Catatan Etja

June 7, 2012

The Bubble Boy, A Story of a Boy with Severe Combined Immunodeficiency

Gambar

Let me tell you a story, about a boy named David Phillip Vetter (September 21, 1971 – February 22, 1984). He was a lovely young boy. Perhaps, not so many people know his name, he was often referred as “Bubble Boy”. He was diagnosed with X-Linked Severe Combined Immunodeficiency (SCID), a rare genetic disease which caused failures in his immune system. In this disease, there is a mutation in the long arm of X chromosome. This mutated area prevents the construction of many immune related cytokine receptors, most notably IL-2 receptor. IL-2 Receptor plays an important role in proliferating T Lymphocyte cells. Lack of T-Cells, particularly T-Helper Cells, will also negatively affects the other arm of adaptive immunity, B-Cells. To put it simply, people with this disease are extremely vulnerable to any kind of infection, be it intracellular or extracellular pathogens.

David Vetter was already diagnosed with this disease even before his birth. However, at the time he was born, treatment for this disease was not fully established yet. The only option for him was living an isolated life in a sterile environment for his entire life. Just a moment after he was being removed from his mother’s womb, David entered a sterile plastic room. And that was how he got his nickname “Bubble Boy”. His doctors at the beginning, planned to perform bone marrow transplant with his sister as a donor. However this plan was canceled because his sister was not a match.

Initially, though there were many problems, there was no major problem. Everything seemed to be just fine. His parent and his doctors tried their best to provide David with a normal life. Problem begun to arise when little David started showing his interest in interacting with outside world, which unfortunately was impossible for him. Can you imagine how would that feel for a little kid to live in his extremely sterile “prison”? David started having nightmare, in his dream he saw “King of Germs”. He was really distressed. Ultimately, he started to become more and more aggressive, throwing tantrum, and even some said exhibiting psychotic tendency.

Finally, in an attempt to set David free from his plastic prison, the family and the doctors decided to proceed with unmatched bone marrow transplant. The transplantation went well. However few months later, David became ill with diarrhea, severe vomiting, and GI bleeding. He did not make it. At the age of 12, David died. Autopsy result had shown that the cause of his death is Burkitt Lymphoma. Apparently there was undetected contaminant of Epstein-Barr Virus in the donor tissue. The virus went rampant spreading cancer all over his body. Many questions arose from this case, ethical aspect, psychological aspect, medical aspect, and many more. However, the most important thing is David had raised medical world awareness toward many immunodeficiency diseases. Subsequent researches have saved many person diagnosed with congenital immunodeficiency. At the very least, David Vetter will always be known as the “Bubble Boy” who contributed to the advance of medical science.

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