Atypical Hemolytic Uremic Infusion Therapy

Atypical Hemolytic Uremic Syndrome (aHUS) is a rare illness that runs in families. It harms blood cells and kidneys. The body's defense system gets too active and attacks itself. People with aHUS may feel tired, bruise easily, and pee less. Doctors use special drugs to calm the body's overactive defenses. Without help, aHUS can make the kidneys stop working.

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Symptoms

Symptoms

Symptoms of Atypical Hemolytic Uremic Syndrome (aHUS) include:

Tiredness
Pale skin
Easy bruising or bleeding
Confusion
Less peeing
Swelling (edema)
High blood pressure
Stomach pain
Nausea or throwing up
Shortness of breath

These signs can come on suddenly or develop slowly. Some people might have only a few symptoms. If you notice these problems, especially together, it's important to see a doctor quickly.

Treatment Options

Treatment options for Atypical Hemolytic Uremic Syndrome (aHUS) include:

Complement inhibitors:

Eculizumab (Soliris)
Ravulizumab (Ultomiris)

Plasma therapy:

Plasma exchange
Plasma infusion

Kidney support:

Dialysis (temporary or long-term)

Kidney transplant:

For severe cases with permanent kidney damage

Blood transfusions:

To address severe anemia

Blood pressure medications:

To manage hypertension

Supportive care:

Fluids and electrolyte management
Nutritional support

Genetic counseling:

For families with inherited forms of aHUS

The main treatment goal is to control the overactive complement system and prevent organ damage. Complement inhibitors are now the primary treatment, often replacing plasma therapy as the first-line option.

Living With

Living with aHUS involves regular medical care, including doctor visits and treatments to control the condition. Patients focus on maintaining overall health through diet, exercise, and infection prevention. They must be alert to potential flare-ups and have an action plan. Lifestyle adjustments are often necessary, including work or school accommodations. Mental health support can help cope with the challenges of a chronic illness. While aHUS requires ongoing management, many patients can lead fulfilling lives with proper care and support. Staying informed about the condition and new treatments is important for effective long-term management.