Lost in the Wind - A Journey with ALS

During the spring of 2001 a friend from the East Coast came to Portland, Oregon for a visit. It was wonderful to see her even though most of our conversation was one-way. I am able to communicate only with the use of an electronic augmentative communication system. It is slow and labored. I will discuss this system and the meaning of one-way communication in further detail later. On the Sunday my friend was here, it was a gorgeous day. We went downtown to the waterfront. I wanted to burst out of my body and feel my feet touch the earth, feel the breeze, and smell the air. We spent time looking at the river while she and my family had appetizers and drinks. We stopped at Salmon Springs for a few minutes to watch the children play in the water. Then we drove home through the city observing the crowds of people.

When I got home I cried for four hours because I had begun to feel like a piece of meat, but with feelings and a fully functional brain. Being on the waterfront with my friend and experiencing those feelings prompted my attempt to describe the day and its impact to my speech and language pathologist. She suggested I write about the experiences that led to those feelings. Over the next few months, by using my communication system, I typed most of my experiences that had left me virtually motionless, voiceless, and disconnected from everyone except through a process both tedious and mechanical.

As I write this, I am a young woman of 40 years who was diagnosed in 1999 with Amyotrophic Lateral Sclerosis (ALS). The diagnosis came only after at least two years of progressive physical changes. The disease ended my career as an ASIC engineer. My dreams of getting married and having a family were shattered. My body has weakened to the extent that I require a lift and an electric wheelchair for all movement. I breathe connected to a ventilator 24 hours a day. My entire life has become centered on this disease, and on my attempt to sustain life.

Within a few months of returning to the San Francisco Bay area in 1996, I began to notice that I was chronically tired and had no energy for anything. I felt like I was falling into a deep canyon. I had no control. And that fall has never stopped. For example, once I lay on the living room floor from Friday after work until Monday morning. I didn't eat or drink or use the bathroom. I was in a shallow sleep state with no dreaming. My brain seemed to be stopped! I was more frightened than I had ever been in my life.

A former employer that I respected encouraged me to see my physician. He thought I might be depressed. I was stunned. I disagreed and said I have been sad at times but certainly not sad all the time. My former employer explained that sadness is not depression. I began a series of appointments with my primary care physician (PCP). Every time I met with her I would cry. She started me almost immediately on 10 mg of Prozac a day with a scheduled increase to 20 mg a day. Although it lifted me some, it didn't help much.

My primary care physician ran a series of blood tests but found nothing wrong. Although she was concerned, she was honest. She said, "I don't know what is wrong with you." Soon after, my crying increased significantly and was often accompanied with a frantic feeling. I saw my doctor and talked with her on the phone several times. Within a few weeks, she said she thought I should see a psychiatrist. She said, "I know a good psychiatrist, and I think you should go see her." I accepted her recommendation. It was a good place to cry. This psychiatrist knew I was on 20 mg of Prozac a day, and she encouraged me to continue. I had my suspicions about what was happening to me but I knew neither my doctor nor the psychiatrist was open to my insights.

During the time of these initial medical appointments, I was preparing to go to Iran for my brother's wedding. It took me almost a full 24 hours to get to Iran because of political and security reasons. By the time we finally arrived at my family home, it was time for breakfast. I was exhausted. For the first time in my life, I had physically hit rock bottom. I couldn't eat, visit, or connect with my surroundings. I couldn't even sleep or rest until long after nightfall.

Throughout the visit to my homeland, I never felt rested or like my normal self. In fact, I would later look back and see that I was to never feel like myself again. During that trip, I became aware that I had absolutely no control over my tears. I had a hard time engaging myself in the various conversations. I grew fatigued easily and frequently.

About a week before my flight back to America, I went to Tehran. I wanted to do some last minute shopping. I asked my brother to take me to a particular bookstore near the front of the University of Tehran. While I waited for my brother at the stairway entrance, I felt as though a main switch in the center of my brain turned off.

