After six months without any medication for her Stiff Person Syndrome (SPS), a rare neurological disorder characterized by painful muscle rigidity and debilitating muscle spasms, Angela Davis finally scored an appointment for a North Carolina neurology specialist. 

Despite the fact that the neurologist has been “dismissive of the way SPS is handled” during her first appointment, Angela had hope that she would finally receive the pain medication needed to manage her condition – and at least some kindness or understanding from her doctor. 

Unfortunately, Angela faced the exact opposite. Rather than get support from the person who is meant to treat her, she was ridiculed. Harmed, even. 

Angela, a rare disease patient advocate since 2016, especially for people with SPS, has had enough. In her interview with Rareatives, published just before Stiff Person Syndrome Awareness Day on March 15, Angela speaks more about the “zip code effect,” the declining compassion she’s faced from providers as a patient, and the immediate need for a more collaborative healthcare model. 

The Astonishing Lack of Care and Compassion

During her recent appointment, Angela’s doctor worked in tandem with a medical resident. Both checked her reflexes, which were slowed. Then the doctor left the room, leaving the resident to his own devices. He asked Angela what life was like with SPS. 

“I looked at my husband and he looked at the ceiling,” she tells me. “We’ve already been through a quarter of a century explaining this. I know the resident was new, but he had all of my medical records. Why am I trying so hard to get help when I have such a detailed history? Why am I still talking to a brick wall? People get so hopeful when they’re diagnosed that they can find help; I thought that way too. And then you realize that many doctors don’t know what they’re doing.” 

The resident asked Angela to climb onto the exam table, offering no help. “My SPS most affects me when I push myself too hard physically,” Angela explains. “Laundry, housework, medical appointments – that’s when I most feel my muscles seize up.”

After Angela’s husband stepped in, assisting her onto the exam table, the resident continued down an uncomfortable and potentially dangerous path. The resident grabbed and forcefully manipulated her head despite her protests. He wanted to induce a seizure. The pain was immediate and intense. 

“Stop,” Angela told him repeatedly. But the resident would not relent. It wasn’t until Angela’s husband physically stood up to intervene that the resident finally let go.

“I told him never to do that to someone with SPS,” she says, her voice wavering. “He put me in the danger zone with no idea how to treat me. I don’t want to go to the emergency room because he felt like messing around. My neck muscles are in permanent contracture. When someone yanks my head around, it will trigger a major pain event. I had an acute migraine for four days.” 

This incident was neither an anomaly or an accident. Instead, it represents the medical failures and trauma that have permeated Angela’s experience as a patient since she was a child. Doctors believe that her SPS originated from birth trauma, but Angela had no pediatric support: “I was punished because I was sick, but nobody knew what was going on. Nobody took it as an emergency.”

Now, as an adult, Angela finds herself frustrated by physicians’ unwillingness to listen to her needs and her experiences. Despite 26 years of SPS medical records, which her neurologist and resident had already reviewed electronically, and the printed copies of records from the UCLA Neuromuscular Clinic and Johns Hopkins that Angela brought with her, her information was ignored entirely. 

In another effort to induce a seizure, the resident began clapping loudly in Angela’s ears. She says, “There was zero empathy or concern for me. It was heartbreaking. I kept thinking, ‘My life is in your hands. What are you doing?’ But it felt more like I was seen as the enemy and they were trying to break me down. I don’t understand that setup for a patient appointment, and I wish nobody in rare had to fight this hard.” 

As they left the appointment, Angela’s normally chatty husband was unusually silent. Eventually, his voice cracking, he said: “I can’t believe we’re here. I can’t believe you had to go through that.” 

An Urgent Need to Improve Awareness

These dangerous interactions with physicians happen partially because the medical community remains largely uninformed about SPS, despite the condition’s recent spotlight through Celine Dion’s public diagnosis. Even though Angela and others in the SPS community have lived with SPS for years, doctors continue to treat them as if they’re only worth studying – and not deserving of care. 

