Playing the Odds

I carry a rare mutation that has formed tumors in my bones.

Growing up, I was told these tumors usually occur on one side of the body and tend to form in the hands and long bones. Rarely in the pelvis, ribs, and/or skull. (Since then, there has been a lot more research on how Ollier's can present:  Natural history of Ollier disease and Maffucci syndrome: Patient survey and review of clinical literature - PMC (nih.gov) )

I only have them on my right side in the long bones of my leg, pelvis, and ribs. 

These tumors can be problematic in several ways. 

Firstly, they can cause issues with growth and malformations that, in their turn, can cause mechanical issues. 

Having a shorter leg causes back problems, for example. My right leg is over 4 cm shorter than my left, and that is after two surgeries to correct the problem (fewer than many, I am acutely aware!). A lift helps, but it doesn't fix the problem entirely.  

I also had a knocked knee, which caused pain in the joint. The surgery to correct the problem – now that was something! (I corrected it at 37, having left it quite late). 

The tumors can also weaken the bone. In general, I have to be careful to not break my bones, but that is good advice for anyone.  :)

The final, and for me, the main problem is that the tumors can become cancerous. The ones in the pelvis seem to carry more risk. 

When I was about 10, I read somewhere there was a 25% chance of degeneration by 40. 

Because my disease is so rare, I didn't know for sure that data was correct (and I have never found that article again.).

It didn't matter though, because it became my focus and a sort of unwelcome mantra: 

1 in 4 by 40, 

1 in 4 by 40, 

1 in 4 by 40.

I went ahead and lived under the sword of Damocles for over 30 years.

You see, I always knew I would be the “one”.  Each year that I drew closer to 40, I knew I was getting closer.

And I was! 

Exactly two weeks after my 40th birthday, I was due to be diagnosed with my total body MRI.  

COVID pushed it back though, so I was nearly 40 ½. (Thankfully, the tumor didn't grow too much in those extra 6 months).  

"Well, that was punctual" I declared to my husband after I found out.  "See, I always told you I had until 40, and I was right!".  

It was a stressful rush to get additional scans and then treatment, but now, all is well. Unbelievably, I ended up being successfully treated in a couple of hours. I am being literal!  A lot of worry over the years for not much muss, thankfully. I will share this story soon. 

Anyway, I am happy to have been the one, with hopes I could spare the 3.  This is not purely generosity of spirit, though that is a part of it.

I had prepared myself for years with my mantra and had shaped myself with the expectation.

I was lucky enough to have excellent medical and familial support. Thankfully, I was well-placed to cope, and will be again, should my number be redialled. 

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 Ollier disease: MedlinePlus Genetics