Evan’s Syndrome

Who is affected?
Most people with Evan’s syndrome are likely to be diagnosed during childhood, but people can go from childhood to adulthood without realizing they have the disease. It is a rare disease, but the median age for when the disease presents itself is 7.7 years.
What is it?
Evan’s syndrome is an autoimmune disorder in which the body makes antibodies that destroy red blood cells, white blood cells, and platelets. There is no specific cause for it, and it is said that a cause may be different for every single case. Usually, patients are first diagnosed with either Autoimmune hemolytic anemia or idiopathic thrombolytic purpura (ITP). With this disease, the patient can be affected with low levels oexternal image redbloodcells.jpgf all three types of blood cells at once, or it can be affected by one or two types.
How it affects the body and where?
-If the red blood cell count is down, your body will become weak, you may experience fatigue, or you will experience shortness of breath.
-If you have a low number of platelets, you are susceptible to bleeding and bruising from minor bumps or cuts.
-If the white blood cell count is low, you are susceptible to infections and have difficulty fighting off infections.

Treatment:
-There is no definite cure for Evan’s syndrome
-Steroids are frequently used to suppress the immune system or decrease production of “bad antibodies”
-Intravenous immune globulin(IVIG) is used as chemotherapy
-Blood transfusions are performed in crisis situations, but they usually do not last long since the cells are destroyed quickly by the body external image 19449.jpg

-Rituxin or Romiplostimmay may also be administered
  • -Some physicians choose to treat the disease with splenectomy, but it has a low success rate and a considerable recovery period



Living with Evan’s syndrome…

Most people with Evan’s syndrome acquire a lot of bruises, mainly on their arms and legs. They continually have illness after illness and are regularly in the hospital treating infections. Patients also go to the hospital regularly for blood transfusions once their cell count is really low. One patient, Katie Addington, was diagnosed as a child, and she is injected with IVIG at home every three weeks which allows her to stay out of the hospital and fight infections better.

Sources:
  1. http://www.evanssyndrome.org
  2. http://www.evanssyndrome.net/index.php
  3. http://emedicine.medscape.com