Fibrodysplasia Ossificans Progressiva

Fibrodysplasia Ossificans Progressiva; GA 3.19
Topic Started: Aug 9 2008, 11:21 AM (94 Views)
Carter	Aug 9 2008, 11:21 AM Post #1
O'Malley Forever Posts: 5,361 Group: Admin Member #1 Joined: August 2, 2008 Favorite Character George O'Malley Favorite Couple Callie & Arizona	MY FAVORITE MISTAKE Episode 319 Air date 03/22/07 Well, this week on Grey's Anatomy we did it! We were the very first TV program to do a fictional story about someone with FOP. (Usually we think we've found something totally cool and original and then we discover Doogie Houser MD did it.) What's FOP? It stands for Fibrodysplasia Ossificans Progressiva, which is why those of us in the know say "FOP". It's a rare (600 known cases in the world right now) congenital (from birth) disorder that causes muscles and connective tissue to turn to bone when injured. I had trouble visualizing and understanding this when I first heard about it. I thought -- well, someone would be stiff... I had an Aunt with Parkinson's disease (horrible in its own right); she was stiff, I figured it would be like that. But, that doesn't really fully explain it. See, everywhere I have skin and everywhere I have bone -- I have muscle. So, if my muscle started to turn into bone -- it would be like having a second skeleton over the one I already have. Only this tissue responsible for moving my bones -- the muscle -- would be gone -- because it would have turned to BONE. If the connective tissue of my joints -- tendons and ligaments - turned to bone, I would lose all of the motion of my limbs. That second skeleton would impair my ability to move to the extent that it would make it difficult for me to BREATHE. My chest would no longer go in and out. That's when I started to understand it. (People with advanced FOP breathe only with their diaphragm.) So, is it possible that I have this disease? EVERY PERSON with FOP has a deformity in their big toe. They are born with a big toe that is shorter than their next toe and the big toe joint sticks out -- as though they have a bunion. Dr. Kaplan (brilliant FOP specialist) wants you to know about the BIG TOE DEFORMITY. We asked him what the one thing he wanted Grey's fans to know about FOP. He said& There is a BIG TOE DEFORMITY. Why is that so important? FOP first manifests like bony tumors on the back, usually on a child less than 10. The "tumors" grow FAST. The doctors get scared and OPERATE. That's the worse thing they can possibly do and it causes irreparable harm. If more doctors knew about FOP and how to diagnose it -- if they knew about the BIG TOE DEFORMITY -- then they wouldn't operate and the kids would keep mobility for longer. But, why does surgery cause more bone growth? Well, it seems that FOP thinks that it should heal all injury by making bone. So, if you hurt muscle -- bone grows. If you hurt tendon, bone grows, if you hurt their bone it more bone grows. Imagine if every bruised muscle you ever had turned to bone. The heart and lungs are muscle -- are they affected? Nope. It only affects skeletal muscle. The organs aren't affected either. Is there a specific pattern to the disease progression? Typically, in the first twenty years of life a patient will have their first "flare up", painful bumpy things on the back. (Doctors call these painful bumps fibrous nodules.) At first the "flare ups" happen in the upper body. Then they move to the lower body. Wanna hear something strange? In the womb, it's how babies develop their skeletons -- neck, back shoulders and then the lower extremities. There is so much that is unknown about this rare congenital disease. But, Dr. Kaplan and his colleagues are re-searching for a cure. They've found the gene mutation that causes the condition. This amazing discovery gives them insight into not only FOP but all bone diseases. If I sound all gushy about this disease process there are three reasons. The medical geek in me is fascinated by this complicated disease process. It's like a diabolical arch-villain, cunning in its maniacal attempts for complete domination of the body. This disease comes with true heroes. Dr. Kaplan and the folks at IFOPA are fighting the good fight. They're getting the word out and are relentless in their struggle for a cure. Truth be told, their passion is infectious -- it feels good to know them. I met someone with FOP -- Jud Bogard - and I found him inspiring. But, then again, he was charming and funny and cracked me up and I'm a sucker for a great sense of humor. For more information about FOP please visit: http://www.ifopa.org/ Credit: Sara (mcobsessed)



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