Friday, November 13, 2009

The story so far....

In August of 2009 I was diagnosed with Pseudomyxoma Peritonei, or PMP. Pseudomyxoma Peritonei is a rare disease (one in a million! yay, Dan!) that is characterized by the progressive accumulation of mucus-secreting tumor cells within the abdomen and pelvis. The disorder develops after a small growth or polyp located within the appendix (causes unknown, but it is not genetic) bursts through the wall of the appendix, and spreads mucus-producing tumor cells throughout the abdominal cavity. As mucinous tumor cells accumulate, the abdominal area becomes swollen and digestive and other functions become impaired as the organs get coated with this mucin, and the organs can fail. This swollen state is called "jelly belly" when it gets bad. You can end up looking like you are 7-8 months pregnant. I don't have jelly belly yet, as they found my PMP early, by accident. Which is good! Most people find it later, much later, when their bellies are huge or they have some organ problems. Here's one of the major support groups for this disease:

My tumor was not technically a "malignant" cancer, in that it won't metastasize to other parts of my body. So that's also very good! And the tumor was successfully removed during my surgery on August 31, 2009, along with my appendix and some other stuff in there that I apparently don't need. But the tumor was leaking and mucin had already spread inside my abdomen. While most of it was removed, some of the goo no doubt remains in the nooks and crannies, and on a cellular level, so it will almost certainly cause more tumors to grow, and thus create more mucin, then more tumors, and so forth, etc. So while it is good it was not malignant, it will still spread and cause problems if untreated.

So, the plan is to let my current surgery wounds heal, stay healthy and in shape, get another CT scan soon, and see if any new tumors have appeared. The doctor doesn't think any tumors will have appeared by then, as this is typically a very slowly progressing disease. In any event, they will check then.

Regardless of the appearance of any new tumors, in early 2010 I will have what is known as cytoreduction surgery, along with hyperthermic intraperitoneal chemotherapy ("HIPEC"). More on that later. The hospital stay can be pretty long if there are complications. I think a week if I am lucky. The treatment is very promising, though. I could have this surgery before any tumors reappear, or wait for new tumors to show up. My thinking is it would be better to do it while I am healthy, before any new tumors, if possible. The current doctor seems to agree.

So, the good news is they caught this very early, and I'm other than this I'm generally healthy and in good shape. I'm researching the doctors that perform this cytoreduction and HIPEC procedure on a regular basis (there aren't many in the U.S.) to get a second opinion, and to decide where to have the surgery. I have some time to think about these things, which is also good!

So there you have it! PMP!


Anonymous said...

Thanks for creating this. I certainly learned a lot about this rare condition. Glad it was caught early and I'm sending the healing vibe your way. Stay strong! - Tanja in Edina, MN

Tash said...

I've just been diagnosed with the same thing, thanks so much for all the info here, hard to find much and great to see what it's like from someone who went through it. I live in Australia and waiting to hear when the surgeon in Sydney can book me in, looks like it's gonna be a hell of a journey, can't say i'm looking forward to it but reading this has made me a bit more prepared.

thank you

Anonymous said...

What a God-send to be able to read your experience. We are searching for a doctor now as my husbands cancer is also rare. Thanks so much as we need to hear from the survivors right now! We are taking one day at a time and praying for good doctors to help us thru this.

Thank You from Kansas