We have been The Transverse Myelitis Association for twenty-five years. Our organization advocates for people who have Acute Disseminated Encephalomyelitis (ADEM), Acute Flaccid Myelitis (AFM), MOG Antibody-Associated Disease (MOG-Ab disease), Neuromyelitis Optica Spectrum Disorder (NMOSD), Optic Neuritis (ON), and Transverse Myelitis (TM). All these conditions are immune-mediated disorders of the central nervous system.
Our membership includes people with all these disorders and their family members. Our membership also includes people with vascular causes of spinal cord disorders, such as arteriovenous malformations and spinal strokes, radiation myelopathies, neoplastic disorders (tumors in the spinal cord) and Lyme disease. I’m sure there are people with other disorders of the central nervous system who have joined our Association, because they had nowhere else to go. Their symptoms are the same as the rare neuro-immune disorders, and the treatments for these symptoms are the same, as well. They likely found the TMA by searching for their symptoms. Often, the immune-mediated disorders are distinguished from the “other” disorders by referring to the former with the suffix, ‘itis’ and the later, myelopathies. Itis means inflammation. Myelopathy is a more general concept referring to conditions of the spinal cord. Our membership also includes people with multiple sclerosis, likely because their first attack was in the spinal cord, and they were given a diagnosis of transverse myelitis. They could have been given a diagnosis of clinically isolated syndrome. The medical community can be imprecise in their use of nomenclature.
People with NMO and ADEM were also finding us, because their doctors were telling them that the inflammatory attack in their spinal cords was ‘transverse myelitis.’ TM was being described as a ‘symptom’ of ADEM and NMO; the spinal cord inflammation. This use of the term transverse myelitis has caused tremendous confusion for both physicians and patients. Many people with ADEM and NMO who were told they had TM believed that they had two disorders, TM and ADEM or TM and NMO.
The TMA has always had an open arms policy. TM is such a rare disorder. Most people who have gone through this experience have developed some serious empathy. It has been a very natural and compassionate approach for us to warmly accept anyone who could benefit from what the TMA has had to offer. In the early history of our organization, we took in everyone to offer support. We didn’t understand enough about these other disorders to think about all the benefits that could accrue from having these disorders exist under one umbrella.
Are these rare neuro-immune disorders related to each other? They could be. We really don’t understand enough today to say what the relationships could be. It is important to understand the way disorders are determined or diagnosed to appreciate what these relationships might mean. Disorders are characterized by a unique set of characteristics. No other disorder can possess the exact same characteristics. When Dr. Douglas Kerr developed the diagnostic criteria for transverse myelitis through his work at the TM Center at Johns Hopkins, this is precisely what he was doing. He defined the criteria to arrive at a TM diagnosis by excluding every other possibility. He had to do it this way, because there is no biomarker for TM and we understand so little about the disease process. TM is diagnosed through observable, clinical characteristics. Observable means using diagnostic tests like MRI and analysis of spinal fluid. TM must be differentiated from NMOSD, ADEM, AFM and MS.
Only MOG-Ab disease and NMOSD have unique biomarkers. All the other rare neuro-immune disorders are diagnosed in the same way as TM; by differentiating the characteristics from related disorders, ruling those out, and then determining that the person possesses the unique criteria for their specific diagnosis. Developing biomarkers would be much easier and faster, and we are advocating for this research across all these disorders. The current approach for diagnosis and treatment is not the most effective.
We understand very little about these rare neuro-immune disorders. All these disorders are thought to be auto-immune. It is believed that people who get an auto-immune disorder have a genetic predisposition for this auto-immunity and are exposed to certain environmental factors that trigger the inflammatory attack. We know next to nothing about either the genetics or the environmental factors that could be involved in these disorders. Having the biomarkers for NMOSD and MOG-Ab disease gets us to a more rapid diagnosis. These biomarkers are first steps in figuring out the disease process, but these are only small steps and we remain a long way from understanding the disease processes. With ADEM, AFM, ON and TM, we haven’t even made these first small steps.
