I published a book this past May; The Patient Experience with Transverse Myelitis: An Anthropological Perspective. I'm selling more than twenty years of accumulated knowledge about transverse myelitis on Amazon for $18. If you own a Kindle, you can get everything I know about transverse myelitis for $2.85. I have no motivation to earn any profit from this book. Pauline and I strongly believed that the work we were doing for our community should never involve any kind of financial gain. We adopted that approach because we never wanted our integrity to be questioned. This philosophy also made it easier for us to ask for donations. Among the hardest things we ever had to do was ask people for money. We came to realize, however, that without money, we could accomplish nothing. No institutions in our society are held to a higher standard than not for profits. I'll have to write a blog sometime as to why that is the case in American culture. For the first five or more years of our existence, Pauline and I bankrolled the operation from our personal bank account. We have continued to make our financial support of the organization our priority.
Amazon told me that if I wanted to sell my book with no profit, $13 would be the price. That would cover their cost. When I told Nancy the suggested price, she told me that it was bad luck to sell it for $13. I agreed. While I'm very much a science person, I was raised in a culture of shtetl superstition. I should probably write a blog one of these days discussing the relationship between magic, superstition and the perceived and real control or lack of control a people have over the forces of nature, the acts of humankind and the universe in general.
Nancy suggested that I sell the book for $18. Brilliant. Many cultures apply spiritual significance to numbers. In many western cultures, the number 3 is important; I'm guessing the derivation of which is the holy trinity. In most Native American cultures, the number 4 is spiritually important. In religious ceremonies, rituals are repeated in sets of 4. In Judaism, the number 18 has mystical significance. The letters of the Hebrew word chai add up to 18. The word chai means life. I am donating all proceeds from sales to the Pauline H. Siegel Eclipse Fund.
The book isn't meant to serve as a medical treatise, although it does contain a great deal of medical information. I tried to steer clear of medical advice, because medicine isn't my background or expertise. I'm a cultural anthropologist and a photography student.
I have been doing advocacy work for my Association for 25 years. Pauline was diagnosed with TM in 1994. Most of what I learned came from my experiences with Pauline. The hospitals, the rehabilitation centers, the medications, the adaptive equipment, the doctor appointments, the social service agencies, the legal issues (reasonable accommodations), and the emotional and psychological issues; I was involved in all of it. I also learned from the people in our community. I learned so much through my role as the editor of our newsletter and journal and serving as president of The Transverse Myelitis Association; now the Siegel Rare Neuroimmune Association (SRNA). I have spoken to thousands of people with transverse myelitis who have called over the years seeking information and support. I have conducted extensive research on transverse myelitis. I have been immersed in the medical literature. I have attended almost every education program that has been offered by our Association and various academic centers across the country. I do know a lot about transverse myelitis.
The primary research methodology in cultural anthropology is participant-observation. I have most definitely been a participant in this community of people with rare neuroimmune disorders, their family members and caregivers. The observations in anthropology attempt to be as objective as possible. My perspective on objectivity is that if it involves a human being, there is going to be bias. The only issue is the magnitude of bias, where it comes from, how much one can be aware of, and whether there's anything you can do about it by virtue of knowing it exists. My observations were not as objective as they should be even from a social science perspective. I have always been too emotionally involved through my relationship with Pauline and also my personal connections in our community. What objectivity I did bring to my observations and experiences came in large part from none of this happening to my own body.
The book describes what a person goes through when they get a diagnosis of transverse myelitis. While it is a unique experience for everyone who travels this journey, there are similarities. Transverse myelitis is an inflammatory attack in the spinal cord that is thought to be immune-mediated; the immune system attacks myelin in the spinal cord. The resultant damage to the spinal cord is what causes the symptoms of TM. Those symptoms can include muscle weakness to paralysis, nerve pain, spasticity, bowel, bladder and sexual dysfunction, fatigue, depression, and cognitive dysfunction. The attack for most people is a one-time event. And that makes TM a rarity among auto-immune disorders, because once the immune system becomes deranged, as for instance, in multiple sclerosis or lupus or rheumatoid arthritis, the person is at risk for multiple attacks.
The nature and severity of symptoms in TM depends on where in the spinal cord the inflammatory attack occurs and the extent of myelin and neuronal damage. For people who are impacted high on the cord, they can become full quadriplegic and lose the ability to breathe. Pauline was fortunate to only be paralyzed from the waist down. I say fortunate because being able to breath on one's own is the game changer. Not being able to use one's hands has a devastating impact on independence and quality of life. I also say fortunately, because it wasn't my body.
The attack can happen to children as young as four months old and to people into their 70s and 80s. It happens to both males and females and there doesn't appear to be a concentration of cases among any particular group of people. The geographic and demographic distribution of cases looks to be random.
