It’s not surprising that Jess Jacobs’ death this past Saturday hit close to home. Besides our shared name we also share, loosely, professional interests, where we live, and, most remarkably, a statistically improbable combination of illnesses. Jess was 29 and had accomplished an enormous amount in the last decade, especially considering the hours spent sick and in the hospital.
She used the moniker, “Unicorn Jess,” to draw attention to the rare nature of her illness and, more pointedly, to the difficulties she faced receiving proper medical care as one of the one-percent of patients who stubbornly do not fit the prescribed protocols.
To quote her blog, “These real problems can’t be solved with hospital wide technology updates or private rooms. While there’s a huge focus on prevention and population health (as there should be), we need to be careful we don’t forget the 1% of unicorns who make up 20% of healthcare spending. And, while there’s obviously no easy answer, there is an answer.”
She was a unique patient who suffered from a rare disease, possessed a magnetic sense of humor, and maintained a razor-sharp ability to observe, summarize, and then explain the reasons for a situation. Her education, professional training, and natural abilities meant that she was able to exist within the medical system while simultaneously observing it from a distance. Through Twitter and her blog she picked apart minor inefficiencies, egregious errors, and dehumanizing attitudes. But instead of drowning in the details of injustice she was able to explain the underlying systemic issues at the core.
She studied health administration at Georgetown and at The University of California, Berkeley and was a prodigy in her field. If it weren’t for her own health struggles her impact would have likely been reserved to the academic, federal, and corporate systems in which she operated. As it is, those of us in the network of the chronically ill and disabled have been able to benefit from both reading and sharing her insights. Jess Jacob’s illnesses aren’t what made her a unicorn. Her generosity of spirit coupled with a relentless commitment to destabilize a dangerous system are what set her apart.
In the spirit of Jess and coming off of hours immersed in her writing and the writing about her, my mind is spinning with thoughts on the years that I have spent with a rare disease and insights on our broken system. My own experience since becoming ill five years ago mirrors that of almost every patient with a poorly-understood illness. Like Jess, I have both Postural Orthostatic Tachycardia Syndrome and Ehlers Danlos Syndrome.
POTS, though not actually rare, is treated as such by health care providers as a result of its limited research and nebulous origin. It’s technically a neurological condition but its most measurable impacts are cardiac. To further complicate matters, its most debilitating symptoms can be, on any given day, digestive distress, temperature deregulation, neurological impairment, or cardiac complications. POTS is, at its core, a lack of proper control of one’s autonomic nervous system, which regulates nearly every system of one’s body. Neurologic in cause but wide-reaching in effect, we are often passed from doctor to doctor, to quote Jess, like a “hot potato.” Our healthcare system is not designed to respond well to mystery. It’s much easier to patient-blame or pass us on to someone else than to dig in with us. Rare is the doctor who sees our complicated bodies and chooses to devote the time and care to a patient with low potential for recovery.
Perhaps most cruelly, the symptoms of POTS can overlap with the symptoms of psychiatric conditions. A poorly regulated autonomic nervous system mimics severe anxiety. The stigmatization of mental illness is its own deplorable reality. Being mis-diagnosed with a mental illness is essentially removing a patient’s foundation. After years of being told my symptoms were likely stress, I found myself unable to trust my own physical experience and, even worse, my own interpretation of that experience. I was physically miserable and worried that I was causing my own misery. Trying to prove that you are sane is a humiliating and losing battle. Our physical symptoms are blamed on hysteria and we become sicker and sicker. According to most studies, women make up over ninety percent of POTS patients. The odds are against us. The gender gap in patient care is well-documented and women with physical ailments are often called anxious and attention-seeking.
A woman with POTS is in the appalling position of having to prove the existence of an illness that is poorly understood within a system that is predisposed to blame her for her symptoms. It’s important to remember that the women who are forced to prove these symptoms, more often than not, wake up one day to a body that no longer functions. In my case I was in Greece on vacation when suddenly I felt like I was dying. My legs were weak, my skin clammy, I started losing weight and I felt constantly on the verge of fainting. It took me two years and dozens of doctors to be diagnosed. The fight to be heard paled in comparison to the actual physical misery I experienced. Days before getting sick I was a runner. Within a month of onset I couldn’t sit through dinner. The loss of health is terrifying, painful, and lonely. Something is deeply broken when the place we turn for help turns its back on us.
