In October 2017, at the Q&A following the London theatrical premiere of Unrest, a woman stood up and asked, “Why aren’t you talking about recovery?” She claimed to have gotten well from Myalgic Encephalomyelitis (ME) after a combination of what I can only describe as Lightning Process-like “cognitive training” and group dancing.
I’ve always hated recovery stories. Not because I am not thrilled for the person who managed to find their way to better health — I am. Rather, I hate the way these stories can distract from the essential truth: there is no scientifically validated path out of this disease. There is not even a solid understanding of what causes it. When it comes to recovery, all we have are a collection of stories, one usually very different from the other, all difficult to interpret. Implicit in these stories (and some recovered patients actively promote this message) is the idea that if you only did what I did, you would be well, too.
Recovery stories, which are inevitably stories of individuals, also distract from the most important message: the challenges we face are systemic. Lack of recognition. Stigma. Lack of medical education. Essentially zero access to healthcare. Lack of social and financial support. Lack of funding for research.
Whenever someone shared a recovery story, I tried very hard not to disbelieve them, even if I did not understand it at the time. After all, why would we treat fellow patients the way the medical profession has, for decades, treated us? I have always thought there is value in all of our stories. An individual story might tell us something about misdiagnosis that can help us understand how to better diagnose others. An individual story might also provide a clue to the etiology of this disease. And if that individual story becomes two stories? Twenty? Well, that’s when I think you have to start to looking twice.
My reply to the woman at the screening was something to the effect of:
I have also made improvements thanks to antivirals, mold avoidance, fludrocortisone and Mestinon, but without an understanding of the root pathology, without a biomarker, I cannot not know if I had what you had or if what helped you will help me. We cannot know if what helped me will help others. What we are fighting for is public health, and the delivery of effective treatment at scale. We do not yet know how to match the right treatments to the right patients, or what the universe of those treatments might be. To get there, we need to fund good science.
I never imagined that just one year later, I would find my pathology. It would be treatable. I would get better.
Now that I am a recovery story, I am finding it a very complicated place to be, especially as a very public patient. I ask myself every week, “Am I that person who just bought a Honda and now sees them everywhere I drive?” The difference here is that what started as two anecdotes — me and Jeff Wood — has begun, tentatively, to become anecdata. Since he and I came out about our diagnoses and recoveries, at least 121 people with ICC/CCC ME have been diagnosed with craniocervical instability, 48 with cervical stenosis, 25 with Chiari malformation (still a common, undiagnosed pathology in our patient population), and 12 with tethered cord syndrome. 25 have been given other structural diagnoses. (Many people are diagnosed with more than one problem, so these numbers are overlapping.)
According to a poll on Phoenix Rising, 75.4% of 61 patients who have pursued scans have received one or more neurosurgical diagnoses. Yes, people who have reason to believe they have a craniocervical junction (CCJ)-related condition are more likely to pursue imaging. People with negative results might be less likely to come back and take the poll. Even accounting for self-selection and reporting bias, this is still an astonishing figure.
These conditions have been known to the Ehlers-Danlos (EDS) community for quite some time. This very public conversation has led to several ME patients being diagnosed with hypermobile EDS (hEDS) and in a handful of cases, even classical or vascular EDS. What is new is that some doctors are starting to see cases of craniocervical junction/spinal diagnoses in viral and mold-onset ME patients who do not have hEDS or even any apparent history of trauma. At the same time, we are learning that hEDS, hypermobility, Chiari malformation and intracranial hypertension may all be more common among ME patients than we previously thought.
Of an unknowable number of people who have had surgery, at least nine of us have had a total remission of post-exertional malaise (PEM) and postural orthostatic tachycardia (POTS), and several others, varying degrees of improvement. (These figures include me and Jeff Wood.) Most of those diagnosed have not had surgery and those who have are still very early in their recovery process. One member of our community, Jenny Rowbury, who had surgery in England for both a Chiari malformation and craniocervical instability, and also has vascular EDS, has had a devastating outcome.
Many have been active members of our community for a very long time. Some are #MillionsMissing organizers. Others, patients in Unrest. Some got sick after an accident, surgery or pregnancy. Others, following a viral infection, mold exposure, or tick-born infection. Some of us have hEDS. Others do not. In other words, their stories span the range of experiences in our community. It is growing more and more likely that we constitute a significant subset of people with ME. All this raises more questions than we have answers. So like everyone living with this horrible condition, I say to our clinicians and researchers, we need your help.
