Communicating with ALS One Blink at a Timeby Shaheen E Lakhan, MD, PhD, MEd, MS, FAAN | January 28, 2015
In their new book One Blink at a Time, Ismail and Cheryl Tsieprati share how they teamed up and overcame each and every challenge ALS placed before them.
Ismail has lived with ALS for more than thirty years. His wife, Cheryl, has been challenged over the years to build, train and maintain a reliable and effective nursing team. In addition to Ismail and Cheryl’s inspiring story, the book contains practical advice from training caregivers, to preparing for emergencies, to surviving the hospital. Also included is an extensive glossary and helpful resources. Here, I interview both Cheryl and Ismail.
Lakhan: Can you tell us about the moment you found out that your husband had ALS?
Cheryl: I was attending a support group meeting for post-polio syndrome survivors when I first feared that Ismail had ALS. I had been writing feature articles for a local newspaper and had interviewed a young woman who was a polio survivor. She invited me to attend the meeting which she helped organize. She wanted me to write an article about the group. During the meeting a question came up about the similarities and differences between post-polio syndrome and ALS. A lot of the people in the room had no idea what ALS was. The guest speaker, a neurologist, explained what ALS is, and said that some of the symptoms are very similar to post-polio syndrome. “But the prognosis for persons with ALS is very poor,” she said. “Most people with ALS die within five years of diagnosis.”
As she described the symptoms of ALS, I began to panic. They were exactly the same symptoms that Ismail had been having for so long: progressive weakness and wasting away of muscles. The room started to close in. I desperately needed air. I wanted to run out of the room, but I forced myself to stay until the end of the talk. Then, I excused myself and went home, terrified that I had just discovered what so many doctors hadn’t been able to — that Ismail had ALS. I prayed that I was wrong, but the following week, Ismail went in for testing and Dr. Rebecca Hanson, his neurologist, confirmed my worst fears.
Dr. Hanson called me at home and asked if I could come into her office that day so that she could talk to both Ismail and me together about her diagnosis. That was the moment I knew for certain that my worst fear was true. Unable to wait until I got to her office, I asked her over the phone whether it was ALS. She hesitated and then said “yes.” In that instant, my world crumbled around me. I couldn’t believe such a terrible thin could happen to Ismail and I.
Lakhan: How did your life change from that point on?
Cheryl: My life turned upside-down in that instant. The diagnosis changed everything forever. I believed that all of the dreams Ismail and I had of a happy and successful life together were dead, that we would never be happy again. This is how I describe it in our book:
Will we ever be able to smile and laugh again, I wondered? Gone were our hopes of building a successful video production company, for writing screenplays and musicals together, for traveling the world. We would never grow old together, like those sweet old couples we often watched strolling hand-in-hand through the park at Santa Monica Beach. I would soon be a widow. I would lose the man I loved so deeply, wanted so desperately. I’d live the rest of my life alone and lonely. And Ismail, so sweet, so cheerful, so full of life, would die young. How could this happen to us?
Looking back, I realize that everything I predicted that day about our future was wrong. We have smiled and laughed a lot throughout the last 30 years, even produced some videos. Ismail wrote another screenplay using a computer system and an infrared switch, we have written a couple of musicals together, and we have written a book. We took a European tour before Ismail started using a ventilator, and for many years, took overnight trips to beautiful places in California like Carmel, Monterey, La Jolla, San Diego, and Santa Barbara. To everyone’s amazement, Ismail is still alive and happy, and we continue to enjoy our lives and one another’s company.
It’s true, however, that our lives were irreversibly changed the instant Dr. Hanson made that dark diagnosis. Our journey has been different from most other married couples, our challenges are often more intense. Life is harder. Our daily routine is complicated with nurses, medical protocols, medical equipment, ventilators, hospital beds, and our ongoing fight to keep Ismail healthy and happy despite his ALS. But, throughout these 30 years, our relationship and our love of life and for one another have remained as strong as ever.
Lakhan: Healthcare providers often focus too much on the disease and not the patient (and even less so their loved ones and caregivers). What would you like for them to know?
