Bubble Boy Disease



Bubble Boy Disease also known as SCID, is a congenital problem occurring among children leading to failure in their immune system effectively functioning, thus making them more prone to fungus, bacteria and virus-related diseases.  The term ‘bubble boy disease’ came into existence due to the familiar SCID kid several years back that was placed in a specialised ‘sterile bubble’ for safeguarding him from infections. When SCID is undetected and not treated, such infants rarely live past a year’s time.

SCID treatment is conducted by transfusion of bone marrow cells from a matching donor in good health. These transfused stem cells attain maturity in the receiver’s body transforming into T and B cells – the specialized WBC’s known as lymphocytes that don crucial roles as infection-combatants and help safeguard the immune system.

bubble boy diseaseA live shining example of the efficacy of this treatment procedure is A.J.Williams who was a mere 8 pounds in weight when he was 4 months old. His mother Erika Williams recollects in utter aghast how his tiny chest caved in due to the breathing distress he experienced during those times. Many doctors located in close proximity to their home in Atlanta, Ga., had failed to detect anything unusual in A.J. According to the several doctors, A.J.’s puking, loose motions and weight reduction was some condition that was happening a lot in those places. The child was also suffering from an oral thrush – an infection that occurs in the mouth – that failed to subside.

After 5 days when A.J. parents paid a visit to the doctor’s office to get their son checked did they come to the realisation that their petite son was indeed in deep trouble, gravely ill and required major medical assistance.

A.J.’s mother, Erika recollects how on seeing their son, the nurse at the local hospital told them to immediately stop penning down the paperwork and instead rush their son to the emergency room. On diagnosing A.J., they realised that he had a congenitally defective heart value.

During the course of the ultrasound procedure, the doctors came to the realization that his heart was not the offender, but that he was ailing from a kind of pneumonia generally observed among AIDS-inflicted individuals. Further tests excluded the presence of AIDS, but revealed that A.J. ailed from SCID.

Unfortunately, no matching donor was found in A.J.’s case. He required a specialised type of transplant wherein the bone marrow cells taken from the parent undergo purification process before the transplant procedure. His mother was finally chosen as the nearest match.

Another unanticipated snag subsequent to the successful marrow transplant was that A.J. had lost the bottle sucking habit. To overcome this problem and aid in increasing his much-required weight the parents had to employ a feeding tube for nearly a year.

A.J. now past three years of age is a bundle of mischief doing all the usual boyish stuff and in good health.

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