We don’t understand why the attack happens. We don’t know why one person is impacted only in the spinal cord and others have attacks in the brain and or optic nerve or optic nerves. We don’t know why a person has an attack in the thoracic region while another has one in the cervical region. We don’t know why some populations are more prone to receiving an NMOSD diagnosis. We don’t understand why AFM is happening with so much greater frequency in children. We don’t understand why some people have only one attack while others have multiple attacks. Why does TM only occur in the spinal cord?
We don’t have the slightest idea why a person who receives no acute therapy can experience a good recovery from the inflammatory attack. We don’t have the slightest idea why a person who receives high course, intravenous steroids, plasma exchange and Cytoxan can experience no recovery from the inflammatory attack.
Regardless of the rare neuro-immune disorder, physicians are going to treat them in about the same way. They will receive the same acute treatments, they will go through a similar rehabilitation regime, and then their long-term symptoms will be treated in the same way. These similarities are likely a reflection of how much we don’t understand. If we understood the disease processes, it is possible that we would develop more refined targets for acute therapy, and we might also figure out more effective strategies for rehabilitation and long-term therapies and symptom management.
Is it possible that ADEM, AFM and TM are the same disorder? Is it possible that their disease processes are closely related? Is it possible that TM is really several different disorders and that there is some overlap between what happens to a person with TM and a person with ADEM or AFM? In my mind, that there could be relationships is not only possible, it is likely. In my mind, there are no bright classificatory lines around any of these diagnoses. We classify and diagnose disorders based on our current understandings. That information is severely limited. The limits are created by knowledge (or the absence of knowledge), by technology and by resources (money). What we understand about any of these disorders is based on what we can observe. Today’s technology allows us to observe only so much. And limits on knowledge create barriers to where we ‘look,’ ‘what we see,’ and how we make sense of it. We move forward in our knowledge through research. Research is predicated on money. There was a time when I was waiting for Jerry Lewis to get one of these rare neuro-immune disorders. That didn’t work out. I’ve also waited for the NIH to fund a ton of our research. That hasn’t worked out all that well either. There’s just way too much competition for medical research dollars. I believe our best strategy for funding research is to raise the money ourselves. We’re growing our discipline through the James T. Lubin Fellowship. Our program to develop clinicians and researchers has been a great success. We need to do the same with our Pauline H. Siegel Eclipse Fund for Research on the Rare Neuro-immune Disorders! We need to control our own destiny.
As we learn about one of these disorders, it will inform our understanding of all the other disorders. As we develop more effective therapies and treatments, all these disorders will benefit. Our inclusive strategy is the most effective approach for understanding all these disorders. I have no doubt in my mind that we are going to discover even more disorders as we continue to learn. It is likely that some of those new disorders are currently being diagnosed under the term transverse myelitis today.
What occurred in our organization through serendipity has resulted in an important approach to thinking about and understanding all these disorders. I didn’t recruit people with ADEM, AFM, MOG-Ab disease, NMOSD, and ON into our organization. (And how come MOG-Ab gets to be a disease?) I didn’t know about any of them. Like so many occurrences in my life, I get to appear smart and insightful by accident. All these people came to us. We just have a ton of compassion and took them in. That it all makes so much sense from a research and education and support perspective was an after-thought. By looking at each of these disorders separately, we lose the synergy or the collaboration that accrues from a comparative and comprehensive strategy. That this happens doesn’t have anything to do with biology or what makes sense from a research perspective. Unfortunately, some of this has to do with institutional forces that have everything to do with human behavior.
Every time a new organization develops that represents one of these rare neuro-immune disorders, our advocacy and our ability to pursue our research goals is diluted. We are a rare disorder community. We cannot afford too much watering down. If there are organizations that exclusively focus on one disorder and actively ignore other disorders, they artificially turn attention away from seeing relationships. The process of operating with blinders can occur through the development of support groups that raise money for research. They often raise money that dictates how those research dollars are spent, and they are most often focused on ‘their disorder.’ For example, the MS organizations aren’t funding research on the rare neuro-immune disorders. It would however make sense for them to do so from a sound research perspective, as NMOSD was considered MS in a by-gone era, and people who are diagnosed with clinically isolated syndrome could just as easily be told they have TM. As there is no biomarker for either ADEM or MS, how can anyone really be so certain that they aren’t the same thing or that there is some classificatory overlap?
