Saturday, January 1, 2011

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THE BOY IN THE PLASTIC BUBBLE


"The social instincts of men is not based on love society, but the fear of loneliness. "

Arthur Schopenhauer


we bring a topic that Today was not addressed in this blog. This is a rare group of diseases specific care subsidiaries to try to prevent serious complications to those who have, fortunately, the constant technological advances that have achieved perfect bone marrow transplants, along with other more complex techniques of gene therapy, have come to represent a giant step in your treatment.

"The Boy in the Plastic Bubble" ( Randal Kleiser, 1976) is a telefilm based on the real life of two patients, David Vetter and Ted De Vita who suffered severe immune system disorders.

THE CASE OF DAVID VETTER.

David Vetter (1971 - 1984)

David was born with a rare genetic disorder called Syndrome Severe Combined Immunodeficiency (SCID ). Affects 1 in every 100000 children. Is due to an autosomal recessive disorder that will ultimately affect the functioning of T lymphocytes B and because of the deficit adenosine deaminase (ADA ), a crucial enzyme in the metabolism of purines .

At first these children are normal, while in their blood have sufficient maternally transferred antibodies duante pregnancy. But precisely because of the involvement in the development of their own immune system in the first year of life often have many serious infections that do not respond to treatment: pneumonia, sepsis, meningitis, hepatitis, dermatitis, gastroenteritis ..., and may even cause death.

From the medical point of view, the case of David Vetter has some peculiarities worthy of reflection from the point of view Bioethics. This child was the third child of the marriage between David Joseph Vetter Jr and Carol Ann Vetter . The second of their offspring, called David Joseph Vetter III , died at 7 months old victim also d and a SCID. Vetter had the older daughter, Katherine , completely healthy and normal. His deep religious convictions (ultraconservative Catholic) led them to conceive a baby, despite knowing he would have a 50% chance of being born with other SCID.

Incredibly, several doctors at Baylor Medical Center of Texas (U.S. ), Drs John Montgomery, Mary Ann South and Raphael Wilson , they proposed to Vetter, in the event that affected the small born, was raised in a large sterile plastic bubble to find a cure for extreme immune deficiency. Apparently, this disturbing research project had substantial federal aid. The child was born by caesarean and went straight to a sterile plastic bubble that would become his home forever.



Everything went into the bubble (air, food, clothes ...) should be previously sterilized. The child was manipulated through gloves, and as it grew, new modules were being added special plastic ...

At first, doctors thought of Katherine as the ideal donor to attempt a bone marrow transplant able to save the life of his sick brother. Soon realized that despite the direct relationship between the two support did not guarantee success.

David continued to grow in your bubble, being aware of its uniqueness. Could interact with parents, relatives, friends, but always shy away from direct contact with them. His case was becoming as popular as uncomfortable. Until the very NASA stepped in to design a special outfit for the boy could wander away from their protective environment. David used this costume in very rare cases, it was a huge fear about the possibility of contamination.

In 1984, the physician decided to try again bone marrow transplantation. Katherine remained the first choice as a donor. To prevent rejection caused by the incompatibility, the doctors decided this time to completely destroy David's bone marrow by radiation and chemotherapy, and later replaced with cells from the healthy sister.

Just remember here that for these transplants require a donor succeed HLA identical. Only 25-30% of the time these patients have a sibling with the HLA compliance these strict conditions. Nor should we forget that the bone marrow transplant related donor is associated with mortality in the first 100 days of 20%, fatal percentage rises to 30-40% when it is shared between donor and recipient relationship.
At first, the innovative treatment appeared successful, but David fell ill a few months later, debuting with fever , diarrhea and vomiting . His condition gradually worsened to the point of even having to leave their protective bubble. It appears that the transplanted marrow cells that contained traces of Epstein-Barr virus and the boy would eventually develop a Burkitt lymphoma which led him to the grave.

Regardless of the moral and personal drama, some experts say the treatment of disease of David Vetter, irresponsibly bred by their parents and supported by unethical medicine, involved in its day cost about $ 1.3 million ...

For those interested in knowing:




IF TED DE VITA.

