Being Rare: A Curse or a Golden Ticket to Good Medical Care

Expensive or subpar healthcare is the norm in the United States. There’s a staggering number of inadequacies in our system. But having a rare disease can suddenly give you a golden ticket to exceptional medical care.

I became ill in September 2020 and assumed I had COVID-19 for a second time. I figured I’d wait several hours in the emergency room and leave with a doctor’s note for work. But when my blood pressure set off literal alarms at 245/165, they freaked. In less than an hour, I was whisked through a gantlet of tests that ended with Dr. Prakash Prabhu, the nephrologist who told me I was dying. My kidneys and liver were shutting down, and my red blood cells were breaking down, or hemolyzing.

Once admitted to the ICU, I was questioned about my symptoms for three weeks. I had one to four blood draws daily, dietary restrictions, varied procedures, dialysis, 18 blood transfusions … but no diagnosis.

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One night I heard a commotion in the hall. At 12:30 a.m., Dr. Prabhu rushed into my room, in his pajamas, followed by three nurses. He was pacing and talking about idiopathic thrombocytopenic purpura, atypical hemolytic uremic syndrome (aHUS), and thrombotic thrombocytopenic purpura (TTP). But I couldn’t focus on anything he was saying because he was in his pajamas! They were navy blue, with tiny gold dots and a long matching robe.

I finally asked, “What’s going on?” He stopped in his tracks, as if he realized where he was and how crazy this seemed as we stared at him.

“Ms. Jordan, I apologize for barging in at this hour,” the doctor said. “I’ve been studying your case, and I’m certain you have TTP or aHUS. If I’m correct, we need to exchange your plasma.”

He turned and pointed at the head nurse. She jumped as though he’d zapped her. “Nurse, make the call to the Red Cross. They have this machine, they wheel it in, they can access her port, and we can treat her.”

She timidly interjected, “But it’s almost 1 in the morning.”

That sent Dr. Prabhu into a frenzy again. “I’ll call the Red Cross myself!” he said, pulling out his phone.

We watched him walk to the hall, past my window, pacing and flailing his free arm, yelling at some unsuspecting person with the Red Cross. We all looked at one another and burst into laughter. (Yes, I was dying, but I was the most lively patient in ICU. So we found opportunities to laugh.)

By 2 a.m., the Red Cross wheeled in this massive plasmapheresis machine that looked like the Dalek robots from the TV show “Doctor Who.” (“Exterminate!”) There were buttons, levers, and spinning components.

As I sat hooked up, I felt bad that Dr. Prabhu had come so late. “I’m sorry. This could have waited until tomorrow morning,” I said.

He softly said, “Ms. Jordan, I had to come tonight because I wasn’t sure if there’d be any tomorrow mornings left for you.”

I alternated dialysis and plasmapheresis daily until the test came nine days later. Afterward, I was diagnosed with aHUS, for which I have the rarest gene mutation. So I began chemotherapy, which I still do today.

My rare disease turned me into a priority patient, which was a huge change from my past medical experiences, when the color of my skin drastically affected my care. My rare disease is fascinating and exciting for doctors.

My hospital is a teaching hospital, so I was introduced to between two and 10 students daily. I was one-stop shopping for multiple medical anomalies. They all knew who I was. Everyone thought I was going to die. I’d had liver and kidney failure and 18 blood transfusions, and survived.

Another great example of priority treatment happened when I needed a hysterectomy. My surgeon was all but “fan girling” at the opportunity to operate on me. He even came in on his day off to check on me. He said, “I’m just amazed that I’m able to see this disease in my lifetime.”

Women of color are disproportionately at risk of receiving inadequate healthcare. I’ve experienced this firsthand. Statistically we have higher chances of dying in a medical facility. But having a rare disease has unlocked some kind of cheat code for me.

It shouldn’t be this way, but it is. So we all should do whatever we can to advocate, not only for ourselves, but for anyone navigating the medical jungle that’s the U.S. healthcare system.

I’ve worked with my hospital, infusion center, and former dialysis clinic to shed light on several issues. And I’ve begun a dialogue with one of our South Carolina senators about disability and aid for chronically sick people. And now I’m a columnist here for Bionews, the parent company of aHUS News.

Use your golden ticket to help give others a voice, to begin these difficult conversations, and to bring attention to healthcare shortcomings. There’s always a bright side in any situation. Find yours.

Note: aHUS 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 aHUS News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to aHUS.