This isolation process can occur in the development of medical centers and specializations. This process can occur in government institutions. It is a complicated proposition and can create artificial barriers to better understanding all these disorders, developing more effective diagnostic approaches, acute therapies and long-term disease and symptom management. This process has everything to do with power, prestige and money and nothing to do with biology. We humans can be way too complicated for our own good.
I am certain that the approach of including all these disorders under our umbrella will create the most comprehensive knowledge about and treatments for these disorders. I am also certain that the numbers of the disorders we advocate for will increase. I am certain that the disorders we advocate for today will change. We will find out that they are something different than what we think they are today. My thinking about these matters is often informed by Dr. Leonard McCoy, and how he characterized the stone age of medicine (today).
Given our expansive and comprehensive thinking about the rare neuro-immune disorders, the name of our organization has been and remains a significant problem. In 1994 we were all about transverse myelitis. Nothing else. Today, we are so much more than The Transverse Myelitis Association. I’ve known this was a problem for a long time. I felt horribly about it. I’ve also felt horribly about war, famine and poverty, and have felt equally capable of attacking all these problems in the same manner. Honestly, the thought of changing our name just seemed way too overwhelming while working full time, raising two teenage sons, serving as Pauline’s primary caregiver, taking care of a home, and managing the TMA as a part-time volunteer.
It has been imperative that our name be inclusive and represent the comprehensive approach we take to advocacy, education, support and research.
Together we are stronger.
On November 1, 2019, The Transverse Myelitis Association became the Siegel Rare Neuroimmune Association.
As the Board of Directors adopted our new name, I was filled with such strong and complicated emotions. There is no escaping that this honor has come as I’ve lost Pauline. In my mind and in my work, this organization, my experiences with Pauline and my love for Pauline have been, and will always be, inextricably bound. Pauline and I were on a truly amazing journey. Her getting transverse myelitis and our work for the Association became such a large part of that journey. Speaking to people on the phone or responding to emails was a daily activity. We traveled across the country attending support group meetings and all our education programs. We made visits to the centers of excellence and attended science meetings with other academic centers and organizations. And we made our annual visits to camp. This organization encompassed so much of our lives. The work I did for all these years was motivated by Pauline … from beginning to end. I still do the work and I still care, because of Pauline. This is her legacy. Having transverse myelitis changed her life; it changed everything about her life. All of what she wanted for a better life I also wanted for her, and I wanted to do my best to make it happen. I do all that I can every day to continue to make a difference.
The Siegel Rare Neuroimmune Association. It is humbling to think about; and primarily because it wasn’t something Pauline and I thought about. We almost never counted coup. We didn’t think much about what we accomplished. We thought more about all the work that needed to be done. I am so proud of the work that Jim, Debbie, Paula, Pauline, and the many volunteers over the years accomplished to grow this organization and to create such a positive and supportive culture.
In the mid-1990s our membership was fewer than 200 people. We are now almost 14,000 people from more than 100 countries around the world. It was nothing short of a miracle that a small group of volunteers could create and keep an all-volunteer organization moving forward for almost 20 years.
I am humbled and so honored that Pauline’s and my work will be recognized in the name. I am grateful that Pauline’s memory is enshrined in the name and the important work of this organization. Thinking about the name is surreal. Mostly it makes me profoundly sad. It will be impossible for me to do this work without feeling the sadness from my loss.
I’m grateful for my time with Pauline and all of what we shared together. I am grateful that we were able to help people when they most needed someone to care. I am grateful that our work has become a way to bring hope to people who so desperately need a reason to hope.
The Siegel Rare Neuroimmune Association.
Humbled. Honored. Grateful. Sad.
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