In 2007, the Blatter family was excited to welcome their son, Hayden, into the world. What they were not expecting on this journey was to hear that at 5 months old, Hayden was suffering from Wiskott – Aldrich syndrome (WAS), an autoimmune disease that without a bone marrow transplant is fatal.
What Is It?
WAS is an inherited disorder of the immune system that is characterized by recurring infections, low numbers of circulating blood platelet cells (thrombocytes) and a skin rash known by the medical name of eczema. As a direct result of the disease’s malfunctioning affect on the body’s T-cells and B-cells, coupled with its interfering with platelet production, individuals with WAS are highly susceptible to viral and bacterial infections, as well as excessive bleeding. Because the genetic nature of this disorder is associated with a mutation found on the X chromosome, WAS can only affect males.
What Are Some Other Symptoms of WAS?
In addition to sensitivity to infection and bleeding, autoimmune disorders are a frequent occurrence in individuals suffering from WAS. This includes conditions such as vasculitis, arthritis and various types of anemia. An increase in chances for malignancies including brain tumors, leukemia and lymphomas are characteristic of WAS as well.
What Are the Treatment Options?
A bone marrow transplant is the only known cure for WAS. When starting the search for a bone marrow donor, it is always best to begin with family members, as they serve as the most immediate chance of being an exact match. If an individual does not have a relative who is a match, enrolling in a donor program is the appropriate next step.
In addition to a bone marrow transplant, an individual suffering from WAS usually receives monthly infusions of gamma globulin, or as it is commonly known, IVIg. Other symptoms associated with WAS, such as eczema, are treated with various forms of skin care medication.
As for Hayden, his family found a donor match and recently celebrated his being WAS-free for more than 2.5 years.
Sources:
St. Jude Children’s Research Hospital – Disease Information for Wiskott – Aldrich syndrome: http://www.stjude.org/stjude/v/index.jsp?vgnextoid=dd8c061585f70110VgnVCM1000001e0215acRCRD&vgnextchannel=bc4fbfe82e118010VgnVCM1000000e2015acRCRD
Genetics Home Reference – Wiskott – Aldrich syndrome: http://www.cnn.com/HEALTH/library/polymyositis/DS00334.html