Are you aware of this rare kidney disease? (Atypical Hemolytic Uremic Syndrome - aHUS)

What would happen if blood clots formed inside the tiny blood vessels in your kidneys, blocking blood flow to your kidneys? It's scary to think about, isn't it? That's exactly what we're talking about today. This is called Atypical Hemolytic Uremic Syndrome, or aHUS for short. Although it can't be cured, it can be managed well with the right treatment. So let's learn more about it.

What is the difference between Atypical and Typical HUS?

You may have heard of a condition called HUS. We call it "Typical HUS." It's usually caused by a bacterial infection like E. coli, which causes diarrhea. But the condition we're talking about today, aHUS, is different.

This condition, called aHUS, can occur at any age. In young children, it affects both boys and girls equally. However, as they get older, women are more likely to develop the disease. This is because pregnancy can be a major trigger for the disease. It is so rare that some studies suggest that only two in a million people have the disease.

Why does aHUS develop?

Simply put, many people who develop aHUS have a genetic change, or gene mutation, in their bodies. Think of it like there's a 'button' in your body that can cause this disease. But just having that button on doesn't automatically cause the disease. It needs something external to it to be 'turned on', or activated. We call these 'triggers'.

The main triggers can be:

• Pregnancy
• Certain infectious conditions
• Cancer
• Some medications
• An organ transplant
• Some long-term medical conditions (such as systemic sclerosis or malignant hypertension)

What are the symptoms?

aHUS symptoms usually appear suddenly after a trigger has occurred. Flare-ups can be mild or severe. In mild cases, the kidneys may not be affected much, but in severe cases, they can lead to kidney failure .

Symptoms you may experience in the early stages:

• Body aches and extreme fatigue
• Feeling generally unwell

This is a progressive disease. This means that symptoms get worse over time, especially if not diagnosed early. aHUS mainly affects your kidneys. However, other organs, such as the brain, liver, lungs, and heart, can also be affected by blood vessel blockages.

How to diagnose the disease?

If no one in your family has had the disease before, it can be a little difficult to diagnose aHUS. Usually, a nephrologist or hematologist is the best person to know about this. They will pay special attention to the factors mentioned in the table above and refer you for the necessary tests.

What are the treatment options?

Fortunately, there are two approved medications for aHUS.

• `Eculizumab (Soliris)`
• `Ravulizumab-cwcz (Ultomiris)`

Both of these belong to a class of drugs called `monoclonal antibodies.` Simply put, these are artificially made proteins that act like our body's natural antibodies. They control a part of the body's immune system and stop blood clotting.

A drug called ``Eculizumab`` can increase the number of platelets and red blood cells in your blood. If treatment is started as soon as possible , this drug can even reverse some of the damage to the kidneys.

Both of these drugs are given by injection. They can cause minor side effects, such as headache, flu-like symptoms, stomach upset, and body aches. Most importantly, there is a risk of serious infections, such as meningitis (meningococcal disease), when taking these drugs. So your doctor will tell you to get a shot for that, and may also give you antibiotics to prevent the infection.

In addition to these medications, plasma therapy can also be used. This involves giving the patient plasma (the liquid part of the blood) taken from a healthy person (plasma infusion) or removing the patient's plasma and replacing it with healthy plasma (plasma exchange).

If the kidneys do not respond to any of these treatments, the last option is kidney dialysis or a kidney transplant .

Possible complications and emergencies

In addition to kidney failure, aHUS can cause other complications.

Living with this disease and mental health

It's normal to feel depressed and anxious when living with a long-term illness like this. It's not your fault. That's why it's important to take care of your mental health as well as your physical health .

• Ask your medical team for help: Doctors, nurses, and social workers can guide you to places where you can get the psychological support you need.
• See a counselor: Talking to a licensed counselor can help ease your burden and build the mental strength you need to cope with this situation.
• Take care of yourself: Get enough sleep. Find ways to reduce stress. Eat a kidney-friendly diet. Get as much exercise as possible. These things can have a big impact on your overall well-being.
• Talk to your loved ones: Talking about this with family and friends will give you great relief.

aHUS, Atypical Hemolytic Uremic Syndrome, Kidney Disease, Blood Clots, Kidney Failure, Nephrologist, Hematologist, Eculizumab