Symptoms

Symptoms of Atypical HUS | Research & Information

Most of the time, atypical HUS does not present with severe initial symptoms. The initial onset can appear to be flu like, with lethargic behavior, pale color and loss of appetite. It has been our experience that adult hospitals may often send the patient home with a flu diagnosis, while children’s hospitals will often do a blood draw and conduct basic tests, such as Hemoglobin, Hematocrit, while blood cell counts, and platelet counts. With this simple blood test, an initial Diagnosis of atypical HUS is quite possible, and the hospital may then conduct Creatinine and BUN tests to further confirm their suspicions.

The flu like symptoms described above will continue to worsen when episodes are active. At this point, kidney function may begin to fall, often quite dramatically. Other organs sometime experience problems in some cases. Quite often, Seizures have been reported, along with other Neurological issues. Sometimes gastronomical problems occur.

During an extended atypical attack or episode, the tell-tale signs of aHUS are very obvious. Hemoglobin levels may fall to 6-7, when normal levels should be 11-13: Hematocrit levels may fall in the low 20s, when normal levels should be in the mid 30s. Creatinine and BUN levels start to rise, characteristics of failing kidney function. Blood Pressure will become a nagging, recurring problem. Diarrhea and vomiting may also be present (sometimes that occurs with the initial onset , at other times it occurs later).

Severe Symptoms

Sometimes, severe symptoms present themselves right away, and is some severe symptoms do not occur until later. These can include stroke, seizures, severe stomach pain, inflamed colon, blood vessel damage and heart attacks. Patients who have recurring episodes will usually experience some sort of major event that occurs elsewhere in the system.

Symptoms of Atypical HUS | Research & Information

Triggers vs Cause

It is important not to confuse “triggers” of atypical HUS with the root cause. In normal life, many of use get colds, the flu, infections, and the body’s immune system deal with those properly. In aHUS, a person may get a cold, and it “sets off or triggers” a full blown aHUS episode. This simply occurs because the body’s immune system is not proper reacting to the event. What happens is a bit complex, but there is a summary. There is a genetic mutation on one of the proteins that regulate Complement. Complement plays a role in the body’s immune system. So the complement system overreacts, and ends up doing damage to the Endothelium walls. The most susceptible part of the endothelium resides in the kidneys, in the narrow openings that clean the blood. So the vessels of the kidney become clogged up, which results in a myriad of problems. The Platelets clog up the blood vessel walls, the red blood cells are now impeded, and may get shattered (Shistocytes) and kidney function starts to fail.