It’s been 15 months since my craniocervical fusion and tethered cord release surgeries. Last March, I announced that my postural orthostatic tachycardia (POTS) was gone. Then in May, I announced that all my ME symptoms (PEM, muscle fatigability, and all of the other symptoms in the table at the bottom of this page) were gone as well.
This was not the order or timeframe in which things happened, though. My ME symptoms had already remitted by the time I woke up in recovery on November 28, 2018, having resolved in all likelihood during hour four of my fusion surgery, the moment I was put in intraoperative traction; made permanent thanks to six screws, two rods and a heaping serving of bone matrix protein and cadaver bone. Aside from POTS, which came and went for several weeks after my post-surgical swelling kicked in, my ME symptoms have never come back.
The fIfteen months since that day have not been easy or linear. My recovery story has become my recovering story. And since I hate simple stories, now that mine is proving to be anything but simple, I’m ready to share a little more of what my journey has been.
Yes, my ME is really gone
I’ve heard people say, “Well, maybe she just thinks she’s better because some of her symptoms have improved, and when you’ve been severely ill, better can feel like well.” The bottom of my remission post has a full table of all the symptoms I experienced pre-surgery and which have since resolved. My ME is well and truly gone.
I suspect it will remain gone for the rest of my life, unless I develop a new problem with my brainstem or cervical spinal cord.
My mast cell activation syndrome has dramatically improved
I came to realize over time that my mold sensitivity was due to mast cell activation syndrome (MCAS). I think the relationship between CCI and MCAS can work in both directions: mast cell activation (e.g., due to mold exposure) might break down collagen, leading to an unstable CCJ. But spinal injuries can also cause mast cell activation. After an initial post-surgical mast cell flare, my reactivity has been steadily decreasing. I can eat foods I could not eat before. I can tolerate environments that used to be intolerable. Mold doesn’t bother me as much as it used to. Chemical fragrances don’t bother me at all. I can go longer than I care to admit without taking showers! MCAS is still definitely there in the background, but most days I don’t even notice I have it. Life has become a whole lot more manageable as a result.
I’ve stopped all my ME-related prescription drugs
I do still need to take thyroid replacement hormone and will for life (I had a total thyroidectomy in 2018 for Stage 1 papillary thyroid cancer). However, all the prescriptions I was taking to manage my ME and POTS––Valcyte, Valtrex, Mestinon, and fludrocortisone––I was able to stop within a month following surgery.
I can get “normal sick” again
Since I first became bedridden due to ME, I stopped being able to be “normal sick.” Every time my husband got a cold or the flu, I would “crash” terribly. But my temperature would never spike. I would not get as much as a sniffle. Doctors would say, “What are you complaining about? Your immune system must be really strong!” I have always said that the day I can muster a temperature over 100 degrees will be the day that I know I am truly recovered.
Since my surgery, I have had a cold — twice! Sore throat, runny nose, the whole shebang. It’s not fun, but it really is just a cold. I take it as a sign that my immune system is functioning more normally again.
And then in November, a minor miracle: I had my first fever since in almost nine years.
I have normal physical energy
Since surgery, I have not been able to find a physical activity intense enough to crash me. I can do an hour-long aerobics class. I can hike on hilly terrain for four days in a row. I can close out a dance floor at 1am.
Drinking used to make me sick. Now, I can even drink gin & tonics and martinis again (thanks, I think, due to improved MCAS).
I have normal cognitive function and stamina
My brain works now — really, really well. I believe this is due to a normalization of cerebrospinal fluid flow, blood flow, and intracranial pressure (see my Path to Diagnosis articles). I can read and can write long-form again, which I found nearly impossible to do for years. My ability to multi-task and my executive function have also dramatically improved. Cognitively, I cannot remember the last time I felt this well.
Surgery was brutal
I am not even sure what I can say about this. My surgeries were brutal. They were (and I hope will always remain) the greatest traumas of my life. I had it harder than most. Most people having fusions are “in and out” in 5–10 days. I had three surgeries over the course of six weeks and several complications including a spinal fluid leak and severe mast cell flare. I cannot tell you how many times I found myself waking up in the middle of the night, screaming with a level of pain I did not know was possible, or becoming non-responsive after simply trying to walk down the hallway.
