Sometimes I feel like I need a PhD to understand my own body
It's hard to sort among the overlapping yet varied symptoms of 4 diseases
Immune thrombocytopenic purpura (ITP), thrombotic thrombocytopenia purpura (TTP), and atypical hemolytic uremic syndrome (aHUS) can make for a confusing “word soup,” as I’ve heard people say. These three strange and dangerous diseases can occur concurrently, despite how rare each one is. I have them all, and sometimes I feel like I need a PhD to understand my own body.
ITP, TTP, and aHUS are all diseases that affect elements of blood production, but each in a different way. When I became sick in 2018, my medical team narrowed the possibilities down to an ITP diagnosis that had been triggered by my lupus. I was weak, exhausted, covered in bruises, had a nonstop menstrual cycle, and overall felt unwell. ITP causes your immune system to attack your blood platelets, which leads to excess bleeding and bruising. While there’s no definitive test for ITP, my medical team arrived at the diagnosis through process of elimination.
So the search ended there. I was given corticosteroids and blood transfusions, and for the most part, they fixed me. ITP explained a lot of things about my body that had always plagued me, including my anemia, mysterious bruises, and debilitating menstrual cycles that lasted three weeks. ITP checked all the boxes. I felt better for a while — until 2020.
Dovetailing diagnoses
That September, I hadn’t felt well for months. Medicaid was no longer paying for necessary care, and I couldn’t afford it otherwise. I brushed off my symptoms, figuring I’d just been overdoing it. But in those first two weeks of September, I declined quickly and badly.
I had exhaustion, weakness, and dark purple bruises all over. OK, I thought, it must be a lupus and ITP flare. I’ll go to urgent care or the emergency room if it gets worse, where I’ll get a couple of blood transfusions and be good. But I began coughing and spiked a 103 F fever at work. My legs kept going numb, so I often stopped what I was doing to rub them. My lower back pain was almost blinding. And I began vomiting blood. With much despair, I went to the hospital the next morning.
The first concerning symptom was my blood pressure, which literally set off blaring alarms at 245/165 mmHg — a lot given that the normal for an adult is 120/80. That was odd because my blood pressure has never been high; I’m normally hypotensive, in fact. Did I have COVID-19? I wondered. Was something else wrong?
A nephrologist told me I was dying from kidney and liver failure. If I’d waited 18 more hours, my children would’ve found me dead in bed. I tasted bile in the back of my throat and nearly vomited. Dying. Can ITP be this dangerous? Maybe it’s lupus nephritis?
Living with lupus for most of my life meant that kidney damage was always a risk. But my doctors said I showed little to no signs of a lupus flare.
While my body was destroying my platelets, however, more was going on: My red blood cells were being torn apart (hemolytic anemia), and blood in several organs, particularly my kidneys, liver, left eye, and uterus, was forming small clots in the tissue, which was killing them. That’s TTP. How was I having platelet destruction while also having blood clots in my organs? It made no sense.
My team knew then that ITP wasn’t working alone. The immediate concern was keeping me alive long enough to solve the puzzle. After my children left, I was rushed to emergency surgery for a dialysis port and started dialysis that night. My first session removed 20 pounds of fluid from my body. My face, neck, legs, and feet became much smaller. My kidneys, however, hadn’t been able to process and remove fluids properly.
I also needed lots of blood — 18 transfusions’ worth, to be exact.
I spent nearly two months in intensive care. Things finally began to turn around after the first four weeks. My original nephrologist had a guess and was so excited that he came to my floor at 1 a.m. still in his pajamas. While they’d tossed around the idea that my ITP had been misdiagnosed in 2018, he realized that beyond my lupus, I might also have ITP, TTP, and aHUS: three rare autoimmune diseases that all affect blood production in different ways.
The test for aHUS took a week and confirmed this doctor’s suspicions. Then came the task of treating each disease. That involved continued dialysis for my kidneys, more blood transfusions, and monoclonal antibody infusions with eculizumab (well known as Soliris) to stop aHUS symptoms and complete plasma exchange with plasmapheresis.
Having full diagnoses after many weeks in the dark was both monumental and overwhelming. Nearly five years later, I still get confused sometimes. I have a hard time separating my daily symptoms, as many of them overlap. Being able to discern which disease is flaring at any given moment can be a matter of life and death, so I have to stay vigilant.
Note: Bleeding Disorders News is strictly a news and information website about the syndrome. 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 Bleeding Disorders News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to bleeding disorders.
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