Severe+Combined+Immunodeficiency

Severe Combined Immunodeficiancy is a group of rare, sometimes fatal congetial disorders, characterized by little or no immune response. The Syndrome is commonly reffered to as "bubble boy" syndrome. The genetic disorder is a defect in the white blood cells. Pateints contract many fatal diseases, such as pneumonia, chicken pox, and meningitis many times.
 * // ﻿ Description://**

SCID is a defect that is inherited on the X chromosome. There are also two other ways to contract it, both in chromosome mutations.
 * Severe combined immunodeficiency (SCID) represents a group of rare, sometimes fatal, congenital disorders characterized by little or no immune response.
 * The defining feature of SCID, commonly known as "bubble boy" disease, is a defect in the specialized white blood cells (B- and T-lymphocytes) that defend us from infection by viruses, bacteria and fungi.
 * Without a functional immune system, SCID patients are susceptible to recurrent infections such as pneumonia, meningitis and chicken pox, and can die before the first year of life.
 * //Chromosome Location: //**

All types of SCID are inherited, with half of SCID cases linked to the X chromosome, passed on by the mom. X-linked SCID results from a mutation in the interleukin 2 receptor gamma (IL2RG) gene which produces the common gamma chain subunit, a component of several IL receptors. IL2RG activates an important signalling molecule, JAK3. A mutation in JAK3, located on chromosome 19, can also result in SCID. Defective IL receptors and IL receptor pathways prevent the proper development of T-lymphocytes that play a key role in identifying invading agents as well as activating and regulating other cells of the immune system

New treatments, such as bone marrow and stem cell transplantation save as many as 80% of SCID patients. Though invasive, new treatments such as bone marrow and stem-cell transplantation save as many as 80% of SCID patients. For children with SCID, the first concern is to prevent infections. Children with SCID need to be protected from germs. This includes keeping them away from crowds and sick people. They are treated with antibiotics to prevent infections such as Candida albicans (a type of yeast) and Pneumocystis pneumonia (PCP) infections. They will also be given intravenous immune globulin (IVIG). Immune globulin is also called immunoglobulin or gammaglobulin. It contains antibodies that would normally be made by healthy B cells to help the body fight infection. Immune globulin is usually infused into a vein
 * //Treatment://**

//**Life Expectancy:**// Pateints usually die after often contracting the fatal diseases listed above many times, they do not usually live past the first year of life. When new treatments are used, the life expectancy becomes higher, but is different for every patient.  Patients usually die within the first year of life if the disease is not immediatly treated. For the first months of life though, it is hard to treat. //**Support Organizations:**// More information on the disease can be found under this web adress link: [] [] //Text under this coloring and font style has been submitted by Harry (stars82). // //﻿Hannah Saunders //