After I came home, there were many more months of swelling and inflammation around my surgical sites that, in essence, intermittently re-compressed my spinal cord. Some symptoms returned. I wondered whether my early gains would be permanent. This is proving to be a common experience. The first three months are hell. Then at the end of three months, things suddenly get much better. They get even better at six, and again at twelve. (If you are pursuing diagnosis or contemplating having surgery, go on Facebook, search for groups dedicated to your diagnosis, and get peer support. Selecting a skilled and experienced surgeon is important. Recovery is a long process and no one gives you a roadmap.)
Despite all that, I would do it again in a heartbeat.
My body does not work as well as my nervous system now does
For a long time, it felt like someone had taken a beat up, 1996 Chevy and replaced it with a 1500 horsepower engine but left the body. Yes, I could now go 300 miles per hour now but if I did, the wheels would spin off.
When I first got out of the hospital, I felt like I had more energy than I could ever remember having. Yet, I was so weak and in so much pain, I could barely walk across the room or sit in a chair. For months, I struggled. Then, as the post-surgical swelling subsided, I realized the more I did, the more I could do. First I walked in pools. Then, on dry land. I lifted one pound weights. Then three, then five. Every day, I pushed myself to the limit. At night, I would curl up in bed, exhausted. The next morning, it was like a truck had run me over. I think my muscles were sore for six months straight. But I did not crash. I knew that if I forced myself to get out of bed and move around, I would feel better. Then, I would do it all again. Physical therapy, exercise, and strength training became the center of my recovery. I can now hold a plank for two minutes. I can do thirty squats. I can walk 20,000 steps in a day. Yesterday, I did this “bird dog pose,” ipsilaterally (on the same side) for the first time.
This all would have been impossible when I had ME. In fact, attempting to do even basic, daily activities of living would have crashed me in bed for a month and possibly made me permanently worse. Exercise was poison. But post-surgery, I was no longer an ME patient. I was “simply” a severely deconditioned patient.
Now, the more I do, the better I feel.
Up is up again.
I suspect I have a connective tissue disorder
It is not hypermobile EDS, at least not by any of our past or current standards. (As I have written many times, I do not check a single box on this list.) I would not even call it hypermobility spectrum disorder (HSD). But like many ME patients have reported, I had some significant connective tissue changes after my viral onset. (For one, my nails became ridged and their lunulae disappeared.)
What I and a growing number of people have started to wonder is whether MCAS can cause many of the same complications people with hEDS are at risk to develop, even in those of us without genetic hypermobility, resulting in a distinct but overlapping phenotype? Or, might there be other connective tissue genes at play that are just not as clinically obvious as those that cause hypermobility?
If true, it would mean that while I have made remarkable gains, I can always develop new problems in the future. I am grateful for what I now have, for as long as I have it. I am doing everything I can to stabilize my mast cells, avoid mast cell triggers, and promote healthy collagen metabolism, despite scant evidence and all that is still unknown.
I have had trouble with recurring spinal fluid leaks
My days of hiking the great southwest were cut short after I developed a spinal fluid leak in late August. My physical therapist recommended I start doing daily massage of my tethered cord scar, that 8-inch doozy on my back (I have a much longer than normal scar. In addition to the tethered cord release, my surgeon had to evacuate a hematoma that developed following an earlier blood patch.) Direct massage of the scar immediately caused swelling and neurological symptoms, and triggered a recurrent spinal fluid leak. My neurosurgeon thinks the spinal fluid leak happened because that same part of my spine was found to have diverticula or “blebs.” Blebs are weak spots in the dura, that thick membrane of connective tissue that surrounds the brain and spinal cord. And they have a tendency to leak.
For two and half months, I spent most of my time flat in bed. I leaked and self-patched four times. When I leaked, I would get a headache in the front of my head, feel like I wanted to vomit, and think I was going to pass out. This was due to intracranial hypotension (low pressure in my skull). After 1–2 weeks flat in bed, I would develop wicked rebound hypertension in the form of a pressure headache. Now, I had the inverse problem: I could not tolerate lying in bed. However, if I stood too long or sat in a chair for even a short period of time, I would start leaking, due in part to the rebound pressure, and have to lay flat in bed again. Rinse, repeat.
