Monday, March 2, 2009
Some of the basics
Cushing's Syndrome can be caused by either a pituitary tumor, an adrenal tumor or an ectopic source. In Alex's case once testing proved positive for excess cortisol and the surgeon could visualize a tumor (or irregularity) on her MRI she was approved for pituitary surgery. Testing was long and involved! Her surgery in May of 2008 was not successful, but it took another round of testing to prove the excess cortisol was still an issue. In her case the pathology from the tissue removed was classified as ACTH hyperplasia. In lay terms I guess I'd say the cells in her pituitary have run amok and a producing ACTH, the chemical messenger that tells her adrenals to make more cortisol. The hyperplasia can be through out the gland and may be hard to identify, even by a skilled neurosurgeon. Alex's surgeon took out what he could see, but if he were to go again again he would plan on removing half of her pituitary and hope he got all of the tumor/hyperplasia. Some people manage fine with only half a pituitary (or less) but the chance of hypopituitaryism, permanent damage to one or more of the 7 functions performed by the pituitary, would be quite high. For this reason we are choosing BLA. The ACTH producing cells will stay, and continue sending out their signal, but there will be no adrenals to receive the faulty message. Of course no adrenal glands means she will forever have to take medications to replace the steroids/hormones her adrenals would normally produce.