This first week of May is National Osteogenesis Imperfecta Week and May 6 is Wishbone Day, a day of international awareness for Osteogenesis Imperfecta. And, as it happens, your venerable host ALSO has OI. So guess what we're talking about today!
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TRANSCRIPT OF “ME OH MY OI”
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MALE VO [00:03]
This is Bad Attitudes.
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Hello friends and strangers! Welcome to another episode of Bad Attitudes: An Uninspiring Podcast about Disability. I’m your host, Laura.
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As always, I want to remind you that disability is not a monolith. My experience as a disabled person is going to be different from the experiences of other disabled people. I am one voice for the disabled community but I am not the only voice.
Today, we’re talking about osteogenesis imperfecta, which if you’re just joining us, is what I have. May 6 is Wishbone Day, a day to raise awareness of OI, so the timing could hardly be better. OI is also known as brittle bone disease because the hallmark of OI is the fact that people with OI break bones frequently and often without known cause. My most recent break was in 2018 and I still have no idea how it happened.
People with OI can have hundreds of fractures in their lifetime. My family stopped counting my breaks around 60, which occurred when I was still in the single digits. Most breaks occur between infancy and puberty, with a drop off after puberty, but breaks can increase in frequency later in life. I had many, MANY fractures growing up but was very lucky to have no breaks between 2000 and 2018.
OI is considered a rare disease, affecting between 25,000 and 50,000 people in the US. When I was a kid, nobody — including a lot of medical professionals — had even heard of OI. Thanks to popular media, it’s starting to become more recognized. On “Grey’s Anatomy”, Jackson and April’s first child had and subsequently died as a result of OI. OI is a focal point of the Jodi Picoult book, “Handle With Care.” In the movie “Unbreakable,” Samuel L. Jackson’s character has OI. Atticus Schafer, one of the stars of the sitcom “The Middle,” has OI. Sammi Haney of Netflix’s “Raising Dion” has OI. Michael J. Anderson, known as the Man from Another Place on “Twin Peaks,” also has OI. I’m just saying, for a rare disease, it’s pretty popular.
While OI is known to most affect the skeletal structure, there are other characteristics, including: short stature (got that, check); spinal curvature (check); low bone density (check); loose joints; ligament laxity; muscle weakness; distinctive skull features; blue sclerae (whites of the eyes, check); hearing loss that may begin in the early 20s and by middle age is present in 50 percent of people with OI; brittle teeth (checkity check, more on that in a minute); respiratory problems; vision problems; skin hyperlaxity; easy bruising; cardiac issues; fatigue (um, doesn’t everybody?); and skin, blood vessels, and internal organs that may be fragile.
One of the things I’m most self-conscious about regarding my OI is my teeth. I have dentinogenesis imperfecta — brittle teeth — meaning my teeth don’t have a lot of enamel, making them look gray. The first time I was aware of my teeth looking different was in second grade, when a boy pointed out that my teeth were black. Enamel is what makes teeth look white and is also what protects them against the decay that leads to cavities. I had NO cavities until I was about 21. At that point, apparently, my teeth went into revolt. All things considered, my teeth are in pretty good shape, but from my twenties onward, cavities and broken teeth became the norm. Another thing about having teeth with no enamel is that braces have a hard time sticking. During the time I had braces, brackets would just randomly pop off for no reason. But when it came time to remove them, that process went hella smooth.
I also have scoliosis, or curvature of the spine. I had two spinal fusion surgeries, first at 11 and then at 14, to put steel rods in my spine to prevent my spine from curving further. I also had multiple rodding surgeries in which steel rods were inserted into the bones of my legs. The primary purpose of these rods is to keep the bones in place in the event of a fracture. Without these rods, when I suffered a break, my bones might have gone out of alignment, which would cause even more problems. Although when I was growing up, I still had to wear casts after a break, following my most recent break in 2018, the doctor didn’t see much point because the rod acted as an internal cast.
