Tuesday, March 21, 2006

What is Pulmunary Alveolar Proteinosis?

Parker was diagnosed with PAP from the Lung Biopsy that he had. The toxins in his body can cause problems for Parker and in his case, it made his lungs have to work harder so that he can breathe.

In 90% of the cases of patients with PAP the disorder is genetic and occurs in 1 in 100, 000. But in the other 10% of the people with PAP it is caused by a secondary reason. Heavy smokers and miners usually account for the other 10% as they dirty their lungs.

PAP causes the function of the patient's aveoli to be comprised. This is where the oxygen and carbon dioxede exchange occurs. The problem is that a substance called surfactant (mostly fat) fills the alveoli and the body is unable to remove it. The reason for 90% of the people who have this diagnosis is because cells called macrophages that normally act as a catalist for the cleaning mechanism for the alveoli. The removal of the surfactant is hindered by some other types of cells in the immune system as the interfere with the macrophages.

This interference with the macropahges is not expected to be the problem for Parker, as he does not have much of an immune system. His problem is that the macrophages that act as a catalist for the cleaning process are lacking in numbers. The solution for Parker is to stimulate the production of these macrophages. This will be done with a medication called GM-CSF.

Parker will begin this treatment within the week. However, the difficulty with treating Parker is that infants are not normally diagnosed with PAP, and even fewer have been diagnosed that also have ADA. There is only one other known case of PAP in conjunction with ADA that Sick Kids has dealt with.

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