The Last Place in the World to Be in a Rare Disease Crisis

The emergency room was busier than I’d ever seen it, and due to severe acute porphyria attacks, I’ve been in that waiting room many times. I couldn’t get comfortable in the hospital-commissioned wheelchair, no matter how I shifted my body. And there were no other places to sit.

I avoid seeking emergency care at all costs. This time, I’d waited six days after the pain set in before I sought help. I tried managing my symptoms at home, but the agony in my abdomen grew to a torturous roar and I was so nauseous I couldn’t eat or drink. Finally, when I felt my bowels slowing down, I made my decision out of fear. Lying in bed alone in my house, no one was monitoring my vital signs. I didn’t feel safe. I called my partner, who dropped everything to take me in.

I removed my AirPods; I couldn’t bear to watch another episode of “The Circle.” Soon, I began hearing rumors the hospital was full, that people weren’t moving through the ER because there was nowhere for them to go. A man came in, out of breath, desperately searching for word on his wife, who’d suffered a heart attack and was arriving by ambulance. A woman pulled up to the circle drive with a leg break so bad her bone was exposed. When a nurse accessed my port, he used a family gathering room because it was the only space available.

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We had been waiting four hours and still the crowd around us remained the same. I studied the people, the families and couples. Many were there when I arrived. Through bleary eyes, I noticed their movements, trying to guess what ailment brought them in.

Despite knowing precisely my treatment protocol and the severity of my pain, I doubted my rare disease crisis gave me an advantage in being seen. Especially because I’ve yet to meet an ER doctor who was familiar with acute hepatic porphyria. It was anyone’s guess how much longer before I’d be called back.

A nationwide ER crisis

I expected this chaos to a certain extent. It was January, the height of flu season, and according to the U.S. Centers for Disease Control and Prevention, the country is experiencing its fourth highest rate of new COVID-19 hospitalizations since the pandemic began. Not to mention, the healthcare workforce shortage hit my state and local hospital hard. Even if rooms were available, there weren’t nurses to staff them.

I’ll never know why the people who surrounded me that night were waiting to see a medical professional, but I know they must be desperate for care. Otherwise, they wouldn’t be willing to sacrifice eight or more hours of their lives alone in the waiting room.

According to a 2017 study from the University of Maryland School of Medicine, many people seek care at an ER because they are unable to access healthcare from a primary care physician or specialist during business hours. Knowing they can receive a high level of evaluation and treatment in one place is another benefit. While that report doesn’t take into account the COVID-19 pandemic’s impact on the healthcare delivery system, there remain opportunities for flexibility and support to accommodate patients outside of ER settings.

Rare disease and the ER

What many familiar with this crisis may not understand is how people like me depend on the ER as a cornerstone of our disease management. When my pain gets to a level I cannot manage at home, the ER is my only option. When my bowels start to shut down and I haven’t been able to eat or drink for days, my doctor will send me to the ER so my electrolytes can be monitored. If I have a paralytic ileus, I’ll be admitted to the hospital.

I shifted in my seat and tried plugging back into my reality TV binge, but I was too uncomfortable. It had been six hours, with no end in sight, and I had two choices: continue waiting or try to get some sleep in my own bed.

Feeling disappointed, frustrated, and inferior, that night I chose to leave. I requested that a nurse de-access my port and was wheeled to the door. At that point, it was the most compassionate choice for me, my partner, and my kenneled dog back home. I was willing to tough out the night, knowing I’d be seen by my specialist the following morning.

This experience changes nothing for how I’ll manage my disease moving forward. The next time I have pain so uncontrolled and torturous that I’m scared for my life, I’ll have to return to the ER. I have no other choice.

Note: Porphyria News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Porphyria News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to porphyria.