“I’m the lab monkey. I’m more practice for them than anything,” she tells me. “I don’t mind informing doctors and teaching the future doctors, but I am paying to be seen as a patient and to have my concerns addressed.” 

Individuals with rare diseases face a heavy burden to educate providers on their condition, which takes valuable time away from addressing symptoms or developing treatment plans. The responsibility to prevent medical harm falls onto the patient, inverting how healthcare should function. 

At the same time, Angela faces a catch-22: although physicians expert her to educate them and be the expert on her condition, they also frequently refuse to listen. After a recent fall, her neurologist refused to discuss her injury with her. When she asks for certain treatment or management options, she’s shot down. 

Angela even had to suggest a new anti-seizure med, saying: “I had to tell them what to do, what to prescribe, and how much.”

Then, with a laugh, “I should be paying myself.” 

Her neurologist has refused to give her any infusions, saying that they’re too dangerous. “I’ve had them before,” Angela sighs. “I had a mild reaction, but I’m accustomed to that. I’m used to neurologists who know Stiff Person Syndrome and know that I need more than just diazepam. I need something to control the acute, intractable pain.”

Back at Square One

Yet doctors also give her trouble about how she chooses to manage her pain. When Angela and her husband lived in Maine from 2018-2023, Angela had a medical cannabis prescription. 

A 2016 study in the Journal of Pain Management found that medical cannabis use contributed to a better quality of life in people with chronic pain. Angela experienced these effects firsthand. Not only did real cannabis gummies reduce her intractable pain, but these gummies also improved her mobility and decreased her myoclonic seizing.

“I miss it a lot,” she reflects. North Carolina, where she currently lives, has much stricter regulations — and cannabis remains illegal. 

In fact, according to reporting from Jane Monreal in WCNC Charlotte, North Carolina is just one of twelve states nationwide without some version of legal medical marijuana use. 

“There’s a bipartisan bill in the North Carolina senate to legalize medical marijuana in the state, but the neurologist said there’s no telling how long that’ll take,” Angela says. “He said I could go to Cherokee where cannabis is legal, but gave me no other guidance. Even though medical cannabis has shown benefits, my doctor seems freaked out about anybody being on a ‘controlled substance.’” 

The current approach to pain management in the United States, heavily influenced by concerns about opioid addiction, creates particular hardships for people living with chronic pain. 

Angela has been taking her pain medication for over a decade. Doctors acknowledge this fact, and the severe pain that Angela is in, but they sometimes treat her requests for treatment as a form of drug-seeking behavior. One doctor even told her that he knew she wasn’t drug-seeking, but the clinic had a big problem with addicts. 

Says Angela, “I feel like I’m starting back at square one. Like I’ve lost over twenty years of progress. The lack of care and compassion is astonishing. It’s like all the knowledge of how to treat a patient – medically, personally, and professionally – has been erased.” 

Angela recently ordered legal, hemp-derived CBD gummies after her husband noticed her “literally writhing in my chair,” which she didn’t realize until her husband pointed it out. While the gummies are beneficial for sleep, they haven’t managed to take the edge off of the pain.

“The pain can be the most debilitating part of SPS. I have a strong ability to disassociate from what my body is enduring – I’ve learned to do that from being forced for so long – but shouldn’t we have a medical system where patients don’t have to endure their symptoms? I want doctors to hear me and know that I know what’s going on with my body better than they do,” Angela tells me. 

Angela sighs before continuing, the frustration of being dismissed and gaslit evidenced in her voice. “I’m at a point where I’m progressing pretty quickly and it’s difficult to move at all. You think that would catch their attention. What makes me most frustrated is they aren’t listening to a word I’m saying. They nod their heads, but they’re not hearing me.” 