I could feel every sense and organ within my body shutting down simultaneously. It started from my head and ran down to my toes. The sensations then reversed. It was as if I had died and was coming back to life. I didn't see any bright light nor did I feel as though it were an "out of body" experience. Although I was frightened at first, I realized there had been no pain. This was my first experience of noticing there would not be pain in dying for me. There was some comfort in that realization.
A few days later, before we were to leave for America, we were invited to my cousin's house for dinner. The atmosphere was festive. As I was sitting down, suddenly my experience repeated itself. This time I felt angry. It was as though I were being reminded that there is no pain with death. That type of incident never happened again. At the same time, I was not ready for what was ahead for me.

I didn't mention these experiences to anyone. I was looking forward to returning home and to getting rest. On my first day back in America, I noticed that I was limping on the right side. Actually, it was my pinkie toe. I didn't have any pain so I ignored the limping. Soon after that, while I was at work in my office, I lost total control of myself. I couldn't stop crying. My physician expressed concern, especially when I told her I hadn't experienced any pain during the two incidents in Iran. She stated that I might have Multiple Sclerosis (MS). I panicked. I was speechless. I was in a state of shock and couldn't respond. She ordered blood work, and gave me the names of neurologists. I took her advice, and made an appointment with the first name on the list for a full month later.

One evening after leaving the office, I got into my car and unconsciously turned on the ignition with my left hand. "How odd," I said to myself. I am right-handed and there was no reason to use my left hand. I turned off the ignition and then tried to turn it on with my right hand. I could not. I sat there in the dark for a few minutes before using my left hand to go home.

When my appointment with the neurologist arrived, I met a short man with pants riding above his ankles. Upon entering his office, I noticed that he had my chart and was preparing to take notes. I told him about my incidents in Iran, and that I was seeing a psychiatrist. I was attempting to openly provide him all my recent history. His reply was, "So, you are seeing a shrink to unload and to feel better."

He continued, saying, "I think you might have had a seizure. By California law, I should report that to the DMV and have your driver's license voided." I panicked. I told him I live alone and have no family nearby. I asked, "Are you sure I had a seizure?" He said he was not and that he needed to run some medical tests. In the meantime, he said he thought I might be dangerous on the road. I explained that the incident had not repeated itself in many months. I then asked, "Could this be a virus that has attacked my nervous system?"

He became defensive saying, "You are an engineer. I don't tell you how to do your job so don't tell me how to do my job." I thought to myself that I am not some case study for this arrogant doctor. I left his office calmly and civilly. He had no idea that I would never return for another visit with him.
The next neurologist I saw was an associate professor at Stanford University and a solo practitioner in a large medical office in Atherton, California. Atherton is in one of the most elite areas of Northern California. I thought I would now be in good hands. I did decide that this time I would not talk about the uncontrolled crying or the incidents in Iran. I focused instead on my body weakness.
When I arrived for my appointment, Dr. Black was at a conference. Dr. Smith, a colleague of Dr. Black's also from Stanford University who specializes in MS, saw me instead. He did a thorough examination and arrived at the decision that I did not have MS. Unfortunately, he didn't tell me much else. At my next appointment Dr. Black reported to me that I didn't have MS. He didn't do any examination. For whatever reason, he was convinced that there was nothing wrong with me. In turn, I then had to convince him that I wanted more tests run, which he did.

In the subsequent appointments, I was seen by his physician assistant (PA). I requested to see Dr. Black but was refused. I asked my PCP and my psychiatrist to call Dr. Black to request that he see me personally. He continued to refuse. By this time, I was experiencing severe twitching in my muscles. The PA recognized this and recommended I have an EMG (electromyogram)* completed. But during my next appointment, Dr. Black came in abruptly and stated, "You don't need an EMG. All your tests are negative. Continue to see your psychiatrist."

I looked into his eyes without blinking and said, "You are chasing the wrong thing." There was a pause. I maintained eye contact with him. He made no response and I realized that I had no respect for him. As I was leaving, he scheduled me for a spinal tap saying he would do this procedure himself in his office.

