Saturday, April 21, 2012

Pseudomyxoma Peritonei and Other Words Too Hard To Explain

To begin, for reference, PMP =Pseudomyxoma Peritonei. There, now at least I don't have to type it any more!

As promised, I am following up last nights blog post with a sequel version tonight. I am still lacking much emotion about the whole thing still, so it's probably a good time to write, before I get too keyed up. Also, my lack of emotion also probably stems from the fact that I don't have many details yet, and I may just be rolling things out of proportion. That is something we will find out when I finally speak with the specialist though.

So, for now, let's work from the standpoint that I don't have this condition, and my risk for developing it is extremely low. Working from there, maybe you can set aside your grave worries for my well being (which I'm sure all of you have! Just kidding!) and let's learn what the small group of people in this world who are dealing with PMP have on their plates.

PMP is an orphan disease, it's pathological origins and best modes of treatment are shrouded in a veil of medical journals and published articles that only the most highly skilled Google searchman could find. I don't profess to be a genius on the subject, but I will attempt to paraphrase some of the probably 60 published research articles on the subject that I have read over and over.  So keep in mind this is my interpretation of the facts.
An orphan disease is one that is so rare, no one really knows about it. It's sort of forgotten. Only a handful of doctors in the country are considered specialists.
Let's go from here on out in chronological order.
The appendix is connected to the cecum.  The cecum is connected to the right ascending colon.  (think of the child's song)  The beginnings of PMP start when the pathway between the appendix and cecum becomes blocked.  The appendix makes a thick gelatinous mucus (think soft jello) and fills itself up.  This process can be benign, malignant, or somewhere in between.  If malignant or somewhere in between, the bad cells mix in with the mucus.  Given enough time or a heavy handed surgeon, and the thin walls of the appendix can break.  This causes this thick mucus stuff to spread into the peritoneal cavity (that is the bag that holds most of your abdominal organs in it.)  If the mucus has any malignant or pre malignant cells in it, that is bad because it spreads those cells around the abdomen.  But even if the mucus is clean as a whistle, it is still bad because the mucus continues to multiply and grow in your abdomen until it squeezes your digestive tract shut and the result is less than ideal...
The "Pseudo" in PMP comes from the fact that it doesn't act like most other cancers by spreading through lymph nodes and blood vessels to other organs and parts of the body.  It stays in the peritoneal cavity for the most part.  But, it is much more difficult to deal with because unlike other cancers, it doesn't have a solid tumor that can be cut out or dealt with.  It is jello like, and if only one cell is left behind in your abdomen, it can start the whole process over again.  You cannot just wash it out with saline.  That would be like trying to wash jello out of a cup by filling the cup with water and sucking the water out.  The jello would just sit there.  The commonly accepted treatment for this diagnosis is cytoreductive surgery (scooping out the bad stuff) and then giving your abdominal cavity a heated chemo bath where they take chemo medicine, heat it up, and then wash out your insides.  That is how they get the bad cells out of there.  The folks who have had to have this surgery lovingly call it MOAS (Mother of All Surgeries), because it is very difficult to go through.  I feel very much for anyone who has dealt with this disease because it is apparently not a breeze to deal with.

Now that we have all of that mumbo jumbo out of the way, I will state again for the sake of anyone's worries, we are not sure that I even have this.  I am waiting on an actual conversation with the specialist.  His voicemail only said that I was at risk for the disease because of what happened during surgery with my appendix.  Many times doctors will suggest a simple wait and see attitude.  The disease can be slow growing and could take 10, 20, or 40 years to show up.  No one really knows (remember orphan disease).      Keep checking back here for any updates, and as soon as we have more info I will post an update.
Thank you all for your concern and for reading these blog updates.

Feel free to comment, and if I messed any of the information up and you know more about it, please comment and correct me.
-Ryan


6 comments:

Anonymous said...

Excellent explanation of PMP (I have it). There is a great support group on Facebook - look for pmp appendix cancer and a great site here - http://www.pseudomyxomasurvivor.co.uk

Kristi said...

Ryan, I am so sorry that you and your family are having to go through all this!!! But now that you are an expert in PMP, maybe you should be a doctor :) Please keep us updated and even though I am far away, please let me know if there is ANYTHING I can do for you guys!

Daniela said...

praying that is NOT the case!

Harold said...

Keeping you in our prayers, and you are on our prayer list at church. Thanks for your updating. Love you.

Jennifer said...

Great post. Glad you are contacting the specialists. Having caught this early you have a great chance to beat it. My husband's appendix ruptured (like a balloon popping) and spread the PMP around the abdomen. His surgeon told us that he knew of NO ONE in the WHOLE state of Pennsylvania that could help us (back in 2004, didn't find out about Bartlett until later). My husband had the MOAS with Dr Sardi in 2005. He has been PMP-free for seven years now. Best of luck to you and your family!

Richard Sams said...

I do not know anything about PMP but I do know your writing and vocabulary are EXCEPTIONALLY improved. (I think it must be the beard making you wise.) I can relate to learning medical words you never really wanted to know.

You have come to the time of your life when you would pay extra for just a few days of "boring" but remember, it is an adventure. You have faced many things, good and bad, in your life. The good have made you happy and the bad have made you strong. Use that strength now to get well. I've always suspected that a car wash would do you good. I just never thought you might consider washing the INSIDE of the car. Hmmmm....

Also remember, no matter what has happened, you are special to God. (I know because I was there the day He spoke to you directly.) He still has you in His hand. We will be praying for you.

If there is ANYTHING we can do just say the word.

"Richie"