Atypical HUS

So your doctor has gotten your test results back and has told you that you have ATypical HUS. You've never even heard of this disease or known anyone who has gotten it. So lets get you started with the facts about this disease and current methods of managing the symptoms.

Atypical HUS

Atypical HUS is a disease of the blood. It was discovered as a disease in 1965. At that time there was no medicine for the disease. The medicine to treat the disease was only released for use in 2011 after many trials. Until then the mortality rate for this disease was anywhere from 40 to 65 percent. Before the medicine Eculizumab was released the only treatment was Plasma exchange. For many this didn't work. The disease can mainly attack the kidneys. This can occur at any age and it causes abnormal blood clots to form. The blood clots can form in the kidneys, liver, heart, brain etc. They then block the flow of blood and can then cause organ failure or death. There are certain symptoms you will notice. One symptom is puffiness of the body. You can also notice confusion in the affected person. Another symptom is extreme tiredness. You can also note that the person may stop urinating. The disease can also cause loss of appetite. Diarrhea is also a symptom.


1. Dialysis

2. Plasmapheresis.

3. Kidney transplants

4. Soliris infusions

1a. Dialysis will take the place of the job the kidneys do but it won't cure the disease.

2a. Plasmpheresis will clean the blood and remove the clots but this is only temporary

3a. Kidney transplants can't stop the disease which will then damage the new kidneys.

4a. Solaris is the one hope for people with this disease. It supplies the necessary protein to stop the body from damaging the red blood cells.

People with this disease need to watch their diet, and make sure they don't eat foods with too many phosphates. These are found in many prepared foods. They also need to restrict salt in their diet. The bi-weekly infusions with Eculizumab must be done to stay in remission. It is very important to keep away from people with illness and a flu shot every year is mandatory. The person needs to work closely with a dietician to make sure they do everything possible to stay healthy.

How common is Atypical HUS

Atypical HUS is diagnosed to 1, person out of 500,000 people. It is extremely rare and can be identified by genetic testing although not every person has the known genetic markers. Scientists are still attempting to identify other markers.
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What is the long name for Atypical HUS is Atypical hemolytic-uremic syndrome

People who have ATypical HUS need to manage their disease. If you go to the website below you will find a Grab N Go Tool kit which will help you do this. Here you will fine a downloadable LabTracker to record your labs. There is also a Patient Self-Advocacy Tool that you can also download. Lastly there is an urgent care directive. You need to keep this filled out form with you at all times so when you are away from home and you many need urgent care, the doctors will know how to treat you.

Edward Nielsen 12/1/14 D2