While POTS isn’t rare, Ehlers Danlos is. Jess and I share a diagnosis of EDS, a genetic condition that causes POTS. Those with EDS have connective tissue that is made of material that is not-quite-right. For a reason not understood, faulty connective tissue leads to autonomic dysfunction, another term for POTS. Complete recovery from POTS is rare in general but nearly impossible in those with EDS. Besides doling out my POTS life-sentence EDS causes unremitting pain. Most days range from six to eight on the pain scale. Doctors are quick to question claims of severe daily pain, especially on account of an illness that is statistically rare.
Reading the tributes to Jess online I was struck by her friends’ and colleagues’ horror that while she was uniquely prepared to advocate for herself, the medical system still ultimately failed her. Like Jess, I consider myself lucky. I have the mental and economic resources to fight for myself, many of my friends are doctors, I don’t have to devote all of my energy to surviving financially, I am durable emotionally, and my friends and family are relentless in their support.
Our conditions are rare but so is our privilege. I have the resources to fight but I shouldn’t have to. The status quo is maintained because the burden is on the sick to complain. It can take years to develop some understanding of the subtleties and shortcuts of how our care is organized. Our confusion keeps us weak and silent. The experience of a patient is that of constant disorientation. We are afraid to speak up or make trouble because our lives are on the line. We are afraid of our illness but often we are more afraid of our providers. We want to be liked and compliant because we want to be saved.
Jess fought back. She studied the regulations and the protocols and the unspoken rules and then she pushed against it all. She armed herself with a masters degree and Twitter and fought for transparency and patient-centered care. She didn’t blame the doctors or nurses and neither do I. We are all doing our best to function within limiting regulations and organizations. They want to help us and we desperately need their help but we are both trapped.
I would be remiss if I didn’t acknowledge those of my providers who have provided exceptional care. Their commitment to me and respect for my experience have been lifelines during my years of illness. I’ve been fortunate to see a cardiologist for a few years who is not only brilliant but treats me as valuable. Her team’s care has been exceptional but that should not be the exception. She has been, sadly, her own kind of one-percenter.
We didn’t choose to be sick. For most of us we turned to the first doctor with hope and trust after becoming ill, thankful that there was a place to go for answers. Realizing that the people we rely on to keep us alive aren’t able to provide the care we need is terrifying. Many of us have held onto hope for years too long. We cross our fingers for each new appointment and new provider, hoping this is the one that will come alongside us. We don’t expect them to have all of the answers, but we want to be seen as people and to be heard.
At some point each physically limited person realizes that we are truly in this alone. We must manage our own care, a task for which we are ill-prepared. Not only are we weak and tired but we lack the education to manage a complex illness. But when you are one of the tragic one-percenters the burden is on you, the patient. Our burden is to prove our value. We must never stop asking for what we need. A rare illness is lonely but we must never be ashamed. Despite subtle messages to the contrary, we deserve the best.
Jess pursued healthcare transparency by making her own condition visible. She fought a larger battle by displaying the deficiencies in her own care. Fighting an illness can feel unbearable and unfortunately that is often unavoidable. Jess understood the system on a micro and macro level, something that most patients can never do. She was brilliant and powerful and she used her voice.
For those of us who will not get our degrees in health administration we can share with Jess the most basic of understanding. It doesn’t take her background to know that there is something deeply wrong with the way sick people are treated. The issue isn’t limited to hospitals and doctors offices. The false belief that sick people are inferior permeates everything from film, literature, travel, building design, and the educational system. We are fully human, we are not inferior because we are sick, we are not to blame, our needs matter, and we have something to offer. I want to honor Jess’s legacy by continuing to push against the suffering that is avoidable. With each refusal to accept that we deserve substandard care we are inching closer to a system that treats each person as full, complex, important, and worthy.
@jessjacobs on Twitter