Disappointment and gratitude can go hand in hand with aHUS

This column describes the author’s own experiences with Soliris (eculizumab). Not everyone will have the same response to treatment. Consult your doctor before starting or stopping a therapy.

Dialysis, blood transfusions, muscle loss, isolation, loss of appetite, infusions with damaging side effects, and the hallway echoes of the slow beeping of patients’ hearts on monitors: With all that, it’s a miracle I was even alive to see each day while in intensive care. But with so much change and an unknown future, I felt like I was riding a thin line between disappointment and gratitude.

In September 2020, my life changed forever. I hadn’t felt well for a while, but when I went to the emergency room, I learned I was dying. Multiple organs were shutting down, I had almost no blood or platelets in my body, and I was getting weaker and weaker as each second passed.

It took over a month to get a diagnosis. It turned out that atypical hemolytic uremic syndrome (aHUS), an ultrarare disease, was the main culprit. After five weeks of being barely kept alive, I was astonished to have a diagnosis, a treatment plan, and a direction. But at that moment, I had no idea how hard the road ahead would be.

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The cumulative toll

The damage was unimaginable. The tiny blood clots that the disease caused in my organs had nearly destroyed my kidneys and liver. They’d damaged my heart and left eye, too. Unbeknownst to us, it had also destroyed my uterus, but it took 10 months and a hysterectomy to discover that. I also had new comorbidities and symptoms that came with my severe kidney and liver damage: chronic kidney disease, uncontrolled hypertension, brain fog, muscle loss, severe edema, and an always upset stomach.

To make matters worse, two other diseases were complicating my health by attacking some element of my blood. While aHUS was causing my immune system to attack my red blood cells, immune thrombocytopenia (ITP) was attacking whatever platelets I had left, and thrombotic thrombocytopenic purpura (TTP) was making small but deadly blood clots throughout my organs. I was much sicker then I realized at the time.

I knew more blood transfusions would be in my future. But we had no idea how long I’d need dialysis. Another month? A year? The rest of my life? The uncertainty of a major life change like dialysis was more terrifying to me than the illnesses themselves. And as I learned more about aHUS, I realized that it came with yet another change: infusions for the rest of my life.

aHUS causes an uncontrolled activation of the immune complement system, which then attacks my blood. The easiest way to stop that is to suppress my immune system. Only a few medications do this job well, and the one that works for me is Soliris (eculizumab), a monoclonal antibody infusion. But there are also consequences.

Life with Soliris

After five years on this medicine, I experience severe hair loss, bone and joint pain, dental deterioration, brain fog, extreme fatigue, migraines, and slow but uncontrolled weight gain. This treatment affects people differently, though, as some may experience no side effects while others might have side effects that are worse than mine.

When my medical team introduced Soliris to me, I wish they’d better prepared me for what it would do. I feel immensely grateful and dejected at the same time. Soliris is what we all call a miracle drug; it’s helped heal many aHUS patients and allowed our kidneys a break from the disease so they can heal. I don’t think I’d be here today without it.

Without Soliris, I never would’ve been able to stop dialysis after five months. That was my biggest goal, and I achieved it. But I wish I’d been prepared to experience the severe side effects, possibly for the rest of my life. Prepared to plan trips and events around infusions for the rest of my life. Prepared to be locked into U.S. healthcare for the rest of my life. Disappointment and gratitude, I’ve learned, can both factor into a person’s chronic disease world; they have their places in mine.

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.