Ted Vita (1962 - 1980)

We commented at the beginning that writers of "The boy plastic bubble "had been inspired by the life experiences of two boys affected by severe immunodeficiency. The second question is Vita Ted, son of Vince de Vita , a renowned oncologist and researcher at the National Institute of healths ( NIH ). Ted suffered a serious aplastic anemia that forced him and living in hospital in a clean room. He stayed and just over 8 years, admitted in a special room laminar flow at the 10 th floor NIH.

aplastic anemia a reduction of all blood cells (erythrocytes, leukocytes and platelets) and in many cases suspected to be an autoimmune disease.


Ted also had a sister in this case lower. Her name is Elizabeth Vita-Raeburn, a journalist specializing in science, which has left us "The Empty Room" their particular witness to the illness of his brother.


Like David Vetter, Ted also had a special diving suit that allowed him to leave his closure. The psychological effects caused by the permanent isolation of this patient was higher than in the case of David, because Ted did reach adolescence. He died in 1980 as a result of hemochromatosis caused by excessive blood transfusions needed throughout life. Today, with proper treatment, 80% of cases of aplastic anemia usually resolved satisfactorily.

FILM ...

data Taking the life of both patients, screenwriter Douglas Day Stewart was commissioned to develop a unique script that presents the history of Lubitch Tod, a young victim of a severe congenital immunodeficiency, whose role fell to a then-twentysomething John Travolta , months before his breakthrough triumph in the screens thank "Saturday Night Fever" ( John Badham, 1977) and "Grease" ( Randal Kleiser, 1978).

The patient's childhood begins in a kind of sterile incubator, which is gradually incorporating modules are sterile, in the same way that they would go on adding the different phases of a space station.

This
initial stage probably was inspired by the experiences of David Vetter and his family. In this case, parents are John Tod ( Robert Reed) and Mickey Lubitch ( Diana Hyland ). The doctor responsible for your care is Dr. Gunther (played by veteran and versatile Ralf Bellamy).

Ralf Bellamy is Dr. Gunther

With expertise, screenwriter and director introduced a simple metaphor in the story, a tender and emotional element in the patient's life. It is a structure consisting of several tubes interconnected lives a laboratory mouse that comes with its isolation in the young patient. Paradox of existence as the life of David Vetter went the way of a helpless animal experimentation.

As the child grows, the old sterile plastic bubble turns into a special room equipped with laminar flow positive and several areas of trade, from which the teen can even go Lubitch class through a closed circuit television. Of course, love can not be absent from this history and is the blonde neighbor Giggs Gina (Glynnis O'Connor ) that awakens the deep affection of our misunderstood protagonist.

A Young Glynnis O'Connor

Later, Tod is interned in a hospital sterile where all patients were immunosuppressed isolated by different diseases (leukemia, for example) or by treatment (chemotherapy) ... They share the premises with Roy Slater ( John Friedrich ).

cinephilia ...

NASA experts involved in costume design "space - especially" (50000 dollars) to David Vetter could leave his plastic bubble. In this film involved astronaut Buzz Aldrin , who visits his retirement Tod Lubitch in hospital ... It was the second man to walk on the lunar surface. After suffering a slump in the early 70's came to suffer from with the drink, which was restored completely. Aldrin, like all his colleagues, had to undergo extreme training conditions in which the insulation in confined spaces, as in the case of boys of bubbles, was particularly hard to bear ...

In this film there is a blonde character named Tom Shuster ( Kelly Ward), another teenager Tod rivals for the affections of Gina. Tom lost a bet with Tod and establishing a $ 10 bill in the sterile environment as payment for his defeat. This fact shows that the film would be impossible in fact, considering the microbial load is home to huge money flow ... But, continuing with Kelly Ward, just two years later, would play Putzie , a member of the T-Birds , the gang of rockers who captained slicked Danny Zuko (John Travolta) in "Grease "...

This movie is public domain and may be entirely at the following link:




ANOTHER BUBBLE BOY ...

"The Bubble Boy" ( Blair Hayes, 2001) is remake in key comedy shows us the vicissitudes of Jimmy Livingston (Jake Gyllenhaal ), a boy which has no immune system and is forced to live in his room in a plastic bubble. A film that will surely bring more elements to the fun for reflection ...

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