Cheryl: Ismail and I were fortunate to have two compassionate, supportive neurologists who each delivered their diagnosis in a kind and caring manner and made themselves available to provide ongoing support and continue to answer our questions whenever they came up. Unfortunately, other patients often complain that their neurologists told them they had ALS in a cold, clinical manner and then sent them away to “get their affairs in order” without any sense of hope or helpful resource information to take with them. These families often feel frightened, lost, and abandoned.
Without providing a sense of “false” hope to newly diagnosed patients, I feel doctors should explain that ALS affects everyone differently and that many people with ALS live longer than the 3-5 year average that professionals quote; that some people with ALS, like Ismail, live for many decades. I think doctors should also explain that there are options available, such as bipap machines and portable ventilators, that can, when the time comes, help extend the lives of those who are interested in exploring these options and who have the resources necessary to support living with them. ALS patients and their caregivers should be given as much resource information as possible at the time of diagnosis and should be referred to the ALS Association and the Muscular Dystrophy Association, both of which provide helpful practical information and support to persons with ALS and their families.
Ismail and I feel that our book, One Blink at a Time, will show people living with ALS and other life-altering conditions that happiness and success are possible, even in the face of adversity. We believe our book will inspire people to live every day to the fullest with optimism, courage, and hope. Alternate chapters are written by Ismail and me and explore each of our battles, triumphs, and lessons learned. Our book includes practical advice about things such as training caregivers, preparing for emergencies, and surviving the hospital. It also includes an appendix containing an extensive glossary and helpful resources.
Lakhan: What was it like to meet Stephen Hawking? If you saw the movie “The Theory of Everything“, what did you make of it?
Cheryl: Stephen Hawking, one of the greatest minds of all time, is my hero and inspiration. His remarkable story of surviving ALS for so many years and continuing to make a tremendous contribution to science and the world despite his paralysis and inability to talk, profoundly touched me and helped to lead me out of my intense depression after Ismail’s diagnosis. If this world-renowned scientist, I thought, can continue to work and travel and accomplish great things despite being in a wheelchair and dependent on a computer voice system for communication, so can Ismail. Emerging from my deep depression, I resolved to help Ismail live his life to the fullest for as long as he could.
On August 14, 1992, Ismail and I were guests of the ALS Association at the world premiere of A Brief History of Time, a movie about Professor Hawking’s life and work. We were also invited to a private reception for Professor Hawking at the Peninsula Hotel in Beverly Hills prior to the movie’s screening. It was one of the most exciting nights of our lives. Our hero was right there in front of us, speaking to us with his computerized voice! His gracious companion repeated to us his words that had been swallowed by the loud buzz of the crowded room: “It is a pleasure to meet you.” Meeting Stephen Hawking was the thrill of a lifetime.
Sitting on a shelf in our home is a framed snapshot of Stephen Hawking and Ismail, sitting side-by-side in their wheelchairs at the Peninsula Hotel reception. Ismail and I both treasure the picture and the memory of meeting this amazing man.
I thought the movie The Theory of Everything was well-made and touching and that it realistically portrayed many of the challenges and conflicts faced by people living with ALS and their families. Both Eddie Redmayne and Felicity Jones gave outstanding performances. I found Eddie Redmayne totally believable as Stephen Hawking. He did an amazing job portraying a man with ALS and simulating the various stages of his body’s gradual deterioration resulting from progressing ALS. Some scenes were painful for me to watch, not only because they showed the struggle Stephen Hawking had to deal with, but also because they reminded me of the pain I suffered watching Ismail struggle through those same stages of ALS.
Lakhan: Ismail, how were you diagnosed with ALS?
Ismail: The first thing I experienced was pain in my right arm, my right thumb, and my neck. The pain came and went. As far back as the late 1960s, a doctor prescribed a neck collar for me to use while I worked. I began to experience weakness in my right arm in 1976. By 1980, I was unable to hold my niece, then a baby, in my right arm. A doctor told me it was nothing but a pinched nerve and suggested I take Tylenol. Later, when I discovered that I had lost muscle between my thumb and index finger, another doctor suggested I practice squeezing a soft rubber ball to build up the muscle. He prescribed a neck brace. Finally, during an annual physical examination, my family doctor happened to notice twitching in my breast and gave me a referral to Dr. Rebecca Hanson, a great doctor who was head of the neurology department.
