Multiple Hereditary Exostoses Awareness Week: October 15-21
October 15-21st is Multiple Hereditary Exostoses Awareness Week.
Multiple Hereditary Exostoses (MHE) is an inherited disorder of bone growth, wherein people with the condition grow exostoses or boney bumps on their bones that vary in size, location, and number. Any bone can be affected, but the most commonly affected areas seem to be the long bones (legs, arms, fingers, and toes), pelvis and shoulders. It is said to affect roughly 1 in 50,000.
This condition is painful because these exostoses can irritate and compress surrounding tissues, blood vessels, arteries, tendons, nerves, and muscles. So far, the only methods of treatment are pain management and surgeries to remove exostoses that cause additional problems with mobility and surrounding tissues.
I was diagnosed with Multiple Hereditary Exostoses at 18 months old, after my parents discovered an exostosis above my knee and one on the back of my shoulder. They'd always suspected something wasn't quite right with me, as I felt way more fragile to hold than my older sister, who'd been born just thirteen months before me. My bones all seemed to shift whenever I was picked up, they could feel and hear it.
I had my first surgery when I was about seven or eight to remove the one above my left knee my parents had found, as well as one below the same knee and one from my ankle. It was during that surgery that we also discovered I had bleeding disorder (later to be diagnosed as a platelet secretion deficiency). When my doctor went to removed the exostoses, I bled from the bone. It took another two emergency surgeries: one to stop the bleeding and put in some drains, and another to remove the drains and stitch me back up before I could go home. In less than a week, my little body had undergone three surgeries.
Luckily once they determined I had some kind of unknown bleeding disorder, the surgeries got a little less dramatic. I still gave my doctor a run for his money more often than not with unforeseen additional issues and problems, but I didn't have issues with bleeding again, as they started giving me DDAVP before procedures.
I'm not sure what the official number is of how many surgeries I've actually had, because I stopped counting years ago as a child. It used to overwhelm me, and back then...I didn't want to be defined by how many surgeries I had, but I have had roughly one surgery every year/year and a half, from grade two up until the end of high school. Each time I had to go under the knife, my doctor would operate on multiple areas, hoping to remove as many of the pesky ones as we could while limiting the amount of times I'd have to go under anesthesia. This meant a single surgery was often addressing the removal of 3 or more problematic exostoses.
My most recent surgery was in November of 2019. They removed four exostoses total during this surgery. One exostosis was removed from one of my lower right rib: it had grown so large that it actually broke the rib, and was preventing it from healing properly. My doctor figured I was walking around with a broken rib for over two years, which certainly explains why I couldn't draw in a deep breath, or put on my socks without excruciating pain. They removed another two exostoses from underneath my right scapula, and they had to detach the muscles to get at them. The final exostosis was removed from the right side of my pelvic bone. That one was probably the most "dangerous" exostosis I've had, as the sharpest point of it ran right beneath my femoral artery.
Sometimes, the surgeries do cause even more problems what with nerve damage: but leaving exostoses that are pressing against tendons and nerves can be just as damaging. Most times, surgery can improve the quality of life and elevate some of the more unbearable pain symptoms.
Treatment for MHE has come a long way since I was a child, they no longer advise removing every visible exostoses: only the ones that actually cause problems.
I have two sons of my own now, ages 11 and 9, and they both inherited MHE from me. We have it on the EXT-1 gene, and I was a spontaneous case. I've written about my experiences as a parent with MHE parenting kids with MHE, and those writings can be found over at the MHE Coalition Blog if you'd like to check them out.
For Multiple Hereditary Exostoses Awareness week, I wanted to include a list of my favourite MHE Creators and Entrepreneurs:
Tina is an artist that channels her pain into creating beautiful pieces of art! Check out her MHE fish:
Lizzie is a jeweller who makes custom orders on her Etsy website, HempWerksCo.
Mandy is an artist and photographer based in Metro Detroit. She specializes in graphic design and photography (she did our wedding photos), and makes custom jewellery in her Etsy shop.
And as for me...I write books! Contemporary romance and new adult books, mainly...but I do have a few historical romances up my sleeve. You probably already knew that, though. If you didn't, all of my books are available on Amazon.