I finally broke the cycle by taking treatments to bring the rebound pressure down to a manageable level (an herb, actually — Diamox doesn’t really help in my case). I made sure to lay flat for several days after self-patching. Then, very slowly and carefully, I increased the amount of time I spent out of bed. It took a lot of trial and error to get the balance right.
Aftercare and rehabilitation are everything
Well…almost. The most important thing is to find a surgeon who knows what they are doing. Because I got good advice, that was the easy part. Much harder was what came after.
I remember my surgeon telling me the day after my fusion, “50% of your recovery just happened in the operating room. 50% is the work you are going to do over the next year.”
“Great!” I thought. “I’m in. I want to be as well as possible. I’ll do the work!” But no one tells you what that work is supposed to entail, the help you’ll need, or how to find it. I actually don’t think anyone really knows.
Eleven months after surgery, I finally found the right rehabilitative help in the form of a chiropractor who specializes in sports medicine and in particular, helping athletes who have had surgery recover from their injuries. I had avoided going to this clinic, even though it’s not far from my house. The doctor is a serious weightlifter. He’s huge! All the photos on his Yelp page are of him with pro athletes. I thought, “This guy is definitely going to push me too hard. I might get hurt.” Then one day, desperate with back pain, I made an appointment. He ended up being the kindest, gentlest healthcare practitioner I’ve encountered in my life. When we started he was cautious, respectful of my boundaries, and observant of where my body actually is. His practice is a mix of chiropractic, physical therapy, and soft tissue work (e.g., massage, Graston technique). He focused on building up my core strength, which all of the other physical therapists I’ve worked with (and there were many) mostly ignored. He is also working on progressively stretching and reeducating muscles in my hips and legs that had grown too short or stiff from all those years in bed. He’s the reason I can now do planks for minutes, why I can sit in a chair without springing a leak. Without this help, I would still be profoundly debilitated.
I don’t know what comes next
The post-surgery healing is definitely behind me. They told me it would take a year, and it did. But eight years of being bedridden and a wheelchair user? Again, I don’t really have a roadmap. I think it will take another year to fully build up my strength and maximize my recovery. Healing from all this is a long process. And with the surgeries I’ve had and the underlying connective tissue disorder that I might still have, I don’t consider myself out of the woods. I have six screws lodged in my skull and first two vertebrae. I have three laminectomies in my upper lumbar and lower thoracic spine. I will have to strength train for the rest of my life to keep my core strong. I fear what having a fusion and back surgery will be like in old age.
All that, I can deal with.
What I haven’t quite figured out yet is what to do with what remains. One, the emotional trauma. Eight years of ME. Six months of daily, waking apnea and paralysis. It gets better with time but I don’t know if the ground will ever feel solid.
Two, the knowledge that there are a whole lot of other people out there, in this world and in our own community, who suffer from an array of connective tissue disorder-related complications/pathologies (Chiari, craniocervical instability, atlantoaxial instability, cervical stenosis, Eagle’s Syndrome, median arcuate ligament syndrome, the list goes on…), but who cannot access the extensive diagnostic and treatment options I’ve had. Because I live in the United States, where many of the EDS specialist neurosurgeons practice, and my husband works for a large organization that offers exceptional health insurance, all of these diagnostics and surgeries have been almost free.
Three, the knowledge that we aren’t doing nearly enough to define, measure, understand, and ultimately fix this problem. Many clinicians have known about these diagnoses since the 1990s, when the neurosurgery boards issued a moratorium on performing surgery on any patient with a chronic fatigue syndrome or fibromyalgia diagnosis. (Craniocervical instability is the exception. In the context at least of non-traumatic cases, its diagnosis and treatment is still somewhat new.) Maybe they thought surgery was all too serious, all too scary, and far too dangerous for such trivial-seeming illnesses. (Fusion surgery at least is not nearly as dangerous as some think.) Did we miss something big because we were too afraid to look?
I can’t help but wonder what might have happened if we had all collectively stared these pathologies in the face in the 90s. If we had tried to understand the underlying disease process that causes them, named all the risk factors, could we have learned how to prevent or even repair the damage? Would I have ever needed surgery?
Read all the posts in my CCI + tethered cord series
Read this disclaimer. Crucially, surgery carries risks and it’s important to remember that in medicine, the same exact symptoms can have multiple, different causes. We have no idea how prevalent CCI is in our community and there’s been no research into its relationship with ME. We do know that it is more common among patients with EDS.