Starting in the 1970s, a list was kept of the different types of OI. In the Dark Ages when I was born (AKA 1981) there were four known types of OI. Today, there are 15. Not all people who have OI share the same characteristics, even those who have the same type. OI is an incredibly variable disease; I didn’t even realize HOW variable until preparing this episode. Truthfully, beyond my own experience, I know very little about OI. I don’t even know which type of OI I have. Based on the available information, I can probably hazard a guess.
I’ll tell you about the most common types of OI. If I tried going over all 15, we’d be here for a WHILE. But just know that a lot of the characteristics carry across multiple types of OI and severity can vary from person to person.
Type I is the most common and least severe type of OI. Individuals with Type I may only experience a handful of fractures in their lifetime or as many as a few dozen. These people are generally of average height and have little bone deformity.
Type II is the most severe type of OI and is usually lethal shortly after birth. This is the Type it was expected I had at birth. Mostly I think it’s just because not much was known about OI at the time and the doctors were guessing. My parents were told to expect me not to live past the age of 2 and to be no bigger than a six-month-old.
People with Type III OI are born with fractures or with fractures that healed in utero. Common characteristics include short stature, spinal curvature, long bone deformities, and a barrel-shaped ribcage. Some people may experience severe and even fatal respiratory issues in infancy and childhood. The severity of Type III can vary.
Type IV is considered the moderate type of OI and is similar to Type I or Type III. People with this type of OI may be shorter than their family members, experience fractures that decrease in frequency after puberty, and have mild to moderate bone deformity.
Type V is moderate and similar to Type IV. A defining characteristic of this type is that a type of callus may form at fracture or surgical sites, and this can limit joint mobility. I don’t know if this happens in the average person, but I do have one fracture site near my elbow that developed a knot of bone, and over the years, I’ve lost some of the range of motion in that elbow.
Type VI is another moderate form of OI and looks similar to Type IV. The distinguishing characteristic here is a mineralization defect visible in biopsied bone. Type VI is actually incredibly rare.
Based on the information here, I would guess that I have a less severe variation of Type III. I was born with healed fractures. I am short. I already mentioned my spinal curvature and I have a barrel-shaped ribcage, which, not gonna lie, makes finding a good bra difficult. I do not have severe respiratory issues, although I do have a lower lung capacity. I’ve had my breathing tested multiple times since respiratory issues can arise with OI and have good respiratory function. I have some minor long bone deformity, mostly due to the breaking and healing of fractures before I was born.
There is no cure for OI. It is caused by genetic mutation, or is inherited from a parent with a genetic mutation. There are over 1,000 different mutations affecting a dozen genes associated with OI. Yet, more than 90 percent of people with OI have a mutation in one of the genes associated with producing type 1 collagen. These mutations either reduce the amount of collagen or reduce the quality. When people hear that OI affects the bones, the mind generally makes the incorrect leap to assuming the problem is with calcium. And while people with OI often take calcium supplements (I don’t), if you’ve ever seen calcium in its mineral form, it is VERY brittle. Collagen is what gives bones a little “spring,” which is why people who don’t have OI can do things like gymnastics and not snap their tibia the first time they land a back handspring. Obviously, people who don’t have OI can break their bones, but collagen keeps it from happening quite so easily.
So that’s your surface-level introduction to OI. If you’d like to learn more, I’d highly recommend checking out the OI Foundation’s website, oif.org, which is where I got all my facts and figures for this episode. Don’t forget, this week is National OI Awareness Week, and May 6 is Wishbone Day, an international day of OI awareness.
If you’re interested in learning more about disability and other marginalized groups, I’d like to suggest the podcast out_cast. Out_cast is a podcast about being multiply marginalized, specifically about queerness, disability, neurodivergence, mental health, and sometimes other stuff. That’s out underscore cast, available wherever you listen to podcasts. Let me know if you listen and tell me what you think.
Thanks for listening and I’ll talk to you in the next one.
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