The Atypical HUS Foundation


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courtesy of Bill Biermann

There is no cure for HUS. In fact, there is no standard treatment, as each case is different. Children with typical HUS are in a life threatening situation, but if they survive the intial stages of the disease, they usually respond good to the supportive treatment.

The symptoms are analyzed and a treatment plan is developed. Neprhrologist have tried drugs that interfere with clotting, drugs that interfere with platelet function, blood transfusions, Plasma infusions (plasma is the liquid part of the blood),plasmapheresis (a blood filtering process). The best therapy is to closely monitor the disease, and provide supportive techniques to control blood pressure and minimized the damage to kidneys thru plasma and blood tranfusions. Early dialysis may be life saving until the kidney function is able to return to normal. Recurring cases must be watched very closely, and immediate treatment should begin to prevent another full blown HUS episode.

What combination of drugs and treatments show promise in atypical HUS?

  • Antiplatelet agents act by inhibition of platelet aggregation and thrombus formation. These agents shorten the duration of thrombocytopenia but are not beneficial alone; they are used in combination with plasma exchange.
  • Plasma infusion (fresh frozen plasma [FFP]) is effective in select patients. Plasma infusion supplements deficient plasma factors, with an overall response of 60%. Most patients require a large volume of plasma, which creates the possibility of circulatory overload. Dialysis may be required.
  • Plasma exchange is the treatment of choice in TTP, with an overall response of 80%. This procedure removes insulting agents and supplements deficient plasma factors.

Malvinder S Parmar, MD, FRCPC, FACP, Medical Director, Medical Program, Timmins & District Hospital, Assistant Professor (VPT), Faculty of Medicine, University of Ottawa, Department of Internal Medicine.

Treatments with plasma
Plasma, the liquid portion of the blood, has been quite successful in alleviating many of the symptoms involved in Atypical HUS. A plasma transfusion is similar to any blood transfusion, as the patient is administered plasma over a period of 3 - 4 hours. Plasma contains thousands of different proteins. One of these proteins, factor H, is believed to be the source of the problem for a fairly large group of Atypical HUS patients.

Treatment with Plasmapheresis – MD Website
Plasmapheresis is a process in which the fluid part of the blood, called plasma, is removed from blood cells by a device known as a cell separator. The separator works either by spinning the blood at high speed to separate the cells from the fluid or by passing the blood through a membrane with pores so small that only the fluid part of the blood can pass through. The cells are returned to the person undergoing treatment, while the plasma, which contains the antibodies, is discarded and replaced with other fluids. Medication to keep the blood from clotting (an anticoagulant) is given through a vein during the procedure.

What's involved in a plasmapheresis treatment?
A plasmapheresis treatment takes several hours and can be done on an outpatient basis. It can be uncomfortable but is normally not painful. The number of treatments needed varies greatly depending on the particular disease and the person's general condition. An average course of plasma exchanges is six to 10 treatments over two to 10 weeks. In some centers, treatments are performed once a week, while in others, more than one weekly treatment is done.



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