Miss Diagnosis: How Rare Is It?

Friday, April 6, 2012

How Rare Is It?

We have reached a point where it is getting nearly impossible to find doctors who can help; the rarity of the disease was bad enough but we have moved beyond that now, to a land where doctors haven't been. We are now trail blazers. To help you understand, here are some statistics (believable or not), as they relate to Alex...

One in a million people are diagnosed with Cushing's Disease in the U.S. every year
Only 10% of those diagnosed are children (Alex was 14)
Possibly 10% of those are cyclic (Alex is)
Most (60-90%) are cured with one pituitary surgery (her tumor couldn't be completely removed)
Nearly all (95%) are cured with bilateral adrenalectomy (hers was not a cure, only a remission)

It's like being attacked by a bear and struck by lighting on the same day. Alex isn't one on a million, she's one in a billion, or maybe that's trillion? Math was never my thing.

No comments:

Post a Comment

Transphenoidal Pituitary Surgery- From the UCSF web site

What happens during surgery?
The surgical removal of the pituitary adenoma is performed by a technique called a transsphenoidal operation. The word 'transsphenoidal' describes the path the surgeon follows to reach the pituitary gland. The word comes from 'trans', meaning 'to cross or pass through', and 'sphenoid', the name of the cavity (the sphenoid sinus) that is passed through to reach the pituitary gland. Newer techniques and improvements in technology now allow removal of these tumors without the need for an incision under the lip or within the nostril. The surgeon begins the approach by entering through the nostril on one side. The surgeon uses a brilliant fiber optic light to illuminate the internal anatomy, a microscope to magnify the area of surgery to 12 times the actual size, and very tiny microsurgical instruments especially designed for this particular operation. The surgeon guides the surgical instrument into the nasal cavity and an opening is made in the sphenoid bone. The sphenoid sinus is entered, and then an opening is made in the wall of the sella turcica to expose the pituitary gland. The adenoma can be readily distinguished from the normal pituitary gland tissue and is removed without removing the normal gland. After the surgeon removes all the tumor tissue, the small cavity that is left is treated with alcohol to destroy any tumor cells that may remain. This cavity is then sealed, sometimes with a piece of fat that the surgeon removes from the patient's abdomen. The surgeon then applies a biological 'glue' that helps seal the pituitary area from the nose and promotes natural healing. No nasal packs are used with this approach.