Dr. Hanson gave me a very thorough examination. In addition to my other symptoms, she discovered a twitching and a weakness of control of my tongue. She tested the strength of my tongue by having me push it against the inside of my cheek while she pushed back on the outside of my cheek with her hand. Afterwards, she sent me for an electromyogram (EMG), which is a test that checks muscles and the nerves that control muscles. Needles that were attached by wires to an electrical recorder were put in my tongue. The test was very painful. When Dr. Hanson got the results of the test, she determined that I had ALS. Even though Dr. Hanson was certain of her diagnosis, she arranged for an appointment for me to see Dr. W. King Engel, Director of the USC Neuromuscular Center at the Good Samaritan Hospital, for a second opinion.
Dr. Engel, another great doctor, is a world-renowned specialist in neuromuscular disease. He took a long time to examine me and told me he was not sure that I had ALS. He made arrangements for me to have a special liver test and ordered a muscle biopsy. The biopsy was taken from my thigh. After getting the result of that test, Dr. Engel was convinced that I had ALS.
Lakhan: When did you realize all the clinical manifestations of ALS?
Ismail: When I was first diagnosed with ALS, I did not know anything about the disease. Dr. Hanson and Dr. Engel explained to me and Cheryl how ALS kills a human being by destroying nerve cells that allow the brain to control muscle movement until eventually the person becomes paralyzed. The muscles of the diaphragm eventually stop working, causing respiratory failure.
Because I have a slowly progressing form of ALS, I experienced the symptoms of the disease gradually over many years.
Lakhan: What words do you have for others newly diagnosed with ALS?
Ismail: Take one day at a time and enjoy every minute you can. Focus on the things you can do, not on the things you can’t do. Never stop dreaming.
Do not give up. There is always hope. With all the research that is going on, soon science will provide answers. Hopefully this disease will be eliminated, and it will be a thing of the past.
Lakhan: The Ice Bucket Challenge was a real success in raising funds for ALS. However, we recently wrote on the subject and exposed that most of the participants didn’t know what ALS stood for let alone what the disease actually entailed. What are your thoughts?
Ismail: The Ice Bucket Challenge has been the greatest thing to ever happen to people with ALS. When I first heard about it, I never dreamed how big and important it was going to become. Cheryl and I thought it would die out after a couple of weeks. We were wrong!
When I was first told I had ALS, I didn’t know what it was. When I told other people I had ALS, most of them didn’t know what it was either. Even when we said it was “Lou Gehrig’s Disease” a lot of people didn’t know what we were talking about. But thanks to the Ice Bucket Challenge, people started talking about ALS. Celebrities, politicians, and CEOs of big companies poured buckets of ice water over their heads and challenged other famous people to do it, too. People from around the world began doing the Ice Bucket Challenge, and everybody was talking about ALS! Unfortunately, even with all of the publicity from the Ice Bucket Challenge, many people, including some of those who participated in the challenge, still do not know very much about the disease. They thought the challenge was a fun thing to do, but didn’t take the time to learn about the reason they were doing it. That is why it is important for everyone to continue talking about ALS and educating the public about what it is, what it does to people, and why it is important to find a cure.
The ALS Association’s annual “Walks to Defeat ALS” and other fundraising events help to raise awareness of what ALS is and why it’s important to raise money for research and family services. Cheryl and I feel our book, One Blink at a Time. also helps educate people about ALS and how families can face its challenges. It includes an appendix with lots of resources that can be helpful to people with ALS and their families.
Lakhan: We received a number of questions from our readers that we would love for you to answer.
BB Readers: What does it mean to be “locked in”?
Ismail: Sometimes a person can become so totally paralyzed that they have no muscle movement at all, not even eye movement. Not only can’t they talk, but they can’t even blink their eye like I do to communicate and become unable to communicate in any way with the outside world. This is what is called being “locked in.” This is how Cheryl describes it in our book:
Those who are “locked in” survive in complete isolation. They can see and hear everything around them, comprehend everything, think complex thoughts, experience joy, sorrow, and pain, and yearn to communicate with their loved ones and caregivers, to express their feelings, their discomfort, and their desires, but they are hopelessly trapped inside their bodies with no way of communicating with anyone.
