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Severe Combined Immunodeficiency Disorder, or SCID.

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Introduction

Severe Combined Immunodeficiency Disorder, or SCID, is a rare genetic disorder in which components of the individual's immune system is missing. This results in making the victim susceptible to disease without much chance of survival. A simple sickness could be fatal to a SCID patient. In order for these people to live, they must reside in germ-free isolation their whole lives because their immune system can't fight against bacteria or viruses that may be easy for a person with a normal immune system to overcome. Much work has been done to change the devastating effects of SCID, including gene therapy, which so far, has proven to be successful. Most cases of SCID are diagnosed within the first few months of life. They are very likely to come down with pneumonia, meningitis, or sepsis, which can be fatal diseases to the young victims. Medication is futile because their immune system does not function properly. They either have irregular or absent B-cells or T-cells, which are the white blood cells that fight off disease. ...read more.

Middle

Children with the disease usually fail to gain weight or grow normally and without proper and immediate diagnosis and treatment, the child usually will not survive longer than one year old. They develop chronic illnesses such as skin infections, yeast infections in the mouth, diarrhea, liver infections, as well as many other life threatening ailments. So they are sentenced to a life of solitude. The typically curious and adventurous child is condemned to confinement, with no friends to play with (because they may bring in outside germs) as well as having all the child's toys constantly sterilized before being able to play with, and only sporadic trips to the hospital for any breaks in the child's monotonous day. "This once-fatal disease should now be seen as a pediatric emergency, a condition that needs immediate diagnosis and treatment," said Dr. Rebecca Buckley, Duke's chief in pediatric allergy and immunology division in regard to the early detection of SCID. Parents with history of the disease in the family may get a procedure called CVS or amniocentesis done to for an early diagnosis of the child's condition. ...read more.

Conclusion

Now with more advanced technology, more and more children have been saved from this disease. In 2000, the first two cases of curing the disease by means of gene therapy in France were successful. Gene therapy is a very intricate process. It involves the removal of some stem cells from the SCID patient and in a laboratory, a normal gene is added to the abnormal stem cells. The now genetically altered stem cells reproduce, all harmless because of the addition of the normal gene, and are introduced back into the patient's bone marrow. The stem cells reproduce quickly and in a matter of weeks, the patient can now produced B-cells and T-cells independently, which fight off any foreign infections. Because this a relatively new method of curing a genetic disease, the long term effects are unknown because all the patients have this procedure done at a very early age (Tesla 1043). Luckily, the children who got gene therapy are able to live normal, healthy lives and can finally live outside the bubble. ...read more.

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