Dealing with the Zip Code Effect

Angela has asked to be referred to the Duke Neurological Disorders Clinic, where the doctors are “much more serious and know their stuff.” But even this hasn’t been an easy endeavor. Angela has had to call — repeatedly — after being put on the waiting list. And at the time of writing this article, she is still pushing to get in. 

“Persistence is key,” she shares. “When my first primary care provider told me I wasn’t special or important, and that I’m just one patient, it stung. But I kept going. You have to, even when you want to crawl under the duvet and disappear from the world. I just keep pushing forward. I don’t know what else to do. We’re used to pushing in rare disease. We’re really good at that, and we’ve gotten a lot done thanks to our persistence. But it is exhausting. We’re the sick people!”

But Angela’s persistence also highlights another issue that many rare disease patients and families face: access to care. Healthcare disparities based on location – what Angela refers to as “the zip code effect” – can create life-threatening situations for patients and families.

2016 research from the Aetna Foundation discovered that “in various cities across America, average life expectancies in certain communities are 20-30 years shorter than those mere miles away.” But the problem is compounded by the fact that 95% of rare diseases have no FDA-approved treatment. Since specialized care is primarily located in urban medical centers, many rare patients, and especially those with mobility challenges, face seemingly insurmountable barriers. 

As Angela emphasizes, “Where you physically reside determines the quality of your care and whether you’re getting a knowledgeable doctor or not. When there are such human, life-or-death implications, why are we letting it get to that point? Honestly, I’ve told my husband that if anything happens to me, sue the doctors because they haven’t taken care of me and they don’t care.” 

For people with SPS, from infants and children to adults, appropriate care is a necessity. The progressive nature of SPS means that worsening symptoms can lead to potentially fatal complications — and if doctors don’t know how to manage these complications, outcomes worsen significantly.Angela has seen this firsthand. Over the past few years, she has lost six friends to SPS, including one who “had a massive seizure that squeezed her lungs and cut off her ability to breathe.”

As she recounts this, Angela’s voice suddenly grows passionate: “It’s completely embarrassing, the state of our healthcare. I hear about other countries that have universal healthcare and wonder why we can’t dedicate the same level of care to our communities. To the people who need it.” 

Forging a Path Forward

Despite the trauma and structural barriers Angela has faced, she remains determined to make a difference. She shares her story, finds ways to support the SPS community, raises her voice, and continues to affirm the necessity of a collaborative patient-doctor relationship. 

“I tell doctors — there’s always something you can do. Even if there’s nothing tangible or physical you can do, refer me to someone else. I’m not leaving here until you do something,” she tells me. Then, with a laugh, “I can’t move anyways, so I’ll keep my butt in this seat.” 

Looking at Angela’s experience, it’s clear that our healthcare system could make several improvements to benefit rare disease patients. The first, and arguably one of the most important, is educational reform. Medical education typically focuses on more common conditions, centering the idea that “if you hear hoofbeats, think horse, not zebra.” But this approach leaves doctors unprepared to recognize or treat rare diseases. 

Improved education has benefits outside of the immediate geographic area. If a doctor in one state learns more about SPS, or chronic pain, or hematology, or comorbidities, they can share those insights with others. Not every healthcare facility can retain rare disease specialists. But better referral systems, telehealth consultations, and provider training can extend expertise beyond major medical centers to areas of the country where this knowledge is slim. 

Another possible reform is a heightened focus on patient-centricity. Patient expertise and voices must be recognized as valuable. The dismissal of patient-reported history and experiences does nothing but dissuade people who need help from seeking care. By learning from, rather than combatting, patient voices, doctors could develop a foundational understanding of the rare space that poises them to provide future help. 

As Angela’s experiences demonstrate, the current approach to care is not working and takes a heavy toll on people already carrying the weight of a rare disease. A truly patient-centered healthcare system would allow patients to focus their energy rather than fighting for basic care. But if you’re in the middle of the fight now? “Keep your spirits up,” Angela reminds you. “You may think you’re alone, but you’re not.”