In the meantime, I was becoming weaker. I told a family friend, Parv. I shared the frustration that I felt with the two neurologists. Parv told me that her son, Hooman, was visiting San Francisco, and that he is a neurosurgeon. She suggested I get my medical file and allow her son to review it. I was hesitant, knowing all the medical tests were negative. She assured me that he is an excellent doctor, and would be willing to support me.

After much persuasion I agreed. An appointment was scheduled. Parv's son, Hooman, proceeded to complete a thorough medical examination. At one point he took a piece of paper and wadded it into a ball. "Rabe'eh, try to catch each one as I throw it to you." He threw these balls in different directions. We were sitting no more than three feet apart. Some balls I was able to catch and some I could not. He looked concerned.

I calmly asked, "Do you know what I have?" "Do you really want to know?" Hooman asked in response. "Yes," I said. "I think you have ALS (Amyotrophic Lateral Sclerosis or Lou Gehrig's Disease)." "What is that?" I wanted to know. "You will soon be dragging your right foot." He stood and demonstrated. I thought that was all there was to it, and I didn't want to accept this. At this point I had no idea of what was ahead of me. He strongly encouraged me to go to the University of California at San Francisco (UCSF).

On my way home, I turned off the music and watched the green hills and the beautiful San Francisco Bay. I also reflected on what Hooman had said. What if he was right about my leg? I rejected that thought immediately. I was looking for the medicine that would cure me and not something that would mean permanent damage. This simply couldn't be happening to me.

After all, I had never used drugs or even tried cigarettes. I drank alcohol only moderately. I would occasionally have a glass of red wine with a meal. I had been a vegetarian for well over a decade. I exercised regularly. In my more than twenty years in America, I would guess I hadn't drunk even twenty cans of soda. Neither side of my family had ever been diagnosed with a neurological disease. My motto was to die healthy. I desperately hoped Hooman was wrong.

During all the months of change, frustration, and mounting fear, I still had not told anyone about what was happening to me. I had not told my family nor my friends, and none of my co-workers. I had not even informed my boss. What was I to say? If myown physicians and neurologists were not listening to me or believing me, how could I expect other people to believe me?

About that time I took two months leave from work. I knew I was not being taken seriously. I had to find another alternative. Among the alternatives I considered was acupuncture. I had always been frightened of needles so I put that idea on hold.

My condominium was on the second floor of the building where I lived. Soon I had to pull myself up the stairs using the railing. Taking groceries and other objects up to my condo became a major chore. I was able to carry only one bag at a time, and I had to take one step at a time. I would lift the bag up one step and then pull myself up one step. By the time I had my groceries inside I would be in a full-body sweat and completely exhausted.

At night the twitching in my thigh muscles became so intense that I would wake consumed with fear. I would repeat a prayer as many as fifty times until the fear would subside.

It was now time to reconsider acupuncture. After a month of daily acupuncture treatments, I knew this was not helping me either. I continued into the second month, as I believed it was my only hope at that time. Unfortunately, by the end of two months, there was no improvement.

About this time, my family was visiting me from Portland. I went to the airport to see them catch a flight to Hawaii. When I was leaving the airport, I became suddenly short of breath. I wasn't able to take a deep breath. I couldn't seem to control my breathing at all. I saw an airport cart nearby. I pulled myself over to it and used it to lean on as I dragged myself through the airport. I continued this difficult and exhausting process until I located my car.

"Oh my God, now my breathing is affected!"

When my family returned from their week's vacation, I finally shared with them all that was happening to me. I was now weakening rapidly so I made an urgent appointment with Dr. Black. By the time I arrived at his office, I was exhausted and struggling for breath.

When I went into his exam room, the first thing he said to me was, "You are obviously very emotional." "Look at my hand! I have lost all muscle function in my thumb!" I exclaimed with frustration. I was upset, angry, and concerned. I was not being emotional.