While we know more about transverse myelitis today than we did when Pauline and I first started doing this work, so much remains not understood. Most physicians believe that autoimmunity involves a genetic predisposition in combination with environmental factors that trigger the immune attack. While the paucity of information about transverse myelitis is discouraging, it is the case that little is understood about almost all auto-immune disorders. We are learning more and more about the immune system, but we still have so much more to learn. It wasn't until the 1950's that medicine figured out that the immune system could become dysfunctional and attack 'self.' The purpose of the immune system is to find 'not self' (bacterial, viral and fungal infections) and to destroy it. The immune system is supposed to leave 'self' alone.
There is no known biomarker for TM. That means there is nothing in the blood, spinal fluid or anything else in the body that is unique to a person who has TM, distinguishing them from a healthy person or a person with a related disorder, such as multiple sclerosis. As a result, a diagnosis of TM involves observable clinical characteristics; it is a diagnosis of exclusion. Physicians see inflammation in the spinal cord through an MRI with contrast agent, and then begin a long battery of tests to rule out everything they know that could cause inflammation in the spinal cord. When everything is ruled out, and the involvement of the immune system is confirmed through a spinal tap, the person is given an idiopathic transverse myelitis diagnosis. Idiopathic means they have no idea how or why you had this inflammatory attack in your spinal cord.
If we could raise the money for research, we would almost certainly be able to figure it out. Raising money in a rare disease community is a challenge. But we keep at it. We owe it to our community to find these answers.
When Pauline died, the board established the Pauline H. Siegel Eclipse Fund to support research on the rare neuroimmune disorders. You can learn about how we came up with the name Eclipse if you follow the link. While you're there, please consider making a donation. Thank you!
I wrote the book because I felt obligated to do so. I spent a significant portion of my life devoted to helping the people in our community. Support and education were central to our mission. How could I possibly take the chance that I would leave this earth without passing on what I learned. It took me years to write. I wanted the book to be comprehensive. It was important to me that a person who was diagnosed with TM could read my book and learn just about everything that was possible to happen from their experience. It is a horrible experience to go through even when you know what is possible. It's way more than just horrible when every aspect of the experience occurs as a totally unpleasant surprise.
I wish Pauline and I had this book when she was diagnosed. We knew absolutely nothing. We knew nothing for a very long time. We had no idea whether Pauline was going to keep getting worse. We didn't know if TM was fatal. We were entirely vacuous. One of the more important benefits of the book is that a person can develop some expectations about what is possible; and think about what the future might hold. The book will also help a person feel less alone in their experience, something many people feel when they have a very rare disorder.
The book is also going to make a person or family members, better advocates for their medical care. We have more specialists today because our organization started a fellowship program to train clinicians and researchers in the rare neuroimmune disorders. Specialists were being trained in a few medical centers across the country, but the process was happening too slowly. Our fellowship program has accelerated this process. Still not as quickly as needed, but better than it was before we got involved. Again, it is all about the money.
As cases of TM are spread across the country and around the world, the odds are good that most people will be cared for by a physician who has little to no experience with TM. To receive the best care possible, patients and family members need to be educated and they need to become strong advocates. Our web site is a wealth of information to support these education efforts. My book is a contribution to that critical education effort.
The book is for the person with the disorder and it will also help family members and caregivers. Importantly, the book will help people who have the other rare neuroimmune disorders that we advocate for under the SRNA umbrella: Acute Dissemination Encephalomyelitis (ADEM), MOG Antibody-Associated Disease (MOG-Ab disease), Neuromyelitis Optice Spectrum Disorder (NMOSD) and Optic Neuritis (ON). The clinical differences between these disorders involves where in the central nervous system the immune system attacks and whether the attacks are monophasic (a one-time event) or recurrent. There is significant overlap in symptoms and treatments between all of them. There are so many similarities in experiences, for the person with the disorder and for their family members.
As there was much I didn't know, because, 'hey, I'm human, I found contributors from our community who were willing to write articles describing their experiences. For instance, two women with TM describe their experiences going through a pregnancy and taking care of an infant. More then twenty people contributed articles for my book. These included people with TM, parents of children with TM, and medical professionals who specialize in caring for people with this disorder.
The first specialist in transverse myelitis was Dr. Douglas Kerr. He established the first Center of Excellence in TM at Johns Hopkins. The second specialist was Dr. Benjamin Greenberg. He was trained at Johns Hopkins, under Dr. Kerr, and has since established the second Center of Excellence in TM at the University of Texas Southwestern in Dallas. Doug and Ben co-authored the Forward to my book.
