The word 'congenital' means that children are born with a condition. What causes severe congenital immune deficiencies?

It is important to know that severe congenital immune deficiencies are very different from acquired immune deficiencies such as HIV and AIDS, which cannot be cured by a standard allogeneic HSCT.įinally, there is a family of diseases called the Histiocytic Disorders, which are life-threatening diseases that are due to an inherited immune defect. Once discovered, a child with SCID is usually should be kept in protective isolation to prevent catching a possibly fatal viral infection.Īt this time the only known effective and curative therapy is hematopoietic stem cell transplantation (HSCT) from a healthy donor. The most severe is severe combined immune deficiency (SCID), where both the disease-fighting immune system T-cells and B-cells are absent or broken. What is severe combined immune deficiency (SCID)?īecause the human immune system is so complex, there are many possible genetic mutations that can result in severe congenital immune deficiency. When present, SCID and other congenital severe immune deficiencies, like Wiskott Aldrich Syndrome, are life-threatening diseases because the child cannot effectively fight off common infections.Ĭhildren with severe immune deficiencies often need to be hospitalized and can die from common respiratory viruses like flu, RSV or adenovirus, other common viruses like chickenpox or opportunistic infections that only affect the severely immune-compromised (people with weak immune systems) like certain types of pneumonia. This condition is called an immune deficiency or severe combined immune deficiencies (SCID). These are a group of genetic diseases in which a child is born without a normal ability to fight infection.

At what age do people get scids