He compared my hands. It was obvious that I had lost most of the muscle function in my right hand. My left hand was weak as well but not as measurably as the right hand. He set up the EMG machine. He positioned the needle marking three sites. He remarked that he now had some evidence.
I thought he now believed me so I proceeded to tell him that whatever I had was in my entire body. I explained that it was more on the right side but was spreading throughout my body. He looked at me as though I was crazy. I realized that he still did not see me or believe something progressive was happening to me.

"My sister, Haleh, has obtained a recommendation to see a neurologist at UCSF," I told him calmly. "When is your appointment?" he asked. "It is about a month from now but we are calling almost daily to see if there are any cancellations," I responded.

Haleh started calling the ALS Center daily begging for an appointment as soon as possible. A neurologist there finally agreed to see me on August 10, 1999, during his lunch hour. Haleh then called Dr. Black's office and demanded to see him. She confronted him with how slow he had been in coming to any medical diagnosis. Direct communication with Dr. Black was of no functional benefit regardless of whether he was speaking to me or one of my family members.

I didn't sleep the night before the appointment at UCSF. I kept experiencing one extreme fear after another. I was consumed with terror and fear of the unknown. At the appointment the next day, the doctor kept coming and going from the exam room. I finally asked, "Dr. Fields, do you know what is wrong with me?" "I will tell you at the end of today's examination," he replied.

One time when he returned to the exam room, he asked me to remove my shoes. "Now, hold my hands and try to stand up tall on your toes," he directed. I held his hands and then realized that there was no way I could lift myself even a fraction of an inch without falling. "I can't do it but I might be able to do it if I hold onto your shoulders," I said. "My hands should give you enough support. I want you to try, please," he insisted. I lifted my left foot since it was the stronger of the two. I was only able to lift it a fraction of an inch before my entire leg started to shake uncontrollably. "I just can't do it," I pleaded. "All right," he answered, and left the room again.

This time it was at least 25 minutes before he returned. "Do you cry often?" he asked upon re-entering the room. "Yes I do," I said. "Do you cry without any reason for crying?" he asked further. "Yes!" Finally I felt as though someone understood me. It was a huge relief. "If you feel like a good laugh, are you able to do that?" he probed. "No, not at all. I long for a good laugh. Laughter keeps me alive," I replied. Dr. Field's face turned as white as the walls. He seemed shocked but attempted to hide his feelings. I knew the news he would be sharing would not be good.

I was impatient to have a diagnosis. I hadn't slept for three days. On the way home that day, I was despondent and fearful. I had no doubt that tomorrow's news would be unfavorable and serious. Haleh disagreed. I felt hopeless that even my own sister didn't seem to understand. It seemed as though no one had any concept of the sheer terror I was feeling and experiencing everyday.

Upon arriving home, we had to talk with our parents. Haleh was optimistic. I was consumed with fear. I sat down with them as Haleh reported the day to them. Then I went and got into bed. That night my mother lay with me in my bed. I asked her to hold me. I had the loudest and deepest cry of my life. She held me tightly. "Mom, please don't leave me," I pleaded. "I would never leave you alone," she said repeatedly to me. Dad and Haleh rushed into my bedroom. They were speechless. I couldn't sleep that night either.

I was utterly exhausted the next day. Haleh went with me to Dr. Fields' office. Finally, the doctor entered the room. I stood up immediately, and before he could speak, I shouted, "Please tell me I don't have ALS!" "Unfortunately, I can't say that," he responded.

This silenced and paralyzed me. I sat down. "What are you afraid of?" Dr. Fields asked me. "A wheelchair," I answered. "Oh! You have at least a year before you have to get a wheelchair. I also think you have four to six years ahead of you." "Excuse me! ALS claims life!" I remarked. "Most patients have only one or two years but you are young. I believe you have four to six years," he repeated. It was as though he was presenting the one good aspect in consideration of this catastrophic diagnosis.