Pauline wrote the conclusion; the last words in the book belong to Pauline.
After Pauline died, the last thing on my mind was completing this book. I had finished writing the book before we left for our vacation. The book still required a great deal of editing.
My grief journey was complicated and very difficult. I was in such a bad place. My shock and sorrow were magnified by the tragic way Pauline died, the more than 20 years of struggles she had with transverse myelitis, and losing her when she was so young, and just a year into her much anticipated and wonderful retirement. Everything about the situation put me into a tailspin. There was nothing more that Pauline and I enjoyed together than spending time with our five grandchildren. (Our sixth grandchild appeared more than a year after Pauline died, so she didn't even know about him). It was hard for me to be with the grandkids because her absence was most acutely felt when I was with them. I was a monumental mess.
With the same neurotic focus and energy I apply to all life endeavors, I decided to tackle my loss of Pauline. I refused any kind of anti-depressants or sleep medication, I stopped using any kind of alcohol, I started reading books on the subject of grieving the loss of a loved one, and I got myself into intensive therapy with a wonderful clinical psychologist. I depended on close friends and family who generously offered support to me all along the way. I was and remain so blessed to have such wonderful people in my life. I am learning to be sad from my loss of Pauline while being grateful for all of what remains in my life - Nancy, my family and friends, and my passions, i.e., the SRNA and photography.
I started talking to my therapist about the book. We discussed the reasons I wrote it, and what the book could mean to me after Pauline's death. The meaning of the process and the product definitely changed for me. In addition to all of the information I was motivated to share with the community, I began thinking about the book as a way to honor Pauline's memory and to recognize her wonderful and meaningful life. So, during that first year of mourning, I began editing the book. As noted above, Pauline wrote the last words of the book. I recognized that if I started from the beginning, and worked my way to the end, the build up to the crescendo of emotions from reading her words would be devastating. So, the first thing I did was to go to her words at the end and I read those. I thought about what she said, and I sobbed. Then I went back to the very beginning and started my work.
So much of what I wrote about in the book described Pauline's experiences. All of it was written when she was alive. I didn't have the emotional energy to change the book to reflect that she had died. That process would have been brutal. So, I rewrote the Preface to describe what had happened. I concluded the Preface with the following words:
I have not changed Pauline's story in the book to convey that she died. Her story is told as she was when I wrote it, full of life and hope, and filled with love for the people around her.
I included Pauline's biography. After almost a year of editing, I finally had it completed. It was nothing short of a miracle.
I decided to use my photographs in the book as another way to honor Pauline. I had taken so many beautiful photographs of Pauline from the time I became a photography student. I had taken an environmental and studio portraiture class that gave me the opportunity to take some wonderful photographs of Pauline. I also decided to include images from our vacations, as well as some of Pauline's favorite photographs.
The book is self-published through Kindle Direct Publishing. I have no doubt in my mind that if it was possible, my images would have been color. I was forced by production constraints and cost to use black and white images. It was critical for me to produce a book that was affordable for our community. I didn't want cost to be a barrier to access. Additionally, if you want to use color images, the book needs to be fewer than 250 pages. My book is just short of 600 pages. Remember, neurotically comprehensive.
One of the classes I've taken at Columbus State was digital black and white photography. I had an excellent instructor who was both an artist and a technician. It is possible to shoot black and white images in camera, but I never do so. I always shoot in raw and I shoot in color. This approach gives you the greatest flexibility in how you want to show your images. Once you shoot in black and white, there's no way to retrieve the color from those files. If you shoot in color, there are numerous ways to convert that image to a black and white. The class offered a number of different approaches to get to a good black and white image. My go-to approach for black and white images is to use NIK's Silver Efex Pro as a starting place. I work the image from there.
I very rarely create black and white images. I have great respect and appreciation for the history of photography and the wonderful images that were created by the masters, such as Alfred Stieglitz, Edward Weston, Ansel Adams, Dorothea Lange and Robert Frank. Black and white is just not my thing. Color is a relatively late arrival in photography.
My first serious foray into photography involved my two years of shooting during my cultural anthropology research on the Fort Belknap Reservation in Northcentral Montana. I was on the reservation in the mid 1970s. I used Kodachrome film for all of my outdoor photography. Montana is a spectacularly beautiful state in all seasons. The slightly dark, saturated colors from Kodachrome film was a great way to capture the majesty of the Montana landscape. I returned from this experience with thousands of photographs that I still cherish.
Once I reluctantly accepted that I was only going to be able to use black and white images, I set about selecting the photographs that I thought would be most suited to the drama and detail that are accentuated in black and white photography.
You can find the gallery of these black and white images on this web site:
I hope that these images do honor to Pauline's and Kazu's memories.
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