Cheryl and I are always looking for new technologies that will help me communicate if there comes a time when my eye blink becomes so weak that I’m no longer able to communicate as I do now. I have signed up to be an early adopter of a new eye gaze technology called EyeSpeak. With EyeSpeak I hope to be able to select letters from a virtual keyboard by looking at each letter and then selecting a button with my eyes that will make the glasses read out loud what I have typed. I might also be able to use a computer with the help of these glasses.
BB Readers: How do you communicate?
Ismail: I communicate with eye blink. Many years ago, one of my nurses helped me design a spelling chart (above) that worked well for me. The chart has alphabet letters divided into six rows. Each row is numbered. For example, row #1 is a-b-c-d and row #2 is e-f-g-h. Row #7 says “end of word,” which I select if I want to tell my caregiver that I’ve finished spelling a word or a sentence. Row #8 contains numbers. The chart also indicates that one blink means “yes” and two blinks means “no.” Cheryl or one of my nurses calls out the numbers of each row. I blink when I hear the number of the row I want. I blink again when I hear the letter I want. Letter by letter, blink by blink, I spell out words.
Spelling out words by blinking is not the only way I communicate. I grind my teeth whenever I need to get someone’s attention (for example, when there is something wrong or I need something urgently). I can also make my ventilator beep by holding my breath and building up pressure in my ventilator circuit. My caregivers all know my code: if the ventilator beeps twice in a row, I am calling them. I also “point” at objects by looking at them. That way, I can call attention to things.
BB Readers: If you muscles are too weak, how do you breathe? Eat? Move about?
Ismail: The more my ALS advances, the more dependent I become on machines and devices to help me. Because I can no longer walk or even stand up on my own, I use a power wheelchair to move around. The wheelchair has a control switch that Cheryl and my nurses use to drive it and to lower the back when I need to recline. My caregivers also use a Hoyer lift to transfer me to and from my bed and wheelchair. When it was no longer possible for me to eat because I could not chew or swallow my food, a doctor inserted a feeding tube into my stomach so that I can be given formula and pureed food through the tube.
When I started to develop respiratory failure, I had a tracheotomy and began using a ventilator to breathe. That was 24 years ago, and the ventilator has kept me alive all this time. There are other machines available, such as the C-PAP, BIPAP, and oxygen concentrator, that can help some people breathe who have different needs than I do.
I am thankful that so much great technology exists today that can help people continue to live and enjoy life like I do.
BB Readers: When you wake up every day, what keeps you going?
Ismail: Every day I get up and look forward to being with my wife and seeing family and friends. I enjoy going out and visiting different places, and I love working with Cheryl on books, musicals, and other projects.
Death is an end of all things on earth. Life is a celebration. I want to enjoy the part of the world that belongs to me and celebrate life.
This is what I wrote in One Blink at a Time:
People are amazed that I have survived ALS for thirty years and still feel good and enjoy life. They want to know how I’ve done it. They ask me how I can continue to wake up every morning, totally paralyzed and unable to talk or to eat, and still want to go on after so many years. The answer is simple. I have a lot to live for. I love my wife and enjoy being with her. She is my best friend and collaborator. I want to continue to be with her as long as I can. I dream of living many more years with Cheryl and giving other people in situations like mine encouragement and hope.
Lakhan: Any closing remarks for our readers?
Cheryl: Life is short. It’s not always perfect, but I believe in living life to the fullest, despite challenges and limitations. I consider every day I have with Ismail a gift, which I cherish and for which I am grateful. Together, we make the most of every day.
Ismail: I hope that One Blink at a Time will be helpful and informative for other people in situations such as mine and will educate the general public about ALS. I hope that it will inspire people to live every day to the fullest with optimism, courage, and hope.
People can learn more about us and our book, read what people are saying about the book, and click on a link to Amazon to buy a copy by visiting our website at www.oneblinkatatime.com.
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