Isn't there a medicine?" I asked. "There is no medicine that cures ALS but there is medication** that slows the disease process by 20 percent." "What about research?" I asked. "Unfortunately, research is too far into the future for finding a cure to aid you," he said.

On our way home, many thoughts raced through my mind. For example, how is it in this great, compassionate nation in which I live, with all its wealth and brain research resources, that in 130 years there has been no identified cure for ALS? Is it because ALS patients die fast and the scientists have no founded medical courses to defend and care for the patients? Is it because the diagnosis takes so long that the person is already dying?

I was exhausted from not being able to sleep. If I didn't die from this ALS, I would surely die from lack of sleep. After sharing this devastating news with my family, I went to bed and finally slept for more than twelve hours. When I awakened, I felt numb and in a persistent state of shock. I felt as though I had a memory block of some kind. The shock was preventing me from integrating this medical diagnosis. The word of my diagnosis began to spread quickly to family and friends, both in America and in Iran. The phone calls started to pour in. I appreciated every call, but I absolutely refused to talk to them. I simply couldn't do it.

Within two months, I couldn't walk without holding someone's hand or without holding on to the walls. By Christmas, I had to use a cane. Two months later I had to have a walker and a month after that I required a manual wheelchair. The disease was progressing much faster than either my doctors or I had thought it would. Exactly six months after my diagnosis I got an electric wheelchair.

It was only a matter of weeks before I had to have a BiPAP *** machine for breathing, and then I had to begin assessments for an augmentative communication system. Soon I could not correctly pronounce words. I had to select my words carefully before speaking. My voice continued to worsen. I could not believe that I would lose my voice completely.

The first speech pathologist I was sent to was confident and knowledgeable. She did not, however, seem to be taking everything into consideration. Didn't she know what this ALS was doing? Perhaps she did but felt this was all that was available. I had a few sessions with her. Every time I left a session, I experienced a severe headache. I knew she was doing her best work. At the same time, I can state that I did not feel she comprehended the significance of what losing my verbal abilities meant to me. She had no idea of how terrifying and devastating this process was.

I couldn't and didn't make any decision about communication devices or software for my computer. I was being expected to make one choice after another during this brief span of a few months. I kept thinking that my laptop would be too heavy to carry around for communication. How could that be practical? There was this head mouse that operated with a dot placed on my forehead. I would scan with this head mouse and make communication selections with head movements. That required strong and stable head and neck support, which I knew I would not have for much longer. In spite of my limitations, that was her recommendation. I stopped seeing that particular speech pathologist.
I met my next speech pathologist; she was warm and friendly. I looked into her eyes closely. She in turn asked if she could touch and examine me. "Of course," I said. Her touch was gentle, and her energy was pure. I felt as though I had never been examined so carefully by anyone. I could feel a strong connection between us. I had finally met someone who seemed to understand my feelings and concerns. We decided to meet Wednesdays at my home.

Even after only one visit, she perceived the exertion it would take for me to attend outpatient appointments. She wanted me to conserve my energy for communication and connection with her.
It turned out that Wednesdays with Carol were better than "Tuesdays With Morrie"**** . She introduced me to many different communication devices, and always took time to discuss the benefits as well as the limitations. More importantly, every Wednesday we took time to connect and not just to cover the technical issues. I had finally found someone who would address all of me and not just one piece or part or issue. I felt real with Carol.

None of the communication devices were any more efficient for me than with the other speech pathologist but it was nearing the time to make a decision. I was losing my voice and my physical functions. I decided on what is known as the LightWRITER***** . I selected it because it was light, simple to learn, and had speech output. Carol also showed me that I could later add a scanner connected to a switch when my hands became too weak to access the keyboard. There are switches available that allow the slightest of movements to make a scanned choice.

This brings us to the point in my life with ALS where communication has become a one-way process even with the LightWRITER. In the next issue of this publication, I will cover this impact and how it influences all areas of my motionless and voiceless life. Before closing this installment, my speech pathologist will provide some further insights we have developed together.

