being with Carey. However, it was often easier to stay at home ... and certainly less stressful for her.
It was about this time that my business manager thought of another way to ease my transition into retirement. She gave me the opportunity to work out of the house. What initially appeared to be an opportunity to reduce my stress, as well as the wear and tear on my body, was actually another step in withdrawing from the world.
My only contact with my business associates and friends was now over the phone or by email. I missed the interaction, stimulation, camaraderie, and, most importantly, the challenge and rewards of working in an office environment with other professionals. In the past, I was a problem solver with people coming to me for help.
Now, I was on the outside and, if consulted at all, it was an afterthought and mostly done as a courtesy. My self-worth slipped even further. If I could no longer contribute at work, maybe it was time to retire.
With those thoughts, came another one even more frightening: if I couldn’t help around the house, and if I was no longer needed at work, what value did I contribute to the world?
While wallowing in my loneliness, self-pity, and worthlessness, I realized that as my self-reliance, strength, and capability to work diminished, so had my self-worth. I looked at my life and found that I hated what I had become more than I hated having the disease. I decided that it was worse living in the gutters of my mind and always thinking the worst about everything than just accepting me for what I was and what I was becoming. It was time to take some action!
The first thing I needed was knowledge. I went on the Internet and read everything I could find on Kennedy's Disease. In the process of becoming educated on the subject, I happened across the Kennedy's Disease Association (KDA) website. The KDA had a wealth of information on the disease, but even more importantly, it provided me with access to doctors and researchers who were working diligently to find a cure.
The association also had a 'chat room' - a place for people with KD to share their experiences. There was something healing in knowing that I wasn't the only one afflicted with the disease. I found the chat room to be helpful in another important way. Even though it was hard to believe, others with KD had many of the same fears and concerns. In fact, the more chats that I attended, the more I learned about the disease and, probably more importantly, how to live with my disability.
As with most everything in life, time heals most wounds. Many of my initial fears of being helpless didn’t materialize. Instead, I learned that if I used my head, I could still do many things. I also found that people were very accommodating and would go out of their way to help. Instead of being considered a burden, I was still a valued friend and business associate that had much to contribute.
With acceptance, and armed with the additional knowledge that researchers were working toward finding a treatment and potentially a cure, I discovered something that had been missing in my life recently – HOPE.