Comments from Rabe'eh's Speech and Language Pathologist and Neuro Psychotherapist, Carol L. Nugent, MS, CCC:

Rabe'eh has been a client for more than two years. Our relationship has deepened and grown with each visit. Because Rabe'eh has been clear, credible and authentic regarding her life experience with ALS, I encouraged her to put all her thoughts, opinions and feelings into a text both for herself and to share with others. We have used the writing as a process of healing and as a means of further educating professionals and the general public about what ALS means to the unique and special individual.

This first article was essential before writing about the one-way communication process. It is critically important to begin with the process of diagnosis of the progressive illness. As you have read and, I hope, gained some insight into, the diagnosis process was more than tedious, frustrating and confusing. Sadly, it was a humiliating process that further crippled Rabe'eh. This time it was crippling in an emotional and psychological way. No one deserves humiliation. It is an unacceptable approach from anyone, let alone from physicians and professionals that we trust with our lives.

After many months of not being listened to or heard, Rabe'eh had come to believe that she could not trust anyone within the medical system. In fact, she had come to distrust many friends and family members as well. To protect herself, she became more defended and rigid with each passing month. These behaviors added to her body's weakening and betrayal while further contributing to her non-verbal status.

Every human needs to be approached and treated with respect for his or her whole self. We are not pieces and parts to be acted upon analytically and with detachment. It would have served Rabe'eh much better if any one medical or professional individual had taken the time to listen to what Rabe'eh knew was evolving within her body. She may not have had a name for the disease process but she knew it terrified her and that it was progressive and serious.

If Rabe'eh had been treated with dignity and interactive attention, it is possible and maybe probable that diagnosis could have emerged earlier. Rabe'eh could have approached her lifetime rehabilitation process as a living process rather than an immediate, pending dying process.

At the time of her referral to C. L. Nugent & Associates, a Speech Pathology and Neuro Psychotherapy service, Rabe'eh was dependent across most modalities of daily function. Her speech was severely dysarthric with minimal volume or projection subsequent to the respiratory involvement of the ALS. Had there been early diagnosis and comprehensive education with referrals to all the disciplines skilled in the care of individuals having ALS, Rabe'eh might have embraced this catastrophic disease differently.

Unfortunately, this is not an isolated case review of someone having ALS. There are likely many reasons and excuses why the medical community does not approach individuals with more humane behaviors. The point, however, is that with articles from the patients themselves, such as Rabe'eh, perhaps there can be change?one issue and change-one individual at a time.

In the next installment, Rabe'eh will share what the experience of losing her verbal communication skills has been. She will continue with a discussion about how the process of communication has become a "one-way process" and what she means by that. Carol will present how augmentative communication (AAC) might better serve the individual when the working professional is more of an interactive, humane caregiver and less the authoritative and detached power source.

Note: Please forward your feedback regarding this article to [email protected], as this is a section of an upcoming book. Your comments will guide and support us for the rest of the book.

* Electromyogram - This is a record of the intrinsic electrical activity in a skeletal muscle. This data is used in the diagnosis of neuromuscular diseases. The data is obtained by applying surface electrodes or by inserting a needle into the muscle.
** Rilutek is a medication with the active ingredient of riluzole prescribed by a physician for some having ALS. Rilutek is not a cure for ALS, although studies have shown it can extend tracheostomy-free survival.
*** BiPAP is a lightweight portable ventilator that helps individuals with advanced breathing weakness. The BiPAP gives the breathing muscles an opportunity to rest. Air is delivered through a cushioned nasal mask, which fits comfortably over the nose.
**** "tuesdays with Morrie," by Mitch Albom, 1997, Doubleday
***** LightWRITER: A dedicated augmentative communication aid that includes DECtalk, a speech synthesizer, allowing the user to engage in interactive conversation. This AAC aid has many features and is easy to learn for almost immediate functional uses. For more information about this AAC aid please contact ZYGO Industries, Inc.